Echocardiography in Pulmonary Arterial Hypertension: from Diagnosis to Prognosis

doi:10.1016/j.echo.2012.10.009

Journal of the American Society of Echocardiography

Volume 26, Issue 1, January 2013, Pages 1-14

https://doi.org/10.1016/j.echo.2012.10.009 Get rights and content

Pulmonary arterial hypertension is most often diagnosed in its advanced stages because of the nonspecific nature of early symptoms and signs. Although clinical assessment is essential when evaluating patients with suspected pulmonary arterial hypertension, echocardiography is a key screening tool in the diagnostic algorithm. It provides an estimate of pulmonary artery pressure, either at rest or during exercise, and is useful in ruling out secondary causes of pulmonary hypertension. In addition, echocardiography is valuable in assessing prognosis and treatment options, monitoring the efficacy of specific therapeutic interventions, and detecting the preclinical stages of disease.

Section snippets

Pulmonary Hemodynamics in the Echocardiography Lab

Table 3 lists Doppler echocardiographic indices for the evaluation of patients with clinical suspicion of PH.7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26 Doppler echocardiography enables the reliable estimation of PAP, because in the absence of pulmonary flow obstruction, tricuspid regurgitation (TR) peak velocity (TRV) and RV outflow tract acceleration time have linear positive and negative correlations, respectively, with systolic PAP (SPAP) and MPAP measured by

Echocardiographic Features in Pulmonary Arterial Hypertension

Figure 1 and Table 3 describe echocardiographic features in PAH. Because of chronic RV pressure overload, at the time of diagnosis, most patients present with enlarged right-side chambers, RV hypertrophy, increased interventricular septal thickness, an abnormal interventricular septum/posterior LV wall ratio (>1), and reduced global RV systolic function. Furthermore, the abnormal pressure gradient between the left and right ventricles results in shape distortion and motion of the

Transesophageal Echocardiography

Transesophageal echocardiography should be considered in the following circumstances: (1) to confirm and assess congenital systemic-to-pulmonary shunts, (2) to assess the severity and contribution of mitral valve disease, (3) to characterize a right-sided intracardiac mass not well visualized with transthoracic echocardiography or other imaging techniques, and (4) to guide interventional procedures, such as balloon atrial septostomy.³⁶

Exercise-Induced Pulmonary Hypertension: Looking beyond the Scene

The pulmonary circulation is a high-flow, low-pressure, low-resistance system. PVR is approximately one tenth of comparable systemic values. In healthy subjects, moderate exercise induces mild increases in PAP that are linear with CO and decreases in PVR secondary to the dilation of compliant small vessels and/or the recruitment of additional vessels in the upper portion of normal lungs.41, 42, 43

In elite athletes, substantial increases in PAP have been shown to occur during intense exercise as

Echocardiography and Prognosis

PAH is a progressive disease with a relatively poor prognosis even in the modern era (reported overall survival rates are 87% [95% CI, 84%–90%], 76% [95% CI, 73%–80%], and 67% [95% CI, 63%–71%] at 1, 2, and 3 years, respectively).⁷² The natural history is heterogeneous and influenced mostly by “treatability” and/or “reversibility” of the underlying etiology, with systemic sclerosis and portopulmonary hypertension having the worst and CHD the best prognosis (neonatal right ventricles may also

Nonconventional Echocardiography

The accuracy of conventional two-dimensional (2D) echocardiography in delineating RV structure and function is challenged by several factors, including (1) the asymmetric and complex pyramidal shape of the right ventricle, along with its retrosternal location; (2) limited definition of the endocardial surface; and (2) marked load dependence of many functional indices.83, 84, 85, 86 Emerging ultrasound techniques, namely, Doppler tissue imaging, strain imaging, and real-time three-dimensional

Screening for Pulmonary Arterial Hypertension: The Pivotal Role of Echocardiography

The substantial time delay from symptom onset to definite diagnosis in PAH remains an unresolved issue. This has relevant clinical implications, especially when considering the better prognosis and response to treatment with early detection of the disease (WHO class I or II, 6-min walk distance > 450 m, normal or mildly increased B-type natriuretic peptide, no evidence of right-heart failure). Regular echocardiographic screening of patients at high risk for PAH or with unexplained symptoms of

Conclusions

Echocardiography is a pivotal screening test in symptomatic patients at risk for PAH. As an imaging modality, it has the advantage of being widely available, cost effective, and safe. It also plays an important role in assessing outcomes, monitoring the efficacy of specific therapeutic interventions for PH, and detecting the preclinical stages of disease. Newer ultrasound techniques may provide key additional information in the assessment of right-heart structure and function.

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Right ventricle-pulmonary artery coupling in pulmonary artery hypertension its measurement and pharmacotherapy
2024, Current Problems in Cardiology
The right ventricular (RV) function correlates with prognosis in severe pulmonary artery hypertension (PAH) but which metric of it is most clinically relevant is still uncertain. Clinical methods to estimate RV function from simplified pressure volume loops correlate with disease severity but the clinical relevance has not been assessed. Evaluation of right ventricle pulmonary artery coupling in pulmonary hypertensive patients may help to elucidate the mechanisms of right ventricular failure and may also help to identify patients at risk or guide the timing of therapeutic interventions in pulmonary hypertension. Complete evaluation of RV failure requires echocardiographic or magnetic resonance imaging, and right heart catheterization measurements. Treatment of RV failure in PAH relies on decreasing afterload with drugs targeting pulmonary circulation; fluid management to optimize ventricular diastolic interactions; and inotropic interventions to reverse cardiogenic shock. The ability to relate quantitative metrics of RV function in pulmonary artery hypertension to clinical outcomes can provide a powerful tool for management. Such metrics could also be utilized in the future as surrogate endpoints for outcomes and evaluation of response to therapies. This review of literature gives an insight on RV-PA coupling associated with PAH, its types of measurement and pharmacological treatment.
Machine Learning for Diagnosis of Pulmonary Hypertension by Echocardiography
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To evaluate a machine learning (ML)–based model for pulmonary hypertension (PH) prediction using measurements and impressions made during echocardiography.
A total of 7853 consecutive patients with right-sided heart catheterization and transthoracic echocardiography performed within 1 week from January 1, 2012, through December 31, 2019, were included. The data were split into training (n=5024 [64%]), validation (n=1275 [16%]), and testing (n=1554 [20%]). A gradient boosting machine with enumerated grid search for optimization was selected to allow missing data in the boosted trees without imputation. The training target was PH, defined by right-sided heart catheterization as mean pulmonary artery pressure above 20 mm Hg; model performance was maximized relative to area under the receiver operating characteristic curve using 5-fold cross-validation.
Cohort age was 64±14 years; 3467 (44%) were female, and 81% (6323/7853) had PH. The final trained model included 19 characteristics, measurements, or impressions derived from the echocardiogram. In the testing data, the model had high discrimination for the detection of PH (area under the receiver operating characteristic curve, 0.83; 95% CI, 0.80 to 0.85). The model’s accuracy, sensitivity, positive predictive value, and negative predictive value were 82% (1267/1554), 88% (1098/1242), 89% (1098/1241), and 54% (169/313), respectively.
By use of ML, PH could be predicted on the basis of clinical and echocardiographic variables, without tricuspid regurgitation velocity. Machine learning methods appear promising for identifying patients with low likelihood of PH.
Dynamic Mitral Regurgitation: Scratching Beneath the Surface
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Signal intensity coefficient as a detector of aortic stenosis-induced myocardial fibrosis and its correlation to the long term outcome
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Despite advanced aortic valve replacement techniques, aortic stenosis (AS)-induced irreversible myocardial fibrosis contributes to poorer outcomes. Therefore, in addition to early diagnosis of AS, detecting myocardial fibrosis is crucial for physicians to determine the timing of surgery. The Signal Intensity Coefficient (SIC) was used to detect subtle myocardial deformation. Hence, we aimed to investigate whether SIC correlated with myocardial dysfunction and fibrosis from both clinical and preclinical perspectives.
We collected medical records and echocardiography images, including the SIC of patients who underwent surgical aortic valve replacement (AVR) for AS from 2010 to 2015. The endpoint of the study was mortality. Median follow-up period was 80 months.
Among 109 patients, 15 died due to cardiovascular causes. Although SIC decreased in all patients post-AVR, patients with an SIC ≥0.34 before surgeries presented with a higher probability of cardiovascular death. In contrast, changes in the left ventricular (LV) ejection fraction, LV mass index, and LV volume failed to predict outcomes. Similarly, SIC was obtained in mice undergoing aortic banding and debanding surgery for comparison with the degree of myocardial fibrosis. SIC was continuously elevated after aortic banding and declined gradually after debanding surgery in mice. Debanding surgery indicated the regression of aortic banding-induced myocardial fibrosis.
Pre-AVR SIC was associated with the risk of cardiovascular death and reflected the degree of myocardial fibrosis. Further investigations are required to study the clinical application of SIC in patients with AS.
Prognostic Implication of Pulmonary Hypertension in Low-Flow Low-Gradient Aortic Stenosis After Transcatheter Aortic Valve Replacement
2023, American Journal of Cardiology
Prognostic implications of pulmonary hypertension (PH) in low-flow low-gradient (LG) aortic stenosis (AS) after transcatheter aortic valve replacement (TAVR) remains unexplored. We aimed to investigate the impact of baseline and changes in PH after TAVR. In this single-center retrospective study, we included patients who underwent TAVR for low-flow LG AS. Patients were categorized into 2 groups: baseline pulmonary artery systolic pressure (PASP) <46 mm Hg (no-to-mild PH) and PASP ≥46 mm Hg (moderate-to-severe PH). On the basis of changes in PASP after TAVR, patients were stratified into increased (ΔPASP ≥ + 5 mm Hg), no change (−4 to +4 mm Hg), and decreased (≤ −5 mm Hg) groups. Primary end point was a composite of all-cause mortality and heart failure rehospitalization. In total, 210 patients were included, 148 in the no-to-mild PH group and 62 in the moderate-to-severe PH group. Median follow-up was 13.2 months. The moderate-to-severe PH group was at an increased risk of composite end point (adjusted hazard ratio [HR] 3.5, 95% confidence interval [CI] 1.8 to 6.9), all-cause mortality (HR 2.4, 95% CI 1.1 to 5.6), and heart failure rehospitalization (HR 8.3, 95% CI 2.9 to 23.7). There were no differences in clinical outcomes among those with increased (32%), no change (28%), and decreased (39%) PASP after TAVR. In conclusion, moderate-to-severe PH at baseline is an independent predictor of worse clinical outcomes in patients with low-flow LG AS who undergo TAVR, and this cohort of patients do not seem to derive the benefits of postoperative reduction of PASP.
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The Quality Enhancement Research Initiative (QuERI) in adults with congenital heart disease (ACHD) was developed to improve detection of pulmonary arterial hypertension (PAH) after repair of systemic-to-pulmonary arterial shunt lesions.
This study sought to standardize use of accepted criteria for PAH diagnosis and evaluate utility in at-risk patients with ACHD.
Patients ≥18 years of age with ACHD repaired ≥1 year before enrollment and with additional risk factors for developing PAH were eligible. History, physical examination, electrocardiogram, transthoracic echocardiogram, World Health Organization functional class, and 6-minute walk distance were evaluated at baseline and yearly for 3 years. Pop-up reminders of patient-specific evidence-based recommendations for PAH detection appeared during data entry.
Among 217 eligible patients, mean age (enrollment) was 44.0 ± 15.9 years, 72.3% were women, and 82.0% were World Health Organization functional class I. Electrocardiogram was performed in >80% and TTE in >70% of patients annually; capture of required transthoracic echocardiography (TTE) measures and alignment between study- and core-center interpretation improved over time, with more frequent assessment of pulmonary arterial flow acceleration time and documentation of right ventricular outflow tract Doppler notching. Approximately 40% of patients had ≥2 high-risk features for PAH on TTE, but only 7% (6/82) underwent right heart catheterization (RHC). Using current definitions, 2 patients were confirmed by RHC to have a diagnosis of PAH (maximum follow-up 3 years).
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State-of-the-Art Review ArticleEchocardiography in Pulmonary Arterial Hypertension: from Diagnosis to Prognosis

Section snippets

Pulmonary Hemodynamics in the Echocardiography Lab

Echocardiographic Features in Pulmonary Arterial Hypertension

Transesophageal Echocardiography

Exercise-Induced Pulmonary Hypertension: Looking beyond the Scene

Echocardiography and Prognosis

Nonconventional Echocardiography

Screening for Pulmonary Arterial Hypertension: The Pivotal Role of Echocardiography

Conclusions

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Respir Med

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Heart Fail Clin

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State-of-the-Art Review Article
Echocardiography in Pulmonary Arterial Hypertension: from Diagnosis to Prognosis