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Absent pulmonary valve syndrome--a case report
1Department of Paediatrics, Hospital Universiti Sains Malaysia, Kelantan.
Insights
A child with recurrent bronchopulmonary issues was diagnosed with a large perimembranous ventricular septal defect and absent pulmonary valve. Further evaluation revealed complex cardiac anomalies requiring corrective surgery.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Recurrent bronchopulmonary symptoms in infants can indicate underlying congenital heart defects.
- Early diagnosis and management are crucial for complex cardiac anomalies.
Observation:
- A 7-year-old female presented with a history of recurrent bronchopulmonary issues since one week of life.
- Initial diagnosis of ventricular septal defect was managed conservatively.
Findings:
- Repeat echocardiogram revealed a large perimembranous ventricular septal defect.
- Associated anomalies included absent pulmonary valve, aortic overriding, narrow pulmonary artery annulus, and dilated pulmonary arteries.
Implications:
- Complex congenital heart disease requires specialized surgical intervention.
- This case highlights the importance of comprehensive cardiac evaluation for persistent respiratory symptoms in children.
Abstract:
A 7-year old female child was admitted for recurrent bronchopulmonary since one week of life. She was diagnosed to have ventricular septal defect and was treated conservatively. At seven years of life, repeat echocardiogram revealed a large perimembranous ventricular septal defect, absent pulmonary valve with overriding of aorta, narrow pulmonary artery annulus, and dilated main pulmonary artery and its branches. She was treated conservatively, discharged and follow-up at the National Heart Institute Kuala Lumpur, for corrective surgery.