Peripheral Pulmonary Artery Stenosis Research Articles

Pulmonary Artery Diverticulum: An Angiographic Marker for Williams Syndrome

We evaluated the presence of pulmonary artery diverticulum in patients with Williams syndrome in comparison with other conditions causing peripheral pulmonary artery stenosis (PPS). Angiographic characteristics of patients with a definitive diagnosis of Williams syndrome, by fluorescence in situ hybridization, between 1990 and 2008 were reviewed. These data were compared with those diagnosed with those for patients with PPS without Williams syndrome. Differentiating morphological features on angiography were compared between the groups, along with demographic and echocardiographic data. Twelve patients with a chromosomal diagnosis of Williams syndrome who underwent cardiac catheterization were identified. Seven were male. Eleven patients (91%) had supravalvar aortic stenosis and nine (81%) had PPS. Pulmonary valve stenosis was seen in two patients. Eight patients who were negative for Williams syndrome and had PPS were identified during the same period. Two had Alagille syndrome and one had Noonan syndrome. Mean age at catheterization was 5 years in the Williams group versus 8 years in the non-Williams group. Pulmonary artery diverticulum involving the main pulmonary artery was documented in all patients with Williams syndrome, while none of the patients in the other group had it. It originated at the bifurcation of the pulmonary artery in all. In conclusion, the angiographic appearance of a diverticulum as an extension of the main pulmonary artery is a consistent finding in patients with Williams syndrome. Compared to the classically described findings of supravalvar aortic stenosis or PPS, pulmonary artery diverticulum can be considered as a pathognomonic feature of Williams syndrome.

Correlation between cardiac catheterization and contrast enhanced MRI in pulmonary artery stenosis

Introduction Peripheral pulmonary artery stenosis (PPAS) is present in 2-3% of all congenital heart defects. Catheter interventions guided by conventional X-ray angiography (XRA) have replaced cardiac surgery in the management of PPAS. However, X-ray exposure has negative health effects. Interventional XRA would benefit from non-ionizing and noninvasive planning using contrast enhanced magnetic resonance imaging (CE-MRI) due to its multi-planar capability, but the correlation of images in the two modalities is not always perfect, possibly due to different projections or time delays in capture.

Cardiovascular Abnormalities, Interventions, and Long-term Outcomes in Infantile Williams Syndrome

To determine the prevalence of cardiovascular abnormalities (CVA) and outcomes in patients with Williams syndrome presenting before 1 year of age. A retrospective review was undertaken of consecutive patients with WS at our institution from January 1, 1980, through December 31, 2007. WS was diagnosed by an experienced medical geneticist and/or by fluorescence in situ hybridization. CVA were diagnosed with the use of echocardiography, cardiac catheterization, or computerized tomographic angiography. Freedom from intervention was determined using Kaplan-Meier analysis. The study group was 129 patients with CVA. Age at presentation was 127 +/- 116 days, with follow-up of 8.0 +/- 7.5 years (0 to 42 years). The most common lesions were peripheral pulmonary artery stenosis (62%) and supravalvar aortic stenosis (57%). Other CVA were common. CV interventions were performed in 29%, with 58% of those before 1 year. Freedom from intervention was 85%, 73%, and 66% at 1, 5, and 25 years, respectively. Four patients died. CVA are the most common manifestations of infantile Williams syndrome and occur with greater frequency than previously reported. In those with CVA, interventions are common and usually occur by 5 years of age. Most of these patients do not require intervention on long-term follow-up, and overall mortality is low.

Congenital Pulmonary Artery Stenoses Masquerading as Chronic Thromboembolic Disease

Stenotic pulmonary vascular lesions, often amenable to balloon angioplasty, can erroneously appear to suggest chronic thromboembolic disease on ventilation/perfusion (V/Q) scan. We report a case of multiple peripheral pulmonary artery stenoses and describe radiologic findings and treatment options.

Dynamic Left Main Coronary Artery Obstruction Associated With Williams Syndrome

A 5-year-old boy with Williams syndrome (WS) presented with a history of diffuse recurrent obstructive vascular disease. At the age of 12 months, he underwent pericardial patch augmentation of the ascending aorta and pulmonary arteries. He subsequently required Dacron patch repair of the ascending aorta and aortic arch at 3 years of age. In the interim, he had undergone percutaneous transluminal angioplasty and stenting for recurrent peripheral pulmonary artery stenoses and aortic recoarctation. On routine follow-up, he was found to have worsening right ventricular hypertension associated with disparate lung perfusion on a nuclear perfusion scan. He was brought to the catheterization laboratory for further investigation. Angiography showed recurrent pulmonary artery in-stent restenosis and aortic recoarctation. Coronary …

Tetralogy of Fallot: A Possible Etiology for Narrowing of the Right Pulmonary Artery

Patients with tetralogy of Fallot have right ventricular outflow and pulmonary valve stenosis. They may also have more distal peripheral pulmonary artery stenoses. Peripheral stenosis is believed to be part of the spectrum of the genetic manifestation of tetralogy. Our hypothesis was that narrowing of the right pulmonary artery may be due to compression from the adjacent dilated ascending aorta. We identified 27 patients with tetralogy of Fallot who underwent magnetic resonance imaging scanning postoperatively, most often for evaluation of pulmonary valve stenosis and regurgitation. On the axial image at the level of the right pulmonary artery, we measured the transverse diameters of the ascending and descending aorta, and the minimum and maximum right pulmonary artery diameters. There was a significant correlation between the ratios of the ascending:descending aorta diameters and the maximum:minimum right pulmonary artery diameters. Thus, increasing ascending aorta size is associated with decreasing size of the adjacent right pulmonary artery. In our patient population with tetralogy of Fallot, right pulmonary artery narrowing is at least partly because of compression from the adjacent enlarged ascending aorta.

How We Approach Peripheral Pulmonary Stenosis in Williams-Beuren Syndrome

Williams-Beuren syndrome is associated with supravalvar aortic stenosis and peripheral pulmonary artery stenosis in the majority of affected individuals. Among patients in whom surgery for supravalvar aortic stenosis is contemplated, stenosis of the branch pulmonary arteries is common. For asymptomatic patients with subsystemic right ventricular pressure, the natural history is favorable and no intervention is necessary. For patients with important proximal branch pulmonary artery, stenoses patch arterioplasty can be accomplished at the time of surgery for supravalvar aortic stenosis. For patients with important peripheral pulmonary stenosis, preoperative catheter-based therapy is indicated. Surgical repair of peripheral pulmonary stenosis is indicated when preoperative intervention is unsuccessful and can include a combination of patch arterioplasty as well as intraoperative application of catheter-based techniques. This report will address the approach to this lesion with catheter-based approaches, surgical approaches, and their combination.

Moyamoya Disease with Peripheral Pulmonary Artery Stenoses and Coronary Artery Fistulae

Moyamoya is a progressive disorder of the cerebral vasculature. Our report describes a rare case of Moyamoya disease with distal peripheral pulmonary artery stenoses and coronary fistulae in a 12-year-old Caucasian female patient.

Hybrid transcatheter–surgical approach in complex pulmonary artery stenosis due to arterial tortuosity syndrome

Arterial tortuosity syndrome is a connective tissue disorder characterized by elongation and tortuosity of large elastic arteries, potentially resulting in multiple vascular stenoses. This paper reports a novel hybrid strategy to treat 'complex' bilateral peripheral pulmonary artery stenoses consisting of transcatheter stent implantation via midline sternotomy and reductive angioplasty of the proximal pulmonary arteries.

Congenital Supravalvular Aortic Stenosis and Sudden Death Associated with Anesthesia: What’s the Mystery?

Patients with congenital supravalvular aortic stenosis and associated peripheral pulmonary artery stenoses, the majority of whom have Williams-Beuren syndrome, are inherently at risk for development of myocardial ischemia. This is particularly true in the setting of procedural sedation and anesthesia. The biventricular hypertrophy that accompanies these lesions increases myocardial oxygen consumption and compromises oxygen delivery. In addition, these patients often have direct, multifactorial compromise of coronary blood flow. In this article, we review both the pathophysiology of congenital supravalvular aortic stenosis and the literature regarding sudden death in association with sedation and anesthesia. Recommendations as to preoperative assessment and management of these patients are made based on the best available evidence.

A novel 2.43 Mb deletion of 7q11.22–q11.23

We present a patient with a novel heterozygous deletion of 7q11.22-q11.23. Standard cytogenetic analysis using the ELN cosmid 82C and the ELN/ LIMK1 cosmid 34B FISH probes suggested a diagnosis of Williams syndrome. Although he has supravalvular aortic stenosis and peripheral pulmonary artery stenosis, which are common in this condition, he does not have the clinical gestalt of Williams syndrome. 44k oligo array CGH analysis showed a 2.43 Mb deletion, encompassing the proximal 1.43 kb of the Williams syndrome critical region and extending approximately 1 Mb beyond it. The deletion of further genes outside the Williams syndrome critical region does not appear to be having a phenotypic effect at present.

Hybrid Transcatheter-Surgical Strategy in Arterial Tortuosity Syndrome

Arterial tortuosity syndrome is a rare connective tissue disorder characterized by elongation and tortuosity of large elastic arteries resulting in multiple vascular stenoses. This article reports on a novel hybrid transcatheter-surgical strategy to treat bilateral peripheral pulmonary artery stenoses due to this syndrome. Operation consisted of stent implantation by midline sternotomy and reductive reconstruction of the proximal pulmonary arteries. This approach resulted in right ventricular overload relief and sudden improvement of the patient's clinical condition.

Progress of interventional strategies in the management of peripheral Pulmonary artery stenosis

周围肺动脉狭窄(PPAS)应用外科手术治疗可导致受治肺动脉进一步扭曲或狭窄,位于肺动脉分支远端难以暴露手术野而成为临床上较为棘手的问题.介入治疗是新近发展起来的一种治疗PPAS较理想的方法.PPAS介入治疗的发展先后出现了球囊扩张血管成形术、切割球囊血管成形术、血管内支架置入术三种方法,该文分别对三种方法的研究过程、治疗的禁忌证、适应证、并发症和有待改进的问题等进行概述,并对近年来在球囊治疗采用较保守的连续性扩张、输送系统的改进、支架应用的长度与再狭窄处理等方面的研究情况作一综述,以期促进对PPAS介入治疗的进一步研究。

Spontaneous Development and Rupture of Pulmonary Artery Aneurysm: A Rare Complication in an Infant with Peripheral Pulmonary Artery Stenoses Due to Mutation of the Elastin Gene

We present a 3-year-old boy with an elastin gene mutation and multiple peripheral pulmonary stenoses, who developed aneurysms of the pulmonary arteries spontaneously. We performed transcatheter occlusion of the aneurysms with detachable coils. While pulmonary arterial aneurysms may develop following pulmonary balloon angioplasty, spontaneous development is exceedingly rare. To the best of our knowledge, this is the first report describing spontaneous development of pulmonary artery aneurysms in a patient with peripheral pulmonary artery stenoses due to mutation of the elastin gene or Williams-Beuren syndrome.

Matrix GLA Protein, an Inhibitory Morphogen in Pulmonary Vascular Development

Deficiency of matrix GLA protein (MGP), an inhibitor of bone morphogenetic protein (BMP)-2/4, is known to cause arterial calcification and peripheral pulmonary artery stenosis. Yet the vascular role of MGP remains poorly understood. To further investigate MGP, we created a new MGP transgenic mouse model with high expression of the transgene in the lungs. The excess MGP led to a disruption of the pulmonary pattern of BMP-4, and resulted in significant morphological defects in the pulmonary artery tree. Specifically, the vascular branching pattern lacked characteristic side branching, whereas control lungs had extensive side branching accounting for as much as 40% of the vascular endothelium. The vascular changes could be explained by a dramatic reduction of phosphorylated SMAD1/5/8 in the alveolar epithelium, and in epithelial expression of the activin-like kinase receptor 1 and vascular endothelial growth factor, both critical in vascular formation. Abnormalities were also found in the terminal airways and in lung cell differentiation; high levels of surfactant protein-B were distributed in an abnormal pattern suggesting lost coordination between vasculature and airways. Ex vivo, lung cells from MGP transgenic mice showed higher proliferation, in particular surfactant protein B-expressing cells, and conditioned medium from these cells poorly supported in vitro angiogenesis compared with normal lung cells. The vascular branching defect can be mechanistically explained by a computational model based on activator/inhibitor reaction-diffusion dynamics, where BMP-4 and MGP are considered as an activating and inhibitory morphogen, respectively, suggesting that morphogen interactions are important for vascular branching.

The negative impact of Alagille syndrome on survival of infants with pulmonary atresia

Alagille syndrome (AGS) is a complex disorder with multisystemic involvement, including the liver, heart, kidneys, cerebral vasculature, skeleton, eyes, and face.' A structural heart defect is one of the diagnostic criteria for AGS. These vary in severity, with peripheral pulmonary arterial stenosis being a common problem. Pulmonary atresia (PA) is a rare presentation in AGS, but we were impressed by the poor outcome of such infants following review of our institutional experience over the last 20 years. The information has particular relevance in this era where prenatal diagnosis of both AGS and PA can be made.

Anästhesie für Herzkatheteruntersuchungen bei Kindern

Cardiac catheterization in children with congenital heart disease or in adults with completely or partially corrected cardiac defects, is a growing field of activity for anaesthesiologists. This requires not only the willingness for interdisciplinary co-operation, but also detailed knowledge about the pathophysiology of congenital heart diseases. In interventional paediatric cardiology significant innovations have occurred during recent years including stenting of a patent ductus arteriosus and of peripheral pulmonary artery stenosis. Furthermore, radiofrequency catheter ablation for recurrent tachyarrhythmia, or resynchronisation therapy with biventricular pacing in the setting of congestive heart failure, or implantation of cardioverter defibrillators are increasingly being employed which require anaesthesia support.

PERIPHERAL PULMONARY ARTERY STENOSIS IN A FOUR‐MONTH‐OLD WEST HIGHLAND WHITE TERRIER

A 4-month-old West Highland White Terrier was evaluated for dyspnea. Severe cardiac silhouette enlargement was present radiographically. Severe right ventricular hypertrophy and dilation with a dilated pulmonary trunk and a stenosis of the right pulmonary artery were diagnosed via echocardiography. Additional areas of peripheral pulmonary artery stenosis were diagnosed with nonselective computed tomography (CT) angiography and selective fluoroscopic angiography. Balloon dilation therapy was unsuccessful and the imaging findings were confirmed at necropsy.

Expanding indications for the treatment of pulmonary artery stenosis in children by using cutting balloon angioplasty

To evaluate the role of cutting balloon angioplasty in children with pulmonary artery stenosis. Pulmonary artery stenoses can be either congenital or secondary to postoperative scar formation. Isolated multiple small-vessel pulmonary artery stenoses are very rare. No surgical procedures for their treatment are currently available. We report on four patients in whom standard and high-pressure balloon angioplasty had failed. Three of the four (2.5-, 3-, and 3.5-years-old; two girls) had isolated multiple peripheral pulmonary artery stenosis. The fourth patient was an 11-month-old girl (8 kg) with tetralogy of Fallot and hypoplastic pulmonary artery branches treated with the implantation of two stents in the pulmonary arteries. During the follow-up this patient developed severe intrastent restenosis and showed severely hypoplasic distal left pulmonary artery. We treated 11 vessels. The mean vessel diameter increased by 81% (P<0.0001) and RV/LV pressure ratio decreased from 1.15 to 0.75 (P=0.05). Patient treated for intrastent restenosis underwent successful complete tetralogy of Fallot repair. None of the patients suffered procedure-related complications. At a median follow-up of 18 months, results were stable and no late complications had occurred. Cutting balloon angioplasty is a promising technique for the treatment of highly challenging pathologies such as small vessel pulmonary artery stenoses and intrastent restenosis.

Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome

We described a case of Alagille syndrome with severe peripheral pulmonary artery stenosis and very high right ventricular pressure that underwent successful living-related liver transplantation without any peri-operative and mid-term postoperative complication because of this cardiac malformation. The aim of this report is to point out that the severe pulmonary artery stenosis may be a risk factor but not a contraindication to liver transplantation in patients with Alagille syndrome.