The target audience for this post are healthcare professionals of all levels. Many clinicians, including cardiologists and pulmonologists, find it often difficult to diagnose CTEPHChronic Thromboembolic Pulmonary Hypertension CTEPH is short for chronic thromboembolic pulmonary hypertension, a rare form of high blood pressure in the lungs caused by blood clots or narrowed blood vessels. High blood pressure in the lungs is often also called pulmonary hypertension. and seem unaware of BPABalloon Pulmonary Angioplasty Balloon pulmonary angioplasty, or BPA for short, is a technique for widening narrowed or blocked blood vessels in the lungs of CTEPH patients. Small balloons attached to a catheter restore blood flow to areas of the lungs that do not receive blood due to the disease.  When successful, BPA eliminates or reduces shortness of breath, increases exercise tolerance and quality of life, and extends life expectancy.’s potential. To counteract this situation, I have put together these research papers and presentations.

I discovered most of the material presented here while researching my illness. There’s a lot more out there. In recent years, a wealth of new research on these topics has appeared. Please see the references and citations and search the relevant databases for more information.

Note: not all materials on this page are “Open Access.” Some are behind a pay wall, which should be fine for health professionals who have access to libraries through their institutions.

BPA & CTEPH Resources

Also note: Knowledge about health and medicine is constantly evolving. This information may become out of date.

I am not a doctor

It’s important to note that I am not a healthcare professional. A medical expert may have selected different resources. Nevertheless, I hope that my post serves its intended purpose.

If you have any suggestions for additional resources, please don’t hesitate to contact me.

Background

I had the privilege of being treated by Dr. Hiromi Matsubara, a leading figure in the development of modern balloon pulmonary angioplastyBalloon Pulmonary Angioplasty Balloon pulmonary angioplasty, or BPA for short, is a technique for widening narrowed or blocked blood vessels in the lungs of CTEPH patients. Small balloons attached to a catheter restore blood flow to areas of the lungs that do not receive blood due to the disease.  When successful, BPA eliminates or reduces shortness of breath, increases exercise tolerance and quality of life, and extends life expectancy. (BPA) and a frequent author of scientific publications.

A photo of Dr. Hiromi Matsubara and I immediately after my 2nd BPA treatment. I am still on the operating table.
Dr. Hiromi Matsubara, still in scrubs, and I, right after my 2nd BPA procedure, asking: Why is BPA so simple? – Image: OMC published with permission.

I had a total of six BPA procedures at the Okayama Medical Center (OMC) in Japan. Four in October and November 2022, and two more during my follow-up visit in June 2023. All procedures went without clinically significant complications.

I had so much fun (yeah!) at OMC and became so impressed with BPA’s elegance and effectiveness that I decided to start this blog.

Please do not hesitate to contact me if you want additional information or have questions about my experience with BPA or CTEPH.

The Okayama Method: a modern and safe approach

Higher risks and more severe complications, including fatalities, are frequently reported in materials until around 2017. The safety and efficacy of BPA have improved substantially in recent years, primarily due to the efforts of Japanese doctors. Modern BPA (particularly the Okayama Method as described by Ogawa & Matsubara in 2018 and again in 2022 in chapter 5 of the book referenced below) has emerged as a safe and effective treatment with a fair chance of curing inoperable CTEPH.

Balloon pulmonary angioplasty in patients with CTEPH

the title page of the book "Balloon pulmonary angioplasty in patients with CTEPH."
Balloon pulmonary angioplasty in patients with CTEPH. Springer 2022.

This recently published book (Springer 2022):

  • Describes a novel, effective, and relatively safe treatment that has yet to be widely acknowledged and still needs standardization.
  • Is of interest to an interdisciplinary audience.
  • It uniquely covers balloon pulmonary angioplasty (BPA) in CTEPH patients and briefly explains the disease and its diagnosis. 

Balloon pulmonary angioplasty (BPA) is a newer, recently refined treatment option for patients with inoperable CTEPH. Extensive data clearly shows the procedure’s efficacy and relative safety. Unfortunately, many clinicians are unaware of CTEPH and of BPA’s potential.

This book is an excellent reference tool for individuals interested in starting or expanding a BPA program, as well as clinicians who work with this complex patient population. It delves into the clinical indications and technical aspects of BPA.

This book is a must-read for every cardiologist and pulmonologist! CTEPH is frequently underdiagnosed or misdiagnosed in daily practice because patients often present with subtle or nonspecific symptoms and because of a need for more awareness among clinicians. Early diagnosis of CTEPH is crucial as it is the only potentially curable form of PHPulmonary Hypertension (PH) High pressure in the blood vessels leading from the heart to the lungs is called pulmonary hypertension. The arteries in the lungs and the right side of the heart are affected by pulmonary hypertension. If untreated, the right side of the heart will weaken over time, eventually leading to heart failure and death. The WHO has defined five distinct groups of pulmonary hypertension, each with a different cause. CTEPH is a form of PH (WHO group 4). - References: mayoclinic.org and cdc.gov and because of its poor prognosis if left untreated (Chapter 2 of this book and Bagalas et al., 2014).

This book is also available on Amazon as a hardcover or Kindle edition.

Saia, F., Galiè, N., Matsubara, H. (Eds.). (2022). Balloon pulmonary angioplasty in patients with CTEPH. Springer. https://doi.org/10.1007/978-3-030-95997-5

Haemodynamic effects of BPA

Balloon pulmonary angioplasty in distal-type chronic thromboembolic pulmonary hypertension (CTEPH). Representative angiography. images: before BPA; immediately after; long-term after.
Balloon pulmonary angioplasty in distal-type chronic thromboembolic pulmonary hypertensionChronic Thromboembolic Pulmonary Hypertension CTEPH is short for chronic thromboembolic pulmonary hypertension, a rare form of high blood pressure in the lungs caused by blood clots or narrowed blood vessels. High blood pressure in the lungs is often also called pulmonary hypertension. (CTEPH). Representative angiography a) before, b) immediately after, and c) at follow-up for a characteristic lesion with complete obstruction in a patient with inoperable CTEPH. At follow-up, there was no restenosis in the treated lesion (arrow). – Credit: Fukui et al., 2014; Figure 1.

Balloon Pulmonary Angioplasty for CTEPH

A brief introduction. This is the shortest (about 15 minutes), somewhat recent (2019) video presentation on BPA that I could find. It provides context, introduces the technique, and discusses the outcomes and potential risks.

Presented by Dr. David Boshell at the Structural Heart Disease Australia Right Heart Symposium on 23rd February 2019.

Balloon Pulmonary Angioplasty as a Treatment in Chronic Thromboembolic Pulmonary Hypertension: Past, Present, and Future.

A scientific review. Read the Abstract here.

Until recently, BPA was little known as a possible option to treat CTEPH effectively. The primary treatment choices were pulmonary endarterectomyPulmonary Endarterectomy For patients with operable CTEPH, pulmonary endarterectomy (PEA) remains the treatment of choice. Obstructive material (old blood clots and scar tissue) is surgically removed from the arteries in the lungs. PEA is technically challenging, requiring intermittent total circulatory arrest conducted under severe hypothermia. Yet, PEA has been demonstrated to improve pulmonary hemodynamics and survival rate, which is otherwise poor in CTEPH patients. PEA is also known as pulmonary thromboendarterectomy (PTE). - References: Darocha et al., 2021. doi: 10.3390/jcm10051038 and Pulmonary Endarterectomy - St Vincent's Lung Health. Retrieved 6 November 2022 (PEAPulmonary Endarterectomy For patients with operable CTEPH, pulmonary endarterectomy (PEA) remains the treatment of choice. Obstructive material (old blood clots and scar tissue) is surgically removed from the arteries in the lungs. PEA is technically challenging, requiring intermittent total circulatory arrest conducted under severe hypothermia. Yet, PEA has been demonstrated to improve pulmonary hemodynamics and survival rate, which is otherwise poor in CTEPH patients. PEA is also known as pulmonary thromboendarterectomy (PTE). - References: Darocha et al., 2021. doi: 10.3390/jcm10051038 and Pulmonary Endarterectomy - St Vincent's Lung Health. Retrieved 6 November 2022 ) and targeted drug therapy.

With the development of BPA, the therapy options for CTEPH have expanded. This review aims to better understand and assess BPA as a therapeutic option for CTEPH patients.

Data from around the world confirm that BPA is a challenging but potentially valuable intervention for treating patients with inoperable CTEPH.

Karyofyllis, P., Demerouti, E., Papadopoulou, V., Voudris, V., & Matsubara, H. (2020). Balloon Pulmonary Angioplasty as a Treatment in Chronic Thromboembolic Pulmonary Hypertension: Past, Present, and Future. Current treatment options in cardiovascular medicine, 22(3), 7. https://doi.org/10.1007/s11936-020-0807-3

2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary HypertensionPulmonary Hypertension (PH) High pressure in the blood vessels leading from the heart to the lungs is called pulmonary hypertension. The arteries in the lungs and the right side of the heart are affected by pulmonary hypertension. If untreated, the right side of the heart will weaken over time, eventually leading to heart failure and death. The WHO has defined five distinct groups of pulmonary hypertension, each with a different cause. CTEPH is a form of PH (WHO group 4). - References: mayoclinic.org and cdc.gov

The guidelines were updated in 2022 for the first time in seven years, based on the most recent scientific findings and clinical practices.

These guidelines cover all types of pulmonary hypertension (PH), including CTEPH (WHO group 4). – The World Health Organization (WHO) classifies pulmonary hypertension (PH) into five groups based on etiology or comorbidity (Wijeratne et al., 2018). – Besides pulmonary endarterectomy (PEA), and medical therapies, BPA is listed as a treatment option for CTEPH (see Recommendation Table 24 in the guidelines).

A discussion of the 2022 guidelines with the first author, Dr. Marc Humbert, is available on Medscape in episode 6 of their InDiscussion series of podcasts.

Humbert, M., Kovacs, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Escribano-Subias, P., Ferrari, P., Ferreira, D. S., Ghofrani, H. A., Giannakoulas, G., Kiely, D. G., Mayer, E., Meszaros, G., Nagavci, B., Olsson, K. M., Pepke-Zaba, J., Quint, J. K., … ESC/ERS Scientific Document Group (2022). 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European heart journal, 43(38), 3618–3731. https://doi.org/10.1093/eurheartj/ehac237

More resources

BPA is significantly less invasive than pulmonary endarterectomy; it does not require general anesthesia or circulatory arrest and can be performed via a small surgical incision […]

Madani, M., Ogo, T., & Simonneau, G. (2017). The changing landscape of chronic thromboembolic pulmonary hypertension management. European respiratory review : an official journal of the European Respiratory Society, 26(146), 170105. https://doi.org/10.1183/16000617.0105-2017

Balloon pulmonary angioplasty is a new percutaneous vascular technique for patients with inoperable CTEPH. This review summarises the available evidence for BPA, patient selection, recent technical refinements and periprocedural imaging, and discusses the potential future role of BPA in the management of CTEPH.

Lang, I., Meyer, B. C., Ogo, T., Matsubara, H., Kurzyna, M., Ghofrani, H. A., Mayer, E., & Brenot, P. (2017). Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. European respiratory review : an official journal of the European Respiratory Society, 26(143), 160119. https://doi.org/10.1183/16000617.0119-2016

Medscape’s InDiscussion: Pulmonary Arterial Hypertension series includes two recent podcasts on CTEPH and BPA.

Transcripts of the podcasts are also available on the respective show pages.

The hero image for the blog "Post-PE Patient Management: Suggestions by a CTEPH Sufferer"

Post-PE Patient Management: Suggestions by a CTEPH Sufferer

After a pulmonary embolism, regular screening for chronic thromboembolic pulmonary hypertension (CTEPH) is essential. Detecting this condition as early as possible minimizes damage to the heart and dramatically enhances the chances of recovery. The suggestions in this post are based on my personal experience as a CTEPH patient and my understanding of the literature.

Go To Suggestions

This post was first published on December 6, 2022, and later revised on June 20, 2023, after my second visit to the OMC. On September 11, 2023, I added the Post-PE Patient Management Suggestions.

Additional references

Ogawa, A., & Matsubara, H. (2018). After the Dawn　- Balloon Pulmonary Angioplasty for Patients With Chronic Thromboembolic Pulmonary Hypertension. Circulation journal : official journal of the Japanese Circulation Society, 82(5), 1222–1230. https://doi.org/10.1253/circj.CJ-18-0258

Wijeratne, D. T., Lajkosz, K., Brogly, S. B., Lougheed, M. D., Jiang, L., Housin, A., Barber, D., Johnson, A., Doliszny, K. M., & Archer, S. L. (2018). Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension: A Population-Based Cohort Study in Ontario, Canada. Circulation. Cardiovascular quality and outcomes, 11(2), e003973. https://doi.org/10.1161/CIRCOUTCOMES.117.003973

Fukui, S., Ogo, T., Morita, Y., Tsuji, A., Tateishi, E., Ozaki, K., Sanda, Y., Fukuda, T., Yasuda, S., Ogawa, H., & Nakanishi, N. (2014). Right ventricular reverse remodelling after balloon pulmonary angioplasty. The European respiratory journal, 43(5), 1394–1402. https://doi.org/10.1183/09031936.00012914

Bagalas, V., Paspala, A., Sourla, E., Akritidou, S., Tsolakidou, K., Boutou, A., Pitsis, A. A., Manika, K., Kioumis, I. P., Stanopoulos, I., & Pitsiou, G. (2014). Chronic thromboembolic pulmonary hypertension: do not miss the chance for an early diagnosis. The American journal of case reports, 15, 378–381. https://doi.org/10.12659/AJCR.891014