Rupture Of Pulmonary Artery Aneurysm Research Articles

Idiopathic Pulmonary Artery Aneurysm: A Case Report of a Rare Disease

SESSION TITLE: Fellow Case Report Poster - Obstructive Lung Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary artery aneurysm (PAA) is defined as dilation of the proximal pulmonary artery or one of its branches. It is difficult to diagnose and often is an autopsy finding. It is classified as central, which arises from the main pulmonary artery, or peripheral, which arises from segmental or sub-segmental arteries. Pulmonary hypertension is an important risk factor for development and rupture of PAA. We present a case of idiopathic PAA which has remained stable for past 10 years. CASE PRESENTATION: A 32 year old male with a history of PAA presented to the ER with complaints of atypical chest pain. Physical exam findings and laboratory studies were unremarkable. Contrast-enhanced CT scan of the chest revealed aneurysmal dilation of the right pulmonary artery with a clot in it. This was compared to multiple previous CT scans and did not show any changes. Review of past records revealed that 10 years ago, the patient was admitted for similar complaints, found to have a right hilar mass on X-ray and chest CT suspicious for neoplasm. Thoracotomy was done for biopsy and patient was found to have a 4x3 cm right PAA. Echocardiography showed normal pulmonary artery pressure. On this visit VQ scan was done which was negative for pulmonary embolism. Patient was discharged after 2 days with outpatient follow-up. DISCUSSION: PAA is a rare condition with an incidence of 1 in 14,000 autopsies. Unlike aortic aneurysm it has equal prevalence in both genders and occurs in younger patients. The likely pathology seems to be increased hemodynamic stresses combined with underlying vessel wall weakness. It is commonly associated with congenital heart defects (shunts, PDA or VSD), pulmonary artery hypertension and connective tissue disorders such as Marfan’s syndrome and infections like syphilis and TB. It typically presents with hemoptysis, chest pain, cough and/or dyspnea but is also not infrequently discovered as an incidental finding. The advent of contrast-enhanced CT, MRI and echocardiograms have assumed a primary role in diagnosis. Complications may include pulmonary hypertension, thrombus formation, bronchial compression or aneurysmal rupture. Management is surgical coupled with treatment of the underlying disorders. CONCLUSIONS: Controversy still exists regarding indications for surgical management, with some pursuing conservative approach while others in favor of surgical intervention even in asymptomatic patients. One third of the patients die from rupture of the aneurysm emphasizing the need for follow up and prompt intervention if it enlarges or become symptomatic. Therefore, close follow-up is required. Reference #1: Deterling RA, Clagett OT. Aneurysm of the pulmonary artery: Review of the literature and report of a case. Am Heart J. 1947;34(4):471-499. Reference #2: Arom KV, Richardson JD, Grover FL, Ferris G, Trinkle JK. Pulmonary artery aneurysm. Am Surg. 1978;44(10):688-692. Reference #3: Deb SJ, Zehr KJ, Shields RC. Idiopathic pulmonary artery aneurysm. Ann Thorac Surg. 2005;80(4):1500-1502. DISCLOSURE: The following authors have nothing to disclose: Siddique Chaudhary, Joseph Ramzy, Susan Smith No Product/Research Disclosure Information

Ruptured pulmonary artery aneurysm: a surgical emergency

Idiopathic pulmonary artery aneurysm rupture was diagnosed in a 79-year-old man who presented with a dry cough. He was considered unlikely to tolerate extensive pulmonary artery reconstruction or lung resection; hence, he was salvaged by timely ligation of the distal pulmonary artery at the origin of the aneurysm.

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.

Issue Highlights

HomeCirculationVol. 114, No. 10Issue Highlights Free AccessIn BriefPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessIn BriefPDF/EPUBIssue Highlights Originally published5 Sep 2006https://doi.org/10.1161/circ.114.10.985Circulation. 2006;114:985WILD-TYPE AND MUTANT HCN CHANNELS IN A TANDEM BIOLOGICAL-ELECTRONIC CARDIAC PACEMAKER, by Bucchi et al.andBIOARTIFICIAL SINUS NODE CONSTRUCTED VIA IN VIVO GENE TRANSFER OF AN ENGINEERED PACEMAKER HCN CHANNEL REDUCES THE DEPENDENCE ON ELECTRONIC PACEMAKER IN A SICK-SINUS SYNDROME MODEL, by Tse et al.Advances in technology have improved reliability of pacemaker therapy for bradycardia due to sinus node dysfunction or complete atrioventricular block. However, these electronic devices remain imperfect and have finite battery longevity. Recent manufacturers’ advisories and US Food and Drug Administration recalls due to component failures highlight the need for continued improvement. In this issue of Circulation, 2 separate studies by Bucchi and colleagues and by Tse and colleagues report their findings of experiments using bioengineered pacemaker channel technology. Both groups found a decrease in electronic pacing when constructs with engineered modified HCN channels were transfected into cardiac tissue. Bucchi and colleagues demonstrated that a mutant biological pacemaker implanted in a canine atrioventricular block model conferred catecholamine-sensitive rate improvement and less reliance on electronic pacemaker. Tse and colleagues overexpressed a different mutant HCN channel in a porcine sinus node dysfunction model, and similarly showed that transduction in the atria induced spontaneous automaticity and a catecholamine-responsive physiological heart rate that reduced electronic pacing. Although not yet ready to render pacemakers obsolete, these studies further our understanding of cardiac impulse generation and conduction. These hybrid electronic-biological pacemaker systems illustrate important advances in gene therapy for electrophysiological abnormalities. See p 992 and p 1000 (editorial on p 986).SPECIFIC TEMPORAL PROFILE OF MATRIX METALLOPROTEINASE RELEASE OCCURS IN PATIENTS AFTER MYOCARDIAL INFARCTION: RELATION TO LEFT VENTRICULAR REMODELING, by Webb et al.Experimental studies in animals have shown that matrix metalloproteinases (MMPs) and their endogenous inhibitors, the tissue inhibitors of MMPs (TIMPs), play an important role in regulating myocardial remodeling after myocardial infarction (MI). Webb et al measured plasma levels of MMP-8 and -9 and TIMP-1, -2, and -4 sequentially over the first 180 days after MI to characterize the temporal pattern of MMP and TIMP in this clinical setting. A specific MMP/TIMP temporal profile was found, with early increases in MMP-8 and -9 and a later decrease in TIMP-4. These findings raise the possibility that the MMP/TIMP profile can be used as a biomarker and may yield diagnostic and prognostic information in the post-MI period. See p 1020.DISTRIBUTION OF LIPOPROTEINS BY AGE AND GENDER IN ADOLESCENTS, by Jolliffe and JanssenAtherosclerosis can begin at a young age, and the rate of progression is related to plasma lipoprotein concentrations. Lipoprotein concentrations tend to persist from youth into adulthood, providing further evidence that it is imperative to identify and manage high-risk lipoprotein concentrations at an early age. The current pediatric National Cholesterol Education Program (NCEP) classification system does not recognize that lipoprotein concentrations fluctuate naturally with growth and maturation. Jolliffe and Janssen have devised an age-specific lipoprotein classification system for adolescents. The adolescent thresholds, which were developed using nationally representative data sets, were anchored to the adult NCEP thresholds using growth curve modeling. These adolescent lipoprotein classification systems are presented in a series of growth curves and tables that are practical for both clinical and research settings. It is hoped that the classification systems developed in this study will better identify adolescents with high-risk lipoprotein concentrations; validation studies are needed, however. See p 1056.Visit http://circ.ahajournals.org:Images in Cardiovascular MedicineScimitar Syndrome: Complete Diagnosis by Transthoracic Echocardiography. See p e373. Download figureDownload PowerPointMyocardial Fibroma in Gorlin Syndrome by Cardiac Magnetic Resonance Imaging. See p e376.Large Pulmonary Artery Aneurysm Rupture in Hughes-Stovin Syndrome: Multidetector Computed Tomography Pattern and Endovascular Treatment. See p e380.Book ReviewManual of Vascular Diseases. See p e382.CorrespondenceSee p e383. Previous Back to top Next FiguresReferencesRelatedDetails September 5, 2006Vol 114, Issue 10 Advertisement Article InformationMetrics https://doi.org/10.1161/circ.114.10.985 Originally publishedSeptember 5, 2006 PDF download Advertisement

Rupture of pulmonary artery aneurysm associated with persistent ductus arteriosus.

Fatal haemopericardium occurred in a 14-year-old boy after rupture of a pulmonary artery aneurysm. Persistent ductus arteriosus with severe pulmonary hypertension was confirmed by cardiac catheterisation when he was 4 weeks old. Attempted closure of the ductus at 4 years had not been possible because of apparent high pulmonary resistance. Exercise tolerance had been good enough to permit competitive horse riding up to the day of death. Light and electron microsocpy showed widespread cystic medionecrosis of the elastic pulmonary arteries.