Ignacio Barriuso, Jara Gayán-Ordas, Pablo Pastor-Pueyo
{"title":"[迟做总比不做好:老年患者致心律失常心肌病的评估]。","authors":"Ignacio Barriuso, Jara Gayán-Ordas, Pablo Pastor-Pueyo","doi":"10.24875/ACM.22000070","DOIUrl":null,"url":null,"abstract":"*Correspondence: Ignacio Barriuso E-mail: barriusobarrado@gmail.com Available online: 23-01-2023 Arch Cardiol Mex. 2023;93(3):366-368 www.archivoscardiologia.com Date of reception: 23-02-2022 Date of acceptance: 26-07-2022 DOI: 10.24875/ACM.22000070 A 74-year-old woman without previous medical history except for the left bundle branch block was admitted for evaluation of recurrent syncopes. During admission, she experienced a sustained self-limited monomorphic ventricular tachycardia together with new syncope. Initial echocardiography displayed moderate biventricular systolic dysfunction. Cardiac magnetic resonance imaging (CMRI) confirmed these findings (Fig. 1) and revealed patchy subepicardial areas of late gadolinium enhancement within the left ventricular inferolateral and apical segments (red arrow) and an aneurysm was found in the right ventricular apex, containing a rounded thrombus (blue arrow) which persisted 10 days after intravenous anticoagulation therapy. Due to the clinical suspicion of biventricular arrhythmogenic cardiomyopathy with sustained ventricular arrhythmias, cardioverter-defibrillator (ICD) implantation was decided. Subcutaneous approach, initially preferred due to persistent thrombus, was finally dismissed due to predicted high risk of inappropriate therapies in the screening test. Finally, a transvenous ICD was implanted with defibrillation electrode located in the posterior interventricular vein and left bundle branch pacing (Fig. 2) with a significant reduction of paced QRS duration (Fig. 3) and partial recovery of biventricular function during the follow-up. Subsequently, a genetic study was performed confirming a pathogenic variant in the DSG2 gene, which not only explained the phenotype but also allowed familiar cascade screening (Fig. 4).","PeriodicalId":8360,"journal":{"name":"Archivos de cardiologia de Mexico","volume":"93 3","pages":"366-368"},"PeriodicalIF":0.7000,"publicationDate":"2023-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/92/68/7567AX223-ACM-93-366.PMC10406476.pdf","citationCount":"0","resultStr":"{\"title\":\"[Better late than never: assessment of arrhythmogenic cardiomyopathy in an elderly patient].\",\"authors\":\"Ignacio Barriuso, Jara Gayán-Ordas, Pablo Pastor-Pueyo\",\"doi\":\"10.24875/ACM.22000070\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"*Correspondence: Ignacio Barriuso E-mail: barriusobarrado@gmail.com Available online: 23-01-2023 Arch Cardiol Mex. 2023;93(3):366-368 www.archivoscardiologia.com Date of reception: 23-02-2022 Date of acceptance: 26-07-2022 DOI: 10.24875/ACM.22000070 A 74-year-old woman without previous medical history except for the left bundle branch block was admitted for evaluation of recurrent syncopes. During admission, she experienced a sustained self-limited monomorphic ventricular tachycardia together with new syncope. Initial echocardiography displayed moderate biventricular systolic dysfunction. Cardiac magnetic resonance imaging (CMRI) confirmed these findings (Fig. 1) and revealed patchy subepicardial areas of late gadolinium enhancement within the left ventricular inferolateral and apical segments (red arrow) and an aneurysm was found in the right ventricular apex, containing a rounded thrombus (blue arrow) which persisted 10 days after intravenous anticoagulation therapy. Due to the clinical suspicion of biventricular arrhythmogenic cardiomyopathy with sustained ventricular arrhythmias, cardioverter-defibrillator (ICD) implantation was decided. Subcutaneous approach, initially preferred due to persistent thrombus, was finally dismissed due to predicted high risk of inappropriate therapies in the screening test. Finally, a transvenous ICD was implanted with defibrillation electrode located in the posterior interventricular vein and left bundle branch pacing (Fig. 2) with a significant reduction of paced QRS duration (Fig. 3) and partial recovery of biventricular function during the follow-up. Subsequently, a genetic study was performed confirming a pathogenic variant in the DSG2 gene, which not only explained the phenotype but also allowed familiar cascade screening (Fig. 4).\",\"PeriodicalId\":8360,\"journal\":{\"name\":\"Archivos de cardiologia de Mexico\",\"volume\":\"93 3\",\"pages\":\"366-368\"},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2023-07-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/92/68/7567AX223-ACM-93-366.PMC10406476.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Archivos de cardiologia de Mexico\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.24875/ACM.22000070\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archivos de cardiologia de Mexico","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.24875/ACM.22000070","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
[Better late than never: assessment of arrhythmogenic cardiomyopathy in an elderly patient].
*Correspondence: Ignacio Barriuso E-mail: barriusobarrado@gmail.com Available online: 23-01-2023 Arch Cardiol Mex. 2023;93(3):366-368 www.archivoscardiologia.com Date of reception: 23-02-2022 Date of acceptance: 26-07-2022 DOI: 10.24875/ACM.22000070 A 74-year-old woman without previous medical history except for the left bundle branch block was admitted for evaluation of recurrent syncopes. During admission, she experienced a sustained self-limited monomorphic ventricular tachycardia together with new syncope. Initial echocardiography displayed moderate biventricular systolic dysfunction. Cardiac magnetic resonance imaging (CMRI) confirmed these findings (Fig. 1) and revealed patchy subepicardial areas of late gadolinium enhancement within the left ventricular inferolateral and apical segments (red arrow) and an aneurysm was found in the right ventricular apex, containing a rounded thrombus (blue arrow) which persisted 10 days after intravenous anticoagulation therapy. Due to the clinical suspicion of biventricular arrhythmogenic cardiomyopathy with sustained ventricular arrhythmias, cardioverter-defibrillator (ICD) implantation was decided. Subcutaneous approach, initially preferred due to persistent thrombus, was finally dismissed due to predicted high risk of inappropriate therapies in the screening test. Finally, a transvenous ICD was implanted with defibrillation electrode located in the posterior interventricular vein and left bundle branch pacing (Fig. 2) with a significant reduction of paced QRS duration (Fig. 3) and partial recovery of biventricular function during the follow-up. Subsequently, a genetic study was performed confirming a pathogenic variant in the DSG2 gene, which not only explained the phenotype but also allowed familiar cascade screening (Fig. 4).