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Anatomically corrected malposition of the great arteries, inflow ventricular septal defect, and subaortic stenosis:
F X Schmid1, H Oelert, H Jakob
1Division of Cardiothoracic and Vascular Surgery, University Clinics Mainz, FRG.
Insights
This study reports a successful intracardiac correction for a rare congenital heart defect in a young boy, involving complex anatomical abnormalities and subaortic stenosis. The surgical intervention effectively addressed multiple cardiac defects, leading to a stable recovery.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Anatomy
Background:
- The case involves a rare combination of congenital heart defects: anatomically corrected malposition of the great arteries, severe subaortic stenosis (muscular and fibrous), and ventricular septal defects.
- The patient, a 2 1/2-year-old boy, also presented with a large secundum atrial septal defect.
Observation:
- Surgical correction utilized a combined transatrial and transaortic approach for resection of subaortic stenosis, aiming to restore a wide outflow tract for the systemic ventricle.
- Ventricular and atrial septal defects were initially closed using Dacron patches.
Findings:
- Postoperative assessment revealed a residual pressure gradient across the systemic ventricle outflow of less than 10 mmHg.
- A secondary ventricular septal defect in the anterior muscular septum was identified and successfully closed in a reintervention.
- The child was discharged in excellent condition with stable sinus rhythm two weeks after the initial surgery.
Implications:
- This case highlights the feasibility of intracardiac correction for complex congenital heart lesions, even with multiple coexisting defects.
- The surgical strategy demonstrated effective management of severe subaortic stenosis and septal defects, offering a potential treatment pathway for similar rare conditions.
- Successful outcomes underscore the importance of precise surgical techniques and vigilant postoperative monitoring in pediatric cardiac surgery.
Abstract:
Intracardiac correction of the combination of rare congenital heart lesion of anatomically corrected malposition of the great arteries, severe muscular as well as fibrous subaortic stenosis, and ventricular septal defects in the inflow and the trabecular septum in a 2 1/2 years old boy is reported. There was an additional large secundum atrial septal defect. The operation consisted of resection of the subaortic stenosis by a combined transatrial and transaortic approach, thereby regaining a wide outflow tract of the systemic ventricle. Both the ventricular and atrial septal defects were closed with Dacron patches. Reinvestigation 10 days after the operation revealed a residual pressure gradient across the outflow of the systemic ventricle of less than 10 mmHg. A secondary ventricular septal defect was detected in the anterior muscular septum at the same time and closed with a patch in a reintervention. At discharge from the hospital two weeks later the child was in excellent condition and had a stable sinus rhythm. The preoperative differential diagnosis of a double outlet right ventricle with L-malposition of the aorta as well as possible surgical approaches are discussed.