Bicuspid aortic valve with associated aortopathy, significant left ventricular hypertrophy or concomitant hypertrophic cardiomyopathy: A diagnostic and therapeutic challenge

Due to its prevalence of 0.5%to 2%in the general population,with a 75%predominance among men,bicuspid aortic valve is the most common congenital heart defect.It is frequently accompanied by other cardiac congenital anomalies,and clinical presentation can vary significantly,with stenosis being the most common manifestation,often resulting in mild to moderate concentric hypertrophy of the left ventricle.Echocardiography is the primary diagnostic modality utilized for establishing the diagnosis,and it is often the sole diagnostic tool relied upon by clinicians.However,due to the heterogeneous clinical presentation and possible associated anomalies(which are often overlooked in clinical practice),it is necessary to employ various diagnostic methods and persist in finding the accurate diagnosis if multiple inconsistencies exist.By employing this approach,we can effectively manage these patients and provide them with appropriate treatment.Through a clinical case from our practice,we provide an overview of the literature on bicuspid aortic valve with aortophaty and the possible association with hypertrophic cardiomyopathy,diagnostic methods,and treatment options.This review article highlights the critical significance of achieving an accurate diagnosis in patients with bicuspid aortic valve and significant left ventricular hypertrophy.It is crucial to exclude other possible causes of left ventricular outflow tract obstruction,such as sub-or supra-aortic obstructions,and hypertrophic cardiomyopathy.

Quadricuspid aortic valve and right ventricular type of myocardial bridging in an asymptomatic middle-aged woman:A case report

BACKGROUND Quadricuspid aortic valve(QAV)is a very rare congenital cardiac defect with the incidence of 0.0125%-0.033%(<0.05%)predominantly causing aortic regurgitation.A certain number of patients(nearly one-half)have abnormal function and often require surgery,commonly in their fifth or sixth decade.QAV usually appears as an isolated anomaly but may also be associated with other cardiac congenital defects.Echocardiography is considered the main diagnostic method although more and more importance is given to computed tomography(CT)and magnetic resonance imaging(MRI)as complementary methods.CASE SUMMARY A 60-year-old female patient was referred for transthoracic ultrasound of the heart as part of a routine examination in the treatment of arterial hypertension.She did not have any significant symptoms.QAV was confirmed and there were no elements of valve stenosis with moderate aortic regurgitation.At first,it seemed that in the projection of the presumed left coronary cusp,there were two smaller and equally large cusps along with two larger and normally developed cusps.Cardiac CT imaging was performed to obtain an even more precise valve morphology and it showed that the location of the supernumerary cusp is between the right and left coronary cusp,with visible central malcoaptation of the cusps.Also,coronary computed angiography confirmed the right-type of myocardial bridging at the distal segment of the left anterior descending coronary artery.Significant valve dysfunction often occurs in middle-aged patients and results in surgical treatment,therefore,a 1-year transthoracic echocardiogram control examination and follow-up was recommended to our patient.CONCLUSION This case highlights the importance of diagnosing QAV since it leads to progressive valve dysfunction and can be associated with other congenital heart defects which is important to detect,emphasizing the role of cardiac CT and MRI.