A case series of six children with primary pulmonary hypertension.
BACKGROUND
Primary pulmonary hypertension is an uncommon but serious disease in children. Management is difficult despite recent advances in pharmacotherapy.


METHODS
We reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome.


METHODS
These children were treated with individualised combinations of oxygen, diuretics and calcium antagonists.


RESULTS
In our follow-up of 5 children, 4 had died.


CONCLUSIONS
This condition is irreversible and progressive with a high mortality rate. A better understanding of, and research into, the pathogenesis would hopefully lead to the formulation of improved therapeutic strategies for this condition.
Quek
S C
SC
Department of Paediatrics, National University of Singapore, 5 Lower Kent Ridge Road, Singapore 119074.
Yip
C L
CL
Wong
M L
ML
Chan
K Y
KY
Wong
C L
CL
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy*; D008297:Male; D011379:Prognosis