Background: Apical ventricular septal defects (VSDs) are difficult to visualize and close transatrially. We described their distinctive anatomic features, which have seldom been documented angiocardiographically and pathologically, in order to develop an effective approach for their surgical management.
Methods: Fourteen postmortem cases, two explanted hearts, 9 successfully operated patients, and 1 unoperated living patient were included in this report. Angiocardiographic documentation of the apical VSD was available in 14 of 16 (87.5%) of the postmortem and transplanted cases, and in 6 of 10 (60%) of the living patients. Echocardiograms were available in 23 of all 26 cases (88%).
Results: Severe associated malformations were present in 14 of 16 (87%) of the pathologically documented cases. Large VSDs allowed extensive communication between the left ventricular and the right ventricular sinuses in 4 patients. In 12 of the pathologically documented cases and in the 10 living patients, the left ventricular apex communicated with the right ventricular apical infundibular recess.
Conclusions: Extremely large apical VSDs with severe biventricular dysplasia and dysfunction may require cardiac transplantation. Large apical VSDs can be successfully closed through a small apical infundibulotomy. This approach, applicable even in small infants, can avoid pulmonary artery banding or left ventriculotomy.
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