Cardiomyopathy Pathology Study Guide

Cardiomyopathy Pathology Video

Cardiomyopathy

Cardiomyopathy is a progressive heart disease that may affect heart function.

Examples of cardiomyopathy include:

Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Ischemic cardiomyopathy

Cardiomyopathy. Gross pathology of idiopathic cardiomyopathy. Opened left ventricle of heart shows a thickened, dilated left ventricle with subendocardial fibrosis manifested as increased whiteness of endocardium. Autopsy. CDC/Dr. Edwin P. Ewing, Jr. Not altered. Public Domain
Cardiomyopathy. Structural categories of cardiomyopathy Npatchett Not altered. CC BY-SA 3.0
Cardiomyopathy. Stained microscopic section of heart muscle in hypertrophic cardiomyopathy Koshiro Fujisawa. Not altered. CC BY-SA 3.0
Histological abnormalities in cardiomyopathy: (A) Dilated cardiomyopathy, increased interstitial fibrosis at the blue arrow. (B) Hypertrophic cardiomyopathy, increased interstitial fibrosis (blue), and myocyte disarray. (C) Fibro-fatty replacement in arrhythmogenic right ventricular cardiomyopathy. Taken from Hughes, Asimaki and Saffitz, Gulati.146–148. Taxonomy of segmental myocardial systolic dysfunction European Heart Journal. Not Altered. CC.
Cardiomyopathy Pathology. The arrhythmia, ventricular fibrillation, seen on an ECG Jer5150. Not altered. CC BY-SA 3.0

Dilated Cardiomyopathy

In dilated cardiomyopathy the heart’s four chambers enlarge.

Dilated cardiomyopathy is the most common type of cardiomyopathy.

Dilated cardiomyopathy leads to biventricular congestive heart failure (CHF) and systolic dysfunction (the inability of the ventricles to pump blood), with the comorbidities of mitral and tricuspid valve regurgitation and arrhythmia.

Dilated cardiomyopathy is typically idiopathic.

Factors associated with dilated cardiomyopathy include:

Genetic mutation (typically autosomal dominant)
Myocarditis
Alcohol abuse
Drugs (e.g. doxorubicin)
Pregnancy (seen in the final stages of pregnancy or shortly (weeks to months) after delivery)

Coxsackie viruses (coxsackie A or coxsackie B) are typically associated with myocarditis.

A lymphocytic infiltrate in the myocardium characterizes myocarditis.

Symptoms of myocarditis include chest discomfort, and arrhythmias.

Dilated cardiomyopathy is a late complication of myocarditis.

The treatment for dilated cardiomyopathy is heart transplant.

Dilated Cardiomyopathy. Dilated cardiomyopathy on CXR James Heilman, MD. Not altered. CC BY-SA 4.0
Chest x-ray in a child with dilated cardiomyopathy.Dilated cardiomyopathy in childhood. Franklin O, Burch M - Images in paediatric cardiology (2000). Not Altered. CC.
Dilated Cardiomyopathy. Dilated cardiomyopathy on CXR James Heilman, MD. Not altered. CC BY-SA 4.0
Dilated Cardiomyopathy. Serial 12-lead ECGs from a 49-year-old black man with cardiomyopathy. (TOP): Sinus tachycardia (rate about 101/min) with LBBB accompanied by RAD (here about 108°). Frequent multifocal PVCs (both singly and in pairs) and left atrial enlargement. (BOTTOM): Same patient about 5 months later status-post orthotopic heart transplant. - Not altered. CC BY-SA 3.0
Dilated Cardiomyopathy. Illustration of a Normal Heart vs. Heart with Dilated Cardiomyopathy Bruce Blaus. Not altered. CC BY 3.0

Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is the massive hypertrophy of the left ventricle.

Hypertrophic cardiomyopathy may be due to an autosomal dominant genetic alteration of sarcomere proteins that result in a large heart.

The classic cases vignettes of hypertrophic cardiomyopathy involve sudden death of young athletes.

Clinical features of hypertrophic cardiomyopathy include:

Diastolic dysfunction is caused by decreased cardiac output due to left ventricular hypertrophy (ventricle cannot fill)
Sudden death
Ventricular arrythmias
Syncope with exercise

Hypertrophic Cardiomyopathy. Saddle thrombus in the feline aorta. 1 opened Aorta with thrombus, 2 A. iliaca externa, 3 common trunk for both Aa. iliacae internae, 4 A. circumflexa ilium profunda, 5 A. mesenterica caudalis, 6 Colon descendens. Not altered. CC BY-SA 4.0
Hypertrophic Cardiomyopathy. An ECG showing HOCM W.G. de Voogt, MD, PhD, SLAZ, The Netherlands - W.G. de Voogt, MD, PhD, SLAZ, Not altered. CC BY-SA 3.0
Cardiomyopathy. Stained microscopic section of heart muscle in hypertrophic cardiomyopathy Koshiro Fujisawa. Not altered. CC BY-SA 3.0
Hypertrophic Cardiomyopathy. Illustration of asymmetric septal hypertorphy in HCM Npatchett. Not altered CC BY-SA 3.0
Transthoracic echocardiogram. A: Parasternal long axis view showing asymmetric septal hypertrophic cardiomyopathy in end diastole. B: A four-chamber view showing a combination of basal and mid-ventricular hypertrophic cardiomyopathy and an apical aneurysm (*). (LA = left atrium, RA = right atrium, RV = right ventricle, LV = left ventricle, Ao = aorta).Subaortic and mid-ventricular obstructive hypertrophic cardiomyopathy with an apical aneurysm: a case report. Cianciulli TF, Saccheri MC, Konopka IV, Serans DF, Acunzo RS, Escudero AM, Masoli OH, Prezioso HA - Cardiovascular ultrasound (2006). Not Altered. CC.

Restrictive Cardiomyopathy

Restrictive cardiomyopathy results in decreased ventricular endocardial compliance that prevents cardiac chamber filling during diastole.

Causes of restrictive cardiomyopathy include:

Endocardial fibroelastosis in children
Sarcoidosis
Hemochromatosis
Amyloidosis
Loeffler syndrome

Loeffler syndrome is a type of restrictive cardiomyopathy with endomyocardial fibrosis with eosinophilic infiltrates and eosinophilia.

Restrictive cardiomyopathy clinically manifests as congestive heart failure

The electrocardiogram (ECG) findings of restrictive cardiomyopathy show a low-voltage EKG with diminished QRS amplitude.

Restrictive Cardiomyopathy. High magnification micrograph of senile cardiac amyloidosis. Congo red stain. Autopsy specimen. The micrograph shows amyloid (extracellular washed-out red material) and abundant lipofuscin (yellow granular material). Nephron. Not altered. CC BY-SA 3.0
Right and left ventricular tracing with concordant change in pressures during expiration and inspiration confirming the diagnosis of restrictive cardiomyopathy. Cardiac amyloidosis responding to bortezomib: case report and review of literature. Charaf E, Iskandar SB, Blevins A, Abi-Saleh B, Fahrig S - Current cardiology reviews (2009). Not Altered. CC.