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Survival With Respect to Morphology in Pulmonary Atresia and Intact Ventricular Septum in Sweden
Stina Manhem1, Katarina Hanséus2, Håkan Berggren1
1Department of Pediatrics, Institution for Clinical Sciences. Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Insights
Survival rates for pulmonary atresia with intact ventricular septum have improved, particularly for membranous cases. However, muscular pulmonary atresia remains a significant risk factor, necessitating further research and focused interventions.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Medical Outcomes Research
Background:
- Pulmonary atresia with intact ventricular septum (PA/IVS) presents significant challenges in pediatric cardiology.
- Understanding survival trends and morphological impacts is crucial for improving patient outcomes.
Purpose of the Study:
- To investigate changes in survival rates for children with PA/IVS in Sweden over a 36-year period.
- To analyze survival variations based on specific morphological subtypes of PA/IVS.
Main Methods:
- Retrospective analysis of medical records and echocardiographic data from 1980 to 2016.
- Comparison of survival outcomes between two cohorts: early (1980-1998) and late (1999-2016) birth periods.
Main Results:
- Overall incidence of PA/IVS remained stable. One-year survival significantly improved from 76% to 92% between the early and late groups.
- Survival increased notably for membranous atresia (78% to 98%) but less so for muscular atresia (68% to 85%).
- Muscular pulmonary atresia and ventriculocoronary arterial communications were associated with no significant survival improvement; low birth weight and early birth period were risk factors for death.
Conclusions:
- While overall survival for PA/IVS has improved, particularly for membranous forms, muscular PA remains a critical risk factor.
- Targeted interventions for patients with muscular pulmonary atresia and ventriculocoronary arterial communications are essential for further survival gains.
Background:
Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years.
Methods:
A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group).
Results:
The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group (P = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) P = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) P < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) P = .0010.
Conclusion:
The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.
