Prenatal Diagnosis Improves the Postnatal Cardiac Function in a Population-Based Cohort of Infants with Hypoplastic Left Heart Syndrome

Markkanen, Hanna K.; Pihkala, Jaana; Salminen, Jukka; Saarinen, Maiju; Hornberger, Lisa K.; Ojala, Tiina

doi:10.1016/j.echo.2013.05.005

Cited by 25 publications

(28 citation statements)

References 29 publications

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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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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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“…The comparison between the two groups with pre-natal and post-natal diagnosis with regard to the post-operative morbidity showed discrepancies exactly as for the survival ( 3 , 6 , 26 , 29 , 31 ).…”

Section: General Morbiditymentioning

confidence: 83%

“…Few studies showed reduced post-operative morbidity in the group with pre-natal diagnosis ( 3 , 6 , 31 ). The reasons to support the decreased morbidity were the better pre-operative conditions, particularly because of the reduced metabolic acidosis ( 3 , 31 ), reduced duration of mechanical ventilation ( 6 ), and reduced need for emergency operation ( 6 ), and the observations of better post-operative ventricular function ( 3 , 31 ).…”

Section: General Morbiditymentioning

confidence: 99%

â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis

Corno

2015

Front. Pediatr.

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Within the last few decades the pre-natal echocardiographic diagnosis of congenital heart defects has made substantial progresses, particularly for the identification of complex malformation. "Functionally" univentricular hearts categorize a huge variety of heart malformations. Since no one of the patients with these congenital heart defects can ever undergo a bi-ventricular type of repair, early recognition and decision-making from the neonatal period are required in order to allow for appropriate multiple-step diagnostic and treatment procedures, either of interventional cardiology and/or surgery, on the pathway of "univentricular" heart. In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects. This review of the recent reports has been undertaken to better understand the impact of pre-natal diagnosis in "functionally" univentricular hearts taking into consideration the following topics: pre-natal screening, outcomes and survival, general morbidity, neurologic and developmental consequences, pregnancy management and delivery planning, resources utilization and costs/benefits issues, ethical implications, parents counseling, and interruption of pregnancy versus treatment.

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“…4 Studies 3,5-7 have indicated that although prenatal diagnosis may not have a marked effect on mortality, it is related to an improvement in morbidity, specifically with regard to neurodevelopmental outcomes. Neonates in whom HLHS is not diagnosed prenatally are more likely to have shock, more severe preoperative lactic acidosis, and a need for inotropic support, which further increases the risk for right ventricular dysfunction and tricuspid regurgitation, both of which are important risk factors for survival of stage I Norwood palliation.…”

Section: Perinatal Factors That Affect Preoperative Managementmentioning

confidence: 99%

“…Neonates in whom HLHS is not diagnosed prenatally are more likely to have shock, more severe preoperative lactic acidosis, and a need for inotropic support, which further increases the risk for right ventricular dysfunction and tricuspid regurgitation, both of which are important risk factors for survival of stage I Norwood palliation. 4,8 Prenatal diagnosis has clear advantages, such as providing time for counseling and educating the infant's parents and allowing a delivery plan with immediate initiation of prostaglandins and prompt transport to an appropriate care setting. Additionally, prenatal diagnosis of HLHS allows further and more precise delineation of the anatomy, and conditions such as an intact or restrictive atrial septum can be monitored so that plans can be made for prompt balloon atrial septostomy immediately after delivery as needed.…”

Section: Perinatal Factors That Affect Preoperative Managementmentioning

confidence: 99%

Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation

Donnellan

Justice

2016

Critical Care Nurse

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Hypoplastic left heart syndrome is a severe form of congenital heart disease that results in single-ventricle physiology. Although surgical palliation in infants with this syndrome is widely agreed on in practice, variation occurs in both surgical and medical management. Perinatal factors that affect preoperative management include the subtype of the syndrome and the patient's birth weight, gestational age, and genetic abnormalities. The general aspects of perioperative stabilization and management are monitoring, use of vasoactive infusions, ventilatory support, and nutrition. Management strategies for balancing single-ventricle physiology are aimed at addressing the 3 major causes of desaturation: diminished pulmonary blood flow, low mixed venous oxygen saturation, and pulmonary venous desaturation. (Critical Care Nurse. 2016; 36[1]:52-59)

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Prenatal Diagnosis Improves the Postnatal Cardiac Function in a Population-Based Cohort of Infants with Hypoplastic Left Heart Syndrome

Cited by 25 publications

References 29 publications

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

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

â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis

Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation

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Prenatal Diagnosis Improves the Postnatal Cardiac Function in a Population-Based Cohort of Infants with Hypoplastic Left Heart Syndrome

Cited by 25 publications

References 29 publications

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

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

â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis

Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation

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â€œFunctionallyâ€ Univentricular Hearts: Impact of Pre-Natal Diagnosis