Hajira Akbar, Waqar Ahmed, Maadullha.
A 14 days old neonate with intracardiac mass.
Pak J Cardiol Jan ;16(2):100-2.

Amjad was a 14 days old neonate, presented with progressive bluish coloration. He initially started on breast feeding but later on was noticed by mother to stop frequently during feeds, cry excessively and developed bluish disceleration of mucosa and lips. On examination, the baby was blue, cold and dehydrated. He had pulse of 150/min, respiratory rate of 60/ min, blood pressure of 80/50 mm of Hg, oxygen saturation of 78% and afebrile. The neonate had a severe perianal rash extending into groins. On cardiovascular examination, apex beat was in left 4th intercostal space lateral to midclavicular line. He had a 3/6 pansystolic murmur at lower left sternal edge with normal heart sound. An S3 gallop was noticed as well as prominent neck veins were seen and liver was tender on palpation. The relevant investigations were carried out revealing Hb of 21g/dl. The 12 lead ECG revealed heart rate of 140/min, PR interval of 80 msec, RSR` in V1 QT interval of 0.2 sec. On chest X-ray cardiomegaly was noticed along with plethoric lung fields. Two dimensional echocardiogram revealed situ solitus, levocardia, AV and VA concordance, systemic venous return to LA, pulmonary venous return to RA, and huge mass was noticed in right atrium extending to right ventricle causing obstruction to pulmonary venous return. On the basis of history, examination and investigations a diagnosis of cardiac tumor was made and diagnostic procedure was preformed. The child was put on intravenous antibiotics, cefitraxone and vitamin K. A big growth from right ventricle was removed adherent to RV cavity and tricuspid valve. It was extending to right atrium. Mass excision was done resulting in a small RV cavity and small pulmonary arteries were seen. The tricuspid valve was dilated. After the tumor resection, RVOT reconstruction was done and RA was closed. Multiple attempts to wean off from bypass were not successful as the right ventricle cavity was not able to cope with systemic venous return and the child expired. The specimen was sent for histopathology which revealed a right sided rhabdomyoma.

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