Supravalvular aortic stenosis in adult: the three-patch technique:

Supravalvular aortic stenosis is a rare congenital malformation seen in pediatric age group as a result of an abnormal thickening of the aortic wall. This entity in adult age is further rare and we report a case of successful repair (Brom’s procedure) in an adult.

Prof. Brom worked in the UAE as the head of the department of cardiac surgery in Mafraq Hospital. During this time, he had his coronary bypass surgery done by Dr. Y. A. Nazer. Prof. Brom is considered as the father of pediatric cardiac surgery.

Introduction

Congenital supravalvular aortic stenosis (SVAS) is an uncommon anomaly presented as a narrowing situated at the level of the sinotubular junction. An early diagnosis is necessary in order to avoid future complications. Surgical treatment should ideally be performed in infancy to prevent early aortic valve degeneration, coronary artery pathology and left ventricular hypertrophy.

Case history

A 36-year-old male expatriate foreign national who presented with shortness of breath and chest pain of one-year duration. He had a detailed cardiology workup and his transthoracic echocardiogram showed supraaortic stenosis with post stenotic dilatation of the ascending aorta.

Preoperative transesophageal echocardiogram showed a supraaortic gradient more than 100 mmHg with mild aortic regurgitation.

Surgical technique

Under cardiopulmonary bypass and cardioplegic arrest, the aorta is transected just above the stenosis. Intraoperative findings were severe narrowing of the sinotubular junction with thick aortic valve. Aortic valve is tricuspid and normal leaflets. Left coronary osteum shifted more towards the left side.

Three longitudinal incisions were made in each sinus of Valsalva and rectangular shaped glutaraldehyde treated autologous pericardial patches were sutured. Care is taken to design the correct size of the patch. We have taken all precautions to avoid commissural extension and possible aortic regurgitation. An enlargement of the distal ascending aorta is also done with another piece of autologous pericardium (modified Brom’s procedure, four-patch technique).

Both coronary ostea were inspected and were found normal. Aorta closed in two layers and came off bypass in stable hemodynamics. Post-operative transesophageal echocardiography showed no gradient across the LVOT and aorta and no aortic regurgitation. The patient had a smooth post-operative recovery and discharged on seventh post-operative day. He is back to his normal activities.

Discussion

The three-patch technique, designed by Prof. Brom, offered a new concept for restoration of the aortic tract that continues to be the ideal model to repair supravalvular aortic stenosis both in pediatric and adult age group. There are multiple advantages in this technique. It provides a more symmetric reconstruction, less distortion of the aortic root and the ascending aorta. Glutaraldehyde treated autologous pericardium provides a normal thickness vascular tissue. This technique is easier than the other techniques and the individual patches can be designed in such a way that aortic root restoration will be optimal.

Brom’s procedure in adult done by Brom’s surgeon - Burjeel