No abstract

There is a severe decrease of CD4+ lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4+ lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the Fontan procedure.

In our opinion, Cook detachable coils are safe and effective especially in the treatment of persistent ductus arteriosus with a diameter < or = 2.5 mm. Due to the low costs these coils appear to be superior to other devices in this subgroup of patients.

Objective-To describe the morphology of the pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries with and without monosomy 22q11. Design-A retrospective analysis of all patients with this congenital heart defect who are being followed at the University Children's Hospital Erlangen. Setting-A tertiary referral centre for paediatric cardiology and paediatric cardiac surgery. Patients-21 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Monosomy 22q11 was diagnosed by fluorescent in situ hybridisation using the D22S75 probe (Oncor). The morphology of the pulmonary arteries was assessed on the basis of selective angiograms. Results-10 patients (48%) were shown to have a microdeletion in 22q11 (group I). There was no diVerence with respect to the presence of confluent central pulmonary arteries between these patients (80%) and the remaining 11 patients (group II) without monosomy 22q11 (91%). Patients of group I, however, more often had arborisation anomalies of the pulmonary vascular bed (90% in group I v 27% in group II). Because of the more severe abnormalities of the pulmonary arteries, a biventricular repair had not been possible in any of the children with monosomy 22q11, though repair had been carried out in 64% of the children in group II. Conclusions-The developmental disturbance caused by monosomy 22q11 seems to impair the connection of the peripheral pulmonary artery segments to the central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, resulting in a lower probability of biventricular repair. (Heart 1998;79:180-185 In the majority of cases with tetralogy of Fallot and pulmonary atresia, collateral lung perfusion is provided either by a unilateral or bilateral arterial duct or by major aortopulmonary collateral arteries.9 10 According to the findings of Momma et al the presence of major aortopulmonary collateral arteries is associated in a high percentage with monosomy 22q11.2 Among their cohort of 49 patients with pulmonary atresia and ventricular septal defect, monosomy 22q11 was present in 21 of 34 children with collateral lung perfusion by major aortopulmonary collateral arteries.2 The purpose of our study was to determine additional anomalies of the pulmonary vascular bed and to define their impact on the treatment in the subgroup of patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. MethodsAll 21 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries who are at present being followed at our institution were included in this retrospective study. These patients were not strictly consecutive, since several children who had been diagnosed during recent years had been lost to follow up or had died.Metaphase chromosomes of peripheral blood lymphocytes were analysed after GTG banding and b...