Rapid two-stage Starnes procedure for a symptomatic neonate with Ebstein anomaly.

We present a rapid two-stage Starnes procedure for a seriously symptomatic neonate with the prenatal diagnosis of Ebstein anomaly. At 16 hours after birth, we performed an emergency operation consisting of main pulmonary artery ligation, plication of the right atrial and right ventricular wall, modified Blalock-Taussig shunt, and patent ductus arteriosus ligation, without cardiopulmonary bypass. At age 12 days, we then performed the Starnes procedure using a glutaraldehyde-treated autologous pericardial patch with a 4-mm fenestration to close the tricuspid valve orifice. The infant's postoperative course was excellent. A rapid two-stage Starnes procedure is useful for treating a seriously symptomatic neonate with Ebstein anomaly.

Imaging and clinical characteristics of children with multiple foci of microsusceptibility changes in the brain on susceptibility-weighted MRI.

BACKGROUND: Microsusceptibility changes in the brain are well known to correspond with microbleeds or micrometal fragments in adults, but this phenomenon has not been explored well in children. OBJECTIVE: To assess imaging and clinical characteristics of children with multiple foci of microsusceptibility changes using susceptibility-weighted imaging (SWI). MATERIALS AND METHODS: Between 2006 and 2008, 12 children with multiple foci of microsusceptibility on SWI without corresponding abnormal signal on conventional MRI were identified and were retrospectively assessed. RESULTS: The locations of foci of microsusceptibility included the cerebral white matter, basal ganglia, brainstem and cerebellar white matter, without any clear systematic anatomic distribution. CT (n=5) showed no calcification at the locations corresponding to the microsusceptibility on SWI. Conventional MR imaging showed white matter volume loss (n=5), delayed myelination (n=2), acute infarction (n=1), chronic infarction (n=1), meningitis (n=1), slight signal abnormality in the white matter (n=1) and no abnormal findings (n=1). Follow-up SWI (n=3) showed no change of the microsusceptibility foci. Interestingly, all children had a history of heart surgery under extracorporeal circulation for congenital heart disease. CONCLUSION: Multiple foci of microsusceptibility can be seen in the brain on SWI in children with congenital heart disease who underwent heart surgery with extracorporeal circulation.

Impact of 3-mm Blalock-Taussig shunt in neonates and infants with a functionally single ventricle.

Functionally single ventricle (f-SV) is susceptible to volume overload. Atrioventricular valve regurgitation (AVVR) tends to develop and ventricular function deteriorates due to excessive pulmonary blood flow following modified Blalock-Taussig shunt (mBTS). On the other hand, a small caliber graft has risks of early obstruction and poor growth of pulmonary vascular beds. We assessed the effect of mBTS with a 3-mm graft to circumvent volume overload in f-SV on achievement of the right heart bypass. Eleven neonates and infants with f-SV at the median age of 24 days underwent mBTS using a 3-mm graft between August 2004 and June 2007. There were no early deaths, but there was one late death. All survivors achieved bidirectional cavopulmonary shunt (BCPS) at 4.2 months after mBTS. Cardiac catheterization demonstrated sufficient growth of the pulmonary artery (pulmonary artery index, 268+/-98 cm(2)/m(2)), low pulmonary vascular resistance (1.4+/-0.9 U.m(2)). The AVVR remained mild or less. Ventricular end-diastolic volume and ejection fraction were 171+/-61% of the normal value and 64+/-6%, respectively. We conclude that a 3-mm mBTS was useful in preventing f-SV from volume overload and was effective for growing good pulmonary vasculature and achieving a right heart bypass.

Evaluation of blood rheology in patients with cyanotic congenital heart disease using a microchannel array flow analyzer.

Blood hyperviscosity due to secondary erythrocytosis is a common pathologic feature of cyanotic congenital heart disease (CCHD). In CCHD, it is possible that hematological parameters other than red blood cells influence blood rheology. We measured blood passage time to evaluate the blood rheology in patients with CCHD (n=18, age: 15.3+/-11.9 years, mean +/- SD) and age-matched control subjects (n=27) using the microchannel array flow analyzer (MC-FAN), and the results [several hematological parameters, including hematocrit (Hct)] were compared. Blood passage time in the CCHD group was prolonged, compared with the control group (67.6+/-27.2 s vs. 44.6+/-6.7 s). For the CCHD group, blood passage time correlated significantly with red blood cell (RBC) count, hemoglobin (Hb) concentration, Hct, mean corpuscular hemoglobin concentration (MCHC), platelet (Plt) count, high-density lipoprotein cholesterol (HDL-C) level, and triglycerides (TG) level (RBC, r=0.77; Hb, r=0.69; Hct, r=0.73; MCHC, r=-0.64; Plt, r=-0.49; TG, r=0.53; HDL-C, r=-0.49, p<0.05 for each variable). For all 45 subjects, blood passage time correlated significantly with HbA1c level (r=0.45, p<0.01) and tissue-type plasminogen activator (t-PA) antigen level (r=0.46, p<0.01). Our results indicated that blood rheology is reduced in patients with CCHD as expressed by prolonged blood passage time, and it may be defined by several blood parameters in addition to erythrocytosis.