Right ventricular assessment in pulmonary hypertension

Lyana Labrada; Anika Vaidy; Anjali Vaidya

doi:10.1097/MCP.0000000000000980

. 2023 Jul 6;29(5):348–354. doi: 10.1097/MCP.0000000000000980

Right ventricular assessment in pulmonary hypertension

Lyana Labrada ¹, Anika Vaidy ¹, Anjali Vaidya ¹

PMCID: PMC10408730 PMID: 37410491

Purpose of review

The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis.

Recent findings

The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme.

Summary

Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.

Keywords: pulmonary hypertension, pulmonary vascular resistance, right ventricle

INTRODUCTION

Thorough evaluation of the right ventricle (RV) is of utmost importance in evaluating pulmonary hypertension. Complete assessment includes both echocardiographic and hemodynamic assessment, and provides insight to disease severity and prognosis. We will review unique characteristics of right ventricular anatomy and physiology, the relationship between right ventricular function and the pulmonary vasculature, methods for echocardiographic and hemodynamic assessment, and ways in which right ventricular assessment is contributory to risk assessment and stratification in patients with pulmonary hypertension.

RIGHT VENTRICULAR ANATOMY

The RV is anatomically unique from the left ventricle (LV). The RV is positioned anterior to the LV within the chest cavity, sits immediately posterior to the sternum, and forms the inferior border of the heart. The tricuspid valve, comprised of three leaflets and secured by multiple papillary muscles, forms the superior border of the RV. Perfusion to the RV is supplied primarily by the right coronary artery, and notably occurs during both systole and diastole secondary to low RV wall tension in normal physiology.

Wall thickness is normally 2–5 mm, which comparatively is one-sixth that of the LV. A deep layer of subendocardial muscle fibers lies longitudinally from base to apex, whereas a more superficial layer of muscle fibers is arranged circumferentially, parallel to the atrioventricular groove. Due to the unique orientation of muscle fibers within the RV, its contractile pattern is primarily within the longitudinal plane. Both the interventricular septum and the free wall, comprise the distinct crescent-like shape of the RV, in contrast to the ellipsoid shape of the LV [1^▪,2]. The anatomic differences between the ventricles allow for unique differences in physiology.

RIGHT VENTRICULAR PHYSIOLOGY AND INTERACTION WITH PULMONARY VASCULATURE

Despite a markedly reduced myocardial mass, the RV generates an equivalent stroke volume and cardiac output (CO) to the LV, made possible by a low-impedance, highly compliant pulmonary arterial system comprised of thin-walled vessels, which provides a low degree of afterload for the RV. Pulmonary vascular resistance (PVR) under normal circumstances is a fraction of systemic vascular resistance.

With progressive pulmonary vascular disease in pulmonary arterial hypertension (PAH), a rising PVR and right ventricular afterload leads to increased right ventricular wall stress and subsequently right ventricular hypertrophy (RVH), allowing for increased contractility. These adaptive changes maintain ‘RV–PA coupling’, a concept describing the RV's ability to adapt to an increased afterload while maintaining stroke volume. With increasing PVR and maximized RVH, the RV dilates to accommodate increased volumes and maintain CO. Right ventricular dilation is a maladaptive response, and leads to an increase in wall stress, oxygen consumption, and cumulatively a loss of efficient right ventricular contractile function. In this setting, the RV can no longer compensate for its degree of afterload to maintain CO, leading to ‘RV–PA uncoupling’ [1^▪,2]. Although maladaptive changes of the RV occur with progressive pulmonary vascular disease, the reverse is also true. A maladaptive RV has the potential to reverse remodel with normalization of size, morphology and function with a significant reduction in PVR [3].

CAUSE OF PULMONARY HYPERTENSION: ECHOCARDIOGRAPHY TO DISTINGUISH ELEVATED PULMONARY VASCULAR RESISTANCE VS. PULMONARY VENOUS HYPERTENSION

Pulmonary hypertension is widely defined as a mean pulmonary artery pressure greater than 20 mmHg [4^▪▪]. However, there are multiple causes of pulmonary hypertension that may lead to elevation in pressure. Differentiation between PAH (characterized by elevated PVR), and pulmonary venous hypertension (PVH – characterized by increased left-heart pressures), is of utmost importance to determine appropriate treatment. Meaningful echocardiographic assessment of the right and left heart may often offer insight into the cause of pulmonary hypertension (Fig. 1).

Echocardiographic parameters to distinguish between elevated pulmonary vascular resistance and pulmonary venous hypertension. While elevated RVSP may be present in both elevated PVR and pulmonary venous hypertension, there are multiple echocardiographic parameters distinct to each phenotype. PVR, pulmonary vascular resistance; RVSP, right ventricular systolic pressure.

Evaluation of the right ventricular systolic pressure (RVSP) by Doppler echocardiography (D_E) is often the most common method of evaluating pulmonary hypertension in routine clinical settings. Although Doppler-derived RVSP estimation remains a useful pulmonary hypertension screening tool, it has many limitations, including the potential to both overestimate and underestimate the degree of pulmonary hypertension, as it relies on accurate assessment of the tricuspid regurgitation jet velocity and inferior vena cava assessment. Further, estimation of RVSP is unable to predict an elevated PVR, as it may be markedly elevated in both PAH and PVH [5].

Although RVSP is limited in determining the cause of pulmonary hypertension, other D_E-derived factors may provide noninvasive hemodynamic insight (Fig. 2). The pulse-waved Doppler envelope of the right ventricular outflow tract (RVOT) interrogates the velocity of blood leaving the RVOT through the pulmonic valve during systole, which is affected by the timing and degree of arterial wave reflection. Under normal circumstances, the pulmonary circulation is a low resistance system, and the small wave that is reflected backwards after pulmonic valve closure does not affect the Doppler envelope. However, with progressive PVR elevation, a larger reflected arterial wave with increased velocity arrives at the RVOT during systole, leading to blood flow deceleration, and in turn mid-systolic notching of the Doppler envelope. Consistent with this physiology, the presence of RVOT Doppler notching has been shown to be strongly suggestive of pulmonary hypertension in the setting of PVR elevation. Further, the time to peak velocity of blood flow within the RVOT, known as the acceleration time, has been shown to be shorter with PVR elevation [6]. In contrast, pulmonary hypertension without RVOT systolic notching is strongly suggestive of PVH in the setting of left-sided congestion, and the absence of PVR elevation.

Right ventricular echocardiographic parameters associated with elevated pulmonary vascular resistance. Panel a: severe right ventricular dilation (RV >LV), with decreased base : apex ratio (demonstrated by lines measuring basal and apical diameters), and increased right atrial size with septal bowing to the left. Panel b: severe systolic interventricular septal flattening in the short-axis view. Panel c: mid-systolic notching (MSN) of the RVOT Pulse Wave Doppler and short acceleration time (AccT).

Additional insight into cause of pulmonary hypertension can be obtained via evaluation of the morphology of the RV, the structural relationship between the RV and LV, and evaluation of left-sided structures and function. Normally, the RV is tapered at the apex, with the RV base being twice as wide as the apex, and overall smaller in size than the LV. With progressive PVR elevation, both the apex and base dilate, leading to a base–apex ratio approaching 1. A base–apex ratio less than 1.5 has been shown to have a strong echocardiographic association with elevated PVR [7]. Flattening of the interventricular septum in systole has been associated with increased RV afterload and PVR elevation [8] (Fig. 2). In contrast to PVR elevation, echocardiographic parameters supportive of PVH include increased left atrial size, presence of atrial septum bowing to the right, transmitral E/A greater than 1, decreased LV tissue Doppler velocities, and lateral E/e′ greater than 10 [8–10] (Fig. 1).

RIGHT VENTRICULAR ECHOCARDIOGRAPHIC ASSESSMENT

Given the complexity of RV shape and function, in addition to its position within the chest immediately posterior to the sternum, echocardiographic assessment can be challenging. For initial assessment, both the apical four-chamber and subcostal windows offer the most optimal views of the RV. The RV should appear triangular, with a broad base and narrow apex [11–14]. Qualitatively, RV size is often described in comparison to the LV. Normally, the RV should be approximately two-thirds of the size of the LV in basal diameter measurement. Moderate or severe RV dilation occurs when the RV is equal or greater in size than the LV [1^▪,2].

RV thickness should be measured at end-diastole in the subcostal view and should not include epicardial fat or myocardial trabeculations. RV hypertrophy is suggestive of chronically elevated RV afterload and is defined by RV free wall thickness greater than 0.5 cm [2,11,14]

RV function is assessed with a number of measures, including tricuspid annular planar systolic excursion (TAPSE), RV fractional area change (FAC), tissue Doppler peak systolic velocity at the tricuspid annulus, and right ventricular index of myocardial performance (RIMP) (Table 1). TAPSE, often considered the surrogate of RV function, is a measure of longitudinal contraction of the RV and is assessed using M-mode from an apical four-chamber view. It measures excursion of the annulus from end-diastole to peak systole. Compared with other echocardiographic measurements of RV function, TAPSE offers advantages in that it is highly reproducible and is independent of geometric assumptions [12–14].

Table 1.

Right ventricular echocardiographic normal values

RV basal diameter (mm)	≤41
RV mid diameter (mm)	≤35
RVOT distal diameter (mm)	≤35
RV wall Thickness (mm)	≤5
TAPSE (cm)	≥1.6
FAC (%)	≥35
Tissue Doppler Velocity (cm/s)	≥9.5

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Data from [12–14]. RV, right ventricle; RVOT, right ventricular outflow tract; TAPSE, tricuspid annular planar systolic excursion.

It should be noted that there are certain contexts in which the predominant motion of the RV is nonlongitudinal. Vaidya et al. compared right ventricular contractile patterns in patients who underwent cardiac surgery with controls [15]. They found that, in the surgical cohort, there was a dramatic decrease in longitudinal shortening, but overall right ventricular function was normal due to a gain in transverse shortening. In these cases, global assessment of right ventricular function may prove more useful.

RIGHT VENTRICULAR HEMODYNAMIC ASSESSMENT

Right ventricular assessment in patients with pulmonary hypertension includes a thorough evaluation of hemodynamic parameters reflective of right ventricular function. Although significant hemodynamic data may be obtained from echocardiography, right heart catheterization is the gold standard for invasive hemodynamic assessment in pulmonary hypertension. Assessment is important both for initial diagnosis, and for further classification of the phenotype of pulmonary hypertension to correctly determine management, risk assessment and prognosis [16].

Hemodynamic parameters supportive of PAH include PVR at least 2 Woods units (WU) and pulmonary capillary wedge pressure (PCWP) less than 15 mmHg. PVH secondary to left heart congestion may be classified by PVR less than 2 WU, and PCWP greater than 15 mmHg. A mixed picture in the setting of multifactorial pulmonary hypertension may be classified by PVR at least 2 WU, and PCWP greater than 15 mmHg [4^▪▪,16,17].

Once the phenotype of pulmonary hypertension has been classified, this information must be combined with a hemodynamic evaluation of right ventricular function for a full clinical assessment. Right atrial pressure is an important marker of right ventricular function. In a normal heart, right atrial pressure is between 3 and 7 mmHg, and is usually approximately 1/2 of PCWP. The 1 : 2 relationship between right atrial pressure and PCWP is generally maintained in normal right ventricular function. However, with progressive right ventricular dysfunction, right atrial pressure may rise independently, leading to elevation out of proportion to PCWP. Therefore, both elevated right atrial pressure alone, in addition to a higher right atrium : PCWP ratio, is suggestive of worsening right ventricular dysfunction [18].

In addition to hemodynamic variables of right ventricular function, brain natriuretic peptide (BNP), and N-terminal pro-BNP have an increasingly important role both in clinical assessment and prognosis in pulmonary hypertension. BNP is released by the cardiac myocytes in proportion to increased wall stretch, and therefore often correlates with increased filling pressures. BNP has been shown to increase in proportion to worsening right ventricular dysfunction [19]. Further, NT-proBNP has been found to be predictive in identifying patients with poor long-term prognosis, and is incorporated into risk stratification tools for pulmonary hypertension [20–22].

RIGHT VENTRICULAR FUNCTION AS A GOAL FOR PULMONARY ARTERIAL HYPERTENSION THERAPY

Complete assessment of right ventricular function is key in both initial diagnosis of pulmonary hypertension and serial evaluation of PAH. The degree of right ventricular dysfunction remains a critical marker for disease severity. Multiple hemodynamic variables predictive of right ventricular function are associated with mortality in PAH. Elevated right atrial pressure [22], increased right atrium : PCWP ratio, reduced cardiac index, and reduced stroke volume have all been associated with increased mortality in pulmonary hypertension [18,21,23,24]. These parameters are predictive of mortality both at baseline, and when serially assessed during the clinical trajectory of disease. Response to therapy resulting in changes in these hemodynamic parameters (either worsening or improvement), evaluated by serial right heart catheterization, has been shown to be of similar importance in predicting mortality as baseline parameters [21].

Further, several markers of right ventricular dysfunction obtained with echocardiography are shown to be predictive of mortality in PAH, and may serve as goal metrics for titration of pulmonary hypertension therapies. These are listed in Table 2, and include but are not limited to TAPSE, right ventricular end diastolic volume, right ventricular mass, right atrial pressure, and cardiac index. TAPSE is a representative parameter of right ventricular function, as it measures systolic motion of the tricuspid annulus in the longitudinal plane, in line with the primary contractile pattern of the RV. TAPSE less than 1.8 cm has been associated with increased mortality in a subgroup of patients with PAH [25]. When assessed serially after pulmonary hypertension treatment was initiated, Mazurek et al. [26] demonstrated that TAPSE greater than 2 cm was highly predictive of survival. When combined with other echocardiographic markers of right ventricular dysfunction, TAPSE becomes an even more powerful predictive tool. Ghio et al. demonstrated that combining markers of right heart dysfunction with markers of systemic venous congestion was predictive of increased mortality. In this study, a reduced TAPSE combined with a dilated inferior vena cava on echocardiogram was predictive of a higher mortality than an abnormal TAPSE alone [27]. As two independent predictors of mortality in PAH, TAPSE, and the degree of tricuspid regurgitation (TR) have been shown to be useful in further risk stratifying patients traditionally considered to be intermediate risk, which may subsequently guide therapy decisions [28^▪▪]. More recently, Moceri et al. demonstrated a prognostic significance of serial assessment of right ventricular area strain in patients with pulmonary hypertension, again underscoring the importance of right ventricular function as a goal for titrating pulmonary hypertension therapies, in addition to the importance of serial assessment [29^▪].

Table 2.

Right heart parameters predictive of mortality in pulmonary arterial hypertension

TAPSE <1.8 cm [25]

TAPSE ≤1.7 cm + dilated IVC [27]

RV end-diastolic volume index ≥84 ml/m²[34^▪]

Stroke volume index ≤25 ml/m²[34^▪]

RV mass index ≥59 g/m²[34^▪]

RA pressure [21,23,24]

RA : PCWP [18]

Cardiac index[21,23]

NT-pro BNP [20,21]

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BNP, brain natriuretic peptide; IVC, inferior vena cava; PAH, pulmonary arterial hypertension; PCWP, pulmonary capillary wedge pressure; RA, right atrium; RV, right ventricle; TAPSE, tricuspid annular planar systolic excursion.

Despite that multiple echocardiographic parameters of right ventricular assessment have been shown to predict outcomes in patients with pulmonary hypertension, echocardiographic assessment is not well represented in our current risk assessment tools. Right atrial area and pericardial effusion are the only echocardiographic parameters of right ventricular function included in contemporary risk calculators. Although these two parameters are included in the European Society of Cardiology/European Respiratory Society (ESC/ERS) risk calculator, the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk calculator includes only pericardial effusion, and REVEAL 2 Lite contains no other representative parameters of right ventricular function [22,30,31]. Pericardial effusion is certainly an important parameter of right ventricular dysfunction. However, its presence is representative of late-stage disease, and patients lacking a pericardial effusion may still carry higher risk based on other echocardiographic parameters. El-Kersh et al. recently demonstrated the use of an echocardiographic risk score for risk stratification in pulmonary hypertension. Four parameters including right ventricular chamber enlargement, right ventricular reduced systolic function, tricuspid regurgitation severity, and pericardial effusion were combined into an echocardiographic risk score based on the REVEAL Registry. Incorporation of these echocardiographic parameters was found to be predictive of survival in PAH [32^▪▪]. Given the importance of right ventricular function in mortality in pulmonary hypertension, incorporation of right ventricular assessment in risk stratification may continue to improve risk stratification tools in the future.

Prognostic information in PAH may also be derived from right ventricular assessment in cardiac MRI. Van Wolferan et al. demonstrated that baseline parameters of right ventricular function, including right ventricular end-diastolic volume, right ventricular mass index, and a low stroke volume are associated with a poor prognosis. In the same study, it was noted that progressive right ventricular dilation and reduced stroke volume at follow-up predicted treatment failure, and a poor long-term outcome [33], highlighting the theme that serial follow-up of right ventricular function in addition to baseline data is critical for prognosis and risk assessment. Further studies have re-emphasized the prognostic significance of cMRI data, and have proven it to be at least equal to risk assessment based on hemodynamic data [34^▪].

CONCLUSION

Accurate assessment of right ventricular function is crucial for multiples facets of assessment of pulmonary hypertension and PAH. Thorough right ventricular assessment may be completed through analysis of both hemodynamic and echocardiographic parameters, and provides insight into the cause of pulmonary hypertension in addition to disease severity. Further, many parameters of right ventricular function are associated with prognosis in pulmonary hypertension, highlighting its importance and supporting the concept of right ventricular function as a target in titrating pulmonary hypertension therapies. Although baseline assessment is crucial, the importance of serial assessment in risk assessment remains an emerging theme.

Acknowledgements

None.

Financial support and sponsorship

None.

Conflicts of interest

There are no conflicts of interest.

REFERENCES AND RECOMMENDED READING

Papers of particular interest, published within the annual period of review, have been highlighted as:

▪ of special interest
▪▪ of outstanding interest

REFERENCES

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[R1] 1▪.Rajagopal S, Ruetzler K, Ghadimi K, et al. on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, and the Council on Cardiovascular and Stroke Nursing. Evaluation and management of pulmonary hypertension in noncardiac surgery: a scientific statement from the American Heart Association. Circulation 2023; 147:1317–1343. [DOI] [PubMed] [Google Scholar]; This scientific statement addresses assessment and management of patients with pulmonary hypertension undergoing noncardiac surgery, and highlights the importance of right ventricular performance in risk assessment.

[R2] 2.Vaidya A, Kirkpatrick J. Otto C. Right ventricular anatomy, function and echocardiographic evaluation. The practice of clinical echocardiography Elsevier Publishing, 5th edAmsterdam: 2016. [Google Scholar]

[R3] 3.Badagliacca R, Papa S, Manzi G, et al. Usefulness of adding echocardiography of the right heart to risk-assessment scores in prostanoid-treated pulmonary arterial hypertension. JACC Cardiovasc Imaging 2020; 13:2054–2056. [DOI] [PubMed] [Google Scholar]

[R4] 4▪▪.Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43:3618–3731. [DOI] [PubMed] [Google Scholar]; Recently published guidelines by ESC/ERS outline recommendations for diagnosis and management of pulmonary hypertension. Throughout the guidelines, right ventricular performance is emphasized as an important factor in assessing the severity of disease and risk assessment, in addition to deciding on further management.

[R5] 5.Fisher MR, Forfia PR, Chamera E, et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179:615–621. [DOI] [PMC free article] [PubMed] [Google Scholar]

[R6] 6.Arkles JS, Opotowsky AR, Ojeda J, et al. Shape of the right ventricular doppler envelope predicts hemodynamics and right heart function in pulmonary hypertension. Am J Respir Crit Care Med 2011; 183:268–276. [DOI] [PubMed] [Google Scholar]

[R7] 7.Raza F, Dillane C, Mirza A, et al. Differences in right ventricular morphology, not function, indicate the nature of increased afterload in pulmonary hypertensive subjects with normal left ventricular function. Echocardiography 2017; 34:1584–1592. [DOI] [PubMed] [Google Scholar]

[R8] 8.Vaidya A, Golbus JR, Vedage NA, et al. Virtual echocardiography screening tool to differentiate hemodynamic profiles in pulmonary hypertension. Pulm Circ 2020; 10:1–10. [DOI] [PMC free article] [PubMed] [Google Scholar]

[R9] 9.Mclaughlin VV, Shah SJ, Souza R, Humbert M. Management of pulmonary arterial hypertension. J Am Coll Cardiol 2015; 65:1976–1997. [DOI] [PubMed] [Google Scholar]

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PERMALINK

Right ventricular assessment in pulmonary hypertension

Lyana Labrada

Anika Vaidy

Anjali Vaidya