The association of cardiac ventricular hypertrophy with congenital hyperinsulinism.

OBJECTIVE: Ventricular hypertrophy (VH) has been observed in children with congenital hyperinsulinism (CHI), a condition of hypoglycaemia characterised by dysregulated insulin secretion, but the prevalence is not known. PATIENTS AND METHODS: Cardiac assessment was performed in children (n=49) with CHI at diagnosis and follow-up. Two dimensional and Doppler echocardiography studies were used to assess cardiac structures, while M-mode study was used to measure left ventricular (LV) dimensions, subsequently converted to Z scores. Where possible, LV hypertrophy was confirmed by LV mass index (g/m(2.7)) >95th centile. RESULTS: Cardiac structural lesions were found in 14 (28%) children. At initial echocardiography, VH was present in 31 (65%) children with median (range) LV posterior wall dimension in diastole Z scores of +1.6 (-2.4 to +5.8) and interventricular septal wall dimension in end diastole Z scores of +1.9 (-1.7 to +17.2). At follow-up echocardiography, performed after an interval of 178 (45-390) days, VH persisted in 16 (33%) children. In regression analysis, the presence of VH (odds ratio (95% confidence intervals) 1.1 (1.0-1.2), P=0.04) at initial echocardiography was correlated with maximum glucose requirement at diagnosis, indicating that severity of CHI at presentation may play a role in the pathogenesis of VH. CONCLUSIONS: A significant proportion of children with CHI have cardiac structural lesions. A majority also have VH, which may be associated with the severity of CHI at diagnosis. VH may persist in some children, which requires careful long-term cardiac review.

Truncus arteriosus communis: early and midterm results of early primary repair.

BACKGROUND: Repair of truncus arteriosus communis (TAC) in the neonatal and early infant period has become standard practice in many centers. We report our experience on early primary repair of TAC, with a focus on early and midterm results. METHODS: From July 1993 to December 2005, 29 patients with median age 28 days (range, 11 to 127), and median body weight 3.1 kg (range, 2.6 to 5.9 kg), underwent primary repair of TAC. The anatomical type of TAC was as follows: A1-2, 27; A3, 0; and A4, 2. Right ventricular outflow tract was reconstructed with an aortic (n = 7) or pulmonary homograft (n = 8), or a bovine (n = 11) or porcine valved xenograft (n = 3). Follow-up was complete for all patients. RESULTS: Hospital mortality was 3.4% (1 death due to respiratory infection). At a mean follow-up of 74 months (range, 2 to 149), 1 patient died suddenly 2 months after surgery (6-year actuarial survival 93%). Of the 27 midterm survivors, 14 (52%) underwent 30 interventional procedures including percutaneous balloon dilation with or without stenting for right ventricular outflow tract or branch pulmonary artery obstruction. Eight of them were reoperated on for right ventricle-to-pulmonary artery conduit replacement (n = 8, 23%), and aortic valve regurgitation (n = 1, 3.4%). The overall freedom from any reintervention at 6 years was 50%. Aortic valve regurgitation was trace in 15 patients, mild in 8, moderate in 4. All midterm survivors but 1 (26 of 27) had good ventricular function. CONCLUSIONS: Truncus arteriosus communis repair can be performed early with very low perioperative mortality and satisfactory midterm morbidity; the latter is mainly attributed to right ventricular outflow tract reconstruction. Interventional cardiac catheterization delays inevitable conduit replacement.

Intra-aortic balloon pumping in children undergoing cardiac surgery: an update of the Liverpool experience.

OBJECTIVE: Intra-aortic balloon pumping in children remains a rarity. We report our experience in supporting pediatric cardiac surgical patients with intra-aortic balloon pumping. METHODS: We reviewed the cases of 24 children supported with intra-aortic balloon pumping after cardiac surgery in our institution from 1994 through 2003. RESULTS: Mean age at the time of the operation was 5.0 +/- 5.6 years (range, 7 days-17.5 years). Ten patients were infants less than 6 months old. Mean weight was 18.9 +/- 18.1 kg (range, 3.5-58.7 kg). Indications for intra-aortic balloon pump deployment were postoperative hemodynamic deterioration (n = 11, 8 survivors), failure to wean off cardiopu(n = 7, 5 survivors), and prophylaxis before weaning off cardiopulmonary bypass (n = 6, 5 survivors). The balloon was inserted through the ascending aorta in infants and through the femoral artery in children. Eighteen children (7 infants) were weaned off the intra-aortic balloon pump successfully (intra-aortic balloon pump survival, 75%). Mean duration of intra-aortic balloon pump support was 121.3 +/- 140.60 hours (range, 8-670 hours). There were 3 post-intra-aortic balloon pump in-hospital deaths (survival to hospital discharge, 62.5%). Severe intra-aortic balloon pump-related complications were mesenteric ischemia in 1 patient and lower limb ischemia requiring intra-aortic balloon pump removal in 1 patient. At a mean follow-up of 85 +/- 31 months (range, 18-124 months), all 15 long-term survivors were alive and well. CONCLUSIONS: Use of an intra-aortic balloon pump is an effective modality of cardiac support in properly selected pediatric cardiac surgical patients with refractory low cardiac output. It can be safely used in small infants and neonates. In selected cases with known left ventricular dysfunction, there is a place for prophylactic use of an intra-aortic balloon pump.

Review of an institutional experience of coronary arterial fistulas in childhood set in context of review of the literature.

Coronary artery fistulas are uncommon in children. We conducted a retrospective review of the case notes of 17 children who presented to our institution with the diagnosis of a coronary fistula since 1970. Their median age was 2.3 years. In five patients, there were associated congenital cardiac anomalies, with four having these as part of the spectrum of tetralogy of Fallot. Surgical correction was performed in nine patients, while in five closure was achieved using percutaneous embolization with coils. No complications or deaths were encountered related to the treatments chosen. In two patients, further procedures were needed to close residual fistulas. The median follow-up was 16 years, and on echocardiography, all fistulas appeared to be successfully closed, albeit that we lost three patients to follow up. We also conducted a review of 426 cases of coronary fistulas reported in children in the English literature. We have compared these findings with our institutional experience.

Anomalous origin of the left coronary artery from the pulmonary artery: results of surgical correction in five infants.

BACKGROUND: Five infants operated on for anomalous origin of the left coronary artery from the pulmonary artery were retrospectively analyzed. The mean age at operation was 12 +/- 6.7 weeks (95% confidence interval, 3.5 to 20 weeks), and mean weight at operation was 4.43 +/- 0.68 kg (95% confidence interval, 3.7 to 5.27 kg). All babies presented in infancy with left ventricular failure. Three had evidence of ischemia with left ventricular strain, and two had Q waves in anterolateral leads on electrocardiograph. Cross-sectional echocardiography showed dilated left ventricles with poor contractility in all babies with fractional shortening of 15.8% +/- 4.02% (95% confidence interval, 12% to 20%); moderate mitral regurgitation was seen in all babies. METHODS: All babies underwent operation as soon as the diagnosis was made. Four babies had direct reimplantation of left coronary artery into the aorta, and 1 had tunnel repair. Intraaortic balloon counterpulsation was used in 1 baby for hemodynamic instability and as prophylaxis in the remaining 4 babies postoperatively for 115 +/- 26.2 hours (95% confidence interval, 72 to 144 hours). RESULTS: All babies had delayed closure of the chest. There was no operative mortality. One baby was reoperated on for tunnel stenosis as well as pulmonary stenosis 4 months after primary repair. All babies were followed for 192 patient-months and show an improved fractional shortening. CONCLUSIONS: Early operation, early institution of intraaortic balloon counter pulsation for left ventricular support, and delayed sternal closure are the key to good results.

Nutritional support via percutaneous endoscopic gastrostomy in children with cardiac disease experiencing difficulties with feeding.

Adequate nutrition is crucial to the management of children and infants with cardiac disease. Difficulties with feeding are extremely common, and maintaining an adequate caloric intake, in order to achieve sustained growth, is often not possible without nutritional support. We retrospectively reviewed our experience between 1995 and 1999 in treating 37 children with cardiac disease who underwent percutaneous endoscopic construction of a gastrostomy to augment nutritional needs. We stratified the patients into those with cyanotic heart disease, when saturations of oxygen were less than 95%; those with non-cyanotic heart disease with saturations greater than 95%, and those with minor cardiac disease associated with a systemic disorder. Each group was compared to control children matched for age, sex, and diagnosis. We evaluated, the variation in standard deviation score for body weight over a median period of follow-up of 295 days. Improvements in the standard deviation score for body weight occurred in each of the groups, whereas children in the control groups demonstrated a decrease in standard deviation score for body weight. The median change of the score for body weight was significantly higher in patients managed with gastrostomy compared to controls. We conclude that supplementation using a gastrostomy tube allows the safe delivery of the caloric intake needed to support malnourished children with cardiac disease.