Clinical and genetic profiles of patients with hereditary and wild-type transthyretin amyloidosis: the Transthyretin Cardiac Amyloidosis Registry in the state of São Paulo, Brazil (REACT-SP).

Background: Transthyretin amyloidosis (ATTR) is a multisystem disease caused by the deposition of fibrillar protein in organs and tissues. ATTR genotypes and phenotypes are highly heterogeneous. We present data on physical signs and symptoms, cardiac and neurological assessments and genetic profile of patients enrolled in the Transthyretin Cardiac Amyloidosis Registry of the State of São Paulo, Brazil.

Assessment of potential transthyretin amyloid cardiomyopathy cases in the Brazilian public health system using a machine learning model.

Objectives: To identify and describe the profile of potential transthyretin cardiac amyloidosis (ATTR-CM) cases in the Brazilian public health system (SUS), using a predictive machine learning (ML) model.

Methods: This was a retrospective descriptive database study that aimed to estimate the frequency of potential ATTR-CM cases in the Brazilian public health system using a supervised ML model, from January 2015 to December 2021. To build the model, a list of ICD-10 codes and procedures potentially related with ATTR-CM was created based on literature review and validated by experts.

Focused Endocardial Septal Ablation: A New Technique to Treat Postmyectomy Recurrent Gradient.

This report describes the case of a 64-year-old woman with a previous diagnosis of obstructive hypertrophic cardiomyopathy who underwent surgical myectomy but who had a persistent midventricular residual gradient. The patient was symptomatic despite medical treatment and chose to undergo percutaneous radiofrequency (RF) ablation focused on the gradient. RF delivery was performed, and the gradient was reduced from the initial 105/68 mm Hg (during Valsalva maneuver/at rest before ablation) to 24/10 mm Hg.

Electrocardiography scar quantification correlates with scar size of hypertrophic cardiomyopathy seen by multidetector computed tomography.

Background: Hypertrophic cardiomyopathy (HCM), a genetically transmitted disease, is the most common genetic cardiovascular disease. Current strategies to stratify risk are expensive and concentrated in wealthy centers. Twelve-lead electrocardiography (ECG) is inexpensive, universally available, and can be readily used for Selvester QRS scoring, which estimates scar size.