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
What is constrictive pericarditis and what are the 3 most common causes?
It is when the pericardium becomes thickened and fibrotic forming a “concrete shell” around the heart making it difficult to relax in diastole, but not changing systolic function.
- previous cardiac surgery
- tuberculous pericarditis- constriction
- idiopathic (maybe viral)
What is the presentation of a patient with constrictive pericarditis?
- insidious onset of abdominal swelling
- peripheral edema
- fatigue and Exertional dyspnea from decreased CO
What are the physical exam findings of someone with constrictive pericarditis?
What is heard on auscultation?
What is seen on a venous pressure curve?
Just like restrictive cardiomyopathy, they have symptoms of RHF:
- increased jugular veins with Kussmaul’s sign
- ascites/ hepatosplenomegaly
- peripheral edema
On auscultation there will be a pericardial knock (higher frequency S3 heard with diaphragm)
On the venous pressure curve, there will be a dip and plateau in diastole with the ventricular pressures being “elevated and equal”
How do you treat patients with mild constrictive pericarditis?
How do you treat patients who have failed medical therapy?
- diuretics
2. pericardiectomy
What are the findings associated with pericarditis on CT and CXR?
What tests are used to assess pericardial thickness?
How can restrictive cardiomyopathy and constrictive pericarditis be differentiated from pulmonary hypertension as the cause of RHF?
CXR- nike check calcification around the heart
CT and MRI assess thickness of the pericardium (calcification is white on CT, black on MRI).
Pulm hypertension will have a dilated RV with decreased systolic function (less contraction). Cons. pericarditis and restrictive cardiomyopathy have maintained ventricle size and systolic function
If you use cardiac cath and measure the RA, RV, PCW, LV filling and they are all elevated and equal, what is you next step to determining if it is pericarditis of restrictive cardiomyopathy?
Which has a better prognosis?
- CT/MRI/echo- is the pericardium thick?
If yes, it is probably pericarditis and you should do pericardial stripping
If no, do endocardium biopsy for amyloid, sarcoid or hemochromatosis. If nothing, do pericardial stripping. If one is present it is probably restrictive cardiomyopathy
Constrictive pericarditis has a better prognosis than restrictive cardiomyopathy.
What is acute pericarditis and what are the four major causes?
It is inflammation of the pericardium with or without pericardial effusion.
- following acute MI
- viral infection (or other infection)
- uremia (renal failure)
- cardiac surgery
How does a patient present with acute pericarditis?
- Retrosternal chest pain that is worse when lying down and when taking deep breaths.
- dyspnea, cough, hoarseness dysphagia (due to compression of bronchi, espophagus, laryngeal nerve)
What is heard on auscultation for acute pericarditis?
What is seen on the EKG?
Pericardial friction rub- audible over entire pericardium that appears and disappears from one hour to the next.
EKG has diffuse ST elevation with PR depression
What 4 tests are routinely ordered for acute pericarditis diagnosis?
Why are they ordered?
These are ordered to try to find the underlying cause of the acute pericarditis to better cater treatment.
- EKG
- chest radiograph
- serum creatinine
- cardiac enzymes
If there is no specific cause found (idiopathic) for acute pericarditis, what is the treatment?
Give aspirin/NSAIDs to decrease inflammation and relieve pain.
The pericardial effusion and inflammation should resolve within days-weeks.
What is pericardial tamponade? What are the 2 mechanisms by which it can form?
It is when pericardial effusion pressure in the pericardial sac affects the function of the heart.
- rapid accumulation of a small volume of fluid
- slower accumulation of a large volume of fluid
This pressure rise decreases diastolic filling and CO starts to be compromised.
What is noted on the pulse of someone with pericardial tamponade?
Pulsus paradoxus because the RV can only fill at the expense of the LV. On inspiration, the right heart fills and the left is underfilled dropping systolic by more than 15mmHg
What are the 2 main changes in a patient with pericarditis that would suggest they have tamponade?
- tachycardia- due to decrease SV and CO and sympathetic reflex
- hypotension - due to decrease SV and CO
What are the 7 signs of pericardial tamponade?
- decreased BP
- increased HR
- increased respiratory rate
- increased JVP
- pulsus paradoxus
- faint heart sounds
- clear lungs
How is pulsus paradoxus measured?
- Inflate BP cuff to above systolic pressure
- deflate to first heart sound (systolic)
- seal off cuff and listen to a few rounds of respiration. The sound will come and go bc there is an inspirational drop of 15mmHg.
- deflate to a level where the Karatkoff sounds do NOT disappear in the respiration cycle.
Subtract the second pressure from the first. if the difference is >10 it is pulsus paradoxus
What does the EKG reveal for acute pericarditis with pericardial tamponade?
- diffuse ST elevation (PR depression)
- low voltage (small QRS)
- electrical alternans (QRS has different heights beat to beat because heart is swinging in fluid)
What does the echocardiogram show for acute pericarditis with cardiac tamponade?
RV diastolic collapse with equalizing of diastolic pressures in RA and RV
What is treatment for cardiac tamponade?
- volume expansion (because of the decreased CO and hypotension)
- pericardial drainage to get the fluid off the heart using pericardiocentesis by needle or window to drain into pleural space
