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Isolated peripheral pulmonary artery stenoses in the adult

  [ 30 votes ]   [ Discuss This Article ] • April 1, 1996

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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