Hypereosinophilic syndrome (HES) is a heterogeneous group of conditions that is defined at its core by hypereosinophilia (HE) (blood eosinophil count of >1.5×10(9)/L) and organ damage directly attributable to the HE. Cardiac dysfunction occurs frequently in all forms of HES and is a major cause of morbidity and mortality. Once a significantly elevated eosinophil count is identified, it must be confirmed on repeat testing and the aetiology for the HE must be rigorously sought out with a focus on identifying whether organ dysfunction is occurring. Echocardiography is routinely performed to assess for cardiac involvement, looking for evidence of left ventricular and/or right ventricular apical obliteration or thrombi or a restrictive cardiomyopathy. Cardiac magnetic resonance imaging and CT are often useful adjuncts to establish the diagnosis but endomyocardial biopsy remains the gold standard. To decrease the degree of eosinophilia, treatment can include corticosteroids and/or imatinib based on the aetiology. Anticoagulation, standard heart failure therapy for a restrictive cardiomyopathy and finally cardiac transplantation may be indicated in the treatment algorithm.

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To evaluate 2-dimensional speckle tracking echocardiography as a diagnostic and prognostic tool in patients with acute myocarditis. In this retrospective cohort study, 45 patients (age, 39 ± 15 years; 32 male) with suspected acute myocarditis and 83 healthy controls (age, 39 ± 13 years; 27 male) underwent 2-dimensional speckle tracking echocardiography. Main outcome measures were circumferential and longitudinal strain and strain rate as prognostic and diagnostic markers. Patients with myocarditis had lower circumferential strain (-13.3 ± 5.6 % vs. -22.3 ± 4 %), circumferential strain rate (-0.9 ± 0.3 vs. -1.4 ± 0.3 s(-1)), longitudinal strain (-11.7 ± 4 % vs. -17.7 ± 1.9 %), and longitudinal strain rate (-0.7 ± 0.2 vs. -1.0 ± 0.1 s(-1)) (all P < .001). For diagnostic purposes, longitudinal strain had the greatest area under the curve, 0.93 (optimal cutoff value, -15.1 %; sensitivity, 78 %; specificity, 93 %). Future events were defined as cardiac death, heart transplant, placement of left ventricular assist device or implantable cardioverter-defibrillator, pulmonary edema-related respiratory failure, cardiogenic shock, and rehospitalization due to cardiac events. For every 1 % decline in longitudinal or circumferential strain, the hazard ratios (95 % CIs) were 1.26 (1.10-1.47) and 1.34 (1.14-1.63), respectively; for every 0.1 s(-1) decline in longitudinal or circumferential strain rate, the hazard ratios (95 % CIs) were 1.43 (1.09-1.89) and 1.52 (1.19-2.01), respectively (P< .01). Kaplan-Meier curve and log-rank test showed event-free survival significantly related to these 4 measurements. In acute myocarditis, left ventricular strain and strain rate may be promising diagnostic and prognostic tools, even in patients with preserved left ventricular ejection fraction. Most importantly, this imaging technique had a role in predicting deterioration and overall event-free survival.

Prognosis and therapeutic decisions are often based on left ventricular ejection fraction (LVEF), which means the LVEF needs to be accurately measured. Many imaging modalities can measure LVEF. Each of these modalities is subject to measurement errors that can lead to the inaccurate calculation of LVEF. This article reviews the most common non-invasive imaging modalities – i.e., echocardiography, magnetic resonance imaging (MRI), computed tomography (CT), radionuclide angiography, gated myocardial perfusion single-photon emission computed tomography (SPECT) and gated myocardial perfusion positron emission tomography (PET) – used to measure LVEF, as well as the common sources of error with each of them. It is important to understand these sources of errors in order to prevent them, and recognise them when they do occur so that they can be corrected if possible.

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PPV/RVPAC IE is associated with significant morbidity, mortality and high risk of requiring operative intervention. TTE and TEE are marginal diagnostic tools when used independently; they should be used as complementary techniques in the evaluation of those patients. Severe PPV/RVPAC stenosis was more common than regurgitation in patients with IE; thus IE should be considered in patients presenting with new PPV/RVPAC obstruction.

Objective. The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation. Design. We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database. A follow-up questionnaire was mailed to all patients not known to be deceased at the time of study. Patients with available contraception and pregnancy data were included in the study. Results. Of the 138 women with available contraception data, 44% used no contraception, 12% each used barrier methods, combination hormone therapy or sterilization, 8% used Depo-Provera, 7% had intrauterine devices, 4% had a partner with a vasectomy and 1% used progestin pills. Six women had thrombotic complications (only one using oral contraceptives). Thirty-five women had pregnancy data available. Prior to the Fontan operation there were 10 pregnancies (8 miscarriages, 2 therapeutic abortions, and no live births). After the Fontan operation there were 70 pregnancies resulting in 35 miscarriages (50%), 29 live births (41%), and 6 therapeutic abortions (9%). There were no maternal deaths during pregnancy. During long-term follow up (26 ± 6 years since the Fontan), 1 death, and 1 cardiac transplant occurred. Mean gestational age of the newborns (n = 22/29) was 33.1 ± 4.0 weeks; mean birth weight (n = 20/29) was 2086 ± 770 g. There was 1 neonatal death because of prematurity and two children were born with congenital heart disease (one patent ductus arteriosus and one membranous ventricular septal defect). Conclusions. Pregnancy after the Fontan operation is associated with a high rate of miscarriages, preterm delivery, and low birth weight. Further studies are needed to identify specific variables influencing risk stratification of pregnancy in this patient population.

Objective To evaluate the utility of magnetic resonance elastography (MRE) in screening patients for hepatic fibrosis, cirrhosis, and hepatocellular carcinoma after Fontan operation. Patients and Methods Hepatic MRE was performed in conjunction with cardiac magnetic resonance imaging in patients with prior Fontan operation between 2010-2014. Liver stiffness was calculated using previously reported techniques. Comparisons to available clinical, laboratory, imaging, and histopathologic data were made. Results Overall, 50 Fontan patients aged 25 (21-33) years were evaluated. The median interval between Fontan creation and MRE was 22 (16-26) years. Mean liver stiffness values were increased; 5.5±1.4 kilopascals relative to normal subjects. Liver stiffness directly correlated with liver biopsy total fibrosis score, time since operation; mean Fontan pressure; γ-glutamyl transferase (GGT); MELD score; creatinine; and pulmonary vascular resistance index (PVR). Liver stiffness was inversely correlated with cardiac index (CI). All 3 subjects with hepatic nodules exhibiting decreased contrast uptake on delayed post-contrast imaging and elevated nodule stiffness had biopsy-proven hepatocellular carcinoma. Conclusion The association between hepatic stiffness and fibrosis scores, MELD scores, and GGT, suggests that MRE may be useful to detect (and possibly quantify) hepatic cirrhosis in Fontan patients. Correlation between stiffness and post-Fontan time interval, mean Fontan pressure, PVR and reduced CI suggests a role for long term hepatic congestion in creating these hepatic abnormalities. MRE was useful in detecting abnormal nodules ultimately diagnosed as hepatocellular carcinoma. The relationship between stiffness with advanced fibrosis and hepatocellular carcinoma provides a strong argument for additional study and broader application of MRE in these patients.