Incidence of new pulmonary hypertension and determining factors during follow-up of patients with systemic sclerosis after negative right heart catheterisation

The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide (DLCO) <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses.96 patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at baseline were followed for 2.95±0.7 years (median 3 years). Of these, 71 had a second RHC; 18 of these 71 patients (25.3%) developed PH, and five (7%) developed SSc-associated pulmonary arterial hypertension. For patients with an mPAP of 21–24 mmHg at baseline, the likelihood of presenting with PH as opposed to normal pressures on follow-up was significantly higher (p=0.026). Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up.In a selected cohort of SSc patients with a DLCO <60%, pulmonary pressures appeared to rise progressively during follow-up. In this population, it was possible to identify manifest PH in almost 25% of patients using prospective RHC during follow-up. Therefore, regular clinical assessment including RHC might be useful in patients with SSc.

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Right heart catheterisation: best practice and pitfalls in pulmonary hypertension

European Respiratory Review ◽

10.1183/16000617.0062-2015 ◽

2015 ◽

Vol 24 (138) ◽

pp. 642-652 ◽

Cited By ~ 51

Author(s):

Stephan Rosenkranz ◽

Ioana R. Preston

Keyword(s):

Pulmonary Hypertension ◽

Clinical Practice ◽

Best Practice ◽

Data Interpretation ◽

Volume Measurement ◽

Right Heart ◽

Heart Catheterisation ◽

Right Heart Catheterisation ◽

Pulmonary Arterial

Right heart catheterisation (RHC) plays a central role in identifying pulmonary hypertension (PH) disorders, and is required to definitively diagnose pulmonary arterial hypertension (PAH). Despite widespread acceptance, there is a lack of guidance regarding the best practice for performing RHC in clinical practice. In order to ensure the correct evaluation of haemodynamic parameters directly measured or calculated from RHC, attention should be drawn to standardising procedures such as the position of the pressure transducer and catheter balloon inflation volume. Measurement of pulmonary arterial wedge pressure, in particular, is vulnerable to over- or under-wedging, which can give rise to false readings. In turn, errors in RHC measurement and data interpretation can complicate the differentiation of PAH from other PH disorders and lead to misdiagnosis. In addition to diagnosis, the role of RHC in conjunction with noninvasive tests is widening rapidly to encompass monitoring of treatment response and establishing prognosis of patients diagnosed with PAH. However, further standardisation of RHC is warranted to ensure optimal use in routine clinical practice.

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Exercise right heart catheterisation before and after pulmonary endarterectomy in patients with chronic thromboembolic disease

European Respiratory Journal ◽

10.1183/13993003.00458-2018 ◽

2018 ◽

Vol 52 (3) ◽

pp. 1800458 ◽

Cited By ~ 10

Author(s):

Stefan Guth ◽

Christoph B. Wiedenroth ◽

Andreas Rieth ◽

Manuel J. Richter ◽

Ekkehard Gruenig ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Thromboembolic Disease ◽

Pulmonary Endarterectomy ◽

Right Heart ◽

Heart Catheterisation ◽

Right Heart Catheterisation ◽

Before And After ◽

The Mean ◽

Chronic Thromboembolic Disease ◽

The Impact

Symptomatic patients with chronic thromboembolic disease (CTED) without pulmonary hypertension often show an excessive increase in mean pulmonary arterial pressure (MPAP) during exercise.We report on the impact of pulmonary endarterectomy (PEA) on pulmonary haemodynamics in a prospective series of 32 consecutive CTED patients who underwent PEA. All patients had a comprehensive diagnostic work-up including right heart catheterisation at baseline and 12 months after PEA. Furthermore, in 12 patients exercise right heart catheterisation was performed before and after PEA.After PEA, MPAP was lower at rest (20±3 versus 17±3 mmHg; p=0.008) and during maximal exercise (39±8 versus 31±6 mmHg; p=0.016). The mean total pulmonary resistance (TPR) decreased from 3.6±0.8 Wood Units (WU) pre-operatively to 2.7±0.7 WU 1 year after PEA (p=0.004) and the mean slope of the MPAP/cardiac output (CO) relationship decreased from 3.6±1.0 to 2.3±0.8 WU (p=0.002). Peak oxygen uptake increased from 1.2±0.4 to 1.5±0.3 L·min−1 (p=0.014) and ventilatory equivalents of carbon dioxide decreased from 39±2 to 30±2 (p=0.002). There was a significant improvement in quality of life assessed by the Cambridge Pulmonary Hypertension Outcome Review questionnaire.In CTED patients, PEA resulted in haemodynamic and clinical improvements. The means of TPR and MPAP/CO slopes decreased to <3.0 WU.

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Residual Pulmonary Hypertension More than 20 Years after Repair of Shunt Lesions

Medicina ◽

10.3390/medicina56060297 ◽

2020 ◽

Vol 56 (6) ◽

pp. 297

Author(s):

Dovilė Jančauskaitė ◽

Virginija Rudienė ◽

Gabrielius Jakutis ◽

Laurie W Geenen ◽

Jolien W Roos-Hesselink ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Surgical Repair ◽

Significant Proportion ◽

University Hospital ◽

Right Heart ◽

Long Term Outcome ◽

Right Heart Catheterisation ◽

Study Results

Background and Objectives: After successful surgical repair of a congenital shunt lesion, pulmonary hypertension (PH) often disappears. However, PH can persist long-term after the closure. This study aimed to assess the prevalence of PH long-term after surgical repair of congenital heart disease (CHD), and to evaluate the outcomes and preoperative factors related to residual PH. Materials and Methods: In this retrospective cohort study, we reviewed patients who underwent right heart catheterisation in Vilnius University Hospital Santaros Klinikos during the period of 1985–2007. Among 4118 right heart catheterisations performed, 160 patients underwent congenital systemic-to-pulmonary shunt repair at a young age (<18 years) and had pre-operative PH. Half of the patients were foreigners whose follow-up data were unavailable. Eventually, 88 patients with available follow-up data were included in this study. Results: The median age at diagnosis of CHD with PH was 0.8 (0.6–3.0) and 1.1 (0.6–3.9) years at surgery (50% females). Residual PH was assessed 9.5 years after surgery and observed in 30.7% (n = 27) of the patients. It was associated with having more than one shunt (44.4% (n = 12), p = 0.016) and higher median pulmonary vascular resistance (3.4 (2.5–6.5) vs. 2.2 (1.0–3.7), p = 0.035) at baseline. After a median follow-up of 21 (15–24) years, 9.1% of the patients were deceased. Kaplan–Meier survival analysis revealed significantly higher mortality in the residual PH group (p = 0.035). Conclusions: Residual PH affects a significant proportion of patients after surgical repair of a shunt lesion and is associated with worse long-term outcome.

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Faculty Opinions recommendation of Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.722035117.793510066 ◽

2015 ◽

Author(s):

Marilyn Telen

Keyword(s):

Pulmonary Hypertension ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease ◽

Right Heart ◽

Heart Catheterisation ◽

Right Heart Catheterisation

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Exercise pulmonary haemodynamics predict outcome in patients with systemic sclerosis

European Respiratory Journal ◽

10.1183/13993003.00990-2016 ◽

2016 ◽

Vol 48 (6) ◽

pp. 1658-1667 ◽

Cited By ~ 33

Author(s):

Adriana Stamm ◽

Stéphanie Saxer ◽

Mona Lichtblau ◽

Elisabeth D. Hasler ◽

Suzana Jordan ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Systemic Sclerosis ◽

Pulmonary Artery ◽

Cox Regression ◽

Early Stage ◽

Free Survival ◽

Right Heart Catheterisation ◽

Exercise Induced ◽

Wedge Pressure

The aim of the present study was to investigate the prognostic value of exercise haemodynamics measured during right heart catheterisation (RHC) in patients with systemic sclerosis (SSc) referred for evaluation of pulmonary hypertension.SSc patients undergoing RHC at rest and during maximal supine incremental cycle exercise were grouped into resting precapillary pulmonary hypertension (PHrest) (mean pulmonary artery pressure (mPAP) ≥25 mmHg, pulmonary artery wedge pressure <15 mmHg), exercise-induced pulmonary hypertension (PHex) (mPAP ≥30 mmHg and mPAP/cardiac output >3 mmHg·L−1·min−1 at maximal exercise), and without pulmonary hypertension (PHnone). Patients' characteristics, haemodynamics and follow up data were compared between groups.72 SSc patients were followed for median (interquartile range) 33 (15–55) months. Mean (95% CI) survival without transplantation estimated by Kaplan-Meyer analysis was 4.4 (0.8–2.9) years in PHrest (n=17), 5.2 (4.4–6.1) years in PHex (n=28) and 9.5(8.4–10.6) years in PHnone (n=27; p<0.05 versus others). In Cox regression models, the exercise-induced increase in mPAP (hazard ratio (HR) 1.097, 95% CI 1.002–1.200) and the coefficient of pulmonary vascular distensibility alpha (HR 0.100, 95% CI 0.012–0.871) controlled for age, but not resting haemodynamics predicted transplant-free survival.Among SSc patients with normal mPAP at rest, an excessive increase in mPAP during exercise and an impaired vascular distensibility may indicate an early stage of pulmonary vasculopathy, associated with reduced survival similar to resting pulmonary hypertension patients.

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