Aortic valve repair in adult congenital heart disease

Abstract

Aortic valve repair in adult congenital heart disease (ACHD) went through a major development during the last two decades to become an increasingly established treatment option in experienced heart valve repair centers. This mini-review addresses valve-sparing treatment strategies in the two most common clinical entities of patients with adult congenital aortic valve disease, namely those presenting with bicuspid (BAV) and unicuspid (UAV) aortic valve disease. Both diseases are integral components of the continuum of congenital aortic valve diseases and represent one of the most common reasons of cardiovascular morbidity in young and otherwise healthy adult patients. The review will highlight the most important advantages of aortic valve sparing procedures as compared to the conventional valve replacement strategy. New treatment aspects will be reviewed including minimally-invasive surgical approaches for aortic valve repair as well as modern protocols of enhanced perioperative recovery which will potentially improve the perioperative recovery and quality of life of the patients undergoing valve-sparing surgical procedures in the future.

Keywords: Aortic valve repair; bicuspid aortic valve (BAV); enhanced perioperative recovery; heart valve reconstruction; minimally invasive surgery; unicuspid aortic valve (UAV).

Figure 1

Forms of aortic valve annulus dilatation in ACHD patients. (A) Dilatation of the basal ring; (B) STJ dilatation; (C) dilatation of the whole aortic root. ACHD, adult congenital heart disease; STJ, sinotubular junction.

Figure 2

Types of cusp disease in ACHD patients and the corresponding surgical techniques. (A) Type I: normal cusp movement and the surgical technique of valve sparing root surgery. Arrow indicates Dacron prosthesis which is used for reimplantation procedure; (B) type II: cusp prolapse addressed by central plication of the fused cusp. Arrow indicates cusp plication in the central part of the cusp to correct the prolapse; (C) type III: restrictive cusp movement treated by cusp augmentation using pericardial patch. Arrow indicates decellularized bovine pericardial patch implantation to address cusp restriction. ACHD, adult congenital heart disease.

Figure 3

The most common congenital-morphologic substrate of BAV regurgitation. Arrows indicate prolapse of the fused cusp. BAV, bicuspid aortic valve.

Figure 4

Recreation of the geometric configuration of the functional bicuspid aortic root. (A) Asymmetric Sievers type I R/L BAV with dilatation of basal ring to 34 mm, prolapse of the fused cusp (*), normal non-coronary cusp (#) and aneurysm of the aortic root which has been excised. Arrow indicates raphe between right and left coronary cusp; (B) valve sparing root replacement by remodeling technique (*) with an additional reduction of basal ring diameter to 25 mm, prolapse correction of both cusps (arrow) and recreation of the symmetric configuration of bicuspid valve. Cross marks indicate the bottom of right coronary ostium.

Figure 5

Echocardiographic and intraoperative appearance of the unicuspid aortic valve. Red arrows indicate: (A) two fused commissures and a typical posterior opening towards the left atrium of UAV in a transesophageal echocardiographic image; (B,C) and the intraoperative appearance of unicuspid aortic valve before and after excision during the surgery.

Figure 6

Complex aortic valve repair using a partial upper sternotomy approach.