A comprehensive echocardiographic method for risk stratification in pulmonary arterial hypertension.

QUESTION ADDRESSED: Echocardiography is not currently considered as providing sufficient prognostic information to serve as an integral part of treatment goals in pulmonary arterial hypertension (PAH). We tested the hypothesis that incorporation of multiple parameters reflecting right heart function would improve the prognostic value of this imaging modality. METHODS AND MAIN RESULTS: We pooled individual patient data from a total of 517 patients (mean age 52±15 years, 64.8% females) included in seven observational studies conducted at five European and United States academic centres. Patients were subdivided into three groups representing progressive degrees of right ventricular dysfunction based on a combination of echocardiographic measurements, as follows. Group 1 (low risk): normal tricuspid annular plane systolic excursion (TAPSE) and nonsignificant tricuspid regurgitation (TR) (n=129); group 2 (intermediate risk): normal TAPSE and significant TR or impaired TAPSE and nondilated inferior vena cava (IVC) (n=256); group 3 (high risk): impaired TAPSE and dilated IVC (n=132). The 5-year cumulative survival rate was 82% in group 1, 63% in group 2 and 43% in group 3. Low-risk patients had better survival rates than intermediate-risk patients (log-rank Chi-squared 12.25; p<0.001) and intermediate-risk patients had better survival rates than high-risk patients (log-rank Chi-squared 26.25; p<0.001). Inclusion of other parameters such as right atrial area and pericardial effusion did not provide added prognostic value. ANSWER TO THE QUESTION: The proposed echocardiographic approach integrating the evaluation of TAPSE, TR grade and IVC is effective in stratifying the risk for all-cause mortality in PAH patients, outperforming the prognostic parameters suggested by current guidelines.

Cardiac MRI right ventricle / left ventricle (RV/LV) volume ratio improves detection of RV enlargement.

PURPOSE: To determine the normal range of the ratio of right ventricular (RV) end-diastolic volume to left ventricular (LV) end-diastolic volume by magnetic resonance imaging (MRI) and examine whether combining this volume ratio with RVEDV indexed to body surface area (RVEDVi) increased the detection of RV dilation in patients with pulmonary arterial hypertension (PAH). MATERIALS AND METHODS: MRI-derived ventricular function and volumes were measured in a control group (n = 152) and in patients with PAH (n = 46). Images were acquired with a 1.5T Siemens or a 1.5T Philips scanner using a steady-state free procession sequence. Proposed criteria for the detection of RV enlargement, including RVEDVi alone, RV/LV volume ratio alone, and combining both criteria, were evaluated in both groups. RESULTS: The range (mean ± 2 standard deviations) for the volume ratio in the normal population was found to be 0.906-1.266; there was no difference between genders (P = 0.70). Combining this ratio with RVEDVi detected RV enlargement in 21.7% (P < 0.001) PAH patients (volume ratio ≥1.27) who were not identified by the RVEDVi alone (>104 mL/m(2) for females and >113 mL/m(2) for males). CONCLUSION: Combining RV/LV volume ratio with indexed RVEDV increased detection of RV enlargement in a PAH population. This result may have potential impact in RV size assessment. J. Magn. Reson. Imaging 2016;43:1379-1385.

Changes in right heart haemodynamics and echocardiographic function in an advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis.

BACKGROUND: Pulmonary hypertension (PH)-targeted therapy in the setting of pulmonary fibrosis (PF) is controversial; the main clinical concern is worsening of systemic hypoxaemia. We sought to determine the effects of gentle initiation and chronic administration of parenteral treprostinil on right heart function in patients with PF associated with an advanced PH phenotype. METHODS: Open-label, prospective analysis of patients with PF-PH referred for lung transplantation (LT). Advanced PH was defined as mean pulmonary artery pressure (mPAP) ≥35 mm Hg. We compared haemodynamics, Doppler echocardiography (DE), oxygenation, dyspnoea and quality of life indices, and 6 min walk distance (6MWD) before and 12 weeks after parenteral treprostinil. RESULTS: 15 patients were recruited in the study. After therapy, there were significant improvements in right heart haemodynamics (right atrial pressure (9.5 ± 3.4 vs 6.0 ± 3.7); mPAP (47 ± 8 vs 38.9 ± 13.4); CI (2.3 ± 0.5 vs 2.7 ± 0.6); pulmonary vascular resistance (698 ± 278 vs 496 ± 229); transpulmonary gradient (34.7 ± 8.7 vs 28.5 ± 10.3); mvO2 (65 ± 7.2 vs 70.9 ± 7.4); and stroke volume index (29.2 ± 6.7 vs 33 ± 7.3)) and DE parameters reflecting right heart function (right ventricular (RV) end diastolic area (36.4 ± 5.2 vs 30.9 ± 8.2 cm(2)), left ventricular eccentricity index (1.7 ± 0.6 vs 1.3 ± 0.5), tricuspid annular planar systolic excursion (1.6 ± 0.5 vs 1.9 ± 0.2 cm)). These changes occurred without significant alteration in systemic oxygenation, heart rate, or mean systemic arterial pressure. In addition, improvements were seen in 6MWD (171 ± 93 vs 230 ± 114), 36-Item Short Form Health Survey Mental Component Summary aggregate (38 ± 11 vs 44.2 ± 10.7), University of California, San Diego Shortness of Breath Questionnaire (87 ± 17.1 vs 73.1 ± 21), and brain natriuretic peptide (558 ± 859 vs 228 ± 340). CONCLUSIONS: PH-targeted therapy may improve right heart haemodynamics and echocardiographic function without affecting systemic oxygen saturation in an advanced PH phenotype associated with RV dysfunction in the setting of PF.

Estimation of oxygen consumption in elderly patients with aortic stenosis.

BACKGROUND: Invasive evaluation of aortic stenosis requires measuring cardiac output. With the Fick equation, a measure of oxygen consumption (VO2) is required. Standard equations for estimating VO2 were derived in younger and healthier populations than the ones referred for possible transcatheter aortic valve replacement. The goal of this study was to determine the best method of estimating VO2 in elderly patients with aortic stenosis. METHODS: We directly measured VO2 in elderly patients undergoing invasive assessment of aortic stenosis. We compared standard equations estimating VO2 and two prespecified hypothesized equations for VO2 to determine which was most accurate. We also examined the subgroup of patients with low flow. RESULTS: Among 51 patients, aged 80-97 years, the mean VO2 was 198 mL/min. Using 125*body surface area (BSA) to estimate VO2 the average error was 35 mL/min, and 67% of values were within 25% of the true value. Using 3*Weight to estimate VO2, those numbers were 29 mL/min and 65%. The hypothesized equations did better: 100*BSA had error of -12 mL/min and 90% within 25% of measured VO2; for 2.5*Weight it was -9 mL/min and 84%. Among the 20 patients with low flow, hypothesized equations performed best. Using 2.5*Weight and 100*BSA there were 90% and 85% within 25% of measured VO2, respectively, compared to 55% and 75% when 3*Weight and 125*BSA were used. Weight and BSA were the only independent predictors of VO2. CONCLUSIONS: When estimating VO2 in an elderly population with aortic stenosis, the best equations are 2.5*Weight and 100*BSA.

Assessment of right ventricular function by transthoracic echocardiography following aortic valve replacement.

BACKGROUND: Tricuspid annular plane systolic excursion (TAPSE) is a widely used clinical measure of right ventricular (RV) systolic performance. However, postsurgical changes in the pattern of RV contraction may limit the utility of TAPSE for assessing global RV function. We retrospectively examined pre- and postoperative TAPSE and RV fractional area change (FAC) in patients undergoing 3 different types of aortic valve replacement (AVR). METHODS: Fifty-two patients enrolled in the Placement of AoRTic TraNscathetER Valve Trial at our institution were randomized to receive open AVR or transcatheter AVR (TAVR) by either the transapical or transfemoral access routes. Thirty-seven of these patients had analyzable transthoracic echocardiography (TTE) before and after AVR. Using M-mode echocardiography, TAPSE was measured in the apical four-chamber view. Using two-dimensional echocardiography, RV FAC was measured in the apical four-chamber view. RESULTS: The mean change in TAPSE was -0.7 ± 0.6 cm for open AVR (P = 0.002), -0.2 ± 0.4 cm for transapical TAVR (P = 0.26), and 0.1 ± 0.5 cm for transfemoral TAVR (P = 0.64). The mean change in RV FAC was -1 ± 5% for open AVR (P = 0.91), 2 ± 4% for transapical TAVR (P = 0.37), and 7 ± 10% for transfemoral TAVR (P = 0.07). CONCLUSIONS: The normal pattern of RV contraction was unchanged by transapical and transfemoral TAVR, while open AVR led to a significant decrease in TAPSE with preserved RV FAC. Thus, RV FAC is a preferable method for assessing RV function in the postoperative patient.

[Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology]. / Pulmoner arter hipertansiyonuna sag kalp adaptasyonu: Fizyoloji ve patobiyoloji.

Survival in patients with pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Although pulmonary load is an important determinant of RV systolic function in PAH, there remains a significant variability in RV adaptation to pulmonary hypertension. In this report, the authors discuss the emerging concepts of right heart pathobiology in PAH. More specifically, the discussion focuses on the following questions. 1) How is right heart failure syndrome best defined? 2) What are the uderlying molecular mechanisms of the failing right ventricle in PAH? 3) How are RV contractility and function and their prognostic implications best assessed? 4) What is the role of targeted RV therapy? Throughout the report, the authors highlight differences between right and left heart failure and outline key areas of future investigation. (J Am Coll Cardiol 2013;62:D22-33) a 2013 by the American College of Cardiology Foundation).

Pelvic Vein Obstruction in Chronic Thromboembolic Pulmonary Hypertension: A Novel Association.

Background: Pelvic venous obstruction (PVO), defined as greater than 50% stenosis or occlusion of pelvic veins, is a known risk factor for deep vein thrombosis (DVT). DVT is a known risk factor for chronic thromboembolic pulmonary hypertension (CTEPH), but the prevalence of PVO in CTEPH is unknown. Methods: This cross-sectional study at Temple University's tertiary referral center for Pulmonary Hypertension, Right Heart Failure, and CTEPH sought to identify the presence of PVO in patients with CTEPH who underwent cardiac catheterization, pulmonary angiography, and venography. Results: A total of 193 CTEPH patients were referred for pulmonary angiography, and among these, 148 underwent venography. PVO was identified in 65 (44%) patients. Lower extremity (LE) DVT was associated with PVO (p = 0.004). The severity of pulmonary hypertension was similar with and without PVO (mean pulmonary artery pressure 43.0 ± 10.3 mm Hg vs. 43.8 ± 12.4 mm Hg, p = 0.70), as was the need for pulmonary thromboendarterectomy (69.2% vs. 61.4%, p = 0.32). Conclusions: Pelvic vein obstruction is common and a novel clinical association in patients with CTEPH, particularly in patients with a history of LE DVT. PVO and its role in CTEPH warrants further study, including the potential role of revascularization to mitigate further risk.

Potential effects of digoxin on long-term renal and clinical outcomes in chronic heart failure.

BACKGROUND: Digitalis glycosides are known to improve the hemodynamic and neurohormonal perturbations that contribute to heart failure (HF)-induced renal dysfunction (RD). The objective of this study was to determine if randomization to digoxin is associated with improvement in renal function (IRF) and to evaluate if patients with digoxin-induced IRF have improved clinical outcomes. METHODS AND RESULTS: Patients in the Digitalis Investigation Group (DIG) dataset with protocol-driven 1-year serum creatinine levels (performed in a central laboratory; n = 980) were studied. IRF was defined as a postrandomization ≥20% increase in estimated glomerular filtration rate (eGFR). IRF occurred in 15.5% of the population (mean improvement in eGFR 34.5 ± 15.4%) and was more common in patients randomized to digoxin (adjusted odds ratio 1.6; P = .02). In patients without IRF, digoxin was not associated with reduced death or hospitalization (adjusted hazard ratio [HR] 0.96, 95% CI 0.8-1.2; P = .67). However, in the group with IRF, digoxin was associated with substantially improved hospitalization-free survival (adjusted HR 0.49, 95% CI 0.3-0.8; P = .006; P interaction = .026). CONCLUSIONS: In this subset of the DIG trial, digoxin was associated with long-term improvement in kidney function and, in patients demonstrating this favorable renal response, reduction in death or hospitalization. Additional research is necessary to confirm these hypothesis-generating findings.

Enhancing the accuracy of echocardiography in the diagnosis of pulmonary arterial hypertension: looking at the heart to learn about the lungs.

PURPOSE OF REVIEW: Although routine use of Doppler echocardiography has led to an increased recognition of pulmonary hypertension, the role of Doppler echocardiography has largely remained as a screening tool with the primary emphasis on the presence or absence of an increased Doppler-estimated pulmonary artery systolic pressure (PASP). However, the utility of Doppler echocardiography in the workup of pulmonary hypertension extends far beyond that of a screening tool, with the integration of relevant Doppler echocardiography parameters providing a wealth of hemodynamic insight into not only if a patient has pulmonary hypertension, but why they have pulmonary hypertension. This review summarizes some of the recent advances in the use of Doppler echocardiography in evaluating the pathophysiology of pulmonary hypertension. RECENT FINDINGS: Distinguishing pulmonary hypertension related to pulmonary vascular disease (i.e. pulmonary arterial hypertension, pulmonary hypertension due to lung disease, or chronic thromboembolic pulmonary hypertension; PHPVD), from those with left heart disease associated pulmonary hypertension (pulmonary venous hypertension; PVH) is crucial as workup and treatment options differ dramatically. Recent studies have identified easily obtainable Doppler echocardiography parameters that can reliably distinguish between PHPVD and PVH, allowing for rapid triage of patients with evidence of PHPVD to invasive right heart catheterization whiles avoiding invasive investigation and the inappropriate use of pulmonary hypertension specific therapy in patients with PVH. SUMMARY: This review highlights the importance of integrating two-dimensional and Doppler parameters in order to inform the clinician as to the hemodynamic cause of pulmonary hypertension, thus enhancing the diagnostic accuracy of Doppler echocardiography, rapidly identifying those with PHPVD and right heart dysfunction and assisting in triage of patients to further invasive hemodynamic assessment.

Virtual Echocardiography Screening Tool Identifies Pulmonary Arterial Hypertension Significantly Earlier Than High-Risk Clinical Diagnosis.

Pulmonary arterial hypertension (PAH) is often a progressive, fatal disease. Because of nonspecificity of symptoms and limited awareness of PAH, patients are often diagnosed and referred late to accredited pulmonary hypertension (PH) centers, contributing to worsening survival and overall prognosis. The objective of the present study was to determine if the virtual echocardiography screening tool (VEST), a simple scoring system using routinely reported echocardiographic metrics, could capture earlier diagnoses of PAH before clinical recognition and referral to expert PH centers. This study is a retrospective analysis of 132 patients with PAH evaluated consecutively at 2 accredited referral PH centers. VEST scores and time to evaluation at PH center were quantified based on the first available echocardiogram before referral. Clinical risk assessment was calculated at initial evaluation by the PH center using the REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) 2.0 calculator. An overwhelming majority (93%) of the study participants had markedly abnormal VEST scores predictive of PAH before evaluation at a PH referral center. The median delay from VEST to evaluation was >6 months at 206 days (quartile 1, quartile 3: 55, 757). At initial evaluation, 72% were intermediate or high-risk based on REVEAL 2.0 risk assessment. In conclusion, we propose that VEST is a powerful yet simple scoring tool that can capture high-risk patients with PAH, prompting earlier diagnosis and referrals to accredited PH centers, and allowing for earlier expert implementation of PH medical therapies.

Shape of the right ventricular Doppler envelope predicts hemodynamics and right heart function in pulmonary hypertension.

RATIONALE: Systolic deceleration or "notching" of the right ventricular outflow tract Doppler flow velocity envelope (FVE(RVOT)) relates to pathologic wave reflection in the setting of elevated pulmonary artery impedance. OBJECTIVES: We investigated whether simple visual assessment of FVE(RVOT) morphology aids in hemodynamic differentiation and detection of pulmonary vascular disease among a referral pulmonary hypertension (PH) cohort. METHODS: We reviewed hemodynamics, echocardiography, and clinical data for 88 patients referred for PH and 32 subjects with systolic heart failure and PH. The FVE(RVOT) was categorized as normal (no notch [NN]); late systolic notch (LSN); or midsystolic notch (MSN). MEASUREMENTS AND MAIN RESULTS: The pulmonary vascular resistance (PVR) was highest in the MSN group (9.2 ± 3.5 Wood's units [WU]; P < 0.001) versus the LSN (5.7 ± 3.1 WU) and NN (3.3 ± 2.4 WU) groups. The ratio of stroke volume to pulse pressure (compliance) also differed by FVE(RVOT) morphology (MSN = 1.2 ± 0.5; LSN = 1.7 ± 0.8; NN = 2.6 ± 1.7; P = 0.001 and 0.04, respectively, vs. NN). MSN was 96% specific and 71% sensitive for a PVR >5 WU (positive predictive value, 98%). The MSN group had severe right ventricular dysfunction (tricuspid annular plane systolic excursion 1.6 ± 0.5 cm) relative to the LSN and NN groups (tricuspid annular plane systolic excursion 1.9 ± 0.6 vs. 2.2 ± 0.6 cm; both P < 0.05). In the PH cohort, any FVE(RVOT) notching (MSN or LSN) was highly associated with PVR >3 WU (odds ratio, 22.3; 95% confidence interval, 5.2-96.4), whereas the NN pattern predicted a PVR less than or equal to 3WU and pulmonary artery wedge pressure greater than 15 mm Hg (odds ratio, 30.2; 95% confidence interval, 6.3-144.9). CONCLUSIONS: Visual inspection of the shape of the FVE(RVOT) provides insight into the hemodynamic basis of PH in a referral PH cohort. MSN is associated with the most severe pulmonary vascular disease and right heart dysfunction.

Risk Stratification of Patients with Pulmonary Arterial Hypertension: The Role of Echocardiography.

Background: Given the morbidity and mortality associated with pulmonary arterial hypertension (PAH), risk stratification approaches that guide therapeutic management have been previously employed. However, most patients remain in the intermediate-risk category despite initial therapy. Herein, we sought to determine whether echocardiographic parameters could improve the risk stratification of intermediate-risk patients. Methods: Prevalent PAH patients previously enrolled in observational studies at 3 pulmonary hypertension centers were included in this study. A validated PAH risk stratification approach was used to stratify patients into low-, intermediate-, and high-risk groups. Right ventricular echocardiographic parameters were used to further stratify intermediate-risk patients into intermediate-low- and intermediate-high-risk groups based on transplant-free survival. Results: From a total of 146 patients included in our study, 38 patients died over a median follow-up of 2.5 years. Patients with intermediate-/high-risk had worse echocardiographic parameters. Tricuspid annular plane systolic excursion (TAPSE) and the degree of tricuspid regurgitation (TR) were highly associated with survival (p < 0.01, p = 0.04, respectively) and were subsequently used to further stratify intermediate-risk patients. Among intermediate-risk patients, survival was worse for patients with TAPSE < 19 mm compared to those with TAPSE ≥ 19 mm (estimated one-year survival 74% vs. 96%, p < 0.01) and for patients with moderate/severe TR compared to those with no/trace/mild TR (estimated one-year survival 70% vs. 93%, p < 0.01). Furthermore, among intermediate-risk patients, those with both TAPSE < 19 mm and moderate/severe TR had an estimated one-year survival (56%) similar to that of high-risk patients (56%), and those with both TAPSE ≥ 19 mm and no/trace/mild TR had an estimated one-year survival (97%) similar to that of low-risk patients (95%). Conclusions: Echocardiography, a routinely performed, non-invasive imaging modality, plays a pivotal role in discriminating distinct survival phenotypes among prevalent intermediate-risk PAH patients using TAPSE and degree of TR. This can potentially help guide subsequent therapy.

May-Thurner Anatomy in Patients With Chronic Thromboembolic Pulmonary Hypertension: An Important Clinical Association.

OBJECTIVES: The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors. BACKGROUND: MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown. METHODS: A retrospective chart review was conducted in patients referred to Temple University Hospital's cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA. RESULTS: A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015). CONCLUSIONS: MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.

Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension.

RATIONALE: Transthoracic Doppler echocardiography is recommended for screening for the presence of pulmonary hypertension (PH). However, some recent studies have suggested that Doppler echocardiographic pulmonary artery pressure estimates may frequently be inaccurate. OBJECTIVES: Evaluate the accuracy of Doppler echocardiography for estimating pulmonary artery pressure and cardiac output. METHODS: We conducted a prospective study on patients with various forms of PH who underwent comprehensive Doppler echocardiography within 1 hour of a clinically indicated right-heart catheterization to compare noninvasive hemodynamic estimates with invasively measured values. MEASUREMENTS AND MAIN RESULTS: A total of 65 patients completed the study protocol. Using Bland-Altman analytic methods, the bias for the echocardiographic estimates of the pulmonary artery systolic pressure was -0.6 mm Hg with 95% limits of agreement ranging from +38.8 to -40.0 mm Hg. Doppler echocardiography was inaccurate (defined as being greater than +/-10 mm Hg of the invasive measurement) in 48% of cases. Overestimation and underestimation of pulmonary artery systolic pressure by Doppler echocardiography occurred with a similar frequency (16 vs. 15 instances, respectively). The magnitude of pressure underestimation was greater than overestimation (-30 +/- 16 vs. +19 +/- 11 mm Hg; P = 0.03); underestimates by Doppler also led more often to misclassification of the severity of the PH. For cardiac output measurement, the bias was -0.1 L/min with 95% limits of agreement ranging from +2.2 to -2.4 L/min. CONCLUSIONS: Doppler echocardiography may frequently be inaccurate in estimating pulmonary artery pressure and cardiac output in patients being evaluated for PH.

Virtual echocardiography screening tool to differentiate hemodynamic profiles in pulmonary hypertension.

This study validated a novel virtual echocardiography screening tool (VEST), which utilized routinely reported echocardiography parameters to predict hemodynamic profiles in pulmonary hypertension (PH) and identify PH due to pulmonary vascular disease (PHPVD). Direct echocardiography imaging review has been shown to predict hemodynamic profiles in PH; however, routine use often overemphasizes Doppler-estimated pulmonary artery systolic pressure (PASPDE), which lacks discriminatory power among hemodynamically varied PH subgroups. In patients with PH of varying subtypes at a tertiary referral center, reported echocardiographic findings needed for VEST, including left atrial size, E:e' and systolic interventricular septal flattening, were obtained. Receiver operating characteristic analyses assessed the predictive performance of VEST vs. PASPDE in identifying PHPVD, which was later confirmed by right heart catheterization. VEST demonstrated far superior discriminatory power than PASPDE in identifying PHPVD. A positive score was 80.0% sensitive and 75.6% specific for PHPVD with an area under the curve of 0.81. PASPDE exhibited poorer discriminatory power with an area under the curve of 0.56. VEST's strong discriminatory ability remained unchanged when validated in a second cohort from another tertiary center. We demonstrated that this novel VEST using three routine parameters that can be easily extracted from standard echocardiographic reports can successfully capture PH patients with a high likelihood of PHPVD. During the Covid-19 pandemic, when right heart catheterization and timely access to experts at accredited PH centers may have limited widespread availability, this may assist physicians to rapidly and remotely evaluate PH patients to ensure timely and appropriate care.

Cangrelor and Heparin for Pulmonary Thromboendarterectomy in Heparin-Induced Thrombocytopenia.

Perioperative anticoagulation management for patients with heparin-induced thrombocytopenia requiring cardiopulmonary bypass and deep hypothermic circulatory arrest presents a clinical challenge. Alternative anticoagulants have been used but can cause significant postoperative bleeding. We report the successful use of cangrelor and heparin in a 30-year-old patient with severe heparin-induced thrombocytopenia undergoing urgent pulmonary thromboendarterectomy.