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Objective: The aim of this study was to assess right atrial (RA) function, including RA phase strain, via speckle-tracking echocardiography (STE) in a cohort of systemic lupus erythematosus (SLE) patients with pulmonary arterial hypertension (PAH) and in particular to explore the relationship between RA phase strain and the occurrence of cardiovascular events., Methods: STE analyses of RA function were evaluated in patients with SLE-PAH and in 33 healthy control subjects. Clinical associations, serum biomarkers, echocardiographic data, survival times, and adverse cardiovascular events were evaluated., Results: A total of 66 patients with SLE-PAH were enrolled; they were divided into two groups based on the occurrence of adverse clinical events. RA phase strain was significantly reduced in patients with events than in patients without events. The endpoint was defined as the combined outcome of all-cause mortality, right heart failure, and rehospitalization due to disease progression. During a mean follow-up of 17.2 ± 9.9 months, 23 patients (35%) reached the endpoint. Compared with patients with RA reservoir strain (RASr) ≥33.45%, patients with RASr < 33.45% had more adverse long-term outcomes (log rank p < .0001). RASr was independently associated with adverse clinical outcomes according to multivariate analysis (p = .010)., Conclusion: Our data suggest that RA function has prognostic value for SLE-PAH patients, and strain analysis revealed that the worse the RA function is, the worse the prognosis., (© 2024 Wiley Periodicals LLC.)

Background: Accurately stratifying patients with pulmonary arterial hypertension (PAH) is very important, and traditional risk scores still have internal heterogeneity. This study aimed to construct a risk stratification model that can accurately identify clinical worsening (CW) events in conventional low-intermediate risk patients with pulmonary hypertension under targeted drug treatment by using echocardiographic parameters., Methods: This study is a single-center, prospective study, including 105 PAH patients who underwent regular follow-up at Guangdong Provincial People's Hospital from October 2021 to April 2023. The primary endpoint was the occurrence of CW, including death, hospitalization due to pulmonary hypertension, escalation of targeted drug therapy, and worsening of PAH. The predictive value of the echocardiography-based three-strata risk model was assessed using Kaplan-Meier curves and COX regression analysis., Results: A total of 98 PAH patients were ultimately included in this study. The median follow-up duration was 26 months (range 7-28 months). The echocardiography-based three-strata model included the ratio of tricuspid annular plane systolic excursion and pulmonary artery systolic pressure (TAPSE/PASP) and inferior vena cava (IVC). The echocardiography-based three-strata model had higher diagnostic value (C-index = .76) compared to the 2022 ESC/ERS three-strata model and four-strata model (C-index = .66 and C-index = .61, respectively). PAH patients with lower TAPSE/PASP and wider IVC showed a higher CW rate compared to patients with higher TAPSE/PASP and normal IVC (HR = 15.1, 95%CI:4.4-51.9, p < .001)., Conclusion: The echocardiography-based three-strata model based on TAPSE/PASP and IVC can effectively improve the stratification of low-intermediate risk PAH patients under targeted treatment., (© 2024 Wiley Periodicals LLC.)

Background: Systemic Sclerosis (SSc), an intricate autoimmune disease causing tissue fibrosis, introduces cardiovascular complexities, notably pulmonary hypertension (PH), affecting both survival and quality of life. This study centers on evaluating echocardiographic parameters and endothelial function using flow-mediated dilatation (FMD) in SSc patients, aiming to differentiate those with and without pulmonary arterial hypertension (PAH). The emphasis lies in early detection, given the heightened vulnerability of the right ventricle (RV) in the presence of PH., Methods: Fifty-nine SSc patients and 48 healthy subjects participated, undergoing clinical examinations, echocardiography, FMD assessments, blood analyses, and right heart catheterization (RHC) according to the ESC/ERS guidelines for diagnosis and treatment of PH., Results: SSc-PAH patients displayed lower FMD, higher frequency of TAPSE < 18 mm, RA area > 18 cm 2 , act RVOT < 105 ms and TRV > 280 cm/s compared to those without PAH and healthy controls. Resting resistivity index (RI) was higher in SSc patients, with no significant difference between those with and without PAH. Lower FMD% serves as a predictive marker for adverse cardiovascular outcomes in both SSc and SSc-PAH patients. Stratification by TRV levels and PAH presence reveals notable FMD% variations, emphasizing its potential utility., Conclusions: Early identification of endothelial dysfunction and impaired RV echocardiographic parameters, such as TAPSE and TRV, could aid in predicting right ventricular dysfunction and PAH in SSc patients., (© 2024 Wiley Periodicals LLC.)

Purpose: Chronic thromboembolic pulmonary disease (CTEPD) can lead to exercise limitations even without right ventricular (RV) dysfunction or pulmonary hypertension at rest. Combining exercise stress echocardiography with cardiopulmonary exercise testing (ESE-CPET) for RV function and pressure changes combined measuring overall function may be useful for CTEPD evaluation. This study aims to investigate CPET and ESE results to elucidate the mechanisms of exercise limitation in mild CTEPD cases., Methods: Among our CTEPD registry, 50 patients who performed both right heart catheterization data of mild disease (less than 30 mm Hg of mean pulmonary arterial pressure (mPAP)) and ESE-CPET were enrolled. Echocardiography and CPET-derived parameters were compared with hemodynamic parameters measured through right heart catheterization., Results: Peak VO 2 (maximal oxygen consumption) was decreased in overall population (71.3 ± 16.3% of predictive value). Peak VO 2 during exercise was negatively correlate with mPAP and pulmonary vascular resistance at rest. A substantial increase in RV systolic pressure (RVSP) was observed during exercise (RVSP: pre-exercise 37.2 ± 11.8 mm Hg, postexercise 64.3 ± 24.9 mm Hg, p-value < .001). Furthermore, RV function deteriorated during exercise when compared to the baseline (RV fractional area change: 31.5 ± 10.0% to 37.8 ± 7.0%, p-value < .001; RV global longitudinal strain: -17.1 ± 4.2% to -17.7 ± 3.3%, p-value < .001) even though basal RV function was normal. While an excessive increase in RVSP during exercise was noticed in both groups, dilated RV and RV dysfunction during exercise were demonstrated only in the impaired exercise capacity group., Conclusion: CTEPD patients with mild PH or without PH exhibited limited exercise capacity alongside an excessive increase in RVSP during exercise. Importantly, RV dysfunction during exercise was significantly associated with exercise capacity. ESE-CPET could aid in comprehending the primary cause of exercise limitation in these patients., (© 2024 Wiley Periodicals LLC.)

Background: Myocardial fibrosis can result in right ventricular (RV) dysfunction, a critical factor in poor clinical outcomes and high mortality rates among patients with pulmonary hypertension (PH). Decreased RV myocardial strain rates have been reported in PH patients. The expression of FOXO3A may play a crucial role in myocardial fibrosis; however, the relationship between myocardial fibrosis, speckle-tracking echocardiography (STE), and the transcription factor FOXO3A remains unclear. This study aimed to explore the relationship between the molecular mechanisms of myocardial fibrosis and noninvasive ultrasound evaluation indices to provide a reliable molecular foundation for the early diagnosis of right heart dysfunction in clinical settings., Methods: A progressive right heart failure (RHF) rat model was established through subcutaneous injections of monocrotaline. Rats were divided into baseline, 2-week, 4-week, and 6-week groups based on the disease course. RV structure, function, and myocardial strain were assessed via echocardiography. Myocardial fibrosis severity was determined using PSR staining. The correlation between myocardial strain and RV myocardial fibrosis was analyzed. FOXO3A, collagen I, collagen III, and BNP expressions were tested using western blotting., Results: As the disease progressed, the right ventricle significantly expanded, and the RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), RV global longitudinal strain (RVLS global), and RV free wall longitudinal strain (RVLS FW) gradually declined. However, the reductions in RVLS global and RVLS FW occurred earlier than that of RVFAC, TAPSE. Significant correlations were observed between RVLS global, RVLS FW, and collagen deposition. FOXO3A expression gradually decreased with disease progression, while BNP, collagen I, and collagen III expressions gradually increased., Conclusions: Decreases in RVLS global and RVLS FW in RHF rats occurred earlier than RVFAC and were associated with RV myocardial fibrosis. Furthermore, FOXO3A may have a protective role in the process of RV myocardial fibrosis., (© 2023 The Authors. Echocardiography published by Wiley Periodicals LLC.)

Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) may occur as a severe complication of the disease. Herein, we report a patient diagnosed with fibrosing mediastinitis and pulmonary hypertension, whose imaging tests showed obstruction of right pulmonary artery and veins., (© 2022 Wiley Periodicals LLC.)

Objectives: We sought to determine the cardiac magnetic resonance (CMR) indicators of intermediately to highly probable pulmonary hypertension (IHpPH) in patients with thalassemia referred for myocardial iron overload assessments to prevent further cardiac complications., Methods: The study population consisted of 152 patients with thalassemia (major or intermedia) (49.3% women, mean age = 33 ± 10.1 years) who underwent non-contrast CMR and echocardiographic examinations on the same day. Functional, T2*, and global strain parameters via a feature-tracking method were extracted from CMR. The probability of PH was defined based on the tricuspid regurgitation velocity and echocardiographic parameters. The catheterization-derived hemodynamic data of patients with moderate to high probable PH was registered., Results: Twenty-two (14.5%) patients suffered from IHpPH. The multivariate logistic regression analysis revealed that the right ventricular end-systolic volume index (RVESVI) was the strongest of all the CMR parameters for the prediction of IHpPH (OR: 1.044, 95% CI: 1.021-1.067). The other powerful IHpPH predictor was age (OR: 1.066, 95% CI: 1.009-1.126). A cutoff point of greater than 47 ml for RVESVI (AUC: .801, 95% CI: .728-.861) was found to predict IHpPH with 73.91% sensitivity and 70.31% specificity. The single most robust CMR-derived strain parameter for IHpPH prediction was the right ventricular global longitudinal strain (OR: .887, 95% CI: .818-.961). A p value of less than 0.05 was considered significant., Conclusions: Both CMR functional and global strain parameters were strong predictors of IHpPH in our patients with thalassemia., (© 2021 Wiley Periodicals LLC.)

Background: Identification of elevation in pulmonary pressures during exercise may provide prognostic and therapeutic implications in patients with connective tissue disease (CTD). Interstitial lung disease (ILD) is common in CTD patients and subtle interstitial abnormalities detected by lung ultrasound could predict exercise-induced pulmonary hypertension (PH)., Methods and Results: Echocardiography and lung ultrasound were performed at rest and bicycle exercise in CTD patients (n = 41) and control subjects without CTD (n = 24). Ultrasound B-lines were quantified by scanning four intercostal spaces in the right hemithorax. We examined the association between total B-lines at rest and the development of exercise-induced PH during ergometry exercise. Compared to controls, the number of total B-lines at rest was higher in CTD patients (0 [0, 0] vs 2 [0, 9], P < .0001) and was correlated with radiological severity of ILD assessed by computed tomography (fibrosis score, r = .70, P < .0001). Pulmonary artery systolic pressure (PASP) was increased with ergometry exercise in CTD compared to controls (48 ± 14 vs 35 ± 13 mm Hg, P = .0006). The number of total B-lines at rest was highly correlated with higher PASP (r = .52, P < .0001) and poor right ventricular pulmonary artery coupling (tricuspid annular plane systolic excursion/PASP ratio, r = -.31, P = .01) during peak exercise. The number of resting B-lines predicted the development of exercise-induced PH with an area under the curve .79 (P = .0003)., Conclusions: These data may suggest the value of a simple resting assessment of lung ultrasound as a potential tool for assessing the risk of exercise-induced PH in CTD patients., (© 2021 Wiley Periodicals LLC.)

Introduction: The use of echocardiography to evaluate the probability for pulmonary hypertension (PH) in pregnant women has not been reported or correlated with outcomes. We hypothesized that in women with elevated right ventricular systolic pressure (RVSP) on echocardiography first identified during pregnancy, those with low probability for PH would have fewer major adverse cardiac events (MACE)., Methods: We performed a retrospective cohort study of pregnant women with RVSP >35 mm Hg on echocardiogram first identified during pregnancy. Women were classified as intermediate-high probability for PH (HP) or low probability for PH (LP) based on simplified European Society of Cardiology echocardiographic criteria. Maternal cardiac, obstetric, and fetal outcomes were assessed., Results: A total of 77 women met inclusion criteria (mean age 30 ± 5 years), with 45 (58%) classified as HP and 32 (42%) as LP. There were 21 (27%) women who experienced MACE, more commonly in the HP cohort (HP 18 (40%) women vs. LP 3 (9%) women, P = .01). The echocardiographic criteria for intermediate-high probability of PH identified women at risk for MACE with 85% sensitivity and 52% specificity. The negative predictive value for MACE in women meeting low echocardiographic probability for PH criteria was 91%., Conclusions: In women with elevated RVSP on echocardiography first identified during pregnancy, those with low echocardiographic PH probability are at significantly lower risk for MACE during pregnancy, though the risk is not eliminated. This may be useful to risk stratify pregnant women with suspected PH, guiding tertiary care referral and invasive catheterization., (© 2021 Wiley Periodicals LLC.)

Background: Pericardial effusion drainage in patients with significant pulmonary hypertension (PH) has been questioned because of hemodynamic collapse concern, mainly because of right ventricular (RV) function challenging assessment. We aimed to assess RV function changes related to pericardiocentesis in patients with and without PH., Methods: Consecutive patients with symptomatic moderate-to-large pericardial effusion who had either echocardiographic or clinical signs of cardiac tamponade and who underwent pericardiocentesis from 2013 to 2018 were included. RV speckle-tracking echocardiography analysis was performed before and after pericardiocentesis. Patients were stratified by significant PH (pulmonary artery systolic pressure [PASP] ≥50 mm Hg)., Results: The study cohort consisted of 76 patients, 23 (30%) with PH. In patients with PH, both end-diastolic and end-systolic areas (EDA, ESA) increased significantly after pericardiocentesis (22.6 ± 8.0 cm 2 -26.4 ± 8.4 cm 2 , P = .01) and (15.9 ± 6.3 cm 2 -18.7 ± 6.5 cm 2 , P = .02), respectively. However, RV function indices including fractional area change (FAC: 30.6 ± 13.7%-29.1 ± 8.8%, P = .61) and free-wall longitudinal strain (FWLS: -16.7 ± 6.7 to -15.9 ± 5.0, P = .50) remained unchanged postpericardiocentesis. In contrast, in the non-PH group, after pericardiocentesis, EDA increased significantly (20.4 ± 6.2-22.4 ± 5.9 cm 2 , P = .006) but ESA did not (14.9 ± 5.7 vs 15.0 ± 4.6 cm 2 , P = .89), and RV function indices improved (FAC 27.9 ± 11.7%-33.1 ± 8.5%, P = .003; FWLS -13.6 ± 5.4 to -17.2 ± 3.9%, P < .001)., Conclusion: Quantification of RV size and function can improve understanding of echocardiographic and hemodynamic changes postpericardiocentesis, which has the potential to guide management of PH patients with large pericardial effusion., (© 2021 Wiley Periodicals LLC.)

Background: Right ventricular (RV) function is a prognostic marker of chronic thromboembolic pulmonary hypertension (CTEPH). We used two-dimensional (2D) speckle-tracking echocardiography (STE) to evaluate the therapeutic effects of balloon pulmonary angioplasty (BPA) in CTEPH patients., Methods: A total of 46 CTEPH patients who underwent 2D STE before and after BPA were enrolled in this retrospective study. The following RV functional parameters were measured: tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RVFAC), RV index of myocardial performance (RIMP), and free wall longitudinal strain (RVFWLS). Satisfactory BPA was defined as mean pulmonary arterial pressure (mPAP) <25 mm Hg or improvement in mPAP > 10 mm Hg after BPA. Patients were divided into two groups according to mPAP values: group I had satisfactory BPA outcomes; group Ⅱ had unsatisfactory BPA outcomes. The area under the curve (AUC) of the receiver operating characteristic (ROC) curve was used to determine the optimal cutoff values and the ability of RVFWLS to predict successful BPA outcomes., Results: After BPA, SPAP measured by echocardiography (SPAP echo ) and RIMP decreased, but TAPSE, RVFAC, and RVFWLS increased. Before BPA, group Ⅰ had significantly better RV function than group Ⅱ. Multifactor logistic regression analysis identified RVFWLS as an independent factor associated with satisfactory BPA outcomes. The optimal cutoff value for RVFWLS in predicting satisfactory BPA outcomes was -12.2%., Conclusions: Balloon pulmonary angioplasty improves RV function in CTEPH patients. RVFWLS is a valuable noninvasive tool with which to assess the treatment effects of BPA. CTEPH patients with lower RVFWLS may have limited benefit from BPA., (© 2020 Wiley Periodicals LLC.)

Objectives: Right ventricular (RV) function is identified as a key determinant of the outcome in patients with pulmonary hypertension (PH). Several studies have assessed the role of peak global longitudinal RV strain in PH patients; however, less emphasis was given to the RV regional longitudinal strain. The aim of this study was to evaluate the regional RV systolic strain in PH patients and investigate the relationship of these parameters with the severity of PH., Methods: RV regional longitudinal peak systolic strain (LPSS) and strain rate (LPSSR) were measured using speckle tracking echocardiography on 100 patients with PH who underwent right heart catheterization, and 29 control subjects. Severe PH was identified by a decreased cardiac index (CI) (<2.0 L/min/m 2 )., Results: LPSS and LPSSR of the RV free wall were significantly lower in PH patients than control subjects, especially when comparing the basal and mid regions (P < .001). When comparing severe PH and nonsevere PH, basal and mid LPSS and LPSSR were significantly lower (P < .001). RV free wall mid LPSSR correlated with CI (r = -.703, P < .001). In the multiple logistic regression analysis, mid LPSSR was identified as an independent predictor of severe PH (odds ratio 1.82; 95% confidential interval 1.39-2.40; P < .001). In the receiver operating characteristics curve analysis, a cutoff value of mid LPSSR of -0.92 s -1 predicted severe PH, with a sensitivity and specificity of 75.0% and 93.7%, respectively (AUC = 0.889, P < .001)., Conclusions: RV free wall mid longitudinal peak systolic strain rate may be useful for the detection of severely impaired RV performance in PH., (© 2020 Wiley Periodicals, Inc.)

Long-standing left-to-right shunting across a patent ductus arteriosus (PDA) can result in Eisenmenger syndrome. In this report, we present echocardiographic findings of a 27-year-old female patient with pulmonary hypertension. In diagnostic work-up especially Doppler findings of the pulmonary artery suggested the presence of a reversed PDA as a cause of pulmonary hypertension. The diagnosis was confirmed by contrast study and computed tomography., (© 2020 Wiley Periodicals, Inc.)

Objective: To evaluate whether global peak systolic strain (PSS) and peak systolic strain rate (PSSR) derived from velocity vector imaging (VVI) allow early recognition of regional and global right ventricular (RV) dysfunction and the impact of this on left ventricular (LV) function in patients with pulmonary hypertension (PHT)., Background: RV function is an important determinant of prognosis in patients with heart failure, pulmonary hypertension, heart transplant, and congenital heart diseases. However, evaluation of the right ventricle is often limited by its complex geometry and inadequate visualization of RV free wall. Furthermore, the impact of RV dysfunction on the LV function is not well elucidated., Methods: Ninety-nine participants, 35 control patients with normal RV systolic pressure (RVSP) (<30 mm Hg) and 64 patients with PHT (25 with mild-to-moderate increase in RVSP [≥36 and <60 mm Hg] and 39 with severe increase in RVSP [≥60 mm Hg]), underwent comprehensive echo-Doppler assessment and velocity vector imaging (VVI) for strain rate analysis. RV regional peak systolic and diastolic tangential velocity, strain, and strain rate were obtained from the basal, mid and apical segments of the RV free wall and interventricular septum (IVS) from apical 4-chamber view at end-expiration. Similar data were obtained from eighteen LV segments from apical 4-chamber, 2-chamber, and long-axis views., Results: Peak systolic strain, strain rate, and tangential velocity at all segments in the RV free wall and IVS were decreased compared to controls in patients with PHT (P < 0.001). Significant correlation (r > 0.60; P < 0.001) was noted between RVSP and systolic and diastolic strain and strain rate at basal segment in IVS and global RV function. Peak early diastolic strain rate at all segments was also decreased in PHT patients compared with control patients (P < 0.01). Furthermore, RV systolic and diastolic strain and strain rate were lower in group 2 with mild-to-moderate hypertension while the conventional echo parameters were normal. Except for IVS segments, other LV segments had no statistical differences in systolic and diastolic velocity, strain, and strain rate compared to controls. However, they were lower than the published normal range., Conclusions: Strain and strain rate derived from VVI demonstrates early recognition of systolic and diastolic RV dysfunction in patients with PHT compared to controls. PHT is associated with global and regional RV systolic and diastolic dysfunction. Systolic and diastolic strain and strain rate from LV was lower compared to controls but were not statistically significant. This may indicate subclinical LV dysfunction in these patients, suggesting that conventional LV function parameters may not be sensitive to recognize subclinical LV dysfunction., (© 2019 Wiley Periodicals, Inc.)

Objectives: Right ventricular (RV) contractile reserve reflects the ability of RV to accommodate the increased afterload and may play an essential role in the evaluation of precapillary pulmonary hypertension (PH). This study aimed to assess RV contractile reserve based on exercise stress echocardiography (ESE) and to determine the echocardiographic determinants of exercise capacity in patients with precapillary PH., Methods: A total of 31 patients with precapillary PH and 15 age- and sex-matched healthy control subjects were prospectively recruited. All subjects underwent ESE to assess RV function at rest and under peak exercise. Changes in these parameters during exercise were calculated to quantify the RV contractile reserve. Patients with precapillary PH also underwent cardiopulmonary exercise test (CPET), and data pertaining to peak oxygen uptake (peak VO 2 ) and minute ventilation/carbon dioxide production (VE/VCO 2 ) were collected., Results: Right ventricular contractile reserve including change in tricuspid annular plane systolic excursion (∆TAPSE), change in RV fractional area change (∆RVFAC), and change in Doppler-derived tricuspid lateral annular peak systolic velocity (∆S') was significantly depressed in precapillary PH patients compared with control subjects (P < 0.05). Parameters of RV function and RV contractile reserve were markedly associated with maximal exercise capacity (P < 0.05). ∆RVFAC was an independent predictor of peak VO 2 (r 2  = 0.601, P < 0.05)., Conclusions: Assessment of RV contractile reserve facilitates identification of subclinical dysfunction and evaluation of clinical status and severity of precapillary PH. ESE as a noninvasive method may provide a comprehensive clinical assessment and facilitate therapeutic decision-making for these patients., (© 2019 Wiley Periodicals, Inc.)

Objective: Pulmonary hypertension (PH) impairs right ventricular (RV) systolic and diastolic function, which in turn induces compensatory changes in right atrial (RA) function; the diverse effects on RA function are subject to much debate. We hypothesized that RA function plays a more important role in compensating RV dysfunction, than mere prevention of clinical failure in patients with PH., Methods: We studied 54 patients with PH and 23 healthy controls. RA volume, including maximum RA volume, minimum RA volume, and the volume before atrial systole, was evaluated by 3DE. RA maximum volume index (V max I), total emptying volume index (TotEVI), passive emptying volume index (PassEVI), and active ejection fraction (ActEF) were calculated. Receiver operating characteristic curve analysis was used to determine the sensitivity and specificity of various cutoff levels of the variables measured for predicting World Health Organization functional class (WHO-FC) IV in patients with PH., Results: RAV max I in patients with PH was higher than that in controls. In patients with PH, the TotEVI was significantly higher, while PassEVI was significantly lower as compared to that in controls. ActEF was increased in patients with WHO functional class (WHO-FC) III PH as compared to that in controls (P = .003) but was reduced in more advanced cases (WHO-FC IV). In addition, the area under the curve of 3D RA ActEF was larger than those of 2D RA ActEF, RA GLS, RA area, FAC, TAPSE, and RIMP (P < .01 for all) for predicting WHO-FC IV., Conclusion: We demonstrated that RA function plays a more important role in compensating RV dysfunction then mere prevention of clinical failure in PH., (© 2017, Wiley Periodicals, Inc.)

A 7-year-old girl with severe mitral regurgitation and pulmonary artery hypertension with good ventricular function was planned for mitral valve repair, but was subsequently diagnosed to have anomalous left coronary artery from pulmonary artery. We describe the pitfalls in the diagnosis of this rare anomaly, particularly in the presence of severe pulmonary artery hypertension and absence of circumstantial evidence of coronary steal and ventricular dysfunction., (© 2017, Wiley Periodicals, Inc.)

A 71-year-old male with a medical history of hypertension was admitted to emergency department with hypertensive pulmonary edema. The patient appeared anxious and diaphoretic, and physical examination revealed tachypnea, crepitant rales in the both lungs and a systolic ejection murmur at the right sternal border, radiating to the both carotid arteries. The electrocardiography showed sinus tachycardia with indications of left ventricle hypertrophy Before the aortic valve surgery, transesophageal echocardiography (TEE) was performed for detailed evaluation of the aortic valve. Two-dimensional TEE showed mild tricuspid valve regurgitation and only two of three leaflets of the tricuspid valve at a time., (© 2017, Wiley Periodicals, Inc.)

Background: The assessment of hemodynamic change by echocardiography is clinically useful in patients with pulmonary hypertension. Recently, mild elevation of the mean pulmonary arterial pressure (PAP) has been shown to be associated with increased mortality. However, changes in the echocardiographic indices of right ventricular (RV) function are still unknown. The objective of this study was to validate the relationship between echocardiographic indices of RV function and right heart catheterization variables under a mild RV pressure overload condition., Methods and Results: Echocardiography and right heart catheterization were performed in dog models of mild RV pressure overload induced by thromboxane A 2 analog (U46619) (n=7). The mean PAP was mildly increased (19.3±1.1 mm Hg), and the cardiac index was decreased. Most echocardiographic indices of RV function were significantly impaired even under a mild RV pressure overload condition. Multivariate analysis revealed that the RV free wall longitudinal strain (RVLS), standard deviation of the time-to-peak longitudinal strain of RV six segments (RV-SD) by speckle-tracking echocardiography, and Tei index were independent echocardiographic predictors of the mean PAP (free wall RVLS, β=-0.60, P<.001; RV-SD, β=0.40, P=.011), pulmonary vascular resistance (free wall RVLS, β=-0.39, P=.020; RV-SD, β=0.47, P=.0086; Tei index, β=0.34, P=.047), and cardiac index (Tei index, β=-0.65, P<.001)., Conclusions: Free wall RVLS, RV-SD, and Tei index are useful for assessing the hemodynamic change under a mild RV pressure overload condition., (© 2017, Wiley Periodicals, Inc.)

Pulmonary arterial pressure is an important index in cardiovascular disorders, especially for pulmonary hypertension (PH). Doppler echocardiography (DE) is widely used as a noninvasive method to assess pulmonary arterial pressure. However, recent studies have found several hemodynamic factors that affect its accuracy in estimating systolic pulmonary arterial pressure (sPAP). But the effect of tricuspid regurgitation (TR) has not been investigated. Therefore, our study is aimed to determine whether the severity of TR will affect the accuracy of sPAP measured by DE in an unselected patient population. We retrospectively studied 177 patients who underwent DE and right heart catheterization (RHC) examinations. Patients were categorized into 3 groups according to the severity of TR (mild, moderate, and severe). The discrepancy in sPAP measured by DE and RHC was calculated and compared in each group. Determinants of discordant results between two methods were also evaluated. Age, gender, interval between DE and RHC, sequence of DE and RHC were similar among groups (all P>.05). Differences in sPAP, RAP, and tricuspid regurgitation pressure gradient (TR-PG) were similar in group 1 and 2 (all P>.05), while all significantly higher in group 3 (all P<.05). The difference in sPAP between DE and RHC was affected independently by severe TR and severe PH (both P<.05). Severe TR and severe PH affect the accuracy of sPAP measured by DE. Modification of echocardiographic sPAP measurements by taking into consideration of these factors may lead to reduced systemic errors., (© 2017, Wiley Periodicals, Inc.)

Background: Even though the tricuspid annulus (TA) is anatomically and functionally related to right atrioventricular dependence and tricuspid annular plane systolic excursion (TAPSE) is a well-known measure of right ventricular (RV) systolic function, there is paucity of data regarding the potential impact that right atrial (RA) size and function have on TAPSE. Hence, we sought to determine whether RA volumetric and longitudinal measures affect TAPSE in patients with chronic pulmonary hypertension (cPH)., Methods: In this retrospective study, echocardiographic data from 110 patients were reviewed and the studied population was divided into Group I consisting of 50 patients (50±11 years) without PH and Group II that included 60 patients (55±14 years) with cPH., Results: Even though RA areas, RA fractional area change, and maximal long-axis length measurements were abnormal in cPH patients, TA tissue Doppler imaging systolic velocity and RV fractional area change were the only useful variables in identifying patients with abnormal TAPSE (P<.0001 and P=.018, respectively). Additionally, TAPSE was independent of all RA measures, left ventricular ejection fraction, and age (P>.05)., Conclusions: Based on these results, TAPSE does not appear to be influenced by either RA size or function. Additional studies using strain echocardiography to interrogate RA mechanics might now be very useful to advance our understanding of TA motion in cPH patients given recent data showing that RA function accounts for a significantly greater proportion of total right heart function in patients with cPH than in normal subjects., (© 2017, Wiley Periodicals, Inc.)

Background: Right ventricular (RV) dysfunction is a major determinant of outcomes in patients with pulmonary arterial hypertension (PAH), although the optimal measure of RV function is poorly defined. We evaluated the utility of RV free-wall speckle tracking strain as an assessment tool for RV function in patients with PAH who are already under specific treatment compared with conventional echocardiographic parameters and investigated the relationship of RV free-wall strain with clinical hemodynamic parameters of RV performance., Methods: Right ventricular free-wall strain was evaluated in 92 patients (Group-1 and Group-4 pulmonary hypertension) who were on PAH-specific treatment for at least 3 months. Right atrial (RA) area, RV FAC, TAPSE, tricuspid S, functional class, 6-minute walking distance, and NT-proBNP were studied. The mean duration of follow-up was 222±133 days., Results: All patients were under PAH-specific treatment, and mean RV free-wall strain was -13.16±6.3%. RV free-wall strain correlated well with functional class (r=.312, P=.01), NT-proBNP (r=.423, P=.0001), RA area (r=.427, P=.0001), FAC (r=-.637, P=.0001), TAPSE (r=-.524, P=.0001), tricuspid S (r=-.450, P=.0001), 6-minute walking distance (r=-.333, P=.002). RV free-wall strain significantly correlated with all follow-up adverse events, death, and clinical right heart failure (RHF) (P=.04, P=.03, P=.02, respectively). According to the receiver operator characteristic analysis, the cutoff value for RV free-wall strain for the development of clinical RHF was -12.5% (sensitivity: 71%, specificity: 67%) and for all cardiovascular adverse events (death included) was -12.5% (sensitivity: 54%, specificity: 64%)., Conclusion: Assessment of RV free-wall strain is a feasible, easy-to-perform method and may be used as a predictor of RHF, clinical deterioration, and mortality in patients already under PAH-specific treatment., (© 2017, Wiley Periodicals, Inc.)

Background: Pulmonary arterial hypertension (PAH) is characterized by elevated mean pulmonary arterial pressure with abnormal right ventricular (RV) pressure overload that may alter left ventricular (LV) function. The aim of this study was to assess the impact of RV pressure overload on LV function in PAH patients using two-dimensional (2D) speckle tracking strain., Methods: The study enrolled 37 group 1 PAH patients and 38 age- and gender-matched healthy controls. LV longitudinal and radial 2D strains were measured with and without including the ventricular septum. Six-minute walk test (6MWT) and brain natriuretic peptide (BNP) levels were also obtained in patients with PAH., Results: The mean age of patients was 46.4 ± 14.8 years, 76% women, and 16 patients (43%) had schistosomiasis. Sixteen patients (43%) were in WHO class III or IV under specific treatment for PAH. The overall 6MWT distance was 441 meters, and the BNP levels were 80 pg/mL. Patients with PAH more commonly presented with LV diastolic dysfunction and impairment of RV function when compared to controls. LV global longitudinal and radial strains were lower in patients than in controls (-17.9 ± 2.8 vs. -20.5 ± 1.9; P < 0.001 and 30.8 ± 10.5 vs. 49.8 ± 15.4; P < 0.001, respectively). After excluding septal values, LV longitudinal and radial strains remained lower in patients than in controls. The independent factors associated with global LV longitudinal strain were LV ejection fraction, RV fractional area change, and tricuspid annular systolic motion., Conclusions: This study showed impaired LV contractility in patients with PAH assessed by speckle tracking strain, irrespective of ventricular septal involvement. Global LV longitudinal strain was associated independently with RV fractional area change and tricuspid annular systolic motion, after adjustment for LV ejection fraction., (© 2016, Wiley Periodicals, Inc.)

Objectives: We hypothesized that left atrial deformation during atrial systole (LASRa) correlates with pulmonary capillary wedge pressure (PCWP), thus enabling echo-derived pulmonary vascular resistance (PVR) estimation in a wide range of different subsets of patients., Background: Various etiologies of pulmonary hypertension (PH) have different mechanisms and treatments for breathlessness. Irrespective of the location of the underlying pulmonary vascular pathology, pre- or postcapillary, the resulting PH is fairly easy to assess by Doppler echocardiography, but PVR remains a challenge., Methods: We prospectively included 46 patients (mean age 61 ± 13 years) in sinus rhythm, who underwent right heart catheterization because of dyspnea. According to the NICE guidelines classification, 22 belonged to group 1 pulmonary artery hypertension (PAH), 19 belonged to group 2 congestive heart failure (CHF), 1 belonged to group 4 chronic thromboembolic pulmonary hypertension (CTEPH), and 4 had normal hemodynamics. Simultaneous Doppler echocardiography using spectral, tissue Doppler, and speckle tracking echocardiography techniques for assessing LA structure and function was performed., Results: PCWPrhc correlated with LASRa (r(2) = 0.65, P < 0.001). PCWPecho was calculated using the equation (PCWPecho = 26.12 - 11.09 × LASRa), and the resulting PVR echo strongly correlated with the respective catheter-based measurements PVRrhc (r(2) = 0.69. P < 0.001) with a sensitivity of 85% and specificity of 74% identifying a PVR ≥ 3 WU., Conclusions: Left atrial strain rate during atrial systole correlates closely with pulmonary capillary wedge pressure and consequently the calculated pulmonary vascular resistance, irrespective of the etiology of PH., (© 2016, Wiley Periodicals, Inc.)

Background: Right ventricular (RV) systolic function is one of the most important determinants of outcome for pulmonary hypertension (PH) patients, but the factors influencing prognosis vary widely. Elevated right atrial (RA) pressure is reported to be one of these prognostic factors, but its functional importance has scarcely been assessed., Methods: Eighty-two PH patients, all of whom underwent echocardiography and right heart catheterization, were recruited. RV function was assessed by two-dimensional speckle tracking longitudinal strain from RV-focused apical four-chamber view and calculated by averaging the three regional peak strains from the RV free wall (RV-free). RA function was determined as the sum of three peak strain values comprising reservoir, conduit, and contractile function (sum of RA strain)., Results: Sum of RA strain correlated significantly with hemodynamic parameters such as mean right atrial pressure (r = -0.35, P = 0.002) and end-diastolic RV pressure (r = -0.29, P = 0.008). Patients with sum of RA strain ≥30.2% experienced more favorable outcomes than those with sum of RA strain <30.2% (log-rank P = 0.001). Furthermore, patients with impaired RV systolic function (RV-free <20%) and RA function (sum of RA strain <30.2%) showed the worst outcome (P = 0.001). A sequential Cox model based on clinical variables (χ(2) = 5.8) was improved by addition of RV-free (χ(2) = 8.7; P < 0.05) and further improved by addition of sum of RA strain (χ(2) = 12.0; P < 0.01)., Conclusion: Right atrial strain appears to be a valuable additive factor for predicting outcomes for PH patients, and comprehensive functional assessment of right-sided heart using speckle tracking strain may have potential clinical implications for better management of PH patients., (© 2016, Wiley Periodicals, Inc.)

Background: Pulmonary hypertension (PH) is a common problem in the neonatal intensive care unit and is associated with significant morbidity and mortality. The aim of this study was to identify a quantitative echocardiographic marker of septal curvature that can be used to accurately identify PH in NICU infants with concern for PH., Methods: Echocardiograms of infants who were prematurely born and infants with persistent pulmonary hypertension of the newborn were performed using a defined protocol for evaluation of PH. Qualitative assessment by a single pediatric cardiologist was used as a reference standard. Qualitative and quantitative parameters of right ventricular (RV) size, pressure, and function were documented. Left ventricular end-systolic eccentricity index (EI) was defined as the ratio of the anterior-inferior and septal-posterolateral cavity dimensions at the mid-ventricular level., Results: A total of 216 infants at risk for PH were included in this study. One hundred forty-three (66%) had an interpretable tricuspid regurgitation jet velocity. While systolic septal flattening was recognized at EIs ≥ 1.15, more than half-systemic RV pressure became apparent at EIs ≥ 1.3. Unlike qualitative assessment of septal flattening, there was high inter-observer agreement for EIs. Quantitative parameters of RV systolic function were impaired only at EIs ≥ 1.3., Conclusions: We suggest that EIs should be incorporated into routine protocols when there is a concern for PH in neonates. This may lead to a more reliable assessment of PH and may reduce inter-observer variability. Correlation of EIs with invasive hemodynamic data is needed to validate our results., (© 2016, Wiley Periodicals, Inc.)

Background: Tricuspid annular plane systolic excursion (TAPSE) has emerged as a reliable marker of right ventricular (RV) systolic function. Recently, TAPSE derived using 2D images (2D-TAPSE) was shown to correlate with M-mode TAPSE (MM-TAPSE). We have developed a novel technique for semiautomatic evaluation of TAPSE (SA-TAPSE). The purpose of this study was to determine the accuracy of this novel technique and validate it on normal hearts and pulmonary hypertension (PH)., Methods: A total of 110 patients (56 with normal heart and 54 with PH) were retrospectively identified for analysis. The semiautomatic algorithm tracked the lateral tricuspid valve hinge point (TVHP) and the apex in the apical 4-chamber view. SA-TAPSE was calculated as displacement of the TVHP in end-diastole (ED) and end-systole (ES). The same points were manually identified to derive 2D-TAPSE., Results: The system was able to accurately identify ED and ES in 304/330 heartbeats within three cardiac frames. The automatically identified TVHP points were within 1.2 ± 0.7 mm from the manually identified points. Intra-class correlation between SA-TAPSE and 2D-TAPSE was 0.96 (95% CI 0.93-0.98) for normal hearts and 0.92 (95% CI 0.87-0.96) for those with PH. Bland-Altman analysis showed a strong agreement between SA-TAPSE and 2D-TAPSE for normal hearts and those with PH., Conclusion: Using the novel custom-made software, SA-TAPSE could be measured in majority of our patients and was accurate when applied to normal hearts and those with PH. Future work will focus on fully automating the system for a rapid retrospective analysis of TAPSE., (© 2016, Wiley Periodicals, Inc.)

Background: Current guidelines recommend the routine use of tricuspid annular plane systolic excursion (TAPSE) as a simple method for estimating right ventricular (RV) function. However, when ventricular apical longitudinal rotation (apical-LR) occurs in pulmonary hypertension (PH) patients, it may result in overestimated TAPSE., Methods: We studied 105 patients with PH defined as mean pulmonary artery pressure >25 mmHg at rest measured by right heart cardiac catheterization. TAPSE was defined as the maximum displacement during systole in the RV-focused apical four-chamber view. RV free-wall longitudinal speckle tracking strain (RV-free) was calculated by averaging 3 regional peak systolic strains. The apical-LR was measured at the peak rotation in the apical region including both left and right ventricle. The eccentricity index (EI) was defined as the ratio of the length of 2 perpendicular minor-axis diameters, one of which bisected and was perpendicular to the interventricular septum, and was obtained at end-systole (EI-sys) and end-diastole (EI-dia). Twenty age-, gender-, and left ventricular ejection fraction-matched normal controls were studied for comparison., Results: The apical-LR in PH patients was significantly lower than that in normal controls (-3.4 ± 2.7° vs. -1.3 ± 1.9°, P = 0.001). Simple linear regression analysis showed that gender, TAPSE, EI-sys, and EI-dia/EI-sys were associated with apical-LR, but RV-free was not. Multiple regression analysis demonstrated that gender, EI-dia/EI-sys, and TAPSE were independent determinants of apical-LR., Conclusions: TAPSE may be overestimated in PH patients with clockwise rotation resulting from left ventricular compression. TAPSE should thus be evaluated carefully in PH patients with marked apical rotation., (© 2015, Wiley Periodicals, Inc.)

Aims: To elucidate right ventricular (RV) function in patients with idiopathic pulmonary fibrosis (IPF) with and without pulmonary hypertension (PH) and its relation to other features of the disease., Methods and Results: Clinical evaluation, standard Doppler echo, Doppler myocardial imaging (DMI), and 2D strain echocardiography (STE) of RV septal and lateral walls were performed in 52 IPF patients (66.5 ± 8.5 years; 27 males) and in 45 age- and sex-comparable controls using a commercial US system (MyLab Alpha, Esaote). Pulmonary artery mean pressure (mPAP) was estimated by standard echo Doppler. RV global longitudinal strain (RV GLS) was calculated by averaging RV local strains. The IPF patients were divided into 2 groups by noninvasive assessment of PH: no PH (mPAP<25 mmHg; 36 pts) and PH (mPAP ≥25 mmHg; 16 pts). Left ventricular diameters and ejection fraction were comparable between controls and IPF, while GLS was impaired in IPF (P < 0.01). RV end-diastolic diameters, wall thickness andmPAP were increased in IPF patients with PH. In addition, pulsed DMI detected in PH IPF impaired myocardial RV early diastolic (Em) peak velocity. Also peak systolic RV strain was reduced in basal and middle RV lateral free walls in IPF, as well as RV GLS (P < 0.0001). The impairment in RV wall strain was more evident when comparing controls with the no PH group than comparing the no PH group with the PH group. By multivariate analysis, independent association of RV strain with both six-minute walking test distance (P < 0.001), mPAP (P < 0.0001), as well as with forced vital capacity (FVC) % (P < 0.005) in IPF patients were observed., Conclusions: Impaired RV diastolic and systolic myocardial function were present even in IPF patients without PH, which indicates an early impact on RV function and structure in patients with IPF., (© 2015, Wiley Periodicals, Inc.)

Background: Right atrial (RA) enlargement has been associated with worse clinical outcomes in chronic pulmonary hypertension (cPH) patients. Even though current guidelines only recommend measurement of RA dimensions at the end of ventricular systole in these patients, there is paucity of information regarding the potential utility of RA dimensions obtained at the end of ventricular diastole., Methods: In this retrospective study, standard echocardiographic data were collected from 80 studies. The population studied was divided into Group I that consisted of 35 patients (52 ± 10 years) without PH while Group II included 45 patients (56 ± 14 years; P = 0.2) with cPH. RA measurements were obtained not only at the end of ventricular systole, but also at the end of ventricular diastole to determine which RA measurement was more indicative of abnormal right ventricular afterload., Results: Even though all RA measurements were abnormal, RA area (>8.4 cm(2) ) measured at the end of ventricular diastole was the most useful RA variable to identify cPH patients with elevated pulmonary pressures (P < 0.0001) and with an abnormal pulmonary vascular resistance (P = 0.001)., Conclusions: Based on these results, it appears that isolated RA measurements made at the end of ventricular systole are insufficient to fully explain the hemodynamic load of cPH. Additional studies are now required to determine whether sequential monitoring of the composite change in RA dimensions that occur throughout the cardiac cycle over time correlates better with response to vasodilator therapy and overall clinical outcomes., (© 2015, Wiley Periodicals, Inc.)

Background: Although takotsubo cardiomyopathy (TTC) has been reported to have a favorable outcome, many complications may occur in the acute phase. Heart failure is the most common clinical complication in patients with TTC. We aimed to investigate determinants of secondary pulmonary hypertension (PH) in patients with TTC., Methods: This study consisted of 55 patients with TTC. Detailed echocardiographic measurements were taken, including pulmonary artery systolic pressure (PASP). PH was identified PASP >35 mmHg. The severity of mitral regurgitation (MR) was evaluated by measuring effective regurgitant orifice area (EROA). Follow-up echocardiography was performed in 45 patients (81.8%) within 4 weeks after initial presentation., Results: All patients were stratified into PH or no PH (NPH) group (average PASP: 46.2 ± 6.7 vs. 29.8 ± 3.3 mmHg, P < 0.001); 25 patients (45.5%) were categorized into the PH group. Left ventricular (LV) volume, LV ejection fraction, and troponin I levels did not significantly differ between the two groups. Age and EROA were significantly greater in PH group than NPH group (age; 74.6 ± 9.1 vs. 63.5 ± 17.7, EROA; 0.22 ± 0.17 vs. 0.03 ± 0.05 cm(2) , all P < 0.01). The multivariate analysis revealed that age and EROA were independent predictors for PH in patients with TTC (all P < 0.001). PASP was significantly improved at follow-up compared to those at initial presentation (35.8 ± 8.4 vs. 30.3 ± 7.9 mmHg, P < 0.01)., Conclusion: Age and the severity of MR were independent predictors for secondary PH in patients with TTC., (© 2015, Wiley Periodicals, Inc.)

Background: Pericardial effusion in pulmonary arterial hypertension (PAH) is an indicator of right heart failure and poor prognosis; its significance on serial transthoracic echocardiograms (TTE) is not clear., Methods: Baseline and follow-up TTE (1.0 ± 0.5 years), clinical parameters, and outcomes were studied (N = 200) in consecutive patients with PAH who underwent TTE at our center between October 1999 and November 2007. Study baseline TTE was 2.8 ± 4.0 years from initial PAH diagnosis., Results: Over median follow-up of 3.6 ± 2.6 years from baseline TTE, 106 patients (53%) died. Pericardial effusion was present in 20% at baseline, and at any time during the study in 29%. Patients with any pericardial effusion during follow-up were more likely to have underlying connective tissue disease. They also had significantly higher mean right atrial pressure and pulmonary vascular resistance, had lower cardiac output by invasive hemodynamic studies, had higher serum creatinine, and were more likely to be treated with prostanoids. Patients were also significantly likely to have more echocardiographic right atrial dilation and right ventricular dilation and dysfunction, and worse tricuspid regurgitation with higher peak velocity. During follow-up, there was significantly increased use of prostanoids (58% vs. 28%) and combination therapy (8% vs. 2%) compared to baseline. Persistence of pericardial effusion on both baseline and follow-up TTE was associated with worse outcome, and an independent predictor of survival after adjusting for age, creatinine, functional class, and hemodynamics (P < 0.01)., Conclusion: Persistence of pericardial effusion in PAH despite vasoactive therapy predicts worse outcomes; absence or resolution of pericardial effusion with therapy suggests better prognosis., (© 2015, Wiley Periodicals, Inc.)

Background: Tricuspid annular plane systolic excursion (TAPSE) has emerged as a novel and reliable measure of right ventricular (RV) function. The purpose of this study was to determine the effect of pulmonary hypertension (PH) therapy on TAPSE in pediatric patients and compare TAPSE to other quantitative measures of RV function., Methods: A retrospective review of medical records and echocardiograms of patients in the PH clinic from January 2011 to August 2013 was done. Echocardiograms were analyzed prior to initiation or addition of a PH drug and at least 8 weeks later. Following quantitative measures of RV function were compared: TAPSE, TAPSE age-based z-score, RV fractional area change (RVFAC), tricuspid annular S', tricuspid inflow E/tricuspid annular E' velocity (TV E/E'), and RV myocardial performance index (RVMPI)., Results: Of the 37 patients included in this study (median age 0.6 years), 23 (62.2%) were treatment naive and others had a new PH drug added to their regimen at the time of the baseline echocardiogram. The median duration between the baseline and follow-up echocardiogram was 8 (2-25) months. There was a significant improvement in TAPSE and TAPSE age-based z-score on the follow-up echocardiogram. RVFAC, tricuspid S', TV E/E', and RVMPI did not show a statistically significant change., Conclusion: In contrast to the other echocardiographic markers of RV function, TAPSE, and TAPSE age-based z-score significantly improve after initiation or addition of PH therapy and can be used for serial noninvasive monitoring of RV function in pediatric PH patients., (© 2014, Wiley Periodicals, Inc.)

Background and Aim: Pulmonary hypertension (PH) is common in patients with severe aortic stenosis (AS). The prognostic effect of PH in high-risk patients undergoing transcatheter aortic valve implantation (TAVI) remains unknown. The aim of this study was to examine the feasibility of TAVI in patients with PH and to determine the effect of TAVI on PH., Methods and Results: TAVI was performed in 70 patients (mean age, 77.6 years; 51 females and 19 males) between July 2011 and December 2012, in our hospital. The patients were divided into three groups based on their systolic pulmonary artery pressure (sPAP) values. Group 1 comprised patients with sPAP values <40 mmHg; group 2 included patients with sPAP values ranging from 40 to 59 mmHg; and group 3 included patients with sPAP values >60 mmHg. Seventy percent of the patients were in groups 2 and 3. After TAVI, the sPAP values of the patients in groups 2 and 3 were significantly decreased (47.4 ± 4.6 and 36.6 ± 6.3, P < 0.001 and 64.5 ± 4.7 and 43.2 ± 9.2, P < 0.001, respectively). However, this reduction was sustained for 6 months in group 2 (P = 0.006), whereas the reduction lost its statistical significance (P = 0.07) after 1 month in group 3 (64.5 ± 4.7 and 40.8 ± 8.0, P = 0.001). Significant differences between the sPAP values in all three groups before the procedure were sustained after TAVI (P ≤ 0.001) and after the 1st month (P = 0.02); however, no statistically significant difference was observed after the 6th month (P = 0.06)., Conclusion: In this study, we demonstrated that TAVI could be reliably and successfully performed in PH patients with severe AS and that TAVI results in a permanent and significant reduction in sPAP., (© 2014, Wiley Periodicals, Inc.)

Background: As survival and outcomes continue to improve in neonates born prematurely, there is an increasing need to promptly identify and treat pulmonary hypertension (PHT) in this population. Several echocardiographic indices have been used to evaluate for PHT. There is no clear consensus on how to utilize these parameters specifically for the evaluation of PHT in infants with chronic lung disease of prematurity., Objectives: Τhe objectives of the study were (1) to identify the different echocardiographic techniques for assessment of PHT in infants with chronic lung disease of prematurity in an evidence-based manner and (2) to establish an echocardiographic screening protocol based on available literature using different echocardiographic techniques., Methods: We conducted a systematic review of the literature regarding use of echocardiographic techniques for evaluation of PHT in infants with bronchopulmonary dysplasia. On the basis of the available evidence, we came up with a screening algorithm using various echocardiographic techniques., Results: We identified nine techniques that had been employed for detection of PHT noninvasively using echocardiography in 23 studies. Using these echocardiographic parameters, we came up with a flow chart to diagnose PHT in infants born prematurely, based on presence or absence of tricuspid regurgitation, presence or absence of patent ductus arteriosus, and direction of flow., Conclusions: We have proposed a new screening strategy for assessment of PHT using echocardiography in infants with chronic lung disease of prematurity. Further studies will be necessary to confirm its validity., (© 2014, Wiley Periodicals, Inc.)

Background: Right ventricular (RV) strain is a potentially useful prognostic marker in patients with pulmonary arterial hypertension (PAH). However, published reports regarding the accuracy of two-dimensional echocardiography (2DE)-derived RV strain against an independent reference in this patient population are limited. The aims of this study were: (1) to study the relationship between 2DE RV longitudinal strain and cardiovascular magnetic resonance (CMR)-derived RV ejection fraction (RVEF) in patients with PAH; (2) to compare 2DE-derived and CMR-derived RV longitudinal strain in these patients; and (3) to determine the reproducibility of these measurements., Methods: Thirty patients with PAH underwent 2DE and CMR imaging within a 2-hour time period. 2DE RV longitudinal strain was measured from a focused RV apical four-chamber view using speckle tracking software. CMR RV longitudinal strain was measured from short-axis slices acquired using fast-strain-encoded sequence. Global peak systolic RV longitudinal strain was calculated for both 2DE and CMR., Results: RV longitudinal strain using 2DE software correlated well with CMR-derived RVEF (R = 0.69, P = 0.0006). There was moderate agreement when comparing 2DE to CMR RV longitudinal strain (R = 0.74, P = 0.0002; bias -1%, limits of agreement -9 to 7%). Inter-observer variability and intra-observer variability for RV longitudinal strain were lower for 2DE than CMR., Conclusions: RV longitudinal strain by 2DE provides a good alternative for CMR-derived RVEF in patients with PAH. The moderate agreement in strain measurements between 2DE and CMR suggests that further software improvements are needed before these measurements can be used interchangeably in clinical practice., (© 2014, Wiley Periodicals, Inc.)

Purpose: Few studies describe diastolic (presystolic) tricuspid regurgitation (DTR) mainly in the context of atrioventricular conduction abnormalities. Little is known about its occurrence in the other clinical settings., Methods: We identified patients with DTR recorded during routine echocardiographic examinations. DTR was defined as low velocity backward flow through the tricuspid valve orifice during end-diastole recorded with a continuous and/or pulsed-wave Doppler and/or color-coded M-mode Doppler echocardiography., Results: Diastolic tricuspid regurgitation was present in a wide variety of clinical entities. Of the 23 patients with DTR 6 patients had ischemic and 10 dilated cardiomyopathy. Others had clinical conditions including: inappropriate pacemaker settings, decompensated hypertrophic cardiomyopathy, biventricular dysfunction following orthotropic heart transplantation, torrential aortic regurgitation, low ejection fraction aortic stenosis, advanced endocardial fibroelastosis, and complex congenital heart disease. Twenty of 23 patients had significantly impaired right ventricle (RV) systolic function. Systolic tricuspid regurgitation was estimated as moderate or severe in 13 cases and mild in the remaining 10 cases. RV systolic pressure was significantly elevated in all but 2 cases. In all but 4 cases DTR was transient. The persistence of DTR was associated with severe pulmonary hypertension, severe biventricular failure, and persistent severe pulmonary regurgitation., Conclusions: Diastolic tricuspid regurgitation may be encountered in a variety of clinical settings and should be sought for especially in patients with advanced RV systolic dysfunction, pulmonary hypertension, pulmonary regurgitation, or conduction abnormalities. Significant systolic regurgitation is not prerequisite for the development of DTR., (© 2014, Wiley Periodicals, Inc.)

Pulsed-wave Doppler interrogation of the hepatic veins (HVs) provides a window to right heart hemodynamics and function. Various pathologies that involve the right heart are manifested on the HV Doppler depending on the location and severity of the involvement and its hemodynamic consequences. Pulmonary hypertension (PHTN), a common finding on echocardiographic studies, imparts changes on the HV Doppler that are important to recognize. In this article, we provide a review of the main abnormalities that are encountered on the HV Doppler in patients with PHTN., (© 2014, Wiley Periodicals, Inc.)

Background: Even though systolic eccentricity index has been traditionally categorized based on right ventricular (RV) pressure overload, which hemodynamic component is particularly responsible for modulating leftward ventricular septal bowing (LVSB) in chronic pulmonary arterial hypertension (PAH) is less well-known., Methods: Therefore, currently used echocardiographic variables, as suggested by the American Society of Echocardiography, for evaluation of RV performance were evaluated in 60 patients to determine which of these commonly used parameters best determine an abnormal LVSB in chronic PAH patients., Results: In a stepwise multiple regression analysis of the echocardiographic variables used in this study, elevated pulmonary artery systolic pressures was the best discriminatory variable to identify an abnormal LVSB at any level along the interventricular septum in chronic PAH patients followed by smaller left ventricular end-diastolic volumes and larger RV end-systolic areas., Conclusion: Progressive RV failure in chronic PAH not only is associated with a decline in longitudinal base to apical motion of the annulus; but also with reduced inward displacement of the RV free wall. Additional reduction in RV systolic function also occurs as a result of progressive displacement of the interventricular septum toward the left ventricle. Therefore, as measures of LVSB along the interventricular septum not only are easily obtained and related to RV systolic performance in chronic PAH patients, routine use of these parameters might be helpful in monitoring decline of RV function as well as progression of end-stage PAH., (© 2014, Wiley Periodicals, Inc.)

Speckle tracking echocardiography-derived myocardial strain has useful clinical applications in adults with pulmonary hypertension (PH) as well as preterm infants with chronic lung disease. It is considered more sensitive compared to conventional indices. This report presents a 3-month-old infant with PH and poor right ventricular function who was treated with inhaled nitric oxide. Myocardial strain was noted to be impaired with paradoxical segmental strain. Impairment in strain improved after inhaled nitric therapy. Strain analysis can help improve understanding of cardiac adaptation in critical clinical situations., (© 2014, Wiley Periodicals, Inc.)

Aims: The objective of this study was to determine the factors independently associated with septal curvature in patients with pulmonary arterial hypertension (PAH)., Methods: Eighty-five consecutive patients with PAH who had an echocardiogram and a right heart catheterization within 24 hours of each others were included in the study. Septal curvature was assessed at the mid-papillary level using the eccentricity index (EI). Marked early systolic septal anterior motion was defined as a change in EI > 0.2 between end-diastole and early systole. Inter-ventricular mechanical delay was calculated as the percent time difference between right ventricular (RV) to left ventricular (LV) end-ejection time normalized for the RR interval., Results: Average age was 45 ± 11 years and the majority of patients were women (75%). Mean right atrial pressure was 11 ± 7 mmHg, mean PAP was 52 ± 13 mmHg, relative RV area 1.8 ± 0.9, and RV fractional area change 24 ± 8%. End-diastolic EI was 1.6 ± 0.4 and systolic EI was 2.5 ± 0.8. On multivariate analysis relative pulmonary pressure, relative RV area, and inter-ventricular mechanical delay were independently associated with systolic EI (R(2) = 0.72, P < 0.001). Independent determinants of diastolic EI included relative RV area and mean PAP (R(2) = 0.69, P < 0.001). A systolic EI >1.08 differentiated patients with PAH from healthy controls with an AUC = 0.99. Patients with early systolic septal anterior motion (44% of subjects) had lower exercise capacity, more extensive ventricular remodeling, and worst ventricular function., Conclusion: Septal curvature is a useful marker of structural, hemodynamic, and electromechanical ventricular interdependence in PAH., (© 2013, Wiley Periodicals, Inc.)

Background: Pulmonary hypertension (PH) in patients with systemic hypertension and preserved ejection fraction (PEF) has been described. However, the pathophysiology and consequences are not entirely clear. We sought to distinguish the clinical and anatomic features among hypertensive patients with or without coexistent PH., Methods: Echocardiograms and records of hypertensive patients with left ventricular (LV) hypertrophy and PEF from January 2009 to January 2011 were reviewed. We identified 174 patients, including 36 with PH (calculated pulmonary artery systolic pressure [PASP] ≥ 35 mmHg), and 138 with normal pulmonary pressures., Results: Hypertensive patients with PH were older (76 ± 13 vs. 65 ± 13 years, P < 0.0001), more often female (91, 70%), had lower estimated glomerular filtration rate (eGFR) (63 ± 44 vs. 88 ± 48 mL/min, P = 0.002), and higher pro-BNP levels (3141 ± 4253 vs. 1219 ± 1900 pg/mL, P = 0.003). PH patients also had larger left atrial areas (23.7 ± 3.8 vs. 20.8 ± 4.6 cm(2) , P = 0.002), evidence of diastolic dysfunction (i.e., septal E/e' 17.6 ± 8.6 vs. 12.7 ± 4.4, P = 0.0005), and higher calculated peripheral vascular resistance (PVR) (2.3 ± 1.1 vs. 1.6 ± 0.4, P < 0.0001). Both PVR and septal E/e' showed strong linear correlation with PASP (P < 0.0001 and P < 0.0001, respectively)., Conclusions: Hypertension in elderly patients is frequently complicated by LV diastolic dysfunction and secondary PH. These hypertensive patients tended to have reduced renal function and higher pro-BNP. Because of the known morbidity and mortality associated with PH, these observations have potentially important implications for target medical therapy., (© 2013, Wiley Periodicals, Inc.)

Background: Pulmonary hypertension (PH) is known to affect the right ventricular (RV) function., Aims: To assess the extent of global and regional RV dysfunction in PH patients., Methods: We performed a cross-sectional study on 20 controls (age 62 ± 15 years, 7 males) and 35 patients (age 67 ± 12 years, 13 males) with PH of mixed etiologies and assessed RV inflow and outflow tracts (OTs) function, using speckle tracking echocardiography (STE) based myocardial deformation and its time relations. RV inlet and OT dimensions (2D), inlet myocardial velocities (TDI), myocardial strain and strain rate (SR), TAPSE (M-mode), ejection and filling times (pulsed-wave [PW] Doppler), and pulmonary artery acceleration (PAc) were measured., Results: RV inlet and OT were dilated (P < 0.001 for both) and TAPSE (P < 0.001), inlet velocities (P < 0.001), basal and mid-cavity strain, SR and longitudinal displacement reduced (P < 0.001 for all). The time to peak systolic SR at basal, mid-cavity (P < 0.001 for both), and RVOT (P = 0.007) was short as was that to peak displacement (P < 0.001 for all). The time to peak pulmonary ejection correlated with time to peak SR at RVOT (r = 0.7, P < 0.001) in controls, but with that of the mid-cavity in patients (r = 0.71, P < 0.001). PAc was faster (P = 0.001) and RV filling time shorter in patients (P = 0.03) with respect to controls., Conclusions: PH has drastic effects on RV structure and intrinsic myocardial function, significantly disturbing its ejection time relations and overall pump performance. Increased RV afterload results in RV configuration changes with the inflow tract determining peak ejection rather than OT., (© 2013, Wiley Periodicals, Inc.)

Unlabelled: The ratio of tricuspid regurgitation velocity divided by the velocity-time integral of right ventricular outflow tract pulsed-wave Doppler tracing (TRV/VTI(RVOT) ) has been used to estimate pulmonary vascular resistance (PVR). However, this method has not been validated in chronic thromboembolic pulmonary hypertension (CTEPH). We assessed the utility of TRV/VTI(RVOT) in patients with CTEPH and PVR from 2 to 20 WU. All had right heart catheterization (RHC) within 2 days of echocardiography. TRV/VTI(RVOT) was calculated and RHC-derived pressures, PVR, and cardiac outputs were recorded. Mean pulmonary artery pressure was 47 ± 12 mmHg, cardiac output: 4.2 ± 1.1 L/min, PVR: 9 ± 4 WU, right atrial pressure: 12 ± 6 mmHg. Mean VTI(RVOT) was 13 ± 5 cm; mean TRV was 4.2 ± 0.8 m/s, mean tricuspid regurgitation severity was 2.5 ± 0.8 (1 = trace, 2 = mild, 3 = moderate, 4 = severe). Regression analysis demonstrated a correlation between RHC PVR and TRV/VTI(RVOT) : PVR = 19.4 × (TRV/VTI(RVOT) ) + 2.4 (r = 0.74, P < 0.001). However, Bland-Altman analysis found a poor degree of agreement between echo-derived PVR and RHC PVR. We also studied 28 patients with non-CTEPH pulmonary hypertension. Similar analysis revealed a regression equation of PVR = 20.1 × (TRV/VTIR(RVOT) ) + 0.3 (r = 0.57, P < 0.01)., Conclusion: TRV/VTI(RVOT) is only marginally useful for estimating PVR in CTEPH (r = 0.74). Moreover, the regression equation in CTEPH differs significantly from previous studies in pulmonary hypertension. Reasons for this may include the markedly elevated PVR levels in this population and specific effects on VTI(RVOT) from CTEPH., (© 2013, Wiley Periodicals, Inc.)

Background: In recent years, right ventricular (RV) function has acquired greater relevance as a clinical and prognostic marker in many physiopathological conditions. The study aims to point out the value of real time three-dimensional echocardiography (RT3DE) and tissue Doppler imaging (TDI) in the evaluation of patients affected by pulmonary hypertension (PH), compared with conventional two-dimensional (2D) echocardiography., Methods: We enrolled 44 subjects affected by PH who underwent 2D and Doppler echocardiography, RT 3D Echocardiography and TDI evaluation of the RV, and a healthy control group. PH itself can induce severe functional and structural abnormalities of the RV, such as RV hypertrophy, RV dilation, and RV systolic and diastolic dysfunction., Results: In this study, RV FAC, and TAPSE showed marked alterations in patients with PH compared to the control group (C): (RVFAC: [PH] 0.29 ± 0.07 vs. [C] 0.49 ± 0.05%, P < 0.0001; TAPSE: [PH] 15.3 ± 3.2 vs. [C] 21.1 ± 2.6 mm, P > 0.0001). The 3D RV end-diastolic volume was significantly higher in PH than in C (PH) (138.7 ± 25.3 vs. [C] 82.8 ± 12.5 mL, P < 0.0001] as well as 3D RV end-systolic volume (PH) (97.6 ± 21.5 vs. [C] 39.3 ± 9.5 mL, P < 0.0001). The 3D RV ejection fraction (EF) was significantly lower in the pulmonary hypertension group than in healthy subjects (31.8 ± 6.8 vs. [C] 52.5 ± 4.7%, P < 0.0001)., Conclusions: In patients with PH, evaluation of the RV diastolic and systolic volume and EF by RT3DE has shown a higher discriminating power in comparison, respectively, with 2DRV diastolic area and the relative fractional area changes., (© 2013, Wiley Periodicals, Inc.)

Introduction: We followed patients with pulmonary arterial hypertension (PAH) receiving specific vasodilator therapy and tested for predictors of clinical outcome., Methods: Thirty-two patients (mean age 39 ± 15 years, 22 women, diagnosed with pulmonary hypertension; PH): 29 with PAH and 3 patients with inoperable chronic thromboembolic PH received therapy with either bosentan, sildenafil, or both and were evaluated with clinical parameters, biomarkers (B-type natriuretic peptide values), and echocardiography before receiving specific medication and every 3 months thereafter. A right heart catheterization was performed at baseline. A composite endpoint of death, worsening of functional class, or the need of a second vasodilator agent was used to define the clinical nonresponders., Results: Patients were followed for 14 months (7.5-21). The endpoint was reached by 15 patients: four patients died (two idiopathic PAH and two PAH in context of Eisenmenger syndrome), seven patients showed 1 functional class worsening, and four patients needed to be switched to combination therapy. Patients who remained clinically stable or improved had at baseline a better cardiac output with a less remodeled right ventricle (RV) and better functioning RV (all P < 0.05). A RV fractional area change (RVFAC) lower than 25.7% and a RV global strain value higher than -13.4% predict with 87% sensitivity and 83% specificity (AUC 87.3%, P = 0.001) and 73% sensitivity and 91% specificity (AUC 84.2%, P = 0.003), respectively, patients who will deteriorate clinically under specific vasodilator therapy. A multivariate model showed RVFAC to be the only independent predictor of the endpoint with a HR of 0.87 (0.8-0.96), P = 0.007., Conclusions: Over an average period of 1 year, almost half of patients showed signs of clinical deterioration despite specific vasodilator therapy. Parameters of right ventricular morphology and function had prognostic value in these patients., (© 2012, Wiley Periodicals, Inc.)