Dr. Sushant Srivastava
Director & Sr. Consultant
CTVS, BLK Heart Centre
BLK Super Speciality
Hospital, New Delhi

Tetralogy of Fallot (TOF) is a complex cyanotic birth defect of the heart with four components, namely, a large Ventricular Septal Defect (VSD) Infundibular and Valvular Pulmonary Stenosis, aorta overriding the VSD to a variable degree and Right Ventricular Hypertrophy. The patients, invariably infants, experience breathlessness, failure to thrive and blue discolouration of nails, lips and tongue. Some infants or children develop hypoxic spells which are potentially fatal.

THE CASE

A 3-year old Iraqi boy was admitted in the BLK Heart Centre with complaints of breathlessness, blue discolouration of the lips and nails. He had a medical history suggestive of hypoxic spells. An Echocardiogram was done and a diagnosis of TOF was established. However, the left and specially the right pulmonary arteries were small and also had stenotic segments at their origin from the main pulmonary artery. A CT Angiogram was done to assess pulmonary artery size and the diagnosis of origin stenosis confirmed small sized pulmonary arteries. This presented a dilemma with regard to choice of treatment between shunt and total correction. Inadequate pulmonary arteries can lead to right heart failure and even failure to wean from cardio pulmonary bypass after total repair. From careful evaluation of the CT Angiogram, it appeared that stenotic segments and small pulmonary arteries could be successfully enlarged.

THE PROCEDURE

Risks and benefits were discussed with parents and a decision to undertake open heat repair was made. The heart was approached through a median sternotomy. A pericardial patch was harvested. The patient was placed on heart lung machine. The right heart was entered through the right atrium and infundibular muscle was resected to enlarge the right ventricular outflow. The incision was carried into the main pulmonary artery and branch pulmonary arteries up to the hila. The VSD was closed with a dacron patch. To enlarge the right and left pulmonary arteries, a pericardial patch was stitched to the left and right pulmonary arteries, on the right side it was carried as far as possible behind and across the aorta.

THE RESULT

The child recovered well from surgery and was discharged on the seventh post operative day. Cyanosis disappeared completely, his lips and nails became healthy pink in colour. This was a technically difficult case, but the well planned operation led to a successful one-stage repair.

DISCUSSION

Pre-operative decision making in surgery for complex congenital heart disease is of paramount importance. As near as complete correction of anatomical defects is necessary to restore optimal physiology.