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[Pulmonary valve agenesis. Clinico-surgical evaluation of 32 patients]
L N Bustamante1, E Atik, A A Lopes
1Instituto do Coração do Hospital das Clínicas--FMUSP, São Paulo.
Insights
Surgical correction for absent pulmonary valve is best delayed until at least two years of age. Early intervention in infants with absent pulmonary valve and associated defects, like Tetralogy of Fallot, showed high mortality.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
Background:
- Absent pulmonary valve is a rare congenital heart defect.
- Often associated with other cardiac anomalies, particularly Tetralogy of Fallot.
- Clinical presentation includes cyanosis and murmurs, with potential for severe respiratory and cardiac insufficiency.
Purpose of the Study:
- To analyze the surgical and clinical outcomes of 32 infants with absent pulmonary valve.
- To determine the optimal timing for surgical correction in these patients.
Main Methods:
- Retrospective analysis of 32 infants under 12 months of age (1980-1993).
- Evaluation of clinical symptoms, ECG, chest X-ray, echocardiography, and cardiac catheterization.
- Assessment of outcomes related to surgical timing.
Main Results:
- Tetralogy of Fallot was present in 30 patients.
- 84% presented with early cyanosis, 90% with a "to and fro" murmur.
- Mortality was high (50%) in infants operated on before 12 months due to refractory insufficiency.
- Survivors undergoing repair were between 2 and 11 years old.
Conclusions:
- Conservative management is recommended until bronchial walls mature.
- Delaying surgical repair until at least two years of age improves survival.
- This approach allows for better tolerance of pulmonary artery pressure post-operatively.
Purpose:
To study the surgical and clinical evolution of 32 cases with absent pulmonary valve to propose the ideal period of time for surgical correction.
Methods:
Clinical and laboratorial analysis were performed in 32 infants, under 12 months of age, between 1980 an 1993, in an evolutive character. From the clinical viewpoint, hypoxic and/or congestive features were considered in previous and late periods related to surgical repair. Laboratorial studies as ECG (cavities overload), chest X-ray (cardiac size and pulmonary vascular markings) and echocardiogram (associated defects, pressure gradients and anatomical aspects of pulmonary arteries) were also analyzed. Cardiac catheterization was performed in 15 patients.
Results:
Early cyanosis in 84% of cases and "to and for "murmur in 90% of them facilitate clinical diagnosis in whom tetralogy of Fallot was associated in 30 patients. Refractory respiratory and cardiac insufficiency were responsible for operative indication in 12 patients, half of them, operated on under 12 months of age, died. Survival patients were repaired between two to 11 years old. Four deaths occurred early in life, before any surgical consideration and the 16 remaining patients will electively be considered for an opportune repair.
Conclusion:
Conservative clinical treatment is indicated, waiting for a more rigid bronchial wall can support the pressure of the dilated pulmonary arteries. This way, surgical repair is postponed for at least two years of age.