Goetz C. Mueller

Impedance cardiography (electrical velocimetry) and transthoracic echocardiography for non-invasive cardiac output monitoring in pediatric intensive care patients: a prospective single-center observational study

IntroductionElectrical velocimetry (EV) is a type of impedance cardiography, and is a non-invasive and continuously applicable method of cardiac output monitoring. Transthoracic echocardiography (TTE) is non-invasive but discontinuous.MethodsWe compared EV with TTE in pediatric intensive care patients in a prospective single-center observational study. Simultaneous, coupled, left ventricular stroke volume measurements were performed by EV using an Aesculon® monitor and TTE (either via trans-aortic valve flow velocity time integral [EVVTI], or via M-mode [EVMM]). H0: bias was less than 10% and the mean percentage error (MPE) was less than 30% in Bland–Altman analysis between EV and TTE. If appropriate, data were logarithmically transformed prior to Bland–Altman analysis.ResultsA total of 72 patients (age: 2 days to 17 years; weight: 0.8 to 86 kg) were analyzed. Patients were divided into subgroups: organ transplantation (OTX, n =28), sepsis or organ failure (SEPSIS, n =16), neurological patients (NEURO, n =9), and preterm infants (PREM, n =26); Bias/MPE for EVVTI was 7.81%/26.16%. In the EVVTI subgroup analysis for OTX, NEURO, and SEPSIS, bias and MPE were within the limits of H0, whereas the PREM subgroup had a bias/MPE of 39.00%/46.27%. Bias/MPE for EVMM was 8.07%/37.26% where the OTX and NEURO subgroups were within the range of H0, but the PREM and SEPSIS subgroups were outside the range. Mechanical ventilation, non-invasive continuous positive airway pressure ventilation, body weight, and secondary abdominal closure were factors that significantly affected comparison of the methods.ConclusionsThis study shows that EV is comparable with aortic flow-based TTE for pediatric patients.

Retrospective analysis of the effect of angiotensin II receptor blocker versus β-blocker on aortic root growth in paediatric patients with Marfan syndrome

Objectives Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). β-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS. Methods 215 patients with MFS (9.01±5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation. Results Treatment by ARB and BB leads to significant reduction of SV dilation (p<0.05). The deviation of SV enlargement from normal as expressed by the rate of change in z scores was significantly reduced by a mean difference of −0.56±0.71 z scores (p<0.001) under ARB therapy and by a mean difference of −0.35±0.68 z scores (p<0.05) under BB therapy. The prophylactic effect of ARB and BB on aortic root dilation is similar in both groups (p>0.05). Conclusions Both concepts lead to a significant reduction of SV dilation. The effect of ARB and BB is similar. This is the first study concerning the comparison of ARB and BB in previously untreated paediatric patients with MFS. The results of the study show that both treatment strategies are beneficial in paediatric and adolescent patients.

The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome

Due to age‐dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long‐term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified.

First experience with a new drug-eluting balloon for the treatment of congenital pulmonary vein stenosis in a neonate.

Paclitaxel-eluting balloons are a new and innovative method in the treatment of in-stent stenosis and small vessel disease in adult cardiac pathology. The treatment of congenital pulmonary vein stenosis is difficult to manage, and results in a high mortality rate due to residual or recurrent stenosis. We report the first case of treatment for neonatal pulmonary vein restenosis with a paclitaxel-eluting balloon.

Cardiovascular biomarkers in paired maternal and umbilical cord blood samples at term and near term delivery

BACKGROUND Cardiovascular biomarkers might help to identify fetuses or pregnancies at risk. AIM To examine the umbilical cord neonatal and maternal levels of cardiovascular biomarkers at the time of delivery, and to correlate maternal and fetal biomarker levels to each other, to gestational age and to delivery mode. STUDY DESIGN In a prospective, observational, cross-sectional, single-center study biomarkers were measured in paired maternal and umbilical venous cord blood samples. SUBJECTS The sample cohort included 66 sets of fetal and maternal blood samples (11 after multiple gestation, 53 after cesarean section, 17 after exposure to labor). OUTCOME MEASURES Midregional pro-adrenomedullin (MRproADM), midregional-pro atrial natriuretic peptide (MRproANP), brain natriuretic peptide (BNP), n-terminal-pro brain natriuretic peptide (NTproBNP), copeptin, and high sensitive troponin I (hsTnI) levels were measured. RESULTS Mean ± SEM for biomarker levels in umbilical venous/maternal blood were: MRproADM [nmol/L] 1.02 ± 0.04/1.24 ± 0.08, MRproANP [pmol/L] 215.53 ± 12.96/54.65 ± 3.41, BNP [pg/mL] 32.02 ± 3.37/19.76 ± 3.29, NTproBNP [pg/mL] 1228.94 ± 91.73/71.48 ± 8.65, copeptin [pmol/L] 103.42 ± 22.89/10.41 ± 1.71, and hsTnI [pg/mL] 13.54 ± 5.17/4.91 ± 2.37. Fetal MRproANP, NTproBNP, and BNP were inversely correlated with gestational age. Maternal and fetal MRproANP (r=0.472, p=0.002) and copeptin (r=0.572, p<0.001) levels were correlated, whereas there was no feto-maternal correlation for the other biomarkers. Fetal copeptin was elevated after exposure to labor. CONCLUSIONS Biomarker levels appear to be regulated independently in mother and fetus. Fetal biomarkers are influenced by gestational age and delivery mode. In this study on term and near term pregnancies without specific fetal pathology, correlation between paired maternal and fetal biomarker levels was weak or not demonstrable.

Health-related quality of life is unimpaired in children and adolescents with Marfan syndrome despite its distinctive phenotype

Marfan syndrome (MFS) is a progressive, life‐threatening genetic disorder of the connective tissue, which causes impaired quality of life (QoL) in adults. This study investigated the quality of life in children and adolescents, taking into account their gender, age and how MFS affected their organs.

Prevalence of Anemia in Children with Congestive Heart Failure due to Dilated Cardiomyopathy

Introduction. Anemia is prevalent in adult heart failure patients and appears to be an independent risk factor for morbidity and mortality. The purpose of this work is to determine the prevalence of anemia in children with heart failure from dilated cardiomyopathy (DCM) and to evaluate its influence on morbidity and mortality. Methods. A homogenous group of 58 children with congestive heart failure from DCM was evaluated for heart failure symptoms, appearance of anemia, hospitalization, age of first clinical appearance, necessity of transfusion, and death during medical attendance. Anemic and nonanemic patients were analyzed for differences in age distribution, morbidity, and mortality. Results. Anemia was present in 64% of DCM patients. Hospitalization secondary to heart failure was significantly elevated in heart failure patients with anemia (mean 35.1 ± 40.5 versus 9.97 ± 9.65 days per year, P < 0.05). However, mortality was not elevated. Significant relations of age and prevalence of anemia or age and severity of anemia did not appear. Conclusion. Anemia is prevalent in pediatric patients with congestive heart failure from DCM and appears in all age classes. Hospitalization as a surrogate of morbidity is elevated in heart failure patients developing anemia, but mortality risk did not increase.

Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.

The Pulmonary Artery in Pediatric Patients with Marfan Syndrome: An Underestimated Aspect of the Disease

Aortic root dilatation and its complications are known to be the most important and life limiting features in patients with Marfan syndrome (MFS). Since monitoring of patients, preventive medical and surgical treatments are available nowadays, other MFS pathologies are becoming more relevant for the outcome of the disease. Main pulmonary artery (MPA) dilatation is a cardiac finding, which has not been fully investigated in children. Due to the similarities in tissue composition of the aortic and pulmonary root, MPA dilatation may cause complications and require treatment. In addition, it may be a predictor for severe connective tissue involvement. We retrospectively examined 135 pediatric patients with MFS. 8.1% showed MPA dilatation. MPA dilatation was associated with earlier occurrence of aortic dilatation, mitral valve prolapse, and systemic manifestations of MFS compared with patients without MPA dilatation (p < 0.05). The presence of MPA dilatation was also associated with a higher incidence of ectopia lentis (p < 0.05). Medical treatment was started earlier in MPA dilatation patients than in those without (p < 0.05). We conclude that MPA dilatation is a sign of more severe vascular and connective tissue involvement. Regular examination of the pulmonary artery is essential in MFS to avoid complications. As medical treatment of life threatening MFS events has improved, other features of MFS need to be investigated to improve quality of life.

Kid-Short Marfan Score (Kid-SMS) Is a Useful Diagnostic Tool for Stratifying the Pre-Test Probability of Marfan Syndrome in Childhood

Due to age dependent organ manifestation, diagnosis of Marfan syndrome (MFS) is a challenge, especially in childhood. It is important to identify children at risk of MFS as soon as possible to direct those to appropriate treatment but also to avoid stigmatization due to false diagnosis. We published the Kid-Short Marfan Score (Kid-SMS) in 2012 to stratify the pre-test probability of MFS in childhood. Hence we now evaluate the predictive performance of Kid-SMS in a new cohort of children. We prospectively investigated 106 patients who were suspected of having MFS. At baseline, children were examined according to Kid-SMS. At baseline and follow-up visit, diagnosis of MFS was established or rejected using standard current diagnostic criteria according to the revised Ghent Criteria (Ghent-2). At baseline 43 patients were identified with a risk of MFS according to Kid-SMS whereas 21 patients had Ghent-2 diagnosis of MFS. Sensitivity was 100%, specificity 77%, negative predictive value 100% and Likelihood ratio of Kid-SMS 4.3. During follow-up period, three other patients with a stratified risk for MFS were diagnosed according to Ghent-2. We confirm very good predictive performance of Kid-SMS with excellent sensitivity and negative predictive value but restricted specificity. Kid-SMS avoids stigmatization due to diagnosis of MFS and thus restriction to quality of life. Especially outpatient pediatricians and pediatric cardiologists can use it for primary assessment.