Does prenatal diagnosis of hypoplastic left heart syndrome make a difference? – A systematic review

Thakur, Varsha; Munk, Nadia; Mertens, Luc; Nield, Lynne E.

doi:10.1002/pd.4873

Cited by 21 publications

(17 citation statements)

References 29 publications

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“…Prenatal diagnosis did not result in a better survival similar to the findings of others. 33,34 For patients that survived to stage III surgery, the 10-year transplantation-free survival was 88%. Long-term results after stage III have been reported from the population of New Zealand and Australia with a 97% survival.…”

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confidence: 99%

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

et al. 2020

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Background:Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia.Methods:Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993–2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016.Results:The 1993–2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome.Conclusion:The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.

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confidence: 99%

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

et al. 2020

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“…Hypoplastic left heart syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation ( 44 , 64 , 65 ). However, borderline cases of HLHS can go undetected in many early to mid-gestation fetal exams.…”

Section: Discussionmentioning

confidence: 99%

Model of Human Fetal Growth in Hypoplastic Left Heart Syndrome: Reduced Ventricular Growth Due to Decreased Ventricular Filling and Altered Shape

Dewan¹,

Krishnamurthy

Kole³

et al. 2017

Front. Pediatr.

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IntroductionHypoplastic left heart syndrome (HLHS) is a congenital condition with an underdeveloped left ventricle (LV) that provides inadequate systemic blood flow postnatally. The development of HLHS is postulated to be due to altered biomechanical stimuli during gestation. Predicting LV size at birth using mid-gestation fetal echocardiography is a clinical challenge critical to prognostic counseling.HypothesisWe hypothesized that decreased ventricular filling in utero due to mitral stenosis may reduce LV growth in the fetal heart via mechanical growth signaling.MethodsWe developed a novel finite element model of the human fetal heart in which cardiac myocyte growth rates are a function of fiber and cross-fiber strains, which is affected by altered ventricular filling, to simulate alterations in LV growth and remodeling. Model results were tested with echocardiogram measurements from normal and HLHS fetal hearts.ResultsA strain-based fetal growth model with a normal 22-week ventricular filling (1.04 mL) was able to replicate published measurements of changes between mid-gestation to birth of mean LV end-diastolic volume (EDV) (1.1–8.3 mL) and dimensions (long-axis, 18–35 mm; short-axis, 9–18 mm) within 15% root mean squared deviation error. By decreasing volumetric load (−25%) at mid-gestation in the model, which emulates mitral stenosis in utero, a 65% reduction in LV EDV and a 46% reduction in LV wall volume were predicted at birth, similar to observations in HLHS patients. In retrospective blinded case studies for HLHS, using mid-gestation echocardiographic data, the model predicted a borderline and severe hypoplastic LV, consistent with the patients’ late-gestation data in both cases. Notably, the model prediction was validated by testing for changes in LV shape in the model against clinical data for each HLHS case study.ConclusionReduced ventricular filling and altered shape may lead to reduced LV growth and a hypoplastic phenotype by reducing myocardial strains that serve as a myocyte growth stimulus. The human fetal growth model presented here may lead to a clinical tool that can help predict LV size and shape at birth based on mid-gestation LV echocardiographic measurements.

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“…Critical congenital heart defects (CHD) are the most common congenital anomalies that may lead to infant death when undiagnosed . Prenatal detection of severe cardiac defects allows optimal follow up of pregnancy and planning of delivery of a critically ill child, and reduces mortality and morbidity . Heart defects such as univentricular heart (UVH) and transposition of the great arteries (TGA) benefit from prenatal diagnosis and should be prioritised in prenatal CHD screening.…”

Section: Introductionmentioning

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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Does prenatal diagnosis of hypoplastic left heart syndrome make a difference? – A systematic review

Abstract: There is no significant impact of prenatal diagnosis of hypoplastic left heart syndrome on preoperative or post Stage I mortality. Neonates with prenatal diagnosis were hemodynamically more stable. © 2016 John Wiley & Sons, Ltd.

Cited by 21 publications

References 29 publications

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

Model of Human Fetal Growth in Hypoplastic Left Heart Syndrome: Reduced Ventricular Growth Due to Decreased Ventricular Filling and Altered Shape

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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