Key result
Absent pulmonary valve syndrome with dextrocardia progresses to hydrops and intrauterine fetal demise.
Case Report (n=1)
This case highlights the rapid progression to hydrops fetalis and intrauterine demise in a fetus with suspected Tetralogy of Fallot with absent pulmonary valve and dextrocardia, underscoring the importance of preconception glycemic control in maternal diabetes.
Progressive hydrops and demise in this fetal TOF/APV case with maternal diabetes supports close surveillance; leaves open preconception glycemic optimization benefits.
Early fetal echocardiography and serial surveillance are crucial in pregnancies complicated by pregestational diabetes. Tetralogy of Fallot with absent pulmonary valve syndrome may cause progressive cardiomegaly, ventricular hypertrophy, hydrops, and fetal demise. Timely diagnosis enables informed parental counseling and multidisciplinary perinatal planning.
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Golbabaei et al. (2026) conducted a case report in Fetal Tetralogy of Fallot with absent pulmonary valve syndrome and dextrocardia (n=1). Maternal pre-gestational diabetes mellitus was evaluated on Fetal outcome and progression of cardiac anomalies. A fetus with suspected Tetralogy of Fallot with absent pulmonary valve and dextrocardia in a mother with pre-gestational diabetes developed progressive cardiomegaly and hydrops fetalis, resulting in intrauterine fetal demise at 30 weeks.
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