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.
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).
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 symptomatology.
[Show abstract][Hide abstract] ABSTRACT: Multiple peripheral pulmonary artery stenoses were detected in three patients with congenital generalised lipodystrophy. This association, which has not been described before, may be clinically important in patients with lipodystrophy who present with impaired exercise tolerance or heart murmurs.
Archives of Disease in Childhood 06/1997; 76(5):456-7. DOI:10.1136/adc.76.5.456 · 2.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Stenosis of pulmonary arteries is one of the most challenging problems requiring treatment in the care of patients with congenital and acquired cardiopulmonary disease. Surgical approaches have been met with difficulty over the years, and may themselves lead to further distortion of the treated arteries. Balloon dilation first came into use in the 1980s, and has proved moderately effective. Its use has been extended to proximal pulmonary valve stenosis in order to improve distal flow and artery growth in some variants of tetralogy of Fallot. More recently, the judicious application of stent implantation has improved the outlook for pulmonary artery stenosis. The etiology, treatment (with balloon dilation and stent placement), and prognosis of pulmonary arterial stenosis will be discussed.
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