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Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study
F L Hanley1, R M Sade, E H Blackstone
1Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, MA.
Insights
Pulmonary atresia with intact ventricular septum in neonates is linked to small tricuspid valve diameter and coronary fistulas. These factors, along with initial procedure type, impact survival and need for further interventions.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Neonatal Surgery
Background:
- Pulmonary atresia with intact ventricular septum (PA/IVS) is a severe congenital heart defect.
- Understanding risk factors for outcomes in PA/IVS is crucial for surgical planning and patient management.
- Previous studies highlight anatomical variations and their impact on surgical success.
Purpose of the Study:
- To prospectively evaluate risk factors influencing survival and reintervention in neonates with PA/IVS.
- To identify patient-specific and procedural factors associated with mortality and need for subsequent surgeries.
- To analyze the long-term outcomes of different surgical repair strategies in this complex population.
Main Methods:
- Prospective multiinstitutional study of 171 neonates with PA/IVS from 1987-1991.
- Data collection included Z-value of tricuspid valve diameter, right ventricular size, coronary artery-right ventricular fistulas, and birth weight.
- Multivariable analysis was used to identify risk factors for time-related death and need for subsequent procedures.
Main Results:
- 52% of patients had tricuspid valve Z-value < -2, strongly correlated with small right ventricular size.
- 45% had coronary artery-right ventricular fistulas; both were negatively correlated with tricuspid valve diameter.
- Survival was 81% at 1 month and 64% at 4 years. Small tricuspid valve diameter, RV coronary dependency, birth weight, and initial procedure type were risk factors for death. 51% required subsequent procedures, with small tricuspid valve size predicting shunt need.
Conclusions:
- Tricuspid valve size is a critical determinant of outcomes in PA/IVS, influencing survival and the need for shunts and transannular patches.
- Right ventricular coronary dependency and initial surgical approach significantly impact long-term results.
- Patient-specific factors like tricuspid valve Z-value are key predictors for achieving a two-ventricle repair.
Abstract:
A total of 171 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective multiinstitutional study between January 1, 1987, and January 1, 1991. Treatment was not assigned randomly but was selected by the responsible physicians. The Z-value (standard deviation units) of the diameter of the tricuspid valve was less than -2 in 52% of patients and less than -4 in 26%; it was highly correlated with right ventricular cavity size (which was small in 90% of patients and was severely reduced in 54%). Coronary artery-right ventricular fistulas were present in 45% of patients, and right ventricular dependency was severe in 9%; diameter (Z-value) of the tricuspid valve was negatively correlated (P < 0.0001) with the prevalence of both. Survival was 81% at 1 month after the first intervention and 64% at 4 years; the hazard function (instantaneous risk of death) declined rapidly after the initial procedure but remained appreciable for 24 months. Multivariable analysis showed small diameter of the tricuspid valve, severe right ventricular coronary dependency, birth weight, and the date and type of initial procedure to be risk factors for time-related death. Subsequent procedures were performed in 51% of patients. Among patients undergoing an initial procedure that did not include a systemic-pulmonary artery shunt, only 49% did not receive a shunt in the subsequent 1 month; small size of the tricuspid valve was the only risk factor for receiving the subsequent shunt. Ninety-eight percent of living patients whose initial procedure did not include a transannular patch were free of a subsequently placed transannular patch within 1 month, but only 45% were free of it 3 years later; no risk factors were identified. Eighteen percent of living patients had received a one-ventricle repair within 3 years, and 32% had received a two-ventricle repair; the remainder (50%) had incompletely separated pulmonary and systemic circulations. The only patient-specific risk factor for not receiving a two-ventricle repair was the Z-value of the tricuspid valve.