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A Late Complication in a Surgically Corrected ALCAPA Patient.

ABSTRACT: Late complications in surgically corrected ALCAPA patients are rare. We describe an interesting case of a patient with a thrombosed giant right coronary artery aneurysm which was discovered on a chest X-ray. (Level of Difficulty: Intermediate.).

SUBMITTER: Visser PC

PROVIDER: S-EPMC9207942 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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A Late Complication in a Surgically Corrected ALCAPA Patient.

Visser Pascal C PC Keijzers Mitran M Delewi Ronak R Lobatto Mark E ME Bot Pieter T PT

JACC. Case reports 20220615 12

Late complications in surgically corrected ALCAPA patients are rare. We describe an interesting case of a patient with a thrombosed giant right coronary artery aneurysm which was discovered on a chest X-ray. (<b>Level of Difficulty: Intermediate.</b>). ...[more]

PMID: 35734524

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Project description:BackgroundEbstein's anomaly occurs when there is an apical displacement of the tricuspid valve with septal and posterior valve leaflets tethering. This condition often occurs in association with other congenital, structural, or conduction system diseases, including intracardiac shunts, valvular lesions, arrhythmias, accessory conduction pathways, and first-degree atrioventricular (AV) block. We present for the first time a case of a patient with Ebstein's anomaly who presented with second-degree Mobitz II AV block and was successfully treated with conduction system pacing (CSP) due to her young age and the likelihood of a long-term high percentage of pacing.Case summaryWe present a case of a 42-year-old lady with a background of complex congenital heart disease, including severe pulmonary stenosis, Ebstein anomaly, and atrial septal defect (ASD). She required complex surgical intervention, including tricuspid valve (TV) repair and subsequently replacement, ASD closure, and pulmonary balloon valvuloplasty. She presented to our hospital with symptomatic second-degree Mobitz II AV block (dizziness, shortness of breath, and exercise intolerance) and right bundle branch block (RBBB) on her baseline ECG. Her echocardiogram showed dilated right ventricle (RV) and left ventricle (LV) with low normal LV systolic function. Due to her young age and the likelihood of a long-term high percentage of RV pacing, we opted for CSP after a detailed discussion and patient consent. The distal HIS position is the preferred pacing strategy at our centre. We could not cross the TV with the standard Medtronic C315 HIS catheter, so we had to use the deflectable C304 HIS catheter. Mapping and pacing of the distal HIS bundle were achieved by Medtronic Selectsecure 3830, 69 cm lead. HIS bundle pacing led to the correction of both second-degree Mobitz II AV block and pre-existing RBBB. The implantation was uneventful, and the patient was discharged home the next day without any acute complications.DiscussionDistal HIS pacing is feasible in patients with surgically treated complex Ebstein anomaly and heart block. This approach can normalize the QRS complex with a high probability of preserving or improving LV function.

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A Late Complication in a Surgically Corrected ALCAPA Patient.

Publications

A Late Complication in a Surgically Corrected ALCAPA Patient.

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