Interventional Strategies in the Management of Peripheral Pulmonary Artery Stenosis

Trivedi, Kalyani R.; Benson, Lee

doi:10.1046/j.1540-8183.2003.08031.x

Cited by 72 publications

(59 citation statements)

References 84 publications

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“…9 Recurrent stenosis is not uncommon, as it occurs in approximately 35% of successfully dilated vessels. 32,33 Stent placement in the central pulmonary arteries has been performed with success, 34 but stenting of small segmental arteries, particularly in patients with congenital PPAS, has been less successful.…”

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“…32,33 Stent placement in the central pulmonary arteries has been performed with success, 34 but stenting of small segmental arteries, particularly in patients with congenital PPAS, has been less successful. 9,35,36 In recent years, surgical intervention for PPAS has been associated with low mortality and a reduction of the RV/aortic pressure ratio in half, a hemodynamic improvement that has been maintained during follow-up. 31 However, surgery is limited to proximal pulmonary artery lesions; distal branches are difficult for the surgeon to repair.…”

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confidence: 99%

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Peripheral Pulmonary Artery Stenosis as a Cause of Pulmonary Hypertension in Adults

et al. 2015

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Peripheral pulmonary artery stenosis (PPAS) is an underrecognized condition in the adult population. PPAS can lead to pulmonary hypertension but is likely misdiagnosed as either idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. We retrospectively identified adult patients with PPAS either in its isolated form or related to other congenital defects from January 1998 to September 2012. We reviewed the patients' clinical data by using our hospital electronic medical records and/or their paper charts. We identified 6 adult patients with PPAS with an age range of 16-56 years (1 woman and the rest men). Presenting signs and symptoms were thoracic murmurs, progressive dyspnea, and syncope. Three patients had Williams-Beuren syndrome. Pulmonary angiography showed that PPAS was predominantly located in main branches or lobar pulmonary arteries in 5 patients, while in 1 patient the arterial narrowing was at the level of the segmental pulmonary arteries. Right heart catheterization showed a mean pulmonary artery pressure (PAP) ranging from 35 to 60 mmHg. Balloon dilation was performed in all patients, predominantly in the lobar arteries, and it caused a decrease in mean PAP that ranged from 16% to 46% in 5 patients. In 1 patient the mean PAP did not decrease. All but 1 patient had follow-up echocardiograms at 1 year that showed stable echocardiographic findings. Pulmonary hypertension due to PPAS continues to presents a diagnostic challenge. Therefore, a high index of suspicion during the initial evaluation of pulmonary hypertension is essential for its prompt diagnosis and adequate treatment.

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Peripheral Pulmonary Artery Stenosis as a Cause of Pulmonary Hypertension in Adults

et al. 2015

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“…Angioplasty and stent implantation have been shown to increase vessel diameter and decrease RV pressure in patients with PA stenosis in various clinical circumstances. [9][10][11][12][13][14] Although several studies have focused on acute outcomes of catheterization in patients with Williams syndrome, 12,15 little is known about long-term outcomes in patients who underwent transcatheter intervention for severe PPS early in life. Characterizing long-term outcomes in this population and identifying genetic, procedural, and hemodynamic factors associated with outcomes may help inform decision-making and prognosis.…”

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Outcomes After Primary Transcatheter Therapy in Infants and Young Children With Severe Bilateral Peripheral Pulmonary Artery Stenosis

Cunningham

McElhinney

Bergersen

et al. 2013

Circ: Cardiovascular Interventions

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Patients with bilateral PPS, who underwent transcatheter intervention at Children's Hospital Boston at ≤5 years of age from July 1984 through July 2009, were ascertained from the computer database of the Department of Cardiology. This series does not include patients with PA stenosis in the context of other anomalies, such as tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, single ventricle heart disease, congenitally discontinuous PAs, or patients with congenital rubella. Patients with isolated atrial or ventricular septal defects, or systemic arterial disease (eg, supravalvar aortic Background-Angioplasty and stent implantation have become accepted therapies for isolated peripheral pulmonary stenosis, and have been shown to increase vessel diameter and reduce right ventricular (RV) pressure acutely in patients with pulmonary artery (PA) stenosis. The purpose of this study was to assess long-term outcomes after primary transcatheter therapy for peripheral pulmonary stenosis. Methods and Results-We studied 69 patients who underwent primary transcatheter intervention for severe isolated peripheral pulmonary stenosis at ≤5 years of age. Genetic/syndromic diagnoses included Williams syndrome (n=23), non-Williams familial arteriopathy (n=12), and Alagille syndrome (n=3). At the initial PA intervention, median RV:aortic pressure ratio decreased from 1.00 to 0.88 (median decrease, 0.18; P<0.001). Patients with a higher preintervention RV:aortic pressure ratio had a greater reduction (P<0.001). During follow-up (median, 8.5 years), 10 patients died, 5 from complications of PA catheterization (all before 1998). Thirteen patients underwent surgical PA intervention, most within 1 year and along with repair of supravalvar aortic stenosis. Freedom from any PA reintervention was 38±6% at 1 year and 22±6% at 5 years. The median RV:aortic pressure ratio decreased from 1.0 at baseline to 0.53 at the most recent catheterization (P<0.001), and 82% of patients with available clinical follow-up were asymptomatic. Conclusions-Transcatheter therapy for infants with severe peripheral pulmonary stenosis has become safer, regardless of genetic condition. Coupled with reintervention and surgical relief in selected cases, RV:aortic pressure ratios decrease substantially and most patients are asymptomatic at late follow-up. (Circ Cardiovasc Interv. 2013;6:460-467.)

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“…12) There are several reports of congenital peripheral pulmonary artery stenosis in children. 3,13) However, congenital peripheral pulmonary artery stenosis in adults is especially rare. 8,9) Kreutzer, et al reported 12 cases of peripheral pulmonary artery stenosis in adults.…”

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Pulmonary Artery Stenosis due to External Compression by a Calcified Pericardial Band

et al. 2004

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SUMMARYA 60-year-old male with exertional dyspnea was referred to our hospital. Right pulmonary artery stenosis due to external compression by a calcified band was diagnosed by echocardiography, computed tomography, and magnetic resonance imaging. Percutaneous transluminal angioplasty was conducted in vain due to vascular recoil and failure of stent delivery. Pulmonary bypass grafting was performed successfully. The surgery indicated a probable etiology of chronic pericarditis. This is an extremely rare case of adult pulmonary artery stenosis without a known history of congenital disease, constrictive pericarditis, tuberculosis, or surgery. (Jpn Heart J 2004; 45: 527-533)

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Interventional Strategies in the Management of Peripheral Pulmonary Artery Stenosis

Cited by 72 publications

References 84 publications

Peripheral Pulmonary Artery Stenosis as a Cause of Pulmonary Hypertension in Adults

Peripheral Pulmonary Artery Stenosis as a Cause of Pulmonary Hypertension in Adults

Outcomes After Primary Transcatheter Therapy in Infants and Young Children With Severe Bilateral Peripheral Pulmonary Artery Stenosis

Pulmonary Artery Stenosis due to External Compression by a Calcified Pericardial Band

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