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Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases
A Benatar1, R H Kinsley, S Milner
1Department of Paediatrics, Johannesburg Hospital, University of the Witwatersrand, South Africa.
Insights
Five infants with anomalous pulmonary artery origin from the ascending aorta underwent surgical repair. This rare congenital heart defect, often presenting with severe pulmonary hypertension, showed improved pressures post-surgery in survivors.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Disease
Background:
- Anomalous origin of a pulmonary artery from the ascending aorta is a rare congenital heart defect.
- This anomaly can lead to severe pulmonary hypertension and heart failure in infants.
Purpose of the Study:
- To report on the surgical management and outcomes of infants with anomalous pulmonary artery origin from the ascending aorta.
- To highlight the importance of considering this anomaly in infants with specific cardiac presentations.
Main Methods:
- Retrospective review of 5 infants diagnosed with anomalous pulmonary artery origin from the ascending aorta over a 10-year period.
- Surgical re-implantation of the anomalous vessel.
- Pre- and post-operative cardiac catheterization to assess pulmonary artery pressures.
Main Results:
- All 5 patients presented with severely elevated pulmonary artery pressures.
- Substantial reduction in pulmonary artery pressures was observed immediately after surgery.
- Four survivors showed decreased pulmonary artery pressures on long-term follow-up, with one experiencing mild residual elevation.
- Two patients developed mild stenosis at the reimplantation site.
Conclusions:
- Anomalous pulmonary artery origin from the ascending aorta is a critical diagnosis in infants with large left-to-right shunts, tricuspid incompetence, and right ventricular hypertrophy.
- Prompt surgical repair can prevent mortality from heart failure and irreversible pulmonary vascular disease.
Abstract:
Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.