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Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct form of pulmonary hypertension, uniquely characterized by pulmonary artery narrowing and occlusion from clot material. With advances in medical education and therapeutic options, awareness of CTEPH has grown significantly in recent years. The diagnostic evaluation remains complex, warranting an integrated assessment of history, physical exam, echocardiogram, chest imaging including computerized tomography with angiography, ventilation–perfusion scanning, right heart catheterization, catheter-based pulmonary angiography, and assessment for medical and mechanical CTEPH risk factors. The diagnostic evaluation of CTEPH is reviewed here.

Objectives The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors. Background MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown. Methods A retrospective chart review was conducted in patients referred to Temple University Hospital’s cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA. Results A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015). Conclusions MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.

Tumoral obstruction is a small, but broadly defined, category of pulmonary hypertension that encompasses microvascular tumor emboli, tumor thrombotic microangiopathy, and macrovascular tumor obstruction within the pulmonary circulation. We present 4 patients with solid tumors, severe pre-capillary pulmonary hypertension, right ventricular failure, and pulmonary veno-occlusive–like disease. (Level of Difficulty: Advanced.), Central Illustration

Background: Given the morbidity and mortality associated with pulmonary arterial hypertension (PAH), risk stratification approaches that guide therapeutic management have been previously employed. However, most patients remain in the intermediate-risk category despite initial therapy. Herein, we sought to determine whether echocardiographic parameters could improve the risk stratification of intermediate-risk patients. Methods: Prevalent PAH patients previously enrolled in observational studies at 3 pulmonary hypertension centers were included in this study. A validated PAH risk stratification approach was used to stratify patients into low-, intermediate-, and high-risk groups. Right ventricular echocardiographic parameters were used to further stratify intermediate-risk patients into intermediate-low- and intermediate-high-risk groups based on transplant-free survival. Results: From a total of 146 patients included in our study, 38 patients died over a median follow-up of 2.5 years. Patients with intermediate-/high-risk had worse echocardiographic parameters. Tricuspid annular plane systolic excursion (TAPSE) and the degree of tricuspid regurgitation (TR) were highly associated with survival (p < 0.01, p = 0.04, respectively) and were subsequently used to further stratify intermediate-risk patients. Among intermediate-risk patients, survival was worse for patients with TAPSE < 19 mm compared to those with TAPSE ≥ 19 mm (estimated one-year survival 74% vs. 96%, p < 0.01) and for patients with moderate/severe TR compared to those with no/trace/mild TR (estimated one-year survival 70% vs. 93%, p < 0.01). Furthermore, among intermediate-risk patients, those with both TAPSE < 19 mm and moderate/severe TR had an estimated one-year survival (56%) similar to that of high-risk patients (56%), and those with both TAPSE ≥ 19 mm and no/trace/mild TR had an estimated one-year survival (97%) similar to that of low-risk patients (95%). Conclusions: Echocardiography, a routinely performed, non-invasive imaging modality, plays a pivotal role in discriminating distinct survival phenotypes among prevalent intermediate-risk PAH patients using TAPSE and degree of TR. This can potentially help guide subsequent therapy.

Perioperative anticoagulation management for patients with heparin-induced thrombocytopenia requiring cardiopulmonary bypass and deep hypothermic circulatory arrest presents a clinical challenge. Alternative anticoagulants have been used but can cause significant postoperative bleeding. We report the successful use of cangrelor and heparin in a 30-year-old patient with severe heparin-induced thrombocytopenia undergoing urgent pulmonary thromboendarterectomy.

The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors.MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown.A retrospective chart review was conducted in patients referred to Temple University Hospital's cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA.A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015).MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.

A major outcome determinant in patients with precapillary pulmonary hypertension (PH) is right ventricular (RV) function. We studied the effect of ranolazine on RV function over 6 months using cardiovascular magnetic resonance (CMR) imaging in patients with precapillary PH (groups I, III, and IV).We enrolled patients with PH and RV dysfunction (CMR imaging ejection fraction [EF] of45%) in a longitudinal, randomized, double-blinded, placebo controlled, multicenter study of ranolazine treatment. All enrolled patients were on stable PH-specific therapy. Enrolled patients were assessed using CMR imaging, New York Heart Association functional class, N-terminal pro brain natriuretic peptide, 6-minute walk test, and quality of life health outcomes at baseline and repeated at the end of treatment. The primary outcome was change in RVEF after 6 months of treatment. Analysis of covariance was used to analyze the longitudinal changes taking into account baseline values, age, and sex, based on per protocol population. Twenty-two patients were enrolled, and 9 patients completed follow-up CMR imaging after ranolazine treatment and 6 completed placebo treatment. There was significant increase in RVEF at end of treatment compared with baseline in the ranolazine group adjusted for baseline values, age, and sex. There were no statistically significant changes in secondary outcomes such as changes in New York Heart Association functional class, 6-minute walk distance, N-terminal pro brain natriuretic peptide, or quality of life measures. Ranolazine treated patients experienced a higher number of adverse events, but only one was discontinued owing to side effects.Ranolazine may improve RV function in patients with precapillary PH. Larger studies are needed to confirm the beneficial effects of ranolazine.

Patient: Female, 58-year-old Final Diagnosis: Tumor embolism Symptoms: Dyspnea Medication:— Clinical Procedure: Percutaneous embolectomy • surgical embolectomy Specialty: Cardiac surgery • Cardiology • Critical Care Medicine • Oncology Objective: Rare disease Background: Tumor embolism is a rare neoplastic complication that occurs when there is intravenous invasion by a benign or malignant tumor. We present the case of an asymptomatic patient with an incidentally discovered leiomyosarcoma tumor emboli, which was initially misdiagnosed as “thrombus in transit.” Case Report: The patient was a 58-year-old woman who was incidentally found on echocardiogram to have a large tubular mass within the inferior vena cava and right atrium. Although initially characterized as “thrombus in transit”, this mobile right atrial mass was present without clinical, echocardiographic, or radiographic evidence of pulmonary embolism or increased pulmonary arterial impedance. Given that a thrombus in transit is nearly always associated with submassive or massive pulmonary emboli and their attendant right heart sequelae, these pertinent negative findings led us to seek an alternative diagnosis. After a trial of conservative management with anticoagulation and attempted removal of the mass with the AngioVac system, the patient ultimately underwent median sternotomy and surgical embolectomy on cardiopulmonary bypass to remove the mass, which was later identified on pathology as a leiomyosarcoma. Conclusions: With rare exceptions, “thrombus in transit” is accompanied by large pulmonary emboli and the presence of increased pulmonary artery pressure and right heart strain. The absence of clinical, echocardiographic, or radio-graphic evidence of these hemodynamic sequelae should raise suspicion for an alternative diagnosis. Tumor embolism should be considered in the differential diagnosis of any patient with a history of malignancy who presents with evidence of intracardiac mass or embolism.

Question addressedEchocardiography is not currently considered as providing sufficient prognostic information to serve as an integral part of treatment goals in pulmonary arterial hypertension (PAH). We tested the hypothesis that incorporation of multiple parameters reflecting right heart function would improve the prognostic value of this imaging modality.Methods and main resultsWe pooled individual patient data from a total of 517 patients (mean age 52±15 years, 64.8% females) included in seven observational studies conducted at five European and United States academic centres. Patients were subdivided into three groups representing progressive degrees of right ventricular dysfunction based on a combination of echocardiographic measurements, as follows. Group 1 (low risk): normal tricuspid annular plane systolic excursion (TAPSE) and nonsignificant tricuspid regurgitation (TR) (n=129); group 2 (intermediate risk): normal TAPSE and significant TR or impaired TAPSE and nondilated inferior vena cava (IVC) (n=256); group 3 (high risk): impaired TAPSE and dilated IVC (n=132). The 5-year cumulative survival rate was 82% in group 1, 63% in group 2 and 43% in group 3. Low-risk patients had better survival rates than intermediate-risk patients (log-rank Chi-squared 12.25; pAnswer to the questionThe proposed echocardiographic approach integrating the evaluation of TAPSE, TR grade and IVC is effective in stratifying the risk for all-cause mortality in PAH patients, outperforming the prognostic parameters suggested by current guidelines.

Background: Significant pulmonary hypertension [PH] is often considered a contraindication for closure of atrial septal defects [ASD]. Treatment of pulmonary arterial hypertension [PAH] with selective pulmonary vasodilators can allow for surgical repair of ASD otherwise considered prohibitive. We describe our experience in the assessment and treatment of adults with ASD and PAH with pulmonary vasodilator therapy who underwent successful surgical repair. Methods: Retrospective case series of patients with atrial level shunts and significant PAH who met clinical criteria for repair. Clinical and demographic data were collected through review of the electronic medical records. Echocardiographic data were analyzed by a single cardiologist. Results: Four patients with ASD and PAH were identified. All four patients experienced a decrease in pulmonary vascular resistance and increase in shunt fraction with institution of PH-directed therapy. Each underwent successful surgical repair with improvements in six-minute walk distance, right ventricular systolic function, and natriuretic peptide levels. All patients remained improved and stable on PH medications at last follow-up. Conclusion: With appropriate selection and application of therapy, patients with atrial level shunts and severe PAH can successfully undergo surgical closure following medical treatment of their pulmonary arterial hypertension.

Uterine fibroids have been described as an associate to acute venous thromboembolism (VTE), with case reports showing an association between large uterine fibroids, acute deep venous thrombosis (DVT), and acute pulmonary embolism (PE). However, there is little known about the association or causation between uterine fibroids, chronic thromboembolic disease (CTED), and chronic thromboembolic pulmonary hypertension (CTEPH). We report on six women with uterine fibroids and CTEPH, as well as one woman with CTED, all of whom presented with exertional dyspnea, lower extremity swelling, and in the cases of CTEPH, clinical, echocardiographic, and hemodynamic evidence of pulmonary hypertension and right heart failure. Compression of the pelvic veins by fibroids was directly observed with invasive venography or contrast-enhanced computed tomography in five cases. All seven women underwent pulmonary thromboendarterectomy (PTE) followed by marked improvement in functional, clinical, and hemodynamic status.

Background The aim of study was to assess whether a specific morphology of the right ventricle (RV) by 2D echo predicts the hemodynamic nature of pulmonary hypertension (PH). Methods We reviewed clinical, 2D echo, and hemodynamic data of 100 patients with PH: divided into three groups: PH from pulmonary vascular disease (PHPVD ; n = 34) with pulmonary vascular resistance (PVR) > 3 mm Hg/L/min (Wood unit [WU]) and pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg, pulmonary venous hypertension (PVH; n = 33) with PVR 15 mm Hg and PHMIXED (n = 33) with PVR > 3 WU and PAWP > 15 mm Hg. We analyzed several two-dimensional parameters of right heart morphology and function, including the degree of tapering of the RV diameter from base (just above tricuspid annulus) to apex (level of moderator band) in the apical four-chamber view. P = Results Baseline characteristics were similar in all three groups: age 62 ± 14.4 years, 69% females, 57% Caucasians. Hemodynamics and 2D echo data of PHPVD vs PVH vs PHMIXED were as follows: PVR 13 ± 6 vs 2 ± 1 vs 7 ± 2 WU, mean pulmonary artery pressure 53 ± 14 vs 34 ± 8 vs 49 ± 8 mm Hg and cardiac index 2.0 ± 0.5 vs 2.8 ± 0.7 vs 2.2 ± 0.7 L/m2 , RV base/apex ratio during systole (sRVb/a ) 1.3 ± 0.2 vs 2.6 ± 0.5 vs 1.5 ± 0.3. Thus, sRVb/a was twofold higher in the PVH vs PHPVD cohort. On ROC analysis, the AUC for sRVb/a for predicting PVR > 3 WU was 0.873, with optimal cutoff of 1.5. Conclusion Systolic RV base/apex ratio is a simple 2D index of RV shape that powerfully predicts a PVR > 3 WU and provides powerful discriminating ability between PVH and PHPVD .

Few studies have examined the utility of serial echocardiography in the evaluation, management, and prognosis of patients with pulmonary arterial hypertension (PAH). Therefore, we sought to evaluate the prognostic significance of follow-up tricuspid annular plane systolic excursion (TAPSE) in PAH. We prospectively studied 70 consecutive patients with PAH who underwent baseline right heart catheterization (RHC) and transthoracic echocardiogram, who survived to follow-up echocardiogram after initiation of PAH therapy. Baseline TAPSE was 1.6 ± 0.5 cm which increased to 2.0 ± 0.4 cm on follow-up (P

The transition from acute to chronic thromboembolic disease can occur to an unpredictable degree in patients who survive an acute pulmonary embolism. In the vast majority of such patients on anti-thrombotic therapy, near complete resolution of the thrombus occurs. In 2–3% of patients, chronic thromboembolic pulmonary hypertension will develop. However, in an unknown percentage of pulmonary embolic survivors, organized thrombotic residua can involve enough of the pulmonary vascular bed to cause cardiopulmonary symptoms in the absence of pulmonary hypertension or right heart dysfunction. This is referred to as chronic thromboembolic disease or CTED, and the case presentation to follow illustrates some of challenges in treatment recommendations for this unique disease.

Pulmonary arterial compliance (PAC), invasively assessed by the ratio of stroke volume to pulmonary arterial (PA) pulse pressure, is a sensitive marker of right ventricular (RV)-PA coupling that differs across the spectrum of pulmonary hypertension (PH) and is predictive of outcomes. We assessed whether the echocardiographically derived ratio of RV outflow tract velocity time integral to PA systolic pressure (RVOT-VTI/PASP) (a) correlates with invasive PAC, (b) discriminates heart failure with preserved ejection-associated PH (HFpEF-PH) from pulmonary arterial hypertension (PAH), and (c) is associated with functional capacity. We performed a retrospective cohort study of patients with PAH (n = 70) and HFpEF-PH (n = 86), which was further dichotomized by diastolic pressure gradient (DPG) into isolated post-capillary PH (DPG

A 66-year-old man with a medical history significant for multiple pulmonary embolism and morbid obesity was evaluated for chronic thromboembolic pulmonary hypertension. Echocardiogram and right heart catheterisation were significant for severe pulmonary hypertension. Therefore, he was started on pulmonary hypertension medical therapy with riociguat and ambrisentan, in addition to anticoagulation. He experienced a dramatic clinical response to medical therapy. Despite haemodynamic improvement, the patient remained symptomatic with significant fatigue, exertional dyspnea and poor functional status as highlighted by a 6 min walk distance of only 128 m. Patient was referred for bariatric surgery with a gastric sleeve, after which he successfully lost 95 lbs in 6 months. Postoperative right heart catheterisation demonstrated normal pulmonary vascular resistance and cardiac output. His echocardiogram revealed normal right ventricular size and function. His 6 min walk distance also nearly quadrupled from 128 to 512 m, consistent with WHO Functional Class I.

Total right heart function requires normal function of both the right ventricle and the right atrium. However, the degree to which right atrial (RA) function and right ventricular (RV) function each contribute to total right heart function has not been quantified. In this study, we aimed to quantify the contribution of RA function to total right heart function in a group of pulmonary arterial hypertension (PAH) patients compared to a cohort of normal controls without cardiovascular disease. The normal cohort comprised 35 subjects with normal clinical echocardiograms, while the PAH cohort included 37 patients, of whom 31 had echocardiograms before and after initiation of PAH-specific therapy. Total right heart function was measured via tricuspid annular plane excursion (TAPSE). TAPSE was broken down into two components, the excursion occurring during RA contraction (TAPSERA) and that occurring before RA contraction (TAPSERV). RA fractional area change (RA-FAC) was also compared between the two groups. In the PAH cohort, more than half of the total TAPSE occurred during atrial systole, compared to less than one-third in the normal cohort (51.0% vs. 32.1%; P < 0.0001). There was a significant correlation between RA-FAC and TAPSE in the PAH cohort but not in the normal cohort. TAPSE improved significantly in the posttreatment cohort (1.7 vs. 2.1 cm), but TAPSERA continued to account for about half of the total TAPSE after treatment. RA function accounts for a significantly greater proportion of total right heart function in patients with PAH than in normal subjects.

Right ventricular function (RVF) carries great prognostic significance in heart failure and pulmonary hypertension (PH). Although there is considerable focus on RVF in pulmonary arterial hypertension, RVF is also of great importance in group 2 PH. This article will discuss assessment of RVF and evaluation of the Right Ventricle-Pulmonary Artery (RV-PA) coupling relationship. Cardiac imaging modalities allow direct visualization and assessment of RVF. Imaging modalities include the commonly utilized echo-Doppler imaging evaluating RV fractional area change, tricuspid annular plane systolic excursion and Tissue Doppler Imaging, in addition to the increasingly utilized cardiac magnetic resonance. Invasive hemodynamic assessment also plays an important role and can also be employed during exercise to help elucidate functional reserve. Cardiopulmonary exercise testing provides added insight into the mechanisms of cardiopulmonary disease. Cardiac imaging, invasive hemodynamics, and gas exchange stress testing can be combined to give a more sophisticated understanding of RVF. The RV-PA coupling relationship can be assessed using practical and clinically available metrics in order to gain clinically relevant understanding of the patients' physiologic state. RV-PA coupling assessments can be done using invasive, combined noninvasive-invasive, or non-invasive approaches. We also discuss our approaches in the assessment of the RV-PA coupling relationship.

Background Tricuspid regurgitation (TR) is an under treated disease. Although surgery for TR remains an effective therapy, many patients are considered to be at a high risk or otherwise inoperable. Caval valve implant (CAVI) offers an alternative to surgery in these patients. Trials assessing the safety and efficacy of caval valve implant are lacking. Methods: The Heterotopic Implantation Of the Edwards-Sapien XT Transcatheter Valve in the Inferior VEna cava for the treatment of severe Tricuspid Regurgitation (HOVER) trial is an FDA approved, physician initiated, prospective, non-blinded (open label), non-randomized safety and feasibility study to determine the safety and efficacy of the heterotopic implantation of the Edwards-Sapien XT valve in the inferior vena cava for the treatment of severe TR in patients who are at high risk or inoperable. Patients with severe TR in the absence of severe pulmonary hypertension will be recruited. They will be evaluated by a multi-disciplinary team who will agree by consensus that the patients’ symptoms are from TR. They will undergo imaging to assess the size of the inferior vena cava (IVC) to determine feasibility of the procedure. If patients meet the inclusion criteria and are free from exclusion criteria, after informed consent they will be eligible for enrollment in the study. A total of 30 patients will be enrolled. The primary objective of the study will be to demonstrate procedural success at 30-days and patient success at 1-year. Conclusion: Caval valve implant may present an alternative for patients who are at high risk or inoperable for tricuspid valve surgery (TVS) for TR. © 2016 Wiley Periodicals, Inc.

Background Post-operative atrial arrhythmias (POAA) after cardiac surgery are common and known to prolong hospital length of stay (LOS), but have been reported grouping atrial fibrillation (POAF), atrial flutters (AFL), and atrial tachycardias (AT). The impact of POAA vs. specifically POAF on intensive care unit time (ICUT) and hospital LOS after pulmonary thromboendarterectomy surgery (PTE) remains unknown. Objective To determine the impact of POAA and POAF on ICUT and hospital LOS after PTE. Method We identified consecutive patients undergoing PTE at our medical center from June 2013 to March 2020. After assessing baseline patient characteristics, we compared ICUT and LOS in patients with and without POAA and POAF after PTE. Results A total of 224 patients underwent PTE. POAA occurred in 31% (n = 68), of which 71% (n = 48) were POAF. Patients who developed POAA were older on average (63.5 vs. 51.5 years, p Conclusion In an adjusted analysis, in patients undergoing PTE, neither development of POAA nor specifically POAF were associated with increased ICUT. However, both POAA and POAF were associated with longer hospital LOS, denoting that POAA and POAF prolong hospitalization outside the ICU.

Introduction: Pulmonary arterial hypertension (PAH) is defined by elevation in PA pressure (PAP) and pulmonary vascular resistance (PVR) resulting in right ventricular (RV) failure and is associated with significant morbidity and mortality. Prior studies have evaluated invasive hemodynamic and biomarker parameters of PAH progression and prognostication, including the prognostic role of ST2, a member of the IL-1 receptor family, which is upregulated in cardiopulmonary disease, including PAH. Hypothesis: Serum ST2 (sST2), is upregulated and prognostic of transplant free survival in PAH in 2 independent cohorts and is associated with parameters of RV function. Methods: The primary cohort comprised 191 largely prevalent PAH patients from the University of California San Francisco (UCSF) with 41 largely incident PAH patients from the University of Pennsylvania (Penn) in the validation cohort. The primary outcome was transplant free survival. The Presage high sensitivity ELISA was used to measure sST2. Results: The UCSF cohort was 72% female, mean age 50±15 years, 25% idiopathic & 25% connective tissue disease-PAH (CTD-PAH). Those with sST2 > median (32.5 (21.9, 53.0) U/ml) had decreased semi-quantitative RV function (p=0.0003) by echo and increased right atrial pressure ([RAP]; 11.5 vs. 8.0 mmHg, p=0.003) and decreased cardiac index ([CI]; 2.23 vs. 2.45 L/min/m2, p=0.04), vs. sST2 ≤ median. Patients with sST2 ≤ median had significantly increased survival compared to those with sST2 > median (figure). The Penn cohort was 70% female, mean age 56±11 years, 54% idiopathic & 26% CTD-PAH. Median sT2 was 34 (21.7, 45.5) U/ml with those > median having significantly increased RAP (10.5 vs. 6.2 mmHg), mean PAP (53.3 vs. 39.8 mmHg) and PVR (12.7 vs. 6.0 WU), and lower CI (2.21 vs. 3.05 L/min/m2) (all p-values median (figure). Conclusions: In 2 independent PAH cohorts, sST2 is predictive of survival and serves as a marker of invasive parameters of RV failure. That sST2 is associated with differences in mean PAP and PVR in the largely incident Penn cohort, as compared to the prevalent UCSF cohort, warrants further study, as does the mechanism behind the robust association of ST2 and PAH.

Right ventricular (RV) end-diastolic volume (EDV) to left ventricular (LV) EDV ratio using cardiovascular magnetic resonance imaging (CMR) is an important parameter for RV size evaluation in additional to indexed EDV. We explore the severity partition for RV dilation using mortality in a population of 62 patients with pulmonary hypertension (PH). Cine short-axis images were acquired with a 1.5 T MR scanner using a steady-state free precession sequence. The optimal cutoff to classify severe RV dilation was determined by a receiver-operating curve (ROC) analysis based on mortality. We further defined mild and moderate categories by the standard deviation distance between normal and severely dilated and found the categories RV dilation by RV/LV volume ratio to be “mild” (1.27–1.69), “moderate” (1.70–2.29) and “severe” (≥2.30). There were significant differences in RVEDV and RV ejection fraction between “mild”, “moderate” and “severe” groups (p

Background Outcomes in peripartum cardiomyopathy ( PPCM ) vary. We sought to determine whether severity of left or right ventricular dysfunction (RVD) at PPCM diagnosis differentially associates with adverse outcomes. Methods and Results We conducted a single‐center retrospective cohort study of 53 patients with PPCM. The primary outcome was a composite of left ventricular assist device implantation, cardiac transplantation, or death. We used Kaplan‐Meier curves to examine event‐free survival and Cox proportional hazards models to examine associations of left ventricular (LV) ejection fraction RVD at PPCM diagnosis with the primary outcome. Median ( interquartile range ) follow‐up time was 3.6 (1.4–7.3) years. Seventeen patients (32%) experienced the primary outcome, of whom 11 had moderate‐to‐severe RVD at time of PPCM diagnosis. Overall event‐free survival differed by initial RVD severity and LV ejection fraction end‐diastolic diameter ≥60 mm. In univariable analyses, LV ejection fraction RVD were associated with the outcome (hazard ratios [95% confidence intervals] of 4.85 [1.11–21.3] and 4.26 [1.47–11.6], respectively). In a multivariable model with LV ejection fraction LV end‐diastolic diameter ≥60 mm, and moderate‐to‐severe RVD , only moderate‐to‐severe RVD was independently associated with the outcome (hazard ratio [95% confidence interval ] , 3.21 [1.13–9.10]). Although most outcomes occurred within the first year, nearly a third occurred years after PPCM diagnosis. Conclusions Initial moderate‐to‐severe RVD is associated with a more advanced cardiomyopathy phenotype and increased risk of adverse outcomes in PPCM , within and beyond the first year of diagnosis. By identifying a worse PPCM phenotype, initial moderate‐to‐severe RVD may prompt earlier consideration of advanced heart replacement therapies.

To determine the normal range of the ratio of right ventricular (RV) end-diastolic volume to left ventricular (LV) end-diastolic volume by magnetic resonance imaging (MRI) and examine whether combining this volume ratio with RVEDV indexed to body surface area (RVEDVi) increased the detection of RV dilation in patients with pulmonary arterial hypertension (PAH).MRI-derived ventricular function and volumes were measured in a control group (n = 152) and in patients with PAH (n = 46). Images were acquired with a 1.5T Siemens or a 1.5T Philips scanner using a steady-state free procession sequence. Proposed criteria for the detection of RV enlargement, including RVEDVi alone, RV/LV volume ratio alone, and combining both criteria, were evaluated in both groups.The range (mean ± 2 standard deviations) for the volume ratio in the normal population was found to be 0.906-1.266; there was no difference between genders (P = 0.70). Combining this ratio with RVEDVi detected RV enlargement in 21.7% (P 104 mL/m(2) for females and >113 mL/m(2) for males).Combining RV/LV volume ratio with indexed RVEDV increased detection of RV enlargement in a PAH population. This result may have potential impact in RV size assessment. J. Magn. Reson. Imaging 2016;43:1379-1385.

Objective Determination of clinical outcomes following resuscitation from cardiac arrest remains elusive in the immediate post-arrest period. Echocardiographic assessment shortly after resuscitation has largely focused on left ventricular (LV) function. We aimed to determine whether post-arrest right ventricular (RV) dysfunction predicts worse survival and poor neurologic outcome in cardiac arrest patients, independent of LV dysfunction. Methods A single-center, retrospective cohort study at a tertiary care university hospital participating in the Penn Alliance for Therapeutic Hypothermia (PATH) Registry between 2000 and 2012. Patients 291 in- and out-of-hospital adult cardiac arrest patients at the University of Pennsylvania who had return of spontaneous circulation (ROSC) and post-arrest echocardiograms. Measurements and main results Of the 291 patients, 57% were male, with a mean age of 59±16 years. 179 (63%) patients had LV dysfunction, 173 (59%) had RV dysfunction, and 124 (44%) had biventricular dysfunction on the initial post-arrest echocardiogram. Independent of LV function, RV dysfunction was predictive of worse survival (mild or moderate: OR 0.51, CI 0.26–0.99, p p =0.008) and neurologic outcome (mild or moderate: OR 0.33, CI 0.17–0.65, p =0.001; severe: OR 0.11, CI 0.02–0.50, p =0.005) compared to patients with normal RV function after cardiac arrest. Conclusions Echocardiographic findings of post-arrest RV dysfunction were equally prevalent as LV dysfunction. RV dysfunction was significantly predictive of worse outcomes in post-arrest patients after accounting for LV dysfunction. Post-arrest RV dysfunction may be useful for risk stratification and management in this high-mortality population.

Pulmonary hypertension, which is related to right ventricular (RV) failure, indicates a poor prognosis in heart failure (HF). Increased ventilatory response and exercise oscillatory ventilation (EOV) also have a negative impact. We hypothesized that the severity classification of HF and risk prediction could be improved by combining functional capacity with cardiopulmonary exercise testing (CPET) and RV-pulmonary circulation coupling, as evaluated by the tricuspid annular plane systolic excursion (TAPSE)-pulmonary artery systolic pressure (PASP) relationship.Four hundred fifty-nine patients with HF were assessed with Doppler echocardiography and CPET and were tracked for outcome. The subjects were followed for major cardiac events (cardiac mortality, left ventricular assist device implant, or heart transplant). Cox regression and Kaplan-Meier analyses were performed with TAPSE and PASP as individual measures that were then combined into a ratio form.The TAPSE/PASP ratio (TAPSE/PASP) was the strongest predictor, whereas the New York Heart Association classification and EOV added predictive value. A four-quadrant group prediction risk was created based on TAPSE (16 mm or ≥ 16 mm) vs PASP (40 mm Hg or ≥ 40 mm Hg) thresholds and the CPET variables distribution as follows: group A (TAPSE16 mm and PASP40 mm Hg) presented the lowest risk (hazard ratio, 0.17) and best ventilation; group B exhibited a low risk (hazard ratio, 0.88) with depressed TAPSE (16 mm) and normal PASP, a preserved peak oxygen consumption (V.o2), but high ventilation. Group C had an increased risk (hazard ratio, 1.3; TAPSE ≥ 16 mm, PASP ≥ 40 mm Hg), a reduced peak V.o2, and a high EOV prevalence. Group D had the highest risk (hazard ratio, 5.6), the worse RV-pulmonary pressure coupling (TAPSE16 and PASP ≥ 40 mm Hg), the lowest peak V.o2, and the highest EOV rate.TAPSE/PASP, combined with exercise ventilation, provides relevant clinical and prognostic insights into HF. A low TAPSE/PASP with EOV identifies patients at a particularly high risk of cardiac events.

Patients with end-stage renal disease (ESRD) with arteriovenous dialysis access (AVDA) can develop symptoms of heart failure and pulmonary hypertension (PH). We report on 5 patients with ESRD and AVDA who presented with shortness of breath, heart failure, and PH. All patients had partial or complete closure of AVDA and were reevaluated after AVDA revision. All 5 subjects had clinical and echocardiographic evidence of heart failure, hypertensive heart disease, left ventricular diastolic dysfunction, and PH at baseline. After complete closure ([Formula: see text]) or partial banding ([Formula: see text]) of AVDA, mean New York Heart Association class improved from [Formula: see text] to [Formula: see text] ([Formula: see text]). Mean 6-minute walk distance improved from [Formula: see text] to [Formula: see text] m ([Formula: see text]). Serial echocardiography revealed a decrease in the right ventricle∶left ventricle ratio from [Formula: see text] to [Formula: see text] ([Formula: see text]) and improved diastolic dysfunction parameters. On right heart catheterization before definitive AVDA revision, acute manual fistula or graft occlusion led to an average decrease in cardiac output of 1.1 L/min with no other changes in hemodynamics: [Formula: see text] to [Formula: see text] L/min ([Formula: see text]). However, the average decrease in cardiac output after definitive revision of the AVDA (mean, 90 days) was 4.0 L/min with marked improvements in biventricular filling pressures and pulmonary artery pressure. In patients with ESRD and AVDA presenting with heart failure and PH, revision or closure of AVDA can markedly improve dyspnea as well as the clinical, echocardiographic, and hemodynamic manifestations of heart failure and PH.

Background Pulmonary thromboendarterectomy (PTE) is the treatment of choice for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, access to CTEPH and PTE care is limited. There is a paucity of published data on PTE efficacy and outcomes from alternative, regional centers of excellence in CTEPH and PTE care in the USA, outside a single national and international referral center. Methods We performed a retrospective review of patients undergoing PTE at our institution from June 2013 to December 2016 (42 months), and collected clinical, echocardiographic and hemodynamic data on our patients pre- and post-PTE (N.=71). Results Patients age ranged between 20-83 years (mean±SD: 56±16), with 54% of patients female and 61% Caucasians. The predominant symptom was shortness of breath with a median duration of symptoms of 17 months. Following PTE, clinical improvements included a reduction in NYHA class from 3.1±1.1 to 2.2±1.2. There were major improvements in hemodynamics and echocardiographic parameters pre- versus post-PTE: mean pulmonary artery pressure (mmHg) 45±11 to 24±8, cardiac index (L/min/m2) 2.1±0.5 to 2.8±0.5, pulmonary vascular resistance (mmHg/L/min) 8.9±4.5 to 2.8±1.8, ratio of right ventricle (RV): left ventricle (LV) 1.2±0.3 to 0.9±0.2, RV fractional area change (%) 23±14 to 44±13, reduction in the incidence of RV outflow tract Doppler notching and improved pulmonary artery acceleration time (96% to 30%, and 74±19 to 111±21). In-hospital mortality was 4.2% (3 patients). Conclusions Herein, we report for the first time, the improvements in patient functionality, hemodynamics, right heart function and outcomes at a major regional PTE program.

Several studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (Tp-e) may correspond to the transmural dispersion of repolarization and that increased Tp-e interval and Tp-e/QT ratio are associated with malignant ventricular arrhythmias. The aim of this study was to evaluate ventricular repolarization by using the Tp-e interval and Tp-e/QT ratio in patients with rheumatoid arthritis (RA), and to assess the relation with inflammation.Ninety-six patients (72 females, 24 males; mean age 43.8±11.8 years) with RA and 50 controls (35 females, 15 males; mean age 44.2±11.1 years) were included. From the 12-lead electrocardiogram, Tp-e interval and Tp-e/QT ratio were measured. Blood samples were taken for erythrocyte sedimentation rate (ESR) and plasma levels of C-reactive protein (CRP). These parameters were compared between groups. The relationship between ventricular repolarization and inflammation was assessed by Pearson correlation coefficients.Tp-e interval and Tp-e/QT ratio were increased in RA patients compared to the controls (72.6±8.2 vs 66.4±8.5 ms, 0.20±0.02 vs 0.18±0.02; p0.001 and p0.001, respectively). The Tp-e interval was significantly correlated with CRP, ESR, and disease activity score (DAS-28) (r=0.56, p0.001, r=0.57, p0.001, and r=0.29, p=0.02, respectively). The Tp-e/QT ratio was also correlated with CRP, ESR, and DAS-28 score (r=0.43, p0.001, r=0.53, p0.001, and r=0.25, p=0.03, respectively).In RA patients, the increased frequency of ventricular arrhythmias may be explained by increased indexes of ventricular repolarization and their relationship with inflammation.

Background Pulmonary hypertension (PH) in the setting of left heart disease (PH-LHD) is the most common cause of PH in the general population, with a prevalence of up to 80% and is associated with high morbidity and mortality. We have previously demonstrated the utility of right ventricular outflow tract (RVOT) Doppler notching as a composite marker of pulmonary vascular disease (PVD) in patients with PH-associated with heart failure with preserved ejection fraction (HFpEF). Our study examines the ability of RVOT Doppler notching to predict survival in patients with PH-HFpEF. Methods We retrospectively evaluated consecutive patients referred to the Penn PH program between January 2007 and March 2013 diagnosed with PH-HFpEF. Patients were included who had right heart catheterization with mean pulmonary artery pressure ≥ 25mmHg and pulmonary artery wedge pressure > 15 mmHg. All patients underwent echocardiographic and hemodynamic assessment as indicated clinically. Statistical analysis included student's t-test and chi-squared to assess continuous and categorical variables, respectively, along with use of Cox regression and the Kaplan-Meier estimator to evaluate survival. Results Of 86 patients, 53 had notching and 33 did not. Baseline characteristics were similar across both groups. Patients with notching had higher diastolic pulmonary gradient (8.2±6.4 vs 1.4±4.3, p Conclusions In PH-HFpEF, RVOT Doppler notching is a composite marker of PVD and is a strong predictor of long-term survival. Further work is needed to validate this marker in larger cohorts.

Objectives 1) Outline a case in which suspected PAH was falsely diagnosed and treated as pulmonary venous hypertension due to incorrect RHC data 2) Reinforce importance of standardized technique during RHC to ensure accurate hemodynamic data 3) Emphasize importance of reviewing source data when there is a discrepancy between diagnostic data and the clinical picture. A 59 year old female with scleroderma, non-ischemic cardiomyopathy (30-35% EF), GERD, and T2DM presented from an outside hospital for evaluation of chronic dyspnea. Initial RHC data (RHC 1) indicated pulmonary venous hypertension and cardiogenic shock. Diuretics, afterload reduction, and Milrinone were initiated. However, her echocardiogram showed an RV:LV ratio of 1.8 (Image 1) and right ventricular outflow tract (RVOT) doppler notching (Image 2), consistent with PAH physiology. Due to patient deterioration and a discrepancy between echocardiographic and hemodynamic data, a repeat RHC was performed. The results (RHC 2) were significantly different from prior and diagnostic of PAH, which was consistent with the initial echocardiographic findings and the clinical picture. Although RHC is the definitive diagnostic tool for diagnosing PAH, it is prone to operator discrepancies. The initial RHC data was incorrect due to under-occlusion, a phenomenon that can occur in patients with significant PAH due to enlargement of the proximal pulmonary artery. Tracings 1 and 2 show the wedge pressures from the initial and subsequent RHC, respectively. There is clearly a significant difference in the reported left sided filling pressure. Additionally, RHC 1 data shows the wedge pressure to be higher than the diastolic pulmonary artery pressure, indicating an erroneous wedge pressure due to partial or incomplete pulmonary artery occlusion. Many centers confirm wedge position by checking a wedge saturation. A standardized technique for performing RHC could help mitigate operator discrepancies. After starting Sildenafil for PAH, the patient began to improve and was eventually discharged home on Milrinone, Sildenafil, and oral diuretics. This case highlights the importance of reviewing all source data especially when there is discrepancy between different diagnostic data and the clinical picture.