We report the case of a 18 days of life newborn who presented, after a normal pregnancy and first days, an acute respiratory distress. He need to be intubated, to receive sedative drugs, and to be transfered in a paediatric intensive care unit.
In this unit, because of the intense hypoxemia, inhaled nitric oxide (NO) was administered, concomitantly with ventilation with 100% of oxygen. We can note a real worsening after NO administration and particularly his chest radiography was really asymetric, with an aspect of right pulmonary hypoperfusion and left pulmonary oedema.
An echocardiography was performed, and the important elements were: supra-systemic pulmonary hypertension, asymetric pulmonary arteries (PA) with hypoplastic right PA and dilated left PA, moreover we could see that left atrium received the pulmonary veins but with one hypoplastic and others dilated, with acceleration of 2 m/sec and suspicion of stenosis of pulmonary veins. Atrial septal defect was shunting right to left.
A CT-scan completed the investigations and gave the precise diagnosis: this baby suffered from an intra-lobar sequestration concerning all right superior and medium lobes, with 6 systemic collateral arteries, responsible for the pulmonary hypertension and the increased cardiac output.
The therapeutic decision was to stop NO, administer diuretics, and to embolize during a catheterization 4 of the 6 systemic arteries. The evolution after was good, even if the baby suffered from persistent pulmonary hypertension after procedure. The extubation was possible six days after procedure.
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