Project description:Quadricuspid aortic valves are rare congenital anomalies which can be diagnosed by various imaging modalities. Described is the case of a 77 year old female with a quadricuspid aortic valve diagnosed by cardiac CT.
Project description:Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly causing aortic regurgitation usually in the fifth to sixth decade of life. Earlier, the diagnosis was mostly during postmortem or intraoperative, but now with the advent of better imaging techniques such as transthoracic echocardiography, transesophageal echocardiography (TEE), and cardiac magnetic resonance imaging, more cases are being diagnosed in asymptomatic patients. We present a case of a 39-year-old male who was found to have QAV, with the help of TEE, while undergoing evaluation for a diastolic murmur. The patient was found to have Type B QAV with moderate aortic regurgitation. We also present a brief review of classification, pathophysiology, and embryological basis of this rare congenital anomaly. The importance of diagnosing QAV lies in the fact that majority of these patients will require surgery for aortic regurgitation and close follow-up so that aortic valve replacement/repair is done before the left ventricular decompensation occurs.
Project description:Coronary artery obstruction is an uncommon yet devastating complication of transcatheter aortic valve replacement (TAVR) and may necessitate leaflet modification. A 38-year-old man presented to our center with quadricuspid aortic valve with severe aortic regurgitation. Double leaflet modification was performed with the Bioprosthetic or native Aortic Scallop Intentional Laceration to prevent Iatrogenic Coronary Artery obstruction (BASILICA) technique prior to TAVR, creating 6 leaflets from 4. The patient tolerated the procedure well with symptomatic improvement. Follow-up transthoracic echocardiogram showed normal bioprosthetic aortic valve function. This case demonstrates feasibility of this procedure with comprehensive preprocedural analysis and intraprocedural imaging guidance.
Project description:BackgroundQuadricuspid aortic valve (QAV) is a rare congenital malformation that can present with aortic regurgitation or aortic stenosis (AS)), requiring surgical treatment. Transcatheter aortic valve replacement (TAVR) is an alternative treatment for older patients and its prognosis for QAV therapy remains challenging. We sought to examine our early experience with TAVR in patients with QAV.Materials and methodsProspectively collected data were retrospectively reviewed in patients with QAV undergoing TAVR in our institution.ResultsFive patients with QAV and AR or AS were treated with TAVR between January 2016 and January 2022. The mean age was 73.8 years (range 69-82 years), and the median Society of Thoracic Surgeons score was 7.51% (range 2.668-18.138%). Two patients had type B and three had either type A, D, or F according to the Hurwitz and Roberts classification for QAV. Four patients with pure aortic regurgitation underwent transapical TAVR using the J-Valve system, and the patient with severe AS underwent transfemoral TAVR using the Venus-A system. Procedural success was achieved in all five patients. Trivial paravalvular leak was only detected in one case after the procedure, and one patient received a permanent pacemaker due to high-degree atrioventricular block three days later. The median follow-up period was 18 (12-56) months. After discharge, no deaths occurred during the 1 year follow-up. All patients improved by ≥1 New York Heart Association functional class at 30 days; four patients were in functional class ≤II later in the follow-up period. All patients' heart failure symptoms improved considerably.ConclusionOur early experience with TAVR in QAV demonstrates these procedures to be feasible with acceptable early results. Further follow-up is necessary to determine the long-term outcomes of this modality.Clinical trial registration[ClinicalTrials.gov], identifier [NCT02917980].
Project description:A quadricuspid aortic valve is rarely diagnosed in children, but it can be associated with significant aortic regurgitation. It is important for pediatric cardiologists to be aware of this pathologic entity. We present a nine-year-old male, diagnosed with a quadricuspid aortic valve and mild aortic regurgitation.
Project description:BackgroundQuadricuspid aortic valve (QAV) is a rare congenital heart defect usually accompanied with different hemodynamic abnormalities. Due to the rarity of QAV, treatment and prognosis of QAV patients with aortic regurgitation still remain challenging. We here present the first case of a patient with severe QAV regurgitation who underwent successful treatment and performed favorable prognosis with transapical aortic valve implantation (TAVI) using J-Valve system.Case presentationA 62-year-old man experienced intermittent palpitation, shortness of breath and chest pain. Echocardiography revealed congenital QAV with massive aortic regurgitation and mild aortic stenosis, left ventricular enlargement. Aortic valve replacement was successfully performed with TAVI using J-Valve system. The postoperation and follow-up was uneventful.ConclusionTAVI using J-Valve system has emerged as a new high success rate method for treatment of patients with simple non-calcified aortic valve insufficiency.