Related Experiment Videos
Masked subaortic stenosis in ostium primum atrial septal defect: recognition and treatment
Insights
Patients with ostium primum atrial septal defect (ASD) and cleft mitral valve can develop subaortic stenosis after surgery. Preoperative imaging may reveal a "goose-neck" deformity indicating potential obstruction.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- Ostium primum atrial septal defects (ASD) with cleft mitral valves are congenital heart conditions.
- Left ventricular (LV) outflow tract obstruction is a potential complication that may not be evident preoperatively.
Purpose of the Study:
- To investigate the development of subaortic stenosis after surgical repair of ostium primum ASD with cleft mitral valves.
- To identify diagnostic imaging signs of potential or actual subaortic obstruction in these patients.
Main Methods:
- Review of preoperative and postoperative cardiac catheterization data and LV angiograms in 5 patients.
- Analysis of preoperative echocardiograms for characteristic deformities.
- Correlation of imaging findings with surgical outcomes.
Main Results:
- All 5 patients developed subaortic stenosis post-surgery, with pressure gradients from 10 to 120 mm Hg.
- Preoperative and postoperative LV angiograms showed systolic narrowing of the outflow tract.
- A diastolic "goose-neck" deformity during LV systole, visible on echocardiography, indicated potential obstruction.
Conclusions:
- Surgical repair of ostium primum ASD with cleft mitral valve can unmask or cause subaortic stenosis.
- The "goose-neck" deformity is a crucial diagnostic sign of subaortic obstruction, detectable via echocardiography.
- Surgeons must be aware of this sign and explore the LV outflow tract during surgery to prevent or manage obstruction.
Abstract:
Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic "goose-neck" deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.