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.

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.

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.

Canadian Cardiovascular Society/Canadian Cardiac Transplant Network Position Statement on Heart Transplantation: Patient Eligibility, Selection, and Post-Transplantation Care.

Significant practice-changing developments have occurred in the care of heart transplantation candidates and recipients over the past decade. This Canadian Cardiovascular Society/Canadian Cardiac Transplant Network Position Statement provides evidence-based, expert panel recommendations with values and preferences, and practical tips on: (1) patient selection criteria; (2) selected patient populations; and (3) post transplantation surveillance. The recommendations were developed through systematic review of the literature and using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. The evolving areas of importance addressed include transplant recipient age, frailty assessment, pulmonary hypertension evaluation, cannabis use, combined heart and other solid organ transplantation, adult congenital heart disease, cardiac amyloidosis, high sensitization, and post-transplantation management of antibodies to human leukocyte antigen, rejection, cardiac allograft vasculopathy, and long-term noncardiac care. Attention is also given to Canadian-specific management strategies including the prioritization of highly sensitized transplant candidates (status 4S) and heart organ allocation algorithms. The focus topics in this position statement highlight the increased complexity of patients who undergo evaluation for heart transplantation as well as improved patient selection, and advances in post-transplantation management and surveillance that have led to better long-term outcomes for heart transplant recipients.

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.

Usefulness of cardiac resonance imaging in Churg-Strauss syndrome.

: Churg-Strauss syndrome (CSS) is a rare entity that is characterized by widespread vasculitis, which affects both small and medium-sized blood vessels of nearly all organs. More than 50% of these cases have cardiac involvement, which is the major cause of morbidity and mortality. We describe a case of a patient with cardiac biopsy proven CSS, and we discuss the usefulness of cardiovascular MRI for its diagnosis.

Effects of chronic sildenafil use on pulmonary hemodynamics and clinical outcomes in heart transplantation.

BACKGROUND: Elevated pulmonary vascular resistance (PVR) in heart transplant (HT) candidates is associated with poor survival after HT. This study assessed the effect of peri-operative sildenafil administration on pulmonary hemodynamics and clinical outcomes in patients with advanced heart failure who were considered high-risk for HT because of elevated PVR and transpulmonary gradient (TPG). METHODS: The study included 119 consecutive patients who underwent HT between 2004 and 2011. Fifteen patients (Group A) had severe pulmonary hypertension (PH), defined as mean pulmonary pressure (MPAP)>25 mm Hg and PVR>2.5 Wood units (WU), and/or TPG>12 mm Hg after vasodilator test or the continuous administration of inotropics drugs, and 104 patients (Group B) were without severe PH. Group A received sildenafil therapy. Pulmonary hemodynamics were evaluated before HT with and without sildenafil therapy. Right catheterization was performed early after HT with sildenafil therapy and late after HT without sildenafil. Survival after HT was compared between the groups. RESULTS: The sildenafil dosage was 109±42 mg/day during 163±116 days before HT. After sildenafil therapy MPAP, PVR, and TPG decreased from 43.9±12.5 to 33.4±5.8 mm Hg, 5.0±1.1 to 3.0±1.6 WU, and 17.3±3.2 to 10.2±4.1 mm Hg, respectively (p<.01). All patients underwent successful HT. Sildenafil dosage was 140±70 mg/day for 43±45 days after HT. There were no differences in PVR and TPG with sildenafil therapy early after HT and without sildenafil 6 months after HT. Survival after HT was similar between the groups. CONCLUSION: Sildenafil therapy before and after HT in patients with severe PH is associated with improved pulmonary hemodynamics and successful HT, without an increase in post-HT mortality.

Usefulness and limitations of rituximab in managing patients with lymphoproliferative disorder after heart transplantation.

Posttransplant lymphoproliferative disorders remain an uncommon complication of heart transplant with a high mortality rate reported after conventional therapies. Four patients with posttransplant lymphoproliferative disorders, of whom 3 were CD20 positive, received intravenous dosages of rituximab, 375 mg/m(2), weekly, for 6 ± 2 weeks. The overall response rate was 75% with 3 complete responses (CD20 positive) and 1 case of progressive disease (CD20 negative). Rituximab should be considered as a first-line therapy for patients with CD20 positive posttransplant lymphoproliferative disorders.

Effect of thoracic epidural analgesia on clinical outcomes following transapical transcatheter aortic valve implantation.

OBJECTIVE: To determine the impact of perioperative thoracic epidural analgesia (TEA) on acute and late outcomes following transapical transcatheter aortic valve implantation (TA-TAVI). PATIENTS AND INTERVENTION: A total of 135 consecutive patients who underwent TA-TAVI were included. All patients received catheter-based pain control, either via TEA (TEA group, n=74) or intercostal local analgesia with a catheter placed at the surgical incision site (non-TEA group, n=61), depending on the preference of the anaesthesiologist responsible for the case. MAIN OUTCOME MEASURES: Pain level during early postoperative period (verbal rating scale from 1 to 10), 30-day/in-hospital complications and mortality, and 1-year mortality. RESULTS: There were no differences in baseline or procedural characteristics between groups except for a lower left ventricular ejection fraction in the TEA group. The maximal pain score related to thoracotomy in the postoperative period was higher in the non-TEA group as compared with the TEA group (4 (IQR: 3-5)) vs 2 (IQR: 1-3), p<0.001). Non-TEA was associated with a higher rate of pulmonary complications (p<0.05 for nosocomial pneumonia, reintubation and tracheostomy). The 30-day/in-hospital mortality rate was higher in the non-TEA group (22.9% vs 2.7% in the TEA group, p<0.001). At 1-year follow-up, overall mortality remained higher in the non-TEA group (31.1%) compared with the TEA group (10.8%), p=0.005. Similar periprocedural and late results were obtained in a propensity score-matched analysis that included 100 matched patients. In the multivariable analysis, STS score (p=0.027) and absence of TEA (p=0.039) were independent predictors of increased cumulative late mortality. CONCLUSIONS: TEA provided superior analgesia following TA-TAVI, and was associated with a dramatic reduction in periprocedural respiratory complications, and both, short- and long-term mortality. These results highlight the importance of obtaining optimal analgesia following TA-TAVI to improve the results associated with this procedure.

Splenic infarction associated with a mobile aortic valve lesion: a case report and review of the literature.

The present report describes a 67-year-old woman with a mobile aortic valve lesion discovered by transesophageal echocardiography performed in the setting of abdominal pain. Definitive identification of the lesion was not possible by transesophageal echocardiography alone. Pathological examination of the excised lesion led to the diagnosis of papillary fibroelastoma (PFE). PFEs are rare benign tumours that primarily affect cardiac valves. Because left-sided PFEs are associated with a high risk of systemic embolization including cerebral ischemia and myocardial infarction, they require preventive surgical correction. The present case of PFE is reviewed for a better understanding of its pathology.

Usefulness of Doppler echocardiography guidance during balloon aortic valvuloplasty for the treatment of congenital aortic stenosis.

INTRODUCTION: Few data exist regarding the potential benefits of continuous echocardiographic guidance during balloon aortic valvuloplasty (BAV) for the treatment of congenital aortic stenosis (AS). The objectives of this study were 1) to prospectively evaluate, in a series of consecutive patients with severe AS, the efficacy of BAV guided by Doppler echocardiography (DE) in relieving AS while preventing the appearance of significant aortic regurgitation (AR), and 2) to compare the results obtained by BAV-DE with those obtained in a historical series of patients who underwent BAV without echocardiographic guidance (BAV guided by angiography, BAV-A). METHODS: From 1995 to 2006 a total of 36 consecutive patients with AS (median age 6 years, range, 1 day to 26 years) underwent BAV in our center, with systematic application of continuous DE guidance since 2003. BAV-DE consisted of measuring the aortic annulus, choosing balloon diameters and evaluating the results of each balloon dilation on the basis of DE. RESULTS: Seventeen patients underwent BAV-DE (transthoracic and transesophageal DE in 3 and 14 patients, respectively) with successful transaortic gradient relief in 88% of them. None of the patients complicated with moderate or severe AR. At 17+/-13 months follow-up there had been 3 cardiac events (18%), all of them related to aortic restenosis. BAV-A was associated with longer fluoroscopic times (35 min vs 16 min, p=0.005 after adjusting for age and weight differences between groups) and a higher degree of AR following BAV (>or=2 degrees increase in AR, 32% vs 0%, p=0.045 after adjusting for age and weight). Angiographic measurements of the aortic annulus were higher than those obtained by DE (mean overestimation+2.5+/-1.8 mm, range 0 to +6 mm, p<0.0001). CONCLUSION: BAV-DE provides successful gradient relief of severe AS with lower fluoroscopy time and a lower degree of AR compared to BAV-A. Overestimation of aortic annulus diameters by angiographic measurements might partially explain the high rate of significant AR associated with BAV in the absence of echocardiographic guidance.

Incidental mobile aortic valve lesion: a case of aortic valve fenestration.

The case is reported of a 72-year-old patient with a mobile aortic valve lesion discovered incidentally by echocardiography performed in the setting of coronary artery bypass graft (CABG) surgery. Definitive identification of this lesion was not possible by transthoracic echocardiography or transesophageal echocardiography alone. Pathological examination of the excised aortic valve led to a diagnosis of aortic valve fenestration (AVF) bridging strand rupture. AVFs are common, usually asymptomatic, and of little clinical significance. However, rupture of a bridging strand can produce aortic insufficiency and require surgical correction.