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一名五十多岁男性的不完全性弯刀综合征病例。

A Case of Incomplete Scimitar Syndrome in a Quinquagenarian Male.

作者信息

Franca Hudson P, Sole Priscila, Abdelmoneim Sahar S, Razavian Matthew, Gomez Sabas

机构信息

Internal Medicine, Larkin Community Hospital - Palm Springs Campus, Hialeah, USA.

Medicine, Centro Universitário Lusíada, São Paulo, BRA.

出版信息

Cureus. 2025 Jan 31;17(1):e78315. doi: 10.7759/cureus.78315. eCollection 2025 Jan.

Abstract

Scimitar syndrome is a rare congenital cardiopulmonary condition characterized by an anomalous pulmonary venous return from the right lung to the inferior vena cava (IVC). Most patients are diagnosed during infancy. Incomplete scimitar syndrome refers to a variant, where only a portion of the right lung drains abnormally into the IVC without associated cardiac defects. This case involves a 59-year-old man who presented with severe dizziness and hypertensive emergency with a blood pressure of 234/122 mmHg. His past medical history includes hypertension, cerebrovascular accident, prior myocardial infarction, and diabetes mellitus. A computed tomography (CT) without contrast demonstrated an aberrant connection of the right lower pulmonary vein to the IVC. The patient did not have associated congenital heart defects (incomplete scimitar syndrome). He was conservatively treated for his comorbidities with clinical improvement. Incomplete scimitar syndrome manifests late in adulthood as dyspnea during exertion secondary to the development of pulmonary hypertension. Hence, the cornerstone of treatment for pulmonary hypertension is medical control. By concentrating on the adult appearance of incomplete scimitar syndrome, long-term treatment and care may result from the identification of more individuals with incomplete scimitar syndrome as imaging methods advance and awareness increases. Future longitudinal outcome research for such rare conditions is crucial to fill up these information gaps.

摘要

弯刀综合征是一种罕见的先天性心肺疾病,其特征是右肺异常肺静脉回流至下腔静脉(IVC)。大多数患者在婴儿期被诊断出来。不完全性弯刀综合征是一种变异型,其中只有一部分右肺异常引流至下腔静脉,且无相关心脏缺陷。该病例涉及一名59岁男性,他因严重头晕和高血压急症就诊,血压为234/122 mmHg。他既往有高血压、脑血管意外、既往心肌梗死和糖尿病病史。非增强计算机断层扫描(CT)显示右下肺静脉与下腔静脉存在异常连接。该患者无相关先天性心脏缺陷(不完全性弯刀综合征)。他的合并症接受了保守治疗,临床症状有所改善。不完全性弯刀综合征在成年后期表现为运动时呼吸困难,继发于肺动脉高压的发展。因此,肺动脉高压治疗的基石是药物控制。随着成像方法的进步和认识的提高,通过关注不完全性弯刀综合征的成人表现,可能会识别出更多不完全性弯刀综合征患者,从而实现长期治疗和护理。对于此类罕见疾病,未来的纵向结局研究对于填补这些信息空白至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6b7/11873357/bf7fb9ee6fdc/cureus-0017-00000078315-i01.jpg

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