Eric V Krieger

Seattle Children's Hospital, Seattle, Washington, United States

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Publications (13)35.84 Total impact

  • Eric V Krieger, Anne Marie Valente
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    ABSTRACT: Heart failure (HF) is the leading cause of death in adults with repaired congenital heart disease (CHD). However there is currently little evidence to guide treatment strategies in this growing group of patients. Unlike the majority of HF, which is usually caused by LV systolic or diastolic dysfunction, CHD-HF is more often a consequence of RV disease, valve dysfunction, shunting or pulmonary hypertension. It is therefore not appropriate to extrapolate from the acquired HF literature and apply it to this heterogeneous population of CHD patients. Additionally, patients with CHD have been excluded from most large trials of medical or device therapy of HF, which has resulted in small retrospective and underpowered studies in the CHD population. This article critically reviews the current knowledge about CHD-HF, paying particular attention to medical therapy in different CHD populations, cardiac resynchronisation therapy and implantable cardiac defibrillators, and the challenges of heart transplantation and mechanical circulatory support in CHD patients.
    Heart (British Cardiac Society) 06/2014; · 5.01 Impact Factor
  • Eric V Krieger, Susan M Fernandes
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    ABSTRACT: There are diverse mechanisms by which congenital left-sided cardiac lesions can precipitate heart failure. Left heart outflow obstruction can impose abnormal pressure load on the left ventricle, inducing adverse remodeling, hypertrophy, and diastolic and systolic dysfunction. Abnormalities in left ventricular inflow can increase pulmonary venous pressure and predisposing to pulmonary edema. In addition, inborn abnormalities in left ventricular myocardial structure and function can impair both systolic and diastolic function and manifest as heart failure later in life. In this article, the different mechanisms, outcomes, and treatments of heart failure in patients with congenital left-sided lesions are discussed.
    Heart Failure Clinics 01/2014; 10(1):155-65.
  • Eric V Krieger, Karen K Stout
    Heart (British Cardiac Society) 11/2013; · 5.01 Impact Factor
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    ABSTRACT: The aim of this study was to determine associations between aortic morphometry evaluated by cardiovascular magnetic resonance (CMR) and pregnancy outcomes in women with aortic coarctation (CoA). Consecutive women with CoA seen with CMR within 2 years of delivery were reviewed. Aortic dimensions were measured on CMR angiography. Adverse outcomes (cardiovascular, obstetric, and foetal/neonatal) were documented. We identified 28 women (4 with native and 24 with repaired CoA) who had 30 pregnancies. There were 29 live births (1 stillbirth) at mean gestation 38 ± 2 weeks. Mean maternal ages at first cardiac intervention and pregnancy were 6 ± 8 and 29 ± 6 years, respectively. There were nine cardiovascular events (hypertensive complications in five; stroke in two and arrhythmia in two) occurring in seven pregnancies. Minimum aortic dimensions were smaller in women with cardiovascular events (12.1 vs. 14.3 mm, P = 0.001), specifically in those with hypertensive complications (11.6 vs. 14.4 mm, P < 0.001). From receiver operator curve analysis, optimal discrimination for the development of adverse cardiovascular events occurred at the 12 mm diameter threshold [sensitivity 78%, specificity 91%, area under the curve 0.86 (95% CI: 0.685-1)]. All hypertensive events occurred in conjunction with a minimum aortic diameter of 12 mm (7mm/m(2)) or less. No adverse outcomes occurred if minimum diameter exceeded 15 mm. Smaller aortic dimensions relate to increased risk of hypertensive events in pregnant women with CoA. CMR can aid in stratification of risk for women with CoA who are considering pregnancy.
    European heart journal cardiovascular Imaging. 09/2013;
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    ABSTRACT: OBJECTIVES: To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA). BACKGROUND. : Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease. METHODS. : We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010. RESULTS. : Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement. CONCLUSION. : In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV.
    Congenital Heart Disease 06/2013; · 1.01 Impact Factor
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    ABSTRACT: AIMS: There is a fivefold increase in the frequency of intracranial aneurysm (IA) in adults with coarctation of the aorta (CoA). Current guidelines for management of adults with CoA recommend computed tomography angiography (CTA) or magnetic resonance imaging of the intracranial vessels. However, this recommendation has not been universally accepted. The purpose of our study was to prospectively perform CTA of the intracranial vessels in adults with CoA to evaluate the prevalence and identify high-risk features of this complication. METHODS AND RESULTS: From January 2008 to February 2011, adults ≥18 years of age with CoA were prospectively enrolled in a screening program with CTA of the intracranial vessels. Analyses of prognostic variables were performed with both Fisher's exact and two sample t-test. Forty-three patients (58% female, 33.55 ± 10.21 years) with CoA completed CTA of the intracranial vessels. Five patients (11%) were found to have IA. Patients with IA were older than those without (45.6 ± 8.17 vs. 30.89 ± 7.89, P = 0.0003). There were no statistically significant differences detected between measurements of fasting lipid profiles, C-reactive protein, brain natriuretic peptide, and homocysteine levels among CoA patients with and without IA (P = not significant). CONCLUSION: Prospective screening of adults with CoA confirmed the increased prevalence of IA but also identified increased age as the sole risk factor. These data suggested that screening is justified particularly in the fourth and fifth decades of life. Further studies are required that focus on the development, natural history, and treatment of IA.
    Congenital Heart Disease 12/2012; · 1.01 Impact Factor
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    ABSTRACT: The role of exercise testing to risk stratify patients with repaired coarctation of the aorta (CoA) is controversial. Concentric left ventricular (LV) hypertrophy, defined as an increase in the LV mass-to-volume ratio (MVR), is associated with a greater incidence of adverse cardiovascular events. The objective of the present study was to determine whether a hypertensive response to exercise (HRE) is associated with increased LVMVR in patients with repaired CoA. Adults with repaired CoA who had a symptom-limited exercise test and cardiac magnetic resonance imaging examination within 2 years were identified. A hypertensive response to exercise was defined as a peak systolic blood pressure >220 mm Hg during a symptom-limited exercise test. The LV mass and volume were measured using cardiac magnetic resonance by an investigator who was unaware of patient status. We included 47 patients (median age 27.3 years, interquartile range 19.8 to 37.3), who had undergone CoA repair at a median age of 4.6 years (interquartile range 0.4 to 15.7). Those with (n = 11) and without (n = 36) HRE did not differ in age, age at repair, body surface area, arm-to-leg systolic blood pressure gradient, gender, or peak oxygen uptake with exercise. Those with a HRE had a greater mean systolic blood pressure at rest (146 ± 18 vs 137 ± 18 mm Hg, p = 0.04) and greater median LVMVR (0.85, interquartile range 0.7 to 1, vs 0.66, interquartile range 0.6 to 0.7; p = 0.04) than those without HRE. Adjusting for systolic blood pressure at rest, age, age at repair, and gender, the relation between HRE and LVMVR remained significant (p = 0.001). In conclusion, HRE was associated with increased LVMVR, even after adjusting for multiple covariates.
    The American journal of cardiology 11/2012; · 3.58 Impact Factor
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    ABSTRACT: BACKGROUND: Single ventricle (SV) patients with Fontan physiology have multiple risk factors for liver disease but the prevalence of liver disease remains unknown in this population. We sought to determine whether hospitalized patients with a SV diagnosis have higher rates of nonalcoholic cirrhosis than patients without congenital heart disease. METHODS: We used the 1998-2009 Healthcare Cost and Utilization Project's Nationwide Inpatient Sample, a nationally representative dataset, to identify patients 18-49years old admitted to an acute care hospital. We compared the rate of nonalcoholic cirrhosis between those with a SV diagnosis and patients without congenital heart disease. RESULTS: There were 7968 hospitalizations of patients with a SV diagnosis and 13,602,149 hospitalizations of patients without congenital heart disease. SV patients were more likely to have nonalcoholic cirrhosis than those without congenital heart disease (4.3±0.7 vs. 0.3±0.01%, univariate OR 15.2, 95%CI 10.9-21.3), even after adjusting for viral or chronic hepatitis and other cirrhosis risk factors (multivariable OR 21.6, 95%CI 4.3-32.5). The proportion of all hospitalizations among SV patients for nonalcoholic cirrhosis increased by 173% between 1998/9 and 2008/9, from 2.3% to 6.2% (p=0.009). Among those with nonalcoholic cirrhosis, SV patients were more likely to have congestive hepatopathy (6.6±3.1 vs. 0.1±0.0001%, OR 63.2, 95%CI 19.2-207.8), longer hospital stays and higher hospital charges. CONCLUSIONS: A single ventricle diagnosis is associated with markedly higher risk for nonalcoholic cirrhosis in a population-based sample of hospitalized patients. The proportion of patients with single ventricle anatomy admitted for nonalcoholic cirrhosis or its complications is increasing rapidly.
    International journal of cardiology 06/2012; · 6.18 Impact Factor
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    ABSTRACT: Described is a 42-year-old man with complex congenital heart disease with single-ventricle physiology who underwent a Fontan operation at 27 years of age. He presented with sudden-onset chest pain and dyspnea and was initially misdiagnosed with a pulmonary embolism. This case is described in order to highlight the challenges in the evaluation of common presentations in adults with congenital heart disease.
    Congenital Heart Disease 04/2012; 7(5):E73-7. · 1.01 Impact Factor
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    Journal of Cardiovascular Magnetic Resonance 02/2012; 14 Suppl 1:O68. · 4.44 Impact Factor
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    ABSTRACT: Hypertension is a common consequence of coarctation of the aorta. The frequency of hypertensive complications of pregnancy in women with coarctation in the general population is undefined. In this study, we used the 1998 to 2007 Nationwide Inpatient Sample, a nationally representative data set, to identify patients admitted to an acute care hospital for delivery. The frequency of hypertensive complications of pregnancy was compared between women with and without coarctation. Secondary outcomes, including length of stay, hospital charges, Caesarean delivery, and adverse maternal outcomes, were also assessed. There were an estimated 697 deliveries among women with coarctation, compared to 42,601,409 deliveries by women without coarctation. The frequency of hypertensive complications of pregnancy was 24.1 ± 3.3% for women with coarctation compared to 8.0 ± 0.1% for women without coarctation (multivariate odds ratio [OR] 3.6, 95% confidence interval [CI] 2.5 to 5.2). Preexisting hypertension complicating pregnancy (10.2 ± 2.5% vs 1.0% ± 0.02%, multivariate OR 10.8, 95% CI 5.9 to 19.8) and pregnancy-induced hypertension (13.9 ± 3.0% vs 7.0% ± 0.1%, multivariate OR 2.1, 95% CI 1.3 to 3.3) were more common in women with coarctation. Women with coarctation were more likely to deliver by Caesarean section (41.6 ± 3.3% vs 26.4% ± 0.2%, multivariate OR 2.0, 95% CI 1.4 to 2.8), have adverse cardiovascular outcomes (4.8 ± 2.2% vs 0.3 ± 0.01%, multivariate OR 16.7, 95% CI 6.7 to 41.5), have longer hospital stays, and incur higher hospital charges (both p values <0.0001) than women without coarctation. In conclusion, women with coarctation are more likely to have hypertensive complications of pregnancy, deliver by Caesarean section, have adverse cardiovascular outcomes, have longer hospitalizations, and incur higher hospital charges than women without coarctation.
    The American journal of cardiology 03/2011; 107(10):1529-34. · 3.58 Impact Factor
  • Eric V Krieger, Karen Stout
    Heart (British Cardiac Society) 10/2010; 96(20):1676-81. · 5.01 Impact Factor
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    ABSTRACT: The growing number of adult patients with congenital heart disease represents an evident success of paediatric cardiology. However, these patients continue to present long-term cardiovascular mortality and morbidity. The interplay between aging and vascular health is likely to affect the natural history and clinical effectiveness of cardiovascular care in this group of patients. We review the evidence regarding the epidemiology of cardiovascular risk factors in adult patients with congenital heart disease. Conventional cardiovascular risk factors are present in this population of patients with a prevalence that is higher than expected. The relationship between peculiar anatomy and cardiac physiology in complex congenital heart disease and conventional risk factors has been summarized. Endothelial dysfunction, diastolic dysfunction and high incidence of glucose metabolism impairment will likely have a detrimental effect on surgical palliation or correction of complex congenital heart diseases. Cardiovascular prevention is of primary importance in this group of patients to improve long-term outcome and reduce cardiac morbidity.Received for publication 22 February 2010; accepted for publication 29 March 2010.
    High Blood Pressure & Cardiovascular Prevention 02/2010; 17(1):15-25.