Fontan repair for hypoplastic left heart syndrome is categorized under which WHO maternal CV risk group?

Fontan repair for hypoplastic left heart syndrome is categorized under which WHO maternal CV risk group?

• A. Group II
• B. Group III
• C. Group IV
• D. Not categorized

Answer: (B)

In the WHO risk classification for pregnancy with cardiovascular disease, Fontan physiology after hypoplastic left heart syndrome is placed in Group III. This reflects a significantly increased risk of maternal complications and mortality during pregnancy, necessitating management by a specialized multidisciplinary team in a tertiary center. Why this fits: Fontan circulation creates a single-ventricle system with limited cardiac reserve and passive pulmonary blood flow. Pregnancy brings substantial hemodynamic changes—rising blood volume, increased cardiac output demand, and hormonal effects—that can overwhelm the single ventricle, raising the risk of heart failure, arrhythmias, thromboembolism, and hepatic or systemic congestion. While not an absolute contraindication in all cases (which would imply Group IV), the level of risk is substantial enough to categorize it as Group III, requiring careful preconception counseling, optimized medical therapy, and planning for delivery and postpartum care with expert cardiology–obstetric support.

In the WHO risk classification for pregnancy with cardiovascular disease, Fontan physiology after hypoplastic left heart syndrome is placed in Group III. This reflects a significantly increased risk of maternal complications and mortality during pregnancy, necessitating management by a specialized multidisciplinary team in a tertiary center.

Why this fits: Fontan circulation creates a single-ventricle system with limited cardiac reserve and passive pulmonary blood flow. Pregnancy brings substantial hemodynamic changes—rising blood volume, increased cardiac output demand, and hormonal effects—that can overwhelm the single ventricle, raising the risk of heart failure, arrhythmias, thromboembolism, and hepatic or systemic congestion. While not an absolute contraindication in all cases (which would imply Group IV), the level of risk is substantial enough to categorize it as Group III, requiring careful preconception counseling, optimized medical therapy, and planning for delivery and postpartum care with expert cardiology–obstetric support.