198 Intrapulmonary shunt assessment in pulmonary arterial hypertension

Abstract Aims Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. It is characterized by the presence of mean pulmonary arterial pressure (mPAP) ≥25 mmHg along with a pulmonary arterial wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) >3 Wood units at right heart catheterization, in the absence of other causes of pre-capillary PH such as PH due to lung diseases, chronic thromboembolic PH, or other rare diseases. Hypoxaemia is a frequent finding in patients with PAH and could be related to ventilation–perfusion mismatch, reduced diffusing capacity, decreased cardiac output, or the opening of intrapulmonary (IP) or intracardiac shunt. Purpose of the present study is to detect IP shunts in PAH patients and its determinants. Methods and results We retrospectively enrolled 29 PAH patients, collecting clinical parameters, haemodynamic and blood gas analysis at baseline and after specific therapies at follow-up. Shunt fraction was calculated by the formula (Cc—Ca)/(Cc—Cv) during oxygen supplementation (FiO2 100%). Intracardiac defects were excluded by echo contrast examination. As expected, after treatments our results showed a significant decrease of PAPm (−7.2 ± 11.6 mmHg) and a significant decrease of PVR (−2.1 ± 3.9 WU). However, it was reported a statistically significant decrease in Hb value (−1.2 ± 1.7 g/dl), in SpO2 (−2.1 ± 3.8%) and in the alveolar–arterial oxygen gradient (a-ADO2) (+54.5 ± 113.1 mmHg). 6MWT and NYHA decreased at follow-up but not statistically significance was detected. IP shunt increase was detected at follow-up after specific treatments (delta Shunt +6.9 ± 6.5%). At multivariate analysis delta PVR remains the only independent determinants of delta Shunts with a significative increment of shunts when PVR are reduced by more than three WU. Conclusions Specific PAH treatments determine a decrease of PVR, but a reverse correlation with IP shunt was noticed. Increase of IP shunt could be not a favourable clinical feature. In fact high IP shunt fraction could determine hypoxaemia and the need of a chronic oxygen supply therapy. Although a not statistically significant decrease of 6MWT and NYHA was found, it is reasonable that a long-standing hypoxaemia could reduce the aerobic function capacity. Ours results unfortunately were deeply influenced by the loss of a great part of elective patients during the pandemic, with the most part of data coming from patients needing hospitalization. This could explain why the delta shunt fraction is higher than expected.

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Follow-up tricuspid annular plane systolic excursion predicts survival in pulmonary arterial hypertension

Pulmonary Circulation ◽

10.1177/2045893217694175 ◽

2017 ◽

Vol 7 (2) ◽

pp. 361-371 ◽

Cited By ~ 24

Author(s):

Jeremy A. Mazurek ◽

Anjali Vaidya ◽

Stephen C. Mathai ◽

Justin D. Roberts ◽

Paul R. Forfia

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Prognostic Significance ◽

Functional Class ◽

Heart Catheterization ◽

Pulmonary Arterial ◽

Group 2 ◽

One Year ◽

Group 1

Few studies have examined the utility of serial echocardiography in the evaluation, management, and prognosis of patients with pulmonary arterial hypertension (PAH). Therefore, we sought to evaluate the prognostic significance of follow-up tricuspid annular plane systolic excursion (TAPSE) in PAH. We prospectively studied 70 consecutive patients with PAH who underwent baseline right heart catheterization (RHC) and transthoracic echocardiogram, who survived to follow-up echocardiogram after initiation of PAH therapy. Baseline TAPSE was 1.6 ± 0.5 cm which increased to 2.0 ± 0.4 cm on follow-up ( P < 0.0001). The cohort was dichotomized by TAPSE at one-year follow-up: Group 1 (n = 37): follow-up TAPSE ≥ 2 cm; Group 2 (n = 33): follow-up TAPSE < 2 cm. Group 1 participants were significantly more likely to reach WHO functional class I–II status and achieve a higher six-minute walk distance on follow-up. Of the 68 patients who survived more than one year, 18 died (26.5%) over a median follow-up of 941 days (range, 3–2311 days), with significantly higher mortality in Group 2 versus Group 1 (41.9% vs. 13.5%; P = 0.003). While baseline TAPSE stratified at 2 cm did not predict survival in this cohort, TAPSE ≥ 2 cm at follow-up strongly predicted survival in bivariable models (hazard ratio, 0.21; 95% confidence interval, 0.08–0.60). In conclusion, follow-up TAPSE ≥ 2 cm is a prognostic marker and potential treatment target in a PAH population.

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

Rheumatology ◽

10.1093/rheumatology/kez336 ◽

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.

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Seizures in Idiopathic Pulmonary Arterial Hypertension

International Journal of Epilepsy ◽

10.1055/s-0039-1688528 ◽

2018 ◽

Vol 05 (02) ◽

pp. 107-109

Author(s):

Jamir Pitton Rissardo ◽

Ana Letícia Fornari Caprara

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Supplemental Oxygen ◽

Right Heart Catheterization ◽

Idiopathic Pulmonary Arterial Hypertension ◽

Heart Catheterization ◽

Right Heart ◽

Systemic Disorder ◽

Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a progressive pulmonary vasculopathy. A 29-year-old female patient presenting with dyspnea and syncope within 6 hours of onset was admitted to our hospital. The patient stated that she looked for a neurologist months ago because she experienced abrupt shaking limbs occurring during physical activity. She was diagnosed with focal seizure, and carbamazepine (CBZ) was started. On admission, she reported that the dyspnea had started in the last week and recurrent episodes of syncope in the last few hours. A right heart catheterization was diagnostic of PAH. She was started on spironolactone, furosemide, sildenafil, warfarin, and supplemental oxygen. On 10th admission day, the patient was seizure free and the dose of CBZ was tapered. In the follow-up, the patient remained seizure free. An investigation to search for a chronic lung disease or hypoxemia, systemic disorder, hematological disorder, and metabolic disorder was negative.

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4972Upfront triple combination therapy with ambrisentan, tadalafil and subcutaneous treprostinil in incident patients with severe pulmonary arterial hypertension

European Heart Journal ◽

10.1093/eurheartj/ehz746.0031 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

M D'Alto ◽

E Romeo ◽

P Argiento ◽

R Badagliacca ◽

S Papa ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Combination Therapy ◽

Arterial Hypertension ◽

Arterial Compliance ◽

Right Atrial ◽

Heart Catheterization ◽

Triple Combination ◽

Triple Combination Therapy ◽

Pulmonary Arterial

Abstract Aim Current treatments strategies for high-risk patients with pulmonary arterial hypertension (PAH) are based on the use of parenteral prostanoids. The evidence to support triple upfront combination therapy remains largely based on expert consensus or small studies. Aim of this study was to evaluate the efficacy and safety of an upfront triple combination therapy with ambrisentan, tadalafil and subcutaneous (sc) treprostinil in patients with severe PAH. Methods This is a multi-center retrospective analysis of patients with newly diagnosed severe PAH treated with upfront triple combination therapy with ambrisentan, tadalafil and sc treprostinil between 2014 and 2018. Clinical evaluations, WHO functional class (FC), 6-min walk distance, biomarkers and right heart catheterization were collected from the patients' medical records at baseline and during the follow-up. Results Overall, 20 patients (mean age 44±15 years, 15 female) were included. Over a median follow-up of 12 months, all patients were still alive on triple combination therapy. At baseline 11 patients were in WHO-FC 3 and 9 patients in WHO-FC 4. At follow-up, WHO-FC (2.0±0.5 vs 3.5±0.5, p<0.001, improved in all: 2, 16, and 2 patients in FC 1, 2 and 3, respectively), exercise capacity (431±67 vs 152±130 m, p<0.001), NT-proBNP (423±260 vs 3492±1864 pg/ml; p<0.001), and haemodynamics (right atrial pressure 5±2 vs 13±3 mmHg, p<0.001; mean pulmonary artery pressure 42±5 vs 60±9 mmHg, p<0.001; cardiac index 3.5±0.8 vs 1.8±0.3 l/min/m2, p<0.001; pulmonary vascular resistance 5.5±1.3 vs 16.4±4.4 Wood units, p<0.001; pulmonary arterial compliance 2.5±0.9 vs 0.8±0.3 ml/mmHg, p<0.001) significantly improved compared with baseline. No patient discontinued the therapy due to serious adverse events. Conclusions Triple upfront combination therapy with ambrisentan, tadalafil and sc treprostinil is safe and offers clinical and heamodynamics benefits in incident patients with severe PAH.

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The haemodynamic assessment of patients with pulmonary arterial hypertension

Global Cardiology Science and Practice ◽

10.21542/gcsp.2020.4 ◽

2020 ◽

Vol 2020 (1) ◽

Author(s):

Stefano Ghio

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Drug Efficacy ◽

Right Heart Catheterization ◽

Heart Catheterization ◽

Right Heart ◽

Accurate Knowledge ◽

Pulmonary Arterial ◽

Technical Recommendations

[No abstract. Showing first paragraph of article]Right heart catheterization (RHC) has a crucial role in the management of patients with pulmonary hypertension (PH). Firstly, it is mandatory in subjects with the suspicion of PH to confirm the diagnosis. Secondly, it is strongly recommended in the follow-up of patients with confirmed pulmonary arterial hypertension (PAH) to evaluate drug efficacy and in case of clinical deterioration.In order to always perform a complete and correct RHC, clinicians carrying out the examination must be guided not only by technical recommendations, but also by the accurate knowledge of the different clinical issues which have to be addressed in these different situations.

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SAT0240 THE PROGNOSIS OF TWO DISTINCT CLINICAL PHENOTYPES OF SLE-PAH

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4520 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1063.2-1063

Author(s):

J. Wang ◽

Y. Feng ◽

Y. Lei ◽

X. Zhang

Keyword(s):

Systemic Lupus Erythematosus ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Lupus Erythematosus ◽

Right Heart Catheterization ◽

Heart Catheterization ◽

Systemic Lupus ◽

Clinical Phenotypes ◽

Weighted Score ◽

Pulmonary Arterial

Background:Based on the characteristics of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH), Sunet alhas put forward a scoring system to distinguish two clinical phenotypes as vasculitic and vasculopathic subtypes[1]. A weighted score ≥2 suggested a vasculitic subtype by combining two factors: The time interval between SLE and PAH diagnosis <2 years and ≥2 years were 1 and 0 point; SLE Disease Activity Index (SLEDAI) >9, 5-9 and <5 were 2, 1, 0 point, respectively. While the vasculitic subtype seemed to have poorer prognosis in Sun’s research, other study has shown controversial result[2].Objectives:To find out the prognosis of two distinct clinical phenotypes of SLE-PAH.Methods:Between 2008 and 2019, a SLE-PAH cohort confirmed by right heart catheterization (RHC) from Guangdong Provincial People’s Hospital was included. Other groups of pulmonary hypertension were excluded. Based on the scoring system, patients were divided into vasculitic (weighted score≥2) and vasculopathic subtypes (weighted score<2). The endpoint was PAH-related mortality. Survival status were confirmed by clinic follow-up data or phone call.Results:A total of 53 SLE-PAH patients were enrolled. The cases of vasculitic and vasculopathic subtype were 14 and 39, respectively. Ten endpoint events occurred. Eight attributed to PAH and the cause could not be traced in two which were still included in study. The pooled 1-, 3-, 5-year survival rates were 85.7%, 78.6%, 65.5% in vasculitic subtype, and 93.9%, 87.5%, 87.5% in vasculopathic subtype, respectively. Kaplan-Meier analysis showed vasculitic subtype tended to have a poorer prognosis than vasculopathic subtype (p=0.16, HR 2.4, 95%CI 0.5-13.8, figure 1).Figure 1.Survival curves for patients with systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) in two distinct subtypes. RHC, Right Heart Catheterization.Conclusion:The prognosis of the two phenotypes of SLE-PAH was statistically indifferent while the vasculitic subtype showed a trend of worse prognosis. Further studies are needed.References:[1]F. Sun, Y. Lei, W. Wu, L. Guo, K. Wang, Z. Chen, W. Xu, X. Wang, T. Li, X. Zhang, S. Ye, Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus, Ann Rheum Dis 78(1) (2019) 148-150.[2]J. Qian, M. Li, J. Zhao, Q. Wang, Z. Tian, X. Zeng, Inflammation in SLE-PAH: good news or not?, Ann Rheum Dis (2018).0:1–2. doi:10.1136/annrheumdis-2018-214605Disclosure of Interests:None declared

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Change of myocardial fluorodeoxyglucose uptake distribution after specific therapy of pulmonary arterial hypertension patients

European Heart Journal ◽

10.1093/ehjci/ehaa946.2306 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

R Kazimierczyk ◽

P Szumowski ◽

L.M Malek ◽

P Blaszczak ◽

D Jurgilewicz ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Glucose Uptake ◽

Funding Source ◽

Hemodynamic Parameters ◽

Fdg Uptake ◽

Baseline Visit ◽

Pulmonary Arterial ◽

Rv Function

Abstract Background Right ventricular (RV) function is a major determinant of survival in patients with pulmonary arterial hypertension (PAH). In our previous study, we confirmed that increased RV fluorodeoxyglucose (FDG) uptake in positron emission tomography (PET) (presented as higher ratio of FDG uptake of RV to LV) was associated with progressive RV dysfunction and preceded hemodynamic and clinical deterioration in PAH patients. Now, we obtained second PET/MRI assessments of the study group after 2-years of PAH-targeted treatment. Aim The aim of the study was to obtain change of cardiac FDG uptake in PAH patients after follow-up period and to indicate factors mainly affecting this change. Methods Twenty-eight PAH patients (51.32±15.91 years) had simultaneous PET/MRI scans performed during baseline visit. FDG was used and its uptake was quantified as mean standardized uptake value (SUV) for both left (LV) and RV. Second PET/MRI assessments were done after 2 years in the group of twenty patients (four deaths, four patients did not agree to perform additional scans). Results After follow-up period, we observed significant change of MRI-derived RV ejection fraction (45±10% to 51.2±12.7%, p=0.03), and improvement in hemodynamic parameters obtained from right heart catheterization (RHC) e.g. mean pulmonary artery pressure, mPAP (48.5±17.2 to 41.8±17.1 mmHg, p=0.01) and pulmonary vascular resistance, PVR (8.7±5.3 to 7.0±4.2 WU, p=0.04). Follow-up SUVRV/SUVLV ratio significantly correlated with follow-up RV hemodynamic parameters confirming relationship between RV function and cardiac metabolic alterations (Table 1). Interestingly, patients who had improvement in SUVRV/SUVLV (lower follow-up value than baseline, n=11) had significantly higher mPAP at baseline visit (58.9±18.7 vs 40.3±11.8 mmHg, p=0.02), suggesting that RV FDG accumulation in advanced PAH may decrease after the PAH-specific treatment in accordance with the degree of reduction in the pulmonary vascular resistance. Conclusion Impaired RV function is associated with increased glucose uptake of RV myocytes estimated by FDG PET in PAH patients. Targeted treatment may improve RV function and thus affect previously altered cardiac glucose uptake. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Polish National Science Centre

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