Pulmonary Arterial Hypertension: The Key Role of Echocardiography

doi:10.1378/chest.127.5.1836

Chest

Volume 127, Issue 5, May 2005, Pages 1836-1843

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Given the nonspecific nature of its early symptoms and signs, pulmonary arterial hypertension (PAH) is often diagnosed in its advanced stages. Although clinical assessment is essential when initially evaluating patients with suspected PAH, echocardiography is a key screening tool in the diagnostic algorithm. It not only provides an estimate of pulmonary pressure at rest and during exercise, but it may also help to exclude any secondary causes of pulmonary hypertension, predict the prognosis, monitor the efficacy of specific therapeutic interventions, and detect the preclinical stage of the disease.

Section snippets

2D-echo

Because of chronic RV pressure overload, most patients present with enlarged right-side chambers, RV hypertrophy, and reduced global RV systolic function.⁹ This is accompanied by a systolic flattening of the interventricular septum (IVS), with increased thickness and an abnormal IVS/posterior left ventricular (LV) wall ratio (> 1) in response to pressure overload. The LV appears D-shaped with reduced diastolic and systolic volumes, but preserved global systolic function⁹ (Fig 1, top).

Prognostic Implications

The prognosis of PAH is relatively poor and directly related to the severity of right-heart dysfunction.¹²²⁷²⁸²⁹³⁰³¹³²³³³⁴³⁵³⁶³⁷³⁸³⁹⁴⁰⁴¹⁴²⁴³⁴⁴ Together with a number of hemodynamic and noninvasive parameters, the echocardiographic indicators of right-heart impairment (Table 4), which include indexed right atrial area, the degree of septal shift in diastole, a high Doppler RV performance index, and the severity of PE, have been associated with unfavorable outcomes (death or lung transplantation).

Ex-echo

Ex-echo is a reliable means of monitoring PAP during exercise and thus differentiating physiologic and pathologic pulmonary PAP responses.⁷⁶²⁶³⁶⁴⁶⁵ It can be used to assess the pulmonary vascular response to increasing blood flow and may help identify patients with ExPH. In healthy subjects, moderate exercise leads to an increase in stroke volume and a relatively small increase in PAP. Pulmonary vascular resistance falls with exercise as a physiologic response that allows the thin-walled RV to

Future Directions

The extensive application of new echocardiographic methods (pulsed-wave tissue Doppler imaging, contrast, tridimensional, and intracardiac echocardiography) may open up new horizons in the noninvasive assessment of RV function and structure in patients with PAH. Systolic and diastolic tissue Doppler imaging-derived velocity profiles of RV free wall and the lateral tricuspid annulus have been related to right chamber hemodynamic and function, and may be useful in detecting RV dysfunction early,

Conclusions

Echocardiography is an essential component of the diagnostic algorithm of PAH insofar as it makes it possible to confirm the diagnosis. It may also help to exclude PH related to congenital systemic-to-pulmonary shunts or secondary to pulmonary venous hypertension, thromboembolic disease or obstructed pulmonary vessels, predict the prognosis, monitor the efficacy of specific therapeutic interventions, and detect the preclinical stage of the disease.

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To assess the prevalence of PHT in patients with BCR-ABL1–negative CMPN and to evaluate impact of PHT on survival during long-term follow-up.
A total of 122 patients with BCR-ABL1–negative CMPN underwent transthoracic echocardiographic (TTE) evaluation at the beginning of study. Patients undergoing PHT on TTE examination were also evaluated by a pulmonologist. Patients were divided into 3 groups. Group A comprised patients with CMPN-related PHT; group B, patients with no PHT; and group C, patients with PHT due to secondary causes. Patients were evaluated again every 3 to 6 months.
PHT was detected in 33 (27%) of 122 patients. Eight (6.5%) had CMPN-related PHT and the remaining 25 (20.5%) had non–CMPN-related PHT. Positivity for JAK2 V617F mutation in the study population was 72.9%. Groups were similar with respect to hematologic parameters and gender. Follow-up times were as follows: median (range) time from diagnosis to TTE and study end were 34 (1-158) months and 107 (16-251) months, respectively, and from TTE to study end was 88 (7-110) months. No significant differences found among the groups in terms of median time from diagnosis to TTE, follow-up, and overall survival.
BCR-ABL1–negative CMPN patients had a lower prevalence of PHT compared to earlier studies. There was no statistically significant difference in median overall survival between patients with or without PHT. This may be because patients with PHT were asymptomatic and PHT was mild. The impact of PHT on survival was negligible.
Effect of membrane insertion for tricuspid regurgitation using immersed-boundary lattice Boltzmann method
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Tricuspid regurgitation is treated by valve repair or replacement. However, these methods have limitations, and alternative treatment methods are therefore required.
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Our results suggest that membrane insertion can form an effective new method for the treatment of tricuspid regurgitation.
Pulmonary Hypertension in HIV
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Human immunodeficiency virus–associated pulmonary arterial hypertension (HIV-PAH) is important to recognize given its association with significant morbidity and mortality. With the introduction of antiretroviral therapy, the focus of disease management has largely shifted from treating immunodeficiency-related opportunistic infections to managing chronic cardiopulmonary complications. Symptoms are nonspecific, and a high index of clinical suspicion is needed to avoid significant delay in the diagnosis of HIV-PAH. Although several viral proteins have been implicated in the pathogenesis of HIV-PAH, the exact mechanism remains uncertain. Further studies are needed to elucidate precise pathogenic mechanisms, early diagnostic tools, and novel therapeutic targets to improve prognosis of this severe complication.
Il est important de reconnaître l’hypertension artérielle pulmonaire (HTAP) associée au virus de l'immunodéficience humaine (VIH) compte tenu de son lien avec une morbidité et une mortalité élevées. L’arrivée des antirétroviraux a fait en sorte que l’attention se porte désormais bien plus sur la prise en charge des complications cardiopulmonaires chroniques que sur le traitement des infections opportunistes imputables à une immunodéficience. Les symptômes étant aspécifiques, l’indice de suspicion clinique doit être élevé pour éviter des retards indus dans le diagnostic de l’HTAP associée au VIH. Bien que plusieurs protéines virales aient été incriminées dans la pathogenèse de cette affection, son mécanisme exact reste nébuleux. Il faudra réaliser de nouvelles études pour élucider les processus pathologiques en cause, pour trouver des outils permettant un diagnostic précoce et découvrir de nouvelles cibles thérapeutiques afin d’améliorer le pronostic de cette grave complication.
Prognostic Value of Right Ventricular Strain Using Speckle-Tracking Echocardiography in Pulmonary Hypertension: A Systematic Review and Meta-analysis
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Right ventricular (RV) strain imaging using speckle-tracking echocardiography (STE) is a quantitative method of assessing RV systolic function that has shown prognostic utility in patients with pulmonary hypertension (PH). However, its prognostic value for a large and mixed PH population remains poorly defined.
A systematic review and meta-analysis was performed using the MedLine, Embase, and Cochrane Library databases for publications reporting the prognostic value of RV strain calculated using 2-dimensional STE in PH patients for the clinical end point of all-cause mortality.
Screening of 687 publications yielded 10 that were included in the meta-analysis, representing data for 1001 PH patients, among whom 76% had pulmonary arterial hypertension with the remainder having a range of PH etiologies. The pooled free wall RV strain was −16.2% (95% confidence interval [CI], −14.3 to −18.1; I² = 94.1%; Q = 102.8; P < 0.001), and the global (free wall and septum) RV strain was −14.5% (95% CI, −12.9 to −16; I² = 84.9%; Q = 20; P < 0.001). There were 193 (18%) deaths (follow-up period range, 7.4 months to 4.2 years). From 6 publications, the pooled unadjusted hazard ratio for a binary cut off RV strain value for the primary outcome was 3.67 (95% CI, 2.82-4.77; P < 0.001; I² = 0; Q = 1.8; P = 0.87), whereas the pooled unadjusted hazard ratio of RV strain as a continuous variable (per 1% change) was 1.14 (95% CI, 1.11-1.8; P < 0.001; I² = 0; Q = 2.0; P = 0.85), and were superior to corresponding values for tricuspid annular systolic plane excursion (1.45; P = 0.071, hazard ratio = 1.00, and P = 0.82, respectively).
RV strain performed using 2-dimensional STE provides important prognostic value within a large and mixed population of PH patients.
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Un examen systématique et une méta-analyse ont été réalisés à partir des bases de données MedLine, Embase et Cochrane Library pour trouver des articles faisant état de la valeur pronostique de la tension VD calculée par STE bidimensionnelle chez des patients atteints d’HP en tant que paramètre d’évaluation clinique de la mortalité toutes causes confondues.
Dix articles sur 687 sélectionnés ont été inclus dans la méta-analyse. Les données dont font état ces articles ont été recueillies auprès de 1 001 patients atteints d’HP, dont 76 % étaient atteints d’hypertension artérielle pulmonaire, le reste des cas relevant d’étiologies variées. La tension VD combinée au niveau de la paroi libre a été de -16,2 % (intervalle de confiance [IC] à 95 % : de -14,3 à -18,1; I² = 94,1 %; Q = 102,8; p < 0,001) et la surcharge VD globale (paroi libre et septum), de -14,5 % (IC à 95 %: de -12,9 à -16; I² = 84,9 %; Q = 20; p < 0,001). Cent quatre-vingt-treize décès (18 %) ont été observés (période de suivi : de 7,4 mois à 4,2 ans). Dans 6 articles, le rapport de risque non ajusté combiné pour la limite binaire de la valeur de la tension VD associé au paramètre d’évaluation principal a été de 3,67 (IC à 95 % : 2,82 à 4,77; p < 0,001; I² = 0; Q = 1,8; p = 0,87), tandis que le rapport de risque non ajusté combiné pour la tension VD en tant que variable continue (par variation de 1 %) a été de 1,14 (IC à 95 % : 1,11 à 1,8; p < 0,001; I² = 0; Q = 2,0; p = 0,85), des valeurs supérieures aux valeurs correspondantes pour l’excursion systolique du plan de l’anneau tricuspide (1,45; p = 0,071, rapport de risque = 1,00 et p = 0,82, respectivement).
La tension VD calculée à l’aide d’une STE bidimensionnelle présente un intérêt pronostique important dans une vaste population mixte de patients atteints d’HP.
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Citation Excerpt :
Thus, inotropic catecholamine stimulation affects both systole and diastole and may enhance diastolic dysfunction, whereas reduced LVEF leads to increased end-systolic volume and prolongs the diastolic phase of the cardiac cycle.33 The principal pathophysiologic mechanisms of RV dysfunction include increased RV preload, increased RV afterload, impaired right coronary artery perfusion, and decreased contractility.34-36 The specific features of RV perfusion and their alterations during increased pulmonary artery pressure are important to understand.
The Prognostic Utility of a Simplified Biventricular Echocardiographic Index of Cardiac Remodeling in Patients with Pulmonary Hypertension
2016, Journal of the American Society of Echocardiography
Right ventricular (RV) remodeling has been associated with outcomes in patients with pulmonary hypertension (PH). However, the additive prognostic significance of RV remodeling and left ventricular (LV) morphology in PH is unclear. The objective of this study was to test the hypothesis that the ratio of RV end-diastolic area to LV end-diastolic area is a biventricular index predictive of outcome in patients with PH.
In total, 139 patients with precapillary PH (mean age, 55 ± 15 years; 75% women) and 22 control subjects (mean age, 40 ± 17 years; 73% women) were studied. The apical four-chamber view was used to measure the RV-to-LV end-diastolic area ratio as an index of biventricular cardiac remodeling. RV free wall and global strain were measured using speckle-tracking echocardiography. The study design was prospective, with all-cause mortality over 5 years predefined as the outcome event.
Patients with PH had significantly larger RV to LV end-diastolic area ratios than normal subjects, as expected (1.06 vs 0.67, P < .0001). There were 72 deaths over 5 years. Using a cutoff value of 0.93, patients with RV-to-LV ratios ≥ 0.93 had significantly higher all-cause mortality (hazard ratio,1.84; 95% CI, 1.14-2.96; P = .019). RV global strain was also significantly associated with survival using a cutoff of ≥−15% (hazard ratio, 1.66; 95% CI, 1.03-2.67; P = .044). In a multivariate analysis, only age and biventricular index were independent predictors of survival among other clinical and echocardiographic features.
The RV-to-LV end-diastolic area ratio is a simplified biventricular echocardiographic index of cardiac remodeling that is predictive of long-term survival in patients with PH.

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Chest

ReviewsPulmonary Arterial Hypertension: The Key Role of Echocardiography

Section snippets

2D-echo

Prognostic Implications

Ex-echo

Future Directions

Conclusions

Chest

J Am Soc Echocardiogr

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Chest

Lancet

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

Am Heart J

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Chest

Am Heart J

Am J Cardiol

Chest

J Am Coll Cardiol

Chest

Chest

Lancet

Am J Cardiol

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

Am Heart J

Prog Cardiovasc Dis

J Am Soc Echocardiogr

ACCP consensus statement: primary pulmonary hypertension

Chest

Electrocardiogram in primary pulmonary hypertension

Eur J Cardiol

Radiologic features in primary pulmonary hypertension [abstract]

Chest

The electrocardiogram in the diagnostic algorithm for primary pulmonary hypertension: the need for clinical correlation [abstract]

Chest

Echocardiographic assessment of overt or latent unexplained pulmonary hypertension

Can J Cardiol

Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension

Circulation

Non-invasive estimation of pulmonary artery systolic pressure with Doppler ultrasound

Br Heart J

Estimation of pulmonary artery pressure by pulsed Doppler echocardiography [abstract]

Circulation

Non invasive evaluation of pulmonary hypertension by a pulsed Doppler technique

Circulation

Reviews
Pulmonary Arterial Hypertension: The Key Role of Echocardiography