Background Optimally treated patients with coarctation of the aorta remain at risk for late vascular dysfunction. The effect of treatment modality on vascular function is unknown. The LOVE‐COARCT (Long‐term Outcomes and Vascular Evaluation After Successful Coarctation of the Aorta Treatment) study was done to compare vascular function in patients with coarctation of the aorta treated with surgery, balloon dilation (BD), or stent implantation. Methods and Results In treated coarctation of the aorta patients without residual coarctation, we prospectively compared aortic stiffness by applanation tonometry and cardiac magnetic resonance; endothelial function by endothelial pulse amplitude testing; blood pressure ( BP ) phenotype by office BP , ambulatory BP monitoring, and BP response to exercise; left ventricular mass by cardiac magnetic resonance; and blood biomarkers of endothelial function, inflammation, vascular wall function, and extracellular matrix. Participants included 75 patients treated with surgery (n=28), BD (n=23), or stent (n=24). Groups had similar age at enrollment, coarctation of the aorta severity, residual gradient, and metabolic profile, but differed by age at treatment. Prevalence of systemic hypertension, aortic stiffness, endothelial function, and left ventricular mass were similar among treatment groups. However, BD patients had more‐distensible ascending aortas, lower peak systolic BP during exercise, less impairment in diurnal BP variation, and lower inflammatory biomarkers. Results were unchanged after adjustment for potential confounders, including age at treatment. Conclusions In our cohort of patients without residual coarctation, treatment modality was not associated with major vascular outcomes, even though there were some favorable vascular characteristics in the BD patients. Although this suggests that choice of treatment modality should continue to be driven by likelihood of achieving a good anatomical result, more long‐term studies are required to assess the clinical significance of the more‐optimal results of secondary markers of vascular function in BD patients. Clinical Trial Registration URL : http://www.clinicaltrials.gov . Unique identifier: NCT 03262753.

Hemodialysis catheters are used to support blood filtration, yet there are multiple fundamentally different approaches to catheter tip design with no clear optimal solution. Side-holes have been shown to increase flow rates and decrease recirculation but have been associated with clotting/increased infection rates. This study investigates the impact of changing the shape, size and number of side-holes on a simple symmetric tip catheter by evaluating the velocity, shear stress and shear rate of inflowing blood. A platelet model is used to examine the residence time and shear history of inflowing platelets. The results show that sideholes improve the theoretical performance of the catheters, reducing the maximum velocity and shear stress occurring at the tip compared to non-side-hole catheters. Increasing the side-hole area improved performance up to a point, past which not all inflow through the hole was captured, and instead a small fraction slowly 'washed-out' through the remainder of the tip resulting in greater residence times and increasing the likelihood of platelet adhesion. An oval shaped hole presents a lower chance of external fibrin formation compared to a circular hole, although this would also be influenced by the catheter material surface topology which is dependent on the manufacturing process. Overall, whilst side-holes may be associated with increased clotting and infection, this can be reduced when side-hole geometry is correctly implemented though; a sufficient area for body diameter (minimising residence time) and utilising angle-cut, oval shaped holes (reducing shear stress and chances of fibrin formation partially occluding holes).

The mitral valve is a complex anatomical structure whose physiological functioning relies on the biomechanical properties and structural integrity of its components.Their compromise can lead to mitral valve dysfunction, associated with morbidity and mortality. Therefore, a review on the morphometry of the mitral valve is crucial, more specifically on the importance of valve dimensions and shape for its function.This review initially provides a brief background on the anatomy and physiology of the mitral valve, followed by an analysis of the morphological information available.A characterisation of mathematical descriptions of several parts of the valve is performed and the impact of different dimensions and shape changes in disease is then outlined. Finally, a section regarding future directions and recommendations for the use of morphometric information in clinical analysis of the mitral valve is presented.

SummaryAddison’s disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison’s disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. She had been to the emergency department of her local hospital various times during the first trimester presenting with a clinical scenario suggestive of glucocorticoid under-replacement (nausea, persistent vomiting and hypotension), but this was interpreted as normal pregnancy symptoms. Hydrocortisone dose was adjusted, and the patient maintained regular follow-up. No complications were reported for the remainder of gestation and delivery. Pregnant patients with Addison’s disease should be monitored during gestation and in the peripartum period by multidisciplinary teams. Adjustments in glucocorticoid and mineralocorticoid replacement therapy are often necessary, and monitoring should be based mainly on clinical findings, which becomes increasingly difficult during pregnancy. Patient education and specialized monitoring are key to avoiding complications from under- or over-replacement therapy in this period.Learning points:An increase in glucocorticoid replacement dose is expected to be necessary during pregnancy in a woman with Addison’s disease.Patient education regarding steroid cover and symptoms of acute adrenal crisis are fundamental.Monitoring in this period is challenging and remains mainly clinical.The increase in hydrocortisone dose often obviates the need to increase fludrocortisone dose.

SummaryAddison’s disease (AD) is the most common endocrine manifestation of antiphospholipid syndrome (APS), but it remains a very rare complication of the syndrome. It is caused by adrenal venous thrombosis and consequent hemorrhagic infarction or by spontaneous (without thrombosis) adrenal hemorrhage, usually occurring after surgery or anticoagulant therapy. We present a clinical case of a 36-year-old female patient with a previous diagnosis of APS. She presented with multiple thrombotic events, including spontaneous abortions. During evaluation by the third episode of abortion, a CT imaging revealed an adrenal hematoma, but the patient was discharged without further investigation. A few weeks later, she presented in the emergency department with manifestations suggestive of adrenal insufficiency. Based on that assumption, she started therapy with glucocorticoids, with significant clinical improvement. After stabilization, additional investigation confirmed AD and excluded other etiologies; she also started mineralocorticoid replacement. This case illustrates a rare complication of APS that, if misdiagnosed, may be life threatening. A high index of suspicion is necessary for its diagnosis, and prompt treatment is crucial to reduce the morbidity and mortality potentially associated.Learning points:AD is a rare but life-threatening complication of APS.It is important to look for AD in patients with APS and a suggestive clinical scenario.APS must be excluded in patients with primary adrenal insufficiency and adrenal imaging revealing thrombosis/hemorrhage.Glucocorticoid therapy should be promptly initiated when AD is suspected.Mineralocorticoid replacement must be started when there is confirmed aldosterone deficiency.Hypertension is a common feature of APS; in patients with APS and AD, replacement therapy with glucocorticoids and mineralocorticoids may jeopardize hypertension management.

Woodhouse-Sakati Syndrome is a very rare autosomal recessive disorder caused by pathogenic variants in the DCAF17 gene, which encodes DDB1-and CUL4-associated factor 17. It is a multisystemic disorder characterized by hypogonadism, adolescent-to young adult-onset diabetes mellitus, hypothyroidism, and alopecia. Neurologic involvement includes childhood-onset moderate bilateral sensorineural hearing loss, mild intellectual disability adolescent-to young adult-onset of extrapyramidal findings, dysarthria, and dysphagia. Brain imaging typically reveals iron deposition in the globus pallidus and periventricular leukodystrophy. We report the case of a 31-year-old Portuguese female, the only child of a consanguineous couple. She presented with cognitive impairment, spastic paraparesis, lower limb dystonia, dysarthria, and dysphagia. She also had hypergonadotrophic hypogonadism associated with primary amenorrhea, insulin-dependent diabetes mellitus with retinopathy, primary hypothyroidism, moderate bilateral sensorineural hearing loss, and alopecia. Serial brain magnetic resonance imaging showed a progressive periventricular leukodystrophy with pontine involvement and significant bilateral iron deposition in the globus pallidus, substantia nigra, and red nucleus.

assessment of bicuspid aortic valve phenotypes: a fluid-structure interaction modelling approach', Cardiovascular Engineering and Technology.

Central venous catheters are widely used in haemodialysis therapy, having to respect design requirements for appropriate performance. These are placed within the right atrium (RA); however, there is no prior computational study assessing different catheter designs while mimicking their native environment. Here, a computational fluid dynamics model of the RA, based on realistic geometry and transient physiological boundary conditions, was developed and validated. Symmetric, split and step catheter designs were virtually placed in the RA and their performance was evaluated by: assessing their interaction with the RA haemodynamic environment through prediction of flow vorticity and wall shear stress (WSS) magnitudes (1); and quantifying recirculation and tip shear stress (2). Haemodynamic predictions from our RA model showed good agreement with the literature. Catheter placement in the RA increased average vorticity, which could indicate alterations of normal blood flow, and altered WSS magnitudes and distribution, which could indicate changes in tissue mechanical properties. All designs had recirculation and elevated shear stress values, which can induce platelet activation and subsequently thrombosis. The symmetric design, however, had the lowest associated values (best performance), while step design catheters working in reverse mode were associated with worsened performance. Different tip placements also impacted on catheter performance. Our findings suggest that using a realistically anatomical RA model to study catheter performance and interaction with the haemodynamic environment is crucial, and that care needs to be given to correct tip placement within the RA for improved recirculation percentages and diminished shear stress values.