Dr. Sushant Srivastava
Director & Sr. Consultant
CTVS, BLK Heart Centre
BLK Super Speciality
Hospital, New Delhi
A Mighty Heart
A successful major surgery to save an infant with
Tetralogy of Fallot (TOF)
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.
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.
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 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.
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.