Unlocking Diversity in Cardiovascular Clinical Research: Lessons from the Screening for Cardiac Amyloidosis With Nuclear Imaging in a Minority Populations Study.

BACKGROUND: The Screening for Cardiac Amyloidosis with Nuclear Imaging in Minority Populations study seeks to determine the prevalence of transthyretin cardiac amyloidosis (ATTR-CA) among older Black or Caribbean Hispanic individuals with heart failure and an increased wall thickness. We noticed varied recruitment percentages across the recruiting sites and sought to determine the factors associated with greater percentage enrollment of eligible participants. METHODS: The percentage of enrolled to eligible participants was calculated across study sites. Baseline demographic and clinical characteristics, health literacy, trust in providers, perceived discrimination, area deprivation index (ADI) and English proficiency were compared by site using Kruskal-Wallis's test or one-way ANOVA for continuous variables and the Chi-Square test or Fisher's exact test for categorical variables. Wilcoxon rank sum and Chi-Square tests, with multiple comparisons correction using the false discovery rate (FDR) method, were used as post-hoc analysis when results were statistically significant. RESULTS: Among the four recruiting sites, Boston Medical Center, Columbia University Irving Medical Center, Harlem Hospital and Yale University, which employed different recruitment approaches, the percentage of participants enrolled among eligible participants differed, with the highest rate at Harlem Hospital (n=149 of 310, 48%), followed by Yale University (n=27 of 67, 40%), Boston University (n=247 of 655, 38%), and Columbia University (n=137of 442, 32%), p <0.01. Direct recruitment by the primary cardiovascular care team providing clinical care was associated with higher percent enrolled across sites as were higher education levels and English proficiency. Enrollment differences across sites were not associated with the number of chronic diseases, physician trust, perceived discrimination, or health literacy. CONCLUSIONS: Recruitment of eligible under-represented minorities (URMs) in SCAN-MP was associated with approaches employed in recruitment, including direct initial contact by the primary cardiovascular care team providing the potential participant's clinical care. Such data may help improve approaches to more successful recruitment of URMs in clinical research.

Understanding family dynamics of obesity: Do parents and children lose and gain weight together?

BACKGROUND: Obesity is prevalent among children and adults. Yet, understanding the relationship between parent and child weight trajectories is limited. OBJECTIVE: (1) Examine the association between parent/child undesirable body mass index (BMI) category change. (2) Assess whether parental BMI category predicts child modified BMI z-score (mBMIz) annual change. METHODS: We conducted a cross-sectional study of weight trajectories of 3821 parent-child dyads between March 2020 and December 2021 within the NYC Health + Hospitals system. Undesirability of child and parental BMI category change and the magnitude of mBMIz change by parental BMI are analysed. RESULTS: Of 3821 children (mean [SD] baseline age, 9.84 [3.51]), 1889 were female. Of the 3220 parents (mean [SD] baseline age, 39.9 [8.51]), 2988 were female. Most children (53.52%) and parents (81.94%) presented with overweight and obesity. Undesirable BMI change in children was associated with concordant change in parents (adjusted OR: 1.7, 95% CI [1.45, 2.01], adjusted p < 0.001). Children of parents with obesity (adjusted coef: 0.076, 95% CI [0.004, 0.147], p < 0.038) and severe obesity (adjusted coef: 0.1317, 95% CI [0.024, 0.239], adjusted p < 0.016) demonstrated greater change in mBMIz than those of parents with normal weight or underweight. CONCLUSION: Parents and children have concordant weight trajectories, and public health interventions targeting both populations are essential.

Clinical Penetrance of the Transthyretin V122I Variant in Older Black Patients With Heart Failure: The SCAN-MP (Screening for Cardiac Amyloidosis With Nuclear Imaging in Minority Populations) Study.

Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed cause of heart failure (HF) among patients ≥60 years of age. Although the V122I (valine to isoleucine substitution at position 122 of the transthyretin protein) variant associated with hereditary ATTR-CM is present in 3.4% of self-identified Black individuals in the United States (or 1.5 million people), the phenotypic penetrance is not known. Methods and Results The SCAN-MP (Screening for Cardiac Amyloidosis With Nuclear Imaging in Minority Populations) study is a currently accruing prospective multisite study designed to determine the prevalence of ATTR-CM using technetium-99m-pyrophosphate imaging in older (≥60 years of age) self-identified Black and Hispanic individuals with HF. Calculations of the penetrance and prevalence of the V122I allele, along with analyses of functional, biochemical, and echocardiographic parameters, were performed for the first 278 Black participants in SCAN-MP. The prevalence of ATTR-CM was 6.8% (95% CI, 4.2-10.5; n=19 cases), of whom 63% were ATTR wild-type. The prevalence of V122I was 6.5% (n=18 carriers), of whom 7 had ATTR-CM, yielding a phenotypic penetrance of 39% (95% CI, 17-64). V122I carriers with ATTR-CM evidenced more advanced HF than carriers without ATTR-CM. Prealbumin concentration was lowest among V122I carriers with ATTR-CM (12.9 mg/dL) versus carriers without ATTR-CM (21.0 mg/dL) and HF controls (25.0 mg/dL, P<0.0001). Conclusions Among older Black individuals with HF and increased left ventricular wall thickness, of those with ATTR-CM, 63% had wild-type, and of those with V122I, the phenotypic penetrance of ATTR-CM was 39% (95% CI, 17-64), suggesting that genotype alone is insufficient for diagnosis. Prealbumin concentration may be useful to identify V122I carriers with ATTR-CM. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03812172.

Physical Performance in Black and Hispanic Outpatients With Heart Failure: The SCAN-MP Study.

Background: Deficits of physical function are associated with poor quality of life and adverse health outcomes, but data informing the association of these assessments among Black and Hispanic outpatients with heart failure (HF) are limited. Methods: The multicentre, prospective Screening for Cardiac Amyloidosis With Nuclear Imaging for Minority Populations (SCAN-MP) study identified Black and Hispanic subjects with stable HF, collected baseline characteristics, and took measures using the short physical performance battery. Subjects completed a Kansas City Cardiomyopathy Questionnaire (KCCQ), and the clinical outcomes of HF hospitalization and death were ascertained by telephone and review of the electronic health record. Results: Of 320 participants, 227 (70.9%) had physical deficits, defined by a battery score of ≤ 9. Patients with severe physical deficits reported overall lower KCCQ scores compared to those with no deficits (KCCQ score of 57.0 vs 72.4, P < 0.001). Physical limitation was significantly associated with risk of HF hospitalization, after adjustments for age, sex, and New York Heart Association class (severe physical deficit hazard ratio, 3.61; 95% confidence interval [CI], 1.19-10.93; P = 0.024; mild physical deficit hazard ratio, 2.59; 95% CI, 0.86-7.75; P = 0.090). Conclusions: Reduced physical performance is highly prevalent among Black and Hispanic outpatients with HF, and it is associated with overall KCCQ score, as well as an increased risk for HF hospitalization.

Contexte: La limitation physique est associée à une détérioration de la qualité de vie et à une aggravation de l'état de santé, mais il y a peu de données sur la corrélation entre ces paramètres chez les patients externes noirs et hispaniques atteints d'insuffisance cardiaque. Méthodologie: Dans l'étude multicentrique et prospective de dépistage de l'amylose cardiaque chez les populations minoritaires (SCAN-MP), on a ciblé des sujets noirs et hispaniques atteints d'insuffisance cardiaque stable, recueilli les caractéristiques initiales et mesuré les capacités au moyen du court test d'évaluation de la performance physique (short physical performance battery [SPPB]). Les sujets ont répondu au questionnaire de cardiomyopathie de Kansas City (KCCQ), et les critères cliniques des hospitalisations et des décès liés à l'insuffisance cardiaque ont été évalués par téléphone et par examen des dossiers de santé électroniques. Résultats: Des 320 participants, 227 (70,9 %) avaient des déficits physiques, définis par un score au SPPB de 9 ou moins. Les patients ayant des déficits physiques graves ont obtenu des scores globaux au KCCQ inférieurs aux patients sans déficit (score KCCQ de 57,0 contre 72,4; p < 0,001). La limitation physique est fortement associée au risque d'hospitalisation liée à l'insuffisance cardiaque, après ajustement pour tenir compte de l'âge, du sexe et de la classe d'insuffisance cardiaque de la New York Heart Association (rapport des risques instantanés [RRI] du déficit physique grave : 3,61; intervalle de confiance [IC] à 95 % de 1,19 à 10,93; p = 0,024; RRI du déficit physique léger : 2,59; IC à 95 % de 0,86 à 7,75; p = 0,090). Conclusions: La diminution de la performance physique est très fréquente chez les patients externes noirs et hispaniques atteints d'insuffisance cardiaque, et elle est corrélée au score global au KCCQ ainsi qu'à une augmentation du risque d'hospitalisation liée à l'insuffisance cardiaque.

Design and Rationale the SCAN-MP (Screening for Cardiac Amyloidosis With Nuclear Imaging in Minority Populations) Study.

Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is an important cause of heart failure in older individuals. Misfolding and deposition of transthyretin or prealbumin protein causes ATTR-CM in the context of a normal (wild-type) or variant TTR sequence. Variant ATTR-CM is most commonly caused by the substitution of valine for isoleucine at position 122 in transthyretin (Val122Ile or pV142I, almost exclusively observed in individuals of West African ancestry), demonstrated in 3.4% of self-identified Black individuals in the United States with an estimated 1.5 million carriers. Despite the large number of known pV142I carriers, the proportion of older Black patients with heart failure attributable to ATTR-CM remains unknown. Methods To address this knowledge gap, the SCAN-MP (Screening for Cardiac Amyloidosis with Nuclear Imaging in Minority Populations) study was funded by the National Institutes of Health/National Heart, Lung, and Blood Institute (R01HL139671) to enroll a targeted population of self-identified, community-dwelling Black or Caribbean Hispanic patients (many of whom are of West African ancestry) >60 years of age with heart failure and identify ATTR-CM by noninvasive nuclear imaging. The principal objective of SCAN-MP is to determine the prevalence of ATTR-CM in this population. Secondary objectives will explore TTR genotype, demographics, progression of variant versus wild-type ATTR-CM, and biochemical mechanisms of transthyretin amyloid fibril formation. Conclusions The SCAN-MP study is the largest, prospective study of cardiac amyloidosis in Black and Hispanic individuals. Both wild-type and variant ATTR-CM are now treatable with the US Food and Drug-approved drug tafamidis. The insights gained from SCAN-MP are likely to improve those at risk for or afflicted with ATTR-CM. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03812172.

Clinical and social determinants of health features of SARS-CoV-2 infection among Black and Caribbean Hispanic patients with heart failure: The SCAN-MP Study.

Patients with heart failure (HF) often have multiple chronic conditions and are at increased risk for severe disease and mortality when infected by SARS-CoV-2, the virus that causes COVID-19. Furthermore, disparities in outcomes with COVID-19 have been associated with both racial/ethnic identity but also social determinants of health. Among older, urban-dwelling, minority patients with HF, we sought to characterize medical and non-medical factors associated with SARS-CoV-2 infection. Patients with HF living in Boston and New York City over 60 years of age participating in the Screening for Cardiac Amyloidosis with Nuclear Imaging (SCAN-MP) study between 12/1/2019 and 10/15/2021 (n = 180) were tested for nucleocapsid antibodies to SARS-CoV-2 and queried for symptomatic infection with PCR verification. Baseline testing included the Kansas City Cardiomyopathy Questionnaire (KCCQ), assessment of health literacy, biochemical, functional capacity, echocardiography, and a novel survey tool that determined living conditions, perceived risk of infection, and attitudes towards COVID-19 mitigation. The association of infection with prevalent socio-economic conditions was assessed by the area deprivation index (ADI). There were 50 overall cases of SARS-CoV-2 infection (28%) including 40 demonstrating antibodies to SARS-CoV-2 (indicative of prior infection) and 10 positive PCR tests. There was no overlap between these groups. The first documented case from New York City indicated infection prior to January 17, 2020. Among active smokers, none tested positive for prior SARS-CoV-2 infection (0 (0%) vs. 20 (15%), p = 0.004) vs. non-smokers. Cases were more likely to be taking ACE-inhibitors/ARBs compared to non-cases (78% vs 62%, p = 0.04). Over a mean follow-up of 9.6 months, there were 6 total deaths (3.3%) all unrelated to COVID-19. Death and hospitalizations (n = 84) were not associated with incident (PCR tested) or prior (antibody) SARS-CoV-2 infection. There was no difference in age, co-morbidities, living conditions, attitudes toward mitigation, health literacy, or ADI between those with and without infection. SARS-CoV-2 infection was common among older, minority patients with HF living in New York City and Boston, with evidence of infection documented in early January 2020. Health literacy and ADI were not associated with infection, and there was no increased mortality or hospitalizations among those infected with SARS-CoV-2.

Diagnostic performance characteristics of planar quantitative and semi-quantitative parameters of Tc99m pyrophosphate (PYP) imaging for diagnosis of transthyretin (ATTR) cardiac amyloidosis: the SCAN-MP study.

BACKGROUND: The optimal heart-to-contralateral chest (H/CL) ratio threshold for non-invasive diagnosis of transthyretin cardiac amyloidosis (ATTR-CA) using Tc99m pyrophosphate (PYP) imaging in a population with low pretest probability is not known. METHODS: Using myocardial PYP retention by SPECT as the reference standard, we evaluated the diagnostic performance of different semi-quantitative and quantitative (H/CL chest ratio) planar parameters obtained from 3-hour PYP imaging in a prospectively recruited cohort of minority older adults with heart failure and increased LV wall thickness. RESULTS: Of 229 patients, 14 were found to have ATTR-CA (6.1%). No PYP uptake (grade 0) was observed in 77% of scans, all grade 3 scans were ATTR-CA, and only 4 of 11 (36%) grade 2 scans were ATTR-CA. An H/CL threshold of ≥ 1.4 maximized specificity (99%) and positive predictive value (93%) but resulted in decreased sensitivity (93%), compared to the ≥ 1.3 threshold which had 100% sensitivity. CONCLUSION: Among patients with a low pretest likelihood of ATTR-CA, planar interpretation, while useful to exclude disease, must be interpreted with caution. H/CL ratio threshold of ≥ 1.3 resulted in clinically important misclassifications. These data suggest that quantitative planar imaging thresholds may not be appropriate to apply in low pretest likelihood populations being evaluated for ATTR-CA.

Relation of Body Mass Index to Transthyretin Cardiac Amyloidosis Particularly in Black and Hispanic Patients (from the SCAN-MP Study).

Heart failure with preserved ejection fraction is a heterogeneous clinical syndrome that includes distinct subtypes with different pathophysiologies, genetics, and treatment. Distinguishing heart failure with preserved ejection fraction caused by transthyretin cardiac amyloidosis (ATTR-CA) is critical given its specific treatment. We analyzed a single-center retrospective cohort to determine the association of body mass index (BMI) with a composite of either ATTR-CA or the valine-to-isoleucine substitution (Val122Ile) variant genotype (ATTR-CA+Val122Ile). These BMI differences were prospectively evaluated in the multicenter Screening for Cardiac Amyloidosis using nuclear imaging for Minority Populations (SCAN-MP) study of Black and Hispanic patients with heart failure. The association of BMI with ATTR-CA+Val122Ile was compared by Wilcoxon rank sum analysis and combined with age, gender, and maximum left ventricle wall thickness in multivariable logistic regression. In the retrospective analysis (n = 469), ATTR-CA+Val122Ile was identified in n = 198 (40%), who had a lower median BMI (25.8 kg/m2, interquartile range [IQR] 23.4 to 28.9) than other patients (27.1 kg/m2, IQR 23.9 to 32.0) (p <0.001). In multivariable logistic regression, BMI <30 kg/m2 (odds ratio 2.6, 95% confidence interval 1.5 to 4.5) remained independently associated with ATTR-CA+Val122Ile with a greater association in Black and Hispanic patients (odds ratio 5.8, 95% confidence interval 1.7 to 19.6). In SCAN-MP (n = 201), 17 (8%) had either ATTR-CA (n = 10) or were Val122Ile carriers (n = 7) with negative pyrophosphate scans. BMI was lower (25.4 kg/m2 [IQR 24.3 to 28.2]) in ATTR-CA+Val122Ile patients than in non-amyloid patients (32.7 kg/m2 [28.3 to 38.6]) (p <0.001), a finding that persisted in multivariable analysis (p = 0.002). In conclusion, lower BMI is associated with ATTR-CA+Val122Ile in heart failure with increased left ventricle wall thickness, particularly in Black and Hispanic patients, and may aid in the identification of those benefiting from ATTR-CA evaluation.

Coronavirus Disease 2019 and Hospital Readmissions: Patient Characteristics and Socioeconomic Factors Associated With Readmissions in an Urban Safety-Net Hospital System.

BACKGROUND: It is not yet known whether socioeconomic factors (ie, social determinants of health) are associated with readmission following hospitalization for coronavirus disease 2019 (COVID-19). METHODS: We conducted a retrospective cohort study of 6191 adult patients hospitalized with COVID-19 in a large New York City safety-net hospital system between March 1 and June 1, 2020. Associations between 30-day readmission and selected demographic characteristics, socioeconomic factors, prior health care utilization, and relevant features of the index hospitalization were analyzed using a multivariable generalized estimating equation model. RESULTS: The readmission rate was 7.3%, with a median of 7 days between discharge and readmission. The following were risk factors for readmission: age 65 and older [adjusted odds ratio (aOR): 1.32; 95% confidence interval (CI): 1.13-1.55], history of homelessness, (aOR: 2.03 95% CI: 1.49-2.77), baseline coronary artery disease (aOR: 1.68; 95% CI: 1.34-2.10), congestive heart failure (aOR: 1.34; 95% CI: 1.20-1.49), cancer (aOR: 1.68; 95% CI: 1.26-2.24), chronic kidney disease (aOR: 1.74; 95% CI: 1.46-2.07). Patients' sex, race/ethnicity, insurance, and presence of obesity were not associated with increased odds of readmission. A longer length of stay (aOR: 0.98; 95% CI: 0.97-1.00) and use of noninvasive supplemental oxygen (aOR: 0.68; 95% CI: 0.56-0.83) was associated with lower odds of readmission. Upon readmission, 18.4% of patients required intensive care, and 13.7% expired. CONCLUSION: We have found some factors associated with increased odds of readmission among patients hospitalized with COVID-19. Awareness of these risk factors, including patients' social determinants of health, may ultimately help to reduce readmission rates.

Effect of parenteral nutrition in oxygen escalation/de-escalation in SARS-CoV-2 infected patients who are pre-intubation: A multicenter, observational study.

BACKGROUND & AIMS: SARS-CoV-2 infection includes a variety of gastrointestinal manifestations along with the usual viral symptoms of malaise and myalgias. The objective of this study was to determine if intravenous parenteral nutrition (PN) affected the risk of intubation in SARS-CoV-2 patients who were dependent on non-invasive ventilation. METHODS: Retrospective, multicenter case-control study which analyzed oxygen requirements for 1974 adults with SARS-CoV-2, who were admitted to the local public hospital system between March 1 and May 17, 2020. Relevant baseline biomarkers were studied over 5 days. The main outcome was an escalation or de-escalation of oxygen requirements relative to the exposure of PN. RESULTS: 111 patients received PN while on non-invasive ventilation. Patients who received PN had a significantly lower odds (p < 0.001) of oxygen escalation in comparison to their control group counterparts (OR = 0.804, 95% CI 0.720, 0.899) when matched for age, body mass index, Charlson comorbidity index, and gender. CONCLUSION: Initiating PN in the setting of non-invasive ventilation of SARS-CoV-2 infected patients was significantly associated with a lower odds of oxygen escalation. PN does not independently exacerbate oxygen requirements in SARS-CoV-2 infected pre-intubated patients.

A curious case of Lyme carditis in an urban hospital.

Lyme carditis (LC), a manifestation of early disseminated Lyme disease, most commonly presents with cardiac conduction abnormalities. It is a transient condition with good prognosis but in extremely rare cases may be life-threatening. We describe a 42-year-old man who presented with progressively worsening generalized weakness, presyncope and dyspnea on exertion for 2 weeks after sustaining a tick bite. He subsequently developed a 'bull's eye rash' on his flank 2 days before his presentation. He was found to have symptomatic third-degree AV conduction blockade with a ventricular escape rhythm resulting in a brief cardiac arrest. Intravenous (IV) ceftriaxone was commenced empirically and a temporary transvenous pacemaker was placed. In a few days he showed dramatic, rapid improvement; the pacemaker was removed, and the patient was discharged on oral doxycycline to complete a 24-day course. This case is unique due to its occurrence in an urban hospital where such cases are uncommon. Cardiac arrest, although brief in this case, is a rare occurrence. Lyme carditis was a surprise diagnosis in our hospital due to the patient's geographical dislocation during the COVID-19 pandemic.

Thrombus in Transit on Point-of-Care Ultrasound in COVID-19 Pneumonia: A Cause of Refractory Hypoxia Requiring Systemic Thrombolysis.

Coronavirus disease 2019 (COVID-19) is predominantly a pulmonary disease due to infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with underlying systemic involvement associated with coagulopathy. The reported number of events of venous thromboembolism and refractory hypoxia remains high despite being maintained on prophylactic or therapeutic doses of anticoagulation in patients with a high clinical indication, which has shown a reduction in mortality otherwise. This report is of a case of severe COVID-19 pneumonia in a 37-year-old Hispanic man who developed coagulopathy with left popliteal vein thrombosis and subsequently a right ventricle thrombus in transit diagnosed by point-of-care ultrasound requiring systemic thrombolysis. Although patients with severe COVID-19 pneumonia are routinely given therapeutic anticoagulants, this case has shown that monitoring acute thrombotic events, D-dimer levels, and the presence of refractory hypoxia may indicate a thrombotic event that requires further intervention. This report has demonstrated the value of point-of-care ultrasound in the diagnosis of thromboembolism and venous thrombosis in a patient with severe COVID-19 pneumonia.

Regadenoson administration and QT interval prolongation during pharmacological radionuclide myocardial perfusion imaging.

The objective of our study is to assess change in QTc interval with Regadenoson administration during myocardial perfusion imaging (MPI). We conducted a retrospective, observational analysis of 1497 consecutive patients who underwent pharmacological radionuclide MPI. On multivariate logistic regression analyses, there was no statistical significance of QTc prolongation when adjusted for ischemia/fixed perfusion defect on MPI and QT prolonging medications being taken prior to stress testing. However, a positive stress ECG after Regadenoson injection had a statistical significance (p value 0.0004). Regadenoson is a safe drug for use in MPI with little, if any, side effects of major clinical significance.

Giant Left Ventricular Pseudoaneurysm and Myocardial Dissection as a Complication of Multiple Ventricular Tachycardia Ablations in a Patient with Cardiac Sarcoidosis.

Late development of left ventricular (LV) pseudoaneurysms after ventricular tachycardia (VT) catheter ablation is a rare phenomenon, and very few cases have been reported in the medical literature. We describe the case of a giant LV pseudoaneurysm as a late complication of multiple epicardial and endocardial VT ablations in a female in her 50s with known cardiac sarcoidosis.

Association of hyponatremia and outcomes in pulmonary hypertension.

BACKGROUND: Hyponatremia is known to be an important marker and prognosticator in left-sided heart failure. However, less is known about the significance of hyponatremia in pulmonary hypertension, particularly in the absence of left ventricular dysfunction. METHODS AND RESULTS: We identified 635 patients with pulmonary hypertension and preserved ejection fraction who were normonatremic (n = 493) or hyponatremic (n = 142). End points were mortality and readmission at 1 year. Overall, 27% of all of the patients died within 1 year. Hyponatremia was significantly associated with an increased rate of 1-year mortality (hazard ratio [HR] 1.82, 95% confidence interval [CI] 1.27-2.61; P = .001) and trended toward an association with the composite of mortality and readmission (HR 1.25, 95% CI 0.97-1.62; P = .08). Additionally, the severity of hyponatremia was directly related to the rate of 1-year mortality (P < .001). CONCLUSIONS: Hyponatremia is an indicator of poor prognosis in patients with echocardiographic evidence of pulmonary hypertension.

Not all obstructive cardiac lesions are created equal: double-chamber right ventricle in pregnancy.

Double-chambered right ventricle (DCRV) is a rare form of right ventricular outflow tract (RVOT) obstruction accounting for approximately 1% of patients with congenital heart disease. It consists of an anomalous muscle bundle that divides the right ventricle usually between the sinus (inlet) and the infundibulum (outlet). This division creates a proximal chamber with high pressure and a distal chamber with low pressure. The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. The diagnosis of DCRV should be considered in the young patient with an elevated right ventricular systolic pressure and intracavitary gradient. Echocardiography and cardiac MRI are the principal diagnostic tools for the assessment of DCRV. This entity is often misdiagnosed as pulmonary hypertension in the young patient, and can often go overlooked and untreated for many years. Definitive therapy involves surgical resection of the muscle bundle. This can often be curative and if done in a timely fashion, may prevent right ventricular remodeling. We describe the unique diagnostic dilemma, the course and management of a young adult with DCRV during pregnancy.

Ablation of peri-"mitral" flutter in a patient with congenitally corrected transposition of great vessels and situs inversus.

This case reports the successful ablation of a peri-"mitral" flutter in a patient with congenitally corrected transposition of the great vessels and situs inversus using an anterior mitral line.

Polymorphisms in serine hydroxymethyltransferase 1 and methylenetetrahydrofolate reductase interact to increase cardiovascular disease risk in humans.

The enzymes serine hydroxymethyltransferase 1 (gene name SHMT1) and methylenetetrahydrofolate reductase (gene name MTHFR) regulate key reactions in folate-mediated one-carbon metabolism. Common genetic variants with the potential to influence disease risk exist in both genes. A prior report from the Normative Aging Study indicated no association of the SHMT1 rs1979277 SNP with cardiovascular disease (CVD), but a strong gene-gene interaction was detected with MTHFR rs1801133. We investigated the effect of the SHMT1 rs1979277 SNP and the SHMT1 rs1979277-MTHFR rs1801133 interaction in 2 epidemiologic cohort studies. In the Nurses' Health Study (NHS), the MTHFR rs1801133 variant genotypes were associated with an increased CVD risk and there was an interaction between SHMT1 and MTHFR such that the association of the MTHFR rs1801133 CT genotype (vs. CC; the TT genotype could not be evaluated) was stronger in the presence of the SHMT1 rs1979277 TT genotype (OR = 4.34, 95% CI = 1.2, 16.2; P = 0.049). In the Health Professionals Follow-Up Study, the MTHFR rs1801133 genotype was not associated with CVD risk, nor was there an interaction with SHMT1 rs1979277. The association of genetic variation in the SHMT1 gene, alone and in interaction with MTHFR, in relation to CVD risk is relatively understudied at the population level and results in the NHS confirmed a past report of gene-gene interaction, which is consistent with mechanisms suggested by basic science studies.

US public opinion regarding proposed limits on resident physician work hours.

BACKGROUND: In both Europe and the US, resident physician work hour reduction has been a source of controversy within academic medicine. In 2008, the Institute of Medicine (IOM) recommended a reduction in resident physician work hours. We sought to assess the American public perspective on this issue. METHODS: We conducted a national survey of 1,200 representative members of the public via random digit telephone dialing in order to describe US public opinion on resident physician work hour regulation, particularly with reference to the IOM recommendations. RESULTS: Respondents estimated that resident physicians currently work 12.9-h shifts (95% CI 12.5 to 13.3 h) and 58.3-h work weeks (95% CI 57.3 to 59.3 h). They believed the maximum shift duration should be 10.9 h (95% CI 10.6 to 11.3 h) and the maximum work week should be 50 h (95% CI 49.4 to 50.8 h), with 1% approving of shifts lasting >24 h (95% CI 0.6% to 2%). A total of 81% (95% CI 79% to 84%) believed reducing resident physician work hours would be very or somewhat effective in reducing medical errors, and 68% (95% CI 65% to 71%) favored the IOM proposal that resident physicians not work more than 16 h over an alternative IOM proposal permitting 30-h shifts with > or =5 h protected sleep time. In all, 81% believed patients should be informed if a treating resident physician had been working for >24 h and 80% (95% CI 78% to 83%) would then want a different doctor. CONCLUSIONS: The American public overwhelmingly favors discontinuation of the 30-h shifts without protected sleep routinely worked by US resident physicians and strongly supports implementation of restrictions on resident physician work hours that are as strict, or stricter, than those proposed by the IOM. Strong support exists to restrict resident physicians' work to 16 or fewer consecutive hours, similar to current limits in New Zealand, the UK and the rest of Europe.