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Background: Patients with obstructive hypertrophic cardiomyopathy have increased symptomatic burden. Mavacamten was recently approved for treatment of obstructive hypertrophic cardiomyopathy based on 2 randomized controlled trials. However, its use under real-world conditions and in diverse populations is under-studied., Methods and Results: This was a prospective observational cohort study of patients seen at the Johns Hopkins HCM center and prescribed mavacamten for obstructive hypertrophic cardiomyopathy between July 7, 2022 and January 6, 2024. Patients were followed longitudinally, with serial echocardiography and clinical evaluation as mandated by the risk evaluation and mitigation strategy program. Sixty-six patients received mavacamten (mean age 59 years, 47% male, 29% non-White [Black, Hispanic/Latino, Asian, Native Hawaiian or Pacific Islander], 47% obese). Before treatment, all patients had New York Heart Association class II (51.5%) or III (48.5%) heart failure symptoms. Initial maximum peak left ventricular outflow tract gradient was 107±46 mm Hg. Median treatment duration was 9 months. For patients on mavacamten after ≥6 months (n=43), symptoms improved by ≥1 New York Heart Association class in 72% of patients, and peak left ventricular outflow tract gradient decreased by 80±46 mm Hg, eliminating hemodynamically significant left ventricular outflow tract obstruction in 79.1% of patients. Mavacamten was temporarily discontinued in 3 patients due to left ventricular ejection fraction decrease <50%. There were no medication-related adverse events. Effectiveness and safety were similar between White and non-White patients, but symptomatic relief was attenuated in patients with body-mass index ≥35 kg/m 2 ., Conclusions: Mavacamten was effective and safe when used under real-world conditions in a racially diverse population of symptomatic patients with obstructive hypertrophic cardiomyopathy. Patients with comorbid obesity were less likely to experience symptomatic improvement while on mavacamten.

Background: The best regional anesthesia plan with the best clinical results for Carotid endarterectomy (CEA) has not been defined yet., Methods: Prospective multicentric observational study of two non-randomized matched cohorts including patients undergoing elective unilateral CEA (N.=100) between January-October 2021. Main outcomes were cerebral oximetry measurements, verbal numeric pain score assessment, peripheral nerve blockades and in-hospital stay. The main objective is to compare results achieved after carotid endarterectomy (CEA) performed under loco-regional blockade (LRB) versus general anesthesia (GA), in terms of intraoperative hemodynamic and neurologic variability. Patients undergoing LRB were performed under ultrasound (US) guidance and mild sedation., Results: The LRB and GA groups showed no differences in comorbidities and risk factors. However, there was a significant difference in the intraoperative hemodynamic behavior due to the amount of vasoactive drugs used (0% vs. 16% for phenylephrine, P=0.006). The results showed neurological stability through the cerebral oximetry measurements during the procedure except for the left hemisphere de-clamp values, which were higher in the GA group (68.7±9.9 vs. 72.7±8.8; P=0.035). There were also significant differences in the verbal pain scale scores assessed 6 hours and 12 hours after the procedure; better pain control was evidenced in the LRB group (0[0-1] vs. 1[0-3], P=0.01; 1[0.5-2] vs. 0[0-2], P=0.01). An increased transient hypoglossal and laryngeal nerves blockade was observed in the LRB group (30% vs. 4%; P<0.001). The in-hospital length of stay was longer in the GA group (77.2±36.3 hours vs. 129.1±41.1 hours; P<0.001)., Conclusions: Although the use of intermediate-deep cervical plexus blockade for CEA confers similar neurologic stability as GA does, there is a difference on the hemodynamic behaviour due to the differences in vasoactive drug consumption. Loco-regional techniques provide a better postoperative pain control and shorten in-hospital length of stay.

(1) Introduction: The aim was to describe the anesthetic and surgical technique of eversion carotid endarterectomy performed under intermediate-deep cervical block with sedation, and to analyze the intraoperative and postoperative results. (2) Material and Methods: Thirty cases of unilateral eversion carotid endarterectomy (n = 30), performed between 2019-2020 in a tertiary center under intermediate-deep ultrasound-guided cervical plexus block and sedation, were prospectively observed and analyzed. Hemodynamic (blood pressure, heart rate) and neurological (cerebral oximetry) variables were measured in four intraoperative phases: at the beginning of the operation, prior to carotid clamping, after unclamping and at the end of the operation. We assessed acute postoperative pain in a numerical rating scale at 6, 12 and 24 h, early and 30-day complications, and length of stay. (3) Results: Baseline mean arterial pressure values were 100.4 ± 18 mmHg, pre-clamping 95.8 ± 14 mmHg, post-clamping 94.9 ± 11 mmHg, and at the end of the operation 102.4 ± 16 mmHg. Cerebral oximetry values were 61.7 ± 7/62.7 ± 8, 68.5 ± 9.6/69.1 ± 11.7 and 68.1 ± 10/68.1 ± 10 for the left and right hemispheres at baseline, pre- and post-clamping, respectively. The pain assessment showed a score less than or equal to 3. The incidence of residual nerve block, early complications, and major complications in the first 30 days was 40%, 16.7% and 3.3%, respectively. (4) Conclusions: The combination of intermediate-deep cervical plexus block and low-dose sedation is an effective and safe alternative in awake eversion carotid endarterectomy.

Background: The ratio of tricuspid annular plane systolic excursion (TAPSE) to pulmonary artery systolic pressure (PASP) is a validated index of right ventricular-pulmonary arterial (RV-PA) coupling with prognostic value. We determined the predictive value of TAPSE/PASP ratio and adverse clinical outcomes in hospitalized patients with COVID-19., Methods: Two hundred and twenty-nine consecutive hospitalized racially/ethnically diverse adults (≥18 years of age) admitted with COVID-19 between March and June 2020 with clinically indicated transthoracic echocardiograms (TTE) that included adequate tricuspid regurgitation (TR) velocities for calculation of PASP were studied. The exposure of interest was impaired RV-PA coupling as assessed by TAPSE/PASP ratio. The primary outcome was in-hospital mortality. Secondary endpoints comprised of ICU admission, incident acute respiratory distress syndrome (ARDS), and systolic heart failure., Results: One hundred and seventy-six patients had both technically adequate TAPSE measurements and measurable TR velocities for analysis. After adjustment for age, sex, BMI, race/ethnicity, diabetes mellitus, and smoking status, log(TAPSE/PASP) had a significantly inverse association with ICU admission (p = 0.015) and death (p = 0.038). ROC analysis showed the optimal cutoff for TAPSE/PASP for death was 0.51 mm mmHg -1 (AUC = 0.68). Unsupervised machine learning identified two groups of echocardiographic function. Of all echocardiographic measures included, TAPSE/PASP ratio was the most significant in predicting in-hospital mortality, further supporting its significance in this cohort., Conclusion: Impaired RV-PA coupling, assessed noninvasively via the TAPSE/PASP ratio, was predictive of need for ICU level care and in-hospital mortality in hospitalized patients with COVID-19 suggesting utility of TAPSE/PASP in identification of poor clinical outcomes in this population both by traditional statistical and unsupervised machine learning based methods., (© 2022 Wiley Periodicals LLC.)

Purpose: The present international survey among healthcare providers aimed to collect data on theoretical knowledge and clinical practices in the diagnosis and management of cow's milk protein allergy (CMPA) and lactose intolerance (LI) in infants., Methods: A global survey was conducted in several countries with diverse health care settings. The survey consisted of multiple-choice questions in 3 main domains: (1) understanding and clinical practices around CMPA and LI; (2) case scenarios; and (3) disease-specific knowledge and potential educational needs., Results: Responses were available from 1,663 participants. About 62% of respondents were general practitioners or general pediatricians, and the remainder were pediatric allergists/gastroenterologists (18%) or other health practitioners (20%). The survey identified knowledge gaps regarding the types of CMPA (IgE-mediated vs. non-IgE-mediated) and the clinical overlap with LI. The survey suggested diverse clinical practices regarding the use of hypoallergenic formulas, as well as misconceptions about the prebiotic benefits of lactose in extensively hydrolyzed formulas in non-breastfed infants with CMPA. Responses to the two case scenarios highlighted varying levels of awareness of the relevant clinical practice guidelines. While respondents generally felt confident in managing infants with CMPA and LI, about 80% expressed an interest for further training in this area., Conclusion: The current survey identified some knowledge gaps and regional differences in the management of infants with CMPA or LI. Local educational activities among general and pediatric healthcare providers may increase the awareness of clinical practice guidelines for the diagnosis and treatment of both conditions and help improve clinical outcomes., Competing Interests: Conflicts of Interest: AJ and RGH are employees of Nestlé Health Science. All other authors declared no conflict of interest., (Copyright © 2022 by The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition.)

Purpose: To describe the use of large-diameter balloon-expandable stent-grafts (BeGraft aortic stent-graft, Bentley InnoMed GmbH, Hechingen, Germany) in the treatment of infrarenal penetrating aortic ulcer (iPAU)., Materials and Methods: Retrospective analysis of patients undergoing endovascular treatment with the BeGraft aortic stent-graft in 8 European centers from January 2017 to October 2020. Demographics, perioperative data, and midterm outcomes were collected. Endpoints of the study were technical feasibility, early mortality, and morbidity., Results: A total of 40 patients were included. The mean age was 73.9±7.05 years and 63.2% were male. Indications for treatment included size and morphology (65%), presence of symptoms (29.5%), and contained ruptures (5.5%). Urgent treatment was performed in 5% of cases. Technical success was 97.5%. Median operation time was 58 minutes (19-170 minutes), with 27.5% of patients having additional procedures during the main intervention (1 additional repair with a C-TAG (W.L. Gore & Associates, Inc, Flagstaff, AZ, USA) thoracic endoprosthesis, 5 covered endovascular reconstruction of aortic bifurcation procedures, 3 extensions with proximal cuffs, and 2 percutaneous angioplasties of the common iliac arteries). Percutaneous femoral access was used in 72.5%, while groin cut-down was performed in 27.5%. Repair was successful with only 1 stent in 45% of cases, while 37.5% required 2 stents and nearly 17.5% required 3/4 stent-grafts. The 30-day mortality was 0%, with a 2.5% reintervention rate (1 patient required evacuation of an intra-abdominal hematoma). Median follow-up was 13.9 months (2-39 months), during which no vascular-related reinterventions or deaths were reported. In 4 patients, a type II endoleak was observed. No cases of graft migration, thrombosis, or stent-fracture were observed., Conclusions: The treatment of iPAU with the BeGraft aortic stent-graft in a selective patient group is feasible with low rate of perioperative morbidity and mortality. Balloon-expandable stent-grafts offer the option to repair iPAUs with a shorter coverage of the aorta using low-profile sheath, that enables treatment in the presence of calcified access vessels and small diameter aortic bifurcations.

Objective: the aim of this study was to assess the results of mechanochemical endovenous ablation either in the primary or recurrent saphenous vein insufficiency, including only patients with veno-venous shunt type III., Methods: retrospective analysis of a prospective study of patients with symptomatic chronic venous insufficiency who underwent ClariVein® technique. A total of 134 saphenous veins were included between August 2017 and August 2018. Follow-up was performed by Duplex ultrasound at 1, 6 and 12 months. Primary endpoints were technical and anatomical success. Secondary endpoints were the need for further treatment of varicose collateral veins by sclerotherapy, outcomes regarding recurrent insufficiency and clinical success., Results: A total of 111 great saphenous veins and 23 small saphenous veins were treated with a technical success of 95.6%. The overall anatomical success rates at 1, 6 and 12 month were 96.2%, 88.8% and 84.4%, respectively, without differences between primary and recurrent insufficiency. Deferred sclerotherapy over varicose collaterals was carried out in 28% of the patients with anatomical success. Clinical improvement was achieved in 87.3%., Conclusions: MOCA technique has proven to be an effective technique, although additional treatment over varicose collaterals could be necessary in up to one-third. Atrophy of the saphenous vein and the lack of persistent varicose collateral veins during follow-up seem to be indicators of successful therapy.

Since the emergence of the COVID-19 pandemic, the most affected population group has been that of older people living in long-term care facilities (LTCFs), which has accumulated between 30 and 60 % of total number of deaths in the world. In Mexico, outbreaks have been reported in LTCFs of at least eight states. Various factors make this population group and LTCFs susceptible to COVID-1 outbreaks, mainly due to coexistence in common spaces, shared bedrooms and permanent physical contact with the personnel who work there, coupled with a lack of protocols and standards of care of mandatory observance, as well as personnel training limitations. There is evidence of the need to formally develop a National Care System that provides support to those in need of care and their families, and that includes LTCFs. In view of the challenges due to the lack of information and competencies in infection prevention and control at LTCFs, a group of experts, in collaboration with different public institutions, joined efforts with the purpose to update the guidelines in order to allow LTCFs face the pandemic and to contribute to the generation of said National Care System., (Copyright: © 2020 Permanyer.)

Background: Endovascular treatment of complex aortoiliac disease is seeing a growing popularity despite the Trans-Atlantic Inter-Society Consensus (TASC) II recommendations for open surgery in this cases. However, the available evidence does not focus particularly on patients with complete unilateral iliac axis obstruction (CIAO) (TASC II D4 group). This study reports mid-term results of endovascular therapy with covered stents for CIAO., Methods: This is single-center retrospective review of patients with CIAO endovascular treatment from January 2015 to December 2017 (3 years). Two types of covered stents were used, alone or combined: the Viabahn self-expandable stent (W. L. Gore, Flagstaff, AZ) and the Advanta V12 balloon-expandable stent (Atrium-Maquet, Hudson, NH). Thirty-day outcomes, long-term patency (assessed with Kaplan-Meier estimates), in-hospital stay, and limb salvage were analyzed., Results: Thirty-nine patients with CIAO were treated in the period (87.2% male, mean age 64.3 ± 9 years). A majority presented with critical limb ischemia (56.4%, n = 22). Recanalization could be accomplished from an ipsilateral or contralateral femoral access in 82.1% of patients (1 case needed the use of a re-entry device), and from a left brachial access in 17.9%. Technical success was 100%. About 66.7% of cases received an aortic kissing stent technique. Common femoral artery/profundoplasty with prosthetic or bovine patch was associated with 74.3% of cases. Thirty-day mortality was 2.6% (1/39). Primary, assisted, and secondary patency rates at 24 months were all 96.8%. Mean in-hospital stay was 5 days; no limb loss was registered during follow-up., Conclusions: Endovascular treatment of complete iliac axis occlusions can offer comparable midterm patency rates to open surgery aortoiliac femoral bypass, when an adequate combination of balloon and self-expandable covered stents is used and an appropriate outflow through the common femoral artery is warranted., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: The objective of this report is to present a challenging case of a mycotic aneurysm of the innominate artery (IA) in a patient with a bovine aortic arch., Materials and Methods: An 85-year-old woman presented with intermittent fever and positive blood cultures for Staphylococcus aureus. An echocardiogram and a positron emission tomography-computed tomography were performed, showing a hypermetabolic dilation of the IA compatible with a mycotic aneurysm with a type one bovine aortic arch. Conventional open arch repair and total endovascular repair with a custom-made aortic endograft were rejected given the elderly age and need for urgent repair. Treatment was achieved with a hybrid procedure, including a left carotid transposition and exclusion of the aneurysm with a modified Endurant II ® iliac limb (two stents were cut off and it was resheathed in an inverted fashion) released through a prosthetic graft sutured onto the right axillary artery, followed by coil embolization of the sac. One year after the repair, the patient is well with complete exclusion of the aneurysm., Conclusions: Under the need for urgent repair, "on-the-table" modification of standard endograft components can be an effective solution for aneurysm exclusion when off-the-shelf endovascular stent grafts do not meet the anatomical requirements., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose:: Endograft infection is an infrequent but one of the most serious and challenging complications after endovascular aortic repair. The aim of this study was to assess the management of this complication in a tertiary center., Case Series:: A retrospective analysis of a prospective database was performed including all patients who underwent elective endovascular abdominal aortic repair (EVAR) from 2003 to 2016 in a tertiary center. Seven cases of endograft infection were identified during the follow-up period from a total of 473 (1.48%) EVAR. Most frequent symptoms at presentation were fever (71.4%) and lumbar pain (57.1%). One case developed an early infection, while 6 cases were diagnosed as late infections. Mean time from endograft placement to symptom presentation was 28.3 months (2-91.5 months). Gram-positive cocci were the microorganisms most commonly isolated in blood cultures (66%). Two cases were managed with endograft removal and aortic reconstruction with a cryopreserved allograft, 2 cases with surgical drainage, and 2 cases exclusively with antibiotic therapy. In 1 case, the diagnosis was performed postoperatively based on intraoperative findings associated with positive graft cultures; and graft explantation was performed with "in situ" reconstruction using a Dacron graft. Perioperative mortality was 42.9%. One-year mortality was 57.1%. Mean follow-up was 21.5 months., Conclusion:: Endograft explantation is the gold standard of treatment; however, given the overall high morbi-mortality rates of this pathology, a tailored approach should always be offered depending on the patient's overall condition. Conservative management can be an acceptable option in those patients with short life expectancy and high surgical risk.

Echocardiography is the foundation for diagnostic cardiac testing, allowing for direct identification and management of various conditions. Point-of-care ultrasound (POCUS) has emerged as an invaluable tool for bedside diagnosis and management. The objective of this review is to address the current use and clinical applicability of POCUS to identify, triage, and manage a wide spectrum of cardiac conditions. POCUS can change diagnosis and management decisions of various cardiovascular conditions in a range of settings. In the outpatient setting, it is used to risk stratify and diagnose a variety of medical conditions. In the emergency department (ED) and critical care settings, it is used to guide triage and critical care interventions. Furthermore, the skills needed to perform POCUS can be taught to noncardiologists in a way that is retained and allows identification of normal and grossly abnormal cardiac findings. Various curricula have been developed that teach residents and advanced learners how to appropriately employ point-of-care ultrasound. In conclusion, POCUS can be a useful adjunct to the physical exam, particularly in critical care applications., (Copyright © 2018. Published by Elsevier Inc.)

Purpose: To describe the uses and advantages of hybrid vascular grafts (HVGs) for complex iliofemoral revascularization procedures., Case Report: We report 3 cases of iliofemoral occlusions repaired with the Gore HVG, used as an alternative technique to standardized common femoral artery (CFA) endarterectomy associated with iliac stenting or open repair. The indication in each of the 3 cases was different: In the first case, there was an absent CFA secondary to a mycotic pseudoaneurysm, in the second case, an iatrogenic lesion of the posterior wall of the CFA during the intervention was performed, and in the third case, the indication was because of thrombosis of a previous iliofemoral revascularization procedure., Conclusion: Although we only present 3 cases, the Gore HVG can be useful for complex iliofemoral revascularizations, serving as an alternative to standardized CFA endarterectomy plus iliac stenting in cases where there is a damaged or absent CFA, especially in high-surgical risk patients.

Lactose is the main carbohydrate in human and mammalian milk. Lactose requires enzymatic hydrolysis by lactase into D-glucose and D-galactose before it can be absorbed. Term infants express sufficient lactase to digest about one liter of breast milk daily. Physiological lactose malabsorption in infancy confers beneficial prebiotic effects, including the establishment of Bifidobacterium-rich fecal microbiota. In many populations, lactase levels decline after weaning (lactase non-persistence; LNP). LNP affects about 70% of the world's population and is the physiological basis for primary lactose intolerance (LI). Persistence of lactase beyond infancy is linked to several single nucleotide polymorphisms in the lactase gene promoter region on chromosome 2. Primary LI generally does not manifest clinically before 5 years of age. LI in young children is typically caused by underlying gut conditions, such as viral gastroenteritis, giardiasis, cow's milk enteropathy, celiac disease or Crohn's disease. Therefore, LI in childhood is mostly transient and improves with resolution of the underlying pathology. There is ongoing confusion between LI and cow's milk allergy (CMA) which still leads to misdiagnosis and inappropriate dietary management. In addition, perceived LI may cause unnecessary milk restriction and adverse nutritional outcomes. The treatment of LI involves the reduction, but not complete elimination, of lactose-containing foods. By contrast, breastfed infants with suspected CMA should undergo a trial of a strict cow's milk protein-free maternal elimination diet. If the infant is not breastfed, an extensively hydrolyzed or amino acid-based formula and strict cow's milk avoidance are the standard treatment for CMA. The majority of infants with CMA can tolerate lactose, except when an enteropathy with secondary lactase deficiency is present., Competing Interests: Not required.All authors have reviewed the manuscript and have provided their consent for publication.Dr. Ralf Heine has been a member of the scientific advisory boards of Nestlé Health Science / Nestlé Nutrition Institute, Australia/Oceania and Nutricia Australia. He has received honoraria from industry for educational activities. All authors have received reimbursement for travel expenses for this project from Nestlé Health Science, Switzerland. The authors otherwise declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Background: Physicians spend less time at the bedside in the modern hospital setting which has contributed to a decline in physical diagnosis, and in particular, cardiopulmonary examination skills. This trend may be a source of diagnostic error and threatens to erode the patient-physician relationship. We created a new bedside cardiopulmonary physical diagnosis curriculum and assessed its effects on post-graduate year-1 (PGY-1; interns) attitudes, confidence and skill., Methods: One hundred five internal medicine interns in a large U.S. internal medicine residency program participated in the Advancing Bedside Cardiopulmonary Examination Skills (ACE) curriculum while rotating on a general medicine inpatient service between 2015 and 2017. Teaching sessions included exam demonstrations using healthy volunteers and real patients, imaging didactics, computer learning/high-fidelity simulation, and bedside teaching with experienced clinicians. Primary outcomes were attitudes, confidence and skill in the cardiopulmonary physical exam as determined by a self-assessment survey, and a validated online cardiovascular examination (CE)., Results: Interns who participated in ACE (ACE interns) by mid-year more strongly agreed they had received adequate training in the cardiopulmonary exam compared with non-ACE interns. ACE interns were more confident than non-ACE interns in performing a cardiac exam, assessing the jugular venous pressure, distinguishing 'a' from 'v' waves, and classifying systolic murmurs as crescendo-decrescendo or holosystolic. Only ACE interns had a significant improvement in score on the mid-year CE., Conclusions: A comprehensive bedside cardiopulmonary physical diagnosis curriculum improved trainee attitudes, confidence and skill in the cardiopulmonary examination. These results provide an opportunity to re-examine the way physical examination is taught and assessed in residency training programs.

We report our experience with the use of a Spanish version of the rapid geriatric assessment of 30 patients in 2 long-term care institutions in Mexico City by a group of healthcare students without prior experience in geriatric medicine. The Spanish version of the rapid geriatric assessment is a very easy tool to administer that can provide overall good results for identifying frailty, sarcopenia, anorexia, weight loss, and cognitive impairment., (Copyright © 2017 AMDA – The Society for Post-Acute and Long-Term Care Medicine. Published by Elsevier Inc. All rights reserved.)

In urban populations, worsening renal function (WRF) is well established in patients hospitalized with acute decompensated heart failure with preserved ejection fraction (HFpEF). However, the mechanisms for development of WRF in the setting of acute HF in HFpEF are unclear. In the present study, we sought to characterize conventional echocardiographic measures of right ventricular (RV) chamber size and function to determine whether RV dysfunction and/or adverse RV remodeling is related to WRF in patients with HFpEF. Our study included 104 adult patients with HFpEF (EF ≥ 55%) with technically adequate 2-dimensional echocardiograms performed during their hospitalization for acute decompensated HF to determine echocardiographic predictors of WRF, defined as a serum creatinine (Cr) increase of ≥ 0.3 mg/dl within 72 hours of hospitalization. Thirty-eight of the 104 patients (36%) developed WRF (mean Cr increase = 0.9 ± 0.1 mg/dl) during the hospitalization (mean age ± SD of 64 ± 12 years, 27 women [71%], 29 black [76%]). There were no significant differences in LV medial E/e' ratio and RV systolic pressure by WRF status or in linear dimensions of RV and right atrial size. RV fractional area change, a measure of RV function, however, was significantly decreased in HFpEF patients with WRF compared with the no WRF group (p = 0.003), whereas RV free wall thickness (p = 0.001) was increased. In conclusion, linear and volumetric measures of dimensions of right atrial and RV chamber size did not distinguish HFpEF patients with and without WRF. However, in HFpEF patients with WRF during acute HF hospitalization, there was a significant decrease in RV function and a significant increase in RV free wall thickness compared with matched patients with no WRF. These findings suggest that adverse RV remodeling and RV dysfunction occur in HFpEF patients with WRF., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Conventional open surgery still remains as the gold standard of care for aortic arch and thoracoabdominal pathology. In centers of excellence, open repair of the arch has been performed with 5% immediate mortality and a low rate of complications; however overall mortality rates are around 15%, being up to 40% of all patients rejected for treatment due to their age or comorbidities. For thoracoabdominal aortic pathology, data reported from centers of excellence show immediate mortality rates from 5% to 19%, spinal cord ischemia from 2.7% to 13.2%, and renal failure needing dialysis from 4.6% to 5.6%. For these reasons, different alternatives that use endovascular techniques, including debranching procedures, have been developed. The reported results for hybrid debranching procedures are controversial and difficult to interpret because series are retrospective, heterogenic and including a small number of patients. Clearly, an important selection bias exists: debranching procedures are performed in elderly patients with more comorbidities and with thoracoabdominal aortic aneurysms that have more complex and extensive disease. Considering this fact, debranching procedures still remain a useful alternative: for aortic arch pathology debranching techniques can avoid or reduce the time of extracorporeal circulation (ECC) or cardiac arrest which may be beneficial in high-risk patients that otherwise would be rejected for treatment. And compared to pure endovascular techniques, they can be used in emergency cases with applicability in a wide range of anatomies. For thoracoabdominal aortic aneurysms, they are mainly useful when other lesser invasive endovascular options are not feasible due to anatomical limitations or when they are not available in cases where delaying the intervention is not an option., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Background: Pressure overload resulting from aortic stenosis causes maladaptive ventricular and vascular remodeling that can lead to pulmonary hypertension, heart failure symptoms, and adverse outcomes. Retarding or reversing this maladaptive remodeling and its unfavorable hemodynamic consequences has the potential to improve morbidity and mortality. Preclinical models of pressure overload have shown that phosphodiesterase type 5 inhibition is beneficial; however, the use of phosphodiesterase type 5 inhibitors in patients with aortic stenosis is controversial because of concerns about vasodilation and hypotension., Methods and Results: We evaluated the safety and hemodynamic response of 20 subjects with severe symptomatic aortic stenosis (mean aortic valve area, 0.7 ± 0.2 cm(2); ejection fraction, 60 ± 14%) who received a single oral dose of sildenafil (40 or 80 mg). Compared with baseline, after 60 minutes, sildenafil reduced systemic (-12%; P<0.001) and pulmonary (-29%; P=0.002) vascular resistance, mean pulmonary artery (-25%; P<0.001) and wedge (-17%; P<0.001) pressures, and increased systemic (13%; P<0.001) and pulmonary (45%; P<0.001) vascular compliance and stroke volume index (8%; P=0.01). These changes were not dose dependent. Sildenafil caused a modest decrease in mean systemic arterial pressure (-11%; P<0.001) but was well tolerated with no episodes of symptomatic hypotension., Conclusions: This study shows for the first time that a single dose of a phosphodiesterase type 5 inhibitor is safe and well tolerated in patients with severe aortic stenosis and is associated with improvements in pulmonary and systemic hemodynamics resulting in biventricular unloading. These findings support the need for longer-term studies to evaluate the role of phosphodiesterase type 5 inhibition as adjunctive medical therapy in patients with aortic stenosis.

Background: Giant cell arteritis (GCA) is the most common primary systemic vasculitis worldwide, although it seems to be very rare in some areas, such as Latin America., Objectives: The objective of the study was to describe the clinical, laboratory, and treatment features in a Mexican Mestizo population with GCA., Methods: Retrospective data chart review (1989-2010)., Results: Twenty-two patients with GCA were identified, 18 women and 4 men. Mean age was 73 (SD, 7.9) years. Diagnosis was made at a mean of 67 (SD, 83.6) days from symptom onset. Most frequent presenting symptoms included headache (90%), constitutional symptoms (86%), and polymyalgia rheumatica (59%). Severe cranial ischemic complications were present in 32%. Amaurosis fugax and blindness were present in 36% and 27%, respectively. High erythrocyte sedimentation rate was present in 89% of patients. Rapid response to prednisone treatment was seen, but in 10 patients, relapse occurred, possibly related to fast tapering. Additional treatment was methotrexate (n = 8), azathioprine (n = 5), and cyclophosphamide (n = 3). Median follow-up was 242 (SD, 214) weeks., Conclusions: Giant cell arteritis is rarely recognized in Latin America. We report on characteristics of GCA in a population of Mexican Mestizos, as ours is the largest series to be reported from Latin America so far. When compared with other series, age at onset is similar, females are more affected, and although a good response to corticosteroid treatment was seen, a higher frequency of amaurosis fugax and blindness was observed, accounting for an unfavorable functional outcome in 6 (27%) of 22 patients.

Background: The diabetic heart exhibits increased left ventricular (LV) mass and reduced ventricular function. However, this relationship has not been studied in patients with aortic stenosis (AS), a disease process that causes LV hypertrophy and dysfunction through a distinct mechanism of pressure overload. The aim of this study was to determine how diabetes mellitus (DM) affects LV remodeling and function in patients with severe AS., Methods and Results: Echocardiography was performed on 114 patients with severe AS (mean aortic valve area [AVA], 0.6 cm(2)) and included measures of LV remodeling and function. Multivariable linear regression models investigated the independent effect of DM on these aspects of LV structure and function. Compared to patients without diabetes (n=60), those with diabetes (n=54) had increased LV mass and LV end-systolic and end-diastolic dimensions, and decreased LV ejection fraction (EF) and longitudinal systolic strain (all P<0.01). In multivariable analyses adjusting for age, sex, systolic blood pressure, AVA, body surface area, and coronary disease, DM was an independent predictor of increased LV mass (β=26 g, P=0.01), LV end-systolic dimension (β=0.5 cm, P=0.008), and LV end-diastolic dimension (β=0.3 cm, P=0.025). After also adjusting for LV mass, DM was associated with reduced longitudinal systolic strain (β=1.9%, P=0.023) and a trend toward reduced EF (β=-5%, P=0.09). Among patients with diabetes, insulin use (as a marker of disease severity) was associated with larger LV end-systolic dimension and worse LV function. LV mass was a strong predictor of reduced EF and systolic strain (both P<0.001)., Conclusions: DM has an additive adverse effect on hypertrophic remodeling (increased LV mass and larger cavity dimensions) and is associated with reduced systolic function in patients with AS beyond known factors of pressure overload.

Common causes of heart failure are associated with derangements in myocardial fuel utilization. Evidence is emerging that metabolic abnormalities may contribute to the development and progression of myocardial disease. The peroxisome proliferator-activated receptor (PPAR) family of nuclear receptor transcription factors has been shown to regulate cardiac fuel metabolism at the gene expression level. The three PPAR family members (alpha, beta/delta and gamma) are uniquely suited to serve as transducers of developmental, physiological, and dietary cues that influence cardiac fatty acid and glucose metabolism. This review describes murine PPAR loss- and gain-of-function models that have shed light on the roles of these receptors in regulating myocardial metabolic pathways and have defined key links to disease states including the hypertensive and diabetic heart.