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A right heart catheterization with measurements of pulmonary artery wedge pressure (PAWP) may be necessary for the diagnosis of left heart failure as a cause of pulmonary hypertension or unexplained dyspnea. Diagnostic cutoff values are a PAWP of ≥ 15 mm Hg at rest or a PAWP of ≥ 25 mm Hg during exercise. However, accurate measurement of PAWP can be challenging and heart failure may be occult. Left heart catheterization, with measurement of left ventricular end-diastolic pressure, may also be indecisive. Measurements are then best repeated in stress conditions. Exercise is an option, but the equipment is not universally available, and interpretation can be difficult in patients with wide respiratory pressure swings. An alternative is offered by a fluid challenge. Studies have gathered data supporting infusion of 500 mL or 7 mL/kg saline and a PAWP of 18 mm Hg as a diagnostic cutoff. The procedure is simple and does not take much catheterization laboratory time. Combining echocardiography with invasive measurements may increase the diagnostic accuracy of diastolic dysfunction. Cardiac output after a fluid challenge may be of prognostic relevance.

Aim: To investigate the prevalence and prognostic impact of right heart failure and right ventricular-arterial uncoupling in Corona Virus Infectious Disease 2019 (COVID-19) complicated by an Acute Respiratory Distress Syndrome (ARDS). Methods: Ninety-four consecutive patients (mean age 64 years) admitted for acute respiratory failure on COVID-19 were enrolled. Coupling of right ventricular function to the pulmonary circulation was evaluated by a comprehensive trans-thoracic echocardiography with focus on the tricuspid annular plane systolic excursion (TAPSE) to systolic pulmonary artery pressure (PASP) ratio Results: The majority of patients needed ventilatory support, which was noninvasive in 22 and invasive in 37. There were 25 deaths, all in the invasively ventilated patients. Survivors were younger (62 ± 13 vs. 68 ± 12 years, p = 0.033), less often overweight or usual smokers, had lower NT-proBNP and interleukin-6, and higher arterial partial pressure of oxygen (PaO2)/fraction of inspired O2 (FIO2) ratio (270 ± 104 vs. 117 ± 57 mmHg, p < 0.001). In the non-survivors, PASP was increased (42 ± 12 vs. 30 ± 7 mmHg, p < 0.001), while TAPSE was decreased (19 ± 4 vs. 25 ± 4 mm, p < 0.001). Accordingly, the TAPSE/PASP ratio was lower than in the survivors (0.51 ± 0.22 vs. 0.89 ± 0.29 mm/mmHg, p < 0.001). At univariate/multivariable analysis, the TAPSE/PASP (HR: 0.026; 95%CI 0.01–0.579; p: 0.019) and PaO2/FIO2 (HR: 0.988; 95%CI 0.988–0.998; p: 0.018) ratios were the only independent predictors of mortality, with ROC-determined cutoff values of 159 mmHg and 0.635 mm/mmHg, respectively. Conclusions: COVID-19 ARDS is associated with clinically relevant uncoupling of right ventricular function from the pulmonary circulation; bedside echocardiography of TAPSE/PASP adds to the prognostic relevance of PaO2/FIO2 in ARDS on COVID-19., SCOPUS: ar.j, info:eu-repo/semantics/published

Noninvasive estimation of systolic pulmonary artery pressure (SPAP) during exercise stress echocardiography (ESE) is recommended for pulmonary hemodynamics evaluation but remains flow-dependent. Our aim was to assess the feasibility of pulmonary vascular reserve index (PVRI) estimation during ESE combining SPAP with cardiac output (CO) or exercise-time and compare its value in three group of patients: with invasively confirmed pulmonary hypertension (PH), at risk of PH development (PH risk) mainly with systemic sclerosis and in controls (C) without clinical risk factors for PH, age-matched with PH risk patients. We performed semisupine ESE in 171 subjects: 31 PH, 61 PH at risk and 50 controls as well as in 29 young, healthy normals. Rest and stress assessment included: tricuspid regurgitant flow velocity (TRV), pulmonary acceleration time (ACT), CO (Doppler-estimated). SPAP was calculated from TRV or ACT when TRV was not available. We estimated PVRI based on CO (peak CO/SPAP*0.1) or exercise-time (ESE time/SPAP*0.1). During stress, TRV was measurable in 44% patients ACT in 77%, either one in 95%. PVRI was feasible in 65% subjects with CO and 95% with exercise-time (p

The recent outbreak of 2019 severe acute respiratory syndrome coronavirus-2 is having major repercussions on healthcare services provision in Italy and worldwide. Data suggest the virus has a strong impact on the cardiovascular system, and cardiac imaging will play an important role in patients affected by coronavirus disease-2019. Although paediatric patients are mildly affected, they represent a clear accelerator in spreading the virus, and healthcare workers are at higher risk of infection. The aim of this position paper is to provide clinical recommendation regarding the execution of imaging investigations for the cardiac diagnostic work-up of paediatric patients with suspected or confirmed infection.

Doppler echocardiography is extensively used in clinical practice for the screening and detection of pulmonary hypertension (PH). It allows for accurate estimates of pulmonary artery pressures, but with moderate precision, which explains why it is more appropriate for population studies than for definitive diagnosis of PH in individual patients. Moreover, echocardiography allows one to distinguish different patterns of right ventricular remodelling in various forms of PH and enables clinically satisfactory differentiation between pre- and post-capillary PH. This article will review the methods for evaluating PH by echocardiography, while also providing an insight into specific strengths and weaknesses.

Background Pulmonary hypertension (PH) is a well-known independent prognostic factor in chronic obstructive pulmonary disease (COPD) and a sufficient criterion for lung transplant candidacy. Limited data are currently available on the hemodynamic and clinical effect of phosphodiesterase 5 inhibitors in patients with severe PH associated with COPD. This study assessed the effect of sildenafil on pulmonary hemodynamics and gas exchange in severe PH associated with COPD. Methods After screening, this multicenter, randomized, placebo-controlled double-blind trial randomized patients to receive 20 mg sildenafil or placebo 3 times a day (ratio 2:1) for 16 weeks. The primary end point was the reduction in pulmonary vascular resistance. Secondary end points included BODE (body mass index, airflow obstruction, dyspnea, and exercise capacity) index, 6-minute walk test, and quality of life questionnaire. Changes in the partial pressure of arterial oxygen were evaluated as a safety parameter. Results The final population included 28 patients, 18 in the sildenafil group and 10 in the placebo group. At 16 week, patients treated with sildenafil had a decrease in pulmonary vascular resistance (mean difference with placebo –1.4 WU; 95% confidence interval, ≤ –0.05; p = 0.04). Sildenafil also improved the BODE index, diffusion capacity of the lung for carbon monoxide percentage, and quality of life. Change from baseline in the partial pressure of arterial oxygen was not significantly different between the sildenafil and placebo groups. Conclusions This pilot study found that treatment with sildenafil reduced pulmonary vascular resistance and improved the BODE index and quality of life, without a significant effect on gas exchange.

BRACKGROUND: The most important determinant of long-term survival in patients with idiopathic pulmonary fibrosis is the right ventricular (RV) adaptation to the increased pulmonary vascular resistance. Our aim was to explore RV contractile reserve during stress echocardiography in early-stage IPF.METHOD: Fifty early-stage patients with IPF and 50 healthy control patients underwent rest and stress echocardiography, including RV two-dimensional speckle tracking echocardiography. At peak exertion, blood gas analysis and spirometry were also assessed.RESULTS: At rest, RV diameters were mildly increased in IPF; however, although RV conventional systolic function indexes were similar between the IPF and control groups, RV global longitudinal strain and RV lateral wall longitudinal strain (LWLS) were significantly reduced in the IPF cohort. During physical exercise, patients with IPF showed a reduced exercise tolerance with lower maximal workload (P < .01), level of oxygen saturation (P < .001), and peak heart rate (P < .01). Systolic and diastolic BP values were similar in both groups. Systolic pulmonary artery pressure (PAPs) increase (Delta PAPs) during exertion was higher in IPF vs healthy subjects (P < .0001); RV LWLS increase (Delta RV LWLS) during exercise was lower in patients with IPF vs control patients (P < .00001). By multivariable analysis, RV LWLS at rest and Delta RV LWLS were directly related to peak exertion capacity, PAPs, and blood oxygen saturation level (Spo(2) ; P < .0001). Delta RV LWLS was directly related to diffusion lung carbon monoxide (P < .0001).CONCLUSION RV myocardial dysfunction is already present at rest in early-stage IPF and worsens during exertion as detected by two-dimensional speckle-tracking echocardiography. The RV altered contractile reserve appears to be related to reduced exercise tolerability and impaired pulmonary hemodynamic.

Transvenous pacemaker (PM) catheters can be unintentionally placed in the left ventricle (LV) during the implantation procedure. An 8-year-old girl was discovered with a malpositioned pm wire, seven years after the implant. Trans-thoracic echocardiogram revealed the lead traversing the inter-atrial septum, crossing the mitral valve and embedded in the basal lateral wall of the LV. This is a report of a 14-year long follow-up after the surgical extraction of the malpositioned PM lead. come easier. Although nowadays a transvenous lead can be placed in children, there is a greater risk of complication in small patients [3]. One extremely rare complication associated with transvenous PM implantation is the inadvertent positioning of pacing lead in the left ventricle [4]. The greater risks depend on the delay of diagnosis of lead malpositions. This report concerns a case of long standing malpositioned PM wire in a paediatric patient and the successive long term follow up after the successful surgical extraction.

The cardiopulmonary vascular system represents a key determinant of prognosis in several cardiorespiratory diseases. Although right heart catheterization is considered the gold standard for assessing pulmonary hemodynamics, a comprehensive noninvasive evaluation including left and right ventricular reserve and function and cardiopulmonary interactions remains highly attractive. Stress echocardiography is crucial in the evaluation of many cardiac conditions, typically coronary artery disease but also heart failure and valvular heart disease. In stress echocardiographic applications beyond coronary artery disease, the assessment of the cardiopulmonary vascular system is a cornerstone. The possibility of coupling the left and right ventricles with the pulmonary circuit during stress can provide significant insight into cardiopulmonary physiology in healthy and diseased subjects, can support the diagnosis of the etiology of pulmonary hypertension and other conditions, and can offer valuable prognostic information. In this state-of-the-art document, the topic of stress echocardiography applied to the cardiopulmonary vascular system is thoroughly addressed, from pathophysiology to different stress modalities and echocardiographic parameters, from clinical applications to limitations and future directions.

In early stage uncomplicated systemic hypertension (HT), increased pulmonary vascular resistance (PVR) has been reported at rest, but more rarely during exercise. Recently, limits of normal for stress echocardiography in the evaluation of the pulmonary circulation have been better defined. We therefore used this approach to assess the pulmonary circulation in early HT.One hundred thirteen patients with mild to moderate untreated, uncomplicated HT (blood pressure, 152 ± 19/89 ± 11 mm Hg, heart rate, 70 ± 13 beats per minute) and 345 age- and sex-matched healthy control subjects underwent resting Doppler echocardiography with estimation of mean pulmonary arterial pressure (mPAP), left atrial pressure (LAP), and cardiac output (CO). Measurements were repeated at exercise stress test in 25 patients from each group.At rest, hypertensive patients had normal right and left ventricular structure and function, higher systemic vascular resistance, mPAP (16 ± 5 vs 14 ± 5 mm Hg; P0.0001), and PVR (1.3 ± 1.1 vs 1.0 ± 1.2 Wood units; P = 0.006) than control participants, but similar LAP. During exercise, hypertensive patients showed a lower maximum workload and CO and higher peak mPAP than control subjects, but a similar increase in LAP. PVR determined according to multipoint mPAP-CO relationships was also higher in hypertensive patients than in control subjects (2.5 ± 1.1 vs 1.5 ± 0.7 mm Hg/L/min; P0.05), with no changes in pulmonary resistive vessel distensibility coefficient α (0.012 ± 0.007 vs 0.012 ± 0.010% change in diameter for each mm Hg increase in mPAP).Resting and exercise PVR are increased in uncomplicated HT, without this being related to increased pulmonary venous pressure or resistive vessel stiffness, suggesting an early increase in pulmonary vascular tone.

Background The differential diagnosis between pre- and postcapillary pulmonary hypertension (PH) is of major therapeutic relevance and thus requires optimal clinical probability assessment with echocardiography. Methods We prospectively analyzed 152 consecutive patients referred to a PH center over a 1-year period undergoing quasi-simultaneous (within 1 hour) echocardiography and right heart catheterization. Echocardiography was performed as usually recommended for the assessment of PH and left heart conditions. PH was defined as a mean pulmonary artery pressure ≥ 25 mm Hg. Postcapillary PH was diagnosed on the basis of a pulmonary capillary wedge pressure >15 mm Hg. Results Ten of 152 patients (7%) had no PH, 81 of 152 (53%) had precapillary PH, and 61 of 152 (40%) had postcapillary PH. The following five echocardiographic variables were found to predict precapillary PH: right heart chamber larger than the left ( P = .0018), left ventricular eccentricity index > 1.2 ( P = .0039), dilated inferior vena cava without inspiratory collapse ( P = .0076), E/e′ ratio ≤10 ( P = .00001), and the right ventricle forming the heart apex ( P = .0144). Beta coefficients from multiple logistic regression were significant for dilated inferior vena cava without inspiratory collapse ( P = .0464) and E/e′ ratio ≤ 10 ( P = .0002). The score based on β coefficients, ranging from 3 to 34 points, resulted in optimal discrimination at >5, with a positive predictive value of 67.9% and a negative predictive value of 77.5% for precapillary PH. Conclusion Echocardiography enables a clinically satisfactory differential diagnosis between pre- and postcapillary PH.

Aim: To determine whether right atrial (RA) function has prognostic value in patients with idiopathic pulmonary arterial hypertension (PAH).Methods and results: Overall, 104 patients (70 female, mean age 58 +/- 13 years) with idiopathic PAH underwent standard Doppler echocardiography and strain and strain rate (SR) analysis before right heart catheterization. At a mean follow-up of 22 +/- 7 months, 30 patients (29%) had clinical worsening. On Cox multivariable proportional-hazards regression analysis, RA reservoir function measured as peak longitudinal SR (hazard ratio [HR] 0.5; P < 0.0001), RA area (HR 1.2; P < 0.01), right ventricular (RV) SR (HR 0.6; P < 0.0001), cardiac index (HR 0.79; P < 0.01), and mixed venous oxygen saturation (HR 0.82; P < 0.01) were found to be independent correlates of cardiac events. A RA SR reservoir cut-off value of

AIMS A simple echocardiographic score was designed for diagnosing precapillary vs postcapillary pulmonary hypertension and for discriminating between isolated postcapillary pulmonary hypertension (Ipc-PH) and combined precapillary and postcapillary pulmonary hypertension (Cpc-PH). METHODS The score comprised 7 points (2 for E/e' ratio ≤10, 2 for a dilated non-collapsible inferior vena cava, 1 for a left ventricular eccentricity index ≥1.2, 1 for a right-to-left heart chamber dimension ratio >1 and 1 for the right ventricle forming the heart apex) and was applied to 230 consecutive patients referred for evaluation of pulmonary hypertension. RESULTS Precapillary pulmonary hypertension and postcapillary pulmonary hypertension were diagnosed in 160 and 70 patients, respectively. In the latter, Ipc-PH was found in 51 and Cpc-PH in 19. The echo score was higher in precapillary vs postcapillary pulmonary hypertension patients (4.2 ± 1.7 vs 1.6 ± 1.7, P

The aim of the study was to analyze possible correlations between strain echocardiography (STE) and PET myocardial perfusion in a population of heart transplantation (HTx) recipients showing preserved left ventricular (LV) ejection fraction. By STE, LV global longitudinal strain (LV GLS) was lower in HTx. PET showed no transient or chronic ischemia in 83 of 115 HTx (73%). Fixed perfusion defects were observed in 17% of HTx and reversible ischemia in 10%. Significant coronary stenosis was observed only in 10 cases. GLS was independently associated with age at HTx and fixed perfusion defects (HR 0.41; P

Background Fluid challenge may help in the differential diagnosis between pre- and postcapillary pulmonary hypertension (PH). However, the test is still in need of standardization and better defined clinical relevance. Methods Two hundred twelve patients referred for PH underwent a right-sided heart catheterization with measurements before and after rapid infusion of 7mL/kg of saline. PH was defined as mean pulmonary artery pressure≥ 25mmHg, and postcapillary PH was defined as pulmonary artery wedge pressure (PAWP) > 15mmHg. An increase in PAWP≥ 18mmHg was considered diagnostic for postcapillary PH. At baseline, 66 patients received a diagnosis of no PH; 22, of postcapillary PH; and 124, of precapillary PH (mostly pulmonary arterial hypertension). Results After fluid challenge, five of 66 patients with no PH (8%) and eight of 124 with precapillary PH (6%) had the diagnosis reclassified as postcapillary PH. Fluid challenge was associated with an increase in PAWP by 7 ± 2mmHg in postcapillary PH and 3 ± 1mmHg in both precapillary PH and no-PH groups. Between-group differences were significant, but there was overlap. There were no adverse events related to fluid challenge. Prediction bands calculated from quadratic fits of the PAWP responses in pooled control subjects with no PH and patients with precapillary PH helped confirm 18mmHg as the cutoff for diagnosing postcapillary PH. Conclusions Fluid challenge with 7mL/kg saline increases PAWP, more in postcapillary than in precapillary PH or in control subjects with no PH. A cutoff value of 18mmHg allows reclassification of 6%to 8%of patients with precapillary PH or normal hemodynamic characteristics at baseline.

Background: Exercise-induced increase in pulmonary artery systolic pressure (PASP) as a possible measure of right ventricular (RV) contractile reserve has been shown to predict survival in severe pulmonary hypertension. However, RV contractile reserve can also be measured by changes in stroke volume (SV), tricuspid annular plane systolic excursion (TAPSE), or tricuspid annular systolic velocity (S'). The limits of normal values and the functional significance of these changes in healthy subjects are not well known. Methods: In this prospective study, 90 healthy subjects (45 male, mean age 39 ± 13years) underwent exercise stress echocardiography with measurement of TAPSE, S', TAPSE/PASP, SV, and PASP at rest and peak exercise. Maximum and minimum normal values were reported for all indices. Results: Normal values of exercise-induced changes (Δ) were 4 to 10mm for TAPSE, 6 to 14cm/s for S', 12 to 57mm Hg for PASP, 0 to 96mL for SV, and −1.2 to 0mm/mm Hg for TAPSE/PASP. At peak exercise, women showed lower ΔTAPSE/PASP, ΔPASP, ΔS', and ΔSV, but higher TAPSE/PASP than men. Aging was associated with decreased ΔTAPSE/PASP, ΔTAPSE, ΔS', ΔPASP, and ΔSV. In addition, ΔS', ΔTAPSE/PASP, ΔPASP, and ΔSV, but not ΔTAPSE, were directly correlated with maximum workload. Conclusions: Our results provide age- and sex-related limits of normal for RV contractile reserve as assessed by exercise stress echocardiography and demonstrate that RV systolic function indices (PASP, TAPSE, S', and TAPSE/PASP) correlate with maximum exercise capacity.

Background In patients with obstructive sleep apnea syndrome (OSAS), repetitive hypoxia due to sleep-induced apnea adversely affects the interaction between myocardial oxygen demand and supply, resulting in the development of subclinical cardiac dysfunction. The purpose of the study was to analyze the different involvement of left and right heart myocardial function in patients with OSAS treated with noninvasive ventilation (NIV). Methods Conventional Doppler echocardiography, Doppler myocardial imaging (DMI), and two-dimensional speckle tracking echocardiography (2DSTE) of left (LV) and right ventricular (RV) longitudinal and right atrial (RA) deformation were performed in 55 patients with OSAS undergoing NIV (M/F 38/17; mean age 67.8 ± 11.2 years). LV and RV global longitudinal strain (GLS) was calculated by averaging local strain along the entire right and left ventricle, before and during NIV, and after 6 months of nocturnal NIV therapy. Results LV morphology was comparable before and during NIV, whereas LV ejection fraction and LV DMI early diastolic peak velocity were significantly improved in patients with OSAS during NIV, as was LV regional peak myocardial strain (P < 0.001). RV diameters were slightly increased in patients with OSAS during ventilation, whereas pulmonary artery systolic pressure (PASP), RV GLS, and regional peak myocardial RV strain were significantly reduced during ventilation (P < 0.0001). RA transverse diameters and RA area were also slightly increased during NIV, whereas RA lateral wall strain was reduced (P < 0.001). Acute RV myocardial impairment completely reversed at follow-up, with a decrease in PASP and subsequent increase in both RV and RA myocardial performance. Conclusions Conventional 2DSTE is a useful tool for assessing left and right heart morphology and myocardial deformation in patients with OSAS and for monitoring both acute and chronic effects of NIV.

During the last 15 years, a real “paradigm-shift” occurred, due to the development of PAH-targeted drugs, leading to crucial improvements in symptoms, exercise capacity, hemodynamics and outcome of PAH patients. In order to describe differences regarding epidemiology and therapy in PAH according to gender, we performed a review of the available literature in “PubMed” and “Web of Science” databases. In order to find relevant articles, we combined each of the following the keywords “pulmonary arterial hypertension”, “gender”, “sex”, “men”, “woman”, “male”, “female”, “phosphodiesterase inhibitors”, “endothelin receptor antagonists”, “prostanoids”. While there is a substantial agreement among epidemiological studies in reporting an increased prevalence of pulmonary arterial hypertension (PAH) among women, male PAH patients are affected by a higher impairment of the right ventricular function and consequently experience poorer outcomes. With regards to PAH-targeted drug administration, endothelin receptor antagonists (ERAs) and prostacyclin analogues (PC) show better treatment results in female PAH patients, while phosphodiesterase-5 inhibitors (PD5-I) seem to exert a more beneficial effect on male patients. However, to date no clear consensus could be formed by the available literature, which is constituted mainly by retrospective studies. Females with PAH are more prone to develop PAH, while males experience poorer outcomes. Females PAH might benefit more from ERAs and PC, while males seem to have more beneficial effects from PD5-I administration. However, more research is warranted in order to assess the most effective treatment for PAH patients according to gender.

Enlargement and dysfunction of the right atrium might be an early sign for pulmonary hypertension in systemic sclerosis (SSc). This is the first study to analyse right atrial morphology and function in SSc patients compared to healthy controls by speckle-tracking two-dimensional strain echocardiography (2DSE) at rest and during exercise. Furthermore, right atrial function was correlated with further clinical findings. Adult patients with SSc for > 3 years (n = 90) and 55 age- and gender-matched healthy controls underwent a panel of non-invasive assessments including transthoracic echocardiography, pulsed Doppler myocardial imaging and 2DSE at rest and during exercise. Furthermore, serological tests and high-resolution chest computed tomography were performed. SSc patients showed significant impairment of right atrial function and the right atrial enlargement, measured by 2DSE at rest and during exercise compared to controls (both p < 0.001). These findings were more evident in SSc patients with pulmonary fibrosis (p < 0.001) and in patients with high pulmonary artery systolic pressures (PAPs) during exercise. In the SSC patients, right atrial lateral strain was significantly associated with PAPs during effort, right atrial area, left ventricle stroke volume and inferior vena cava diameter using multivariable analysis. The findings of this study suggest that a high proportion of SSc patients reveal right atrial dysfunction even without manifest pulmonary hypertension. Impaired right atrial function occurred mostly in patients with pulmonary fibrosis and/or elevated PAPs during exercise, was independently associated with prognostic factors and may therefore be useful for risk stratification. Further studies are needed to analyse if right atrial dysfunction assessed by 2DSE may help to improve early diagnosis of pulmonary hypertension.

This study analyses the frequency and the potential role of two polymorphisms, the +134del/insA, located in the gene encoding for Endothelin-1 (EDN1), and the His323His in the gene encoding for Endothelin receptor type A (EDNRA) in a cohort of 98 consecutive patients with pulmonary arterial hypertension from two different Cardiology Units (Mid-South of Italy), and in 100 healthy Caucasian subjects randomly recruited from the same area. Cardiac anatomy and function were analysed by non invasive diagnostic imaging techniques (Echocardiography standard m-mode, 2D, colour-Doppler) and by invasive studies (cardiac catheterization). Molecular screening of the region of interest was performed by automated sequencing. At univariate analysis, patients with the His323His TT genotype show a lower cardiac index (2 ± 0.6 vs. 2.3 ± 0.6; p = 0.05) and a higher indexed pulmonary vascular resistance (18.8 ± 9.6 vs. 14.2 ± 6.9; p = 0.01) at cardiac catheterization. A logistic multivariate model shows idiopathic disease (p = 0.01; OR = 3.8; CI = 1.3-11) and indexed pulmonary vascular resistances (p = 0.01; OR = 1.1; CI = 1-1.2) as independent predictors of TT genotype. Our findings may suggest a potential link between specific genotypes in the EDNRA gene and susceptibility for PAH.

We report the case of a 16-year-old boy with cardiomyopathy induced by inappropriate sinus tachycardia (IST). The patient was resistant to treatment with conventional rate-decreasing medications. Therapy with the selective sinus node I(f) current inhibitor ivabradine was started. After 3 months of ivabradine therapy, an improvement in ejection fraction and a successful decrease in heart rate were observed. No side effects occurred. We suggest that ivabradine, currently used to treat stable angina, could be considered as a second-line treatment in patients with symptomatic and refractory IST.

An unusual case of two different types of atrial arrhythmia after orthotopic heart transplantation is reported: a macro-reentrant atrial tachycardia in the recipient atrium, and a common-type, counterclockwise, isthmus-dependent atrial flutter in the donor atrium. The clinical symptoms were caused by atrial flutter arising from the donor atrium. Radiofrequency catheter ablation of the electrically active, donor-derived portion of the cavotricuspid isthmus was performed successfully.

Background Recent data show that there is an unexpectedly high prevalence of ‘inappropriate’ pulmonary responses to exercise among patients with systemic sclerosis (SS). However, no consensus exists as to which threshold of pulmonary artery systolic pressure (PASP) can be considered diagnostically relevant. Aim To evaluate pulmonary vascular reserve and right ventricular function changes induced by exercise in SS patients without overt pulmonary arterial hypertension. Methods and results The study enrolled 172 consecutive SS patients in NYHA class IeII, with a peak tricuspid regurgitant jet velocity at echocardiography not greater than 3 m/s, and 88 control subjects. Echocardiography was performed at rest and at the end of a maximal exercise test. SS patients showed a higher exercise-induced PASP than control subjects (36.968.7 vs 25.963.3 mm Hg, p¼0.00008). The response to effort was higher in the presence of moderate interstitial lung disease (39.769.3 vs 36.068.4 mm Hg, p¼0.016) or subclinical left ventricular diastolic dysfunction (42.365.8 vs 37.068.6 mm Hg, p¼0.015). In control subjects, PASP values were normally distributed at rest and after exercise. In SS patients, the distribution was normal at rest but bimodal after exercise, with a second peak at 52.2 mm Hg including 13% of the total SS population. Patients in this subgroup showed subtle abnormalities of right ventricular function at rest and, most importantly, a blunted increase in right ventricular systolic function with exercise. Conclusion Exercise echocardiography may identify a subset of SS patients with an inappropriate exercise-induced increase in PASP and early signs of right ventricular dysfunction.

Exercise stress tests have been used for the diagnosis of pulmonary hypertension, but with variable protocols and uncertain limits of normal. The pulmonary haemodynamic response to progressively increased workload and recovery was investigated by Doppler echocardiography in 25 healthy volunteers aged 19-62 yrs (mean 36 yrs). Mean pulmonary artery pressure ((Ppa)) was estimated from the maximum velocity of tricuspid regurgitation. Cardiac output (Q) was calculated from the aortic velocity-time integral. Slopes and extrapolated pressure intercepts of (Ppa)-Q plots were calculated after using the adjustment of Poon for individual variability. A pulmonary vascular distensibility alpha was calculated from each (Ppa)-Q plot to estimate compliance. (Ppa) increased from 14+/-3 mmHg to 30+/-7 mmHg, and decreased to 19+/-4 mmHg after 5 min recovery. The slope of (Ppa)-Q was 1.37+/-0.65 mmHg x min(-1) x L(-1) with an extrapolated pressure intercept of 8.2+/-3.6 mmHg and an alpha of 0.017+/-0.018 mmHg(-1). These results agree with those of previous invasive studies. Multipoint (pa)-Q plots were well described by a linear approximation, from which resistance can be calulated. We conclude that exercise echocardiography of the pulmonary circulation is feasible and provides realistic resistance and compliance estimations. Measurements during recovery are unreliable because of rapid return to baseline.

Echocardiography is a key screening tool in the diagnostic algorithm of pulmonary arterial hypertension (PAH). It provides an estimate of right ventricular function and pulmonary artery pressure, either at rest or during exercise, and is useful in ruling out secondary causes of pulmonary hypertension (PH) such as left heart disease or congenital heart disease. Several studies have showed that echocardiography is insufficiently precise as single tool for the ultimate diagnosis of PH respect to the right heart catheterization, considered the gold standard technique. Echocardiography is valuable in assessing prognosis and treatment options, monitoring the efficacy of specific therapeutic interventions, and detecting the preclinical stages of disease. The ideal imaging modality for accurate noninvasive assessment of the right heart should be accurate and precise, not influenced by loading conditions, routinely practicable and easily repeatable. For all such reasons and considering that PAH is a rare and severe condition, a complete noninvasive assessment of right heart function requires a deep knowledge of the disease and a multimodality approach.

The electrocardiographic manifestations of hyperkalemia have been well described. We report the case of a patient with permanent atrial fibrillation for 13 months, who converted to normal sinus rhythm after treatment for hyperkalemia. This is the first case of hyperkalemia-induced conversion of permanent atrial fibrillation to sinus rhythm with a well-documented transitional tracing of atrial arrest. J Cardiovasc Med 11:000–000 Q 2010 Italian Federation of Cardiology.

A 41-year-old asymptomatic Italian male underwent cardiologic screening before blood donation. A transthoracic echocardiogram showed a huge right coronary artery (RCA) aneurysm continuing into a very twisted, worm-like channel, apparently draining into the right atrium (RA), suggesting the diagnosis of RCA-RA coronary fistula. Multidetector computed tomography showed a mild dilatation of the proximal RCA that gave rise to a giant 'snake-like' aneurysm (6.6 × 7 cm) followed by a long, tortuous and circumvolved channel spreading all over the right atrium and right ventricular free wall and finally draining into the coronary sinus.

Background Echocardiographic studies have contributed to progress in the understanding of the pathophysiology of the pulmonary circulation and have been shown to be useful for screening for and prognostication of pulmonary hypertension, but are considered unreliable for the diagnosis of pulmonary hypertension. We explored this apparent paradox with rigorous Bland and Altman analysis of the accuracy and the precision of measurements collected in a large patient population. Methods A total of 161 patients referred for a suspicion of pulmonary hypertension were prospectively evaluated by a Doppler echocardiography performed by dedicated cardiologists within 1h of an indicated right heart catheterization. Results Nine of the patients (6%) were excluded due to an insufficient signal quality. Of the remaining 152 patients, 10 (7%) had no pulmonary hypertension and most others had either pulmonary arterial hypertension (36%) or pulmonary venous hypertension (40%) of variable severities. Mean pulmonary artery pressure, left atrial pressure and cardiac output were nearly identical at echocardiography and catheterization, with no bias and tight confidence intervals, respectively ±3mm Hg, ±5mm Hg and ±0.3L/min. However, the ±2SD limits of agreement were respectively of +19 and −18mm Hg for mean pulmonary artery pressure, +8 and −12mm Hg for left atrial pressure and +1.8 and −1.7L/min for cardiac output. Conclusions Doppler echocardiography allows for accurate measurements of the pulmonary circulation, but with moderate precision, which explains why the procedure is valid for population studies but cannot be used for the individual diagnosis of pulmonary hypertension.

article i nfo Article history: Background: Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome. Methods: WHO functional class, resting oxygen saturation, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in patients with CHD-related PAH with and without Down's syndrome. Results: Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12 months of bosentan therapy, both with and without Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5±0.5 vs 2.9±0.6, p=0.005; controls: 2.5±0.5 vs 2.9±0.5, p=0.000002), 6-minute walk distance (Down: 288±71 vs 239±74 m, p=0.0007; controls: 389±80 vs 343±86 m, p=0.00003), and hemodynamics (pulmonary flow, Down: 4.0±1.6 vs 3.5±1.4 l/m/m 2 ,p =0.006; controls: 3.5±1.4 vs 2.8±1.0 l/m/m 2 , p=0.0005; pulmonary to systemic flow ratio, Down: 1.4±0.7 vs 1.0±0.4, p=0.003; controls: 1.1±0.7 vs 0.9±0.3,p=0.012; pulmonary vascular resistanceindex, Down: 15±9 vs 20± 13 WU m 2 , p=0.007; controls: 20±10 vs 26±15 WU m 2 , p=0.002). No differences in the efficacy of therapy were observed between the two groups. Conclusions: Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.

Bicuspid aortic valve (BAV) represents the most common cardiac congenital malformation in the adult age. It is frequently associated with dilatation, aneurysm and dissection of the ascending aorta. The purpose of the following study was to evaluate in patients with BAV: 1) the elastic properties of the ascending aorta, 2) the mechanical function of the left ventricle and 3) stiffness, elasticity and strain of the epi-aortic vessels wall.Forty BAV patients (28M/12F; age 20.9 ± 4.7 years; range 17-26) with no or mild valvular impairment were recruited with 40 control subjects (25M/15F; age 23.4 ± 3.4 years; range 15-31) matched for age, gender and body surface area (BSA). Aortic strain, aortic distensibility (AoDIS) and aortic stiffness index (AoSI) were derived. Left ventricular strain was acquired. Elastic properties of epi-aortic vessels were evaluated.BAVs vs. controls had increased systolic and diastolic aortic diameters (p0.001). Aortic strain (%) was lower in BAVs than in controls (8.3 ± 3.6 vs. 11.2 ± 2.6; p0.001) as well as AoDIS (10(-6)cm(2)dyn(-1)) (6.5 ± 2.8 vs. 8.8 ± 2.9; p=0.002), while AoSI was greater in BAVs (6.4 ± 3.5 vs. 3.9 ± 1.2; p0.001). Both AoDIS and aortic strain were related to aortic size in BAVs and controls. Left ventricular longitudinal (p=0.01), circumferential (p=0.01) and radial (p0.001) strain (%) were lower in BAVs. No significant differences were found in elastic properties of epi-aortic vessels.Bicuspid aortic valve is associated with an increased aortic stiffness and with a reduction of the aortic and left ventricular deformation properties. Epi-aortic vessels do not seem to be interested by the disease. The use of an echocardiographic method that can estimate the degree of aortic and left ventricular remodeling can provide great benefits in the selection of patients with BAV to be treated and in determining the time for beginning drug therapy.

cardiac autonomic control in patients with subclinical and overt hyperthyroidism. Eur J Endocrinol 2001;145:691–6. [15] Biondi B. Should we treat all subjects with subclinical thyroid disease the same way? Eur J Endocrinol Sep 2008;159(3):343–5 Electronic publication 2008 Jul 22. [16] Haentjens P, Van Meerhaeghe A, Poppe K, Velkeniers B. Subclinical thyroid dysfunction and mortality: an estimate of relative and absolute excess all-cause mortality based on time-to-event data from cohort studies. Eur J Endocrinol Sep 2008;159(3):329–41 Electronic publication 2008 May 29. [17] Patane S, Marte F, Di Bella G, Turiano G. Acute myocardial infarction and subclinical hyperthyroidism without significant coronary stenoses. Int J Cardiol 2009 May 29;134(3):e135–7 [Electronic Publication ahead of print 2008 Jun 24]. [18] Patane S, Marte F. Paroxysmal atrial fibrillation during acute myocardial infarction associated with subclinical hyperthyroidism, severe three vessels coronary artery disease and elevation of prostate-specific antigen after Turp. Int J Cardiol 2010;138:e28–30. [19] Patane S, Marte F. Paroxysmal ventricular tachycardia and paroxysmal atrial fibrillation associated with subclinical hyperthyroidism, chronic renal failure and elevation of prostate-specific antigen during acute myocardial infarction. Int J Cardiol 2010 Feb 4;138(3):e44–6 [Electronic Publication ahead of print 2008 Aug 21]. [20] Patane S, Marte F. Atrial fibrillation associated with subclinical hyperthyroidism. Int J Cardiol 2009 May 29;134(3):e155–8 [Electronic Publication ahead of print 2009 Jan 29]. [21] Patane S, Marte F. Intermittent changing axis deviationwith intermittent left anterior hemiblock during atrial flutter with subclinical hyperthyroidism. Int J Cardiol 2009 Jun 26;135(2):e37–9 [Electronic Publication ahead of print 2008 Jan 24]. [22] Patane S, Marte F. Changing axis deviation and paroxysmal atrial flutter associated with subclinical hyperthyroidism. Int J Cardiol 2010 Oct 8;144(2):e31–3 [Electronic Publication ahead of print 2009 Jan 26]. [23] Erem C. Blood coagulation, fibrinolytic activity and lipid profile in subclinical thyroid disease: subclinical hyperthyroidism increases plasma factor X activity. Clin Endocrinol (Oxf) Mar 2006;64(3):323–9. [24] Coban E, Aydemir M, Yazicioglu G, Ozdogan M. Endothelial dysfunction in subjects with subclinical hyperthyroidism. J Endocrinol Invest Mar 2006;29(3):197–200. [25] Coban E, Aydemir M. Levels of plasma fibrinogen and D-dimer in subjects with subclinical hyperthyroidism. Med Sci Monit Jan 2008;14(1):CR42–46. [26] Patane S, Marte F. Atrial fibrillation and acute myocardial infarction without significant coronary stenoses associated with subclinical hyperthyroidism and erythrocytosis. Int J Cardiol 2010 Nov 5;145(1):e36–9 [Electronic Publication ahead of print 2009 Jan 30]. [27] Patane S, Marte F, Curro A, Cimino C. Recurrent acute pulmonary embolism and paroxysmal atrial fibrillation associated with subclinical hyperthyroidism. Int J Cardiol 2010 Jul 9;142(2):e25–6 [Electronic Publication ahead of print 2009 Jan 9]. [28] Coats AJ. Ethical authorship and publishing. Int J Cardiol 2009;131:149–50.

BACKGROUND: Although the physiologic range of pulmonary artery systolic pressure (PASP) has been reported, data on how it is manifested in athletes are limited. The aim of the present study was to explore the full spectrum of PASP and the long-term training impact in a large population of highly trained athletes. METHODS: Six hundred fifteen consecutive athletes (370 endurance-trained athletes [ATEs] vs 245 strength-trained athletes [ATSs]; 28.4 ± 10.1 years old) and 230 healthy control subjects (27.5 ± 11.3 years old) underwent transthoracic echocardiography. PASP was estimated by measuring maximal tricuspid regurgitant jet velocity (TRV) with the modified Bernoulli equation. The ratio of TRV to right ventricular outflow tract time-velocity integral (TRV/RVOTTVI) was obtained as a correlate of pulmonary vascular resistance (PVR). RESULTS: Left ventricular (LV) mass index and ejection fraction did not differ significantly between the two groups of athletes. Conversely, ATSs showed an increased sum of wall thickness and relative wall thickness, whereas LV end-diastolic diameter, LV stroke volume, peak TRV, and PASP were significantly higher in ATEs. The ratio between transmitral E wave and tissue Doppler e' wave was not different among the three groups. The ratio TRV/RVOTTVI was ≤ 0.2 (ie, normal PVR) in all subjects. A TRV value > 2.5 m/s was observed in 76 athletes (12.3%). By multivariable analysis, age (P < .01), type and duration of training (P < .01), and LV stroke volume (P < .001) were the only independent predictors of PASP in athletes. CONCLUSIONS: This study delineates the full range of resting TRV and derived PASP in highly trained athletes. The upper physiologic limit of PASP in endurance athletes may reach 40 mm Hg, in line with the greater increase in stroke volume. This should be considered a "physiologic phenomenon" when evaluating athletes for sports eligibility.

Background Vasoreactivity testing is recommended in the management of pulmonary arterial hypertension (PAH), but its clinical relevance in congenital heart disease (CHD)-associated PAH has not been established. Objective To determine whether residual pulmonary vascular responsiveness to intravenous. epoprostenol is predictive of clinical outcome in patients with CHD-PAH and Eisenmenger syndrome. Methods and results A diagnostic right heart catheterisation with reversibility testing using epoprostenol infusion was performed in 38 consecutive patients with CHD-PAH and Eisenmenger syndrome. Patients were treated with bosentan and were assessed every 3 months. Clinical worsening was defined as death from any cause, heart–lung or lung transplantation (or on the waiting list for this procedure), hospitalisation for PAH, or symptom exacerbation defined as a ≥20% decrease in the 6 min walking distance on two consecutive tests, an increase in WHO functional class, or worsening right heart failure. The mean follow-up was 33±17 months. Sixteen patients showed clinical worsening. Although they did not differ from the other patients in their baseline exercise capacity, haemodynamic characteristics and underlying CHD, pulmonary vascular resistance index (PVRi) was less reversible (ΔPVRi 29±21 vs 52±14%, p = 0.0003). At univariate analysis, systemic vascular resistance, PVRi and ΔPVRi were significant predictors of clinical worsening. At multivariate Cox proportional hazards regression model, ΔPVRi was found to be the only independent predictor of clinical worsening (HR=0.973, 95% CI 0.95 to 0.99; p = 0.01). ΔPVRi ≥25% had a positive and negative predictive value for clinical worsening of 100% and 75.9%, respectively. Conclusion Pulmonary vasoreactivity is a significant predictor of clinical worsening in patients with CHD-PAH.

A 37-year-old woman, nonsmoker, with a history of chronic migraines with aura, was referred to our emergency room due to dysarthria and left faciobrachial paresis. Her family history was unremarkable. Physical examination was normal except for a mild oxygen desaturation (88% on room air). Electrocardiogram, echocardiography, peripheral venous and arterial Doppler ultrasound, and brain magnetic resonance imaging were normal. However, transcranial Doppler analysis showed a significant (shower pattern) right-to-left shunt late after isotonic saline. This pattern was present at rest and did not significantly change after Valsalva maneuver. At chest radiograph, a lobular smoothmargined opacity in the right lower lobe made us suspect the diagnosis of pulmonary arteriovenous malformation (PAVM). Chest computed tomography (CT) showed a large complex PAVM fed by two large and tortuous descending pulmonary arteries and draining through a straight vessel directed posterosuperiorly to the right lower pulmonary vein (Fig. 1). Thus, cardiac catheterization was planned to occlude the vascular malformation. The patient’s informed consent to the procedure and agreement with the thoracic surgeons were obtained. The procedure was performed with local anesthesia through the femoral vein entry. Pulmonary angiography confirmed the presence of multiple large PAVMs into the right lower lobe fed by a single 1.1-cm-large vessel originating from the right inferior pulmonary artery (Fig. 2a). Transcatheter embolization of the feeding vessel was achieved with a 14-mm-large Amplatzer Vascular Plug type II (AGA Medical, Golden Valley, Minnesota, USA) device (Fig. 2b) and resulted in a sudden increase in the oxygen saturation, from 88 to 96% on room air. Clinical status and

Skin erosion caused by the pacemaker is widely documented, but the complete extrusion of the device is very rare. We describe the case of a 54-year-old woman who was admitted to hospital because of skin erosion, followed by the complete extrusion of the pacemaker pulse generator out of the subcutaneous pocket. The patient underwent a lead extraction procedure and a new pacemaker, in the contralateral side, was implanted. This case demonstrates that the early stages of skin erosion favoured by the device, if neglected, may cause more serious complications that may require the removal of the hardware.

Objective The present study aimed to evaluate the management of fetal cardiac dysrhythmias based on prior identification of the underlying electrophysiological mechanism. Methods We studied 36 consecutive fetuses with cardiac dysrhythmia. Rhythm diagnosis was based on M-mode, pulsed wave Doppler and tissue Doppler imaging (TDI). Only fetuses with: (i) incessant tachycardia (> 12 h) and mean ventricular rate > 200 beats/min, (ii) signs of left ventricular dysfunction, or (iii) hydrops, were treated using oral maternal drug therapy. Results The mean gestational age at diagnosis was 24.3 +/- 4.5 weeks. Twenty-one fetuses had tachycardia with a 1: 1 atrial-ventricular (AV) conduction. Based on ventricular-atrial interval, prenatal diagnosis was: permanent junctional reciprocating (n = 6), atrial ectopic (n = 6) or atrial-ventricular re-entry tachycardia (n = 9). One had atrial flutter, one ventricular tachycardia and four congenital AV block. Nine showed premature atrial or ventricular beats. Fifteen fetuses with incessant tachycardia, left ventricular dysfunction or hydrops were prenatally treated with maternal administration of digoxin, sotalol or flecainide. The total success rate (sinus rhythm or rate control) was 14/15 (93%). Seven fetuses were hydropics. Three of these died (one at 28 weeks of gestation, two in the first week of life). The prenatal diagnosis of dysrhythmia was confirmed at the birth in 31 of 35 live-born. No misdiagnosis was made using TDI. At 3 +/- 1.1-year follow-up, 33/35 children were alive and well. Conclusions Fetal echocardiography could clarify the electrophysiological mechanism of fetal cardiac dysrhythmias and guide the therapy.

A 12-year-old female with the Wolff-Parkinson-White syndrome underwent an electrophysiologic study followed by radiofrequency catheter ablation of the left-lateral accessory pathway. After several unsuccessful attempts, the procedure was stopped because of early recurrence of accessory pathway conduction. Twenty-four hours after the procedure, the patient was found without ventricular pre-excitation pattern on the electrocardiogram. During a 12-month follow-up, the Wolff-Parkinson-White pattern was no longer present. A transesophageal electrophysiologic study showed no further tachycardia induction and the patient is still asymptomatic. This report raises the issue that ablation-induced lesions may evolve considerably during the first few days after ablation, leading to either recurrent accessory pathway conduction or long-lasting complete cure.

Subtotal anomalous pulmonary venous connection (APVC) is a rare congenital heart disease (CHD) in which all pulmonary veins but one drain into the right cardiac chambers. Similar to total APVC, the presence of an unrestrictive atrial septal defect is critical for survival in the first months of life. Reduced exercise tolerance, severe dyspnea, and palpitations are early symptoms and generally survival beyond infancy is rare without surgical treatment. To date, no cases of this rare CHD without associated atrial septal defect have been diagnosed in adulthood. In addition, our patient also showed a type IIIA2 left cor triatriatum, in which the pulmonary venous chamber is separated from the true left atrium by a fibromuscular septum. To date, this latter malformation has been reported only in association with partial APVC.

OBJECTIVE Left ventricular (LV) remodelling in prenatally diagnosed LV outflow tract obstructive lesions such as aortic stenosis and aortic coarctation is important for prenatal counselling and postnatal management. The purpose of this study was to document the spectrum and the progression of different LV remodelling patterns and to identify prenatal markers of hypoplastic left heart syndrome (HLHS). METHODS We studied 29 fetuses with LV outflow tract obstruction: 13 with isolated aortic stenosis, 14 with isolated aortic coarctation and two with combined aortic stenosis and aortic coarctation. Echocardiographic evaluation was performed 4 and 8 weeks after the first observation and at birth. RESULTS None of the fetuses had HLHS (LV end-diastolic diameter z score higher than -2) at first prenatal echocardiography (24.5 + or - 3.6 weeks). Fetuses were divided into two groups: group A (n = 25) with a LV end-diastolic volume at birth > 20 ml/m(2); group B (n = 4) with a LV end-diastolic volume at birth < 20 ml/m(2) (LV hypoplasia). At first echocardiographic evaluation, the two groups showed a significantly different aorta to pulmonary ratio (0.44 + or - 0.08 vs. 0.86 + or - 0.14; P < 0.001); other LV echocardiographic features were not significantly different. The growth of the mitral (0.10 + or - 0.02 vs. 0.43 + or - 0.28 mm/week; P < 0.02) and aortic annulus (0.08 + or - 0.01 vs. 0.26 + or - 0.14 mm/week; P < 0.05) was significantly slower in group B. CONCLUSIONS Our data suggest that LV outflow tract obstruction can progressively evolve in HLHS during pregnancy. A smaller aorta to pulmonary ratio was the only significant difference at initial echocardiographic evaluation in the two groups. Moreover, serial echocardiographic examinations are necessary to recognize fetuses at risk for HLHS caused by a subnormal growth rate of the mitral and aortic annulus.

Patients with severe heart failure require large quantities of health care resources, and more intensive interventions are not always related to a decrease in need for medical care, including hospitalization. A thoughtful approach to the efficient and expeditious allocation of these resources is required. In fact, the nonpharmacologic therapy of patients with chronic congestive heart failure (CHF) has to include the complete explanation of various topics, such as causes of heart failure, symptoms, diet, salt and fluid restriction, drug regimen, compliance, physical and work activities, lifestyle changes, and measures of self-control. This article describes how the relationship among patient, family, friends, and different heart-care workers (physicians, nurses, psychologists, rehabilitation technicians, dietitians, hospital personnel) can greatly affect therapeutic efficacy.

Ambrisentan, an oral endothelin-1-receptor antagonist (ERA) selective for the endothelin A, showed an improvement in WHO functional class, 6 minute walk distance, and time to clinical worsening relative to placebo in randomised, double-blind, placebo-controlled, multicenter, 12-week clinical trials (ARIES study 1 and 2, total n=394) [1]. At this time, the long-term effects of ambrisentan on haemodynamics have not been reported in a prospective study. The aim of this study was to evaluate prospectively the efficacy and safety of ambrisentan treatment in adult patients with pulmonary arterial hypertension by assessing its long term effects on clinical status, exercise capacity, and cardiopulmonary haemodynamics. This was a single-centre, open-label, single-arm, prospective study. All patients with PAH starting oral ambrisentan as first line therapy or combination therapy (based on recommendations of the current ESC/ERS guidelines on pulmonary hypertension) [2] were enrolled after obtaining the informed consent. Clinical status, resting transcutaneous oxygen saturation (SpO2), 6-minute walk distance, serology and right heart catheterization (RHC) were assessed at baseline (before starting ambrisentan therapy) and at 1 year follow-up. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [3]. Clinical status evaluation included medical history, assessment of WHO functional class, measurement of systemic arterial pressure, transcutaneous oxygen saturation (SpO2) and heart rate.

Anthracyclines are effective chemotherapeutic agents against various malignancies but their therapeutic value is limited by well-known dose-related cardiotoxicity, mainly induced by oxygen free radicals. Left ventricular diastolic and systolic functional abnormalities precede the clinical evidence of cardiotoxicity. The aim of this study was to evaluate the possible cardiotoxicity of epidoxorubicin administered as "high-dose short-term" protocol. Twenty patients (mean age 50.4 +/- 7.9 years) without cardiac disease, affected by advanced breast cancer were studied. All patients were treated with epidoxorubicin as neoadjuvant chemotherapy according to the new protocol "high-dose short-term" (cumulative dose 475.8 +/- 35.6 mg/m2, range 450-600 mg/m2, in 4-6 weeks). The effectiveness of cancer chemotherapy was monitored by clinical evaluation and mammography performed before and after treatment. All patients underwent color Doppler echocardiography and resting radionuclide angiocardiography in baseline condition and 30 +/- 10 days after the last cycle of chemotherapy. All patients showed a significant reduction of tumor lesion after chemotherapy. Left ventricular systolic and diastolic function parameters obtained by echocardiography (fractional shortening 33.1 +/- 4.5% vs 32.4 +/- 4.8%; ejection fraction 63.6 +/- 6.2% vs 62.9 +/- 5.7%; E/A ratio 1.73 +/- 0.64 vs 1.82 +/- 0.67; E wave deceleration time 204 +/- 24.6 ms vs 208.5 +/- 31.7 ms;isovolumetric relaxation time 79 +/- 15.7 ms vs 80 +/- 17.8 ms) and radionuclide angiocardiography (ejection fraction 62.4 +/- 7% vs 61.8 +/- 5.9; peak ejection rate 2.87 +/- 0.44 VTD/s vs 2.74 +/- 0.46 VTD/s; peak filling rate 2.72 +/- 0.54 VTD/s vs 2.6 +/- 0.58 VTD/s) did not show significant changes after treatment. In conclusion, our results suggest that epidoxorubicin administration using the "high-dose short-term" protocol in patients with breast cancer does not induce early significant abnormalities of left ventricular systolic and diastolic function.

To verify the impact of sever obesity (defined as body mass index31 kg/m2) on left ventricular (LV) function, 32 asymptomatic obese but otherwise healthy subjects (16 men; age 38 +/- 11 years) voluntarily underwent first-pass and equilibrium 99mTc radionuclide angiography at rest and, in 22 of them, during bicycle supine exercise. Data were compared to those obtained from 10 normal volunteers (age 48 +/- 13; p0.05, vs. obeses). End-diastolic and stroke volumes did not differ between the two groups, whereas end-systolic volume was significantly higher in obese subjects (67 +/- 20 vs. 49 +/- 20 ml; p0.05), and, as a consequence, LV ejection fraction at rest was decreased in obese subjects (59 +/- 7%) compared to normals (65 +/- 6%; p0.05). Due to the higher heart rate in obese subjects (81 +/- 13 vs. 69 +/- 10 pbm, respectively; p0.05) cardiac output was significantly greater compared to normals (7.1 +/- 0.8 vs. 6.2 +/- 0.2 liters/min, respectively; p0.01). During exercise, ejection fraction normally increased in normals (70 +/- 7%; p0.001, vs. baseline) but not in obese subjects (60 +/- 9%; p = nonsignificant vs. baseline). In addition, systolic blood pressure/end-systolic volume ratio was significantly decreased in obese subjects (23 +/- 1.3) compared to normals (2.8 +/- 1.6; p0.05). Peak filling rate, normalized to end-diastolic counts per second, was significantly lower in obese subjects (2.2 +/- 1.3) compared to normals (2.8 +/- 1.6; P0.05). This difference was also true when peak filling rate was computed in stroke counts per second (3.8 +/- 0.8 in obeses vs. 4.4 +/- 0.4 in normals; p0.05). Repeat analysis in a subgroup of 10 young obese subjects (ageor = 30 years) confirmed decreased ejection fraction at rest (60 +/- 4%; p0.05) and peak filling rate (2.4 +/- 0.4 end-diastolic counts/s; p0.05), as well as the lack of ejection fraction increase during exercise (59 +/- 9%). Thus, these data indicate a subclinical impairement of LV systolic and diastolic function at rest and during exercise in asymptomatic severely obese but otherwise healthy subjects.

The aim of the present study was to evaluate the variation in left ventricular function at 15 and 120 days after bypass surgery. Ten male patients (mean age 59.6 +/- 7 years) with previous myocardial infarction and coronary stenosis of at least two main vessels, underwent a radionuclide ambulatory monitoring of left ventricular function (VEST) during: handgrip test, mental stress test, walking, climbing stairs. These tests were carried out 8 +/- 2 days before (T0), 15 +/- 3 days (T1) and 120 +/- 11 days after coronary bypass; heart rate (HR), ejection fraction (EF), stroke volume (SV), and cardiac output (CO) were evaluated beat by beat before and during the test until the maximal HR was reached. Handgrip and mental stress tests did not induce significant variations in cardiac indices both before and after cardiac surgery. No variation in HR was observed before and after the test. During walking, at T0, HR increased from rest to maximal effort (p0.01) with a significant decrease in EF (p0.05); at T1 HR, EF, SV and CO increased with respect to rest (p0.01); AT T2 HR, EF (p0.05) and CO (p0.01) increased with respect to rest. Climbing stairs, at T0, HR increased (p0.01) and EF decreased (p0.05); at T1 HR, EF, CO (p0.05) and SV (p0.01) increased with respect to rest. At T2 an increase in HR (p0.01) and CO (p0.05) was observed at maximal effort with respect to rest. The statistical analysis on the percentage variations between baseline and maximal effort (climbing stairs) showed a significant increase in SV at T2 compared to T0 (p0.01). In conclusion, VEST during daily normal activities 15 days and 4 months after bypass surgery showed a significant increase in cardiac function indices. The best results obtained at T1 can be explained with the transient increase in adrenergic tone at the time of early postoperative period.

The aim of this study was to analyze the relationship between heart rate and QT interval (HR-QT) during exercise in control subjects (Group A) and in patients with coronary artery disease (CAD) with effort angina and without previous myocardial infarction (MI) (Group B). The diagnosis of CAD was confirmed by coronarographic examination. The correlation HR-QT was significant (p0.001) in both groups on effort and at recovery. The analysis of the regression HR-QT was carried out separately, both on effort in upright position and at rest in supine position, to avoid the influence of posture on QT length. During effort, the regression line showed lower slope and intercept values in Group B (p0.001) than those for Group A. A similar behavior was also observed at rest. Thus, at the highest heart rate, where ECG signs of ischemia (ST depression1 mm) frequently occurred, a longer QT interval was present in Group B. Moreover, in Group B, the QT interval in the presence of ECG signs of ischemia was significantly longer (p0.01) than in Group A at comparable heart rates both on effort and at rest, thereby confirming the result obtained by comparing both regression lines. The same effort protocol was repeated in Group B patients after acute administration of atenolol 100 mg per os. After atenolol administration, the analysis of the regression HR-QT in Group B clearly showed a shorter QT interval than that obtained in washout period during the baseline test at the highest heart rates where the ECG frequently showed signs of ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)

123I-radiolabeled metaiodobenzylguanidine (123I-MIBG) cardiac imaging has been used to evaluate the distribution of sympathetic nervous system (SNS) in the heart. Different heart diseases have shown impaired cardiac SNS distribution as reflected by MIBG activity. The aim of this study was to assess the cardiac distribution of SNS in normal subjects, using MIBG imaging. Ten normal subjects (1 male and 9 females, mean age 46 +/- 9 years) with no cardiac abnormalities underwent myocardial 123I-MIBG scintigraphy, Tc-99m methoxyisobutylisonitrile (MIBI) cardiac perfusion imaging and equilibrium radionuclide angiography (RNA). Regional myocardial MIBG and MIBI activities were quantitatively evaluated using a region of interest analysis. For this purpose, the left ventricle was divided into 6 myocardial regions as anterior, apical, inferior, septum, lateral and posterolateral. In particular, myocardial MIBG and MIBI activities were measured as myocardium to mediastinum ratio. Regional left ventricular function was assessed by RNA. Myocardial MIBG uptake was homogeneous in anterior (2.2 +/- 0.5), inferior (2.5 +/- 0.7), septal (2.4 +/- 0.4), lateral (2.3 +/- 0.4), and posterolateral (2.3 +/- 0.4) regions. Conversely, MIBG uptake was significantly lower in the apical region (1.9 +/- 0.3) compared to all other left ventricular segments (p0.05). Regional myocardial perfusion, as measured by MIBI uptake, was homogeneous in all regions. No regional left ventricular wall motion abnormalities were observed by RNA. In conclusion, our data suggest that a decreased MIBG uptake may be observed in the left ventricular apical region of normal subjects reflecting reduced sympathetic innervation of the apex. This finding is not related to myocardial perfusion or wall motion abnormalities. The knowledge of cardiac sympathetic innervation in normal subjects may be helpful to assess SNS abnormalities in heart disease.