Hyponatraemia: A strong predictor of mortality in adults with congenital heart disease.

AIMS: We studied the prevalence of hyponatraemia and its prognostic implications in a large population of adult patients with congenital heart disease (ACHD). METHODS AND RESULTS: A total of 1004 ACHD patients were retrospectively entered in this study (mean age 36.2 +/- 14.4 years, 48.7% male). Cox regression was used to estimate mortality associated with hyponatraemia, adjusted for potential confounders using both multivariable regression and propensity score matching. Mean sodium concentration in this ACHD cohort was 137.6 +/- 2.6 mmol/L. The overall prevalence of hyponatraemia in this cohort was 15.5% and was highest in congenitally corrected transposition (33.3%), after Fontan operation (29.6%), and in patients with Eisenmenger syndrome (22.0%). Predictors of hyponatraemia were worse functional class, cyanosis, higher serum creatinine levels, and treatment with diuretics. Patients were followed for a median of 4.1 years, during which there were 96 deaths. Hyponatraemia was a strong predictor of death, independent of age, previous surgery, functional class, systemic ventricular function, creatinine levels, and the use of diuretics (adjusted HR 2.82, 95% CI: 1.72-4.63, P < 0.0001). CONCLUSION: Hyponatraemia is relatively common in ACHD. Hyponatraemia carries a three-fold higher risk of death in ACHD and is a simple, cheap but powerful marker of mortality.

Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease.

BACKGROUND: Renal insufficiency in patients with ischemic heart disease and acquired heart failure is associated with higher mortality and morbidity. We studied the prevalence of renal dysfunction in adult patients with congenital heart disease (ACHD) and its relation to outcome. METHODS AND RESULTS: A total of 1102 adult patients with congenital heart disease (age 36.0+/-14.2 years) attending our institution between 1999 and 2006 had creatinine concentration measured. Glomerular filtration rate (GFR) was calculated with the Modification of Diet in Renal Disease equation. Patients were divided into groups of normal GFR (> or =90 mL . min(-1) . 1.73 m(-2)), mildly impaired GFR (60 to 89 mL . min(-1) . 1.73 m(-2)), and moderately/severely impaired GFR (<60 mL . min(-1) . 1.73 m(-2)). Survival was compared between GFR groups by Cox regression. Median follow-up was 4.1 years, during which 103 patients died. Renal dysfunction was mild in 41% of patients and moderate or severe in 9%. A decrease in GFR was more common among patients with Eisenmenger physiology, of whom 72% had reduced GFR (<90 mL . min(-1) . 1.73 m(-2), P<0.0001 compared with the remainder), and in 18%, this was moderate or severe (P=0.007). Renal dysfunction had a substantial impact on mortality (propensity score-weighted hazard ratio 3.25, 95% CI 1.54 to 6.86, P=0.002 for moderately or severely impaired versus normal GFR). CONCLUSIONS: Deranged physiology in adult patients with congenital heart disease is not limited to the heart but also affects the kidney. Mortality is 3-fold higher than normal in the 1 in 11 patients who have moderate or severe GFR reduction.

Effect of pregnancy on clinical status and ventricular function in women with heart disease.

BACKGROUND: Pregnant women with heart disease (HD) are at an increased risk for maternal and neonatal adverse events. However, the effect of pregnancy on clinical status and ventricular function in women with HD has not been examined in a controlled study. METHODS AND RESULTS: Ninety-three women with HD were studied longitudinally. Of these, fifty-three underwent clinical and echocardiographic evaluation before and 1.5+/-1.1 years after pregnancy (pregnancy group), whereas forty served as controls matched for age (28.6+/-4.6 versus 28.5+/-6.6, p=0.88), diagnosis, and length of follow-up (2.9+/-1.4 versus 2.6+/-1.1, p=0.23). NYHA functional class remained unchanged in both groups during follow-up. End diastolic and end systolic dimensions and shortening fraction of the morphologic left ventricle also remained unchanged. Furthermore, systemic and subpulmonary ventricular function remained unchanged in the pregnancy and control groups on semiquantitative analysis. Pregnancy, however, was associated with a persisting increase in subpulmonary ventricular size in patients with tetralogy of Fallot (ToF) which was not present in tetralogy controls. Furthermore, diagnosis of ToF was the only predictor of an increase in subpulmonary ventricular size after pregnancy on univariate logistic regression analysis (OR 8.8[95% CI 1.9-41.1], p=0.006). CONCLUSIONS: In this longitudinal controlled study amongst women with HD no deleterious midterm effects of pregnancy on clinical status and right and left ventricular function were found. However, pregnancy was associated with a persisting increase in subpulmonary ventricular size, attributable to patients with repaired ToF. This may have prognostic implications and merits further investigation.

Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease.

OBJECTIVE: To examine long-term safety and efficacy of bosentan--an oral dual endothelin receptor antagonist--in patients with pulmonary hypertension associated with congenital heart disease or Eisenmenger's syndrome. DESIGN: Retrospective study. SETTING: Tertiary cardiology referral centre. PATIENTS: All adult patients with pulmonary arterial hypertension associated with congenital heart disease treated with bosentan at the Royal Brompton Adult Congenital Heart Centre were included. MAIN OUTCOME MEASURES: Oxygen saturation, functional (WHO) class, 6-minute walk test distance and liver enzymes were analysed. RESULTS: Eighteen patients (14 female) with pulmonary arterial hypertension associated with congenital heart disease (15 patients with Eisenmenger's syndrome) with a mean (SD) age of 41 (9) years (range 23-69) were included. Median follow-up was 29 months (range 1-39). One patient died during follow-up. Patients tolerated bosentan well and no significant rise in liver transaminases was seen. Arterial oxygen saturation remained stable throughout follow-up. Mean (SD) functional class (p = 0.001) and the 6-minute walk test distance improved compared with baseline (284 (144) vs 363 (124) m, 380 (91) m and 408 (114) m at baseline, 0-6 months, 6-12 months and 1-2 years of treatment, respectively; p<0.05 for each). CONCLUSIONS: Bosentan appears to be safe and well tolerated in adults with pulmonary arterial hypertension associated with congenital heart disease or Eisenmenger's syndrome during mid- to long-term follow-up. In addition, functional class and the 6-minute walk test distance improved and this effect was maintained for up to 2 years of bosentan treatment.