This large contemporary cohort included 3,035 patients (median age 58 years, 57% men); 56% had LGE, including 242 (8%) with LGE > 10%. Most patients have obstructive HCM (71%), 1498 (69%) of which subsequently underwent septal reduction therapy (SRT) at a median of 43 days (interquartile range [IQR] 5-107) from the CMR date (1,485 myectomies and 13 alcohol septal ablations). Over a median of 8.8 years, 331 (11%) primary endpoints occurred (295 cardiovascular deaths [9.7%] and 36 appropriate ICD discharges [1.3%]). On multivariable Cox proportional hazards analysis (adjusted for standard clinical and CMR variables, higher %LGE (1.05 per 1% increase, 95% CI 1.03-1.06; p < 0.001) was associated with primary events. Compared with no LGE, LGE ≥10% was associated with higher risk of the composite endpoint.

Background Left ventricular (LV) myocardial extracellular volume (ECV), from cardiac magnetic resonance (CMR) T1 mapping, primarily reflects diffuse fibrosis and is a marker of adverse myocardial remodeling in hypertrophic cardiomyopathy (HCM). Objectives The authors sought to evaluate the prognostic value of ECV beyond established clinical and imaging variables in a large contemporary HCM cohort. Methods We evaluated 1,050 consecutive patients with HCM and preserved LV ejection fraction (≥50%) who underwent CMR, including quantification of ECV (%) and late gadolinium enhancement (LGE) burden (% LV mass, >6 SDs above remote myocardium), between 2012 and 2021. The primary endpoint was a composite of mortality, appropriate implantable cardioverter-defibrillator (ICD) therapy, or heart transplantation. Statistical methods included multivariable Cox proportional hazards regression, restricted cubic spline analysis, assessment of incremental discrimination using changes in the C-statistic, and net reclassification improvement analysis. Results Median age was 60 years (IQR: 49-68 years) with 44% women. Median follow-up was 5.6 years (IQR: 2.5-7.8 years). The primary endpoint occurred in 124 patients (11.8%), including 111 deaths (10.6%), 12 appropriate ICD discharges (1.1%), and 1 heart transplantation (0.1%). In multivariable Cox regression, older age (HR: 1.05, 95% CI: 1.03-1.06; P < 0.001), NYHA functional class ≥II (HR: 1.96, 95% CI: 1.23-3.11; P = 0.004), diabetes mellitus (HR: 1.89, 95% CI: 1.27-2.81; P = 0.002), and higher ECV (HR: 1.05 per 1% increase, 95% CI: 1.02-1.09; P = 0.001) were independently associated with the primary outcome. Patients with ECV ≥32% had lower event-free survival than those with ECV <32% (adjusted HR: 1.81, 95% CI: 1.24-2.64; P < 0.001). Conclusions Myocardial ECV was independently associated with long-term adverse outcomes. Multiparametric CMR assessment may improve risk stratification in HCM.

Cardiovascular complications are increasingly recognised as a major driver of morbidity and early mortality in patients with sickle cell disease (SCD), yet they remain underdiagnosed and underappreciated. This contemporary review synthesises current knowledge across a spectrum of cardiovascular manifestations—including myocardial dysfunction, pulmonary hypertension, cardiac iron overload, arrhythmias, myocardial infarction, stroke and sudden death—with emphasis on their unique pathophysiological mechanisms in SCD. We highlight emerging diagnostic tools such as cardiac magnetic resonance with T2* mapping and extracellular volume sequences, speckle-tracking echocardiography and invasive exercise testing, which can revealing a distinct phenotype combining restrictive cardiomyopathy and high-output heart failure. Practical algorithms for risk stratification and disease monitoring are presented alongside evidence-based and SCD-specific management approaches, including the role of hydroxyurea, transfusions, anticoagulation and gene therapy. By integrating multimodality imaging, updated guideline recommendations and recent clinical insights, this review provides a comprehensive resource to support early recognition, personalised therapy and improved cardiovascular outcomes in SCD.

Bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, characterized by the presence of two functional aortic valve leaflets instead of the usual three. It affects approximately 1 – 2% of the population and is often associated with other cardiovascular complications, including aortic stenosis, aortic regurgitation, and aortic root dilation. BAV can present with a wide range of clinical manifestations, from asymptomatic individuals to those with severe aortic valve dysfunction or aortic dissection. The diagnosis of BAV is commonly established through non-invasive imaging techniques such as transthoracic and transesophageal echocardiography, with advanced modalities, such as computed tomography and magnetic resonance imaging providing superior delineation of valve morphology, aortic root measurements, and associated pathology. Management of BAV is multifaceted and depends on the severity of valvular and aortic involvement. Lifelong surveillance is often necessary, with serial imaging to monitor disease progression. Surgical intervention is indicated for patients with severe valve dysfunction, symptomatic disease, or aortic aneurysm exceeding established threshold diameters. Common procedures include aortic valve replacement (AVR) and composite graft repair, such as the Bentall or valve-sparing David procedure. Transcatheter AVR has also emerged as a less invasive alternative to surgical valve replacement within this patient population, with its indications expanding significantly in recent years due to growing interest and advancements in the field. Despite substantial progress in diagnostic and therapeutic strategies, BAV poses clinical challenges due to its heterogeneous presentation, genetic underpinnings, and the requirement for tailored management approaches.

Background Cardiac tamponade is a rare but potentially lethal complication of transcatheter aortic valve replacement (TAVR). There is paucity of evidence assessing the incidence and outcomes of patients with cardiac tamponade after TAVR. Methods A retrospective review was performed of all patients who underwent TAVR at our institution from January 2013 to January 2019. The clinical characteristics of patients who developed cardiac tamponade in the periprocedural period were compared to the patients who did not develop tamponade. Qualitative and quantitative assessment of aortic annular calcium distribution on cardiac computerized tomography was analyzed. Results Twenty out of 2030 patients (0.9%) developed cardiac tamponade post-TAVR. The mean age of the cohort developing cardiac tamponade was 81.7 years, and 50% of them were men. Most of these were intraprocedural (70%) while the remaining were identified in the postprocedural period. The site of injury resulting in pericardial tamponade was thought to be from the injury to aortic annulus (50%), right ventricle (40%), and left ventricle (10%). Tamponade due to annular or left ventricular trauma was mostly identified intraprocedurally (91%; n ​= ​10 of 11), while patients with tamponade due to presumed right ventricular injury were mostly identified in the postprocedural period (62.5%; n ​= ​5 of 8) (p ​= ​0.009). Conservative management with supportive therapies was employed in 90% of patients with cardiac tamponade, while two patients had cardiac surgery. There was one in-hospital mortality, and another patient died within 30 days of the TAVR procedure. Conclusion The incidence of cardiac tamponade after TAVR (0.9%) was low, and this serious complication can be managed successfully in the majority of patients with streamlined processes in high-volume centers.

BACKGROUND:Merkel cell carcinoma (MCC) is an aggressive neuroendocrine malignancy with high metastatic potential. Cardiac involvement is rare and usually occurs late in the disease course. FIRST-IN-HUMAN/EARLY REPORTS SUMMARY:A 76-year-old man with recurrent MCC was found to have a large epicardial mass encasing the right coronary artery. Surgical and external percutaneous biopsy approaches were considered high risk. DISCUSSION:A transcatheter biopsy was performed using multimodality imaging. Intracardiac echocardiography enabled real-time visualization, while transesophageal echocardiography provided higher resolution to confirm accurate forceps positioning. Diagnostic tissue was successfully obtained, confirming recurrent MCC and informing subsequent oncologic therapy. NOVELTY:This appears to be the first description of transcatheter biopsy of an epicardial mass guided by both intracardiac and transesophageal echocardiography. TAKE-HOME MESSAGES:Multimodality imaging can facilitate safe biopsy of select cardiac masses. Tissue confirmation remains important when management decisions depend on histology.

With rising life expectancy, the population of older adults over the age of 65 is rapidly expanding, with women comprising the majority. Structural heart disease is common in this group, and transcatheter interventions have transformed its management. Optimal outcomes require careful consideration of sex-specific differences. This review examines transcatheter structural heart interventions in older adults with a focus on sex-based outcomes, procedural planning, and current knowledge gaps.

Cardiac SPECT-CT and PET-CT studies often have clinically actionable non-cardiac pathologies, making thorough interpretation critical. The American Society of Nuclear Cardiology (ASNC) emphasizes importance of reviewing both cardiac and extracardiac regions to ensure that significant incidental findings are not overlooked. Although CT images used for attenuation correction are often low in resolution and lack diagnostic detail, they can still reveal incidental findings that warrant further evaluation with dedicated imaging. In this manuscript, we highlight clinically relevant extracardiac and limited upper abdominal findings that may be of value to the interpreting physicians.