Pamela Moceri

Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life—single centre experience and review of published data

AIMS We aimed to investigate the distribution of exercise capacity across the spectrum of adult congenital heart disease (ACHD) using own data and the published experience and to provide diagnosis, gender-, and age- specific reference values. METHODS AND RESULTS Publications describing exercise capacity in ACHD patients using cardiopulmonary exercise testing (CPET) were identified (n = 2286 patients in 23 papers). In addition, we included 2129 patients who underwent CPET at our own institution. The majority of patients (80%) had reduced peak oxygen uptake (peak VO(2)) compared with normal values (defined as <90% of predicted peak VO(2)). There were significant differences in peak VO(2) between subgroups of patients, with the lowest values seen in patients with Eisenmenger syndrome and complex heart disease. However, even in patients with simple lesions, peak VO(2) was on average significantly reduced compared with normal values. Based on a large number of observations we herewith provide gender- and age-specific peak VO(2) centile plots for the most common lesions (Tetralogy of Fallot, systemic right ventricle, Ebstein anomaly and Fontan-palliation) and relate disease-specific exercise capacity to that required for specific activities of daily life, sports, and occupations. CONCLUSION We provide age-, gender-, and diagnosis-specific data on peak VO(2) levels across the spectrum of ACHD allowing to compare the exercise capacity of individual patients with that of their peer patients. These data should be helpful in interpreting CPET results, guiding therapy, and advising patients on activities of daily life, sports participation, and choice of occupation.

Pregnancy outcomes in patients with pulmonary arterial hypertension associated with congenital heart disease

Objective There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD. Methods Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes. Results 28 pregnancies in 20 women (26±6 years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20 weeks’ gestation (WG)), 8 abortions and 2 miscarriages. Six (33%, 95% CI (11.9 to 54.3)) patients experienced severe cardiac events. The concerned women had lower resting oxygen saturation (79.6±4.1% vs 89.3±3.8%, p<0.01). The most common cardiac complications during the complete pregnancies were heart failure (n=4) and severe hypoxaemia (n=5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality=5.0% 95% CI (0.1 to 24.9)). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred. Conclusions Outcome of pregnancy in women with PAH-CHD is better than previously reported, with only 5% maternal mortality in our cohort. However, because of the severity of heart failure and the high rate of neonatal complications, patients should still be advised against pregnancy.

Imaging in pulmonary hypertension: Focus on the role of echocardiography

Patients with pulmonary hypertension must be evaluated using a multimodality approach to ensure a correct diagnosis and basal evaluation as well as a prognostic assessment. Beyond the assessment of pulmonary pressures, the echocardiographical examination allows the evaluation of right ventricular adaptation to elevated afterload. Numbers of variables are commonly used in the assessment of the pulmonary hypertension patient in order to detect changes in right heart geometry, right-to-left interaction and right ventricular dysfunction. Whereas an isolated change in one echocardiographical variable is not meaningful, multiple echocardiographical variable modifications together provide accurate information. In this review, we will link pulmonary hypertension pathophysiological changes with echocardiographical indices and describe the clinical implications of echocardiographical findings.

Real time three-dimensional echocardiographic assessment of left ventricular function in heart failure patients: underestimation of left ventricular volume increases with the degree of dilatation.

Background: Accurate quantification of left ventricular (LV) volumes and ejection fraction (EF) is of critical importance. Cardiac magnetic resonance (CMR) is considered as the reference and three‐dimensional echocardiography (3DE) is an accurate method, but only few data are available in heart failure patients. We therefore sought to compare the accuracy of real time three‐dimensional echocardiography (RT3DE) and two‐dimensional echocardiography (2DE) for quantification of LV volumes and EF, relative to CMR imaging in an unselected population of heart failure patients. Methods and Results: We studied 24 patients (17 men, age 58 ± 15 years) with history of heart failure who underwent echocardiographic assessment of LV function (2DE, RT3DE) and CMR within a period of 24 hours. Mean LV end‐diastolic volume (LVEDV) was 208 ± 109 mL (121 ± 64 mL/m2) and mean LVEF was 31 ± 12.8%. 3DE data sets correlate well with CMR, particularly with respect to the EF (r: 0.8, 0.86, and 0.95; P < 0.0001 for LVEDV, LVESV, and EF, respectively) with small biases (–55 mL, –44 mL, 1.1%) and acceptable limits of agreement. RT3DE provides more accurate measurements of LVEF than 2DE (z= 2.1, P = 0.037) and lower variability. However, 3DE‐derived LV volumes are significantly underestimated in patients with severe LV dilatation. In patients with LVEDV below 120 mL/m2, RT3DE is more accurate for volumes and EF evaluation. Conclusion: Compared with CMR, RT3DE is accurate for evaluation of EF and feasible in all our heart failure patients, at the expense of a significant underestimation of LV volumes, particularly when LVEDV is above 120 mL/m2.

From adolescents to adults with congenital heart disease: the role of transition

AbstractImproved surgical care during the last decades, together with advances in medical management, led to a remarkable increase in survival of patients with congenital heart disease (CHD). However, aging of the CHD population brings new challenges, and loss of follow-up of adolescents and adults with CHD is a major concern. It is crucial to optimize the transfer of patients with CHD from paediatric to adult health care services to prevent loss to follow-up. The transition process plays a central role in the future health and follow-up of the patient. The aim of this review is to explain and discuss the clinical impact of the transition process in adolescents with CHD. We will also discuss specific CHD adolescents’ problems. Conclusion: Adolescence is a crucial phase for the formation of the personality. Understanding and acceptance of the responsibility for health at this stage through a transition process with a multidisciplinary team will determine the quality of future medical follow-up and probably limit psychosocial issues in their adult life.What is known:• Aging of the congenital heart disease population brings new challenges to the organisation of care.• Loss of follow-up is a major concern for patients with congenital heart disease.What is new:• The quality of a formal transition process during adolescence will determine future outcomes in patients with congenital heart disease.

Doubling the dose of Clopidogrel restores the loss of antiplatelet effect induced by esomeprazole

INTRODUCTION Concerns have been raised about the potential adverse interaction between clopidogrel and PPIs. We studied the impact of esomeprazole and ranitidine on the antiplatelet action of clopidogrel and aspirin and sought to determine whether doubling the dose of clopidogrel could restore its efficacy. MATERIALS AND METHODS In a randomized prospective crossover study, we tested platelet reactivity to aspirin and clopidogrel (75 and 150 mg) with and without esomeprazole or ranitidine using the VerifyNow system (Accumetrics Inc, San Diego, CA, USA) in 4 stages, each lasting 7 days: T1, 160 mg aspirin and 75 mg clopidogrel; T2 : 160 mg aspirin+75 mg clopidogrel+20 mg esomeprazole, T3 : 160 mg aspirin+150 mg clopidogrel+20 mg esomeprazole and T4 : 160 mg aspirin+75 mg clopidogrel+150 mg ranitidine. Results are expressed in P2Y12 Reaction Units (PRU%) and Aspirin Reaction Units (ARU). RESULTS In 21 patients with stable coronary artery disease, esomeprazole reduced the effect of clopidogrel with a 38.6%±24 loss in PRU% (p<0.001) (absolute mean difference -16.7 PRU% [-21;-12.5]), increasing 8-fold the prevalence of low responders to clopidogrel (defined as patients with PRU% below 20%). Doubling clopidogrel dosage to 150 mg restored the basal response. Ranitidine did not modify the antiplatelet effect of clopidogrel. CONCLUSION Our study demonstrates a strong negative clopidogrel/esomeprazole interaction, compensated by increasing the dose of clopidogrel to 150 mg or replacing esomeprazole with ranitidine. That could offer a simple solution to the PPI-induced clopidogrel resistance.

Tako-Tsubo cardiomyopathy presenting with cardiogenic shock successfully treated with milrinone: A case report

We report the case of a middle age patient presenting with Tako-Tsubo cardiomyopathy (TTC) complicated by cardiogenic shock that was successfully handled with milrinone. A 64-year old man presented with cardiogenic shock after benzodiazepine and alcohol intoxication. A slight elevation of troponin and typical left ventricular ballooning without coronary lesions suggested TTC. Within a few hours milrinone infusion normalized the cardiac index. TTC is responsible for severe transient left ventricular dysfunction occurring after physical or psychological stress. The major pathophysiological mechanism involved is disproportionate catecholamine secretion, which may stun the myocardium. We considered if treatment of this unique physiopathology with catecholamines could be dangerous in these patients and if alternative inotropes such as milrinone should be preferred.

Left main coronary artery compression associated with primary pulmonary hypertension.

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4] A 61-year-old man with known primary pulmonary arterial hypertension had typical angina when lying on his back. Strangely, in the left lateral decubitus position, the pain disappeared completely. Coronary 64-slice multidetector

Early detection of cardiac involvement in sarcoidosis with 2-dimensional speckle-tracking echocardiography.

BACKGROUND/OBJECTIVES Cardiac sarcoidosis (CS) is associated with high morbidity and sudden death. The absence of specific symptoms and lack of diagnostic gold standard technique is challenging. New imaging methods could improve the diagnosis of CS. The aim of our study was to assess the role of left ventricular (LV) longitudinal and circumferential strain as estimated by 2D speckle-tracking imaging in patients with diagnosed sarcoidosis without cardiac involvement according to the current guidelines. We investigated the prevalence of LV strain impairment in this population and assessed its relationship with clinical outcomes, composite of mortality, heart failure, arrhythmia and/or secondarily development of CS and cardiac device implantation. METHODS AND RESULTS We performed a prospective case-control longitudinal study including 35 patients with diagnosed sarcoidosis and normal cardiac function as assessed by standard transthoracic echocardiography and 35 healthy age- and gender-matched controls. All patients underwent a comprehensive echocardiographic study. Mean age of patients was 47.9±14.8years old (22 women). Compared with controls, global LV longitudinal strain (LV GLS) was reduced in sarcoidosis patients: (-17.2±3.1 vs -21.3±1.5%, p<0.0001). Circumferential LV strain was preserved in patients compared to controls (-19.9±-4.3% vs -21.3±1.5%, p=0.12). Impaired LV GLS was significantly associated with clinical outcomes (HR 1.56; [1.16-2.11], p<0.01) on univariate analysis. CONCLUSION Speckle-tracking echocardiography revealed decreased longitudinal LV strain in sarcoidosis patients that was associated with outcomes. LV GLS may represent an early marker of myocardial involvement in sarcoidosis patients that needs to be studied further.

Arterial Tortuosity Syndrome: Early Diagnosis and Association With Venous Tortuosity

![Figure][1] [![Graphic][3] ][3] A newborn, first child of consanguineous parents was admitted to intensive care because of persistent pulmonary hypertension. The diagnosis of arterial tortuosity syndrome (ATS) was suspected due to severe tortuosity of the aorta (A) and pulmonary