**5.2. Signs and symptoms**

RCM presents with signs and symptoms of both right and left heart failure. Patients complain of exercise intolerance because of diminished cardiac output. Patients often have a low volume pulse, an audible third heart sound, regurgitant murmurs, and a raised JVP with rapid X and Y descent that increases or fails to decrease on inspiration. Low blood pressures are often seen, complicating heart failure management. Pulmonary oedema is uncommon. Syncope occurs occasionally, often exertional, reflecting the limited ability of the heart to increase diastolic filling and is an ominous sign. Syncope may also be aggravated by antihypertensive medications. Concomitant autonomic neuropathy can precipitate orthostatic hypotension as can volume contraction from nephrotic syndrome.

Arrhythmias and conduction disturbances are frequent. Less frequent cardiac manifestations include dynamic LV outflow obstruction, often confused with hypertrophic cardiomyopathy; cardiac ischaemia caused by amyloid deposition in intramural coronary arteries; and intracardiac thrombosis caused by atrial wall standstill, with a risk for systemic embolisation.

The ECG may demonstrate conduction abnormalities. The chest X-ray shows signs of pulmonary congestion and/or pleural effusion, but cardiomegaly is absent. Echocardiographybased two-dimensional and Doppler are essential for determining diastolic dysfunction and for distinguishing patients with RCM from patients with restrictive physiology because of constrictive pericarditis. Echocardiography may also provide information to suggest a specific diagnosis such as the presence of regional wall motion abnormalities in a non-coronary distribution and aneurysms, which would raise the suspicion for cardiac sarcoidosis (CS). Cardiac magnetic resonance (CMR) imaging can aid in the diagnostic process, but the use should be determined on an individual basis. Endomyocardial biopsy (EMB) may be helpful for establishing a diagnosis in some cases. Ultimately, diagnosis of any of the RCMs relies on a constellation of clinical, laboratory, and imaging findings.

#### **5.3. Management**

Medical management: Treatment of RCM includes treating the underlying cause (if identified) and heart failure management. Diuretics are the mainstay of treatment to reduce volume overload. However, volume status in patients with RCM may be challenging to manage, as patients with RCM rely on high filling pressures to maintain cardiac output and excessive diuresis may result in tissue hypoperfusion. Digoxin must be used with great caution because it is potentially dysrhythmogenic in patients with amyloidosis. The use of β-blockers or calcium channel blockers to increase filling time or to manage arrhythmias should be carefully introduced, as some patients may be intolerant. Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers may also be considered, but the proof of benefit is lacking, and these agents may not be well tolerated. Anticoagulation is required in patients with atrial fibrillation, mural thrombus, or evidence for systemic embolisation and may be helpful in most patients because of propensity for thrombus formation in the left atrial appendage.

ventricle. It is a rare type of cardiomyopathy. It occurs if the muscle tissue in the right ventricle dies and is replaced by a scar tissue. This disrupts the pathway of the heart's electrical

Current Perspectives on Cardiomyopathies http://dx.doi.org/10.5772/intechopen.79529 119

ARVC usually affects teenagers or young adults. ARVC has a prevalence of 1 in 5000 among healthy young people. ARVC is seen in up to 20% of all causes of sudden death in young

Histologically, the myocardial cells are replaced by the adipose and fibrous tissues. These alterations can form a re-entry electrical circuit triggering arrhythmias. ARVC usually starts as a localised disease with regional wall abnormalities. As the disease progresses, the right ventricle continues to lose the healthy tissue and dilates and becomes thin walled. Patients can develop right bundle branch block before they finally present with the symptoms of right

Young patients often present with syncope, arrhythmia, cardiac arrest, or sudden death. The

An accurate diagnosis of ARVC is important due to the high risk of drug-resistant arrhythmias and sudden cardiac death. The diagnosis can be established by myocardial biopsy, which shows adipocytes and fibrous tissue. However, these changes can be localised and may not be present at the exact site of biopsy. The availability of cardiac MRI and the gadolinium enhancement techniques are now fundamental in diagnosing ARVC eliminating the need for

The main aim of medical management is to prevent or reduce the risk of fatal arrhythmias.

• Due to the recurrence of arrhythmias and drug resistance, continuous Holter monitoring, or an electrophysiological study may be required. Catheter ablation can be used as a palliative rather than a curative intervention. It is indicated in patients with monomorphic VT due to alocalised ARVC, with a drug-resistant arrhythmia, or with frequent intervention

• ECG showing ventricular tachycardia with left bundle branch block morphology.

• Premature ventricular complexes with left bundle branch block morphology.

people. It is a complex genetic condition due to its genetic variation.

ventricular failure between the fourth and fifth decades of life.

signals leading to arrhythmias.

**6.1. Pathophysiology**

**6.2. Signs and symptoms**

biopsies.

**6.3. Management**

diagnosis of ARVC should be considered in:

• T-wave inversion in leads V1–V3.

• Young male athletes with cardiac symptoms.

• Spontaneous non-sustained ventricular tachycardia.

• Sotalol, verapamil, and amiodarone can be used.

following ICD implantation [7].

Surgical management: No corrective surgery has yet been proposed that would be 100% effective in improving the heart function in RCM. Advanced heart failure therapies, including cardiac transplantation, may be beneficial for selected patients. Heart transplantation is the only effective surgery that can be offered to the patients with restrictive cardiomyopathy. It may be the best option for those who are already symptomatic at the time of diagnosis or in whom reactive pulmonary hypertension exists. Left ventricular assist device (LVAD) therapy may be particularly applicable in patients with RCM as a bridge to transplant or as definitive therapy.
