Oxygen inhalation can selectively dilate pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension before balloon angioplasty

Abstract Background Balloon pulmonary angioplasty (BPA) improves hemodynamics, symptoms and exercise capacity in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA). However, certain patients still have hypoxemia after BPA or PEA despite normalization of hemodynamics. In CTEPH, hypoxemia is related increased dead space ventilation caused by vascular obstruction. Purpose This study was aimed to clarify whether additional BPA can improve hypoxemia of CTEPH patients after normalization of hemodynamics. Methods A total of 335 patients who underwent initial series of BPA in our institute were followed up. Sixty-four patients with mean pulmonary artery pressure (mPAP) <30mmHg and percutaneous oxygen saturation (SpO2) <95% without oxygen inhalation at more than 6 months after the initial series of BPA and of patients who could reevaluate hemodynamics and oxygenation after additional BPA were enrolled. These patients were divided into two groups with or without additional BPA procedures. Change of hemodynamics and SpO2 were retrospectively investigated. Results Thirty-three of 64 patients underwent additional BPA procedures. Patients' age was older in BPA group than those in non-BPA group (71.3±10.4 vs. 66.5±9.4 years old, p=0.02). mPAP and pulmonary vascular resistance (PVR) was significantly higher in BPA group (mPAP: 23.9±3.2 vs. 20.7±3.8 mmHg, p=0.001, PVR: 4.2±1.2 vs. 3.5±1.4 wood unit, p=0.03, respectively). Among the 1.8±1.4 BPA procedures per person, total 6.6±3.8 segmental pulmonary arteries per person were treated. While no obvious improvements were observed in non-BPA group, PVR and SpO2 in BPA group were significantly improved (4.2±1.2 to 3.7±1.3 wood unit, p=0.002, 90.7±3.1% to 94.1±3.6%, p<0.001, respectively). In the multivariate logistic regression analysis, additional BPA procedures were associated with further improvement of SpO2 (hazard ratio, 3.7; 95% confidence interval, 1.2–11.5; P=0.02). Conclusions Additional BPA procedure was associated with improvement of hypoxemia in CTEPH patients after normalization of hemodynamics. Treating as many lesions as possible in BPA might relieve the patients' residual dyspnea.

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Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-12.4.186 ◽

2014 ◽

Vol 12 (4) ◽

pp. 186-192 ◽

Cited By ~ 1

Author(s):

David Poch ◽

Victor Pretorius

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Pulmonary Artery Pressure ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Pulmonary Artery Wedge Pressure ◽

Pulmonary Endarterectomy ◽

Pulmonary Thromboendarterectomy ◽

Thromboembolic Pulmonary Hypertension ◽

Wedge Pressure

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

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The soluble guanylate cyclase stimulator riociguat is the first-line therapy for chronic thromboembolic pulmonary hypertension patients

Kardiologiia ◽

10.18087/cardio.2020.8.n1198 ◽

2020 ◽

Vol 60 (8) ◽

pp. 115-123

Author(s):

Z. S. Valieva ◽

S. E. Gratsianskaya ◽

T. V. Martynyuk

Keyword(s):

Pulmonary Hypertension ◽

Guanylate Cyclase ◽

Soluble Guanylate Cyclase ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

First Line ◽

Pulmonary Thromboendarterectomy ◽

First Line Therapy ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Microcirculation

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary type of pulmonary hypertension with chronic obstruction of large and medium branches of pulmonary arteries along with secondary alterations in pulmonary microcirculation, which cause progressive increases in pulmonary vascular resistance and pulmonary arterial pressure and ensuing severe right heart dysfunction and heart failure. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for CTEPH; however, this procedure is available not for all patients. Although the surgery performed in the conditions of centers with advanced experience generally shows good results, up to 40% of patients are technically inoperable or PTE is associated with a high risk of complications. At present, riociguat, the only officially approved drug from the class of soluble guanylate cyclase stimulators, is considered as a first-line treatment for inoperable and residual forms of STEPH. Introduction of riociguat to clinical practice can be called a real breakthrough in the treatment of patients with STEPH who cannot undergo PTE or those with relapse or persistent STEPH after the surgery.

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Chronic thromboembolic pulmonary hypertension

ESC CardioMed ◽

10.1093/med/9780198784906.003.0668 ◽

2018 ◽

pp. 2800-2804

Author(s):

Irene M. Lang ◽

Walter Klepetko ◽

Hiromi Matsubara

Keyword(s):

Pulmonary Hypertension ◽

Gold Standard ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Vascular Remodelling ◽

Pulmonary Endarterectomy ◽

Medical Treatments ◽

Group 4 ◽

Major Vessel ◽

Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions represent group 4 in the pulmonary hypertension classification. Angiosarcoma, other intravascular tumours, arteritides, congenital pulmonary arteries stenoses, and parasites (hydatidosis) remain rare differential diagnoses within group 4. CTEPH comprises an occlusive vascular remodelling process of major vessel pulmonary thromboembolism in combination with a pulmonary arteriopathy at the pre-capillary level. The gold standard treatment has been pulmonary endarterectomy that is able to restore normal pulmonary haemodynamics at rest. Because about half of the patients are felt to be unsuited for pulmonary endarterectomy, medical treatments and balloon pulmonary angioplasty have gained grounds in the management of patients with CTEPH.

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Successful right heart remodelling and subsequent pregnancy in a patient with chronic thromboembolic pulmonary hypertension undergoing balloon pulmonary angioplasty: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytz063 ◽

2019 ◽

Vol 3 (2) ◽

Cited By ~ 2

Author(s):

Nobutaka Ikeda ◽

Masaru Hatano ◽

Takeshi Nagamatsu ◽

Masato Nakamura

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Right Heart Catheterization ◽

Subsequent Pregnancy ◽

Heart Catheterization ◽

Right Heart ◽

Promising Alternative ◽

Balloon Pulmonary Angioplasty ◽

Thromboembolic Pulmonary Hypertension

AbstractBackgroundTo date, the management of pregnancy in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and the associated risk of maternal mortality have not been established. Although balloon pulmonary angioplasty (BPA) in patients with CTEPH is still an emerging procedure, this approach represents a promising alternative to pulmonary endarterectomy (PEA), especially in patients with inoperable CTEPH.Case summaryWe present a case of a 34-year-old woman with CTEPH who desired to have a child. Right heart catheterization showed a mean pulmonary artery pressure of 54 mmHg. Since the lesions were observed in the distal part of subsegmental pulmonary arteries, there was no indication for PEA. After improvement in her haemodynamic status by BPA, she became pregnant. At 40 weeks of gestation, a normal baby was delivered vaginally. Both mother and baby have made satisfactory progress.DiscussionIn cases in which the haemodynamic status is improved by effective BPA, pregnancy and childbirth may be possible, even in patients with CTEPH.

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Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension

European Respiratory Review ◽

10.1183/16000617.0111-2016 ◽

2017 ◽

Vol 26 (143) ◽

pp. 160111 ◽

Cited By ~ 89

Author(s):

David Jenkins ◽

Michael Madani ◽

Elie Fadel ◽

Andrea Maria D'Armini ◽

Eckhard Mayer

Keyword(s):

Pulmonary Hypertension ◽

Treatment Algorithm ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Pulmonary Endarterectomy ◽

European Respiratory Society ◽

Complex Procedure ◽

Thromboembolic Pulmonary Hypertension ◽

Definition Of ◽

European Society Of Cardiology

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are <5% and survival is >90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual's operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team.Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an “expert centre” for the management of this condition.

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Chronic Thromboembolic Pulmonary Hypertension – What Have We Learned From Large Animal Models

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.574360 ◽

2021 ◽

Vol 8 ◽

Author(s):

Kelly Stam ◽

Sebastian Clauss ◽

Yannick J. H. J. Taverne ◽

Daphne Merkus

Keyword(s):

Pulmonary Hypertension ◽

Animal Models ◽

Current Knowledge ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Large Animal ◽

Large Animal Models ◽

Microvascular Remodeling ◽

Thromboembolic Pulmonary Hypertension ◽

The Right

Chronic thrombo-embolic pulmonary hypertension (CTEPH) develops in a subset of patients after acute pulmonary embolism. In CTEPH, pulmonary vascular resistance, which is initially elevated due to the obstructions in the larger pulmonary arteries, is further increased by pulmonary microvascular remodeling. The increased afterload of the right ventricle (RV) leads to RV dilation and hypertrophy. This RV remodeling predisposes to arrhythmogenesis and RV failure. Yet, mechanisms involved in pulmonary microvascular remodeling, processes underlying the RV structural and functional adaptability in CTEPH as well as determinants of the susceptibility to arrhythmias such as atrial fibrillation in the context of CTEPH remain incompletely understood. Several large animal models with critical clinical features of human CTEPH and subsequent RV remodeling have relatively recently been developed in swine, sheep, and dogs. In this review we will discuss the current knowledge on the processes underlying development and progression of CTEPH, and on how animal models can help enlarge understanding of these processes.

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Chronic Thromboembolic Pulmonary Hypertension Medical Management

Methodist DeBakey Cardiovascular Journal ◽

10.14797/ichn7633 ◽

2021 ◽

Vol 17 (2) ◽

pp. e28-e32

Author(s):

Ryan Logue ◽

Zeenat Safdar

Keyword(s):

Pulmonary Hypertension ◽

Medical Management ◽

Soluble Guanylate Cyclase ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Standard Of Care ◽

Pulmonary Angiography ◽

Thromboembolic Pulmonary Hypertension ◽

Soluble Guanylate Cyclase Stimulator

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.

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