External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome

Background: Personalized external aortic root support (PEARS) was introduced in 2004 for prevention of aortic root dilatation in Marfan patients. The individual's aortic root is replicated by 3-dimensional printing. A polymer mesh sleeve is manufactured, which is implanted with the aim to support and stabilize the aortic wall. / Objectives: The aim of this study was to assess effectiveness of PEARS for prevention of aortic root dilatation in Marfan patients. / Methods: A total of 24 consecutive Marfan patients operated 2004 to 2012 were prospectively monitored with magnetic resonance imaging. Following a pre-defined protocol, baseline and follow-up aorta measurements were made in a blinded random sequence. / Results: The mean age of the patients was 33 ± 13.3 years (range: 16 to 58 years), and the mean aortic root diameter was 45 ± 2.8 mm (range: 41 to 52 mm). Follow-up was 6.3 ± 2.6 years. There was no increase in the aortic root and ascending aorta diameters, but there was a tendency toward reduction: annulus diameter 28.9 ± 2.3 mm to 28.5 ± 2.4 mm (change −0.39 mm, 95% confidence interval [CI]: −1.05 to 0.27 mm), sinus of Valsalva diameter 44.9 ± 2.9 mm to 44.5 ± 3.0 mm (change −0.37 mm, 95% CI: −1.23 to 0.51 mm), and ascending aorta diameter 32.4 ± 3.6 mm to 32.3 ± 3.7 mm (change −0.10 mm, 95% CI: −0.92 to 0.74 mm). In the same period, the descending aorta diameter increased from 22.9 ± 2.4 mm to 24.2 ± 3.0 mm (change 1.32 mm, 95% CI: 0.70 to 1.94 mm; p < 0.001) with a tendency toward increase in aortic arch diameter 24.1 ± 2.0 mm to 24.5 ± 2.8 mm (change 0.41 mm, 95% CI: −0.56 to 1.37 mm). / Conclusions: PEARS is effective in stabilizing the aortic root and preventing its dilatation. It is a viable alternative for prevention of aortic root dissection in Marfan patients

Three-dimensional late gadolinium enhancement cardiovascular magnetic resonance predicts inducibility of ventricular tachycardia in adults with repaired tetralogy of Fallot

Background - Adults with repaired tetralogy of Fallot (rTOF) die prematurely from ventricular tachycardia (VT) and sudden cardiac death. Inducible VT predicts mortality. Ventricular scar, the key substrate for VT, can be non-invasively defined with late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) but whether this relates to inducible VT is unknown. Methods - Sixty-nine consecutive rTOF patients (43 male, mean 40{plus minus}15 years) clinically scheduled for invasive programmed VT-stimulation were prospectively recruited for prior 3D LGE CMR. Ventricular LGE was segmented and merged with reconstructed cardiac chambers and LGE volume measured. Results - VT was induced in 22(31%) patients. Univariable predictors of inducible VT included increased RV LGE (OR 1.15;p=0.001 per cm3), increased non-apical vent LV LGE (OR 1.09;p=0.008 per cm3), older age (OR 1.6;p=0.01 per decile), QRS duration ≥180ms (OR 3.5;p=0.02), history of non-sustained VT (OR 3.5; p=0.02) and previous clinical sustained VT (OR 12.8;p=0.003); only prior sustained VT (OR 8.02;p=0.02) remained independent in bivariable analyses after controlling for RV LGE volume (OR 1.14;p=0.003). An RV LGE volume of 25cm3 had 72% sensitivity and 81% specificity for predicting inducible VT (AUC 0.81;p10cm3 was 100% sensitive and >36cm3 was 100% specific for predicting inducible VT. Conclusions - 3D LGE CMR-defined scar burden is independently associated with inducible VT and may help refine patient selection for programmed VT-stimulation when applied to an at least intermediate clinical risk cohort

Plasma copeptin may not be a sensitive marker of perinatal stress in healthy full-term growth-restricted fetuses

Objective: Intrauterine-growth-restriction-(IUGR) is associated with chronic fetal stress, as well as a phase of enhanced fetal/early postnatal insulin sensitivity, followed by a later emergence of insulin resistance. We aimed to prospectively investigate concentrations of copeptin, a sensitive marker of stress and insulin resistance, in IUGR versus appropriate-for-gestational-age-(AGA) fetuses. Methods: Cord blood copeptin concentrations were determined by ELISA in well-defined, non-distressed at birth, asymmetric IUGR (n = 30) and AGA (n = 20) full-term pregnancies. Doppler studies were indicative of placental insufficiency. Results: Cord blood copeptin concentrations were similar in IUGR cases and AGA controls, after controlling for delivery mode. Copeptin concentrations were markedly elevated in vaginally delivered fetuses (p = 0.001). No association was recorded between fetal copeptin concentrations and maternal age, parity, gestational age, or fetal gender. Conclusions: Cord blood copeptin concentrations are probably not affected by IUGR at term, in the absence of fetal distress, possibly due to a balance between copeptin up-regulation by chronic fetal stress, on one hand, and copeptin down-regulation in the presence of increased insulin sensitivity, on the other hand; thus, copeptin may not be a sensitive marker of chronic perinatal stress in healthy asymmetric IUGR infants. Cord blood copeptin seems to primarily reflect perinatal stress associated with delivery mode. © 2016 Informa UK Limited, trading as Taylor &amp; Francis Group

Caesarean section: Impact on mother and child

Maternal/foetal morbidity/mortality consequent to uterine rupture, placenta previa/increta and stillbirth may occur in repeat caesarean section (CS). Elective CS before 39 weeks increases respiratory complications, hypoglycaemia, sepsis and intensive care unit admissions. Different gut colonization in neonates born by CS accounts for increased incidence of food allergy, asthma and possibly type 1 diabetes. Epigenetic changes might be responsible - among others - for childhood malignancies. Conclusion: Decision for primary CS should take into consideration possible maternal/neonatal complications. © 2011 The Author(s)/Acta Pædiatrica

Cord blood copeptin concentrations in fetal macrosomia

Background/aim Excessive fetal growth is associated with increased adiposity and reduced insulin sensitivity at birth. Copeptin, a surrogate marker of arginine vasopressin (AVP) secretion, is upregulated in states of hyperinsulinemia and is considered one of the mediators of insulin resistance. We aimed to investigate cord blood concentrations of copeptin (C-terminal fragment of AVP pro-hormone) in healthy large-for-gestational-age (LGA) infants at term. Methods This prospective study was conducted on 30 LGA (n = 30) and 20 appropriate-for-gestational-age (AGA, n = 20) singleton full-term healthy infants. Cord blood copeptin and insulin concentrations were determined by ELISA and IRMA, respectively. Infants were classified as LGA or AGA, based on customized birth-weight standards adjusted for significant determinants of fetal growth. Results Cord blood copeptin concentrations were similar in LGA cases, compared to AGA controls, after adjusting for delivery mode. However, in the LGA group, cord blood copeptin concentrations positively correlated with birth-weight (r = 0.422, p = 0.020). In the AGA group, cord blood copeptin concentrations were elevated in cases of vaginal delivery vs elective cesarean section (p = 0.003). Cord blood insulin concentrations were higher in LGA cases, compared to AGA controls (p = 0.036). No association was recorded between cord blood copeptin concentrations and maternal age, parity, gestational age or fetal gender in both groups. Conclusions Cord blood copeptin concentrations may not be up-regulated in non-distressed LGA infants. However, the positive correlation between cord blood copeptin concentrations and birth-weight in the LGA group may point to the documented association between AVP release and increased fat deposition. Vaginal delivery vs elective cesarean section is accompanied by a marked stress-related increase of cord blood copeptin concentrations. © 2016 Elsevier Inc. All rights reserved

Cord blood chemerin and obestatin levels in large for gestational age infants

Objective: To investigate possible alterations in cord blood levels of adipokines, chemerin and obestatin (secreted by adipose tissue and associated with later development of insulin resistance/metabolic syndrome), as well as insulin, in large for gestational age (LGA) and appropriate for gestational age (AGA) pregnancies, granted that these groups differ in body fat mass and metabolic/endocrine mechanisms. Methods: Cord blood chemerin, obestatin, and insulin concentrations were prospectively measured in 40 LGA (9 born from diabetic and 31 from nondiabetic mothers) and 40 AGA singleton full-term infants. Results: Cord blood chemerin concentrations were significantly higher in LGA compared with AGA neonates (b = 38.91, SE 9.29, p &lt; 0.001). In contrast, no significant differences in obestatin concentrations were observed between groups. Insulin levels were significantly elevated as customized centiles increased (b = 0.003, SE = 0.001, p = 0.032). Conclusions: Higher chemerin concentrations in LGA neonates possibly reflect the increased adipose tissue in this group. Lack of difference between the two groups in circulating levels of obestatin-possibly a sensitive marker of insulin resistance-might be due to development of metabolic disorders later in life. © 2013 Informa UK, Ltd

Potential prognostic biomarkers of cardiovascular disease in fetal macrosomia: the impact of gestational diabetes

Objective: Fetal macrosomia is associated with cardiac hypertrophy and increased cardiovascular risk. Cardiac biomarkers may play diagnostic/prognostic role in cardiovascular disease. We tested whether cardiac biomarkers are differentially expressed in cord blood samples of full-term singleton large-for-gestational-age (LGA), as compared to appropriate-for-gestational-age (AGA) pregnancies. Methods: Cardiotrophin-1 (CT-1), Titin, pentraxin (PTX-3) and soluble CD36 (sCD36) concentrations were determined in 80 cord blood samples from a) LGA pregnancies due to maternal diabetes (n = 8), overweight/obese (n = 11), excessive weight gain (n = 7), without specific pathology (n = 14), b) AGA normal pregnancies (controls, n = 40). Neonates were classified as LGA or AGA based on customized birth weight (BW) standards. Results: CT-1 and Titin concentrations were higher in LGA than AGA pregnancies (p &lt;.001 and p =.023, respectively). A subgroup analysis (in the LGA group) showed increased CT-1 concentrations only in diabetic pregnancies. PTX-3 and sCD36 concentrations were similar in LGA and AGA fetuses. In the LGA group, PTX-3 concentrations positively correlated with birth-weight (r =.416, p =.008) and respective sCD36 concentrations (r =.443, p =.004). Conclusion: Higher Titin concentrations in LGAs possibly represent a candidate molecular mechanism underlying the association between fetal macrosomia and cardiomyocyte/diastolic dysfunction. CT-1 is up-regulated only in LGAs exposed to maternal diabetes. PTX-3 and sCD36 are probably not affected by excessive fetal growth. © 2017 Informa UK Limited, trading as Taylor &amp; Francis Group