Susan A Casey

Minneapolis Heart Institute, Minneapolis, Minnesota, United States

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Publications (44)553.5 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%. This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients. We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45 ± 8 years) over 7.2 ± 5.2 years of follow-up. Of 1,000 patients, 918 (92%) survived to 53 ± 9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% [0.53%/year]) at 50 ± 10 years from the following events: progressive heart failure (n = 17); arrhythmic sudden death (SD) (n = 17); and embolic stroke (n = 2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n = 33) or heart transplantation for advanced heart failure (n = 18 [0.79%/year]). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p = 0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension. In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of 0.5%/year and an opportunity for extended longevity. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
    Journal of the American College of Cardiology 05/2015; 65(18):1915-28. DOI:10.1016/j.jacc.2015.02.061 · 15.34 Impact Factor
  • Journal of the American College of Cardiology 03/2015; 65(10):A789. DOI:10.1016/S0735-1097(15)60789-6 · 15.34 Impact Factor
  • Journal of the American College of Cardiology 03/2015; 65(10):A269. DOI:10.1016/S0735-1097(15)60269-8 · 15.34 Impact Factor
  • Journal of the American College of Cardiology 03/2015; 65(10):A359. DOI:10.1016/S0735-1097(15)60359-X · 15.34 Impact Factor
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    ABSTRACT: -In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An under-appreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function.
    Circulation Heart Failure 09/2014; DOI:10.1161/CIRCHEARTFAILURE.114.001435 · 5.95 Impact Factor
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    ABSTRACT: OBJECTIVES: To determine ICD efficacy in children/adolescents with HCM. BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden death (SD) in the young. Availability of the implantable cardioverter-defibrillator (ICD) over the last decade for HCM has demonstrated the potential for SD prevention, predominantly in adult patients. METHODS: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for SD was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and non-referral institutions in U.S., Europe and Australia. RESULTS: Defibrillators activated appropriately to terminate ventricular tachycardia/fibrillation (VT/VF) in 43 of 224 patients (19%) over a 4.3 ± 3.3 years period. ICD intervention rates were 4.5%/year overall, 14.0%/year for secondary prevention after cardiac arrest, and 3.1%/year for primary prevention based on risk factors (5-year cumulative probability; 17%). Implant to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). Primary prevention discharge rate terminating VT/VF was the same in patients implanted for 1, 2, or ≥ 3 risk factors (12/88, 14%; 10/71, 14%; 4/29, 14%; respectively; p = 1.00). Extreme LV hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17/26; 65%). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%), at 17 ± 5 years. CONCLUSIONS: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme LV hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.
    Journal of the American College of Cardiology 03/2013; 61(14). DOI:10.1016/j.jacc.2013.01.037 · 15.34 Impact Factor
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    ABSTRACT: BACKGROUND: Hypertrophic cardiomyopathy (HCM) is prominently associated with risk for sudden death (SD) and disease progression, largely in young patients. Whether patients of more advanced age harbor similar risks is unresolved, often creating clinical dilemmas, particularly in decisions for primary prevention of SD with implantable defibrillators. METHODS AND RESULTS: We studied 428 consecutive HCM patients presenting at ≥ 60 years, followed 5.8 ± 4.8 years; 53% were women. Of 428 patients, 279 (65%) survived to ages 73 ± 7 (range 61-96), most of these (n = 245; 88%) with no/mild symptoms, including 135 with ≥ 1 conventional SD risk factors and 50 (37%) with late gadolinium enhancement. Over follow-up, 149 (35%) died at80 ± 8 years, most from non-HCM related causes (n = 133; 31%), including a substantial proportion from non-cardiac disease (n = 54). Sixteen patients (3.7%) had HCM-related mortality events (0.64%/year), including embolic stroke (n = 6), progressive heart failure or transplant (n = 3), postoperative complications (n = 2), arrhythmic SD events (n = 5; 1.2%; 0.20%/year). All-cause mortality was increased in HCM patients ≥ 60 years compared to an age matched U.S. general population, due predominantly to non-HCM-related diseases (p < 0.001; SMR 1.5). CONCLUSIONS: HCM patients surviving into the seventh decade of life are at low risk for disease-related morbidity/mortality, including SD, even with conventional risk factors. These data do not support aggressive prophylactic defibrillator implantation at advanced ages in HCM. Other cardiac or non-cardiac co-morbidities have greater impact on survival than HCM in older patients.
    Circulation 12/2012; 127(5). DOI:10.1161/CIRCULATIONAHA.112.136085 · 14.95 Impact Factor
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    ABSTRACT: Hypertrophic cardiomyopathy (HC) is the most common cause of sudden death in the young, but survival to particularly advanced age is less well appreciated. The investigators report the prevalence, clinical features, and demographics of patients with HC surviving to ≥90 years of age. Of 1,297 patients with HC in the Hypertrophic Cardiomyopathy Center database (Minneapolis Heart Institute Foundation), 26 (2.0%) were identified who had achieved the age of ≥90 years; 18 (69%) were women. HC diagnosis came late in life, at 61 to 92 years (mean 80 ± 8; ≥75 years in 21 patients), recognized fortuitously by the detection of a heart murmur or during family screening (n = 6) or after onset of new symptoms (n = 20). At most recent evaluation (or death) patients were aged 90 to 96.7 years (mean 92.2 ± 2), with 6 presently alive at 91 to 96 years of age; HC did not appear to be the primary cause of death in any patient. Left ventricular wall thicknesses were 15 to 31 mm (mean 20 ± 3); 8 patients (31%) had obstruction to left ventricular outflow at rest (peak instantaneous gradients, 38 to 135 mm Hg). Significant HC-related complications occurred in 13 patients (50%), including progressive heart failure symptoms, atrial fibrillation, and nonfatal embolic stroke. Although no patient died suddenly, 13 (50%) nevertheless carried conventional HC risk markers. A greater proportion of cohort patients reached ≥90 years of age (2.0%) than expected in the general population (0.8%) (p <0.001). In conclusion, HC may be unrecognized until late in life and is consistent with survival to particularly advanced age into the 10th decade of life without the need for major HC-related treatment interventions, and with demise ultimately largely unrelated to this disease. This principle regarding the natural history of HC can afford a measure of reassurance to many patients.
    The American journal of cardiology 02/2012; 109(9):1341-7. DOI:10.1016/j.amjcard.2011.12.027 · 3.43 Impact Factor
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    ABSTRACT: The goal of this study was to assess gender-related differences in a multicenter population with hypertrophic cardiomyopathy (HCM). Little is known regarding the impact of gender on the heterogeneous clinical profile and clinical course of HCM. We studied 969 consecutive HCM patients from Italy and the U.S. followed over 6.2 +/- 6.1 years. Male patients had a 3:2 predominance (59%), similar in Italy and the U.S. (p = 0.24). At initial evaluation, female patients were older and more symptomatic than male patients (47 +/- 23 years vs. 38 +/- 18 years; p < 0.001; mean New York Heart Association [NYHA] functional class 1.8 +/- 0.8 vs. 1.4 +/- 0.6; p < 0.001), and more frequently showed left ventricular outflow obstruction (37% vs. 23%; p < 0.001). Moreover, female patients were less often diagnosed fortuitously by routine medical examination (23% vs. 41% in male patients, p < 0.001). Female gender was independently associated with the risk of symptom progression to NYHA functional classes III/IV or death from heart failure or stroke compared with male gender (independent relative hazard 1.5; p < 0.001), particularly patients > or =50 years of age and with resting outflow obstruction (p < 0.005). Hypertrophic cardiomyopathy-related mortality and risk of sudden death were similar in men and women. Women with HCM were under-represented, older, and more symptomatic than men, and showed higher risk of progression to advanced heart failure or death, often associated with outflow obstruction. These gender-specific differences suggest that social, endocrine, or genetic factors may affect the diagnosis and clinical course of HCM. A heightened suspicion for HCM in women may allow for timely implementation of treatment strategies, including relief of obstruction and prevention of sudden death or stroke.
    Journal of the American College of Cardiology 09/2005; 46(3):480-7. DOI:10.1016/j.jacc.2005.04.043 · 15.34 Impact Factor
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    ABSTRACT: Two-dimensional echocardiography is currently the standard test for the clinical diagnosis of hypertrophic cardiomyopathy (HCM). The present study was undertaken to determine whether cardiac MRI (CMR) affords greater accuracy than echocardiography in establishing the diagnosis and assessing the magnitude of left ventricular (LV) hypertrophy in HCM. Forty-eight patients (age 34+/-16 years) suspected of having HCM (or with a confirmed diagnosis) were imaged by both echocardiography and CMR to assess LV wall thickness in 8 anatomic segments (total n=384 segments) and compared in a blinded fashion. Maximum LV thickness was similar by echocardiography (21.7+/-9.1 mm) and CMR (22.5+/-9.6 mm; P=0.21). However, in 3 (6%) of the 48 patients, echocardiography did not demonstrate LV hypertrophy, and CMR identified otherwise undetected areas of wall thickening in the anterolateral LV free wall (17 to 20 mm), which resulted in a new diagnosis of HCM. In the overall study group, compared with CMR, echocardiography also underestimated the magnitude of hypertrophy in the basal anterolateral free wall (by 20+/-6%; P=0.001), as well as the presence of extreme LV wall thickness (> or =30 mm) in 10% of patients (P<0.05). CMR is capable of identifying regions of LV hypertrophy not readily recognized by echocardiography and was solely responsible for diagnosis of the HCM phenotype in an important minority of patients. CMR enhances the assessment of LV hypertrophy, particularly in the anterolateral LV free wall, and represents a powerful supplemental imaging test with distinct diagnostic advantages for selected HCM patients.
    Circulation 08/2005; 112(6):855-61. · 14.95 Impact Factor
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    ABSTRACT: Twelve-lead electrocardiography, a traditional component in evaluations of patients with hypertrophic cardiomyopathy (HC), is often regarded as a marker for the magnitude of left ventricular (LV) hypertrophy, which in turn has been linked to sudden death risk. To determine whether electrocardiographic (ECG) patterns have clinical utility by accurately reflecting phenotypic expression or predicting clinical outcome, voltages and patterns were compared with LV wall thicknesses assessed by echocardiography and with clinical outcomes in 448 consecutive patients with HC. Significant but relatively weak correlations were evident between maximum LV wall thickness and ECG voltage: r = 0.295 (p <0.01) for the sum of R- and S-wave voltages in all 12 leads, r = 0.254 (p <0.01) for the maximum R or S wave in any lead, and r = 0.210 (p <0.01) for the sum of SV(1) (or SV(2)) and RV(5) (or RV(6)). Of 55 patients with extreme LV hypertrophy (LV wall thickness > or =30 mm), only 24 (44%) showed greatly increased ECG voltage > or =30 mm in any lead. Of 102 patients with outflow gradients > or =30 mm Hg at rest, only 43 (42%) had ECG voltage > or =30 mm in any lead. Normal ECG results were uncommonly associated with HC-related death (1 of 40 patients, 2.5%) but had similar prevalence in surviving patients (17 of 376 patients, 4.5%; p = NS). In conclusion, in HC, 12-lead ECG voltages are not a reliable clinical marker for the magnitude of LV hypertrophy or outflow obstruction. Diverse ECG patterns, consistent with heterogeneous expression of this disease, did not predict HC-related death. Scalar electrocardiography has selective but limited power in routine clinical assessments of patients with HC.
    The American Journal of Cardiology 07/2005; 96(2):270-5. DOI:10.1016/j.amjcard.2005.03.058 · 3.43 Impact Factor
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    ABSTRACT: In hypertrophic cardiomyopathy (HC), an important subgroup of patients develop progressive and disabling symptoms that are related to heart failure and death. Although a direct relation has been demonstrated between left ventricular (LV) wall thickness and likelihood of sudden and unexpected death (usually in patients who are asymptomatic or mildly symptomatic), it is unresolved whether magnitude of hypertrophy is similarly associated with severity of heart failure. To determine the relation of LV wall thickness to heart failure symptoms in HC, 700 consecutive patients who had HC were assessed by 2-dimensional echocardiography. The relation between maximum level of heart failure symptoms by New York Heart Association functional class and maximum LV wall thickness was not linear but rather parabolic. Therefore, marked symptoms were most commonly associated with moderate degrees of LV hypertrophy (wall thickness 16 to 24 mm; 27%) but less frequently with extreme hypertrophy (>/=30 mm 13%) or mild hypertrophy (</=15 mm; 19%, p = 0.0001). Mean New York Heart Association functional class showed a similar pattern with respect to moderate hypertrophy (1.9 +/- 0.8), mild hypertrophy (1.6 +/- 0.9), and extreme hypertrophy (1.6 +/- 0.7, p = 0.005). Multivariable regression analysis showed the parabolic relation between heart failure symptoms and magnitude of LV hypertrophy to be independent of other hypertrophic cardiomyopathy related clinical variables. In conclusion, no direct relation was evident between symptoms of heart failure and magnitude of LV wall thickness, with implications for the natural history of HC.
    The American Journal of Cardiology 06/2005; 95(11):1329-33. DOI:10.1016/j.amjcard.2005.01.077 · 3.43 Impact Factor
  • ACC Current Journal Review 06/2005; 14(6):32. DOI:10.1016/j.accreview.2005.05.070
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    ABSTRACT: In this study we assessed the long-term efficacy and safety of disopyramide for patients with obstructive hypertrophic cardiomyopathy (HCM). It has been reported that disopyramide may reduce left ventricular outflow gradient and improve symptoms in patients with HCM. However, long-term efficacy and safety of disopyramide has not been shown in a large cohort. Clinical and echocardiographic data were evaluated in 118 obstructive HCM patients treated with disopyramide at 4 HCM treatment centers. Mortality in the disopyramide-treated patients was compared with 373 obstructive HCM patients not treated with disopyramide. Patients were followed with disopyramide for 3.1 +/- 2.6 years; dose 432 +/- 181 mg/day (97% also received beta-blockers). Seventy-eight patients (66%) were maintained with disopyramide without the necessity for major non-pharmacologic intervention with surgical myectomy, alcohol ablation, or pacing; outflow gradient at rest decreased from 75 +/- 33 to 40 +/- 32 mm Hg (p < 0.0001) and mean New York Heart Association functional class from 2.3 +/- 0.7 to 1.7 +/- 0.6 (p < 0.0001). Forty other patients (34%) could not be satisfactorily managed with disopyramide and required major invasive interventions because of inadequate symptom and gradient control or vagolytic side effects. All-cause annual cardiac death rate between disopyramide and non-disopyramide-treated patients did not differ significantly, 1.4% versus 2.6%/year (p = 0.07). There was also no difference in sudden death rate, 1.0%/year versus 1.8%/year (p = 0.08). Two-thirds of obstructed HCM patients treated with disopyramide could be managed medically with amelioration of symptoms and about 50% reduction in subaortic gradient over >/=3 years. Disopyramide therapy does not appear to be proarrhythmic in HCM and should be considered before proceeding to surgical myectomy or alternate strategies.
    Journal of the American College of Cardiology 05/2005; 45(8):1251-8. DOI:10.1016/j.jacc.2005.01.012 · 15.34 Impact Factor
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    ABSTRACT: The goal of this study was to assemble a profile and assess the significance of arrhythmias in a nontertiary-based hypertrophic cardiomyopathy (HCM) cohort. Hypertrophic cardiomyopathy is associated with arrhythmia-related consequences, particularly sudden death. Ventricular tachyarrhythmias on Holter electrocardiograms (ECG) have been reported as markers for sudden death in highly selected HCM populations. We assessed the profile of ventricular and supraventricular ectopy and bradyarrhythmia on ambulatory 24-h Holter ECG and also related these findings to clinical outcome in 178 HCM patients. Of the 178 study patients, 157 (88%) had premature ventricular complexes (PVCs), including 21 (12%) with >/=500 PVCs, 74 (42%) had couplets, 67 (37%) had supraventricular tachycardia (SVT), and 56 (31%) had nonsustained ventricular tachycardia (NSVT). Mean number of PVCs was 330 +/- 763 (range 1 to 5,435) and increased with age (p < 0.01); NSVT was associated with greater left ventricular hypertrophy (p = 0.01) and severe symptoms (New York Heart Association functional classes III and IV) (p = 0.04); SVT occurred more commonly in patients with outflow obstruction (p = 0.02). Over a follow-up of 5.5 +/- 3.4 years, 11 (6%) patients died suddenly (annual mortality rate, 1.1%) including 5 patients with NSVT. For sudden death, NSVT on Holter ECG had negative and positive predictive values of 95% and 9%, and sensitivity and specificity of 45% and 69%, respectively. In this nontertiary-based HCM cohort, ventricular and supraventricular tachyarrhythmias were particularly frequent and demonstrated a broad spectrum on ambulatory (Holter) ECG. Paradoxically, despite such a highly arrhythmogenic substrate, sudden death events proved to be relatively uncommon. Ventricular tachyarrhythmias had a low positive and relatively high negative predictive value for sudden death in this HCM population.
    Journal of the American College of Cardiology 03/2005; 45(5):697-704. DOI:10.1016/j.jacc.2004.11.043 · 15.34 Impact Factor
  • Circulation 11/2004; 110(16):e450. DOI:10.1161/01.CIR.0000145174.74063.C1 · 14.95 Impact Factor
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    ABSTRACT: Hypertrophic cardiomyopathy (HCM) has a diverse clinical spectrum that often includes progressive heart failure symptoms and disability. Assessment of symptom severity may be highly subjective, encumbered by the heterogeneous clinical presentation. Plasma B-type natriuretic peptide (BNP) has been used widely as an objective marker for heart failure severity and outcome, predominantly in coronary heart disease with ventricular dilatation and systolic dysfunction. We prospectively assessed plasma BNP as a quantitative clinical marker of heart failure severity in 107 consecutive HCM patients. BNP showed a statistically significant relationship to magnitude of functional limitation, assessed by New York Heart Association (NYHA) functional class: I, 136+/-159 pg/mL; II, 338+/-439 pg/mL; and III/IV, 481+/-334 pg/mL (P<0.001). Multivariable analysis showed that BNP was independently related to NYHA class as well as age and left ventricular wall thickness (each with a value of P=0.0001). BNP > or =200 pg/mL was the most reliable predictor of heart failure symptoms, with positive and negative predictive values of 63% and 79%, respectively. BNP power in distinguishing patients with or without heart failure symptoms was less than that for differentiating between no (or only mild) and severe symptoms (area under receiver operating characteristic curve=0.75 and 0.83, respectively). Plasma BNP is independently related to the presence and magnitude of heart failure symptoms in patients with HCM. As a clinical marker for heart failure, BNP is limited by considerable overlap in values between categories of heart failure severity as well as confounding variables of left ventricular wall thickness and age.
    Circulation 03/2004; 109(8):984-9. DOI:10.1161/01.CIR.0000117098.75727.D8 · 14.95 Impact Factor
  • Journal of the American College of Cardiology 03/2004; 43(5). DOI:10.1016/S0735-1097(04)90698-5 · 15.34 Impact Factor
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    B J Maron, S A Casey, A K Almquist
    Heart (British Cardiac Society) 02/2004; 90(1):24. · 6.02 Impact Factor
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    ABSTRACT: The morphologic apical form of hypertrophic cardiomyopathy (HC), in which left ventricular (LV) wall thickening is confined to the most distal region at the apex, has been regarded as a phenotypic expression of nonobstructive HC largely unique to Japanese patients. To investigate this question further, we directly compared unselected and regional hospital-based cohorts of adult patients with HC ( > or =18 years of age) from Japan (Kochi; n=100) and from the United States (US) (Minneapolis; n=361). Japanese and American patients with HC had similar clinical features and did not differ significantly with regard to the severity of symptoms and frequency of outflow obstruction. Although Japanese and American patients also showed similar maximum LV thickness, they differed significantly with respect to the distribution of LV hypertrophy. In particular, the segmental form of HC, with hypertrophy confined to the LV apex, was more frequent in Japanese patients (i.e., apical HC, 15% in Japan vs 3% in US, p<0.0001). Giant negative T waves were also more common in Japanese patients with HC (26% vs 2%, p<0.001), including those with the apical form (64% vs. 30%, p<0.05). Each patient with apical HC had either no or only mild symptoms, and all survived. The morphologic form of nonobstructive HC with hypertrophy limited to the LV apex (apical form of HC) was 5 times more common in an unselected Japanese population. These findings document variability in the phenotypic expression of HC between countries and races, which may be due to differences in environmental factors or genetic background. Patients with the apical form of HC had a benign clinical course.
    The American Journal of Cardiology 11/2003; 92(10):1183-6. DOI:10.1016/j.amjcard.2003.07.027 · 3.43 Impact Factor

Publication Stats

3k Citations
553.50 Total Impact Points


  • 1999–2015
    • Minneapolis Heart Institute
      Minneapolis, Minnesota, United States
  • 2005
    • University of Minnesota Medical Center, Fairview
      Minneapolis, Minnesota, United States
  • 2003
    • Kochi Medical School
      Kôti, Kōchi, Japan
    • Tufts Medical Center
      • Division of Cardiology
      Boston, Massachusetts, United States