Publications by authors named "Eric Krieger"

60 Publications

AHA/ACC vs ESC Guidelines for Management of Adults With Congenital Heart Disease: JACC Guideline Comparison.

J Am Coll Cardiol 2021 Nov;78(19):1904-1918

Cincinnati Children's Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA. Electronic address:

The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications.
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http://dx.doi.org/10.1016/j.jacc.2021.09.010DOI Listing
November 2021

How to write an image challenge multiple choice question.

Authors:
Eric V Krieger

Heart 2021 Nov 16;107(21):1762-1763. Epub 2021 Aug 16.

Cardiac Clinic and Services, University of Washington Medical Center, Seattle, Washington, USA

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http://dx.doi.org/10.1136/heartjnl-2021-319175DOI Listing
November 2021

Long-term adult congenital heart disease survival after heart transplantation: A restricted mean survival time analysis.

J Heart Lung Transplant 2021 07 13;40(7):698-706. Epub 2021 Mar 13.

Department of Medicine, Division of Cardiology, University of Washington School of Medicine, Seattle Washington.

Background: Adult Congenital Heart Disease (ACHD) heart transplant recipients may have lower post-transplant survival resulting from higher peri-operative mortality than non-ACHD patients. However, the late risk of mortality appears lower in ACHD recipients. This study seeks to establish whether long-term heart transplant survival is reduced among ACHD recipients relative to non-ACHD recipients.

Methods: Adult patients who received a heart transplant between January, 2000 and December, 2019 in the United Network for Organ Sharing database were stratified by the presence of ACHD. Propensity-matched cohorts (1:4) were created to adjust for differences between groups. Graft survival at time points from 1 to 18 years was compared between groups using restricted mean survival time (RMST) analysis.

Results: The matched cohort included 1,139 ACHD and 4,293 non-ACHD patients. Median age was 35 years and 61% were male. Average survival time at 1 year was 0.85 years for ACHD patients and 0.93 years for non-ACHD patients (average difference: -0.08 years, 95% Confidence Interval [CI] -0.10 to -0.06, p < 0.001), reflecting higher immediate post-transplant mortality. Average survival time at 18 years was not clinically or statistically different: 11.14 years for ACHD patients and 11.40 years for non-ACHD patients (average difference: -0.26 years, 95% CI: -0.85 to + 0.32 years, p = 0.38).

Conclusions: Despite increased medium-term mortality among ACHD patients after heart transplant, differences in long-term survival are minimal. Allocation of hearts to ACHD patients results in acceptable utility of donor hearts.
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http://dx.doi.org/10.1016/j.healun.2021.02.019DOI Listing
July 2021

Heart murmur in a young man.

Heart 2021 May;107(9):733-770

Cardiac Clinic and Services, University of Washington Medical Center, Seattle, Washington, USA

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http://dx.doi.org/10.1136/heartjnl-2020-318834DOI Listing
May 2021

Management of Women With Congenital or Inherited Cardiovascular Disease From Pre-Conception Through Pregnancy and Postpartum: JACC Focus Seminar 2/5.

J Am Coll Cardiol 2021 04;77(14):1778-1798

Cardiovascular Medicine, University of Florida College of Medicine, Gainesville, Florida, USA.

Maternal morbidity and mortality continue to rise in the United States, with cardiovascular disease as the leading cause of maternal deaths. Congenital heart disease is now the most common cardiovascular condition encountered during pregnancy, and its prevalence will continue to grow. In tandem with these trends, maternal cardiovascular health is becoming increasingly complex. The identification of women at highest risk for cardiovascular complications is essential, and a team-based approach is recommended to optimize maternal and fetal outcomes. This document, the second of a 5-part series, will provide practical guidance from pre-conception through postpartum for cardiovascular conditions that are predominantly congenital or heritable in nature, including aortopathies, congenital heart disease, pulmonary hypertension, and valvular heart disease.
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http://dx.doi.org/10.1016/j.jacc.2021.02.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8061781PMC
April 2021

COVID-19 in Adults With Congenital Heart Disease.

J Am Coll Cardiol 2021 04;77(13):1644-1655

Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, USA.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.

Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.

Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined.

Results: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not.

Conclusions: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.
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http://dx.doi.org/10.1016/j.jacc.2021.02.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8006800PMC
April 2021

The heart as a spring, the measurement of myocardial bounce to assess left ventricular function on cardiac MR.

Int J Cardiovasc Imaging 2021 May 23;37(5):1699-1707. Epub 2021 Feb 23.

Department of Radiology, University of Washington Medical Center, Seattle, WA, USA.

Little has been reported on the left ventricular myocardial distension (bounce) and its utility to assess cardiac function. The purpose of this study is to determine whether myocardial bounce at end diastole is reproducibly visualized by blinded observers and to determine whether it corresponds to systolic and diastolic function. 144 Consecutive cardiac MR exams between September and December 2017 were selected for analysis. The bounce was graded by two blinded observers, and the change in LV diameter pre and post bounce was measured. The bounce was defined as the rapid change in LV volume that occurs at the end of diastole during atrial contraction just prior to systolic ejection. Inter-reader agreement was summarized using Cohen's kappa. Spearman's rank correlation coefficient was used to evaluate associations between bounce grade and cardiac physiology parameters. Overall agreement was good with unweighted kappa = 0.69 (95% CI 0.60-0.79). Bounce grade was significantly correlated with the average change in LV diameter before and after the bounce (Spearman's rho = 0.76, p < 0.001). Median diameter changes were 0.0, 1.9, and 4.2 mm in grades 0 (no bounce), 1 (small bounce), and 2 (normal), respectively. The bounce lasted 8 to 12 ms in all patients. Bounce grade was significantly correlated with LV EF (Spearman's rho = 0.43, p < 0.001). Median EF was 44%, 51%, and 58% in grades 0, 1, and 2, respectively. Of the 87 patients who had E/A ratio or E/e' ratio measured, bounce grade was also significantly correlated with E/A ratio (r =  - 0.24, p = 0.034) and E/e' ratio (r =  - 0.24, p = 0.022), with lower grades having higher ratio values on average (Table 4). Of the 15 patients with a bounce grade of 0 by one or both readers and EF ≥ 50%, 8 had E/A ratio measurements and 7 had E/e' ratio measurements. The E/A ratio values ranged from 1 to 2.7 (median 1.5). The E/e' ratio values ranged from 4.8 to 9.6 (median 7.7). The simple observation of a normal myocardial bounce during cine loop review of cardiac MR exams was predictive of normal diastolic and systolic cardiac function. Lack of myocardial bounce was highly associated with both systolic and diastolic dysfunction. The subpopulation of patients with loss of myocardial bounce and normal ejection fraction appear to represent patients with early diastolic dysfunction. Further studies with more diastolic dysfunction MRs are needed to examine this relationship. This study suggests changes to the myocardial bounce seen on cardiac MR may be a simple useful tool for detecting cardiac dysfunction. This study is not to replace, but rather aid the clinical diagnosis and management of both diastolic and systolic dysfunction.
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http://dx.doi.org/10.1007/s10554-020-02129-4DOI Listing
May 2021

2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

J Am Coll Cardiol 2021 02 17;77(4):450-500. Epub 2020 Dec 17.

Aim: This executive summary of the valvular heart disease guideline provides recommendations for clinicians to diagnose and manage valvular heart disease as well as supporting documentation to encourage their use.

Methods: A comprehensive literature search was conducted from January 1, 2010, to March 1, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, Cochrane, Agency for Healthcare Research and Quality Reports, and other selected database relevant to this guideline.

Structure: Many recommendations from the earlier valvular heart disease guidelines have been updated with new evidence and provides newer options for diagnosis and treatment of valvular heart disease. This summary includes only the recommendations from the full guideline which focus on diagnostic work-up, the timing and choice of surgical and catheter interventions, and recommendations for medical therapy. The reader is referred to the full guideline for graphical flow charts, text, and tables with additional details about the rationale for and implementation of each recommendation, and the evidence tables detailing the data considered in developing these guidelines.
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http://dx.doi.org/10.1016/j.jacc.2020.11.035DOI Listing
February 2021

2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Circulation 2021 Feb 17;143(5):e35-e71. Epub 2020 Dec 17.

Aim: This executive summary of the valvular heart disease guideline provides recommendations for clinicians to diagnose and manage valvular heart disease as well as supporting documentation to encourage their use.

Methods: A comprehensive literature search was conducted from January 1, 2010, to March 1, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, Cochrane, Agency for Healthcare Research and Quality Reports, and other selected database relevant to this guideline. Structure: Many recommendations from the earlier valvular heart disease guidelines have been updated with new evidence and provides newer options for diagnosis and treatment of valvular heart disease. This summary includes only the recommendations from the full guideline which focus on diagnostic work-up, the timing and choice of surgical and catheter interventions, and recommendations for medical therapy. The reader is referred to the full guideline for graphical flow charts, text, and tables with additional details about the rationale for and implementation of each recommendation, and the evidence tables detailing the data considered in developing these guidelines.
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http://dx.doi.org/10.1161/CIR.0000000000000932DOI Listing
February 2021

Ebstein's Anomaly of the Tricuspid Valve: an Overview of Pathology and Management.

Curr Cardiol Rep 2020 10 9;22(12):157. Epub 2020 Oct 9.

Division of Cardiology, Department of Medicine, University of Washington, 1959 Pacific Street, Box 356422, Seattle, WA, 98195, USA.

Purpose Of Review: Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population.

Recent Findings: Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence. Despite an improvement in understanding and recognition of EA, significant disease heterogeneity and complex treatment options continue to challenge providers, with the best outcomes achieved at expert congenital heart disease centers.
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http://dx.doi.org/10.1007/s11886-020-01412-zDOI Listing
October 2020

Acute Increase in Deaths Among Patients With Adult Congenital Heart Disease During COVID-19: Single-Center Experience.

JACC Case Rep 2020 Jul 13;2(9):1275-1278. Epub 2020 Jun 13.

Division of Cardiology, Department of Medicine, University of Washington Heart Institute, Seattle, Washington.

Fear of acquiring severe acute respiratory syndrome coronavirus-2 infection is a major contributor to underutilization of the health care system during the current pandemic. In this report, we describe 4 cases of unexpected deaths that occurred within a short time period in patients with adult congenital heart disease without warning symptoms. ().
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http://dx.doi.org/10.1016/j.jaccas.2020.06.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7293490PMC
July 2020

Cardiovascular outcomes of pregnancy in Turner syndrome.

Heart 2021 01 15;107(1):61-66. Epub 2020 Jul 15.

Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands.

Objectives: Women with Turner syndrome (TS) are frequently counselled against pregnancy due to lack of data and unclear aortic dissection risk. However, with advances in fertility therapy, more women with TS are contemplating pregnancy. This study compared rates of adverse cardiovascular (CV) outcomes among: (1) pregnant and non-pregnant women with TS and (2) pregnant women with TS with/without structural heart disease.

Methods: Retrospective analysis of pregnant and age-matched non-pregnant controls with TS (2005-2017) across 10 CV centres was done. Data were collected at initial evaluation in pregnancy and outcomes were assessed to 6 months postpartum. Adverse CV events were defined as CV death, aortic dissection/rupture and/or aortic intervention. Non-pregnant age-matched controls were followed over the same time period.

Results: Sixty-eight pregnancies were included (60 women, mean age 33 years, 48% primigravid, 49% fertility therapy, 80% structurally normal heart, 25% XO karyotype). Based on American Society of Reproductive Medicine criteria, 10 pregnancies occurred in women stratified to high-risk category. There were no CV events in the pregnant women or in the non-pregnant women with TS. Obstetric events complicated 12 (18%) pregnancies with 9 (13%) attributed to hypertensive disorder of pregnancy. Fetal events included small for gestational age neonates (18%), preterm delivery (15%) and fetal death (3%).

Conclusions: This study helps to refine the approach to pregnancy in women with TS. Among women with TS without structural heart disease, pregnancy does not impose an increased risk of CV outcomes. Among women with TS with structural heart disease, the risk of pregnancy is not as prohibitive as previously described but does require ongoing evaluation.
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http://dx.doi.org/10.1136/heartjnl-2020-316719DOI Listing
January 2021

Tetralogy of Fallot.

Cardiol Clin 2020 Aug 10;38(3):365-377. Epub 2020 Jun 10.

Department of Cardiology, Boston Children's Hospital, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Bader 208, Boston, MA 02115, USA. Electronic address:

Repaired tetralogy of Fallot is one of the most common conditions managed by adult congenital heart disease providers. Recent comprehensive review articles and book chapters are devoted to this topic. The purpose of this article is to address several common clinical questions encountered in the management of patients with repaired tetralogy of Fallot. These answers are not intended to supplant Practice Guidelines.
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http://dx.doi.org/10.1016/j.ccl.2020.04.009DOI Listing
August 2020

Chest pressure and dyspnoea in a 47-year-old man.

Heart 2020 Feb;106(4):260-298

Cardiology, University of Washington, Seattle, Washington, USA.

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http://dx.doi.org/10.1136/heartjnl-2019-316086DOI Listing
February 2020

Improved Outcomes of Heart Transplantation in Adults With Congenital Heart Disease Receiving Regionalized Care.

J Am Coll Cardiol 2019 12;74(23):2908-2918

Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington; Division of Cardiology, Seattle Children's Hospital, Seattle, Washington.

Background: The number of adult congenital heart disease (CHD) patients undergoing heart transplantation is increasing rapidly. CHD patients have higher surgical risk at transplantation. High-volume adult CHD transplant centers may have better transplant outcomes.

Objectives: This study aimed to evaluate the effect of center CHD transplant volume and expertise on transplant outcomes in CHD patients.

Methods: The authors studied heart transplantations in CHD patients age ≥18 years using the United Network of Organ Sharing (UNOS) database for the primary outcomes of waitlist mortality and post-transplant outcomes at 30 days and 1 year. Transplant centers were assessed by status as the highest CHD transplant volume center in a UNOS region versus all others, presence of Adult Congenital Heart Association accreditation, and adult versus pediatric hospital designation.

Results: Between January of 2000 and June of 2018, 1,746 adult CHD patients were listed for transplant; 1,006 (57.6%) of these underwent heart transplantation. After adjusting for age, sex, listing status, and inotrope requirement, waitlist mortality risk was lower at Adult Congenital Heart Association accredited centers (hazard ratio: 0.730; p = 0.020). Post-transplant 30-day mortality was lower at the highest volume CHD transplant center in each UNOS region (hazard ratio: 0.706; p = 0.014).

Conclusions: Designated expertise in CHD care is associated with improved waitlist outcomes for CHD patients listed for transplantation. Post-transplant survival was improved at the highest volume regional center. These findings suggest a possible advantage of regionalization of CHD transplantation.
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http://dx.doi.org/10.1016/j.jacc.2019.09.062DOI Listing
December 2019

"Unstable angina" in a man aged 53 years.

Heart 2019 09 1;105(18):1431-1446. Epub 2019 Jun 1.

Department of Medicine, Division of Cardiology, University of Washington, Seattle, Washington, USA.

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http://dx.doi.org/10.1136/heartjnl-2018-314651DOI Listing
September 2019

Predictors of extracorporeal membrane oxygenation support after surgery for adult congenital heart disease in children's hospitals.

Congenit Heart Dis 2019 Jul 5;14(4):559-570. Epub 2019 Mar 5.

Division of Cardiology, Seattle Children's Hospital, Seattle, Washington.

Objective: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals.

Design: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups: ECMO-free survival, requiring ECMO, and DWE. Univariate analyses were performed, and multinomial logistic regression models were constructed examining ECMO and DWE as independent outcomes.

Setting: Tertiary care children's hospitals.

Results: A total of 4665 adult patients underwent ACHD surgery in 39 children's hospitals with 51 (1.1%) patients requiring ECMO and 64 (1.4%) patients experiencing DWE. Of the 51 ECMO patients, 34 (67%) died. Increasing patient age, surgical complexity, diagnosis of single ventricle heart disease, preoperative hospitalization, and the presence of noncardiac complex chronic conditions (CCC) were risk factors for both outcomes. Additionally, low and medium hospital ACHD surgical volume was associated with an increased risk of DWE in comparison with ECMO.

Conclusions: There are overlapping but separate risk factors for ECMO support and DWE among adults undergoing congenital heart surgery in pediatric hospitals.
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http://dx.doi.org/10.1111/chd.12758DOI Listing
July 2019

The Use of Transesophageal Echocardiography in the Management of Baffle Leaks in a Patient With Transposition of the Great Arteries.

A A Pract 2018 Nov;11(10):282-284

Cardiology Division, Department of Medicine, University of Washington, Seattle, Washington.

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http://dx.doi.org/10.1213/XAA.0000000000000805DOI Listing
November 2018

Histamine H Receptor Polymorphisms, Myocardial Transcripts, and Heart Failure (from the Multi-Ethnic Study of Atherosclerosis and Beta-Blocker Effect on Remodeling and Gene Expression Trial).

Am J Cardiol 2018 01 20;121(2):256-261. Epub 2017 Oct 20.

Department of Medicine, University of Colorado, Denver, Colorado.

Myocardial H receptor activation contributes to heart failure (HF) in preclinical models, and H receptor antagonists are associated with decreased HF incidence. This study evaluated whether H histamine receptor (HRH2) single nucleotide polymorphisms (SNPs) are associated with HF incidence and whether myocardial transcript abundance is associated with HF recovery. The association of SNPs in HRH2 with incident HF was characterized using Cox proportional hazards regression among participants in the Multi-Ethnic Study of Atherosclerosis. Differences in myocardial HRH2 transcripts were characterized in participants with dilated cardiomyopathy comparing 6 "super-responders" with 6 nonresponders to β blockade in the Beta-Blocker Effect on Remodeling and Gene Expression Trial. In MESA, no candidate SNP was associated with HF in black, Hispanic, or white participants. The rs2241562 minor allele was present only in Chinese participants and the adjusted HF hazard among those with 1 or more copies of this allele was 3.7, 95% confidence interval 1.0 to 13.4. In BORG, super-responders to β blockade had higher levels of myocardial HRH2 transcript at baseline compared with nonresponders (fragments per kilobase per transcript per million mapped reads: Variant 2, 5.5 ± 1.1 compared with 3.2 ± 0.8 in nonresponders, p = 0.002; Variant 1 + 2, 32.1 ± 7.4 compared with 23.3 ± 4.2 in nonresponders, p = 0.04). In conclusion, the presence of a minor allele at rs2241562 was associated with increased HF incidence in Chinese participants. Differences in myocardial HRH2 transcript abundance were seen in participants with dilated cardiomyopathy who responded to β blockade. These observations support the hypothesis that HRH2 is involved in the pathogenesis of HF.
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http://dx.doi.org/10.1016/j.amjcard.2017.10.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5742297PMC
January 2018

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Circulation 2017 Nov 9;136(20):e348-e392. Epub 2017 Oct 9.

Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.
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http://dx.doi.org/10.1161/CIR.0000000000000535DOI Listing
November 2017

The Role of Pulmonary Scintigraphy in the Evaluation of Adults with Congenital Heart Disease.

Semin Nucl Med 2017 11 12;47(6):660-670. Epub 2017 Aug 12.

Department of Radiology, Division of Nuclear Medicine, University of Washington, Seattle, WA.

Adults with congenital heart disease represent a growing population with challenging and complex medical management. Pulmonary scintigraphy can play a valuable role in the evaluation and care of this patient population. We present a review of the variety of clinical scenarios where pulmonary scintigraphy can be helpful in the evaluation of adults with congenital heart disease, along with technical considerations associated with these studies.
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http://dx.doi.org/10.1053/j.semnuclmed.2017.07.006DOI Listing
November 2017

Exertional dyspnoea in a 28-year-old woman.

Heart 2017 11 27;103(22):1779-1829. Epub 2017 Sep 27.

Department of Medicine, Division of Cardiology, University of Washington Medical Center, Seattle, Washington, USA.

Clinical Introduction: A 28-year-old woman with a history of critical pulmonic stenosis, status postsurgical valvotomy and subsequent pulmonary valve replacement, presented to the cardiology clinic with 1 year of progressive exertional dyspnoea. She has a heart rate of 75 bpm and blood pressure of 110/55 mm Hg. Cardiac auscultation reveals a 1/6 systolic ejection murmur along the left sternum and an early 3/6 diastolic decrescendo murmur. A transthoracic echocardiogram is obtained (figure 1).

Questions: Which of the following would be most likely found during right heart catheterisation?Ratio of pulmonary to systemic blood flow (Qp:Qs) >1.5Pulmonary vascular resistance >3 Wood unitsRight atrial pressure >10mm HgPulmonary artery systolic pressure >45mm Hg E. Pulmonary artery diastolic pressure <10mm Hg.
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http://dx.doi.org/10.1136/heartjnl-2017-312174DOI Listing
November 2017

Bicuspid aortic valve type: it takes two.

Heart 2018 04 27;104(7):544-545. Epub 2017 Sep 27.

Division of Cardiology, Massachusetts General Hospital, Boston, Massachusetts, USA.

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http://dx.doi.org/10.1136/heartjnl-2017-312133DOI Listing
April 2018

Evaluation of aortic regurgitation with cardiac magnetic resonance imaging: a systematic review.

Heart 2018 01 19;104(2):103-110. Epub 2017 Aug 19.

Division of Cardiology, Department of Medicine, University of Washington, Seattle, Washington, USA.

This review summaries the utility, application and data supporting use of cardiac magnetic resonance imaging (CMR) to evaluate and quantitate aortic regurgitation. We systematically searched Medline and PubMed for original research articles published since 2000 that provided data on the quantitation of aortic regurgitation by CMR and identified 11 articles for review. Direct aortic measurements using phase contrast allow quantitation of volumetric flow across the aortic valve and are highly reproducible and accurate compared with echocardiography. However, this technique requires diligence in prescribing the correct imaging planes in the aorta. Volumetric analytic techniques using differences in ventricular volumes are also highly accurate but less than phase contrast techniques and only accurate when concomitant valvular disease is absent. Comparison of both aortic and ventricular data for internal data verification ensures fidelity of aortic regurgitant data. CMR data can be applied to many types of aortic valve regurgitation including combined aortic stenosis with regurgitation, congenital valve diseases and post-transcatheter valve placement. CMR also predicts those patients who progress to surgery with high overall sensitivity and specificity. Future studies of CMR in patients with aortic regurgitation to quantify the incremental benefit over echocardiography as well as prediction of cardiovascular events are warranted.
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http://dx.doi.org/10.1136/heartjnl-2016-310819DOI Listing
January 2018

Durable mechanical circulatory support in teenagers and adults with congenital heart disease: A systematic review.

Int J Cardiol 2017 Oct 2;245:135-140. Epub 2017 Aug 2.

University of Washington, Division of Cardiothoracic Surgery, Seattle, WA, United States.

Background: Heart failure is the leading cause of morbidity and mortality for adults with congenital heart disease (ACHD). Many patients are ineligible for transplantation, and those who are eligible often face long wait times with high wait-list morbidity. Durable mechanical circulatory support (MCS) may be an option for many patients. This systematic review evaluates the published literature on the use of durable MCS in teenagers and adults with congenital heart disease.

Methods: A comprehensive search of MEDLINE (PubMed), EMBASE, and the Cochrane Library was performed electronically in July 2015 and updated in March 2016, guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines.

Results: Individual case reports and several case series identified 66 patients with ACHD treated with durable MCS. More than half were INTERMACS 1 or 2 at the time of implantation. Patients with Fontan repairs were more frequently classified as INTERMACS 1 or 2 (89% compared to 59% or less among other groups). Cases published after 2010 showed a trend toward less severe INTERMACS status, and patients were less likely to have received transplants by the time of reporting (31% compared to 61% prior). Durable MCS was implanted as bridge-to-transplant in 77%. Patients with Fontan repair accounted for 14% of cases.

Conclusion: Reports of durable MCS utilization in patients with ACHD are becoming more frequent and devices are being implanted in more stable patients. Reports are mostly case reports or small case series so reporting bias is likely and prospective protocoled reporting is needed.
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http://dx.doi.org/10.1016/j.ijcard.2017.07.107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5667943PMC
October 2017

Maternal and Fetal Outcomes of Anticoagulation in Pregnant Women With Mechanical Heart Valves.

J Am Coll Cardiol 2017 Jun;69(22):2681-2691

Division of Cardiology, Department of Medicine, University of Washington School of Medicine, Seattle, Washington.

Background: Anticoagulation for mechanical heart valves during pregnancy is essential to prevent thromboembolic events. Each regimen has drawbacks with regard to maternal or fetal risk.

Objectives: This meta-analysis sought to estimate and compare the risk of adverse maternal and fetal outcomes in pregnant women with mechanical heart valves who received different methods of anticoagulation.

Methods: Studies were identified using a Medline search including all publications up to June 5, 2016. Study inclusion required reporting of maternal death, thromboembolism, and valve failure, and/or fetal spontaneous abortion, death, and congenital defects in pregnant women treated with any of the following: 1) a vitamin K antagonist (VKA) throughout pregnancy; 2) low-molecular-weight heparin (LMWH) throughout pregnancy; 3) LMWH for the first trimester, followed by a VKA (LMWH and VKA); or 4) unfractionated heparin for the first trimester, followed by a VKA (UFH and VKA).

Results: A total of 800 pregnancies from 18 publications were included. Composite maternal risk was lowest with VKA (5%), compared with LMWH (16%; ratio of averaged risk [RAR]: 3.2; 95% confidence interval [CI]: 1.5 to 7.5), LMWH and VKA (16%; RAR: 3.1; 95% CI: 1.2 to 7.5), or UFH and VKA (16%; RAR: 3.1; 95% CI: 1.5 to 7.1). Composite fetal risk was lowest with LMWH (13%; RAR: 0.3; 95% CI: 0.1 to 0.8), compared with VKA (39%), LMWH and VKA (23%), or UFH and VKA (34%). No significant difference in fetal risk was observed between women taking ≤5 mg daily warfarin and those with an LMWH regimen (RAR: 0.9; 95% CI: 0.3 to 2.4).

Conclusions: VKA treatment was associated with the lowest risk of adverse maternal outcomes, whereas the use of LMWH throughout pregnancy was associated with the lowest risk of adverse fetal outcomes. Fetal risk was similar between women taking ≤5 mg warfarin daily and women treated with LMWH.
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http://dx.doi.org/10.1016/j.jacc.2017.03.605DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5457289PMC
June 2017
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