Right heart failure: Causes and management

Author: Barry A Borlaug, MD

Section Editor: Donna Mancini, MD
Deputy Editor: Todd F Dardas, MD, MS

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Feb 2024. | This topic last updated: Nov 03, 2022.

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
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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