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“心音五联征”:法洛四联症根治术后远期出现右心衰竭并肺瓣反流

"The Heart Sound Quintet": A Case Report of Right-Sided Heart Failure Due to Free Pulmonary Regurgitation Long After Intracardiac Repair of Tetralogy of Fallot.

机构信息

Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan.

出版信息

Am J Case Rep. 2020 Jul 17;21:e924636. doi: 10.12659/AJCR.924636.

Abstract

BACKGROUND Intracardiac repair of tetralogy of Fallot (TOF) is generally performed during childhood. However, the majority of patients develop the sequelae long after surgical repair, which results in significant right ventricular (RV) dilation, RV myocardial dysfunction, and, ultimately, in right-sided heart failure. CASE REPORT A 52-year-old man was referred to our institution for the evaluation of sudden-onset ventricular tachycardia. His medical history included RV outflow tract reconstruction at 5 years of age. Auscultation revealed a harsh diastolic regurgitant murmur, widely split first heart sound (S₁), and a single second heart sound (S₂), indicating a severely dilated RV due to severe pulmonary regurgitation (PR) and the presence of a non-functioning pulmonary valve. Moreover, the right-sided third heart sound (S₃) and fourth heart sound (S₄) were present, consistent with elevated RV filling pressure and the presence of a non-compliant RV. Eventually, the aforementioned "heart sound quintet" was confirmed using multimodal evaluation as right-sided heart failure with a concomitant severely dilated RV because of complete regression of the pulmonary valve and resultant free PR. CONCLUSIONS We encountered a case with a "heart sound quintet" that was composed of a widely split S₁, single S₂ with a harsh diastolic regurgitant murmur, and right-sided S₃ and S₄ The logical interpretation of the findings from physical examination will contribute to understanding the pathophysiology and aid clinical decision-making.

摘要

背景

法洛四联症(TOF)的心脏内修复通常在儿童时期进行。然而,大多数患者在手术后很长时间都会出现后遗症,导致右心室(RV)明显扩张、RV 心肌功能障碍,并最终导致右心衰竭。

病例报告

一名 52 岁男性因突发室性心动过速到我院就诊。他的病史包括 5 岁时进行 RV 流出道重建。听诊显示严重的舒张期反流性杂音,第一心音(S₁)广泛分裂,第二心音(S₂)单一,表明由于严重的肺动脉瓣反流和无功能肺动脉瓣导致 RV 严重扩张。此外,还存在右侧第三心音(S₃)和第四心音(S₄),提示 RV 充盈压升高,RV 顺应性差。最终,通过多模态评估确认了上述“心音五联征”,即右心衰竭合并严重扩张的 RV,原因是肺动脉瓣完全退化,导致自由的肺动脉瓣反流。

结论

我们遇到了一个病例,存在“心音五联征”,包括 S₁ 广泛分裂、有舒张期反流性杂音的单一 S₂,以及右侧 S₃ 和 S₄。对体格检查结果进行逻辑解释有助于理解病理生理学并辅助临床决策。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fbb6/7386553/7dec3fb48f9f/amjcaserep-21-e924636-g001.jpg

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