Diagnosis and clinical investigation of patients presenting with pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Investigation ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Reference Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Invasive Test ◽  
Pulmonary Arterial

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.

scholarly journals Right ventricular dimension index by cardiac magnetic resonance for prognostication in connective tissue diseases and pulmonary hypertension

Rheumatology ◽  
2019 ◽  
Author(s):  
Nobuya Abe ◽  
Masaru Kato ◽  
Michihito Kono ◽  
Yuichiro Fujieda ◽  
Hiroshi Ohira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Dimension Index

Abstract Objectives Pulmonary hypertension (PH) in patients with CTD is a heterogeneous condition affected by left heart disease, chronic lung disease and thromboembolism as well as pulmonary vascular disease. Recent studies using cardiac magnetic resonance (CMR) have shown that right ventricular dysfunction is predictive for mortality in patients with PH, but limited to pulmonary arterial hypertension. This study aimed to analyse prognostic factors in PH-CTD. Methods This retrospective analysis comprised 84 CTD patients, including SSc, who underwent both CMR and right heart catheterization from 2008 to 2018. Demographics, laboratory findings, and haemodynamic and morphological parameters were extracted. The prognostic value of each parameter was evaluated by multivariate analysis using covariables derived from propensity score to control confounding factors. Results Of 84 patients, 65 had right heart catheterization-confirmed PH (54 pulmonary arterial hypertension, 11 non-pulmonary arterial hypertension). Nine out of these PH patients died during a median follow-up period of 25 months. In 65 patients with PH, right ventricular end-diastolic dimension index (RVEDDI) evaluated by CMR was independently associated with mortality (hazard ratio 1.24; 95% CI: 1.08–1.46; P = 0.003). In a receiver operating characteristic analysis, RVEDDI highly predicted mortality, with area under the curve of 0.87. The 0.5–2-year follow-up data revealed that RVEDDI in both survivors and non-survivors did not significantly change over the clinical course, leading to the possibility that an early determination of RVEDDI could predict the prognosis. Conclusion RVEDDI simply evaluated by CMR could serve as a significant predictor of mortality in PH-CTD. A further validation cohort study is needed to confirm its usability.

scholarly journals Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Verônica Silva Vilela ◽  
Marcio Macri Dias ◽  
Ângelo Antunes Salgado ◽  
Bruno Rangel Antunes da Silva ◽  
Agnaldo José Lopes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Right Heart Catheterization ◽  
Diagnostic Tools ◽  
Heart Catheterization ◽  
Systematic Screening ◽  
Mean Pulmonary Arterial Pressure ◽  
Common Group ◽  
Pulmonary Arterial

Abstract Background Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. Methods A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. Results Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. Conclusions Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

scholarly journals Experience with exercise right heart catheterization in the diagnosis of pulmonary hypertension: a retrospective study

2014 ◽  
Vol 9 ◽  
Author(s):  
Stephan Keusch ◽  
Anina Bucher ◽  
Séverine Müller-Mottet ◽  
Elisabeth Hasler ◽  
Marco Maggiorini ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Life Experience ◽  
Real Life ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Mean Pulmonary Arterial Pressure ◽  
Exercise Hemodynamics ◽  
Pulmonary Arterial

Background: Data on exercise pulmonary hemodynamics in healthy people and patients with pulmonary hypertension (PH) are rare. We analyzed exercise right heart catheterization (RHC) data in a symptomatic collective referred with suspected PH to characterize the differential response by diagnostic groups, to correlate resting with exercise hemodynamics, and to evaluate safety. Methods: This is a retrospective single-center study reviewing data from patients in whom an exercise RHC was performed between January 2006 and January 2013. Patients with follow-up RHC under PH -therapy were excluded. Results: Data from 101 patients were analyzed, none of them had an adverse event. In 35% we detected a resting PH (27.8% precapillary, 6.9% postcapillary). Exercise PH (mean pulmonary arterial pressure (mPAP) >30 mmHg at exercise) was found in 38.6%, whereas in 25.7% PH was excluded. We found a remarkable number of exercise PH in scleroderma patients, the majority being postcapillary. 83% of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH. Patients with resting PH had worse hemodynamics and were older compared with exercise PH ones. Conclusion: In this real-life experience in symptomatic patients undergoing exercise RHC for suspected PH, we found that exercise RHC is safe. The facts that the vast majority of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH and the older age of patients with resting PH may indicate that exercise PH is a precursor of resting PH. Whether earlier treatment start in patients with exercise PH would stabilize the disease should be addressed in future studies.

scholarly journals Right heart catheterization for pulmonary hypertension during the coronavirus disease 2019 pandemic

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402094878 ◽  
Author(s):  
Kanza N. Qaiser ◽  
James E Lane ◽  
Adriano R. Tonelli
Keyword(s):  
Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Clinical Practices ◽  
Right Heart ◽  
Diagnostic Modality ◽  
Hypertension Therapy ◽  
Pulmonary Arterial ◽  
Pulmonary Arterial Hypertension Therapy

Right heart catheterization is an essential diagnostic modality in the evaluation of pulmonary hypertension. The coronavirus disease 2019 pandemic has resulted in deferral of elective procedures including right heart catheterization. The benefits of proceeding with right heart catheterization, such as further characterization of hemodynamic subtype and severity of pulmonary hypertension, initiation of targeted pulmonary arterial hypertension therapy, as well as further hemodynamic testing, need to be carefully balanced with the risk of potentially exposing both patients and health care personnel to coronavirus disease 2019 infection. This review article aims to provide best clinical practices for safely performing right heart catheterization in pulmonary hypertension patients during the coronavirus disease 2019 pandemic.

scholarly journals An autopsy case of pulmonary arterial hypertension in an elderly patient with multimorbidity: a case report

2021 ◽  
Author(s):  
Masashi Yokose ◽  
Takashi Tomoe ◽  
Takehiko Yamaguchi ◽  
Takanori Yasu
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Elderly Patients ◽  
Arterial Hypertension ◽  
Diagnostic Delay ◽  
Right Heart Catheterization ◽  
World Health ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial

Abstract Background There is an increasing number of elderly patients with pulmonary arterial hypertension, and their characteristics differ from those of young or middle-aged patients with this condition. Case Summary A 73-year-old woman with a history of myocardial infarction and cardiovascular risk factors was admitted to the hospital with 2-week exertional dyspnoea. Her initial diagnosis was heart failure with preserved ejection fraction, but the symptoms persisted despite receiving treatment with diuretics. Additional tests showed a significant decrease in diffusing capacity of carbon monoxide and findings suggestive of severe pulmonary hypertension. Contrast-enhanced computed tomography of the chest, and pulmonary angiography, showed no narrowing or obstruction of the pulmonary arteries. Right heart catheterization revealed hemodynamic data implying pre-capillary pulmonary hypertension. Her condition gradually deteriorated to World Health Organization functional class IV, and sequential combination therapy with tadalafil, macitentan, and selexipag was initiated with a pulmonary arterial hypertension diagnosis; however, she died 1 month later. Pathological findings in autopsy were consistent with pulmonary arterial hypertension, and some parts of the lungs revealed the presence of obstructive and interstitial lung disease. Discussion The majority of elderly patients with pulmonary arterial hypertension might have multimorbidity. However, there is no specific treatment strategy. It is associated with diagnostic delay and worse prognosis; therefore, early suspicion and comprehensive tests, including right heart catheterization, are essential for better management.

scholarly journals Impact of Three Different Algorithms for the Screening of SSc-PAH and Comparison with the Decisions of a Multidisciplinary Team

Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1738
Author(s):  
Valentin Coirier ◽  
Céline Chabanne ◽  
Stéphane Jouneau ◽  
Nicolas Belhomme ◽  
Alice Ballerie ◽  
...  
Keyword(s):  
Multidisciplinary Team ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Tertiary Center ◽  
Previous Definition ◽  
Pulmonary Arterial ◽  
Definition Of ◽  
The Impact

Background: to compare three existing screening algorithms of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) with the results of a multidisciplinary team (MDT) meeting from a tertiary center. Methods: we conducted a monocentric longitudinal study from 2015 to 2018. All patients with SSc according to LeRoy’s classification were eligible. Patients were excluded in the case of missing data required by any of the three screening algorithms. The algorithms were applied for each patient at inclusion. Right heart catheterization (RHC) was performed based on the MDT decision. MDT members were all blinded from the results of the three algorithms regarding RHC recommendations. The RHC recommendations of each algorithm were compared with the MDT decision, and the impact on diagnosis and management was evaluated. Results: 117 SSc patients were consecutively included in the study, and 99 had follow-up data over the three-year duration of the study (10 deaths). Among the 117 patients, the MDT suggested RHC for 16 patients (14%), DETECT algorithm for 28 (24%), ASIG for 48 (41%) and ESC/ERS 2015 for 20 (17%). Among the 16 patients who had RHC, SSc-PAH was diagnosed in seven. Among patients with an initial recommendation of RHC based on at least one algorithm but not according to the MDT meeting, no SSc-PAH was diagnosed during the three-year follow-up. Results were unchanged when the new 2018 definition of PAH was applied instead of the previous definition. Conclusion: a MDT approach appears interesting for the screening of SSc-PAH, with a significant reduction of RHC performed in comparison with dedicated algorithms. The specific relevance of a MDT for the management and follow-up of patients with RHC recommended by existing algorithms but with no PAH warrants further studies.

scholarly journals Accuracy of Echocardiographic Estimates of Pulmonary Artery Pressures in Pulmonary Hypertension: Insights From the KARUM Hemodynamic Database

2021 ◽  
Author(s):  
Ashwin Venkateshvaran ◽  
Natavan Seidova ◽  
Hande Oktay Tureli ◽  
Barbro Kjellström ◽  
Lars H Lund ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Population Studies ◽  
Right Heart Catheterization ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Limits Of Agreement ◽  
Right Heart ◽  
Minimal Bias

Abstract BACKGROUND. Accurate assessment of pulmonary artery (PA) pressures is integral to diagnosis, follow-up and therapy selection in pulmonary hypertension (PH). Despite wide utilization, the accuracy of echocardiography to estimate PA pressures has been debated. We aimed to evaluate echocardiographic accuracy to estimate right heart catheterization (RHC) based PA pressures in a large, dual-centre hemodynamic database. METHODS. Consecutive PH referrals that underwent comprehensive echocardiography within 3 hours of clinically indicated right heart catheterization were enrolled. Subjects with absent or severe, free-flowing tricuspid regurgitation (TR) were excluded. Accuracy was defined as mean bias between echocardiographic and invasive measurements on Bland-Altman analysis for the cohort and estimate difference within ±10mmHg of invasive measurements for individual diagnosis. RESULTS. In 419 subjects, echocardiographic PA systolic and mean pressures demonstrated minimal bias with invasive measurements (+2.4 and +1.9mmHg respectively) but displayed wide limits of agreement (-20 to +25 and -14 to +18mmHg respectively) and frequently misclassified subjects. Recommendation-based right atrial pressure (RAP) demonstrated poor precision and was falsely elevated in 32% of individual cases. Applying a fixed, median RAP to echocardiographic estimates resulted in relatively lower bias between modalities when assessing PA systolic (+1.4mmHg; 95% limits of agreement +25 to –22mmHg) and PA mean pressures (+1.4mmHg; 95% limits of agreement +19 to -16mmHg).CONCLUSIONS. Echocardiography accurately represents invasive PA pressures for population studies but may be misleading for individual diagnosis owing to modest precision and frequent misclassification. Recommendation-based estimates of RAPmean may not necessarily contribute to greater accuracy of PA pressure estimates.

scholarly journals Feasibility and safety of the antecubital venous access for right heart catheterization in patients with pulmonary hypertension

2020 ◽  
Vol 10 (1) ◽  
pp. 204589401987538
Author(s):  
Avital Avriel ◽  
Michael Kassirer ◽  
Avi Shimony ◽  
Gal Tsaban ◽  
Amir Bar-Shai ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Center ◽  
Academic Medical Center ◽  
Venous Access ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial

Background The diagnosis of pulmonary arterial hypertension requires right heart catheterization (RHC) which is typically performed via proximal venous access (PVA). Antecubital venous access (AVA) is an alternative approach for RHC that can minimize complications, decrease procedural duration and allow for immediate patient recovery. A direct comparison between the two procedures in patients with pulmonary hypertension (PH) is lacking. Objectives To determine the feasibility, safety, and adoption rates of AVA-RHC as compared with ultrasound-guided PVA in a subpopulation of patients with PH. Methods All patients who underwent RHC for evaluation of PH between December 2014 and March 2017 at a single large academic medical center were included in this study. Demographic, procedural and outcomes data were retrieved from the medical records. Results In total, 159 RHC were included (124 AVA, 35 PVA). The duration of RHC was significantly shorter in the AVA compared with PVA group (53 (IQR 38–70) vs. 80 (IQR 56–95) min, respectively, p < 0.001). 19% of AVA (24/124) procedures were switched to PVA. Failed attempts at AVA were more common in scleroderma (50% failure rate). Success rate of AVA increased from 81.2% to 93.3% from the first to last quartile. Fluoroscopy time was similar in both groups, the difference between the groups in the radiation dose are not statistically significant (54.5 (IQR 25–110) vs. 84.5 (IQR 30–134)). Conclusion AVA-RHC is a feasible and safe alternative to PVA in patients with PH who are evaluated for pulmonary arterial hypertension diagnosis. Our experience and rapid adoption rate support the use of AVA as the preferred access site for RHC in uncomplicated PH patients.

scholarly journals Anti-synthetase syndrome-associated pulmonary veno-occlusive disease

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093528 ◽  
Author(s):  
Dana Kay ◽  
Ferdous Kadri ◽  
Garrett Fitzpatrick ◽  
Hassan Alnuaimat ◽  
Raju Reddy ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Occlusive Disease ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Pulmonary Arterial Hypertension Therapy

Pulmonary arterial hypertension has been reported with a prevalence of 7.9% in patients with anti-synthetase syndrome; however, anti-synthetase syndrome associated with pulmonary veno-occlusive disease (PVOD) has never before been described in the literature. We present a novel case of anti-synthetase syndrome-associated PVOD in a patient who presented with hypoxic respiratory failure associated with right heart failure and was diagnosed with anti-synthetase syndrome based on his autoimmune serology and pre-capillary pulmonary hypertension on right heart catheterization. He was initiated on pulmonary arterial hypertension therapy, but with escalating dose of parenteral epoprostenol, experienced acute clinical worsening with chest imaging concerning for PVOD that was confirmed on autopsy. Anti-synthetase syndrome can be associated with PVOD, and it should be suspected in patients who have evidence of pre-capillary pulmonary hypertension and who deteriorate with the initiation of pulmonary hypertension-specific therapy.

scholarly journals Cardiopulmonary exercise testing in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis

Rheumatology ◽  
2019 ◽  
Vol 59 (7) ◽  
pp. 1581-1586 ◽  
Author(s):  
Alessandro Santaniello ◽  
Rosa Casella ◽  
Marco Vicenzi ◽  
Irene Rota ◽  
Gaia Montanelli ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Positive Predictive Value ◽  
Predictive Value ◽  
Cardiopulmonary Exercise Testing ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Cardiopulmonary Exercise ◽  
Pulmonary Arterial

Abstract Objectives The DETECT algorithm has been developed to identify SSc patients at risk for pulmonary arterial hypertension (PAH) yielding high sensitivity but low specificity, and positive predictive value. We tested whether cardiopulmonary exercise testing (CPET) could improve the performance of the DETECT screening strategy. Methods Consecutive SSc patients over a 30-month period were screened with the DETECT algorithm and positive subjects were referred for CPET before the execution of right-heart catheterization. The predictive performance of CPET on top of DETECT was evaluated and internally validated via bootstrap replicates. Results Out of 314 patients, 96 satisfied the DETECT application criteria and 54 were positive. PAH was ascertained in 17 (31.5%) and pre-capillary pulmonary hypertension in 23 (42.6%) patients. Within CPET variables, the slope of the minute ventilation to carbon dioxide production relationship (VE/VCO2 slope) had the best performance to predict PAH at right-heart catheterization [median (interquartile range) of specificity 0.778 (0.714–0.846), positive predictive value 0.636 (0.556–0.750)]; exploratory analysis on pre-capillary yielded a specificity of 0.714 (0.636–0.8) and positive predictive value of 0.714 (0.636–0.8). Conclusion In association with the DETECT algorithm, CPET may be considered as a useful tool in the workup of SSc-related pulmonary hypertension. The sequential determination of the VE/VCO2 slope in DETECT-positive subjects may reduce the number of unnecessary invasive procedures without any loss in the capability to capture PAH. This strategy had also a remarkable performance in highlighting the presence of pre-capillary pulmonary hypertension.

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