Hisashi Nikaidoh

Myocardial inflammatory activation in children with congenital heart disease

Objective In several cardiac-related diseases, there is a strong association between systemic endotoxemia, myocardial cytokine production, and cardiac failure. Because pre- and postoperative endotoxemia recently was reported in children with congenital heart disease, we sought direct evidence of myocardial inflammatory activation in a cohort of children undergoing congenital heart surgery on cardiopulmonary bypass. Inflammatory activation was prospectively defined as the presence of nuclear factor-&kgr;B nuclear translocation in myocardial tissue samples. Design Prospective observational study. Setting Tertiary care pediatric intensive care unit. Patients Fifteen children with congenital heart disease undergoing operative repair on cardiopulmonary bypass. Interventions All patients underwent operative repair of congenital heart disease on cardiopulmonary bypass and had plasma samples obtained for endotoxin and tumor necrosis factor-&agr;, both pre- and postoperatively. Myocardial tissue samples were obtained intraoperatively, both before and during cardiopulmonary bypass. Measurements and Main Results Elevated plasma endotoxin concentrations were documented in all 15 patients during the study period. In 12 patients, plasma endotoxin was elevated before cardiopulmonary bypass. The median preoperative tumor necrosis factor-&agr; concentration was 16.4 pg/mL, which is higher than concentrations reported in adults with New York Heart Association class III congestive heart failure. Examination of myocardial tissue samples revealed nuclear factor-&kgr;B nuclear translocation (predominantly p50/p65 heterodimers) in nine of 15 patients (60%). Four of these nine patients had nuclear factor-&kgr;B nuclear translocation before initiation of cardiopulmonary bypass, with p50/p50 homodimers present in two of the four. Conclusions These data provide the first evidence of nuclear factor-&kgr;B activation in children with congenital heart disease and the first evidence of myocardial nuclear factor-&kgr;B translocation in human hearts before explant for transplantation. Furthermore, these data suggest that, similar to adults with advanced congestive heart failure, the myocardial inflammatory cascade may contribute to the pathophysiology of congenital heart disease in infants and children.

Two-dimensional and Doppler echocardiography alone can adequately define preoperative anatomy and hemodynamic status before repair of complete atrioventricular septal defect in infants <1 year old

OBJECTIVES We sought to assess the ability of two-dimensional and Doppler echocardiography alone, without cardiac catheterization, to evaluate infants < 1 year of age for complete open heart repair of complete balanced atrioventricular (AV) septal defect. BACKGROUND Two-dimensional echocardiographic-Doppler examinations provide accurate anatomic detail in patients with AV septal defect. Lung biopsy data have shown that patients rarely develop significant inoperable pulmonary vascular disease before 7 months of age. Although calculated pulmonary arteriolar resistance is often elevated in young infants with this heart defect, this elevation rarely reflects significant pulmonary vascular changes in infants < 7 to 12 months of age. METHODS We performed a retrospective review of 34 patients who underwent complete repair of AV septal defect at our institution between January 1, 1988 and September 1, 1992. Some patients had both catheterization and echocardiographic-Doppler studies (group I, n = 16); others had only echocardiographic-Doppler studies (group II, n = 18). RESULTS The groups were comparable with regard to age at echocardiography and operation, days in the hospital, days with ventilatory and inotropic support and occurrence of postoperative pulmonary hypertension. One child (2.9%) died during the early postoperative period, and one child in each group (5.8%) died within the 1st year of life. Preoperative echocardiography allowed better detailing of anatomy, valve commitment and regurgitation than was possible with catheterization alone. Knowledge of preoperative pulmonary resistance did not alter the surgical decision or predict postoperative pulmonary hypertension. There was no apparent difference in mortality between the two groups (0 vs. 5.5%), but the small number of patients in each group provides for a very low power (beta = 0.04) calculation. This mortality rate is not different from that reported in recent studies. CONCLUSIONS Patients with AV septal defect can safely undergo surgical correction of this defect on the basis of echocardiographic-Doppler data alone.

Thrombolytic treatment with streptokinase for late intraatrial thrombosis after modified fontan procedure

Abstract Although the role of the Fontantype operation in providing high grade palliation for patients with tricuspid atresia and ventriculoarterial concordance has been clearly established, complications have been reported.1–3 In this report, we describe a patient who developed late intracardiac thrombosis occluding the anastomosis between the right atrium and pulmonary artery who was successfully treated with intravenous streptokinase.

Major intrahepatic venovenous fistulas after a modified Fontan operation

After the Fontan operation, systemic venous hypertension drives transpulmonary blood flow. Owing to this physiology, there is a significant incidence of effusions, splanchnic congestion, and generalized edema. To attentuate the effusive problems, partial exclusion of the hepatic veins has been recently practiced by Jacobs and Norwood. This report concerns a patient we recently treated for profound progressive cyanosis due to an acquired intrahepatic venovenous shunt after a Fontan operation with partial hepatic vein exclusion.

Echocardiographic Diagnosis of Anomalous Drainage of the Superior Vena Cava Into the Left Atrium

A 1-day-old, 2805 g baby girl who was carried to term was cyanotic shortly after birth. Pulse oximetry documented an oxygen saturation of 65%. The saturation improved to 80% with hyperoxic challenge. Physical examination revealed a grade II/VI long systolic murmur best heard at the base of the heart, and a mid-diastolic rumble at the left lower sternal border. The ECG exhibited left ventricular hypertrophy. Chest roentgenogram demonstrated cardiomegaly with …

Usefulness of external counterpulsation early postoperatively after the Fontan procedure in children

temic venous return, and reduces ventricular afterload. 1‐3 We hypothesized that EC could benefit patients who underwent the Fontan procedure by improving cardiac output in the early postoperative period. EC had not been studied in the postoperative setting, and had never previously performed in the pediatric population. Therefore, this investigation sought to provide preliminary evidence of EC safety and short-term efficacy in children after Fontan surgery. ••• We performed a prospective, within-patient, controlled investigation of EC in children who underwent the Fontan procedure (fenestrated and nonfenestrated) at Children’ s Medical Center, Dallas, from May 1999 to June 2001. Children were considered for participation in the study if they were consecutive pediatric patients for whom parental consent was obtained, if there was an EC machine available, and if EC cuff sizes were deemed appropriate (pediatric-sized cuff straps able to be tightened over a sheepskin barrier to prevent excessive movement and skin irritation during EC). Patients were excluded from study for the following reasons: significant intraoperative surgical complications that may have compromised postoperative neurologic status (defined as 10 minutes of hypotension off bypass with blood pressure less than the fifth percentile for age), extubation before or immediately upon arrival to the cardiac intensive care unit, presence of a cardiac arrhythmia or paced heart rhythm, invasive line(s) in the femoral or lower extremities, peripheral vascular disease, musculoskeletal anomaly involving the lower extremities, significant and prolonged postoperative hemorrhage (3 ml/kg/ hour persisting for 10 hours after arrival to the cardiac intensive care unit), or aortic insuf ficiency. After admission to the cardiac intensive care unit, each patient was assessed for clinical stability. Blood products and/or volume (5% albumin) were administered and inotropes were adjusted at the discretion of the attending physician based on hemodynamic status, chest tube drainage, hemoglobin level, and degree of anticoagulation. Intravenous fluid was administered before the investigation if systemic perfusion was determined to be clinically poor, or if systolic blood pressure decreased below the fifth percentile for age at any time. The study protocol was initiated while patients were sedated and anesthetized with midazolam and fentanyl, before extubation, and after mediastinal blood loss was controlled (3 ml/kg/hour). Inotropes and vasoactive medications were unchanged during the period of investigation. The study protocol was divided into 3 10-minute periods. During the first period, baseline parameters were measured before initiation of EC. This was immediately followed by a 10-minute EC period, and directly thereafter, a postintervention period. The hemodynamic parameters obtained during all 3 periods included cardiac index (CI) by ascending aortic pulse Doppler echocardiography (averaged over 8 cardiac cycles), 4 vital signs (heart rate, blood pressure), and central venous pressure (CVP). Each parameter was measured every minute during the 3 study intervals, except ascending aortic Doppler flow, which was evaluated every 2 minutes. Echocardiographic determinations of CI were made off-line by a single interpreter who was blinded to the period of testing. Blinded interpretation was feasible because EC does not alter ascending aortic Doppler flow patterns.5 Chest tube output and oxygen saturation were recorded throughout the investigation. EC was performed using the Cardiomedics Cardi