Isolated peripheral pulmonary artery stenoses in the adult.
ABSTRACT 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.
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ABSTRACT: Objectives Peripheral pulmonary artery stenosis (PPS) is a heterogeneous disease associated with numerous clinical or syndromic disorders including Williams syndrome. Previous studies focusing on patients with Williams syndrome have documented spontaneous improvement in branch pulmonary artery (PA) stenosis and right ventricular pressure. The goal of this study is to identify angiographic evidence of spontaneous occlusion or loss of segmental PAs in patients with PPS.Methods Patients undergoing serial catheterization for PPS at Boston Children's Hospital between 1999 and 2014 were selected. Patients with structural heart disease such as tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, or single ventricle were excluded. Hemodynamic data were extracted from catheterization reports, all available angiograms were reviewed, and evidence of segmental PA loss was documented based on defined angiographic criteria.ResultsForty-two patients were included in the study. Each patient underwent an average of 5 catheterizations over the 15-year study period. The average RV pressure at initial catheterization was near systemic levels. Evidence of segmental PA loss was present in 40% of patients. Almost 30% of patients with vessel loss demonstrated loss at their initial catheterization, and 65% of patients experienced loss of multiple vessels over time. Patients with vessel loss tended to be older at initial catheterization and have higher average RV pressures at their most recent catheterization than those without vessel loss.Conclusions Progressive segmental PA loss in patients with systemic arteriopathy and PPS has not been well described previously. In this cohort, segmental PA loss occurred in 40% of patients, resulting in persistently elevated PA pressures relative to patients who did not develop vessel loss. These findings highlight the importance of surveillance in this population of patients with systemic arteriopathy and severe PPS.Congenital Heart Disease 09/2014; DOI:10.1111/chd.12211 · 1.01 Impact Factor
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ABSTRACT: Peripheral pulmonary artery stenosis is a common congenital heart lesion associated with several genetic syndromes. We have reviewed the genetics of the lesion and present an unusual case of peripheral pulmonary stenosis involving a newly reported genetic deletion on chromosome 16. Further studies will be needed to confirm association of this genotype and phenotype.Congenital Heart Disease 06/2014; DOI:10.1111/chd.12198 · 1.20 Impact Factor
Dataset: KT NakanishiIVUS99