Vikram Bhangale, 35, of Mumbai, was referred by Dr Rajiv Karnik for corrective surgery for ALCAPA on August 12, 2011.
Case history given by Doctor:
Patient is a known case of anomalous location of coronary artery arising from Pulmonary Artery since birth, with mild hypertension and a history of gout with associated pain occasionally in ankles. He had a history of femur bone fracture and was operated in January 2006 and a rod was put in the right leg in Feb’10. He was admitted to AHI for corrective surgery for left coronary artery arising from main coronary artery.
He also had a Low Ejection Fraction.
Case history given by patient:
As a child the patient was asked to take medication and avoid rigorous activity. He was advised to visit the doctor if he felt uneasy. In spite of the medical condition, the patient had normal, fun childhood and participated in all activities. For the past two years he was experiencing heavy breathing and decided to get it checked and underwent an ECG which came out to be abnormal. He underwent Angiography before going to the US, where he underwent 2D echo tests and sent the reports to his doctor in India, Dr Rajiv Karnik. Dr. Rajiv Karnik referred him to AHI and provided all his history. The meeting with Dr Ramakanta Panda was excellent and the patient and his family felt very confident and comfortable after being briefed about the procedure. The meeting was smooth-sailing since Dr Panda already had the history of the patient and all the corresponding reports with him.
What makes this case interesting.
Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) is a rare but serious congenital anomaly.
The ALCAPA occurs when the baby’s heart is developing, early in the pregnancy. The developing blood vessels in the heart do not connect correctly. In normal individuals the left and right coronary arteries arise from the aorta at the point where the aorta meets the left ventricle & these supply oxygen rich blood to the heart. However in the case of this patient the left coronary artery was arising from the pulmonary artery which carries deoxygenated blood. As a result the left coronary artery carried deoxygenated blood to the heart as well. This is when the heart does not get enough oxygen and it begins to die which causes a condition leading to a heart attack in the baby.
If left untreated, the condition results in a mortality rate of up to 90% within thefirst year of life.
The survival of the individual depends on two factors – dominance and collaterals. In layman’s terms, Dominance determines which coronary artery supplies more blood to the heart. Collateral blood supply is the flow of blood from a coronary artery to an adjacent artery. This collateral circulation provides alternate routes of blood flow to the heart in cases when the heart isn’t getting the blood supply it needs.
Therefore, in the case of Patient Bhangale, his right coronary artery was dominant and the collateral blood supply from the right coronary artery to the left coronary artery was sufficient. Hence the patient could pass through childhood with relatively minor symptoms.
The procedure he underwent is called a Takeuchi repair. Due to the difficult location of the left coronary artery, an intrapulmonary artery tunnel was required to redirect oxygenated blood into the vessel from the aorta through an aortopulmonary window.
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