**6.1. Pathophysiology**

**5.3. Management**

118 Current Topics in Intensive Care Medicine

therapy.

**5.4. Patients posted for non-cardiac surgery**

The overall aims of anaesthesia are:

• sinus rhythm to be maintained if possible;

• to maintain adequate filling pressures;

• to manage electrolyte disturbances;

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.

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

RCM presents a huge challenge for anaesthetists due to the high risk of morbidity and mortality. General anaesthesia causes vasodilation, suppresses the myocardium, and reduces venous return. The latter can be worsened by intermittent positive pressure ventilation resulting in cardiac arrest. Invasive arterial blood pressure monitoring and transesophageal echocardiog-

• to use anaesthetic agents with minimal cardiovascular effect like ketamine or etomidate.

Arrhythmogenic right ventricle cardiomyopathy (ARVC) is characterised by structural abnormalities and cardiac dysfunction of mainly the right ventricle, but it can also involve the left

raphy are useful in identifying the causes of cardiovascular instability [6].

• to maintain SVR in the presence of relatively fixed cardiac output.

**6. Arrhythmogenic right ventricle cardiomyopathy**

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 ventricular failure between the fourth and fifth decades of life.
