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Right ventricular growth potential in neonates with pulmonary atresia and intact ventricular septum
Insights
Pulmonary valvotomy in infants with pulmonary atresia and intact ventricular septum promotes right ventricular growth and improves outcomes. This intervention is recommended to enhance the right ventricle's function in circulation.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Pulmonary atresia with intact ventricular septum is a complex congenital heart defect.
- Assessing right ventricular (RV) growth potential is crucial for surgical planning.
Purpose of the Study:
- To evaluate the impact of transventricular pulmonary valvotomy on RV growth in neonates with pulmonary atresia and intact ventricular septum.
- To compare outcomes between infants treated with shunts alone versus those undergoing pulmonary valvotomy.
Main Methods:
- Retrospective review of cardiac catheterization and angiogram data from 30 infants.
- Utilized a standardized index for RV size based on tricuspid valve annulus, inlet, and outlet dimensions.
- Group I (n=14) received shunts only; Group II (n=16) underwent pulmonary valvotomy, with some also receiving shunts.
Main Results:
- Group I showed no RV growth (RV index 7.0 preop vs. 7.0 postop) and persistent RV hypertension (121 mmHg preop vs. 120 mmHg postop).
- Group II demonstrated significant RV cavity increase in 9/11 infants after valvotomy (RV index 7.7 preop vs. 11.0 postop, p<0.01).
- RV systolic pressure decreased in Group II (132 mmHg preop vs. 83 mmHg postop, p<0.1), with lower mortality (19% vs. 50%).
Conclusions:
- Pulmonary valvotomy should be considered in neonates with pulmonary atresia and intact ventricular septum when an outflow tract is identified.
- This intervention maximizes RV growth potential and its contribution to systemic circulation.
- Valvotomy improves functional outcomes and potentially reduces mortality in this patient population.
Abstract:
The cardiac catheterization data and angiograms of 30 infants with pulmonary atresia and intact ventricular septum were reviewed to evaluate the growth potential of the right ventricle after transventricular pulmonary valvotomy. An index of right ventricular size based upon the tricuspid valve anulus, right ventricular inlet, and right ventricular outlet dimensions was used. Fourteen infants (Group I) were treated with systemic-pulmonary arterial shunts only, whereas 16 infants (Group II) underwent pulmonary valvotomy and 14 had shunting as well. Follow-up studies demonstrated the lack of right ventricular growth in Group I (right ventricular index of 7.0 +/- 3.2 preoperatively versus 7.0 +/- 2.0 postoperatively) and persistence of severe right ventricular hypertension (systolic pressure of 121 +/- 31 versus 120 +/- 48 mm Hg). In contrast, the right ventricular cavity increased in nine of 11 Group II infants who underwent valvotomy. Right ventricular index increased from 7.7 +/- 1.6 to 11.0 +/- 3.1 (p less than 0.01) and systolic pressure fell from 132 +/- 31 to 83 +/- 50 mm Hg (p less than 0.1). Early and late mortality in Group I was 50% (7/14), whereas only three of 16 Group II infants died (p greater than 0.1). It is concluded that pulmonary valvotomy should be attempted in all neonates with pulmonary atresia and intact ventricular septum in whom an outflow tract is identified angiographically to maximize the potential for right ventricular growth and increase its functional contribution to normal circulation.