Nobody knew the reason for 18-year-old Naveen’s height in his small village. He was more than a foot taller than the tallest person in his family.
While the tallest in his family did not cross five-and-a-half feet Naveen reached 6.6 feet and was still growing. Things went on smoothly until it was found that it was not just his height that was increasing. The size of his heart was also growing, but at an unhealthy rate. While the width of the heart is normally 4 cm, in Naveen it had increased to 8.5 cm.
Diagnosed with Marfan Syndrome, Naveen can grow up to 7 feet, but there is no scope for his heart to grow any further. Cardiac surgeons found it difficult to find an artificial valve of proper size for him and they had to improvise.
The youngster developed breathlessness and palpitation because of the enormous size of his heart. There was no doctor who could diagnose his condition in his native Chinthalapudi village in West Godavari district. Naveen had to run from pillar to post until he was diagnosed with the rare Marfan Syndrome. Marfan Syndrome is an inherited disease in which a faulty gene leads to insufficient production of a protein causing formation of defective connective tissue.
It was in this critical stage that Naveen approached The Brain and Heart Institute of the Andhra Group of Hospitals. The hospital, in an eight-hour operation, replaced the defective valve and put a graft on the aorta. It had to use deep hypothermic circulatory arrest (DHCA), a surgical technique that involves cooling the body temperatures below 20 degrees Celsius and stop blood circulation and brain function for up to one hour. This had to be done to put a graft on the aneurysm on the aorta. After the successful surgery, Naveen was discharged on Thursday.
Size and fit matter
The big problem for cardiac surgeon R. Dileep Kumar is finding a valve to fit Naveen’s heart. Again size become a problem for him. While artificial aortic valve is available in size 31, Naveen required 33. “I had to reverse (valves are unidirectional) the bigger Mitral value (between Left Atrium and Left Ventricle chambers of the heart) and use it. This innovation had to be done on the operation table,” Dr. Kumar said.
