Guidelines for the diagnosis and treatment of circulatory diseases in the context of the COVID-19 pandemic

Hypertrophic cardiomyopathy associated with damaging variants in the ALPK3 gene is a fairly recent discovery, and only a small number of patients have been described thus far. Here we present two additional patients with hypertrophic cardiomyopathy caused by biallelic variants in ALPK3. Genetic investigation was performed using a targeted gene panel consisting of known cardiomyopathy-associated genes and whole exome sequencing. The patients showed a large difference in the age of onset, and both presented with extracardiac features that are often seen in ALPK3 patients. The patient with the later onset showed milder extracardiac symptoms, such as decreased muscle tone and distal muscular dystrophy, but had fast progression of cardiac complications leading to the need of heart transplantation. This study further elucidates the variability of both symptoms and age of onset among these patients.

The extent of the problem of heart failure determines the priority of the intention of developing and implementing an optimal model of medical care for this group of patients. The article describes the key components of the heart failure medical care (continuity, patient routing, educational activities) and provides examples of documents, protocols, checklists that can be used in real clinical practice by specialists in medical organizations in the regions of the Russian Federation.

Objective: to estimate the survival of patients in the heart transplantation waiting list (HTx WL) at Almazov National Medical Research Centre (V.A. Almazov NMRC), Saint-Petersburg, Russia, from 2010 to 2018 and to define factors for death among these patients.Materials and methods: it was a single-centre retrospective study involving 151 patients with end-stage chronic heart failure (CHF) classes III-IV (NYHA) in the HTx WL.Results: the dynamic of decreasing mortality was revealed. Based on the results of constructing the discriminant function, four factors predicting the survival of patients in the HTx WL, namely, ACE inhibitors/ARBs, beta-blockers, status 1 of UNOS, CHF NYHA class IV, were pointed out. The model has sufficient resolving power but is not capable of predicting the outcome in the presence of acute decompensation of CHF.Conclusion: mortality in the HTx WL for the period 2010-2018 decreased that was associated with an active implementation of cardiac surgical methods as a «bridge» to transplant and optimal medical therapy, including ACE inhibitors/ARBs and beta-blockers in patients supported with inotropes (UNOS 1B). The greatest risk of mortality was found in CHF class IV and status 1 by UNOS.

The article highlights the central components of Russian heart failure (HF) management programs in actual clinical practice. The experience of the Competence Center of Almazov National Medical Research Center, as well as opportunities and prospects for improving the monitoring of decompensated HF.

This article is focused on the topicality of assessing complications and mortality from diseases of the circulatory system during the COVID-19 pandemic. The main variants of damage to the cardiovascular system, the mechanisms of their development, and risk factors are given. The long-term consequences of the new coronavirus infection for the heart and blood vessels are considered. In addition, the necessary measures to reduce the burden of disease after the pandemic are discussed.