Peter Lauerer

The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities.

Background—The classification of clinical severity of Ebstein anomaly still remains a challenge. The aim of this study was to focus on the interaction of the pathologically altered right heart with the anatomically—supposedly—normal left heart and to derive from cardiac magnetic resonance (CMR) a simple imaging measure for the clinical severity of Ebstein anomaly. Methods and Results—Twenty-five patients at a mean age of 26±14 years with unrepaired Ebstein anomaly were examined in a prospective study. Disease severity was classified using CMR volumes and functional measurements in comparison with heart failure markers from clinical data, ECG, laboratory and cardiopulmonary exercise testing, and echocardiography. All examinations were completed within 24 hours. A total right/left-volume index was defined from end-diastolic volume measurements in CMR: total right/left-volume index=(RA+aRV+fRV)/(LA+LV). Mean total right/left-volume index was 2.6±1.7 (normal values: 1.1±0.1). This new total right/left-volume index correlated with almost all clinically used biomarkers of heart failure: brain natriuretic peptide (r=0.691; P=0.0003), QRS (r=0.432; P=0.039), peak oxygen consumption/kg (r=−0.479; P=0.024), ventilatory response to carbon dioxide production at anaerobic threshold (r=0.426; P=0.048), the severity of tricuspid regurgitation (r=0.692; P=0.009), tricuspid valve offset (r=0.583; P=0.004), and tricuspid annular plane systolic excursion (r=0.554; P=0.006). Previously described severity indices ([RA+aRV]/[fRV+LA+LV]) and fRV/LV end-diastolic volume corresponded only to some parameters. Conclusions—In patients with Ebstein anomaly, the easily acquired index of right-sided to left-sided heart volumes from CMR correlated well with established heart failure markers. Our data suggest that the total right/left-volume index should be used as a new and simplified CMR measure, allowing more accurate assessment of disease severity than previously described scoring systems.

Successful treatment of a large hemangioma with propranolol

Hemangiomas are the most common vascular tumors in children. They occur in 8–12% of all infants and in 22% of premature infants (female: male = 3: 1). Hemangiomas are usually sporadic; their etiology is unknown [ 1 ].

BNP and haematological parameters are markers of severity of Ebstein's anomaly: correlation with CMR and cardiopulmonary exercise testing

AIMS Ebsteins anomaly (EA) involves a displaced and dysplastic tricuspid valve resulting in an atrialized portion of the right ventricle and an enlargement of the functional right ventricle and right atrium. Biomarkers targeting heart failure such as brain natriuretic peptide (BNP) or haematological parameters [haemoglobin (Hb) and haematocrit (Hct)] are upregulated in states of pulmonary hypoperfusion. We hypothesized that decreased pulmonary perfusion dependent on the stage of right heart failure is a possible mechanism in EA, and that it can be correlated with cardiac magnetic resonance (CMR) parameters. The aim of this study was to investigate the relationship between BNP and haematological parameters with functional parameters from CMR and exercise testing in patients with EA. METHODS AND RESULTS Twenty-five patients with non-corrected EA were studied prospectively (mean age 26 ± 14 years). BNP level was increased (74 ± 127 ng/L), and in 16% markedly above the heart failure cut-off level of 100 ng/L. Hb and Hct were increased above normal levels in 20 and 24% of patients, respectively. BNP and Hct/Hb correlated with CMR [total right/left (R/L)-Volume-Index, right atrium-end-diastolic volume index (EDVi), functional right ventricle (fRV)-EDVi, fRV-ejection fraction (EF), tricuspid regurgitation, pulmonary artery flow, and left ventricular EF] and exercise testing [workload/kg, oxygen uptake (VO2), ventilatory response to carbon dioxide production (VE/VCO2), oxygen (O2) pulse, and heart rate reserve]. The higher BNP and haematological parameters, the higher was the disease severity and the more limited was the physical exercise capacity. CONCLUSION In this EA cohort, BNP levels and haematological parameters correlated well with functional data from CMR and exercise testing. The total R/L-Volume-Index and BNP, and to some extent hematological parameters, may be useful as prognostic markers in patients with EA.

Combination therapy of infantile hemangiomas with pulsed dye laser and Nd:YAG laser is effective and safe

Infantile hemangiomas (IH) can cause severe complications such as obstruction, ulceration or heart failure. Therefore, in certain difficult‐to‐treat areas, or when there is no sign of involution, early and effective therapy is required. In rare instances, systemic treatments, like the beta‐blocker propranolol and oral corticosteroids, can cause serious side effects. Effective and well‐tolerated local treatment options are thus desirable as additive or alternative methods.

Cardiac MRI underlines the role of BNP and hematologic parameters as heart failure markers in patients with Ebstein's anomaly of the tricuspid valve

Background Ebstein’s anomaly of the tricuspid valve involves a congenitally displaced and dysplastic tricuspid valve (TV). The displacement of the TV towards the apex results in an enlarged right atrium (RA) with an atrialzied portion of the right ventricle (aRV) and an enlarged functional right ventricle (fRV). Brain natriuretic peptide (BNP) is a heart failure marker. Its role in Ebstein’s anomaly has not been evaluated in conjunction with cardiac MRI (CMR) parameters. Hematologic parameters (hemoglobin (Hb), hematocrite (Hct)) are upregulated in states of pulmonary hypo perfusion as in congenital cyanotic heart disease. Intermittent Pulmonary hypo perfusion may be a an indicator of right heart failure in patients with Ebstein’s anomaly, but has not been studied so far. The aim of the present study was to correlate CMR functional parameters and severity of disease with BNP and hematologic parameters in patients with Ebstein’ sa nomaly. Methods 26 patients with non-corrected Ebstein’ sa nomaly were studied prospectively. Laboratory parameters (BNP, Hb, Hct), CMR data (RA, aRV, fRV, left atrial (LA) and left ventricular (LV) volumes and functional parameters like ejection fraction (EF), stroke volume (SV) and others) as well as exercise capacity (maximal oxygen uptake (VO2 max.), CO2 equivalent (VE/VCO2), maximum heart rate (max. HR) using a cycle ergometer) were all measured on the same day for an individual patient. Results

Cardiac magnetic resonance imaging in pediatric patients ≤ 18 years with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC): a correlation to genetics

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with increased risk of sudden cardiac death. It is a progressive disease that can already manifest itself during childhood and adolescence. Since little is known about the early stage in pediatric patients, this study sought to determine the influence of right and left ventricular findings from cardiac magnetic resonance imaging (CMR) in pediatric patients ≤ 18 years with suspected ARVC in conjunction with positive genetic testing.