Background and diagnosis

Vinay Goyal was born March 2, 1990, in St. Boniface Hospital, to Vipan and Sheena Goyal. He was diagnosed at birth with a number of heart defects, including Tetralogy of Fallot. He also had Trisomy-21 or Down's syndrome. After 16 days in hospital, he was discharged home on no specific treatment or medication.

On Monday, June 4, 1990, Vinay was seen in the cardiology clinic at the Winnipeg VCHC. He was breathing rapidly, and his mother reported that his lips were blue when feeding. When seen again on November 12, 1990, following his family's return from India, Vinay appeared deeply cyanosed. He also had clubbing of his fingers. (Clubbing occurs in the fingers and toes of some patients with heart or lung diseases. The tips of the fingers and toes become bulbous and swollen, giving them a 'club-like' appearance.)

An echocardiogram showed:

• a ventricular septal defect
• right ventricular hypertrophy
• significant subvalvular muscular obstruction (This meant that the passageway to the pulmonary valve was obstructed by a build-up of muscle.)
• a small pulmonary valve with a degree of supra-pulmonary valve narrowing (This means that the pulmonary artery was slightly narrowed above the valve.)

In addition, his main pulmonary artery and subsequent pulmonary artery branches were small and underdeveloped.

It was determined that Vinay was in urgent need of surgery.

On his November 12 clinic visit, a significant upper respiratory infection was noted. However, surgery was planned for November 26, with the provision that the infection had cleared by that date. Evidently the infection cleared and on November 26, Dr. Kim Duncan placed a Blalock-Taussig (B-T) shunt in Vinay's heart to assist his heart function. Then, when Vinay was 27 months of age, the first B-T shunt was replaced by a second, larger shunt. The first shunt had been originally scheduled for replacement in June 1992 but the operation had been cancelled to allow investigation of another condition - thrombocytopenia (or a decrease in the number of platelets in the blood).

On July 14, 1992, Dr. Rachel Yanofsky, a hematologist, determined that Vinay had compensatory polycythemia with secondary thrombocytopenia. This condition meant that Vinay had an increase in the number of red blood cells, probably because his heart condition made him cyanotic or lacking in oxygen, so his body produced more red cells to carry more oxygen. As a result of the increase in the number of red blood cells, Vinay appeared to have a reduced number of platelets, but this was because the platelets were actually diluted by the increased number of red blood cells. Yanofsky advised that the operation could go ahead and Vinay could be transfused with platelets if necessary. There were no bleeding problems during the second shunt operation on July 16, 1992.

The long-term medical plan was to undertake a more definitive repair when Vinay was older. As he grew, his increasing cyanosis and decreasing exercise tolerance indicated that a total correction was required. By the fall of 1993, Vinay was clearly cyanotic and clubbed. An October 27, 1993, catheterization showed a Tetralogy-type double outlet right ventricle, nearly complete pulmonary valve atresia with subpulmonary muscular obstruction, and a 14 millimetre non-restrictive subaortic VSD. (A subaortic VSD is a VSD that is found just below the aorta in the septal wall.)

In a November 9, 1993, letter to Dr. W. Robinson, the Goyals' family doctor, Giddins wrote, "There are no surprises at catheterization, and I believe his anatomy is suitable for definitive repair in the new year (a decision has been made regarding a surgeon who is to start in January)." (Exhibit 7, page GOY 585) At that point Giddins discussed the need for surgery with the Goyals.

Healthy heart

Diagram 6.3 Vinay Goyal - pre-operative heart 1 - Patent foramen ovale 2 - Nearly atretic (or small) pulmonary valve 3 - Right ventricular hypertrophy 4 - Blalock-Taussig shunts 5 - Ventricular septal defect (with overriding aorta) 6 - Right ventricular outflow tract obstruction (Subvalvular muscular obstruction)