Incidence Rates of Penn Classes and Class-Specific Mortality in Acute Type A Aortic Dissection Patients: An Epidemiologic Systematic Review and Meta-Analysis.

Acute type A aortic dissection (ATAAD) is a life-threatening emergency that is associated with a high morbidity and mortality rate. One of the complications is end-organ ischemia, a known predictor of mortality. The primary aims of this meta-analysis were to summarize the findings of observational studies investigating the utility of the Penn classification system and to analyze the incidence rates and mortality patterns within each class. The electronic databases PubMed, MEDLINE, and Embase were searched through to April 2023. These were filtered by multiple reviewers to give 10 studies that met the inclusion criteria. The extracted data included patient characteristics, and primary outcomes were the incidence rates of different Penn classes, along with the corresponding mortality for each class. Out of 1,512 studies identified during the initial search, 10 studies, including 4,494 patients, met the inclusion criteria. The pooled incidence of Penn A was highest at 0.55 (95% CI 0.52, 0.58), followed by Penn B at 0.21 (95% CI 0.17, 0.25), and finally Penn C at 0.14 (95% CI 0.11, 0.17). Patients with Penn BC were found to be at the highest risk of death, as their early mortality rates were 0.36 (95% CI 0.31, 0.41). Within those populations, the subtype with the highest individual mortality was Penn C at 0.21 (95% CI 0.15, 0.27), followed by Penn B at 0.19 (95% CI 0.15, 0.23) and Penn A at 0.07 (95% CI 0.05, 0.10). Among patients presenting with ATAAD, class A was most frequently observed, followed by classes B, C, and BC. These findings indicate an incremental increase in mortality rates with the progression of Penn classification.

Blastic plasmacytoid dendritic cell neoplasm: a rare external ear lesion presenting with leukaemia.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy, typically characterised by cutaneous lesions and bone marrow involvement. We present a unique case of a woman in her 70s, initially seen for a spontaneous swelling on her left external ear resembling a haematoma, which recurred after initial treatment, triggering further evaluation.Diagnostic challenges arose as the patient displayed positive markers for Myeloperoxidase (MPO) (p-ANCA), suggesting vasculitis. Dermatology considered various differential diagnoses, but imaging and tests ruled out significant pathology. Steroid treatment led to improvement, but coincided with a surge in white cell count (WCC), prompting an urgent haematological review.Subsequent investigations, including a punch biopsy of the external ear and a bone marrow biopsy revealed BPDCN concurrent with chronic myelomonocytic leukaemia. This case highlights the challenging diagnostic journey, emphasising the need for multidisciplinary collaboration and the potential for unique BPDCN presentations, expanding our understanding of this malignancy.

Advances in pharmacotherapy for heart failure and reduced ejection fraction: what's new in 2024?

INTRODUCTION: Updated guidelines for heart failure with reduced ejection fraction (HFrEF) and acute decompensation have improved outcomes, but ongoing efforts are focused on uncovering new evidence and developing novel therapies. This review examines the limitations of current treatments and the potential impact of emerging therapies. AREAS COVERED: A literature search focused on studies investigating drugs for HFrEF. We review recent clinical trials and emerging therapies to assess evidence strength, explore guideline updates, and identify strategies to optimize patient outcomes. EXPERT OPINION: The HFrEF treatment landscape is rapidly evolving, with advances in therapies like sodium/glucose cotransporter inhibitors and sacubitril-valsartan. Though managing acute decompensated heart failure remains challenging, recent trials suggest improvements in diuretic strategies and anti-inflammatory treatments. Ongoing research is essential for validating these therapies and incorporating them into standard practice.

Mitral valve diseases: Pathophysiology and interventions.

Valvular heart disease is common and increasingly prevalent among the elderly. The end result of valvular pathologies is cardiac failure and can lead to sudden death; thus, diagnosis and interventions are very important in the early stages of these diseases. The usual treatment methods of mitral regurgitation include percutaneous mitral valve repair, mitral valve replacement and minimally invasive surgery, whereas the treatment methods of mitral stenosis include percutaneous transluminal mitral commissurotomy and mitral commissurotomy as well as open surgical repair. Nonetheless, ongoing clinical trials are a clear indicator that the management of valve diseases is ever evolving. The focus of this paper is on the various pathologies of the mitral valve, their etiology and clinical management, offering a comprehensive view of mitral valve diseases.