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Research Reports in Clinical Cardiology

Publishes open access research on cardiovascular diseases and prevention, arrythmia, heart failure, adult congenital disease, methodologies and treatment.

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Research Reports in Clinical Cardiology publishes evidence-based original research, reports, editorials, perspectives, reviews and commentaries covering the following topics:

  • metabolic disorders influencing cardiac risk
  • coronary heart disease
  • arrhythmias and pacing
  • heart failure
  • cardiomyopathies
  • methodologies
  • percutaneous and invasive techniques
  • adult congenital heart disease
  • cardiovascular genetics

Emphasis is placed upon translation of important basic research, updated information about classical topics, scholarly discussions of controversial topics, and incisive, salient manuscripts relevant either to conceptualization or practice of cardiology.

Research Reports in Clinical Cardiology will no longer consider meta-analyses for publication.

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Clinical Cardiology Research and Reports

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Clinical Cardiology Research and Reports (CCRR) is a Peer-reviewed, Open Access journal that publishes original research articles, review articles, and clinical studies that focus on the diagnosis and treatment of cardiovascular disease. The journal welcomes submissions related to systemic hypertension, arrhythmia, congestive heart failure, valvular heart disease, vascular disease, congenital heart disease, and cardiomyopathy. Manuscript types include original article, review, case report, short communication, book review, letter to the editor, etc.

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research reports in clinical cardiology

Research Reports in Clinical Cardiology

Issn: 1179-8475.

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research reports in clinical cardiology

Editor-in-Chief: Dr Richard Kones

Research Reports in Clinical Cardiology publishes evidence-based original research, reports, editorials, perspectives, reviews and commentaries covering the following topics:   • prevention • metabolic disorders influencing cardiac risk • coronary heart disease • arrhythmias and pacing • heart failure • cardiomyopathies • methodologies • treatment • percutaneous and invasive techniques • outcomes • adult congenital heart disease • cardiovascular genetics

Emphasis is placed upon translation of important basic research, updated information about classical topics, scholarly discussions of controversial topics, and incisive, salient manuscripts relevant either to conceptualization or practice of cardiology.

Research Reports in Clinical Cardiology will no longer consider meta-analyses for publication. 

Related journals you may also be interested in:

  • Diabetes, Metabolic Syndrome and Obesity
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  • v.39(1); 2016 Jan

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Objectives and Design of the Russian Acute Coronary Syndrome Registry (RusACSR)

Vladimir i. gridnev.

1 Department of New Cardiological Informational Technologies, Research Institute of Cardiology, Saratov State Medical University n.a. V.I. Razumovsky, Saratov, Russia

Anton R. Kiselev

Olga m. posnenkova, yulia v. popova, viktor a. dmitriev.

2 Department of Registries of Cardiovascular Diseases, Russian Cardiology Research and Production Complex, Moscow, Russia

Mikhail D. Prokhorov

3 Laboratory of Nonlinear Dynamics Modelling, Saratov Branch of the Institute of Radio Engineering and Electronics of the Russian Academy of Sciences, Saratov, Russia

Pavel Ya. Dovgalevsky

Elena v. oschepkova, associated data.

The Russian Acute Coronary Syndrome Registry ( RusACSR ) is a retrospective, continuous, nationwide, Web‐based registry of patients with acute coronary syndromes ( ACS ). The RusACSR is a database that uses a secure Web‐based interface for data entry by individual users. Participation in the RusACSR is voluntary. Any clinical center that provides health care to ACS patients can take part in the RusACSR . The RusACSR enrolls ACS patients who have undergone care in Russian hospitals from February 2008 to the present. Key data elements and methods of data analysis in the RusACSR are presented in this article. Up to 2015, 213 clinical centers from 36 regions of Russia had participated in the RusACSR . Currently, the database contains data on more than 250 000 ACS patients who underwent care from 2008 to 2015. Some current problems are highlighted in this article. The RusACSR is a perspective project for different epidemiologic studies in Russian ACS patients.

Introduction

There are a number of registries from various countries that include patients with acute coronary syndromes (ACS). 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 The main goal of these registries is to fill the gap between probative data of randomized controlled trials and real clinical practice. 12 , 13 The main functions of the ACS registries are (1) to report on clinical and demographic characteristics of patients with ACS in covered cohorts; and (2) to assess the treatment of ACS in accordance with the current guidelines.

The registries can also be used for epidemiologic studies, original studies, risk modeling, improving the quality of health care, and other objectives. The registry is an ideal tool for studying the real clinical practice, especially when it is necessary to improve the quality of health care.

It is known that the quality of health care in ACS patients, especially revascularization in ACS patients with ST‐segment elevation on electrocardiogram (STE‐ACS), requires improvement in many countries. 14 , 15 , 16 Within the framework of the Russian Federal Target Program Prevention and Control of Social Diseases (2007–2011) and the All‐Russian Vascular Program, in 2008 the Russian Acute Coronary Syndrome Registry (RusACSR; https://federalregistry.ru ) was established. This registry is still functioning.

The aim of this article is to describe the objectives and design of the RusACSR. The presented results may be interesting to a wide audience, as the RusACSR is currently the largest registry of ACS patients in Russia.

Description of the Russian Acute Coronary Syndrome Registry

The RusACSR has the following main objectives: (1) to create a national database with information on health care in ACS patients treated in Russia; (2) to obtain data on the demographic, clinical, and laboratory characteristics of ACS patients treated in Russia; (3) to identify the national features of associations between the characteristics of ACS and clinical outcomes, including mortality, complications, length of hospital stay, and quality of health care; and (4) to propose a practical guide for improving the quality and efficiency of ACS treatment in each clinical center participating in the RusACSR.

The Russian Cardiology Research and Production Complex (Moscow, Russia) was responsible for the development of the RusACSR and centralized data analysis at a federal level. The RusACSR was established in 2007 and 2008 by researchers, cardiologists, and information technology specialists from the Saratov Research Institute of Cardiology (Saratov, Russia). The current support of the RusACSR is carried out by the staff of both above‐mentioned organizations.

Participation

Participation in the RusACSR is voluntary and free of charge. Any clinical center that provides health care for ACS patients can participate in the RusACSR by sending a request to the technical support staff of the RusACSR. Starting in 2008, most of the main centers participating in the All‐Russian Vascular Program were invited to take part in the RusACSR by the Russian Cardiology Research and Production Complex. Up to 2015, 213 clinical centers from 36 regions of Russia had participated in the RusACSR (Figure ​ (Figure1) 1 ) . 17

CLC-22495-FIG-0001-c

Maps with clinical centers participated in the RusACSR. Abbreviations: RusACSR, Russian Acute Coronary Syndrome Registry.

The majority of centers participating in the RusACSR are located in the Central Federal Region of the Russian Federation, and several centers are located in the North‐West Federal Region, South Federal Region, Ural Federal Region, and Siberian Federal Region. Thus, registry centers represent federal districts where the majority of the Russian population lives. Of course, these regions are socioeconomically heterogeneous, but an annual public report contains the data on each region separately. 17

Design of the Russian Acute Coronary Syndrome Registry

The RusACSR is established as a retrospective, continuous, nationwide, Web‐based registry operating online ( https://federalregistry.ru ). The design of the RusACSR is based on the main points of the clinical guidelines on diagnosis and treatment of ACS. 18 , 19 , 20 , 21

Access to the Registry is given to registered members. Each user has a unique identification number and password to log in to the database. The Web forms are designed to be interactive. They limit or exclude certain options to avoid entry of conflicting or spurious data. Wherever possible, the data are entered by selection from drop‐down menus to minimize the number of keyboard errors. The purpose of all these measures is to maximize the accuracy of data.

The Web interface of the RusACSR contains 11 Web forms with the following titles: (1) personal data of ACS patients; (2) history of present event of ACS; (3) past history; (4) results of physical examination; (5) results of instrumental examinations; (6) results of laboratory tests; (7) invasive intervention; (8) prior therapy; (9) drug treatment of ACS; (10) recommendations at discharge; and (11) complications and outcomes.

The RusACSR enrolls patients who were admitted for ACS (ie, unstable angina or myocardial infarction [MI]) and underwent care in hospitals in Russia. Enrollment of patients started in February 2008 and has continued to the present day.

Inclusion criteria comprise any type of ACS (ie, unstable angina or MI) as a presumptive diagnosis, age ≥18 years, finished patient hospital chart, and absence of any exclusion criteria. 22

The RusACSR exclusion criteria are the following: (1) symptoms considered as consistent with acute cardiac ischemia are absent within the last 24 hours prior to admission; (2) patient was transferred into a registry hospital >24 hours after admission to the initial hospital; (3) patient was transferred out of a registry hospital <24 hours after admission; (4) symptoms considered as ACS at admission were not consistent with acute cardiac ischemia; and (5) ACS accompanied by a significant comorbidity, such as a motor vehicle accident, trauma, or severe gastrointestinal bleeding, and operation or procedure directly before admission. 22

A flow diagram of patient selection for inclusion in the RusACSR is presented in Figure ​ Figure2 2 .

CLC-22495-FIG-0002-b

Flow diagram of patients' selection for inclusion to the RusACSR. Abbreviations: ACS, acute coronary syndrome; RusACSR, Russian Acute Coronary Syndrome Registry.

Data Elements

The key data elements and definitions of the RusACSR database were developed using the American College of Cardiology (ACC) 2001 Key Data Elements and Definitions for Measuring the Clinical Management and Outcomes of Patients with ACS 23 and the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) 2011 Key Data Elements and Definitions of a Base Cardiovascular Vocabulary for Electronic Health Records. 24 After 2013, we adopted the RusACSR to the 2013 ACCF/AHA Key Data Elements and Definitions for Measuring the Clinical Management and Outcomes of Patients with ACS and Coronary Artery Disease. 25

Data on patient demographics, clinical characteristics, previous and hospital drug treatment, and reperfusion therapy are collected. Data on post‐hospital treatment of ACS patients are not included in the Registry. There is no capacity to follow patients after hospital discharge. (For details on the key data elements of the RusACSR database, see Supporting Information, Appendix A, in the online version of this article).

Data Collection

The centers participating in the RusACSR were asked to include all patients following inclusion/exclusion criteria treated for ACS during the year prior to the year of participation. The source of patient data is the hospital chart. To help the participants, a detailed user manual was developed. 22 This user manual is available on the RusACSR Web site.

The centers are interested in accurate data collection because these data are analyzed further by experts separately for each center and used on‐site for health care quality management. In each center, ≥1 physicians have been trained to log patient data into the Registry. The content of data‐entry Web forms is intuitive for 88% of untrained users. 26 Each year, experts from the RusACSR check the validity of entered data by reconciling randomly selected records with the data of patients' hospital charts. Currently, the RusACSR contains data on >250 000 ACS patients treated from 2008 to 2015. The main baseline characteristics of patients included in the RusACSR from February 1, 2008, to October 22, 2015, are presented in the Table ​ Table1 1 .

Baseline Characteristics of ACS Patients Included in the RusACSR From February 1, 2008 to October 22, 2015

ParametersValues
Demographic and clinical characteristics (n = 254 584)
Male sex49.8
Age, y67 (58–77)
Prior MI28.6
Prior chest pain55.5
Prior PCI4.5
Prior CABG<0.1
Prior CHF43.8
Prior stroke and/or TIA7.6
PVD6.3
Family history of CAD19.9
Hypertension79.3
Smoking22.8
DM15.3
STE‐ACS patients33.6
NSTE‐ACS patients49.2
Heart rate at admission, bpm76 (68–86)
SBP at admission, mm Hg140 (120–160)
DBP at admission, mm Hg85 (80–90)
Killip class at admission (n = 169 007)
I71.4
II21.3
III4.5
IV2.8
TC, mmol/L5.0 (4.2–6.0)
TG, mmol/L1.4 (1.0–2.0)
Cr, µmol/L92 (76–111)
Blood glucose, mmol/L5.6 (4.9–7.0)
ACS patients who underwent PCI16.6
Prior medical therapy (n = 197 428)
ASA45.6
Clopidogrel9.5
Nitrates34.6
ACEIs47.8
ARBs7.1
β‐Blockers43.2
Dihydropyridine CCBs9.1
Non‐dihydropyridine CCBs2.0
Warfarin1.9
Reperfusion therapy in STE‐ACS patients (n = 85 496)
Fibrinolitic therapy27.6
Fibrinolitic therapy before hospital arrival8.0
Time from chest pain (or its equivalent) to fibrinolitic therapy, min (n = 20 544)180 (120–300)
PCI (n = 85 496)29.4
Primary PCI (n = 85 496)24.0
Time from chest pain (or its equivalent) to PCI, min (n = 20 436)330 (200–750)
Drug therapy in ACS patients (STE‐ACS patients, n = 85 496; NSTE‐ACS patients, n = 125 228)
Antiplatelet agents
STE‐ACS patients97.8
NSTE‐ACS patients97.3
ASA
STE‐ACS patients96.4
NSTE‐ACS patients95.5
Clopidogrel
STE‐ACS patients84.4
NSTE‐ACS patients76.4
Anticoagulants
STE‐ACS patients95.3
NSTE‐ACS patients94.0
β‐Blockers
STE‐ACS patients89.0
NSTE‐ACS patients89.8
Statins
STE‐ACS patients85.4
NSTE‐ACS patients87.1
ACEIs or ARBs
STE‐ACS patients82.8
NSTE‐ACS patients87.0
Outcomes (STE‐ACS patients, n = 85 496; NSTE‐ACS patients, n = 125 228)
In‐hospital cardiogenic shock
STE‐ACS patients5.6
NSTE‐ACS patients1.2
Death during hospital stay
STE‐ACS patients7.3
NSTE‐ACS patients2.1
Death during first day after admission
STE‐ACS patients3.4
NSTE‐ACS patients0.8
GRACE risk score for in‐hospital death (STE‐ACS patients, n = 85 496; NSTE‐ACS patients, n = 125 228)
STE‐ACS patients with data for calculating GRACE risk of in‐hospital death, n (%)40 214 (47.0)
NSTE‐ACS patients with data for calculating GRACE risk of in‐hospital death, n (%)27 896 (22.3)
STE‐ACS patients (n = 40 214)
Low risk22.2
Medium risk32.9
High risk44.9
NSTE‐ACS patients (n = 27 896)
Low risk26.6
Medium risk29.8
High risk43.6

Abbreviations: ACEI, angiotensin‐converting enzyme inhibitors; ACS, acute coronary syndrome; ARB, angiotensin II receptor blocker; ASA, aspirin; CABG, coronary artery bypass grafting; CAD, coronary artery disease; CCB, calcium channel blocker; CHF, chronic heart failure; Cr, creatinine; DBP, diastolic blood pressure; DM, diabetes mellitus; GRACE, Global Registry of Acute Coronary Events; IQR, interquartile range; MI, myocardial infarction; NSTE‐ACS, non–ST‐segment elevation acute coronary syndrome; PCI, percutaneous coronary intervention; PVD, peripheral vascular disease; RusACSR, Russian Acute Coronary Syndrome Registry; SBP, systolic blood pressure; STE‐ACS, ST‐segment elevation acute coronary syndrome; TC, total cholesterol; TG, triglycerides; TIA, transient ischemic attack.

Data are presented as % or median (IQR), unless otherwise noted.

As for the proportion of ACS presentations included in the Registry since 2008, we have only an approximate estimation because the public statistics contain only the data on MI, and not on ACS morbidity across Russia. There are about 185 000 MIs per year in Russia. The RusACSR contains data on about 100 000 MI patients.

Data Security

Some of the main features of database and Web security issues should be mentioned briefly. As mentioned above, all users are assigned a unique username/password combination that is used to log on to the RusACSR. In this way, all transactions are recorded automatically in the Web server's log.

All the data are pseudonymously entered into a Web‐based database protected by a password on a safe server of the Russian Cardiology Research and Production Complex using secure sockets layer (SSL) connections. Subject identification is possible only at the local study site, and participating centers are exclusively able to review and modify the patient data. The data on ACS patients added to the RusACSR can be changed but cannot be removed. The transmitted data are stored in the central database on the central server at the Russian Cardiology Research and Production Complex.

The purpose of all the above‐mentioned measures is to ensure the confidentiality of the data.

Ethical Aspects

The study protocol including patient information and consent forms has been reviewed and approved by the Ethics Commission of the Russian Cardiology Research and Production Complex.

All patients must give informed consent before inclusion of their personal and clinical data in the RusACSR. The standard informed‐consent form is available on the RusACSR Web site. Patients gave their informed consent after transfer from an intensive care unit to a cardiac/coronary care unit. If patients died in the intensive care unit, consent was given by their relatives.

The appropriate measures are used to guarantee maximal data confidentiality. All patient‐related clinical data are anonymized locally.

Data Analysis

Within the RusACSR, an analytical module was created for the assessment of health care quality for ACS patients in Russia. The main aim of this analytical module is to implement the system analysis of clinical cases for the development of health care quality indicators for ACS patients to achieve the clinical result (for example, decrease of mortality).

We present the structure of a statistical report on health care in ACS patients that could be calculated in the RusACSR for each clinical center, region, or the whole of Russia (see Supporting Information, Appendix C, in the online version of this article). This report is calculated for any selected date range.

In the RusACSR, the completeness of execution of clinical guidelines in real clinical practice is evaluated using the developed clinical indicators, which are calculated automatically by using the database query for a required cohort of patients (in clinical units, in clinics, in regions of Russia, or in all participating clinics). These indicators were developed according to the ACCF/AHA methodology for the development of quality measures for cardiovascular technology. 27 (For details of clinical indicators, see Supporting Information, Appendix D, in the online version of this article.)

Based on the presented clinical indicators, the quality of care in ACS patients was compared among Russian hospitals. This approach allows us not only to evaluate the quality of care in a particular hospital based on clinical guidelines, but also to carry out a comparative evaluation with other hospitals in the city, region, or whole of Russia.

Disease registries allow data to be collected from large patient populations. Currently, the RusACSR is used for different goals by Russian researchers. A number of public reports on the quality of health care in ACS patients have been published recently. 17 , 28 Some authors already use the data from the RusACSR to assess the mortality 29 , 30 and appropriateness of percutaneous coronary intervention (PCI) in ACS patients in Russia, 31 to study the quality of health care in ACS patients, 32 , 33 , 34 , 35 , 36 , 37 and to analyze the clinical factors associated with PCI performance in Russia. 30 The results of the RusACSR have caused debate among Russian cardiologists. 38 Some authors reported their comments and suggestions with a view to improving this registry.

Because the RusACSR uses key data elements and definitions recommended by the ACCF/AHA, the results of our study have the potential to be used for cross‐country comparison with different registries in other European countries and the United States.

The ACC/AHA have proposed the performance measures for STEMI (ST‐segment elevation myocardial infarction) and NSTEMI (non–ST‐segment elevation myocardial infarction) patients. 39 The clinical indicators of the RusACSR have a similar goal. But our indicators were proposed in accordance with the national features of health care in ACS patients.

The evaluation of PCI in ACS patients has already become the target of some registries. 40 These registries have provided useful insights regarding the practice of interventional cardiology in various countries. The RusACSR plays a similar role in Russia.

According to some authors, the employment of ACS registries in small territories (small countries, separate regions, or cities) can improve the quality of health care in ACS patients. 41 The international experience shows that the most complete fulfillment of clinical guidelines in the care of STE‐ACS patients is achieved by using STEMI networks. 42 , 43 In Russia, several regions have already had a positive experience in implementing similar projects. Since 2008, the local STEMI networks based on the independent Samara STEMI Registry have been used successfully in the Samara region of Russia. 44 The RusACSR can become a basis for future development of similar networks in the whole of Russia.

The use of the RusACSR in practical health care is associated with the need to reorganize the workload of staff in centers providing care for ACS patients. Untrained users may have some difficulties with the use of the RusACSR. To solve this problem, we developed a user manual. 22 Another problem with the RusACSR is the formalization of medical information by users during the data transfer from a hospital chart to the Registry database. In our previous study, some problems were identified as the result of a test audit of 26 untrained users of the RusACSR. 26 This study showed that the main errors are the incompleteness of data entry and the semantic content of entered data. The errors relating to incompleteness of data entry were most frequent for heart rate and blood pressure data (33%), results of instrumental examinations (55%), results of laboratory tests (55%), invasive interventions (38%), and advice at discharge on smoking, nutrition, and weight management (28%). The semantic errors were most frequent in data on past history (68%), main symptom at admission (51%), date and time of arrival of ambulance with the patient (49%), electrocardiogram parameters (48%), creatine phosphokinases and troponins (38%), coronary angiography (55%), and advice at discharge on smoking, nutrition, and weight management (50%). It was found that the error rate does not depend on the medical experience of users. After the training, these users entered about 80% of the data correctly. That is why obligatory annual training was organized for all Registry users. However, the problem of human error has still not been completely solved.

Another problem with the RusACSR is covering the Russian population of ACS patients. At first, only clinical centers from the All‐Russian Vascular Program participated in the RusACSR. Currently, different centers participate in the Registry. Nevertheless, covering the Russian population of ACS patients is insufficiently representative. 38 The majority of centers that have participated in the RusACSR have quite good quality of health care, whereas most of the centers with low‐quality health care do not want to participate in the Registry. Widespread implementation of the RusACSR is the only solution to this problem.

The RusACSR is a perspective project for different epidemiologic studies in Russian ACS patients.

Supporting information

Appendix A. Key data elements of the database of the RusACSR

Appendix B. Addition data elements for coronary anatomy used in the RusACSR

Appendix C. Structure of statistical report on healthcare in ACS patients

Appendix D. Clinical indicators for assessment of quality of healthcare in ACS patients

Acknowledgments

The authors thank all participants of the RusACSR. This Registry is funded by the Russian Ministry of Health as a part of the Health National Project.

Drs. Gridnev, Kiselev, Posnenkova, and Prokhorov took part in the manuscript preparation. Drs. Gridnev, Kiselev, Posnenkova and Dmitriev designed the RusACSR. Drs. Dovgalevsky and Oschepkova contributed to the establishment of the federal RusACSR. Drs. Gridnev, Dovgalevsky, and Oschepkova contributed to the establishment of the local RusACSR. All authors reviewed and approved the final manuscript. The RusACSR is funded by the Russian Ministry of Health as a part of the Health National Project. The Russian Ministry of Health was not involved in the collection, analysis, and interpretation of data; in the manuscript preparation; or in making the decision to submit the article for publication. The authors have not received any financial support for the preparation of this article.

The authors have no other funding, financial relationships, or conflicts of interest to disclose.

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  • Published: 22 June 2024

Cardiac evaluation of patients with juvenile dermatomyositis

  • Gökmen Akgün 1 ,
  • Betül Sözeri 2 ,
  • Eviç Zeynep Başar 3 ,
  • Nihal Şahin 4 ,
  • Yunus Emre Bayrak 4 ,
  • Kadir Ulu 2 ,
  • Hüseyin Salih Güngör 3 ,
  • Mustafa Doğan 3 ,
  • Taliha Öner 5 ,
  • Mehmet Karacan 5 ,
  • Kadir Babaoğlu 3 ,
  • Yonca Anık 6 &
  • Hafize Emine Sönmez 4  

Pediatric Research ( 2024 ) Cite this article

Metrics details

The present study aims to evaluate possible cardiac involvement in juvenile dermatomyositis (JDM) patients by conventional methods and cardiac magnetic resonance imaging (MRI) along with a systematic review of the literature on cardiac features in JDM.

The study group consisted of JDM patients who underwent cardiac MRI. We conducted a systematic review of the published literature involving JDM patients with cardiac involvement.

In the present study, although baseline cardiologic evaluations including electrocardiography and echocardiography were within normal limits, we showed late gadolinium enhancement on cardiac MRI in 3 of 11 JDM patients. In the literature review, we identified 25 articles related to cardiac involvement in JDM. However, none of them, except one case report, included cardiac MRI of JDM patients.

Cardiac abnormalities have been reported among the less frequent findings in patients with JDM. Cardiovascular complications during the long-term disease course are a leading cause of morbidity and mortality in these patients. Early detection of cardiac involvement by cardiac MRI in patients with JDM and aggressive treatment of them may improve the clinical course of these patients.

The myocardium in patients with JDM may be involved by inflammation.

Myocardial involvement may be evaluated by using contrast-enhanced cardiac MRI.

This is the first study evaluating cardiac involvement by cardiac MRI in JDM patients.

MRI may show early cardiac involvement in patients whose baseline cardiologic evaluations are within normal limits.

Early detection of cardiac involvement by cardiac MRI may improve the long-term prognosis of patients with JDM.

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Introduction.

Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy presenting with the characteristic rash and proximal muscle weakness during childhood. 1 Although proximal muscle weakness and skin findings are the predominant features of the disease, cardiac findings may be accompanied at initiation or during the course of the disease. 2 Cardiac involvement in JDM is usually underestimated and studies regarding this issue are scarce. However, cardiac involvement may cause morbidity and mortality in the long-term period. Nonspecific sinus tachycardia is the most frequent cardiac abnormality in JDM. Left ventricular diastolic and systolic dysfunction, hypertension, atherosclerosis, coronary artery disease, and metabolic syndrome may also occur. 2 Increasingly, magnetic resonance imaging (MRI) studies begin to take an important place both in the diagnosis of the disease and detection of subclinical organ involvement. Adult studies showed that cardiac MRI may help to detect subclinical myocardial involvement. 3 , 4 However, there is no data about cardiac MRI findings in JDM patients. The present study aims to evaluate possible cardiac involvement in JDM patients by conventional methods and cardiac MRI along with a systematic review of the literature on cardiac features in JDM.

Material and methods

This cross-sectional study was conducted between June 2022 and December 2022. Patients who were followed up with a diagnosis of JDM were enrolled in the study. All patients fulfilled the Bohan and Peter 5 , 6 or EULAR/ACR 2017 classification criteria. 7 Demographic data, clinical manifestations, laboratory findings, treatments, and outcomes were obtained from patient charts. The disease activity was evaluated with the Childhood Myositis Assessment Scale (CMAS). 8

Initially, a standard 12-lead electrocardiogram (ECG) was performed in all patients. Subsequently, all of them were examined using transthoracic echocardiography (GE Vivid E9 ultrasound system, General Electric Healthcare, Horten, Norway). B-Mode and M-Mode echocardiographic images were obtained. Finally, all patients underwent cardiac MRI in a 1.5 T MR scanner (Philips Gyroscan Intera Master; Philips, Eindhoven, The Netherlands) equipped with a 30 mT/m maximum gradient strength and 150 mT/m/ms slew rate. Data were collected by utilizing a synergy body coin with the patient in the supine position. The vital signs of patients were monitored and recorded throughout the MR examination. The left ventricle (LV) was evaluated in the standard 17-segment model 9 (Supplementary Fig.  1 ). All images were analyzed by an experienced pediatric cardiologist (KB) and radiologist (YA).

The study respected the guidelines of the Helsinki Declaration concerning medical research in humans and received local Ethics Committee approval. Informed consent was obtained from each patient and/or parents.

Systematic review of the literature

A systematic review of the literature on cardiac involvement in JDM was conducted. Relevant documents were searched according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) principles by using Medline and PubMed databases (Supplementary Fig.  2 ). 10 The following keywords were used: (“Juvenile dermatomyositis” OR “JDM” OR “Dermatomyositis, Juvenile” OR “Juvenile Myositis” OR “Myositis, Juvenile”) AND (“cardiac involvement” OR “cardiac manifestation” OR “heart involvement” OR “heart manifestation”). Articles only in English were included in the search. All related articles, including randomized and nonrandomized controlled trials, observational studies (case-control, cohort studies, and case series), and single case reports, were included. Two authors (GA and HES) independently reviewed all possibly eligible studies. Finally, discrepancies were resolved by discussion with the two experienced authors (KB and BS).

Baseline characteristics of our patients

A total of 11 patients were included in the study. Four (36.4%) were male and 7 (63.6%) were female. The median age at evaluation was 11 (7–17) years. The median duration of the disease was 24 (1–102) months. All the patients had heliotrope rash and proximal muscle weakness. Gottron papules were present in 10 patients, calcinosis in 2, and lipodystrophy in 2, concomitantly. The median CMAS score was 35 (22–48) at the time of diagnosis and 52 (41–52) at the time of cardiac evaluation. None of the patients had pulmonary or gastrointestinal involvement. Anti-nuclear antibody (ANA) was positive in 10 patients, anti-transcription intermediary factor 1γ (Anti-TIF1γ) in 2, anti-NXP2 in 1, and anti-Mi2 alfa and beta in 1 patient. Nailfold capillaroscopy findings revealed scleroderma pattern in 5 patients and non-scleroderma pattern in 5 patients (Table  1 ). None of them had additional cardiac risk factors including smoking, obesity, hypertension, dyslipidemia, and metabolic syndrome.

Cardiac features of our patients

Electrocardiographic examination showed normal sinus rhythm with normal QRS voltage and axis in all patients. None of them had arrhythmia at the time of evaluation. Conventional echocardiographic parameters were within the normal range (Table  1 ). Three of 11 patients (27.2%) had myocardial late gadolinium enhancement (LGE) on cardiac MRI similar to the appearance in myocarditis. Segment 17 (apex) was involved in 2 patients, and segments 1,2 5,6,9,10,11,14 (basal anterior, basal anteroseptal, basal inferolateral, basal anterolateral, mid inferoseptal, mid inferior, mid inferolateral, and apical septal) were involved in 1 patient (Fig.  1 ). Furthermore, one patient showed impaired right ventricular ejection fraction (EF) which was not shown in conventional echocardiography. Patients with myocardial late gadolinium enhancement were younger than others, but there were no differences between these two groups in terms of clinical and laboratory findings.

figure 1

Myocardial images of T2 scans ( a , c , e ) and late gadolinium enhancement T1 scans ( b , d , f ) on cardiac MRI. Cardiac MRI axial plane: ( a , c , e ) are T2 weighted fat saturated images and ( b , d , f ) are postcontrast late enhancement T1 weighted images. Myocardial edema is seen on segment 17 (apex) on images ( a ) and ( e ) and on segments 1, 2, 5, 6, 9, 10, 11, and 14 (basal anterior, basal anteroseptal, basal inferolateral, basal anterolateral, mid inferoseptal, mid inferior, mid inferolateral and apical septal) on the image ( c ). On postcontrast images ( b ) ( d ) and ( f ) contrast enhancement is seen on the segments parallel with myocardial edema areas on T2W-fat-saturated images.

Cardiac features of patients with JDM in the literature

Supplementary Fig.  2 lists the schematic analyzes of the systematic literature review. At first, 84 related articles were identified. After the title and abstract review, 25 articles were included. 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 Of these 25 articles, 7 were case report and 18 were observational studies (Table  2 ). However, none of them, except one, included cardiac MR findings of JDM patients. Stewart et al. 32 reported a 14-year-old girl diagnosed with JDM who had first-degree heart block and low voltages on ECG. Her disease evolved into multisystem involvement during hospitalization and treatment. Conduction disturbance progressed to atrial fibrillation. Her echocardiographic examination and cardiac MRI were normal. The disease course of the patient was complicated by macrophage activation syndrome. She was treated successfully with immunosuppressive therapy. Karaca et al. 16 reported another case report of conduction disturbance in an 11-year-old boy with JDM. The patient had sinus bradycardia with a heart rate of 40–44 per minute. After treatment with intravenous immunoglobulin (IVIG) and steroids, bradycardia was resolved spontaneously. A case-control study by ref. 13 including 25 patients with JDM reported one patient whose initial presentation was cardiac failure due to myocarditis and another two patients with asymptomatic ECG abnormalities. Ghosh et al. 30 reported a 10-year-old girl with a complete heart block secondary to JDM. She had normal systolic and diastolic functions. After treatment with methylprednisolone, ECG returned to sinus rhythm. Another case-control study by ref. 19 reported that diastolic dysfunction detected by tissue Doppler imaging was present in 22% of patients with JDM, pericarditis in 11%, and ECG abnormalities in 17% during 16 years of clinical follow-up. In their ensuing study, ref. 21 reported that patients with JDM had systolic dysfunction represented by decreased long-axis strain on color tissue Doppler echocardiography. Barth et al. 23 investigated 55 JDM patients aged 6–48 years using 24-h ambulatory Holter ECG and stated that patients with JDM had decreased heart rate variability (HRV). HRV is related to the heart rate. Insufficient response of inflamed myocardium and conduction tissue to vagal and sympathetic stimulus may be the reason for decreased HRV. Cantez et al. 27 reviewed retrospectively 105 patients with JDM. They found that ECG abnormalities (prolonged QTc and PR interval, bundle branch block) were present in 6% of the patients and echocardiographic abnormalities (atrioventricular-semilunar valve regurgitations) in 25%. However, their ECG and echocardiographic findings were mild or trivial and unlikely to be linked to the underlying disease. Diniz et al. 31 investigated 35 patients with JDM using conventional 2D and speckle-tracking echocardiography. They found that patients with JDM had lower LV global peak longitudinal systolic strain as well as lower peak circumferential systolic strain values than controls, although all the patients had normal LV ejection fraction (EF) on 2D conventional echocardiography. These results indicate that speckle-tracking echocardiography may be more sensitive than conventional 2D echocardiography in detecting early subclinical myocardial impairment in JDM patients. Diastolic dysfunction represented by low early diastolic tissue velocity (e’) and systolic dysfunction represented by decreased longitudinal strain were reported by ref. 34 including 57 patients with JDM. Patients with JDM generally have subclinical silent cardiac involvement during childhood, although there are few case reports of pericardial tamponade resulting in congestive heart failure which was treated by open drainage 11 and ventricular arrhythmias treated by amiodarone. 36 Overt cardiovascular disease and death resulting from cardiovascular involvement in JDM are more common in adult populations during long-term follow-up.

The myocardium in patients with JDM can be involved by inflammation just as the skeletal muscle. In the present study, we showed myocardial involvement by using contrast-enhanced cardiac MRI in 3 of 11 JDM patients whose baseline cardiologic evaluations including ECG and echocardiography were within normal limits. To our best knowledge, this is the first study evaluating cardiac involvement by cardiac MRI in JDM patients. Adults, with dermatomyositis and polymyositis frequently have cardiac involvement such as conduction abnormalities, atrial and ventricular arrhythmias, pericarditis, and myocarditis. 16 Cardiac involvement in JDM may either develop acutely or manifest during the disease course. Systemic vasculopathy and elevated interferon signature play a central role in the pathogenesis of idiopathic inflammatory myopathies. Both factors result in endothelial dysfunction which leads to cardiovascular manifestation during the disease. 2 Rhythm and conduction problems and myocarditis are the most observed cardiac abnormalities in the acute phase of the disease. Rider et al. 20 evaluated 374 patients with juvenile idiopathic inflammatory myopathy. Abnormal ECG findings were present in 56 (15%) of the patients. However, the study did not specify the abnormal ECG findings. Cantez et al. 27 showed prolonged QTc or prolonged PR in 6 of 69 JDM patients. Barth et al. 29 demonstrated pathologic ECG findings in 10 (17%) of 58 JDM patients. Furthermore, patients presenting with varying degrees of heart block have been reported. 30 , 32 Besides these acute complications, reduced aerobic capacity, systolic and diastolic dysfunction, and impaired cardiometabolic measures have been demonstrated in long-term follow-up. 14 , 15 , 19 , 21 , 26 , 28 , 33 , 35

Current recommendation guidelines do not provide a recommendation on how often and by which method patients with JDM should be screened for cardiac aspects. Cardiac MRI may be a valuable prospective technique to detect subclinical myocardial involvement. However, it is recommended that cardiac MRI should only be performed in patients with abnormalities on ECG or echocardiography. 37 Cardiac MRI studies conducted in recent years have revealed that myocardial involvement is higher than previously thought. For instance, Rosenbohm et al. 38 demonstrated signs of myocardial inflammation in 62.3% of 53 polymyositis (PM)/dermatomyositis (DM) patients by cardiac MRI. In the aforementioned study, they reported that the lateral segments of the left ventricle were significantly more often affected than the anterior or septal segments. Another study by ref. 4 showed late gadolinium enhancement (LGE) involving around 5% of the mass of the myocardium in 9 of 16 adult patients with PM/DM. The most affected part of the heart was interventricular septum ( n  = 8) followed by lateral wall ( n  = 5), inferior wall ( n  = 3), apex ( n  = 2), and anterior wall of the left ventricle ( n  = 1). They also found that LGE was more common in patients with PM compared to those with DM. 4 Diederichsen et al. 39 evaluated cardiac abnormalities by cardiac MRI in newly diagnosed, untreated patients with idiopathic inflammatory myopathies. They detected systolic dysfunction by cardiac MRI in 2 of 14 patients with idiopathic inflammatory myopathies. Most recently, ref. 40 suggested that characteristics of cardiac dysfunction may be different between subgroups of idiopathic inflammatory myopathies, and they confirmed their hypothesis by mapping cardiac MRI parameters. They found higher global extracellular volume values in the PM group compared to the DM group. The number of affected ventricular segments in PM patients was higher than in DM patients. In our study, 3 of 11 patients had intramyocardial late gadolinium enhancement. Of them, 2 were diagnosed 3 months ago and the other patient was following for 12 months after the diagnosis. All three patients were in clinically inactive stage.

Due to the rare nature of the disease, there is no randomized controlled study on this subject. Accurate and timely treatment may prevent permanent cardiac damage in patients with JDM. Long-term follow-up of patients with JDM have shown frequent cardiovascular complications such as coronary artery disease, hypertension, atherosclerosis in adolescence and adulthood. Despite the fact that JDM can often cause significant cardiovascular morbidity and mortality during the long-term course of the disease, cardiac involvement is often overlooked at the onset of the disease. A cardiac MRI study by ref. 41 showed that myocardial LGE on contrast-enhanced magnetic resonance imaging of 4 adult patients with idiopathic inflammatory myopathies had markedly regressed after 6 months of corticosteroid and immunosuppressive therapy. They suggested that patients with cardiac involvement may require more aggressive treatments. IVIG is an anti-inflammatory and immunomodulatory drug commonly used in myocarditis to reduce the damage of inflammatory cytokines in cardiac myositis. 42 In our study, IVIG was given to 2 patients after cardiac MRI while one could not be treated with IVIG due to financial problems.

Late gadolinium enhancement on cardiac MRI may be seen in various cardiac diseases. Cardiomyopathies, autoimmune and inflammatory diseases, and metabolic and storage diseases cause LGE on cardiac MRI. Accumulation of amyloid fibrils in amyloidosis and glycosphingolipids in Anderson-Fabry disease deteriorate myocardial structure and cause intramyocardial fibrosis. Viral myocarditis, sarcoidosis, and systemic sclerosis are other causes of intramyocardial fibrosis due to inflammation with ischemic damage. Intramyocardial fibrosis and LGE can be found in arrhythmogenic right ventricular cardiomyopathy and dilated or hypertrophic cardiomyopathies. 43 Myocardial segments involved by contrast media and the pattern of gadolinium distribution may vary from patient to patient even if they have the same disease. Therefore, the clinical value of LGE distribution patterns in providing precise diagnosis remains to be clarified. In our 2 patients, intramyocardial LGE was present in the left ventricular apex. The remaining patient had more diffuse myocardial contrast enhancement (basal anterior, basal anteroseptal, basal anterolateral, basal inferolateral, mid inferior, mid inferoseptal, mid inferolateral, and apical septal segments).

Although conventional echocardiography and ECG findings were completely normal, myocardial involvement was detected in 3 patients in our study. All patients underwent repeat cardiac MRIs. Intramyocardial LGE was resolved in both patients after 6 months of treatment. A similar result was present in Allanore et al.’s study. 41

The small sample size is one of the important limitations of the study. Furthermore, all patients were at different periods of the disease. Another limitation is that our findings are not enough to make a contribution to predictive factors for cardiac involvement. Cardiac MRIs are difficult to implement in childhood patients. Unfortunately, it was inevitable that motion artifacts would occur. Changes seen may be the result of slow flow within the trabeculae and could be artifacts. However, in the present study, complete myocardial involvement suggests actual myocardial involvement rather than an artifact. Furthermore, there was an absence of involvement in the basal segment of the left ventricular free wall. Thus, the present involvement should not be construed as an artifact. Additionally, myocardial suppression was performed via a look-locker program that provides different TI values, and the best one was chosen. However, due to motion artifacts and high cardiac rate, the images included were a little inferior quality. Therefore, we believe that further studies are needed to decide on which JDM patients should be evaluated by cardiac MRI, and further studies in a larger cohort are needed to confirm our results.

Juvenile dermatomyositis is a multisystemic disease characterized by diffuse inflammation of skin and muscles. Cardiac involvement may occur in the course of the disease, however, remains unrecognized due to the lack of overt clinical manifestations in childhood. When considering that long-term cardiovascular complications seen in patients with JDM have significant effects on morbidity and mortality, early detection of cardiac involvement by cardiac MRI and aggressive treatment of these patients may improve the long-term prognosis of patients with JDM.

Data availability

All data generated or analyzed during this study are included in this published article. For additional information concerning these data, the corresponding author can be contacted.

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G.A., B.S., E.Z.B., and Y.A. were involved in gathering data, formal analysis, and writing the original draft. N.Ş., Y.E.B., K.U., H.S.G., M.D., T.Ö., M.K., K.B., supported gathering data and contributed to analyzing and interpreting data. H.E.S. conceived the study and revised the manuscript. All authors reviewed and revised the manuscript and approved the final version of the manuscript.

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Akgün, G., Sözeri, B., Başar, E.Z. et al. Cardiac evaluation of patients with juvenile dermatomyositis. Pediatr Res (2024). https://doi.org/10.1038/s41390-024-03336-8

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Value of comparative studies of "real clinical practice" in modern cardiology. Position paper based on the expert council discussion dated 12/18/2020

Affiliations.

  • 1 I. M. Sechenov First Moscow State Medical University (Sechenov University). Moscow, Russia.
  • 2 Russian National Research Medical University named after Pirogov, Moscow, Russia.
  • 3 Research Institute for Complex Issues of Cardiovascular Diseases, Kemerovo, Russia.
  • 4 Russian State University of Peoples' Friendship, Moscow, Russia.
  • 5 Almazov National Medical Research Center, St. Petersburg, Russia.
  • 6 Kazan State Medical University, Kazan, Russia.
  • 7 Russian Medical Academy of Postgraduate Education, Moscow, Russia.
  • 8 Samara Regional Clinical Cardiological Dispensary, Russia Samara State Medical University, Samara, Russia.
  • 9 Perm State Medical University named after Academician Wagner E.A., Perm, Russia.
  • 10 Volgograd State Medical University, Volgograd, Russia.
  • 11 National Medical Research Centre for Therapy and Preventive Medicine, Moscow, Russia Robertson Centre for Biostatistics, Glasgow, Great Britain.
  • 12 National Medical Research Centre for Therapy and Preventive Medicine, Moscow, Russia.
  • 13 Scientific Medical Research Center of Cardiology, Moscow, Russia.
  • 14 Privolzhsky Research Medical University of the Ministry of Health of the Russia, Nizhny Novgorod, Russia.
  • 15 Novosibirsk State Medical University, Novosibirsk, Russia.
  • PMID: 34112079
  • DOI: 10.18087/cardio.2021.5.n1646

On December 18, 2020, an expert council was held with the participation of members of the Russian Society of Cardiology, the Eurasian Association of Ther-apists, the National Society for Atherothrombosis, the National Society for Evi-dence-Based Pharmacotherapy, and the Russian Heart Failure Society. The event was devoted to the discussion of the correct use of research data of "real clinical practice" in decision making.

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1.6 million excess black deaths owed to inequities in cardiac care, jacc report card reveals.

The American College of Cardiology (ACC) this week published a report card on highlighting the excess cardiovascular mortality among Black Americans between 2000-2022.[1] The ACC said it highlights the "persistent and tragic inequities" in cardiovascular care and outlines the years of life lost to the Black community because of their higher cardiovascular disease death rates. #healthdisparities

The Journal of the  American College of Cardiology (JACC) this week published a report card on the excess cardiovascular mortality among Black Americans between 2000-2022.[1] The ACC said it highlights the "persistent and tragic inequities" in cardiovascular care and outlines the years of life lost to the Black community because of higher cardiovascular disease death rates.

The report showed the Black population experienced 1.6 million excess deaths overall and millions of potential life-years lost. Heart disease was the leading cause of age-adjusted excess mortality among Black Americans. This includes deaths due to ischemic heart disease, hypertension, cerebrovascular disease and heart failure.

“Our study reveals that Black Americans, because of their higher cardiovascular mortality rates compared with white Americans, have suffered almost 800,000 excess deaths, which translates to about 24 million additional years of life lost between 2000 and 2022,” JACC incoming editor-in-chief Harlan M. Krumholz, MD , SM, FACC, who is also senior author of the study, said in a statement. “This staggering figure highlights the critical need for systemic changes in addressing cardiovascular inequities.”

Krumholz said the goal of the report card is to promote accountability and serve as a catalyst for action that addresses the ongoing problem.

“We are reminded of the stark reality that Black Americans continue to face significant disparities in cardiovascular outcomes,” explained Jennifer H. Mieres, MD , FACC, in an accompanying editorial.[2] She is the chair of the ACC Diversity and Inclusion Committee and senior vice president of the Center for Equity of Care at Northwell Health in New Hyde Park, New York. She said the report card serves as a reminder that all Americans have not benefited equally from significant advances made in the treatment and prevention of cardiovascular diseases.

“Bringing an equity lens to the redesign of CV health by addressing the social determinants of health and the systemic barriers that contribute to structural racism are critical for solving for cardiovascular health disparities and ensuring equity of care,” Mieres said. 

Despite advancements in medicine, access to care has impacted the higher numbers of Black cardiovascular deaths 

This report highlights persistent and significant racial disparities in cardiovascular disease outcomes between Black and white Americans. The key finding is that between 2000 and 2022, Black Americans had almost 800,000 excess age-adjusted deaths and 24 million excess years of potential life lost due to cardiovascular disease. 

"Despite the triumphant reduction in cardiovascular morbidity and mortality over the last 50 years, those declines evolved at racially disproportionate rates resulting in not just health inequities, but life inequities. The disparities are evident across different subcategories, including ischemic heart disease, hypertension, cerebrovascular disease, and heart failure. Moreover, the sharp increases during the pandemic indicate the specific vulnerability of this group during a public health crisis and the need to mitigate this risk in future pandemics," the authors of the report wrote.

The ACC said in a statement the social determinants of health have a detrimental impact in creating barriers that prevent the most vulnerable Americans from receiving the cardiovascular care they need. The ACC has established multiple programs to address these inequities, including the Diversity and Inclusion Program . Its Internal Medicine Program introduces groups who have been historically underrepresented in cardiology to a career in cardiology by connecting them with the mentors, peer network and resources they need to understand career opportunities in the field. 

The ACC Clinical Trials Research (CTR) program is also designed to increase the number of historically underrepresented minorities in cardiology to serve as leaders in cardiovascular clinical trials research to ensure diversity of thought, experience and perspective. The ACC also wants to ensure that the evidence base in studies includes data more closely reflecting the demographics of the actual cardiovascular patient population.

How the study was conducted

Krumholz and fellow researchers used the U.S. Centers for Disease Control and Prevention’s Wide-ranging ONline Data for Epidemiologic Research (WONDER) national death certificate data to collect primary cause of death stats in 5-year age groups from Black and white populations. 

They calculated age-adjusted mortality rates (AAMR) for each disease condition by weighting the crude death rate by the fraction of individuals in that age group according to the 2000 population distribution. The excess AAMR was computed by subtracting the estimated AAMR of white people from the AAMR of Black people. Years of life lost (YPLL), defined as the number of years a person would have lived had they not died when they did, was estimated by multiplying the 5-year age group crude mortality rate by the life expectancy of white people for that age group, gender, and year. The excess YPLL was computed by subtracting the estimated YPLL of white people from the estimated YPLL of Black people.

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References: 

1. Adith S. Arun, Mitsuaki Sawano, Yuan Lu, Frederick Warner, César Caraballo, Rohan Khera, Melvin R. Echols, Clyde W. Yancy, and Harlan M. Krumholz. Excess Cardiovascular Mortality Among Black Americans 2000-2022: A JACC Report Card. J Am Coll Cardiol. Jun 18, 2024. Epublished DOI: 10.1016/j.jacc.2024.06.004. 

2. Jennifer H. Mieres, Jeffrey T. Kuvin, and Robert O. Roswell. Cardiovascular Health, Juneteenth 2024: Are We Thriving Together or Not? J Am Coll Cardiol. Jun 18, 2024. Epublished DOI: 10.1016/j.jacc.2024.06.005.

Dave Fornell is a digital editor with Cardiovascular Business and Radiology Business magazines. He has been covering healthcare for more than 16 years.

Dave Fornell has covered healthcare for more than 17 years, with a focus in cardiology and radiology. Fornell is a 5-time winner of a Jesse H. Neal Award, the most prestigious editorial honors in the field of specialized journalism. The wins included best technical content, best use of social media and best COVID-19 coverage. Fornell was also a three-time Neal finalist for best range of work by a single author. He produces more than 100 editorial videos each year, most of them interviews with key opinion leaders in medicine. He also writes technical articles, covers key trends, conducts video hospital site visits, and is very involved with social media. E-mail: [email protected]

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Millions fewer people may need statins, a new study suggests. But guidelines have yet to agree

Elizabeth Cooney

By Elizabeth Cooney June 10, 2024

A close up photograph of the prescription label on a bottle of lipitor, a statin medication used to treat high cholesterol

I t’s a familiar scene for patients during a routine primary care visit. The doctor scans blood test results, notes high cholesterol flagged by a standard calculator to assess risk of heart attack or stroke, then decides — and ideally discusses — whether to recommend taking a statin to cut the risk over time.

That conversation may happen less often if changes in the risk model presented by the American Heart Association in November translate into new guidelines for prescribing statins. Those guidelines haven’t been recalibrated yet, but a new analysis suggests that the new risk model could mean far fewer Americans — as many as 40% less than current calculators say — would be candidates for cholesterol-lowering drugs to prevent cardiovascular disease.

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To reach this conclusion, published Monday in a JAMA Internal Medicine study , researchers analyzed data from 3,785 adults who were 40 to 75 years old and took part in the National Health and Nutrition Examination Survey from January 2017 to March 2020. Their 10-year risk of artery-narrowing cardiovascular disease was computed using the AHA’s Predicting Risk of cardiovascular disease EVENTs (PREVENT) equations from 2023 and then compared to risk estimates using the previous tool from 2013, the Pooled Cohort Equations (PCE) on which current guidelines are based.

Those 2013 equations were widely criticized as overestimating risk . The 2023 version, drawing on billing and electronic health record data from a more diverse real-world population, incorporated current statin use as well as metabolic and kidney diseases.

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Chiadi Ndumele, chair of the American Heart Association’s CKM Scientific Advisory Group, emphasized that the actual PREVENT risk thresholds for statin use in cardiovascular prevention will need to be decided in clinical guidelines, and that has not yet occurred. He also acknowledged criticism of the earlier risk model.

“We updated the AHA risk prediction model to PREVENT reflecting the growing influence of inter-related metabolic risk factors (obesity, diabetes, metabolic syndrome) and chronic kidney disease on cardiovascular disease risk,” Ndumele, director of obesity and cardiometabolic research at Johns Hopkins University, told STAT in an email. “It is therefore not surprising that the investigators found about twice the predicted event rate for the PCEs vs. PREVENT, reflecting this difference.”

Under the current guidelines, most people with a 10-year risk of 7.5% or more for developing cardiovascular disease are advised to take a statin, while at a 5% risk, they’re told only that they and their doctors should consider doing so.

“Analyses are underway,” Ndumele said. “Guidelines will have to consider whether and how to update recommendations to include PREVENT risk thresholds to guide clinical decision making.”

Related: Reversing progress, stroke rates are rising, especially in working-age adults

What’s changed in the JAMA Internal Medicine analysis is how many people might be at risk, based on the new components put into the calculator. Overall, 4% of people had a 10-year risk of developing cardiovascular disease, compared to the 8% previously predicted by the PCE. The number of adults recommended for statins could drop from 45.4 million to 28.3 million.

Race, now recognized as a social not biological construct, was excluded in the newer equations. That meant 5.1% of Black adults were computed to be at risk, compared to 10.9% from the previous calculator. For older adults ages 70 to 75, the proportion at risk was 10.2%, down from 22.8%.

In a paradox, the study found that while fewer people might be eligible for statins, which now can cost as little as $40 a year, the estimates also say most people who would be advised to take them aren’t doing so.

“The prior risk equations and the PREVENT equations that we focus on in this study really seek to give doctors and patients sort of a starting percentage to say, is it worth having a conversation about statins?” lead study author Timothy Anderson, a primary care physician and an assistant professor of medicine at University of Pittsburgh Medical Center, told STAT. “When we’re seeing risk rates cut in half, I think that really is something that’s likely to impact how doctors and patients talk about these meds.”

The biggest predictor of risk remains age, Anderson said. “If you’re a borderline risk now, you’re likely to be higher risk in five years. And that’s a complicated set of conversations for primary care doctors and patients to have.”

That concerns Steven Nissen, a cardiologist at the Cleveland Clinic, who was not part of the study. “Age is the most powerful factor in the calculators, so if you wait until somebody is 60 or 65, you’re playing catch-up,” he said. “I tend to lean toward treating rather than not treating when it’s a borderline case, but only when the patient and I have a conversation.”

Nissen has been leading an effort in collaboration with AstraZeneca to make the 5-milligram dose of its drug, rosuvastatin, available without a prescription. He urged shared decision-making between doctor and patient, aware that busy primary care physicians may be pressed for time.

“Good medicine involves judgment. And the calculator is not a replacement for good medical judgment, which may come to a different conclusion,” he said. “I’m not very supportive of either calculator because I think that in general, it’s good to have a lower LDL,” or “bad” cholesterol.

There are a multitude of factors affecting cardiovascular health, and statins are just one piece, said Gregg Fonarow, chief of cardiology at UCLA, citing the AHA’s recent projection that 61% of the U.S. population will likely have cardiovascular disease. He did not take part in the current study.

Related: Decline in heart failure deaths has been undone, led by people under 45

“So many cardiovascular events are preventable, not just through medication but through lifestyle modification. We need to do such a better job with prevention,” Fonarow said. “This really represents an opportunity to use the new enhanced PREVENT risk score and better inform individuals of risk, but importantly, not just for 10-year risk, but their lifelong risk for disease.”

Ndumele said PREVENT will help guide use of preventive therapies beyond statins, relevant for people with cardiovascular-kidney-metabolic syndrome, a disorder in which metabolic risk factors, chronic kidney disease, and the cardiovascular system interact to cause multi-organ dysfunction and poor cardiovascular outcomes.

“I think the challenge with this paper is the assumption that the same threshold will be used for the recommendation of statin use,” Ndumele said. “Risk estimates from PREVENT are much closer to what is observed in reality than they were for PCEs, but there is need for discussion about the optimal risk threshold for preventive statin use in guidelines.”

Nissen said any changes should be thought through carefully, with this caveat: “The take-home message is that any of these calculators are the best guess about risk,” he said, “but the decision to treat is different from simply calculating a risk.”

STAT’s coverage of chronic health issues is supported by a grant from Bloomberg Philanthropies . Our financial supporters are not involved in any decisions about our journalism.

About the Author Reprints

Elizabeth cooney.

Cardiovascular Disease Reporter

Elizabeth Cooney is a cardiovascular disease reporter at STAT, covering heart, stroke, and metabolic conditions.

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Kiran K. Mallula, MD, MS

Kiran K. Mallula, MD, MS

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Languages Spoken: English, Hindi, Telugu, Sanskrit

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Interventional MRI

Research Interests

Principal Investigator, Utility of ECHOPIXEL 3D viewer in procedural planning

Principal investigator, Transcatheter Fontan fenestration in children: A multicenter experience.

McPeters R, Chicarelli E, Manning D, Mallula KK , Krulisky J. Case of bronchopulmonary sequestration in a newborn

Krulisky J, Siwik E, Mallula KK . Comparative outcomes of transcatheter closure of secundum ASDs with the ASO and GSO devices: A single center experience.

Mallula KK , Patel S, Avula S, Sernich S, Pettitt T, Siwik ES. Utility of a hybrid approach for Post-Fontan reinterventions: Case series and current review of literature.

Participation in A Prospective Data Registry for the Congenital Cardiovascular Interventional Study Consortium: Risk Stratification for Cardiac Catheterization Procedures

Uppu SC, Chandrasekaran S, Mallula KK. Constrictive pericarditis in a patient with sinus venosus atrial septal defect and anomalous right upper pulmonary venous return. Ann Pediatr Cardiol 2009;2(1):87-8.

Mallula K , Amin Z. Recent changes in instructions for use for the Amplatzer atrial septal defect occluder: How to incorporate these changes while using transesophageal echocardiography or intracardiac echocardiography? Pediatr Cardiol 2012;33(7):995-1000.

Mallula KK . et. al. Sinus venosus atrial septal defect: Postoperative outcomes after repair http://search.proquest.com/docview/1026799439

Ghawi H, Gendi S, Mallula K , Zghouzi M, Faza N, Awad S. Fetal left and right ventricle myocardial performance index: Defining normal values for the second and third trimesters – single tertiary center experience. Pediatr Cardiol 2013;34(8):1808-15.

Mallula K , Patel N, Amin Z. New design of the amplatzer membranous VSD occluder: A step forward? Pediatr Cardiol 2013;34(8):2068-72.

Mallula KK , Patel ND, Hijazi ZM, Joshi S, Naheed Z. Double-outlet right ventricle with an intact ventricular septum: A unique stage 1 palliation. Pediatr Cardiol 2013;34(8):2086-8

Mallula KK , Sosnowski C, Awad S. Spectrum of Cantrell’s pentalogy: Case series from a single tertiary care center and review of the literature. Pediatr Cardiol 2013;34(7):1703-10

Severin PN, Awad S, Shields B, Hoffman J, Bonney W, Cortez E, Ganesan R, Patel A, Barnes S, Al-Anani S, Mallula K , et. al. The pediatric cardiology pharmacopeia: 2013 update. Pediatr Cardiol 2013;34(1):1-29.

Mallula KK , Gupta U, Hasbani K. A rare case of Cardiac Aspergilloma diagnosed by cardiac MRI. Case of the week Number 14-02. Society of Cardiovascular Magnetic Resonance http://scmr.org/caseoftheweek/2014/6994.html#.Uw0kiLTiZRk (2/2014)

Patel ND, Mallulla KK , Abdulla RI. Atrial flutter demonstrated by M-mode echocardiography. Pediatr Cardiol 2014;35(5):893-5.

Mallula K , Vaughn G, El-Said H, Lamberti JJ, Moore JW. Comparison of ductal stenting versus surgical shunts for palliation of patients with pulmonary atresia and intact ventricular septum. Catheter Cardiovasc Interv 2015;85(7):1196-202

Mallula KK , Kenny D, Hijazi ZM. Transjugular melody valve placement in a small child with protein losing enteropathy. Catheter Cardiovasc Interv 2015;85(2):267-7

Mallula KK , Patel ND, Abdulla RI, Bokowski JW. Tetralogy of fallot with left superior vena cava and coronary sinus atrial septal defect: a rare association. Pediatr Cardiol 2015;36(5):1100-1

Vaughn GR, Moore JW, Mallula KK, Lamberti JJ, El-Said HG. Transcatheter stenting of the systemic-to-pulmonary artery shunt: A 7-year experience from a single tertiary center. Catheter Cardiovasc Interv 2015

Guillot M, Ascuitto R, Ross-Ascuitto N, Mallula KK, Siwik E. Computational fluid dynamics as a complementary study for transcatheter endovascular stent implantation for recoarctation of the aorta associated with minimal pressure drop: An aneurysmal ductal ampulla with aortic isthmus narrowing, Cardiol Young. 2019 Jun; 29(6):768-776.

Krulisky J, Mallula KK. Transcatheter exclusion of a large RVOT aneurysm in a systemic right ventricle. Journal of American College of Cardiology: Case Reports (In press, March 2020)

Guruchandrasekar S, Mallula KK , Siwik ES. Endovascular repair of thoracic aortic pseudoaneurysms in children. Journal of American College of Cardiology: Case Reports (In Press, April 2020)

Non-refereed

Lilje C, Mallula KK , Ward K, Congeni J. A teenager with shortness of breath and headaches. Consultant360:volume 15(7), July 2016. https://www.consultant360.com/articles/teenager-history-shortness-breath-and-headaches

Book Chapters:

Mallula KK , Kenny D, Hijazi Z. Tricuspid valve insufficiency: SAPIEN valve in the tricuspid position. Structural, Valvular and Congenital Heart Disease Interventions, 2nd Edition (Horst Sievert, Shakeel A. Qureshi, Neil Wilson, Ziyad M. Hijazi, eds), October 2012

Mallula KK , Amin Z. Pulmonary atresia with intact ventricular septum. Atlas of Neonatal Cardiology, 1st edition, July 2013

Mallula KK , Amin Z. Hybrid closure of Muscular Ventricular Septal Defects. Chapter in Fetal and Hybrid Procedures in Congenital Heart diseases, 1st edition, November 2015

Book Reviews:

Mallula KK , Hijazi, Z. iBook Review, The Illustrated Field Guide to Congenital Heart Disease and Repair, 3rd Edition. Congenital Cardiology Today, August 2013.

2014  Medium to longer term outcomes of percutaneous transcatheter closure of congenital ventricular septal defects. Oral presentation at PICS-AICS 2013, Miami, FL. https://youtu.be/_hWFK2zGDpc?t=4412

2015  Ductal stenting: Is it time to redefine the palliative strategy in patients with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis? Oral presentation at PICS-AICS 2014, Chicago, IL.  https://youtu.be/awfuFwmNdls?t=4681

2015  Update on 2014 live cases, PICS-AICS 2015, Las Vegas, NV. https://youtu.be/Y4ZKGIKSeBM

2017  Live Cases Update, PICS-AICS 2017, Miami, FL.  https://youtu.be/pV8faM-FIWQ?t=81

Electronic and Multi-Media:

01/16 https://www.medtronic.com/physicianlocator/googleMaps/showResults?therapy=47&locationRadio=allUS&searchByFilterName=zipCode&zipOrCityRadius=50&searchByFilterValue=77584

08/18  https://www.chnola.org/blog/2018/august/understanding-heart-failure-in-children/ Blog from Children’s Hospital for parents with kids with heart failure.

10/18 https://childrensconnection.blog/2018/10/11/fixing-broken-hearts-with-cardiac-catheterization/ Blog from Children’s Hospital related to anticipatory guidance for parents with kids who need a catheterization procedure.

02/19  https://www.wdsu.com/article/heart-surgery-saves-8-month-old-babys-life/26112715 Local news report about the care provided by the Heart team at Children’s Hospital to treat a Honduran child.

Fellow, SCAI

Fellow, PICS

Fellow, AAP

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IMAGES

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  2. Clinical Case Reports on Cardiology

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  3. Global Journal of Cardiology Research and Cardiovascular Diseases

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COMMENTS

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