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Background: Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD)., Objectives: The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure., Methods: Patients who underwent surgical VSD closure during childhood between 1968 and 1980 were reexamined every 10 years. In 2012, we invited eligible patients to undergo another examination, which included electrocardiography, Holter monitoring, echocardiography, bicycle ergometry, measurement of N-terminal pro-B-type natriuretic peptide, and subjective health assessment., Results: Cumulative survival was 86% at 40 years. Causes of mortality were arrhythmia, heart failure, endocarditis, during valvular surgery, pulmonary hypertension, noncardiac causes, and unknown causes. Cumulative event-free survival after surgery was 72% at 40 years. Symptomatic arrhythmias occurred in 13% of patients and surgical or catheter-based reinterventions in 12%. Prevalence of impaired right ventricular systolic function increased from 1% in 2001 to 17% in 2012 (p=0.001). Left ventricular systolic function was impaired but stable in 21% of patients. Aortic regurgitation occurred more often in the last 20 years (p=0.039), and mean exercise capacity decreased (p=0.003). N-terminal pro-B-type natriuretic peptide (median: 11.6 pmol/l [interquartile range: 7.0 to 19.8 pmol/l]) was elevated (>14 pmol/l) in 38% of patients. A concomitant cardiac lesion, for example, patent ductus arteriosus, and aortic cross-clamp time were determinants of late events (hazard ratio: 2.84 [95% confidence interval: 1.23 to 6.53] and hazard ratio: 1.47 per 10 min [95% confidence interval: 1.22 to 1.99], respectively). Patients rated their subjective health status significantly better than a reference population., Conclusions: Survival up to 40 years after successful surgical VSD closure is slightly lower than in the general Dutch population. Morbidity is not negligible, especially in patients with a concomitant cardiac lesion., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to determine the value of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in adults with congenital heart disease (CHD) and investigate its relationship with ventricular function and exercise capacity., Background: NT-proBNP may detect early deterioration in cardiac function., Methods: In this cross-sectional study, extensive echocardiography, exercise testing, and NT-proBNP measurements were performed on the same day in consecutive adult patients with CHD., Results: In total, 475 patients were included in this study (mean age of 34 ± 12 years, 57% male, 90% New York Heart Association class I). The median NT-proBNP level was 15.1 pmol/l (interquartile range [IQR]: 7.1 to 31.3 pmol/l), and the NT-proBNP level was >14 pmol/l in 53% of patients. The highest NT-proBNP levels were observed in patients with Fontan circulation (36.1 pmol/l [IQR: 14.4 to 103.8 pmol/l]) and a systemic right ventricle (RV) (31.1 pmol/l [IQR: 21.8 to 56.0 pmol/l]), and the lowest values were seen in patients with aortic coarctation (7.3 pmol/l [IQR: 2.8 to 19.5 pmol/l]). NT-proBNP levels correlated with age (r = 0.39, p < 0.001) and were higher in women (median of 21.7 vs. 10.4 pmol/l; p < 0.001). In patients with aortic stenosis or aortic coarctation, NT-proBNP levels correlated with diastolic function parameters of E/E' ratio (r = 0.40, p < 0.001) and left atrial dimension (r = 0.36, p < 0.001). In patients with a systemic RV, NT-proBNP levels correlated with RV annulus diameter (r = 0.31, p = 0.024). In patients with tetralogy of Fallot, the strongest correlations were observed with left atrial dimension (r = 0.46, p < 0.001) and left ventricular ejection fraction (r = 0.37, p < 0.001). NT-proBNP levels were associated with exercise capacity (n = 198) (maximum workload: β = -0.08, p = 0.021) and peak oxygen uptake (β = -0.012, p = 0.011) in a multivariable regression model adjusted for age and sex., Conclusions: NT-proBNP levels in adults with CHD clearly differ by diagnosis and are related to echocardiographic parameters and exercise capacity. Disease-specific correlations contribute to the understanding of the main hemodynamic problems per diagnosis. Follow-up data are needed to elucidate the additional prognostic value., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Congenital heart disease (CHD) accounts for nearly one-third of all major congenital anomalies. CHD birth prevalence worldwide and over time is suggested to vary; however, a complete overview is missing. This systematic review included 114 papers, comprising a total study population of 24,091,867 live births with CHD identified in 164,396 individuals. Birth prevalence of total CHD and the 8 most common subtypes were pooled in 5-year time periods since 1930 and in continent and income groups since 1970 using the inverse variance method. Reported total CHD birth prevalence increased substantially over time, from 0.6 per 1,000 live births (95% confidence interval [CI]: 0.4 to 0.8) in 1930 to 1934 to 9.1 per 1,000 live births (95% CI: 9.0 to 9.2) after 1995. Over the last 15 years, stabilization occurred, corresponding to 1.35 million newborns with CHD every year. Significant geographical differences were found. Asia reported the highest CHD birth prevalence, with 9.3 per 1,000 live births (95% CI: 8.9 to 9.7), with relatively more pulmonary outflow obstructions and fewer left ventricular outflow tract obstructions. Reported total CHD birth prevalence in Europe was significantly higher than in North America (8.2 per 1,000 live births [95% CI: 8.1 to 8.3] vs. 6.9 per 1,000 live births [95% CI: 6.7 to 7.1]; p < 0.001). Access to health care is still limited in many parts of the world, as are diagnostic facilities, probably accounting for differences in reported birth prevalence between high- and low-income countries. Observed differences may also be of genetic, environmental, socioeconomical, or ethnic origin, and there needs to be further investigation to tailor the management of this global health problem., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Although recommended by several investigators, the benefit of early surgery in patients with fixed subaortic stenosis has not been proved. Findings were reviewed of 57 patients with isolated fixed subaortic stenosis, including 27 surgically treated patients, with special emphasis on the occurrence of aortic regurgitation during a mean follow-up period of 6.7 years. The number of patients with aortic regurgitation increased preoperatively in the total group (23% at diagnosis to 54% after 3.7 years of follow-up). The prevalence of aortic regurgitation in the 27 surgically treated patients was higher (81%) than that in the nonsurgically treated group but remained unchanged after a mean postoperative period of 4.7 years. In all patients but one, aortic regurgitation remained of minor hemodynamic significance. One patient died during follow-up. After surgery, 15 patients (55%) showed a relapse; 11 redeveloped a subvalvular pressure gradient greater than 30 mm Hg and discrete subvalvular ridges (range 6 months to 24 years after surgery, mean 7 years). In those patients with fixed subaortic stenosis, follow-up did not reveal any benefit from early surgery. The unpredictable course and sometimes very severe progression of this disease make frequent and careful follow-up necessary.

Transesophageal echocardiography was used prospectively in 22 children scheduled for interventional cardiac catheterization (9 with pulmonary valvuloplasty, 5 with aortic valvuloplasty, 1 with pulmonary angioplasty, 2 with aortic angioplasty, 2 with patent ductus arteriosus occlusion and 3 with Mustard baffle dilation) to determine its potential value as a monitoring technique. The patients ranged in age from 0.9 to 14.6 years (mean 5.4) and in weight from 9.5 to 49.2 kg (mean 21.1). Studies were completed in all patients without complications. Preintervention studies provided important new information in two patients, leading to cancellation of the planned procedure. Major contributions of transesophageal monitoring included 1) a real time assessment of catheter placement across either atrioventricular valve and the aortic valve during balloon valvuloplasty; 2) immediate assessment of aortic valve and aortic wall morphology during balloon dilation; and 3) detailed morphologic and hemodynamic information together with enhanced catheter guidance during Mustard baffle dilation. After pulmonary valvuloplasty, partial chordal rupture of the tricuspid valve was documented in one patient. In two patients, balloon catheter position was modified according to the transesophageal findings. The assessment of changes in pulmonary valve morphology and transcatheter occlusion of a patent ductus arteriosus was not enhanced by single-plane transesophageal monitoring. Pulsed wave Doppler studies contributed additional information in the assessment of immediate hemodynamic changes after interventional procedures. Transesophageal echocardiography is a new important guiding and monitoring technique during interventional cardiac catheterization procedures in children. It can provide additional real time imaging information, immediate identification of complications and assessment of hemodynamic changes.

The etiology of diastolic motion of the pulmonary valve seen on the M-mode echocardiogram has been the subject of much debate. To further investigate diastolic events in the pulmonary artery, the patterns of diastolic pulmonary artery blood flow velocity were studied using pulsed Doppler echocardiography in patients with a normal heart. Two diastolic waveforms were found, one in early diastole related to passive filling of the right ventricle and one in late diastole related to atrial contraction. These waveforms were also related to the two recognized phases of diastolic pulmonary valve motion detected by M-mode echocardiography. The presence of biphasic diastolic blood flow in the pulmonary artery was confirmed by electromagnetic flow velocimetry in four additional patients with various cardiac diseases and normal right heart pressures. It is concluded that both atrial contraction and passive right ventricular filling produce blood flow in the pulmonary artery.

Intraoperative epicardial two-dimensional echocardiography was used in 195 patients undergoing surgery for congenital heart disease to evaluate its potential to identify new diagnostic information immediately before and after surgical correction. In 168 patients the preoperative diagnosis was confirmed by intraoperative echocardiography. In four patients, unsuspected findings were revealed, which resulted in modification of the surgical approach. In 18 patients additional morphologic information was obtained which contributed to alteration or refinement of surgical management. The adequacy of cardiac repair was assessed before closure of the chest in all patients. In six patients this information led to immediate reoperation and in four other patients to inotropic drug therapy. During congenital heart surgery, epicardial two-dimensional echocardiography may yield important information for surgical management. The technique is an essential adjunct when preoperative diagnostic studies are not conclusive or when the initial response to repair is unsatisfactory.