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Cardiac amyloidosis has recently garnered substantial attention. Although the advent of noninvasive diagnostic algorithms revolutionized diagnosis, endomyocardial biopsy may still be considered in select cases to determine the amyloidosis subtype definitively. We report a case of a patients with a known mutation causing hereditary apolipoprotein A-I–associated cardiac amyloidosis. (Level of Difficulty: Advanced.), Central Illustration

A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after completion of chemotherapy for osteosarcoma. Five months after completion of chemotherapy, at the age of 10 years, heart transplantation was performed. At 29 months since transplantation, the patient remains free of rejection and recurrence of osteosarcoma. (Level of Difficulty: Intermediate.), Central Illustration

A patient with a history of heart block and longstanding chloroquine use presented in cardiogenic shock refractory to medical therapy and mechanical circulatory support. Autopsy supported antimalarial-induced cardiomyopathy (AMIC). Progression of AMIC may be halted with early recognition and cessation of antimalarial therapy, highlighting importance of screening and timely diagnosis. (Level of Difficulty: Beginner.), Graphical abstract

Immune checkpoint inhibitor (ICI) therapy has significantly improved the prognosis of many advanced cancers. ICIs target cytotoxic T lymphocyte-associated antigen-4 (CTLA-4), programmed cell death-1 (PD-1), or programmed cell death-ligand 1 (PD-L1) and increase the antitumor immune response. Several

A 39-year-old man, painter by profession, presented with symptoms of heart failure. His work up was unrevealing except for elevated blood lead levels (BLL). He was started on guideline-directed medical therapy and was referred to occupational therapy. No improvement in his ejection fraction was noted until his BLL decreased. (Level of Difficulty: Intermediate.), Graphical abstract, A 42-year-old man, painter by profession, presented with symptoms of heart failure. His work up was unrevealing except for elevated blood lead levels (B…

We report a case of giant cell myocarditis in a 76-year-old patient managed with combined immunosuppression and biventricular intravascular microaxial blood pumps. This case highlights a feasible approach for managing such patients who are not candidates for transplantation or durable ventricular assist devices. (Level of Difficulty: Intermediate.), Graphical abstract, We report a case of giant cell myocarditis in a 76-year-old patient managed with combined immunosuppression and biventricular intravascular m…

A 25-year-old African-American woman with end-stage renal disease presented with new-onset heart failure. Transthoracic echocardiography indicated a significantly hyperechoic myocardium, and computed tomography noted a circumferential hyperattenuated myocardium. Endomyocardial biopsy revealed focal interstitial and intramyocyte calcium deposition in the heart, confirming a rare diagnosis of massive myocardial calcium deposition. (Level of Difficulty: Beginner.), Graphical abstract, A 25-year-old African American woman with end-stage renal disease presented with new-onset heart failure. Transthoracic echocardiography indicated a…

We describe a case of fulminant eosinophilic myocarditis as the first presentation of eosinophilic granulomatosis with polyangiitis, promptly managed with extracorporeal membrane oxygenation. This case highlights the multidisciplinary work involving all health care professionals in the acute management of these patients and discusses it from an educational point of view. (Level of Difficulty: Intermediate.), Graphical abstract, We describe a case of fulminant eosinophilic myocarditis as the first presentation of eosinophilic granulomatosis with polyangiitis, promptly managed…

A young man presented with syncope. He was diagnosed with triglyceride deposit cardiomyovasculopathy and skeletal myopathy secondary to adipose triglyceride lipase (ATGL) deficiency. Despite optimal medical therapy, he required heart transplantation to treat his heart failure. Five years post-transplant, the graft function was normal with no evidence of triglyceride deposits. (Level of Difficulty: Intermediate.), Graphical abstract, A young man presented with syncope. He was diagnosed with triglyceride deposit cardiomyovasculopathy and skeletal myopathy secondary to adipose…

Visual Abstract, Highlights • Quantitative multiplexed immune-phenotyping of cardiac allograft biopsies provides novel diagnostic and prognostic information about allograft health. • Reduced proportions of cells expressing PD-L1 and FoxP3 are associated with clinical evidence of serious allograft injury, even when conventional histologic analysis provides falsely reassuring histologic rejection grades. • The proportions of PD-L1- and FoxP3-expressing cells are dynamic within cardiac allografts, and reduced levels can precede future rejection., Summary Recognizing that guideline-directed histologic grading of endomyocardial biopsy tissue samples for rejection surveillance has limited diagnostic accuracy, quantitative, in situ characterization was performed of several important immune cell types in a retrospective cohort of clinical endomyocardial tissue samples. Differences between cases were identified and were grouped by histologic grade versus clinical rejection trajectory, with significantly increased programmed death ligand 1+, forkhead box P3+, and cluster of differentiation 68+ cells suppressed in clinically evident rejections, especially cases with marked clinical-histologic discordance. Programmed death ligand 1+, forkhead box P3+, and cluster of differentiation 68+ cell proportions are also significantly higher in “never-rejection” when compared with “future-rejection.” These findings suggest that in situ immune modulators regulate the severity of cardiac allograft rejection.

We describe a patient with ventricular tachycardia and complete atrioventricular block. Remarkable thinning of the basal interventricular septum preceded left ventricular dysfunction. Endomyocardial biopsy demonstrated giant cell myocarditis. The patient received combined immunosuppressive therapy and a cardioverter-defibrillator. Eligibility screening for heart transplantation was initiated. (Level of Difficulty: Advanced.), Graphical abstract, This study describes a patient with ventricular tachycardia and complete atrioventricular block. Remarkable thinning of the basal interventricular…

Objectives The aim of this study was to characterize left atrial (LA) pathology in explanted hearts with transthyretin amyloid cardiomyopathy (ATTR-CM); LA mechanics using echocardiographic speckle-tracking in a large cohort of patients with ATTR-CM; and to study the association with mortality. Background The clinical significance of LA involvement in ATTR-CM is of great clinical interest. Methods Congo red staining and immunohistochemistry was performed to assess the presence, type, and extent of amyloid and associated changes in 5 explanted ATTR-CM atria. Echo speckle tracking was used to assess LA reservoir, conduit, contractile function, and stiffness in 906 patients with ATTR-CM (551 wild-type (wt)-ATTR-CM; 93 T60A-ATTR-CM; 241 V122I-ATTR-CM; 21 other). Results There was extensive ATTR amyloid infiltration in the 5 atria, with loss of normal architecture, vessels remodeling, capillary disruption, and subendocardial fibrosis. Echo speckle tracking in 906 patients with ATTR-CM demonstrated increased atrial stiffness (median [25th-75th quartile] 1.83 [1.15-2.92]) that remained independently associated with prognosis after adjusting for known predictors (lnLA stiff: HR: 1.23; 95% CI: 1.03-1.49; P = 0.029). There was substantial impairment of the 3 phasic functional atrial components (reservoir 8.86% [5.94%-12.97%]; conduit 6.5% [4.53%-9.28%]; contraction function 4.0% [2.29%-6.56%]). Atrial contraction was absent in 22.1% of patients whose electrocardiograms showed sinus rhythm (SR) “atrial electromechanical dissociation” (AEMD). AEMD was associated with poorer prognosis compared with patients with SR and effective mechanical contraction (P = 0.0018). AEMD conferred a similar prognosis to patients in atrial fibrillation. Conclusions The phenotype of ATTR-CM includes significant infiltration of the atrial walls, with progressive loss of atrial function and increased stiffness, which is a strong independent predictor of mortality. AEMD emerged as a distinctive phenotype identifying patients in SR with poor prognosis., Central Illustration

Synopsis The clinical manifestations of COVID-19 are widely variable and may involve several districts. Although the clinical course is mostly characterized by respiratory involvement, up to 30% of hospitalized patients have evidence of myocardial injury due to acute coronary syndrome, cardiac arrhythmias, myocarditis, and cardiogenic shock. In particular, myocarditis is a well-recognized severe complication of COVID-19 and is associated with fulminant cardiogenic shock and sudden cardiac death. The pathophysiology of cardiac injury remains poorly understood and the management and outcomes of myocarditis are not yet clarified. Our aim is to present a comprehensive review about COVID-19 related myocarditis, including clinical characteristics, diagnostic workup, and management.

Cardiac amyloidosis is associated with very high morbidity and mortality. Only if treated early, cardiac amyloidosis responds well to therapy, and early recognition with a full differential diagnostic workup including multimodality imaging is therefore critical at first presentation. Closely meshed clinical monitoring and imaging are indispensable to ensure optimal individualized treatment. (Level of Difficulty: Beginner.), Graphical abstract, Cardiac amyloidosis is associated with very high morbidity and mortality. Only if treated early, cardiac amyloidosis responds well to therapy, and…

A 56-year-old woman with mixed connective tissue disease, who was on maintenance immunosuppression, developed asymptomatic left ventricular…, A 56-year-old woman with mixed connective tissue disease, who was on maintenance immunosuppression, developed asymptomatic left ventricular dysfunction, ventricular arrhythmia, and high troponin I. Heart catheterization showed normal coronaries and biopsy-proven, virus-negative lymphocytic myocarditis. A biopsy-guided immunosuppression upgrade effectively treated autoimmune myocarditis, which resulted in ventricular function recovery, resolution of arrhythmia, and of troponin release. (Level of Difficulty: Advanced.), Graphical abstract

Background Prevalence of both degenerative severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR-CA) increases with age. Dual disease (AS+myocardial ATTR-CA) occurs in significant proportion of patients undergoing surgical aortic valve replacement (SAVR). Objectives This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis. Methods Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation. Results SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477). Conclusions Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common., Central Illustration

To investigate whether severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2)–induced myocarditis constitutes an important mechanism of cardiac injury, a review was conducted of the published data and the authors’ experience was added from autopsy examination of 16 patients dying of SARS-CoV-2 infection. Myocarditis is an uncommon pathologic diagnosis occurring in 4.5% of highly selected cases undergoing autopsy or endomyocardial biopsy. Although polymerase chain reaction–detectable virus could be found in the lungs of most coronavirus disease-2019 (COVID-19)–infected subjects in our own autopsy registry, in only 2 cases was the virus detected in the heart. It should be appreciated that myocardial inflammation alone by macrophages and T cells can be seen in noninfectious deaths and COVID-19 cases, but the extent of each is different, and in neither case do such findings represent clinically relevant myocarditis. Given its extremely low frequency and unclear therapeutic implications, the authors do not advocate use of endomyocardial biopsy to diagnose myocarditis in the setting of COVID-19., Central Illustration, Highlights • Whether myocarditis is a cause of myocardial injury in patients with COVID-19 is uncertain. • Myocarditis is uncommon in autopsy or EMB in cases of COVID-19. • Further work is needed to fully understand the cardiac effects of COVID-19 infection.

Standard evaluation and management of the patient with suspected or proven cardiovascular complications of COVID-19, the disease caused by severe acute respiratory syndrome–related coronavirus-2 (SARS-CoV-2), is challenging. Routine history, physical examination, laboratory testing, electrocardiography and plain x-ray imaging may often suffice for such patients but given overlap between COVID-19 and typical cardiovascular diagnoses such as heart failure and acute myocardial infarction, need frequently arises for advanced imaging techniques to assist in differential diagnosis and management. This document provides guidance in several common scenarios among patients with confirmed or suspected COVID-19 infection and possible cardiovascular involvement, including chest discomfort with electrocardiographic changes, acute hemodynamic instability, newly-recognized left ventricular dysfunction, as well as imaging during the sub-acute/chronic phase of COVID-19. For each, we consider the role of biomarker testing to guide imaging decision-making, provide differential diagnostic considerations, and offer general suggestions regarding application of various advanced imaging techniques. Condensed Abstract Standard evaluation and management of the patient with suspected or proven cardiovascular complications due to COVID-19 infection often requires advanced imaging techniques to assist in differential diagnosis and management. This document provides guidance in several common scenarios among patients with COVID-19 infection and for each provides advice regarding the role of biomarker testing to guide imaging decision-making, provides differential diagnostic considerations, and offers general suggestions regarding application of various advanced imaging techniques.

Human coronavirus-associated myocarditis is known, and a number of coronavirus disease 19 (COVID-19)-related myocarditis cases have been reported. The pathophysiology of COVID-19-related myocarditis is thought to be a combination of direct viral injury and cardiac damage due to the host's immune response. COVID-19 myocarditis diagnosis should be guided by insights from previous coronavirus and other myocarditis experience. The clinical findings include changes in electrocardiogram and cardiac biomarkers, and impaired cardiac function. When cardiac magnetic resonance imaging is not feasible, cardiac computed tomographic angiography with delayed myocardial imaging may serve to exclude significant coronary artery disease and identify myocardial inflammatory patterns. Because many COVID-19 patients have cardiovascular comorbidities, myocardial infarction should be considered. If the diagnosis remains uncertain, an endomyocardial biopsy may help identify active cardiac infection through viral genome amplification and possibly refine the treatment risks of systemic immunosuppression. Arrhythmias are not uncommon in COVID-19 patients, but the pathophysiology is still speculative. Nevertheless, clinicians should be vigilant to provide prompt monitoring and treatment. The long-term impact of COVID-19 myocarditis, including the majority of mild cases, remains unknown.

Cardiac amyloidosis (CA) has emerged as a previously underestimated cause of heart failure and mortality. Underdiagnosis resulted mainly from unawareness of the true disease prevalence and the non-specific symptoms of the disease. CA results from extracellular deposition of misfolded protein fibrils, commonly derived from transthyretin (ATTR) or immunoglobulin light chains (AL). A significant proportion of older patients with heart failure and other extracardiac manifestations suffer from ATTR-CA, whereas AL-CA is still considered a rare disease. This article provides an overview of CA with a special focus on current and emerging diagnostic modalities. Furthermore, we provide a diagnostic algorithm for the evaluation of patients with suspected CA in every-day practice. CA - Papathanasiou

Eosinophilic myocarditis is a type of inflammatory cardiomyopathy characterized by eosinophilic infiltration into myocardial tissue. The accurate myocarditis incidence rate is difficult to determine because of the clinical limitations of an endomyocardial biopsy. The primary pathogenesis of eosinophilic myocarditis is the release of related substances by eosinophils, leading to cell membrane damage and cell destruction. However, evidence suggests that specific genes play a role in myocarditis development.As CMR imaging availability increases, the diagnosis rate of eosinophilic myocarditis will increase. The diagnosis of myocarditis mainly depends on an endocardial biopsy. Glucocorticoids can relieve patients’ symptoms, but the early use of steroids may prevent intermediate disease stage development (i.e., thrombonecrosis and fibrosis with wall thrombosis). Anticoagulant therapy may also affect disease development. In addition to routine follow-up, a regular myocardial biopsy should be considered for discharged patients, if possible., Highlights • Eosinophilic myocarditis is eosinophilic infiltration into myocardial tissue. • Regular biopsies may help monitor disease progression. • Define eosinophilic myocarditis pathologically and histologically and distinguish it from similar diseases. • Cardiac MR,are helpful in the diagnosis of eosinophilic myocarditis, especially when myocardial biopsy cannot be performed. • Explore eosinophilic myocarditis at the gene level.