The Long Shadow of Repair: Late-Onset Atrioventricular Block and Atrial Arrhythmias After Scimitar Syndrome and Mitral Annuloplasty

Background and Clinical Significance: Scimitar Syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return, often requiring early surgical correction. It may coexist with other congenital or acquired cardiovascular anomalies, including valvular diseases such as mitral regurgitation. When surgical correction of Scimitar Syndrome is combined with mitral valve annuloplasty, the proximity to the atrioventricular node may potentially predispose patients to late-onset conduction disturbances, although causality remains speculative. Case Presentation: We describe the case of a 53-year-old male who developed paroxysmal atrial fibrillation, atrial flutter, and intermittent second-degree AV block decades after undergoing surgical correction of Scimitar Syndrome with concomitant mitral annuloplasty. Multimodal echocardiographic evaluation revealed preserved left atrial volume, normal intra-atrial conduction time, mildly reduced strain, and maintained atrial synchrony. The patient was treated with direct oral anticoagulants and beta-blockers and underwent the implantation of a ventricular leadless pacemaker. Conclusions: This case highlights the supportive role of atrial function imaging in assessing atrial health and informing rhythm management and procedural choices in surgically corrected congenital heart disease.

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Publication Details

Journal
Reports — Medical Cases Images and Videos
Published
2025-05-18
DOI
https://doi.org/10.3390/reports8020072
Primary Topic
Congenital Diaphragmatic Hernia Studies
Type
article
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article

The Long Shadow of Repair: Late-Onset Atrioventricular Block and Atrial Arrhythmias After Scimitar Syndrome and Mitral Annuloplasty

Ciro Mauro, Salvatore Crispo, Fulvio Cacciapuoti, Mario Volpicelli et al.
Reports — Medical Cases Images and Videos
Congenital Diaphragmatic Hernia Studies
article

The Long Shadow of Repair: Late-Onset Atrioventricular Block and Atrial Arrhythmias After Scimitar Syndrome and Mitral Annuloplasty

Ciro Mauro, Salvatore Crispo, Fulvio Cacciapuoti, Mario Volpicelli, Gerardo Carpinella
article en

Abstract

Background and Clinical Significance: Scimitar Syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return, often requiring early surgical correction. It may coexist with other congenital or acquired cardiovascular anomalies, including valvular diseases such as mitral regurgitation. When surgical correction of Scimitar Syndrome is combined with mitral valve annuloplasty, the proximity to the atrioventricular node may potentially predispose patients to late-onset conduction disturbances, although causality remains speculative. Case Presentation: We describe the case of a 53-year-old male who developed paroxysmal atrial fibrillation, atrial flutter, and intermittent second-degree AV block decades after undergoing surgical correction of Scimitar Syndrome with concomitant mitral annuloplasty. Multimodal echocardiographic evaluation revealed preserved left atrial volume, normal intra-atrial conduction time, mildly reduced strain, and maintained atrial synchrony. The patient was treated with direct oral anticoagulants and beta-blockers and underwent the implantation of a ventricular leadless pacemaker. Conclusions: This case highlights the supportive role of atrial function imaging in assessing atrial health and informing rhythm management and procedural choices in surgically corrected congenital heart disease.

Reports — Medical Cases Images and VideosVol. 8(2)
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Congenital Diaphragmatic Hernia Studies
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The Long Shadow of Repair: Late-Onset Atrioventricular Block and Atrial Arrhythmias After Scimitar Syndrome and Mitral Annuloplasty — Ciro Mauro, Salvatore Crispo, et al. · Reports — Medical Cases Images and Videos (2025) | TGRS Research Map | TGRS