av canal defect of my son

My 18 yr old son is having partial av canal defect.Disease was diagnosed at one year age, and doctors advised for surgery.But due to poor physical growth I could"nt take decision. He is 24kg &133 cms at 18 yr age.His recent echo evaluation is done in Escorts Heart Institute New Delhi, and according to doctors, lungs pressure  is still normal, and heart is still operable.Further I want to add that my elder son was also having the same partial av canal defect, and was operated in All India Medical Institute & Research Center, New Delhi in 1990 at the age of 3year.He could"nt survive.Post operation in ICU, he expired.Thats why I am hesitating for surgery.Kindly guide me.

[Jeffrey R Boris, M.D.]

Dear Vikram,

A partial atrioventricular canal is a type of a congenital cardiac defect between the upper two chambers of the heart.  Without evaluating your son more fully, including history and examination as well as his echocardiogram, I cannot advise you on what to do.  I am extremely concerned about his small size, and wonder whether he may have an abnormal genetic syndrome.  At this point, the first thing that I would do is to find out why he is so small.  Then, what needs to be determined is if the reason for his small size could potentially influence his ability to tolerate surgery.  I do not know anything about the specific cardiac center to which you refer, so I am not able to say whether your first son died because of his medical condition or because of the medical care he received.  

The only other thing that I can say that may help with the decision-making process is that if the defect is small AND if the atrioventricular valves are normal and are not leaking significantly, it may not need to be closed and can be observed.  Otherwise, moderate-sized and large defects have been demonstrated to shorten lifespan and increase the risk for exercise intolerance, arrhythmias, pulmonary hypertension (high blood pressure across the lungs), and strokes.