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[Mortality and survival rate in pulmonary atresia with intact intraventricular septum. Determining factors]
G Cabezuelo Huerta1, P Frontera Izquierdo
1Departamento de Pediatria, Hospital Infantil La Fe, Universidad de Valencia.
Insights
Pulmonary atresia with intact ventricular septum in children had a high mortality rate historically. Advances in prostaglandin infusion and surgical techniques have significantly improved survival rates for this congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Context:
- Pulmonary atresia with intact ventricular septum (PA/IVS) is a severe congenital heart defect.
- Historically, PA/IVS presented a dismal prognosis, particularly in neonates.
- This study reviews outcomes from 1971-1988, a period preceding widespread advanced interventions.
Purpose:
- To analyze the outcomes and mortality rates of children diagnosed with PA/IVS between 1971 and 1988.
- To identify factors influencing survival, including surgical intervention and anatomical features.
- To evaluate the impact of evolving medical practices on the natural history of PA/IVS.
Summary:
- A review of 69 children with PA/IVS revealed an overall mortality of 72.4%.
- Mortality was higher before 1982 and before the routine use of prostaglandin infusion.
- Surgical mortality decreased from 74% (1971-1982) to 54% (1983-1988), with right ventricular hypoplasia being a key negative prognostic factor.
- Actuarial survival improved significantly in later years, especially with improved surgical techniques and prostaglandin use.
Impact:
- The study highlights the critical role of prostaglandin infusion and surgical advancements in improving outcomes for PA/IVS.
- Findings underscore the importance of timely intervention and surgical expertise in managing complex congenital heart diseases.
- This research provides historical context for understanding the evolution of treatment strategies for PA/IVS.
Abstract:
Sixty nine children with pulmonary atresia and intact ventricular septum diagnosed by catheterization from 1971 to 1988 are reviewed. These patients are the 2.9% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in this 17 year period. The overall mortality was 72.4%. Twenty children died at a mean age of 37 days before cardiac surgery was performed, mostly before 1982, before the use of prostaglandin infusion. Forty seven children received surgical treatment, with a surgical mortality rate of 74% at the period 1971-1982 and 54% at the period 1983-1988. The right ventricular hypoplasia was the anatomic factor of dismal outlook. The actuarial survival in the patients without hypoplastic right ventricle and surgical treatment was 52% in the period 1971-1988 and near 80% in the first 3 years of life in the period 1983-1988. The use of preoperative prostaglandin infusion and improved operative techniques has changed the natural history of this malformation.