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Natural history and surgical outcomes for isolated discrete subaortic stenosis in children
C V Rohlicek1, S F del Pino, M Hosking
1Division of Cardiology, Montréal Children's Hospital, 2300 Tupper Street, Montréal, Québec H3H 1P3, Canada.
Insights
Many children with mild discrete subaortic stenosis (DSS) show minimal progression and may not require immediate surgery. However, severe DSS can progress rapidly, benefiting from early surgical intervention despite associated risks and recurrence potential.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Discrete subaortic stenosis (DSS) is a congenital heart defect characterized by a fibrous ridge or tunnel below the aortic valve.
- It can lead to left ventricular outflow tract obstruction and aortic insufficiency, potentially progressing over time.
- Understanding the natural history and surgical outcomes is crucial for optimal patient management.
Purpose of the Study:
- To document the natural history of discrete subaortic stenosis in children.
- To evaluate the surgical outcomes for children with discrete subaortic stenosis.
- To identify factors predicting disease progression and the need for intervention.
Main Methods:
- Retrospective review of 92 children diagnosed with discrete subaortic stenosis between 1985 and 1998.
- Analysis of echocardiographic data, including left ventricular outflow tract gradient (echograd) and aortic insufficiency (AI).
- Comparison of outcomes between medically managed and surgically treated patients.
Main Results:
- The mean echograd and incidence of AI increased significantly over time in the overall cohort.
- Preoperative echograd and AI incidence were higher in patients who underwent surgery compared to those managed medically.
- Postoperative AI incidence remained high (82%), and 8 patients required reoperation for recurrent stenosis.
- Medically followed patients with lower initial echograd showed less progression of obstruction and AI.
Conclusions:
- Children with mild DSS may not require immediate surgery due to limited progression.
- More severe DSS cases can progress rapidly, benefiting from early surgical resection.
- Surgical intervention carries risks of morbidity and recurrence, necessitating careful patient selection.
Objective:
To document the natural history and surgical outcomes for discrete subaortic stenosis in children.
Design:
Retrospective review.
Setting:
Tertiary care paediatric cardiology centres.
Patients:
92 children diagnosed between 1985 and 1998.
Main Outcome Measures:
Echocardiographic left ventricular outflow gradient (echograd), and aortic insufficiency (AI).
Results:
The mean (SEM) age at diagnosis was 5.3 (0.4) years; the mean echograd was 30 (2) mm Hg, with AI in 22% (19/87) of patients. The echograd and incidence of AI increased to 35 (3) mm Hg and 53% (36/68) (p < 0.05) 3.6 (0.3) years later. The echograd at diagnosis predicted echograd progression and appearance of AI. 42 patients underwent surgery 2.2 (0.4) years after diagnosis. Preoperatively echograd and AI incidence increased to 58 (6) mm Hg and 76% (19/25) (p < 0.05). The echograd was 26 (4) mm Hg 3.7 (0.4) years postoperatively, with AI in 82% (31/38) of patients. Surgical morbidities included complete heart block, need for prosthetic valves, and iatrogenic ventricular septal defects. Eight patients underwent reoperation for recurrent subaortic stenosis. The age at diagnosis of 44 patients followed medically and 42 patients operated on did not differ (5.5 (0.6) v 5. 0 (0.6) years, p < 0.05). However, the echograd at diagnosis in the former was less (21 (2) v 40 (5) mm Hg, p < 0.05) and did not increase (23 (2) mm Hg) despite longer follow up (4.1 (0.4) v 2.2 (0. 4) years, p < 0.05). The incidence of AI at diagnosis and at last medical follow up was also less (14% (6/44) v 34% (13/38); 40% (17/43) v 76% (19/25), p < 0.05).
Conclusions:
Many children with mild subaortic stenosis exhibit little progression of obstruction or AI and need not undergo immediate surgery. Others with more severe subaortic stenosis may progress precipitously and will benefit from early resection despite risks of surgical morbidity and recurrence.