Comment
CLVD often represent novel territory with regards to management and, at times, diagnosis for most heart teams. Not only is the prevalence fairly low, ubiquitously lower than 1% as previously described, but additionally the natural history of the disease is not well delineated. There are two distinct pathologies that are clearly described in the literature, with CLVD representing an outpouching from the ventricle, with synchronous contraction, and histologically similar to that of the ventricular wall from which it protrudes. Congenital left ventricular aneurysm (CLVA) represents the other end of the spectrum, whereby the outpouching is dyskinetic or altogether akinetic, and histologically composed of fibrous tissue, dissimilar to that of the ventricular wall [5]. Despite the dearth of epidemiological data, CLVD are not clinically silent, with reports of important cardiac complications such as thromboembolism, ventricular arrhythmias, and myocardial ischemia. Additionally, catastrophic CLVD rupture has been described, leading to sudden death [6].
Due to the rarity of CLVD, there is little guidance for the clinician regarding appropriate treatment paradigms, be it a surgical or conservative approach. In a review of 809 patients published since 1816 with either CLVD or CLVA, 4.5% of patients with CLVD were found to have an episode of rupture, and a 5.0% reported rate of cardiac death. Other significant complications included ventricular tachycardia/fibrillation in 13.1%, embolic events 3.6%, and syncope 5.1% [7].
Despite the existence of a concise review of the cases reported to date, there is little in the way of a detailed surgical approach. Some authors have reported closure of the diverticulum neck with a patch, with surgical glue closure and plication with aneurysmorrhaphy [8]. Other authors have described their technique as suture reapproximation in a double layered fashion with the use of felt [9], while some describe removal of the outpouching cavity and closure with an in-situ patch [10].
Due to the catastrophic events associated with CLVD, our institution prefers surgical management for patients with symptomatic CLVD. The first patch of our two patch technique helps retain the native geometry of the left ventricle. Meanwhile, linear closure of the CLVD may cause a reduction of the ventricular diameter and distortion of contractile force vectors. The addition of a second patch to the ventricular epicardial surface in our approach decreases early postoperative bleeding and should minimize long term aneurysm formation and rupture. Further study is necessary to determine the benefits of this technique.