Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?

Vincenti, Marie; Guillaumont, Sophie; Clarivet, Béatrice; Macioce, Valérie; Mura, Thibault; Boulot, P.; Cambonie, Gilles; Amedro, Pascal

doi:10.1016/j.acvd.2018.11.013

Cited by 17 publications

(22 citation statements)

References 35 publications

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“…Several studies present a trend towards more cardiac surgery in infants with complex CHDs and severe comorbidity, such as trisomy 18 and 13. 31 32 In the study by Vincenti et al 28 , all infants received active treatment regardless of the severity of CHDs or comorbidities, but the mortality rate was similar to the mortality in our study. This may indicate that a palliative approach mainly changes the manner of death without increasing the rate of mortality.…”

Section: Discussionsupporting

confidence: 83%

“…In contrast, a recent study reported no difference in mortality in infants with and without prenatal diagnosis of severe CHDs and questioned the impact of prenatal diagnosis on infant survival. However, the two groups (with and without prenatal diagnosis) differed in the types of heart diagnoses 28. In the present study, the 2-year mortality rate among live-born infants was 10% and almost 50% died in palliative care.…”

Section: Discussioncontrasting

confidence: 49%

“…However, the two groups (with and without prenatal diagnosis) differed in the types of heart diagnoses. 28 In the present study, the 2-year mortality rate among live-born infants was 10% and almost 50% died in palliative care. This proportion was stable despite increased termination rates.…”

Section: Discussionmentioning

confidence: 47%

“…Termination of about half of pregnancies with prenatally diagnosed severe CHDs has been recently reported. 28 We previously reported that the prenatal detection rate in Norway in 2016 was 58%. 29 In the present study, we were not able to include the prenatal detection rate for all fetuses with severe CHDs, but we registered an increased rate of terminated pregnancies during the study period.…”

Section: Discussionmentioning

confidence: 98%

“…Termination of about half of pregnancies with prenatally diagnosed severe CHDs has been recently reported 28. We previously reported that the prenatal detection rate in Norway in 2016 was 58% 29.…”

Section: Discussionmentioning

confidence: 98%

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Severe congenital heart defects: incidence, causes and time trends of preoperative mortality in Norway

et al. 2020

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Background and aimsSevere congenital heart defects (CHDs) still represent one of the main causes of infant death. The risk factors associated with cardiac surgery and postoperative mortality are well known. We aimed to describe the rates, causes and time trends of mortality before surgery—including termination of pregnancies and palliative care—in fetuses and children below 2 years of age with severe CHDs.Methods and resultsData concerning all 789 345 pregnancies in Norway from 2004 to 2016 were retrieved from the Medical Birth Registry of Norway, the Oslo University Hospital’s Clinical Registry for Congenital Heart Defects, the Norwegian Cause of Death Registry, the National Registry, Statistics Norway, autopsy reports and medical records. When including termination of pregnancy and stillbirths, the number of fetuses and children with severe CHDs that did not reach the age of 2 years was 31%. Mortality among the 2359 live-born infants with severe CHDs was 10%, of whom 58% died before surgery. Of the preoperative deaths, 81% died in a palliative care setting, and comorbidity and univentricular CHDs were common among these infants. Together, palliative care and termination of pregnancy accounted for 86% of deaths in cases of severe CHDs, and this proportion increased during the study period (annual percent changes 1.3, 95% CI 0.4 to 2.1, p<0.001), mainly due to an increased termination rate.ConclusionsTermination of pregnancy accounted for the majority of the deaths in fetuses and children with severe CHDs. Among live-born children, most preoperative deaths occurred in a palliative care setting and were strongly related to comorbidities and/or univentricular hearts.

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Section: Discussionsupporting

confidence: 83%

Section: Discussioncontrasting

confidence: 49%

Section: Discussionmentioning

confidence: 47%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 98%

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Severe congenital heart defects: incidence, causes and time trends of preoperative mortality in Norway

et al. 2020

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Impact of prenatally diagnosed congenital heart defects on outcomes and management

Wong

Kohari

Bahtiyar

et al. 2022

J of Clinical Ultrasound

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Fetal echocardiogram aids in prenatal identification of neonates at high risk for congenital heart defects (CHD). Prenatal detection rates for CHD have increased with improved ultrasound technology, the use of the early fetal echocardiography, and standardization of the performance of the fetal echocardiogram. Accurate prenatal detection of CHD, particularly complex CHD, is an important contributor to improved survival rates for patients with CHD. Early detection allows for families to choose whether or not to continue with pregnancy, referral to pediatric cardiology specialists for patient education, and delivery planning. Better psychosocial supports are needed for families with CHD.

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Risk factors for early occurrence of malnutrition in infants with severe congenital heart disease

et al. 2023

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Purpose: To identify the risk factors of early occurrence of malnutrition in infants with severe congenital heart disease (CHD) during their rst year of life.Methods and Results: Retrospective longitudinal multicentre study carried out from January 2014 to December 2020 in two tertiary care CHD centres. Four CHD hemodynamic groups were identi ed.Malnutrition was de ned by a Waterlow score under 80% and/or the need for calorie-enriched formula. A total of 216 infants with a severe CHD, e.g., requiring cardiac surgery, cardiac catheterization, or hospitalization for heart failure during their rst year of life were included in the study. Malnutrition was observed among 43% of the cohort, with the highest prevalence in infants with increased pulmonary blood ow (71%) compared to the others hemodynamic groups (p<0.001). In multivariate analysis, low birthweight (OR 0.62, 95% CI 0.44-0.89, p=0.009), CHD with increased pulmonary blood ow (OR 4.80, 95% CI 1.42-16.20, p=0.08), heart failure (OR 9.26, 95% CI 4.04-21.25, p<0.001), and the number of hospitalizations (OR 1.35, 95% CI 1.08l-1.69, p=0.009) during the rst year of life were associated with malnutrition (AUC 0.85, 95% CI 0.79-0.90). Conclusion:In infants with a severe CHD, early occurrence of malnutrition during the rst year of life affected a high proportion of subjects. CHD with increased pulmonary blood ow, low birthweight, heart failure, and repeated hospitalizations were risk factors for malnutrition. Further studies are required to identify optimal nutritional support in this population. What Is KnownMalnutrition is a known morbidity and mortality factor in children with severe congenital heart disease. What is new:Early occurrence of malnutrition during the rst year of life in infant severe congenital heart disease (CHD) was high (43%).CHD with increased pulmonary blood ow, low birthweight, heart failure, and repeated hospitalizations during the rst year of life were risk factors for malnutrition.These results suggest the need for further studies to identify optimal nutritional support in this population.

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Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?

Cited by 17 publications

References 35 publications

Severe congenital heart defects: incidence, causes and time trends of preoperative mortality in Norway

Severe congenital heart defects: incidence, causes and time trends of preoperative mortality in Norway

Impact of prenatally diagnosed congenital heart defects on outcomes and management

Risk factors for early occurrence of malnutrition in infants with severe congenital heart disease

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