Symptoms of Bicuspid Aortic Valve Disease
Many patients with bicuspid aortic valve remain asymptomatic for years or decades, but when symptoms develop, they typically reflect progressive valve dysfunction (stenosis or regurgitation) or aortic complications, with dyspnea on exertion being the most common initial symptom. 1
Asymptomatic Phase
- The majority of patients with bicuspid aortic valve and minimal hemodynamic abnormality are asymptomatic and may remain so for many years. 1, 2
- Patients may be completely unaware of the condition until detected incidentally during routine examination or imaging. 2, 3
- The asymptomatic phase can persist even with mild-to-moderate valve dysfunction. 2
Symptoms Related to Aortic Stenosis
When stenosis develops and progresses, symptoms emerge in a predictable pattern:
- Dyspnea on exertion or decreased exercise tolerance is typically the first symptom to appear, reflecting early heart failure. 1, 4
- Syncope, particularly with exertion, occurs due to inability to increase cardiac output in response to peripheral vasodilation. 1, 4
- Angina pectoris develops from reduced coronary reserve and myocardial ischemia, even without coronary artery disease. 1, 4
- These classical symptoms (dyspnea, syncope, angina) typically appear late in the disease course and signal poor prognosis without intervention. 1, 4
Symptoms Related to Aortic Regurgitation
When regurgitation is the predominant lesion:
- Patients remain asymptomatic during the chronic compensated phase, often for many years. 1
- Dyspnea on exertion appears as left ventricular dysfunction progresses. 1
- Arrhythmias (palpitations) develop as the disease advances. 1
- Angina occurs in advanced cases due to increased myocardial oxygen demand from volume overload. 1
Associated Aortic Complications
Bicuspid aortic valve is associated with aortopathy that can produce distinct symptoms:
- Most aortic dilatation and aneurysm formation is asymptomatic until complications occur. 5, 6
- Acute aortic dissection presents with sudden severe chest or back pain (though this is rare in the natural history). 1
Other Clinical Manifestations
- Fatigue may be reported as cardiac function declines. 1
- Heart murmur is often the first clinical finding that prompts further evaluation, detected on routine examination. 1
- Patients may subconsciously reduce their activity level, masking symptoms and falsely reporting themselves as "asymptomatic." 1
Critical Clinical Pitfall
The absence of symptoms does not guarantee benign disease. Careful questioning is essential because patients often unconsciously limit their activities to avoid symptoms. 1 Exercise testing may reveal functional limitations not apparent from history alone. 1
Prognostic Significance of Symptoms
- Once symptoms develop in severe aortic stenosis, prognosis is extremely poor without intervention, with average survival of only 2 years after heart failure onset, 3 years after syncope, and 5 years after angina. 1, 4
- Asymptomatic patients with bicuspid aortic valve and minimal dysfunction have excellent long-term survival comparable to the general population. 2
- However, even asymptomatic patients face frequent cardiovascular events over time (42% at 20 years), particularly with progressive valve dysfunction. 2