Different Patterns of Adaptation of the Right Ventricle to Pressure Overload: A Comparison between Pulmonary Hypertension and Pulmonary Stenosis

doi:10.1016/j.echo.2011.07.016

Journal of the American Society of Echocardiography

Volume 24, Issue 10, October 2011, Pages 1109-1117

https://doi.org/10.1016/j.echo.2011.07.016 Get rights and content

Background

The study was designed to compare RV morphological and functional parameters derived from conventional and myocardial deformation echocardiography in two instances of right heart pressure overload: pulmonary arterial hypertension (PAH) and pulmonary stenosis (PS).

Methods

Sixty-two individuals were included: 22 patients with pulmonary arterial hypertension (PAH), 19 patients with PS and 21 healthy individuals who served as a control group. All patients had clinical evaluation with 6-minute walking test, standard and two-dimensional strain echocardiography and B-type natriuretic peptide evaluation.

Results

At similar levels of pressure overload (RV systolic pressure, 88.2 ± 31.5 vs 73.4 ± 34.9 mm Hg; P = NS) the right ventricles of patients with PS were less dilated (RV end-diastolic diameter, 31.7 ± 3.7 vs 43.7 ± 10.5 mm; P < .001) and performed significantly better than those of patients with PAH (RV strain, −27.4 ± 5.8% vs 16.2 ± 7.9%; RV fractional area change, 51.1 ± 9.2% vs 29.1 ± 11.3%; P < .001). Although some of the RV functional parameters were comparable with those in healthy individuals, strain rate showed lower values, suggesting subclinical longitudinal dysfunction in patients with PS. Myocardial stress biomarkers were correlated with RV systolic pressure only in patients with PAH (r = 0.64, P = .03), not in those with PS (r = 0.22, P = .50).

Conclusions

At similar levels of pressure overload, the right ventricle is less dilated and performs better in patients with PS compared with those with PAH.

Section snippets

Study Population

We analyzed data from 62 individuals: 22 patients with PAH, 19 patients with PS, and 21 healthy individuals who served as a control group. Patients with PAH were enrolled consecutively after the diagnosis of PAH was reached at our institution. Of the 22 patients with PAH, 16 had idiopathic PAH and six patients had PAH related to connective tissue disease (scleroderma). The etiologic diagnosis of PAH was reached using the stepwise approach suggested by the recent European Society of Cardiology

Results

The demographic, clinical, hemodynamic, and BNP data of patients and controls included in the study are presented in Table 1. The three groups had similar ages and gender distribution. All patients had normal LV ejection fractions (P = NS) (Table 2). RVSP was similar between patients with PAH and those with PS (P = NS) and higher than in controls (ANOVA P < .001). Eleven patients with PAH had severe tricuspid regurgitation, whereas none of the patients with PS or controls had severe tricuspid

Discussion

In the present study, we compared different echocardiographic parameters of RV morphology and function as well as functional status and BNP levels between two different conditions causing pressure overload of the right ventricle: PAH and PS. We found that (1) at similar levels of pressure overload, the right ventricles of patients with PS are less dilated and maintain significantly better global and regional function than those of patients with PAH; (2) although some RV functional parameters

Conclusions

At similar levels of pressure overload, the right ventricle is less dilated and performs better in patients with PS compared with those with PAH. This suggests that the right ventricle could activate intrinsic mechanisms that allow it to cope with increased afterload. This in turn would suggest that novel therapies in PAH not only should be aimed at decreasing pulmonary pressure and resistance but should also target the cellular and molecular mechanisms involved in RV dysfunction.

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Pulmonary hypertension (PH) is a pathophysiological condition defined as an increase in mean pulmonary artery pressure of ≥ 25 mm Hg at rest. Although right heart catheterization remains an essential step in the diagnostic algorithm—particularly for pulmonary arterial hypertension—noninvasive multimodality imaging plays an important role in defining the cause, assessing outcome, monitoring the efficacy of specific therapeutic interventions, and detecting the preclinical stage of disease. We review the role of multimodality imaging in the evaluation of PH—including echocardiography, chest computed tomography, cardiovascular magnetic resonance imaging, and nuclear imaging—the choice of which depends on individual patient-based indications provided by the clinical team in accordance with current guidelines, also taking into account institutional expertise, patient safety and convenience, and repeatability. Recent advances in cardiac imaging (3-dimensional echocardiography and strain imaging, tissue characterization, and 18F-fluorodeoxyglucose positron emission tomography) may provide new insights into the pathophysiological characteristics of the right heart–pulmonary circulation unit.
L’hypertension pulmonaire (HP) est une affection physiopathologique définie par l’augmentation de la pression artérielle pulmonaire moyenne de ≥ 25 mm Hg au repos. Bien que le cathétérisme du cœur droit demeure une étape essentielle de l’algorithme diagnostique, particulièrement pour l’hypertension artérielle pulmonaire, l’imagerie multimodale non invasive joue un rôle important dans la définition de la cause, l’évaluation du résultat, la surveillance de l’efficacité des interventions thérapeutiques particulières et la détection de la maladie au stade préclinique. Nous passons en revue le rôle de l’imagerie multimodale dans l’évaluation de la HP, à savoir l’échocardiographie, la tomodensitométrie du thorax, l’imagerie cardiovasculaire par résonance magnétique et l’imagerie nucléaire, dont le choix dépend des indications individuelles de chaque patient fournies par l’équipe clinique conformément aux lignes directrices actuelles, et en tenant compte de l’expertise institutionnelle, de la sécurité et du confort du patient et de la répétabilité. Les avancées récentes dans le domaine de l’imagerie cardiaque (échocardiographie tridimensionnelle et imagerie de déformation, caractérisation tissulaire et tomographie par émission de positons au [¹⁸F]-fluorodésoxyglucose) peuvent offrir de nouvelles perspectives sur les caractéristiques physiopathologiques de l’ensemble de la circulation pulmonaire et du coeur droit.
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Citation Excerpt :
RV pressure overload causes RV hypertrophy and dilation. Jurcut et al.24 demonstrated in their study that patients with PH respond to high afterload differently than in patients with pulmonary valve stenosis. In their study, patients with PH had more dilated right ventricles compared with those with pulmonary valve stenosis.
Pulmonary hypertension (PH) increases right ventricular (RV) pressure, resulting in septal shift and RV dilation. Few echocardiographic measures have been used to evaluate severity and outcomes in children with PH. The aims of this study were to compare the RV to left ventricular (LV) diameter ratio at end-systole (RV/LV ratio) in normal controls and patients with PH, to correlate the RV/LV ratio with invasive hemodynamic measures, and to evaluate its association with outcomes in children with PH.
The RV/LV ratio was compared retrospectively between 80 matched normal controls and 84 PH patients without shunts. Of the patients with PH, 49 children underwent 94 echocardiographic studies and cardiac catheterizations within 48 hours (13 patients had simultaneous measurements). The RV/LV ratio was correlated against hemodynamic measures. Kaplan-Meier curves and a Cox proportional-hazards regression model were used to assess relationships between RV/LV ratio and time until an adverse clinical event (initiation of intravenous prostacyclin therapy, atrial septostomy, death, or transplantation).
RV/LV ratios were lower in controls compared with patients with PH (mean, 0.51 [95% confidence interval, 0.48–0.54] vs 1.47 [95% confidence interval, 1.25–1.70], P < .01). The RV/LV ratio correlated significantly with mean pulmonary artery pressure, systolic pulmonary artery pressure, systolic pulmonary artery pressure as a percentage of systemic pressure, and pulmonary vascular resistance index (r = 0.65 [P < .01], r = 0.6 [P < .01], r = 0.49 [P < .01], and r = 0.43 [P < .01], respectively). Twenty-two patients with PH with RV/LV ratios > 1 had adverse events within a median of 1.1 years from their earliest echocardiographic studies. Increasing RV/LV ratio was associated with an increasing hazard for a clinical event (hazard ratio, 2.49; 95% confidence interval, 1.92–3.24).
The RV/LV end-systolic diameter ratio can easily be obtained noninvasively in the clinical setting and can be used in the management of patients with PH. The RV/LV ratio incorporates both pathologic septal shift and RV dilation in children with PH and correlates with invasive measures of PH. An RV/LV ratio > 1 is associated with adverse clinical events.

View all citing articles on Scopus

: This work was supported by grant ID_246/2008 from the Romanian National Research Programme II and by a research grant from the European Association of Echocardiography (M.-S.A.).

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Clinical InvestigationVentricular FunctionDifferent Patterns of Adaptation of the Right Ventricle to Pressure Overload: A Comparison between Pulmonary Hypertension and Pulmonary Stenosis

Background

Methods

Results

Conclusions

Section snippets

Study Population

Results

Discussion

Conclusions

Int J Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Am Soc Echocardiogr

Chest

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Thorac Cardiovasc Surg

J Am Coll Cardiol

Additional predictive value of both left and right ventricular ejection fractions on long-term survival in idiopathic dilated cardiomyopathy

Eur Heart J

Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension

Eur Heart J

Right ventricular function in cardiovascular disease, part I: anatomy, physiology, aging, and functional assessment of the right ventricle

Circulation

The echocardiographic assessment of the right ventricle: what to do in 2010?

Eur J Echocardiogr

Right ventricular function and failure: report of a National Heart, Lung, and Blood Institute working group on cellular and molecular mechanisms of right heart failure

Circulation

Ventricular performance in congenital heart disease

Circulation

Current options, and long-term results for interventional treatment of pulmonary valvar stenosis

Cardiol Young

Exercise testing in children with pulmonary valvar stenosis

Pediatr Cardiol

Prevalence of congenital heart defects in newborns in Germany: results of the first registration year of the PAN Study (July 2006 to June 2007)

Klin Padiatr

Clinical Investigation
Ventricular Function
Different Patterns of Adaptation of the Right Ventricle to Pressure Overload: A Comparison between Pulmonary Hypertension and Pulmonary Stenosis