A 40-year-old male presented with exertional dyspnoea and was diagnosed as having restrictive cardiomyopathy with severely decreased LV systolic function. [ncbi.nlm.nih.gov]

This presents as increased "contractility" because of Frank-Starling mechanisms, but there isn't an intrinsic positive ionotropy. It's a mechanical effect. [forums.studentdoctor.net]

Semigran Journal of the American College of Cardiology Efficacy of Chemotherapy for Light-Chain Amyloidosis in Patients Presenting With Symptomatic Heart Failure Brett W. Sperry, Mazen Hanna, et al. [onlinejacc.org]

• Hypoxemia

  61 Solitary Pulmonary Nodule in a 58YearOld Male Case 42 483 Chapter 62 Persistent Jaundice in a 1MonthOld Infant Case 43 497 Chapter 63 Newborn Baby with a Large Abdominal Wall Defect Case 44 503 Chapter 64 Newborn Infant with Labored Breathing and Hypoxemia [books.google.com]

  The chest X-ray revealed an increased cardiothoracic index and arterial blood gas analysis showed mild hypoxemia. Blood analysis revealed increased proBNP (1781 pg/ml), without anemia or renal impairment. [revportcardiol.org]

• Pharyngitis

  Muscle- weakness of various muscles including pharyngeal and respiratory muscles. Liver- hepatomegaly. Heart- infiltrative cardiomyopathy. Intelligence is usually normal. Onset of Pompe’s disease may be infantile, juvenile or adult. [heartpearls.com]

• Heart Disease

  Stay on top of the latest advances in molecular imaging, intravascular ultrasound, cardiovascular regeneration and tissue engineering, device therapy for advanced heart failure, atrial fibrillation management, structural heart disease, Chagasic heart [books.google.com]

  Jaber, MD Author and Disclosure Information PDF PD F PDF Read the Full Article Next Article: Strengthening the standards for preventing heart disease and stroke: The recent AHA guidelines Cardiology Imaging Immunology Pulmonology [mdedge.com]

  Disease François-Pierre Mongeon, Raymond Y. [imaging.onlinejacc.org]

  He had no history of hypertension, diabetes mellitus, or heart disease. On general physical examination, he had ascitis and bilateral pedal edema. [ijpmonline.org]

• Hypotension

  […] physical hx depends on the classification - CHF, dyspnea, orthonpnea, fatigue, weakness, anorexia, angina, poor exercise tolerance, peripheral edema, ascites, hepatomegally, hypotension, jugular venous distention, cardiac tumors Restrictive (Infiltrative [quizlet.com]

  Cardiac: CHF, arrhythmias, hypotension, imaging abnormalities CHFpEF CHFrEF in amyloidosis Conduction disease with heart block, tachyarrhythmias, and low volts on EKG LVH and increased LV mass on TTE Combination of increased LV mass & low volts is very [ucsfmed.wordpress.com]

  There are five useful clinical signs to establish the presence and severity of cardiomyopathy: general appearance (cachexia and dyspnea at rest indicate severe impairment), hypotension, tachycardia, elevated jugular venous pressure, and displaced left [clevelandclinicmeded.com]

  Accordingly, angiotensin receptor enzyme inhibitors are generally avoided, because these drugs blunt an important axis for blood pressure control, and in the presence of preexisting autonomic dysfunction and diminished vascular tone can lead to profound hypotension [circ.ahajournals.org]

  There is a high susceptibility to digitalis intoxication, nifedipine-induced hemodynamic deterioration, verapamil-induced left ventricular dysfunction, and ACE-inhibitor induced profound hypotension. [textbookofcardiology.org]

• Akinesia

  […] drugs: epi-pen, cymbalta, synthroid can cause CM -recovery w/in 1 month echocardiographic signs: hyperkinetic base and akinetic or dyskinetic LV apex (apex alone bulges or bulges and elongates), BANANA SHAPE variations exist: reverse takotsubo (basal akinesia [quizlet.com]

  […] ejection fraction 40% By RV angiography: Regional RV akinesia, dyskinesia, or aneurysm Minor By 2D echo: Regional RV akinesia or dyskinesia and 1 of the following (end diastole): PLAX RVOT 29 to 2 ) PSAX RVOT 32 to 2 ) or fractional area change 33% to [textbookofcardiology.org]

• Ground Glass Appearance

  LVH, RVH GROUND GLASS appearance of endocardium and myocardium- speckled - nl or dec LV syst fxn - abnl diastolic filling fxn (dec compliance) - poss pericardial eff - w endomyocardial fibrosis- endocardium appears echogenic Restrictive (Infiltrative) [quizlet.com]

• Mediastinal Mass

  Chapter 57 Gastric Bypass Case 39 451 Chapter 58 Coronary Revascularization Case 40 463 Coronary Bypass 470 Chapter 60 Mediastinal Mass in a 61YearOld Male Case 41 475 Chapter 61 Solitary Pulmonary Nodule in a 58YearOld Male Case 42 483 Chapter 62 Persistent [books.google.com]

• Nodular Glomerulosclerosis

  Moulin B, Deret S, Mariette X, Kourilsky O, Imai H, Dupouet L, Marcellin L, Kolb I, Aucouturier P, Brouet JC, Ronco PM, Mougenot B: Nodular glomerulosclerosis with deposition of monoclonal immunoglobulin heavy chains lacking C[H]1. [karger.com]

• Poor R Wave Progression

  Against it was the ECG showing poor R-wave progression and echocardiographic findings suggestive of an infiltrative disease, with symmetric biventricular wall thickening and pericardial effusion. [revportcardiol.org]

  Sinus tachycardia at 112 bpm, frequent premature ventricular contractions and 1.0 mm ST depression in V6, left axis deviation, left atrial enlargement, poor R-wave progression and lateral ST/T-wave abnormalities were all noted on his electrocardiogram [centrallakesclinic.biz]

  Figure 3 shows atrial fibrillation, poor R wave progression, and diffuse ST-segment abnormalities in an ECG from a patient with ischemic cardiomyopathy. [clevelandclinicmeded.com]

• Short PR Interval

  Tags: enzyme replacement therapy, glycogen storage disease, infant, infiltrative cardiomyopathy, Pompe's disease, short PR interval [heartpearls.com]

In this article, three fundamental risk factors are considered for "secondary" diastolic dysfunction and HF-hypertension, diabetes, and obesity-with an emphasis on the clinical epidemiology, pathophysiologic mechanisms, and treatment implications of each [ncbi.nlm.nih.gov]

These may include the use of steroidal treatments, blood-thinning medications, diuretics, and anti-arrhythmia drugs If RCM occurs because of other medical conditions, then getting treated for those medical conditions may help delay its progression. [dovemed.com]

Content posted on, created for, or compiled by Lybrate is not intended or designed to replace your doctor's independent judgment about any symptom, condition, or the appropriateness or risks of a procedure or treatment for a given person. [lybrate.com]

Treatment of Pompe’s disease is alglucosidase alpha, which is an analogue of the deficient enzyme. It is very costly, but very effective. In the LOTS study, it was proven to prevent deterioration of vital capacity. [heartpearls.com]

Recent clinical studies suggest that patients with congestive heart failure and preserved LV function may benefit from specific medical treatment. [books.google.com]

[…] individuals may improve their quality of life by leading an active life, losing excess weight, stopping smoking, discontinuing drug and alcohol use, and eating a balanced and nutritious meals Restrictive Cardiomyopathy worsens over time, and therefore, the prognosis [dovemed.com]

[…] tissue biopsy and demonstration of amyloid. [2], [4], [5] Subcutaneous fat pad aspiration with a reported sensitivity of 84% to 88% [4] is the preferred technique because of ease and simplicity of the procedure. [3] AL cardiac amyloidosis has the worst prognosis [ijpmonline.org]

[…] indicated in patients with LVEF The prognosis of LVNC has currently not yet been defined. [textbookofcardiology.org]

They usually portend an adverse prognosis, although in rare instances (e.g. Fabry’s disease) early diagnosis can result in potentially curative treatment. [link.springer.com]

Prognosis In patients with cardiac-related symptoms, 5-year survival is less than 50%. Major events include progressive heart failure and sudden death due to arrhythmia. [orpha.net]

A family history of neurologic and/or cardiac disease may suggest TTR etiology. [orpha.net]

Etiology Although a variety of etiologies can result in a dilated cardiomyopathy which are listed as below. Some are classified as separate entities. [radiopaedia.org]

Distinguishing between ischemic and nonischemic etiology by echocardiography can be challenging. [centrallakesclinic.biz]

II – Forms A) Etiology The etiology of DCM is heterogeneous. In developed countries CAD and myocardial infarction (MI) are the most common causes of HF, approximating 50-75% of all HF patients. [escardio.org]

Perugini E, Guidalotti PL, Salvi F, et al Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol. 2005;46:1076–1084 CrossRef PubMed Google Scholar 14. [link.springer.com]

In this article, three fundamental risk factors are considered for "secondary" diastolic dysfunction and HF-hypertension, diabetes, and obesity-with an emphasis on the clinical epidemiology, pathophysiologic mechanisms, and treatment implications of each [ncbi.nlm.nih.gov]

Summary Epidemiology Prevalence is unknown. Patients present during adulthood (usually after 30 years of age) with restrictive cardiomyopathy (with varying degrees of chronic heart failure and possible brady/tachyarrhythmias). [orpha.net]

Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study. Circulation. 2015 Feb;131(7):624-32. DOI 10.1161/CIRCULATIONAHA.114.011522. (13.) Houston BA, Mukherjee M. [aprendeenlinea.udea.edu.co]

Images Review Date: 5/15/2008 Reviewed By: Alan Berger, MD, Assistant Professor, Divisions of Cardiology and Epidemiology, University of Minnesota, Minneapolis, MN. Review provided by VeriMed Healthcare Network. [lutheranhealthcare.org]

Published in 2014 Reference Pocket Guidelines Abriged version Published in 2014 Reference ESC-2014-Slide-set-Hypertrophic-Cardiomyopathy Table of contents: Full Text (ESC Clinical Practice Guidelines) Preamble Introduction Definition Scope of Guidelines Epidemiology [escardio.org]

Abstract The detailed pathophysiology of heart failure with preserved ejection fraction (HF-PEF) remains an area of active research and controversy; however, abnormalities of diastolic function are generally believed to play an important role. [ncbi.nlm.nih.gov]

Cardiomyopathies may be classified as either primary or secondary, on the basis of etiology, or on the pathophysiology of the lesion: hypertrophic, dilated, or restrictive. [icd10data.com]

Medscape Education Clinical Briefs, September 10, 2012 Landmark Hypertrophic Cardiomyopathy Recommendations Issued Heartwire CME, November 14, 2011 About Cardiomyopathy Cardiomyopathy is a disease of the heart muscle that is classified according to pathophysiologic [medscape.org]

New frontiers and insights in pathophysiology, diagnosis, and management. Texas Heart Inst J. 2005;32(2):178-84. [ Links ] 22 Gertz MA, Benson MD, Dyck PJ, Grogan M, Coelho T, Cruz M, et al. [scielo.br]

Retrieved 15 October 2011. " restrictive cardiomyopathy " at Dorland's Medical Dictionary a b c d e Pathophysiology of heart disease : a collaborative project of medical students and faculty. Lilly, Leonard S., Harvard Medical School. (5th ed.). [en.wikipedia.org]

Science JACC: CardioOncology JACC: Cardiovascular Interventions JACC: Case Reports JACC: Clinical Electrophysiology JACC: Heart Failure Issues Current Articles in Press Archives Topics Multimedia Cardiology Hour Future of JACC: CV Imaging Guidelines Prevention [imaging.onlinejacc.org]

Jaber, MD Author and Disclosure Information PDF PD F PDF Read the Full Article Next Article: Strengthening the standards for preventing heart disease and stroke: The recent AHA guidelines Cardiology Imaging Immunology Pulmonology [mdedge.com]

In the LOTS study, it was proven to prevent deterioration of vital capacity. The cardiac manifestations of Pompe’s disease can be classified into structural and electrical. [heartpearls.com]

[…] few individuals may live beyond 10 years following diagnosis of RCM Please find comprehensive information on Restrictive Cardiomyopathy regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention [dovemed.com]

Is It Possible to Prevent Cardiomyopathy? Cardiomyopathy is a term that describes the end result of many diseases and illnesses. [emedicinehealth.com]