Key result
Prenatal diagnosis of mitral valve dysplasia syndrome allowed for appropriate delivery planning and successful neonatal management including orthotopic heart transplantation at 4 months of age.
Why the study?
Mitral valve dysplasia syndrome is a rare cardiac defect requiring accurate prenatal identification, appropriate counseling, delivery planning, and postnatal management.
Case Report (n=1)
Prenatal diagnosis of mitral valve dysplasia syndrome, a rare variant of hypoplastic left heart syndrome, is crucial for perinatal preparedness and can lead to favorable outcomes through planned interventions.
Prenatal MVDS identification may inform counseling and delivery planning; leaves open standardized postnatal protocols pending larger series.
BACKGROUND: Mitral valve dysplasia syndrome (MVDS) is a rare cardiac defect requiring accurate prenatal identification and appropriate counseling, delivery planning, and postnatal management. CASE SUMMARY: A 39-year-old woman underwent fetal echocardiography at 34 weeks' gestation owing to cardiomegaly and hydrops. Biatrial enlargement, mild to moderate atrioventricular valve insufficiency, dysplastic mitral valve apparatus, biventricular dilation with systolic dysfunction, endocardial fibroelastosis, hypoplastic aortic outflow, and severely restrictive atrial septum were attributed to MVDS. The plan for critical (class IV) delivery was modified with premature onset of labor at 36 weeks' gestation, leading to urgent cesarean section. Emergent balloon atrial septostomy was performed on the female neonate, followed initially by hybrid palliation and orthotopic heart transplantation at 4 months of age. DISCUSSION: Identifying distinct anatomical features of MVDS prenatally differentiates it from various types of hypoplastic left heart syndrome and guides appropriate delivery and neonatal management, resulting in favorable outcomes. TAKE-HOME MESSAGE: MVDS is a unique variant of hypoplastic left heart syndrome that is able to be diagnosed prenatally, with high perinatal morbidity and mortality requiring perinatal preparedness.
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Goyal et al. (2025) conducted a case report in Mitral valve dysplasia syndrome (MVDS) (n=1). Prenatal diagnosis and multidisciplinary management was evaluated on Clinical outcome. Prenatal diagnosis of mitral valve dysplasia syndrome allowed for appropriate delivery planning and successful neonatal management including orthotopic heart transplantation at 4 months of age.
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