OBJECTIVE:Vascular Ehlers-Danlos syndrome (vEDS) is a rare monogenetic disease caused by pathogenic variants in procollagen 3A1. Arterial rupture is the most serious clinical manifestation. A randomised controlled trial, the Beta-Blockers in Ehlers-Danlos Syndrome Treatment (BBEST) trial, reported a significant protective effect of the beta blocker celiprolol. The aim was to study the outcome of celiprolol treatment in a cohort of Swedish patients with vEDS. METHODS:Uppsala is a national referral centre for patients with vEDS. They are assessed by vascular surgeons, angiologists, and clinical geneticists. Family history, previous and future clinical events, medication, and side effects are registered. Celiprolol was administered twice daily and titrated up to a maximum dose of 400 mg daily. Logistic regression was used to analyse predictors of vascular events. RESULTS:Forty patients with pathogenic sequence variants in COL3A1 were offered treatment with celiprolol in the period 2011-2019. The median follow up was 22 months (range 1-98 months); total follow up was 106 patient years. In two patients, uptitration of the dose is ongoing. Of the remaining 38, 26 (65%) patients reached the target dose of 400 mg daily. Dose uptitration was unsuccessful in six patients because of side effects; one died before reaching the maximum dose, and five terminated the treatment. Five major vascular events occurred; four were fatal (ruptured ascending aorta; aortic rupture after type B dissection; ruptured cerebral aneurysm; and ruptured pulmonary artery). One bled from a branch of the internal iliac artery, which was successfully coiled endovascularly. The annual risk of a major vascular event was 4.7% (n = 5/106), similar to the treatment arm of the BBEST trial (5%) and lower than in the control arm of the same trial (12%). No significant predictor of vascular events was identified. CONCLUSION:Treatment with celiprolol is tolerated in most patients with vEDS. Despite fatal vascular events, these observations suggest that celiprolol may have a protective effect in vEDS.

Purpose: To investigate contemporary results of percutaneous transluminal renal angioplasty (PTRA). Materials and Methods: A multicentre retrospective study analysing all patients treated with PTRA for primary symptomatic renal artery stenosis (RAS) between 2010 and 2013 at four tertiary centres. Procedures during the preceding four years were counted to evaluate for change in PTRA frequency. Results: The number of PTRA procedures decreased by approximately 50% from 2006 to 2013. Patients treated in the post-ASTRAL period (n = 224) had a significant reduction in mean systolic pressure (168 to 146 mmHg, p < 0.01), diastolic pressure (84 to 76 mmHg, p < 0.01), number of anti-hypertensive drugs (3.54 to 3.05, p < 0.01), and anti-hypertensive treatment index (21.75 to 16.92, p < 0.01) compared to before PTRA. These improvements were maintained at one year and at the last clinical evaluation after a mean follow-up of 4.31 years. Renal function increased transiently without sustained improvement, or deterioration, during later follow-up. Thirteen patients (5.8%) eventually required dialysis, nine of these had eGFR Conclusion: The frequency of PTRA has decreased, indicating a higher threshold for invasive treatment of RAS in recent years. The reduction in blood pressures, the reduced need for anti-hypertensive medication, and stabilization of renal function over time suggest a clinical benefit for most patients who are now being treated with PTRA.

Introduction: Renal artery stenosis (RAS) can cause severe hypertension and renal impairment. Pharmacological treatment has improved, and the additional benefit of invasive treatment with percutaneous transluminal renal angioplasty (PTRA) has been questioned since the publication of the ASTRAL trial in 2009 (1). The aim of this study was to investigate contemporary results of PTRA. Methods: Consecutive patients treated with PTRA for primary symptomatic RAS between 2010 and 2013 at four tertiary referral centres were retrospectively reviewed. Information on indications, co-morbidities, procedural details, and treatment results were retrieved from medical records of hospitals, interventional units, and primary care. Measures of outcomes were systolic and diastolic blood pressures, anti-hypertensive medication evaluated by number of drugs and by a calculated anti-hypertensive treatment index, and renal function evaluated by the estimated glomerular filtration rate (eGFR). The number of PTRA procedures done at the participating centres during the preceding four years (2006‒2009) were recorded for comparison, to evaluate change in PTRA frequency Results: The number of PTRA procedures decreased by approximately 50% from 2006 to 2013. Patients treated in the post-ASTRAL period, 2010‒2013, had a significant reduction in mean systolic blood pressure (from 168 to 146 mmHg, p<0.01), mean diastolic blood pressure (84 to 76 mmHg, p<0.01), mean number of anti-hypertensive drugs (3.54 to 3.05, p<0.01), and mean anti-hypertensive treatment index (21.75 to 16.92, p<0.01) compared to before PTRA. These improvements were maintained at one year and at the last clinical evaluation after a mean follow-up of 4.31 years. In contrast, renal function (eGFR) increased only transiently following the PTRA procedure, without sustained improvement during later follow-up. There was no significant difference in outcomes between subgroups differentiated by different indications for PTRA. Conclusion: The frequency of PTRA has decreased, indicating a higher threshold for invasive treatment of RAS in recent years compared to before the publication of the ASTRAL trial. The reduction in systolic and diastolic blood pressures, and the reduced need for anti-hypertensive medication, suggest a clinical benefit for patients who are now being treated with PTRA. References 1. Wheatley K, Ives N, Gray R, Kalra PA, Moss JG, Baigent C, et al. Revascularization versus medical therapy for renal-artery stenosis. The New England journal of medicine. 2009;361(20):1953-62.

Background: Renal denervation (RDN) is a catheter-based intervention to treat patients with resistant hypertension. The biological effects of RDN are not fully understood, and randomized controlled trials have generated conflicting evidence. This report presents data from the Swedish Registry for Renal Denervation, an investigator-driven nationwide registry. Purpose: To assess the safety and efficacy of RDN on patients with resistant hypertension in a real-world clinical setting. Methods: This nationwide database contains patient characteristics, procedural details, and follow-up data on all RDN procedures performed in Sweden. Consecutive procedures between 2011 and 2015 were included. Results: The data analysis consists of 252 patients (mean age 61 ± 10 years, 38% women; mean 4.5 ± 1.5 antihypertensive drugs). Office SBP and DBP and 24-h ambulatory blood pressure (BP) decreased 6 months after RDN (176 ± 23/97 ± 17 to 161 ± 26/91 ± 16 mmHg, both P < 0.001; and 155 ± 17/89 ± 14 to 147 ± 18/82 ± 12 mmHg, both P < 0.001). Significant office and ambulatory BP reductions persisted throughout the observation period of 36 months. Major procedure-related vascular complications occurred in four patients. Renal function and number of antihypertensive drugs were unchanged during follow-up. Conclusion: In this complete national cohort, RDN was associated with a sustained reduction in office and ambulatory BP in patients with resistant hypertension. The procedure proved to be feasible and associated with a low-complication rate, including long-term adverse events.