Eric V. Krieger

Prospective Comparison of Valve Regurgitation Quantitation by Cardiac Magnetic Resonance Imaging and Transthoracic Echocardiography

Background—Both transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) imaging allow quantification of chronic aortic regurgitation (AR) and mitral regurgitation (MR). We hypothesized that CMR measurement of regurgitant volume (RVol) is more reproducible than TTE. Methods and Results—TTE and CMR performed on the same day in 57 prospectively enrolled adults (31 with AR, 26 with MR) were measured by 2 independent physicians. TTE RVolAR was calculated as Doppler left ventricular outflow minus inflow stroke volume. RVolMR was calculated by both the proximal isovelocity surface area method and Doppler volume flow at 2 sites. CMR RVolAR was calculated by phase-contrast velocity mapping at the aortic sinuses and RVolMR as total left ventricular minus forward stroke volume. Intraobserver and interobserver variabilities were similar. For AR, the Bland–Altman mean interobserver difference in RVol was −0.7 mL (95% confidence interval [CI], −5 to 4) for CMR and −9 mL (95% CI, −53 to −36) for TTE. The Pearson correlation was higher (P=0.001) between CMR (0.99) than TTE readers (0.89). For MR, the Bland–Altman mean difference in RVol between observers was −4 mL (95% CI, −21 to 13) for CMR compared with 0.7 mL (95% CI, −30 to 32) for the proximal isovelocity surface area and −10 mL (95% CI, −76 to 56) for TTE volume flow at 2 sites. Correlation was similar for CMR (0.94) versus TTE readers (0.90 for the proximal isovelocity surface area). Conclusions—Compared with TTE, CMR has lower intraobserver and interobserver variabilities for RVolAR, suggesting CMR may be superior for serial measurements. Although RVolMR is similar by TTE and CMR, variability in measured RVol by both approaches suggests that caution is needed in clinical practice.

Comparison of Risk of Hypertensive Complications of Pregnancy Among Women With Versus Without Coarctation of the Aorta

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.

Single ventricle anatomy is associated with increased frequency of nonalcoholic cirrhosis.

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 Projects Nationwide Inpatient Sample, a nationally representative dataset, to identify patients 18-49 years 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.

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

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.

Heart failure treatment in adults with congenital heart disease: where do we stand in 2014?

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.

Congestive hepatic fibrosis score: a novel histologic assessment of clinical severity

Chronic right heart failure predisposes to hepatic passive congestion and centrizonal necrosis that may lead to hepatic fibrosis (cardiac sclerosis). Although there have been several studies on the histologic features of congestive hepatopathy, there is no available grading system. In this study we developed a novel grading system for congestive hepatic fibrosis. Liver biopsies were examined in patients with chronic heart failure of various etiologies including congenital heart disease, idiopathic cardiomyopathy, ischemic heart disease, and valvular heart disease. The cases with available echocardiography and/or right heart catheterization were included. Cases with other types of underlying chronic liver diseases, alcoholic liver disease, significant steatosis (>20%), malignant neoplasm, and acute heart failure or shock were excluded. After exclusion, 42 cases were included in the study. We herein proposed a novel congestive hepatic fibrosis score and correlated it with the right heart structure and function obtained by echocardiography and/or right heart catheterization. Our results showed that congestive hepatic fibrosis score is well correlated with the right atrial pressure (P for trend <0.001). The presence of portal fibrosis (congestive hepatic fibrosis scores 2 and 3) is associated with significantly higher right atrial pressure than those with no fibrosis (P<0.001) or with centrizonal fibrosis only (P=0.02). Congestive hepatic fibrosis score is also significantly associated with increasing severity of right atrial dilatation (P=0.03) and right ventricular dilatation (P=0.02), indicators for chronic volume and/or pressure overload. Other histopathologic features include sinusoidal dilatation and centrizonal hepatocyte atrophy. In summary, although sinusoidal dilatation and centrizonal fibrosis are the hallmarks of hepatic passive congestion, the presence of portal fibrosis is suggestive of more advanced disease, as it correlates with more severe impairment of right heart function, regardless of the etiologies of right heart failure. Congestive hepatic fibrosis score is a useful indicator of clinical severity.

Diagnosis and management of ebstein anomaly of the tricuspid valve.

Opinion StatementEbstein anomaly (EA) is a rare congenital heart defect that may not be detected until late in adolescence or adulthood. Since the original description in a 19-year-old laborer with severe tricuspid valve (TV) regurgitation in 1866, our understanding of this rare condition has increased to the recognition that it is an abnormality not only of the TV, but also of the right ventricle (RV). EA is the result of failure of delamination of the TV leaflets from the interventricular septum, resulting in adherence of the leaflets to the underlying myocardium. This results in a wide variety of abnormalities, including apical and posterior displacement of the dilated TV annulus; dilation of the “atrialized” portion of the RV; and fenestrations, redundancy, and tethering of the anterior leaflet of the TV. The malformed TV is usually regurgitant, but may rarely be stenotic. The clinical manifestations of EA in the adult depend on several factors, including the extent of TV leaflet distortion, degree of tricuspid regurgitation (TR), right atrial pressure, and presence of a right-to-left atrial level shunt. Over the past several decades, advances in diagnostic imaging and surgical techniques have contributed to our current management of this challenging congenital heart defect.

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

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.

Quantitation of mitral regurgitation with cardiac magnetic resonance imaging: a systematic review.

In this review discuss the application of cardiac magnetic resonance (CMR) to the evaluation and quantification of mitral regurgitation and provide a systematic literature review for comparisons with echocardiography. Using the 2015 Preferred Reporting Items for Systematic Reviews and Meta-Analyses methodology, we searched Medline and PubMed for original research articles published since 2000 that provided data on the quantification of mitral regurgitation by CMR. We identified 220 articles of which 33 were included. Four main techniques of mitral regurgitation quantification were identified. Reproducibility varied substantially between papers but was high overall for all techniques. However, quantification differed between the techniques studied. When compared with two-dimensional echocardiography, mitral regurgitation fraction and regurgitant volume measured by CMR were comparable but typically lower. CMR has high reproducibility for the quantification of mitral regurgitation in experienced centres, but further technological refinement is needed. An integrated and standardised approach that combines multiple techniques is recommended for optimal reproducibility and precise mitral regurgitation quantification. Definitive outcome studies using CMR as a basis for treatment are lacking but needed.

Cardiovascular magnetic resonance imaging predictors of pregnancy outcomes in women with coarctation of the aorta

AIMS 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). METHODS 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. RESULTS 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. CONCLUSION 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.