Red flags to diagnose infiltrative cardiomyopathies

Infiltrative cardiomyopathies are a group of diseases characterized by the deposition of abnormal substances in heart tissues, which leads to thickening of the walls or dilation of chambers with a secondary decrease in wall thickness and the development of diastolic, less often systolic, ventricular dysfunction. Most often, these are progressive diseases that, in the absence of adequate therapy, have an unfavorable prognosis. Clinical manifestations of infiltrative cardiac diseases are variable, which often leads to diagnostic difficulties and errors. In most cases, specific laboratory and morphological tests are required to confirm or clarify the diagnosis. Early diagnosis is critical to initiating therapy and improving patient prognosis. This article provides characteristic signs and symptoms, the so-called "red flags", making it possible to suspect infiltrative cardiomyopathies, diagnose them at an early stage and start life-saving therapy

Ventricular arrhythmias. Ventricular tachycardias and sudden cardiac death. 2020 Clinical guidelines

Russian Society of Cardiology (RSC).With the participation of Russian Scientific Society of Clinical Electrophysiology, Arrhythmology and Cardiac Pacing, Russian Association of Pediatric Cardiologists, Society for Holter Monitoring and Noninvasive Electrocardiology.Approved by the Scientific and Practical Council of the Russian Ministry of Health

Paediatric and adult congenital cardiology education and training in Europe

Background: Limited data exist on training of European paediatric and adult congenital cardiologists. Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. Results: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R-2 = 0.41). Conclusion: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.Developmen

The genetics of pulmonary hypertension

Recent clinical and experimental studies data are considering relating to the genetic causes of pulmonary arterial hypertension (PAH). The genetic abnormalities were first identified in association with the idiopathic and familial form of PAH, and some cases of secondary PAH following congenital heart defects (CHD). Genetic polymorphism of BMPR2, SMADs, ALK1/ENG were described as a reason of TGF-β-cell signaling disturbances. These genetic abnormalities were found in 6—18,2% of the patients with PAH owing to CHD. There are 70-80% heterozygote carriers of BMPR2-gene mutation among the relatives of familial PAH patients. The effects of the abnormal BMPR2 signaling pathway develop with the participation of ALK-receptors and transcriptional SMAD-proteins. Then BMPR2 и BMPR1B dysfunction culminate in an expressed smooth cells proliferative response that occludes the pulmonary arterial lumen and increases the apoptosis of the endoteliocytes in the small pulmonary arteries

Use of statins in children with familial hypercholesterolemia

Familial hypercholesterolemia Is the most common genetic disease in the world. It is characterized by an increase in the level of total cholesterol and low-density lipoproteins since childhood. It is desired to diagnose and start treatment in childhood prior to development of complications, such as aortic stenosis, atherosclerotic changes in the artery walls. Statins are recommended as the first-line medications. However, due to limited experience, the clinicians have different opinions regarding the age of initiation of hypolipidemic therapy. The article is aimed to critically examine the data on the use of statins in children with familial hypercholesterolemia, their effectiveness and safety available in the world literature