Ventricular septal defect (VSD) is a common congenital heart defect, with an incidence of 1.5-6 cases per 1000 live births. Certain VSDs are associated with AR due to prolapse of the noncoronary and right coronary leaflets. The prolapse of an aortic valve leaflet associated with a VSD is termed Laubry-Pezzi syndrome. This phenomenon is caused by the Venturi effect, which reduces the left-to-right shunt but distorts the aortic valve leaflets, leading to AR due to impaired leaflet coaptation. The development of AR, even if trivial, in the context of a VSD is an indication for patch closure surgery regardless of hemodynamic impact.
The article under review, conducted at Children’s National Hospital in Washington, DC, is a single-center, retrospective study analyzing patients who underwent VSD closure without concomitant aortic valve intervention. The study reviewed cases from April 2007 to March 2016, excluding patients with non-perimembranous or non-subpulmonary VSDs (juxta-arterial in Van Praagh’s terminology), those without AR at the time of surgery, or those with less than 6 months of follow-up.
A total of 37 patients were identified, half of whom were male. The mean age at surgery was 2.7 years, with a mean weight of 13.4 kg. Preoperative echocardiograms showed mild or greater AR in 17 patients. The study reported no mortality, surgical reinterventions, or percutaneous procedures. At a median follow-up of 4.3 years, only five patients presented with mild or greater AR, while 76% (28 patients) had no degree of AR. Multivariable logistic regression identified left ventricular ejection fraction (LVEF) as a predictor of AR persistence during follow-up (p = .002).
The authors concluded that VSD closure, even in cases with mild AR, can yield excellent outcomes without requiring aortic valve intervention. When AR is trivial or mild at the time of surgery, it generally improves during follow-up. Reduced LVEF is a predictor of AR persistence post-surgery.
COMMENTARY:
Risk factors for the development of AR after VSD closure include preoperative AR, large VSDs, structural abnormalities of the aortic leaflets, sinus of Valsalva aneurysm, among others. Aortic valve intervention is usually indicated when AR exceeds a mild grade. However, AR can progress during follow-up even in patients with trivial preoperative AR. Few studies have tracked these patients to determine the progression of AR.
Subpulmonary VSDs rarely close spontaneously, and in the case of perimembranous VSDs, spontaneous closure beyond 1-2 years of life is infrequent. Both entities can be associated with right coronary sinus herniation, with or without AR. The Venturi effect causes leaflet deformation, and AR often manifests between 5 and 8 years of age. Therefore, trivial AR or a subpulmonary VSD are indications for surgical VSD closure, regardless of shunt volume. Other mechanisms contributing to AR in these patients include inadequate leaflet support on the adjacent side of the VSD, abnormal commissural suspension, lack of apposition force, and discontinuity between the aortic medial layer and the annulus.
The main limitation of this study is its retrospective nature, small sample size, and short follow-up duration. As a strength, it is the first study to identify reduced LVEF as a predictor of persistent or worsening AR.
In conclusion, surgical VSD closure provides excellent short- and long-term outcomes. It prevents the onset or worsening of aortic valve disease by eliminating the deleterious effects of the Venturi effect on the aortic leaflets. Therefore, waiting for spontaneous closure in this patient subset is counterproductive.
REFERENCE:
Bukhari SM, Desai M, Zurakowski D, Christopher A, Tongut A, Ozturk M, et al. Fate of aortic regurgitation after isolated repair of ventricular septal defect with concomitant aortic regurgitation in children. JTCVS Open 2023 Mar;13:271–7. DOI: 10.1016/j.xjon.2022.12.015
