Characteristics and Outcomes of Patients With Severe Bioprosthetic Aortic Valve Stenosis Undergoing Redo Surgical Aortic Valve Replacement.

BACKGROUND: With improved event-free survival of patients undergoing primary bioprosthetic aortic valve replacement (AVR), reoperation to relieve severe prosthetic aortic stenosis (PAS) is increasing. We sought to (1) identify of the characteristics of patients with severe bioprosthetic PAS undergoing redo AVR, and (2) assess the outcomes of these patients, along with factors associated with adverse outcomes. METHODS AND RESULTS: We studied 276 patients with severe bioprosthetic PAS (64±16 years, 58% men) who underwent redo-AVR between 2000 and 2012 (excluding mechanical PAS, severe other valve disease, and transcatheter AVR). Society of Thoracic Surgeons score was calculated. Severe PAS was defined as AV area <0.8 cm(2), mean AV gradient ≥40 mm Hg, or dimensionless index <0.25. A composite outcome of death and congestive heart failure admission was recorded. Mean Society of Thoracic Surgeons score and mean AV gradients were 8±8 and 53±17 mm Hg, whereas 28% had >II+ aortic regurgitation. Only 39% had an isolated redo AVR, the rest were combination surgeries (coronary bypass and/or aortic surgeries). At 4.2±3 years, 64 (23%) patients met the composite end point (48 deaths and 19 congestive heart failure admissions, 2.5% 30-day deaths). On multivariable Cox survival analysis, higher Society of Thoracic Surgeons score (hazard ratio, 1.35), higher grades of aortic regurgitation (hazard ratio, 1.29), and higher right ventricular systolic pressure (hazard ratio, 1.3) were associated with worse longer-term outcomes (all P<0.01). CONCLUSIONS: At an experienced center, in patients with severe bioprosthetic PAS undergoing redo AVR, the majority undergo combination surgeries but have excellent outcomes.

Predictors of long-term outcomes in patients with significant myxomatous mitral regurgitation undergoing exercise echocardiography.

BACKGROUND: Significant myxomatous mitral regurgitation leads to progressive left ventricular (LV) decline, resulting in congestive heart failure and death. Such patients benefit from mitral valve surgery. Exercise echocardiography aids in risk stratification and helps decide surgical timing. We sought to assess predictors of outcomes in such patients undergoing exercise echocardiography. METHODS AND RESULTS: This is an observational study of 884 consecutive patients (age, 58 ± 14 years; 67% men) with grade III+ or greater myxomatous mitral regurgitation who underwent exercise echocardiography between January 2000 and December 2011 (excluding functional mitral regurgitation, prior valvular surgery, hypertrophic cardiomyopathy, rheumatic valvular disease, or greater than mild mitral stenosis). Clinical and echocardiographic data (mitral regurgitation, LV ejection fraction, LV dimensions, right ventricular systolic pressure) and exercise variables (metabolic equivalents, heart rate recovery at 1 minute after exercise) were recorded. Composite events of death, myocardial infarction, stroke, and progression to congestive heart failure were recorded. Mean LV ejection fraction, indexed LV end-systolic dimension, resting right ventricular systolic pressure, peak stress right ventricular systolic pressure, metabolic equivalents achieved, and heart rate recovery were 58 ± 5%, 1.6 ± 0.4 mm/m(2), 31 ± 12 mm Hg, 46 ± 17 mm Hg, 9.6 ± 3, and 33 ± 14 beats, respectively. During 6.4 ± 4 years of follow-up, there were 87 events. On stepwise multivariable Cox analysis, percent of age/sex-predicted metabolic equivalents (hazard ratio, 0.99; 95% confidence interval, 0.98-0.99; P=0.005), heart rate recovery (hazard ratio, 0.29; 95% confidence interval, 0.17-0.50; P<0.001), resting right ventricular systolic pressure (hazard ratio, 1.03; 95% confidence interval, 1.004-1.05; P=0.02), atrial fibrillation (hazard ratio, 1.91; 95% confidence interval, 1.07-3.41; P=0.03), and LV ejection fraction (hazard ratio, 0.96; 95% confidence interval, 0.92-0.99; P=0.04) predicted outcomes. CONCLUSIONS: In patients with grade III+ or greater myxomatous mitral regurgitation undergoing exercise echocardiography, lower percent of age/sex-predicted metabolic equivalents, lower heart rate recovery, atrial fibrillation, lower LV ejection fraction, and high resting right ventricular systolic pressure predicted worse outcomes.

Predictors of long-term outcomes in patients with hypertrophic cardiomyopathy undergoing cardiopulmonary stress testing and echocardiography.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) have exercise intolerance due to left ventricular outflow tract (LVOT) obstruction, mitral regurgitation, and left ventricular dysfunction. We sought to study predictors of outcomes in HCM patients undergoing cardiopulmonary stress testing (CPT). METHODS: We studied 1,005 HCM patients (50 ± 14 years, 64% men, 77% on ß-blockers) who underwent CPT with echocardiography. Clinical, echocardiographic, and exercise variables (peak oxygen consumption [VO2] and heart rate recovery [HRR] at first minute postexercise) were recorded. End point was a composite of death, appropriate defibrillator discharges, resuscitated sudden death, stroke, and heart failure admission. RESULTS: Mean left ventricular ejection fraction (LVEF), postexercise LVOT gradient, and peak VO2 were 62% ± 6%, 92 ± 51 mm Hg, and 21 ± 6 mL kg(-1) min(-1), respectively. Despite 789 patients (78%) being in New York Heart Association classes I to II, only 8% achieved >100% age-gender predicted peak VO2, whereas 77% and 15% achieved 50% to 100% and <50%, respectively. Left ventricular outflow tract gradient ≥30 mm Hg was observed in 83% patients, whereas 23% had abnormal HRR. More than 5.5 ± 4 years, there were 94 (9%) events; 511 (50%) patients underwent surgery for LVOT obstruction. Multivariable Cox proportional analysis demonstrated % age-gender predicted peak VO2 (hazard ratio [HR] 0.96 [0.93-0.98]), normal vs abnormal HRR (HR 0.48 [0.32-0.73]), higher LVEF (HR 0.96 [0.93-0.98]), surgery (0.53 [0.33-0.83]), and atrial fibrillation (HR 1.65 [1.04-2.60]) were associated with outcomes (all P < .05). CONCLUSIONS: In HCM patients undergoing CPT, a higher % of achieved age-gender predicted VO2 and surgical relief of LVOT obstruction were associated with better outcomes, whereas abnormal HRR, atrial fibrillation, and lower LVEF were associated with worse outcomes.

Predictors of long-term outcomes in symptomatic hypertrophic obstructive cardiomyopathy patients undergoing surgical relief of left ventricular outflow tract obstruction.

BACKGROUND: We report the predictors of long-term outcomes of symptomatic hypertrophic cardiomyopathy patients undergoing surgical relief of left ventricular outflow tract obstruction. METHODS AND RESULTS: We studied 699 consecutive patients who have hypertrophic cardiomyopathy with severe symptomatic left ventricular outflow tract obstruction (47±11 years, 63% male) intractable to maximal medical therapy, who were referred to a tertiary hospital between January 1997 and December 2007 for the surgical relief of left ventricular outflow tract obstruction. We excluded patients <18 years of age, those with an ejection fraction <50%, those with hypertensive heart disease of the elderly, and those with more than mild aortic or mitral stenosis. Clinical, echocardiographic, and Holter data were recorded. A composite end point of death, appropriate internal cardioverter defibrillator discharges, resuscitated from sudden death, documented stroke, and admission for congestive heart failure was recorded. During a mean follow-up of 6.2±3 years, 86 patients (12%) met the composite end point with 30-day, 1-year, and 2-year event rates of 0.7%, 2.8%, and 4.7%, respectively. The hard event rate (death, defibrillator discharge, and resuscitated from sudden death) at 30 days, 1 year, and 2 years was 0%, 1.5%, and 3%, respectively. Stepwise multivariable analysis identified residual postoperative atrial fibrillation (hazard ratio, 2.12; confidence interval, 1.37-3.34; P=0.001) and increasing age (hazard ratio, 1.49; confidence interval, 1.22-1.82; P=0.001) as independent predictors of long-term composite outcomes. CONCLUSIONS: Symptomatic adult hypertrophic cardiomyopathy patients undergoing surgery for the relief of left ventricular outflow tract obstruction have low event rates during long-term follow-up; worse outcomes are predicted by increasing age and the presence of residual atrial fibrillation during follow-up.

Effect of early treatment in polymyositis and dermatomyositis.

BACKGROUND: Idiopathic inflammatory myopathies, dermatomyositis (DM) and polymyositis (PM) are rare but are potentially treatable. AIM: To compare the effect of early and late treatment in patients with PM and DM. MATERIALS AND METHODS: The study included all the adult patients with definite diagnosis of PM or DM treated for at least 12 months. The patients were divided into two groups: Early Group - treatment within three months and Late Group - treatment after three months. The number of patients with positive therapeutic response, remission in less than one year and the mean time elapsed for reaching the remission were assessed and compared between the two groups. Chi-square test, Fisher's exact test, t-test and Pearson correlation test were used for data analysis. RESULTS: The analysis included 65 patients, 42 with DM and 23 with PM. Late Group included 24 patients (seven PM and 17 DM), while Early Group included 41 patients (16 PM and 25 DM). Positive therapeutic response, remission rate within one year was higher in Early Group (80% vs. 46%, P: 0.004). The mean time needed to achieve remission was much less with early treatment (5.5 vs. 11.9 months, P: 0.003). The relapse rate was also lower in Early Group (5% vs. 25%, P < 0.02). The comparison of treatment outcomes showed the same results in both PM and DM, but it was statistically significant in patients with DM. CONCLUSIONS: Early treatment in patients with PM and DM is associated with higher remission rates, shorter treatment period and low complication rates.

Hypertrophic Obstructive Cardiomyopathy Requiring Ablation via Septals From Both Left and Right Coronary Arteries - A Case Report and Review of Literature.

Alcohol septal ablation has traditionally been performed using septal perforators from the left coronary system. We describe a case in which septal perforators from the left and right coronary arteries were utilized and review current literature on the management of hypertrophic obstructive cardiomyopathy.

Role of Intravascular Ultrasound in Managing a Rare Case of Delayed Stent Deformity.

Delayed stent deformity is a very rare complication. We attempted to dilate the lumen within the crushed stent, but were unsuccessful even after laser atherectomy. At this point, we opted to place a new stent within the deformed old stent. In this case, intravascular ultrasound was extremely useful in guiding its diagnosis and management.

Emergent TandemHeart-ECMO for acute severe mitral regurgitation with cardiogenic shock and hypoxaemia: a case series.

BACKGROUND: Acute severe mitral regurgitation (MR) associated with cardiogenic shock is a life-threatening emergency. Traditional teaching has focused on the need for emergent coronary angiography and/or intra-aortic balloon counterpulsation in preparation for emergent open-heart surgery for repair/replacement. Unfortunately, emergent open-heart surgery in patients with acute MR complicated by cardiogenic shock is associated with 25-46% perioperative mortality. New devices have provided additional options for stabilization prior to emergent surgery which facilitate improved outcomes. CASE SUMMARY: We present two cases of acute severe MR resulting in cardiogenic shock and profound hypoxaemia. TandemHeart® mechanical circulatory support with an oxygenator spliced into the circuit, akin to veno-arterial extracorporeal membrane oxygenation (ECMO), facilitated haemodynamic stabilization and decongestion of the lungs facilitating successful bridge to mitral valve surgery. Successful discharge to home was achieved in both patients with good neurological outcomes and sustained long-term functional recovery at 18 and 14 months, respectively. DISCUSSION: Selective use of the TandemHeart®, with or without ECMO, facilitates management of the critically ill cardiogenic shock patient with acute severe MR.

Exercise capacity in asymptomatic patients with significant primary mitral regurgitation: independent effect of global longitudinal left ventricular strain.

BACKGROUND: Despite preserved left ventricular ejection fraction (LVEF), patients with significant primary mitral regurgitation (MR) often have reduced exercise capacity. In asymptomatic patients with ≥3+ primary MR undergoing rest-stress echocardiography (RSE), we sought to evaluate the incremental impact of left ventricular global longitudinal strain (LV-GLS) on exercise capacity. METHODS: A total of 660 asymptomatic patients with ≥3+ primary MR, non-dilated LV and LVEF ≥60% (mean age, 57±14 years, 66% men, body mass index or BMI 25±4 kg/m2) who underwent RSE at our center between 2001 and 2013 were included. Standard RSE data were obtained. Average resting LV-GLS was measured using Velocity Vector Imaging. RESULTS: Mean mitral effective regurgitant orifice, resting right ventricular systolic pressure (RVSP) and LV-GLS were 0.45±0.2 cm2, 31±12 mmHg and -21.7%±2%, respectively; 28% had flail mitral leaflet. Mean metabolic equivalents (METs) and post-stress RVSP were 9.9±3, and 46±15 mmHg; 28% achieved <100% age-gender predicted METs. No patient had ischemia or significant arrhythmias. On logistic regression, resting LV-GLS [odds ratio (OR), 1.40, 95% confidence interval (CI): 1.21-1.55, BMI (OR, 1.11, 95% CI: 1.06-1.17)] and resting RVSP 1.22 (1.02-1.49) were independent predictors of exercise capacity. Area under the curve for association between 100% age-gender predicted METs and various factors were as follows: (I) BMI (0.60, 95% CI: 0.55-0.65, P<0.001); (II) resting RVSP (0.57, 95% CI: 0.52-0.62, P=0.006) and LV-GLS (0.66, 95% CI: 0.61-0.70, P<0.001). CONCLUSIONS: In asymptomatic patients with ≥3+ primary MR, non-dilated LV and preserved LVEF, LV-GLS is independently associated with exercise capacity, beyond known predictors.

Incremental Prognostic Use of Left Ventricular Global Longitudinal Strain in Asymptomatic/Minimally Symptomatic Patients With Severe Bioprosthetic Aortic Stenosis Undergoing Redo Aortic Valve Replacement.

BACKGROUND: With improved survival of patients undergoing primary bioprosthetic aortic valve replacement (AVR), reoperation to relieve severe prosthetic aortic stenosis (PAS) is increasing. Timing of redo surgery in asymptomatic/minimally symptomatic patients remains controversial. Left ventricular (LV) global longitudinal strain (GLS) is a marker of subclinical LV dysfunction. In asymptomatic/minimally symptomatic patients with severe PAS undergoing redo AVR, we sought to determine whether LV-GLS provides incremental prognostic use. METHODS AND RESULTS: We studied 191 patients with severe bioprosthetic PAS (63±16 years, 58% men) who underwent redo AVR between 2000 and 2012 (excluding mechanical PAS, severe other valve disease transcatheter AVR, and LV ejection fraction <50%). Society of Thoracic Surgeons score was calculated. Standard echocardiography data were obtained. LV-GLS was measured on 2-, 3-, and 4-chamber views using velocity vector imaging. Severe PAS was defined as aortic valve area <0.8 cm2, mean aortic valve gradient ≥40 mm Hg, and dimensionless index <0.25. A composite outcome of death and congestive heart failure admission was recorded. At baseline, mean Society of Thoracic Surgeons score, LV ejection fraction, mean aortic valve gradients, and right ventricular systolic pressure were 7±6, 58±6%, 54±10 mm Hg and 40±14 mm Hg, whereas 50% had >2+ aortic regurgitation. Median LV-GLS was -14.2% (-11.4, -17.1%). At 4.2±3 years, 41 (22%) patients met the composite end point (2.5% deaths and 1% strokes at 30 days postoperatively). On multivariable Cox survival analysis, LV-GLS was independently associated with longer-term composite events (hazard ratio, 1.21; 95% confidence interval, 1.10-1.33), P<0.01. The C statistic for the clinical model (Society of Thoracic Surgeons score, degree of aortic regurgitation, and right ventricular systolic pressure) was 0.64 (95% confidence interval 0.54-0.79), P<0.001. Addition of LV-GLS to the clinical model increased the C statistic significantly to 0.71 (95% confidence interval 0.58-0.81), P<0.001. CONCLUSIONS: In asymptomatic/minimally symptomatic patients with severe bioprosthetic PAS undergoing redo AVR, baseline LV-GLS provides incremental prognostic use over established predictors and could potentially aid in surgical timing and risk stratification.

Strain Echocardiography and Functional Capacity in Asymptomatic Primary Mitral Regurgitation With Preserved Ejection Fraction.

BACKGROUND: The potential additive utility of baseline resting left ventricular global longitudinal strain (LV-GLS) and exercise stress testing in risk stratification of patients with significant mitral regurgitation (MR) has not been studied. OBJECTIVES: The goal of this study was to determine whether resting LV-GLS and exercise testing provide incremental prognostic utility in asymptomatic patients with ≥3+ primary MR and preserved left ventricular ejection fraction. METHODS: Between 2000 and 2011, resting and exercise echocardiography data, Society of Thoracic Surgeons (STS) scores, and death were recorded in 737 patients (mean age 58 ± 13 years; 68% men). RESULTS: Coronary artery disease and flail leaflet were seen in 10% and 28% of patients, respectively. STS score, resting left ventricular ejection fraction, mitral effective regurgitant orifice, resting right ventricular systolic pressure (RVSP), exercise metabolic equivalents (METs), and percentage of age-/sex-predicted METs were 1.5 ± 1%, 62 ± 2%, 0.45 ± 0.2 cm2, 31 ± 12 mm Hg, 9.8 ± 3, and 115 ± 27, respectively. Median LV-GLS was -21.7%. Within 3 months (interquartile range: 1 to 15 months), 65% underwent mitral valve surgery. At 8.3 ± 3 years, 64 (9%) patients died (0% 30-day post-operative deaths). On multivariable Cox survival analysis, higher STS score (hazard ratio [HR]: 1.14), more abnormal resting LV-GLS (HR: 1.60), higher baseline RVSP (HR: 1.35), and lower percentage of age-/sex-predicted METs (HR: 1.13) were associated with higher mortality, whereas mitral valve surgery (HR: 0.82) was associated with improved survival (all p < 0.01). Addition of predicted METs and resting LV-GLS to STS, resting RVSP, left ventricular end-systolic dimension, and mitral effective regurgitant orifice increased the C-statistic for longer-term mortality from 0.61 to 0.69 and 0.78, respectively (all p < 0.01). On quadratic spline analysis, the risk of death progressively increased as resting LV-GLS worsened below -21%. CONCLUSIONS: Reduced exercise capacity and worsening resting LV-GLS were associated with mortality, providing additive prognostic utility.

Impact of additive mitral valve surgery to coronary artery bypass grafting on mortality in patients with coronary artery disease and ischaemic mitral regurgitation: a systematic review and meta-analysis of randomized trials and observational studies.

AIMS: Treatment of ischaemic mitral regurgitation (IMR) remains controversial. While IMR is associated with worse outcomes, randomized controlled trials (RCTs) and observational studies provided conflicting evidence regarding the benefit of mitral valve replacement (MVR) or repair (MVr) in addition to coronary artery bypass grafting (CABG). We conducted a meta-analysis incorporating data from published RCTs and observational studies comparing CABG vs. CABG + MVR/MVr. METHODS AND RESULTS: We searched PubMed, MEDLINE, Embase, Ovid, and Cochrane for RCTs and observational studies comparing CABG (Group 1) vs. CABG + MVR/MVr (Group 2). Outcome was 30-day and 1-year mortality after surgical intervention. Mantel-Haenszel odds ratio (OR) was calculated using random-effects meta-analysis for the outcome. Heterogeneity was assessed by I2 statistics. Four RCTs and 11 observational studies met the inclusion criteria (5781 patients, 507 in RCTs, 5274 in observational studies). Group 1 vs. 2 weighted mean left ventricular ejection fraction in RCTs and combined RCTs/observational studies was 41.5 ± 12.3 vs. 40.3 ± 10.4% ( P -value = 0.24) and 45.5 ± 7.2 vs. 38 ± 10% ( P -value < 0.001), respectively. In RCTs, there was no difference in 30-day [OR: 0.95, 95% confidence interval (95% CI): 0.30-3.08, P = 0.94] or 1-year (OR: 0.90, 95% CI: 0.43-1.87, P = 0.78) mortality, respectively. For combined RCTs/observational studies, there was no difference in mortality at 30 days (OR: 0.67, 95% CI: 0.43-1.04, P = 0.08) or at 1 year (OR: 0.90, 95% CI: 0.7-1.15, P = 0.39). CONCLUSION: In a meta-analysis of RCTs and observational studies of IMR patients, the addition of MVR/MVr to CABG did not improve survival.

Stress echocardiography in valvular heart disease: a current appraisal.

Stress echocardiography is increasingly used in the management of patients with valvular heart disease and can aid in evaluation, risk stratification and clinical decision making in these patients. Evaluation of symptoms, exercise capacity and changes in blood pressure can be done during the exercise portion of the test, whereas echocardiographic portion can reveal changes in severity of disease, pulmonary artery pressure and left ventricular function in response to exercise. These parameters, which are not available at rest, can have diagnostic and prognostic importance. In this article, we will review the indications and diagnostic implications, prognostic implications, and clinical impact of stress echocardiography in decision making and management of patients with valvular heart disease.

Impact of duration of mitral regurgitation on outcomes in asymptomatic patients with myxomatous mitral valve undergoing exercise stress echocardiography.

BACKGROUND: Significant mitral regurgitation (MR) typically occurs as holosystolic (HS) or mid-late systolic (MLS), with differences in volumetric impact on the left ventricle (LV). We sought to assess outcomes of degenerative MR patients undergoing exercise echocardiography, separated based on MR duration (MLS versus HS). METHODS AND RESULTS: We included 609 consecutive patients with ≥III+myxomatous MR undergoing exercise echocardiography: HS (n=487) and MLS (n=122). MLS MR was defined as delayed appearance of MR signal during mid-late systole on continuous-wave Doppler while HS MR occurred throughout systole. Composite events of death and congestive heart failure were recorded. Compared to MLS MR, HS MR patients were older (60±14 versus 53±14 years), more were males (72% versus 53%), and had greater prevalence of atrial fibrillation (16% versus 7%; all P<0.01). HS MR patients had higher right ventricular systolic pressure (RVSP) at rest (33±11 versus 27±9 mm Hg), more flail leaflets (36% versus 6%), and a lower number of metabolic equivalents (METs) achieved (9.5±3 versus 10.5±3), compared to the MLS MR group (all P<0.05). There were 54 events during 7.1±3 years of follow-up. On step-wise multivariable analysis, HS versus MLS MR (HR 4.99 [1.21 to 20.14]), higher LV ejection fraction (hazard ratio [HR], 0.94 [0.89 to 0.98]), atrial fibrillation (HR, 2.59 [1.33 to 5.11]), higher RVSP (HR, 1.05 [1.03 to 1.09]), and higher percentage of age- and gender-predicted METs (HR, 0.98 [0.97 to 0.99]) were independently associated with adverse outcomes (all P<0.05). CONCLUSION: In patients with ≥III+myxomatous MR undergoing exercise echocardiography, holosystolic MR is associated with adverse outcomes, independent of other predictors.

Exercise echocardiography in asymptomatic HCM: exercise capacity, and not LV outflow tract gradient predicts long-term outcomes.

OBJECTIVES: This study sought to assess long-term outcomes in asymptomatic or minimally symptomatic patients with hypertrophic cardiomyopathy (HCM) who underwent exercise echocardiography, without invasive therapies for relief of left ventricular outflow tract (LVOT) obstruction. BACKGROUND: Many HCM patients present with LVOT obstruction, mitral regurgitation (MR), and diastolic dysfunction, often requiring invasive therapies for symptomatic relief. However, a significant proportion of truly asymptomatic patients can be closely monitored. In HCM patients, exercise echocardiography has been shown to be a useful assessment of functional capacity and risk stratification. METHODS: We included 426 HCM patients (44 ± 14 years; 78% men) undergoing exercise echocardiography, excluding hypertensive heart disease of elderly, ejection fraction <50% and invasive therapy (myectomy or alcohol ablation) during follow-up. Clinical, echocardiographic (LV thickness, LVOT gradient, and MR) and exercise variables (percent of age-sex predicted metabolic equivalents [METs] and heart rate recovery [HRR] at 1 min post-exercise) were recorded. A composite endpoint of death, appropriate internal defibrillator discharge, and admission for congestive heart failure was recorded. RESULTS: Patients were asymptomatic or minimally symptomatic on history, but 82% of patients achieved <100% of age-sex predicted METs, and 43% had ≥II+ post-stress MR. The mean LV septal thickness, post-exercise LVOT gradient, and HRR were 2.0 ± 0.5 cm, 62 ± 47 mm Hg, and 31 ± 14 beats/min, respectively. During a mean follow-up of 8.7 ± 3 years, there were 52 events (12%). Patients achieving >100% of age-sex predicted METs had 1% event rate versus 12% in those achieving <85%. On stepwise multivariate survival analysis, percent of age-sex predicted METs (hazard ratio [HR]: 0.76; 95% confidence interval [CI]: 0.64 to 0.90), abnormal HRR (HR: 0.89; 95% CI: 0.82 to 0.97), and atrial fibrillation (HR: 2.73; 95% CI: 1.30 to 5.74) (overall, p < 0.001) independently predicted outcomes. CONCLUSIONS: In asymptomatic or minimally symptomatic HCM patients, exercise stress testing provides excellent risk stratification, with a low event rate in patients achieving >100% of predicted METs.

Importance of exercise capacity in predicting outcomes and determining optimal timing of surgery in significant primary mitral regurgitation.

BACKGROUND: In primary mitral regurgitation (MR), exercise echocardiography aids in symptom evaluation and timing of mitral valve (MV) surgery. In patients with grade ≥3 primary MR undergoing exercise echocardiography followed by MV surgery, we sought to assess predictors of outcomes and whether delaying MV surgery adversely affects outcomes. METHODS AND RESULTS: We studied 576 consecutive such patients (aged 57±13 years, 70% men, excluding prior valve surgery and functional MR). Clinical, echocardiographic (MR, LVEF, indexed LV dimensions, RV systolic pressure) and exercise data (metabolic equivalents) were recorded. Composite events of death, MI, stroke, and congestive heart failure were recorded. Mean LVEF was 58±5%, indexed LV end-systolic dimension was 1.7±0.5 mm/m(2), rest RV systolic pressure was 32±13 mm Hg, peak-stress RV systolic pressure was 47±17 mm Hg, and percentage of age- and gender-predicted metabolic equivalents was 113±27. Median time between exercise and MV surgery was 3 months (MV surgery delayed ≥1 year in 28%). At 6.6±4 years, there were 53 events (no deaths at 30 days). On stepwise multivariable survival analysis, increasing age (hazard ratio of 1.07 [95% confidence interval, 1.03 to 1.12], P<0.01), lower percentage of age- and gender-predicted metabolic equivalents (hazard ratio of 0.82 [95% confidence interval, 0.71 to 0.94], P=0.007), and lower LVEF (0.94 [0.89 to 0.99], P=0.04) independently predicted outcomes. In patients achieving >100% predicted metabolic equivalents (n=399), delaying surgery by ≥1 year (median of 28 months) did not adversely affect outcomes (P=0.8). CONCLUSION: In patients with primary MR that underwent exercise echocardiography followed by MV surgery, lower achieved metabolic equivalents were associated with worse long-term outcomes. In those with preserved exercise capacity, delaying MV surgery by ≥1 year did not adversely affect outcomes.

Graves' disease causing pancytopenia and autoimmune hemolytic anemia at different time intervals: a case report and a review of the literature.

Graves' disease (GD) is associated with various hematologic abnormalities but pancytopenia and autoimmune hemolytic anemia (AIHA) are reported very rarely. Herein, we report a patient with GD who had both of these rare complications at different time intervals, along with a review of the related literature. The patient was a 70-year-old man who, during a hospitalization, was also noted to have pancytopenia and elevated thyroid hormone levels. Complete hematologic workup was unremarkable and his pancytopenia was attributed to hyperthyroidism. He was started on methimazole but unfortunately did not return for followup and stopped methimazole after a few weeks. A year later, he presented with fatigue and weight loss. Labs showed hyperthyroidism and isolated anemia (hemoglobin 7 g/dL). He had positive direct Coombs test and elevated reticulocyte index. He was diagnosed with AIHA and started on glucocorticoids. GD was confirmed with elevated levels of thyroid stimulating immunoglobulins and thyroid uptake and scan. He was treated with methimazole and radioactive iodine ablation. His hemoglobin improved to 10.7 g/dL at discharge without blood transfusion. Graves' disease should be considered in the differential diagnosis of hematologic abnormalities. These abnormalities in the setting of GD generally respond well to antithyroid treatment.