Presentation
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]
Signs and symptoms (orthopnea and paroxysmal nocturnal dyspnea) of left heart failure are also present. [circ.ahajournals.org]
Entire Body System
- Congestive Heart Failure
Over the past decade there has been an increasing awareness of left ventricular (LV) diastolic dysfunction as a mechanism of congestive heart failure, and it appears that more than 30-40 % of all heart failure patients have dominantly diastolic heart [books.google.com]
Thus, in a patient with congestive heart failure, these echocardiographic findings should suggest infiltrative cardiomyopathy. [ncbi.nlm.nih.gov]
[…] muscle) Cardiomyopathy associated with another disorder Cardiomyopathy, primary (heart condition) Congestive heart failure (chf) with cardiomyopathy Congestive heart failure due to cardiomyopathy Primary cardiomyopathy Secondary cardiomyopathy Tachycardia [icd10data.com]
A provisional diagnosis of congestive heart failure (CHF) due to infiltrative cadiomyopathy (possibly due to amyloidosis) was made. An abdominal fat pad aspirate demonstrated Congo red positive deposits of amyloid [Figure 2A]. [ijpmonline.org]
"Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents". American Journal of Cardiology. 55 (13): 1645. doi : 10.1016/0002-9149(85)90995-6. ISSN 0002-9149. PMID 4003314. [ipfs.io]
- Asymptomatic
Besides DCM, the other manifestations of anthracycline cardiotoxicity include restrictive cardiomyopathy (symptomatic and asymptomatic), cardiac arrhythmias [14], asymptomatic left ventricular enlargement, and more subtle changes of cardiac function. [emedicine.medscape.com]
Although cardiomyopathy is asymptomatic in the early stages, symptoms are the same as those characteristically seen in any type of heart failure and may include shortness of breath, fatigue, cough, orthopnea, paroxysmal nocturnal dyspnea, and edema. [aafp.org]
Serial echocardiographic observations in patients with primary systemic amyloidosis: an introduction to the concept of early (asymptomatic) amyloid infiltration of the heart. Mayo Clin Proc 1984; 59:589–597 PubMed Google Scholar 7. [link.springer.com]
Cueto-Garcia L, Tajik AJ, Kyle RA, Edwards WD, Greipp PR, Callahan JA, Shub C, Seward JB: Serial echocardiographic observations in patients with primary systemic amyloidosis: An introduction to the concept of early (asymptomatic) amyloid infiltration [karger.com]
- 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]
Gastrointestinal
- Failure to Thrive
Clinical Presentation and Diagnosis Age of onset may be highly variable, with cyanosis, failure to thrive or dysmorphic features described in the neonatal period, to adult patients presenting with LV failure or ventricular arrhythmia. [textbookofcardiology.org]
Liver, Gall & Pancreas
- Hepatomegaly
Liver- hepatomegaly. Heart- infiltrative cardiomyopathy. Intelligence is usually normal. Onset of Pompe’s disease may be infantile, juvenile or adult. Infantile onset causes death before 2 years of age. [heartpearls.com]
The remainder of the examination was notable for dullness to percussion at the lung bases, ascites, tender hepatomegaly, and marked bilateral lower extremity pitting edema. [circ.ahajournals.org]
Cardiovascular
- 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]
Atrial fibrillation is a common manifestation of hypertensive heart disease. Hypertensive heart disease is the leading identifiable cause of heart failure in older women. [clevelandclinicmeded.com]
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
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]
[…] 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]
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]
Workup
Figure 2 Diagram shows the diagnostic workup and management of cobalt-induced cardiomyopathy. CMR, cardiac magnetic resonance; CT, computed tomography; EKG, electrocardiogram; LGE, late gadolinium enhancement; MRI, magnetic resonance imaging. [pubmed.ncbi.nlm.nih.gov]
QRS Wave
- Poor R Wave Progression
The electrocardiogram showed normal sinus rhythm, QS waves in the inferior leads, poor R progression in the precordial leads and T-wave inversion in I and aVL ( Figure 1 ). [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]
Treatment
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]
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]
Treatment The condition causing the cardiomyopathy is treated when it can be found. Few treatments are known to work well for restrictive cardiomyopathy. The main goal of treatment is to control symptoms and improve quality of life. [stlukes-stl.com]
Current Treatment Options in Cardiovascular Medicine. 2 (5): 431–438. doi : 10.1007/s11936-000-0038-6. ISSN 1092-8464. PMID 11096547. External links Overview at Merck Manual [ipfs.io]
[…] despite conventional treatment. [revespcardiol.org]
Prognosis
[…] 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]
[…] indicated in patients with LVEF The prognosis of LVNC has currently not yet been defined. [textbookofcardiology.org]
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]
Despite optimal medical treatment, prognosis of patients with severe diastolic dysfunction remains poor. [wikiecho.org]
They usually portend an adverse prognosis, although in rare instances (eg, Fabry disease) early diagnosis can result in potentially curative treatment. [heart.bmj.com]
Etiology
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]
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]
Epidemiology
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]
Pathophysiology
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]
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]
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]
^ " 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]
Prevention
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]
Is It Possible to Prevent Cardiomyopathy? Cardiomyopathy is a term that describes the end result of many diseases and illnesses. [emedicinehealth.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]
The following treatments may be used to control symptoms or prevent problems: Blood thinning medicines Chemotherapy (in some situations) Diuretics to remove fluid and help improve breathing Medicines to prevent or control abnormal heart rhythms Steroids [medlineplus.gov]