Minimizing circulatory arrest by using antegrade cerebral perfusion for aortic arch reconstruction in infants causes fewer postoperative adverse events.

BACKGROUND: Because deep hypothermic circulatory arrest (DHCA) carries a risk for neurological damage, antegrade cerebral perfusion (ACP) is used increasingly for aortic arch surgery in infants. We assessed the short-term effects of minimal DHCA (< 30 minutes) versus prolonged DHCA (> 30 minutes) during biventricular aortic arch reconstruction. METHODS: Twenty-six infants (< 3 months of age) who had undergone aortic arch reconstruction were retrospectively analyzed: 15 infants without DHCA (group A) and 11 infants with DHCA (group B). Group B was further divided into < 30 minutes DHCA (group B1, n = 6), and >or= 30 minutes DHCA (group B2, n = 5). Additionally, minimal DHCA (group A + B1) was compared to prolonged DHCA (group B2). RESULTS: In the minimal DHCA group (A + B1), 29 % of the patients had a postoperative adverse event, compared to 80 % in the prolonged DHCA group (B2) ( P < 0.05). Average length of stay in hospital was 25 days shorter for the minimal DHCA group (15 days versus 40 days, P < 0.01). CONCLUSION: Minimal DHCA results in fewer adverse events and a reduced length of stay, compared with prolonged DHCA. Therefore, during aortic arch surgery in infants, DHCA should be minimized by using antegrade cerebral perfusion.

Successful percutaneous balloon valvuloplasty in a preterm infant weighing 1500 g with critical pulmonary valve stenosis.

We describe a successful cardiac intervention in an infant, born at 32 weeks of gestation, with a birth weight of 1040 g, who had a critical pulmonary valve stenosis with the right ventricular pressure twice the systemic pressure. Continuous prostaglandin E infusion kept the arterial duct open and at the age of four weeks and a weight of 1500 g a balloon valvuloplasty was performed, which reduced the systolic right ventricular pressure to below the systemic pressure. Two weeks later the procedure was repeated because of increasing right ventricular pressure. At two months of age the mean systolic transpulmonary Doppler gradient was 30 mmHg with an adequate right ventricular volume. The neurological status of the infant was normal and the femoral vein was patent. Timely interventional heart catheterisation is a successful method of treatment in critical pulmonary valve stenosis in infants with a low birth weight. (Neth Heart J 2008;16:264-6.).

Pentalogy of Cantrell : successful early correction.

The pentalogy of Cantrell is a rare midline developmental field complex often with a dismal outcome. We present a newborn qualifying for Cantrell's pentalogy with an abdominal wall defect, a diaphragmatic defect and a heart defect, a ventricular septal defect (VSD) and a left ventricular diverticulum. Early operative correction following accurate diagnostic work-up was prompted at the age of 11 weeks because of progressive heart failure. Beside correction of the VSD and the abdominal and diaphragmatic defects, resection of the distal part of the diverticulum was possible without damage to the LAD and preserving good contractility. Two years postoperative follow-up is uneventful.

Exercise prescription for patients with a Fontan circulation: current evidence and future directions.

It is well documented that children with a Fontan circulation have a reduced exercise capacity. One of the modalities to improve exercise capacity might be exercise training. We performed a systematic literature review on the effects of exercise training in patients with a Fontan circulation. Six published studies were included that reported on the effects of exercise training in 40 patients. All studies had a small sample size and/or did not include a control group.Based on the six published studies we can conclude that children who have undergone a Fontan operation and who are in a stable haemodynamic condition can safely participate in an exercise training programme and that exercise training results in an improved exercise capacity. However, more research is needed to establish the optimal exercise mode, dose-response relation, and the effects of exercise training on cardiac function, peripheral muscle function, physical activity, and health-related quality of life. (Neth Heart J 2007;15:142-7.).

Aortico-right ventricular tunnel: prenatal diagnosis leading to neonatal survival.

In the 36th week of gestation a large aortico-right ventricular tunnel with an otherwise structurally normal heart was diagnosed by fetal echocardiography. This report describes for the first time the impact of the timely prenatal diagnosis of an aortico-right ventricular tunnel followed by successful management in early infancy.

Diagnostic utility of the pre-incubated acidified glycerol lysis test in haemolytic and non-haemolytic anaemias.

The usefulness of the pre-incubated acidified glycerol lysis test (AGLT), a laboratory test for spherocytosis, has been investigated in a selected hospital population of 348 patients with haemolytic and non-haemolytic anaemia. The AGLT was positive in 58 out of 59 patients with hereditary spherocytosis. In all 32 patients with other types of hereditary haemolytic anaemia the AGLT was normal or equivocal, but clearly different from spherocytosis. Adults with a positive AGLT, but without hereditary spherocytosis, had auto-immune haemolytic anaemia, myelodysplastic syndrome or were pregnant women. In newborn infants the AGLT was positive, in the first week of life, in those babies having hereditary spherocytosis or immune haemolysis due to blood group incompatibility; no positive AGLT results were seen if no haematological explanation for neonatal hyperbilirubinaemia could be found. At the optimal cut-off point the sensitivity of the AGLT for hereditary spherocytosis was 98.3% and the specificity 91.1%, under the most unfavourable conditions. The AGLT is a very useful and simple test for the diagnosis of hereditary spherocytosis.

Rubinstein-Taybi syndrome in a mother and son.

The Rubinstein-Taybi syndrome is a condition characterized by mental retardation, typical facial changes and broad thumbs and big toes. The cause is unknown; almost all cases are sporadic. We describe a mother and son with Rubinstein-Taybi syndrome. Literature search documented at least 413 cases with 558 sibs. An affected offspring has occurred at least twice, possibly six times. In 45 yet undescribed Dutch cases no recurrence was found in 94 sibs. A submicroscopic chromosome deletion seems the most probable explanation, but other alternatives may be possible.

Histopathology of the arterial duct (ductus arteriosus) with and without treatment with prostaglandin E1.

In the light of conflicting data in the literature on the relation between histopathology of the arterial duct and treatment with prostaglandin E1, we restudied our material. Special attention was paid to pathology of the duct not influenced by administration of prostaglandin E1. For this reason, three comparison groups (59 specimens) of non-treated ducts were evaluated. The results were compared with data from ducts obtained from 24 infants treated with prostaglandin E1. In agreement with our earlier findings, and with the results of Calder and her colleagues, there is indeed a marked increase in histopathology of the duct after administration of prostaglandin E1. The histopathology itself, however, is not specific for ducts treated with prostaglandin E1. It can also be found in the non-treated material. This has been previously described by others but had not been noted in our own previous work. The essential difference between those studies that confirmed an increase in pathology after treatment with prostaglandin E1 and those failing to find a marked effect is in the method of study. Changes were observed by those, including ourselves, who investigated complete sets of serial sections. The changes were not seen by those studying selected cross-sections from isolated parts of the duct or, at least in the cases treated with prostaglandin E1, sections taken at intervals.

Histologic studies on normal and persistent ductus arteriosus in the dog.

The process of anatomic closure of the ductus arteriosus was studied at the ultrastructural level in 15 normal beagles (age 0 hour to 13 days) and in 18 specimens from a strain of dogs with hereditary persistent ductus arteriosus (age 4 hours to 27 days). Normal ductal closure takes place from the pulmonary artery to the aortic end. It is accompanied by a series of histologic changes: 1) separation of the endothelial cells from the internal elastic lamina resulting in a wide region of subendothelial edema; 2) ingrowth and infolding of endothelial cells and migration of undifferentiated smooth muscle cells from the inner media into the subendothelial region; 3) apposition of endothelial cells bordering the lumen; and 4) degenerative changes. In persistent ductus arteriosus, these changes do not occur. The endothelial cells remain closely adhered to the internal elastic lamina and the underlying media is abnormal in structure. In the case of partial persistent ductus arteriosus (ductus diverticulum), both the normal and the abnormal type of wall are found in a single ductus arteriosus. The histologic features of the normal and the persistent ductus arteriosus in the dog resemble those of the normal and the persistent ductus arteriosus in humans, suggesting a similar pathogenesis.