Version May 2024
INTRODUCTION — Right heart failure (RHF) is a clinical syndrome in which symptoms and signs are caused by dysfunction of the right heart structures (predominantly the right ventricle [RV], but also the tricuspid valve apparatus and right atrium) or impaired vena cava flow, resulting in impaired ability of the right heart to perfuse the lungs at normal central venous pressures [1-3]. The terms RHF and RV dysfunction are not synonymous, as some patients have asymptomatic RV dysfunction, and not all RHF is caused by RV dysfunction. Many specific cardiovascular disorders lead to the clinical syndrome of RHF through a variety of mechanisms.
This topic will address evaluation of the cause and management of RHF. The clinical manifestations, diagnosis, and pathophysiology of RHF are discussed separately. (See "Right heart failure: Clinical manifestations and diagnosis".)
Other aspects of HF are discussed separately including the diagnosis and evaluation of HF, management of HF with reduced ejection fraction, and management of HF with preserved ejection fraction. (See "Heart failure: Clinical manifestations and diagnosis in adults" and "Determining the etiology and severity of heart failure or cardiomyopathy" and "Overview of the management of heart failure with reduced ejection fraction in adults" and "Heart failure with preserved ejection fraction: Clinical manifestations and diagnosis" and "Treatment and prognosis of heart failure with preserved ejection fraction".)
EVALUATING THE CAUSE OF RIGHT HEART FAILURE
General approach — The process of evaluating the cause of RHF overlaps the process of diagnosing RHF, starting with assessment of clinical manifestations and diagnostic testing that not only supports diagnosis of RHF but also provides information about the cause of RHF, with further evaluation as needed to identify the cause. (See "Right heart failure: Clinical manifestations and diagnosis", section on 'Clinical manifestations' and "Right heart failure: Clinical manifestations and diagnosis", section on 'Diagnosis'.)
Evaluation based upon presentation — Since patients with RHF commonly have an antecedent condition causing or predisposing to RHF, the patient's clinical presentation (eg, medical history, symptoms, signs, initial tests, and echocardiography) often strongly suggests the cause of RHF. Evaluation of each of the following conditions is discussed further in the linked topics. (See "Right heart failure: Clinical manifestations and diagnosis", section on 'Common clinical settings'.)
Acute right heart failure — Causes of acute RHF include the following conditions. These are generally distinguished by clinical presentation and initial testing, including echocardiography, with further testing such as cardiac catheterization in selected patients.
●Causes of acute dyspnea and pulmonary hypertension (PH).
•Pulmonary embolism commonly presents with acute onset dyspnea, pleuritic chest pain, and cough, but symptoms may be nonspecific or absent. The diagnostic approach varies depending on the patient's hemodynamic stability and pretest probability. (See "Clinical presentation, evaluation, and diagnosis of the nonpregnant adult with suspected acute pulmonary embolism" and "Epidemiology and pathogenesis of acute pulmonary embolism in adults".)
•Adult respiratory distress syndrome (ARDS) [4] typically presents with progressive dyspnea, increasing oxygen requirement, and alveolar infiltrates on chest imaging within several hours to days following an inciting event (table 1). ARDS is diagnosed after cardiogenic pulmonary edema and other causes of acute hypoxemic respiratory failure and bilateral infiltrates have been excluded. (See "Acute respiratory distress syndrome: Clinical features, diagnosis, and complications in adults", section on 'Diagnosis'.)
●Causes of new onset dyspnea with primary RV dysfunction – While RV systolic dysfunction is seen with most patients with RHF, in these patients, RV systolic dysfunction is a dominant feature.
•RV myocardial infarction (RVMI) presents with acute symptoms of MI including chest pain, nausea, vomiting, and diaphoresis. The presentation of MI with hypotension, jugular venous distention, and clear lung fields is specific but not sensitive for RVMI. RVMI should be carefully distinguished from acute pulmonary embolus and other causes of similar clinical features. Electrocardiographic findings and troponin levels may suggest RVMI, and echocardiography is generally helpful in identifying RV involvement. Cardiac catheterization with coronary arteriography may be performed, particularly if primary percutaneous coronary intervention is feasible. (See "Right ventricular myocardial infarction", section on 'Diagnosis'.)
•Myocarditis presents variably with a spectrum of subacute or acute symptoms ranging from none to chest pain, fatigue, HF, and cardiogenic shock. Criteria have been developed to identify clinically suspected myocarditis, but a definitive diagnosis is generally made by biopsy (eg, endomyocardial and for some conditions, other affected tissue). Myocarditis may cause RHF through direct involvement of the RV and/or through involvement of the left ventricle (LV) and development of PH, which can cause RV pressure overload. (See "Clinical manifestations and diagnosis of myocarditis in adults" and "Clinical manifestations and diagnosis of cardiac sarcoidosis", section on 'Diagnosis'.)
●Precipitation of RHF in the setting of increased venous return [5,6].
•High-output HF should be suspected in patients with HF but well-perfused extremities, particularly those with a condition commonly associated with a high-output state (eg, presence of an arteriovenous fistula for dialysis access [7]). The diagnosis is confirmed by evaluation of hemodynamics by right heart catheterization. Patients with high-output HF frequently also have LV and/or RV systolic dysfunction, as seen in sepsis. (See "Causes and pathophysiology of high-output heart failure" and "Clinical manifestations, diagnosis, and management of high-output heart failure" and "Sepsis syndromes in adults: Epidemiology, definitions, clinical presentation, diagnosis, and prognosis" and "Evaluation and management of suspected sepsis and septic shock in adults".)
•LV assist device (LVAD) implantation may precipitate early or late (after initial hospital discharge) RHF. Following LVAD implantation, there is an increase in systemic venous return to the right heart; also, decreased LV pressure and chamber size after LVAD implantation cause interventricular septal bowing, which may worsen RV mechanics with decrease in RV stroke volume and increase in tricuspid regurgitation. Evaluation includes right heart catheterization and transthoracic echocardiography. Predictors of RHF after LVAD implantation include greater elevation in central venous pressure to pulmonary capillary wedge pressure (>0.63), reduced pulmonary artery pulsatility index, and decreased RV stroke work index [1]. (See "Management of long-term mechanical circulatory support devices", section on 'Right heart failure'.)
●Subacute or acute onset of dyspnea, often in the setting of a cause of pericardial effusion or hematoma (eg, viral syndrome, post-thoracic surgery).
•Cardiac tamponade may have a subacute or acute presentation, depending on speed of pericardial fluid accumulation and the clinical setting, with symptoms ranging from fatigue and dyspnea to shock. All causes of pericardial effusion may cause tamponade. The clinical diagnosis is generally based upon clinical evaluation, including echocardiography, and confirmed by the clinical and hemodynamic response to pericardial fluid drainage. Cardiac catheterization may be performed to confirm the presence of hemodynamic features consistent with tamponade. (See "Cardiac tamponade".)
Chronic right heart failure — The following cardiovascular disorders are causes of chronic RHF [8-12]. These are generally distinguished largely by clinical presentation and initial testing, including echocardiography.
●Chronic PH – PH in all etiologic groups (table 2) can cause RHF. The symptoms and signs of PH are nonspecific and include dyspnea and fatigue, exertional chest pain, exertional syncope, peripheral edema, anorexia and early satiety, and abdominal discomfort and swelling (table 3). Evaluation of suspected PH includes transthoracic echocardiography, which may provide an estimate of pulmonary artery systolic pressure and guide further evaluation, as discussed separately. Among patients with RHF of uncertain cause, echocardiographic findings suggestive of PH and RV dysfunction (eg, elevated left atrial pressures or pulmonary vascular disease) are the most common indications for cardiac catheterization (table 3). Identification of the cause of PH is important since this guides management. (See "Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults" and 'Specific therapy' below.)
Some of the more common causes are highlighted here:
•PH due to left heart disease (group 2) – PH in patients with left HF is due to both passive backward transmission of left atrial hypertension, as well as pulmonary vasoconstriction and vascular remodeling that opposes blood flow through the lungs [13]. Diagnostic evaluation includes transthoracic echocardiography, which helps determine whether right heart catheterization is indicated. (See "Pulmonary hypertension due to left heart disease (group 2 pulmonary hypertension) in adults", section on 'Diagnosis and evaluation'.)
Common causes of PH due to left heart disease include the following conditions (see "Pulmonary hypertension due to left heart disease (group 2 pulmonary hypertension) in adults"):
-HF with reduced ejection fraction (HFrEF) and HF with mid-range ejection fraction (HFmrEF) – In patients with HF, a diagnosis of HFrEF is generally confirmed by identification of an LV ejection fraction (LVEF) ≤40 percent (most commonly by echocardiography); a diagnosis of HFmrEF is generally confirmed by identification of an LVEF of 41 to 49 percent. HFrEF (and HFmrEF) may be caused by nonischemic (eg, dilated cardiomyopathy, myocarditis) or ischemic heart disease. (See "Heart failure: Clinical manifestations and diagnosis in adults" and "Determining the etiology and severity of heart failure or cardiomyopathy".)
-HF with preserved ejection fraction (HFpEF) – In patients with HF with preserved ejection fraction (HFpEF), the LVEF is (by definition) ≥50 percent, which may make diagnosis more challenging [14]. Physical examination findings, echocardiographic values, and natriuretic peptide levels are generally more abnormal in patients with HFpEF and RHF as compared with patients with HFpEF and no RHF [2,15]. The approach to diagnosis of HFpEF is discussed separately. (See "Heart failure with preserved ejection fraction: Clinical manifestations and diagnosis", section on 'Evaluation'.)
Some patients present with RHF with no clear evidence of provocative factors, and many of these patients have RHF due to previously undiagnosed HFpEF. In a longitudinal study of patients who presented initially with HFpEF and isolated left heart involvement, the incidence of new-onset RV dysfunction was 23 percent over just four years [16]. Over sustained periods of time, ventricular dilation and deterioration in systolic function are much greater in the RV than the LV in HFpEF, and RHF may be an indicator of the duration of left-sided HF [16].
-HF due to left-sided valve disease (mitral or aortic; stenosis or regurgitation). (See "Clinical manifestations and diagnosis of chronic mitral regurgitation" and "Rheumatic mitral stenosis: Clinical manifestations and diagnosis" and "Clinical manifestations and diagnosis of chronic aortic regurgitation in adults" and "Clinical manifestations and diagnosis of aortic stenosis in adults".)
•Pulmonary arterial hypertension (group 1). Evaluation is discussed separately. (See "Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults" and "The epidemiology and pathogenesis of pulmonary arterial hypertension (Group 1)".)
•PH due to lung disease and/or hypoxia (group 3), including the following causes (see "Pulmonary hypertension due to left heart disease (group 2 pulmonary hypertension) in adults" and "Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Treatment and prognosis"):
-Obstructive sleep apnea. (See "Clinical presentation and diagnosis of obstructive sleep apnea in adults".)
-Obstructive and/or restrictive lung disease. (See "Chronic obstructive pulmonary disease: Diagnosis and staging" and "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Chest wall diseases and restrictive physiology".)
●Congenital heart disease – Congenital heart lesions that cause RHF are generally identified by clinical evaluation and echocardiography and include lesions causing RV volume overload, conditions causing RV pressure overload (due to PH and/or RV outflow obstruction), congenital causes of right-sided valve disease, and conditions associated with primary RV dysfunction or absence (including patients with anatomic or functional single ventricle treated with a Fontan procedure) [17].
•Congenital lesions causing RV volume overload include atrial septal defects, tricuspid regurgitation, or pulmonic regurgitation. (See "Clinical manifestations and diagnosis of atrial septal defects in adults" and "Ebstein anomaly: Clinical manifestations and diagnosis" and "Pulmonic regurgitation".)
•PH in patients with congenital heart disease is predominantly group 1 and less commonly group 2; groups 3, 4, and 5 also occur. Group 5 includes some complex congenital heart disease. Various types of intracardiac shunts (eg, ventricular septal defects, atrial septal defects) and aortic shunts (eg, patent ductus arteriosus) increase pulmonary blood flow, leading to progressive pulmonary vascular disease and group 1 PH, with some cases progressing to Eisenmenger syndrome. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis".)
•Right ventricular outflow obstruction is a component of tetralogy of Fallot, and some patients have residual RV outflow obstruction after surgical repair. (See "Tetralogy of Fallot (TOF): Pathophysiology, clinical features, and diagnosis", section on 'Right ventricular outflow tract obstruction' and "Tetralogy of Fallot (TOF): Long-term complications and follow-up after repair", section on 'Residual RVOT obstruction'.)
•In Fontan patients, there is no ventricular pump to push blood into the pulmonary arterial circulation, so systemic venous pressures are chronically elevated. Even small elevations in pulmonary vascular resistance in these patients substantially compromise blood flow and lead to increased risk of RHF due to the absence of a subpulmonary ventricle [18]. (See "Management of complications in patients with Fontan circulation", section on 'Heart failure'.)
●Right-sided valve disease – Right-sided valve (tricuspid or pulmonic) diseases (regurgitation or stenosis) are generally identified and characterized by physical examination and echocardiography. These valve lesions may be congenital (eg, Ebstein anomaly with tricuspid regurgitation) or acquired (eg, right-sided valve dysfunction caused by endocarditis). Patients with atrial fibrillation frequently develop tricuspid regurgitation related to dilation of the tricuspid annulus and tricuspid regurgitation, causing RHF related to volume overload even in the absence of an elevation in pulmonary artery pressure [8,9,16,19].
●Cardiomyopathies – Cardiomyopathies involving the RV and/or LV may cause RHF, including dilated and restrictive cardiomyopathies, as well as arrhythmogenic right ventricular cardiomyopathy [20]. Echocardiography and other cardiac imaging (particularly cardiovascular magnetic resonance imaging) are used to identify and characterize these disorders. (See "Definition and classification of the cardiomyopathies" and "Restrictive cardiomyopathies" and "Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations" and "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics" and "Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis".)
●Constrictive pericarditis should be suspected in patients with RHF with prior history of a potential cause of pericardial constriction (eg, prior thoracic radiation exposure or prior cardiac surgery) and is generally diagnosed by echocardiography with selective use of additional cross-sectional imaging (computed tomography or magnetic resonance imaging). Combined left and right heart catheterization is the clinical gold standard for distinguishing pericardial disease from cardiac causes of RHF (such as restrictive cardiomyopathy). Testing to distinguish these conditions is discussed further separately. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Initial tests' and "Differentiating constrictive pericarditis and restrictive cardiomyopathy".)
MANAGEMENT
General management — General management includes maintaining oxygen delivery to the tissues, monitoring optimizing volume status, hemodynamic support, arrhythmia management, advanced therapies for refractory HF, palliative care, and activity restriction. (See "Treatment of acute decompensated heart failure: General considerations" and "Treatment of acute decompensated heart failure: Specific therapies" and "Overview of the management of heart failure with reduced ejection fraction in adults" and "Treatment and prognosis of heart failure with preserved ejection fraction".)
Supplemental oxygen and ventilatory support — Supplemental oxygen is used as needed to treat hypoxemia (SpO2 <90 percent) with assisted ventilation if needed. In addition to supporting O2 delivery to the body, this intervention may also help to reduce hypoxic vasoconstriction in the lungs that contributes to pulmonary hypertension (PH). (See "Treatment of acute decompensated heart failure: Specific therapies", section on 'Supplemental oxygen and assisted ventilation'.)
Monitoring and sodium and fluid restriction — Measures for acute HF care as well as outpatient HF self-management include daily monitoring of symptoms, signs, and weight. General guidelines for sodium restriction (eg, <3 g/day) and fluid restriction (eg, 1.5 to 2 L/day) apply, although evidence for specific thresholds are limited. (See "Treatment of acute decompensated heart failure: General considerations", section on 'Monitoring' and "Treatment of acute decompensated heart failure: Specific therapies", section on 'Sodium and fluid restriction' and "Heart failure self-management".)
Optimization of volume status
●Volume overload is generally treated with diuretic therapy. Ultrafiltration is an option for patients with refractory volume overload. (See "Use of diuretics in patients with heart failure".)
•Volume overload in patients with acute or chronic RHF due to left HF or RV volume overload due to tricuspid or pulmonic regurgitation are treated with diuretic therapy. In patients with RHF due to left HF, diuretics are a mainstay of therapy to reduce right and left heart filling pressures. Treating left HF will reduce RV afterload by decreasing pulmonary artery pressures [21] and also by increasing pulmonary artery compliance [22]. Patients with RHF frequently have concurrent acute and chronic kidney disease (including cardiorenal syndrome) and often require very high doses of diuretics, typically administered intravenously because gut edema limits oral absorption [23-26]. (See "Use of diuretics in patients with heart failure" and "Pulmonary hypertension due to left heart disease (group 2 pulmonary hypertension) in adults", section on 'Optimized management of left heart disease' and "Cardiorenal syndrome: Prognosis and treatment".)
•Diuretic therapy is used to treat volume overload in selected patients with RV pressure overload (eg, patients with group 1 pulmonary arterial hypertension or tricuspid stenosis), but volume depletion should be avoided given the risk of hemodynamic decompensation. (See "Treatment and prognosis of pulmonary arterial hypertension in adults (group 1)", section on 'Diuretics (treatment of chronic right heart failure)' and "Tricuspid stenosis", section on 'Medical management'.)
●Avoidance of volume depletion is particularly important in the following clinical settings: pulmonary embolism, cardiac tamponade, RVMI, and PH associated with congenital heart disease. (See "Treatment, prognosis, and follow-up of acute pulmonary embolism in adults", section on 'Hemodynamic support' and "Right ventricular myocardial infarction", section on 'Optimization of right ventricular preload' and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis", section on 'Volume depletion or systemic vasodilation' and "Cardiac tamponade", section on 'Supportive care'.)
●Volume repletion – When volume repletion is indicated, this is generally performed by carefully monitored intravenous fluid challenges (eg, aliquots of 200 to 300 mL of normal saline over five minutes).
•Patients with acute pulmonary embolism with hypotension or other signs of low cardiac output (hypoperfusion) are generally treated with volume repletion (generally limited to a total of no more than 500 to 1000 mL of normal saline), which is followed by vasopressor therapy if hypoperfusion persists. (See "Treatment, prognosis, and follow-up of acute pulmonary embolism in adults", section on 'Hemodynamic support'.)
•For patients with cardiac tamponade with hypotension or other signs of low cardiac output, volume repletion is a temporizing measure until pericardial fluid drainage is performed. (See "Cardiac tamponade", section on 'Supportive care'.)
•For patients with RVMI with evidence of low cardiac output, no pulmonary congestion, and low or normal jugular venous pressure, volume repletion is performed to enhance RV preload. (See "Right ventricular myocardial infarction", section on 'Optimization of right ventricular preload'.)
Arrhythmia management — Complications in patients with RHF may include conduction system disease and arrhythmias. Management of these conditions and the risk of sudden death are based upon general treatment principles along with specific approaches for certain causes of RHF, as discussed in individual topic reviews. (See "Overview of the management of heart failure with reduced ejection fraction in adults", section on 'Arrhythmias and conduction system disease' and "Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis" and "Management and prognosis of cardiac sarcoidosis" and "Management and prognosis of cardiac sarcoidosis", section on 'Management of arrhythmias and conduction system disease' and "Treatment and prognosis of heart failure with preserved ejection fraction", section on 'Atrial fibrillation'.)
Activity restriction — Patients with symptomatic HF, active myocarditis, pericardial effusion, or constrictive pericarditis are generally restricted from competitive sports [27]. Specific recommendations apply to certain types of cardiomyopathy, including arrhythmogenic right ventricular cardiomyopathy and myocarditis. (See "Athletes: Overview of sudden cardiac death risk and sport participation".)
Specific therapy — Patients with certain causes of RHF are treated with the appropriate specific therapies, in addition to the above general measures.
For acute right heart failure
●Management of acute pulmonary embolism with RHF includes treatment to address thromboembolism (anticoagulation for stable patients; reperfusion by thrombolysis or thrombectomy for unstable patients) in addition to the supportive care described above. (See "Treatment, prognosis, and follow-up of acute pulmonary embolism in adults".)
●RVMI is treated with reperfusion (primary percutaneous coronary intervention or fibrinolysis) and other therapy for MI (in addition to measures to optimize volume status and provide hemodynamic support, as described above). (See "Right ventricular myocardial infarction", section on 'Treatment'.)
●Certain forms of myocarditis that may cause acute RHF (eg, giant cell myocarditis and necrotizing eosinophilic myocarditis) are treated with immunosuppressive therapy, as discussed separately. (See "Treatment and prognosis of myocarditis in adults" and "Treatment and prognosis of myocarditis in adults", section on 'Giant cell myocarditis' and "Treatment and prognosis of myocarditis in adults", section on 'Eosinophilic myocarditis'.)
●Cardiac tamponade is treated by removal of pericardial fluid, either percutaneously (pericardiocentesis) or surgically. (See "Pericardial effusion: Approach to management".)
●In patients with RHF following LV assist device (LVAD) placement, LVAD settings are adjusted to minimize interventricular septal bowing on echocardiography. Additional measures may be required, including inotropic support, RV mechanical support, or extracorporeal membrane oxygenation. (See "Management of long-term mechanical circulatory support devices", section on 'Right heart failure'.)
●Superior or inferior vena cava obstruction may present with acute or chronic symptoms. Management of these conditions is discussed separately. (See "Malignancy-related superior vena cava syndrome", section on 'Treatment' and "Overview of iliocaval venous obstruction", section on 'Management'.)
For chronic right heart failure
●Patients with RHF and HF with reduced ejection fraction (HFrEF) or HF with mid-range ejection fraction should be treated with standard guideline-directed pharmacologic and device therapy as tolerated [28]. (See "Overview of the management of heart failure with reduced ejection fraction in adults" and "Primary pharmacologic therapy for heart failure with reduced ejection fraction" and "Secondary pharmacologic therapy for heart failure with reduced ejection fraction".)
●Pulmonary vasodilator therapies are used to treat patients with RHF due to pulmonary arterial hypertension group 1 (table 2); selected patients with PH groups 2, 3, or 4 may be candidates for some of these agents, as discussed separately. (See "Treatment of pulmonary arterial hypertension (group 1) in adults: Pulmonary hypertension-specific therapy" and "Pulmonary hypertension due to left heart disease (group 2 pulmonary hypertension) in adults", section on 'Targeted therapy for pulmonary hypertension' and "Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Treatment and prognosis", section on 'Our approach' and "Chronic thromboembolic pulmonary hypertension: Pulmonary hypertension-specific therapy" and "Management of complications in patients with Fontan circulation" and "Management of complications in patients with Fontan circulation", section on 'Pulmonary vasodilator therapy'.)
●Certain forms of myocarditis that may cause chronic RHF (eg, cardiac sarcoidosis) are treated with immunosuppressive therapy. (See "Management and prognosis of cardiac sarcoidosis", section on 'Immunosuppressive therapies' and "Treatment and prognosis of myocarditis in adults", section on 'Management of specific disorders'.)
●The management of arrhythmogenic RV cardiomyopathy includes measures to prevent sudden cardiac death, treat symptomatic arrhythmias, and reduce the risk of progression. (See "Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis".)
●Patients with chronic thromboembolic PH are treated with anticoagulant therapy and should be referred for evaluation for pulmonary thromboendarterectomy. (See "Chronic thromboembolic pulmonary hypertension: Initial management and evaluation for pulmonary artery thromboendarterectomy".)
●Specific interventions (eg, surgery or percutaneous techniques) are indicated for patients with congenital heart disease; an example is atrial septal defect closure in patients with a hemodynamically significant left-to-right shunt without significant PH. Valve surgery is indicated for patients with symptomatic severe right- or left-heart valve dysfunction (regurgitation or stenosis), as discussed in individual topic reviews. (See "Management and prognosis of tricuspid regurgitation", section on 'Tricuspid valve surgery' and "Pulmonic valve stenosis in adults: Management", section on 'Indications for intervention' and "Tricuspid stenosis", section on 'Indications for intervention' and "Pulmonic regurgitation", section on 'Intervention'.)
●Early stage (subacute) hemodynamically stable constrictive pericarditis may respond to a trial of antiinflammatory therapy (a nonsteroidal antiinflammatory agent plus colchicine), but refractory patients and those with late-stage constrictive pericarditis should be referred for surgical pericardiectomy. (See "Constrictive pericarditis: Management and prognosis", section on 'Management of constrictive pericarditis'.)
Management of refractory heart failure — Management of refractory RHF depends on the clinical presentation and cause.
Approach to refractory volume overload — Patients with refractory volume overload despite initial diuretic therapy are treated with uptitration of diuretic dose and diuretic combinations as discussed separately. If these measures are unsuccessful, hemodialysis or hemofiltration can be used for fluid removal. (See "Causes and treatment of refractory edema in adults" and "Causes and treatment of refractory edema in adults", section on 'Management of refractory edema'.)
Approach to low cardiac output — Patients with evidence of low cardiac output with hypoperfusion caused by RV and/or LV systolic dysfunction are treated with intravenous inotropes as a temporizing measure, while treatment for specific causes is provided (see 'Specific therapy' above). As an example, patients with acute MI complicated by acute RV systolic dysfunction may be treated with an inotrope, vasopressor therapy, and/or fluid resuscitation until revascularization and RV recovery occur. (See "Right ventricular myocardial infarction", section on 'Inotropic drugs'.)
For patients with acute RHF with HFrEF, intravenous systemic vasodilators may help improve forward flow [29,30]. The use of pulmonary vasodilator therapy for selected patients with PH is discussed elsewhere in this topic. (See "Treatment of acute decompensated heart failure: Specific therapies", section on 'Vasodilator therapy' and 'For chronic right heart failure' above.)
For patients with isolated RHF, a role for digoxin therapy has not been established, as evidence in this setting is scant [1,31]. A systematic review included four small randomized controlled trials with a total of 76 patients with RHF due to cor pulmonale [32]. The review found no association between digoxin therapy and improvement in New York Heart Association (NYHA) functional class, exercise capacity, or RV ejection fraction. In contrast, digoxin is an established component of therapy in selected patients with HFrEF (including those with RHF) and persistent symptoms while on optimized guideline-directed medical therapy. (See 'Specific therapy' above and "Secondary pharmacologic therapy for heart failure with reduced ejection fraction".)
Mechanical circulatory support is indicated for patients with severe RHF due to LV or RV systolic dysfunction with refractory HF [4,33]. (See "Treatment of advanced heart failure with a durable mechanical circulatory support device", section on 'Device types for common patient groups'.)
Heart or combined heart-lung transplantation is required in some cases of refractory HF due to LV and/or RV dysfunction. (See "Heart transplantation in adults: Indications and contraindications", section on 'Indications for transplantation'.)
Role of palliative care — Supportive primary palliative care is an important component of care for patients with RHF to address symptom control, goals of care, shared decision-making, and execution of advanced care directives. Secondary palliative care is indicated for patients with persistent NYHA functional class IV, with significant comorbidities or progressive frailty, facing decision-making on major interventions (such as mechanical circulatory support), or with preferences shifting the emphasis of care to quality of life. (See "Palliative care for patients with advanced heart failure: Indications and systems of care" and "Palliative care for patients with advanced heart failure: Decision support and management of symptoms".)
PROGNOSIS — Outcomes are highly variable for RHF and depend on the underlying etiology. In general, the presence of RHF or RV dysfunction is among the strongest predictors for adverse outcomes for patients with HF with reduced ejection fraction, HF with preserved ejection fraction, or non-HF-related pulmonary hypertension [8,9,11,34]. Further details on prognosis can be found in the individual topics for specific causes of RHF.
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Heart failure in adults".)
SUMMARY AND RECOMMENDATIONS
●Evaluating the cause of right heart failure – The process of evaluating the cause of right heart failure (RHF) overlaps the process of diagnosing RHF, starting with assessment of clinical manifestations and diagnostic testing (table 3). (See 'Evaluating the cause of right heart failure' above and "Right heart failure: Clinical manifestations and diagnosis", section on 'Clinical manifestations'.)
●Acute right heart failure – Causes of acute RHF include causes of acute dyspnea and pulmonary hypertension (PH; such as pulmonary embolism and adult respiratory distress syndrome), causes of primary right ventricular (RV) dysfunction (such as RV myocardial infarction [RVMI] and myocarditis), disorders associated with increased venous return (such as sepsis and RHF after left ventricular assist device implantation), and cardiac tamponade. (See 'Acute right heart failure' above.)
●Chronic right heart failure – Causes of chronic RHF include causes of chronic PH (including PH due to left heart disease), various types of congenital heart disease that cause RV volume or pressure overload or RV dysfunction, right-sided valve disease, cardiomyopathies, and constrictive pericarditis. (See 'Chronic right heart failure' above.)
●Management
•General management – General management of RHF includes maintaining oxygen delivery to the tissues, monitoring optimizing volume status, hemodynamic support, arrhythmia management, and activity restriction. (See 'General management' above.)
•Specific therapies – Certain causes of RHF are treated with the appropriate specific therapies. Examples include myocardial reperfusion for patients with RVMI and pericardiectomy for patients with late-stage constrictive pericarditis. (See 'Specific therapy' above.)
•Management of refractory right heart failure – Management of refractory RHF includes management of refractory volume overload (with optimized diuretic therapy and if unsuccessful, with hemofiltration), management of low cardiac output (options include intravenous inotropes, mechanical support, and in some cases, heart or heart-lung transplantation), and palliative care. (See 'Management of refractory heart failure' above.)
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