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Objectives The objective of our study was to determine whether a simple score combining indices of right ventricular (RV) function and right atrial (RA) size would offer good discrimination of outcome in patients with pulmonary arterial hypertension (PAH). Background Identifying a simple score of outcome could simplify risk stratification of patients with PAH and potentially lead to improved tailored monitoring or therapy. Methods We recruited patients from both Stanford University (derivation cohort) and VU University Medical Center (validation cohort). The composite end-point for the study was death or lung transplantation. A Cox proportional hazard with bootstrap confidence interval adjustment model was used to determine independent correlates of death or transplantation. A predictive score was developed using the β- coefficients of the multivariate models. Results For the derivation cohort (n=95), the majority of patients were female (79%), average age was 43±11 years, mean pulmonary arterial pressure was 54±14 mmHg, and indexed pulmonary vascular resistance was 25±12 WU m2. Over an average follow-up of 5 years, the composite end-point occurred in 34 patients consisting of 26 deaths and 8 patients undergoing lung transplantation. On multivariate analysis, RV systolic dysfunction grade [HR 3.4, 2.0 to 7.8, P<0.001], severe RA enlargement [HR 3.0, 1.3 to 8.1, P=0.009] and systemic blood pressure <110 mmHg [HR 3.3, 1.5 to 9,4, P<0.001] were independently associated with outcome. A right heart (RH) score was constructed based on these 3 parameters compared favorably to the NIH survival equation (0.88[0.79 to 0.94] vs. 0.60[0.49 to 0.710], P< 0.001) but not statistically different than the REVEAL score c-statistic of 0.80[0.69 to 0.88] with P= 0.097. In the validation cohort (n=87), the RH score remained the strongest independent correlate of outcome. Conclusion In patients with prevalent PAH, a simple RH score may offer good discrimination of long term outcome in PAH.

Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD) who require cardiac surgery. At present, prevention and management of perioperative pulmonary hypertensive crisis is aimed at optimizing cardiopulmonary interactions by targeting prostacyclin, endothelin, and nitric oxide signaling pathways within the pulmonary circulation with various pharmacological agents. This review is aimed at familiarizing the practitioner with the current pharmacological treatment for dealing with perioperative pulmonary hypertensive crisis in PAH-CHD patients. Given the life-threatening complications associated with pulmonary hypertensive crisis, proper perioperative planning can help anticipate cardiopulmonary complications and optimize surgical outcomes in this patient population.

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IntroductionThe evolution in pulmonary arterial hypertension (PAH) management has been summarized in three iterations of the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. No study has assessed whether changes in management, as reflected in the changing guidelines, has translated to improved long-term survival in PAH.MethodsMixed retrospective/prospective analysis of treatment naïve, incident PAH patients (n=392) diagnosed at three major centers in Canada from 2009–2021. Patients were divided into two groups based on their diagnosis date and in accordance with three ESC/ERS guideline iterations: 2009 and 2015. Overall survival was assessed based on date of diagnosis and initial treatment strategy (i.e. mono versus combination).ResultsIn Canada, there was a shift towards more aggressive upfront management with combination therapy after the publication of the 2015 guidelines (10.4% and 30.8% in 2009–2015 patients, and 36.0% and 57.4% in 2016–2021 patients, for baseline and 2-year follow-up respectively). A key factor associated with combination therapy after 2015 was higher pulmonary vascular resistance (p=0.009). The 1, 3 and 5 year survival rates in Canada were 89.2%, 75.6%. and 56.0%, respectively. Despite changes in management, there was no improvement in long-term survival before and after publication of the 2015 ESC/ERS guideline (p=0.53).ConclusionsThere was an increase in the use of initial and sequential combination therapy in Canada after publication of the 2015 ESC/ERS guidelines, which was not associated with improved long-term survival. These data highlight the continued difficulties of managing this aggressive pulmonary disease in an era without a cure.