scholarly journals The Challenge to Decide between Pulmonary Hypertension Due to Chronic Lung Disease and PAH with Chronic Lung Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 311
Author(s):  
Horst Olschewski

Chronic lung diseases are strongly associated with pulmonary hypertension (PH), and even mildly elevated pulmonary arterial pressures are associated with increased mortality. Chronic obstructive pulmonary disease (COPD) is the most common chronic lung disease, but few of these patients develop severe PH. Not all these pulmonary pressure elevations are due to COPD, although patients with severe PH due to COPD may represent the largest subgroup within patients with COPD and severe PH. There are also patients with left heart disease (group 2), chronic thromboembolic disease (group 4, CTEPH) and pulmonary arterial hypertension (group 1, PAH) who suffer from COPD or another chronic lung disease as co-morbidity. Because therapeutic consequences very much depend on the cause of pulmonary hypertension, it is important to complete the diagnostic procedures and to decide on the main cause of PH before any decision on PAH drugs is made. The World Symposia on Pulmonary Hypertension (WSPH) have provided guidance for these important decisions. Group 2 PH or complex developmental diseases with elevated postcapillary pressures are relatively easy to identify by means of elevated pulmonary arterial wedge pressures. Group 4 PH can be identified or excluded by perfusion lung scans in combination with chest CT. Group 1 PAH and Group 3 PH, although having quite different disease profiles, may be difficult to discern sometimes. The sixth WSPH suggests that severe pulmonary hypertension in combination with mild impairment in the pulmonary function test (FEV1 > 60 and FVC > 60%), mild parenchymal abnormalities in the high-resolution CT of the chest, and circulatory limitation in the cardiopulmonary exercise test speak in favor of Group 1 PAH. These patients are candidates for PAH therapy. If the patient suffers from group 3 PH, the only possible indication for PAH therapy is severe pulmonary hypertension (mPAP ≥ 35 mmHg or mPAP between 25 and 35 mmHg together with very low cardiac index (CI) < 2.0 L/min/m2), which can only be derived invasively. Right heart catheter investigation has been established nearly 100 years ago, but there are many important details to consider when reading pulmonary pressures in spontaneously breathing patients with severe lung disease. It is important that such diagnostic procedures and the therapeutic decisions are made in expert centers for both pulmonary hypertension and chronic lung disease.

2017 ◽  
Vol 26 (145) ◽  
pp. 170056 ◽  
Author(s):  
David Launay ◽  
Vincent Sobanski ◽  
Eric Hachulla ◽  
Marc Humbert

Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease). Pulmonary veno-occlusive disease is not uncommon in SSc and may also cause PH in some patients (group 1′). There is a high prevalence of each of these conditions in SSc and, as such, it may be difficult to determine the dominant cause of PH in a particular patient. However, careful phenotyping of PH in SSc is important as the therapy required for each of these underlying conditions is very different. In this review, we will decipher the different phenotypes of SSc-PH.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Lucia Soriente ◽  
Valeria Visco ◽  
Chiara Aliberti ◽  
Michele Ciccarelli ◽  
Gennaro Galasso ◽  
...  

Abstract Aims The definition of pulmonary hypertension (PH) requires the documentation of mean pulmonary arterial pressure (PAPm) ≥20 mmHg at rest, assessed by right cardiac catheterization. This condition can characterize multiple clinical conditions with different pathophysiological and haemodynamic aspects. Specifically, in pulmonary arterial hypertension (IAP) (Group 1), the increase in PAP is due to an intrinsic pathology of the pulmonary microcirculation; Group 2 includes the forms of IP associated with a pathology of the left heart; Group 3 includes all pathologies of the pulmonary parenchyma and/or hypoxic conditions that lead to a secondary impairment of the small circulation; Group 4 identifies patients with chronic thromboembolic pulmonary heart (CPCTE); finally, Group 5 includes rare clinical conditions in which IP is linked to direct involvement or ab extrinsic compression of the pulmonary vessels. Methods and results To examine the number of deaths and the differences between the various subgroups, we analyzed the follow-up of approximately 76 patients (64.30 ± 13.20 years, 37% male) enrolled in the Pulmonary Hypertension Clinic of the San Giovanni AOU Dio and Ruggi d’Aragona of Salerno from 2014 to 2020 excluding patients with IP under definition and those ‘screened’ who did not show pulmonary hypertension at rest. At each visit, the patients were subjected to anamnestic data collection, physical examination, measurement of blood pressure, heart rate, arterial saturation, transthoracic cardiac echo color Doppler at rest, attribution of the functional class NYHA, evaluation of functional capacity by performing the test of the 6-min walk test (6MWT) and possible programming of right cardiac catheterization. From the data analysis it was found that 26 patients (34.21%, 55.81 ± 13.90 years, 27% males) were affected by IP group 1; 15 patients (19.48%, 74.12 ± 6.26 years, 20% male) were affected by IP group 2; 14 patients (18.18%, 63.34 ± 11.52 years, 71% male) were affected by IP group 3; 12 patients (15.58%, 67.22 ± 11.53 years, 33% male) were affected by IP group 4; 2 patients (2.60%, 71.57 ± 12.48 years, 0% male) were affected by IP group 5; 7 patients (9.09%, 70.07 ± 8.27 years, 57% male) were affected by group 2–3 mixed IP. Analysing the number of deaths, of the 76 patients, 17 deaths were recorded in total (22.37%). All patients in group 1 had been treated with specific therapy and survival was 88% at 3 years. Of the three deaths (12%) in this group, one patient had idiopathic PAH non-responder to pulmonary vasoreactivity test, one patient belonged to the IAP subgroup associated with congenital heart shunt but with concomitant lung disease, and the third patient belonged to the IAP subgroup associated with connective tissue disease (specifically Takayasu’s arteritis). From the analysis of the idiopathic IAP subgroup it emerged that the patients were all women, with an average age of 50.81 ± 3.98 years, and that the deceased patient was distinguished at the first visit from the other patients for: a history of arterial hypertension, dysthyroidism, and obesity; worst NYHA class (III vs. II), elevated heart rate (102 vs. 70.00 ± 7.07 b.p.m.) and blood pressure (SBP: 150.00 vs. 127.50 ± 10.61; DBP 90.00 vs. 75.00 ± 7.07 mmHg) at rest at the clinic visit. Transthoracic echocardiography revealed elevated PAPs values (100.00 vs. 42.50 ± 20.51 mmHg), low TAPSE values (20.00 vs. 26.50 ± 3.54 mm), reduced pulmonary acceleration time (ACT 60 ms), enlargement of the right atrium (area 22 cm2), worst exercise tolerance parameters (6MWT 300 m and 86% final SO2 vs. 427.50 ± 74.25 m and 96.50 ± 0.71% final SO2). Conclusions The percentage of deaths in the different groups appears very heterogeneous, especially if we consider the six deaths (42.86%) in group 3 and three deaths (42.86%) in patients with mixed IP groups 2 and 3. In these two groups, age and advanced NYHA class were the most representative prognostic factors. On the other hand, analysing patients belonging to the idiopathic IAP subgroup, a worse prognosis is entrusted to the negativity of the vasoreactivity test, to the presence of cardiovascular comorbidities (arterial hypertension and dysthyroidism in our case), to worse echocardiographic values (PAPs, TAPSE, ACT, atrial area right) and reduced functional capacity at the 6MWT. However, early treatment and innovative drugs together with a careful strategy have been allowed.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Juan Carlos J Grignola ◽  
Leticia L Fernandez-Lopez ◽  
Enric E Domingo-Ribas ◽  
Rio R Aguilar ◽  
Cristian Humberto C Arredondo ◽  
...  

Purpose: The aim of the study is to assess pulmonary vasculopathy (wall fibrosis, pulmonary arterial pulsatility and elastic modulus ) in patients with persistent pulmonary hypertension (pulmonary systolic pressure by ECHO > 50 mmhg ) at least 1 year after mitral valve replacement with normal function of the valve. The evaluation was carried out by intravascular ultrasound (IVUS) in medium sized pulmonary arteries. We compared three groups: Group 1 ( persistent pulmonary hypertension after mitral valve replacement), Group 2 (pulmonary hypertension belonging to the group 1 of the Dana Point classification) and Group 3 (healthy controls). Methods: We studied 43 patients, 15 in Group 1 , 18 Group 2 and 10 in Group 3. Group 1: 13 females, the mean age of this group was 74+-7 years; Group 2: 14 females, 53+-14 years and Group 3: 6 females, 51+-5 years. All patients were submitted to left and right heart catheterization, and IVUS in medium sized elastic PA ( 2-3 mm diameter ) of the inferior lobes. Studied variables were: mean pulmonary artery pressure (PAP, mm Hg), pulmonary wedge pressure, aortic pressure, cardiac output (CO,l/min), pulmonary vascular resistance (PVR, Wood Units), IVUS pulsatility and elastic modulus (EM,mm Hg). Local pulsatility was estimated by IVUS: (systolic- diastolic lumen area/ diastolic lumen area) X 100. PA stiffness was assessed by the elastic modulus (EM= pulse pressure/ IVUSp). Results: In Group 3 all variables were statistically different from the other 2 groups (p<0.01). Variables are shown in table. Conclusions: Group 1, even with a lower mean PAP than Group 2 (p<0.05) showed a similar anatomical ( wall fibrosis ) and similar functional wall remodeling ( EM ).


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Fontes Oliveira ◽  
M Trepa ◽  
R Costa ◽  
A Dias Frias ◽  
I Silveira ◽  
...  

Abstract Introduction Noninvasive echocardiography evaluation of the right ventricle (RV) has been shown to have prognostic value in patients with pulmonary hypertension (PH). Different etiology groups might have different echocardiographic phenotypes. In this study, we aimed to study echocardiographic characterization of the different PH groups and its ability to predict pulmonary vascular disease severity. Methods We collected echocardiographic and right heart catheterization (RHC) data from 97 (75% female, age 65 ± 15 years) consecutive patients referred to an expert tertiary care referral PH centre from 12/2016 to 11/2018. Echocardiographic analysis was performed using Echo-Pac software from GE Healthcare®. Group 3 and 5 were not included in the group comparison analysis due to few patients included. Results Group 2 PH was the most frequent etiology of PH (35), followed by group 1 (26), group 4 (18), group 5 (3) and group 3 (2). The echocardiographic evaluation of this population as a whole showed borderline parameters of RV dysfunction (tricuspid annular plane systolic excursion (TAPSE) 18 ± 4 mm, fractional area change (FAC) 33 ± 10% and S’ tricuspid wave 10 ± 3 cm/sec). Mean RV global strain was -15 ± 5 and RV free wall strain was -17 ± 7. PH group 1 had a significantly lower FAC (26 ± 4%, p = 0.0025), higher eccentricity index (IE) (1.5 ± 0.1, p = 0.01), and more frequently RV outflow tract (RVOT) notching than other groups (62%, p = 0.012). Group 4 presented an intermedium echocardiographic phenotype between group 1 and 2, and showed more abnormal strain values than the other groups. Group 2 had fewer patients in sinus rhythm (atrial fibrillation in 34% of patients, p = 0.02), presented a thicker interventricular septum (11.3 ± 1.8, p = 0.014), a higher FAC (35 ± 3%, p = 0.0025), higher E mitral wave velocity (72 ± 6 cm/s, p &lt; 0.001) and E/E’ ratio (12.7 ± 10.2, p = 0.006), and larger left (45 ± 3 cm3/m3, p &lt; 0.01) and right atria (25 ± 2 cm2, p = 0.03). PH groups 1 and 4 had higher Pulmonary Vascular Resistance (PVR) and Pulmonary Mean Arterial Pressure (PMAP) values than group 2, which significantly correlated with echocardiographic RV function parameters as TAPSE, FAC, RV global strain and IE. In PH group 2, eccentricity index was the only predictor of PVR (β=4.1, p = 0.018). In this population, a left atria volume &lt; 32.7 cm3/m2 (OR 4.25, CI 1.71 - 10.55) and a E/e’ ratio &lt; 12 (OR 4.72, CI 2.05 - 10.87) predicted PECP &lt; 15 mmHg. RV global strain &gt; -17.1 predicted PVR &gt; 3 wood (OR 3.46, CI 1.50 - 8.02) and PMAP &gt; 20 mmHg (OR 4.92, CI 1.67 - 14.51). TAPSE &lt; 18 mm predicted PVR &gt; 3 wood (OR 7.41, CI 2.99 - 18.36, AUC 0.72). Conclusion Different PH groups present mild echocardiographic differences between them. PH group 1 presented with more echocardiographic signs of RV disfunction, and PH group 2 had higher FAC, E/E’ and larger right and left atria. RV function parameters predicted PVR in PH groups 1 and 4, and EI was the only predictor of PVR in PH group 2. Abstract P1505 Figure.


2021 ◽  
Vol 10 (14) ◽  
pp. 3008
Author(s):  
Silvia Papa ◽  
Cristiano Miotti ◽  
Giovanna Manzi ◽  
Gianmarco Scoccia ◽  
Federico Luongo ◽  
...  

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and under-recognized complication of acute pulmonary embolism (PE). Forty consecutive patients with acute PE (Group 1), predominantly female (22, 55%) with a mean age of 69 ± 15 years, were matched for demographic data with 40 healthy subjects (Group 2), 40 systemic hypertension patients (Group 3) and 45 prevalent idiopathic pulmonary arterial hypertension (IPAH) patients (Group 4). The baseline evaluation included physical examination, NYHA/WHO functional class, right heart catheterization (RHC) limited to IPAH patients, echocardiographic assessment and systemic arterial stiffness measurement by cardio-ankle vascular index (CAVI). Patients with PE underwent an echocardiographic evaluation within 1 month from hospital discharge (median 27 days; IQR 21–30) to assess the echo-derived probability of PH. The CAVI values were significantly higher in the PE and IPAH groups compared with the others (Group 1 vs. Group 2, p < 0.001; Group 1 vs. Group 3, p < 0.001; Group 1 vs. Group 4, p = ns; Group 4 vs. Group 2, p < 0.001; Group 4 vs. Group 3, p < 0.001; Group 2 vs. Group 3, p = ns). The predicted probability of echocardiography-derived high-risk criteria of PH increases for any unit increase of CAVI (OR 9.0; C.I.3.9–20.5; p = 0.0001). The PE patients with CAVI ≥ 9.0 at the time of hospital discharge presented an increased probability of PH. This study highlights a possible positive predictive role of CAVI as an early marker for the development of CTEPH.


2018 ◽  
Vol 8 (2) ◽  
pp. 204589401877505 ◽  
Author(s):  
Nader Chebib ◽  
Jean-François Mornex ◽  
Julie Traclet ◽  
François Philit ◽  
Chahera Khouatra ◽  
...  

Group 3 pulmonary hypertension (PH) is a common complication of advanced chronic lung disease. Our hypothesis was that group 3 PH is associated with a more severe baseline presentation and a more severe prognosis compared to group 1 pulmonary arterial hypertension (PAH), chronic thromboembolic PH (group 4), and group 5 PH. We retrospectively analyzed consecutive incident PH patients in a single center between January 2006 and November 2014. Data were acquired from a prospective database. Clinical, functional, and hemodynamic characteristics, as well as survival, were compared between the four groups of precapillary PH. A total of 363 patients were analyzed; 164 patients (45.2%) belonged to group 1 PAH, 109 (30%) to group 3 PH, 65 (17.9%) to group 4 PH, and 25 (6.9%) to group 5 PH. Group 3 patients were predominantly male and were more frequently in New York Heart Association (NYHA) class III/IV. Patients with group 3 and 4 PH were older, had significantly lower 6-min walking distance (6MWD), higher mean pulmonary arterial pressure, higher pulmonary vascular resistance (PVR), and lower cardiac index (CI) than PAH patients. Group 3 and 5 patients had significantly lower total lung capacity (TLC), forced vital capacity (FVC), and FEV1; group 3 patients had the lowest carbon monoxide transfer coefficient values. PH therapy was used in 90.9% of group 3 patients. Univariate analysis of prognostic factors in the overall population showed that age, male gender, NYHA class, groups 3 and 4 PH (vs. PAH), 6MWD, FVC, TLC, carbon monoxide transfer coefficient (KCO), PVR, CI, and venous oxygen saturation were significantly associated with greater mortality. Multivariate analysis showed that age, PH group 4, 6MWD, and KCO but no longer PH group 3 were significantly associated with mortality. Patients with group 3 PH are older, have more severe baseline presentation and lower survival rates than PAH patients in univariate analysis, that seemed to be related to older age.


Author(s):  
Charles D. Burger

Pulmonary hypertension (PH) was defined hemodynamically at the Fifth World Symposium on Pulmonary Hypertension (WSPH) as a mean pulmonary artery (PA) pressure (mPAP) of 25 mm Hg or more. Five diagnostic groups represent the various causes of PH. Group 1 pulmonary arterial hypertension (PAH) requires both an elevated mPAP and a pulmonary vascular resistance (PVR) of at least 3 Wood units; therefore, the left-sided heart filling pressures are not elevated (pulmonary arterial wedge pressure [PAWP] ≤15 mm Hg). Conversely, in group 2 PH, left-sided heart disease causes an elevation in PAWP. Group 3 PH is due to chronic hypoxemia typically from lung disease, group 4 PH is chronic thromboembolic PH, and group 5 PH is a miscellaneous category.


2020 ◽  
Vol 73 (9) ◽  
pp. 1853-1860
Author(s):  
Sylwia Łukasik ◽  
Dariusz Łukasik ◽  
Michał Tomaszewski ◽  
Weronika Topyła ◽  
Agnieszka Wojtowska ◽  
...  

Introduction: Chronic lung disease (WHO group 3) is the second leading cause of pulmonary hypertension (PH). In turn, the development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. The aim: To analyse the difficulties in the diagnosis of pulmonary hypertension due to chronic lung disease. Review and Discussion: According to recent literature, PH in the course of lung diseases develops as a result of both “parenchymal” and vascular pathology in patients with a genetic predisposition. Prolonged infection (especially viral) may be an additional promoting factor. Elevation of pulmonary arterial pressure (PAP) is usually moderate and correlates with severity of lung disease. In a small minority, PAP may reach that seen in WHO group 1 pulmonary arterial hypertension (PAH). Conclusions: Echocardiography and right heart catheterization are the principal tools for the diagnosis of PH in chronic lung diseases. Unfortunately, current medications for treating PAH have not shown benefit in controlled trials of group 3 PH, hence their routine use is not recommended. Patients with severe group 3 PH should be considered for referral to expert centres or entry into clinical trials.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Jasmine Tatah ◽  
Marc R Pritzker ◽  
Thenappan Thenappan ◽  
Kurt W Prins

Background: Iron deficiency promotes pulmonary vascular remodeling in pre-clinical models, and is associated with worse outcomes in pulmonary arterial hypertension. However, the consequences of iron deficiency in patients with pulmonary hypertension due to chronic lung disease (Group 3 PH) are unexplored. Methods: We studied 122 consecutive Group 3 PH patients from the University of Minnesota Pulmonary Hypertension Repository. Serum soluble transferrin receptor (sTR) levels quantified iron deficiency. We evaluated the relationship between iron deficiency and pulmonary vascular disease, right ventricular (RV) function, exercise capacity, and survival. Results: The iron deficient group (<4.8mg/L sTR) had significantly higher mean pulmonary arterial pressure (40±9 mmHg, n=59 vs. 44±13 mmHg, n=61; p =.02) and lower pulmonary arterial compliance (2.2±1.2 mL/mmHg, n=52 vs. 1.7±0.8 mL/mmHg, n=55; p =.01), but there was no difference in pulmonary vascular resistance. Moreover, there were trends for higher right atrial pressure (7±4 mmHg, n=58 vs. 9±6 mmHg, n=61; p =0.08) in iron deficient patients. However, iron deficiency did not significantly alter RV function by echocardiography, 6-minute walk distance, or survival. Conclusions: Iron deficiency in Group 3 PH is associated with worse pulmonary vascular disease. This suggests iron deficiency could contribute to pulmonary vascular disease in Group 3 PH, and future studies are needed to determine if iron replacement could be a therapy for this deadly type of PH.


VASA ◽  
2020 ◽  
Vol 49 (4) ◽  
pp. 281-284
Author(s):  
Atıf Yolgosteren ◽  
Gencehan Kumtepe ◽  
Melda Payaslioglu ◽  
Cuneyt Ozakin

Summary. Background: Prosthetic vascular graft infection (PVGI) is a complication with high mortality. Cyanoacrylate (CA) is an adhesive which has been used in a number of surgical procedures. In this in-vivo study, we aimed to evaluate the relationship between PVGI and CA. Materials and methods: Thirty-two rats were equally divided into four groups. Pouch was formed on back of rats until deep fascia. In group 1, vascular graft with polyethyleneterephthalate (PET) was placed into pouch. In group 2, MRSA strain with a density of 1 ml 0.5 MacFarland was injected into pouch. In group 3, 1 cm 2 vascular graft with PET piece was placed into pouch and MRSA strain with a density of 1 ml 0.5 MacFarland was injected. In group 4, 1 cm 2 vascular graft with PET piece impregnated with N-butyl cyanoacrylate-based adhesive was placed and MRSA strain with a density of 1 ml 0.5 MacFarland was injected. All rats were scarified in 96th hour, culture samples were taken where intervention was performed and were evaluated microbiologically. Bacteria reproducing in each group were numerically evaluated based on colony-forming unit (CFU/ml) and compared by taking their average. Results: MRSA reproduction of 0 CFU/ml in group 1, of 1410 CFU/ml in group 2, of 180 200 CFU/ml in group 3 and of 625 300 CFU/ml in group 4 was present. A statistically significant difference was present between group 1 and group 4 (p < 0.01), between group 2 and group 4 (p < 0.01), between group 3 and group 4 (p < 0.05). In terms of reproduction, no statistically significant difference was found in group 1, group 2, group 3 in themselves. Conclusions: We observed that the rate of infection increased in the cyanoacyrylate group where cyanoacrylate was used. We think that surgeon should be more careful in using CA in vascular surgery.


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