ABSTRACT
UNLABELLED: The management of the preterm fetus with hydrops due to complete congenital heart block is difficult. The outcome is frequently associated with significant morbidity and mortality. Two fetuses presented at the post menstrual age of 29 and 30 weeks respectively with severe hydrops due to complete heart block. The following staged approach was adopted: (1) enhance fetal lung maturation with maternal corticosteroids and thyroid releasing hormone for 48 h; (2) elective Caesarean section; (3) classical neonatal management consisting of intubation and ventilation, drainage of all cavities with effusions; (4) increase neonatal heart rate by administration of i.v. isoprenaline, by bipolar trans-oesophageal pacing or epicutaneo-oesophageal pacing; (5) after the regression of the hydrops, start epicardial pacing after implantation of 2 or 3 temporary epicardial 3/0 pacemaker; (6) implantation of a permanent abdominal pacing system with steroid epicardial tip once the threshold exceeds 20 mA or when the baby weighs more than 1500 g. In these patients a permanent pacing system was implanted at the ages of 8 weeks (2045 g) and 4 weeks (1560 g) respectively. No major complications occurred; the cardiac outcome with 37 and 34 months of follow up is excellent. CONCLUSION: This proposed staged approach with temporary epicardial leads can improve the outcome of hydropic fetuses due to complete congenital AV block.
Subject(s)
Cardiac Pacing, Artificial/methods , Heart Block/congenital , Hydrops Fetalis/therapy , Infant, Premature , Adult , Female , Heart Block/therapy , Humans , Infant, Newborn , Lupus Erythematosus, Systemic , Male , Pregnancy , Pregnancy Complications , Pregnancy in DiabeticsABSTRACT
The effectiveness and safety of a short acting barbiturate, methohexital, was assessed for its use at the time of elective intubation in 18 newborn infants with severe respiratory or cardiac conditions. Evaluation included the speed of action and the degree of relaxation, sedation, and sleep in the first five minutes after administration. All newborn infants were intubated in a fully relaxed and somnolent state. In most infants recovery was completed within five minutes. A slight to moderate oxygen saturation drop was observed during the period of intubation, especially in patients with cyanotic heart disease. The side effects of the drug were twitching and a slight drop in blood pressure. In conclusion, methohexital seems to be a useful drug for short term anaesthesia in neonates, during which, short procedures like elective intubation can be safely performed.
Subject(s)
Anesthesia, General , Anesthetics, Intravenous , Intubation , Methohexital , Respiratory Distress Syndrome, Newborn/therapy , Anesthesia Recovery Period , Blood Pressure/drug effects , Bronchopulmonary Dysplasia/blood , Bronchopulmonary Dysplasia/therapy , Evaluation Studies as Topic , Heart Defects, Congenital/blood , Heart Defects, Congenital/therapy , Heart Rate/drug effects , Humans , Hypertension, Pulmonary/blood , Hypertension, Pulmonary/therapy , Infant , Infant, Newborn , Oxygen/blood , Prospective Studies , Respiration, Artificial , Respiratory Distress Syndrome, Newborn/bloodABSTRACT
We report on a newborn infant with male pseudohermaphroditism and glomerular lesions (Denys-Drash syndrome) but without Wilms tumor. A constitutional heterozygous mutation in the WT1 gene (366Arg to His) was identified. In addition the child had a large diaphragmatic hernia, so far not described in Denys-Drash syndrome. The expression of the WT1 gene in pleural and abdominal mesothelium and the occurrence of diaphragmatic hernia in transgenic mice with a homozygous WT1 deletion strongly suggests that the diaphragmatic hernia in this patient is part of the malformation pattern caused by WT1 mutations.
