Unexpected death in children with severe congenital heart defects in Norway 2004–2016

AimsUpdated knowledge on the rates and causes of death among children with severe congenital heart defects (CHDs) is needed to further improve treatment and survival. This study investigated nationwide mortality rates in children with severe CHDs with an emphasis on unexpected mortality unrelated to cardiac intervention.Methods and resultsData on all pregnancies and live-born children in Norway from 2004 to 2016 were obtained from national registries, the Oslo University Hospital’s Clinical Registry for CHDs and medical records. Among 2359 live-born children with severe CHDs, 234 (10%) died before 2 years of age. Of these, 109 (46%) died in palliative care, 58 (25%) died of causes related to a cardiac intervention and 67 (29%) died unexpectedly and unrelated to a cardiac intervention, either before (n=26) or following (n=41) discharge after a cardiac intervention. Comorbidity (38/67, 57%), persistent low oxygen saturation (SaO2; <95%; 41/67, 61%), staged surgery (21/41, 51%), residual cardiac defects (22/41, 54%) and infection (36/67, 54%) were frequent in children who died unexpectedly unrelated to an intervention. Two or more of these factors were present in 62 children (93%). The medical reports at hospital discharge lacked information on follow-up in many patients who died unexpectedly.ConclusionsThe numbers of unexpected deaths unrelated to cardiac intervention in children <2 years of age without comorbidity were low in Norway. However, close follow-up is recommended for infants with comorbidities, persistent low oxygen saturation, staged surgery or residual cardiac defects, particularly when an infection occurs.

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Out-of-hospital sudden cardiac arrest in children with congenital heart defects

Archives of Disease in Childhood ◽

10.1136/archdischild-2017-312621 ◽

2017 ◽

Vol 103 (1) ◽

pp. 57-60 ◽

Cited By ~ 1

Author(s):

Jarle Jortveit ◽

Jakob Klcovansky ◽

Gaute Døhlen ◽

Leif Eskedal ◽

Sigurd Birkeland ◽

...

Keyword(s):

Cardiac Arrest ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Sudden Cardiac Arrest ◽

Supplementary Information ◽

Clinical Registry ◽

Birth Registry ◽

Live Births ◽

Hospital Cardiac Arrest

AimsOut-of-hospital sudden cardiac arrest (SCA) is a rare but devastating event in children and adolescents. The risk is assumed to be higher in children with congenital heart defects (CHDs) than in healthy individuals. The aim of the present study was to investigate the rate of and survival after out-of-hospital cardiac arrest in children 2–18 years old with CHDs.Methods and resultsData concerning all live births in Norway between 1994 and 2009 were retrieved from the Medical Birth Registry of Norway, the patient administrative systems at all hospitals in Norway, the Oslo University Hospital’s Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012, and supplementary information for the deceased children was retrieved from medical records at Norwegian hospitals. Among the 943 871 live births in Norway from 1994 to 2009, 11 272 (1.2%) children had a CHD. We identified 11 (0.1%) children 2–18 years old with CHDs who experienced out-of-hospital SCA. The estimated rate of out-of-hospital SCA in children 2–18 years old with CHD was 10 per 100 000 person-years. Early cardiopulmonary resuscitation was initiated in all patients. Three children survived.ConclusionsThe incidence of and survival after out-of-hospital SCA in children with CHDs were comparable to the reported rates in the general child population.

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Pulse Oximetry During the First 24 Hours as a Screening Tool For Congenital Heart Defects

The Journal of Critical Care Medicine ◽

10.1515/jccm-2017-0004 ◽

2017 ◽

Vol 3 (1) ◽

pp. 12-17

Author(s):

Mihaela Patriciu ◽

Andreea Avasiloaiei ◽

Mihaela Moscalu ◽

Maria Stamatin

Keyword(s):

Oxygen Saturation ◽

Pulse Oximetry ◽

Sensitivity And Specificity ◽

Congenital Heart Defects ◽

Screening Test ◽

Congenital Heart ◽

Heart Defects ◽

Blood Oxygen ◽

Blood Oxygen Saturation ◽

Routine Pulse Oximetry

Abstract Introduction: Although screening for congenital heart defects (CHD) relies mainly on antenatal ultrasonography and clinical examination after birth, life-threatening cardiac malformations are often not diagnosed before the patient is discharged. Aim: To assess the use of routine pulse oximetry in the delivery room and at 24 hours postpartum, and to study its feasibility as a screening test for CHD. Material and Methods: In this prospective study, all infants born in “Cuza Voda” Maternity Hospital, Iasi, Romania, were enrolled over a thirteen-month period. Preductal oximetry was assessed during the first hour, and postductal oximetry was evaluated at twenty-four hours postpartum. Data were then analyzed to establish the sensitivity and specificity of pulse oximetry, as a screening test for CHD. Results: 5406 infants were included in the study, with a mean gestational age of 38.2 weeks and a mean birth weight of 3175 grams. During the first minute, blood oxygen saturation varied between 40% and 90% and at 24 hours of life, it ranged between 90% and 100%. Following oximetry assessment, 14 infants with critical CHD were identified. Blood oxygen saturation values in infants with CHD were lower throughout the entire period of evaluation. Pulse oximetry had good sensitivity and specificity at 1 hour (Se=87.5%, Sp=95.5%) and 24 hours (Se=92.5%, Sp=97.4%) for the diagnosis of CHD. Blood oxygen saturation values at one minute, 1 hour and 24 hours are strong discriminative parameters for the early diagnosis of CHD. Conclusion: Routine pulse oximetry during the first 24 hours postpartum represents an early indicator of CHD to facilitate timely intervention. Pulse oximetry provides excellent sensitivity and specificity and has tremendous potential as a standard screening test for CHD during the first 24 hours of life.

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Evaluation of heterogeneity in the association between congenital heart defects and variants of folate metabolism genes: Conotruncal and left-sided cardiac defects

Birth Defects Research Part A Clinical and Molecular Teratology ◽

10.1002/bdra.22849 ◽

2011 ◽

Vol 91 (10) ◽

pp. 879-884 ◽

Cited By ~ 7

Author(s):

Jin Long ◽

Philip J. Lupo ◽

Elizabeth Goldmuntz ◽

Laura E. Mitchell

Keyword(s):

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Folate Metabolism ◽

Cardiac Defects

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Head growth in fetuses with isolated congenital heart defects: lack of influence of aortic arch flow and ascending aorta oxygen saturation

Ultrasound in Obstetrics and Gynecology ◽

10.1002/uog.15980 ◽

2016 ◽

Vol 48 (3) ◽

pp. 357-364 ◽

Cited By ~ 8

Author(s):

F. A. R. Jansen ◽

E. W. van Zwet ◽

M. E. B. Rijlaarsdam ◽

E. Pajkrt ◽

C. L. van Velzen ◽

...

Keyword(s):

Oxygen Saturation ◽

Aortic Arch ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Ascending Aorta ◽

Head Growth

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Impact of 3D Printing on Short-Term Outcomes of Biventricular Conversion From Single Ventricular Palliation for the Complex Congenital Heart Defects

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.801444 ◽

2021 ◽

Vol 8 ◽

Author(s):

Bozhong Shi ◽

Yanjun Pan ◽

Weiru Luo ◽

Kai Luo ◽

Qi Sun ◽

...

Keyword(s):

3D Printing ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Vena Cava ◽

Left Ventricular ◽

Long Term Results ◽

Short Term ◽

The Mean

Background: Although Fontan palliation seems to be inevitable for many patients with complex congenital heart defects (CHDs), candidates with appropriate conditions could be selected for biventricular conversion. We aimed to summarize our single-center experience in patient selection, surgical strategies, and early outcomes in biventricular conversion for the complex CHD.Methods: From April 2017 to June 2021, we reviewed 23 cases with complex CHD who underwent biventricular conversion. Patients were divided into two groups according to the development of the ventricles: balanced ventricular group (15 cases) and imbalanced ventricular group (8 cases). Early and short-term outcomes during the 30.2 months (range, 4.2–49.8 months) follow-up period were compared.Results: The overall mortality rate was 4.3% with one death case. In the balanced ventricular group, 6 cases received 3D printing for pre-operational evaluation. One case died because of heart failure in the early postoperative period. One case received reoperation due to the obstruction of the superior vena cava. In the imbalanced ventricular group, the mean left ventricular end-diastolic volume was (33.6 ± 2.1) ml/m2, the mean left ventricular end-diastolic pressure was 9.1 ± 1.9 mmHg, and 4 cases received 3D printing. No death occurred while one case implanted a pacemaker due to a third-degree atrioventricular block. The pre-operational evaluation and surgery simulation with a 3D printing model helped to reduce bypass time in the balanced group (p < 0.05), and reduced both bypass and aorta clamp time in the imbalanced group (p < 0.05). All patients presented great cardiac function in the follow-up period.Conclusion: Comprehensive evaluation, especially 3D printing technique, was conducive to finding the appropriate cases for biventricular conversion and significantly reduced surgery time. Biventricular conversion in selected patients led to promising clinical outcomes, albeit unverified long-term results.

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A Case of Situs Ambiguous and Complex Cardiac Defect Presenting as Fetal Hydrops

Indian Journal of Cardiovascular Disease in Women WINCARS ◽

10.1055/s-0038-1656413 ◽

2017 ◽

Vol 02 (02) ◽

pp. 060-063

Author(s):

Shagun Aggarwal

Keyword(s):

Case Report ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Cardiac Defect ◽

Fetal Hydrops ◽

Cardiac Defects ◽

Live Births ◽

Situs Ambiguous ◽

Laterality Defects

AbstractSitus ambiguous comprises of 3% of congenital heart defects and is present in at least 1 in 10,000 live births. Most cases diagnosed prenatally are associated with complex cardiac defects which can be detected by ultrasonography. This is a case report of a fetus presenting with hydrops, which was detected to have situs ambiguous, a complex cardiac defect and multiple laterality defects on autopsy.

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Congenital Cardiac Defects and Critical Congenital Heart Defects

Fast Facts for The Neonatal Nurse ◽

10.1891/9780826184917.0019 ◽

2020 ◽

Keyword(s):

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Cardiac Defects ◽

Congenital Cardiac Defects

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Blood flow patterns underlie developmental heart defects

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00641.2016 ◽

2017 ◽

Vol 312 (3) ◽

pp. H632-H642 ◽

Cited By ~ 29

Author(s):

Madeline Midgett ◽

Kent Thornburg ◽

Sandra Rugonyi

Keyword(s):

Blood Flow ◽

Congenital Heart Defects ◽

Dose Response ◽

Congenital Heart ◽

Heart Defects ◽

Flow Patterns ◽

Early Embryo ◽

Response Type ◽

Cardiac Defects ◽

Blood Flow Patterns

Although cardiac malformations at birth are typically associated with genetic anomalies, blood flow dynamics also play a crucial role in heart formation. However, the relationship between blood flow patterns in the early embryo and later cardiovascular malformation has not been determined. We used the chicken embryo model to quantify the extent to which anomalous blood flow patterns predict cardiac defects that resemble those in humans and found that restricting either the inflow to the heart or the outflow led to reproducible abnormalities with a dose-response type relationship between blood flow stimuli and the expression of cardiac phenotypes. Constricting the outflow tract by 10–35% led predominantly to ventricular septal defects, whereas constricting by 35–60% most often led to double outlet right ventricle. Ligation of the vitelline vein caused mostly pharyngeal arch artery malformations. We show that both cardiac inflow reduction and graded outflow constriction strongly influence the development of specific and persistent abnormal cardiac structure and function. Moreover, the hemodynamic-associated cardiac defects recapitulate those caused by genetic disorders. Thus our data demonstrate the importance of investigating embryonic blood flow conditions to understand the root causes of congenital heart disease as a prerequisite to future prevention and treatment. NEW & NOTEWORTHY Congenital heart defects result from genetic anomalies, teratogen exposure, and altered blood flow during embryonic development. We show here a novel “dose-response” type relationship between the level of blood flow alteration and manifestation of specific cardiac phenotypes. We speculate that abnormal blood flow may frequently underlie congenital heart defects.

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