Sonographic Pulmonary Abnormalities in Fetuses With Hypoplastic Left Heart Syndrome and Intact Atrial Septum...

Area of Science:

• Fetal Medicine
• Pediatric Cardiology
• Medical Imaging

Background:

• Hypoplastic left heart syndrome (HLHS) with an intact atrial septum presents a severe challenge in fetal cardiology.
• These cases are associated with high perinatal mortality rates (50-70%) despite prenatal diagnosis.
• Prenatal left atrial and pulmonary venous hypertension contribute to abnormal fetal lung development.

Purpose of the Study:

• To correlate lung sonographic findings in fetuses with HLHS and intact atrial septum with neonatal outcomes.
• To evaluate the impact of in utero interventions aimed at opening the atrial septum on lung development and prognosis.
• To investigate the relationship between prenatal lung appearance and postnatal pathology.

Main Methods:

• Retrospective analysis of fetal cases diagnosed with HLHS and intact atrial septum.
• Sonographic assessment of lung appearance, focusing on inhomogeneity.
• Correlation of imaging findings with neonatal outcomes, autopsy findings, and in utero intervention status.

Main Results:

• Lung inhomogeneity on prenatal ultrasound was observed in fetuses with HLHS and intact atrial septum.
• This sonographic finding correlated with peripheral lymphatic dilatation identified at autopsy.
• Lung inhomogeneity was associated with a poor neonatal prognosis, irrespective of successful in utero intervention.

Conclusions:

• Prenatal sonographic detection of lung inhomogeneity is a significant indicator of poor prognosis in fetuses with HLHS and intact atrial septum.
• In utero intervention to open the atrial septum may not fully mitigate the adverse effects of prenatal pulmonary hypertension on lung development.
• Fetal lung appearance on ultrasound provides critical prognostic information for this high-risk group.