Dehradun. A 53 years old lady underwent successful open heart surgery for a complicated and
extremely rare birth defect of the heart at Shri Mahant Indiresh Hospital. In this rare congenital defect of the heart there was formation of a pipe like connection in-between the left Circumflex Coronary Artery, the left Superior Vena Cava and the Coronary Sinus which resulted in abnormal blood flow in the body.
In Medical Science, this disease is called ‘Left Circumflex Coronary Artery to Left Superior Vena Cava and Coronary Sinus Fistula’. In the normal hearts the Superior Vena Cava andthe Coronary Sinus return oxygen-poor blood from the body and the heart respectively to the right chambers of the heart. This oxygen-poor blood arriving in the right chambers of the heart is then pumped into the lungs where it picks oxygen.Next this oxygen-rich blood from the lungs is then pumped by the left chambers of the heart into the whole body being distributed by the branches of Aorta which isthe main artery from the heart to the body.
The Circumflex Coronary Artery originates from the Aorta and supplies oxygen-rich blood and nutrients to the muscle of the heart for the blood-pumping action. In the patient’s heart the left circumflex coronary artery originated normally from the aorta and was supplying oxygenated blood to the heart muscle in a normal fashion. But by its communication to the coronary sinus,the left circumflex coronary artery was also by passing a large volume of this oxygenated blood from the aorta directly into the right heart chambers and then into
the lungs. As a result of this abnormal blood pathway, the body was receiving only half supply of its blood requirement and the lungs were getting over-flooded by the twice normal volume of blood flow.This led to the heart enlarging to double the normal size. The Circumflex Artery and the Coronary Sinus enlarged almost ten times the normal size.
The Tricuspid Valve located in between the right chambers of the heart ware stretched by the large amount of the blood passing across it and the valve started to leak back blood. The lungs were over-distended by the flowing blood and were not able to function properly. All these changes started after birth and had gradually deteriorated over the years. The patient developed progressively worsening breathing difficulty, increased heart beat, pain in chest, swelling of the whole body, restlessness, etc. since the last many years. She suffered from persistent vomiting and poor digestion too. The patient was investigated in the Cardiology Department of Shri Mahant Indiresh Hospital, Dehradun, for these complaints for the first time in 2017 when ECG, chest X-RAY, Echo cardiography, Coronary Angiography, Cardiac CT and CT Angiography were performed. Subsequently the diagnosis of the rare congenital defect was finally made and the prognosis and treatment was explained and discussed with the family and patient.
However, the patient opted for medical management at that time instead of surgical correction. As was expected, in spite of all medicines,the symptoms continued to worsen over the years since 2017. Eventually, when she could not bear it anymore she agreed for the surgical correction of the congenital defect. Accordingly she was planned for the surgical correction of the heart defect.The main motive of the operation was to correct the abnormal circulation of blood in heart and to repair the damaged Tricuspid valve. During the operation, it was also mandatory that no deficiency occurred in the supply of oxygen rich blood to the muscles of the heart otherwise the patient would need Coronary Artery bypass surgery also.Eventually she underwent a 6 hour long open heart surgery during which head of the CTVS department,Dr Ashok Kumar Jayant and his surgical team corrected the abnormal pathway and blood circulation of the heart.
The tricuspid valve which was leaking badly was also repaired. As a result of accurate and successful operation, the need for Coronary bypass surgery did not arise. After the surgery the patient was nursed in the ICU where she had a smooth recovery and was discharged 9 days after the operation. Dr Ashok Kumar Jayant, trained at the Delhi AIIMS, communicated that “This was an extremely complicated and rarest of the rare heart surgeries in the world which was done for the first time in the state of Uttarakhand. In my 37 years experience of Cardio-Vascular surgeries, I encountered such an abnormal heart formation for the first time. The credit of success of this surgery goes to the efficient surgical team of CTVS, experiences anesthesiologists Dr. Parag & Dr.Hariom, Cardiologist Dr. Tanuj Bhatia and hardworking CTVS staff nurses and world-class ultra-modern infrastructure of Shri Mahant Indiresh Hospital”
