Project description:Background While the aneurysms of the membranous septum (AVS) are rare, the possibility that they lead to obstruction is even rarer. To the best of our knowledge, 11 similar cases have been reported since 1982. Case presentation Initially, the five-year-old boy was evaluated for dyspnoea that had been present since birth. He did not receive any medical treatment until the previous year. At the age of four, the transthoracic echocardiography showed a large aneurysm extending to the right ventricular outflow tract (RVOT) and causing RVOT stenosis. Complete surgical resection of the aneurysmal tissue was performed, and the boy was discharged home in satisfactory condition. Conclusions As the occurrence of RVOT obstruction by a membranous ventricular septal aneurysm is very rare, we are reporting the second case in which an aneurysm of the membranous septum dynamically obstructed the RVOT in a child. We are also reviewing all the previously reported similar cases in the literature. Further studies are needed to obtain a more comprehensive understanding of aneurysms of the membranous septum (AVS).
Project description:A 50-year-old man presented with an episode of chest pain. Cardiac magnetic resonance revealed the presence of a large ventricular septal aneurysm partially closing a perimembranous ventricular septal defect, prolapsing into the right ventricular outflow tract, and mimicking a mass. We illustrate the diagnostic approach and management of such ventricular septal aneurysms. (Level of Difficulty: Advanced.).
Project description:A 65-year-old female presenting with worsening dyspnea and notable weight loss were found to have a systolic murmur on physical examination. On workup with computed tomography (CT) angiogram, a solid mass was found extending from the inferior vena cava into the right ventricle. Transesophageal echocardiography demonstrated this mass extension causing right ventricular outflow tract obstruction. After surgical removal, the pathology of the mass was endometrial leiomyosarcoma.
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.
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.
Project description:We present a case of a 58-year-old man for surgical myectomy due to recurrent left ventricular outflow tract (LVOT) obstruction, who had prior transaortic septal myectomy and embolization of the septal branch. On admission, transthoracic echocardiography showed a typical hypertrophic obstructive cardiomyopathy (HOCM) with asymmetric septal hypertrophy, significant LVOT obstruction and severe mitral regurgitation due to the systolic anterior movement of the anterior mitral valve leaflet. We performed a novel procedure of the transapical beating-heart septal myectomy, following which the LVOT obstruction was resolved. And a decreased grade of systolic anterior movement and a reduction in the severity of mitral regurgitation were observed.
Project description:Disease animal models play an extremely important role in preclinical research. Lack of corresponding animal models, many basic research cannot be carried out, and the conclusions obtained are incomplete or even incorrect. Right ventricular (RV) outflow tract (RVOT) obstruction leads to RV pressure overload (PO) and reduced pulmonary blood flow (RPF), which are two of the most important pathophysiological characteristics in pediatric cardiovascular diseases, and seriously affect the survival rate and long-term life quality of many children. Due to the lack of a neonatal mouse model for RVOT obstruction, it is largely unknown how RV PO and RPF regulate postnatal RV and pulmonary development. Thus, many treatment are directly applied from adults despite significant differences in cardiovascular physiology between infants and adults, with limited or even harmful results. Here we firstly introduced a neonatal mouse model of RVOT obstruction by pulmonary artery banding (PAB) on postnatal day 1. PAB induced neonatal RVOT obstruction, RV PO, and RPF. Neonatal RV PO induced cardiomyocyte proliferation, and neonatal RPF induced pulmonary dysplasia, the two features that can not be observed in adult RVOT obstruction. As a result, PAB pups exhibited overall developmental dysplasia, a sign similar to that of children with RVOT obstruction. Since many pediatric cardiovascular diseases are associated with RV PO and RPF, the introduction of neonatal mouse model of RVOT obstruction may greatly enhance our understanding of those diseases, and eventually save or improve the lives of many children. We used C57 mice in this study and divided them into sham and PAB group, each group contain 3 replicates.