Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra‐cardiac malformations: 10‐year national cohort from Finland

Hautala, Johanna; Karstunen, Emma; Ritvanen, Annukka; Rintala, Risto J.; Mattila, Ilkka; Räsänen, Juha; Suominen, Pertti; Ojala, Tiina

doi:10.1111/aogs.13274

Cited by 16 publications

(5 citation statements)

References 15 publications

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“…A limitation of LHR measurement is that the interpretation of its value may vary significantly between different ultrasound users (interobserver error) [8,9], which justifies the efforts to look for new prognostic markers. The authors of this study support the notion that evaluation of the fetal heart is a promising direction in the search for new prognostic parameters [10][11][12][13][14].…”

Section: Introductionsupporting

confidence: 71%

Small prenatal diameter of the ascending aorta is associated with increased mortality risk in neonates with congenital diaphragmatic hernia.

et al. 2022

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IntroductionThe aim of this study was to evaluate if selected prenatal markers obtainded from fetal echocardiography can predict postnatal outcome in congenital diaphragmatic hernia (CDH) patients. We also aimed to verify the prognostic value of lung-to-head ratio (LHR).Material and methodsThe study group included 29 fetuses with CDH. We analyzed potentially prognostic parameters measured using fetal echocardiography and fetal ultrasound. The assessed parameters were compared between the group of patients with CDH who survived to discharge (n=21) and the subset of patients who died before discharge from hospital (n=8).ResultsIn survivors, mean z-score for ascending aorta (AAo) diameter was 0.23 ± 0.98 vs – 1.82 ± 1.04 in patients who died (t-test, p=0.0015). In survivors, the main pulmonary artery/ascending aorta ratio was 1.22 ± 0.17 vs 1.46 ± 0.21 in patients who died (t-test, p=0.017). In survivors, the LHR was 1.81 ± 0.96 vs 0.95 ± 0.6 in patients who died (t-test, p=0.019). In survivors, the observed to expected LHR was 57% ± 30% vs 30% ± 18% in patients who died (t-test, p=0.018).ConclusionsNarrowing of the ascending aorta in CDH fetuses is a poor prognostic factor associated with increased mortality in neonates. Our study also confirmed the prognostic value of LHR.

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Section: Introductionsupporting

confidence: 71%

Small prenatal diameter of the ascending aorta is associated with increased mortality risk in neonates with congenital diaphragmatic hernia.

et al. 2022

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“…All pregnant women are offered two screening ultrasounds (the first between gestational weeks 11 and 13, followed by a fetal anatomy scan between weeks 18 and 22) and combined serum screening for chromosomal defects 7 . The basic data were generated from five different national registries: (a) the National Register of Pediatric Cardiac Surgery; (b) the Register of Congenital Malformations; (c) the Register of Induced Abortions; (d) the Medical Birth Register; and (e) the Cause‐of‐Death Register 8 . The registers are maintained by the Children's Hospital at Helsinki University Hospital (a), the Finnish Institute of Health and Welfare (b‐d), and Statistics Finland (e).…”

Section: Methodsmentioning

confidence: 99%

Perinatal and perioperative factors associated with mortality and an increased need for hospital care in infants with transposition of the great arteries: A nationwide 11‐year population‐based cohort

Hautala

Gissler

Ritvanen

et al. 2020

Acta Obstet Gynecol Scand

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Introduction: Newborn infants with transposition of the great arteries (d-TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11-year population-based cohort of d-TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care. Material and methods: The national cohort consisted of all live-born infants with simple d-TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004-2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated. Results: Preoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but the total mortality rate remained unchanged. All prenatally diagnosed infants (n = 27) survived. Lower gestational age (odds ratio 0.68, P = .012) and higher maternal age at birth (odds ratio 1.16, P = .036) were associated with increased mortality in multivariable analysis. Older infant age at time of operation (P = .002), longer aortic clamp time (P < .001), and higher maternal body mass index (P = .027) were associated with longer initial hospital stay. An extended need for hospital care during the first year of life was multi-factorial. Conclusions: In our cohort, none of the prenatally diagnosed d-TGA infants died. As a result of the limited prenatal detection rates, however, the sample size was insufficient to reach statistical significance. The d-TGA infants born with lower gestational age and to older mothers had increased mortality.

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“…The Finnish Register of Congenital Malformations actively collects data on all fetal/congenital malformations in Finland . We cross‐checked data with the mothers' and live‐born infants' identification numbers and verified all information from hospital records, including prenatal and postnatal reports, karyotype and autopsy results.…”

Section: Methodsmentioning

confidence: 99%

“…The national registers are comprehensive: (1) the National Register of The Finnish Register of Congenital Malformations actively collects data on all fetal/congenital malformations in Finland. 20 We cross-checked data with the mothers' and live-born infants' identification numbers and verified all information from hospital records, including prenatal and postnatal reports, karyotype and autopsy results. One paediatric and fetal cardiologist (TO) confirmed all cardiac diagnoses and one clinical geneticist (AR) confirmed all extra-cardiac malformations.…”

Section: Register Datamentioning

confidence: 99%

The implementation of a nationwide anomaly screening programme improves prenatal detection of major cardiac defects: an 11‐year national population‐based cohort study

Hautala

Gissler

Ritvanen

et al. 2019

BJOG

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Objective To evaluate whether a nationwide prenatal anomaly screening programme improves detection rates of univentricular heart (UVH) and transposition of great arteries (TGA), and whether maternal risk factors for severe fetal heart disease affect prenatal detection. Design Population‐based cohort study. Setting Nationwide data from Finnish registries 2004–14. Population A total of 642 456 parturients and 3449 terminated pregnancies due to severe fetal anomaly. Methods Prenatal detection rates were calculated in three time periods (prescreening, transition and screening phase). The effect of maternal risk factors (obesity, in vitro fertilisation, pregestational diabetes and smoking) was evaluated. Main outcome measures Change in detection rates and impact of maternal risk factors on screening programme efficacy. Results In total, 483 cases of UVH and 184 of TGA were detected. The prenatal detection rate of UVH increased from 50.4% to 82.8% and of TGA from 12.3% to 41.0% (P < 0.0001). Maternal risk factors did not affect prenatal detection rate, but detection rate differed substantially by region. Conclusions A nationwide screening programme improved overall UVH and TGA detection rates, but regional differences were observed. Obesity or other maternal risk factors did not affect the screening programme efficacy. The establishment of structured guidelines and recommendations is essential when implementing the screening programme. In addition, a prospective screening register is highly recommended to ensure high quality of screening. Tweetable abstract Implementation of a nationwide prenatal anomaly screening improved detection rates of UVH and TGA.

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Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra‐cardiac malformations: 10‐year national cohort from Finland

Cited by 16 publications

References 15 publications

Small prenatal diameter of the ascending aorta is associated with increased mortality risk in neonates with congenital diaphragmatic hernia.

Small prenatal diameter of the ascending aorta is associated with increased mortality risk in neonates with congenital diaphragmatic hernia.

Perinatal and perioperative factors associated with mortality and an increased need for hospital care in infants with transposition of the great arteries: A nationwide 11‐year population‐based cohort

The implementation of a nationwide anomaly screening programme improves prenatal detection of major cardiac defects: an 11‐year national population‐based cohort study

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