Unveiling the Rare Quadricuspid Aortic Valve A Case of Severe Aortic Insufficiency in Adults

Introduction Congenital Quadricuspid Aortic Valve (QAV) is a rare heart valve anomaly, with an estimated prevalence of just 0.013% to 0.043%. Despite its uncommon occurrence, QAV is a critical condition often associated with aortic insufficiency (AI) and other cardiac abnormalities. This case report highlights the diagnosis and management of a patient with QAV and severe AI. Visit https://www.cardiologymedjournal.com/jccm for more groundbreaking research in cardiology.

Understanding Quadricuspid Aortic Valve QAV is characterized by the presence of four aortic valve leaflets instead of the usual three. The anomaly can lead to improper valve closure, resulting in AI, which was observed in 75% of QAV cases. This congenital defect may also coexist with coronary artery anomalies, septal defects, and other structural heart issues.

Case Presentation A 58-year-old woman with a history of uncontrolled hypertension and obesity presented with progressive dyspnea and chest pain. Clinical evaluation revealed a grade 2 diastolic murmur, abnormal electrocardiographic findings, and elevated cardiac biomarkers. Transthoracic and transesophageal echocardiography confirmed severe AI due to QAV, necessitating surgical intervention.

Surgical Management and Outcomes The patient underwent aortic valve replacement (AVR) with a mechanical valve. Intraoperatively, an anomalous origin of the right coronary artery was also noted. Postoperatively, complications such as supraventricular tachycardia, severe hemolytic anemia, and renal failure led to a fatal outcome within 11 days of surgery.

Clinical Implications and the Need for Early Detection Due to its rarity, QAV is often diagnosed incidentally during imaging or surgery. With the advancement of echocardiography, cardiac CT, and MRI, early recognition of QAV can improve patient outcomes. The American College of Cardiology (ACC) underscores the significance of timely detection and intervention in congenital valve anomalies to prevent severe AI progression.

Key Takeaways

• QAV is a rare congenital anomaly that predisposes patients to AI.
• Diagnosis relies on advanced imaging techniques, with transesophageal echocardiography being the gold standard.
• Early surgical intervention may be required to prevent complications.
• Comprehensive post-surgical monitoring is essential due to potential complications.

Further Reading and References For a detailed analysis, read the full study at https://com/10.29328/journal.jccm.1001005.

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