Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.

Congenitally corrected transposition of the great arteries is commonly associated with left ventricular outflow tract obstruction. We describe a case of congenitally corrected transposition of the great arteries and previous surgical ventricular septal defect repair with recurrent left ventricular outflow tract obstruction. The patient underwent a hybrid procedure to stent the left ventricular outflow tract, which was successful with no re-intervention through 3 years of follow-up.

Myxoma in neonatal life are extremely rare. We report a case of a neonate with a pedunculated cardiac tumour arising from the anterolateral left ventricular wall protruding across the left ventricular outflow tract and continuously extending into the distal aortic arch. Surgical removal at 14 days of age via combined transaortic approach and apical ventriculotomy was indicated because of the risk of further compromise of aortic valve function and aortic arch obstruction. Histopathologic examination was consistent with a myxoma.

A one-stage repair was performed for correction of the intracardiac malformations associated with coarctation of aorta in five pateints or interrupted aortic arch in eight patients. The ages ranged from four to 294 (median 35) days. The anomalies within the heart were a ventricular septal defect with or without subaortic stenosis (n=7), an aortopulmonary window (n=2), common arterial trunk (n=2), aortic valvar stenosis (n=1) and the TaussigBing anomaly (n=1). Surgery was performed through a median sternotomy employing cardiopulmonary bypass with moderate to deep hypothermia. In terms of the aortic reconstruction, an extended direct anastomosis was performed in 10 patients, while a vascular graft was interposed in three. So long as the aortic cannula did not interfere with the proximal anastomotic site on the aorta, circulatory arrest was avoided. As for surgery within the heart, the ventricular septal defects were closed via the right atrium with myotomy and myectomy if a morphological substrate for subaortic stenosis was confirmed (n=4). There were two hospital deaths (15.4%) due to low cardiac output. In patients who underwent myotomy and myectomy for subaortic stenosis, the postoperative pressure gradient across the aortic valve was negligible. We conclude that surgical results of one-stage repair for the intracardiac malformations associated with interrupted aortic arch or aortic coarctation are reasonable. We suggest that the early relief of obstruction within the left ventricular outflow tract may have played some role in the favorable outcome.

Objective: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. Methods: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 ± 68 months. Catheterization carried out 16 ± 6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16 ± 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Conclusion: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

We report obstruction of the reconstructed left ventricular outflow tract diagnosed 18 months after an arterial switch operation that included closure of a ventricular septal defect. We speculate that either turbulences created by the patch used to close the ventricular septal defect, or postoperative modifications of ventricular geometry, were responsible for producing this unusual complication.