COR PULMONALE is a rare disease in the pediatric age group1 but its recognition is very important since emergency therapy is often essential for survival, and many cases are completely reversible and curable.2 Usually the pulmonary artery hypertension of cor pulmonale is secondary to destructive changes in the lungs, such as fibrosis and/or emphysema, but occasionally the lung parenchyma and lower air passages are normal, and abnormal pulmonary hemodynamics result from extrathoracic disease, such as obesity (the Pickwickian syndrome).3 In these patients, vasoconstrictive pulmonary artery hypertension is caused by the abnormal gaseous exchange of alveolar hypoventilation. (Alveolar hypoventilation is the hypoxemia resulting from insufficient ventilation without restrictive or obstructive pulmonary disease or abnormal diffusion. Alveolar ventilation is the amount of fresh air reaching the alveoli per minute and is measured by: tidal volume [VT]—dead space volume [VDS] × respiratory rate.) A seldom recognized
Cayler GG, Johnson EE, Lewis BE, Kortzeborn JD, Jordan J, Fricker GA. Heart Failure Due to Enlarged Tonsils and Adenoids: The Cardiorespiratory Syndrome of Increased Airway Resistance. Am J Dis Child. 1969;118(5):708–717. doi:10.1001/archpedi.1969.02100040710007
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