Recommendation 95: We recommend right heart failure (RHF) should be considered in patients with unexplained symptoms of exercise intolerance or hypotension in combination with evidence of elevated jugular venous pressure, peripheral edema, hepatomegaly or any combination of these findings. An echocardiogram should be performed to assess cardiac structure and function, and inferior vena cava dispensability. In cases of refractory RHF, or when the diagnosis is not clear, hemodynamic assessment with complete right heart catheterization should be considered (Strong Recommendation, Low Quality Evidence).

Recommendation 96: We recommend that patients with right heart failure (RHF) secondary to or in association with left heart failure (LHF) should be managed as per LHF guidelines (Strong Recommendation, High Quality Evidence).

Recommendation 97: We recommend judicious diuretic therapy for patients with symptomatic RHF, with a goal of euvolemia if feasible and tolerated (Strong Recommendation, Low Quality Evidence).

Recommendation 98: We recommend patients with PH undergo evaluation in centres with experience and expertise in the management of this disorder (Strong Recommendation, Low Quality Evidence).

Recommendation 99: We recommend that right heart catheterization be considered in selected patients with right sided heart failure to determine the true PASP, PVR, TPG, PCWP and to exclude the left sided heart failure as the underlying cause (Strong Recommendation, Low Quality Evidence).

Recommendation 100: We recommend cardiologist referral for patients with any right-sided obstructive cardiac lesion and moderate or severe right-sided regurgitant lesion for assessment of etiology, associated diseases and treatment plan (Strong Recommendation, Low Quality Evidence).

Recommendation 101: We recommend that symptomatic patients with severe right-sided obstructive or severe regurgitant lesions be evaluated and considered for surgical or percutaneous intervention at a center with expertise and experience in the management of these conditions (Strong Recommendation, Low Quality Evidence).

Recommendation 102: We recommend that patients with severe (peak gradient higher than 80 mmHg) or symptomatic moderate (peak gradient 50 mmHg to 79 mmHg) pulmonary valvular stenosis should be referred or considered for balloon valvuloplasty or surgical intervention (Strong Recommendation, Low Quality Evidence).

Recommendation 103: We recommend bioprosthetic rather than metallic prosthesis for replacement of right sided valvular lesions (Strong Recommendation, Low Quality Evidence).

Recommendation 104: We recommend diagnosis of ARVC be made according to the European Society of Cardiology (ESC)/International Society and Federation of Cardiology criteria (revised in 2010) to establish a diagnosis (Strong Recommendation, Low Quality Evidence).

Recommendation 105: We recommend individuals with ARVC avoid strenuous or high-intensity sports activities (Strong Recommendation, Moderate Quality Evidence).

Recommendation 106: We recommend an implantable cardioverter defibrillator (ICD) be offered to all eligible patients with ARVC who have had a cardiac arrest or a history of sustained ventricular tachycardia (Strong Recommendation, Low Quality Evidence).

Recommendation 107: We recommend ICD be considered for the prevention of sudden cardiac death (SCD) in eligible patients with ARVC in whom the risk of SCD is judged to be high (Strong Recommendation, Low Quality Evidence).

Recommendation 108: We recommend all patients with ARVC be referred to a centre with experience and expertise in the management of this condition (Strong Recommendation, Low Quality Evidence).

Recommendation 109: We recommend genetic counselling be considered for families with ARVC for the purpose of screening and/or genetic testing (Strong Recommendation, Low Quality Evidence).

Recommendation 110: We recommend CT scan or CMR be performed in all patients with suspected constrictive pericarditis to assess for pericardial thickening (Strong Recommendation, Low Quality Evidence).

Recommendation 111: We recommend that echocardiography with Doppler assessment of ventricular filling, as well as a right- and left-sided (simultaneous) cardiac catheterization (with manoeuvres if necessary) be performed in all cases of constrictive pericarditis to confirm the presence of a constrictive physiology (Strong Recommendation, Low Quality Evidence).

Recommendation 112: We recommend surgical referral for pericardiectomy be considered for patients with constrictive pericarditis and persistent advanced symptoms despite medical therapy (Strong Recommendation, Moderate Quality Evidence).

Recommendation 113: We recommend that patients with symptomatic constrictive pericarditis be offered referral to a centre with expertise in the management of this condition (Strong Recommendation, Low Quality Evidence).

• Atrial septal defect may be difficult to diagnose and should be suspected in the setting of unexplained RHF or RV enlargement. Bubble study or transesophageal echocardiography may be required for diagnosis.
• Patients with RHF may not have increased left atrial filling pressures and may be more sensitive to change in reduction of cardiac preload and renal dysfunction. This may manifest as light-headedness or elevation of serum creatinine. Careful monitoring of volume status is necessary.
• Patients with RHF may require increased doses of diuretics, which may lead to increased likelihood of hypokalemia. Judicious use of potassium-sparing diuretics may be useful in the maintenance of potassium homeostasis.
• Carefully selected patients with advanced heart failure and severe pulmonary hypertension while on optimal therapy may be considered for therapy with sildenafil for improvement of symptoms and exercise tolerance.
• Ventilation/perfusion lung scanning should be used as a screening test for CTEPH but CT pulmonary angiography or conventional pulmonary angiography will be required for the confirmation for CTEPH diagnosis.
• Pulmonary function testing with diffusion of carbon monoxide should be performed to determine underlying obstructive or interstitial lung disease.
• Lung biopsy may be considered for diagnosis in cases in which the diagnosis is in doubt and will refine treatment.
• Evaluation for lung and heart-lung transplantation should be considered for end-stage cor pulmonale.
• Patients with trivial (mean gradient lower than 25 mmHg) or mild (gradient lower than 50 mmHg) pulmonary stenosis require no intervention or exercise limitation, but should have periodic follow-up (approximately every five years).
• Patients with right-sided valvular stenosis may have underlying carcinoid syndrome or ingestion of appetite suppressants.
• Arrhythmogenic RV cardiomyopathy (ARVC) should be suspected in individuals with unexplained dilation or dysfunction of the right ventricle in whom there is a history of ventricular arrhythmia, syncope or heart failure, or in whom characteristic ECG changes or a positive family history of ARVC is noted.
• Up to 40% of patients with ARVC may have a normal ECG on initial presentation, although almost all patients will develop pathological ECG changes within six years
• Interpretation of CMR for ARVC should be performed at experienced centres. An abnormal scan in isolation is not diagnostic for ARVC.
• Endomyocardial biopsy (EBM) of the RV free wall for ARVC should be performed with extreme caution and at an experienced centre due to the high risk of myocardial perforation and cardiac tamponade.
• Antiarrhythmic drugs or catheter ablation should not be used in the place of ICD therapy for patients with ARVC, but may be considered in patients who refuse or who are not candidates for device therapy.
• TTE is insensitive for detecting pericardial thickening but is a useful first test for examining constrictive physiology; transesophageal echocardiography may further improve the sensitivity over the transthoracic approach.
• When extensive calcification of the pericardium is present, CT may be more effective than CMR for measuring pericardial thickness.
• Provocation testing in the cardiac catheterization laboratory, such as rapid volume loading (e.g., intravenous infusion of 1 L of normal saline over 6 min to 8 min) and simultaneous LV and RV measurement during respiration, may unmask hemodynamic signs of constriction in patients with early or occult forms of constrictive pericarditis.
• The diagnosis of pericardial constriction may be difficult and is made on clinical grounds with supporting information from diagnostic testing. Despite extensive workup, information from EMB or even at open thoracotomy may be required to assist in the diagnosis.