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[Congenital absence of the pulmonary valve. Analysis of 7 cases]
A Cabrera Duro1, L de Benito Basanta, J Alcíbar Villa
1Cardiología Pediátrica, Hospital Infantil de Cruces, Vizcaya.
Insights
Congenital absence of the pulmonary valve is a rare condition. Two-dimensional echocardiography is crucial for diagnosing this defect, aiding in early intervention and management strategies.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects
Background:
- Congenital absence of the pulmonary valve (APV) is a rare and severe congenital heart defect.
- It is characterized by a complete or near-complete lack of the pulmonary valve leaflets.
- APV often presents with other cardiac anomalies, complicating diagnosis and management.
Observation:
- Seven cases of APV were observed over 15 years, with a 5:2 female-to-male ratio.
- Patients ranged from one day to six years old; three neonates with low birth weight (<1.500 g) died within 24 hours.
- Common clinical findings included a continuous systolic-diastolic murmur and a single second heart sound.
Findings:
- Two-dimensional echocardiography enabled definitive diagnosis, revealing a fixed echogenic image in the valvular ring area.
- Pulmonary trunk and branches were dilated, while the valvular ring was normal or small.
- Cardiac catheterization and angiocardiography were performed in four patients.
Implications:
- Early diagnosis via echocardiography is critical for managing APV.
- Surgical interventions, including ventricular septal defect closure and aortic hemograft placement, were performed in two patients.
- Further research is needed to improve outcomes for infants with this rare condition.
Abstract:
We present seven cases of congenital absence of pulmonary valve observed during a 15 years period. There are five females and two males, aged from one day to six years. Three patients with a birth weight under 1.500 g died in the first 24 hours. All the patients had a systolic diastolic murmur and a single second sound. Two dimensional echocardiography was the procedure that allowed a definite diagnosis. In the valvular ring area a fixed ecogenic image was observed during the cardiac cycle. The pulmonary trunk and branches were dilated and the valvular ring was normal or small. In four patients the study was completed with catheterism and angiocardiography. Two were operated upon, with closing of the ventricular septal defect, aortic hemograft in pulmonary situation and reduction of pulmonary artery branches size.