Tuesday 7 September 2021
Here is your daily briefing of all things PH from the ERS Congress 2021.
Dr. Harm Jan Bogaard discusses the difficulty in distinguishing post-capillary due to HFpEF and pre-capillary PH. The OPTICS study derived and validated a risk score for predicting post-capillary PH in community centres. An OPTICS score ≥104 was 100% specific with 100% PPV for post-capillary PH and performed better than the H2FpEF score. This may help avoid unnecessary right heart catheterizations.
2. Low DLCO in pulmonary vascular disease
Dr. Karen M Olsson discusses the differential diagnoses of low DLCO in PH. HFpEF with pulmonary vascular disease, idiopathic PAH, pulmonary veno-occlusive disease and the vanishing capillary syndrome are possibilities when the DLCO is very low. Survival is worse when the DLCO is < 45% and response to PAH therapies is reduced.
3. Portopulmonary hypertension
Dr. Laurent Savale presents the state-of-the-art on portopulmonary hypertension (PoPH), which occurs in approximately 2% of liver transplant candidates. PH can occur due to post-capillary PH, high cardiac output state, and PoPH. Patients with PVR between 2-3 WU are more likely to develop overt PoPH during follow-up. Once established, the survival at 5 years is only 51% and is strongly linked to severity of liver disease. Treatment with PAH therapies improves hemodynamics and may help achieve hemodynamic thresholds that permit liver transplantation.
4. Impact of COVID-19 lockdown on exercise capacity in PAH patients
Dr Claudia Baratto presented a poster on the effect of lockdown on 6MWD in stable PAH patients. Out of 63 stable patients there was a clear drop in 6MWD after a 3-month lockdown, with an average drop of 13 meters. This could have an impart impact on patients in clinical trials during the pandemic and could affect risk assessment for some patients. It is not yet clear whether this drop is clinically meaningful, recoverable or related to disease progression or deconditioning.
Poster ID 3589