[Halasz syndrome revealed in the newborn infant by pulmonary arterial hypertension. Study of 4 cases].
In the 4 studied patients presenting with an Halasz's syndrome, the disease was complicated since the neonatal period by pulmonary arterial hypertension and heart failure. Three out of the 4 infants rapidly died, one after pneumonectomy. The fourth is surviving and benefitted solely from medical cardiotonic treatment. The pathogenesis of early pulmonary arterial hypertension in Halasz's syndrome is complex. The possibly associated cardiac malformations, the persistance of fetal obstructive hypertension due to the increase of the flow in the left pulmonary artery, the left to right shunt induced by the abnormal venous return and predominantly by the systemic blood supply to the right lung, are responsible, at various degrees, for pulmonary hypertension. Among surgical procedures to be proposed, the simple ligation of abnormal arteries which take their origin from the aorta seems to be preferable to pneumonectomy.
D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D008168:Lung / Q000002:abnormalities*; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities*; D011667:Pulmonary Veins / Q000002:abnormalities*; D014682:Vena Cava, Inferior / Q000002:abnormalities
Syndrome de Halasz à révélation néonatale par hypertension artérielle pulmonaire. Etude de quatre observations.