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 36.0Ϯ14.2 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 (Ն90 mL · min Ϫ1 · 1.73 m Ϫ2 ), mildly impaired GFR (60 to 89 mL · min Ϫ1 · 1.73 m Ϫ2), and moderately/severely impaired GFR (Ͻ60 mL · min Ϫ1 · 1.73 m Ϫ2). 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 (Ͻ90 mL · min Ϫ1 · 1.73 m Ϫ2 , PϽ0.0001 compared with the remainder), and in 18%, this was moderate or severe (Pϭ0.007). Renal dysfunction had a substantial impact on mortality (propensity score-weighted hazard ratio 3.25, 95% CI 1.54 to 6.86, Pϭ0.002 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. (Circulation. 2008;117:2320-2328.)Key Words: heart defects, congenital Ⅲ kidney Ⅲ renal function Ⅲ prognosis A s the number of patients with congenital heart disease reaching adulthood (ACHD) continues to increase, it is becoming clear that pathophysiological derangement occurs not only in the heart but in other organs as well. Renal dysfunction has been reported in ACHD patients, but its prevalence and relation to outcome in this population remain unknown. [1][2][3][4][5][6][7][8] In acquired heart disease, renal dysfunction is an ominous sign. 9 -14 We sought to assess the prevalence of renal dysfunction across the spectrum of ACHD and its predictors and impact on survival. Editorial p 2311 Clinical Perspective p 2328 MethodsAll ACHD patients attending our institution from 1999 to June 2006 and in whom serum creatinine concentration was measured were entered into the study. Only the first measurement was used if there were several. For each subject, an estimated glomerular filtration rate (GFR) was calculated from serum creatinine levels by the Modification of Diet in Renal Disease equation, which adjusts for age, gender, and race. 15 Patients were categorized into groups according to the cutoff values suggested by the National Kidney Foundation practice guidelines: GFR Ͼ90 mL · min Ϫ1 · 1.73 m Ϫ2 was considered normal, 60 to 89 mL · min Ϫ1 · 1.73 m Ϫ2 was considered mildly decreased, and Ͻ60 mL · min Ϫ1 ...

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.

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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.