right ventricular outflow tract obstruction

  • 文章类型: Journal Article
    背景:在心脏手术期间,右心室流出道梗阻(RVOTO)定义为右心室收缩压(RVSP)和肺动脉收缩压(PASP)之间的瞬时压差≥6mmHg,时间≥5分钟。RVOTO的风险因素仍然知之甚少。这项队列研究旨在评估发病率,经历RVOTO的患者的特征和结果。
    方法:在心脏手术期间,使用带右心室端口的肺动脉导管,从回顾性(n=295)和前瞻性(n=105)队列中测量RVSP和PASP之间的瞬时压差。
    结果:从回顾性和前瞻性队列中,开始体外循环(CPB)前RVOTO的发生率分别为30.2和36.2%,CPB分离后为43.7和47.6%.CPB启动前,RVOTO患者的心输出量较高(4.2±1.5vs3.8±1.1L·min-1,P=0.033),接受了更多的吸入依前列醇(79%vs61%,P=0.005)和同向异构体(66vs51%,P=0.016)与没有RVOTO的那些相比。CPB分离后,RVOTO患者的心率更高(62±15vs58±13次搏动·min-1,P=0.011),心输出量(4.1±1.4vs3.7±1.1L·min-1,P=0.003),CPB持续时间(90±45vs77±30分钟,P=0.014),较低的液体平衡(758±1123vs1063±1089mL,P=0.021),并且更多暴露于气管内米力农(12vs4%,P=0.015)与没有RVOTO的那些相比。术后28天持续性器官功能障碍(TPOD)的时间在发生RVOTO事件的患者中相似,CPB开始前或CPB分离后,与那些没有的人相比。
    结论:RVOTO在心脏手术中很常见。然而,它与更长的TPOD无关。
    BACKGROUND: During cardiac surgery, right ventricular outflow tract obstruction (RVOTO) is defined as an instantaneous pressure difference ≥6 mmHg between right ventricular systolic pressure (RVSP) and pulmonary artery systolic pressure (PASP), for ≥5 minutes. Risk factors for RVOTO remain poorly understood. This cohort study is designed to evaluate the incidence, characteristics and outcomes of the patients who experienced RVOTO.
    METHODS: Instantaneous pressure difference between RVSP and PASP was measured using a pulmonary artery catheter with a right ventricular port during cardiac surgery from a retrospective (n=295) and a prospective (n=105) cohort.
    RESULTS: From the retrospective and prospective cohort, incidence of RVOTO was 30.2 and 36.2% before cardiopulmonary bypass (CPB) initiation and 43.7 and 47.6% after CPB separation. Before CPB initiation, patients with RVOTO had higher cardiac output (4.2±1.5 vs 3.8±1.1L⋅min-1, P=0.033), received more inhaled epoprostenol (79 vs 61%, P=0.005) and inotropes (66 vs 51%, P=0.016) compared to those without RVOTO. After CPB separation, patients with RVOTO had higher heart rate (62±15 vs 58±13 beats⋅min-1, P=0.011), cardiac output (4.1±1.4 vs 3.7±1.1L⋅min-1, P=0.003), CPB duration (90±45 vs 77±30mins, P=0.014), lower fluid balance (758±1123 vs 1063±1089mL, P=0.021) and were more exposed to intratracheal milrinone (12 vs 4%, P=0.015) compared to those without RVOTO. The time with persistent organ dysfunction (TPOD) at 28 days after surgery was similar among patients who had a RVOTO event, before CPB initiation or after CPB separation, compared to those who did not.
    CONCLUSIONS: RVOTO is common in cardiac surgery. However, it is not associated with longer TPOD.
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  • 文章类型: Case Reports
    Löffler的心内膜炎(LE)是嗜酸性粒细胞增多综合征中经常遇到的疾病,并且与显着的发病率和死亡率有关。
    一名22岁男子出现急性呼吸困难,反复发作的喘息,咳嗽,导致他入院。多模态诊断成像显示在血栓形成阶段表现为嗜酸性粒细胞介导的心脏损伤。此外,壁血栓和心内膜增厚导致右室流出道(RVOT)严重阻塞和右室心尖完全闭塞,导致右心室心输出量显著减少。该患者被诊断为LE,并接受了大剂量皮质类固醇和抗凝剂治疗。为了缓解RVOT阻塞,通过胸骨正中切开术进行紧急手术干预,以切除壁血栓和切除增厚的心内膜.随后,嗜酸性粒细胞计数在1个月内恢复正常。随访影像学检查显示右心室游离壁内存在残留的增厚心内膜。重要的是,RVOT梗阻完全缓解,未发现壁血栓.值得注意的是,术后3个月的经胸超声心动图检查揭示了右心室心内膜心肌纤维化的显著消退.病人的病情表现出明显的改善,没有观察到不良事件。
    多模态成像对于LE的早期诊断和准确分期至关重要。及时手术干预,结合皮质类固醇治疗,是选择LE患者的有效治疗方法。这种方法对于缓解急性期症状和改善预后至关重要。
    UNASSIGNED: Löffler\'s endocarditis (LE) is a frequently encountered condition in hypereosinophilic syndrome and is associated with a significant morbidity and mortality rate.
    UNASSIGNED: A 22-year-old man presented with acute dyspnoea, recurring wheezing, and cough, leading to his hospital admission. Multimodal diagnostic imaging revealed a manifestation of eosinophil-mediated cardiac injury in the thrombus formation stage. Moreover, a mural thrombus and thickened endocardium had caused severe obstruction of the right ventricular outflow tract (RVOT) and complete obliteration of the right ventricular apex, resulting in a significant reduction in right ventricular cardiac output. The patient received a diagnosis of LE and was treated with high-dose corticosteroids and anticoagulants. To alleviate the RVOT obstruction, an emergency surgical intervention was conducted through median sternotomy to the removal of the mural thrombus and resection of the thickened endocardium. Subsequently, eosinophil counts normalized within 1 month. Follow-up imaging examination demonstrated the existence of a residual section of thickened endocardium within the right ventricular free wall. Importantly, no mural thrombus was detected with complete relief of the RVOT obstruction. Notably, a transthoracic echocardiography examination at the 3-month postoperative unveiled a significant regression in right ventricular endomyocardial fibrosis. The patient\'s condition exhibited tangible improvement, with no adverse events observed.
    UNASSIGNED: Multimodal imaging is essential for the early diagnosis and accurate staging of LE. Timely surgical intervention, combined with corticosteroid therapy, is an effective therapeutic approach in selected patients with LE. This approach is crucial to achieve remission of acute phase symptoms and improve prognosis.
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  • 文章类型: Case Reports
    脑室血管瘤是罕见的良性肿瘤,构成诊断和治疗的复杂性。我们报告了一例52岁女性原发性高血压患者,在高血压门诊就诊期间出现收缩期射血杂音。超声心动图显示高回声肿块阻塞右心室流出道,导致右心房和心室增大,右心室射血分数降低.由于死亡的风险,患者接受了紧急手术切除和三尖瓣置换术。术后恢复顺利,随后的心脏磁共振成像显示射血分数改善,没有残留肿瘤。此病例突出了心室血管瘤的诊断和治疗复杂性。有了这份报告,我们的目标是对脑室血管瘤进行全面的回顾,并加强对这种情况的了解,以改善患者的护理。
    Ventricular hemangiomas are rare benign tumors, pose diagnostic and therapeutic complexities. We report a case of a 52-year-old female with essential hypertension who developed a systolic ejection murmur during a hypertension clinic visit. The echocardiogram revealed a hyperechoic mass obstructing the right ventricular outflow tract, causing enlargement of the right atrium and ventricle, with a reduction in the right ventricular ejection fraction. Due to the risk of death, the patient underwent an emergency surgical resection along with tricuspid valve replacement. Postoperative recovery was uneventful, and subsequent cardiac magnetic resonance imaging showed an improvement in ejection fraction without residual tumor. This case highlights the diagnosis and therapeutic complexities of ventricular hemangiomas. With this report, we aim to provide a comprehensive review of ventricular hemangiomas and to enhance understanding of this condition for improved patient care.
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  • 文章类型: Journal Article
    背景:在大动脉转位和动脉转换手术(TGA-ASO)的患者中,右心室流出道(RVOT)阻塞是一种常见的并发症,需要一种或多种RVOT干预措施。
    目的:我们旨在评估RVOT干预类型分层患者的心肺运动能力和右心室功能。
    方法:TGA-ASO患者(≥16岁)按RVOT干预类型进行分层。包括以下结果参数:预测(%)峰值摄氧量(峰值VO2),三尖瓣环平面收缩期偏移(TAPSE),三尖瓣外侧环收缩速度(TVS'),右心室(RV)-动脉耦合(定义为TAPSE/RV收缩压比),和NT-pro-BNP。
    结果:纳入447例TGA患者,平均年龄为25.0岁(四分位距(IQR)21-29岁)。未进行过RVOT干预的患者(n=338,76%)的预测峰值VO2(78.0±17.4%)明显高于进行单入路导管RVOT干预的患者(73.7±12.7%)。单路手术RVOT介入(73.8±28.1%),多途径RVOT干预患者(66.2±14.0%,p=0.021)。与未进行任何RVOT干预的患者相比,先前进行了基于导管和/或手术的RVOT干预的患者的RV-动脉耦合显着降低(p=0.029)。
    结论:TGA患者在动脉转换修复成功后,运动能力下降。与未接受RVOT干预的患者相比,接受导管或手术RVOT干预的TGA患者的表现明显更差。
    BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions.
    OBJECTIVE: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention.
    METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S\'), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP).
    RESULTS: 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21-29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029).
    CONCLUSIONS: TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions.
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  • 文章类型: Journal Article
    目的表征经胎儿镜选择性激光凝固术(FSLC)治疗的双胎对双胎输血综合征(TTTS)后出生的受者的新生儿血液动力学特征。受者双胞胎出生后第一个月的回顾性分析。在11年内出生的480名新生儿中,138例接受产前FSLC的双胞胎分为四组:无血液动力学损害(NoHI,n=102,74%),孤立性高血压(高血压,n=18,13%),右心室流出道梗阻(RVOTO,n=10,7%),和心力衰竭(CF,n=8,6%)。HighBP(36天(23-54))和CF(44天(18-54))组的FSLC与出生之间的时间(中位数(IQR))明显短于RVOTO(91天(68-112))和NoHi(82天(62-104))组(p<0.001)。结论:根据他们的血流动力学确定了四个不同且特征明确的受者组。大约20%的婴儿发生高血压和心力衰竭,并且与激光凝固和出生之间的时间少于2个月有关。已知:•双子对双子输血综合征(TTTS)的特征在于血液动力学失衡,如果不治疗,则导致高胎儿和新生儿死亡率。出生时没有产前胎儿镜激光凝固术(FSLC)的受者双胞胎中有三分之一发展为危及生命的心力衰竭。新增内容:•已经基于其血液动力学鉴定了具有产前FSLC的受体双胞胎的四个不同组。20%的婴儿发生高血压和心力衰竭,并且与FSLC和出生之间的间隔少于2个月有关。
    To characterize the neonatal hemodynamic profiles in recipients born after twin-to-twin transfusion syndrome (TTTS) treated with fetoscopic selective laser coagulation (FSLC). Retrospective analysis during the first month of life of recipient twins. Of the 480 newborns born during an 11-year period, 138 recipient twins with prenatal FSLC were classified into four groups: no hemodynamic impairment (NoHI, n = 102, 74%), isolated high blood pressure (HighBP, n = 18, 13%), right ventricular outflow tract obstruction (RVOTO, n = 10, 7%), and cardiac failure (CF, n = 8, 6%). The time (median (IQR)) between FSLC and birth was significantly shorter in the HighBP (36 days (23-54)) and CF (44 days (18-54)) groups than in the RVOTO (91 days (68-112)) and NoHi (82 days (62-104)) groups (p < 0.001).     Conclusion: Four distinct and well-characterized groups of recipients were identified based on their hemodynamics. High blood pressure and heart failure occurred in approximately 20% of the infants and were associated with a time between laser coagulation and birth of less than 2 months. What is Known: • Twin-to-twin transfusion syndrome (TTTS) is characterized by a hemodynamic imbalance that leads to high fetal and neonatal mortality if left untreated. One-third of recipient twins born without prenatal fetoscopic laser coagulation (FSLC) develop a life-threatening cardiac failure. What is New: • Four distinct groups of recipient twins with prenatal FSLC have been identified based on their hemodynamics. High blood pressure and cardiac failure occurred in 20% of the infants and were associated with an interval between FSLC and birth of less than 2 months.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    目的:跨环形补片(TAP)右室流出道(RVOT)修复法洛四联症的肺动脉瓣(PV)单峰重建在不同的手术方法中具有不同的临床结果。研究目的是系统地评估单核小叶设计参数如何影响体外瓣膜功能。
    方法:3D打印,在三种婴儿生理条件下测试了疾病特异性RVOT模型。将单孔针缝到模型中,天然主肺动脉(MPA)形成后壁,占z评分为零的PV环和MPA直径的重建周长的40%和50%(天然PVz评分-3.52和-2.99对于BSA0.32m2)。各种小叶自由边缘长度(FEL)(相对于后壁),位置(相对于PVSTJ),和扇贝深度在两个模型中进行了研究。压力梯度,返流,采用描述性统计和回归模型进行分析。
    结果:增加FEL超过MPA后壁的100%降低了梯度,但轻度增加了反流,达到25%的峰值。与STJ放置相比,每个FEL的自由边缘超过STJ2mm,梯度略有增加,而反流没有显着变化。扇贝小叶会影响性能。预折叠小叶改善了移动性并略微降低了梯度。
    结论:平衡梯度,返流,为了增长而扩大规模,一组传单设计已被选择用于临床前评估。40%后壁模型中的小叶宽度为140-160%的设计(50%后壁中的110-120%),位于或超过STJ2毫米,展示了最好的结果。离体测试的下一阶段将另外考虑天然RVOT扩张性,天然小叶相互作用,和TAP特征。
    Pulmonary valve (PV) monocusp reconstruction in transannular patch (TAP) right ventricular outflow tract (RVOT) repair for Tetralogy of Fallot has variable clinical outcomes across different surgical approaches. The study purpose was to systematically evaluate how monocusp leaflet design parameters affect valve function in-vitro.
    A 3D-printed, disease-specific RVOT model was tested under three infant physiological conditions. Monocusps were sewn into models with the native main pulmonary artery (MPA) forming backwalls that constituted 40% and 50% of the reconstructed circumference for z-score zero PV annulus and MPA diameters (native PV z-score - 3.52 and - 2.99 for BSA 0.32m2). Various leaflet free edge lengths (FEL) (relative to backwall), positions (relative to PV STJ), and scallop depths were investigated across both models. Pressure gradient, regurgitation, and coaptation were analyzed with descriptive statistics and regression models.
    Increasing FEL beyond 100% of the MPA backwall decreased gradient but mildly increased regurgitation to a peak of 25%. Positioning the free edge 2 mm past the STJ mildly increased gradient for each FEL without significantly changing regurgitation compared to STJ placement. Scalloping leaflets trivially affected performance. Pre-folding leaflets improved mobility and slightly reduced gradient.
    Balancing gradient, regurgitation, and oversizing for growth, a set of leaflet designs have been selected for pre-clinical evaluation. Designs with leaflet widths 140-160% in the 40% backwall model (110-120% in the 50% backwall), positioned at or 2 mm past the STJ, demonstrated the best results. The next stage of ex-vivo testing will additionally consider native RVOT distensibility, native leaflet interactions, and TAP characteristics.
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  • 文章类型: Case Reports
    在儿童,室间隔缺损是最常见的先天性心脏病。一些室间隔缺损有可能自发闭合。在贫穷国家,基于适应症的关闭可能是不可行的。因此可以观察患者的自然病程。Gasul现象,右心室流出道梗阻,是并发症之一。
    一名7岁儿童反复肺炎,体重增加不良,过度出汗最终使这些症状消失。在婴儿期,超声心动图检查发现了大的膜周部室间隔缺损和小的动脉导管未闭。稍后,患者在右心室流出道获得了一条肌脊。行肌脊切除术,动脉导管未闭和室间隔缺损的闭合。患者病情稳定出院。
    通过早期手术闭合室间隔缺损可以避免右心室流出道阻塞。
    UNASSIGNED: In child, ventricular septal defect is the most prevalent congenital cardiac disease. Some ventricular septal defects have the potential for spontaneous closure. In poor nations closure based on indications may not be feasible. The patient\'s natural course may therefore be observed. The Gasul phenomenon, a right ventricular outflow obstruction, is one of the complications.
    UNASSIGNED: A 7-year-old child who had recurrent pneumonia, poor weight gain, and excessive sweating eventually had these symptoms go away. A large peri membranous ventricular septal defect and a small patent ductus arteriosus was detected on echocardiography during infancy. Later, the patient acquired a muscular ridge across the right ventricular outflow tract. Muscular ridge excision and closure of patent ductus arteriosus and ventricular septal defect were done. Patient was discharged in stable condition.
    UNASSIGNED: Right ventricular outflow tract blockage can be avoided by performing early surgical closure of a ventricular septal defect.
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  • 文章类型: Case Reports
    肺下膜是右室流出道(RVOT)梗阻的罕见原因,只有少数病例报告有或没有相关的室间隔缺损。我们报告了一系列三例肺下膜引起RVOT阻塞的病例。其中两个已经手术(第一个病例是在尝试球囊扩张失败后手术),第三个病例目前正在随访中。
    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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  • 文章类型: Case Reports
    我们描述了一例59岁的肥厚型心肌病妇女,在左心室中室的压力梯度被具有负性肌力作用的药物解决后,仍患有右心室流出道阻塞。该患者30年前被诊断为肥厚型心肌病,仅接受低剂量β受体阻滞剂治疗。由于呼吸困难和胸闷的发展和加剧,她在我们医院就诊,怀疑心力衰竭恶化。经胸超声心动图显示左心室中部加速血流3m/s;因此,她开始服用顺式苯并啉,具有负性肌力作用的药物。入院后,心内压力测量显示左腔无压力梯度.然而,右心室心尖和右心室流出道之间有18mmHg的压力梯度,心脏磁共振成像证实右心室流出道梗阻。我们决定通过添加比索洛尔来加强负性肌力作用,2个月后,主观症状和听诊收缩期杂音均消除。
    肥厚型心肌病(HCM)患者可发生右心室心肌肥厚。然而,右心室流出道梗阻在HCM患者中仍然是罕见的,尽管存在形态学异常,如右心室肥大。在患有HCM的患者中,如果症状和听诊结果与左心室影像学检查结果不一致,应考虑右心室阻塞.
    We describe a case of a 59-year-old woman with hypertrophic cardiomyopathy who remained with right ventricular outflow tract obstruction after the pressure gradient in the left midventricle was resolved by a drug with a negative inotropic effect. The patient was diagnosed with hypertrophic cardiomyopathy 30 years previously and was only on low-dose beta-blocker therapy. She presented at our hospital with suspected exacerbation of heart failure because of the development and exacerbation of dyspnea and chest tightness. Transthoracic echocardiography showed an accelerated blood flow of 3 m/s in the middle of the left ventricle; thus, she was started on cibenzoline, a drug with a negative inotropic effect. After admission, intracardiac pressure measurement showed no pressure gradient in the left chamber. However, there was a pressure gradient of 18 mmHg between the apex of the right ventricle and the right ventricular outflow tract, and right ventricular outflow tract obstruction was confirmed on cardiac magnetic resonance imaging. We decided to reinforce the negative inotropic effect by adding bisoprolol, and the subjective symptoms and auscultatory systolic murmur were eliminated 2 months later.
    UNASSIGNED: Hypertrophy of the right ventricular myocardium can occur in patients with hypertrophic cardiomyopathy (HCM). However, right ventricular outflow tract obstruction remains a rare finding in patients with HCM, despite the presence of morphological abnormalities such as right ventricular hypertrophy. In patients with HCM, obstruction of the right ventricle should be considered if the symptoms and auscultatory findings do not match the left ventricular imaging findings.
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