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Natural and unnatural history of pulmonary atresia
H Leonard1, G Derrick, J O'Sullivan
1Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne NE7 7DN, UK.
Insights
Congenital pulmonary atresia has high early mortality, but survival is improving. Most survivors experience significant exercise limitations, especially without definitive surgical repair.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Congenital pulmonary atresia is a severe heart defect.
- Understanding outcomes is crucial for patient management.
Purpose of the Study:
- To investigate mortality, survival, and quality of life in congenital pulmonary atresia.
- To analyze outcomes across different anatomical classifications.
Main Methods:
- Retrospective analysis of live births from 1980-1995.
- Classification into pulmonary atresia with intact septum (PA-IVS), ventricular septal defect (PA-VSD), or complex malformation.
- Assessment of mortality, cause of death, survival duration, and exercise ability.
Main Results:
- 129 cases identified; 56% overall mortality, with 49% dying within the first year.
- Pulmonary atresia with ventricular septal defect was the most common type (60 cases).
- Survivors often have significant exercise limitations (61% with severe limitations), though definitive repair improved outcomes.
Conclusions:
- Early mortality remains high for all congenital pulmonary atresia types.
- Survival rates have shown improvement over time.
- Long-term quality of life is impacted by exercise limitations, highlighting the need for effective surgical interventions.
Objective:
To investigate mortality, cause of death, survival, and quality of life in all types of cardiac malformation with congenital pulmonary atresia.
Design:
Retrospective analysis.
Setting:
The resident population of one health region with a single tertiary referral centre.
Patients:
All babies with pulmonary atresia live born in 1980 to 1995.
Main Outcome Measures:
Anatomical classification, total mortality, cause of death, duration of survival, exercise ability. All cases were classified as pulmonary atresia with intact septum (PA-IVS), pulmonary atresia with ventricular septal defect (PA-VSD), or pulmonary atresia with complex cardiac malformation (complex pulmonary atresia).
Results:
129 cardiac malformations with congenital pulmonary atresia were identified from 601 635 live births (21.4/100 000): 29 had PA-IVS, 60 had PA-VSD, and 40 had complex pulmonary atresia. Total mortality was 72/129 (56%), with 15 deaths in the first week and 49 in the first year. There were 23 surgical deaths, 33 hospital deaths (not related to surgery), and 16 sudden deaths, 12 of which remained unexplained. The sudden death rate was 29/1000 patient years of follow up. Of the 57 survivors, 39% have exercise ability I or II and 61% III or IV. Definitive surgical repair produced better exercise ability.
Conclusions:
Early mortality is high in all types of pulmonary atresia, although survival has improved in recent years. Most children who have not undergone definitive repair have significant exercise limitation.