University:
Western Sydney University
Course:
MEDI 2003 | Clinical Sciences 2
Academic year:

2024
Views:

240

Pages:

2
Author:

Mariam A. A.

Restrictive Cardiomyopathy Definition Heart muscles become stiffer and less complaint Aetiology • • • • Amyloidosis (most common cause) Sarcoidosis Hemochromatosis Systemic sclerosis Pathophysiology • • • • • • • Mutant TTR -> Amyloid are proteins that have been misfolded -> deposit in various tissues and organs Sarcoidosis -> involves the formation of granulomas in the heart endocardial fibroelastosis -> fibrosis develops in endocardium -> tissue less complaint hemochromatosis, or iron overload, iron can deposit in the heart tissue, which contributes to restricted ventricular filling. Loffler syndrome -> eosinophils accumulate in endocardial layer -> inflammation -> endocardial fibrosis -> heart stiffer and less compliance Radiation generates reactive oxygen species in the tissue -> inflammation over time -> myocardial fibrosis -> heart tissue becomes stiffer more restrictive Proliferation of connective tissue -> decrease in the elasticity of the myocardium -> decrease in vascular compliance -> decreased diastolic filling -> atrial congestion -> atrial enlargement & severe diastolic dysfunction -> systemic venous congestion Signs and Symptoms • • • • • • • • • • • • Dyspnoea Jugular vein distention, due to back up of blood Peripheral edema Hepatomegaly, due to backing up of blood Kussmaul sign S4 heart sound Orthopnoea Inability to exercise Swelling of feet Uneven/rapid pulse Chest pain Fatigue Diagnosis • Echocardiography - Images of the heart - Good for initial diagnosis • • • • ECG - S and T wave abnormalities - Left bundle branch block or other conductive disorders evident - Law amplitude signals: peak measurements of QRS complex being less than 5 mm (limb limbs)/ less than 10 mm (precordial leads) - Smaller QRS complexes: these represent ventricular contraction -> restricted tissue -> weaker contraction Chest X-ray - Signs of pulmonary congestion ✓ Pulmonary edema ✓ Pleural effusion Cardiac Catheterization - High atrial pressure - Abnormal ventricular pressure ✓ Diastolic pressure is usually higher within the left ventricle compared t the right ventricle Endo/myocardial biopsy - Fibrosis - May see amyloid deposits depending on the cause Treatment • • • • Medications to slow heart rates - For example, beta blockers etc. Anticoagulation medications Heart transplant Treatment for underlying cause, for example if the condition is because of hemochromatosis then treatment is phlebotomy