Right heart failure overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rim Halaby, Jad Z Al Danaf, Alberto Castro Molina, M.D.
Overview
The right ventricle was previously recognized as a simple conduit between the systemic and the pulmonary circulation, but its importance in maintaining hemodynamic stability and end organ function is now well established.[1] Right heart function is an important prognostic factor in multiple settings, including congenital heart diseases, pulmonary hypertension, and right heart failure.[2][3]
Right heart failure can be defined as failure of the right ventricle to pump blood into the lungs adequately. It is a clinical syndrome resulting from functional and structural cardiovascular disorders that impair right ventricular filling and or ejection. In clinical practice, the main physiologic determinants of right ventricular performance mirror those of the left ventricle and include preload, afterload, contractility, and active relaxation (lusitropy).[1] Right ventricular failure often reflects impaired right ventricle to pulmonary artery coupling and is particularly sensitive to acute increases in pulmonary vascular load.[4]
Furthermore, isolated right ventricular failure, despite its rarity as compared to left ventricular failure, may carry a worse prognosis in selected settings. Right heart failure is also used interchangeably with cor pulmonale when the pathology is caused by an underlying lung disease.[2][5]
Classification
There are many different ways to categorize right heart failure, which includes whether the abnormality is due to insufficient contraction (systolic dysfunction); or due to insufficient relaxation of the heart (diastolic dysfunction), or to both; whether the problem is primarily increased venous back pressure (preload), or failure to supply adequate arterial perfusion (afterload); whether the abnormality is due to low cardiac output with high systemic vascular resistance or high cardiac output with low vascular resistance (low output heart failure vs. high output heart failure); degree of functional impairment conferred by the abnormality (as reflected in the New York Heart Association Functional Classification[6]); and the degree of coexisting illness, such as heart failure with systemic hypertension, pulmonary hypertension, diabetes, or chronic renal failure.
A practical approach is to classify right ventricular failure by time course (acute vs chronic) and predominant mechanism, including pressure overload (afterload), volume overload (preload), and primary myocardial dysfunction affecting contractility or lusitropy.[1][7]
Pathophysiology
The pathophysiological processes underlying right heart failure can be divided broadly into three: decreased right ventricular contractility, right ventricular pressure overload, and right ventricular volume overload.[8] The right ventricle tries to adapt acutely by dilatation and chronically by hypertrophy. Whether dilatation or hypertrophy occur, right heart failure gets further exacerbated as a result of these adaptive mechanisms.
Right ventricular performance is tightly linked to ventricular interdependence and pericardial constraint. The right ventricle and left ventricle are anatomically and physiologically integrated through the interventricular septum, and right ventricular pressure or volume overload can impair left ventricular filling and output by shifting the septum and increasing pericardial restraint.[1] In addition to load dependent mechanisms, cellular and molecular changes such as myocyte hypertrophy, fibrosis, ischemia, neurohormonal activation, and inflammation contribute to progressive right ventricular dysfunction across etiologies.[9][1]
Causes
There are acute and chronic causes of right heart failure. Acute right heart failure is often associated with right ventricular dilation. Chronic right heart failure is often associated with right ventricular hypertrophy.
Common etiologic categories include:
- Acute pressure overload: pulmonary embolism, acute worsening of pulmonary hypertension, severe hypoxemia, and acute respiratory distress with high intrathoracic pressures from mechanical ventilation.[1][7]
- Chronic pressure overload: pulmonary arterial hypertension and other forms of chronic pulmonary hypertension with progressive right ventricle to pulmonary artery uncoupling.[3][10]
- Primary myocardial dysfunction: right ventricular myocardial infarction and inflammatory or infiltrative cardiomyopathies affecting the right ventricle.[11]
- Volume overload: significant tricuspid regurgitation, intracardiac shunts, and chronic right sided valvular lesions.[7]
- Right ventricular dysfunction secondary to systemic illness and critical illness, including sepsis and severe viral infections; right ventricular dysfunction has also been reported in hospitalized patients with COVID 19 and is associated with adverse outcomes.[12]
Epidemiology and Demographics
The prevalence of heart failure has been increasing due to the increase in the aging population, early detection, preventive measures, and improvement in therapy. The prevalence of heart failure in the United States was estimated in 2006 to be 5.8 million people of all ages with an estimated incidence of 10 per 1000 for individuals older than 65 years of age.[13]
Right ventricular failure epidemiology varies by underlying substrate, and is particularly common in advanced pulmonary hypertension, advanced left sided heart failure, and critical illness with acute pulmonary vascular load. Contemporary guidance emphasizes recognizing right ventricular dysfunction as a major determinant of symptoms, exercise limitation, and outcomes across these populations.[1][7]
Natural History, Complications and Prognosis
Right heart failure, if left undiagnosed and untreated, may lead to impaired quality of life and eventually death. There are several factors that define this natural history depending mainly on the underlying etiology or mechanism of injury, the onset of illness, how early treatment was initiated, and other comorbidities.
Progression is often driven by a cycle of rising right sided filling pressures, impaired forward flow, worsening systemic congestion with hepatic and renal dysfunction, and further neurohormonal activation. In pressure overload states, worsening right ventricle to pulmonary artery uncoupling is a key inflection point associated with clinical deterioration.[1][4]
Diagnosis
In the initial approach to a patient with right heart failure, it is important to determine the underlying etiology, assess functional status, determine the presence of end organ dysfunction (liver and kidney most notably), and identify associated conditions.
History and Symptoms
Right heart failure is frequently associated with shortness of breath, exercise intolerance and coughing, and in later stages chest discomfort and swelling of the feet or ankles. According to the 2009 Canadian Cardiovascular Society Consensus Conference update on right sided heart failure, right heart failure should be suspected when a patient presents with unexplained exercise intolerance or hypotension with signs of elevated jugular venous pressure (JVP), peripheral edema, hepatomegaly, or a combination of these findings.[14]
Physical Exam
Physical exam should consist of a thorough cardiovascular exam, an abdominal exam, and examination of the extremities. Findings to be aware of are cyanosis, JVD, S2 and S3 heart sounds, ascites, hepatomegaly, and pedal edema.
Laboratory Findings
Laboratory tests are not diagnostic for heart failure, but they are essential to identify precipitating factors of decompensation, assess severity, monitor adverse effects of therapy, and provide prognostic information.[15] In addition, in patients with right heart failure, an arterial blood gas can be useful in assessing hypoxemia and guiding oxygen therapy.
Electrocardiogram
Right heart failure is often accompanied by right ventricular hypertrophy and right ventricular dilation. General electrocardiographic findings of right ventricular hypertrophy include right axis deviation, an R S ratio greater than 1 in V1, and the presence of P pulmonale.
Chest X Ray
The plain chest radiograph has limited utility in identifying right heart failure. It might show evidence of underlying causes such as pulmonary embolism or congenital heart disease.
Echocardiography
Transthoracic echocardiography plays a key role in the diagnosis of right heart failure by evaluating right ventricular size and function, estimating pulmonary pressures, and assessing tricuspid regurgitation. Recommended measures of right ventricular systolic function include tricuspid annular plane systolic excursion (TAPSE), tissue Doppler S prime, right ventricular fractional area change, and right ventricular longitudinal strain, interpreted in the context of loading conditions and right ventricle to pulmonary artery coupling.[16][1]
Invasive Hemodynamics
Right heart catheterization can define the hemodynamic profile, quantify pulmonary vascular load, and guide therapy in complex or refractory cases. In acute decompensation, invasive monitoring may help assess response to preload adjustment and afterload targeted therapies, particularly when clinical examination and noninvasive findings are discordant.[1][7]
Treatment
Medical Therapy
Currently, the basis of therapy for right heart failure includes cautious diuresis, rhythm management, and treatment of the underlying cause when feasible. Management can be tailored to etiology specific therapy such as anticoagulation for pulmonary embolism or antibiotics for endocarditis. Management also comprises optimizing right ventricular preload, afterload and contractility.
A contemporary approach emphasizes four parallel goals: optimize preload, reduce right ventricular afterload, augment contractility when needed, and maintain systemic perfusion pressure while treating the precipitating cause.[1][7]
Optimization of preload includes careful diuresis and management of venous congestion, while avoiding excessive preload reduction that may lower cardiac output. In selected patients, invasive hemodynamic guidance may be useful for titration.[1][7]
Reduction of afterload depends on etiology and includes reperfusion or anticoagulation for acute pulmonary embolism, correction of hypoxemia and acidosis, minimizing injurious ventilator settings, and targeted pulmonary vasodilator therapy for appropriate forms of pulmonary hypertension.[1][10]
Augmentation of contractility may be required in shock or low output states. Dobutamine can increase cardiac output and stroke volume in right ventricular myocardial infarction and in pulmonary hypertension, and milrinone may be considered with attention to systemic vasodilation and hypotension.[1][7]
Since atrial fibrillation and high grade AV block can cause hemodynamic instability in right heart failure, maintaining sinus rhythm and atrioventricular synchrony is important.[17]
Surgery
Surgical intervention in right heart failure is mainly indicated for valvular pathologies and congenital heart diseases causing progressive symptoms despite adequate medical interventions. Early correction in selected settings can improve symptoms and outcomes. Treating primary pulmonary hypertension may lead to improved functional capacity, and in selected cases, lung transplant or heart lung transplant can extend survival.[17][7]
Mechanical Circulatory Support
Temporary mechanical circulatory support may be considered for patients with refractory acute right ventricular failure and shock despite optimized pharmacotherapy and correction of reversible causes.[1][18] Device selection should account for the mechanism of failure and the presence of pulmonary arterial hypertension, since isolated right ventricular assist strategies may be inappropriate when pulmonary vascular resistance is markedly elevated.[1]
Future or Investigational Therapies
A right ventricular assist device (RVAD) can be a temporary therapy for patients with acute exacerbation of right heart failure. Ongoing research focuses on improved assessment of right ventricle to pulmonary artery coupling and therapies that directly target right ventricular contractility and lusitropy.[1]
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 Houston, Brian A.; Brittain, Evan L.; Tedford, Ryan J. (2023). "Right Ventricular Failure". New England Journal of Medicine. 388 (12): 1111–1125. doi:10.1056/NEJMra2207410.
- ↑ 2.0 2.1 Haddad, F.; Hunt, S.A.; Rosenthal, D.N.; Murphy, D.J. (2008). "Right ventricular function in cardiovascular disease, part I: Anatomy, physiology, aging, and functional assessment of the right ventricle". Circulation. 117: 1436–1448.
- ↑ 3.0 3.1 Hassoun, P.M. (2021). "Pulmonary arterial hypertension". New England Journal of Medicine. 385: 2361–2376.
- ↑ 4.0 4.1 Vonk Noordegraaf, A.; Westerhof, B.E.; Westerhof, N. (2017). "The relationship between the right ventricle and its load in pulmonary hypertension". Journal of the American College of Cardiology. 69: 236–243.
- ↑ McLaughlin, V.V.; Archer, S.L.; Badesch, D.B.; Barst, R.J.; Farber, H.W.; Lindner, J.R.; Mathier, M.A. (2009). "ACCF AHA 2009 expert consensus document on pulmonary hypertension". Journal of the American College of Cardiology. 53: 1573–1619.
- ↑ Criteria Committee, New York Heart Association. Diseases of the heart and blood vessels. Nomenclature and criteria for diagnosis, 6th ed. Boston: Little, Brown and Co., 1964;114.
- ↑ 7.0 7.1 7.2 7.3 7.4 7.5 7.6 7.7 7.8 Konstam, M.A.; Kiernan, M.S.; Bernstein, D. (2018). "Evaluation and management of right sided heart failure: a scientific statement from the American Heart Association". Circulation. 137 (20): e578–e622.
- ↑ Piazza G, Goldhaber SZ (2005). "The acutely decompensated right ventricle: pathways for diagnosis and management". Chest. 128 (3): 1836–52. doi:10.1378/chest.128.3.1836. PMID 16162794.
- ↑ Ryan, J.J.; Archer, S.L. (2014). "The right ventricle in pulmonary arterial hypertension: disorders of metabolism, angiogenesis and adrenergic signaling in right ventricular failure". Circulation Research. 115: 176–188.
- ↑ 10.0 10.1 Humbert, M.; Kovacs, G.; Hoeper, M.M. (2022). "2022 ESC ERS guidelines for the diagnosis and treatment of pulmonary hypertension". European Heart Journal. 43: 3618–3731.
- ↑ O’Rourke, R.A.; Dell’Italia, L.J. (2004). "Diagnosis and management of right ventricular myocardial infarction". Current Problems in Cardiology. 29: 6–47.
- ↑ Corica, B.; Marra, A.M.; Basili, S. (2021). "Prevalence of right ventricular dysfunction and impact on all cause death in hospitalized patients with COVID 19: a systematic review and meta analysis". Scientific Reports. 11: 17774.
- ↑ WRITING GROUP MEMBERS. Lloyd-Jones D, Adams RJ, Brown TM, Carnethon M, Dai S; et al. (2010). "Heart disease and stroke statistics--2010 update: a report from the American Heart Association". Circulation. 121 (7): e46–e215. doi:10.1161/CIRCULATIONAHA.109.192667. PMID 20019324.
- ↑ JG Howlett, RS McKelvie, JMO Arnold, et al. Canadian Cardiovascular Society Consensus Conference guidelines on heart failure, update 2009: Diagnosis and management of right sided heart failure, myocarditis, device therapy and recent important clinical trials. Can J Cardiol 2009;25(2):85-105.
- ↑ Tago WH, Francis GS, Morrow DA, et al. National Academy of Clinical Biochemistry Laboratory Medicine. National Academy of Clinical Biochemistry Laboratory Medicine practice guidelines: Clinical utilization of cardiac biomarker testing in heart failure. Circulation. 2007;116:e90-e109.
- ↑ Rudski, L.G.; Lai, W.W.; Afilalo, J. (2010). "Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography". Journal of the American Society of Echocardiography. 23: 685–713.
- ↑ 17.0 17.1 Haddad, F.; Hunt, S.A.; Rosenthal, D.N.; Murphy, D.J. (2008). "Right Ventricular function in Cardiovascular Disease, Part II: Pathophysiology, Clinical Importance and Management of Right Ventricular Failure". Circulation. 117: 1717–1731.
- ↑ Kapur, N.K.; Esposito, M.L.; Bader, Y. (2017). "Mechanical circulatory support devices for acute right ventricular failure". Circulation. 136: 314–326.