Anatomic Correction of the Syndrome of Aortic Valve Cusp Prolapse and Subaortic VSD via Vertical Right Axillary Thoracotomy

A seven-month-old, 8.5 kg infant was followed clinically for a large ventricular septal defect (VSD) until an echocardiographic evaluation raised concerns regarding aortic valve (AV) regurgitation secondary to a new prolapse of the aortic cusps into the large defect.

The combination of a large subaortic VSD, AV cusp (especially the right and/or non-coronary cusps) prolapse, and dilated associated sinus of Valsalva often present together and occur as a result of loss of continuity between the media and annulus of the AV, which results in sagging of the aortic annulus into the VSD. The repair in this video is done through a transaortic approach and results in an anatomic correction of all the components of the syndrome without using a prosthetic material.

Beginning the procedure with a vertical right axillary thoracotomy, surgeons entered the right chest through the right fourth intercostal space. After retraction of the right lung, the pericardial space was entered anterior to the right phrenic nerve. Heparin was administered systemically, and cardiopulmonary bypass (CPB) was initiated via central aortic and bicaval cannulation. After application of the aortic cross clamp (AXC), a hockey-stick aortotomy was made toward the middle of the noncoronary sinus of Valsalva of the aortic root and cardioplegic arrest was achieved via direct administration of cardioplegia into the coronary ostia. The large VSD was easily visualized via the aortic valve. A series of multiple interrupted 6-0 polypropylene sutures supported with bovine pericardial pledgets were placed in a horizontal mattress fashion passing through the crest of the interventricular septum and the aortic annulus, thus closing the defect, plicating the dilated sinus of Valsalva, and correcting the cusp prolapse. This addressed all the components of the syndrome.

The aortotomy was then closed in a two-layer fashion, the heart was deaired, and AXC was removed. The patient was weaned off CPB and post bypass transesophageal echocardiogram confirmed excellent repair with trace AV regurgitation, no prolapse, and no significant residual ventricular level shunt. The patient was then decannulated, heparin was reversed, and the incision was closed in the standard fashion. The AXC and CPB times were 68 and 91 minutes, respectively.

The patient was extubated in the operating room, received no transfusions, and the rest of the postoperative course was uneventful. He was discharged 48 hours later and continued to do well during his follow up with trivial aortic regurgitation.

References

1. Yacoub MH, Khan H, Stavri G, Shinebourne E, Radely-Smith R. Anatomic correction of the syndrome of prolapsing right coronary aortic cusp, dilation of the sinus of Valsalva, and ventricular septal defect. J Thorac Cardiovasc Surg 1997; Feb 113(2): 253-60
2. Said SM, Greathouse KC, McCarthy CM, Brown N, Kumar S, Salem MI, Kloesel B, Sainathan S. Safety and efficacy of right axillary thoracotomy for repair of congenital heart defects in children. World J Pediatr Congenit Heart Surg 2023 Jan; 14(1): 47-54

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