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Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based
Piers E F Daubeney1, D Wang, D J Delany
1Royal Brompton Hospital, London, United Kingdom. p.daubeney@rbh.nthames.nhs.uk
Insights
Low birth weight, unipartite right ventricle, and dilated right ventricle are key risk factors for poor outcomes in pulmonary atresia with intact ventricular septum. Identifying these features aids in early intervention and improved patient counseling.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Research
- Cardiac Surgery Outcomes
Background:
- Pulmonary atresia with intact ventricular septum (PAIVS) is a complex congenital heart defect.
- PAIVS often presents with right-heart hypoplasia and variable morphology.
- Prognosis for PAIVS can be poor, necessitating identification of risk factors.
Purpose of the Study:
- To identify independent risk factors for poor outcomes in PAIVS.
- To inform surgical strategies and prenatal counseling for PAIVS.
- To analyze survival rates and long-term repair outcomes in a large cohort.
Main Methods:
- Population-based study of 183 patients with PAIVS born between 1991-1995 in the UK and Ireland.
- Direct review of clinical, morphologic, and investigative variables.
- Cox proportional hazards model analysis for risk factor identification.
Main Results:
- One- and 5-year survival rates were 70.8% and 63.8%, respectively.
- Independent risk factors for mortality included low birth weight, unipartite right ventricular morphology, and dilated right ventricle.
- Long-term follow-up showed varying repair strategies, with 41% mortality in the cohort.
Conclusions:
- Specific presenting features identify infants at high risk for PAIVS.
- This data is crucial for effective prenatal counseling and surgical planning.
- Understanding risk factors can guide interventions to improve outcomes for PAIVS patients.
Objectives:
Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved.
Methods:
The UK and Ireland Collaborative study of Pulmonary Atresia with Intact Ventricular Septum is an ongoing population-based study of all patients born with this disease from 1991 through 1995. All available clinical, morphologic, and investigative variables were directly reviewed, and risk factor analysis was performed for poor outcome.
Results:
One hundred eighty-three patients presented with pulmonary atresia with intact ventricular septum. Fifteen underwent no procedure, and all died. Of the remainder, 67 underwent a right ventricular outflow tract procedure (catheter or surgical), 18 underwent an outflow tract procedure with shunt, and 81 underwent a systemic-to-pulmonary shunt alone. One- and 5-year survival was 70.8% and 63.8%, respectively. Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P < .001) were independent risk factors for death. The presence of coronary artery fistulae, right ventricular dependence, or the tricuspid valvar z score did not prove to be risk factors for death. After up to 9 years of follow-up, 29% have achieved a biventricular repair, 3% a so-called one-and-a-half ventricular repair, and 10.5% a univentricular repair, with 16.5% still having a mixed circulation (41% died).
Conclusions:
This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
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