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Treatment of pulmonary atresia with intact ventricular septum in early infancy
1Department of Pediatrics, Veterans General Hospital-Taipei, Taiwan, ROC.
Insights
Neonates with pulmonary atresia and intact ventricular septum (PA-IVS) face high mortality. Small right ventricular volume and specific anatomical features are key risk factors for poor outcomes in PA-IVS patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Neonatal Surgery
Background:
- Pulmonary atresia and intact ventricular septum (PA-IVS) is a critical congenital heart defect requiring prompt intervention.
- Early management strategies include prostaglandin administration, balloon atrial septostomy, and surgical repairs.
Purpose of the Study:
- To identify early treatment strategies and risk factors for neonates with PA-IVS.
- To assess surgical risk and predict outcomes in this patient population.
Main Methods:
- Retrospective review of 29 PA-IVS patients diagnosed between 1987 and 1997.
- Analysis of clinical data, echocardiograms, catheterization, and cineangiograms.
Main Results:
- A right ventricular volume <1 ml was associated with mortality, while >2 ml indicated survival.
- Tricuspid valve area <0.5 cm² and monopartite right ventricle correlated with higher mortality.
- Right ventricle-dependent coronary sinusoids increased mortality risk.
Conclusions:
- Key risk factors for negative outcomes in PA-IVS neonates include small right ventricular volume, coronary sinusoids, and small tricuspid valve area.
- These factors are crucial for surgical risk assessment and outcome prediction.
Background:
Early treatment for the neonate with pulmonary atresia and intact ventricular septum (PA-IVS) is important if a high mortality rate is to be avoided. The treatment includes prostaglandin administration, balloon atrial septostomy, pulmonary valvotomy, a shunting procedure and patch repair for the right ventricular outflow tract (RVOT). This study discusses the early treatment and risk factors of this disease that are essential for assessing the risk of surgery and predicting outcome.
Methods:
The medical records, echocardiograms, catheterization data and cineangiograms of 29 patients with PA-IVS diagnosed at our institution from 1987 to 1997 were reviewed retrospectively. Clinical manifestations including age, body weight, sex ratio, type of surgery and outcome were analyzed.
Results:
Three of four patients with a right ventricular volume of less than 1 ml died, and all patients with a right ventricular volume of greater than 2 ml survived. Four of six patients with a tricuspid valvular area of between 0.25 and 0.5 cm2 died, but patients with an area of greater than 0.5 cm2 survived. One case with a monopartite right ventricle died. Patients with a bipartite or tripartite right ventricle had higher survival rates than those with a monopartite right ventricle. Patients with a the right ventricle dependent coronary sinusoid had a higher risk for mortality, even after surgery, than those with normal coronary circulation.
Conclusions:
A small right ventricular volume, the presence of a coronary sinusoid involving the right ventricle, a short linear length of the tricuspid valve and a small area of the tricuspid valve were the risk factors associated with a negative outcome in neonates with PA-IVS.