Title : The hidden gem of the aortic valve
Abstract:
Background: Quadricuspid aortic valve is a rare congenital cardiac defect with estimated incidence rate of 0.013%. Historically, encountered during open heart surgery or postmortem. Currently diagnosed primarily via transechocardiography. The etiology hypothesis involves the partition of one of the three valve cushions due to an invagination of the endothelial layer on the luminal side during early stage of valve development. Quadricuspid aortic valve frequently progresses to aortic regurgitation with clinical impact in adulthood.
Case: This is the case of a 72 years-old-female with past medical history of paroxysmal supraventricular tachycardia. She presented to the hospital with the complaint of palpitations and dizziness. Denies chest pain, syncope, shortness of breath, prior history of rheumatic fever or acute coronary syndrome. Electrocardiogram remarkable for normal sinus rhythm. On physical examination grade III/VI diastolic murmur at the 4th intercostal left sternal border. Transthoracic echocardiography showed quadricuspid aortic valve with moderate aortic regurgitation. Normal left ventricular ejection fraction, adequate chambers size and filling pressures.
Decision Making: This case is an example of quadricuspid aortic valve with subsequent symptoms that correlates valvular functional status. The most common clinical manifestation observed is aortic regurgitation. Surgical decision making depends on multiple factors, patient symptoms, aortic regurgitation severity, left ventricular dilation or dysfunction. Our case suggests that conservative management with serial imaging, aggressive blood pressure control and close follow-up is a reasonable management because at this moment patient does not meet criteria for surgical intervention.
Conclusion: Finally, quadricuspid aortic valve is a rare congenital cardiac disorder that could be find as isolated defect or in conjunction with other cardiac anomalies. Most cases are detected via echocardiography but due to their rarity may go undiagnosed. There are no guidelines regarding the pathology or the management but based on literature while 20% of cases requires surgery, 80% can be managed conservative.
