Now I will explain about differential diagnosis

Restrictive cardiomyopathy must be distinguished from other pathologic conditions that cause an
increase in wall thickness because of hypertrophy.9 There are some disease as a differential
diagnosis such as acute complications of sarcoidosis, acute pericarditis, aortic stenosis, cardiac
tamponade, constrictive pericarditis, heart failure, hypertensive heart disease, hypertrophic
cardiomyopathy, and imaging in dilated cardiomyopathy

The diasease that most often resemble to restrictive cardiomyopathy is constrictive pericarditis.
Constrictive pericarditis is the result of scarring and consequent loss of the normal elasticity of
the pericardial sac. This leads to impairment of ventricular filling in mid and late diastole.

Restrictive cardiomyopathy as explained before is characterized by a nondilated rigid ventricle,

resulting in severe diastolic dysfunction and restrictive ventricular filling that produces
hemodynamic changes similar to constrictive pericarditis

But there are some differences to distinguish them as shown in this table. We may take some
example such as the atrial enlargement that usually occurred in RC but slighy or moderate in
most case of CP. In MR/CT imaging shows in cp there are thick pericardium and in rm usually
normal. And in endomyocardial byopsi in rm usually show amyloid or there specific infiltratf
disease and in cp usually no specific abnormality

Now we continue to managemnt, so the principal that definitive treatment is based on underlying
disease. Cardiac amyloid is often treated with chemotherapy. Endomyocardial fibrosis and
eosinophilic cardiomyopathy are commonly treated with cytotoxic drugs and glucocorticoids.
Phlebotomy is often helpful in patients with hemochromatosis

These therapies directed at individual causes of restrictive cardiomyopathy have been proven to
be effective.10 However, in most pediatric cases suggesting that the cause is individual genetic
mutations that the currently there are no therapies that can cure this patient. 8 . Heart transplant
is only effective surgery to the pediatric case that offers patient the chance to return to a normal
lifestyle, also perform in pasient with amyloidosis in refracter symptom. When heart transplant
is choosen there are should considere about risk long term complication, as concerns arise, such
as infection, organ rejection, coronary artery disease, and the side effects of medications. This is
the schematic in heart transplantation prosedure

If the transplatiation or definitive therapy cannot perform in some situation. The mainstay
treatments to improve symptoms with restrictive cardiomyopathy. We can given diuretic such as
furosemide and hydrochlorothiazide reduce pulmonary and systemic congestion. Beta bloker
such as carvedilol, metaprolol, propanolo, and atenolol to slow the heartbeat and increase the
relaxation time of the heart and anticoagulants such as aspririn and warfarin are used to prevent
embolism from ventricular thrombus

In prognosis for restrictive cardiomyopathy are varies according to the etiology. In amylodiosis
usually respond to medical or surgical treatment, but in children usually poor prognosis.
Children after few years of diagnosis it reported that 66-100% will die or undergoing cardiac
transplant. And in adult after 5 years diagnosis reported that 32-44% suffers a cardiovascular-
related death

The summary

Restrictive cardiomyopathy is the least common of cardiomyopathy that characterized by

increasing stiffness of ventricular. It usually associate to inflammatory disease, storage diseas
and infiltrative disease. Patient will manifest dispnea, fatigue, dcrese execercise tolerant,
palpitation followed by dizziness. The diagnosis can be established with anamnesis, physical
examination, cardiac diagnosis testing and endomyocardial biopsy. The therapy base on
underlying disease and the prognosis usually poor specially in children

Okey, thats all of our group presentation, thank you for your attention

Is there any question?

Beta blockers and cardioselective calcium channel blockers (eg, verapamil, diltiazem) may be of
benefit, by increasing left ventricular filling time, improving ventricular relaxation, and
decreasing compensatory sympathetic stimulation. In addition, low-medium dose diuretics lower
preload and may provide symptomatic relief. Small initial doses should be administered to
prevent hypotension because patients are frequently extremely sensitive to alterations in left
ventricular volume. Higher doses may be needed if the serum albumin level is low secondary to
concomitant nephrotic syndrome.

ACEIs and angiotensin II inhibitors are poorly tolerated in patients with amyloidosis. Even small
doses may result in profound hypotension, probably secondary to an autonomic neuropathy.
Beta-blockers and calcium channel blockers have not been shown to improve day-to-day
symptoms or to favorably alter the natural history in patients with diastolic heart failure. No
published data are available on the use of intravenous (IV) inotropic or vasodilator drugs.

Patients with a history of atrial fibrillation should be anticoagulated. In patients with

atrial fibrillation, the rate should be controlled adequately. While a donor heart can cure the
symptoms of heart failure and greatly improve survival, it is a major
operation with considerable risks and long-term complications.