Key words asplenia, cytomegalovirus pneumonia, neonate, pediatric cardiac surgery.Cytomegalovirus (CMV) infection, including pneumonia and viral syndrome, is the most prevalent and serious opportunistic complication that occurs following heart and lung transplantation. CMV disease following pediatric cardiac surgery in the non-transplant setting, however, is very rare. 1 Here we report a case of CMV pneumonia following total anomalous pulmonary venous connection (TAPVC) repair in a neonate with heterotaxy syndrome.
Case reportA 39-year-old woman was referred to the Perinatal Medical Center because of suspected fetal congenital heart disease observed on fetal echocardiography at 32 weeks' gestation. The pregnancy was uneventful, and virological screening for cytomegalovirus (CMV) was negative. The baby was delivered by spontaneous vaginal delivery at 39 weeks' gestation.The male infant was born with a birthweight of 2456 g, and Apgar scores were 8 at both 1 and 5 min after birth. Echocardiography showed a single ventricle; common atrioventricular valve, with moderate regurgitation; mixed-type TAPVC (Ib+III), with a peak velocity of 1.9 m/s into the vertical vein; and asplenia. Enteral feeding with breast milk was initiated at day of life (DOL) 5. Because respiratory distress had developed with tachycardia since DOL 7, primary sutureless repair for TAPVC, partial annuloplasty of the common atrioventricular valve, and banding of the main pulmonary artery were performed at DOL 11. He was admitted to the intensive care unit with his chest opened, and delayed sternal closure was performed on postoperative day (POD) 5. During this period including surgery, continuous catecholamine infusion, multiple blood transfusions (red blood cell concentrates, 438 mL; fresh frozen plasma, 1578 mL; platelet concentrates, 172 mL), and i.v. steroid (methylprednisolone, 10-40 mg/day) were required to treat hemodynamic instability due to bleeding tendency and excessive capillary leakage. Postoperative echocardiography showed adequate orifice size of the pulmonary veins with peak velocity 0.6-0.8 m/s, and mild atrioventricular valve regurgitation. After that, the circulatory and respiratory states seemed to stabilize with heart rate 150-160 beats/min, blood pressure 70-80 mmHg, and central venous pressure 10-11 mmHg under i.v. dopamine (8 lg/kg/ min), adrenaline (0.03 lg/kg/min), and milrinone (0.5 lg/kg/ min). There were no signs or symptoms of infection such as mediastinitis, pneumonia, or sepsis, but the infant suddenly developed bradycardia and hypotension without any warning on POD 8, requiring emergency cardiopulmonary resuscitation and veno-arterial extracorporeal membrane oxygenation (ECMO) induction. Laboratory results at ECMO initiation were as follows: white blood cells, 5200/lL; and C-reactive protein, 0.02 mg/dL. Bacteriological cultures of blood, sputum, and urine were all negative. Chest radiograph following ECMO establishment showed gradually enlarging pulmonary infiltrates and consolidation of the right lung and left uppe...