Abstract
This presentation reviews our experience with the syndrome of absent pulmonary valve (SAPV). Ten infants and children with SAPV seen during 1979–83 were analyzed. Their mean age was 11 months with a range of 1 day to 3 years. Five group I patients (< 6 mo.) presented with severe tracheobronchial obstruction, cyanosis, and heart failure; five group II patients (> 6 mo.) presented with history of recurrent respiratory tract infection and a murmur. Clinical, x-ray, echocardiographic (ECHO) findings were classic for this anomaly. Two children were symptom-free on medical management, and two died following surgery. Plication of PAs was performed as the sole procedure in a 5-day-old respirator-dependent infant. Seven patients underwent closure of VSD and relief of PV ring stenosis; two of these had additional PA plication and two additional PV replacement. Follow-up of these patients ranged between 1.5 and 3.5 years and all underwent ECHO and catheterization studies. Symptomatic relief and normalization of cardiac structure and function were greatest in the patients who had plication of PAs as an integral part of total repair as compared with those who had other types of surgical correction or no surgical repair. Based on this experience, we recommend total surgical correction with PA plicaiton but without PV replacement as the procedure of choice in symptomatic patients with SAPV.
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Rao, P., Mardini, M., Feteih, W. et al. 129 SYNDROME OF ABSENT PULMONARY VALVE. Pediatr Res 19, 132 (1985). https://doi.org/10.1203/00006450-198504000-00159
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DOI: https://doi.org/10.1203/00006450-198504000-00159