Unicuspid Aortic Valve, an Extremely Rare Congenital Anomaly in Adults: A Systemic Review

The most common congenital heart defect is the bicuspid aortic valve (BAV) occurring with fusion of one of the three-valve commissures. A rarer valvular phenomenon is the unicuspid aortic valve (UAV) which has an estimated prevalence of 0.02% in the adult population. Two morphologic subtypes of UAV exist: the earlier presenting, acommissural UAV and the later appearing unicommissural. To better characterize and manage patients with UAVs, a systematic review of this rare phenomenon was performed. The objective of our study was to conduct a systematic review of adequate case studies involving UAV patients to describe patient characteristics, clinical presentation, management, and common sequela featured there within. The mean age of presentation in this review was 35.6 years. When diagnosing UAV, TEE was the most utilized diagnostic modality (65%) followed by intraoperative diagnosis (17%) discovered with valve repair or incidentally during another procedure. TTE was utilized to diagnose only 7% of the cases reviewed likely owing to the difficulty and inaccuracies in identifying UAV with this method that were previously established by multiple series. Interventional options for UAV are balloon versus surgical valvotomy, aortic valve replacement, Ross procedure and aortic root replacement. They are performed on an individual basis and all cases ultimately require aortic valve replacement (AVR) or Ross procedure (to avoid anticoagulation). Aortic root replacement is additionally required if aortic root/ascending aorta diameter exceeds 4.5 cm. In this review, Aortic aneurysm (16%) cases resulted in surgical interventions. There may be a need to implant permanent pacemaker (in 3% - 8%) during AVR when calcified UAV has calcification extending into interventricular septum.

A Rare Case of Improved Mitral Regurgitation after the Inter-Atrial Septal Defect Created during an Unsuccessful Percutaneous Mitra-Clip Placement Attempt

Percutaneous mitral valve repair has shown to be a less-invasive treatment option for patients with symptomatic severe mitral regurgitation (MR) with multiple comorbidities. We describe a case of improved mitral regurgitation due to improved atrial fibrillation secondary to left atrial pressure relief after the inter-atrial defect created during an unsuccessful mitraclip placement attempt. Transthoracic Echocardiogram that was performed on admission showed severe mitral valve regurgitation. She was not a surgical candidate due to multiple co-morbidities. Patient was then medically optimized and a percutaneous MitraClip placement (PMCP) was attempted but was unsuccessful due to excessive trans-mitral gradient and the procedure was aborted. However, left atrial pressure decreased, likely secondary to inter-atrial septal defect created by the procedure. Transesophageal echocardiogram performed post-op showed moderate and improved mitral regurgitation and sinus rhythm. Attempts to convert atrial fibrillation to sinus rhythm to improve mitral regurgitation had to be made before continuing with a mitral clip placement procedure in our case. In our case, the procedure itself did not help patient’s symptoms, but the resulting acute atrial pressure relief improved mitral regurgitation overall due to left to right shunt from iASD, which also helped the rhythm.