Abstract 17272: Frequency of Acute Vasodilator Response in Incident and Prevalent Patients With Pulmonary Arterial Hypertension (Group 1): Results From the Pulmonary Vascular Disease Omics (PVDOMICS) Study

Introduction: The prevalence of acute vasodilator response (AVR) to inhaled NO (defined as a reduction in mPAP ≥ 10 mmHg with an absolute value of mPAP ≤ 40 mmHg with increased/unchanged CO) is reported to be around 12% in incident patients with idiopathic PAH (IPAH). The prevalence of AVR, however, is reportedly lower in other disease-associated PAH in Group 1, such as connective tissue disease (CTD). Furthermore, the prevalence of AVR for combined inhaled NO and oxygen, and in patients on PAH therapy (prevalent cohort), is less known. Hypothesis: We hypothesized there would be differences in the prevalence of AVR in PAH subgroups in the large PVDOMICS cohort of incident and prevalent patients. Methods: Group 1 PAH patients enrolled in PVDOMICS underwent right heart catheterization for baseline measurements. AVR was then measured in response to 100% inhaled oxygen (O 2 ) and 100% O 2 plus NO at 40 ppm (O 2 +NO) for 5 min each before hemodynamic measurements. Details of the PVDOMICS methodology and core adjudication of hemodynamic measurements were reported previously. Rates of AVR to 100% O 2 and 100% O 2 +NO were compared between incident and prevalent patients in each PAH subgroup. Results: 351 patients, mostly female (73%), average age of 52.9 years, with mostly prevalent disease (87%) and an average of 4 years from PAH diagnosis, underwent AVR assessment. A positive AVR was found in 0.6% of patients in response to 100% oxygen and 6% of patients in response to 100%+NO for the overall cohort. AVR was similar in incident and prevalent IPAH patients (6 and 6.9%, respectively). It was, however, 0% and 6.9%, in incident vs. prevalent CTD-PAH patients, respectively. There were no responders to either challenge in any other PAH subgroup. Conclusions: The prevalence of AVR is relatively rare in IPAH and CTD-PAH (~ 6%) and absent in other subgroups. There was no response to 100% O2 alone, suggesting specificity of AVR to NO in PAH. AVR is more common than previously reported in CTD-PAH, but only in prevalent disease. Whether the latter is related to long-term PAH therapy affecting pulmonary vasomotor response and/or vascular remodeling in these patients would warrant further studies.

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Potential role of exercise echocardiography and right heart catheterization in the detection of early pulmonary vascular disease in patients with systemic sclerosis

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198319849805 ◽

2019 ◽

Vol 4 (3) ◽

pp. 219-224

Author(s):

Gabor Kovacs ◽

Horst Olschewski

Keyword(s):

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Vascular Disease ◽

Right Heart Catheterization ◽

Pulmonary Vascular Disease ◽

Heart Catheterization ◽

Right Heart ◽

Pulmonary Hemodynamics ◽

Pulmonary Arterial

Pulmonary vascular disease represents one of the most frequent complications in systemic sclerosis leading to increased mortality. The recognition and appropriate clinical management of early pulmonary vascular disease could significantly improve the prognosis of affected patients. Early pulmonary vascular disease is characterized by the histological signs of pulmonary vascular remodeling, mildly increased mean pulmonary arterial pressure (21–24 mmHg) at rest, abnormal pulmonary hemodynamics during exercise, decreased exercise capacity, and a high risk for development of pulmonary arterial hypertension. Pulmonary hemodynamics can be investigated during exercise by echocardiography or by right heart catheterization both representing important clinical tools for the screening and confirmation of early pulmonary vascular disease. Further studies are needed to better understand the clinical course of systemic sclerosis patients with early pulmonary vascular disease and to define the characteristics of patients that will or will not profit from pulmonary arterial hypertension treatment.

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PULMONARY HYPERTENSION STILL AN ‘ORPHAN LUNG DISEASE’ IN PAKISTAN

Pakistan Heart Journal ◽

10.47144/phj.v54i1.2056 ◽

2021 ◽

Vol 54 (1) ◽

Author(s):

Faisal Asad

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Heart Catheterization ◽

Targeted Treatment ◽

Heart Catheterization ◽

Right Heart ◽

Pulmonary Arterial ◽

Group 1

In our country when it comes to diagnosis and treatment of Pulmonary Hypertension (PH) especially Pulmonary Arterial Hypertension (PAH), it seems that it is still an Orphan Lung disease. Whether it is data about its prevalence in our country, available diagnostic services or treatment options, one may easily feel the scarcity in all mentioned areas. Most current classification of PH, categorizes it into 5 groups.1 However this categorization requires availability of diagnostic tools especially right heart catheterization data to classify and know the severity of PH and to offer targeted treatment to patients who belong to group 1 PH i.e. Pulmonary Arterial Hypertension (PAH). Studies done about PH prevalence in Pakistan have mainly focused on data in selected patients group e.g. in patients with Chronic Obstructive Pulmonary Diseases (COPD) or Systemic Lupus Erythematosus (SLE).2,3 All of these studies relied on diagnosis without using the diagnostic bench mark i.e. Right heart catheterization. Moreover, effects of targeted treatment for PAH have also been studied only in selected group of patients e.g. COPD.4,5 While above work by the learned colleagues has to be appreciated, it emphasizes the need to, Spread the awareness about PH more so to reduce inadvertent use of targeted treatment which may prove detrimental outside group 1 PH and should only be considered at specialized centers. Develop specialized centers with required diagnostic services especially Lung functional and anatomical evaluation and Right heart catheterization. Establish PH registries (at least one in each province) which can play a vital role to collect data and ensure effective evidence based patient management. This may be an uphill task however the right direction to move forward. REFERENCES Simonneau G, Montani D, David SC, Christopher PD, Michael AG, Michael K, at el. Paul G. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. Amir S, Azmat A, Raza U, Hamid N, Zafar I, Shahida N, at el. Frequency of Pulmonary Hypertension in COPD patients. Pak J Chest Med. 2017;23(4):144-50. Sadia A, Aflak R, Tafazzul H, Ahmed A. Frequency and predictors of Pulmonary Hypertension in patients with SLE. Pak J Med Sci. 2019;35(1):86-9. Nasir H, Faisal F, Kausar R, Saadia A, Anjum N, Shafi K, at el. Efficacy of bosentan in the treatment of pulmonary hypertension; a prospective study focusing on safety and efficacy in patients with COPD. J Lung Pulm Respir Res. 2018;5(2):67-72. Sharieff S. Effect of sildenafil in primary pulmonary hypertension. Pak J Med Sci. 2007;23(2):264.

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Abstract 17438: Misclassification of the Cause of Dyspnea by Resting Right Heart Catheterization: the Impact of Invasive Cardiopulmonary Exercise Testing

Circulation ◽

10.1161/circ.130.suppl_2.17438 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Mário Santos ◽

Aaron B Waxman ◽

Julie Tracy ◽

Fariha Khalid ◽

Alexander R Opotowsky ◽

...

Keyword(s):

Exercise Testing ◽

Cardiopulmonary Exercise Testing ◽

Right Heart Catheterization ◽

Peak Exercise ◽

Pulmonary Vascular Disease ◽

Heart Catheterization ◽

Right Heart ◽

Cardiopulmonary Exercise ◽

Pulmonary Arterial ◽

The Impact

Introduction: Supine resting right heart catheterization (srRHC) is the standard method to differentiate pulmonary arterial hypertension (PAH) and heart failure with preserved ejection fraction (HFpEF), but most such patients complain of symptoms during exercise. We hypothesized that the upright invasive exercise phenotype of patients with unexplained exertional intolerance provides a distinct and additive perspective compared with supine resting RHC in the diagnosis of PAH and HFpEF. Methods: We reviewed results of consecutive patients with unexplained effort intolerance who underwent same day sequential srRHC and invasive cardiopulmonary exercise testing (iCPET) between March 2011 and October 2013. At rest, patients were classified with PAH if mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg and pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg; as HFpEF if PAWP > 15 mmHg; and as normal if none of the above hemodynamic criteria were met. At peak exercise, patients were categorized as exercise-induced PAH (eiPAH), exercise HFpEF (eHFpEF), normal (eNormal), or peripheral limitation according to the criteria displayed in the table. Results: Of 255 patients, 212 (83%) had normal srRHC. Of these, 46 (22%) had an abnormal iCPET result: eiPAH (n=24), eHFpEF (n=22). A resting mPAP > 18 mmHg discriminated eiPAH reasonably well (ROC AUC: 0.75; 95%CI: 0.67-0.83). Of those with abnormal srRHC, iCPET reclassified diagnosis for 16/43 (37%). Of the 30 patients who had HFpEF by srRHC, 12 (40%) had a normal cardiac hemodynamic profile during upright maximum exercise. 4 (31%) of the 13 patients with PAH at rest had no suggestion of intrinsic pulmonary vascular disease during exercise (3 eHFpEF and 1 non-cardiac limitation) with iCPET. Conclusions: In patients with exertional intolerance, iCPET reveals hemodynamic abnormalities which are overlooked with resting RHC and reclassifies a significant subset of apparent PAH and HFpEF patients by srRHC.

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Pulmonary arterial hypertension unveils itself: a cancer-like progression — a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytab149 ◽

2021 ◽

Vol 5 (5) ◽

Author(s):

Cátia Santos-Ferreira ◽

Daniela Cardoso ◽

Benedita Paiva ◽

Rui Baptista

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Lung Biopsy ◽

Pulmonary Arteries ◽

Right Heart Catheterization ◽

Pulmonary Vascular Disease ◽

Heart Catheterization ◽

Influenza B ◽

Haemodynamic Changes ◽

Pulmonary Arterial

Abstract Background Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis, characterized by progressive remodelling of the small pulmonary arteries that precede the clinical and haemodynamic manifestations of the disease. Thus, a prompt diagnosis and early intervention are crucial. Case summary A 39-year-old pregnant women presented with persistent severe hypoxaemia after the diagnosis of influenza B and an elective caesarean delivery at 33 weeks. Ten months after, an extensive and inconclusive investigation that included a lung biopsy, despite of a spontaneous improvement in oxygen saturation, clinical deterioration led to further testing, namely genetic screening. It revealed a fast-progressing case of hereditary PAH caused by BMRP2 mutation. Discussion This case highlights the challenges of a timely diagnosis of PAH and the importance of close clinical monitoring of patients at high risk of PAH. In addition, it emphasizes the fast development of severe haemodynamic changes associated with a BMPR2 mutation. The availability of a lung biopsy without signs of pulmonary vascular disease (PVD) and a right heart catheterization with mild pulmonary hypertension at the baseline assessment demonstrates that PVD can progress in a neoplastic-like manner in a matter of months.

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Electrical impedance tomography in pulmonary arterial hypertension

PLoS ONE ◽

10.1371/journal.pone.0248214 ◽

2021 ◽

Vol 16 (3) ◽

pp. e0248214

Author(s):

André L. D. Hovnanian ◽

Eduardo L. V. Costa ◽

Susana Hoette ◽

Caio J. C. S. Fernandes ◽

Carlos V. P. Jardim ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Electrical Impedance Tomography ◽

Electrical Impedance ◽

Right Heart Catheterization ◽

Lung Perfusion ◽

Pulmonary Vascular Disease ◽

Heart Catheterization ◽

Impedance Tomography ◽

Pulmonary Arterial

The characterization of pulmonary arterial hypertension (PAH) relies mainly on right heart catheterization (RHC). Electrical impedance tomography (EIT) provides a non-invasive estimation of lung perfusion that could complement the hemodynamic information from RHC. To assess the association between impedance variation of lung perfusion (ΔZQ) and hemodynamic profile, severity, and prognosis, suspected of PAH or worsening PAH patients were submitted simultaneously to RHC and EIT. Measurements of ΔZQ were obtained. Based on the results of the RHC, 35 patients composed the PAH group, and eight patients, the normopressoric (NP) group. PAH patients showed a significantly reduced ΔZQ compared to the NP group. There was a significant correlation between ΔZQ and hemodynamic parameters, particularly with stroke volume (SV) (r = 0.76; P < 0.001). At 60 months, 15 patients died (43%) and 1 received lung transplantation; at baseline they had worse hemodynamics, and reduced ΔZQ when compared to survivors. Patients with low ΔZQ (≤154.6%.Kg) presented significantly worse survival (P = 0.033). ΔZQ is associated with hemodynamic status of PAH patients, with disease severity and survival, demonstrating EIT as a promising tool for monitoring patients with pulmonary vascular disease.

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Systemic Sclerosis and Other Connective Tissue Diseases

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-16.2.55 ◽

2017 ◽

Vol 16 (2) ◽

pp. 55-60

Author(s):

Christopher P. Denton

Keyword(s):

Systemic Sclerosis ◽

Connective Tissue ◽

Lupus Erythematosus ◽

Lung Fibrosis ◽

Connective Tissue Diseases ◽

Right Heart Catheterization ◽

World Health ◽

Heart Catheterization ◽

Pulmonary Arterial ◽

Group 1

Pulmonary hypertension (PH) is an important and relatively frequent complication of connective tissue disease (CTD). It is most often seen as a complication of systemic sclerosis and mixed CTD, but is also recognized in systemic lupus erythematosus and other related conditions. Although PH is defined hemodynamically by mean pulmonary arterial pressure (mPAP) of ≥25 mm Hg at right heart catheterization, and may have a variety of mechanisms, it is World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH) that has deservedly received the most attention in CTD. The inclusion of cases of precapillary PH without other explanation or concurrent lung fibrosis in WHO Group 1 led to availability of licensed therapies and increased the need for screening and treatment. Approaches are now available that detect PAH, and optimal approaches to therapy are included in expert and evidence-based recommendations. Challenges include borderline elevation of mPAP, comorbidity, and mixed-etiology cases. Overall outcomes have improved, and new clinical trials are being performed that will hopefully build on recent progress to further improve outcomes. Other aspects of PH in CTD to consider include forms related to cardiac disease and lung fibrosis or hypoxia, as these may occur as complications of the underlying CTD. Thromboembolic disease may also occur and represents another important differential diagnosis. Finally, it has been reported that pulmonary veno-occlusive disease may occur, which raises diagnostic and treatment difficulties. Overall, there are major challenges in CTDPH, but also opportunities for effective multispecialty care and early detection and diagnosis of potentially treatable cases of PAH.

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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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Plasma exosomal miR-596: a novel biomarker predicts survival in patients with idiopathic pulmonary artery hypertension

Journal of International Medical Research ◽

10.1177/03000605211002379 ◽

2021 ◽

Vol 49 (3) ◽

pp. 030006052110023

Author(s):

Yang Huang ◽

Zuo-Gang Wang ◽

Liang Tang ◽

Su-Gang Gong ◽

Yuan-Yuan Sun ◽

...

Keyword(s):

Right Heart Catheterization ◽

Pulmonary Artery Hypertension ◽

Right Atrial ◽

Heart Catheterization ◽

Distribution Analysis ◽

Poor Overall Survival ◽

Transmission Electron ◽

Exosomal Mirnas ◽

Pulmonary Arterial

Objective To determine if plasma exosomal microRNAs (miRNAs) can predict survival in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods The study enrolled patients with IPAH that underwent right heart catheterization. Plasma was collected and exosomal miRNAs were extracted. Exosomes were evaluated using transmission electron microscopy, Western blot analysis and particle size distribution analysis. MiRNAs were evaluated using a miRNA microarray and validated using real-time polymerase chain reaction. Results This study included 12 patients with IPAH in the study group and 48 patients with IPAH in the validation group. The mean ± SD follow-up duration was 60.3 ± 35.4 months in the overall cohort. The levels of miR-596 were higher in the nonsurvivors compared with the survivors. The levels of miR-596 significantly correlated with survival time, mean right atrial pressure, pulmonary vascular resistance (PVR) and cardiac index. High levels of miR-596 and PVR were significantly associated with poor overall survival. Multivariate analysis demonstrated that exosomal miR-596 (hazard ratio [HR] = 2.119; 95% confidence interval [CI] 1.402, 3.203) and PVR (HR = 1.146; 95% CI 1.010, 1.300) were independent predictors of survival. Conclusions High levels of plasma exosomal miR-596 were significantly associated with disease severity and poor prognosis of patients with IPAH.

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Pathology and Pathobiology of Pulmonary Hypertension

Seminars in Respiratory and Critical Care Medicine ◽

10.1055/s-0037-1606214 ◽

2017 ◽

Vol 38 (05) ◽

pp. 571-584 ◽

Cited By ~ 18

Author(s):

Peter Dorfmüller ◽

Christophe Guignabert

Keyword(s):

Pulmonary Hypertension ◽

Vascular Inflammation ◽

Right Heart Catheterization ◽

Point Of View ◽

Heart Catheterization ◽

Pulmonary Endothelium ◽

Hemodynamic State ◽

Pulmonary Arterial ◽

Genetic And Environmental Factors ◽

And Migration

Pulmonary hypertension (PH) is a hemodynamic state defined by a mean pulmonary artery pressure ≥ 25 mm Hg during resting right heart catheterization. PH can result from precapillary (arterial) or postcapillary (venous) pathophysiological mechanisms. Interestingly, recent PH pathology has shown that pulmonary arterial or pulmonary venous remodelling are rarely independent phenomena, but frequently occur in combined fashion in lungs from patients suffering from different forms of PH, including pulmonary arterial hypertension (PAH). In PAH, it is now becoming clear that aberrant signals present in vessel wall microenvironment, which is largely orchestrated by dysfunctional pulmonary endothelial cells, are key contributors of the pulmonary vascular remodeling process, fostering proliferation, and survival and migration of resident pulmonary vascular cells such as smooth muscle cells, myofibroblasts, and pericytes. In addition, both genetic and environmental factors are also critical in the development of pulmonary vascular inflammation and chronic impairment of the pulmonary endothelium. This article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

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