Erin, born 1993
Erin was born on November 1, 1993. Right before she went home from the hospital, her pediatrician heard a murmur and recommended that she see a pediatric cardiologist. When she was eight days old, she had an echocardiogram and it revealed a moderate size ventricular septal defect, a moderate size atrial septal defect, and a right aortic arch with aberrant left subclavian artery. She was not showing any signs of distress so we took her home with instructions to watch for signs of congestive heart failure.
When she was three weeks old, a bout of RSV sent her into heart failure. She was placed on lasix and digoxin. Over the next eleven months she had recurrent RSV, bronchiolitis, and ear infections. She was in and out of the hospital repeatedly until her first birthday.
At the age of one, it was revealed during a routine echo that the ASD had spontaneously closed. With these findings, her cardiologist felt that it was worthwhile to continue to watch her to see if the VSD would also close. He felt that the vascular ring would eventually need to be repaired but felt it could wait until she was older.
Over the next few years, she did well. She was smaller than other children her age but could still keep up. At the age of eight she began to have problems breathing and swallowing solid food. Her cardiologist felt this was due to the vascular ring which tightly encircled her trachea and esophagus.
In May 2002, she underwent a video assisted thoracotomy (VATS) to repair the vascular ring. The doctors still believed it was premature to repair the VSD even though it had not changed in size over the years. After the VATS procedure she seemed to have fewer problems swallowing, although her breathing was still labored when she was active. The doctors did not feel this indicated any problems and recommended monitoring of the VSD which they felt would not adversely affect her over the course of her life. However, at the age of 10, we were not comfortable continuing this conservative treatment as Erin seemed to tire very easily.
In February of 2004, we traveled to New York City to consult with a cardiologist at NYU Medical Center. After an echocardiogram revealed that her heart was considerately enlarged with leaky valves, they recommended surgical repair. Instead of using the sternal approach, they made an incision through the right side of the chest between the ribs.
During surgery, they discovered that Erin had developed a double chambered right ventricle (DCRV). Without repair, her right ventricle would become so muscular that it would not properly fill with blood. In a 5 1/2 surgical procedure, they were able to repair both the VSD and the DCRV with an uneventful recovery. She was back to school 10 days post op and went skiing just six weeks after surgery!
Today, Erin is healthy and plays soccer and lacrosse competitively.
Story by Erin's mom, Nancy - New Hampshire