Restrictive Cardiomyopathy and Pericardial Diseases Flashcards
What are the clinical manifestation of left heart failure?
(include heart sounds and pulse changes)
- pulmonary venous congestion- dyspnea with exertion, orthopnea, paroxysmal nocturnal dyspnea
- fast breathing
- pulmonary rales
- S3 (increased filling pressure) or S4 (atrial squeeze due to increased pressure)
- pulsus alternans or thread pulse
- hypotension
What are the clinical manifestations of right heart failure?
- central venous congestion- elevated jugular veins
- weight gain and abdominal swelling
- leg edema
- hepatosplenomegaly
- pleural effusion
What is the hepatojugular reflex?
If you push on the liver in someone with RHF, you will see an increase in neck vein distention
What are characteristics of low output state?
- fatigue
- dyspnea, tachycardia
- decreased pulse pressure
What heart failure is usually associated with restrictive cardiomyopathy?
Is it associated with systolic or diastolic dysfunction?
RHF and low output state
It is associated with diastolic dysfunction (decreased filling due to stiff vessels) and normal systolic function (until very late stages of the disease)
What is restrictive cardiomyopathy?
How does it differ from dilated cardiomyopathy and hypertrophic cardiomyopathy?
Disease processes (amyloidosis, hemochromatosis, sarcoidosis) infiltrate the myocardium leading to rigidity in the RV and LV and impairing diastolic function (filling).
It differs from dilated and hypertrophic because it has normal wall thickness and normal systolic function.
What are the 3 most common causes of restrictive cardiomyopathy? which 2 can also cause dilated cardiomyopathy?
- amyloidosis
- sarcoidosis
- hemochromatosis
Amyloid and sarcoidosis both can also cause dilated
In addition to RHF symptoms, what other symptoms might a person with restrictive cardiomyopathy present with?
The infiltrative process can disrupt the cardiac conduction system so they may present with A fib or heart block
What is Kussmaul’s sign?
Usually JVP decreases with inspiration because inspiration reduces intrathoracic pressure and increases return to RV. Kussmaul’s sign is an increase in JVP with inspiration due to restrictive cardiomyopathy (inability to increase return to the heart due to stiff ventricles/ high pressure atria)
What is the course and prognosis of someone with restrictive cardiomyopathy?
Most patients die w/in one to two years of diagnosis as a result of: 1. RHF 2. decreased CO 3. ventricular arrhythmia 4 Heart block
Patients with amyloidosis are extremely sensitive to what drug?
Digoxin binds amyloid myofibrils in the heart so patients with amyloidosis can develop toxicity at really low concentrations
What does restrictive cardiomyopathy show on:
- CXR
- EKG
CXR- normal sized heart, pleural effusion (not pulm venous congestion!!)
EKG- diffuse low voltage (QRS less than 5mm in limb leads) with AV and rhythm disturbances
What does an echo of restrictive cardiomyopathy show?
- normal sized ventricles with normal systolic function
- enlarged atria (potential intracardiac thrombi)
- ground-glass amyloid deposition
What do pressure tracings with cardiac cath reveal in restrictive cardiomyopathy?
While this is diagnostic, what followup might you want to do?
- atrial pressures are elevated and equal
- ventricle pressure is a dip and plateau-rapid early decline at beginning of diastole followed by rapid rise and plateau
Endomyocardial biopsy to see the underlying cause of the restrictive cardiomyopathy.
What are the normal systolic/diastolic pressures of:
1. RA
2. RV
3. LA
4. LV
What would you see in restrictive cardiomyopathy?
- 7/2———–30/24
- 24/4———40/24
- 13/3———-34/24
- 130/7 ——-100/24
All diastolic pressures are elevated and equal