Decreased pulmonary arterial proportional pulse pressure is associated with increased mortality in group 1 pulmonary hypertension

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 ).

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Increased Pulmonary-Systemic Pulse Pressure Ratio Is Associated With Increased Mortality in Group 1 Pulmonary Hypertension

Heart Lung and Circulation ◽

10.1016/j.hlc.2018.05.199 ◽

2019 ◽

Vol 28 (7) ◽

pp. 1059-1066 ◽

Cited By ~ 1

Author(s):

Benjamin K. Ruth ◽

Kenneth C. Bilchick ◽

Manu M. Mysore ◽

Hunter Mwansa ◽

William C. Harding ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulse Pressure ◽

Pressure Ratio ◽

Group 1

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Proximal pulmonary arterial obstruction decreases the time constant of the pulmonary circulation and increases right ventricular afterload

Journal of Applied Physiology ◽

10.1152/japplphysiol.00033.2013 ◽

2013 ◽

Vol 114 (11) ◽

pp. 1586-1592 ◽

Cited By ~ 30

Author(s):

Alberto Pagnamenta ◽

Rebecca Vanderpool ◽

Serge Brimioulle ◽

Robert Naeije

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Time Constant ◽

Pulse Pressure ◽

Pulmonary Circulation ◽

Characteristic Impedance ◽

Ventricular Afterload ◽

Hydraulic Load ◽

Distal Obstruction ◽

Pulmonary Arterial

The time constant of the pulmonary circulation, or product of pulmonary vascular resistance (PVR) and compliance (Ca), called the RC-time, has been reported to remain constant over a wide range of pressures, etiologies of pulmonary hypertension, and treatments. We wondered if increased wave reflection on proximal pulmonary vascular obstruction, like in operable chronic thromboembolic pulmonary hypertension, might also decrease the RC-time and thereby increase pulse pressure and right ventricular afterload. Pulmonary hypertension of variable severity was induced either by proximal obstruction (pulmonary arterial ensnarement) or distal obstruction (microembolism) eight anesthetized dogs. Pulmonary arterial pressures (Ppa) were measured with high-fidelity micromanometer-tipped catheters, and pulmonary flow with transonic technology. Pulmonary ensnarement increased mean Ppa, PVR, and characteristic impedance, decreased Ca and the RC-time (from 0.46 ± 0.07 to 0.30 ± 0.03 s), and increased the oscillatory component of hydraulic load (Wosc/Wtot) from 25 ± 2 to 29 ± 2%. Pulmonary microembolism increased mean Ppa and PVR, with no significant change in Ca and characteristic impedance, increased RC-time from 0.53 ± 0.09 to 0.74 ± 0.05 s, and decreased Wosc/Wtot from 26 ± 2 to 13 ± 2%. Pulse pressure increased more after pulmonary ensnarement than after microembolism. Concomitant measurements with fluid-filled catheters showed the same functional differences between the two types of pulmonary hypertension, with, however, an underestimation of Wosc. We conclude that pulmonary hypertension caused by proximal vs. distal obstruction is associated with a decreased RC-time and increased pulsatile component of right ventricular hydraulic load.

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Classification of Pulmonary Hypertension

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-13.1.17 ◽

2014 ◽

Vol 13 (1) ◽

pp. 17-20

Author(s):

Rogerio Souza ◽

Gerald Simonneau

Keyword(s):

Pulmonary Hypertension ◽

Task Force ◽

Management Strategies ◽

Pathological Findings ◽

Clinical Classification ◽

Current Classification ◽

Pulmonary Arterial ◽

Current Classification System ◽

Group 1

Classification of pulmonary hypertension groups patients with similar pathological findings, hemodynamic profiles, and management strategies. Minor modifications have been made to the current classification system, particularly within Group 1 pulmonary arterial hypertension. This article summarizes the published conclusions of the Fifth World Symposium of Pulmonary Hypertension task force that addressed the updated clinical classification of pulmonary hypertension.

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Advanced Diagnosis and Therapy for Pulmonary Arterial Hypertension

Nano LIFE ◽

10.1142/s1793984420400036 ◽

2020 ◽

Vol 10 (01n02) ◽

pp. 2040003 ◽

Cited By ~ 1

Author(s):

Jing Qi ◽

Yan Xing ◽

Xiaoting Zhao ◽

Daling Zhu ◽

Xiaodong Zheng

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Progressive Disease ◽

Task Force ◽

Pulmonary Arteries ◽

Premature Death ◽

Pulmonary Arterial ◽

Clinical Conditions ◽

Group 1

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and progressive disease, which often leads to premature death. Pulmonary arterial hypertension (PAH, group 1 PH) comprises of diverse diseases that result in similar pathological changes, including the medium-sized pulmonary arteries and pulmonary arterioles characterized by vascular obliteration. The evaluation and diagnosis of PH and PAH were re-defined based on proceedings of the 6th World Symposia on Pulmonary Hypertension (WSPH). Accurate early diagnosis and subsequent therapy of PAH are necessary, as management of this disease is still challenging, and life expectancy is remaining suboptimal. This review task force reflects the multidisciplinary nature of PAH, including the definition, epidemiology, genetics, especially the advanced diagnosis and the therapy development in recent years.

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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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Differential Effects of Terlipressin on Pulmonary and Systemic Hemodynamics in Patients With Cirrhosis and Pulmonary Hypertension

Angiology ◽

10.1177/0003319711411704 ◽

2011 ◽

Vol 63 (3) ◽

pp. 199-205 ◽

Cited By ~ 5

Author(s):

Georgios N. Kalambokis ◽

Konstantinos Pappas ◽

Epameinondas V. Tsianos

Keyword(s):

Pulmonary Hypertension ◽

Cardiac Output ◽

Mean Arterial Pressure ◽

Pulmonary Vascular Resistance ◽

Vascular Resistance ◽

Systemic Hemodynamic ◽

Hemodynamic Improvement ◽

Pulmonary Arterial ◽

Group 2 ◽

Group 1

Terlipressin has been associated with pulmonary arterial vasodilation in patients with pulmonary hypertension (PH). We investigated the effects of terlipressin on pulmonary vascular resistance (PVR) in patients with cirrhosis without and with PH. Pulmonary vascular resistance and cardiac output (CO) by Doppler ultrasound, mean arterial pressure (MAP), and systemic vascular resistance (SVR) were evaluated in patients with cirrhosis with PVR –120 dyne s cm−5 (group 1, n = 20) and PVR >120 dyne s cm−5 (group 2, n = 10) before and 30 minutes after terlipressin infusion (2 mg). After terlipressin, PVR increased significantly in group 1 (96.1 ± 20.2 vs 85.1 ± 18 dyne s cm−5; P = .004) but decreased significantly in group 2 (170.4 ± 37.8 vs 157.8 ± 28.1 dyne s cm−5; P= .04). Pulmonary vascular resistance changes in group 2 correlated significantly with baseline PVR ( r = −0.632; P = .04). Terlipressin induced a significant increase in MAP and SVR and a significant decrease in CO in both groups. Terlipressin significantly reduces pulmonary pressures in patients with cirrhosis having PH together with systemic hemodynamic improvement.

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Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry

Pulmonary Circulation ◽

10.1177/2045894020964342 ◽

2020 ◽

Vol 10 (4) ◽

pp. 204589402096434

Author(s):

Shoaib Fakhri ◽

Kelly Hannon ◽

Kelly Moulden ◽

Ryan Peterson ◽

Peter Hountras ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

High Altitude ◽

Clinical Characteristics ◽

Supplemental Oxygen ◽

Walk Distance ◽

Pulmonary Arterial ◽

Low Altitude ◽

Group 1

Background WHO Group 1 pulmonary arterial hypertension is a progressive and potentially fatal disease. Individuals living at higher altitude are exposed to lower barometric pressure and hypobaric hypoxemia. This may result in pulmonary vasoconstriction and contribute to disease progression. We sought to examine the relationship between living at moderately high altitude and pulmonary arterial hypertension characteristics. Methods Forty-two US centers participating in the Pulmonary Hypertension Association Registry enrolled patients who met the definition of WHO Group 1 pulmonary arterial hypertension. We utilized baseline data and patient questionnaire responses. Patients were divided into two groups: moderately high altitude residence (home ≥4000 ft) and low altitude residence (home <4000 ft) based on zip-code. Clinical characteristics, hemodynamic data, patient demographics, and patient reported quality of life metrics were compared. Results Controlling for potential confounders (age, sex at birth, body mass index, supplemental oxygen use, race, 100-day cigarette use, alcohol use, and pulmonary arterial hypertension medication use), subjects residing at moderately high altitude had a 6-min walk distance 32 m greater than those at low altitude, despite having a pulmonary vascular resistance that was 2.2 Wood units higher. Additionally, those residing at moderately high altitude had 3.7 times greater odds of using supplemental oxygen. Conclusion Patients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Despite these findings, moderately high altitude Pulmonary Hypertension Association Registry patients have better functional tolerance as measured by 6-min walk distance. It is possible that a “high-altitude phenotype” of pulmonary arterial hypertension may exist. These findings warrant further study.

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P4677Balloon pulmonary angioplasty improves pulmonary arterial compliance in patients with inoperable chronic thromboembolic pulmonary hypertension

European Heart Journal ◽

10.1093/eurheartj/ehz745.1059 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

S Umemoto ◽

K Abe ◽

K Horimoto ◽

K Hosokawa ◽

H Tsutsui

Keyword(s):

Pulmonary Hypertension ◽

Stroke Volume ◽

Pulse Pressure ◽

Arterial Compliance ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Heart Catheterization ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Arterial ◽

The Impact

Abstract Background Right ventricular (RV) pressure overload is directly related to the increase in mortality in pulmonary hypertension. Pulmonary arterial compliance (CPA; stroke volume/pulmonary pulse pressure) was reported to be an independent determinant of RV systolic afterload in patients with pulmonary arterial hypertension (PAH). Recently, balloon pulmonary angioplasty (BPA) has been reported to reduce mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (RPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the effects of BPA on CPA remain unclear. Purpose The aim of this study was to investigate the impact of BPA on CPA in patients with inoperable CTEPH. Methods We retrospectively analyzed 78 patients (388 BPA sessions) with inoperable CTEPH who underwent BPA in our hospital from September 2012 to June 2018. Total number of BPA sessions was 5.0±1.8 (range 1–10). The pressure values were obtained from right heart catheterization at baseline (n=78), just after the final BPA (n=78) and follow-up (n=19) periods. The intervals from baseline to the final BPA and the final BPA to follow-up were 593±498 days and 397±276 days, respectively. Results Mean age was 60.5±12.6 years old, and 64 (82%) were female. All patients were symptomatic (WHO functional class II/III/IV 16/55/7). Patients who had pulmonary vasodilators decreased from 70 (90%) at baseline to 23 (28%) at the final BPA and 2 (15%) at follow-up. BPA reduced mPAP and RPA significantly from baseline to the final BPA and follow-up periods. BPA also improved CPA with significant reduction of pulse pressure despite no significant changes in stroke volume between baseline and follow-up (Table). CPA between the final BPA and follow-up was equivalent (p=0.95). Conclusions BPA improved CPA just after the final BPA in inoperable CTEPH patients. In addition, CPA was preserved during the follow-up after the final BPA sessions. These data suggest that BPA consistently unloads RV systolic afterload in those patients.

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

Diagnostics ◽

10.3390/diagnostics11020311 ◽

2021 ◽

Vol 11 (2) ◽

pp. 311

Author(s):

Horst Olschewski

Keyword(s):

Pulmonary Hypertension ◽

Lung Disease ◽

Chronic Lung Disease ◽

Diagnostic Procedures ◽

Disease Group ◽

Group 4 ◽

Pulmonary Arterial ◽

Group 3 ◽

Group 2 ◽

Group 1

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.

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