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 ou r experience with 12 adult patients with isolated PPS, half of whom ha d been previously diagnosed with chronic pulmonary thromboembolic dise ase. Methods and Results The presentation, evolution, and management o f 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 abno rmalities 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 mor e than half-systemic in 7. All had multiple bilateral nonuniform steno ses in segmental and subsegmental arteries. Balloon pulmonary angiopla sty (BPA) to decrease RV hypertension and improve pulmonary flow distr ibution was performed in 11 patients. After BPA, vessel diameter incre ased >50% in 10 patients, distal pulmonary artery pressure increased g reater than or equal to 30% in 6, and RV pressure decreased >30% in 5. One patient died shortly after BPA as a result of pulmonary hemorrhag e. Immediate procedural success was achieved in 9 of 11 patients. At a mean follow-up period of 52+/-32 months, 7 patients had sustained sym ptomatic improvement (NYHA class I-II). Conclusions We describe a seve re syndrome of isolated PPS in the adult that mimics chronic pulmonary thromboembolic disease. Pulmonary hemodynamics and angiography are re quired for definitive diagnosis. BPA may offer these patients successf ul short-term reduction in RV hypertension and alleviation of symptoma tology.