Abstract
Five patients (4-9 yrs.) presented in five years with primary pulmonary hypertension (PPH). Each was initially evaluated for seizures or syncope. Two sisters (#1s2) were Hispanic and resided at 5,200′. Two brothers (#3&4) were black and resided at 5,200′. An only child (#5) resided at 9,000′.
Cardiac catheterization with hyperoxia was performed in all and one received additional prostaglandin 12 (PGI2). Structural heart disease was ruled out in all. Resistance ratios (R.R.) were calculated in room air and 100% O2.
Three of the five patients died 9.2 months after diagnosis. Two of the deaths (#1&2) occurred after steady clinical deterioration at 5,200′. The third child (#5) dramatically improved after moving to sea level but died five days after returning to altitude. One of the black males has significantly improved since moving to sea level (#4) and his brother with milder disease has spontaneously improved while remaining at altitude (#3).
PPH is a rare and rapidly fatal disease in childhood. In patients presenting at altitude, the clinical course may be altered or reversed by moving to sea level.
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Loehr, J., Washington, R., Wiggins, J. et al. PRIMARY PULMONARY HYPERTENSION IN PEDIATRIC PATIENTS AT ALTITUDE. Pediatr Res 21 (Suppl 4), 191 (1987). https://doi.org/10.1203/00006450-198704010-00152
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DOI: https://doi.org/10.1203/00006450-198704010-00152