ALCAPA – the rare heart disease & surgery.
- drpavankumar
- 0
- on Mar 29, 2023
A.L.C.A.P.A. stands for anomalous left coronary artery from the pulmonary artery. It is a rare heart disease where the left coronary artery, which supplies oxygen-rich blood to the heart muscle, arises abnormally from the pulmonary artery instead of the aorta.
This abnormality can cause a variety of symptoms, including chest pain, shortness of breath, fatigue, and dizziness. In some cases, it can lead to a life-threatening condition known as myocardial infarction or a heart attack.
A.L.C.A.P.A. is usually diagnosed in infancy or childhood, but in some cases, it may not be detected until adulthood. Diagnosis typically involves a physical examination, electrocardiogram, echocardiogram, and cardiac MRI or CT scan.
Treatment for A.L.C.A.P.A. involves surgery to correct the abnormality and restore normal blood flow to the heart. The surgery may involve repositioning the coronary artery and attaching it to the aorta or creating a new pathway for blood flow.
With prompt diagnosis and treatment, most individuals with A.L.C.A.P.A. can live normal, healthy lives. However, without treatment, the condition can lead to significant heart damage and even death.
Overall, A.L.C.A.P.A. is a rare but serious heart condition that requires prompt diagnosis and treatment. If you or someone you know is experiencing symptoms such as chest pain or shortness of breath, it is important to seek medical attention immediately. Early diagnosis and treatment can greatly improve outcomes for individuals with A.L.C.A.P.A.
Here is the patient Dilshad Mulani 53 year old lady who hails from Temple town Pandharpur ,
Maharashtra. She never knew that she had this serious and very rare heart disease from birth. All this — she complained of breathlessness and occasional chest pain on exertion. She was checked by local physician and diagnosed to be having leaking mitral heart valve 15 years ago and advised medical management. Her symptoms increased gradually and this while suffering from Dengue she underwent full check up and Echo-cardiography where severe mitral valve regurgitation and heart
enlargement was detected. Further coronary angiography and cardiac CT Scan Angio established the diagnosis of ALCAPA + severe mitral valve regurgitation with myocardial scarring. Apart from this she also had Diabetes and renal failure. She was advised heart surgery. According to senior heart surgeon Dr Pavan Kumar who operated on this lady at Lilavati Hospital on 10/ 11 /2021, this A.L.C.A.P.A is rare and only 10% surviving to adulthood makes it more rarer. Here prolonged lack of oxygenated blood supply and right to left shunt of blood causes two important consequences
1 ) Heart ischemia and silent heart attacks.
2 ) Severe heart failure
3) Ischemic Mitral valve leakage.
Unfortunately all these complications were present in this case with insulin dependent Diabetes and renal failure. In this 7 hour long complex heart surgery, we closed abnormal opening of left coronary system in pulmonary trunk and put two separate graphs of oxygenated blood supply by means of CABG so that pure and impure blood doesn’t mix in coronary system. Then we replaced leaking mitral valve and corrected heart rhythm.
Dr. Pavan Kumar said that this patient had advanced stage of heart failure and signs of many heart attacks / (scarred heart muscles) and Diabetes and renal failure. All this made heart surgery complex and risky. this triple heart operation of ALCAPA repair and Mitral valve replacement and CABG is probably first of its kind reported as per ALCAPA in adult surgery is concerned. Patient did well and has been discharged after successful heart surgery.