Right ventricular hypoplasia: outcome after conservative perinatal management.

Isolated right ventricular hypoplasia is a rare congenital cardiac anomaly. A case described in which prenatal diagnosis was made by echocardiography. Conservative perinatal management produced a favourable short-term outcome without compromising the definitive surgical intervention later. The decisions were based on principles used in the treatment of pulmonary atresia with intact interventricular septum.

Partial abnormal pulmonary venous return in Turner syndrome.

Three cases of partial anomalous pulmonary venous return, in one case combined with coarctation of the aorta and in another with discrete subaortic stenosis, are described in patients with Turner syndrome. In two of them the right and left superior pulmonary veins drained into the right superior vena cava and left innominate vein respectively. Remarkably, in all three cases the atrial septum was intact. We feel that this unique combination probably is not purely coincidental, but might rather be specific for Turner syndrome.

An infant with the scimitar syndrome and pulmonary hypertension; successful surgical intervention.

The case of an infant with the scimitar syndrome and pulmonary hypertension is presented. After surgical intervention with ligation of the abnormal arterial supply to the hypoplastic lung, the pulmonary hypertension disappeared. This was proven by cardiac catheterization after the operation. The abnormal drainage from the right lung into the inferior vena cava was not corrected. The child has had no complaints since except for recurrent pulmonary infections.

[Cardiovascular disorders with respiratory distress in childhood]. / Adembenemende hart- en vaataandoeningen op de kinderleeftijd.

Because of their low prevalence cardiovascular abnormalities are an easily overlooked cause of persistent respiratory distress in childhood. A review is presented of the most common cardiovascular disorders that give rise to respiratory distress by compression of the airway at various levels. At tracheal level the double aortic arch and the aberrant right subclavian artery are the most frequent anomalies. Bronchial compression is mostly due to left to right shunting through either a persistent ductus arteriosus or a ventricular septal defect. Compression of the lung parenchyma itself is in most cases caused by dilatation of the left ventricle. Anatomy, symptomatology, diagnosis and therapy of the cardiovascular disorders are described.

[Wolff-Parkinson-White syndrome; important in childhood also]. / Het Wolff-Parkinson-White syndroom; ook in de kinderleeftijd van betekenis.

In a cooperative, retrospective, study 120 children are reviewed with preexcitation pattern. 80 patients had tachycardias; 54 children were under 1 year, 49 under 3 months of age at their first attack. In 50% the preexcitation pattern disappeared in the first year of life, allthough intermittent preexcitation could be seen in some patients. In 12 patients a circusmovement tachycardia was proved on the surface ecg; in the group of 63 children with a tachycardia of unknown origin probably more may be caused by this mechanism. A beneficial effect of digoxin in childhood is noticed, with good response in 45 cases. A possible explanation for the difference in effect of digoxin during childhood and in adolescence is discussed.