Complex Cardiac Anomalies - Pediatric Oncall

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Rakesh Kumar, Binay Ranjan, Manjul Vijay Department of Pediatrics, Katihar medical college, Katihar...

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	Specialist Answers
Question
My sister is 28 yr old , now 27 weeks pregnant with fetus having complex cardiac anomalies (endocardial cushion defect , VSD ,PS,double outlet right ventricle).she had past history of abortion twice ,first was medically induced , second was spontaneous both in later half of 2003. No other significant family or past history. Kindly guide us regarding management of this at the earliest. yours sincerely, Manish.
Answer
Endocardial cusion defect with complex congenital heart disease ( as mentioned - conotruncal anomalies - DORV+ VSD+ +PS) are usually associated in 60-70% of cases with trisomy 21 and other chromosomal anomalies like di george . Also incidence of non cardiac associations are high -like dandy walker cyst, neural tube defects, omphalocoele etc.Hence amniocentesis for karyotyping and congenital malformation scan are also essential. As the mother is already into 27 weeks of her pregnancy it may be little difficult/ dangerous to attempt MTP at this stage.However they could consult their obstetrician for the same .If they wish to continue pregnancy periodic follow up would be required - as complete heart block and hydrops fetalis are common with these anomalies . Institutional delivery and a complete 2 D Echo evaluation of the newborn child would be required. As regards to outcome after the child is born depends if the cardiac condition is isolated or associated with chromosomal anomalies. If isolated - ECD with DORV+PS+VSD can be operated at 6-9 months of age at good centers ( Chennai, Kochi) with fairly good outcome. Most essential of all for future is - parents karyotype( before Next pregnancy) / fetus karyotype( if abortion opted) / childs karyotype( if pregnancy continued) be done with referral to geneticist for counselling and peripartum folic acid supplementation for the future. Back to All Questions

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