Transient postnatal heart failure caused by noncompaction of the right ventricular myocardium.

Isolated noncompaction of the ventricular myocardium is a cardiomyopathy influencing almost exclusively the left ventricle in children and adults. We report a case of a neonate presenting with right ventricular failure caused by a noncompaction of the right ventricle. However, later course of the disease demonstrated biventricular involvement.

Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries.

Objectives: The goal of this research was to identify predictors for sudden death (SD) in patients with transposition of the great arteries (TGA) who have undergone atrial inflow repair.

Background: Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD.

Sudden unexpected death in children with a previously diagnosed cardiovascular disorder.

Background: It is known that children with previously diagnosed heart defects die suddenly. The causes of death are often unknown.

Objective: The aim of the study was to identify all infants and children within the Netherlands with previously diagnosed heart disease who had a sudden unexpected death (SUD), and to identify the possible cause of death.

Austrian syndrome in a child-aortic valve endocarditis following pneumococcal meningitis.

A 7-year-old girl with a previously healthy heart underwent a Ross procedure after pneumococcal sepsis, meningitis and aortic valve endocarditis with extensive para-valvular involvement. While pneumococcal infections including respiratory tract infections, bacteraemia and meningitis are common in childhood, endocarditis caused by Streptococcus pneumoniae occurs rarely. Pneumococcal meningitis and aortic valve endocarditis is a known combination, described as Austrian syndrome.

Echocardiographic assessment of preload conditions does not help at the neonatal intensive care unit.

To determine the value of noninvasive assessment of right ventricular preload in neonates, a prospective unblinded study was performed. Thirty-seven neonates without heart disease (median birth weight 1390 g, range 900 to 4400) were studied at the neonatal intensive care unit, comparing directly measured central venous pressure (CVP) and two-dimensional echocardiographic measurement of the maximum and minimum diameter of the inferior vena cava and calculated vena cava index (VCI). CVP was higher in conventionally ventilated and high-frequency oscillatory ventilated neonates than in those breathing spontaneously ( p < 0.

Repair of aorto-right ventricular tunnel with pulmonary stenosis and an anomalous origin of the left coronary artery.

A 5-month-old infant with aorto-right ventricular tunnel, pulmonary stenosis and a sole supply of the left coronary artery from the tunnel underwent closure of the ventricular end of the tunnel, subsequent to balloon pulmonary valvuloplasty. Care was taken not to obstruct the aortic end of the tunnel in order to maintain the left coronary artery perfusion.

Low-dose daunorubicin in induction treatment of childhood acute lymphoblastic leukemia: no long-term cardiac damage in a randomized study of the Dutch Childhood Leukemia Study Group.

Background: To investigate late cardiotoxicity in childhood acute lymphoblastic leukemia (ALL) survivors after induction treatment with or without daunorubicin (DNR; 25 mg/m(2), i.v., weekly, x4, cumulative dose 100 mg/m(2)).

Transient foramen ovale incompetence in the normal newborn: an echocardiographic study.

Unlabelled: To assess presence, predominant direction and natural history of interatrial shunt flow in the normal newborn period an uncontrolled pilot study was performed. Twenty term ( > 36 completed weeks gestational age) newborns were studied using cross sectional, M-mode and colour Doppler echocardiography; cardiac, pulmonary or renal disease were excluded before entry to the study. In 11 of 20 normal term newborns a predominant left to right interatrial shunt was detected on the 1st day after birth.

Ultrasonographic prenatal diagnosis of conjoined thoracopagus twins: a case report.

A case of prenatal diagnosed thoracopagus conjoined twins is presented. In thoracopagus twins the cardiovascular system mainly determines the options for separation and survival. Therefore prenatal investigation of this organ system is indispensable.

[The effect of the anthracycline derivative doxorubicin on myocardial function in children].

Echocardiograms of 47 children treated with doxorubicin in combination with other cytostatic agents in the period 1979-1984 were reviewed. The authors compared the first and the last echocardiographic registration of every individual patient, analysing the left ventricular dimensions and the shortening fraction which is the indicator of myocardial contractility. Statistical analysis revealed a significant dilation of the left ventricle with a decrease of contractility in the whole group of patients during the therapy.

Ventricular septal defect with overriding aorta in trisomy-18.

In a child with trisomy-18 prenatal echocardiography demonstrated the presence of a ventricular septal defect with overriding aorta. In a previous anatomical study of 18 hearts with this malformation we found a 50% concurrence rate with trisomy-18. In case of prenatal echocardiographic detection of a ventricular septal defect with overriding aorta, we advise thorough ultrasound examination for additional malformations as seen in trisomy-18; subsequently this procedure should be completed by cytogenetic analysis of amniotic cells.

Prenatal ultrasound diagnosis of the Holt-Oram syndrome.

The Holt-Oram syndrome is an autosomal dominant disorder consisting of a congenital heart defect in combination with characteristic upper limb abnormalities. This report presents the ultrasonographic follow-up of two fetuses at risk for the Holt-Oram syndrome. In the first fetus, the existence of Holt-Oram syndrome was suspected at 22 weeks of gestation; a ventricular septal defect, an atrial septal defect, and a minor skeletal defect were found.

[2 newborn infants with severe arrhythmia caused by hyperkalemia].

Two newborn infants with ventricular arrhythmias secondary to hyperkalaemia are presented. One child also showed a decreased serum calcium concentration. There is scanty literature concerning the often life threatening cardiac arrhythmias due to hyperkalaemia in the newborn infants.

An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes.

An X-linked recessive disease is reported in a large pedigree. The disease is characterised by a triad of dilated cardiomyopathy, neutropenia and skeletal myopathy. The untreated patients, all boys, died in infancy or early childhood from septicemia or cardiac decompensation.