Isolated peripheral pulmonary artery stenoses in the adult

BACKGROUND: Isolated peripheral pulmonary artery stenosis (PPS) in

the adult is rare and frequently unsuspected. We review in

this article our experience with 12 adult patients with

isolated PPS, half of whom had been previously diagnosed with

chronic pulmonary thromboembolic disease.


RESULTS: The presentation, evolution, and management of 12

adults with isolated PPS, 17 to 51 years of age (mean, 36.2

+/- 9.7 years), were evaluated. Presenting symptoms were

dyspnea and fatigue. Three patients had New York Heart

Association (NYHA) functional class III or greater. Lung

perfusion scans revealed multiple segmental abnormalities in

flow distribution in all patients. Oxygen desaturation at

rest was present in 4 patients. At catheterization, right

ventricular (RV) pressure was suprasystemic in 2 patients,

systemic in 1, and more than half-systemic in 7. All had

multiple bilateral non-uniform stenoses in segmental and

subsegmental arteries. Balloon pulmonary angioplasty (BPA) to

decrease RV hypertension and improve pulmonary flow

distribution was performed in 11 patients. After BPA, vessel

diameter increased > 50% in 10 patients, distal pulmonary

artery pressure increased > or = 30% in 6, and RV pressure

decreased > 30% in 5. One patient died shortly after BPA as a

result of pulmonary hemorrhage. Immediate procedural success

was achieved in 9 of 11 patients. At a mean follow-up period

of 52 +/- 32 months, 7 patients had sustained symptomatic

improvement (NYHA class I-II).

CONCLUSIONS: We describe a

severe syndrome of isolated PPS in the adult that mimics

chronic pulmonary thromboembolic disease. Pulmonary

hemodynamics and angiography are required for definitive

diagnosis. BPA may offer these patients successful short-term

reduction in RV hypertension and alleviation of


MCM: Not sure why Medline lists this article under CFS/FM/NA.

Kreutzer J, Landzberg MJ, Preminger TJ, Mandell VS, Treves ST, Reid

LM, Lock JE

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