Primary Angiosarcoma-A Rare Cause of Right Ventricular Outflow Tract Obstruction: Case Report and Literature Review.
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ABSTRACT:
SUBMITTER: Omair M
PROVIDER: S-EPMC6984992 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Subpulmonary membrane is a rare cause of right ventricular outflow tract (RVOT) obstruction, and only a few case reports exist with or without associated ventricular septal defect. We report a series of three cases with subpulmonary membrane causing RVOT obstruction. Two of these have been operated (the first case operated after unsuccessful attempt at balloon dilatation), and the third case is on follow-up at present.
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Project description:Left ventricular outflow tract obstruction (LVOTO) has been reported with bio-prosthetic and mechanical mitral valves (MV), though it is more common with the former. The obstruction can be dynamic or fixed. We hereby report a case of fixed LVOTO following bio-prosthetic MV replacement (MVR).
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Project description:Right ventricular outflow tract obstruction (RVOTO) is a rare cause of hemodynamic instability in the intensive care unit (ICU) after cardiac surgery. We report the first cases of RVOTO diagnosed in the ICU using continuous right ventricular pressure waveform monitoring. Our 2 cases reflect both mechanical and dynamic causes of obstruction, each of which require different approaches to treatment. Inotrope use can exacerbate RVOTO caused by dynamic etiology, whereas surgery is usually the treatment of choice for mechanical obstructions. Inability to recognize RVOTO or the correct etiology can lead to hemodynamic compromise and poor outcomes.
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Project description:A patient with a previous lung transplant and aortic valve replacement had progressive dyspnea. He presented with subacute tamponade secondary to a loculated pericardial effusion that caused impaired left ventricular filling and outflow tract obstruction secondary to distortion of the mitral valve apparatus. We demonstrate the imaging features of this presentation. (Level of Difficulty: Intermediate.).
| S-EPMC8305648 | biostudies-literature
Project description:BackgroundRight ventricle outflow tract (RVOT) dysfunction is a common long-term complication in adult patients with pulmonary atresia/ventricular septal defect (PA/VSD). Common causes include valve thrombosis, stent fractures, and graft calcification. We present, to the best of our knowledge, the first case of malignant invasion of a Gore-Tex conduit, causing severe right ventricle (RV) failure.Case summaryA 30-year-old woman with a history of PA/VSD with major aortopulmonary collateral arteries (MAPCAs) presented with worsening dyspnoea and exercise intolerance. In infancy, she underwent unifocalization of the right- and left-sided AP collaterals utilizing an 18 and 16 mm Gore-Tex graft, respectively. At age 7, she had surgical repair with VSD patch closure and placement of a 20 mm right ventricle-pulmonary artery (RVPA) homograft connected to a 20 mm Gore-Tex graft with linkage to the previously placed right and left unifocalization grafts. A transthoracic echocardiogram revealed a severely dilated RV and a heavily calcified RVOT conduit with severe stenosis. Cardiac computed tomography showed a stenotic RVPA conduit with calcified mural mass. She underwent surgical revision of the RVPA conduit with thromboendarterectomy of bilateral pulmonary arteries. Pathology of the removed conduit revealed fibrin-associated Epstein-Barr virus-positive diffuse large B-cell lymphoma (FA DLBCL).DiscussionOne prior case report has demonstrated invasion of DLBCL involving an aortic synthetic tube graft. However, malignant invasion of the RVOT Gore-Tex conduit has yet to be reported. Pathological review can be essential in guiding management. Malignant invasion of Gore-Tex conduits is a rare phenomenon, but one that should be closely monitored following repair of the RVOT.
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Project description:BackgroundRight ventricular outflow tract obstruction (RVOTO) is a cause of hemodynamic instability that can occur in several situations, including cardiac surgery, lung transplantation, and thoracic surgery, and in critically ill patients. The timely diagnosis of RVOTO is important because it requires specific considerations, including the adverse effects of positive inotropes, and depending on the etiology, the requirement for urgent surgical intervention.MethodsThe objective of this systematic review and meta-analysis was to determine the prevalence of RVOTO in adult patients, and the distribution of all reported cases by etiology.ResultsOf 233 available reports, there were 229 case reports or series, and 4 retrospective cohort studies, with one study also reporting a prospective cohort. Of 291 reported cases of RVOTO, 61 (21%) were congenital, 56 (19%) were iatrogenic, and 174 (60%) were neither congenital nor iatrogenic (including intracardiac tumour). The mechanism of RVOTO was an intrinsic obstruction in 169 cases (58%), and an extrinsic obstruction in 122 cases (42%). A mechanical obstruction causing RVOTO was present in 262 cases (90%), and 29 cases of dynamic RVOTO (10%) were reported. In the 5 included cohorts, with a total of 1122 patients, the overall prevalence was estimated to be 4.0% (1%-9%).ConclusionsRVOTO, though rare, remains clinically important, and therefore, multicentre studies are warranted to better understand the prevalence, causes, and consequences of RVOTO.
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Project description:BackgroundRight ventricular outflow tract obstruction in patients with congenital heart disease is usually assessed using echocardiographic peak instantaneous gradient at rest. Since right ventricular outflow tract obstruction may change during exercise (dynamic right ventricular outflow tract obstruction), we present a case emphasizing the potential use of exercise cardiac magnetic resonance imaging (CMR).Case summaryWe discuss a 15-year-old patient with repaired mid-ventricular sub-pulmonary stenosis type double-chambered right ventricle causing right ventricular outflow tract obstruction and symptoms on exertion. In this case, exercise CMR imaging provided additional information, allowing adequate surgical planning.DiscussionThe additional value of exercise CMR imaging in a case of right ventricular outflow tract obstruction was described. Although exercise cardiac magnetic resonance imaging did not show a significant increase in peak gradient across the right ventricular outflow tract obstruction, shifting and D-shaping of the interventricular septum with subsequent insufficient left ventricular filling (preload) was observed in the patient with recurrent double-chambered right ventricle. This case demonstrates how exercise CMR imaging can be helpful in the clinical decision beyond standard echocardiographic evaluation by providing additional evidence of adverse haemodynamics during exercise.
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