Protocol for 3D photogrammetry and morphological digitization of complex skulls.

Here, we present a protocol for 3D photogrammetry and morphological digitization of skulls, including complex ones with tusks, antlers, and horns, which are challenging to reconstruct digitally. We describe steps for setting up specimens for image acquisition, including camera and lighting configurations, and the subsequent image processing to generate high-quality 3D models. We also outline the extraction of morphological data for accurate geometric morphometric analyses.

Anderson-Fabry Disease: Focus on Ophthalmological Implications.

Fabry disease (FD) is a rare X-linked lysosomal storage disorder with a broad spectrum of clinical manifestations, including severe complications, such as end-stage renal disease, hypertrophic cardiomyopathy, and cerebrovascular disease. Enzyme replacement therapy (ERT), when initiated early, has been shown to reduce the incidence of severe events and slow disease progression. In the classic form, characterized by the absence of α-galactosidase A (α-Gal A) enzyme activity, diagnosis is straightforward.

Cardiac contractility modulation: from molecular patterns to tailored treatment in heart failure subgroups.

Cardiac contractility modulation (CCM) signals are non-excitatory signals that are applied during the myocyte's absolute refractory period. These signals have been demonstrated to have an inotropic effect without increasing myocardial oxygen consumption. This has been observed in both preclinical animal studies and randomized clinical trials.

Thromboembolic Risk in Sinus Rhythm: A New Paradigm in Light Chain Cardiac Amyloidosis.

Thromboembolic risk is increased in cardiac amyloidosis, and this goes beyond the occurrence of atrial fibrillation in these patients. A 56-year-old man was admitted to our hospital for a presyncopal episode. Clinical and instrumental findings led to a diagnosis of light chain cardiac amyloidosis.

Efficacy of Modern Therapies for Heart Failure with Reduced Ejection Fraction in Specific Population Subgroups: A Systematic Review and Network Meta-Analysis.

Introduction: The efficacy and safety of emerging therapies for heart failure with reduced ejection fraction (HFrEF) have never been compared in specific subgroups of patients.

Methods: PubMed, Cochrane Registry, Web of Science, Scopus, and EMBASE libraries were used to extract data. We used the following keywords: (heart failure with reduced ejection fraction OR HFrEF) AND (treatment OR therapy) OR (cardiovascular death) OR (hospitalization for heart failure).

Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study.

Transthyretin-mediated amyloidosis (ATTR) is a systemic disease with protein precipitation in many tissues, mainly the peripheral nerve and heart. Both genetic (ATTRv, "v" for variant) and wild-type (ATTRwt) forms are known. Beyond the steric encumbrance, precipitated transthyretin seems to have a toxic effect.

Usefulness of empiric superior vena cava isolation in paroxysmal atrial fibrillation ablation: a meta-analysis of randomized clinical trials.

Background: The long-term success rate of pulmonary vein isolation (PVI) is suboptimal due to the presence of non-pulmonary vein (PV) foci that can trigger atrial fibrillation (AF) in up to 11%. Among non-PV triggers, the superior vena cava (SVC) is a major site of origin of ectopic beats initiating AF.

Objective: To compare data from randomized controlled trials (RCTs) assessing PVI + empiric SVC isolation (SVCI) versus PVI alone in terms of AF recurrence, procedure-related complications, and fluoroscopic and procedural times.

Immunomodulating and Immunosuppressive Therapy for Virus-Negative Immune-Mediated Myocarditis.

Myocarditis is an inflammatory disease of the myocardium caused by infectious and noninfectious agents. Clinical manifestations range from mildly symptomatic forms to acute heart failure, cardiogenic shock, life-threatening arrhythmias and sudden death. Myocarditis is still a challenging diagnosis because of its wide variability in clinical presentation and unpredictable course.

Probability Score to Predict Spontaneous Conversion to Sinus Rhythm in Patients with Symptomatic Atrial Fibrillation When Less Could Be More?

The probability of spontaneous conversion (SCV) to sinus rhythm (SR) in patients presenting to the emergency department (ED) with hemodynamically stable, symptomatic atrial fibrillation (AF) is not well known. To develop and validate a score to determine the probability of SCV to SR in patients presenting to the ED with hemodynamically stable, symptomatic AF. This retrospective, observational study enrolled consecutive patients admitted with AF to the ED.

Updated Evaluation of Agalsidase Alfa Enzyme Replacement Therapy for Patients with Fabry Disease: Insights from Real-World Data.

The clinical use of agalsidase alfa as enzyme replacement therapy (ERT) for Fabry disease (FD) has spread since 2001, and a large body of evidence of its effectiveness has been collected. This review presents the clinical and laboratory results achieved with agalsidase alfa, which has been published in the literature. Agalsidase alfa infusion slows down or stops the progression of renal damage, expressed by reduction or stabilization of the annual decline of the glomerular filtration rate; yearly decrease of glomerular filtration rate (slope) sometimes is reduced until its stabilization.

Long-Term Clinical-Pathologic Results of Enzyme Replacement Therapy in Prehypertrophic Fabry Disease Cardiomyopathy.

Background: The limited ability of enzyme replacement therapy (ERT) in removing globotriaosylceramide from cardiomyocytes is recognized for advanced Fabry disease cardiomyopathy (FDCM). Prehypertrophic FDCM is believed to be cured or stabilized by ERT. However, no pathologic confirmation is available.

Mechanical Circulatory Support Systems in the Management of Ventricular Arrhythmias: A Contemporary Overview.

Ventricular tachycardias (VTs) and electrical storms (ES) are life-threatening conditions mostly seen in the setting of structural heart disease (SHD). Traditional management strategies, predominantly centered around pharmacological interventions with antiarrhythmic drugs, have demonstrated limited efficacy in these cases, whereas catheter ablation is related with more favorable outcomes. However, patients with hemodynamically unstable, recurrent VT or ES may present cardiogenic shock (CS) that precludes the procedure, and catheter ablation in patients with SHD portends a multifactorial intrinsic risk of acute hemodynamic decompensation (AHD), that is associated with increased mortality.

Inherited Arrhythmias in the Pediatric Population: An Updated Overview.

Pediatric cardiomyopathies (CMs) and electrical diseases constitute a heterogeneous spectrum of disorders distinguished by structural and electrical abnormalities in the heart muscle, attributed to a genetic variant. They rank among the main causes of morbidity and mortality in the pediatric population, with an annual incidence of 1.1-1.

Anderson-Fabry Disease: Red Flags for Early Diagnosis of Cardiac Involvement.

Anderson-Fabry disease (AFD) is a lysosome storage disorder resulting from an X-linked inheritance of a mutation in the galactosidase A (GLA) gene encoding for the enzyme alpha-galactosidase A (α-GAL A). This mutation results in a deficiency or absence of α-GAL A activity, with a progressive intracellular deposition of glycosphingolipids leading to organ dysfunction and failure. Cardiac damage starts early in life, often occurring sub-clinically before overt cardiac symptoms.

[Anticoagulant therapy in left ventricular non-compaction: when, how and why].

Left ventricular non compaction (LVNC) comprises a heterogeneous group of diseases that can cause heart failure, arrhythmias, and thromboembolic events. In particular, the prevalence of thromboembolism in patients with LVNC is relevant compared to the general population. Atrial fibrillation and left ventricular thrombosis are strong predictors and require anticoagulant treatment in primary or secondary prevention, with a significant reduction in the risk of events.

"Function follows form": Role of cardiac magnetic resonance for ventricular arrhythmia risk stratification in patients with cardiac sarcoidosis.

Introduction: Cardiac involvement is common and may become clinically relevant in approximately 5%-10% of patients with systemic sarcoidosis. Although reduced left ventricular ejection fraction is a recognized predictor of mortality, recent studies have suggested an increased risk of ventricular arrhythmia (VAs) and sudden cardiac death (SCD) in patients with cardiac sarcoidosis (CS) and evidence of late gadolinium enhancement-cardiac magnetic resonance (LGE-CMR), irrespective of the underlying left ventricular systolic function. We performed a meta-analysis to assess the correlation between VAs/SCD and presence of LGE-CMR in CS patients.

Does Patisiran Reduce Ocular Transthyretin Synthesis? A Pilot Study of Two Cases.

Background: Variant transthyretin-mediated amyloidosis (ATTR-v) is a well-characterized disease affecting the neurologic and cardiovascular systems. Patisiran has been approved for neurologic involvement as it reduces hepatic synthesis of transthyretin (TTR). Eye involvement is a lateonset feature increasing the risk of glaucoma and cataracts in patients.

The Role of MicroRNA in the Myocarditis: a Small Actor for a Great Role.

Purpose Of Review: Myocarditis is an inflammation of the myocardium secondary to a variety of agents such as infectious pathogens, toxins, drugs, and autoimmune disorders. In our review, we provide an overview of miRNA biogenesis and their role in the etiology and pathogenesis of myocarditis, evaluating future directions for myocarditis management.

Recent Findings: Advances in genetic manipulation techniques allowed to demonstrate the important role of RNA fragments, especially microRNAs (miRNAs), in cardiovascular pathogenesis.

ANMCO position paper: guide to the appropriate use of the wearable cardioverter defibrillator in clinical practice for patients at high transient risk of sudden cardiac death.

Extended risk stratification and optimal management of patients with a permanently increased risk of sudden cardiac death (SCD) are becoming increasingly important. There are several clinical conditions where the risk of arrhythmic death is present albeit only transient. As an example, patients with depressed left ventricular function have a high risk of SCD that may be only transient if there will be a significant recovery of function.