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Heart 2022 Jun 24;108(14):1120-1160. Epub 2022 Jun 24.

Cardiology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Radiol Case Rep 2022 Aug 28;17(8):2598-2602. Epub 2022 May 28.

Department of Magnetic Resonance Imaging, Fuwai Hospital and National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beilishi Road No. 167, Xicheng District, Beijing 100037, China.

Cor triatriatum dexter (CTD) is an extremely rare congenital cardiac malformation in which a membrane divides the right atrium into 2 chambers. Hypertrophic cardiomyopathy (HCM) with restrictive phenotype is also a rare cardiomyopathy. We report a case with an 18-year history of chest discomfort, fatigue and syncope following intense physical activity was finally diagnosed with CTD complicated with HCM, and the HCM is a special type, restrictive phenotype. Read More

J Nucl Cardiol 2022 May 31. Epub 2022 May 31.

Nuclear Medicine Department, Moinhos de Vento Hospital, Porto Alegre, Brazil.

Background: Cardiac amyloidosis (CA) is an under-diagnosed disease presenting as a restrictive cardiomyopathy with high morbidity and mortality. Wild-type transthyretin amyloid cardiomyopathy (ATTR-CM) is mostly seen in elderly patients, with increasing prevalence as life expectancy is growing. New diagnostic imaging techniques and treatments allow for a better prognosis, but lack of clinical awareness delays timely diagnosis and appropriate management. Read More

BMJ Case Rep 2022 Apr 22;15(4). Epub 2022 Apr 22.

Department of Cardiology, Worcestershire Acute Hospitals NHS Trust, Worcester, UK.

A woman in her 70s presented with chest pain, which was initially thought to be an acute coronary syndrome but subsequently felt to be pericarditis. Chest radiography and echocardiography demonstrated striking cardiomegaly and marked biatrial dilatation, likely secondary to undiagnosed restrictive cardiomyopathy. The patient remained well on the ward for some days with only mild discomfort and stable haemodynamics. Read More

Circ Cardiovasc Imaging 2022 04 19;15(4):e013556. Epub 2022 Apr 19.

Department of Cardiology, Cardiovascular Center, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (I.-C.H., Y.E.Y., G.-Y.C.).

Background: The echocardiographic assessment of left ventricular (LV) diastolic dysfunction (LVDD) in patients with hypertrophic cardiomyopathy is complex and not well-established. We investigated whether the left atrial reservoir strain (LARS) could be used to categorize LVDD and whether this grading is predictive of heart failure (HF) events in hypertrophic cardiomyopathy.

Methods: A total of 414 patients with hypertrophic cardiomyopathy (aged 58. Read More

Methodist Debakey Cardiovasc J 2022 14;18(2):1-3. Epub 2022 Mar 14.

Houston Methodist DeBakey Heart & Vascular Center, Methodist J.C. Walter Jr Transplant Center, Houston Methodist, Houston, Texas, US.

Methodist Debakey Cardiovasc J 2022 14;18(2):4-16. Epub 2022 Mar 14.

Department of Cardiovascular Medicine, Houston Methodist Hospital, Houston, Texas, US.

Restrictive cardiomyopathy (RCM) includes a heterogeneous group of diseases that cause increased myocardial stiffness, leading to impaired ventricular relaxation and severe diastolic dysfunction. Given that it is the least common type of cardiomyopathy, it can be a diagnostic challenge due to its varied pathogenesis, clinical presentation, and diagnostic evaluation. In this review, we provide an overview of different etiologies of RCM and examine the diagnostic and treatment approaches for various types. Read More

Methodist Debakey Cardiovasc J 2022 14;18(2):47-58. Epub 2022 Mar 14.

Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist Hospital, Houston, Texas, US.

Cardiac amyloidosis (CA) is the buildup and infiltration of amyloid plaque in cardiac muscle. An underdiagnosed form of restrictive cardiomyopathy, CA can rapidly progress into heart failure. CA is evaluated using a multimodality approach that includes echocardiography, cardiac magnetic imaging, and nuclear imaging. Read More

Qatar Med J 2022 22;2022(1). Epub 2022 Mar 22.

Department of General Medicine & Hematology, St. John's Medical College, Bangalore, India E-mail:

Cardiac amyloidosis is a rare disorder caused by the myocardial deposition of abnormal fibrils. A 52-year-old man was referred to our center with clinical features of heart failure, after cardiac magnetic resonance imaging showed restrictive cardiomyopathy. Abdominal fat pad biopsy showed features of amyloidosis, and after hematological workup, he was diagnosed with Waldenstrom macroglobulinemia (WM). Read More

Int J Cardiol 2022 06 30;357:55-71. Epub 2022 Mar 30.

Department of Clinical and Molecular Medicine, Sapienza University of Rome, Division of Cardiology, Sant'Andrea Hospital, Via di Grottarossa 1035-1039, 00189 Rome, Italy.

Cardiomyopathies (CMPs) are myocardial diseases in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality. Thought for a long time to be rare diseases, it is now clear that most of the CMPs can be easily observed in clinical practice. However, there is a group of specific heart muscle diseases that are rare in nature whose clinical/echocardiographic phenotypes resemble those of the four classical morphological subgroups of hypertrophic, dilated, restrictive, arrhythmogenic CMPs. Read More

G Ital Cardiol (Rome) 2022 Apr;23(4):259-267

Scuola di Specialità in Malattie Cardiovascolari, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), Trieste.

Eosinophilic myocarditis is a rare form of myocardial inflammation characterized by interstitial infiltration by eosinophils. Clinical presentation may vary from complete absence of symptoms to fulminant myocarditis with cardiogenic shock, to chronic heart failure due to progression to restrictive cardiomyopathy. The main causes of eosinophilic myocarditis are hypersensitivity reactions secondary to drug exposure, eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome and infections. Read More

Diagnostics (Basel) 2022 Mar 3;12(3). Epub 2022 Mar 3.

Division of Cardiology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea.

Amyloidosis is a progressive infiltrative disease instigated by the extracellular deposition of amyloid fibrils in various organs such as the heart, kidney, and peripheral nerves. Cardiac amyloid deposits cause restrictive cardiomyopathy, leading to a poor prognosis in systemic amyloidosis. The most common etiologies of cardiac amyloidosis (CA) are immunoglobulin light chain deposits (AL-CA) and misfolded transthyretin deposits (ATTR-CA). Read More

Eur Heart J Case Rep 2022 Feb 22;6(2):ytac081. Epub 2022 Feb 22.

Dr Ram Manohar Lohia Hospital and ABVIMS, 32/31 West Patel Nagar, New Delhi 110001, India.

Background: Cardiac amyloidosis presentation in an affected individual can be varied. We describe a patient who had the entire spectrum of involvement in his life time. Initially presented as an ischaemic heart disease and later developed complete heart block (CHB) and frank cardiomyopathy. Read More

Eur Heart J Case Rep 2022 Feb 7;6(2):ytac058. Epub 2022 Feb 7.

Pediatric Cardiology Unit and Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique (CHUV and HUG), Pediatric Department, University of Geneva and Lausanne, Geneva, Switzerland.

Background: Eosinophilic myocarditis (EM) is a relatively rare form of myocarditis that could progress to restrictive cardiomyopathy and might be fatal if left untreated. Although myocardial biopsy is considered to be the gold standard for the diagnosis of myocarditis, its use in paediatrics remains controversial and not easily applicable in routine practice.

Case Summary: A 10-year-old girl with no prior medical history presented to the emergency department for fever, odynophagia, and gastrointestinal symptoms despite 48 h of antibiotics (Cefaclor). Read More

Heart Fail Rev 2022 Mar 4. Epub 2022 Mar 4.

Houston Methodist Debakey Heart & Vascular Center, Houston, TX, USA.

Cardiac amyloidosis (CA) is an underdiagnosed form of restrictive cardiomyopathy leading to a rapid progression into heart failure. Evaluation of CA requires a multimodality approach making use of echocardiography, cardiac magnetic imaging (CMR), and nuclear imaging. With superior tissue characterization, high-resolution imaging, and precise cardiac assessment, CMR has emerged as a versatile tool in the workup of cardiac amyloidosis with a wide array of parameters both visual and quantitative. Read More

Intern Med 2022 Feb 26. Epub 2022 Feb 26.

Department of Radiology, Jichi Medical University Saitama Medical Center, Japan.

Liver Int 2022 06 8;42(6):1369-1378. Epub 2022 Mar 8.

Children´s Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

Background And Aims: Mulibrey nanism (MUL) is a multiorgan disease caused by recessive mutations in the TRIM37 gene. Chronic heart failure and hepatopathy are major determinants of prognosis in MUL patients, which prompted us to study liver biochemistry and pathology in a national cohort of MUL patients.

Methods: Clinical, laboratory and imaging data were collected in a cross-sectional survey and retrospectively from hospital records. Read More

Adv Clin Exp Med 2022 Feb;31(2):175-185

Department of Bone Marrow Transplantation, Hematology and Transplantology Clinic, Tomasz Sokolowski Public Hospital, Pomeranian Medical University in Szczecin, Poland.

A substantial increase in the interest in transthyretin cardiac amyloidosis (ATTR-CA) is a result of the constantly growing number of patients, the use of clear diagnostic protocols and the availability of the first selective drug for these patients. This has also raised the awareness of the disease among physicians of all specialties. The topic is particularly relevant to cardiologists, who use non-invasive multimodal imaging in their daily practice. Read More

Ann Cardiol Angeiol (Paris) 2022 Apr 17;71(2):63-74. Epub 2022 Feb 17.

CHU Henri Mondor, 1 Rue Gustave Eiffel, 94000 Créteil. Electronic address:

Infiltrative cardiomyopathies are abnormal accumulations or depositions of different substances in cardiac tissue leading to its dysfunction, first diastolic, then systolic. The different infiltrative cardiomyopathies are amyloidosis (both light chain amyloidosis and transthyretin amyloidosis variants), lysosomal and glycogen storage disorders (Fabry-Anderson disease), and iron overload (hemochromatosis and thalassemia associated with blood transfusions), as well as inflammatory diseases such as sarcoidosis. We also evoke hypereosinophilic syndrome associated with endomyocardial fibrosis. Read More

J Clin Med 2022 Jan 24;11(3). Epub 2022 Jan 24.

Cardiovascular Imaging Unit and Inherited Cardiovascular Diseases Unit, Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, 08035 Barcelona, Spain.

Cardiomyopathies are a group of structural and/or functional myocardial disorders which encompasses hypertrophic, dilated, arrhythmogenic, restrictive, and other cardiomyopathies. Multimodality cardiac imaging techniques are the cornerstone of cardiomyopathy diagnosis; transthoracic echocardiography should be the first-line imaging modality due to its availability, and diagnosis should be confirmed by cardiovascular magnetic resonance, which will provide more accurate morphologic and functional information, as well as extensive tissue characterization. Multimodality cardiac imaging techniques are also essential in assessing the prognosis of patients with cardiomyopathies; left ventricular ejection fraction and late gadolinium enhancement are two of the main variables used for risk stratification, and they are incorporated into clinical practice guidelines. Read More

J Magn Reson Imaging 2022 Jan 24. Epub 2022 Jan 24.

Department of Electronics, Information and Bioengineering, Politecnico di Milano, Milan, Italy.

Background: Time-resolved three-directional velocity-encoded (4D flow) magnetic resonance imaging (MRI) enables the quantification of left ventricular (LV) intracavitary fluid dynamics and energetics, providing mechanistic insight into LV dysfunctions. Before becoming a support to diagnosis and patient stratification, this analysis should prove capable of discriminating between clearly different LV derangements.

Purpose: To investigate the potential of 4D flow in identifying fluid dynamic and energetics derangements in ischemic and restrictive LV cardiomyopathies. Read More

Curr Probl Cardiol 2022 Jan 7:101106. Epub 2022 Jan 7.

Department of Transplantation, Mayo Clinic, Jacksonville, FL.

Cardiac amyloidosis (CA) is a complex disease considered to be the most common underdiagnosed form of restrictive cardiomyopathy. Accumulation of misfolded proteins called amyloid fibrils in the extracellular space results in clinical deterioration and late diagnosis is associated with morbidity and mortality. Both types of this disease, light chain CA and transthyretin-related CA share many cardiac and extracardiac features that compromise multiple organs such as kidneys, musculoskeletal system, autonomic nervous system, and gastrointestinal tract. Read More

Kardiol Pol 2022 22;80(3):278-285. Epub 2021 Dec 22.

Magnetic Resonance Unit, National Institute of Cardiology, Warszawa, Poland.

Background: The heart failure (HF) population is estimated to be 64.3 million people worldwide and continues to grow. Identifying the underlying cause of HF is crucial for patient management and prognosis. Read More

Front Cardiovasc Med 2021 29;8:751415. Epub 2021 Nov 29.

Department of Cardiac Sciences, Libin Cardiovascular Institute of Alberta, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.

Chronic Chagas Cardiomyopathy is a unique form of cardiomyopathy, with a significantly higher mortality risk than other heart failure etiologies. Diastolic dysfunction (DD) plays an important role in the prognosis of CCM; however, the value of serum biomarkers in identifying and stratifying DD has been poorly studied in this context. We aimed to analyze the correlation of six biochemical markers with diastolic function echocardiographic markers and DD diagnosis in patients with CCM. Read More

J Investig Med High Impact Case Rep 2021 Jan-Dec;9:23247096211058487

State University of New York Upstate Medical University, Syracuse, USA.

The association between large pericardial effusion and restrictive cardiomyopathy (RCM) is uncommon and has seldom been described. We describe an uncommon case of a 31-year-old male with RCM who presented with large, recurrent pericardial effusion, heart failure, and echocardiographic findings showing progressive worsening of diastolic function even after total pericardiectomy who was eventually transferred for cardiac transplant evaluation. Read More

Am J Case Rep 2021 Nov 24;22:e933677. Epub 2021 Nov 24.

Department of Congenital Heart Diseases, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Moscow, Russian Federation.

BACKGROUND The cardiotoxic effects of chemotherapy in cancer treatment can damage cardiomyocytes. A common link in the pathogenesis is the proliferation of fibroblasts and the increase of collagen synthesis, leading to development of common endomyocardial fibrosis. The walls of ventricles become rigid and their inability to relax prevents them from carrying the required amount of blood. Read More

Lancet 2021 11;398(10314):1913

University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK; Institute of Cardiovascular Sciences, University of Birmingham, Edgbaston, Birmingham, UK.

Heart Fail Rev 2021 Nov 7. Epub 2021 Nov 7.

Houston Methodist Debakey Heart & Vascular Center, Houston, TX, USA.

Cardiac amyloidosis, characterized by progressive restrictive cardiomyopathy, presents unusual diagnostic challenges. Conventional cardiac scintigraphy has shown limited utility in the quantification of disease burden and serial follow-up of cardiac amyloidosis. The advent of specialized positron emission tomography with specific amyloid-binding radiotracers has the potential to change currently employed diagnostic algorithms for the imaging of cardiac amyloidosis. Read More

ESC Heart Fail 2021 12 29;8(6):4863-4872. Epub 2021 Oct 29.

Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, National Clinical Research Center for Cardiovascular Diseases, Beijing, China.

Aims: To distinguish between constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) using cardiac magnetic resonance feature tracking (CMR-FT) left ventricle (LV) diastolic time-strain curve patterns and myocardial strain.

Methods And Results: A total of 32 CP patients, 27 RCM patients, and 25 control subjects were examined by CMR-FT and analysed for global strain, segmental strain, and LV time-strain curve patterns in the longitudinal, circumferential, and radial directions. Speckle tracking echocardiography (STE) strain imaging was performed in some cases. Read More

Heart Fail Rev 2021 Oct 28. Epub 2021 Oct 28.

Houston Methodist Debakey Heart & Vascular Center, Houston, TX, USA.

Cardiac amyloidosis (CA) is an underdiagnosed form of restrictive cardiomyopathy leading to a rapid progression into heart failure. Evaluation of CA requires a multimodality approach making use of echocardiography, cardiac magnetic imaging, and nuclear imaging. Technetium (Tc)-labeled cardiac scintigraphy has witnessed a resurgence in its application for the workup of CA. Read More