Congenital Quadricuspid Aortic Valve: A Rare but Significant Cause of Aortic Insufficiency

Congenital quadricuspid aortic valve (QAV) is a rare cardiac malformation, with a reported prevalence of just 0.013% to 0.043%. Although often asymptomatic in early life, QAV frequently leads to aortic insufficiency (AI) in adulthood, necessitating surgical intervention. A recent case report published in the Journal of Cardiology and Cardiovascular Medicine sheds light on the clinical presentation and challenges associated with QAV.

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Case Summary: Quadricuspid Aortic Valve and Severe Aortic Insufficiency

A 58-year-old woman with a history of uncontrolled hypertension, obesity, and recurrent pancreatitis presented with chest pain and progressive dyspnea. Upon examination, she exhibited a grade 2 diastolic murmur, and echocardiography confirmed severe aortic insufficiency due to a quadricuspid aortic valve. Further imaging revealed an anomalous origin of the right coronary artery, a condition observed in up to 10% of QAV cases.

Despite undergoing an aortic valve replacement (AVR) with a mechanical valve, the patient’s post-surgical course was complicated by supraventricular tachycardia, pericardial effusion, and renal failure, ultimately leading to her demise eleven days post-operation.

Read the full study at https://doi.org/10.29328/journal.jccm.1001005.

Understanding QAV and Its Clinical Implications

While tricuspid aortic valves are the norm, quadricuspid valves feature an additional cusp, disrupting normal valve function and increasing susceptibility to AI. Over 75% of QAV cases develop aortic insufficiency, necessitating valve replacement surgery in nearly half of all diagnosed patients. The condition is often detected incidentally during imaging or surgery.

The Role of Imaging in Diagnosing QAV

With advancements in echocardiography, cardiac MRI, and CT imaging, the detection of QAV has improved. Transesophageal echocardiography (TEE) remains a crucial tool for diagnosing QAV, especially in patients with unexplained AI or unusual aortic valve morphology.

The American College of Cardiology (ACC) emphasizes the role of early imaging diagnostics in congenital valve disorders, which can significantly impact treatment outcomes (ACC).

Surgical Management and Prognosis:

The primary treatment for symptomatic QAV patients with severe AI is aortic valve replacement (AVR). In some cases, valve repair may be an option, but due to abnormal cusp morphology, replacement remains the preferred intervention. Early diagnosis and intervention can improve long-term survival rates, particularly in patients with additional cardiac anomalies.

The Future of QAV Management

With the increasing use of advanced imaging techniques, more cases of quadricuspid aortic valve are being identified earlier, leading to better patient outcomes. Continued research into the genetic and embryological factors behind QAV may provide insights into potential preventive measures.

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