Clinical Outcomes in Patients With Quadricuspid vs Bicuspid Aortic Valve

Area of Science:

• Cardiovascular Medicine
• Congenital Heart Disease
• Valvular Heart Disease

Background:

• Congenital aortic valve disease is common, with bicuspid aortic valve (BAV) being the most frequent type.
• Quadricuspid aortic valve (QAV) is a rare congenital anomaly, and its clinical outcomes compared to BAV are not well-established.
• Understanding the distinct clinical trajectories of QAV and BAV is crucial for patient management and risk stratification.

Purpose of the Study:

• To compare the clinical outcomes between patients diagnosed with quadricuspid aortic valve (QAV) and bicuspid aortic valve (BAV).
• To investigate differences in the rates of aortic valve intervention, aortic surgery, aortic dissection, infective endocarditis, heart failure hospitalization, and all-cause mortality between QAV and BAV cohorts.
• To assess the long-term survival relative to the general population for individuals with QAV versus BAV.

Main Methods:

• A retrospective cohort study was conducted using echocardiographic data from January 1, 2011, to December 31, 2023, at two tertiary hospitals in China.
• Patients diagnosed with QAV or BAV were identified, with QAV patients propensity score matched 1:5 to BAV patients based on age and sex.
• Outcomes including aortic valve intervention, aortic surgery, aortic dissection, infective endocarditis (IE), heart failure hospitalization (HFH), and all-cause death were analyzed using cumulative incidence functions and multivariable adjustments.

Main Results:

• The study included 139 QAV patients and 695 BAV patients, followed for a median of 4.8 years.
• Both QAV and BAV cohorts showed similar rates of aortic valve intervention (77.4% vs 75.7%).
• QAV patients had a lower incidence of aortic surgery (5.1% vs 33.8%) and IE (0% vs 9.6%) but a higher risk of HFH (adjusted hazard ratio [AHR], 2.52) compared to BAV patients. Cardiomyopathy was an independent predictor of HFH in QAV patients (AHR, 4.27).
• Aortic dissection was rare in both groups. The 5-year relative survival rates were comparable to the general population for both QAV (102.9%) and BAV (102.4%).

Conclusions:

• Both QAV and BAV are associated with a high need for aortic valve intervention.
• QAV is linked to an increased risk of heart failure hospitalization, whereas BAV is associated with higher rates of infective endocarditis and aortic surgery.
• Despite significant morbidity, long-term survival for patients with QAV or BAV is similar to that of the general population.