A normal diffusing capacity of the lungs for carbon monoxide is rare in incidental pulmonary arterial hypertension in systemic sclerosis: Data from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma cohort

2018 ◽  
Vol 3 (3) ◽  
pp. 237-241
Author(s):  
Rebecca S Overbury ◽  
Maureen A Murtaugh ◽  
Tracy M Frech ◽  
Virginia D Steen
Keyword(s):  
Carbon Monoxide ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Natriuretic Peptide ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Diffusing Capacity ◽  
Pulmonary Arterial ◽  
Left Atrium Size

Objective: Our purpose was to determine the frequency of normal diffusing capacity for carbon monoxide defined as ⩾70% predicted, in those diagnosed with pulmonary arterial hypertension in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma cohort. We compared those with normal diffusing capacity for carbon monoxide to those with reduced diffusing capacity for carbon monoxide <70% in order to better clarify the role of pulmonary function testing as a screening test for pulmonary arterial hypertension and to better understand this population. Methods: Entry criteria included a right heart catheterization with mean pulmonary artery pressure ⩾25 mm Hg and pulmonary capillary wedge pressure ⩽15 mm Hg. Demographics, echocardiogram variables, B-type natriuretic peptide levels, right heart catheterization findings, and survival were described for both groups. Results: Of (n = 202), 11 (5.4%) had a diffusing capacity for carbon monoxide of ⩾70% versus 191 (94.6%) who had a diffusing capacity for carbon monoxide <70%. There were no identified statistical differences between the groups. Left atrium size was 4.1 cm in the normal diffusing capacity for carbon monoxide patients compared to 3.7 cm in the low diffusing capacity for carbon monoxide group but did not reach statistical significance. There were no statistically significant differences in survival. On repeat testing, seven patients subsequently developed a diffusing capacity for carbon monoxide <70%. Conclusion: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma data suggest that it is very rare for a patient to develop pulmonary arterial hypertension with a preserved diffusing capacity for carbon monoxide. The data support the importance of obtaining diffusing capacity for carbon monoxide and that a patient with a normal diffusing capacity for carbon monoxide while suspected to have systemic sclerosis-pulmonary arterial hypertension should be considered critically. Diffusing capacity for carbon monoxide >70% was present in too few patients to find significant differences in B-type natriuretic peptide and atrium size. Future research should seek to confirm abnormal B-type natriuretic peptide, increased left atrium size, and other evidence of myocardial involvement on diffusing capacity for carbon monoxide.

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 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 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 STILL AN ‘ORPHAN LUNG DISEASE’ IN PAKISTAN

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.

scholarly journals The Right Ventricular Diameter can Predict the Presence of Pulmonary Hypertension

2016 ◽  
Vol 30 (2) ◽  
pp. 48-52 ◽  
Author(s):  
Abrar Kaiser ◽  
Fazilatunnessa Malik ◽  
Tuhin Haque ◽  
Iftekhar Alam ◽  
Abdullah Al Masud ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Non Invasive ◽  
Pulmonary Arterial

Background: Pulmonary arterial hypertension (PAH) is a severe disease characterized by a progressive increase of pulmonary pressure and resistance leading to right heart failure. Pulmonary arterial hypertension is commonly diagnosed at a late stage of the disease and is associated with progressive clinical deterioration and premature death. The assessment of pulmonary artery pressure is important in clinical management and prognostic evaluation of patients with cardiovascular and pulmonary disease. Although PH can be detected invasively by right ventricular (RV) catheterization, accurate non-invasive assessment by echocardiography has many advantages. Reliable non-invasive evaluation of pulmonary pressure at present is still a problem as echocardiographic measurement of pulmonary hypertension relies on the presence of tricuspid regurgitation (TR). Objective: The purpose of this study was to determine whether right ventricular end diastolic diameter can predict the presence of pulmonary hypertension. Methods: Eighty consecutive patients with echo detectable tricuspid regurgitation who underwent right heart catheterization for either diagnostic or therapeutic procedure were recruited. They were divided into two groups on the basis of pulmonary artery systolic pressure (PASP). Group I consists of 40 patients with PASP >35 mm Hg and Group II 40 patients having PASP d• 35 mm Hg. Right ventricular end-diastolic diameter (RVD) was measured in the apical 4 chamber view. PASP was measured from right heart catheterization. Results: The RVD has strong correlation with catheter-derived PASP, at a cutoff value of >3 cm, predicted the presence of PAH with 78% sensitivity and 71% specificity. Conclusion: RVD is a good non-invasive predictor for PAH. RVD can predict the presence of PAH even in absence of TR and correlates well with PASP measured by RV catheterization.Bangladesh Heart Journal 2015; 30(2) : 48-52

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

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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