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Transposition of the great arteries (TGAs) is a condition with ventriculo-arterial discordance where the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. Broadly speaking, two forms exist: (1) a dextro type (referred as D-TGA) and (2) a levo type (referred to as L-TGA). Traditionally, diagnosis is made by echocardiography. Cardiac magnetic resonance imaging (CMR), cardiac computed tomography (CCT), and heart catheterization and angiography are supplemental tools in further characterization of the anatomy. The frequency of routine imaging testing, including echocardiography, CMR, and CCT, depends on severity of heart failure, cyanosis, valvular disease, arrhythmia, pulmonary hypertension, and end-organ damage. The aim of this review is to explore important imaging findings for different types of D-TGA: (1) uncorrected D-TGA, (2) D-TGA after the Mustard/Senning atrial switch surgery; (3) D-TGA after the arterial switch operation; and (4) D-TGA after the Rastelli repair.

Objectives The aim of this study was to enumerate and categorize quality metrics relevant to the pediatric/congenital cardiac catheterization laboratory (PCCL). Background Diagnostic and interventional catheterization procedures are an increasingly important part of the care of young patients with cardiac disease. Measurement of the performance of PCCL programs in a stringent and consistent fashion is a crucial step toward improving outcomes. To the best of our knowledge, a systematic evaluation of current quality metrics in PCCL has not been performed previously. Methods Potential metrics were evaluated by: 1) a systematic review of peer-reviewed research; 2) a review of metrics from organizations interested in quality improvement, patient safety, and/or PCCL programs; and 3) a survey of U.S. PCCL cardiologists. Collected metrics were grouped on 2 dimensions: 1) Institute of Medicine domains; and 2) the Donabedian structure/process/outcome framework. Survey responses were dichotomized between favorable and unfavorable responses and then compared within and between categories. Results In the systematic review, 6 metrics were identified (from 9 publications), all focused on safety either as an outcome (adverse events [AEs], mortality, and failure to rescue along with radiation exposure) or as a structure (procedure volume or operator experience). Four organizations measure quality metrics of PCCL programs, of which only 1 publicly reports data. For the survey, 229 cardiologists from 118 hospital programs responded (66% of individuals and 72% of hospital programs). The highest favorable ratings were for safety metrics (p Conclusions There is a limited number of PCCL quality metrics, primarily focused on safety. Confidence in current risk adjustment methodology is low. The knowledge gaps identified should guide future research in the development of new quality metrics.

Pulmonary hypertension (PH) due to interstitial lung disease (ILD; PH-ILD) can complicate a multitude of ILDs, including idiopathic pulmonary fibrosis, chronic hypersensitivity pneumonitis, and nonspecific interstitial pneumonia. Development of PH-ILD is associated with increased need for supplemental oxygen, reduced mobility, and decreased survival. A high index of suspicion is required to make the diagnosis, given the substantial overlap in symptoms with those of ILD without PH. Severely reduced diffusing capacity or 6-min walk test distance, prominent exertional desaturation, and impaired heart rate recovery after exercise are all suggestive of the development of PH-ILD. Traditional transthoracic echocardiography is the most commonly used screening test for PH-ILD, but it lacks sensitivity and specificity. Newer echocardiographic tools involving 3-dimensional assessment of the right ventricle may have a role in both prognosis and the monitoring of patients with PH-ILD. Right-sided heart catheterization remains the gold standard for confirming a diagnosis of PH-ILD. Although there is little debate about the use of supplemental oxygen and diuretic therapy in the treatment of PH-ILD, treatment with pulmonary vasodilator therapy remains controversial. Although several studies have been terminated prematurely for harm, the recently completed INCREASE trial of inhaled treprostinil appears to validate the concept of treating PH-ILD with pulmonary vasodilators and, we hope, will serve as a foundation from which future studies can be developed.

A physiological approach to the analysis of hemodynamic data in pulmonary hypertension (PH) has the advantage of reducing the large number (well over 100) of potential causal illnesses into four simple mechanisms. A fifth condition is composed of mixtures of the four basic mechanisms. This approach was beautifully described by Paul Wood, the great cardiologist whose name is given to the units of pulmonary vascular resistance (PVR), Wood units. This approach uses well understood physiological contributions to pulmonary vascular pressure. It is powerful, the major uncertainty being in determination of the magnitude of each mechanism in patients that have mixed PH of several causes. It also makes sense of the occasionally awkward clustering of conditions in the clinical classification of the World Symposium, which omits pulmonary vasoconstriction, hyperkinetic states, and the highly prevalent condition of "mixed" PH. This method of analysis is described and demonstrated, much as Wood did in his writings. The method is useful in the office, the ICU, and in consultation. A basic message from this approach is that correct assessment requires measurement of each of the three major inputs, pulmonary arterial pressure (Ppa), pulmonary artery wedge pressure (Pwedge) and cardiac output (CO). Some cases also need left ventricular end diastolic pressure (LVEDP). Other data contributing to analysis will be discussed in each condition. A key to avoiding mistakes is to always remember that PH is simply an elevation in pressure and is not inherently diagnostic of cause.

Background Patients with COPD who experience pulmonary hypertension (PH) have worse mortality than those with COPD alone. Predictors of poor outcomes in COPD-PH are not well-described. Diffusing capacity of the lung (D lco ) assesses the integrity of the alveolar-capillary interface and thus may be a useful prognostic tool among those with COPD-PH. Research Question Using a single center registry, we sought to evaluate D lco as a predictor of mortality in a cohort of patients with COPD-PH. Study Design and Methods This retrospective cohort study analyzed 71 COPD-PH patients from the Johns Hopkins Pulmonary Hypertension Registry with right-sided heart catheterization (RHC)-proven PH and pulmonary function testing data within one year of diagnostic RHC. Transplant-free survival was calculated from index RHC. Adjusted transplant-free survival was modelled using Cox proportional hazard methods; age, pulmonary vascular resistance, FEV1, oxygen use, and N-terminal pro-brain natriuretic peptide were included as covariates. Results Overall unadjusted transplant-free 1-, 3-, and 5-year survivals were 87%, 60%, and 51%, respectively. Survival was associated with reduced D lco across the observed range of pulmonary artery pressures and pulmonary vascular resistance. Severe D lco impairment was associated with poorer survival (log-rank χ2 13.07) (P lco , mortality rates increased by 4% (hazard ratio, 1.04; 95% CI, 1.01-1.07). Interpretation Among patients with COPD-PH, severe gas transfer impairment is associated with higher mortality, even with adjustment for airflow obstruction and hemodynamics, which suggests that D lco may be a useful prognostic marker in this population. Future studies are needed to further investigate the association between D lco and morbidity and to determine the utility of D lco as a biomarker for disease risk and severity in COPD-PH.

Ischemic heart disease secondary to coronary vascular dysfunction causes angina and impairs quality of life and prognosis. About one-half of patients with symptoms and signs of ischemia turn out not to have obstructive coronary artery disease, and coronary vascular dysfunction may be relevant. Adjunctive tests of coronary vasomotion include guidewire-based techniques with adenosine and reactivity testing, typically by intracoronary infusion of acetylcholine. The CorMicA (Coronary Microvascular Angina) trial provided evidence that routine management guided by an interventional diagnostic procedure and stratified therapy improves angina and quality of life in patients with angina but no obstructive coronary artery disease. In this paper, the COVADIS study group provide a comprehensive review of why, how, and when coronary vascular dysfunction should be assessed invasively. They discuss the rationale through a shared understanding of vascular pathophysiology and clinical evidence. They propose a consensus approach to how an interventional diagnostic procedure is performed with focus on practical aspects. Finally, the authors discuss the clinical scenarios in patients with stable and acute coronary syndromes in which measurement of coronary vascular function may be helpful for patient care.

A 56-year-old woman with mixed connective tissue disease, who was on maintenance immunosuppression, developed asymptomatic left ventricular…, A 56-year-old woman with mixed connective tissue disease, who was on maintenance immunosuppression, developed asymptomatic left ventricular dysfunction, ventricular arrhythmia, and high troponin I. Heart catheterization showed normal coronaries and biopsy-proven, virus-negative lymphocytic myocarditis. A biopsy-guided immunosuppression upgrade effectively treated autoimmune myocarditis, which resulted in ventricular function recovery, resolution of arrhythmia, and of troponin release. (Level of Difficulty: Advanced.), Graphical abstract

The etiologic diagnosis of pulmonary hypertension (PH) may be very challenging. Right-heart catheterization (RHC) in isolation cannot classify a precapillary PH patient into group 1, 3, 4, or 5. Moreover, RHC may be not sufficient for reaching a definitive differential diagnosis of precapillary or postcapillary PH if hemodynamic data are not integrated in clinical context and combined with information gleaned from noninvasive imaging. Therefore, only the integration of risk factors, clinical evaluation, invasive and noninvasive tests allows the physician to distinguish between different forms of PH.

Right heart catheterization (RHC) is the gold standard for the diagnosis and classification of pulmonary hypertension. Significant expertise is required for safely performing a full RHC and for the acquisition of reliable and reproducible information. Physicians performing an RHC should have adequate training not only in vascular access, catheter insertion, and manipulation but also in the interpretation of waveforms, potential pitfalls, and strict quality control. This article describes the essential technical aspects of RHC as applied to the pulmonary circulation, the potential pitfalls, and areas of major controversy.

Purpose Cardiac allograft vasculopathy (CAV) is common in patients who have undergone orthotopic heart transplant (OHT) and is associated with significant morbidity and mortality. Non-invasive methods for identifying CAV are an appealing alternative to standard clinical practice of routine invasive angiography. We explored the association of CAV with incidental coronary artery calcium (CAC) from non-ECG gated, clinically indicated chest CT scans. Methods This is a single-center, retrospective, observational study of OHT patients >1 year from transplant who underwent left heart catheterization between 2015 and 2019 and had a clinically indicated, non-ECG gated chest CT obtained for a variety of indications within 6 months of the catheterization. CAC was graded by a blinded reviewer in a semi-quantitative manner based on extent and distribution across the coronary tree. Angiographically significant CAV was obtained from left heart catheterizations results obtained per standard institutional protocol. Results 73 patients were included, 56 (77%) were male, mean age was 60±12 years and median time from heart transplant was 4 years [IQR 2, 8]. 34 patients (47%) were found to have CAC, of which 7 (21%) were found to have moderate or severe CAC. 38 patients (52%) were found to have CAV on angiography. In the CAC group, 25 were found to have CAV (74%). Of those with moderate to severe CAC, 7 (100%) were found to have CAV. In the no CAC group, 13 (33%) were found to have CAV. The presence of CAC had a sensitivity, specificity, positive predictive value and negative predictive value of 66%, 74%, 73%, and 67% respectively for the diagnosis of CAV. Conclusion The absence of CAC on non-ECG gated CT demonstrated a moderate ability to rule out CAV, while moderate to severe CAC was strongly associated with CAV. As chest CT scans are common in this population, informing clinical decision making with this “free” information is potentially beneficial.

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Dilated cardiomyopathy is characterized by structural defects leading to impaired ventricular function. The etiology is often elusive. Viruses are some of the most common causes world-wide (1). Here we present a male with non-ischemic cardiomyopathy and heart failure due to Coxsackie B myocarditis. CASE PRESENTATION: A 36 year old male with history of heart failure with reduced ejection fraction (35%), non-ischemic cardiomyopathy, and ventricular tachycardia presented for syncope. He was found to be in cardiogenic shock. Work-up revealed an elevated troponin and NT-proBNP, 0.064 and 5570, acute kidney injury, creatinine 3.28, and congestive hepatopathy. Prior cardiac work-up consisted of a left and right heart catheterization which showed no atherosclerotic disease and elevated filling pressures (Pulmonary Capillary Wedge Pressure 16 and Pulmonary Artery Mean Pressure 33). Cardiac MRI showed global dilation of the cardiac chambers, global hypokinesis, and late gadolinium enhancement of the base and septum. This was suggestive of dilated cardiomyopathy and prior myocarditis. Echocardiogram revealed a severe decline in ejection fraction, < 15%, and EKG showed a widened QRS complex with left bundle branch block. Serologic tests were positive for Coxsackie B antibodies. Inotropic support was initiated. Bridge therapy and heart transplant were indicated. However, due to his uninsured status and COVID-19 restrictions, he was unable to be transferred to a heart transplant center. Cardiac Resynchronization Therapy was pursued instead. He was clinically stabilized, initiated on GDMT, and was discharged with close follow-up. Even though serologic antibodies lack specificity, given the history, cardiac MRI findings, and diagnosis of NICM, his clinical picture was presumed to be due to Coxsackie B. DISCUSSION: Myocarditis is an inflammatory condition of the myocardium. Viral is the most common cause in Western nations but Rheumatic and Chagas remain prevalent in the developing world. Diagnosis is confirmed with endomyocardial biopsy. This makes it difficult to ascertain the true incidence but there are approximately 1.5 million cases every year. COVID-19 has been frequently associated with myocardial injury and clinical myocarditis. In most cases, tissue diagnosis is lacking. Viral myocarditis can lead to dilated cardiomyopathy through a variety of mechanisms. It is hypothesized to be the result of direct toxicity via receptor mediated entry or an autoimmune response against viral fragments that are not cleared (2). CONCLUSIONS: Unfortunately, this case highlights the disparities of our healthcare system and how that can directly impact patient care. Viral myocarditis remains a difficult clinical scenario. Treatment is largely supportive but the sequelae of disease can be severe. Antivirals and interferon therapy are promising but further research is needed to explore more pharmacologic options (3). REFERENCE #1: Felker GM, Thompson RE, Hare JM, et al. Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med 2000;342:1077. REFERENCE #2: Kang M, An J. Viral Myocarditis. [Updated 2021 May 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing;2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459259/ REFERENCE #3: Coletta AP, Clark AL, Cleland JG. Clinical trials update from the Heart Failure Society of America and the American Heart Association meetings in 2008: SADHART-CHF, COMPARE, MOMENTUM, thyroid hormone analogue study, HF-ACTION, I-PRESERVE, beta-interferon study, BACH, and ATHENA. Eur J Heart Fail 2009;11:214. DISCLOSURES: No relevant relationships by Adhish Singh, source=Web Response No relevant relationships by Michael Vu, source=Web Response

Background Pulmonary hypertension (PH) is diagnosed and classified through right-sided heart catheterization, with a number of hemodynamic markers used to help guide treatment decisions. These markers may not reflect the complex remodeling of the right ventricle or the interplay between ventricles and struggle to predict treatment response. This study investigates the use of a novel marker: the ratio of left ventricular end-diastolic pressure to right ventricular end-diastolic pressure (LVEDP/RVEDP) in predicting treatment outcomes in a cohort of patients with PH. Methods We performed a retrospective analysis of patients with PH at INOVA Fairfax Hospital's advanced lung disease program with simultaneous left-sided and right-sided heart catheterization. The primary end point was the time to clinical improvement, defined by an improvement in distance walked on the 6-min walk test (6MWT) of > 35 m in a year without interceding hospitalization for worsening PH or the need for additional PH therapy. Results A total of 51 patients were included in the final analysis, 21 of whom (41.2%) had a salutary treatment effect with a mean improvement in the 6MWT of 75 m. Treatment responders were more likely to have a lower LVEDP/RVEDP ratio (1.08 vs 1.62; P = .051). This association persisted in the final multivariate regression model after adjustment for age and sex and controlling for severity of PH (OR, 0.17; 95% CI, 0.03-0.65; P = .024). Conclusions The LVEDP/RVEDP ratio is a novel marker for therapeutic response in patients with PH treated with pulmonary vasodilator medications and may offer robust predictive value independent of existing markers of disease severity.

This study evaluated the feasibility of noninvasive intracardiac pressure estimation using subharmonic signals from ultrasound contrast agents in humans. This IRB approved proof-of-concept study included 15 consenting patients scheduled for left and right heart catheterization. During the catheterization procedure, Definity (Lantheus Medical Imaging, N Billerica, MA) was infused intravenously at 4–10 ml/min. Ultrasound scanning was performed with a Sonix RP (BK Ultrasound, Richmond, Canada) using pulse inversion, three incident acoustic output levels, and 2.5 MHz transmit frequency. Radiofrequency data were processed and subharmonic amplitudes were compared with the pressure catheter data. Correlation coefficient between subharmonic signals and pressure catheter data ranged from −0.3 to −0.9. For acquisitions with optimum acoustic output, pressure errors between the subharmonic technique and catheter were as low as 2.6 mmHg. However, automatically determining optimum acoustic output during scanning for each patient remains to be addressed before clinical applicability can be decided.

Background Patients with congenital heart disease and native or palliated conditions are at risk to develop pulmonary hypertension (PH) in later life. Screening for PH is currently performed by regular echocardiographic follow-up, which appears to be difficult in several congenital conditions. This study evaluated the screening for PH in congenital heart disease by cardiopulmonary exercise testing (CPET). Methods We analyzed our database including all patients with congenital heart disease referred for CPET in our institution from June 2001 to September 2013 and identified 683 patients who had an accompanied heart catheterization less than 6 month after CPET. Those 130 patients with proven PH were compared with the other 563 patients with congenital heart disease but without PH. Results Peak oxygen uptake was the most discriminative variable, showing two thresholds at 16.3 mL/min per kg and 25.2 mL/min per kg. The highest specificity of 95% for PH was found in patients with a peak oxygen uptake of 16.3 mL/min per kg or less and a breathing reserve of 37.4% or less. In patients with a peak oxygen uptake exceeding 16.3 mL/min per kg, there was a high specificity of 86.3% but a low sensitivity of 53.1%. With 25.2 mL/min per kg as the threshold, the sensitivity for PH was only 10.0%. Conclusions Detection of PH in patients with congenital heart disease by CPET is difficult because of many falsely positive tests. However, a peak oxygen uptake higher than 25.2 mL/min per kg makes the diagnosis of PH unlikely.

Background HMG CoA Reductase Inhibitors, commonly referred to as statins, are widely used in heart transplant patients as dyslipidemia is common after cardiac transplantation and increases the risk of cardiovascular adverse events including cardiac allograft vasculopathy (CAV). However, not all patients can tolerate statins or see an adequate lowering of the low density lipoprotein cholesterol (LDL-C). Proprotein convertase subtilisin/kexin type 9 (PCSK-9) inhibitors have been shown to be effective at lowering LDL with great tolerance and excellent safety profiles in the general population. Yet, there is limited data regarding effect of PCSK9 inhibitors in heart transplant patients. Objective To study the effect of PSCK-9 inhibitors in post-cardiac transplant patients. Method We reviewed post heart transplant patients who received PCSK9 inhibitors at our University Medical Center from 2017 to March 2020. We found four patients who were eligible. Results The mean age of the four patients was 65.75± 4.7 years (3 Females). Indication for PCSK9 inhibitors in three patients was statin intolerance due to myopathy; the fourth patient had familial hypercholesterolemia that required more aggressive therapy. Average time for initiation of the medication since heart transplant was 10.25 years. The average duration of treatment was 18.25 months. Two patients were on Evolocumab 140mg subcutaneously once every 2 weeks, and the other two were on Alirocumab 75mg every 2 weeks. All four patients continued the medicine with no side effects. Mean LDL-C prior to initiation of the drug was 125.75± 24.42 mg/dL. At 8-12 week follow up there was significant reduction in the LDL 35.75 ±13.74mg/dL (p=0.006).There was no significant dose changes to the immunosuppressive medications. None of the four patients experienced rejection of the transplanted heart. Only one patient with familial hypercholesterolemia had mild CAV on heart catheterization prior to initiation of the drug and there was no progression of the CAV at one year follow up. Conclusion PCSK9-inhibitors were effective in reducing low-density lipoprotein levels in post-cardiac transplant patients and were well tolerated without any rejection or modifications to the transplant medications in this small cohort of patients. The study findings are promising and highlight the need for larger, prospective, multicenter studies.

Congenital portosystemic shunts (CPSS) are rare and underdiagnosed abdominal vascular malformations. Their relationship with cardiovascular (CV) disorders are poorly reported. Herein, we describe CV disorders associated with CPSS in a large cohort to propose adequate diagnosis and management. Among 168 children referred for a CPSS (1996–2019), those presenting at least one clinically significant CV disease including congenital heart diseases (CHD), heart failure (HF), pulmonary hypertension (PH) and/or hepatopulmonary syndrome (HPS) were included. CV disorders were detected using echocardiography complemented by right-sided heart catheterization and/or lung perfusion radionuclide when appropriate. 46/168 children with CPSS (27,4%) were included. In 36 patients, endovascular or surgical CPSS closure was required, including 24 for CV indications. CHD was present in 28 patients (16,6%); 7 had heterotaxy (4,1%). HF occurred in 21 neonates, diagnosed prenatally in 7, including 13 with CHD. HF decreased after cardiac surgery in 10 with CHD but also spontaneously before the age of 3 years in 8 except one without CHD. In 11 patients (mean age: 11 ± 5.2 years) CPSS were diagnosed as part of a work up for fixed PH (mean pressures 46 ± 15.1 mmHg, pulmonary vascular resistances 7 ± 2.5UW/m2). One of them died before CPSS closure, while PH remained stable despite CPSS closure in 10 patients, of which 2 then required lung transplantation. In 6 patients (median age: 5.5 years), hypoxia revealed HPS which resolved within 2 years of CPSS closure. CV disorders are frequently associated with CPSS including CHD with/without heterotaxy. HF is found in fetuses and neonates, regressing in most cases. Later, HPS and PH may develop. After CPSS closure, HPS is reversible while PH may only stabilize. Perinatal HF, complex CHD, heterotaxy, unusual course of CHD, HPS and PH should raise the possibility of CPSS. Conversely CV complications should be searched for in patients with CPSS.

Objectives The aim of this study was to develop a technique to measure regurgitant flow throughout the entire cardiac cycle that would more accurately measure aortic insufficiency (AI) severity in patients with continuous-flow left ventricular assist devices (CF-LVADs). Background AI is a common problem after CF-LVAD implantation. Current echocardiographic evaluation of AI does not take into account the unique flow properties present in patients with CF-LVADs. Methods In this prospective study, patients with LVADs who had varying degrees of AI (N = 20) underwent simultaneous right-sided heart catheterization (RHC) and transthoracic echocardiography (TTE). Regurgitant fraction (RF) across the aortic valve was calculated by subtracting the cardiac output obtained using the Fick method from the total systemic flow measured using the sum of the product of the velocity time integral and the cross-sectional area of the LVAD outflow cannula and aortic valve, respectively. The RFs were then compared with the following: 1) traditional TTE grading parameters; and 2) new TTE parameters unique to LVAD physiology, namely the diastolic flow acceleration and the systolic-to-diastolic peak velocity (S/D) ratio of the LVAD outflow cannula. Results Patients without evidence of AI had an RF approaching zero (2.4 ± 4.6%). Patients with trace and mild AI had an RF of 31.0 ± 5.4%, whereas patients with moderate or severe AI had an RF of 45.8 ± 3.6%. RF correlated better with pulmonary capillary wedge pressure (PCWP) than with vena contracta (correlation coefficient [R] = 0.73 vs. 0.56). The new TTE parameters (S/D ratio and diastolic acceleration) highly correlated with RF (R = 0.91 and 0.94, respectively) and more strongly correlated with PCWP than did vena contracta (R = 0.82 and 0.65 vs. 0.56). Conclusions RF measured by simultaneous RHC and TTE better correlates with clinical filling pressures than do traditional TTE parameters and may identify significant AI that could be underestimated using conventional measures. Novel TTE parameters, unique to CF-LVAD physiology, better correlate with RF and filling pressures than do our current TTE measurements.

BackgroundHemodynamic differentiation between pulmonary arterial hypertension (PAH) and postcapillary pulmonary hypertension (PH) is important because treatment options are strikingly different for the two disease subsets. Whereas patients with PAH can be treated effectively with targeted therapies, their use in postcapillary PH is currently not recommended. Our aim was to establish an algorithm to identify patients who are likely to experience a significant hemodynamic treatment response.MethodsWe determined hemodynamic cutoffs to discriminate between idiopathic PAH and postcapillary PH in a large database of 4,363 stable patients undergoing first diagnostic right and left heart catheterizations. In a second step, we performed a patient-level pooled analysis of four randomized, placebo-controlled trials including 541 patients with PAH who received treprostinil or placebo, to validate hemodynamic cutoffs with regard to treatment response.ResultsReceiver operating characteristic analysis identified mean pulmonary arterial wedge pressure (mPAWP) < 12 mm Hg and diastolic pulmonary vascular pressure gradient (DPG) ≥ 7 mm Hg as the best hemodynamic discriminators between idiopathic PAH and postcapillary PH. In our treatment study, only patients with mPAWP < 12 mm Hg, DPG > 20 mm Hg or a combination of both had a significant placebo-corrected improvement in hemodynamics.ConclusionsmPAWP < 12 mm Hg and DPG > 20 mm Hg identify patients with PAH who are likely to have significant hemodynamic improvement with prostacyclin treatment.

Optical coherence tomography is a catheter-based imaging modality in heart catheterizations, which provides a significantly higher resolution of intravascular pathology by means of using light as opposed to ultrasound. The applications of this modality may include a detailed assessment of atherosclerotic plaques, stent evaluation including coverage and restenosis and percutaneous coronary intervention optimization. In this article, we provide a review of current literature highlighting the advantages and disadvantages of the use of optical coherence tomography in the catheterization lab.

Cobalt toxicity is a rare cause of infiltrative cardiomyopathy. We describe a case of cobalt-induced cardiomyopathy that progressed to end stage heart failure requiring orthotopic heart transplantation. A 67 year old woman with history of bilateral cobalt-chromium hip replacements presented with dyspnea of 3 weeks duration. She was tachycardic and hypotensive, with elevated NT- proBNP. Electrocardiogram demonstrated sinus tachycardia, low voltage and right bundle branch block. Transthoracic echocardiogram revealed left ventricular ejection fraction of 20-25% with global hypokinesis and a moderate circumferential pericardial effusion. Left and right heart catheterization showed normal coronaries, elevated filling pressures and low cardiac index. Dobutamine infusion was initiated for management of cardiogenic shock. Cardiac magnetic resonance imaging (MRI) illustrated diffuse late gadolinium enhancement concerning for infiltrative cardiomyopathy. Extensive non-ischemic and rheumatologic workup was negative. Cobalt and chromium levels were significantly elevated at 121 mcg/L (Nl

Introduction Right heart catheterization is an invasive exam that is currently the gold standard to assess pulmonary hemodynamics for diagnosis and follow-up of pulmonary arterial hypertension (PAH) associated with congenital heart diseases (CHD). Cardiac magnetic resonance 4D flow (4D CMR flow) emerges as a promising non-invasive imaging. We assess the accuracy of 4D CMR flow to measure pulmonary cardiac output (Qp). Methods We prospectively included 28 patients with PAH associated with CHD. Qp was measured invasively using Fick principle (direct oxygen consumption measure) during aright heart catheterization (QpF) and compared to Qp measured by 2D (Qp2D) and 4D flow CMR (Qp4D) on the same day. Results Twenty eight patients was included (median age was 42 years old [35–52]) with PAH and CHD (pre-tricuspid shunt n = 23, 82.1%, median mean pulmonary artery pressure 46 mmHg [40-58]; median pulmonary vascular resistance 15.0 WU.m2 [7.5–25.6]) 4D CMR flow analysis were feasible in all patients. Qp4D and QpF were strongly correlated (rho = 0.87, P Fig. 1 ). Conclusion Qp measured by 4D CMR flow is well correlated to QpF. Further studies are needed to explore other potential interests of using 4D CMR flow to assess PAH, including the derivation of additional hemodynamic parameters such as pulmonary artery compliance, wall shear stress and pulse wave velocities, which could provide further insights into pulmonary artery remodeling and interactions between pulmonary arterial stiffening and right ventricular dysfunction.

Objectives This study sought to assess the impact of a wide range of mean right atrial pressure (P ra ) on fractional flow reserve (FFR) measurements. Background FFR invasively assesses the ischemic potential of coronary stenoses. FFR is calculated as the ratio of mean distal coronary pressure (P d ) to mean aortic pressure (P a ) during maximal hyperemia. The P ra is considered to have little impact if it is within normal range, so it is neglected in the formula. Methods In 1,676 stenoses of 1,235 patients undergoing left-right heart catheterization for ischemic (642 [52%]) or valvular heart disease (593 [48%]), the authors compared the FFR values calculated without accounting for P ra (FFR= P d /P a ) to the corresponding myocardial fractional flow reserve (FFR myo ) values accounting for P ra (FFR myo = P d − P ra /P a − P ra ). Results The median P ra was 7 (interquartile range [IQR]: 5 to 10) mm Hg with a maximum of 27 mm Hg. The correlation and agreement between FFR and FFR myo was excellent (R 2 = 0.987; slope 1.096 ± 0.003). The median FFR (0.85; IQR: 0.78 to 0.91) was slightly but statistically significantly higher than the median FFR myo (0.83; IQR: 0.76 to 0.90; p myo ≤0.80 in 110 (9%) stenoses. No FFR value above 0.80 provided an FFR myo ≤0.75. Conclusions The difference between FFR and FFR myo was minimal even in patients with markedly increased P ra . FFR values above the gray zone (i.e., >0.80) did not yield values below the gray zone (i.e., ≤0.75) in any case, which suggests that the impact of right atrial pressure on FFR measurement is indeed negligible.

Background A subset of patients with hereditary hemorrhagic telangiectasia (HHT) develops pulmonary hypertension (PH) by mechanisms including pulmonary arterial hypertension, high flow, and elevated pulmonary arterial wedge pressure (PAWP). We aimed to describe echocardiographic and hemodynamic characteristics of patients with coexisting HHT and PH. Methods We conducted a single-center cohort study of patients with confirmed HHT who underwent right-sided heart catheterization (RHC) and transthoracic two-dimensional echocardiography for suspected PH between June 1, 2003 and September 1, 2013 at Mayo Clinic Rochester, Minnesota. Results Of 38 patients with confirmed HHT who underwent RHC and echocardiography, 28 (74%) had a mean pulmonary artery pressure (MPAP) ≥ 25 mm Hg. Of those 28, 12 (43%) had pulmonary arterial hypertension. Two patients had normal PAWP and pulmonary vascular resistance (PVR), with PH secondary to either an atrial septal defect or high cardiac flow. Fourteen patients (50%) had elevated PAWP (≥ 15 mm Hg), nine with evidence of high flow. RHC in all 28 patients demonstrated a MPAP of 41 ± 11 mm Hg, PAWP of 17 ± 10 mm Hg, and PVR of 4.5 ± 4.2 Wood units. Echocardiography demonstrated moderate/severe right ventricular dysfunction in nine patients (32%). The presence of PH trended toward worse survival ( P = .06). Conclusions PH in patients with HHT occurs by different mechanisms, and there is a trend toward worse survival in patients who develop PH despite the mechanism. The equal predilection toward all subtypes of PH illustrates the necessity of RHC to clarify the hemodynamics.

Background Pulmonary hypertension and associated right ventricular (RV) dysfunction are important determinants of morbidity and mortality, which are optimally characterized by invasive hemodynamic measurements. Objectives This study sought to determine whether metabolite profiling could identify plasma signatures of right ventricular-pulmonary vascular (RV-PV) dysfunction. Methods We measured plasma concentrations of 105 metabolites using targeted mass spectrometry in 71 individuals (discovery cohort) who underwent comprehensive physiological assessment with right-sided heart catheterization and radionuclide ventriculography at rest and during exercise. Our findings were validated in a second cohort undergoing invasive hemodynamic evaluations (n = 71), as well as in an independent cohort with or without known pulmonary arterial (PA) hypertension (n = 30). Results In the discovery cohort, 21 metabolites were associated with 2 or more hemodynamic indicators of RV-PV function (i.e., resting right atrial pressure, mean PA pressure, pulmonary vascular resistance [PVR], and PVR and PA pressure-flow response [ΔPQ] during exercise). We identified novel associations of RV-PV dysfunction with circulating indoleamine 2,3-dioxygenase (IDO)–dependent tryptophan metabolites (TMs), tricarboxylic acid intermediates, and purine metabolites and confirmed previously described associations with arginine–nitric oxide metabolic pathway constituents. IDO-TM levels were inversely related to RV ejection fraction and were particularly well correlated with exercise PVR and ΔPQ. Multisite sampling demonstrated transpulmonary release of IDO-TMs. IDO-TMs also identified RV-PV dysfunction in a validation cohort with known risk factors for pulmonary hypertension and in patients with established PA hypertension. Conclusions Metabolic profiling identified reproducible signatures of RV-PV dysfunction, highlighting both new biomarkers and pathways for further functional characterization.

Pulmonary hypertension is a hemodynamic condition, defined as a mean pulmonary artery pressure by right-sided heart catheterization of at least 25 mm Hg at rest. It is classified into 5 general groups based on the underlying cause, with left ventricular failure and chronic obstructive pulmonary disease being 2 of the most common causes in the United States. Although the specifics of the pathophysiology will vary with the cause, appreciating the risks of pulmonary hypertension and right ventricular failure is critical to appropriately evaluating and resuscitating pulmonary hypertension patients in the emergency department (ED). Patients may present to the ED with complaints related to pulmonary hypertension or unrelated ones, but this condition will affect all aspects of care. Exertional dyspnea is the most common symptom attributable to pulmonary hypertension, but the latter should be considered in any ED patient with unexplained dyspnea on exertion, syncope, or signs of right ventricular dysfunction. Patients with right ventricular failure are often volume overloaded, and careful volume management is imperative, especially in the setting of hypotension. Vasopressors and inotropes, rather than fluid boluses, are often required in shock to augment cardiac output and reduce the risk of exacerbating right ventricular ischemia. Intubation should be avoided if possible, although hypoxemia and hypercapnia may also worsen right-sided heart function. Emergency physicians should appreciate the role of pulmonary vasodilators in the treatment of pulmonary arterial hypertension and recognize that patients receiving these medications may rapidly develop right ventricular failure and even death without these therapies. Patients may require interventions not readily available in the ED, such as a pulmonary artery catheter, inhaled pulmonary vasodilators, and mechanical support with a right ventricular assist device or extracorporeal membrane oxygenation. Therefore, early consultation with a pulmonary hypertension specialist and transfer to a tertiary care center with invasive monitoring and mechanical support capabilities is advised.

Background Cirrhotic cardiomyopathy may lead to heart failure in stressful circumstances, such as after transjugular intrahepatic portosystemic shunt (TIPS) placement. Aim To examine whether acute volume expansion predicts haemodynamic changes after TIPS and elicits signs of impending heart failure. Methods We prospectively evaluated refractory ascites patients (group A) and compensated cirrhotics (group B), who underwent echocardiography, NT-proBNP measurement, and heart catheterization before and after volume load; group A repeated measurements after TIPS. Results 15 patients in group A (80% male; 54 ± 12.4 years) and 8 in group B (100% male; 56 ± 6.2 years) were enrolled. Echocardiography disclosed diastolic dysfunction in 30% and 12.5%, respectively. In group A, volume load and TIPS induced a significant increase in right atrial, mean pulmonary, capillary wedge pressure and cardiac index, and a decrease in systemic vascular resistance (respectively, 4.7 ± 2.8 vs. 9.9 ± 3.6 mmHg; 13.3 ± 3.5 vs. 21.9 ± 5.9 mmHg; 8.3 ± 3.4 vs. 15.4 ± 4.7 mmHg; 3.7 ± 0.7 vs. 4.6 ± 1 lt/min/m 2 ; 961 ± 278 vs. 767 ± 285 dyn s cm −5 ; and 10.1 ± 3.3 vs. 14.2 ± 3.4 mmHg; 17.5 ± 4 vs. 25.2 ± 4.2 mmHg; 12.3 ± 4 vs. 19.3 ± 3.4 mmHg; 3.4 ± 0.8 vs. 4.5 ± 0.91 lt/min/m 2 ; 779 ± 62 vs. 596 ± 199 dyn s cm −5 , p Conclusions Acute volume expansion predicted haemodynamic changes immediately after TIPS. All patients had adequate haemodynamic adaptation to TIPS; none developed signs of heart failure.

Background It is unclear whether recent advances in pulmonary arterial hypertension therapy can be safely applied to sarcoidosis-associated pulmonary hypertension (SAPH). Evidence for prostacyclin (PG) therapy in SAPH is limited. Methods We conducted a single-center, retrospective review of 46 patients with sarcoidosis, 26 of whom had SAPH. Thirteen received PG as monotherapy or in combination with oral vasodilators. Results Follow-up right-sided heart catheterization at a mean of 12.7 months revealed improved cardiac output, cardiac index, and pulmonary vascular resistance. Functional class and N-terminal pro-brain natriuretic peptide levels also improved in patients treated with PG. No significant change in oxygen requirement was seen with vasodilator therapy initiation. At 2 years, 15 patients with SAPH survived, including eight on PG, and at 5 years, seven survived, including five on PG. Survival was significantly reduced in patients with SAPH compared with patients who had sarcoidosis without pulmonary hypertension. Multivariate analysis demonstrated that the use of PG therapy in SAPH is not associated with increased mortality. Conclusions Many patients with severe SAPH showed significant hemodynamic and clinical improvement on long-term IV or subcutaneous PG therapy and had survival outcomes similar to patients with moderate SAPH on oral vasodilator therapy.

BackgroundThe implantation of stents to keep the ductus arteriosus patent in cyanotic congenital heart disease is an alternative to the modified Blalock-Taussig surgery (mBT) in high-risk patients. This study describes the immediate and medium-term outcomes of stent implantation in neonates and infants with duct-dependent pulmonary circulation.MethodsThis was a descriptive and prospective study including different cyanotic congenital heart diseases treated between 2014 and 2015.ResultsFourteen patients with a mean age of 46 days, and mean weight of 4.5kg were assessed, and pulmonary artresia with interventricular communication was the most treated condition. The femoral artery approach was used in 70% of procedures; carotid approach was used in the remaining cases. Stents of 3.5 x 12mm were used in most cases, and implant success was achieved in 78% of interventions (11/14). The failed cases were referred to surgery – one of them was an emergency, which resulted in death. Ductal spasm occurred in < 48hours in three patients who required mBT, with favorable outcome. Complications after discharge and within the first 30 days included stent thrombosis (2/11), one of which was controlled with redilation, another progressed to death, and one sudden death (1/11). The overall mortality was 21.4% (3/14). A patent ductus arteriosus in the first 6 months was present in five cases, which underwent palliative surgery.ConclusionsThe initial experience of ductal stenting showed favorable immediate outcomes, but in the medium term, little more than a third of the cases maintained a patent ductus arteriosus within 6 months.

RESUMOIntroduçãoO implante de stents para manter o ducto arterial patente na cardiopatia congênita cianótica é uma alternativa à cirurgia de Blalock‐Taussig modificada (BTm) em pacientes de alto risco. Descrevemos os resultados imediatos e de médio prazo do implante de stent em neonatos e lactentes com circulação pulmonar ducto‐dependente.MétodosTrata‐se de estudo descritivo e prospectivo, que incluiu diferentes cardiopatias congênitas cianóticas tratadas entre 2014 e 2015.ResultadosAvaliamos 14 pacientes, com média de idade de 46 dias e pesando 4,5kg, sendo a atresia pulmonar associada à comunicação interventricular a cardiopatia mais tratada. A abordagem pela artéria femoral ocorreu em 70% dos procedimentos e, nos demais, por via carotídea. Stents de 3,5 12mm foram usados na maioria dos casos, e o sucesso do implante foi obtido em 78% das intervenções (11/14). Os casos de insucesso foram encaminhados para cirurgia uma delas em situação de urgência, que resultou em óbito. Ocorreu espasmo ductal < 48 horas em três pacientes que necessitaram de BTm, com evolução favorável. Complicações após a alta e nos primeiros 30 dias incluíram trombose de stent (2/11), uma delas controlada com redilatação e outra que evoluiu para óbito, e uma morte súbita (1/11). A mortalidade total foi de 21,4% (3/14). A patência do ducto arterial nos primeiros 6 meses foi obtida em 5 casos que foram submetidos à cirurgia paliativa.ConclusõesA experiência inicial de implante de stent ductal mostrou resultados imediatos favoráveis, e, em médio prazo, mais de um terço dos pacientes com circulação pulmonar ducto‐dependente manteve seus canais patentes.ABSTRACTBackgroundThe implantation of stents to keep the ductus arteriosus patent in cyanotic congenital heart disease is an alternative to the modified Blalock‐Taussig surgery (mBT) in high‐risk patients. This study describes the immediate and medium‐term outcomes of stent implantation in neonates and infants with duct‐dependent pulmonary circulation.MethodsThis was a descriptive and prospective study including different cyanotic congenital heart diseases treated between 2014 and 2015.ResultsFourteen patients with a mean age of 46 days, and mean weight of 4.5kg were assessed, and pulmonary artresia with interventricular communication was the most treated condition. The femoral artery approach was used in 70% of procedures; carotid approach was used in the remaining cases. Stents of 3.5 x 12mm were used in most cases, and implant success was achieved in 78% of interventions (11/14). The failed cases were referred to surgery – one of them was an emergency, which resulted in death. Ductal spasm occurred in < 48hours in three patients who required mBT, with favorable outcome. Complications after discharge and within the first 30 days included stent thrombosis (2/11), one of which was controlled with redilation, another progressed to death, and one sudden death (1/11). The overall mortality was 21.4% (3/14). A patent ductus arteriosus in the first 6 months was present in five cases, which underwent palliative surgery.ConclusionsThe initial experience of ductal stenting showed favorable immediate outcomes, but in the medium term, little more than a third of the cases maintained a patent ductus arteriosus within 6 months.

BACKGROUND Pulmonary arterial hypertension (PAH) is a rare and ultimately fatal disorder of the pulmonary vasculature. There is increasing interest in the worldwide characteristics of patients with PAH, although data coming from the Southern Hemisphere remain scarce. The objective of this study was to describe a cohort of incident patients with PAH from a large reference center in Brazil. METHODS : All consecutive patients who received a diagnosis of PAH by right-sided heart catheterization between 2008 and 2013 were included in the study. RESULTS : A total of 178 patients with newly diagnosed PAH were enrolled in the study (mean age, 46 years; female/male ratio, 3.3:1; 45.5% in New York Heart Association functional class III or IV). Idiopathic PAH (IPAH), connective tissue disease (CTD), and schistosomiasis-associated PAH (Sch-PAH) accounted for 28.7%, 25.8%, and 19.7% of all cases, respectively. The patients were treated with phosphodiesterase type 5 inhibitors (66%), endothelin receptor antagonists (27%), or a combination of both (5%). For the PAH group as a whole, the estimated survival rate 3 years after diagnosis was 73.9%. The prognosis for the patients with CTD was worse than that for the patients with IPAH and Sch-PAH ( P = .03). CONCLUSIONS : The distribution of PAH causes and the baseline characteristics in our registry clearly differ from the previously published European and US-based registries. These differences highlight the importance of regional registries and also raise questions regarding the need to better account for such differences in future clinical trials.

Elevated mean right atrial pressure (RAP) measured by cardiac catheterization is an independent risk factor for mortality. Prior studies have demonstrated a modest correlation with invasive and noninvasive echocardiographic RAP, but the prognostic impact of estimated right atrial pressure (eRAP) has not been previously evaluated in patients with pulmonary arterial hypertension (PAH).A retrospective analysis of 121 consecutive patients with PAH based on right-sided heart catheterization and echocardiography was performed. The eRAP was calculated by inferior vena cava diameter and collapse using 2005 and 2010 American Society of Echocardiography (ASE) definitions. Accuracy and correlation of eRAP to RAP was assessed. Kaplan-Meier survival analysis by eRAP, right atrial area, and Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) risk criteria as well as univariate and multivariate analysis of echocardiographic findings was performed.Elevation of eRAP was associated with decreased survival time compared with lower eRAP (P.001, relative risk = 7.94 for eRAP15 mm Hg vs eRAP ≤ 5 mm Hg). Univariate analysis of echocardiographic parameters including eRAP15 mm Hg, right atrial area18 cm², presence of pericardial effusion, right ventricular fractional area change35%, and at least moderate tricuspid regurgitation was predictive of poor survival. However, multivariate analysis revealed that eRAP15 mm Hg was the only echocardiographic risk factor that was predictive of mortality (hazard ratio = 2.28, P = .037).Elevation of eRAP by echocardiography at baseline assessment was strongly associated with increased risk of death or transplant in patients with PAH. This measurement may represent an important prognostic component in the comprehensive echocardiographic evaluation of PAH.

Aims: The nitric oxide and endothelin systems are key components of a local paracrine hormone network in the heart. We previously reported that diastolic dysfunction observed in mice lacking the endothelial nitric oxide synthase (eNOS-/-) can be prevented by a genetic overexpression of ET-1. Sexual dimorphisms have been reported in both ET-1 and NO systems. Particularly, eNOS-/- mice present sex related phenotypic differences. Main methods: We used the ET-1 transgenic (ET+/+), eNOS-/-, and crossbred ET+/+ eNOS-/- mice, and wild type controls. We measured cardiac function by heart catheterization. Cardiac ventricles were collected for histological and molecular profiling. Key findings: We report here that (i) the level of ET-1 expression in eNOS-/- mice was elevated in males but not in females. (ii) Left ventricular end-diastolic blood pressure was higher in male eNOS-/- mice than in females. (ii) eNOS-/- males but not females developed cardiomyocyte hypertrophy. (iv) Perivascular fibrosis of intra-cardiac arteries developed in female ET+/+ and eNOS-/- mice but not in males. Additionally, (v) the cardiac expression of metalloprotease-9 was higher in eNOS-/- males compared to females. Finally, (vi) cardiac proteome analysis revealed that the protein abundance of the oxidative stress related enzyme superoxide dismutase presented with sexual dimorphism in eNOS-/- and ET+/+ mice. Significance: These results indicate that the cardiac phenotypes of ET-1 transgenic mice and eNOS knockout mice are sex specific. Since both systems are key players in the pathogenesis of cardiovascular diseases, our findings might be important in the context of gender differences in patients with such diseases. (C) 2013 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).

ObjectiveThere is cross-center variability with regard to timing repair of non–ductal-dependent tetralogy of Fallot (TOF). We hypothesized that earlier repair in the neonatal period is associated with increased mortality and morbidity.MethodsThis was a retrospective analysis of the Pediatric Health Information System of tetralogy of Fallot patients undergoing complete repair from 2004 through 2010 between the ages of 1 day to younger than 19 years. Patients with pulmonary valve atresia, those who received prostaglandin during hospital admission, and those who underwent prior shunt palliation were excluded.ResultsA total of 4698 patients met our inclusion criteria, of whom 202 were younger than 30 days old (group A), 861 were 31 to 90 days old (group B), 1796 were 91 to 180 days old (group C), and 1839 were older than 180 days (group D). In-hospital mortality, intensive care unit length of stay, and total hospital length of stay were significantly higher in group A. Patients in group A had a significantly increased postoperative requirement for mechanical ventilation, intravenous blood pressure support, medical diuresis, extracorporeal membrane oxygenation, gastrostomy tube insertion, heart catheterization, and surgical revision. Significant institutional variability was noted for timing of TOF complete repair, with one third of the centers performing 75% of the repairs at younger than 30 days old. The institutional approach to timing TOF complete repair showed no relation to surgical volume.ConclusionsAcross all centers analyzed, primary neonatal elective TOF repair (

Background Sarcoidosis-associated pulmonary hypertension (SAPH) is a common problem in patients with persistent dyspneic sarcoidosis. The objective of this study was to determine the effect of bosentan therapy on pulmonary arterial hemodynamics in patients with SAPH. Methods This 16-week study was a double-blind, placebo-controlled trial of either bosentan or placebo in patients with SAPH confirmed by right-sided heart catheterization. Patients were enrolled from multiple academic centers specializing in sarcoidosis care. They were stable on sarcoidosis therapy and were receiving no therapy for pulmonary hypertension. The cohort was randomized two to one to receive bosentan at a maximal dose of 125 mg or placebo bid for 16 weeks. Pulmonary function studies, 6-min walk test, and right-sided heart hemodynamics, including pulmonary artery mean pressure and pulmonary vascular resistance (PVR), were performed before and after 16 weeks of therapy. Results Thirty-five patients completed 16 weeks of therapy (23 treated with bosentan, 12 with placebo). For those treated with bosentan, repeat hemodynamic studies at 16 weeks demonstrated a significant mean ± SD fall in PA mean pressure (−4 ± 6.6 mm Hg, P = .0105) and PVR (−1.7 ± 2.75 Wood units, P = .0104). For the patients treated with placebo, there was no significant change in either PA mean pressure (1 ± 3.7 mm Hg, P > .05) or PVR (0.1 ± 1.42 Wood units, P > .05). There was no significant change in 6-min walk distance for either group. Two patients treated with bosentan required an increase of supplemental oxygen by > 2 L after 16 weeks of therapy. Conclusions This study demonstrated that bosentan significantly improved pulmonary hemodynamics in patients with SAPH. Trial registry ClinicalTrials.gov ; No: NCT00581607; URL: www.clinicaltrials.gov

The transradial approach is becoming the access method of choice in many countries because of its lower risk for vascular complications, earlier post-procedural ambulation, and better patient satisfaction. Many patients planned for left heart catheterization require simultaneous right heart

Introduction A 62-year-old man was admitted to a local hospital for heart failure. An echo showed severe biventricular dysfunction, severe LV dilatation and a large and mobile finger-like aspect thrombus extending from septum, across apex and posterior wall. He presented severe instability and therefore was transferred to our centre for further management. On arrival, he suffered electrical VT storm and a VA-ECMO was inserted. At this point, heart catheterization and angiography were considered unsafe. After multidisciplinary discussion, a Levitronix LVAD with filters was inserted as bridge to decision. Due to rapid RV function deterioration an intraoperative decision was made to add an RVAD with an oxygenator. He was inserted on the transplant list, but unclear capacity to cope with treatment and significant psychiatric issues emerged after family discussion. Refractory VF developed which lead to a fully thrombosed LV. In the evaluation for candidacy, a total artificial heart (TAH) was implanted. After the procedure, the patient experienced a stroke leading to left hemiplegia and fluctuating level of consciousness. After a period of relative clinical stability, the patient developed a catastrophic intestinal bleeding. Given the multiple complications developed during the ITU stay (renal failure, multi-drug resistant infections, neurological impairment and unsuitability for transplant) a laparotomy appeared not to be in the patient's best interest. After family discussion, palliation was commenced. Native heart histology revealed extensive multifocal sub-endocardial myocardial infarction involving the LV. Right and left coronary arteries showed significant atherosclerotic disease. Discussion Over recent years, mechanical circulatory support devices have revolutionized cardiovascular therapeutics. In the present case, the initial issue was the severe haemodynamic instability condition in which the patient presented, which lead to an unwanted incomplete assessment of the underlying disease. A multidisciplinary discussion was made about further management after VA-ECMO insertion: in order to discuss with the patient and assess candidacy to transplant, no other ways appeared to be reasonable than implanting a Levitronix, which could have allowed to awake the patient. As soon as the medical team was able to discuss with the patient and the family about further management, neuropsychiatric issues arose, delaying the decision to a definitive insertion of the patient to the transplant list. During this assessment period, massive LV thrombus developed rising the issue of Levitronix occlusion, leading to two main life-treating options: heart transplant or total artificial heart. This case shows how complex the management of a severe heart failure with cardiogenic shock may be and how different types of mechanical circulatory support devices may be required for similar severity of the original disease depending on the phase of the treatment and the comorbidities.

Background A left heart catheterization optimization protocol was recently developed at our institution and several physiologic indices, including LV end diastolic pressure (LVEDP) and trans-aortic gradient (TAG), previously only available in computational or animal models, were evaluated in patients supported on LVAD therapy. We sought to evaluate the prognostic significance of these markers. Hypothesis Inability to achieve adequate ventricular unloading will result in worse heart failure hospitalization-free survival. Methods All patients undergoing combined left and right heart catheterization protocol at our institution between 2015 and 2018 were evaluated. Patients whose left ventricles were inaccessible or who underwent non-standard protocols were excluded. Comprehensive clinical data were obtained. Primary endpoints included a composite outcome of hospitalization or death and overall survival. Results Thirty one patients were analyzed from the cohort. Optimization resulted in normalization of hemodynamic parameters on average (RA 11 mmHg, PA 25 mmHg, PCWP 13 mmHg, LVEDP 12 mmHg, cardiac index 2.53 L/min/m2), which was significantly improved from baseline for all variables [p ≤ 0.05]). On univariate modeling, only LVEDP was associated with the primary endpoint (HR 1.2 per 1 mmHg increase, CI[1.1-1.3], p Conclusions Left ventricular offloading as measured by left ventricular end-diastolic pressure was a significant marker of poor outcomes over time. Further research should focus on pathophysiology corresponding to poor unloading.

Purpose There is a paucity of data describing influences on cardiac output in patients supported on LVAD therapy. We describe, for the first time, the independent impact of afterload on cardiac output in the LVAD population. Methods Between 2015 and 2018, 54 patients on LVAD therapy were evaluated with simultaneous left and right heart catheterization ramp study. All patients with significant aortic regurgitation or device malfunction/thrombosis were excluded. Hemodynamic parameters from the 30 remaining patients undergoing speed optimization were analyzed. Results Mean central aortic pressure (MAP) was 87 ± 13 mmHg at baseline and correlated significantly with RPM for each pump type (R > 0.24, p ≤ 0.05 for all). No association between MAP or RPM and cardiac index (CI) was found. However, there was a significant correlation between systemic vascular resistance (SVRi) and CI (R = -0.76, p 90, n=8) received nitrate therapy and experienced a mean increase in cardiac index from 2.3 L/min/m2 to 2.8 L/min/m2 (mean increase of 23%) at a given RPM, validating the physiologic afterload findings. Conclusion Afterload as measured by LV-EA or SVRi negatively influences cardiac output in LVAD therapy and is ameliorated by nitrates. These findings serve as a foundation to evaluate the dynamic effects of high afterload and chronic vasodilator therapy in patients with durable LVADs.

Pulmonary arterial hypertension (PAH) is a progressive and devastating condition characterized by vascular cell proliferation and is associated with several metabolic derangements. We hypothesized that metabolic derangements in PAH can be detected by measuring metabolic by-products in exhaled breath.We collected breath and blood samples from patients with PAH at the time of right-sided heart catheterization (n=31) and from healthy control subjects (n=34). Breath was analyzed by selected ion flow tube-mass spectrometry in predetermined training and validation cohorts.Patients with PAH were 51.5±14 years old, and 27 were women (85%). Control subjects were 38±13 years old, and 22 were women (65%). Discriminant analysis in the training set identified three ion peaks (H3O+29+, NO+56+, and O2+98+) and the variable age that correctly classified 88.9% of the individuals. In an independent validation cohort, 82.8% of the individuals were classified correctly. The concentrations of the volatile organic compounds 2-propanol, acetaldehyde, ammonia, ethanol, pentane, 1-decene, 1-octene, and 2-nonene were different in patients with PAH compared with control subjects. Exhaled ammonia was higher in patients with PAH (median [interquartile range]: 94.7 parts per billion (ppb) [70-129 ppb] vs 60.9 ppb [46-77 ppb], P.001) and was associated with right atrial pressure (ρ=0.57, P.001), mean pulmonary artery pressure (ρ=0.43, P=.015), cardiac index by thermodilution (ρ=-0.39, P=.03), pulmonary vascular resistance (ρ=0.40, P=.04), mixed venous oxygen (ρ=-0.59, P.001), and right ventricular dilation (ρ=0.42, P=.03).Breathprint is different between patients with PAH and healthy control subjects. Several specific compounds, including ammonia, were elevated in the breath of patients with PAH. Exhaled ammonia levels correlated with severity of disease.

PAH associated with connective tissue diseases is associated with significant functional impairment and morbidity, and carries with it a poor prognosis. The mortality is as high as 10% to 15% in the first year after diagnosis; making it a devastating disease. The availability of ever-increasing numbers of treatment options in the recent era have improved survival in this patient population and have made early and accurate diagnosis a more important goal. According to the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL), 1-year, 3-year, 5-year, and 7-year survival rates from time of diagnostic right-sided heart catheterization in patients with PAH were found to be 85%, 68%, 57%, and 49%, respectively, which is a considerable improvement since the National Institutes of Health registry 2 decades previously. In a study by Condliffe and colleagues, survival rates in patients with SSC-associated PAH have improved to 78%at 1 year and 47% at 3 years. Patients with SLE-related PAH have a much higher survival rate of up to 75% at 3 years. Proper screening, early diagnosis, and early treatment can have a significant impact in reducing morbidity and mortality. A small study to assess outcomes in patients with asymptomatic CTD found to have exercise induced PAH suggest that bosentan may be safe and effective in improving the hemodynamics and outcomes in these patients. This study included only 10 patients, and additional randomized trials with larger numbers of subjects are needed to affirm this hypothesis. Studies are under way to find additional therapeutic modalities in the form of PDGF receptor blockers, VEGF blockers, tyrosine kinase inhibitors, endothelial dysfunction inhibitors, multikinase inhibitor of Raf-1, serotonin receptor antagonists,and rho kinase inhibitors. Despite these, clinical suspicion, early diagnosis, early

Background Pulmonary arterial hypertension (PAH) is characterized by a pulmonary capillary wedge pressure (PCWP) of ≤15 mm Hg, given a normal left ventricular filling pressure (LVFP). However, recent studies have shown that, in PAH patients, diagnosis based on PCWP can erroneously classify a significant number of patients compared with diagnosis based on left ventricular end-diastolic pressure (LVEDP). Therefore, we sought to compare the diagnostic accuracy of end-expiratory PCWP and LVEDP measurements in patients suspected of having pulmonary hypertension (PH). Methods We reviewed the hemodynamic data from 122 patients suspected of having PH who underwent simultaneous right- and left-side heart catheterizations at a PH referral center from 2006 to 2011. Results PH was diagnosed in 105 patients, 79% of whom ( n = 83) showed a pre-capillary pattern according to the LVEDP measurement. Ninety percent of patients with PCWP ≤15 mm Hg were correctly classified as having pre-capillary PH. However, 39% of patients with a PCWP >15 mm Hg had LVEDP ≤15 mm Hg and would have been erroneously diagnosed with pulmonary venous hypertension based on their PCWP measurements alone. The sensitivity and specificity was 0.89 and 0.64, respectively. A Bland-Altman analysis of the PCWP and LVEDP measurements revealed a mean bias of 0.3 mm Hg with 95% limits of agreement of −7.2 to 7.8 mm Hg. Conclusions A PCWP ≤15 mm Hg was found to be a reliable indicator of normal LVFP in pre-capillary PH patients. When measured properly and analyzed in the clinical context, PCWP is a valuable tool for accurate diagnosis of PAH.

World Health Organization (WHO) group 2 pulmonary hypertension (PH) due to left-side heart disease (ie, heart failure or left-sided valvular heart disease) is the most common form of PH in western countries. Distinguishing patients with WHO group 2 PH, particularly the subset of patients with PH due to heart failure with preserved ejection fraction (HFpEF), from those with WHO group 1 pulmonary arterial hypertension (PAH) is challenging. Separating the two conditions is of vital importance because treatment strategies differ completely. Furthermore, therapies that are indicated for WHO group 1 PAH may be harmful in patients with WHO group 2 PH. We review the somewhat confusing PH nomenclature and the WHO classification system and rationale behind it. We then focus on left-side heart disorders that cause PH. An aging population and advances in the medical management of common cardiovascular disorders have caused the prevalence of heart failure to rise significantly, with more than one-half of patients having HFpEF. We review contemporary studies that focus on clinical and echocardiographic findings that help to distinguish HFpEF from PAH in the patient with PH. We discuss the typical, and sometimes atypical, hemodynamic profiles that characterize these two groups, review challenges in the interpretation of data obtained by right-sided heart catheterization, and highlight special maneuvers that may be required for accurate diagnosis. Finally, we review the largely disappointing studies on the use of PAH-specific therapies in patients with WHO group 2 PH, including the use of prostacyclins, endothelin receptor antagonists, and the more promising phosphodiesterase-5 inhibitors.

Objectives Inferior vena caval tumor thrombus (IVC-TT) occurs in 10% of patients diagnosed with renal cell carcinoma (RCC). The perioperative management of these patients remains challenging. Despite multiple publications outlining surgical approaches and outcomes there have been few studies detailing the best peri-operative management of patients with IVC-TT. Our goal was to define the optimal management of patients with RCC and IVC-TT. Materials and methods A review of all published literature regarding the management of RCC with IVC-TT was performed utilizing Pub Med and the Cochrane Database. Reviews were also made of all relevant literature regarding the need for cardiopulmonary bypass and recommendations regarding thrombus in any location in patients with malignancy. Specific items critically examined included: need for preoperative heart catheterization, need for anticoagulation and type of anticoagulation, need for additional studies such as lower extremity duplex or venogram, and indications for vena caval filter placement. The results were then presented to a multidisciplinary group made up of experts in the fields of Urology, Hematology, Oncology, Cardiothoracic Surgery, Interventional Radiology, and Pulmonary/Critical Care. Based on the available literature a best practice guidelines regarding the management of RCC with IVC-TT was established at our institution. Results Our institutional recommendations include (1) preoperative cardiac catheterization in all patients believed to require cardiopulmonary bypass for removal of the thrombus but only cardiac clearance for those who bypass is unlikely, (2) preoperative anticoagulation using a low molecular weight heparin such as enoxaparin unless contraindicated due to bleeding from the tumor or other contraindication, (3) avoidance of vena caval filters whenever possible is recommended due the potential for caval thrombosis and the difficulties they present during surgical resection. Conclusion This study identified the available literature on the management of IVC-TT in association with RCC and was carefully reviewed by a multidisciplinary team. As a result, we have established a set of practice guidelines at our institution to help optimally manage patients with renal cell carcinoma and an inferior venal caval thrombus.

Cardiac comorbidity is one of the most important prognostic factors in lung disease, especially in chronic obstructive pulmonary disease (COPD). The imaging techniques available for the study of this systemic manifestation concomitant with COPD include heart catheterization, transthoracic echocardiography, and magnetic resonance imaging. Multidetector computed tomography (MDCT) represents a significant advance in this field because it enables the acquisition of simultaneous studies of the cardiopulmonary anatomy that go beyond anatomic and morphologic analysis to include a functional approach to this condition. In this article, we review the practical aspects necessary to evaluate cardiac comorbidity in patients with COPD, both from the point of view of pulmonary hypertension and of the analysis of ventricular dysfunction and coronary heart disease.

Background Right-sided heart catheterization (RHC) and pulmonary digital subtraction angiography (PDSA) are the standard methods used in diagnosing suspected or definite chronic thromboembolic pulmonary hypertension (CTEPH). We studied the ability of 320-slice CT imaging to detect simultaneously chronic thromboembolic findings in the pulmonary arteries and pulmonary hemodynamics based on the curvature of the interventricular septum (IVS) in CTEPH. Methods Forty-four patients with high clinical suspicion of CTEPH underwent RHC, PDSA, and enhanced double-volume retrospective ECG-gated 320-slice CT scan. We measured the sensitivity and specificity of CT imaging to detect thrombi in the pulmonary arteries compared with PDSA. We also compared IVS bowing (expressed as curvature) measured on the short-axis cine heart image with pulmonary arterial pressure (PAP) obtained by RHC. Results Compared with PDSA, the sensitivity and specificity of CT imaging to detect chronic thromboembolic findings were 97.0% and 97.1% at the main/lobar level and 85.8% and 94.6% at the segmental level, respectively. The correlation coefficients of IVS curvature with systolic PAP and mean PAP were −0.79 ( P P Conclusions The use of 320-slice CT imaging allows for less invasive and simultaneous detection of thrombi and evaluation of pulmonary hemodynamics for the diagnostic work-up of CTEPH.

Background The Pulmonary Arterial Hypertension-Quality Enhancement Research Initiative (PAH-QuERI) was created to help clinicians to implement a guidelines-based approach to the diagnosis and management of pulmonary arterial hypertension (PAH). Patients with PAH represent a heterogeneous population, and physician evaluation and treatment paradigms may vary considerably. Methods Using an electronic data management system, participating physicians recorded data on diagnostic workup, disease management, and outcomes of patients with PAH. Queries were generated automatically following each follow-up visit if the tests recommended by the American College of Chest Physicians (ACCP) were not performed at least once. Results Of 791 patients enrolled in PAH-QuERI, 77% were women; 64% received a diagnosis > 3 months prior to enrollment; 9% were in New York Heart Association functional class I, 39% in II, 48% in III, and 5% in IV; and the median age was 55 years (interquartile range, 45-66 years). At enrollment, all ACCP-recommended tests had been performed in only 6% of patients. The automated program generated 1,530 reminders for 642 patients (81%) with validated enrollment data. The proportion of recommended tests performed was 91% for CBC count, 91% for liver function test, 50% for connective tissue disease screen, 29% for HIV screen, 88% for chest radiograph, 82% for ECG, 97% for two-dimensional echocardiogram, 83% for pulmonary function tests, 41% for oximetry, 57% for ventilation/perfusion scan, 79% for 6-min walk distance, and 90% for right-sided heart catheterization. Regarding management, 78% of patients were on disease-specific therapy, and the use of these therapies tended to increase with the functional disability of the patient. One hundred seventy patients were taking calcium channel blockers, 91 specifically for PAH. Only six of 91 patients (7%) who received calcium channel blockers specifically for PAH had met the current guideline for acute vasoreactivity. Conclusions When comparing reported clinical practice with ACCP guidelines-recommended strategies, a diagnostic care gap is apparent such that certain essential and recommended diagnostic tests may be underused despite the availability of detailed guidelines and reminders.

Heart valve disease and coronary heart disease are very prevalent in the general population and often coincide in the same patient. Cardiac computed tomography (CT) makes it possible to noninvasively rule out coronary disease before valve surgery and to potentially avoid invasive heart catheterization in 66---75% of patients. The same imaging test provides abundant anatomic and functional information that complements the information from echocardiography, making it possible to characterize the etiology of the valve disease and its repercussions on the heart and aorta, as well as to quantify the severity of disease affecting the valves of the left side of the heart. In this article, we describe the anatomy of the heart valves and the technical requisites of cardiac CT for the study of the valves. We go on to explore the usefulness of CT in the preop- erative study of the coronary arteries and in the morphological and functional characterization of valve disease, with special emphasize on the valves of the left side of the heart. © 2011 SERAM. Published by Elsevier Espana, S.L. All rights reserved.

Background The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry) is a multicenter, US-based, observational study of patients diagnosed with group 1 pulmonary hypertension enrolled consecutively from March 2006 to December 2009. Of 3,128 patients in this analysis, inclusion criteria permitted enrollment of 268 patients with mean pulmonary capillary wedge pressure (PCWP) 16 to 18 mm Hg at diagnostic right-sided heart catheterization (RHC) (above currently accepted pulmonary arterial hypertension [PAH] diagnostic criteria). This study compared the demographics and outcomes of those 268 patients with an elevated mean PCWP to patients with a mean PCWP ≤ 15 mm Hg. Methods Demographic characteristics and outcomes were compared for patients with mean PCWP ≤ 12, 13 to 15, and 16 to 18 mm Hg at diagnostic and follow-up RHC. Results At diagnostic RHC, patients with PCWP 16 to18 mm Hg were older, had more severe hemodynamic impairments, and were more likely to be obese and have other comorbidities than patients with PCWP ≤ 15 mm Hg. There were no clinically relevant differences in 5-year survival rates from diagnostic RHC regardless of PCWP at diagnosis (≤15 mm Hg vs 16-18 mm Hg, P = .07). Two-year survival rates of 108 patients with PAH whose PCWP increased to ≥ 19 mm Hg (regardless of PCWP at diagnosis) were significantly lower than that of patients with PAH with PCWP ≤ 18 mm Hg at subsequent RHC. Conclusion Patients with PCWP 16 to 18 mm Hg who were diagnosed and treated for PAH were older, heavier, and more likely to have comorbidities associated with left ventricular diastolic dysfunction at diagnosis than those with PCWP ≤ 15 mm Hg. Five-year survival rates were similarly low for all PCWP subgroups. Trial registry ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov

Background Pulmonary hypertension (PH) is a well-recognized complication of COPD. The impact of PH on exercise tolerance is largely unknown. We evaluated and compared the circulatory and ventilatory profiles during exercise in patients with COPD without PH, with moderate PH, and with severe PH. Methods Forty-seven patients, GOLD (Global Initiative for Chronic Obstructive Lung Disease) stages II to IV, underwent cardiopulmonary exercise testing and right-sided heart catheterization at rest and during exercise. Patients were divided into three groups based on mean pulmonary artery pressure (mPAP) at rest: no PH (mPAP, vo 2 ) was used for evaluating the circulatory reserve. Pa co 2 and the calculated breathing reserve were used for evaluation of the ventilatory reserve. Results Patients without PH (n = 24) had an end-exercise S vo 2 of 48% ± 9%, an increasing Pa co 2 with exercise, and a breathing reserve of 22% ± 20%. Patients with moderate PH (n = 14) had an exercise S vo 2 of 40% ± 8%, an increasing Pa co 2 , and a breathing reserve of 26% ± 15%. Patients with severe PH (n = 9) had a significantly lower end-exercise S vo 2 (30% ± 6%), a breathing reserve of 37% ± 11%, and an absence of Pa co 2 accumulation. Conclusion Patients with severe PH showed an exhausted circulatory reserve at the end of exercise. A profile of circulatory reserve in combination with ventilatory impairments was found in patients with COPD and moderate or no PH. The results suggest that pulmonary vasodilation might only improve exercise tolerance in patients with COPD and severe PH.

Background Precapillary pulmonary hypertension (PH) is a complication of pulmonary Langerhans cell histiocytosis (PLCH) associated with increased mortality. However, outcomes and efficacy of pulmonary arterial hypertension (PAH) therapies in patients with PH complicating PLCH (PLCH-PH) remain unknown. Methods Consecutive patients with PLCH with PH confirmed by right-sided heart catheterization were included in the study. Characteristics at baseline and during follow-up as well as survival were analyzed. Results Twenty-nine patients were studied. Baseline characteristics of patients with PLCH-PH were as follows: 83% of patients in World Health Organization (WHO) functional class III to IV, mean 6-min walk distance of 355 ± 95 m, mean pulmonary arterial pressure (mPAP) of 45 ± 14 mm Hg, cardiac index of 3.2 ± 0.9 L/min/m 2 , and pulmonary vascular resistance (PVR) of 555 ± 253 dyne/s/cm 5 . Use of PAH therapy in 12 patients was followed by an improvement in mPAP (56 ± 14 mm Hg and 45 ± 12 mm Hg, P = .03) and PVR (701 ± 239 dyne/s/cm 5 and 469 ± 210 dyne/s/cm 5 , P = .01) between baseline and follow-up evaluations. No significant oxygen worsening was observed in the treated group. The 1-, 3-, and 5-year survival estimates of the 29 patients were 96%, 92%, and 73%, respectively. Except a trend toward a better survival rate associated with the use of PAH therapy, WHO functional class was the only variable significantly associated with death. Conclusions In this group of patients, PAH therapies improved hemodynamics without oxygen worsening or pulmonary edema. WHO functional class was the only prognostic factor identified. Prospective clinical trials focusing on this population of patients are warranted.