In Eisenmenger syndrome, treatment with bosentan significantly improved walking distance, pulmonary blood flow, and PVR. Adding sildenafil to bosentan did not significantly improve walking distance but did increase saturation at rest. http://www.ClinicalTrial.gov: NCT00303004.

Background-End-stage heart failure is associated with impaired cardiac output (CO) and organ blood flow. We determined whether CO and peripheral perfusion are maintained during exercise in patients with an axial-flow left ventricular assist device (LVAD) and whether an increase in LVAD pump speed with work rate would increase organ blood flow. Methods and Results-Invasively determined CO and leg blood flow and Doppler-determined cerebral perfusion were measured during 2 incremental cycle exercise tests on the same day in 8 patients provided with a HeartMate II LVAD. In random order, patients exercised both with a constant (Ϸ9775 rpm) and with an increasing pump speed (ϩ400 rpm per exercise stage). At 60 W, the elevation in CO was more pronounced with increased pump speed (8.7Ϯ0.6 versus 8.1Ϯ1.1 L ⅐ min Ϫ1 ; meanϮSD; Pϭ0.05), but at maximal exercise increases in CO (from 7.0Ϯ0.9 to 13.6Ϯ2.5 L ⅐ min Ϫ1 ; PϽ0.01) and leg blood flow [0.7 (0.5 to 0.8) to 4.4 (3.9 to 4.8) L ⅐ min Ϫ1 per leg; median (range); PϽ0.001] were similar with both pumping modes. Normally, middle cerebral artery mean flow velocity increases from Ϸ50 to Ϸ65 cm ⅐ s Ϫ1 during exercise, but in LVAD patients with a constant pump speed it was low at rest (39Ϯ14 cm ⅐ s Ϫ1 ) and remained unchanged during exercise, whereas in patients with increasing pump speed, it increased by 5.2Ϯ2.8 cm ⅐ s Ϫ1 at 60 W (PϽ0.01). Conclusions-With maximal exercise, the axial-flow LVAD supports near-normal increments in cardiac output and leg perfusion, but cerebral perfusion is poor. Increased pump speed augments cerebral perfusion during exercise. (Circ Heart Fail. 2011;4:554-560.)

There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

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When compared to arterial blood gas analysis of blood samples drawn by arterial puncture, the gold standard in the field, fundus oximetry was found to be in good overall agreement with the arterial blood samples. Blood flow measurements will be needed to determine whether the systemic hypoxia is completely compensated, as suggested by oxygen extraction being comparable between the two groups.

AimsPatients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. The aim of this study was to collect clinical outcome data and to identify risk factors for a complicated course of COVID-19 in patients with ACHD.MethodsTwenty-five ACHD centres in nine European countries participated in the study. Consecutive patients with ACHD diagnosed with COVID-19 presenting to one of the participating centres between 27 March and 6 June 2020 were included. A complicated disease course was defined as hospitalisation for COVID-19 requiring non-invasive or invasive ventilation and/or inotropic support, or a fatal outcome.ResultsOf 105 patients with a mean age of 38±13 years (58% women), 13 had a complicated disease course, of whom 5 died. In univariable analysis, age (OR 1.3, 95% CI 1.1 to 1.7, per 5 years), ≥2 comorbidities (OR 7.1, 95% CI 2.1 to 24.5), body mass index of >25 kg/m2 (OR 7.2, 95% CI 1.9 to 28.3) and cyanotic heart disease (OR 13.2, 95% CI 2.5 to 68.4) were associated with a complicated disease course. In a multivariable logistic regression model, cyanotic heart disease was the most important predictor (OR 60.0, 95% CI 7.6 to 474.0).ConclusionsAmong patients with ACHD, general risk factors (age, obesity and multiple comorbidities) are associated with an increased risk of complicated COVID-19 course. Congenital cardiac defects at particularly high risk were cyanotic lesions, including unrepaired cyanotic defects or Eisenmenger syndrome.

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The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.