Background:Fulminant myocarditis (FM) is a life-threatening cause of cardiogenic shock. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) is often employed as a bridge to recovery, but data on long-term outcomes and prognostic markers post-discharge remain limited. We described long-term outcomes and explored the association between discharge left ventricular ejection fraction (LVEF) and post-discharge events. Methods:This retrospective, international, multicentre cohort study included adult FM patients who required VA-ECMO and survived to hospital discharge. Patients were stratified by LVEF ≥ 50% or < 50% at discharge. Cox proportional hazards models were used to assess the association between discharge LVEF and the primary composite endpoint of death, heart transplantation, or durable mechanical circulatory support postdischarge. Results:A total of 106 patients from 11 centres were included, with a median age of 43 years (interquartile range 29-57); 42% were male. At discharge, LVEF was ≥ 50% in 69 patients and < 50% in 37 patients. Over a median follow-up of 4.4 years (interquartile range 1.6-7.9), 3 patients (2.8%) experienced the composite outcome (2 deaths, 1 left ventricular assist device implantation). Compared with patients discharged with an LVEF ≥ 50%, those with an LVEF < 50% had a numerically higher risk of the composite endpoint (hazard ratio 2.9, 95% confidence interval 0.19-42.9), although this difference was not statistically significant. Conclusions:Given the low number of postdischarge events, this study was underpowered to assess the prognostic value of discharge LVEF. However, long-term outcomes were favourable among survivors of FM supported with VA-ECMO, regardless of their LVEF at discharge.

Tropheryma whipplei, the causative agent of Whipple's disease, is a rarely diagnosed pathogen that typically presents as a chronic, multisystemic illness. While its role in blood culture-negative endocarditis (BCNE) is increasingly recognised-accounting for approximately 4% of such cases-its other cardiac manifestations remain underappreciated. We report a case that underscores the broader cardiac implications of Whipple's disease, emphasising the diagnostic challenges faced when typical findings are absent. This report aims to raise awareness among cardiologists about the spectrum of cardiac involvement in Whipple's disease and to highlight the importance of considering this elusive diagnosis in patients with unexplained cardiac and systemic symptoms.

Cardiac imaging is a cornerstone in the initial diagnosis, management, and follow-up of cardiac sarcoidosis. However, ordering thresholds, access, and follow-up imaging vary across the globe. A Delphi study was conducted to define areas of consensus and areas requiring further study in the use of cardiac imaging in suspected or established cardiac sarcoidosis. An international, multidisciplinary panel of experts in cardiac sarcoidosis completed a modified 2-round Delphi study. The study evaluated clinical decision making regarding the use of cardiac imaging, including indication thresholds, interpretation, and interval follow-up imaging. Consensus was defined a priori as ≥70% agreement or disagreement. A total of 89 experts in cardiac sarcoidosis (89 in round 1 and 75 in round 2) participated, representing 61 centers in 13 countries. Consensus was reached on 22 of 46 items (48%) in round 1 and 21 of 29 items (72%) in round 2. There was a low threshold to order advanced cardiac imaging for new rhythm abnormalities or ventricular dysfunction detected on echocardiography in patients with established extracardiac sarcoidosis. 18F-fluorodeoxyglucose (FDG) positron emission tomography was an important co-primary modality with cardiac magnetic resonance (CMR) for initial diagnosis. If CMR was the first test, there was consensus to proceed to FDG-positron emission tomography after any abnormal CMR result or even after normal CMR result in the setting of moderate or high pretest probability for cardiac sarcoidosis. There was consensus that late gadolinium enhancement quantification was important, but there was no consensus on the threshold of risk or on how best to quantify late gadolinium enhancement. Similarly, reduction in FDG uptake was an important factor in guiding treatment response, but there was no consensus on how to best quantify FDG uptake or what constituted an adequate radiographic response. Several consensus areas for cardiac imaging in suspected and established cardiac sarcoidosis were identified. This consensus study identified areas of priority for future prospective, controlled, multicenter research studies.

Background:In some patients with left ventricular assist devices (LVADs), unloading of the left ventricle (LV) and medical therapy may lead to improvement in LV systolic function, allowing for LVAD weaning. There are no guideline-directed parameters to help identify candidates for weaning and long-term outcomes remain imperfectly documented. In this study we aimed to assess the clinical and echocardiographic characteristics of weaned patients and evaluate their event-free survival after weaning. Methods:This investigation was a single-center retrospective study of patients who underwent a second- or third-generation LVAD implantation between 2009 and 2021. Results:Ninety-eight patients were included. Fourteen patients (14%) with LV recovery underwent LVAD weaning after a median support time of 309 days. Heart failure etiologies in weaned patients included toxic (recreational drugs) (n = 8, 57%), toxic (medication) (n = 2, 14%), ischemic (n = 2, 14%), or idiopathic dilated (n = 2, 14%) cardiomyopathy. In unweaned patients, heart failure was mostly attributed to ischemic (n = 35, 42%) and idiopathic dilated (n = 27, 32%) cardiomyopathy. Three months after implantation, patients who were eventually weaned had a higher LV ejection fraction (LVEF) (35% vs 19%, P = 0.001) and lower left ventricular end-diastolic diameter (LVEDD) (52 vs 60 mm, P = 0.03) than unweaned patients. At last follow-up after weaning, mean LVEF was 44 ± 6% and no death nor heart transplant had occurred. Conclusions:LVADs can induce LV reverse remodeling leading to myocardial recovery in a significant proportion of patients, especially those with toxic and nonischemic cardiomyopathies. Early reverse remodeling with decreasing LVEDD and improving LVEF at 3 months after implantation may suggest potential candidacy for LVAD weaning. Weaned patients maintain satisfactory LVEF recovery after weaning and have good long-term event-free survival.

Identification of a cardiomyopathy phenotype should prompt a systematic search for the underlying aetiology, which may be genetic or acquired and can be associated with extracardiac manifestations. Fabry Disease (FD) is an inherited metabolic disorder causing left ventricular hypertrophy along with multiples other cardiac symptoms. Due to its X-linked transmission, FD was long thought to only affect men, although women are also involved but with a different spectrum of presentations, with less important symptoms that tend to appear later or with atypical phenotype.

Background:Aortic valve replacement (AVR) for chronic aortic regurgitation (AR) in the presence of reduced left ventricular ejection fraction (LVEF) is associated with greater surgical risk. Long-term outcomes remain poorly described in contemporary cohorts. Methods:Between 2004 and 2019, 122 patients underwent AVR for chronic severe AR with LVEF < 50%. Patients with severely reduced LVEF (< 35%; n = 37) were compared with those with mild to moderately reduced LVEF (35%-50%; n = 85). Results:Preoperative and intraoperative characteristics were similar in both groups. Operative mortality for the entire cohort was 1.6% (n = 2) and similar across the LVEF spectrum. Postoperatively, optimal medical therapy was achieved in most patients and > 25% of patients with LVEF < 35% benefited from cardiac resynchronization therapy. At latest follow-up, mean LVEF was 42 ± 12% in the severely reduced LVEF group (vs baseline 28 ± 5%; P < 0.001) and 51 ± 9% in the mild to moderately reduced LVEF group (vs baseline 46 ± 4%; P < 0.001). Freedom from cardiovascular death at 10 years was 87.2% in the severe group and 94.7% in the mild to moderate group (P = 0.10). Freedom from heart failure hospitalization at 10 years was higher in the mild to moderate group (96.3%) than in the severe group (88.3%; P = 0.009). Conclusions:In this contemporary cohort of patients who underwent AVR for chronic severe AR, patients with severely reduced LVEF treated with optimal medical therapy had operative mortality and freedom from cardiovascular death similar to patients with mild to moderately reduced LVEF. Favourable left ventricular remodelling was observed in both groups. Hospitalization for heart failure was < 15% in both groups at 10-year follow-up.

The increasing prevalence of amphetamine use has become a major public health concern in Canada. Among the many cardiovascular impacts of amphetamine use, dilated cardiomyopathy is emerging as a rare but morbid complication in young adults. This case report highlights the evolution of a patient with amphetamine-induced cardiomyopathy. We present a common clinical presentation of this disease and a novel approach to address certain treatment challenges in advanced heart failure in this patient population.

Background: Although recent studies have showed improved outcomes, aortic valve replacement (AVR) for chronic aortic regurgitation (AR) in the presence of reduced left ventricular ejection fraction (LVEF) is associated with a higher surgical risk. Contemporary long-term outcome remains poorly investigated. Methods: Between January 2004 and August 2019, we identified 122 patients who underwent AVR for pure chronic severe AR with LVEF less than 50%. Patients with severe reduced LVEF (<35%, n=37) were compared with those with mild to moderate reduced LVEF (35% to 50%, n=85). Results: Preoperative and intraoperative characteristics were similar in both groups. Operative mortality for the entire cohort was 1,6% (n=2) and similar across the LVEF spectrum. Postoperative intra-aortic balloon pump has been used in 2/37 patients with LVEF < 35% compared to 0/85 patients with LVEF 35-50% (p = 0.09). Patients with severely reduced LVEF had a longer length of stay (10.1±5.5 vs 7.8±3.5 days, p=0,02) while the rate of stroke, atrial fibrillation, acute renal failure, and pulmonary infection were similar between groups. Postoperatively, optimal medical therapy was achieved in most patients and more than a quarter of patients with LVEF < 35% benefited from cardiac resynchronization therapy. At the latest follow-up, mean LVEF was 42±12% in the severe reduced LVEF (vs. baseline 28±6%, p<0.001) and 51±9% in the mild to moderate reduced LVEF group (vs. baseline 45±4%, p<0.001). Freedom from cardiovascular (CV) death at ten years was 87.2% in the severe reduced LVEF group and 94.7% in patients with mild to moderate reduced LVEF (p=0.10). Freedom from heart failure (HF) hospitalization at ten years was higher in the mild to moderate reduced LVEF group (96.3%) than in the severely reduced LVEF group (88.3%) (p=0.009). Conclusion: In this contemporary cohort of patients undergoing AVR for chronic severe AR, patients with severely reduced EF treated with optimal guideline-directed medical therapy had a similar operative mortality and freedom from CV death than patients with mild to moderate reduced LVEF. Favorable left ventricular remodeling was observed in both groups at 1 year follow-up. Hospitalization for HF was low with less than 15% in both groups at 10 years follow-up; although rehospitalisation occurred more frequently in severe reduced LVEF group.

Left ventricular assist devices (LVAD) are increasingly used in refractory heart failure, mainly as a bridge to transplant or as destination therapy. LVAD unloading of the left ventricle (LV) can also promote myocardial recovery leading to improvement of the LV systolic function, allowing LVAD explantation. However, parameters associated with LVAD weaning and long-term outcomes are not well documented. The aim of this study was to assess the clinical and echocardiographic characteristics of weaned patients and to evaluate their event-free survival post-explantation. We conducted a single-center retrospective study recruiting patients who underwent a second or third generation LVAD implantation at a Canadian cardiology center between November 2009 and October 2021. A total of 98 patients underwent LVAD implantation during this period. All patients were on guideline-directed medical therapy (GDMT). Fourteen patients (14%) had significant LV recovery and were explanted/decommissioned after a median support time of 309 days (range 55-595 days). Toxic cardiomyopathy, defined as abuse of amphetamines (n=2), energy drinks (n=2) or multiple substances (n=6), was the most likely diagnosis in 10 of the weaned patients (71%). The LVEF was significantly higher and the left ventricular end-diastolic diameter (LVEDD) was significantly lower in weaned patients compared to unweaned patients after 3 months of support. Myocardial recovery manifested as pump thrombosis in 4 of the weaned patients (29%). Prior to explantation, 12 patients had a decrease in GDMT doses to minimize post explantation vasoplegia. After explantation, median follow-up time was 40 months (range 8-109 months) and the majority of patients were anticoagulated for 3 months. At last follow up, the LVEF was 44±6% and no patient had a LVEF below 35%. All patients were NYHA I or II and no death, heart transplant, or mechanical support initiation occurred. LVAD therapy can induce LV reverse remodeling leading to myocardial recovery in a significant proportion of patients, especially young patients with toxic and non-ischemic cardiomyopathies. Early reverse remodeling with decreasing LVEDD and improving LVEF at 3 months following implantation is associated with successful weaning. Pump thrombosis seems to be associated with LV reverse remodeling and weaning of mechanical support in certain patients. Weaned patients maintain satisfactory LVEF recovery after explantation and have good long-term event-free survival.

Background/Objectives: Patients with cardiogenic shock on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) frequently develop left ventricular (LV) distension and pulmonary edema due to an increased LV afterload. A balloon atrial septostomy (BAS) is a technique used to alleviate LV pressure and facilitate left atrial decompression. While primarily performed in pediatric populations, this procedure's feasibility in adult patients is less studied. This study aimed to evaluate the procedural outcomes, including the safety and effectiveness, of BASs in adult patients with cardiogenic shock supported by VA-ECMO. Methods: This single-center retrospective study included 11 adult patients with cardiogenic shock on VA-ECMO, who underwent a BAS between 2012 and 2023. Multiple parameters were used to evaluate the global clinical impact of a BAS on patients with cardiogenic shock. Results: Between 2012 and 2023, 11 patients with cardiogenic shock on VA-ECMO underwent a BAS procedure in our institution. The mean time from the BAS to advanced therapy was 6.4 days. Procedural success was achieved in all patients with no complications. Nine patients (82%) had an improvement in PaO2/FiO2 24 h post-BAS procedure. All patients had an improvement in the pulmonary edema on the chest X-ray 24 to 48 h after the procedure, with clear radiography achieved in nine patients (82%) in a mean time of 7 days (range: 1.5-13 days). A total of five patients (45%) had in-hospital mortality due to non-procedural complications and the mortality timing from BAS was between 5 to 23 days. Among those discharged, all six patients were alive at the 1-year follow-up. Conclusions: A BAS is a feasible and safe technique for decompressing the left atrium in adult patients on VA-ECMO. It significantly improved pulmonary edema and oxygenation in most cases. Further studies with larger populations are needed to evaluate its impact on long-term outcomes.

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.

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.

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/m(2) [46 to 68], p <0.01), LV end-systolic volume (56 [53 to 70] vs 36 ml/m(2) [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/m(2) [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.

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.

Sarcoidosis is an inflammatory multisystemic disease characterized by the development and accumulation of noncaseating granulomas in different organs such as the heart and lungs. Cardiac sarcoidosis (CS) may be a life-threatening condition. Diagnosis, optimal treatments, and follow-up remain challenging. We believe that collaboration among different specialties, often led by expert cardiologists, is key to optimal patient care. A dedicated CS clinic is also an added value owing to the complexity and diversity of cardiac presentations. The need for combined and long immunosuppression-corticosteroid treatment also require specialized expertise. We outline the role of a CS clinic and of each specialty.

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.