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To assess maternal and fetal outcome of pregnancy in patients with congenitally corrected transposition of the great arteries, we reviewed 19 patients (18 retrospectively) who had 45 pregnancies. Their ages ranged from 18 to 40 years (mean 27) at the time of pregnancy. Thirty-six percent of the pregnancies were undertaken while patients were cyanosed, 7% in patients with unpaced complete heart block, and 16% were undertaken after surgical repair of the associated anomalies. Change in functional class and maternal cardiovascular complications during pregnancy were analyzed as well as number of live births, miscarriages, elective termination of pregnancies, timing of delivery, and incidence of cardiac defects in the live offspring. Five patients (26% of patients) developed cardiovascular complications during pregnancy, namely congestive heart failure (3 patients), worsening cyanosis (1 patient), and cerebrovascular accident (1 patient). No maternal deaths occurred. There were 27 live births (60%), 12 miscarriages (27%), and 6 elective terminations of pregnancy (13%). Cyanosis was a significant risk factor for miscarriage. One live offspring had congenital heart disease. Close follow-up of these patients during pregnancy, by a team of experienced physicians, is recommended. [ABSTRACT FROM AUTHOR]

As an increasing number of patients with congenital heart disease reach adulthood, more information is needed regarding outcomes. The first signs of impaired heart function may appear during exercise testing. The aim of the present study was to establish mean values for maximal oxygen uptake in adults with various congenital heart diseases. Patients from 6 major diagnostic groups were identified, including patients with atrial septal defect (ASD, n = 93), transposition of the great arteries corrected with the Mustard procedure (n = 84), congenitally corrected transposition of the great arteries (CCTGA, n = 41), Tetralogy of Fallot (n = 168), Ebstein's anomaly (n = 37), and Modified Fontan procedure (n = 52). Diminished maximal oxygen uptake was found in all diagnostic groups across age compared with healthy subjects. A significant decrease in maximal oxygen uptake with aging was found in those with ASD (p <0.0001), CCTGA (p = 0.01), and Tetralogy of Fallot (p <0.0001). There was no significant decline, however, in Ebstein's anomaly (p = 0.270), Fontan procedure (p = 0.182), and in the Mustard patients (p = 0.188). All patients achieved significantly lower heart rates than predicted (mean for all groups, p <0.0001). Forced vital capacity values (3.51 L, mean SD +/- 1.02) were lower than predicted values (4.10 L, mean SD +/- 0.90, p <0.0001) for all patients groups except those with ASD. Mean values, however, were within the accepted 20% range of variance. This study showed diminished aerobic capacity in all diagnostic groups when compared with a healthy population. The maximal oxygen uptake values across age groups can be used as reference values in patients with similar diagnoses and as the basis for further research. [ABSTRACT FROM AUTHOR]

Examines right ventricular (RV) function before and after epoprostenol therapy using the myocardial performance index (MPI), and tests its utility as a marker of change in patients' clinical status. Improvement in RV MPI after therapy; Differences in estimated systolic pulmonary arterial pressure between the group that improved clinically and the group that did not.

Angiotensin-converting enzyme inhibitors had no significant effect on cardiopulmonary exercise function in 14 patients who had undergone a Mustard operation for transposition of the great arteries. In some patients aerobic capacity improved and maximum systolic blood pressure decreased. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) increases the risk of infective endocarditis (IE), though the lesion-specific risk and mortality are poorly defined. Using the population-based Quebec CHD database, we sought to describe the predictors of IE and to evaluate if IE was associated with mortality among adult CHD (ACHD) patients without prior valve replacement surgery. We extracted data on ACHD patients with IE and assessed the lesion-specific incidence of IE, risk factors for IE acquisition, and all-cause 1-year mortality. Among 29,866 ACHD patients, 285 (0.95%) developed IE during follow-up period of 378,901 patient-years, from 1988-2010. The highest and lowest lesion-specific incidences of IE were observed with left-sided lesions (1.61/1000 patient-years) and patent ductus arteriosus (0.24/1000 patient-years), respectively. The following predicted the risk of IE acquisition (odds ratio (OR), 95% confidence interval [CI]): cardiac surgery in the previous 6 months (9.07, 3.98-20.67), endocardial cushion defects (6.65, 3.84-11.53), left-sided lesions (5.11, 3.60-7.25), cyanosis at birth (4.82, 3.12-7.46), ventricular septal defect (2.81, 1.87-4.21), diabetes mellitus (1.65, 1.10-2.48), and recent medical interventions (12.52, 5.23-29.97). Twenty-five (8.77%) patients died within 1-year of IE diagnosis, a substantially elevated rate compared to patients without IE (OR 31.07, 95%CI 16.23-59.49). The risk of death following IE diagnosis was similarly elevated among patients with left-sided, cyanotic and other CHD lesions. In conclusion, the risk of IE in ACHD patients is lesion-specific and is greatest in the context of recent medical interventions. IE is associated with increased 1-year mortality, irrespective of broad CHD lesion grouping., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Atrial arrhythmias (AAs) are a common complication in adult patients with congenital heart disease. We sought to compare the lifetime prevalence of AAs in patients with right- versus left-sided congenital cardiac lesions and their effect on the prognosis. A congenital heart disease diagnosis was assigned using the International Disease Classification, Ninth Revision, diagnostic codes in the administrative databases of Quebec, from 1983 to 2005. Patients with AAs were those diagnosed with an International Disease Classification, Ninth Revision, code for atrial fibrillation or intra-atrial reentry tachycardia. To ensure that the diagnosis of AA was new, a washout period of 5 years after entry into the database was used, a period during which the patient could not have received an International Disease Classification, Ninth Revision, code for AA. The cumulative lifetime risk of AA was estimated using the Practical Incidence Estimators method. The hazard ratios (HRs) for mortality, morbidity, and cardiac interventions were compared between those with right- and left-sided lesions after adjustment for age, gender, disease severity, and cardiac risk factors. In a population of 71,467 patients, 7,756 adults developed AAs (isolated right-sided, 2,229; isolated left-sided, 1,725). The lifetime risk of developing AAs was significantly greater in patients with right- sided than in patients with left-sided lesions (61.0% vs 55.4%, p <0.001). The HR for mortality and the development of stroke or heart failure was similar in both groups (HR 0.96, 95% confidence interval [CI] 0.86 to 1.09; HR 0.94, 95% CI 0.80 to 1.09; and HR 1.10, 95% CI 0.98 to 1.23, respectively). However, the rates of cardiac catheterization (HR 0.63, 95% CI 0.55 to 0.72), cardiac surgery (HR 0.40, 95% CI 0.36 to 0.45), and arrhythmia surgery (HR 0.77, 95% CI 0.6 to 0.98) were significantly less for patients with right-sided lesions. In conclusion, patients with right-sided lesions had a greater lifetime burden of AAs. However, their morbidity and mortality were no less than those with left-sided lesions, although the rate of intervention was substantially different.

Adults with repaired tetralogy of Fallot and significant chronic pulmonary regurgitation are at risk for progressive right ventricular (RV) dilatation and dysfunction. The assessment of RV function is important in the management in these patients. There is still a lack of an adequate geometric model to quantify RV function by echocardiography. The myocardial performance index (MPI) is a nonvolumetric method to quantify global ventricular function. In this study, the accuracy of MPI obtained by echocardiography to quantify RV function was assessed in 57 adults with repaired tetralogy of Fallot. The MPI measurement was compared with the RV ejection fraction (EF) derived by cardiac magnetic resonance imaging. There was a negative linear correlation between the MPI and the RVEF (r = 0.73, p <0.001). A MPI cutoff of > or =0.40 had a sensitivity of 81% and a specificity of 85% to diagnose a RVEF <35%. A MPI cutoff of <0.25 had a sensitivity of 70% and a specificity of 89% to identify patients with RVEFs > or =0.50. In a multivariate regression model, the MPI was not affected by the degree of pulmonary regurgitation, the presence of tricuspid regurgitation, or the QRS duration. In conclusion, the Doppler-derived MPI is a simple and reliable method for the evaluation of RV systolic function in adults with repaired tetralogy of Fallot.

The timing of pulmonary valve replacement in adult patients with repaired tetralogy of Fallot remains controversial. A magnetic resonance imaging study in 17 adult patients with repaired tetralogy of Fallot reveals a statistically significant decrease in right ventricular (RV) volume (RV end-diastolic volume 163 +/- 34 to 107 +/- 26 ml/m2, p <0.001; RV end-systolic volume 109 +/- 27 to 69 +/- 22 ml/m2, p <0.001) at a mean follow-up of 21 months after pulmonary valve replacement; whereas RV systolic function remained unchanged (mean RV ejection fraction 32 +/- 7% to 34 +/- 10%, p = 0.12). In no patients with a RV end-diastolic volume >170 ml/m2 or a RV end-systolic volume >85 ml/m2 before pulmonary valve replacement were RV volumes "normalized" after surgery.