Persistent right venous valve as a cause of fetal and neonatal pathology from prenatal to postnatal periods: a case report and review.

IF 2.1 3区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS Cardiovascular diagnosis and therapy Pub Date : 2023-12-15 Epub Date: 2023-11-16 DOI:10.21037/cdt-23-288
Dan Liu, Ting Wu, Yu Wang, Ke Dian, Hanmin Liu, Xiaolan He, Jiao Chen
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Abstract

Background: Neonatal cyanosis is a clinical manifestation of hypoxemia and is usually pathological. Persistent right venous valve (PRVV) is a rare cause of cyanosis in newborns and can cause prenatal abnormalities, the clinical significance of which varies depending on the severity of the abnormality. There have been few reports on the intrauterine detection of these abnormalities and their follow-up during infancy. Here, we report a case of PRVV causing supravalvular tricuspid valve (TV) obstruction and secondary right ventricle (RV) hypoplasia. This case is unique in terms of its early prenatal detection, distinct cardiac anomalies, and successful surgery that reversed the symptoms, and the findings offer insights into the diagnosis and management of such rare cardiac conditions.

Case description: We report a case of a newborn diagnosed with PRVV at 31 weeks of gestation at our center. There was no underlying family history of congenital heart disease. Prenatal sonography identified an echogenic membrane in the right atrium, suggesting TV obstruction and subsequent RV hypoplasia. After birth, the neonate suffered hypoxia with decreased arterial oxygen saturation (SaO2). Minimally invasive surgery successfully corrected the membrane. Postoperative SaO2 improved immediately. Three months later, follow-up echocardiography revealed normalized TV and RV dimensions. The patient demonstrated steady progress without any complications. We also reviewed previous cases of PRVV before and after birth and summarized the sonographic and clinically relevant features.

Conclusions: Although PRVV is typically considered as a benign structure, it may lead to significant clinical complications, particularly in fetuses and neonates. The precise identification of its variant forms and related flow patterns is crucial to inform decisions regarding patient management.

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从产前到产后,作为胎儿和新生儿病理原因的持续性右静脉瓣膜:病例报告和综述。
背景:新生儿紫绀是低氧血症的一种临床表现,通常是病理性的。持续性右静脉瓣膜(PRVV)是新生儿发绀的罕见原因,可导致产前畸形,其临床意义因畸形的严重程度而异。有关在宫内发现这些异常并在婴儿期对其进行随访的报道很少。在此,我们报告了一例 PRVV 导致瓣上三尖瓣(TV)阻塞和继发性右心室(RV)发育不良的病例。该病例的独特之处在于其早期产前发现、明显的心脏畸形以及成功逆转症状的手术,其研究结果为此类罕见心脏疾病的诊断和管理提供了启示:我们报告了本中心一例在妊娠31周时被诊断为PRVV的新生儿。该新生儿无先天性心脏病家族史。产前超声检查发现右心房有一层回声膜,提示电视阻塞和随后的 RV 发育不良。新生儿出生后缺氧,动脉血氧饱和度(SaO2)下降。微创手术成功矫正了膜。术后SaO2立即得到改善。三个月后,随访超声心动图显示 TV 和 RV 尺寸恢复正常。患者病情稳定,未出现任何并发症。我们还回顾了以往出生前后的 PRVV 病例,总结了声像图和临床相关特征:尽管 PRVV 通常被认为是一种良性结构,但它可能导致严重的临床并发症,尤其是在胎儿和新生儿中。准确识别其变异形式和相关血流模式对患者管理决策至关重要。
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来源期刊
Cardiovascular diagnosis and therapy
Cardiovascular diagnosis and therapy Medicine-Cardiology and Cardiovascular Medicine
CiteScore
4.90
自引率
4.20%
发文量
45
期刊介绍: The journal ''Cardiovascular Diagnosis and Therapy'' (Print ISSN: 2223-3652; Online ISSN: 2223-3660) accepts basic and clinical science submissions related to Cardiovascular Medicine and Surgery. The mission of the journal is the rapid exchange of scientific information between clinicians and scientists worldwide. To reach this goal, the journal will focus on novel media, using a web-based, digital format in addition to traditional print-version. This includes on-line submission, review, publication, and distribution. The digital format will also allow submission of extensive supporting visual material, both images and video. The website www.thecdt.org will serve as the central hub and also allow posting of comments and on-line discussion. The web-site of the journal will be linked to a number of international web-sites (e.g. www.dxy.cn), which will significantly expand the distribution of its contents.
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