2,407 results match your criteria Cardiomyopathy Restrictive


Mechanical and interventional support for heart failure with preserved ejection fraction: A review.

Artif Organs 2022 May 17. Epub 2022 May 17.

Division of Cardiac Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

Introduction: Restrictive cardiomyopathy (RCM) and hypertrophic cardiomyopathy (HCM) are two disease processes that are known to progress to heart failure with preserved ejection fraction (HFpEF). Pharmacologic therapies for HFpEF have not improved patient outcomes or reduced mortality in this patient cohort; thus, there continues to be substantial interest in other treatment strategies, including surgical interventions and devices. In this article, we explore and report the current utility of percutaneous therapies and surgically implanted mechanical support in the treatment of patients with HFpEF. Read More

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Effect of left ventricular geometric remodeling on restrictive filling pattern and survival in ischemic cardiomyopathy.

Indian Heart J 2022 May 2. Epub 2022 May 2.

Department of Cardiology, St. John's Medical College Hospital, India.

Background: To evaluate the effects of Left ventricular remodeling patterns in patients with left ventricular restrictive filling pattern (RFP; E/A>2) in ischemic cardiomyopathy (ICM) on prognosis.

Methods: Patient data was retrospectively analyzed over a period of 4.5 years to determine the effect of LV geometry by Echocardiographic parameterson survival and re-admission for heart failure. Read More

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Pre-existing Ab against vimentin leads to false-positive HLA Ab results in two pediatric heart transplant candidates.

Pediatr Transplant 2022 May 4:e14302. Epub 2022 May 4.

Cardiology, Phoenix Children's Hospital, Phoenix, Arizona, USA.

Background: HLA Ab analysis is carried out as a routine assay both pre- and post-heart transplantation to identify Abs directed against HLA with a focus post-transplant on those Abs that are donor-specific. While virtual crossmatching has decreased the requirement for prospective crossmatching in many cases, the management of highly sensitized children on the heart transplant waitlist remains challenging and can delay the ability to successfully identify a suitable organ.

Methods: This report describes the histocompatibility assessment and management of identical twin boys with familial restrictive cardiomyopathy serially listed for transplant. Read More

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Trends in Annual Incidence Rates of Newly Diagnosed Endomyocardial Fibrosis Cases at the Uganda Heart Institute: A 14-Year Review.

Front Cardiovasc Med 2022 15;9:841346. Epub 2022 Apr 15.

Uganda Heart Institute, Mulago Hospital Complex, Kampala, Uganda.

Background: First described in Uganda over seven decades ago, Endomyocardial fibrosis (EMF) is a rare form of restrictive cardiomyopathy found in the tropics. EMF occurs mainly in two phenotypes; biventricular involvement and right ventricular (RV) form. Previously endemic in several countries, there are reports suggesting that the disease is on the decline. Read More

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Sex-Related Differences in Genetic Cardiomyopathies.

J Am Heart Assoc 2022 May 26;11(9):e024947. Epub 2022 Apr 26.

Cardiomyopathy Unit Careggi University Hospital Florence Italy.

Cardiomyopathies are a heterogeneous collection of diseases that have in common primary functional and structural abnormalities of the heart muscle, often genetically determined. The most effective categorization of cardiomyopathies is based on the presenting phenotype, with hypertrophic, dilated, arrhythmogenic, and restrictive cardiomyopathy as the prototypes. Sex modulates the prevalence, morpho-functional manifestations and clinical course of cardiomyopathies. Read More

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Establishment of a Dedicated Inherited Cardiomyopathy Clinic: From Challenges to Improved Patients' Outcome.

J Am Heart Assoc 2022 May 26;11(9):e024501. Epub 2022 Apr 26.

Hartford HealthCare, Heart and Vascular Institute Hartford CT.

Background Inherited cardiomyopathies (ICs) are relatively rare. General cardiologists have little experience in diagnosing and managing these conditions. International societies have recognized the need for dedicated IC clinics. Read More

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Isolated cardiac amyloidosis with normal interventricular septum thickness: a case report.

Authors:
Zhanwen Xu Yaqin Li

Cardiol Young 2022 Apr 25:1-3. Epub 2022 Apr 25.

Department of Cardiology, Affiliated Hospital of Hebei University, Baoding, China.

Cardiac amyloidosis presented with normal interventricular septum is an extremely rare entity, and diagnosis may be difficult. This report discusses a 44-year-old female who presented with worsening dyspnoea on exertion, orthopnoea, and lower-extremity oedema. Electrocardiogram depicted low voltage in limb leads and a pseudoinfarct pattern. Read More

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Dual-processing theory helps to explain delay in diagnosis of Stanford type A aortic dissection.

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

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Mixed-Etiology Restrictive Cardiomyopathy (Desminopathy and Hemochromatosis) with Complex Liver Lesions.

Genes (Basel) 2022 03 24;13(4). Epub 2022 Mar 24.

Laboratory of Medical Genetics, B.V. Petrovsky Russian Research Center of Surgery, 119991 Moscow, Russia.

A 28 year-old male with restrictive cardiomyopathy (RCM) and endocardium thickening, conduction disorders, heart failure, and depressive disorder treated with paroxetine was admitted to the clinic. Blood tests revealed an increase in serum iron level, transferrin saturation percentage, and slightly elevated liver function tests. Sarcoidosis, storage diseases and Loeffler endocarditis were ruled out. Read More

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Genetic Insights into Primary Restrictive Cardiomyopathy.

J Clin Med 2022 Apr 8;11(8). Epub 2022 Apr 8.

Comprehensive Heart Failure Center (CHFC), Department of Medicine I, University Clinic Würzburg, Am Schwarzenberg 15, 97078 Würzburg, Germany.

Restrictive cardiomyopathy is a rare cardiac disease causing severe diastolic dysfunction, ventricular stiffness and dilated atria. In consequence, it induces heart failure often with preserved ejection fraction and is associated with a high mortality. Since it is a poor clinical prognosis, patients with restrictive cardiomyopathy frequently require heart transplantation. Read More

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Left Atrial Reservoir Strain-Based Left Ventricular Diastolic Function Grading and Incident Heart Failure in Hypertrophic Cardiomyopathy.

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

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TTR variants in patients with dilated cardiomyopathy: An investigation of the DCM Precision Medicine Study.

Genet Med 2022 Apr 18. Epub 2022 Apr 18.

Division of Human Genetics, Department of Internal Medicine, College of Medicine, The Ohio State University, Columbus, OH; Division of Cardiovascular Medicine, Department of Internal Medicine, College of Medicine, The Ohio State University, Columbus, OH. Electronic address:

Purpose: The cardiac phenotype of hereditary transthyretin amyloidosis (hTTR) usually presents as a restrictive or hypertrophic cardiomyopathy, and, although rarely observed as dilated cardiomyopathy (DCM), TTR is routinely included in DCM genetic testing panels. However, the prevalence and phenotypes of TTR variants in patients with DCM have not been reported.

Methods: Exome sequences of 729 probands with idiopathic DCM were analyzed for TTR and 35 DCM genes. Read More

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Insights on Infiltrative and Restrictive Cardiomyopathies.

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.

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Overview of Restrictive Cardiomyopathies.

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

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It Takes a Village: Multimodality Imaging of Cardiac Amyloidosis.

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

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Cardiac Amyloidosis Treatment.

Methodist Debakey Cardiovasc J 2022 14;18(2):59-72. Epub 2022 Mar 14.

Smidt Heart Institute, Cedars-Sinai, Los Angeles, California, US.

Cardiac amyloidosis (CA) is a restrictive cardiomyopathy with a traditionally poor prognosis. Until recently, CA treatment options were limited and consisted predominantly of managing symptoms and disease-related complications. However, the last decade has seen significant advances in disease-modifying therapies, increased awareness of CA, and improved diagnostic methods resulting in earlier diagnoses. Read More

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Rare Complication of a Rare Malignancy: Case Report of Cardiac Amyloidosis Secondary to Waldenstrom Macroglobulinemia.

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

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Diagnosis and Management of Rare Cardiomyopathies in Adult and Paediatric Patients. A Position Paper of the Italian Society of Cardiology (SIC) and Italian Society of Paediatric Cardiology (SICP).

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

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Spontaneous Bacterial Peritonitis in a Patient With Nonportal Hypertensive Ascites.

Ochsner J 2022 ;22(1):100-103

Department of Hospital Medicine, Ochsner Clinic Foundation, Ochsner Medical Center - West Bank Campus, Gretna, LA.

Spontaneous bacterial peritonitis (SBP) is a life-threatening condition classically found as a complication of cirrhotic ascites, but it has rarely been documented in a case of nonportal hypertensive ascites. We report the case of a 54-year-old male with SBP arising from nonportal hypertensive ascites in the setting of end-stage renal disease and restrictive cardiomyopathy, both secondary to primary amyloidosis (AL type, kappa light chain). Peritoneal fluid analysis showed a serum-ascites albumin gradient of 1. Read More

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January 2022

Restrictive cardiomyopathy: from genetics and clinical overview to animal modeling.

Rev Cardiovasc Med 2022 Mar;23(3):108

Heart Institute, Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN 38103, USA.

Restrictive cardiomyopathy (RCM), a potentially devastating heart muscle disorder, is characterized by diastolic dysfunction due to abnormal muscle relaxation and myocardial stiffness resulting in restrictive filling of the ventricles. Diastolic dysfunction is often accompanied by left atrial or bi-atrial enlargement and normal ventricular size and systolic function. RCM is the rarest form of cardiomyopathy, accounting for 2-5% of pediatric cardiomyopathy cases, however, survival rates have been reported to be 82%, 80%, and 68% at 1-, 2-, and 5-years after diagnosis, respectively. Read More

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[Ten questions about eosinophilic myocarditis].

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

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Deficiency Caused Restrictive Cardiomyopathy via Disrupting Proteostasis.

Int J Biol Sci 2022 28;18(5):2018-2031. Epub 2022 Feb 28.

State Key Laboratory of Proteomics, Beijing Proteome Research Center, National Center for Protein Sciences, Beijing Institute of Lifeomics, Beijing 100071, China.

The molecular mechanisms underlying restrictive cardiomyopathy (RCM) are not fully understood. Hepatocyte growth factor-regulated tyrosine kinase substrate (HGS) is a vital element of Endosomal sorting required for transport (ESCRT), which mediates protein sorting for degradation and is crucial for protein homeostasis (proteostasis) maintenance. However, the physiological function and underlying mechanisms of HGS in RCM are unexplored. Read More

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Multimodal Imaging and Biomarkers in Cardiac Amyloidosis.

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

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[Non-compaction and restrictive cardiomyopathy in pediatrics: Two types of myocardial diseases that are important to recognize].

Andes Pediatr 2021 Oct;92(5):667-676

Hospital Roberto del Río, Santiago, Chile.

Left ventricular non-compaction (LVNC) and restrictive cardiomyopathies (RCM) are rare diseases with high morbidity and mortality in the pediatric age group, particularly the restrictive. They can be diagnosed at any age even in fetal life, in isolation or association with other cardiomyopathies or congenital heart disease. The causes may be genetic, neuromuscular, metabolic, storage, or idiopathic disorders. Read More

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October 2021

A case report-facing blues in cardiac amyloidosis: no more a zebra.

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

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February 2022

Contribution of imaging modalities to eosinophilic myocarditis diagnosis: a case report.

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

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February 2022

Short-term outcomes of EXCOR Paediatric implantation.

Interact Cardiovasc Thorac Surg 2022 Mar 9. Epub 2022 Mar 9.

Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan.

Objectives: The aim of this study was to review a single institution's experience with EXCOR Paediatric implantation.

Methods: Patients <15 years old who underwent EXCOR implantation as a bridge to transplantation between 2015 and 2021 were enrolled. Major adverse events included death, cerebrovascular event resulting in sequelae, major infection (sepsis or surgical site infection requiring open sternal irrigation or device removal) and device malfunction requiring surgical treatment. Read More

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Changes in transcriptomic landscape in human end-stage heart failure with distinct etiology.

iScience 2022 Mar 17;25(3):103935. Epub 2022 Feb 17.

Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei, China.

Heart failure (HF) is the ultimate outcome of a variety of heart diseases, including restrictive cardiomyopathy (RCM), ischemic heart disease (IHD), and valvular heart disease (VHD). To date, accumulating evidence has suggested an important role of noncoding RNAs (ncRNAs) in HF. We performed RNA-sequencing studies with myocardial mRNAs/lncRNAs/circRNAs/miRNAs from non-failing hearts (donor heart tissue from heart transplantation) and three groups of patients with HF (RCM, IHD, and VHD). Read More

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Cardiovascular magnetic resonance for suspected cardiac amyloidosis: where are we now?

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

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