Abstract
An 11 year-old girl, whose condition was diagnosed as juvenile-type autosomal
recessive polycystic kidney disease (ARPKD) at five years of age, presented with
chest pain and dyspnea that had developed suddenly two months previously.
Two-dimensional echocardiography, Doppler study and cardiac catheterization
confirmed pulmonary hypertension. The underlying mechanism of the diagnosis was
not defined. Two and a half months after the onset of symptoms, the patient died
of pulmonary hypertensive crisis. Careful regular checks of cardiopulmonary
status using two-dimensional echocardiography and Doppler should be considered
for the early detection of pulmonary hypertension even in an asymptomatic
patient with juvenile-type ARPKD.