Pulmonary Valve Stenosis

肺动脉瓣狭窄
  • 文章类型: Journal Article
    背景:经导管肺动脉瓣置换术(TPVR)的研究显示了良好的临床和血流动力学结果。我们的研究分析了东南亚接受旋律瓣膜植入术患者的中期临床和血流动力学结果。
    方法:招募患有环形导管或生物瓣膜并经历术后右心室流出道(RVOT)功能障碍的患者进行旋律TPVR。
    结果:我们的队列(n=14)在儿童和成人患者之间平均分配。中位年龄为19岁(8-38岁),男女比例为6:1,中位随访期为48个月(16-79个月),最小的病人是一个8岁的男孩,体重18公斤。所有TPVR手术都顺利且成功,没有立即死亡或导管破裂。植入物的主要适应症是合并狭窄和反流。平均导管直径为21±2.3mm。71.4%的无旋律瓣膜支架骨折(MSFs)患者同时进行支架置入术。植入瓣膜尺寸包括22-mm(64.3%),20毫米(14.3%),和18毫米(21.4%)。在TPVR之后,在出院时,整个RVOT的平均梯度从41mmHg(10-48mmHg)显着降低到16mmHg(6-35mmHg),p<0.01。2例(14.3%)被诊断为晚期随访感染性心内膜炎(IE)。在79个月的随访中,IE的总体自由度为86%。三名患者(21.4%)出现进行性RVOT梯度。
    结论:对于东南亚患有RVOT功能障碍的患者,在血流动力学和临床改善方面,旋律TPVR结果与美国患者报告的结果相似。采用支架置入前策略,未观察到MSF。植入后的残余狭窄和RVOT的进行性狭窄需要长期监测和再干预。最后,尽管积极预防和围手术期预防细菌性心内膜炎,IE仍然是一个值得关注的问题。
    BACKGROUND: Studies of transcatheter pulmonary valve replacement (TPVR) with the Melody valve have demonstrated good clinical and hemodynamic outcomes. Our study analyzes the midterm clinical and hemodynamic outcomes for patients who underwent Melody valve implantation in Southeast Asia.
    METHODS: Patients with circumferential conduits or bioprosthetic valves and experiencing post-operative right ventricular outflow tract (RVOT) dysfunction were recruited for Melody TPVR.
    RESULTS: Our cohort (n = 14) was evenly divided between pediatric and adult patients. The median age was 19 years (8-38 years), a male-to-female ratio of 6:1 with a median follow-up period of 48 months (16-79 months), and the smallest patient was an 8-year-old boy weighing 18 kg. All TPVR procedures were uneventful and successful with no immediate mortality or conduit rupture. The primary implant indication was combined stenosis and regurgitation. The average conduit diameter was 21 ± 2.3 mm. Concomitant pre-stenting was done in 71.4% of the patients without Melody valve stent fractures (MSFs). Implanted valve size included 22-mm (64.3%), 20-mm (14.3%), and 18-mm (21.4%). After TPVR, the mean gradient across the RVOT was significantly reduced from 41 mmHg (10-48 mmHg) to 16 mmHg (6-35 mmHg) at discharge, p < 0.01. Late follow-up infective endocarditis (IE) was diagnosed in 2 patients (14.3%). Overall freedom from IE was 86% at 79 months follow-up. Three patients (21.4%) developed progressive RVOT gradients.
    CONCLUSIONS: For patients in Southeast Asia with RVOT dysfunction, Melody TPVR outcomes are similar to those reported for patients in the US in terms of hemodynamic and clinical improvements. A pre-stenting strategy was adopted and no MSFs were observed. Post-implantation residual stenosis and progressive stenosis of the RVOT require long term monitoring and reintervention. Lastly, IE remained a concern despite vigorous prevention and peri-procedural bacterial endocarditis prophylaxis.
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  • 文章类型: Journal Article
    背景:采用现代治疗方案,神经内分泌肿瘤患者的生存率有了显著提高。虽然相关类癌综合征可以早期诊断和有效控制,心脏病学家仍然会遇到有心脏表现的患者,特别是在血管活性介质持续高水平的个体中。治疗选择仅限于完全表现疾病的外科瓣膜置换。因为手术并不总是可行的,经导管瓣膜植入正在成为一个有趣的选择。
    方法:介绍一例50岁女性,患有类癌综合征和右侧心脏瓣膜病。在评估和治疗神经内分泌肿瘤时,诊断为中度肺动脉瓣狭窄和重度三尖瓣关闭不全。罕见瓣膜介入的可能性以及诊断中跨学科合作的必要性,必须强调对产生血管活性物质的神经内分泌肿瘤患者的监测和治疗。
    结论:该患者患有典型的类癌综合征,并伴有罕见的心脏表现。尽管监测和治疗是根据建议进行的,并且适合于临床情况,转移性疾病的快速进展最终排除了侵入性心脏介入治疗。
    BACKGROUND: The survival of patients with neuroendocrine tumors has substantially improved with modern treatment options. Although the associated carcinoid syndrome can be diagnosed early and controlled effectively, cardiologists still encounter patients with cardiac manifestations, particularly among individuals with persistently high levels of vasoactive mediators. Treatment options have been limited to surgical valve replacement in fully manifested disease. Since surgery is not always feasible, transcatheter valve implantation is becoming an interesting alternative.
    METHODS: A case of a 50-year-old woman with carcinoid syndrome and right-sided valvular heart disease is presented. Moderate pulmonary valve stenosis and severe tricuspid valve regurgitation were diagnosed during the evaluation and treatment of neuroendocrine tumor. The possibility of rare valve involvement and the need for interdisciplinary cooperation in the diagnosis, monitoring and treatment of patients with neuroendocrine tumors producing vasoactive substances must be emphasized.
    CONCLUSIONS: The patient had a typically presenting carcinoid syndrome with a rare cardiac manifestation. Although monitoring and treatment were carried out in accordance with recommendations and appropriate to the clinical condition, rapid progression of the metastatic disease ultimately precluded invasive cardiac intervention.
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  • 文章类型: Case Reports
    背景技术先天性心脏病(CHDs)是出生缺陷的最常见形式,影响新生儿心脏的结构和功能。肺动脉瓣狭窄(PVS)和室间隔缺损(VSD)是两种更常见的形式,如果不及时治疗,两者都可能导致显著的发病率。经导管技术的出现彻底改变了治疗领域,提供微创但有效的心脏直视手术替代方案,并显着减少相关患者的发病率和恢复时间。案例报告该案例详细介绍了一名患有复杂CHD的19岁男子的管理,强调导致经导管方法的细微差别决策过程。病人的临床表现,以反映显著心脏受损的症状为标志,要求一种量身定制的方法,利用非手术干预的最新进展。使用Amplatzer装置成功闭合VSD,并通过球囊瓣膜成形术解决PVS,无并发症。展示这些技术在管理类似案件中的潜力。干预后的时期出现了显著的复苏,确认手术疗效并提高患者的生活质量。结论该病例的有利结果突出了经导管介入治疗在治疗复杂CHD中的关键作用,并表明心脏护理转向侵入性较小的方法。这个案例为现有的证据提供了宝贵的见解,加强经导管技术成为首选治疗方式的潜力。有了有希望的即时和短期结果,这些技术凸显了需要继续研究其长期疗效和在不同患者人口统计学中的应用.
    BACKGROUND Congenital heart diseases (CHDs) are the most common form of birth defects, affecting the structure and function of neonatal hearts. Pulmonary valve stenosis (PVS) and ventricular septal defects (VSD) are 2 of the more prevalent forms, both of which can lead to significant morbidity if left untreated. The emergence of transcatheter techniques has revolutionized the therapeutic landscape, presenting minimally invasive yet effective alternatives to open-heart surgery and significantly reducing associated patient morbidity and recovery time. CASE REPORT The presented case details the management of a 19-year-old man with complex CHDs, highlighting the nuanced decision-making process that led to a transcatheter approach. The patient\'s clinical presentation, marked by symptoms reflective of significant cardiac compromise, demanded a tailored approach that utilized the latest advancements in non-surgical intervention. The successful closure of the VSD with an Amplatzer device and the resolution of PVS via balloon valvuloplasty were achieved without complications, showcasing the potential of these techniques in managing similar cases. The post-intervention period was marked by a noteworthy recovery, confirming the procedural efficacy and enhancing the patient\'s quality of life. CONCLUSIONS The favorable outcome of this case highlights the pivotal role of transcatheter interventions in treating complex CHDs and suggests a shift towards less invasive approaches in cardiac care. This case contributes valuable insights to the existing body of evidence, reinforcing the potential of transcatheter techniques to become the preferred treatment modality. With promising immediate and short-term results, these techniques highlight the need for continued research into their long-term efficacy and application across diverse patient demographics.
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  • 文章类型: Case Reports
    背景:总结成功治疗4例危重肺狭窄(CPS)新生儿的围手术期护理经验。
    方法:在4例患者中,3有出生后呼吸急促和不同程度的紫癜,因哭泣和噪音而加剧,1例无明显的呼吸急促和紫癜。术前听诊心前区可听到3-4/6收缩期杂音;超声心动图诊断为CPS,合并动脉导管未闭,右心室发育不良,和严重的三尖瓣反流。4例患儿用前列腺素5ng/(kg·min)维持一定程度的肺血流改善低氧血症,有效防止动脉导管闭合,并且在手术前2小时停止输注。其中三名儿童在手术前需要呼吸机辅助呼吸以缓解严重的缺氧并纠正酸中毒。
    方法:诊断为新生儿CPS。
    方法:4例病情迅速发展的新生儿入院,立即组织了一个多学科的医院会诊,成立了一个多学科合作小组,由医疗部门的医生和护士组成,新生儿重症监护病房,心血管医学,心脏超声室,麻醉科,放射学和介入医学部。多学科小组评估了儿童的治疗方式,最终决定进行经皮球囊肺动脉瓣成形术。手术团队包括心血管内科的专家,介入放射科,心脏超声组,麻醉科。
    结果:4例新生儿均手术成功出院。出院后1年进行多学科随访干预,孩子们都很好。
    结论:专科护理主导的多学科协作模式显著提高了各专科护士的专业能力,促进多专业学科的融合和发展,并为儿童提供更优质的服务,是提高新生儿经皮球囊肺动脉瓣成形术成功率的关键。
    BACKGROUND: Summarizing the perioperative nursing experience in the successful treatment of 4 neonates with critical pulmonary stenosis (CPS).
    METHODS: Of the 4 patients, 3 had postnatal shortness of breath and varying degrees of cyanosis, aggravated by crying and noise, and 1 had no obvious shortness of breath and cyanosis. The preoperative auscultation of the precordial region could be heard 3-4/6 systolic murmur; echocardiography was diagnosed as CPS, combined with patent ductus arteriosus, right ventricular dysplasia, and severe tricuspid regurgitation. Four children were treated with prostaglandin 5 ng/(kg-min) to maintain a certain degree of pulmonary blood flow to improve hypoxemia, effectively preventing ductus arteriosus from closure, and the infusion was discontinued 2 hours prior to the operation. Three of the children required ventilator-assisted respiration to relieve severe hypoxia and correct acidosis before surgery.
    METHODS: Neonatal CPS was diagnosed.
    METHODS: Four neonates with rapidly developing conditions were admitted to the hospital, a multidisciplinary in-hospital consultation was organized immediately, and a multidisciplinary collaborative team was set up, consisting of medical doctors and nurses from the medical department, the neonatal intensive care unit, cardiovascular medicine, cardiac ultrasound room, anesthesiology department, and radiology and interventional medicine department. The multidisciplinary team evaluated the treatment modality of the children and finally decided to perform percutaneous balloon pulmonary valvuloplasty. The surgical team included specialists from the Department of Cardiovascular Medicine, Department of Interventional Radiology, Cardiac Ultrasound Unit, and Department of Anesthesiology.
    RESULTS: All 4 neonates were successfully operated and discharged from the hospital. Multidisciplinary follow-up interventions were carried out 1 year after discharge, and the children were in good condition.
    CONCLUSIONS: The specialty nursing-led multidisciplinary collaboration model significantly improves the professional competence of nurses from various specialties, promotes the integration and development of multispecialty disciplines, and provides better quality services for children, which is the key to improving the success rate of percutaneous balloon pulmonary valvuloplasty in neonates.
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  • 文章类型: Case Reports
    背景:经皮肺动脉瓣植入术已成为功能失调的右心室流出道治疗的一种有吸引力的方法。
    方法:我们描述了一个20岁的白种人男性患者的独特病例,患有复杂的紫红色心脏缺陷,即肺动脉闭锁,在5岁的使用同种异体移植的Rastelli样手术后,先天性纠正了大动脉移位和室间隔缺损。在20岁的时候,由于同种异体移植功能障碍,患者需要经皮肺动脉瓣植入术。尽管冠状动脉异常,右心室流出道着陆区的球囊测试排除了潜在的冠状动脉压迫。然后,介绍后,a旋律瓣膜成功植入肺动脉瓣位置。8年的随访是顺利的。
    结论:这可能是对这种解剖结构的经皮肺动脉瓣植入的首次描述。这样的程序是可行的;然而,由于冠状动脉异常,它需要格外小心,这可能是他们压缩的原因。
    BACKGROUND: Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment.
    METHODS: We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful.
    CONCLUSIONS: This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression.
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  • 文章类型: Case Reports
    胎儿肺动脉瓣成形术(FPV)的麻醉管理很困难,需要仔细考虑母亲和胎儿。关于具体麻醉实施和术中管理的报道很少。我们报告了一例孕妇,该孕妇在妊娠中期接受右美托咪定的腰硬联合麻醉(CSEA)下接受FPV治疗。同时,胎儿经脐静脉麻醉的应用效果最佳。在操作过程中,孕妇的生命体征稳定,无并发症,心内注射肾上腺素纠正了胎儿心动过缓。术后四个月,一个男孩通过足月经阴道分娩活着出生。CSEA可能是FPV手术的合适麻醉方法。然而,维持母体血流动力学稳定,有效的胎儿麻醉,及时的胎儿复苏是必要的。
    Anesthesia management of fetal pulmonary valvuloplasty (FPV) is difficult, requiring careful consideration of both the mother and the fetus. Few reports have been published on specific anesthesia implementation and intraoperative management. We report the case of a pregnant woman who was treated with FPV under combined spinal epidural anesthesia (CSEA) with dexmedetomidine in the second trimester of pregnancy. Meanwhile, the application of fetal anesthesia through the umbilical vein was optimal. During the operation, the vital signs of the pregnant woman were stable with no complications and the fetal bradycardia was corrected by intracardiac injection of epinephrine. Four months postoperatively, a boy was born alive by full-term transvaginal delivery. CSEA may be a suitable anesthesia method for FPV surgery. Nevertheless, maternal hemodynamic stability maintenance, effective fetal anesthesia, and timely fetal resuscitation were necessary.
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  • 文章类型: Case Reports
    我们描述了具有完整室间隔的常见动脉干的超声心动图检查结果,二尖瓣和左心室发育不全,闭锁左心室出口和双侧,肺动脉狭窄不平衡.
    We describe the echocardiographic findings of a common arterial trunk with intact ventricular septum, mitral and left ventricular hypoplasia, atretic left ventricular outlet and bilateral, and unbalanced pulmonary artery stenoses.
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  • 文章类型: Case Reports
    背景:肺动脉狭窄(PS)是一种先天性心脏病(CHDs),具有一系列狭窄。单拓扑(MC)双胞胎患冠心病的风险增加,尤其是双胎输血综合征(TTTS)的获得性CHD。PS/肺闭锁(PA)与TTTS是罕见的巧合。由于母亲年龄的增加和辅助生殖技术的广泛使用,MC双胎妊娠在过去几十年中有所增加。因此,注意这个群体对心脏异常很重要,特别是在有TTTS的双胞胎中。由于心脏血液动力学变化,预计患有TTTS的MC双胞胎会出现多种心脏异常,并且可以通过胎儿镜激光光凝治疗来消除。鉴于出生后治疗的重要性,有必要对PS进行产前诊断。
    方法:我们在此介绍一例生长受限的受体双胞胎中TTTS与PS共存的情况,该双胞胎在新生儿期成功接受了球囊肺动脉瓣成形术。此外,我们在接受药物治疗(普萘洛尔)的瓣膜成形术后检测到漏斗状PS.
    结论:检测患有TTTS的MC双胞胎的获得性心脏异常非常重要,并在出生后进行随访,以确定是否需要在新生儿期进行干预。
    Pulmonary stenosis (PS) is a congenital heart diseases (CHDs) with a spectrum of stenosis. Monochorionic (MC) twins are at increased risk of CHDs, especially acquired CHDs in twin-twin transfusion syndrome (TTTS). PS/Pulmonary atresia (PA) is a rare coincidence with TTTS. MC twin pregnancies have increased in last decades due to increasing in maternal age and extensive use of assisted reproductive technologies. Therefore, attention to this group is important for heart abnormalities, especially in twins with TTTS. Multiple cardiac abnormalities in MC twins with TTTS are to be expected due to cardiac hemodynamic changes and may be eliminated by Fetoscopic laser photocoagulation treatment. Prenatal diagnosis of PS is necessary given the importance of treatment after birth.
    We here present a case of coexistence of TTTS with PS in a growth restricted recipient twin who successfully treated with balloon pulmonary valvuloplasty in neonatal period. Also, we detected infundibular PS after valvuloplasty that treated with medical therapy (propranolol).
    It is important to detect acquired cardiac abnormalities in MC twins with TTTS, and follow them up after birth to determine the need of intervention in neonatal period.
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  • 文章类型: Case Reports
    背景:我们讨论了一例罕见的成年患者,该患者具有涉及主动脉瓣和肺动脉瓣的不同病理,需要手术治疗。
    方法:患者有先天性肺静脉狭窄和手术瓣膜成形术的病史。大约50年后,由于主动脉瓣感染性心内膜炎和肺动脉瓣高度再狭窄,患者接受了复杂的第二次心脏手术。主动脉瓣和肺动脉瓣置换术,以及RVOT的重建和持续性卵圆孔的闭合,followed.术后过程顺利,患者在手术后一周出院。
    结论:由于不同的病理而同时进行肺动脉瓣和主动脉瓣手术很少见,但即使在高龄患者也可以成功进行,并且可以改善生活质量。
    BACKGROUND: We discuss a rare case of an adult patient with different pathologies involving the aortic and pulmonary valves in need of surgery.
    METHODS: The patient had a history of congenital PV stenosis and surgical valvuloplasty. Almost 50 years later the patient underwent a complex second heart surgery due to infective endocarditis of the aortic valve and high-grade restenosis of the pulmonary valve. Replacement of the aortic and pulmonary valve, as well as reconstruction of the RVOT and closure of a persistent foramen ovale, followed. Postoperative course was uneventful and the patient was discharged home a week after surgery.
    CONCLUSIONS: Simultaneous surgery of pulmonary and aortic valves due to different pathologies is rare but can be performed successfully even in advanced age and can improve quality of life.
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    文章类型: Case Reports
    We present the case of a young girl in whom pre-natal echocardiography showed double outlet right ventricle associated with severe infundibular- and pulmonary valve stenosis. The genetic testing has shown a mutation on the LZTR1 gene, which confirms the diagnosis of a Noonan Syndrome, also present in the mother and an elder sister. The infant was born premature at 34 weeks and 5 days of gestational age. During the neonatal period, feeding difficulties are noted linked to oral aversion and exacerbated by difficulties in the mother-child bond. At 1 month of age, the child presented hypoxic spells caused by the infundibular stenosis which required emergency aorto-pulmonary anastomosis placement ensuring sufficient pulmonary blood flow. This anastomosis needed to be replaced by a larger one at 9th month of age. The child is now 4 years old and has undergone a complete surgical correction. The multidisciplinary management englobes not only follow up in cardiology, genetics, neurology, ophthalmology and hematology but also feeding support and psychomotor development support. The socio-economic precariousness of the family leads to a constant assistance to allow the best possible development of the child.
    Nous présentons le cas d’une fillette chez qui le diagnostic de cardiopathie congénitale de type ventricule droit à double issue avec communication interventriculaire, sténose infundibulaire et valvulaire pulmonaire sévères a été posé en période fœtale. Le bilan génétique a montré la présence d’une mutation du gène LZTR1, confirmant le diagnostic d’un syndrome de Noonan que présentent également la maman et une sœur aînée. L’enfant naît prématurément à 34 semaines et 5 jours d’aménorrhée. La période néonatale est marquée par des difficultés alimentaires liées à des troubles de l’oralité exacerbés par un attachement mère-enfant compromis. à l’âge de 1 mois, l’enfant présente des malaises hypoxiques en raison de la sténose infundibulaire nécessitant la mise en place en urgence d’une anastomose aorto-pulmonaire assurant un débit pulmonaire suffisant, anastomose qui devra être remplacée par une plus large à l’âge de 9 mois. La fillette actuellement âgée de 4 ans vient de bénéficier d’une cure chirurgicale complète. Le suivi multidisciplinaire comprend, outre les suivis cardiologique et génétique, le support à l’alimentation entérale, le suivi neurologique, ophtalmologique et hématologique ainsi que le soutien à la psychomotricité. La précarité psycho-socio-économique familiale nécessite une aide constante pour permettre à l’enfant d’évoluer favorablement dans son milieu familial.
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