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Dr. Lavinia Neubert and Professor Jonigk. Photo: Karin Kaiser / MHH

Improved Diagnosis In Rare Form Of Pulmonary Hypertension

News 2020-265-E EN

A research team from Hannover with participation of the DZL have published a study on molecular markers for various forms of pulmonary hypertension in the American Journal of Pathology.

A multitude of lung diseases is associated with what is referred to as pulmonary hypertension, which means that there is increased pressure in the pulmonary circulation pumping deoxygenated blood to the lungs and oxygenated blood back to the heart. Those affected suffer from respiratory distress, chest pain, and weakness. In the long run, pulmonary hypertension can lead to complete overloading of the right ventricle and even be fatal. For treatment and prognosis of the disease, it is vital to know whether the problem originates in the arteries or the veins of the pulmonary vessels. It is difficult, however, to distinguish between pulmonary arterial hypertension (PAH) and the very rare pulmonary veno-occlusive disease (PVOD). A research team of the Institute of Pathology at Hannover Medical School (MMH) has now come up with a new approach allowing the more accurate definition of the diagno-sis using molecular markers. The study will be published in the American Journal of Pathol-ogy and is available online.

“The development and progression of PVOD have not yet been understood sufficiently“, explains Dr. Lavinia Neubert, principal investigator and member of the Lung Research Study Group as well as the German Center for Lung Research (DZL) at the BREATH site in Hannover. Furthermore, PAH and PVOD are clinically very similar making an accurate diagnosis more difficult. This, however, is important as treatment and diagnosis largely depend on whether vascular remodelling occurs in the arterial or venous pulmonary circulation and consequently leads to constriction, blocking, or complete destruction of blood vessels. “In PVOD, the conventional therapy for PAH to lower pulmonary hypertension by means of what are called vasodilatory agents, may lead to the worsening of symptoms and even life-threatening pulmonary edema“, the pathologist points out. Pulmonary hypertension is incurable anyway and can only be managed by medication. Currently, a lung transplant is the last resort for patients.

In the study, lung samples from female and male patients with PAH, PVOD, idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD) were analysed and compared with healthy lung tissue. The researchers discovered six target genes that quali-fied as molecular markers for the differentiation between PAH and PVOD. “So far, an accurate diagnosis of PVOD could only be established after tissue analysis, even though taking a biopsy always means a great risk for patients with pulmonary hypertension due to the risk of bleeding,” Professor Dr. Danny Jonigk, Head of the Working Group for Lung Research, suggested. The next step will now be to examine if these molecular markers can also be detected in a patient’s blood and urine. If this is the case, it may significantly facilitate an early diagnosis of PVOD and the distinction between PVOD and PAH.

Scientific Contact

Dr. Lavinia Neubert
Telephone (0511) 532-4480

Original publication

Source: idw

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