Outcomes of Subaortic Obstruction Resection in Children

• Published in: Heart, lung & circulation Vol. 26; no. 2; pp. 179 - 186
• Main Authors: Donald, Julia S., MD, Naimo, Phillip S., MD, d’Udekem, Yves, MD, PhD, FRACS, Richardson, Malcolm, MBBS, FRACP, Bullock, Andrew, MBBS, FRACP, Weintraub, Robert G., MBBS, FRACP, Brizard, Christian P., MD, Konstantinov, Igor E., MD, PhD, FRACS
• Format: Journal Article
• Language: English
• Published: Australia Elsevier B.V 01.02.2017
• Subjects: Adult; Age Factors; Aortic Valve Insufficiency - mortality; Aortic Valve Insufficiency - parasitology; Aortic Valve Insufficiency - surgery; Cardiac Surgical Procedures; Cardiovascular; Child; Child, Preschool; Congenital heart disease; Discrete Subaortic Stenosis - mortality; Discrete Subaortic Stenosis - physiopathology; Discrete Subaortic Stenosis - surgery; Female; Follow-Up Studies; Humans; Male; Retrospective Studies; Stroke Volume; Subaortic stenosis; Surgery; Time Factors; SAS; coarctation of aorta; Congenital heart disease; aortic insufficiency; odds ratio; LVOT; left ventricular outflow tract obstruction; PDA; RVOT; HOCM; subaortic stenosis; ASD; Surgery; patent ductus arteriosus; ventricular septal defect; right ventricular outflow tract obstruction; OR; CI; VSD; AI; hypertrophic obstructive cardiomyopathy; Royal Children's Hospital; atrioventricular; CoA; AV; RCH; confidence interval; atrial septal defect; Subaortic stenosis
• ISSN: 1443-9506; 1444-2892
• DOI: 10.1016/j.hlc.2016.05.120

Background Studies of long-term outcomes of discrete subaortic stenosis are rare. Therefore, we reviewed the long-term outcomes of fibromuscular resection in children with subaortic stenosis over 26 years from a single institution. Methods We conducted a retrospective review of all children (n=72) who underwent resection of subaortic obstruction for discrete subaortic stenosis between 1989 and 2015. Results Median age at surgery was 5.0 years (2.7-7.6 years). There were no operative deaths but three late deaths (4.2%, 3/72). Overall Kaplan-Meier survival at 10 years was 93.0 ± 3.9% (95% CI: 79.6, 97.7). Peak instantaneous left ventricular outflow tract Doppler gradient decreased from 74.2 ± 36.7 mmHg (16.0-242.0 mmHg) preoperatively to 12.8 ± 7.4 mmHg (2.6-36.0 mmHg) postoperatively ( p <0.001). Mean left ventricular outflow tract Doppler gradient decreased from 42.4 ± 17.2 mmHg (12.0-98.0) preoperatively to 7.5 ± 2.7 mmHg (1.4-19.3 mmHg) postoperatively ( p <0.001). However, over the mean follow-up period of 7.8 ± 6.1 years (0.1-25.2 years), 29.0% (20/69) of patients had recurrence and 18.8% (13/69) required reoperation at median time of 4.8 years (3.1-9.1 years) after the initial repair. Freedom from reoperation at 10 years was 71.1 ± 7.1% (95% CI: 54.6, 82.3). Risk factors for reoperation were age less than five years at initial repair ( p =0.036) and extension of the membrane to the aortic valve ( p =0.001). Aortic insufficiency was present in 54.2% (39/72) of patients preoperatively. Progression of aortic insufficiency occurred in 38.9% (28/72). Involvement of the aortic valve at initial repair was associated with need for subsequent aortic valve repair or replacement ( p =0.01). Conclusions Resection of subaortic obstruction is associated with low mortality and morbidity. Recurrence and reoperation rates are high and progression of aortic insufficiency following subaortic resection is common. Therefore, these patients warrant close follow-up into adult life.