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Extensive Myocardial Fibrosis in a Patient With Hypertrophic Cardiomyopathy and Ventricular Tachycardia Without Traditional High-Risk Features

2009; Lippincott Williams & Wilkins; Volume: 2; Issue: 4 Linguagem: Inglês

10.1161/circimaging.108.824839

1942-0080

Sergio Bongioanni, Paolo Spirito, A. Masi, Amedeo Chiribiri, Rodolfo Bonamini, Maria Rosa Conte,

Cardiac Structural Anomalies and Repair

HomeCirculation: Cardiovascular ImagingVol. 2, No. 4Extensive Myocardial Fibrosis in a Patient With Hypertrophic Cardiomyopathy and Ventricular Tachycardia Without Traditional High-Risk Features Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBExtensive Myocardial Fibrosis in a Patient With Hypertrophic Cardiomyopathy and Ventricular Tachycardia Without Traditional High-Risk Features Sergio Bongioanni, Paolo Spirito, Andrea Sibona Masi, Amedeo Chiribiri, Rodolfo Bonamini and Maria Rosa Conte Sergio BongioanniSergio Bongioanni From the Ospedale degli Infermi (S.B., A.S.M., M.R.C.), Rivoli, Torino, Italy; Ente Ospedaliero Ospedali Galliera (P.S.), Genova, Italy; and Ospedale Molinette (A.C., R.B.), Torino, Italy. , Paolo SpiritoPaolo Spirito From the Ospedale degli Infermi (S.B., A.S.M., M.R.C.), Rivoli, Torino, Italy; Ente Ospedaliero Ospedali Galliera (P.S.), Genova, Italy; and Ospedale Molinette (A.C., R.B.), Torino, Italy. , Andrea Sibona MasiAndrea Sibona Masi From the Ospedale degli Infermi (S.B., A.S.M., M.R.C.), Rivoli, Torino, Italy; Ente Ospedaliero Ospedali Galliera (P.S.), Genova, Italy; and Ospedale Molinette (A.C., R.B.), Torino, Italy. , Amedeo ChiribiriAmedeo Chiribiri From the Ospedale degli Infermi (S.B., A.S.M., M.R.C.), Rivoli, Torino, Italy; Ente Ospedaliero Ospedali Galliera (P.S.), Genova, Italy; and Ospedale Molinette (A.C., R.B.), Torino, Italy. , Rodolfo BonaminiRodolfo Bonamini From the Ospedale degli Infermi (S.B., A.S.M., M.R.C.), Rivoli, Torino, Italy; Ente Ospedaliero Ospedali Galliera (P.S.), Genova, Italy; and Ospedale Molinette (A.C., R.B.), Torino, Italy. and Maria Rosa ConteMaria Rosa Conte From the Ospedale degli Infermi (S.B., A.S.M., M.R.C.), Rivoli, Torino, Italy; Ente Ospedaliero Ospedali Galliera (P.S.), Genova, Italy; and Ospedale Molinette (A.C., R.B.), Torino, Italy. Originally published1 Jul 2009https://doi.org/10.1161/CIRCIMAGING.108.824839Circulation: Cardiovascular Imaging. 2009;2:349–350Although patients with hypertrophic cardiomyopathy (HCM) and without conventional risk factors for sudden death are generally considered at low risk, the magnitude of risk in such patients remains unknown, and additional markers of prognosis may permit more accurate risk stratification.1–3 We report a patient with HCM and without conventional risk factors who had a sustained life-threatening ventricular tachyarrhythmia 6 months after identification of extensive myocardial fibrosis at contrast-enhanced cardiovascular magnetic resonance (CMR).Case DescriptionA 61-year-old asymptomatic patient with HCM had been followed at our institution for 12 years. The patient had none of the conventional risk factors for sudden death. There was no family history of premature HCM-related sudden death, unexplained syncope, or abnormal blood pressure response during exercise. No episode of nonsustained ventricular tachycardia (≥3 beat run) had been documented in any of the 6 ambulatory Holter ECGs recorded during follow-up. The echocardiogram showed a nondilated and hypertrophied left ventricle (LV) with a maximal LV wall thickness of 22 mm at the level of the anterior septum, which had remained unchanged since initial evaluation. The LV outflow gradient was 35 mm Hg at rest. The patient was treated with 50 mg/d atenolol. Recently, a contrast-enhanced CMR was performed as part of routine clinical evaluation. After gadolinium infusion, multiple and extensive areas of myocardial late enhancement were demonstrated, involving 80% of the septum and 45% of the free wall (Figure 1). Six months after CMR, the patient was admitted to our emergency department for intense palpitations associated with lightheadness. A 12-lead ECG showed sustained ventricular tachycardia with a 320-ms cycle and a heart rate of 190 bpm (Figure 2). Blood pressure was 100/70 mm Hg. Given the impending hemodynamic instability, electric cardioversion was performed with prompt restoration of sinus rhythm. Neither release of cardiac enzymes nor electrolyte imbalance was detected. A coronary arteriogram was performed and showed no significant coronary artery stenoses. Because of the episode of sustained ventricular tachycardia, a cardioverter-defibrillator (ICD) was implanted before hospital discharge. Download figureDownload PowerPointFigure 1. Contrast-enhanced CMR images of the left ventricle in short axis at equatorial (A), basal (B), and apical level (C) and in 2-chamber long-axis view (D). Images show areas of delayed enhancement at the level of the interventricular septum and the free wall, indicating the presence of extensive and transmural fibrosis.Download figureDownload PowerPointFigure 2. Twelve-lead ECG shows monomorphic ventricular tachycardia with a 320-ms cycle and a heart rate of 190 bpm.CommentsThe patient reported here shows that the current risk markers may be insufficient to identify patients with HCM at increased risk of sudden death who may be candidates for the ICD. In the absence of any of the acknowledged risk factors, this patient developed a sustained life-threatening ventricular tachyarrhythmia that required electric cardioversion. However, a recent CMR had identified extensive and potentially arrhythmogenic myocardial scarring. Therefore, our experience in this patient suggests that severe myocardial fibrosis detected in vivo by contrast-enhanced CMR could represent an additional marker of increased risk in patients with HCM.DisclosuresNone.FootnotesCorrespondence to Sergio Bongioanni, MD, Ospedale degli Infermi, Strada Rivalta 47, 10098, Rivoli, Torino, Italy. E-mail [email protected] References 1 Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH III, Spirito P, Ten Cate FJ, Wigle ED. Task Force on Clinical Expert Consensus Documents: American College of Cardiology; Committee for Practice Guidelines European Society of Cardiology. J Am Coll Cardiol. 2003; 42: 1687–1713.CrossrefMedlineGoogle Scholar2 Maron BJ, Spirito P, Shen WK, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NA 3rd, Favale S, Piccininno M, Winters SL, Santini M, Betocchi S, Arribas F, Sherrid MV, Buja G, Semsarian C, Bruzzi P. Outcomes of patients with implantable cardioverter-defibrillator for prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA. 2007; 298: 405–412.CrossrefMedlineGoogle Scholar3 Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, Lesser JR, Hanna CA, Udelson JE, Manning WJ, Maron MS. Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance. J Am Coll Cardiol. 2008; 51: 1369–1374.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Gastl M, Gruner C, Labucay K, Gotschy A, Von Spiczak J, Polacin M, Boenner F, Kelm M, Ruschitzka F, Alkadhi H, Kozerke S and Manka R (2020) Cardiovascular magnetic resonance T2* mapping for the assessment of cardiovascular events in hypertrophic cardiomyopathy, Open Heart, 10.1136/openhrt-2019-001152, 7:1, (e001152), Online publication date: 1-Mar-2020. 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Maron M (2012) Clinical Utility of Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy, Journal of Cardiovascular Magnetic Resonance, 10.1186/1532-429X-14-13, 14:1, Online publication date: 1-Dec-2012. Jonnalagadda N, Jain A, Calkins H and Tandri H (2009) Role of cardiac imaging evaluation of patients with documented or suspected ventricular arrhythmias, Journal of Nuclear Cardiology, 10.1007/s12350-009-9168-8, 17:1, (145-152), Online publication date: 1-Feb-2010. July 2009Vol 2, Issue 4 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCIMAGING.108.824839PMID: 19808616 Originally publishedJuly 1, 2009 PDF download Advertisement SubjectsArrhythmiasCatheter Ablation and Implantable Cardioverter-DefibrillatorComputerized Tomography (CT)