Plasma IL-2 and Symptoms Response after Acute Gluten Exposure in Subjects With Celiac Disease or Nonceliac Gluten Sensitivity.

INTRODUCTION: Treated patients with celiac disease (CeD) and nonceliac gluten sensitivity (NCGS) report acute, transient, incompletely understood symptoms after suspected gluten exposure. To determine whether (i) blinded gluten exposure induces symptoms, (ii) subjects accurately identify gluten exposure, and (iii) serum interleukin-2 (IL-2) levels distinguish CeD from NCGS subjects after gluten exposure. METHODS: Sixty subjects (n = 20 treated, healed CeD; n = 20 treated NCGS; n = 20 controls) were block randomized to a single, double-blind sham (rice flour) or 3-g gluten challenge with 72-hours follow-up. Twelve serial questionnaires (100 mm visual analog scale; pain, bloating, nausea, and fatigue) and 10 serial plasma samples were collected. Mucosal permeability was assessed using both urinary lactulose-13C mannitol ratios and endoscopic mucosal impedance. RESULTS: Thirty-five of 40 (83%) subjects with CeD and NCGS reported symptoms with gluten (8 CeD, 9 NCGS) and sham (9 CeD, 9 NCGS) compared with 9 of 20 (45%) controls after gluten (n = 6) and sham (n = 3). There was no significant difference in symptoms among groups. Only 2 of 10 subjects with CeD and 4 of 10 NCGS identified gluten, whereas 8 of 10 subjects with CeD and 5 of 10 NCGS identified sham. A significant plasma IL-2 increase occurred only in subjects with CeD after gluten, peaking at 3 hours and normalizing within 24 hours postchallenge despite no significant intestinal permeability change from baseline. DISCUSSION: Symptoms do not reliably indicate gluten exposure in either subjects with CeD or NCGS. IL-2 production indicates a rapid-onset gluten-induced T-cell activation in CeD despite long-standing treatment. The effector site is unknown, given no increased intestinal permeability after gluten.

Atopic asthma as a potentially significant but unrecognized risk factor for Kawasaki disease in children.

OBJECTIVES: Childhood asthma is known to be associated with risks of both respiratory and non-respiratory infections. Little is known about the relationship between asthma and the risk of Kawasaki disease (KD). We assessed associations of asthma status and asthma phenotype (e.g. atopic asthma) with KD. METHODS: We performed a population-based retrospective case-control study, using KD cases between January 1, 1979, and December 31, 2016, and two matched controls per case. KD cases were defined by the American Heart Association diagnostic criteria. Asthma status prior to KD (or control) index dates was ascertained by the two asthma criteria, Predetermined Asthma Criteria (PAC) and Asthma Predictive Index (API, a surrogate phenotype of atopic asthma). We assessed whether 4 phenotypes (both PAC + and API+; PAC + only; API + only, and non-asthmatics) were associated with KD. RESULTS: There were 124 KD cases during the study period. The group having both PAC + and API + was significantly associated with the increased odds of KD, compared to non-asthmatics (odds ratio [OR] 4.3; 95% CI: 1.3 - 14.3). While asthma defined by PAC was not associated with KD, asthma defined by PAC positive with eosinophilia (≥4%) was significantly associated with the increased odds of KD (OR: 6.7; 95% CI: 1.6 - 28.6) compared to non-asthmatics. Asthma status defined by API was associated with KD (OR = 4.7; 95% CI: 1.4-15.1). CONCLUSIONS: Atopic asthma may be associated with increased odds of KD. Further prospective studies are needed to determine biological mechanisms underlying the association between atopic asthma and increased odds of KD.

Robotic vs. minimally invasive mitral valve repair: A 5-year comparison of surgical outcomes.

BACKGROUND: Minimally invasive mitral valve repair (MVr) is commonly performed. Data on the outcomes of robotic MVr versus nonrobotic minimally invasive MVr are lacking. We sought to compare the short-term and mid-term outcomes of robotic and nonrobotic MVr. METHODS: We reviewed all patients who underwent robotic MVr (n = 424) or nonrobotic MVr via right mini-thoracotomy (n = 86) at Mayo Clinic, Rochester, MN, from January 2015 to February 2020. Data on baseline and operative characteristics, operative and long-term outcomes were analyzed. Patients were matched 1:1 using propensity scores. RESULTS: Sixty-nine matched pairs were included in the study. The median age was 59 years (interquartile range [IQR]: 54-69) and 75% (n = 103) were male. Baseline characteristics were similar after matching. Robotic and nonrobotic MVr had similar operative characteristics, except that robotic had longer cross-clamp times (57 [48-67] vs. 47 [37-58] min, p < .001) and more P2 resections (83% vs. 68%, p = .05) compared to nonrobotic MVr. There was no difference in operative outcomes between groups. Hospital stay was shorter after robotic MVr (4 [3-4] vs. 4 [4-6] days, p = .003). After a median follow-up of 3.3 years (IQR, 2.1-4.5), there was no mortality in either group, and there was no difference in freedom from mitral valve reoperations between robotic and nonrobotic MVr (5 years: 97.1% vs. 95.7%, p = .63). Follow-up echocardiogram analysis predicted excellent freedom from recurrent moderate-or-severe mitral regurgitation at 3 years after robotic and nonrobotic MVr (90% vs. 92%, p = .18, respectively). CONCLUSIONS: Both short-term and mid-term outcomes of robotic and nonrobotic minimally invasive mitral repair surgery are comparable.

Community-Based Study of Celiac Disease Autoimmunity Progression in Adults.

BACKGROUND & AIMS: Celiac disease can develop at any age, but outcomes of adults with positive results from serologic tests for tissue transglutaminase antibodies (tTGA) without endoscopic determination of celiac disease (called celiac autoimmunity) have not been thoroughly evaluated. We investigated the proportion of adults with celiac autoimmunity at a community medical center and their progression to celiac disease. METHODS: We analyzed waste blood samples from a community clinic from 15,551 adults for tTGA and, if titer results were above 2 U/mL, for endomysial antibody. The blood samples had been collected at 2 time points (median interval, 8.8 years) from 2006 through 2017. We collected data from the clinic on diagnoses of celiac disease based on duodenal biopsy analysis. RESULTS: Of the serum samples collected at the first time point, 15,398 had negative results for tTGA, and 153 had positive results for tTGA (>4 U/mL). Based on medical records, 6 individuals received a diagnosis of celiac disease, for a cumulative incidence of celiac disease diagnosis of 0.06% (95% confidence interval, 0.01-0.11). Forty-nine (0.32%) individuals with a negative result from the first serologic test for tTGA had a positive result from the second test. Among the 153 adults who were tTGA positive at the first time point, 31 (20%) had a subsequent diagnosis of celiac disease, 81 (53%) remained positive for tTGA without a clinical diagnosis of celiac disease, and 41 (27%) had negative test results for tTGA at the second time point. Higher initial tTGA titers, female sex, and a history of hypothyroidism and autoimmune disease were associated with increased risks of subsequent diagnosis of celiac disease. Interestingly, adults whose first blood sample had a positive test result but second blood sample had a negative result for tTGA were older, had lower-than-average initial tTGA titer results, and had a higher mean body mass index than adults whose blood samples were positive for tTGA at both time points and adults later diagnosed with celiac disease. CONCLUSIONS: In an analysis of serum samples collected from a community clinic an average of 8.8 years apart, we found that fewer than 1% of adults with negative results from an initial test for tTGA have a positive result on a second test. Of adults with positive results from the test for tTGA, only 20% are later diagnosed with celiac disease; the remaining individuals maintain persistent increases in tTGA without diagnoses of celiac disease or have negative results from second tests.

Reproductive Characteristics and Pregnancy Outcomes in Hidden Celiac Disease Autoimmunity.

INTRODUCTION: Untreated symptomatic celiac disease (CD) adversely affects female reproduction; however, the effect of hidden CD autoimmunity is uncertain. METHODS: We identified women who were not previously diagnosed with CD and tested positive for tissue transglutaminase and endomysial antibodies between 2006 and 2011 in a community-based retrospective cohort study. We evaluated (i) the rate of adverse pregnancy outcomes and medical complications of pregnancy in successful singleton deliveries and (ii) reproductive characteristics in seropositive women without a clinical diagnosis of CD and age-matched seronegative women. RESULTS: Among 17,888 women whose serum samples were tested for CD autoimmunity, 215 seropositive and 415 seronegative women were included. We reviewed 231 and 509 live singleton deliveries of 117 seropositive and 250 seronegative mothers, respectively. Menarche and menopausal age, gravidity, parity, and age at first child were similar in seropositive and seronegative women. CD seropositivity was not associated with an increased risk of maternal pregnancy complications. Maternal seropositivity was associated with small for gestational age in boys (OR 3.77, 95% CI: 1.47-9.71; P = 0.006), but not in girls (OR 0.57, 95% CI: 0.15-2.17; P = 0.41). CD serum positivity was not associated with prematurity, small for gestational age (birth weight <10th percentile), or 5-minute Apgar score of less than 7. DISCUSSION: Although underpowered, the present study did not show any difference in reproductive characteristics or rates of adverse pregnancy outcomes in women with and without CD autoimmunity, except for birth weight in male offspring. Larger studies are needed to determine the effects of CD autoimmunity on female reproduction.

A diagnostic codes-based algorithm improves accuracy for identification of childhood asthma in archival data sets.

OBJECTIVE: While a single but truncated ICD code (493) had been widely used for identifying asthma in asthma care and research, it significantly under-identifies asthma. We aimed to develop and validate a diagnostic codes-based algorithm for identifying asthmatics using Predetermined Asthma Criteria (PAC) as the reference. METHODS: This is a retrospective cross-sectional study which utilized two different coding systems, the Hospital Adaptation of the International Classification of Diseases, Eighth Revision (H-ICDA) and the International Classification of Diseases, Ninth Revision (ICD-9). The algorithm was developed using two population-based asthma study cohorts, and validated in a validation cohort, a random sample of the 1976-2007 Olmsted County Birth Cohort. Performance of the diagnostic codes-based algorithm for ascertaining asthma status against manual chart review for PAC (gold standard) was assessed by determining both criterion and construct validity. RESULTS: Among eligible 267 subjects of the validation cohort, 50% were male, 70% white, and the median age at last follow-up was 17 (interquartile range, 8.7-24.4) years. Asthma prevalence by PAC through manual chart review was 34%. Sensitivity and specificity of the codes-based algorithm for identifying asthma were 82% and 98% respectively. Associations of asthma-related risk factors with asthma status ascertained by the code-based algorithm were similar to those by the manual review. CONCLUSIONS: The diagnostic codes-based algorithm for identifying asthmatics improves accuracy of identification of asthma and can be a useful tool for large scale studies in a setting without automated chart review capabilities.

Long-term Incidence of Kawasaki Disease in a North American Community: A Population-Based Study.

To assess the longitudinal incidence of Kawasaki disease (KD) within the well-defined predominantly White population of Olmsted County, MN. This retrospective cohort study used a population-based medical record linkage system and manual chart reviews to identify children with KD in Olmsted County, MN between January 1, 1979-December 31, 2016. Age- and gender-adjusted incidence rates were calculated using the 2010 U.S. White population. 124 children with KD were confirmed during the study period (median age 3.5, 61% male, 85% White, 9% Asian). The overall age- and gender-adjusted incidence rates for all ages and < 5 years old were 9.8 and 21.4 per 100,000 person-years, respectively. There was an overall increase in incidence up to 1994 followed by plateau, except among children between the ages of 1-5 years. There was also an overall increase in incidence among females compared to males. 24% of children had cardiac complications. While the overall incidence of KD in Olmsted County appears to be stable since 1994, the incidence of KD in subgroups of children 1-5 years old and females seems to have increased. Given the rising trends and one-quarter of children developing cardiac complications, further studies identifying factors driving these trends are warranted.

Management of Coronary Artery Aneurysms at the Time of Surgical Revascularization.

BACKGROUND: Coronary artery aneurysms (CAAs) represent a rare pathology occurring in 1.5%-5% of routine coronary angiograms. Limited data exist on the management of CAA at the time of cardiac surgery. MATERIALS AND METHODS: A single-institution retrospective review was performed on 53 patients who underwent cardiac surgery in the setting of atherosclerotic CAA between 1993 and 2015. Patients were stratified based on treatment strategy: exclusion and distal bypass (n = 26) versus revascularization alone (n = 27). Comparisons were made with respect to mortality, need for further/concomitant interventions, and long-term cardiac function including myocardial infarctions and congestive heart failure. RESULTS: A total of 53 patients underwent cardiac surgery in the setting of CAA disease. Management strategies included ligation and bypass in 26 patients and distal bypass only in 27 patients (with four of the patients in this group undergoing coronary stenting across the aneurysm). There were no significant differences in patient demographics between the two groups. No significant difference was found in either 30-d (P = 0.74) or long-term mortality when exclusion of the CAA was performed compared with revascularization alone (P = 0.20). More exclusion procedures were performed earlier in the experience (median surgical date 2000), whereas revascularization alone predominated later in the experience (median surgical date 2007; P ≤ 0.001). CONCLUSIONS: The practice of CAA exclusion, while still performed in selected cases, has largely been supplanted in patients undergoing revascularization. Exclusion does not appear to offer any advantage over isolated revascularization, supporting the current trends in managing this rare condition.

Effect of a Gluten-free Diet on Quality of Life in Patients With Nonclassical Versus Classical Presentations of Celiac Disease.

BACKGROUND: Celiac disease (CD) often presents with symptoms of diarrhea and malabsorption, termed classical CD. However, it can also present as nonclassical CD, which is commonly associated with nongastrointestinal symptoms. Studies suggest that nonclassical CD tends to have less severe symptoms than classical CD, which may affect both adherence to a gluten-free diet (GFD) and psychological stress. Therefore, we compared adherence to a GFD and psychological measures, including quality of life (QOL) and somatization, between patients with nonclassical and classical presentations of CD. METHODS: Patients at a tertiary care center with biopsy-proven CD, who completed a Talley Bowel Disease Questionnaire and the Short Form-36 at diagnosis and who had been on a GFD for at least 1 year, were included in this study. Patients were further surveyed to assess gastrointestinal symptoms, QOL, Somatization Symptom Checklist (SSC), and adherence to a GFD. Results were compared between patients with classical versus nonclassical CD presentation. RESULTS: Among 122 patients included in this study, 62 had classical CD and 60 had nonclassical CD. At diagnosis, health-related QOL was lower in the classical CD group than in the nonclassical CD group. After following a GFD, both groups had improved QOL after following a GFD, and body mass index significantly increased in both groups. Most subscales of QOL, SSC scores, and adherence to the GFD were similar between the groups, except the Short Form-36 Mental Component summary scores that were still lower in the classical CD (48.4 vs. 52.6 nonclassical CD group; P=0.03). CONCLUSIONS: Despite QOL at diagnosis being higher in those with nonclassical CD versus lower in those with classical CD, both groups had improved QOL and achieved a similar QOL after following a GFD.

Prevalence and Morbidity of Undiagnosed Celiac Disease From a Community-Based Study.

BACKGROUND & AIMS: Little is known about the prevalence and burden of undiagnosed celiac disease in individuals younger than age 50. We determined the prevalence and morbidity of undiagnosed celiac disease in individuals younger than age 50 in a community. METHODS: We tested sera from 31,255 residents of Olmsted County, Minnesota (<50 y), without a prior diagnosis of celiac disease assay using an assay for IgA against tissue transglutaminase; in subjects with positive test results, celiac disease was confirmed using an assay for endomysial IgA. We performed a nested case-control study to compare the proportion of comorbidities between undiagnosed cases of celiac disease and age- and sex-matched seronegative controls (1:2). Medical records were abstracted to identify potential comorbidities. RESULTS: We identified 338 of 30,425 adults with positive results from both serologic tests. Based on this finding, we estimated the prevalence of celiac disease to be 1.1% (95% confidence interval, 1.0%-1.2%); 8 of 830 children tested positive for IgA against tissue transglutaminase (1.0%; 95% confidence interval, 0.4%-1.9%). No typical symptoms or classic consequences of diagnosed celiac disease (diarrhea, anemia, or fracture) were associated with undiagnosed celiac disease. Undiagnosed celiac disease was associated with increased rates of hypothyroidism (odds ratio, 2.2; P < .01) and a lower than average cholesterol level (P = .03) and ferritin level (P = .01). During a median follow-up period of 6.3 years, the cumulative incidence of a subsequent diagnosis with celiac disease at 5 years after testing was 10.8% in persons with undiagnosed celiac disease vs 0.1% in seronegative persons (P < .01). Celiac disease status was not associated with overall survival. CONCLUSIONS: Based on serologic tests of a community population for celiac disease, we estimated the prevalence of undiagnosed celiac disease to be 1.1%. Undiagnosed celiac disease appeared to be clinically silent and remained undetected, but long-term outcomes have not been determined.

Gender-Based Differences in a Population-Based Cohort with Celiac Disease: More Alike than Unalike.

BACKGROUND: There is a gap in research focused on gender-based differences in non-referral populations with celiac disease. AIMS: The aim of this study was to estimate those differences in a unique population-based cohort of patients with celiac disease with respect to (1) presenting symptoms, (2) associated autoimmune disorders, and (3) survival. METHODS: Clinical data were systematically abstracted from the electronic medical record of a population-based incident cohort of patients with celiac disease. Logistic regression was used to assess the strength of the association of presenting symptoms and gender. Survival differences between genders were evaluated with Cox regression. RESULTS: We included 282 patients (females 65%, median age 39 years) diagnosed between 1990 and 2015. The female to male ratio was 1.85:1. Men and women presented similarly. Women were more likely to present with constipation (OR 2.33; 95% CI 1.06-5.12; p = 0.035). Anemia and abdominal distention or bloating were more frequently seen in women, but not on a statistically significant level. Overall autoimmune diseases were equally prevalent (31.6%) in males (30.2%) and females (32.2%) (p = 0.74). Hypothyroidism predominated in women. Age-adjusted survival was lower among men than women (HR 3.00; 95% CI 1.26-7.21, p = 0.014), but not more so than in the general population. Cancer was the most common cause of death, and there were two possible celiac disease-related deaths. CONCLUSIONS: This study showed that men and women are more alike than unalike when it comes to celiac disease presentation and prevalence of concurrent autoimmune disease.

Predictors of 1-year mortality after transcatheter aortic valve replacement.

OBJECTIVE: To identify variables predictive of increased mortality within 1 year of transcatheter aortic valve replacement (TAVR). METHODS: We retrospectively reviewed the records of 723 consecutive patients who received TAVR from November 2008 through April 2016. Patient and procedure-related characteristics were analyzed with logistic regression for an association with death within 1 year of TAVR. RESULTS: Patient mean age was 81 ± 9 years, male sex was present in 428 patients (59%), and STS predicted risk of mortality was 9.2 ± 6.2%. There were 107 deaths (15%) within 1 year of operation. Multivariable analysis identified increased risk of death with severe chronic lung disease (odds ratio [OR] 2.01; 95% confidence interval [CI] 1.23-3.29; P = 0.006), severe tricuspid valve regurgitation (OR 2.35; 95%CI 1.17-4.30; P = 0.017), vascular injury (OR 2.23; 95%CI 1.15-4.30; P = 0.017), and new-onset dialysis (OR 8.49; 95%CI 3.00-24.03; P < 0.001) (Area under the curve 0.687). When stratified by arterial access, there was increased risk of death following severe tricuspid valve regurgitation, vascular injury, or new-onset dialysis for transfemoral access and severe chronic lung disease or new-onset dialysis for alternative access. CONCLUSION: Patient characteristics and procedure-related complications are both significantly associated with increased risk of death within 1 year of TAVR. Patients with the baseline findings of severe chronic lung disease or severe tricuspid valve regurgitation may not experience mortality benefit from TAVR, and they should be assessed and counselled accordingly. Avoiding procedure-related complications is paramount to a good outcome. The findings have important implications for health care delivery services.

Increasing Incidence and Altered Presentation in a Population-based Study of Pediatric Celiac Disease in North America.

OBJECTIVES: Celiac disease (CD) is a common immune-mediated disorder that affects up to 1% of the general population. Recent reports suggest that the incidence of CD has reached a plateau in many countries. We aim to study the incidence and altered presentation of childhood CD in a well-defined population. METHODS: Using the Rochester Epidemiology Project, we retrospectively reviewed Mayo Clinic and Olmsted Medical Center medical records from January 1994 to December 2014. We identified all CD cases of patients ages 18 years or younger at the time of diagnosis. Incidence rates were calculated by adjusting for age, sex, and calendar year and standardizing to the 2010 US white population. RESULTS: We identified 100 patients with CD. Incidence of CD has increased from 8.1 per 100,000 person-years (2000-2002) to 21.5 per 100,000 person-years (2011-2014). There was an increase in CD prevalence in children from 2010 (0.10%) to 2014 (0.17%). Thirty-four patients (34%) presented with classical CD symptoms, 43 (43%) had nonclassical CD, and 23 (23%) were diagnosed by screening asymptomatic high-risk patients. Thirty-six patients (36%) had complete villous atrophy, 51 (51%) had partial atrophy, and 11 (11%) had increased intraepithelial lymphocytes. Two patients were diagnosed without biopsy. Most patients (67%) had a normal body mass index, 17% were overweight/obese, and only 9% were underweight. CONCLUSIONS: Both incidence and prevalence of CD have continued to increase in children during the past 15 years in Olmsted County, Minnesota. Clinical and pathologic presentations of CD are changing over time (more nonclassical and asymptomatic cases are emerging).

What accounts for the association between late preterm births and risk of asthma?

BACKGROUND: Although results of many studies have indicated an increased risk of asthma in former late preterm (LPT) infants, most of these studies did not fully address covariate imbalance. OBJECTIVE: To compare the cumulative frequency of asthma in a population-based cohort of former LPT infants to that of matched term infants in their early childhood, when accounting for covariate imbalance. METHODS: From a population-based birth cohort of children born 2002-2006 in Olmsted County, Minnesota, we assessed a random sample of LPT (34 to 36 6/7 weeks) and frequency-matched term (37 to 40 6/7 weeks) infants. The subjects were followed-up through 2010 or censored based on the last date of contact, with the asthma status based on predetermined criteria. The Kaplan-Meier method was used to estimate the cumulative incidence of asthma during the study period. Cox models were used to estimate the hazard ratio and 95% confidence interval for the risk of asthma, when adjusting for potential confounders. RESULTS: LPT infants (n = 282) had a higher cumulative frequency of asthma than did term infants (n = 297), 29.9 versus 19.5%, respectively; p = 0.01. After adjusting for covariates associated with the risk of asthma, an LPT birth was not associated with a risk of asthma, whereas maternal smoking during pregnancy was associated with a risk of asthma. CONCLUSION: LPT birth was not independently associated with a risk of asthma and other atopic conditions. Clinicians should make an effort to reduce exposure to smoking during pregnancy as a modifiable risk factor for asthma.

Hemodynamic Comparison of the On-X and Top Hat Mechanical Aortic Valve Prostheses.

BACKGROUND: There are limited data comparing hemodynamic valve function in mechanical aortic valve prostheses. This study compared the hemodynamic function of 2 commonly used mechanical aortic valve (AV) prostheses, the On-X (Artivion) and Top Hat (CarboMedics Inc) valves. METHODS: This study was a retrospective analysis of 512 patients who underwent AV replacement with the On-X (n = 252; 49%) or Top Hat (n = 260; 51%) mechanical valves between 2011 and 2019. Patients were matched on the basis of selected variables. Echocardiographic data were collected preoperatively and postoperatively over a median follow-up of 1.39 years. RESULTS: A total of 320 patients were matched, 160 patients in each group. Despite being matched for left ventricular outflow tract diameter, patients in the Top Hat group received a greater prevalence of smaller tissue annulus diameter valves (≤21 mm) (83% vs 38%; P < .001). Patients in the On-X group had longer aortic cross-clamp times (78 minutes vs 64 minutes; P < .001) during isolated aortic valve replacement. Discharge echocardiography showed no difference in the AV area index between both groups (1.00 cm2/m2 vs 1.02 cm2/m2; P = .377). During longer-term echocardiographic follow-up, the AV area index remained stable for both valves within their respective tissue annulus diameter groups (P = .060). CONCLUSIONS: There was no difference between the 2 valves with respect to the AV area index at discharge, and hemodynamic function was stable during longer-term follow-up. The longer aortic cross-clamp time observed in the On-X group may indicate increased complexity of implantation compared with the Top Hat group.

Incidence of Reoperation After Surgical Procedure for Left Ventricular Outflow Tract Obstruction in Children and Young Adults.

BACKGROUND: The common causes of subaortic left ventricular outflow tract obstruction (LVOTO) are hypertrophic cardiomyopathy (HCM) and membranous/tunnel subaortic stenosis (SAS). Reoperation after corrective surgery may be due to recurrent disease, associated congenital defects, or complications of the initial procedure. This study compares the late outcomes of young patients with HCM and SAS. METHODS: We studied clinical, echocardiographic, and operative data of patients ≤21 years of age at the time of surgery for LVOTO between August 1963 and August 2018. We stratified patients into HCM (n = 152) and congenital SAS (n = 63) groups and compared survival and cumulative incidence of reoperation. RESULTS: At initial repair, patients with HCM were older than patients with SAS (median [interquartile range] age, 15 [10-19] years vs 8 [5-13] years; P < .001), and patients with HCM were more symptomatic with dyspnea (P < .001), chest pain (P = .002), and presyncope/syncope (P = .005). Thirty-day mortality was 1.3% vs 0% for HCM and SAS groups. During a median follow-up of 13.1 years, survival was similar through the first 10 years; but during the second decade, patients with HCM had poorer survival (survival at 20 years, 80% vs 91% for patients with SAS; P = .007). Ten years after repair, reoperation for recurrent LVOTO was performed in 5% of patients with HCM vs 31% in those with SAS (P < .001). CONCLUSIONS: In this surgical cohort, patients with HCM were more symptomatic preoperatively than those with SAS. Late survival of patients with SAS was superior to that of patients with HCM despite a greater need for reoperation.

Partial Anomalous Pulmonary Venous Connection With Intact Atrial Septum: Early and Midterm Outcomes.

BACKGROUND: Partial anomalous pulmonary venous return with intact atrial septum warrants greater understanding and evaluation in the literature. METHODS: From January 1993 to December 2018, 293 patients with partial anomalous pulmonary venous return underwent surgical repair. Of these, 45 patients (15.3%) had an intact atrial septum. The median age was 36 years (interquartile range, 24-48). Direct reimplantation was used in 17 patients (38%), intracardiac baffling in 15 (33%), and caval division (Warden) technique in 13 (29%). Descriptive statistics were used to assess the data, and Kaplan-Meier analysis was used to assess survival. RESULTS: Anomalous veins were right-sided in 27 patients (60%), left-sided in 16 patients (36%), and bilateral in 2 patients (4%). The insertion sites were the superior vena cava in 23 patients (51%), innominate vein in 12 (27%), inferior vena cava in 6 (13%), coronary sinus in 2 (4%), right atrium in 1 patient (2%), and unknown in 1 patient (2%). Scimitar syndrome was noted in 8 patients (18%). There was no postoperative mortality or residual defects. Postoperative echocardiography excluded any obstruction of pulmonary or systemic veins. Postoperative complications included atrial fibrillation in 9 patients (20%) and pneumothorax requiring chest tube in 5 patients (11%). Survival at 1, 5, and 10 years was 100%, 95%, and 95%, respectively. Two patients underwent pulmonary vein dilation, one at 3 years and the other at 7 years. CONCLUSIONS: Surgical repair of partial anomalous pulmonary venous connection with intact atrial septum can be performed with excellent early and midterm outcomes. The overall incidence of midterm systemic or pulmonary vein stenosis is low.

Optimal Management of Mitral Regurgitation Due to Ruptured Mitral Chordae Tendineae in Patients With Hypertrophic Cardiomyopathy.

There is continued controversy regarding surgical management of patients with hypertrophic cardiomyopathy (HCM) and intrinsic mitral valve disease; some clinicians favor prosthetic replacement as this corrects left ventricular outflow tract (LVOT) obstruction and valve leakage. In this study, we investigated the management and late outcome of operation for mitral regurgitation (MR) due to ruptured chordae tendineae in patients with HCM. We analyzed 49 consecutive patients with HCM and MR due to ruptured mitral valve chordae. Echocardiograms and operative reports were reviewed to classify valve anatomy and surgical methods. Information on late outcomes was obtained from electronic medical records and follow-up surveys. The mean age of the 36 men and 13 women was 61.9 ± 12.5 years; significant resting or provoked LVOT obstruction was present at the time of surgery in 46 patients. During the index operation, mitral valve repair was performed in 45 patients, and prosthetic replacement was necessary for 4 patients. Concomitant septal myectomy was performed in 46 patients. There were no hospital deaths or deaths within 30 days of operation. Five and ten-year survival estimates (Kaplan-Meier) were 92% and 71%. During follow-up at a median of 7.9 years, 3 patients underwent reoperation for MV replacement, 5 days, 3 years, and 14 years following valve repair. Ruptured mitral chordae may result in severe mitral valve regurgitation in patients with hypertrophic cardiomyopathy. Valvuloplasty at the time of septal myectomy is safe with an acceptably low rate of recurrent MR requiring prosthetic replacement.

Whole Body Retrograde Perfusion Combined With Central Aortic Perfusion Strategy in the Repair of Distal Arch Pathology Through a Lateral Thoracotomy.

OBJECTIVE: To compare the results of the hypothermic circulatory arrest (HCA) + retrograde whole-body perfusion (RBP) technique with those of deep hypothermic circulatory arrest (DHCA-only) approach. METHODS: Limited data are available on cerebral protection techniques when distal arch repairs are performed through a lateral thoracotomy. In 2012, the RBP technique was introduced as adjunct to HCA during open distal arch repair via thoracotomy. We reviewed the results of the HCA + RBP technique compared with those of the DHCA-only approach. From February 2000 to November 2019, 189 patients (median age, 59 [IQR, 46 to 71] years; 30.7% female) underwent open distal arch repair via lateral thoracotomy to treat aortic aneurysms. The DHCA technique was used in 117 patients (62%, median age 53 [IQR, 41 to 60] years), whereas HCA + RBP was used in 72 patients (38%, median age 65 [IQR, 51 to 74] years). In HCA + RBP patients, cardiopulmonary bypass was interrupted when systemic cooling achieved isoelectric electroencephalogram; once the distal arch had been opened, RBP was then initiated via the venous cannula (flow of 700 to 1000 mL/min, central venous pressure <15 to 20 mm Hg). RESULTS: The stroke rate was significantly lower in the HCA + RBP group (3%, n=2) compared with the DHCA-only (12%, n=14) (P=.031), despite longer circulatory arrest times in HCA + RBP compared with the DHCA-only (31 [IQR, 25 to 40] minutes vs 22 [IQR, 17 to 30] minutes, respectively; P<.001). Operative mortality for patients undergoing HCA + RBP was 6.7% (n=4), whereas for those undergoing DHCA-only it was 10.4% (n=12) (P=.410). The 1-, 3-, and 5-year age-adjusted survival rates for the DHCA group are 86%, 81%, and 75%, respectively. The 1-, 3-, and 5-year age-adjusted survival rates for the HCA + RBP group are 88%, 88%, and 76%, respectively. CONCLUSION: The addition of RBP to HCA in the treatment of distal open arch repair via a lateral thoracotomy is safe and provides excellent neurological protection.

The Role of Individual-Level Socioeconomic Status on Nursing Home Placement Accounting for Neighborhood Characteristics.

OBJECTIVE: Independent living is desirable for many older adults. Although several factors such as physical and cognitive functions are important predictors for nursing home placement (NHP), it is also reported that socioeconomic status (SES) affects the risk of NHP. In this study, we aimed to examine whether an individual-level measure of SES is associated with the risk of NHP after accounting for neighborhood characteristics. DESIGN: A population-based study (Olmsted County, Minnesota, USA). SETTING AND PARTICIPANTS: Older adults (age 65+ years) with no prior history of NHP. METHODS: Electronic health records (EHR) were used to identify individuals with any NHP between April 1, 2012 (baseline date) and April 30, 2019. Association between the (HOUsing-based index of SocioEconomic Status (HOUSES) index, an individual-level SES measure based on housing characteristics of current residence, and risk of NHP was tested using random effects Cox proportional hazard model adjusting for area deprivation index (ADI), an aggregated SES measure that captures neighborhood characteristics, and other pertinent confounders such as age and chronic disease burden. RESULTS: Among 15,031 older adults, 3341 (22.2%) experienced NHP during follow-up period (median: 7.1 years). At baseline date, median age was 73 years old with 55% female persons, 91% non-Hispanic Whites, and median number of chronic conditions of 4. Accounting for pertinent confounders, the HOUSES index was strongly associated with risk of NHP (hazard ratio 1.89; 95% confidence interval 1.66‒2.15 for comparing the lowest vs highest quartiles), which was not influenced by further accounting for ADI. CONCLUSIONS AND IMPLICATIONS: This study demonstrates that an individual-level SES measure capturing current individual-specific socioeconomic circumstances plays a significant role for predicting NHP independent of neighborhood characteristics where they reside. This study suggests that older adults who are at higher risk of NHP can be identified by utilizing the HOUSES index and potential individual-level intervention strategies can be applied to reduce the risk for those with higher risk.