Long-term Management of the Arterial Switch Patient.

PURPOSE OF REVIEW: This review paper describes the management of patients with dextro-transposition of the great arteries (D-TGA) with a focus on the complications seen and the appropriate care required to identify and prevent adverse events. RECENT FINDINGS: D-TGA is a form of cyanotic congenital heart disease (CHD) representing ~ 3% of all CHD and almost 20% of all cyanotic CHD. Since the late 1980s, standard of care is to repair these patients with an arterial switch operation (ASO) as opposed to a Mustard/Senning operation. The long-term survival and complication rates are superior in the ASO. Long-term follow-up is recommended for all D-TGA patients and includes management with adult congenital heart disease specialists and the use of echocardiography and advanced imaging with CT or MRI. The most common complications seen are pulmonary stenosis, coronary artery stenosis, and neo-aortic regurgitation. Careful evaluation of new symptoms or declining function is essential in preventing and treating these long-term sequelae.

Serial scanning with technetium pyrophosphate (99mTc-PYP) in advanced ATTR cardiac amyloidosis.

BACKGROUND: Development of noninvasive imaging modalities to quantify amyloid burden over time is an unmet clinical need. Technetium pyrophosphate (99mTc-PYP) scintigraphy is a simple and widely available radiotracer useful to differentiate transthyretin from light-chain amyloidosis in patients with advanced cardiac amyloidosis. We examined the utility of serial 99mTc-PYP scanning to quantify amyloid burden over time in TTR cardiac amyloidosis (ATTR-CA). METHODS AND RESULTS: Twenty subjects with ATTR-CA (10 wild type, 10 mutant) underwent serial 99mTc-PYP planar cardiac imaging. Cardiac retention was assessed both semiquantitatively (visual score 0, no uptake to 3, uptake greater than bone) and quantitatively (region of interest drawn over the heart, copied, and mirrored over the contralateral chest) to calculate a heart-to-contralateral (H/CL) ratio. Index scan mean visual score and H/CL were 3.0 ± 0.2 and 1.79 ± 0.2, respectively, and after an average 1.5 ± 0.5 years follow-up, did not differ, 3.0 ± 0.2, P = .33 and 1.76 ± 0.2, P = .44. H/CL change was minimal, 0.03 ± 0.17, did not correlate with time between scans, r = 0.19, P = .43, and was observed despite obvious clinical progression (increase in troponin ≥ 0.1 ng/mL, BNP ≥ 400 pg/mL, NYHA class, and/or death). CONCLUSIONS: Serial 99mTc-PYP scanning in subjects with advanced ATTR-CA does not show significant changes over an average 1.5 years of follow-up despite obvious clinical progression.

Spectrum of prevalent cardiovascular diseases in urban Port-au-Prince, Haiti: a population-based cross-sectional study.

Background: Eighty percent of global cardiovascular disease (CVD) is projected to occur in low- and middle -income countries (LMICs), yet local epidemiological data are scarce. We provide the first population-based, adjudicated CVD prevalence estimates in Port-au-Prince, Haiti to describe the spectrum of heart disease and investigate associated risk factors. Methods: Demographic, medical history, clinical, imaging and laboratory data were collected among adults recruited using multistage random sampling from 2019 to 2021. Prevalent CVD (heart failure, stroke, ischemic disease) were adjudicated using epidemiological criteria similar to international cohorts. Multivariable Poisson regressions assessed relationships between risk factors and prevalent CVD. Findings: Among 3003 participants, median age was 40 years, 58.1% were female, 70.2% reported income <1 USD/day, and all identified as Black Haitian. CVD age-adjusted prevalence was 14.7% (95% CI 13.3%, 16.5%), including heart failure (11.9% [95% CI 10.5%, 13.5%]), stroke (2.4% [95% CI 1.9%, 3.3%]), angina (2.1% [95% CI 1.6%, 2.9%]), myocardial infarction (1.0% [95% CI 0.6%, 1.8%]), and transient ischemic attack (0.4% [95% CI 0.2%, 1.0%]). Among participants with heart failure, median age was 57 years and 68.5% of cases were among women. The most common subtype was heart failure with preserved ejection fraction (80.4%). Heart failure was associated with hypertension, obesity, chronic kidney disease, depression, and stress. Interpretation: Early-onset heart failure prevalence is alarmingly high in urban Haiti and challenge modelling assumptions that ischemic heart disease and stroke dominate CVDs in LMICs. These data underscore the importance of local population-based epidemiologic data within LMICs to expedite the selection and implementation of evidence-based cardiovascular health policies targeting each country's spectrum of heart disease. Funding: This study was funded by NIH grants R01HL143788, D43TW011972, and K24HL163393, clinicaltrials.govNCT03892265.

Population-Based Epidemiology of Heart Failure in a Low-Income Country: The Haiti Cardiovascular Disease Cohort.

BACKGROUND: Cardiovascular disease disproportionately affects persons living in low- and middle-income countries and heart failure (HF) is thought to be a leading cause. Population-based studies characterizing the epidemiology of HF in these settings are lacking. We describe the age-standardized prevalence, survival, subtypes, risk factors, and 1-year mortality of HF in the population-based Haiti Cardiovascular Disease Cohort. METHODS: Participants were recruited using multistage cluster-area random sampling in Port-au-Prince, Haiti. A total of 2981 completed standardized history and exam, laboratory measures, and cardiac imaging. Clinical HF was defined by Framingham criteria. Kaplan-Meier and Cox proportional hazard regression assessed mortality among participants with and without HF; logistic regression identified associated factors. RESULTS: Among all participants, the median age was 40 years (interquartile range, 27-55), and 58.2% were female. Median follow-up was 15.4 months (interquartile range, 9-22). The age-standardized HF prevalence was 3.2% (93/2981 [95% CI, 2.6-3.9]). The average age of participants with HF was 57 years (interquartile range, 45-65), and 67.7% were female. The first significant increase in HF prevalence occurred between 30 to 39 and 40 to 49 years (1.1% versus 3.7%, P=0.003). HF with preserved ejection fraction was the most common HF subtype (71.0%). Age (adjusted odds ratio, 1.36 [1.12-1.66] per 10-year increase), hypertension (2.14 [1.26-3.66]), obesity (3.35 [95% CI, 1.99-5.62]), poverty (2.10 [1.18-3.72]), and renal dysfunction (5.42 [2.94-9.98]) were associated with HF. One-year HF mortality was 6.6% versus 0.8% (hazard ratio, 7.7 [95% CI, 2.9-20.6]; P<0.0001). CONCLUSIONS: The age-standardized prevalence of HF in this low-income setting was alarmingly high at 3.2%-5-fold higher than modeling estimates for low- and middle-income countries. Adults with HF were two decades younger and 7.7× more likely to die at 1 year compared with those in the community without HF. Further research characterizing the population burden of HF in low- and middle-income countries can guide resource allocation and development of pragmatic HF prevention and treatment interventions, ultimately reducing global cardiovascular disease health disparities. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; Unique identifier: NCT03892265.

Fetal alcohol spectrum disorders: knowledge and screening practices of university hospital medical students and residents.

BACKGROUND: Fetal alcohol spectrum disorder (FASD) is the leading cause of preventable intellectual disabilities in the United States and a significant public health issue. OBJECTIVES: The purpose of this study is to evaluate the knowledge and screening practices of pre-clinical medical students and clinical providers on FAS, FASD, and alcohol consumption. METHODS: A short survey sent to medical students and residents on the campus of a large medical school and university hospital. RESULTS: On the survey of clinical providers, 38% of respondents stated they always survey pregnant women about their alcohol consumption, 34% stated they always screen patients planning to get pregnant, and 9% screen women of childbearing age. There were a significant percentage of providers who never screen women. When questioned regarding safe amounts of alcohol consumption during pregnancy, 69% of pre-clinical medical students and 67% of clinical providers stated there is no safe amount of alcohol consumption. Clinical providers were much more likely to correctly select the facial features necessary for the diagnosis (p-value < 0.01). CONCLUSIONS: Significant differences exist in the knowledge and screening practices of these different healthcare providers and trainees. Future interventions should seek to improve knowledge on FAS, FASD, and alcohol consumption, in order for practitioners to be more consistent with national guidelines and the Surgeon General recommendations.

Transesophageal echocardiographic screening before atrial flutter ablation: is it necessary for patient safety?

BACKGROUND: Transesophageal echocardiography (TEE) is commonly used before atrial flutter (AFl) ablation to detect atrial thrombus (AT) and thereby identify a heightened risk for systemic embolism both in patients with their initial episodes of AFl and in those with prior episodes whose anticoagulation has been inadequate. This treatment strategy has been extrapolated from guidelines for atrial fibrillation. In fact, limited data exist regarding the prevalence or clinical associations of AT and spontaneous echocardiographic contrast (SEC) in patients with AFl. Both AT and SEC are believed to represent risk factors for systemic embolization. This study was designed to provide further insight into the prevalence of these and their associated clinical findings. METHODS: The results of transesophageal echocardiographic examinations in 347 consecutive patients with AFl in whom radiofrequency ablation procedures were planned were reviewed. In each case, specific care was taken to identify AT and SEC. The presence of either AT or more than mild SEC was considered to reflect a thrombogenic milieu (TM). Clinical and echocardiographic data were analyzed to determine the frequency and relevant clinical associations of these two markers of increased thromboembolic risk. In addition to determining the prevalence of AT and TM, the study sought to identify predictors of their presence short of TEE that might allow that procedure to be avoided. RESULTS: AT were found in 19 of the 347 patients (5.4%). TM was present in 39 patients (11.2%). SEC was associated with reduced left atrial appendage emptying velocity (P < .001). History of myocardial infarction (P = .02) was associated with AT. Reduced left ventricular ejection fraction (P = .01), reduced left atrial appendage emptying velocity (P < .001), diabetes mellitus (P = .02), congestive heart failure (P = .04), and chronic renal insufficiency (P = .05) were associated with a TM. CONCLUSIONS: Allowing for multiple comparisons, the significant markers of the risk for systemic embolization could be obtained only from TEE. Although there are several interesting clinical and echocardiographic associations with AT and a TM, none were strong enough to obviate the need for TEE.