Concomitant symptomatic cardiac sarcoidosis and systemic sclerosis with cardiac involvement: a case report.

Sarcoidosis and systemic sclerosis are two inflammatory multisystemic disorders of unknown etiology that may be life-threatening especially when there is cardiac involvement. Both diseases may coexist, however, there are very few case reports of patients with both cardiac sarcoidosis and systemic sclerosis in the literature. We report the case of a 72-year-old female who was initially referred for dyspnea. A chest computed tomography scan showed multiple hilar and mediastinal adenopathy with a non-specific opacity in the middle pulmonary lobe. FDG-PET-scan showed increased FDG uptake in the adenopathy, the middle lobe and the right ventricular free wall. Sarcoidosis was confirmed with a lung biopsy. Both electrocardiogram and echocardiogram were normal. Four months later, the patient developed a high-grade atrioventricular block deemed secondary to her cardiac sarcoidosis. Two years later, the patient was referred to a rheumatologist for severe Raynaud's symptoms, sclerodactyly and acrocyanosis. After thorough investigations, a diagnosis of limited cutaneous systemic sclerosis with systemic and cardiac sarcoidosis was made. This case demonstrates that both cardiac sarcoidosis and systemic sclerosis may coexist. In the literature, either disease may come first. In cases where cardiac symptoms appear after the diagnosis of concomitant sarcoidosis and systemic sclerosis, it might be difficult for clinicians to confirm which disease is responsible for the heart involvement. This is important since early cardiac sarcoidosis treatment should be done to prevent major complications and may well differ from systemic sclerosis treatment. In this review, we discuss the main clinical manifestations and imaging findings seen with cardiac disease secondary to sarcoidosis and systemic sclerosis.

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.

Left common carotid artery to left innominate vein arteriovenous fistula after transvenous laser lead extraction.

Transvenous lead extraction (TLE) is used for lead infection, lead debulking, venous recanalization and device upgrades. Lead extraction is performed using specialized tools including locking stylets, mechanical or rotating sheaths, femoral snares or laser sheaths. The most feared complications associated with lead extraction are bleeding, vascular tear, cardiac avulsion and tamponade. Despite technological progress, the incidence of major procedural complications including death remains slightly above 1%. This case depicts an asymptomatic left common carotid artery (LCCA) to left innominate vein arteriovenous fistula (AVF) after laser-assisted TLE successfully treated with an endovascular covered stent.

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.

Usefulness of Left Ventricular Assist Device in the Recovery of Severe Amphetamine-Associated Dilated Cardiomyopathy.

Exposure to synthetic drugs such as amphetamines may lead to significant consequences on the cardiovascular system. The prognosis of young adults with amphetamine-induced cardiomyopathy remains poor. We present 2 young patients (aged <40 years) who developed severe dilated cardiomyopathy after chronic amphetamine use. Thorough psychological evaluations demonstrated favorable features with patients being reliable and committed to their health problems. A left ventricular assist device (HeartMate II; Abbott, Chicago, IL) was implanted in the patients shortly after admission to optimize hemodynamic support and evaluate the potential for recovery. Within less than 1 year, significant improvement was achieved and successful explantation of left ventricular assist device was performed in both patients.

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.

Clinical and echocardiographic presentation of postmyocardial infarction papillary muscle rupture.

BACKGROUND: Severe mitral regurgitation (MR) can occur following myocardial infarction (MI) with either partial or complete papillary muscle rupture (pPMR or cPMR). Although the incidence of this complication has significantly decreased, it is still associated with significant mortality. We sought to evaluate the different echocardiographic and clinical presentations of pPMR and cPMR. METHODS AND RESULTS: A review of all the urgent procedures for ischemic MR between January 2000 and June 2016 was performed to identify patients who underwent surgery for PMR. Surgical protocols and echocardiographic studies were used to identify patients with cPMR and pPMR. A total of 37 patients had cardiac surgery for PMR (18 cPMR, 19 pPMR). All patients with cPMR were in cardiogenic shock at the time of diagnosis, as opposed to only 53% of patients with pPMR (P = 0.0008). Between the time of diagnosis and surgery, 7 patients with pPMR developed cardiogenic shock. Transthoracic echocardiography (TTE) led to the diagnosis in 72% of cPMR and 32% of pPMR (P = 0.02). TEE had a yield of 100% for both cPMR and pPMR. Six pathologic varieties of post-MI PMR were recognized on echocardiography and during surgery. Early postoperative, 1 (72% vs 84%), 3 (67% vs 84%), and 5 years (67% vs 74%) survival rates were similar for cPMR and pPMR (P = 0.26). CONCLUSIONS: Partial PMR is associated with a different clinical and echocardiographic presentation than cPMR. Still, most pPMR patients progress toward cardiogenic shock. Prompt diagnosis and referral for surgery are critical and could potentially decrease mortality.

Intramyocardial Injection of Mesenchymal Precursor Cells and Successful Temporary Weaning From Left Ventricular Assist Device Support in Patients With Advanced Heart Failure: A Randomized Clinical Trial.

Importance: Left ventricular assist device (LVAD) therapy improves myocardial function, but few patients recover sufficiently for explant, which has focused attention on stem cells to augment cardiac recovery. Objective: To assess efficacy and adverse effects of intramyocardial injections of mesenchymal precursor cells (MPCs) during LVAD implant. Design, Setting, and Participants: A randomized phase 2 clinical trial involving patients with advanced heart failure, undergoing LVAD implant, at 19 North American centers (July 2015-August 2017). The 1-year follow-up ended August 2018. Interventions: Intramyocardial injections of 150 million allogeneic MPCs or cryoprotective medium as a sham treatment in a 2:1 ratio (n = 106 vs n = 53). Main Outcomes and Measures: The primary efficacy end point was the proportion of successful temporary weans (of 3 planned assessments) from LVAD support within 6 months of randomization. This end point was assessed using a Bayesian analysis with a predefined threshold of a posterior probability of 80% to indicate success. The 1-year primary safety end point was the incidence of intervention-related adverse events (myocarditis, myocardial rupture, neoplasm, hypersensitivity reactions, and immune sensitization). Secondary end points included readmissions and adverse events at 6 months and 1-year survival. Results: Of 159 patients (mean age, 56 years; 11.3% women), 155 (97.5%) completed 1-year of follow-up. The posterior probability that MPCs increased the likelihood of successful weaning was 69%; below the predefined threshold for success. The mean proportion of successful temporary weaning from LVAD support over 6 months was 61% in the MPC group and 58% in the control group (rate ratio [RR], 1.08; 95% CI, 0.83-1.41; P = .55). No patient experienced a primary safety end point. Of 10 prespecified secondary end points reported, 9 did not reach statistical significance. One-year mortality was not significantly different between the MPC group and the control group (14.2% vs 15.1%; hazard ratio [HR], 0.89; 95%, CI, 0.38-2.11; P = .80). The rate of serious adverse events was not significantly different between groups (70.9 vs 78.7 per 100 patient-months; difference, -7.89; 95% CI, -39.95 to 24.17; P = .63) nor was the rate of readmissions (0.68 vs 0.75 per 100 patient-months; difference, -0.07; 95% CI, -0.41 to 0.27; P = .68). Conclusions and Relevance: Among patients with advanced heart failure, intramyocardial injections of mesenchymal precursor cells, compared with injections of a cryoprotective medium as sham treatment, did not improve successful temporary weaning from left ventricular assist device support at 6 months. The findings do not support the use of intramyocardial mesenchymal stem cells to promote cardiac recovery as measured by temporary weaning from device support. Trial Registration: clinicaltrials.gov Identifier: NCT02362646.

Post esophagectomy diaphragmatic hernia: a case report of a rare cause of acute respiratory distress.

BACKGROUND: Diaphragmatic hernia is frequent among the elderly and is usually associated with mild chronic digestive and respiratory symptoms. CASE PRESENTATION: An elderly post-esophagectomy male patient, in the early postoperative period of cardiac surgery, presented with acute respiratory distress. An emergent surgery was performed to reduce a giant diaphragmatic herniation. CONCLUSIONS: An acute transhiatal herniation can cause serious respiratory impairment; surgical repair should be considered in select patients of cardiac surgery.

Cardiac Implantable Electronic Device Infection: Detailed Analysis of Cost Implications.

BACKGROUND: Infections of cardiac implantable electronic devices (CIED) are associated with significant morbidity and mortality. Despite many preventive measures, this condition is associated with significant costs for the health care system. METHODS: We retrospectively analyzed all infection cases referred for lead extraction at a single university hospital over 1 year (2015-2016). We then calculated all costs related to the infection episode per patient using hospital databases and charts review. RESULTS: Thirty-eight patients with CIED infections (29% women-mean age 71 ± 14 years) were referred for lead extraction (27 pocket infections, 11 endocarditis). Devices were mainly pacemakers (60%). When the pathogen was identified, Staphylococcus aureus methicillin sensitive was the main cause. Extraction was performed in all but 3 cases (92%). One death occurred in the nonextracted group. Respective durations of hospitalization and intravenous and antibiotic administration for patients undergoing extraction were 21 and 36 days. The calculated mean total cost for CIED infection management was CAD$29,907 (median: 26,879; range: CAD$4,827-$62,585). Mean hospital charges were CAD$12,291, accounting for 41% of the total costs. CONCLUSIONS: This study represents the first analysis of the direct costs associated with lead extraction in Canada. Device infections are associated with significant costs and increased morbidity. Any preventive measure will have a significant impact on the economic burden of the health care system and patient outcome after lead extraction.

Pregnancy after heart transplantation: a well-thought-out decision? The Quebec provincial experience - a multi-centre cohort study.

Despite reports of successful pregnancies in heart transplant (HTx) recipients, many centers recommend their patients against maternity. We reviewed our provincial experience of pregnancy in HTx recipients by performing charts review of all known gestations following HTx in the province of Quebec (Canada), stratified between planned and unplanned pregnancies. Long-term survival was compared to HTx recipient women of childbearing age who did not become pregnant. Eighteen pregnancies, 56% unplanned, occurred in eight patients, 10.1 (2.6-27.0) years after HTx. Immunosuppression was CNI-based, with a mean dose increase of 48.3% (tacrolimus) and 26.5% (cyclosporine), without rejection. Cardiometabolic complications were high compared to the general Canadian population, including preeclampsia (15.4% vs. 5.5%), hypertension (38.5% vs. 4.6%), and diabetes (15.4% vs. 5.6%). Mean gestational age was 35.1 (23.4-39.6) weeks (72.2% live births; 53.8% prematurity). Mean birthweight was 2418 (660-3612) g. Serum creatinine increased during pregnancy, becoming significant after delivery (P = 0.0239), and returning to preconception level in all but three patients within a year. After 4.6 (1.2-17.2) years of follow-up, two rejection episodes occurred in one patient. Long-term mortality was similar to overall HTx women (Kaplan-Meier; P = 0.8071). Pregnancy in HTx carries high cardiometabolic complications and decreased kidney function, but is feasible with acceptable outcomes and no impact on mother's survival.

Mitral Annular Calcification and Mitral Valve Replacement: A New Approach.

Mitral valve replacement is technically challenging in the context of mitral annular calcification. A new surgical strategy is described that was used in a 71-year-old obese patient, where intraatrial prosthesis insertion and valve fixation into native uncalcified structures were performed without calcium debridement.

Implication of Inflammation and Epigenetic Readers in Coronary Artery Remodeling in Patients With Pulmonary Arterial Hypertension.

OBJECTIVE: Pulmonary arterial hypertension (PAH) is a vascular disease not restricted to the lungs. Many signaling pathways described in PAH are also of importance in other vascular remodeling diseases, such as coronary artery disease (CAD). Intriguingly, CAD is 4× more prevalent in PAH compared with the global population, suggesting a link between these 2 diseases. Both PAH and CAD are associated with sustained inflammation and smooth muscle cell proliferation/apoptosis imbalance and we demonstrated in PAH that this phenotype is, in part, because of the miR-223/DNA damage/Poly[ADP-ribose] polymerase 1/miR-204 axis activation and subsequent bromodomain protein 4 (BRD4) overexpression. Interestingly, BRD4 is also a trigger for calcification and remodeling processes, both of which are important in CAD. Thus, we hypothesize that BRD4 activation in PAH influences the development of CAD. APPROACH AND RESULTS: PAH was associated with significant remodeling of the coronary arteries in both human and experimental models of the disease. As observed in PAH distal pulmonary arteries, coronary arteries of patients with PAH also exhibited increased DNA damage, inflammation, and BRD4 overexpression. In vitro, using human coronary artery smooth muscle cells from PAH, CAD and non-PAH-non-CAD patients, we showed that both PAH and CAD smooth muscle cells exhibited increased proliferation and suppressed apoptosis in a BRD4-dependent manner. In vivo, improvement of PAH by BRD4 inhibitor was associated with a reduction in coronary remodeling and interleukin-6 expression. CONCLUSIONS: Overall, this study demonstrates that increased BRD4 expression in coronary arteries of patient with PAH contributes to vascular remodeling and comorbidity development.