Left ventricular global longitudinal strain and acute myocardial injury in patients with sickle cell disease admitted to the intensive care unit for vaso-occlusive crisis.

In patients with sickle cell disease (SCD), SCD-related cardiomyopathy may be partly due to repeated ischaemic events related to sickling during vaso-occlusive crises, but few clinical studies support this hypothesis. We evaluated the incidence of acute myocardial ischaemia during vaso-occlusive crises as assessed by the left ventricular global longitudinal strain (LVGLS) and high-sensitive cardiac troponin T (hs-cTnT). We included adult patients with SCD admitted to the intensive care unit (ICU) for vaso-occlusive crisis. We collected hs-cTnT and measured LVGLS with echocardiography at admission (day 1), day 2, day 3 and ICU discharge. Among 55 patients included, considering only the first hospitalization of patients admitted several times, 3 (5%) had elevated hs-cTnT at ≥1 time point of the ICU stay. It was ≤2 times the upper limit of normal in two of these patients. LVGLS was altered at ≥1 time point of the ICU stay in 13 (24%) patients. Both hs-cTnT and LVGLS were abnormal at ≥1 time point of the hospital stay in 2 (4%) patients. Acute myocardial injury as assessed by troponin elevation and LVGLS impairment was a rare event during vaso-occlusive crises.

Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation.

BACKGROUND: Heritable pulmonary arterial hypertension (PAH) is most commonly due to heterozygous mutations of the BMPR2 gene. Based on expert consensus, guidelines recommend annual screening echocardiography in asymptomatic BMPR2 mutation carriers. The main objectives of this study were to evaluate the characteristics of asymptomatic BMPR2 mutation carriers, assess their risk of occurrence of PAH and detect PAH at an early stage in this high-risk population. METHODS: Asymptomatic BMPR2 mutation carriers underwent screening at baseline and annually for a minimum of 2 years (DELPHI-2 study; ClinicalTrials.gov: NCT01600898). Annual screening included clinical assessment, ECG, pulmonary function tests, 6-min walk distance, cardiopulmonary exercise testing, chest radiography, echocardiography and brain natriuretic peptide (BNP) or N-terminal (NT)-proBNP level. Right heart catheterisation (RHC) was performed based on predefined criteria. An optional RHC at rest and exercise was proposed at baseline. RESULTS: 55 subjects (26 males; median age 37 years) were included. At baseline, no PAH was suspected based on echocardiography and NT-proBNP levels. All subjects accepted RHC at inclusion, which identified two mild PAH cases (3.6%) and 12 subjects with exercise pulmonary hypertension (21.8%). At long-term follow-up (118.8 patient-years of follow-up), three additional cases were diagnosed, yielding a PAH incidence of 2.3% per year (0.99% per year in males and 3.5% per year in females). All PAH cases remained at low-risk status on oral therapy at last follow-up. CONCLUSIONS: Asymptomatic BMPR2 mutation carriers have a significant risk of developing incident PAH. International multicentre studies are needed to confirm that refined multimodal screening programmes with regular follow-up allow early detection of PAH.

Echocardiographic Evaluation of Left Ventricular Filling Pressure in Patients With Heart Failure With Preserved Ejection Fraction: Usefulness of Inferior Vena Cava Measurements and 2016 EACVI/ASE Recommendations.

CONTEXT: The left ventricular filling pressure (LVFP) is correlated to right atrial pressure (RAP) in heart failure. We compared diagnostic value of the inferior vena cava (IVC) measurements to the one of the 2016 echocardiographic recommendations to estimate LVFP in patients with suspected heart failure with preserved ejection fraction (HFpEF). METHODS: Invasive hemodynamics and echocardiography were obtained within 48 hours in 132 consecutive patients with left ventricular ejection fraction ≥50%, and suspected pulmonary hypertension. Increased LVFP was defined by a pulmonary artery wedge pressure (PAWP) >15 mmHg. RESULTS: Of 83 patients in sinus rhythm, a score of the 2016 recommendations ≥ 2 (E/e' ratio >14 and/or tricuspid regurgitation velocity >2.8 m/s and/or indexed left atrial volume>34 mL /m²) had a positive predictive value (PPV) of 63% for PAWP>15 mmHg, whereas a dilated IVC (>2.1 cm) and/or non-collapsible (≤50%) had a PPV of 82%. The net reclassification improvement was 0.39 (P < .05). In atrial fibrillation (AF), a dilated and/or non-collapsible IVC had an 86% PPV for PAWP>15 mmHg. The correlation between RAP and PAWP was 0.60, with 75.7% concordance (100/132) between dichotomized pressures (both RAP>8 mmHg and PAWP>15 mmHg and vice versa). CONCLUSION: The IVC size and collapsibility is valuable to identify patients with HFpEF with high LVFP in both sinus rhythm and AF.

A Clinical and Echocardiographic Score to Identify Pulmonary Hypertension Due to HFpEF.

BACKGROUND: Heart failure with preserved ejection fraction (HFpEF) is a frequent cause of pulmonary hypertension (PH) that is not easy to differentiate from precapillary PH. We aimed to determine whether the characteristic features of the patients may help differentiate between HFpEF and precapillary PH. METHODS AND RESULTS: Clinical and echocardiographic parameters were analyzed in 156 patients referred to our PH referral center. Right heart catheterization identified 78 PH-HFpEF patients and 78 with precapillary PH. Compared with precapillary PH, PH-HFpEF patients were older, with a smaller proportion of women, a higher proportion of hypertension, diabetes mellitus, atrial fibrillation and sleep apnea syndrome, and a higher body mass index. On echocardiography, PH-HFpEF patients had higher left ventricular mass index, higher left atrial area, and smaller right ventricular end-diastolic area. Following multivariate analysis, a model predicting the probability of PH-HFpEF was built with history of diabetes mellitus, presence of atrial fibrillation, left atrial area, right ventricular end-diastolic area, and left ventricular mass index. The score was internally validated using bootstrap method (area under the curve 0.93 [95% confidence interval 0.918-0.938]). A score <5 ruled out PH-HFpEF. CONCLUSION: A score including clinical and echocardiographic criteria may help physicians to identify PH-HFpEF from precapillary PH.

Exercise Hemodynamics Predict Pulmonary Arterial Hypertension in BMPR2 Mutation Carriers.

BACKGROUND: Exercise hemodynamics are recommended for early detection of pulmonary arterial hypertension (PAH) and have been suggested to be predictive of future development of PAH in high-risk populations such as BMPR2 mutation carriers. However, the optimal exercise hemodynamic screening parameter remains to be determined. Recent data suggest that pulmonary vascular distensibility coefficient (α) may serve as a useful parameter for early detection of PAH. RESEARCH QUESTION: What is the value of exercise hemodynamics, including α, for predicting the occurrence of PAH during long-term follow-up in BMPR2 mutation carriers? STUDY DESIGN AND METHODS: Fifty-two asymptomatic BMPR2 mutation carriers who underwent symptom-limited exercise hemodynamic assessment were followed up for a median of 10 years. The impact of hemodynamics at rest and exercise, presence of exercise pulmonary hypertension, and α on occurrence of PAH during long-term follow-up were assessed. RESULTS: During long-term follow-up, five patients demonstrated PAH. Patients who demonstrated PAH showed a significantly lower α (0.8 ± 0.4%/mm Hg) than patients without PAH (1.8 ± 0.8%/mm Hg; P = .008). The only hemodynamic parameter that predicted the occurrence of PAH during long-term follow-up at regression analysis was α. Receiver operating characteristic analysis showed that α ≤ 1.5%/mm Hg predicted PAH occurrence with a specificity of 75% and sensitivity of 100%. INTERPRETATION: Before development of PAH in BMPR2 mutation carriers, α is reduced markedly and may serve as a useful parameter in the setting of early disease detection. Given the low event rate, caution is warranted in interpreting these results, highlighting the need for validation studies.

Pulmonary hypertension due to left heart disease.

Pulmonary hypertension due to left heart disease, also known as group 2 pulmonary hypertension according to the European Society of Cardiology/European Respiratory Society classification, is the most common cause of pulmonary hypertension. In patients with left heart disease, the development of pulmonary hypertension favours right heart dysfunction, which has a major impact on disease severity and outcome. Over the past few years, this condition has been considered more frequently. However, epidemiological studies of group 2 pulmonary hypertension are less exhaustive than studies of other causes of pulmonary hypertension. In group 2 patients, pulmonary hypertension may be caused by an isolated increase in left-sided filling pressures or by a combination of this condition with increased pulmonary vascular resistance, with an abnormally high pressure gradient between arteries and pulmonary veins. A better understanding of the conditions underlying pulmonary hypertension is of key importance to establish a comprehensive diagnosis, leading to an adapted treatment to reduce heart failure morbidity and mortality. In this review, epidemiology, mechanisms and diagnostic approaches are reviewed; then, treatment options and future approaches are considered.

Long-term effects of pegvisomant on comorbidities in patients with acromegaly: a retrospective single-center study.

CONTEXT: The effect of pegvisomant on IGF1 levels in patients with acromegaly is well documented, but little is known of its long-term impact on comorbidity. AIM: The aim of this retrospective study was to evaluate the effects of long-term pegvisomant therapy on cardiorespiratory and metabolic comorbidity in patients with acromegaly. PATIENTS AND METHODS: We analyzed the long-term (up to 10 years) effect of pegvisomant therapy given alone (n=19, 45%) or in addition to somatostatin analogues and/or cabergoline (n=23, 55%) on echocardiographic, polysomnographic and metabolic parameters in respectively 42, 12 and 26 patients with acromegaly followed in Bicêtre hospital. RESULTS: At the first cardiac evaluation, 20±16 months after pegvisomant introduction, IGF1 levels normalized in 29 (69%) of the 42 patients. The left ventricular ejection fraction (LVEF) improved significantly in patients whose basal LVEF was ≤60% and decreased in those whose LVEF was >70%. The left ventricular mass index (LVMi) decreased from 123±25 to 101±21 g/m(2) (P<0.05) in the 17 patients with a basal LVMi higher than the median (91 g/m(2)), while it remained stable in the other patients. Pegvisomant reduced the apnoea-hypopnea index and cured obstructive sleep apnea (OSA) in four of the eight patients concerned. Long-term follow-up of 22 patients showed continuing improvements in cardiac parameters. The BMI and LDL cholesterol level increased minimally during pegvisomant therapy, and other lipid parameters were not modified. CONCLUSIONS: Long-term pegvisomant therapy not only normalizes IGF1 in a large proportion of patients but also improves cardiac and respiratory comorbidity.

No evidence of a detrimental effect of cabergoline therapy on cardiac valves in patients with acromegaly.

CONTEXT: The effects of cabergoline on cardiac valves have been extensively studied in Parkinson's disease and hyperprolactinemia but not in acromegaly, a condition at risk of cardiac valve abnormalities. OBJECTIVE: We examined the prevalence and incidence of heart valve disease and regurgitation in a series of patients with acromegaly treated with cabergoline, by comparison with matched patients who had never received this drug. DESIGN AND SETTING: We conducted a cross-sectional and longitudinal study in a single referral center. PATIENTS AND METHODS: Forty-two patients who had received cabergoline at a median cumulative dose of 203 mg for a median of 35 months were compared to 46 patients with acromegaly who had never received cabergoline and who were matched for age, sex, and disease duration. A subgroup of patients receiving cabergoline (n = 26) was evaluated longitudinally before and during cabergoline treatment and compared to a group not receiving cabergoline and followed during the same period (n = 26). Two-dimensional and Doppler echocardiographic findings were reviewed by two cardiologists blinded to treatment. RESULTS: Demographic and clinical features were not significantly different between the groups. Compared to acromegalic controls, patients receiving cabergoline did not have a higher prevalence or incidence of valve abnormalities. A slightly higher prevalence of aortic valve regurgitation and remodeling was found in the controls relative to the cabergoline-treated patients (P < 0.02 and P < 0.03, respectively), but this was related to the presence of aortic dilatation. CONCLUSION: Cabergoline therapy is not associated with an increased risk of cardiac valve regurgitation or remodeling in acromegalic patients at the doses used in this study.

Aortic valve translocation for severe prosthetic valve endocarditis: early results and long-term follow-up.

BACKGROUND: Surgical management of ventriculo-aortic disconnection and aortic root abscesses after prosthetic aortic valve endocarditis carries high mortality and morbidity. Initial experience with translocation of the aortic valve and distal coronary artery bypass grafting was disappointing in terms of short-term and long-term success in the few published reports. We describe a technique of translocation of the aortic valve into the ascending aorta with direct antegrade myocardial revascularization. METHODS: Between 1980 and 1992, we included 21 patients and evaluated their long-term outcome. The surgical technique included extracting the aortic valve prosthesis, resecting all infected tissue, restoring the left ventricular outflow tract, and translocating the aortic valve into the ascending aorta, associated with myocardial revascularization of the left main trunk and the proximal right coronary artery. RESULTS: All patients required emergency surgery: 15 patients were in severe congestive heart failure, 3 patients were in cardiogenic shock, and 3 patients had multiple neurologic and peripheral signs of distal embolization. Fifteen patients had active prosthetic valve endocarditis. Intraoperative findings dictated the translocation. The overall hospital mortality was 14%. None of the 18 hospital survivors had prosthetic aortic valve endocarditis recurrence. All patients were observed from 12 to 22 years, are alive, and have resumed normal activities. CONCLUSIONS: In severe forms of prosthetic valve endocarditis, this technique provides a safe and reliable alternative to homograft replacement. The long-term results are satisfactory.