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Background: Risk factors for adverse clinical outcomes have been identified in patients with repaired tetralogy of Fallot before pulmonary valve replacement (PVR). However, pre-PVR predictors for post-PVR sustained ventricular tachycardia and death have not been identified. Methods: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort (International Multicenter TOF Registry), a 4-center international cohort study, who had a comprehensive preoperative evaluation and subsequently underwent PVR were included. Preprocedural clinical, ECG, cardiovascular magnetic resonance, and postoperative outcome data were analyzed. Cox proportional hazards multivariable regression analysis was used to evaluate factors associated with time from pre-PVR cardiovascular magnetic resonance until the primary outcome: death, aborted sudden cardiac death, or sustained ventricular tachycardia. Results: Of the 452 eligible patients (median age at PVR, 25.8 years), 36 (8%) reached the primary outcome (27 deaths, 2 resuscitated death, and 7 sustained ventricular tachycardia) at a median time after PVR of 6.5 years. Cox proportional hazards regression identified pre-PVR right ventricular ejection fraction P =0.02), right ventricular mass-to-volume ratio ≥0.45 g/mL (hazard ratio, 4.08; 95% CI, 1.57–10.6; P =0.004), and age at PVR ≥28 years (hazard ratio, 3.10; 95% CI, 1.42–6.78; P =0.005) as outcome predictors. In a subgroup analysis of 230 patients with Doppler data, predicted right ventricular systolic pressure ≥40 mm Hg was associated with the primary outcome (hazard ratio, 3.42; 95% CI, 1.09–10.7; P =0.04). Preoperative predictors of a composite secondary outcome, postoperative arrhythmias and heart failure, included older age at PVR, pre-PVR atrial tachyarrhythmias, and a higher left ventricular end-systolic volume index. Conclusions: In this observational investigation of patients with repaired tetralogy of Fallot, an older age at PVR and pre-PVR right ventricular hypertrophy and dysfunction were predictive of a shorter time to postoperative death and sustained ventricular tachycardia. These findings may inform the timing of PVR if confirmed by prospective clinical trials.

Aims The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality. Methods and results This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P

Background: The fetal heart at term is exposed to an increase in hemodynamic work as a consequence of fetal growth, increased circulating volume and alteration in loading patterns due to maturational changes in fetoplacental circulation. The extent to which these cardiovascular changes influence the human fetal and neonatal cardiac adaptation has not been fully elucidated. The aim of this study was to evaluate perinatal cardiovascular changes in ventricular geometry and myocardial performance in normal term fetuses.\ud \ud Methods: Prospective study of 108 uncomplicated pregnancies delivering at term. M-mode, twodimensional (2D) or B-mode, pulsed wave (PW) Doppler, PW tissue Doppler and 2D speckle\ud tracking imaging were performed a few days before, and within 24 hours of birth.\ud \ud Results: Analysis of paired fetal and neonatal echoes demonstrated significant perinatal changes (p

Introduction: The prognostic impact and optimal timing of pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) remains a subject of debate. The objective of this study was to determine whether PVR was associated with beneficial outcome. Methods: In this multicenter, case-controlled study, clinical, ECG, cardiac magnetic resonance (CMR) and outcome data of rTOF patients were collected. A propensity score for PVR was created to adjust for baseline differences between PVR and non-PVR patients. A frailty failure time model was used to determine the association of PVR, with matching on propensity score and adjustment for other risk factors, on time to the composite of death/sustained ventricular tachycardia (VT). In addition, differential association between impact of PVR and outcome according to predefined ‘proactive’ and ‘conservative’ (see figure) criteria was studied. Results: A total of 440 PVR patients (age 24±13 years at baseline CMR, 58% male, RV ejection fraction (EF) 47±8%, QRS duration 148±27ms) and 537 non-PVR patients (age 29±15 years, 53% male, RV EF 51±8%, QRS duration 140±27ms) were included. During follow-up of 5.3±3.1 years, 41 events occurred (30 death, 11 sustained VT). The adjusted hazard ratio (HR) for death/sustained VT for patients with versus without PVR was 0.65 (95% CI: 0.31, 1.36, p=0.25). There was an increasing hazard of event across prespecified ordered PVR x criteria subgroups (Figure; P=0.044 for proactive, P=0.002 for conservative). There was a two-fold risk (hazard ratio=2.15, 95% CI: 1.34, 3.46) comparing each adjacent subgroup, suggesting benefit of PVR when conservative criteria were met, and lack of benefit of PVR when criteria were not met. A roughly similar pattern was seen according to proactive criteria. Conclusions: In this multicenter, 5-year follow-up study in patients with rTOF, conservative guidelines seemed useful in selecting patients for PVR. Longer follow-up is necessary to confirm these findings.

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In patients with cyanosis from congenital heart disease, erythropoiesis is governed by many factors that may alter the expected relation between oxygen saturation (O2sat) and hemoglobin concentration. We sought to define the relation between O2sat and hemoglobin in such patients and to predict an ideal hemoglobin concentration for a given O2sat. Adults with congenital heart defects and cyanosis were studied prospectively with blood tests and exercise testing. Non-optimal hemoglobin was defined as any evidence of inadequate erythropoiesis; namely iron, folate, or B12 deficiency, raised erythropoietin, reticulocytosis, or a right-shifted oxygen-hemoglobin curve. For patients without these factors, a linear regression equation of hemoglobin vs.O2sat was used to predict the optimal hemoglobin for all patients. Of 65 patients studied, 21 met all pre-study criteria for optimal hemoglobin. For all patients there was no correlation between O2sat and hemoglobin (r=-0.24), whereas there was a strong linear correlation for those meeting criteria for optimal hemoglobin (r=-0.888, p

Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart disease (CHD). Greater numbers of patients with PAH associated with CHD (PAH-CHD) are now surviving into adulthood, many with increasingly complex cardiac defects. Patients with cardiac defects which result in left-to-right shunting are at risk of developing PAH, owing to the increased shear stress and circumferential stretch induced by increased pulmonary blood flow, which leads to endothelial dysfunction and progressive vascular remodelling and, thus, increased pulmonary vascular resistance. Although PAH-CHD shares similar lung histology with idiopathic PAH, differences do exist between these aetiologies. Management of PAH-CHD can involve surgical correction of the cardiac defect and/or treatment of the PAH, depending on the underlying cardiac defect and status of disease progression. Transplantation surgery can be curative but is not without limitations. The timing of intervention in patients with PAH-CHD is important, but the optimums are sometimes difficult to define, with limited robust data to inform management decisions. Uncontrolled studies suggest that prostacyclin analogues and phosphodiesterase type-5 inhibitors may have benefits in advanced pulmonary vascular disease. In the only randomised controlled trial dedicated to end-stage PAH-CHD, bosentan significantly reduced pulmonary vascular resistance and significantly increased 6-minute walk distance without compromising peripheral oxygen saturation, in patients with Eisenmenger syndrome. These data suggest that targeted therapies are beneficial in the PAH-CHD population, and warrant further research.

Objectives: This study sought to determine what factors are associated with pulmonary artery thrombi in Eisenmenger patients.\ud \ud Background: Pulmonary artery thrombosis is common in Eisenmenger syndrome, although its underlying pathophysiology is poorly understood.\ud \ud Methods: Adult patients with Eisenmenger syndrome underwent computed tomography pulmonary angiography, cardiac magnetic resonance imaging, and echocardiography. Measurement of ventricular function, pulmonary artery size, and pulmonary artery blood flow were obtained. Hypercoagulability screening and platelet function assays were performed.\ud \ud Results: Of 55 consecutive patients, 11 (20%) had a detectable thrombus. These patients were older (p = 0.032), but did not differ in oxygen saturation, hemoglobin, or hematocrit from those without thrombus. Right ventricular ejection fraction by magnetic resonance imaging was lower in those with thrombus (0.41 ± 0.15 vs. 0.53 ± 0.13, p = 0.017), as was left ventricular ejection fraction (0.48 ± 0.12 vs. 0.60 ± 0.09, p = 0.002), a finding corroborated by tissue Doppler and increased brain natriuretic peptide. Those with thrombus also had a larger main pulmonary artery diameter (48 ± 14 mm vs. 38 ± 9 mm, p = 0.007) and a lower peak systolic velocity in the pulmonary artery (p = 0.003). There were no differences in clotting factors, platelet function, or bronchial arteries between groups. Logistic regression showed pulmonary artery velocity to be independently associated with thrombosis.\ud \ud Conclusions: Pulmonary arterial thrombosis among adults with Eisenmenger syndrome is common and relates to older age, biventricular dysfunction, and slow pulmonary artery blood flow rather than degree of cyanosis or coagulation abnormalities. Further work to define treatment efficacy is needed.

Aims To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period. Methods and results Data collected as part of the Euro Heart Survey on adult CHD was analysed. This entailed information transcribed from the files of 4110 patients diagnosed with one of eight congenital heart conditions ('defects'), who consecutively visited the outpatient clinics of one of the participating centres in 1998. The patients were included retrospectively and followed until the end of 2003 for a median follow-up of 5.1 years. Notwithstanding their overall relatively good functional class and low mortality over the follow-up period, a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. There were major differences between the eight defects, both in morbidity and regarding specific characteristics. Outcomes were worst in cyanotic defects and in the Fontan circulation, but a considerable proportion of the other patients also suffer from cardiac symptoms. In particular, arrhythmias are common. Conclusion The spectrum of adult CHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period