Fetal Echocardiographic Findings to Predict Early Surgical Repair and Neonatal Outcomes in Fetuses with Isolated Coarctation of the Aorta.

OBJECTIVE: The aim of this study was to investigate fetal echocardiographic findings in predicting the need for surgical repair in fetuses with coarctation of the aorta (CoA) and to evaluate perinatal outcomes. STUDY DESIGN: In this retrospective study, fetuses diagnosed with CoA in a tertiary center between January 2015 and June 2021 were analyzed. Fetal echocardiographic measurements and quantitative findings, middle cerebral artery (MCA) and umbilical artery (UA) Doppler indices, and perinatal outcomes were recorded. RESULTS: A total of 57 fetuses with CoA were included in the study. In total, 51 (89.5%) pregnancies resulted in live births and 32 (62.8%) of the neonates underwent surgical repair. The left ventricle/right ventricle width ratio and aortic isthmus z-score were significantly lower in fetuses who underwent surgical repair (p = 0.004 0.001, respectively). Retrograde flow in the aortic isthmus (odds ratio [OR]:7.43; 95% confidence interval [CI]: 1.98-27.76), left-to-right foramen ovale shunt (OR: 8.50; 95% CI: 1.68-42.98), and ventricular septal defect (OR: 9.63; 95% CI: 1.90-48.74) were associated with the need for surgical repair. A new scoring system integrating these echocardiographic findings had 89% specificity and 54% sensitivity in predicting surgical repair. Fetal growth restriction rates, preterm birth rates, mean MCA pulsatility index (PI), and mean UA PI were similar in fetuses with and without surgical repair. CONCLUSION: A scoring system integrating echocardiographic findings in fetuses with CoA may improve the prediction of surgical repair need. There is no evidence of an increased risk of FGR, preterm birth, and brain sparing effect in fetuses with CoA who require surgical repair. KEY POINTS: · Coarctation of the aorta is one of the most difficult congenital heart defects to diagnose.. · A new scoring system may improve the prediction of surgical repair need.. · There is no increased risk of fetal growth restriction in fetuses requiring early surgical repair..

Liver parenchymal changes and association with cardiac magnetic resonance imaging findings in repaired tetralogy of Fallot: an intravoxel incoherent motion magnetic resonance imaging study.

BACKGROUND: Liver disease can develop in repaired tetralogy of Fallot (TOF) from hepatic congestion caused by volume and pressure overload of the right ventricle. Noninvasive assessment of the liver is important for diagnosing and managing children with TOF. OBJECTIVE: To evaluate subclinical hepatic changes without liver function test abnormality in adolescents with repaired TOF using intravoxel incoherent motion (IVIM) MRI and cardiac MRI findings. MATERIALS AND METHODS: We included 106 young adults (75 with repaired TOF and 31 healthy individuals) in the study. Liver IVIM MRI examinations were performed with 10 b values (0, 10, 20, 30, 50, 80, 100, 200, 400, 800 s/mm2). Two observers measured IVIM MRI parameters D true, D* and f, as well as apparent diffusion coefficient (ADC) values in liver segments 5-8. RESULTS: D* and f values were significantly lower in adolescents with TOF (P = 0.003 vs. P = 0.05, respectively). ADC values were higher in adolescents with TOF (P = 0.005). However, we found no significant difference between adolescents with and without TOF in terms of Dtrue (P = 0.53). There was a significant correlation between f value and right ventricular ejection fraction. The intraclass correlation coefficient (ICC) analysis of the two observers showed substantial-to-excellent agreement for D, f, D true and ADC (0.7, 0.8, 0.9 and 0.8, respectively). CONCLUSION: The results of our study suggest that impaired microperfusion with increased ADC values in adolescents with repaired TOF reflect hepatic congestion rather than fibrosis. Hepatic congestion characterized by decreased ADC values can be easily differentiated before fibrotic changes occur by using IVIM MRI to assess diffusion and microcapillary perfusion separately.

Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis.

BACKGROUND: We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis. METHODS: From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed. RESULTS: Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0-9.9) years. Ten (34.5%) patients had Williams-Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7-7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08). CONCLUSION: Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

Use of steerable delivery catheter to successfully deliver a Ceraflex septal occluder to close an atrial septal defect in a child with interrupted inferior vena cava with azygos continuation.

The closure of a secundum atrial septal defect through the jugular vein in a child with interrupted inferior vena cava with azygos continuation by steerable delivery catheter is described in the present report. The steerable catheter can be used to correct the perpendicular position of the device over the margins of the defect, and is particularly useful in cases of large defects.

Efficacy of very low-dose prostaglandin E1 in duct-dependent congenital heart disease.

AIM: The present study aims to define the lowest effective prostaglandin E1 dose in patients with inadequacy of pulmonary blood flow and/or intracardiac blood mixing and those with inadequate systemic blood flow. METHODS: Patients with inadequacy of both pulmonary blood flow and/or blood mixing (Group 1) and those with inadequate systemic blood flow (Group 2) were retrospectively evaluated in two separate groups with regard to the prostaglandin E1 starting dose given in the referring facility, the lowest and the highest dose administered in our centre, treatment duration, adverse effects, and administered treatment. RESULTS: No difference between the groups could be detected with respect to sex or birth weight (p=0.95 and 0.42, respectively). Group 1 and Group 2 were statistically similar in aspect of prostaglandin treatment duration (9.73±0.81 days versus 11.6±1.05 days, p=0.064). When compared with Group 2, the initial, maintenance and lowest efficient doses of prostaglandin E1 treatment were significantly lower and the titrated dose of prostaglandin E1 was significantly higher in Group 1 (p=0.001 for each). CONCLUSION: Our findings indicate that the infusion of prostaglandin at a very low dose (0.003-0.005 mcg/kg/minute) is sufficient to maintain the patency of the ductus arteriosus. A higher dose of prostaglandin E1 may be necessary in patients with inadequate systemic blood flow.