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[A report of two cases of absent pulmonary valve syndrome]
Insights
For infants with absent pulmonary valve syndrome, surgical repair using a monocusp patch for right ventricular outflow tract reconstruction may be sufficient, avoiding the need for a pulmonary valve prosthesis in select cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiovascular Surgery
Background:
- Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect.
- Surgical management often involves pulmonary valve replacement, but its necessity in milder cases is debated.
Observation:
- Two infants with APVS, without severe pulmonary symptoms or emphysema, underwent corrective surgery.
- The procedure included infundibular resection, ventricular septal defect closure, and right ventricular outflow tract reconstruction with a monocusp patch.
Findings:
- Patients tolerated pulmonary regurgitation postoperatively.
- Preoperative aneurysmal dilatation of pulmonary arteries was noted.
- The postoperative course was uneventful, suggesting successful management.
Implications:
- A pulmonary valve prosthesis may not be essential in all APVS cases.
- Monocusp patch reconstruction of the right ventricular outflow tract can be a viable alternative in selected milder APVS cases.
- This approach may simplify surgical management and potentially reduce complications.
Abstract:
Two infants, aged 8 and 18 months, with absent pulmonary valve syndrome unaccompanied by severe pulmonary symptoms or roentgenographic evidence of emphysema underwent a corrective operation consisting of infundibular muscle resection, patch closure for ventricular septal defect (perimembranous outlet type), and right ventricular outflow tract reconstruction with a monocusp patch. The patients tolerated pulmonary regurgitation relatively well, although aneurysmal dilatation of the pulmonary arteries was present preoperatively. Since the postoperative course was uneventful, we think that, in milder cases of this syndrome, a pulmonary valve prosthesis, the insertion of which is advocated by some authors, is not essential, and that right ventricular outflow tract reconstruction with a monocusp patch may suffice in selected cases.