The German pulmonary hypertension association, ph e.v., held the 24th edition of its Annual Patient Meeting on October 20-22, 2023, at the Education Center of the Hessian State Sports Association in Frankfurt am Main. The ph e.v. annual meetings consistently draw a very large number of participants from all over the country. The program combines scientific presentations with case studies and workshops on different aspects of living with pulmonary hypertension. There is also a yearly awards ceremony for best journalist.
The original report from ph e.v. in German and photos of the event are available at this link, and what follows is a translated version.
The ph e.v. Chairman, Hans-Dieter Kulla, welcomed a very large number of patients, family members, experts, and supporters to a comprehensive program of presentations, workshops, and information sessions with the aforementioned words. The Hessian Minister for Social Affairs and Integration, Kai Klose, conveyed a greeting via video.
Journalist Award ‘Together Against Pulmonary Arterial Hypertension’ for Volker Arend
Hans-Dieter Kulla explained that the ph e.v. not only maintains its own website but is also present on all social media platforms. Additionally, the association is involved in the new European umbrella organization AfPH (Alliance for Pulmonary Hypertension), which serves for international networking and has established a publicly accessible knowledge sharing platform. This is particularly important because pulmonary arterial hypertension is a serious condition that affects the lungs and the heart, and is often recognised late due to nonspecific symptoms. To ensure that affected individuals receive an early diagnosis and appropriate therapy, it is important to raise awareness not only among the medical community but also among the public. The media plays a crucial role in this. Therefore, ph e.v. awards an annual journalist prize titled ‘Together Against Pulmonary Arterial Hypertension’, endowed with € 3.000,00. For 2023, the Jury, composed of experts from the medical and media fields, selected biologist and science journalist Volker Arend as the awardee. He developed the TV format ‘Abenteuer Diagnose’ (Adventure Diagnosis) for NDR (Norddeutscher Rundfunk, TV broadcaster). In a program titled ‘Lungenhochdruck belastet das Herz’ (Pulmonary Arterial Hypertension Burdens the Heart), broadcast on February 14, 2023, as part of the program ‘Visite’, he narrated the story of a patient – as nformative, touching, and gripping as a true-crime film. Volker Arend is currently filming the next season of ‘Abenteuer Diagnose’ and therefore, unfortunately, could not personally accept the award. However, he sent a thank-you message via video to ph e.v.: The aim of his series is to focus on diagnostically challenging diseases, which is why he is particularly pleased with the award.
Significance of Genetic Testing
Professor Ekkehard Grünig, from Heidelberg, and Professor Silvia Ulrich, from Zurich, moderated the presentations on October 21, ranging from current studies to practical tips for dealing with pulmonary arterial hypertension. Professor Christina A. Eichstaedt, spokesperson of the newly established German Center for Genetics of Pulmonary Hypertension at the University Hospital Heidelberg, spoke about the significance of genetic testing. Pulmonary arterial hypertension can be familial, sporadic, drug-induced, or associated with other conditions. In the majority of patients with familial pulmonary arterial hypertension, mutations in the BMPR2 gene are detectable. This is the gene with the most mutations relevant to pulmonary hypertension, although other genes may also be responsible for the condition. To examine all known genes, the Heidelberg research team has developed a specific gene panel for diagnostics. Not everyone who carries the gene develops pulmonary arterial hypertension. Professor Eichstaedt considers genetic testing to be useful for relatives of affected individuals to identify asymptomatic carriers of mutations, as well as for certain forms of pulmonary arterial hypertension, such as hereditary and idiopathic, to clarify differential diagnoses and plan follow-up examinations.
Groundbreaking Drug Pending Approval
Professor Ekkehard Grünig, Head of the Department of Thoracic Surgery at Kerckhoff Clinic in Bad Nauheim, reported on a groundbreaking new medication, sotatercept, which is the first substance ever to address the underlying causes of pulmonary arterial hypertension. Studies are still ongoing; doctors are hopeful for imminent approval. All pulmonary arterial hypertension patients can benefit from sotatercept, including those without a genetic mutation, because the substance restores the protective effect of the BMPR2 gene, which is reduced in pulmonary arterial hypertension. ‘We hope that sotatercept can significantly improve the disease in a few years,’ said Professor Grünig.
Complex Therapy for Chronic Thromboembolic Pulmonary Hypertension
PD Dr. Stefan Guth, Head of the Department of Thoracic Surgery at Kerckhoff Clinic in Bad Nauheim, spoke about therapeutic options for chronic thromboembolic pulmonary hypertension (CTEPH). This is a special form of pulmonary hypertension, which can occur after a pulmonary embolism, if the blood clot is not dissolved, resulting in the formation of scar tissue that narrows the lung vessels. Chronic thromboembolic pulmonary hypertension is now considered to be treatable. The standard therapy for all operable patients is pulmonary endarterectomy (PEA), a complex operation in complete circulatory arrest, during which the pulmonary arteries are excised. Changes in very fine lung vessels can be treated with pulmonary balloon angioplasty (BPA). First, guide wires are inserted into the vessels, and then the vessels are dilated with balloons. Pulmonary balloon angioplasty is also suitable for non-operable patients. Medications for chronic thromboembolic pulmonary hypertension are also available. Often, a combination of different therapies is indicated. ‘Complex possibilities require complex decisions,’ summarized PD Dr. Guth.
Physical Training at an Individual Level
Professor Silvia Ulrich, Director of the Clinic for Pneumology at the University Hospital Zurich, dedicated her presentation to general measures and supportive therapies for pulmonary arterial hypertension. These include limiting salt and fluid intake, diuretics, if necessary, medications for high blood pressure, iron substitution for low levels of iron, and blood-thinning agents, which are indicated for life in chronic thromboembolic pulmonary hypertension. Nighttime oxygen therapy improves daytime endurance. Regarding exercise, the belief that pulmonary hypertension patients must avoid physical activity under all circumstances is long outdated. ‘Monitored training, adapted to the individual level, is safe and effective for strengthening muscles and improving hemodynamics, emphasized Professor Ulrich.
Measurement of Blood Flow – A Glimpse into Medical History
Hemodynamics – this is the blood flow in the blood vessels, which depends on various factors that mutually influence each other, including the geometry and elasticity of the vessels, as well as the volume and composition of the blood. Professor Heinrike Wilkens, pulmonologist at the University Hospital of Saarland in Homburg/Saar, explained how hemodynamics can be measured in pulmonary hypertension. Despite various newer developments, right heart catheterization remains indispensable. This invasive but now very safe procedure provides unequivocal results in numbers regardless of the examiner. In her presentation, Professor Wilkens also provided insights into an exciting piece of medical history by reporting on the development of right heart catheterization – starting with the later Nobel laureate Werner Forßmann, who, as a young assistant doctor in 1929, performed the very first heart catheterization in a not entirely risk-free self-experiment.
Workshops and Honoring
The ph e.v. patient meeting offered numerous workshops on various topics ranging from nutrition, breathing therapy to traveling with PH, in addition to the presentations. An award ceremony was also part of the program: Ralf Lissel received a certificate from ph e. v. Chairman Hans-Dieter Kulla for leading the ph e.v. regional association of Saxony for ten years, along with special praise for his exemplary dedication.
Learn more about the ph e.v. at this link