Bicuspid aortic valve
What is bicuspid aortic valve?
It is the most common congenital heart disease of cardiac origin in the population. It is estimated to affect 2% of people, and is more common in males.
An aortic valve of normal morphology has 3 sinuses, on each of which a leaflet depends, which act as a ‘gate’ for blood to exit from the left ventricle of the heart, favouring the flow of blood and preventing it from flowing backwards. In the case of the bicuspid valve, there are only two leaflets, which can lead to alterations in its function, either due to early degeneration or lack of closure or insufficiency.
Does it affect other structures?
In half of the patients, in addition to valvular heart disease, other anomalies are usually associated, the most frequent being the presence of aneurysms of the ascending aorta. It is also associated with another pathology known as coarctation of the aorta.
What symptoms does it cause?
The presence of symptoms is usually a consequence of the severity of the valve disease. A heart murmur is usually the first thing a doctor detects, often at a routine check-up, and often without symptoms. In patients with advanced degrees of valve deterioration, dyspnoea or ‘shortness of breath’ on exertion begins to appear, which may limit the ability to perform normal activities. If the disease progresses, heart failure eventually develops, and the heart’s ability to pump blood deteriorates. As a result, peripheral oedema (swelling of the ankles), pulmonary oedema and specific medical treatments are required, which reduce the symptoms but do not treat the main problem, which is the malfunctioning of the valve.
What other pathologies can I have?
Patients with valve disease are at increased risk of valve infections (endocarditis).
Patients with bicuspid aortic valve associated with root dilatation or ascending aorta are at risk of aortic dissection, a life-threatening complication.
I have been diagnosed with bicuspid valve disease, what should my relatives do?
This is a pathology in which, although there is no genetic mechanism of direct inheritance described, there is a familial association. You should inform your first-degree relatives (parents and children) so that they can undergo the corresponding cardiological study.
How is it diagnosed?
The most reliable method of diagnosis is by echocardiogram. This test assesses the anatomy of the valve, as well as its function, and diagnoses whether it is suffering from calcific degeneration, which can lead to stenosis, or whether it is non-closing or insufficient. It is also possible to measure the diameters of the root and ascending aorta and diagnose any associated aneurysms, although for the latter it is more reliable to perform a computerised axial tomography, commonly known as a ‘CAT scan’ or ‘scanner’.
Imaging tests allow us to classify bicuspid valve disease. In most cases (90-95%) there are 3 sinuses of Valsalva, with a fusion between 2 of the leaflets, called a ‘raphe’.
Image source:
International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.
Michelena, Hector I. et al.
The Annals of Thoracic Surgery, Volume 112, Issue 3, e203 – e235
I have bicuspid aortic valve disease, what should I do?
You should lead a healthy lifestyle, taking special care with modifiable cardiovascular risk factors. Smoking is discouraged, and a Mediterranean diet and an active lifestyle are recommended, avoiding a sedentary lifestyle. Cholesterol, blood sugar levels and especially blood pressure should be monitored, especially if associated with dilatation of the root or ascending aorta.
My valve disease is severe, is there a solution?
If the anatomy is favourable, bicuspid valves are surgically repairable valves: their function can be restored in the operating theatre, thus avoiding the need for the implantation of an artificial valve prosthesis. This eliminates the need for lifelong medications such as anticoagulants and improves life expectancy and quality of life for patients.
In cases where there is degeneration due to calcification, the valves are difficult to repair, so the alternative is usually an aortic prosthesis or a Ross procedure.
