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Right heart failure

Updated : September 23, 2023





Background

Heart failure occurs when the heart’s ability to effectively pump blood throughout the body diminishes. One common cause of right heart failure is the result of left ventricular failure, which leads to increased volume and pressure in the right side of the heart. Clinically, individuals with this condition often experience symptoms like chest discomfort, shortness of breath, irregular heartbeats, and swelling in the body.

To assess this condition, various non-invasive methods are employed, including echocardiography, nuclear angiography, MRI, and 64-slice CT scans, in addition to invasive hemodynamic measurements. The management approach focuses on enhancing the contractility of the right ventricle, reducing the afterload on the right ventricle, and optimizing the patient’s fluid balance.

Epidemiology

Right heart failure is often secondary to left heart failure (known as congestive heart failure) and occurs due to increased pressure or volume in the right side of the heart. The prevalence of right heart failure varies depending on the underlying causes, such as pulmonary hypertension, chronic lung diseases, or congenital heart defects. It is a common complication in patients with congestive heart failure.

The incidence of right heart failure can be challenging to determine on its own, as it is often associated with other cardiac or pulmonary conditions. Studies typically focus on the incidence of the underlying conditions that contribute to right heart failure, such as pulmonary hypertension or chronic obstructive pulmonary disease (COPD). Right heart failure can affect individuals of all ages.

However, it is more common in older adults due to the higher prevalence of heart-related and lung-related conditions in this population. Gender may also play a role, as certain conditions, like pulmonary arterial hypertension, are more prevalent in females. The prevalence and incidence of right heart failure may vary by geographic region, as factors like air pollution, access to healthcare, and lifestyle choices can impact the underlying conditions contributing to right heart failure.

Anatomy

Pathophysiology

Right heart failure often results from an increased workload on the right ventricle. This increased afterload can occur due to various factors, including pulmonary hypertension. The right ventricle’s main function is to pump blood into the low-pressure pulmonary circulation. When faced with increased resistance, the right ventricle must generate higher pressure to maintain adequate blood flow to the lungs.

This sustained pressure overload can eventually weaken the right ventricle, impairing its ability to pump effectively. Prolonged pressure and volume overload on the right ventricle can lead to myocardial dysfunction. This dysfunction can manifest as reduced contractility, impaired relaxation, and decreased cardiac output. Eventually, it can progress to right ventricular failure, where the heart cannot adequately pump blood to the lungs.

The pathophysiology of right heart failure is closely interconnected with the underlying causes and conditions that contribute to it. Effective management of right heart failure typically involves addressing the underlying etiology, reducing afterload and preload on the right ventricle, and improving right ventricular function to restore efficient pulmonary blood flow.

Etiology

Right heart failure is typically a consequence of various underlying conditions and diseases that directly or indirectly affect the right side of the heart, primarily the right atrium and right ventricle.

Chronic Lung Diseases: Conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, or severe asthma can cause chronic hypoxia, increased pulmonary vascular resistance, and pulmonary hypertension, ultimately affecting the right ventricle.

Congenital Heart Defects: Certain congenital heart defects, such as atrial septal defects (ASDs), ventricular septal defects (VSDs), or Tetralogy of Fallot, can cause right heart strain and eventual right heart failure.

Cardiomyopathies: Specific types of cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (ARVC) or restrictive cardiomyopathy, can primarily affect the right ventricle’s function and structure.

Pulmonary Embolism: Acute blockage of pulmonary arteries by blood clots can acutely increase right ventricular afterload, leading to right heart failure.

Genetics

Prognostic Factors

Clinical History

Pre-existing co-morbidities may contribute to right heart failure, such as left heart failure, pulmonary hypertension, chronic lung diseases (COPD), congenital heart defects, valvular heart diseases, or a history of pulmonary embolism. One of the hallmark symptoms of right heart failure is dyspnea, often occurring with exertion and progressing to rest. Patients may describe a sensation of breathlessness or difficulty breathing. Ascites is the accumulation of fluid in the abdominal cavity.

Patients with right heart failure often experience fatigue and weakness, which can limit their ability to perform daily activities. Some individuals may develop a persistent dry cough or cough with pink-tinged sputum. Acute right heart failure, as seen in cases of pulmonary embolism, may have a sudden and severe onset. Chronic right heart failure often develops gradually and may be associated with a history of underlying heart or lung conditions.

Physical Examination

Elevated jugular venous pressure (JVP) is a classic sign of right heart failure. The healthcare provider examines the patient’s neck while they are in a semi-recumbent position. JVD is visible as engorged, pulsating jugular veins, typically extending above the clavicle when present. Swelling of the lower extremities, particularly in the legs, ankles, and feet. The edema may be pitting, where pressure on the swollen area leaves a temporary indentation.

Bluish discoloration of the skin and mucous membranes can occur in advanced cases of right heart failure due to inadequate oxygenation of tissues. In some cases, the Parasternal Heave may have visible and palpable pulsations of the chest wall, specifically the left parasternal area, due to right ventricular enlargement.

The presence of an S3 gallop rhythm can indicate fluid overload and impaired ventricular filling. Abnormal breath sounds heard on auscultation of the lungs can indicate pulmonary congestion and fluid accumulation. Patients with hepatomegaly may experience tenderness or discomfort in the right upper quadrant of the abdomen.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Cirrhosis

Community-Acquired pneumonia

Emphysema

Idiopathic pulmonary fibrosis

Myocardial infarction

Nephrotic syndrome

Pulmonary embolism

Respiratory failure

Venous insufficiency

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The management of acute right ventricular failure begins with assessing the severity of the patient’s condition and deciding whether to admit them to an intermediate care unit when appropriate. It is crucial to promptly identify and address any factors that may have triggered RVF, such as arrhythmias, sepsis, or drug withdrawal. In cases where RVF is due to a right ventricular infarct, rapid revascularization is essential. Similarly, reperfusion therapy should be administered promptly for patients at high risk of pulmonary embolism.

Infections pose a significant threat in acute RVF, and preventive measures and early detection and treatment of infections are vital for patient outcomes. The decision to administer fluids should be made carefully, mainly if the patient is hypotensive with low or normal filling pressures. While the right ventricle depends on adequate preload, excessive volume loading can lead to RV overdistension and a further decline in cardiac output. To monitor and manage this, central venous pressure monitoring or placement of a pulmonary artery catheter can be helpful.

In cases of volume overload, intravenous diuresis is recommended. Renal replacement therapy may be considered if volume removal cannot be achieved with medications alone. Diuresis alleviates symptoms, reduces tricuspid regurgitation, restores synchronized RV contraction, and diminishes ventricular interdependence.

To maintain proper fluid balance, sodium restriction, daily weight monitoring, strict fluid intake, and urine output monitoring are advised. When hemodynamic instability is present, vasopressors are indicated to improve systemic blood pressure and restore adequate perfusion to the brain, heart, and other organs. Norepinephrine is the preferred choice of vasopressor in this context.

Inotropic agents such as dobutamine, levosimendan, and milrinone can benefit contractility and cardiac output. Among these, dobutamine is the preferred choice for RVF, as it boosts myocardial contractility through beta receptor stimulation and reduces afterload through beta receptor-mediated vasodilation. However, caution is necessary when using dobutamine and milrinone, as they can lower systemic blood pressure. If this occurs, a vasopressor may need to be added to maintain adequate perfusion.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Surgical interventions are recommended for patients experiencing reversible right ventricular failure (RVF) that does not respond to medical treatment. These surgical options can serve as either a transitional measure to support recovery or as a bridge to heart transplantation.

Furthermore, surgery may be necessary for patients with RVF associated with congenital heart defects, valvular heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH).

It is essential to ensure that patients undergo proper preoperative diuresis before surgical procedures. Additionally, using pulmonary vasodilators and inotropes during the perioperative period may be necessary to optimize patient outcomes.

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Intervention with procedure

Cardiac resynchronization therapy plays a crucial role in restoring mechanical coordination in patients with left ventricular failure (LVF), resulting in improved hemodynamics and a reversal of adverse remodeling. This therapy has been shown to enhance both morbidity and mortality outcomes in LVF. While animal studies and limited case reports suggest that right ventricular (RV) pacing can provide immediate hemodynamic benefits in individuals with RV failure related to pulmonary arterial hypertension (PAH), there is a lack of long-term clinical evidence demonstrating its sustained advantages in this patient group. 

Ultimately, when dealing with advanced RV failure that does not respond to other treatments, heart, lung, or combined heart-lung transplantation (HLT) becomes the treatment of last resort. For individuals with RV failure due to PAH, specific indicators such as a right atrial pressure (RAP) exceeding 15 and a cardiac index (CI) below 2.0 serve as poor prognostic factors, warranting a referral for transplantation. The precise point at which the RV becomes irreversibly damaged remains unclear. However, in general, the RV exhibits remarkable resilience, and in most cases, lung transplantation alone is sufficient.

This approach yields estimated one-year survival rates ranging from 65% to 75% and ten-year survival rates ranging from 45% to 66%. Congenital heart disease patients with RV failure in the context of Eisenmenger syndrome may undergo lung transplantation, sometimes accompanied by the repair of simple shunts like atrial septal defects (ASDs) during surgery, or opt for combined heart-lung transplantation. Studies have indicated a survival benefit in this particular patient population with the latter approach.

Medication

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References

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Right heart failure

Updated : September 23, 2023




Heart failure occurs when the heart’s ability to effectively pump blood throughout the body diminishes. One common cause of right heart failure is the result of left ventricular failure, which leads to increased volume and pressure in the right side of the heart. Clinically, individuals with this condition often experience symptoms like chest discomfort, shortness of breath, irregular heartbeats, and swelling in the body.

To assess this condition, various non-invasive methods are employed, including echocardiography, nuclear angiography, MRI, and 64-slice CT scans, in addition to invasive hemodynamic measurements. The management approach focuses on enhancing the contractility of the right ventricle, reducing the afterload on the right ventricle, and optimizing the patient’s fluid balance.

Right heart failure is often secondary to left heart failure (known as congestive heart failure) and occurs due to increased pressure or volume in the right side of the heart. The prevalence of right heart failure varies depending on the underlying causes, such as pulmonary hypertension, chronic lung diseases, or congenital heart defects. It is a common complication in patients with congestive heart failure.

The incidence of right heart failure can be challenging to determine on its own, as it is often associated with other cardiac or pulmonary conditions. Studies typically focus on the incidence of the underlying conditions that contribute to right heart failure, such as pulmonary hypertension or chronic obstructive pulmonary disease (COPD). Right heart failure can affect individuals of all ages.

However, it is more common in older adults due to the higher prevalence of heart-related and lung-related conditions in this population. Gender may also play a role, as certain conditions, like pulmonary arterial hypertension, are more prevalent in females. The prevalence and incidence of right heart failure may vary by geographic region, as factors like air pollution, access to healthcare, and lifestyle choices can impact the underlying conditions contributing to right heart failure.

Right heart failure often results from an increased workload on the right ventricle. This increased afterload can occur due to various factors, including pulmonary hypertension. The right ventricle’s main function is to pump blood into the low-pressure pulmonary circulation. When faced with increased resistance, the right ventricle must generate higher pressure to maintain adequate blood flow to the lungs.

This sustained pressure overload can eventually weaken the right ventricle, impairing its ability to pump effectively. Prolonged pressure and volume overload on the right ventricle can lead to myocardial dysfunction. This dysfunction can manifest as reduced contractility, impaired relaxation, and decreased cardiac output. Eventually, it can progress to right ventricular failure, where the heart cannot adequately pump blood to the lungs.

The pathophysiology of right heart failure is closely interconnected with the underlying causes and conditions that contribute to it. Effective management of right heart failure typically involves addressing the underlying etiology, reducing afterload and preload on the right ventricle, and improving right ventricular function to restore efficient pulmonary blood flow.

Right heart failure is typically a consequence of various underlying conditions and diseases that directly or indirectly affect the right side of the heart, primarily the right atrium and right ventricle.

Chronic Lung Diseases: Conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, or severe asthma can cause chronic hypoxia, increased pulmonary vascular resistance, and pulmonary hypertension, ultimately affecting the right ventricle.

Congenital Heart Defects: Certain congenital heart defects, such as atrial septal defects (ASDs), ventricular septal defects (VSDs), or Tetralogy of Fallot, can cause right heart strain and eventual right heart failure.

Cardiomyopathies: Specific types of cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (ARVC) or restrictive cardiomyopathy, can primarily affect the right ventricle’s function and structure.

Pulmonary Embolism: Acute blockage of pulmonary arteries by blood clots can acutely increase right ventricular afterload, leading to right heart failure.

Pre-existing co-morbidities may contribute to right heart failure, such as left heart failure, pulmonary hypertension, chronic lung diseases (COPD), congenital heart defects, valvular heart diseases, or a history of pulmonary embolism. One of the hallmark symptoms of right heart failure is dyspnea, often occurring with exertion and progressing to rest. Patients may describe a sensation of breathlessness or difficulty breathing. Ascites is the accumulation of fluid in the abdominal cavity.

Patients with right heart failure often experience fatigue and weakness, which can limit their ability to perform daily activities. Some individuals may develop a persistent dry cough or cough with pink-tinged sputum. Acute right heart failure, as seen in cases of pulmonary embolism, may have a sudden and severe onset. Chronic right heart failure often develops gradually and may be associated with a history of underlying heart or lung conditions.

Elevated jugular venous pressure (JVP) is a classic sign of right heart failure. The healthcare provider examines the patient’s neck while they are in a semi-recumbent position. JVD is visible as engorged, pulsating jugular veins, typically extending above the clavicle when present. Swelling of the lower extremities, particularly in the legs, ankles, and feet. The edema may be pitting, where pressure on the swollen area leaves a temporary indentation.

Bluish discoloration of the skin and mucous membranes can occur in advanced cases of right heart failure due to inadequate oxygenation of tissues. In some cases, the Parasternal Heave may have visible and palpable pulsations of the chest wall, specifically the left parasternal area, due to right ventricular enlargement.

The presence of an S3 gallop rhythm can indicate fluid overload and impaired ventricular filling. Abnormal breath sounds heard on auscultation of the lungs can indicate pulmonary congestion and fluid accumulation. Patients with hepatomegaly may experience tenderness or discomfort in the right upper quadrant of the abdomen.

Cirrhosis

Community-Acquired pneumonia

Emphysema

Idiopathic pulmonary fibrosis

Myocardial infarction

Nephrotic syndrome

Pulmonary embolism

Respiratory failure

Venous insufficiency

The management of acute right ventricular failure begins with assessing the severity of the patient’s condition and deciding whether to admit them to an intermediate care unit when appropriate. It is crucial to promptly identify and address any factors that may have triggered RVF, such as arrhythmias, sepsis, or drug withdrawal. In cases where RVF is due to a right ventricular infarct, rapid revascularization is essential. Similarly, reperfusion therapy should be administered promptly for patients at high risk of pulmonary embolism.

Infections pose a significant threat in acute RVF, and preventive measures and early detection and treatment of infections are vital for patient outcomes. The decision to administer fluids should be made carefully, mainly if the patient is hypotensive with low or normal filling pressures. While the right ventricle depends on adequate preload, excessive volume loading can lead to RV overdistension and a further decline in cardiac output. To monitor and manage this, central venous pressure monitoring or placement of a pulmonary artery catheter can be helpful.

In cases of volume overload, intravenous diuresis is recommended. Renal replacement therapy may be considered if volume removal cannot be achieved with medications alone. Diuresis alleviates symptoms, reduces tricuspid regurgitation, restores synchronized RV contraction, and diminishes ventricular interdependence.

To maintain proper fluid balance, sodium restriction, daily weight monitoring, strict fluid intake, and urine output monitoring are advised. When hemodynamic instability is present, vasopressors are indicated to improve systemic blood pressure and restore adequate perfusion to the brain, heart, and other organs. Norepinephrine is the preferred choice of vasopressor in this context.

Inotropic agents such as dobutamine, levosimendan, and milrinone can benefit contractility and cardiac output. Among these, dobutamine is the preferred choice for RVF, as it boosts myocardial contractility through beta receptor stimulation and reduces afterload through beta receptor-mediated vasodilation. However, caution is necessary when using dobutamine and milrinone, as they can lower systemic blood pressure. If this occurs, a vasopressor may need to be added to maintain adequate perfusion.

Surgical interventions are recommended for patients experiencing reversible right ventricular failure (RVF) that does not respond to medical treatment. These surgical options can serve as either a transitional measure to support recovery or as a bridge to heart transplantation.

Furthermore, surgery may be necessary for patients with RVF associated with congenital heart defects, valvular heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH).

It is essential to ensure that patients undergo proper preoperative diuresis before surgical procedures. Additionally, using pulmonary vasodilators and inotropes during the perioperative period may be necessary to optimize patient outcomes.

Cardiac resynchronization therapy plays a crucial role in restoring mechanical coordination in patients with left ventricular failure (LVF), resulting in improved hemodynamics and a reversal of adverse remodeling. This therapy has been shown to enhance both morbidity and mortality outcomes in LVF. While animal studies and limited case reports suggest that right ventricular (RV) pacing can provide immediate hemodynamic benefits in individuals with RV failure related to pulmonary arterial hypertension (PAH), there is a lack of long-term clinical evidence demonstrating its sustained advantages in this patient group. 

Ultimately, when dealing with advanced RV failure that does not respond to other treatments, heart, lung, or combined heart-lung transplantation (HLT) becomes the treatment of last resort. For individuals with RV failure due to PAH, specific indicators such as a right atrial pressure (RAP) exceeding 15 and a cardiac index (CI) below 2.0 serve as poor prognostic factors, warranting a referral for transplantation. The precise point at which the RV becomes irreversibly damaged remains unclear. However, in general, the RV exhibits remarkable resilience, and in most cases, lung transplantation alone is sufficient.

This approach yields estimated one-year survival rates ranging from 65% to 75% and ten-year survival rates ranging from 45% to 66%. Congenital heart disease patients with RV failure in the context of Eisenmenger syndrome may undergo lung transplantation, sometimes accompanied by the repair of simple shunts like atrial septal defects (ASDs) during surgery, or opt for combined heart-lung transplantation. Studies have indicated a survival benefit in this particular patient population with the latter approach.

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