Survival, ventricular arrhythmia, and implantable cardioverter-defibrillator usefulness in toxic cardiomyopathy due to substance abuse.

BACKGROUND: Toxic dilated cardiomyopathy (T-DCM) due to substance abuse is now recognized as a potential cause of severe left ventricular dysfunction. The burden of ventricular arrhythmias (VA) and the role of a prophylactic implantable cardioverter-defibrillator (ICD) are not well documented in this population. We aim to assess the usefulness of ICD implantation in a T-DCM cohort. METHODS: Patients younger than 65 years with a left ventricular ejection fraction (LVEF) < 35% followed at a tertiary center heart failure (HF) clinic between January 2003 and August 2019 were screened for inclusion. The diagnosis of T-DCM was confirmed after excluding other etiologies, and substance abuse was established according to the DSM-5 criteria. The composite primary endpoints were arrhythmic syncope, sudden cardiac death (SCD), or death of unknown cause. The secondary endpoints were the occurrence of sustained VA and/or appropriate therapies in ICD carriers. RESULTS: Thirty-eight patients were identified, and an ICD was implanted in 19 (50%) of these patients, only one for secondary prevention. The primary outcome was similar between the two groups (ICD vs. non-ICD; p = 1.00). After a mean follow-up of 33 ± 36 months, only two VA episodes were reported in the ICD group. Three patients received inappropriate ICD therapies. One ICD implantation was complicated with cardiac tamponade. Twenty-three patients (61%) had an LVEF ≥35% at 12 months. CONCLUSION: VA are infrequent in the T-DCM population. The prophylactic ICD benefit was not observed in our cohort. The ideal timing for potential prophylactic ICD implantation in this population needs further studies.

Mixed aortic stenosis and regurgitation: a clinical conundrum.

Mixed aortic stenosis (AS) and aortic regurgitation (AR) is the most frequent concomitant valve disease worldwide and represents a heterogeneous population ranging from mild AS with severe AR to mild AR with severe AS. About 6.8% of patients with at least moderate AS will also have moderate or greater AR, and 17.9% of patients with at least moderate AR will suffer from moderate or greater AS. Interest in mixed AS/AR has increased, with studies demonstrating that patients with moderate mixed AS/AR have similar outcomes to those with isolated severe AS. The diagnosis and quantification of mixed AS/AR severity are predominantly echocardiography-based, but the combined lesions lead to significant limitations in the assessment. Aortic valve peak velocity is the best parameter to evaluate the combined haemodynamic impact of both lesions, with a peak velocity greater than 4.0 m/s suggesting severe mixed AS/AR. Moreover, symptoms, increased left ventricular wall thickness and filling pressures, and abnormal left ventricular global longitudinal strain likely identify high-risk patients who may benefit from closer follow-up. Although guidelines recommend interventions based on the predominant lesion, some patients could potentially benefit from earlier intervention. Once a patient is deemed to require intervention, for patients receiving transcatheter valves, the presence of mixed AS/AR could confer benefit to those at high risk of paravalvular leak. Overall, the current approach of managing patients based on the dominant lesion might be too reductionist and a more holistic approach including biomarkers and multimodality imaging cardiac remodelling and inflammation data might be more appropriate.

Heavy Burden of Toxic Dilated Cardiomyopathy Among Young Adults: A Retrospective Study and Review of the Literature.

BACKGROUND: Dilated cardiomyopathy (DCM) is a well described entity for heart failure (HF) with reduced left ventricular ejection fraction (LVEF). Recently, drugs and other substance of abuse have been recognised as potential triggers for DCM. The aim of this study was to assess the survival in patients ≤ 65 years of age with toxic cardiomyopathy (TCM). Left ventricular remodelling and the potential usefulness of left ventricular assist devices (LVADs) was also assessed. METHODS: This was a single-centre retrospective study from January 2003 to August 2019 of 553 patients ≤ 65 years old with LVEF < 40% at a tertiary-care cardiology centre. RESULTS: A total of 201 patients (36%) had a diagnosis of idiopathic DCM. Further analysis identified 38 patients (19%) for which a TCM was the most likely etiology (amphetamine [50%], cocaine [37%], anabolic steroids [8%], and energy drinks [5%]). Despite a mean LVEF of 17 ± 8% at presentation, most patients (n = 27; 71%) had event-free survival with guideline-directed medical therapy, and 61% (n = 23) recovered an LVEF ≥ 40% after a median follow-up of 21 ± 23 months. Seven patients (18%) required an LVAD and 1 patient (3%) a transplantation. All LVADs were explanted or decommissioned after partial or complete LVEF recovery after a median support time of 11 ± 4 months. CONCLUSIONS: TCM induced by substance abuse is a frequent cause of HF, accounting for almost 20% of patients ≤ 65 years of age with DCM of unknown etiology. Treatment must be tailored on an individual basis. Mechanical circulatory support demonstrated its usefulness in carefully selected patients.

Ten Questions Cardiologists Should Be Able to Answer About Cardiac Sarcoidosis: Case-Based Approach and Contemporary Review.

Sarcoidosis is an inflammatory multisystemic disease of unknown etiology characterized by the formation of noncaseating epithelioid cell granulomas. Cardiac sarcoidosis might be life-threatening and its diagnosis and treatment remain a challenge nowadays. The aim of this review is to provide an updated overview of cardiac sarcoidosis and, through 10 practical clinical questions and real-life challenging case scenarios, summarize the main clinical presentation, diagnostic criteria, imaging findings, and contemporary treatment.

La sarcoïdose est une maladie inflammatoire multisystémique de cause inconnue, caractérisée par la formation de granulomes non caséeux composés de cellules épithélioïdes. La sarcoïdose cardiaque est une pathologie potentiellement mortelle qui demeure, à ce jour, difficile à diagnostiquer et à traiter. L'objectif de cet article est de présenter les données les plus à jour concernant la sarcoïdose cardiaque et de résumer, à l'aide de 10 questions cliniques et de cas réels, la présentation clinique, les critères diagnostiques, les trouvailles à l'imagerie et le traitement contemporain.

Effect of Acute Immunosuppression on Left Ventricular Recovery and Mortality in Fulminant Viral Myocarditis: A Case Series and Review of Literature.

BACKGROUND: Fulminant viral myocarditis (FVM) is a rare cause of cardiogenic shock associated with high morbidity and mortality rates. An inappropriately activated immune system results in severe myocardial inflammation. Acute immunosuppressive therapy for FVM therefore gained in popularity and was described in numerous retrospective studies. METHODS: We conducted an extensive review of the literature and compared it with our single-centre retrospective review of all cases of FVM from 2009-2019 to evaluate the possible effect of acute immunosuppression with intravenous immunoglobulins and/or high dose corticosteroids in patients with FVM. RESULTS: We report on 17 patients with a mean age of 46 ± 15 years with a mean left ventricular ejection fraction (LVEF) of 15 ± 9% at admission. Fourteen (82%) of our patients had acute LVEF recovery to ≥ 45% after a mean time from immunosuppression of 74 ± 49 hours (3.1 days). Extracorporeal membrane oxygenation (ECMO) was required in 35% (6/17) of our patients for an average support of 126 ± 37 hours. Overall mortality was 12% (2/17). No patient needed a long-term left ventricular assist device or heart transplant. All surviving patients achieved complete long-term LVEF recovery. CONCLUSIONS: Our cohort of 17 severely ill patients received acute immunosuppressive therapy and showed a rapid LVEF recovery, short duration of ECMO support, and low mortality rate. Our suggested scheme of investigation and treatment is presented. These results bring more cases of successfully treated FVM with immunosuppression and ECMO to the literature, which might stimulate further prospective trials or a registry.

CONTEXTE: La myocardite virale fulminante (MVF) est une cause rare de choc cardiogénique, un état associé à des taux élevés de morbidité et de mortalité. L'activation inappropriée du système immunitaire entraîne une inflammation grave du myocarde. Le recours à un traitement immunosuppresseur aigu en cas de MVF a donc gagné en popularité et a fait l'objet de nombreuses études rétrospectives. MÉTHODOLOGIE: Nous avons effectué une revue exhaustive de la littérature et comparé nos observations avec les résultats de notre examen rétrospectif de tous les cas de MVF traités dans un même centre entre 2009 et 2019, afin d'évaluer l'effet possible d'une immunosuppression aiguë par des immunoglobulines administrées par voie intraveineuse et/ou par une corticothérapie à forte dose chez les patients présentant une MVF. RÉSULTATS: Nous rapportons les cas de 17 patients dont l'âge moyen était de 46 ± 15 ans et qui avaient une fraction d'éjection ventriculaire gauche (FEVG) moyenne de 15 ± 9 % à l'admission. Chez 14 (82 %) d'entre eux, la FEVG aiguë s'est rétablie à une valeur ≥ 45 % dans les 74 ± 49 heures (3,1 jours) en moyenne après l'administration d'un traitement immunosuppresseur. Un soutien par oxygénation extracorporelle par membrane (ECMO) a dû être administré à 35 % (6/17) des patients, pendant 126 ± 37 heures en moyenne. Le taux global de mortalité s'établissait à 12 % (2/17). Aucun patient n'a eu besoin d'assistance ventriculaire gauche de façon prolongée ni d'une transplantation cardiaque. La FEVG a fini par se rétablir complètement chez tous les patients qui ont survécu. CONCLUSIONS: Les 17 patients gravement malades de notre cohorte qui ont reçu un traitement immunosuppresseur aigu ont vu leur FEVG se rétablir rapidement, n'ont eu besoin d'ECMO que pendant une courte période et ont affiché un faible taux de mortalité. Nous présentons notre algorithme d'investigation et de traitement. Nos résultats s'ajoutent à ceux d'autres études témoignant de l'efficacité du traitement de la MVF par immunosuppression et ECMO, ce qui pourrait stimuler la réalisation de nouveaux essais prospectifs ou l'établissement d'un registre.

Non-invasive diagnostic imaging tests largely underdiagnose cardiac cirrhosis in patients undergoing advanced therapy evaluation: How can we identify the high-risk patient?

BACKGROUND: Patients with liver cirrhosis are generally considered ineligible for isolated cardiac transplantation or left ventricular assist device (LVAD) implantation. The aim of this retrospective study is to explore the diagnostic value of abdominal ultrasound, computed tomography scan (CT scan) and liver-spleen scintigraphy to detect the presence of cirrhosis in patients with advanced heart failure. METHODS: Among 567 consecutive patients who underwent pre-transplantation or LVAD evaluation, 54 had a liver biopsy to rule out cardiac cirrhosis; we compared the biopsy results with the imaging investigations. RESULTS: In about 26% (n = 14) of patients undergoing liver biopsy, histopathological evaluation identified cirrhosis. The respective sensitivity of abdominal ultrasound, CT scan and liver-spleen scintigraphy to detect cirrhosis was 57% [29-82], 50% [16-84], and 25% [3-65]. The specificity was 80% [64-91], 89% [72-98], and 44% [20-70], respectively. CONCLUSION: Ultrasonography has the best-combined sensitivity and specificity for the diagnosis of cirrhosis. However, more than a third of patients with cirrhosis will go undiagnosed by conventional imaging. As liver biopsy is associated with a low rate of complication, it should be considered in patients with a high-risk of cirrhosis or with evidence of portal hypertension to assess their eligibility for heart transplantation or LVAD implantation.

Do Energy Drinks Really Give You Wings? Left Ventricular Assist Device Therapy as a Bridge to Recovery for an Energy Drink-Induced Cardiomyopathy.

The deleterious effect of energy drinks is increasingly recognized. We present a 26-year-old woman with inotrope-dependent severe dilated cardiomyopathy, potentially caused by chronic ingestion of energy drinks. The results of extensive investigation-consisting of cardiac magnetic resonance, F-18-fluorodesoxyglucose-positron emission tomography, coronary angiography, and endomyocardial biopsy-were normal. A left ventricular assist device (LVAD) was implanted as a potential bridge to recovery. After 10 months of mechanical support and pharmacological treatment, cardiac function was recovered, and the LVAD was successfully explanted. This is the first case report of energy drink abuse leading to severe heart failure requiring mechanical support for recovery.