Prenatal risk factors for urgent atrial septostomy at birth in fetuses with transposition of the great arteries: a systematic review and meta-analysis.

MATERIAL AND METHODS: Medline, Embase, and Cochrane databases were searched. The primary aim was to explore the differences in prenatal echocardiographic parameters among fetuses diagnosed with TGA that required urgent BAS within 24 h of birth due to life-threatening cyanosis compared to those who did not require such procedure. Random-effect meta-analyses were used to compute the data. RESULTS: Six studies (292 fetuses) were included. Restrictive appearance of the FO was present in 64.5% (95% CI = 39.8-85.7) of fetuses with TGA requiring BAS at birth compared to 7.9% (95% CI = 2.1-16.8) not requiring such procedure (OR = 71.1; 95% CI = 8.3-608.5, p < .0001). Hypermobile appearance of the atrial septum was present in 39.1% (95% CI = 26.4-56.5) of fetuses requiring BAS at birth compared to 9.8% (95% CI = 1.4-24.3) of those which did (OR 3.6; 95% CI = 1.4-9.0, p = .05). There was no difference in the prevalence of redundant (p = .374) or fixed (p = .051) atrial septum, bidirectional flow in the DA (p = .26) or an abnormal size of the DA (p = .06) in fetuses requiring urgent BAS at birth compared to those which did not. Mean (±SD) size of the right atrium was smaller in the fetuses with TGA undergoing urgent BAS at birth (23.4 ± 6.7) compared to those which did not (29.2 ± 6.2, p = .01). The mean (±SD) ratio between the FO and the aortic valve diameters (1.01 ± 0.41 versus 1.41 ± 0.43, p = .009) and the mean (±SD) ratio between the FO diameter and the septal length (0.36 ± 0.13 versus 0.51 ± 0.14, p = .001) were significantly smaller in fetuses requiring compared to those not undergoing urgent BAS at birth. The diagnostic accuracy of each independent ultrasound marker of the need for urgent BAS showed an overall good specificity but a low sensitivity. CONCLUSION: Fetal echocardiography prior to birth can stratify the risk of BAS in fetuses with TGA. Further studies are needed to validate these findings and build individualized multiparametric predictive models in order to more accurately identify those fetuses with TGA at a higher risk of urgent BAS after birth.

Outpatient volumes and medical staffing resources as predictors for continuity of follow-up care during transfer of adolescents with congenital heart disease.

BACKGROUND: Providing continuous follow-up care to patients with congenital heart disease (CHD) remains a challenge in many settings. Previous studies highlight that patients with CHD experience discontinuation of follow-up care, but mainly describe a single-centre perspective, neglecting inter-institutional variations. Hospital-related factors above and beyond patient-related factors are believed to affect continuity of care. The present multicentre study therefore investigated (i) proportion of "no follow-up care"; (ii) transfer destinations after leaving paediatric cardiology; (iii) variation in proportions of no follow-up between centres; (iv) the association between no follow-up and outpatient volumes, and (v) its relationship with staffing resources at outpatient clinics. METHODS: An observational, multicentre study was conducted in seven university hospitals. In total, 654 adolescents with CHD, born between 1991 and 1993, with paediatric outpatient visit at age 14-18 years were included. Transfer status was determined 5 years after the intended transfer to adult care (23y), based on medical files, self-reports and registries. RESULTS: Overall, 89.7% of patients were receiving adult follow-up care after transfer; 6.6% had no follow-up; and 3.7% were untraceable. Among patients in follow-up care, only one remained in paediatric care and the majority received specialist adult CHD care. Significant variability in proportions of no follow-up were identified across centres. Higher outpatient volumes at paediatric outpatient clinics were associated with better continued follow-up care after transfer (OR = 1.061; 95% CI = 1.001 - 1.124). Medical staffing resources were not found predictive. CONCLUSION: Our findings support the theory of hospital-related factors influencing continuity of care, above and beyond patient-related characteristics.

Outcome of surgical commissurotomy for aortic valve stenosis in early infancy.

BACKGROUND: The method of treatment of aortic valve stenosis in early infancy is still controversial. This study was performed to evaluate short-term and long-term outcome in our center during a 14-year period. METHODS: Between 1991 and 2004, 64 consecutive patients younger than 3 months old underwent open surgical commissurotomy because of aortic valve stenosis. Median age was 18 days (range, 1 to 79 days), and median weight was 3.6 kg (range, 1.9 to 6.7 kg). Left ventricular function was good in 44 patients (69%), depressed in 12 (19%), and poor in 8 (12%). The study ended in July 2005. Median follow-up time was 4.1 years (range, 0.4 to 13.6 years). RESULTS: The 30-day mortality was 3 of 64 patients and late mortality was 3 of 61, and the respective mortality in patients younger than 1 month old was 2 of 41 and 2 of 39. There was no early mortality after 1993 and no late mortality after 1999. Thirteen patients required reoperation. Median time to reoperation was 4.3 years (range, 0.2 to 11.3 years) and to aortic valve replacement (7 Ross and 1 homograft) was 6.9 years (range, 1.6 to 9.7 years). At the last follow-up, all had good left ventricular function and 57 of 58 had an ability index of 1. CONCLUSIONS: Surgical commissurotomy for aortic valve stenosis during the first 3 months of life can be done with low mortality and morbidity. The risk for early recurrent stenosis or regurgitation is low, and the need for aortic valve replacement can, in most cases, be delayed until the child is older. The long-term functional ability is excellent.