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1.
Am J Cardiovasc Dis ; 14(2): 121-127, 2024.
Article in English | MEDLINE | ID: mdl-38764547

ABSTRACT

Arrhythmogenic right ventricular cardiomyopathy is an important differential diagnosis in young patients presenting with palpitations and/or dyspnea and must be appropriately investigated. A 23-year-old man presented with cardiogenic shock and monomorphic ventricular tachycardia. He reported palpitations and progressive dyspnea for more than two years, but those symptoms were attributed to anxiety without any further investigation by his family physician. Investigations after the catastrophic presentation in our center suggested terminal right-sided heart failure with severe hepatic insufficiency and acute kidney injury. The patient benefited from extracorporeal membrane oxygenation, followed by an urgent heart transplant 16 days later after the exclusion of liver cirrhosis. Histopathologic analysis of the explanted heart confirmed arrhythmogenic cardiomyopathy.

2.
Article in English | MEDLINE | ID: mdl-38491744

ABSTRACT

A 40-year-old man, newly diagnosed with cardiac sarcoidosis (CS) presented with symptomatic ventricular tachycardia three days after starting steroid-based immunosuppressive therapy (IT). There was no clear guideline indication for implantable cardioverter-defibrillator (ICD) before the initiation of IT. Shortly after ICD implantation and the initiation of anti-arrhythmic drugs, recurring ventricular arrhythmias required titration of the anti-arrhythmic drug therapy. One-year follow-up assessment showed no significant arrhythmias and complete PET scan FDG uptake suppression. This case, along with recent publications, suggests transient pro-arrhythmic effects of steroids in patients with CS, which are not appropriately addressed in the current guidelines. We believe ICD implantation should be considered in clinically manifest CS before initiating IT, particularly in cases with heterogeneous and/or extensive FDG uptake on PET scans.

4.
Am J Cardiol ; 213: 132-139, 2024 Feb 15.
Article in English | MEDLINE | ID: mdl-38114044

ABSTRACT

Tachycardia-induced cardiomyopathy is defined as a reversible left ventricular (LV) systolic dysfunction (SeD) resulting from a sustained fast heart rate. LV remodeling in patients with severe LV dysfunction at diagnosis remains poorly understood. In this retrospective cohort study, we described LV remodeling in 50 patients who underwent atrial flutter ablation. These patients were divided into severe LV SeD (LV ejection fraction [EF] ≤30%) and LV nonsevere SeD (LVEF 31% to 50%) at baseline. All continuous variables are expressed as median and interquartile range. LVEF was 18% (13 to 25) and 38% (34 to 41) in the SeD (n = 29) and LV nonsevere SeD (n = 21) groups, respectively. At baseline, patients with SeD had higher LV end-diastolic diameter (56 [54 to 59] vs 49 mm [47 to 52], p <0.01), LV end-systolic diameter (48 [43 to 51] vs 36 mm [34 to 41], p <0.01), LV end-diastolic volume (71 [64 to 85] vs 56 ml/m2 [46 to 68], p <0.01), LV end-systolic volume (56 [53 to 70] vs 36 ml/m2 [27 to 42], p <0.01), and lower tricuspid annular plane systolic excursion (12 [10 to 13] vs 16 mm [13 to 19], p <0.01). At last follow-up, LVEF was not statistically significantly different between groups. However, LV end-systolic diameter (36 [34 to 39] vs 32 mm [32 to 34], p = 0.01) and LV end-systolic volume (29 [26 to 35] vs 25 ml/m2 [20 to 29], p = 0.02) remained larger in the SeD group. Seven patients (14%), all from the SeD group, had a LVEF ≤35% 2 months after rhythm control, and reverse remodeling was observed up to 9 months. In conclusion, more than half of patients with tachycardia-induced cardiomyopathy and atrial flutter had LVEF ≤30% at baseline. LVEF recovery and LV remodeling were observed beyond 2 months, highlighting the importance of rhythm control and early guideline-directed medical therapy in these patients.


Subject(s)
Atrial Flutter , Cardiomyopathies , Catheter Ablation , Ventricular Dysfunction, Left , Humans , Atrial Flutter/complications , Atrial Flutter/surgery , Retrospective Studies , Cardiomyopathies/complications , Ventricular Dysfunction, Left/etiology , Ventricular Function, Left , Stroke Volume , Tachycardia , Ventricular Remodeling/physiology
5.
Am J Cardiovasc Dis ; 13(4): 283-290, 2023.
Article in English | MEDLINE | ID: mdl-37736350

ABSTRACT

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.

6.
Pacing Clin Electrophysiol ; 46(7): 645-656, 2023 07.
Article in English | MEDLINE | ID: mdl-37247219

ABSTRACT

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.


Subject(s)
Cardiomyopathies , Cardiomyopathy, Dilated , Defibrillators, Implantable , Substance-Related Disorders , Humans , Defibrillators, Implantable/adverse effects , Stroke Volume , Ventricular Function, Left , Arrhythmias, Cardiac/complications , Cardiomyopathies/therapy , Cardiomyopathies/complications , Death, Sudden, Cardiac/etiology , Cardiomyopathy, Dilated/therapy , Substance-Related Disorders/complications , Risk Factors , Treatment Outcome
8.
Am J Med Genet A ; 191(6): 1508-1517, 2023 06.
Article in English | MEDLINE | ID: mdl-36864778

ABSTRACT

Variants of filamin C (FLNC) have been identified as rare genetic substrate for hypertrophic cardiomyopathy (HCM). Data on the clinical course of FLNC-related HCM are conflicting with some studies suggesting mild phenotypes whereas other studies have reported more severe outcomes. In this study, we present a novel FLNC variant (Ile1937Asn) that was identified in a large family of French-Canadian descent with excellent segregation data. FLNC-Ile1937Asn is a novel missense variant characterized by full penetrance and poor clinical outcomes. End stage heart failure requiring transplantation occurred in 43% and sudden cardiac death in 29% of affected family members. Other particular features of FLNC-Ile1937Asn include an early disease onset (mean age of 19 years) and the development of a marked atrial myopathy (severe biatrial dilatation with remodeling and multiple complex atrial arrhythmias) that was present in all gene carriers. The FLNC-Ile1937Asn variant is a novel, pathogenic mutation resulting in a severe form of HCM with full disease penetrance. The variant is associated with a high proportion of end-stage heart failure, heart transplantation, and disease-related mortality. Close follow-up and appropriate risk stratification of affected individuals at specialized heart centers is recommended.


Subject(s)
Atrial Fibrillation , Cardiomyopathy, Hypertrophic , Cardiomyopathy, Restrictive , Heart Failure , Humans , Cardiomyopathy, Restrictive/genetics , Mutation , Filamins/genetics , Canada , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/genetics , Heart Failure/genetics
9.
Clin Transplant ; 37(2): e14869, 2023 02.
Article in English | MEDLINE | ID: mdl-36447131

ABSTRACT

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterized by fibrofatty myocardial replacement, and accurate diagnosis can be challenging. The clinical course of patients expressing a severe phenotype of the disease needing heart transplantation (HTx) is not well described in the literature. Therefore, this study aims to describe the clinical and echocardiographic evolution of patients with ACM necessitating HTx. METHODS: We retrospectively studied all patients who underwent HTx in our institution between 1998 and 2019 with a definite diagnosis of ACM according to the explanted heart examination. RESULTS: Ten patients with confirmed ACM underwent HTx. Only four of them had a diagnosis of ACM before HTx. These patients were 28 ± 15 years old at the time of their first symptoms. Patients received a diagnosis of heart failure (HF) after 5.9 ± 8.7 years of symptom evolution. The mean age at transplantation was 40 ± 17 years old. All the patients experienced ventricular tachycardia (VT) at least once before their HTx and 50% were resuscitated after sudden death. The mean left ventricular ejection at diagnosis and before transplantation was similar (32% ± 21% vs. 35.0% ± 19.3%, p = NS). Right ventricular dysfunction was present in all patients at the time of transplantation. CONCLUSION: Patients with ACM necessitating HTx show a high burden of ventricular arrhythmias and frequently present a biventricular involvement phenotype, making early diagnosis challenging. HF symptoms are the most frequent reason leading to the decision to transplant.


Subject(s)
Arrhythmogenic Right Ventricular Dysplasia , Heart Transplantation , Humans , Retrospective Studies , Arrhythmogenic Right Ventricular Dysplasia/diagnostic imaging , Arrhythmogenic Right Ventricular Dysplasia/etiology , Arrhythmias, Cardiac/etiology , Echocardiography , Heart Transplantation/adverse effects
10.
CJC Open ; 4(11): 913-920, 2022 Nov.
Article in English | MEDLINE | ID: mdl-36444364

ABSTRACT

Background: Peripartum cardiomyopathy (PPCM) is associated with severe morbidity and mortality, and the significance of right ventricular (RV) involvement is unclear. We sought to determine whether RV systolic dysfunction or dilatation is associated with adverse clinical outcomes in women with PPCM. Methods: We conducted a multicentre retrospective cohort study examining the association between echocardiographic RV systolic dysfunction or dilatation at the time of PPCM diagnosis and clinical outcomes. Clinical endpoints of interest were the need for mechanical support, recovery of left ventricular ejection fraction at follow-up, and a combined endpoint of hospitalization for heart failure, cardiac transplant, or death. Results: A total of 67 women, median age 30 years (interquartile range: 7), were diagnosed with PPCM between 1994 and 2015 in 17 participating centres. Twin pregnancies occurred in 11%; 62% of women were multiparous; and 24% had preeclampsia. RV systolic function was impaired in 18 (27%) and dilated in 8 (12%). Seven women required ventricular assistance, and 8 experienced the composite outcome during follow-up (25 [interquartile range 61] months). RV dysfunction was associated with the need for mechanical support (odds ratio 10.10 (95% confidence interval: 1.86-54.81), P = 0.007), but neither RV dysfunction nor dilatation was associated with left ventricular ejection fraction recovery, the need for cardiac transplant, heart failure hospitalization, or death. Conclusions: RV dysfunction is associated with the need for mechanical support in women with PPCM. These findings may improve risk stratification of complications and clinical management.


Introduction: La cardiomyopathie du péripartum (CMP-PP) est associée à la morbidité grave et à la mortalité, mais on ignore l'importance de l'atteinte ventriculaire droite (VD). Nous avons cherché à déterminer si la dysfonction systolique ou la dilatation VD sont associées aux résultats cliniques défavorables chez les femmes atteintes de CMP-PP. Méthodes: Nous avons mené une étude de cohorte rétrospective multicentrique sur l'association entre la dysfonction systolique ou la dilatation VD à l'échographie au moment du diagnostic de CMP-PP et les résultats cliniques. Les critères cliniques d'intérêt étaient la nécessité d'une assistance mécanique, la récupération de la fraction d'éjection ventriculaire gauche (FEVG) au suivi et un critère combiné d'hospitalisation liée à l'insuffisance cardiaque (IC), la transplantation cardiaque ou la mort. Résultats: Un total de 67 femmes, dont l'âge médian était de 30 ans (écart interquartile [EI] : 7), ont reçu un diagnostic de CMP-PP entre 1994 et 2015 dans 17 centres participants. Les grossesses gémellaires sont survenues chez 11 % ; 62 % de femmes étaient multipares ; et 24 % souffraient de prééclampsie. La fonction systolique VD était compromise chez 18 (27 %) femmes et le VD, dilaté, chez huit (12 %) femmes. Sept femmes ont eu besoin d'une assistance ventriculaire, et huit ont subi le critère composite durant le suivi (25 [EI : 61] mois). La dysfonction VD a été associée à la nécessité d'une assistance mécanique (rapport de cotes 10,10 [intervalle de confiance à 95 % : 1,86-54,81], P = 0,007), mais ni la dysfonction ni la dilatation VD n'ont été associées à la récupération de la FEVG, à la nécessité d'une transplantation cardiaque, à une hospitalisation liée à l'IC ou à la mort. Conclusions: La dysfonction VD est associée à la nécessité d'une assistance mécanique chez les femmes atteintes de CMP-PP. Ces conclusions peuvent permettre d'améliorer la stratification des risques de complications et la prise en charge clinique.

11.
J Card Surg ; 37(11): 3598-3606, 2022 Nov.
Article in English | MEDLINE | ID: mdl-36104934

ABSTRACT

BACKGROUND: Atrial fibrillation (AF) is common in patients with reduced left ventricle ejection fraction (RLVEF). The impact of concomitant surgical atrial fibrillation ablation (SAFA) in patients with RLVEF is uncertain. The purpose of this study was to assess the outcomes of concomitant SAFA in patients with RLVEF undergoing heart surgery on heart failure (HF) rehospitalization and mortality. METHODS: Using a local registry and electronic health records linked with provincial civil register survival data from July 2002 to April 2019, we analyzed treatment and outcomes in a cohort of patients with AF and HF defined by left ventricle ejection fraction (LVEF) ≤40%. Health records were used to collect treatment and International Classification of Diseases (ICD 10) codes to determine outcomes. A negative binomial model was used to compare outcomes such as all-cause mortality and rehospitalization for heart failure. RESULTS: The cohort included 682 patients with RLVEF and AF who underwent coronary artery bypass graft and/or valve surgery. A total of 196 patients (29%) underwent concomitant SAFA. After matching, 132 patients with concomitant SAFA were compared to 159 patients who did not undergo concomitant SAFA. At 6.0 ± 3.7 years of follow-up, concomitant SAFA was not associated with lower all-cause mortality (p = .9861) and reduction in rehospitalizations for heart failure decompensation (p = .31) compared to patients who did not have concomitant SAFA performed. Postoperatively, concomitant SAFA might be associated with less vasopressor and mechanical support use (p = .01). CONCLUSIONS: Concomitant SAFA during index cardiac surgery is safe but does not reduce mortality or rehospitalizations for HF. The effects of concomitant SAFA in the context of RLVEF need to be better studied with prospective trials.


Subject(s)
Atrial Fibrillation , Catheter Ablation , Heart Failure , Atrial Fibrillation/complications , Atrial Fibrillation/surgery , Catheter Ablation/adverse effects , Heart Failure/surgery , Heart Ventricles/surgery , Humans , Prospective Studies , Stroke Volume , Treatment Outcome , Ventricular Function, Left
13.
Curr Opin Cardiol ; 37(4): 380-387, 2022 07 01.
Article in English | MEDLINE | ID: mdl-35731683

ABSTRACT

PURPOSE OF REVIEW: Cardiac sarcoidosis (CS) is a potentially fatal condition when unrecognized or not treated adequately. The purpose of this review is to provide new strategies to increase clinical recognition of CS and to present an updated overview of the immunosuppressive treatments using most recent data published in the last 18 months. RECENT FINDINGS: CS is an increasingly recognized pathology, and its diagnostic is made 20 times more often in the last two decades. Recent studies have shown that imaging alone usually lacks specificity to distinguish CS from other inflammatory cardiomyopathies. However, imaging can be used to increase significantly diagnostic yield of extracardiac and cardiac biopsy. Recent reviews have also demonstrated that nearly 25% of patients will be refractory to standard treatment with prednisone and that combined treatment with a corticosteroid-sparing agent is often necessary for a period that remains undetermined. SUMMARY: CS is a complex pathology that should always require a biopsy attempt to have a histological proven diagnosis before starting immunosuppressive therapy consisting of corticosteroids with or without a corticosteroid-sparing agent.


Subject(s)
Cardiologists , Cardiomyopathies , Myocarditis , Sarcoidosis , Adrenal Cortex Hormones/therapeutic use , Cardiomyopathies/diagnosis , Cardiomyopathies/drug therapy , Humans , Retrospective Studies , Sarcoidosis/diagnosis , Sarcoidosis/drug therapy
14.
Can J Cardiol ; 38(1): 49-58, 2022 01.
Article in English | MEDLINE | ID: mdl-34774720

ABSTRACT

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.


Subject(s)
Cardiomyopathy, Dilated/chemically induced , Heart-Assist Devices , Substance-Related Disorders/complications , Ventricular Function, Left/physiology , Ventricular Remodeling/drug effects , Cardiomyopathy, Dilated/therapy , Humans , Retrospective Studies , Ventricular Function, Left/drug effects , Young Adult
18.
J Card Surg ; 36(9): 3100-3111, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34164850

ABSTRACT

OBJECTIVE: Whether patients with severe aortic stenosis (AS) and significant functional mitral regurgitation (MR) should undergo isolated aortic (aortic valve replacement [AVR]) or double aortic-mitral valve procedure (DVP) remains controversial. We sought to determine outcomes of such patients undergoing surgical (surgical aortic valve replacement [SAVR]) and transcatheter AVR (TAVR) or DVP, identify echocardiographic parameters predictive of significant residual MR after isolated AVR, and determine its impact on long-term survival. METHODS: Data prospectively collected from 736 consecutive patients with severe AS and significant MR undergoing AVR or DVP were retrospectively analyzed. Exclusion of organic MR, other valve diseases and concomitant CABG yielded a final population of 74 patients with significant functional MR (32 TAVR, 23 SAVR, 19 DVP). Demographics, postoperative complications and age-adjusted survival were compared. Echocardiographic predictors of significant residual MR and its impact on survival were analyzed for patients undergoing isolated AVR. RESULTS: In the isolated AVR group, MR improvement occurred in 60% of patients and was associated with a significant increase in survival compared to persistence of significant MR (p = .03). Patients with improved MR had significantly greater preoperative left ventricular dilatation (LVEDD: 49 vs. 43 mm, p = .001; LVESD: 35 vs. 29 mm, p = .03; LVEDV: 101 vs. 71 ml, p = .0003; LVESV: 57 vs. 33 ml, p = .002). There was no significant difference in perioperative mortality (5.3 vs. 4.4 vs. 9.4%, p = .85) or age-adjusted long-term survival between isolated AVR and DVP groups (76.3 vs. 84.2% survival at 2-year follow-up, p = .26), or between SAVR, TAVR and DVP groups (78.2 vs. 75.0 vs. 84.2% survival at 2-year follow-up, p = .13). CONCLUSIONS: After isolated AVR, MR improvement occurs in 60% of patients. It is predicted by greater ventricular dimensions and associated with significantly better long-term survival. Whether a staged approach with transcatheter correction of MR should be considered in patients with significant residual MR following AVR remains undetermined.


Subject(s)
Aortic Valve Stenosis , Heart Valve Prosthesis Implantation , Mitral Valve Insufficiency , Transcatheter Aortic Valve Replacement , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve Stenosis/surgery , Humans , Mitral Valve Insufficiency/surgery , Prognosis , Retrospective Studies , Severity of Illness Index , Treatment Outcome
19.
CJC Open ; 3(4): 532-548, 2021 Apr.
Article in English | MEDLINE | ID: mdl-34027358

ABSTRACT

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.

20.
CJC Open ; 3(3): 292-302, 2021 Mar.
Article in English | MEDLINE | ID: mdl-33778446

ABSTRACT

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.

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