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Background COVID-19 infection is known to cause a wide array of clinical chronic sequelae, but little is known regarding the long-term cardiac complications. We aim to report echocardiographic follow-up findings and describe the changes in left (LV) and right ventricular (RV) function that occur following acute infection. Methods Patients enrolled in the World Alliance Societies of Echocardiography-COVID study with acute COVID-19 infection were asked to return for a follow-up transthoracic echocardiogram. Overall, 198 returned at a mean of 129 days of follow-up, of which 153 had paired baseline and follow-up images that were analyzable, including LV volumes, ejection fraction (LVEF), and longitudinal strain (LVLS). Right-sided echocardiographic parameters included RV global longitudinal strain, RV free wall strain, and RV basal diameter. Paired echocardiographic parameters at baseline and follow-up were compared for the entire cohort and for subgroups based on the baseline LV and RV function. Results For the entire cohort, echocardiographic markers of LV and RV function at follow-up were not significantly different from baseline (all P > .05). Patients with hyperdynamic LVEF at baseline (>70%), had a significant reduction of LVEF at follow-up (74.3% ± 3.1% vs 64.4% ± 8.1%, P < .001), while patients with reduced LVEF at baseline (−20%) at baseline had significant improvement at follow-up (−15.2% ± 3.4% vs −17.4% ± 4.9%, P = .004). Patients with abnormal RV basal diameter (>4.5 cm) at baseline had significant improvement at follow-up (4.9 ± 0.7 cm vs 4.6 ± 0.6 cm, P = .019). Conclusions Overall, there were no significant changes over time in the LV and RV function of patients recovering from COVID-19 infection. However, differences were observed according to baseline LV and RV function, which may reflect recovery from the acute myocardial injury occurring in the acutely ill. Left ventricular and RV function tends to improve in those with impaired baseline function, while it tends to decrease in those with hyperdynamic LV or normal RV function.

This case series shows how the 2021 ACC/AHA/SCAI guideline for coronary artery revascularization can be used to decide between revascularization or optimal medical therapy to reduce mortality or cardiovascular events in selected subsets of patients with stable ischemic heart disease and complex coronary disease with or without left ventricular dysfunction. (Level of Difficulty: Advanced.), Graphical abstract

Visual Abstract, Highlights • AAV9 vectors can upregulate Rxfp1 mRNA in murine heart after intravenous injection. • RXFP1 upregulation sensitizes the left ventricle to relaxin-induced inotropy. • RXFP1 overexpression protects heart from ischemia-reperfusion injury., Summary Relaxin is a pleiotropic hormone shown to confer cardioprotection in several preclinical models of cardiac ischemia-reperfusion injury. In the present study, the effects of up-regulating relaxin family peptide receptor 1 (RXFP1) via adeno-associated virus serotype 9 (AAV9) vectors were investigated in a mouse model of myocardial infarction. AAV9-RXFP1 vectors were generated and injected in adult male CD1 mice. Up-regulation of Rxfp1 was confirmed via quantitative polymerase chain reaction, and overexpressing animals showed increased sensitivity to relaxin-induced ventricular inotropic response. Overexpressing animals also demonstrated reduced infarct size and preserved cardiac function 24 hours after ischemia-reperfusion. Up-regulation of RXFP1 via AAV9 vectors has potential therapeutic utility in preventing adverse remodeling after myocardial infarction.

We present the case of a 25-year-old woman with desmoplakin cardiomyopathy–related myocarditis. Her high-sensitivity troponin and symptoms improved with pulse steroid therapy and mycophenolate mofetil. The literature lacks data to effectively guide the management of recurrent myocarditis in desmoplakin cardiomyopathy. (Level of Difficulty: Advanced.), Graphical abstract

Invasive pressure-volume loop analysis allows direct monitoring of changing intraventricular cardiac mechanics during structural heart interventions. Our aim was to illustrate changes in right and left ventricular mechanics during transcatheter edge-to-edge tricuspid repair for severe tricuspid regurgitation. (Level of Difficulty: Advanced.), Graphical abstract

Background Myocytes exposed to stress exhibit significant swelling and reduced contractility. These consequences are ameliorated by adenosine triphosphate–sensitive potassium (KATP) channel opener diazoxide (DZX) via an unknown mechanism. KATP channel openers also provide cardioprotection in multiple animal models. Nitric oxide donors are similarly cardioprotective, and their combination with KATP activation may provide synergistic benefit. We hypothesized that mitochondria-targeted S-nitrosating agent (MitoSNO) would provide synergistic cardioprotection with DZX. Methods Myocyte volume and contractility were compared following Tyrode's physiologic solution (20 minutes) and stress (hyperkalemic cardioplegia [CPG] ± DZX; n = 5-20 each; 20 minutes) with or without MitoSNO (n = 5-11 each) at the end of stress, followed by Tyrode's solution (20 minutes). Isolated mouse hearts received CPG ± DZX (n = 8-10 each) before global ischemia (90 minutes) with or without MitoSNO (n = 8 each) at the end of ischemia, followed by reperfusion (30 minutes). Left ventricular (LV) pressures were compared using a linear mixed model to assess the impact of treatment on the outcome, adjusting for baseline and balloon volume. Results Stress (CPG) was associated with reduced myocyte contractility that was prevented by DZX and MitoSNO individually; however, their combination was associated with loss of cardioprotection. Similarly, DZX and MitoSNO improved LV function after prolonged ischemia compared with CPG alone, and cardioprotection was lost with their combination. Conclusions MitoSNO and DZX provide cardioprotection that is lost with their combination, suggesting mutually exclusive mechanisms of action. The lack of a synergistic beneficial effect informs the current knowledge of the cardioprotective mechanisms of DZX and will aid planning of future clinical trials.

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Acute coronary artery dissection commonly occurs in young women without cardiovascular risk factors. Predisposing factors, including fibromuscular dysplasia or other vasculopathies, have been demonstrated in its etiology. Here we report the case of a 46-year-old-man who presented with left anterior descending coronary artery dissection caused by blast injury after a bomb explosion. (Level of Difficulty: Advanced.), Graphical abstract

A 54-year-old man presented in profound obstructive shock. Investigations revealed a right atrial mass causing severe right ventricular inflow obstruction and compromised cardiac output. The patient was treated with emergency balloon catheter intervention to relieve the obstruction, with resulting hemodynamic stability. The pathology report later returned a positive result for diffuse large B-cell lymphoma. (Level of Difficulty: Intermediate.), Graphical abstract

Background Long-term survivors of Hodgkin lymphoma (HL) and mediastinal non-Hodgkin lymphoma experience late adverse effects of radiotherapy and/or anthracycline-containing chemotherapy, leading to premature cardiovascular morbidity and mortality. Objectives The aim of this study was to identify markers for subclinical cardiovascular disease using cardiovascular magnetic resonance (CMR) in survivors of HL and non-Hodgkin lymphoma. Methods CMR was performed in 80 lymphoma survivors treated with mediastinal radiotherapy with or without anthracyclines, and results were compared with those among 40 healthy control subjects matched for age and sex. Results Of the 80 lymphoma survivors, 98% had histories of HL, the mean age was 47 ± 11 years, and 54% were male. Median radiotherapy dose was 36 Gy (interquartile range: 36-40 Gy), and radiotherapy was combined with anthracyclines in 70 lymphoma survivors (88%). Mean time between diagnosis and CMR was 20 ± 8 years. Significantly lower left ventricular (LV) ejection fraction (53% ± 5% vs 60% ± 5%; P < 0.001) and LV mass (47 ± 10 g/m2 vs 56 ± 8 g/m2; P < 0.001) and higher LV end-systolic volume (37 ± 8 mL/m2 vs 33 ± 7 mL/m2; P = 0.013) were found in lymphoma survivors. LV global strain parameters were also significantly worse in lymphoma survivors (P < 0.02 for all). Native myocardial T1 was significantly higher in lymphoma survivors compared with healthy control subjects (980 ± 33 ms vs 964 ± 25 ms; P = 0.007), and late gadolinium enhancement was present in 11% of the survivors. Conclusions Long-term lymphoma survivors have detectable changes in LV function and native myocardial T1 on CMR. Further longitudinal studies are needed to assess the implication of these changes in relation to treatment and clinical outcome., Central Illustration

Aneurysms of the left atrial appendage (LAA) are rare entities that often require surgical intervention. We demonstrate multimodality imaging features of a giant LAA aneurysm, with a focus on 3-dimensional blood flow dynamics by using 4-dimensional-flow cardiac magnetic resonance. (Level of Difficulty: Advanced.), Graphical abstract

Objectives The purpose of this study was to detect cardiovascular changes after mild severe acute respiratory syndrome coronavirus 2 infection. Background Concern exists that mild coronavirus disease 2019 may cause myocardial and vascular disease. Methods Participants were recruited from COVIDsortium, a 3-hospital prospective study of 731 health care workers who underwent first-wave weekly symptom, polymerase chain reaction, and serology assessment over 4 months, with seroconversion in 21.5% (n = 157). At 6 months post-infection, 74 seropositive and 75 age-, sex-, and ethnicity-matched seronegative control subjects were recruited for cardiovascular phenotyping (comprehensive phantom-calibrated cardiovascular magnetic resonance and blood biomarkers). Analysis was blinded, using objective artificial intelligence analytics where available. Results A total of 149 subjects (mean age 37 years, range 18 to 63 years, 58% women) were recruited. Seropositive infections had been mild with case definition, noncase definition, and asymptomatic disease in 45 (61%), 18 (24%), and 11 (15%), respectively, with 1 person hospitalized (for 2 days). Between seropositive and seronegative groups, there were no differences in cardiac structure (left ventricular volumes, mass, atrial area), function (ejection fraction, global longitudinal shortening, aortic distensibility), tissue characterization (T1, T2, extracellular volume fraction mapping, late gadolinium enhancement) or biomarkers (troponin, N-terminal pro–B-type natriuretic peptide). With abnormal defined by the 75 seronegatives (2 SDs from mean, e.g., ejection fraction 1,072 ms, septal T2 >52.4 ms), individuals had abnormalities including reduced ejection fraction (n = 2, minimum 50%), T1 elevation (n = 6), T2 elevation (n = 9), late gadolinium enhancement (n = 13, median 1%, max 5% of myocardium), biomarker elevation (borderline troponin elevation in 4; all N-terminal pro–B-type natriuretic peptide normal). These were distributed equally between seropositive and seronegative individuals. Conclusions Cardiovascular abnormalities are no more common in seropositive versus seronegative otherwise healthy, workforce representative individuals 6 months post–mild severe acute respiratory syndrome coronavirus 2 infection., Central Illustration

A 33-year-old woman with newly diagnosed disseminated tuberculosis presented with acute heart failure and incidental findings of intracardiac thrombus, demonstrating possible tuberculous myocarditis. (Level of Difficulty: Intermediate.), Central Illustration

We describe the case of an antenatally diagnosed massive cardiac tumor in a fetus requiring cardiorespiratory support immediately following birth. We further discuss the successful management of this case and highlight the importance of a multidisciplinary team in managing such complicated cases. (Level of Difficulty: Advanced.), Central Illustration

We describe the case of a 15-year-old female patient with Peutz-Jeghers syndrome who presented with vomiting and abdominal pain secondary to ileoileal invagination. Initial analgesic treatment was not effective, and subsequent tramadol infusion resulted in clinical manifestations compatible with Kounis and Takotsubo syndromes. However, the patient had an excellent recovery. (Level of Difficulty: Advanced.), Central Illustration

Objective: Knowledge of factors causing pacing-induced cardiomyopathy (PICM) is incomplete. We sought to estimate the incidence and predisposing factors for PICM and evaluate if the risk they portend adds up. Methods: Single centre retrospective study where consecutive patients with preserved LVEF undergoing pacemaker (PM) implantation between 2012 and 2018 were analysed. Results: A total of 749 patients (68.4 % male; mean age 59.2 ± 14.08 years) were included in the analysis. PICM developed in 74 (9.9%) patients over a median follow up of 2.2 years (IQR 1.1–3.2). Pre-implant LVEF, paced QRS duration and RV pacing burden were independent predictors of PICM. Using 90 % specificity cut-off values for LVEF and paced QRS, and the value separating lowest tertile of RV pacing from the higher tertiles, three risk factors were identified: (i) baseline LVEF 160 msec, and (iii) RV pacing burden > 33 %. Patients with two or more risk factors were at the highest risk (OR 11.62, 95 % CI 4.62–29.21, p-value

Radiation therapy is an important component of cancer therapy for many malignancies. With improvements in cardiac-sparing techniques, radiation-induced cardiac dysfunction has decreased but remains a continued concern. In this review, we provide an overview of the evolution of radiotherapy techniques in thoracic cancers and associated reductions in cardiac risk. We also highlight data demonstrating that in some cases radiation doses to specific cardiac substructures correlate with cardiac toxicities and/or survival beyond mean heart dose alone. Advanced cardiac imaging, cardiovascular risk assessment, and potentially even biomarkers can help guide post-radiotherapy patient care. In addition, treatment of ventricular arrhythmias with the use of ablative radiotherapy may inform knowledge of radiation-induced cardiac dysfunction. Future efforts should explore further personalization of radiotherapy to minimize cardiac dysfunction by coupling knowledge derived from enhanced dosimetry to cardiac substructures, post-radiation regional dysfunction seen on advanced cardiac imaging, and more complete cardiac toxicity data., Central Illustration, Highlights • Despite improvements in radiation techniques, radiation-induced cardiac dysfunction can occur in those with thoracic cancers. • Newer studies have examined associations between cardiac substructure doses and cardiac outcomes and/or survival. • Advanced cardiac imaging allows assessment of regional dysfunction after cardiac radiation exposure. • Enhanced dose tracking, imaging, and comprehensive clinical data hold promise for personalized approaches to minimize cardiotoxicity.

Uhl’s anomaly is a rare congenital heart disease characterized by partial or complete absence of the right ventricle myocardium. We report the first case, in a 21-year-old man, of Uhl’s anomaly–associated left ventricular noncompaction. This association represents a unique clinical entity and has important implications for management strategies. (Level of Difficulty: Intermediate.), Central Illustration

Highlights • The prevalence of heart failure with preserved ejection fraction among patients who present with symptoms of heart failure is approximately 50%. • Pharmacologic therapy has not conclusively shown benefits in morbidity and mortality in clinical trials. • Although it represents an active area of research, no device-based therapy has received regulatory approval for the treatment of heart failure with preserved ejection fraction. • Approaches such as atrial shunts, left ventricular expanders, mechanical circulatory support devices, and neurostimulators are at various stages of development., Central Illustration, Summary Characterized by a rapidly increasing prevalence, elevated mortality and rehospitalization rates, and inadequacy of pharmaceutical therapies, heart failure with preserved ejection fraction (HFpEF) has motivated the widespread development of device-based solutions. HFpEF is a multifactorial disease of various etiologies and phenotypes, distinguished by diminished ventricular compliance, diastolic dysfunction, and symptoms of heart failure despite a normal ejection performance; these symptoms include pulmonary hypertension, limited cardiac reserve, autonomic imbalance, and exercise intolerance. Several types of atrial shunts, left ventricular expanders, stimulation-based therapies, and mechanical circulatory support devices are currently under development aiming to target one or more of these symptoms by addressing the associated mechanical or hemodynamic hallmarks. Although the majority of these solutions have shown promising results in clinical or preclinical studies, no device-based therapy has yet been approved for the treatment of patients with HFpEF. The purpose of this review is to discuss the rationale behind each of these devices and the findings from the initial testing phases, as well as the limitations and challenges associated with their clinical translation.

Background The novel severe acute respiratory syndrome coronavirus-2 virus, which has led to the global coronavirus disease-2019 (COVID-19) pandemic is known to adversely affect the cardiovascular system through multiple mechanisms. In this international, multicenter study conducted by the World Alliance Societies of Echocardiography, we aim to determine the clinical and echocardiographic phenotype of acute cardiac disease in COVID-19 patients, to explore phenotypic differences in different geographic regions across the world, and to identify parameters associated with in-hospital mortality. Methods We studied 870 patients with acute COVID-19 infection from 13 medical centers in four world regions (Asia, Europe, United States, Latin America) who had undergone transthoracic echocardiograms. Clinical and laboratory data were collected, including patient outcomes. Anonymized echocardiograms were analyzed with automated, machine learning–derived algorithms to calculate left ventricular (LV) volumes, ejection fraction, and LV longitudinal strain (LS). Right-sided echocardiographic parameters that were measured included right ventricular (RV) LS, RV free-wall strain (FWS), and RV basal diameter. Multivariate regression analysis was performed to identify clinical and echocardiographic parameters associated with in-hospital mortality. Results Significant regional differences were noted in terms of patient comorbidities, severity of illness, clinical biomarkers, and LV and RV echocardiographic metrics. Overall in-hospital mortality was 21.6%. Parameters associated with mortality in a multivariate analysis were age (odds ratio [OR] = 1.12 [1.05, 1.22], P = .003), previous lung disease (OR = 7.32 [1.56, 42.2], P = .015), LVLS (OR = 1.18 [1.05, 1.36], P = .012), lactic dehydrogenase (OR = 6.17 [1.74, 28.7], P = .009), and RVFWS (OR = 1.14 [1.04, 1.26], P = .007). Conclusions Left ventricular dysfunction is noted in approximately 20% and RV dysfunction in approximately 30% of patients with acute COVID-19 illness and portend a poor prognosis. Age at presentation, previous lung disease, lactic dehydrogenase, LVLS, and RVFWS were independently associated with in-hospital mortality. Regional differences in cardiac phenotype highlight the significant differences in patient acuity as well as echocardiographic utilization in different parts of the world.

Background In patients with cardiac resynchronization therapy defibrillators (CRT-Ds), intracardiac impedance measured by dedicated CRT-D software may be used to monitor hemodynamic changes. We investigated the relationship of hemodynamic parameters assessed by intracardiac impedance and by echocardiography in a controlled clinical setting. Methods The study enrolled 68 patients (mean age, 66 ± 9 years; 74% males) at 12 investigational sites. The patients had an indication for CRT-D implantation, New York Heart Association class II/III symptoms, left ventricular ejection fraction 15%–35%, and a QRS duration ≥150 ms. Two months after a CRT-D implantation, hemodynamic changes were provoked by overdrive pacing. Intracardiac impedance was recorded at rest and at four pacing rates ranging from 10 to 40 beats/min above the resting rate. In parallel, echocardiography measurements were performed. We hypothesized that a mean intra-individual correlation coefficient (rmean) between stroke impedance (difference between end-systolic and end-diastolic intracardiac impedance) measured by CRT-D and the aortic velocity time integral (i.e., stroke volume) determined by echocardiography would be significantly larger than 0.65. Results The hypothesis was evaluated in 40 patients with complete data sets. The rmean was 0.797, with a lower confidence interval bound of 0.709. The study hypothesis was met (p = 0.007). A stepwise reduction of stroke impedance and stroke volume was observed with increasing heart rate. Conclusions Intracardiac impedance measured by implanted CRT-Ds correlated well with the aortic velocity time integral (stroke volume) determined by echocardiography. The impedance measurements bear potential and are readily available technically, not requiring implantation of additional material beyond standard CRT-D system., Highlights • Intracardiac impedance measurement feature (ICI-MF) is integrated in some CRT-Ds. • We studied relationship between echo hemodynamic parameters and ICI-MF of CRT-Ds. • Stroke volume by echo correlated well with stroke impedance by ICI-MF of CRT-Ds. • Stepwise reduction in stroke impedance and stroke volume with increasing heart rate. • The ICI-MF bears potential and requires only a ‘standard’ CRT-D system implantation.

We report a case series of 4 patients with transient marked QTc prolongation and ventricular arrhythmias in the setting of inflammation with very high ferritin levels. Three patients were positive for coronavirus disease-2019. In the setting of an acute rise in inflammatory markers, electrocardiography screening for QTc prolongation is warranted. (Level of Difficulty: Beginner.), Central Illustration

A 52-year-old man with acute peritonitis developed severely decreased left ventricular (LV) ejection fraction. Multimodal imaging allowed the diagnosis of sepsis-related myocardial calcification. Moreover, 2-dimensional speckle tracking echocardiography allowed a better understanding of LV dysfunction and confirmed the hypothesis that regional LV dysfunction is in accordance with the localization of calcifications. (Level of Difficulty: Intermediate.), Central Illustration

A 65-year-old immunocompromised woman presented with progressive dyspnea and sacroiliac joint pain. Cardiac magnetic resonance showed abnormal right ventricular filling with septal bounce and abnormal pericardial enhancement, suggestive of constrictive pericarditis. Cultures from pericardium following pericardiectomy grew Coccidioides immitis. She was diagnosed with coccidioidomycosis and responded to pericardiectomy and amphotericin. (Level of Difficulty: Intermediate.), Central Illustration

We present the case of a 16-year-old patient with anomalous left coronary artery from the left pulmonary artery requiring percutaneous coronary intervention in infancy who presented with ventricular fibrillation arrest. A coronary angiogram revealed 40% narrowing of the stent relative to the remainder of the left main coronary artery. Optical coherence tomography was performed and revealed an area stenosis of 70% relative to the native left main coronary artery. The patient had outgrown the stent. (Level of Difficulty: Advanced.), Central Illustration

A 40-year-old woman with a pulmonary embolism, central nervous system infarcts, and eosinophilia was referred for evaluation. Findings on echocardiography and cardiac magnetic resonance were consistent with eosinophilic myocarditis with left ventricular involvement. Further examination led to the diagnosis of Strongyloides stercoralis infection, and treatment with ivermectin and rivaroxaban resulted in clinical, laboratory, and cardiac imaging improvement. (Level of Difficulty: Intermediate.), Central Illustration

Background Radiation therapy (RT) results in myocardial changes consisting of diffuse fibrosis, which may result in changes in diastolic function. Objectives The aim of this study was to explore RT-associated changes in left ventricular (LV) diastolic function. Methods Sixty chemotherapy-naive patients with left-sided, early-stage breast cancer were studied with speckle tracking echocardiography at 3 time points: prior to, immediately after, and 3 years after RT. Global and regional early diastolic strain rate (SRe) were quantified, as were parameters of systolic function. Results Regional changes in SRe, particularly the apical and anteroseptal segments, were observed over time and were more evident than global changes. The apical SRe declined from a median of 1.24 (interquartile range: 1.01 to 1.39) s-1 at baseline to 1.02 (interquartile range: 0.79 to 1.15) s-1 at 3 years of follow-up (p < 0.001). This decline was associated with the left ventricular maximal radiation dose (β = 0.36, p = 0.007). The global SRe was, Central Illustration

Cell therapy is a promising tool to prevent and treat heart failure in congenital heart disease. We report the first case of intramyocardial injection of allogeneic mesenchymal stromal cells as rescue therapy in a neonate with ischemic heart failure following arterial switch procedure for isolated transposition of the great arteries. (Level of Difficulty: Advanced.), Central Illustration

MitraClip (Abbott Vascular, Inc., Santa Clara, California) has emerged as a viable alternative for treatment of symptomatic severe mitral regurgitation. Conduction abnormalities are not a known complication of this procedure. We report a case of complete heart block without a ventricular escape rhythm immediately following MitraClip placement near the medial leaflets (A3-P3) in a patient with underlying trifascicular block. (Level of Difficulty: Intermediate.), Central Illustration

Takeuchi repair is a unique surgical approach in anomalous left coronary artery from the pulmonary artery. We present an adult patient with anomalous left coronary artery from the pulmonary artery with multiple late structural complications after Takeuchi repair who was evaluated using multimodality imaging, including newly developed cardiac fusion imaging with computed tomography and echocardiography. (Level of Difficulty: Advanced.), Central Illustration

Objectives This meta-analysis evaluates assessment of pulmonary arterial hypertension (PAH), with a focus on clinical worsening and mortality. Background Cardiac magnetic resonance (CMR) has prognostic value in the assessment of patients with PAH. However, there are limited data on the prediction of clinical worsening, an important composite endpoint used in PAH therapy trials. Methods The Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, and Web of Science databases were searched in May 2020. All CMR studies assessing clinical worsening and the prognosis of patients with PAH were included. Pooled hazard ratios of univariate regression analyses for CMR measurements, for prediction of clinical worsening and mortality, were calculated. Results Twenty-two studies with 1,938 participants were included in the meta-analysis. There were 18 clinical worsening events and 8 deaths per 100 patient-years. The pooled hazard ratios show that every 1% decrease in right ventricular (RV) ejection fraction is associated with a 4.9% increase in the risk of clinical worsening over 22 months of follow-up and a 2.2% increase in the risk of death over 54 months. For every 1 ml/m2 increase in RV end-systolic volume index or RV end-diastolic volume index, the risk of clinical worsening increases by 1.3% and 0.7%, respectively, and the risk of mortality increases by 0.9% and 1%. Every 1 ml/m2 decrease in left ventricular end-systolic volume index or left ventricular end-diastolic volume index increased the risk of death by 2.1% and 2.3%. Left ventricular parameters were not associated with clinical worsening. Conclusions This review confirms CMR as a powerful prognostic marker in PAH in a large cohort of patients. In addition to confirming previous observations that RV function and RV and left ventricular volumes predict mortality, RV function and volumes also predict clinical worsening. This study provides a strong rationale for considering CMR as a clinically relevant endpoint for trials of PAH therapies., Central Illustration

A patient with history of dilated cardiomyopathy, a cardiac resynchronization therapy defibrillator, and endocardial ablation presented for refractory ventricular tachycardia 3 years after implantation of a Jarvik 2000 left ventricular assist device (Jarvik Heart, Inc., New York, New York). Open-chest epicardial ablation safely and effectively terminated the arrhythmia, without ventricular tachycardia recurrence at 9-month follow-up and in the absence of complications during the hospital stay. (Level of Difficulty: Advanced.), Central Illustration

Mitral regurgitation can have varying hemodynamic parameters dependent on factors such as pressure gradients, exercise, and/or provocative maneuvers. We present a case of unusual dynamic mitral regurgitation resolved by coughing in a patient with hypertrophic cardiomyopathy. (Level of Difficulty: Intermediate.), Central Illustration

A young woman presented with an acute ST-segment elevation myocardial infarction. Her clinical course was complicated by cardiogenic shock and acute renal failure. Work-up revealed thrombocytopenia and hemolytic anemia. A diagnosis of atypical hemolytic-uremic syndrome was made on the basis of clinical and pathological findings. (Level of Difficulty: Intermediate.), Central Illustration

Visual Abstract, Highlights • Myocardial matrix hydrogel preserves LV volumes and apical wall thickening in a chronic MI model. • Myocardial matrix hydrogel trends toward reduced fibrosis. • In vivo differential gene expression analysis shows the matrix modulates cardiac muscle contraction, metabolism, fibrosis, and the inflammatory/immune response in a chronic MI model., Summary A first-in-man clinical study on a myocardial-derived decellularized extracellular matrix hydrogel suggested the potential for efficacy in chronic myocardial infarction (MI) patients. However, little is understood about the mechanism of action in chronic MI. In this study, the authors investigated the efficacy and mechanism by which the myocardial matrix hydrogel can mitigate negative left ventricular (LV) remodeling in a rat chronic MI model. Assessment of cardiac function via magnetic resonance imaging demonstrated preservation of LV volumes and apical wall thickening. Differential gene expression analyses showed the matrix is able to prevent further negative LV remodeling in the chronic MI model through modulation of the immune response, down-regulation of pathways involved in heart failure progression and fibrosis, and up-regulation of genes important for cardiac muscle contraction.

Transcatheter edge-to-edge repair has revolutionized the management of mitral regurgitation in the high surgical-risk population. Iatrogenic atrial septal defects (iASDs) are an obligatory consequence of the procedure. The long-term sequelae of persistent iASDs are unknown but are believed to be dependent on their size, directionality of flow, and underlying hemodynamics. We discuss an uncommon scenario of a post–transcatheter edge-to-edge repair iASD that required immediate closure. (Level of Difficulty: Intermediate.), Graphical abstract

Intra-axial pumps are increasingly used to support cardiogenic shock. The occurrence of electrical storms in this setting is a rising issue, and data remain scarce about optimal management. We report the feasibility of ventricular tachycardia ablation in the presence of a recent surgically inserted Impella 5.0 device (Abiomed, Danvers, Massachusetts). (Level of Difficulty: Intermediate.), Graphical abstract

Currently adopted diagnostic flow charts consider transthyretin and light-chain cardiac amyloidosis as mutually exclusive. Here, we report for the first time, to our knowledge, the demonstration of a biopsy-proven dual pathology in an 80-year-old man with sequential development of both wild-type transthyretin amyloidosis and light-chain cardiac amyloidosis cardiomyopathy over a 3-year timespan. (Level of Difficulty: Intermediate.), Graphical abstract

After a 20-year-old woman suddenly died, autopsy showed characteristic findings of biventricular arrhythmogenic cardiomyopathy. Screening of her family members revealed the same desmoplakin gene mutation and imaging abnormalities predominantly involving the left ventricle. We describe the variable phenotypic expression in a family that shares a common gene variant. (Level of Difficulty: Advanced.), Graphical abstract

This report describes a young adult man presenting with subarachnoid hemorrhage secondary to an intracranial aneurysm who was found to have a short-segment type B interrupted aortic arch. We describe the clinical presentation, evaluation, and management of this patient and highlight imaging findings and percutaneous repair of the aneurysm and interrupted aortic arch. (Level of Difficulty: Intermediate.), Central Illustration

We describe a rare case of infiltrative cardiomyopathy characterized by multiple low-signal myocardial lesions consistent with nodular calcifications. A retrospectively detailed clinical history and the use of multimodality imaging enabled us to identify the final diagnosis. (Level of Difficulty: Advanced.), Central Illustration

ST-segment elevation is a dreadful finding in the emergency department because it is often associated with myocardial infarction and demands a prompt and definitive treatment. However, the clinical and echocardiographic assessment of a patient with electrocardiographic changes trumps any electrocardiology expert and should always lead to a clinical decision. (Level of Difficulty: Intermediate.), Central Illustration

Congenital left ventricular (LV) diverticulum is a rare condition characterized by the presence of a contractile appendix originating usually from the cardiac apex, but with high variability in location, dimension, and clinical presentation. We describe the diagnostic process and clinical management of an isolated apical diverticulum discovered during fetal life. (Level of Difficulty: Advanced.), Central Illustration

Visual Abstract, Highlights • hiPSCs are differentiated into CMs with large-scale 2-dimensional culture system, and refined by metabolic purification. • hiPSC-derived CMs are developed into CSs in special microwell plates. • Intramyocardial transplantation of CSs and GH improves cardiac function in small and large animal models. • Engraftment of CMs and angiogenesis are mechanisms for improvement of cardiac function. • Intramyocardial transplantation of CSs with a transplant injection device is a safe, effective, and feasible strategy for the treatment of HF., Summary The severe shortage of donor hearts hampered the cardiac transplantation to patients with advanced heart failure. Therefore, cardiac regenerative therapies are eagerly awaited as a substitution. Human induced pluripotent stem cells (hiPSCs) are realistic cell source for regenerative cardiomyocytes. The hiPSC-derived cardiomyocytes are highly expected to help the recovery of heart. Avoidance of teratoma formation and large-scale culture of cardiomyocytes are definitely necessary for clinical setting. The combination of pure cardiac spheroids and gelatin hydrogel succeeded to recover reduced ejection fraction. The feasible transplantation strategy including transplantation device for regenerative cardiomyocytes are established in this study.

Visual Abstract, Highlights • Gal-3 is a beta-galactoside–binding lectin that regulates inflammation and fibrosis and is highly predictive of heart failure events and mortality. • In a randomized placebo-controlled trial of MCP, Gal-3 inhibition did not influence collagen markers, echocardiographic measures, or vascular function. • Baseline Gal-3 levels were higher in women compared with men. • Consistent with previous studies, higher Gal-3 levels were associated with diabetes and reduced GFR., Summary We investigated the effect of galectin-3 (Gal-3) inhibition with modified citrus pectin on markers of collagen metabolism in a proof-of-concept randomized placebo-controlled trial of participants with elevated Gal-3 levels and hypertension. Although higher Gal-3 levels were associated with female sex, diabetes, and reduced glomerular filtration rate in cross-sectional analyses, treatment with modified citrus pectin did not change collagen markers. The effect of Gal-3 inhibition among individuals with heart failure warrants further investigation.

Visual Abstract, Highlights • Leptin protects against progression to heart failure after myocardial infarction. • This beneficial effect requires activation of the brain melanocortin system. • Stimulation of brain MC4R recapitulates the cardiac protective effects of leptin. • Leptin-MC4R activation improves cardiac substrate oxidation and mitochondrial function. • It also improves Ca2+ coupling and contractile function in viable cardiomyocytes after MI., Summary Heart failure has a high mortality rate, and current therapies offer limited benefits. The authors demonstrate that activation of the central nervous system leptin-melanocortin pathway confers remarkable protection against progressive heart failure following severe myocardial infarction. The beneficial cardiac-protective actions of leptin require activation of brain melanocortin-4 receptors and elicit improvements in cardiac substrate oxidation, cardiomyocyte contractility, Ca2+ coupling, and mitochondrial efficiency. These findings highlight a potentially novel therapeutic approach for myocardial infarction and heart failure.

A patient with a previous lung transplant and aortic valve replacement had progressive dyspnea. He presented with subacute tamponade secondary to a loculated pericardial effusion that caused impaired left ventricular filling and outflow tract obstruction secondary to distortion of the mitral valve apparatus. We demonstrate the imaging features of this presentation. (Level of Difficulty: Intermediate.), Graphical abstract

Transcatheter aortic valve replacement is currently used off-label for noncalcified aortic valve regurgitation and therefore is restricted to selected cases. In this setting we describe a rare complication of Sapien 3 (Edwards Lifesciences, Irvine, California) embolization from the left ventricle to the descending aorta. Given their technical challenges, such procedures require specific considerations and management. (Level of Difficulty: Advanced.), Graphical abstract

A feared complication of acute myocardial infarction is the formation of a cardiac pseudoaneurysm. We report a case of a gargantuan, arrhythmogenic left-ventricular pseudoaneurysm with contradictory morphological characteristics. The integrative use of high-resolution 3-dimensional magnetic resonance imaging and computed tomography proved essential for the diagnostic discrimination and successful therapeutic intervention. (Level of Difficulty: Advanced.), Graphical abstract