Abstract
Many Fontan patients with and without systolic ventricular dysfunction are being treated with angiotensin-converting enzyme (ACE) inhibitors, despite its effectiveness remaining unclear. In the present study, we evaluated the short-term effect of enalapril on exercise capacity, vascular and ventricular function in pediatric Fontan patients with moderate-good systolic ventricular function. Fontan patients between 8 and 18 years with moderate-good systolic ventricular function and without previous ACE inhibitor treatment were included and were treated with enalapril for 3 months. During the first 2 weeks, the dosage was titrated according to systolic blood pressure (SBP). Exercise tests, ventricular function assessed by echocardiography, arterial stiffness measurements, and plasma levels of N-terminal pro-B-type natriuretic peptide assessed before and after a 3-month enalapril treatment period was compared. A total of 28 Fontan patients (median age 13.9 years, 6 to 15 years after Fontan operation) completed the study with a mean dosage of 0.3 ± 0.1 mg/kg/d. A total of 6 patients (21%) experienced a significant drop in SBP and 6 others (21%) experienced other adverse events. Enalapril treatment lowered the SBP (from 110 to 104 mmHg, p=0.003) and levels of N-terminal pro-B-type natriuretic peptide (from 80 to 72 ng/L, p=0.036). However, enalapril treatment did not improve exercise capacity, ventricular function, or arterial stiffness. In conclusion, short-term ACE inhibition has no beneficial effect in Fontan patients with moderate-good systolic ventricular function.
Highlights
In this study we evaluated the effect of angiotensin-converting enzyme (ACE) inhibition in pediatric Fontan patients with moderate-good systolic ventricular function and hypothesized that it may improve exercise performance and ventricular and vascular function
As only 6 patients participated in the baseline measurements, a total of 30 patients were enrolled in this study and started with enalapril treatment
Our study evaluating the effect of a 3-month enalapril treatment in pediatric Fontan patients demonstrated no improvement of exercise capacity nor systolic and diastolic ventricular function, and arterial stiffness
Summary
With mild to severe systolic heart failure.[3−10] Because its effectiveness in biventricular heart patients, many Fontan patients are currently treated with ACE inhibitors, including those without overt systolic ventricular dysfunction, despite the lack of evidence of its efficacy in this patient population.[11,12] in this study we evaluated the effect of ACE inhibition in pediatric Fontan patients with moderate-good systolic ventricular function and hypothesized that it may improve exercise performance and ventricular and vascular function.
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