Challenges in Managing Ivemark's Syndrome With Complete Congenital Heart Disease and Asplenia: A Rare Case Report From Developing Country

Ivemark's syndrome or right-isomerism sequence is a rare condition in newborn, manifests with asplenia, complex congenital heart disease, and situs ambiguous. Diagnosis and managing this syndrome remain challenging, especially in developing countries. The mainstay of management involves surgical correction of cardiac abnormalities and infection prevention. We present a case of a newborn who exhibited cyanosis since birth and developed persistent respiratory distress despite intubation. The mother had not undergone fetal echocardiography or other prenatal screening. Upon physical examination, he looked respiratory distress with SpO2 ranging from 29-70. A systolic murmur was heard during cardiac examination, with no abnormalities observed in the face, abdomen, and extremities. Plain radiographs revealed situs inversus, right-located stomach and midline-located liver. This finding was supported by abdominal ultrasound and MSCT, which showed absence of the spleen and a right-sided pancreas. Echocardiography and cardiac catheterization identified situs ambiguous, along with complex cardiac anomalies. These findings confirmed the diagnosis of Ivemark's syndrome. Plans were made to transfer the infant to national cardiovascular center for cardiac surgery. Unfortunately, the infant succumbed to infection at three months of age. This case highlights the significant challenges in managing rare diseases, particularly Ivemark's syndrome in developing countries. Limited access to advanced diagnostic tools and specialized cardiac surgical expertise complicate timely diagnosis and intervention. The potential causes of mortality associated with complex cardiac malformation, the increased risk of severe infections due to asplenia, or a combination of both factors.