Age- and Lesion-Related Comorbidity Burden Among US Adults With Congenital Heart Disease: A Population-Based Study.

Background As patients with congenital heart disease (CHD) are living longer, understanding the comorbidities they develop as they age is increasingly important. However, there are no published population-based estimates of the comorbidity burden among the US adult patients with CHD. Methods and Results Using the IBM MarketScan commercial claims database from 2010 to 2016, we identified adults aged ≥18 years with CHD and 2 full years of continuous enrollment. These were frequency matched with adults without CHD within categories jointly defined by age, sex, and dates of enrollment in the database. A total of 40 127 patients with CHD met the inclusion criteria (mean [SD] age, 36.8 [14.6] years; and 48.2% were women). Adults with CHD were nearly twice as likely to have any comorbidity than those without CHD (P<0.001). After adjusting for covariates, patients with CHD had a higher prevalence risk ratio for "previously recognized to be common in CHD" (risk ratio, 9.41; 95% CI, 7.99-11.1), "other cardiovascular" (risk ratio, 1.73; 95% CI, 1.66-1.80), and "noncardiovascular" (risk ratio, 1.47; 95% CI, 1.41-1.52) comorbidities. After adjusting for covariates and considering interaction with age, patients with severe CHD had higher risks of previously recognized to be common in CHD and lower risks of other cardiovascular comorbidities than age-stratified patients with nonsevere CHD. For noncardiovascular comorbidities, the risk was higher among patients with severe than nonsevere CHD before, but not after, the age of 40 years. Conclusions Our data underscore the unique clinical needs of adults with CHD compared with their peers. Clinicians caring for CHD may want to use a multidisciplinary approach, including building close collaborations with internists and specialists, to help provide appropriate care for the highly prevalent noncardiovascular comorbidities.

Echocardiographic determination of pulmonary arterial capacitance.

A growing body of evidence has demonstrated that pulmonary arterial capacitance (PAC) is the strongest hemodynamic predictor of clinical outcomes across a wide spectrum of cardiovascular disease, including pulmonary hypertension and heart failure. We hypothesized that a ratio of right ventricular stroke volume (RVOT VTI) to the associated peak arterial systolic pressure (PASP) could function as a reliable non-invasive surrogate for PAC. We performed a prospective study of patients undergoing simultaneous transthoracic echocardiography and right heart catheterization (RHC) for various clinical indications. Measurements of the RVOT VTI/PASP ratio from echocardiographic measurements were compared against PAC calculated from RHC measurements. Correlation coefficients and Bland-Altman analysis compared the RVOT VTI/PASP ratio with PAC. Forty-five subjects were enrolled, 38% were female and mean age was 54 years (SD 13 years). The reason for referral to RHC was most commonly post-heart transplant surveillance (40%), followed by heart failure (22%), and pulmonary hypertension (18%). Pre-capillary pulmonary hypertension was present in 18%, isolated post-capillary pulmonary hypertension was present in 13%, and combined pre-and post-capillary pulmonary hypertension was present in 29%. The RVOT VTI/PASP ratio was obtainable in the majority of patients (78%), and Pearson's correlation demonstrated moderately-strong association between PAC and the RVOT VTI/PASP ratio, r = 0.75 (P < 0.001). Bland-Altman analysis demonstrated good agreement between measurements without suggestion of systematic bias and a mean difference in standardized units of - 0.133. In a diverse population of patients and hemodynamic profiles, we validated that the ratio of RVOT VTI/PASP to be a reliably-obtained non-invasive marker associated with PAC.

Pregnancy complications and premature cardiovascular events among 1.6 million California pregnancies.

Background: Cardiovascular complications of pregnancy present an opportunity to assess risk for subsequent cardiovascular disease. We sought to determine whether peripartum cardiomyopathy and hypertensive disorder of pregnancy subtypes predict future myocardial infarction, heart failure or stroke independent of one another and of other risks such as gestational diabetes, preterm birth and intrauterine growth restriction. Methods and results: The California Healthcare Cost and Utilization Project database was used to identify all hospitalised pregnancies from 2005 to 2009, with follow-up through 2011, for a retrospective cohort study. Pregnancies, exposures, covariates and outcomes were defined by International Classification of Diseases, Ninth Revision codes. Among 1.6 million pregnancies (mean age 28 years; median follow-up time to event excluding censoring 2.7 years), 558 cases of peripartum cardiomyopathy, 123 603 hypertensive disorders of pregnancy, 107 636 cases of gestational diabetes, 116 768 preterm births and 23 504 cases of intrauterine growth restriction were observed. Using multivariable Cox proportional hazards models, peripartum cardiomyopathy was independently associated with a 39.2-fold increase in heart failure (95% CI 30.0 to 51.9), resulting in ~1 additional hospitalisation per 1000 person-years. There was a 13.0-fold increase in myocardial infarction (95% CI 4.1 to 40.9) and a 7.7-fold increase in stroke (95% CI 2.4 to 24.0). Hypertensive disorders of pregnancy were associated with 1.4-fold (95% CI 1.0 to 2.0) to 7.6-fold (95% CI 5.4 to 10.7) higher risk of myocardial infarction, heart failure and stroke, resulting in a maximum of ~1 additional event per 1000 person-years. Gestational diabetes, preterm birth and intrauterine growth restriction had more modest associations. Conclusion: These findings support close monitoring of women with cardiovascular pregnancy complications for prevention of early cardiovascular events and study of mechanisms underlying their development.

One-Year Outcomes After MitraClip for Functional Mitral Regurgitation.

BACKGROUND: Secondary mitral regurgitation (SMR) occurs in the absence of organic mitral valve disease and may develop as the left ventricle dilates or remodels or as a result of leaflet tethering with impaired coaptation, most commonly from apical and lateral distraction of the subvalvular apparatus, with late annular dilatation. The optimal therapy for SMR is unclear. This study sought to evaluate the 1-year adjudicated outcomes of all patients with SMR undergoing the MitraClip procedure in the EVEREST II (Endovascular Valve Edge-to-Edge Repair Study) Investigational Device Exemption program, which is comprised of the randomized clinical trial, the prospective High-Risk Registry, and the REALISM Continued Access Registry (Multicenter Study of the MitraClip System). METHODS: Patients with 3+/4+ SMR enrolled in EVEREST II were stratified by non-high surgical risk (non-HR) and high surgical risk (HR) status (defined as Society of Thoracic Surgeons risk of mortality ≥12% or predefined risk factors). Clinical, echocardiographic, and functional outcomes at 1 year were evaluated. RESULTS: A total of 616 patients (482 HR, 134 non-HR; mean age, 73.3±10.5 years; Society of Thoracic Surgeons risk, 10.2±6.9%) with SMR underwent the MitraClip procedure. At baseline, 80.5% of patients were in New York Heart Association class III/IV. Major adverse events at 30 days included death (3.6%), stroke (2.3%), and renal failure (1.5%). At discharge, 88.8% had MR ≤2+. At 1 year, there were 139 deaths, and the Kaplan-Meier estimate of freedom from mortality was 76.8%. The majority of surviving patients (84.7%) remained with MR ≤2+ and New York Heart Association class I/II (83.0%). Kaplan-Meier survival at 1 year was 74.1% in HR patients and 86.4% in non-HR patients ( P=0.0175). At 1 year, both groups achieved comparable MR reduction (MR ≤2+, 84.0% versus 87.0%) and improvement in left ventricular end-diastolic volume (-8.0 mL versus -12.7 mL), whereas New York Heart Association class I/II was found in 80.1% versus 91.8% ( P=0.008) of HR and non-HR patients, respectively. In HR patients, the annualized rate of heart failure hospitalizations decreased from 0.68 to 0.46 in the 12 months before to 12 months after the procedure ( P<0.0001). CONCLUSIONS: Transcatheter mitral valve repair with the MitraClip in patients with secondary MR is associated with acceptable safety, reduction of MR severity, symptom improvement, and positive ventricular remodeling. CLINICAL TRIAL REGISTRATION: https://www.clinicaltrials.gov . Unique identifiers: NCT00209274, NCT01940120, and NCT01931956.

Evolution of Echocardiographic Measures of Cardiac Disease From CKD to ESRD and Risk of All-Cause Mortality: Findings From the CRIC Study.

RATIONALE & OBJECTIVE: Abnormal cardiac structure and function are common in chronic kidney disease (CKD) and end-stage renal disease (ESRD) and linked with mortality and heart failure. We examined changes in echocardiographic measures during the transition from CKD to ESRD and their associations with post-ESRD mortality. STUDY DESIGN: Prospective study. SETTING & PARTICIPANTS: We studied 417 participants with CKD in the Chronic Renal Insufficiency Cohort (CRIC) who had research echocardiograms during CKD and ESRD. PREDICTOR: We measured change in left ventricular mass index, left ventricular ejection fraction (LVEF), diastolic relaxation (normal, mildly abnormal, and moderately/severely abnormal), left ventricular end-systolic (LVESV), end-diastolic (LVEDV) volume, and left atrial volume from CKD to ESRD. OUTCOMES: All-cause mortality after dialysis therapy initiation. ANALYTICAL APPROACH: Cox proportional hazard models were used to test the association of change in each echocardiographic measure with postdialysis mortality. RESULTS: Over a mean of 2.9 years between pre- and postdialysis echocardiograms, there was worsening of mean LVEF (52.5% to 48.6%; P<0.001) and LVESV (18.6 to 20.2mL/m2.7; P<0.001). During this time, there was improvement in left ventricular mass index (60.4 to 58.4g/m2.7; P=0.005) and diastolic relaxation (11.11% to 4.94% with moderately/severely abnormal; P=0.02). Changes in left atrial volume (4.09 to 4.15mL/m2; P=0.08) or LVEDV (38.6 to 38.4mL/m2.7; P=0.8) were not significant. Worsening from CKD to ESRD of LVEF (adjusted HR for every 1% decline in LVEF, 1.03; 95% CI, 1.00-1.06) and LVESV (adjusted HR for every 1mL/m2.7 increase, 1.04; 95% CI, 1.02-1.07) were independently associated with greater risk for postdialysis mortality. LIMITATIONS: Some missing or technically inadequate echocardiograms. CONCLUSIONS: In a longitudinal study of patients with CKD who subsequently initiated dialysis therapy, LVEF and LVESV worsened and were significantly associated with greater risk for postdialysis mortality. There may be opportunities for intervention during this transition period to improve outcomes.

Impact of the MitraClip Procedure on Left Atrial Strain and Strain Rate.

BACKGROUND: Although reverse remodeling of the left atrium (LA) has been shown after MitraClip placement in degenerative mitral regurgitation (MR), changes in LA strain and strain rate have not been evaluated in these patients or compared with surgical mitral valve repair. METHODS AND RESULTS: We included 87 subjects (mean age 59.7±12.3 years) with degenerative MR enrolled in the randomized EVEREST II trial (Endovascular Valve Edge-to-Edge Repair Study II). Baseline peak positive LA strain (ε) and strain rates along with longitudinal left ventricular strain (LV ε) were compared with 12-month follow-up measures. At 12 months, all 38 mitral valve repair subjects had ≤2+ MR, and 31 of the 49 MitraClip had ≤2+ MR. Baseline LA strain and strain rate values were similar in MitraClip and mitral valve repair groups. Of the strain rate measures, only peak early diastolic strain rate was consistently decreased in all treatment arms. In patients with reduced baseline LA ε, no significant change in LA ε occurred in either the MitraClip or mitral valve repair group despite significant MR and LA volume reduction. However, in patients with normal or high baseline ε, successful MR reduction by either method resulted in return of LA ε to normative values. There was a significant correlation between LA ε, LV ε values, and change in LA ε and LV ε over time. CONCLUSIONS: Changes seen in LA ε seem to depend on baseline LA and LV function. Changes in LA ε after MR reduction may reflect a decrease in LA expansion but may also be influenced by the degree of pre-existing LA dysfunction. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT00209274.

College students with congenital heart disease: A critical time for transition.

As medical and surgical advances improve, more young adults with congenital heart disease (CHD) are attending college. This case study illustrates some of the issues that these young adults may face as they attend college and discusses the role that college health practitioners can play in easing that transition. PARTICIPANTS: A case of a male with CHD presenting to the college health clinic with a new onset headache. METHODS: The authors discuss some of the unique challenges that college health practitioners may face when caring for students with CHD. In addition, they make recommendations on how best to care for these patients and how best to coordinate care with CHD students other care providers. RESULTS: This student with a history of coarctation of the aorta presented with new onset headaches and was found to have high blood pressure. He was diagnosed with recurrent coarctation, underwent percutaneous treatment with stenting and quickly resumed classes. CONCLUSIONS: As more students with CHD enter college, college health providers will need to understand some of the health risks that CHD students face. In addition, understanding some of the optimal ways to coordinate care with CHD providers can ease the transition that CHD students face as they enter college.

4D flow image quality with blood pool contrast: a comparison of gadofosveset trisodium and ferumoxytol.

To evaluate the effect of MRI blood pool contrast agent on 4D flow image quality and ventricular volume measurements. Adult patients referred for clinical cardiac MRI (n = 22) were imaged with 4D flow. Patients with renal failure (n = 10) received ferumoxytol, and the remainder (n = 12) received gadofosveset trisodium. Image quality was assessed with (1) signal-to-noise ratio (SNR); (2) contrast-to-noise ratio (CNR); and (3) 5-point Likert scale based on endocardial border definition (1 = none; 2 = partial but unable to visualize; 3 = able to roughly estimate; 4 = visible for most of the cardiac cycle; 5 = excellent definition). A subset (n = 15) had short axis steady-state free precession (SSFP) cine imaging allowing for comparison of standard volumetric measurement technique with 4D flow derived volumetric measurements. 4D flow studies using ferumoxytol demonstrated a higher median Likert score of 5 (IQR, 5-5) versus 3 (IQR, 2-3). Median cavity SNR and CNR were higher for ferumoxytol compared to gadofosveset trisodium [65 (IQR, 50-74) versus 22 (IQR, 14-28), p < 0.001; and 40 (IQR, 32-49) versus 4 (IQR, 3-10), p < 0.001]. Good correlation (p < 0.001) was seen between SSFP and 4D flow measured ventricular volumes (ESV and EDV) with ferumoxytol (r = 0.998, mean difference = 1.2 mL, LOA = - 7.7-10.1 mL) and gadofosveset trisodium (r = 0.942, mean difference = - 2.7 mL, LOA = - 35.7-27.1 mL). Ferumoxytol used off-label as an MRI blood pool contrast agent offers an attractive alternative to gadofosveset trisodium in patients with renal failure, with excellent 4D flow image quality and good correlation of volumetric measurements compared to the CMR reference (SSFP).

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation.

BACKGROUND: Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. AIM: To determine the association between CPET variables and LD in adults who have had the Fontan operation. METHODS: We retrospectively reviewed the medical records from two tertiary institutions. RESULTS: We identified 114 adults (≥18 years; mean 30.9±7.4 years) who had undergone the Fontan operation: 56% were women; 63% had total cavopulmonary connection; 66% had New York Heart Association (NYHA) class I status; 42% had arrhythmias; 22% had systemic right ventricle; and 35% had ventricular dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging evidence of cirrhosis, with or without portal hypertension, splenomegaly or varices). There were no differences in clinical or echocardiographic variables between those with and without LD. Among the 58 patients with CPET data, mean peak oxygen consumption (VO2) was 18.6±5.7mL/kg/min, per-cent-predicted peak VO2 was 53.9±15.5%, peak oxygen pulse was 9.3±2.9mL/beat and per-cent-predicted peak oxygen pulse was 82.6±21.5%. Of the 44 patients with liver and CPET data, each standard deviation decrease in per-cent-predicted peak VO2 (16%) and per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold increase in the odds of LD, after adjusting for NYHA, institution and Fontan type (P=0.04). Similarly, each standard deviation decrease in per-cent-predicted peak VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year earlier onset of LD, respectively (P>0.05). CONCLUSIONS: Decline in per-cent-predicted peak VO2 and oxygen pulse was associated with increased odds of LD in adults who had undergone the Fontan operation. Our study supports more rapid hepatic evaluation among patients with abnormal or worsening CPET variables.

Cardiovascular events and hospital resource utilization pre- and post-transcatheter mitral valve repair in high-surgical risk patients.

MitraClip is an approved therapy for mitral regurgitation (MR); however, health care resource utilization pre- and post-MitraClip remains understudied. METHODS: Patients with functional and degenerative MR at high surgical risk in the EVEREST II High-Risk Registry and REALISM Continued-Access Study were linked to Medicare data. Pre- and post-MitraClip all-cause death, stroke, myocardial infarction, heart failure (HF), and bleeding hospitalizations were identified. Inpatient costs, adjusted to 2010 US dollars, were calculated, and event rate ratios and cost ratios were estimated with multivariable modeling. RESULTS: Among 403 linked patients, the mean age was 80 years, 60% were male, mean baseline left ventricular ejection fraction was 49.6%, 83.3% were New York Heart Association class III/IV, 78.2% were MR grade 3+/4+, and 63.3% had functional MR. All-cause hospitalization decreased from 1,854 to 1,435/1,000 person-years (P<.001). HF hospitalization decreased following MitraClip (749 vs 332/1000 person-years, P<.001), but bleeding increased (199 vs 298/1000 person-years, P<.001). Changes in stroke and myocardial infarction were not statistically significant. Overall mean Medicare costs per patient were similar pre- and post-MitraClip, although there was a significant decrease in mean costs among those that survived a full year after MitraClip ($18,131 [SD $25,130] vs $11,679 [SD $22,486], P=.02). CONCLUSIONS: MitraClip was associated with a reduced rate of all-cause and HF hospitalizations and an increased rate of bleeding hospitalizations. One-year Medicare costs were reduced in those who survived a full year after the MitraClip procedure. Payors and providers seeking to reduce HF hospitalizations and associated Medicare costs may consider MitraClip among appropriate patients likely to survive 1 year.

Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease.

Background: Women with congenital heart disease (CHD) may be at increased risk for adverse events during pregnancy and delivery. Objective: To compare delivery outcomes between women with and without CHD. Design, Setting, and Participants: This retrospective study of inpatient delivery admissions in the Healthcare Cost and Utilization Project's California State Inpatient Database compared maternal and fetal outcomes between women with and without CHD by using multivariate logistic regression. Female patients with codes for delivery from the International Classification of Diseases, Ninth Revision, from January 1, 2005, through December 31, 2011, were included. The association of CHD with readmission was assessed to 7 years after delivery. Cardiovascular morbidity and mortality were hypothesized to be higher among women with CHD. Data were analyzed from April 4, 2014, through January 23, 2017. Exposures: Noncomplex and complex CHD. Main Outcomes and Measures: Maternal outcomes included in-hospital arrhythmias, eclampsia or preeclampsia, congestive heart failure (CHF), length of stay, preterm labor, anemia complicating pregnancy, placental abnormalities, infection during labor, maternal readmission at 1 year, and in-hospital mortality. Fetal outcomes included growth restriction, distress, and death. Results: Among 3 642 041 identified delivery admissions, 3189 women had noncomplex CHD (mean [SD] age, 28.6 [7.6] years) and 262 had complex CHD (mean [SD] age, 26.5 [6.8] years). Women with CHD were more likely to undergo cesarean delivery (1357 [39.3%] vs 1 164 509 women without CHD [32.0%]; P < .001). Incident CHF, atrial arrhythmias, ventricular arrhythmias, and maternal mortality were uncommon during hospitalization, with each occurring in fewer than 10 women with noncomplex or complex CHD (<0.5% each). After multivariate adjustment, noncomplex CHD (odds ratio [OR], 9.7; 95% CI, 4.7-20.0) and complex CHD (OR, 56.6; 95% CI, 17.6-182.5) were associated with greater odds of incident CHF. Similar odds were found for atrial arrhythmias in noncomplex (OR, 8.2; 95% CI, 3.0-22.7) and complex (OR, 31.8; 95% CI, 4.3-236.3) CHD, for fetal growth restriction in noncomplex (OR, 1.6; 95% CI, 1.3-2.0) and complex (OR, 3.5; 95% CI, 2.1-6.1) CHD, and for hospital readmission in both CHD groups combined (OR, 3.6; 95% CI, 3.3-4.0). Complex CHD was associated with greater adjusted odds of serious ventricular arrhythmias (OR, 31.8; 95% CI, 4.3-236.3) and maternal in-hospital mortality (OR, 79.1; 95% CI, 23.9-261.8). Conclusions and Relevance: In this study of hospital admissions for delivery in California, CHD was associated with incident CHF, atrial arrhythmias, and fetal growth restriction and complex CHD was associated with ventricular arrhythmias and maternal in-hospital mortality, although these outcomes were rare, even in women with complex CHD. These findings may guide monitoring decisions and risk assessment for pregnant women with CHD at the time of delivery.

AVJ-514 Trial　- Baseline Characteristics and 30-Day Outcomes Following MitraClip® Treatment in a Japanese Cohort.

BACKGROUND: The MitraClip®system is a transcatheter-based therapeutic option for patients with chronic mitral regurgitation (MR) who are at high risk for surgery. A prospective, multicenter, single-arm study was initiated to confirm the transferability of this system to Japan.Methods and Results:Patients with symptomatic chronic moderate-to-severe (3+) or severe (4+) functional or degenerative MR with a Society of Thoracic Surgery (STS) score ≥8%, or the presence of 1 predefined risk factor were enrolled. Patients with left ventricular (LV) ejection fraction (EF) <30% were excluded. MR severity and LV function were assessed by an independent echocardiography core lab. Primary outcome included major adverse events (MAE) at 30 days and acute procedural success (APS). A total of 30 patients (age: 80±7 years; STS score: 10.3%±6.6%) were treated with the MitraClip®. At baseline, all patients had MR 3+/4+ with 53%/47% patients with degenerative/functional etiology with mean LVEF of 50.2±12.8%, and 37% of patients were NYHA class III/IV. APS was achieved in 86.7% with no occurrence of MAE. At 30 days, 86.7% of patients had MR ≤2+ and 96.7% were NYHA class I/II. CONCLUSIONS: The MitraClip®procedure resulted in clinically meaningful improvements in MR severity, function and quality of life measures, and low MAE rates. These early results suggest the transferability of this therapy to appropriately selected Japanese patients. (Trial Registration: clinicaltrials.gov Identifier NCT02520310.).

Dilatation of the ascending aorta is associated with presence of aortic regurgitation in patients after repair of tetralogy of Fallot.

To evaluate the association between aortic morphology and elasticity with aortic regurgitation in surgically corrected of tetralogy of Fallot (TOF) patients. We retrospectively identified 72 consecutive patients with surgically corrected TOF and 27 healthy controls who underwent cardiac MRI evaluation. Velocity-encoded cine MRI was used to quantify degree of aortic regurgitation (AR) in TOF patients. Ascending aorta diameters were measured at standard levels on MRA images. Aortic pulse-wave velocity (PWV) was quantified with MRI. Morphological and functional MRI variables were compared between groups of TOF patients with and without clinically relevant AR and controls. The association between aortic morphology and elasticity with the presence of AR was evaluated using univariate and multivariate logistic regression. The majority of TOF patients had only trace AR. Nine TOF patients (12 %) had an AR fraction higher than 15 %. Indexed aorta diameter at the sinotubular junction (p = 0.007), at the RPA level (p = 0.006), and low left ventricular ejection fraction (LVEF) (p = 0.015) showed the strongest associations with the presence of at least mild AR, which persisted after controlling for age and gender. Increased ascending aorta dimension is associated with AR in patients after repair of TOF. LVEF was also low in the group of patients with relevant AR compared to those without, suggesting even mild to moderate AR may contribute to LV dysfunction in these patients. Enlarged ascending aorta may be an indication for precise quantification of regurgitant fraction with MRI, since symptomatic patients may need aortic valve repair when moderate regurgitation is present.

Racial Differences in Left Atrial Size: Results from the Coronary Artery Risk Development in Young Adults (CARDIA) Study.

Whites have an increased risk of atrial fibrillation (AF) compared to Blacks. The mechanism underlying this association is unknown. Left atrial (LA) size is an important AF risk factor, and studies in older adults suggest Whites have larger LA diameters. However, because AF itself causes LA dilation, LA size differences may be due to greater subclinical AF among older Whites. We therefore assessed for racial differences in LA size among young adults at low AF risk. The Coronary Artery Risk Development in Young Adults (CARDIA) study enrolled White and Black participants between 18 and 30 years of age. LA diameter was measured in a subset of participants using echocardiography at Year 5 (n = 4,201) and Year 25 (n = 3,373) of follow up. LA volume was also assessed at Year 5 (n = 2,489). Multivariate linear regression models were used to determine the adjusted association between race and LA size. In unadjusted analyses, mean LA diameter was significantly larger among Blacks compared to Whites both at Year 5 (35.5 ± 4.8 mm versus 35.1 ± 4.5 mm, p = 0.01) and Year 25 (37.4 ± 5.1 mm versus 36.8 ± 4.9 mm, p = 0.002). After adjusting for demographics, comorbidities, and echocardiographic parameters, Whites demonstrated an increased LA diameter (0.7 mm larger at Year 5, 95% CI 0.3-1.1, p<0.001; 0.6 mm larger at Year 25, 95% CI 0.3-1.0, p<0.001). There was no significant association between race and adjusted Year 5 LA volume. In conclusion, in a young, well-characterized cohort, the larger adjusted LA diameter among White participants suggests inherent differences in atrial structure may partially explain the higher risk of AF in Whites. The incongruent associations between race, LA diameter, and LA volume suggest that LA geometry, rather than size alone, may have implications for AF risk.

Coexistence of abnormal systolic motion of mitral valve in a consecutive group of 324 adult Tetralogy of Fallot patients assessed with echocardiography.

BACKGROUND: The presence of mitral valve prolapse (MVP) in congenital heart disease (CHD) patients is not well described. Tetralogy of Fallot (TOF) is the most common cyanotic CHD associated with overall good long-term survival after palliation. Since MVP is more often identified in adults and TOF patients are now surviving longer, we thus sought to perform this cohort study with a case-control design to (1) determine the prevalence of MVP and systolic displacement of mitral leaflets (SDML) in adult TOF patients, and (2) describe their clinical and imaging characteristics. METHODS: Retrospective interrogation of our echocardiography database identified 328 consecutive TOF patients ≥18 years from 1 January 2000 to 31 December 2014. All images were reviewed to identify patients with concomitant MVP (prolapse >2 mm beyond the long-axis annular plane) or SDML (<2 mm beyond the annular plane). RESULTS: 26 (8%) TOF patients fulfilled criteria for systolic mitral valve abnormality (SMVA) (15 MVP; 11 SDML). 2 had moderate to severe mitral regurgitation requiring repair. When compared with 52 TOF patients without SMVA, those with SMVA were more likely to be females (60.7% vs 33.9%, p=0.03), less likely to have transannular patch (52% vs 97.4%, p<0.0001), had lower right ventricular ejection fraction (36.5% vs 43.8%, p=0.03) and a trend towards increased risk of atrial (44% vs 30.4%, p=0.5) and ventricular arrhythmias (32% vs 25.5%, p=0.6). On multivariate logistic regression, SMVA was independently associated with the absence of transannular patch (p=0.002) and atrial arrhythmias (p=0.04). CONCLUSIONS: In this series of adult TOF patients, we describe a novel finding of a high prevalence of systolic mitral valve abnormalities.

Pregnancy-related cardiovascular deaths in California: beyond peripartum cardiomyopathy.

OBJECTIVE: Maternal mortality rates rose markedly from 2002 to 2006 in California, prompting an in-depth maternal mortality review in a state that comprises one twelfth of the US birth cohort. Cardiovascular disease has emerged as the leading cause of pregnancy-related death in the United States. The primary aim of this analysis was to describe the incidence and type of cardiovascular disease as a cause of pregnancy-related mortality in California. The secondary aims were to describe racial/ethnic and socioeconomic disparities, risk factors, birth outcomes, timing of death and diagnosis, and signs and symptoms of cardiovascular disease and identify contributing factors. STUDY DESIGN: The California Pregnancy-Associated Mortality Review retrospectively examined a case series of 64 cardiovascular pregnancy-related deaths from 2002 through 2006. Two cardiologists independently reviewed complete inpatient and outpatient medical records including laboratory, radiology, electrocardiogram, chest X-ray, echocardiograms, and autopsy findings for each cardiovascular death and classified cause of death by type of cardiovascular disease. Demographic data, racial disparities, risk factors, signs and symptoms, timing of diagnosis and death, birth outcomes, and contributing factors were analyzed using bivariate comparisons with noncardiovascular pregnancy-related deaths and population-based data. RESULTS: Among 2,741,220 California women who gave birth, 864 died while pregnant or within 1 year of pregnancy; 257 of the deaths were deemed pregnancy related, and of these, 64 (25%) were attributed to cardiovascular disease. There were 42 deaths caused by cardiomyopathy, and the pregnancy-related mortality rate from cardiomyopathy was 1.54 per 100,000 births. Dilated cardiomyopathy existed in 29 cases, of which 15 met the definition of peripartum cardiomyopathy. Women with cardiovascular disease were more likely than women who died from noncardiovascular causes to be African-American (39.1% vs 16.1%; P < .01) and more likely to use illicit substances (23.7% vs 9.4%; P < .01). Thirty-seven percent were obese and 20% had a concomitant diagnosis of hypertension or preeclampsia during pregnancy. Health care decisions in the diagnosis or treatment of cardiovascular disease during and after pregnancy contributed to the fatal outcomes. CONCLUSION: African-American race, substance use, and obesity were risk factors for pregnancy-related cardiovascular disease mortality. Chronic disease prevention and better recognition and response to cardiovascular disease during pregnancy are needed to reduce maternal mortality.