Multiple peripheral pulmonary artery stenoses in adults: a rare cause of severe pulmonary hypertension necessitating lung transplantation.
Stenoses of multiple peripheral pulmonary artery branches represent a rare cause of pulmonary hypertension in children, but the prognosis is very poor for such patients. Herein we describe 2 patients with multiple peripheral pulmonary artery stenoses (MPPAS) presenting with severe pulmonary arterial hypertension in adulthood, which has only once been described previously. Both patients lived without significant health problems for decades; however, after onset of symptoms, their medical condition declined rapidly, necessitating lung transplantation several months after the diagnosis despite vasodilator therapy. Because MPPAS mimics chronic thromboembolic pulmonary hypertension, this entity may being underdiagnosed, decreasing the possibility of adequate therapy.
Ulrich
S
S
Department of Internal Medicine, University Hospital Zurich, Zurich, Switzerland. silvia.ulrich@usz.ch
Fischler
M
M
Pfammatter
T
T
Vogt
P
P
Weder
W
W
Boehler
A
A
Speich
R
R
D000328:Adult; D015901:Angiography, Digital Subtraction; D003251:Constriction, Pathologic; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology*; D012129:Respiratory Function Tests