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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

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

Rationale & objectiveAbnormal 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 designProspective study.Setting & participantsWe studied 417 participants with CKD in the Chronic Renal Insufficiency Cohort (CRIC) who had research echocardiograms during CKD and ESRD.PredictorWe 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.OutcomesAll-cause mortality after dialysis therapy initiation.Analytical approachCox proportional hazard models were used to test the association of change in each echocardiographic measure with postdialysis mortality.ResultsOver a mean of 2.9 years between pre- and postdialysis echocardiograms, there was worsening of mean LVEF (52.5% to 48.6%; P

BackgroundThe 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.MethodsRetrospective 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 (

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.

We report a case of sudden death in a clinically stable adult with l-transposition of the great arteries (l-TGA). Sudden death has been reported to be the leading cause of death in l-TGA and is often attributed to arrhythmias in the absence of another identifiable cause. However, the contribution of nonarrhythmic causes to the burden of sudden death in this population is unknown. Comprehensive postmortem investigation, including autopsy and pacemaker interrogation, demonstrated that the cause of death was massive pulmonary hemorrhage due to stenosis of the patient's mechanical tricuspid (systemic AV) valve. This report highlights the important contribution of nonarrhythmic causes of sudden death in this population and the value of autopsy and device interrogation in determining true cause of death.

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.

BackgroundTetralogy of Fallot (TOF) is one of the most common types of congenital heart disease and requires prompt surgical correction. Post-correction pulmonary insufficiency (PI) often ensues in adulthood. At times, the PI is accompanied by residual pulmonary stenosis (PS). Little is known regarding right ventricular (RV) function in the setting of combined PS and PI.PurposeTo compare cardiac magnetic resonance (CMR) parameters for the assessment of RV function between patients with combined pulmonary stenosis and pulmonary insufficiency (PSPI) and isolated PI following surgical repair of TOF.Material and methodsRetrospective review of patients with comparable corrected TOF and similar PI was performed. Seventeen patients (median age, 24 years; range, 10-52 years) had combined PSPI and 30 patients (median age, 30 years; range, 6-70 years) had isolated PI. Cine magnetic resonance (MR) images (Philips Medical Systems, Best, The Netherlands) in the short-axis plane were used to calculate end-systolic, end-diastolic, and stroke volumes (RVESV, RVEDV, RVSV) and to measure RV wall thickness. Velocity-encoded cine MR images were used to measure pulmonary regurgitation fraction (PRF) by calculating the ratio of backward flow and total forward flow, obtained from the main pulmonary flow analysis. Peak pressure gradient across the pulmonary valve was obtained from spectral Doppler echocardiography.ResultsRVEF was 51 ± 8% in the PSPI patients and 39 ± 11%, in the patients with isolated PI (P = 0.001). Additionally, RV wall thickness was 5.2 ± 0.8 mm in the PSPI patients compared to 2.6 ± 0.9 mm in the isolated PI patients (P = 0.001). RVESVi and RVEDVi were significantly lower (P < 0.05) in patients with combined PSPI (60 ± 21 mL/m(2), 121 ± 35 mL/m(2), respectively) compared to the patients with isolated PI (95 ± 48 mL/m(2), 152 ± 61 mL/m(2), respectively).ConclusionRV function is preserved in patients with PSPI when compared to patients with PI following surgical repair of TOF.

BackgroundThe primary objective of this study was to examine levels of B-type natriuretic peptide (BNP) in mechanically ventilated patients with acute lung injury and to test whether the level of BNP would be higher in patients with right ventricular dilatation and would predict mortality.MethodsThis was a prospective, observational cohort study of 42 patients conducted in the intensive care unit of a tertiary care university hospital. BNP was measured and transthoracic echocardiography was performed within 48 hours of the onset of acute lung injury. The left ventricular systolic and diastolic function, right ventricular systolic function, and cardiac output were assessed. BNP was compared in patients with and without right ventricular dilatation, as well as in survivors versus nonsurvivors.ResultsBNP was elevated in mechanically ventilated patients with acute lung injury (median 420 pg/ml; 25-75% interquartile range 156-728 pg/ml). There was no difference between patients with and without right ventricular dilatation (420 pg/ml, 119-858 pg/ml vs. 387 pg/ml, 156-725 pg/ml; p = 0.96). There was no difference in BNP levels between the patients who died and those who survived at 30 days (420 pg/ml, 120-728 pg/ml vs. 385 pg/ml, 159-1070 pg/ml; p = 0.71).ConclusionsIn patients with acute lung injury the level of BNP is increased, but there is no difference in the BNP level between patients with and without right ventricular dilatation. Furthermore, BNP level is not predictive of mortality in this population.

Abstract Background Initial success of electrical cardioversion (ECV) of atrial fibrillation (AF) has been reported in several studies as 50%-90%, of which only 50% patients remain in sinus rhythm (SR) at the end of one year. We conducted this study to see if outcomes of other trials are applicable in managed care setting. Methods We conducted a retrospective study in 370 consecutive patients who underwent ECV for AF. They were reviewed for initial outcome of ECV and recurrence of AF after a successful ECV, with and without prophylactic antiarrhythmic drugs. Results Initial success of ECV for AF was 65.7%. At one year, 47% remained in SR. AF for ≤ 3 months (p = 0.006) and pretreatment with antiarrhythmic drugs (p = 0.032) resulted in improved success. Predictors of recurrence were patients ≤ 65 years (p = 0.019), paroxysmal atrial fibrillation (PAF) (p = 0.0094) and alcohol consumption (p = 0.0074). Conclusion Shorter duration of AF, prophylactic antiarrhythmic drugs and serial ECVs improve outcome of ECV in AF. For younger patients with PAF and alcohol consumption, due to higher recurrence of AF, rate control or ablative therapy may be the preferred strategy.

BackgroundWe studied the pharmacology of l-methamphetamine, the less abused isomer, when used as a nasal decongestant.Methods12 subjects self-administered l-methamphetamine from a nonprescription inhaler at the recommended dose (16 inhalations over 6 hours) then at 2 and 4 (32 and 64 inhalations) times this dose. In a separate session intravenous phenylephrine (200 microg) and l-methamphetamine (5 mg) were given to define alpha agonist pharmacology and bioavailability. Physiological, cardiovascular, pharmacokinetic, and subjective effects were measured.ResultsPlasma l-methamphetamine levels were often below the level of quantification so bioavailability was estimated by comparing urinary excretion of the intravenous and inhaled doses, yielding delivered dose estimates of 74.0 +/- 56.1, 124.7 +/- 106.6, and 268.1 +/- 220.5 microg for ascending exposures (mean 4.2 +/- 3.3 microg/inhalation). Physiological changes were minimal and not dose-dependent. Small decreases in stroke volume and cardiac output suggesting mild cardiodepression were seen.ConclusionInhaled l-methamphetamine delivered from a non-prescription product produced minimal effects but may be a cardiodepressant.

A case study of a 44-year-old man with dyspnea and new atrial fibrillation who received diagnosis of mild mitral regurgitation at 28 years of age but has not visited a physician in many years is presented. Physical examination of the patient which revealed a holosystolic murmur and a soft [S.sub.3] and repeat echocardiography showed a flail posterior leaflet and moderate-to-severe mitral regurgitation suggested surgery at a center experienced in mitral-valve repair on the basis of symptoms and atrial fibrillation.

A study was conducted to evaluate the efficacy of cardiac-resynchronization therapy (CRT) in reducing the risk of mortality or heart-failure events in the case of patients with mild cardiac symptoms. Results indicated that CRT with implantable cardioverter-defibrillator (ICD) greatly reduced the risk of mortality or heart-failure events.

The aim of this study was to determine the feasibility and accuracy of wall motion score index (WMSI) and myocardial performance index (MPI) for measuring regional and global left ventricular (LV) function with use of high-resolution echocardiography after myocardial infarction (MI) in mice. In 48 mice, myocardial infarction was induced by ligation in the middle of the left anterior descending coronary artery. Echocardiography was performed under anesthesia at baseline and 1 mo after MI. WMSI was analyzed by a 16-segment model on short-axis views, and wall motion was scored as 1 for normal, 2 for hypokinetic, 3 for akinetic, 4 for dyskinetic, and 5 for aneurysmal. WMSI was calculated as the sum of scores divided by the total number of segments. MPI was calculated on the basis of isovolumetric contraction time (IVCT), isovolumetric relaxation time (IVRT), and ejection time (ET): MPI = (IVCT + IVRT)/ET. We measured LV ejection fraction (LVEF), end-systolic and end-diastolic volumes (ESV and EDV), fractional shortening (FS), and infarct size (IS). LVEF at 4 wk after MI was reduced at 32.8 [+ or -] 9.0%. Linear correlation analyses showed that WMSI (1.6 [+ or -] 0.3) correlated with LVEF (r = -0.84, P < 0.0005), FS (r = -0.43, P = 0.003), and IS (34.3 [+ or -] 15.3%, r = 0.86, P < 0.0005). MPI (0.67 [+ or -] 0.09) correlated with LVEF (r = -0.67, P < 0.0005) and IS (r = 0.72, P < 0.0005). MPI also correlated with mitral inflow velocity (r = -0.68, P < 0.0005) and deceleration time (r = -0.42, P = 0.003). Stepwise regression analysis revealed that WMSI was independently associated with IS. IS, FS, mitral inflow velocity, and deceleration time were independent determinants of MPI. In conclusion, echocardiographic assessments of WMSI and MPI in mice are feasible and correlate strongly with two-dimensional measurement of LV function and IS. These novel parameters provide additional noninvasive assessment of regional and global LV function in mice after MI. experimental myocardial infarction; echocardiography; infarct size

With the availability of transgenic models, the mouse has become an increasingly important subject for genetic-hemodynamic studies. Recently, we developed a technique to measure left ventricular (LV) pressure in conscious mice with an implanted LV polyethylene tube. We extended our new method by evaluating the LV pressure-volume relationship and examined the feasibility of this method in this study. We studied 17 male mice (age, 11-20 wk) with a conductance catheter inserted into the LV through the polyethylene tube. Load-independent parameters of contractility derived from pressure-volume relationship [slope of the end-systolic pressure-volume relationship ([E.sub.es]), slope of the maximum first derivative of LV pressure ([dP/dt.sub.max])-end-diastolic volume (EDV) relation, and preload-recruitable stroke work (PRSW)] were evaluated by inferior vena caval occlusion with an implanted snare. LV function assessed by this technique on two different days showed that the parameters were very similar, indicating reproducibility. Both linear and nonlinear regression analyses were performed for Eel. Contractility was enhanced by isoproterenol ([E.sub.es], 13.1 [+ or -] 6.6 to 20.8 [+ or -] 8.7 mmHg/[micro]l; dP/[dt.sub.max]-EDV, 496 [+ or -] 139 to 825 [+ or -] 178 mmHg*[s.sup.-1]*[micro][l.sup.-1]; and PRSW, 110 [+ or -] 23 to 127 [+ or -] 21 mmHg), depressed by atenolol ([E.sub.es], 14.5 [+ or -] 6.1 to 4.6 [+ or -] 2.0 mmHg/[micro]l; dP/[dt.sub.max]-EDV, 543 [+ or -] 188 to 185 [+ or -] 94 mmHg*[s.sup. -1]*[micro][l.sup.-1]; and PRSW, 117 [+ or -] 20 to 70 [+ or -] 15 mmHg) and isoflurane ([E.sub.es], 12.3 [+ or -] 6.0 to 5.7 [+ or -] 2.1 mmI-Ig/[micro]l; dP/[dt.sub.max]-EDV, 528 [+ or -] 172 to 164 [+ or -] 68 mmHg/s*[micro]l; and PRSW, 124 [+ or -] 19 to 48 [+ or -] 10 mmHg), significantly. In conclusion, this is the first description of the LV pressure-volume relationship in conscious mice. These findings suggest that this method is feasible to detect changes of contractility in the conscious state, allowing serial assessment of pressure-volume-derived cardiac function indexes over time without anesthesia or repeated surgery. ventricular function; mouse physiology