pulmonary atresia with vsd

[vettu]

hello,
my sister is 21 weeks pregnant. her fetus is diagnosed with double outlet right ventricle, pulmonary atresia and large anterior malaligned vsd.she was told that the baby has to undergo surgeries one in  first month and first year and they are not sure about prognosis and is upto them to continue pregnancy. we r really confused like to continue pregnancy or not? if yes then whats the prognosis of surgeries?  we were also told that it is a very condition.please advice. thanku

[Marie M Gleason, M.D.]

Double outlet right ventricle with pulmonary valve atresia is a very serious congenital heart condition.  The cardiac prognosis depends upon whether or not the child has adequate size pulmonary artery branches, and whether there are 2 good sized ventricles (pumping chambers) and inflow valves.  If there are 2 good pumping chambers and a malalignment ventricular septal defect, then there is a chance to do a 2 ventricle Rastelli repair. We know these patients usually survive to adulthood in most cases. This would be opposed to staging towards a Fontan operation, when one of the pumping chambers or inlet heart valves is smaller.  Either type of approach generally involves multiple surgeries over a lifetime. The long term prognosis for Fontan patients is still evolving, but there is a high likelihood of dying at a younger age than someone with 2 pumping chambers.  That being said, either heart condition has surgical options in most cases.  The decision to pursue that is up to the family.  One should also take into consideration that some children with complex heart problems are genetically abnormal with chromosome anomalies.  Some mother's choose to get an amniocentesis to get that information before deciding to terminate or not.  Either way, decision making for surgery cannot be done completely until after the child is born and the anatomy is clearly delineated. Good luck.

[vettu]

thank u doc. ur reply was helpful.