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Growth of the hypoplastic ascending aorta after radical palliation
1Pediatric Cardiovascular Surgery, California-Pacific Medical Center, San Francisco 94115.
Insights
Surgical palliation for hypoplastic ascending aorta shows promise. This technique can promote growth of heart structures, potentially enabling biventricular repair in infants with specific congenital heart defects.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease
- Cardiovascular Surgery
Background:
- Hypoplasia of the ascending aorta presents a significant surgical challenge.
- A patent aortic outflow with a hypoplastic ascending aorta requires innovative treatment strategies.
Purpose of the Study:
- To evaluate the efficacy of proximal pulmonary artery to distal aorta bypass with distal pulmonary artery banding in neonates and infants with a hypoplastic ascending aorta.
- To assess the potential for growth of cardiac structures and the possibility of biventricular repair following this palliative approach.
Main Methods:
- A cohort of 19 neonates and infants with ascending aorta diameter < 6 mm underwent proximal pulmonary artery to distal aorta bypass and distal pulmonary artery banding.
- Patient characteristics included diagnoses such as transposition of the great arteries, subaortic stenosis, and interrupted aortic arch.
Main Results:
- One operative death occurred. Cerebral cyanosis necessitated reoperation in 3 of 4 patients with transposition of the great arteries, leading to abandonment of this approach in that subgroup.
- All 14 surviving patients with normally related great arteries demonstrated successful palliation and growth of left heart structures.
- Subsequent interventions varied, including Fontan/Damus procedure, aortoplasty, and arch reconstruction.
Conclusions:
- Proximal pulmonary artery to distal aorta bypass with banding can facilitate growth of right and left heart structures in select patients with hypoplastic ascending aorta.
- This palliative strategy offers a potential pathway towards biventricular repair in infants with specific congenital heart defects, excluding those with transposition of the great arteries.
Abstract:
Surgical treatment of hypoplasia of the ascending aorta is a difficult problem. An approach using proximal pulmonary artery to distal aorta bypass with distal pulmonary artery banding was employed in 19 patients with an ascending aorta less than 6 mm in diameter but a patent aortic outflow. There were 11 male and 8 female neonates and infants, and weight ranged from 2 to 4.5 kg (median weight, 3.5 kg). Four patients had transposition of the great arteries, 12 had subaortic stenosis, and 5 had interrupted aortic arch. There was one operative death. Cerebral cyanosis developed in 3 of the 4 patients with transposition of the great arteries and necessitated additional operations within the first year postoperatively. Therefore, palliation with a pulmonary artery to descending aorta conduit and banding was abandoned in such patients. All 14 surviving patients with normally related great arteries had successful palliation and growth of left heart structures. Subsequent procedures included a Fontan/Damus procedure in 1 patient, patch aortoplasty and pulmonary arterioplasty in 5 patients, arch reconstruction with aortic valvulotomy and pulmonary arterioplasty in 2, double arch reconstruction in 2, and simple patch aortoplasty with plans for subsequent pulmonary arterioplasty and ventricular septal defect closure in 1. Results demonstrate that in patients with a hypoplastic ascending aorta, this radical method of palliation can result in growth of right and left heart structures and thus provides the possibility of biventricular repair.