PH by Clinical Cases 2019. A quick overview

The 2019 edition analyzed some of the findings of the sixth World Symposium held in Nice in 2018

On June 14th and 15th, 2019 was held, in the prestigious location of Bologna’s Archiginnasio in Italy, the 8th edition of the annual medical Congress “Pulmonary Hypertension by Clinical Cases”. The well-known format of the event includes a series of 45 minutes of speeches, each one focused on a clinical case. Each speaker has 30 minutes for presentation and 15 for discussion, a generous amount of time to fully examine each case. The 2019 edition analyzed some of the findings of the sixth World Symposium held in Nice in 2018. Professor Nazzareno Galiè made a quick overview of the main topics discussed.


Pathobiology and genetics
Issues of inflammation have been addressed in different aspects. As an example, the case of a patient affected by lupus erythematosus with near normalization of hemodynamics due to immunosuppressive therapy was analyzed.

Discussion on genetics issues focused on rare mutations
The coincidence of congenital disease and PAH has also been addressed; in particular, the different areas in which the new hemodynamic classification is already effective were discussed.
The definition of pulmonary hypertension as pulmonary arterial pressure higher than 20mm of mercury has been discussed as relevant in PAH due to the scleroderma spectrum of disease.
It has been highlighted how some healthy carriers of the BMPR2 mutations may easily progress from a relatively low level of pulmonary hypertension to a more severe condition.
This also affects group 4 patients affected by pulmonary hypertension due to chronic thromboembolism (the group so-called “chronic thromboembolic disease”).
The new hemodynamic definition is relevant for group 2 and group 3 PH, (left heart disease and lung disease). An increase of mortality in the two groups, in fact, can be recognized from a level of 20mm of mercury of mean pulmonary arterial pressure. The discussion on PAH has been focused on risk stratification.

The heterogeneity of the low-risk patient group has been highlighted. The group gathers patients with relatively good prognosis and mild symptoms. The appropriate background therapy, nevertheless, may be different. It is actually possible to recognize greater effectiveness using monotherapy, double combination, or triple combination therapy, depending on the circumstances.

This group gathers the largest number of patients. In this case, the possibility to subclassify this group into 2 subgroups (higher and lower risk patients) has been discussed, specifying the opportunity of different treatments.

High-risk patients require maximal medical therapy including endothelin receptor antagonist, PDE5 inhibitor, intravenous prostacyclin analogues. High-risk patients may also require listing for lung transplantation. It has been argued how, considering the prolonged time on the waiting list, also H-R patients in stable condition should be listed for transplantation. It is possible to recognize relevant progress in the area of chronic thromboembolic pulmonary hypertension. It has been discussed endarterectomy as the treatment of choice but also balloon pulmonary angioplasty, which is an emerging and very fast spreading procedure for not operable patients.

Finally, the largest group with pulmonary hypertension (lung disease and left heart disease) has been analyzed.

Left heart disease
It has been outlined the importance of clinical diagnosis of heart failure with preserved ejection fraction; these patients are not only identified by the hemodynamics but also by the concomitant presence of multiple risk factors for both coronary artery disease and heart failure with preserved ejection fraction. In this case, the lack of efficacy and the possible detrimental effects of drugs approved for pulmonary arterial hypertension has been highlighted.

Lung disease
Similar findings have been reported for pulmonary hypertension due to lung disease. Also in this area, the presence of pulmonary hypertension portend a worse prognosis. However pulmonary hypertension has been recognized not as a target of the treatment but as a flag highlighting the outcome.
The treatment in both, left heart disease and lung disease, is the treatment of the underlying conditions influencing the prognosis.

List of speaker
Fabio Dardi, Nazzareno Galiè, Enrico Gotti, Konrad Hoetzenecker, Gabor Kovacs,
Alessandra Manes, Eckhard Mayer, Horst Olschewski, Davide Pacini, Massimiliano Palazzini,
Laurent Savale, Gérald Simonneau, Olivier Sitbon, Adam Torbicki, Richard C. Trembath,
Jean-Luc Vachiéry.