Pulmonary Hypertension in Children

Pulmonary Hypertension (PHT) is more common in children than in adults. It is defined as an elevation in the pulmonary artery to at least half of the systemic pressure. PHT occurs as a result of many disease processes of childhood, including sickle cell, cystic fibrosis, bronchopulmonary dysplasia, chronic pneumonia as well as several congenital heart lesions which produce left to right shunts.
Children with PHT are at risk for acute decompensation during the perioperative period. The morbidity rate for these kids is eight times that of normal children. They respond to episodes of hypoxemia with prolonged and exaggerated hypoxia, sometimes only responding to long periods of hyperventilation, and pulmonary vasodilators.

For children with the most severe disease - those with pulmonary artery pressures that are equivalent to or higher than systemic pressure. Extreme caution should be used during the perioperative period and practitioners that are familiar with this disorder and the treatment of complications including cardiac arrest should manage them.

Rae Brown, M.D.

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