Congenital Heart Defects in Neonates: Determining the Incidence of Genetic Testing and Follow-up Consultation at UCLA

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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Tissue Engineered Bovine Pericardium (CardioCel) for Repair of Congenital Heart Defects: Histopathology and Outcomes at Mid-Term Follow-Up

Heart Lung and Circulation ◽

10.1016/j.hlc.2017.03.112 ◽

2017 ◽

Vol 26 ◽

pp. S391-S392

Author(s):

Douglas Bell ◽

Sudesh Prabhu ◽

Jane Armes ◽

Robert Justo ◽

Benjamin Anderson ◽

...

Keyword(s):

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Bovine Pericardium

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Longitudinal Analysis of Emotional Problems in Children with Congenital Heart Defects: A Follow-Up from Age 6 to 36 Months

Journal of Developmental & Behavioral Pediatrics ◽

10.1097/dbp.0b013e3182202d2b ◽

2011 ◽

Vol 32 (6) ◽

pp. 461-464 ◽

Cited By ~ 8

Author(s):

Kim Stene-Larsen ◽

Ragnhild Eek Brandlistuen ◽

Henrik Holmstrøm ◽

Markus A. Landolt ◽

Leif T. Eskedal ◽

...

Keyword(s):

Congenital Heart Defects ◽

Longitudinal Analysis ◽

Congenital Heart ◽

Heart Defects ◽

Emotional Problems

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Sense of coherence as a predictor of quality of life in adolescents with congenital heart defects: A register-based 1-year follow-up study

European Psychiatry ◽

10.1016/s0924-9338(11)72267-2 ◽

2011 ◽

Vol 26 (S2) ◽

pp. 560-560 ◽

Cited By ~ 1

Author(s):

B. Neuner ◽

M. Busch ◽

J. Wellmann ◽

U. Nowak-Göttl ◽

H.-W. Hense

Keyword(s):

Quality Of Life ◽

Congenital Heart Defects ◽

Sense Of Coherence ◽

Congenital Heart ◽

Linear Models ◽

Heart Defects ◽

Well Being ◽

Psychological Well Being

ObjectiveSense of coherence (SOC) is a resource for health and quality of life (QoL) in adults. Aim of this investigation was to evaluate the association of SOC and QoL in adolescents with congenital heart defects (CHD).MethodObservational study among 770 adolescents aged 14 – 17 years from a national CHD register. SOC was measured at baseline with the SOC-L9 questionnaire. At baseline and at 12-months follow-up, QoL was measured with the KINDL-R questionnaire, evaluating overall well-being and six subscales. The association of SOC with QoL was evaluated in multi-level linear models separately for overall well-being and KINDL-R subscales. Initial models comprised SOC as only fixed effect while the final models were adjusted for age, gender, medical and socioeconomic status and behavioral factors.ResultsOverall well-being, self-esteem and school-related well-being was significantly higher at follow-up compared with baseline. SOC at baseline (median 50 [range: 16 – 63] points) was positively associated with overall well-being and all KINDL-R subscales. There were significant negative interactions between SOC at baseline and time to follow-up for overall well-being and all KINDL-R subscales except psychological well-being. But even in fully adjusted models associations of SOC at baseline with overall well-being and all KINDL-R subscales at follow-up remained significant.ConclusionSOC is an independent predictor of QoL in adolescents with CHD. Except for psychological well-being, this effect attenuates over one year but remains positive inoverall QoL and sub-dimensions. Further studies should evaluate whether interventions aimed to increase SOC in children with CHD improve QoL.

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Outcomes of Preterm Infants With Congenital Heart Defects After Early Surgery: Defining Risk Factors at Different Time Points During Hospitalization

Frontiers in Pediatrics ◽

10.3389/fped.2020.616659 ◽

2021 ◽

Vol 8 ◽

Author(s):

Po-Yin Cheung ◽

Morteza Hajihosseini ◽

Irina A. Dinu ◽

Heather Switzer ◽

Ari R. Joffe ◽

...

Keyword(s):

Early Childhood ◽

Hearing Loss ◽

Cerebral Palsy ◽

Birth Weight ◽

Preterm Infants ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Neurodevelopmental Outcomes

Background: Compared with those born at term gestation, infants with complex congenital heart defects (CCHD) who were delivered before 37 weeks gestational age and received neonatal open-heart surgery (OHS) have poorer neurodevelopmental outcomes in early childhood. We aimed to describe the growth, disability, functional, and neurodevelopmental outcomes in early childhood of preterm infants with CCHD after neonatal OHS. Prediction models were evaluated at various timepoints during hospitalization which could be useful in the management of these infants.Study Design: We studied all preterm infants with CCHD who received OHS within 6 weeks of corrected age between 1996 and 2016. The Western Canadian Complex Pediatric Therapies Follow-up Program completed multidisciplinary comprehensive neurodevelopmental assessments at 2-year corrected age at the referral-site follow-up clinics. We collected demographic and acute-care clinical data, standardized age-appropriate outcome measures including physical growth with calculated z-scores; disabilities including cerebral palsy, visual impairment, permanent hearing loss; adaptive function (Adaptive Behavior Assessment System-II); and cognitive, language, and motor skills (Bayley Scales of Infant and Toddler Development-III). Multiple variable logistic or linear regressions determined predictors displayed as Odds Ratio (OR) or Effect Size (ES) with 95% confidence intervals.Results: Of 115 preterm infants (34 ± 2 weeks gestation, 2,339 ± 637 g, 64% males) with CCHD and OHS, there were 11(10%) deaths before first discharge and 21(18%) deaths by 2-years. Seven (6%) neonates had cerebral injuries, 7 had necrotizing enterocolitis; none had retinopathy of prematurity. Among 94 survivors, 9% had cerebral palsy and 6% had permanent hearing loss, with worse outcomes in those with syndromic diagnoses. Significant predictors of mortality included birth weight z-score [OR 0.28(0.11,0.72), P = 0.008], single-ventricle anatomy [OR 5.92(1.31,26.80), P = 0.021], post-operative ventilation days [OR 1.06(1.02,1.09), P = 0.007], and cardiopulmonary resuscitation [OR 11.58 (1.97,68.24), P = 0.007]; for adverse functional outcome in those without syndromic diagnoses, birth weight 2,000–2,499 g [ES −11.60(−18.67, −4.53), P = 0.002], post-conceptual age [ES −0.11(−0.22,0.00), P = 0.044], post-operative lowest pH [ES 6.75(1.25,12.25), P = 0.017], and sepsis [ES −9.70(−17.74, −1.66), P = 0.050].Conclusions: Our findings suggest preterm neonates with CCHD and early OHS had significant mortality and morbidity at 2-years and were at risk for cerebral palsy and adverse neurodevelopment. This information may be important for management, parental counseling and the decision-making process.

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Long-Term Follow-Up of Pediatric Patients with Severe Postoperative Pulmonary Hypertension After Correction of Congenital Heart Defects

Pediatric Cardiology ◽

10.1007/s00246-021-02794-9 ◽

2021 ◽

Author(s):

Lars Lindberg

Keyword(s):

Pulmonary Hypertension ◽

Arterial Pressure ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Pulmonary Arterial Pressure ◽

Heart Defects ◽

Pressure Ratio ◽

Targeting Therapy ◽

Pulmonary Arterial

AbstractThe surgical repair of congenital heart defects in children with preoperative pulmonary hypertension (PH) is to varying degree associated with the occurrence of postoperative PH. The objective of this study was to follow up children with severe postoperative PH (pulmonary arterial/aortic pressure ratio ≥ 1.0) to evaluate if pulmonary arterial pressure spontaneously normalized or needed PH-targeting therapy and to identify potential high-risk diagnoses for bad outcome. Twenty-five children who developed clinically significant severe PH on at least three occasions postoperatively were included in the follow-up (20–24 years). Data from chart reviews, echocardiographic investigations, and questionnaires were obtained. Three children died within the first year after surgery. Three children were lost to follow-up. The remaining 17 children normalized their pulmonary arterial pressure without the use of PH-targeting drugs at any time during the follow-up. Two children had a remaining mild PH with moderate mitral valve insufficiency. All three children with bad outcome had combined cardiac lesions causing post-capillary pulmonary hypertension. Normalization of the pulmonary arterial pressure occurred in almost all children with severe postoperative PH, without any need of supplemental PH-targeting therapies. All children with bad outcome had diagnoses conformable with post-capillary PH making the use of PH-targeting therapies relatively contraindicated. These data emphasize the need to perform randomized, blinded trials on the use of PH-targeting drugs in children with postoperative PH before accepting it as an indication for routine treatment.

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Perspectives in the management of congenital heart defects in adult patients

Orvosi Hetilap ◽

10.1556/oh.2015.30072 ◽

2015 ◽

Vol 156 (3) ◽

pp. 92-97

Author(s):

István Hartyánszky ◽

Sándor Varga ◽

Kálmán Havasi ◽

Barna Babik ◽

Márta Katona ◽

...

Keyword(s):

Heart Transplantation ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Diseases ◽

Heart Defects ◽

Adult Patients ◽

Univentricular Heart ◽

Transposition Of Great Arteries ◽

Number Of Patients

Due to improving results in congenital heart surgery, the number of adult patients with congenital heart defect is increasing. The question is: what kind of problems can be managed in this patient-group? The authors review the different problems of management of congenital heart defects in adults based on national and international literature data. Simple defects recognised in adults, postoperative residual problems, changing of small grafts and valves, correction of primary or operated coarctation aortae can be usually managed without problems. A very close follow-up is necessary to establish the correct period for heart transplantation in patients with transposition of great arteries with Senning/Mustard operation, and univentricular heart corrected with “Fontan-circulation” type surgical procedure. The authors conclude that although the number of patients increases, only a few congenital heart diseases may cause problems. It seems important (1) to monitor asymptomatic patient who underwent operation (Fallot-IV, Ross procedure, etc.), (2) follow up regularly patients who underwent Senning/Mustard procedure (magnetic resonance imaging, echocardiography, brain natriuretic peptide measurement), (3) define the proper period of preparation for heart transplantation of patients with a univentricular heart, with special attention to the possibility of multiorgan (lung, liver, etc.) failure. Due to the improvement of foetal diagnosis of congenital heart defects, the number of patients with complex congenital heart defects is decreasing. The standard management of these patients could be primary heart transplantation in infancy. Orv. Hetil., 2015, 156(3), 92–97.

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Quality of life in children with infrequent congenital heart defects: cohort study with one-year of follow-up

Health and Quality of Life Outcomes ◽

10.1186/s12955-019-1265-z ◽

2020 ◽

Vol 18 (1) ◽

Cited By ~ 1

Author(s):

Karen Moreno-Medina ◽

Magally Barrera-Castañeda ◽

Catalina Vargas-Acevedo ◽

Alberto E. García-Torres ◽

Miguel Ronderos ◽

...

Keyword(s):

Quality Of Life ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Wilcoxon Test ◽

Significant Difference ◽

One Year ◽

General Module

Abstract Background The evidence regarding patient related outcomes in children with infrequent congenital heart defects (I-CHD) is very limited. We sought to measure quality of life (QoL) in children with I-CHD, and secondarily, to describe QoL changes after one-year of follow-up, self-reported by children and through their caregivers’ perspective. Methods We assembled a cohort of children diagnosed with an I-CHD in a cardiovascular referral center in Colombia, between August 2016 and September 2018. At baseline and at one-year follow-up, a clinical psychology assessment was performed to establish perception of QoL. The Pediatric Quality of Life Inventory (PedsQL) 4.0 scale was used in both general and cardiac modules for patients and for their caregivers. We used a Mann-Whitney U test to compare scores for general and cardiac modules between patients and caregivers, while a Wilcoxon test was used to compared patients’ and caregivers’ baseline and follow-up scores. Results are presented as median and interquartile range. Results To date, QoL evaluation at one-year follow-up has been achieved in 112/157 patients (71%). Self-reported scores in general and cardiac modules were higher than the QoL perceived through their caregivers, both at baseline and after one-year of follow-up. When compared, there was no statistically significant difference in general module scores at baseline between patients (median = 74.4, IQR = 64.1–80.4) and caregivers scores (median = 68.4, IQR = 59.6–83.7), p = 0.296. On the contrary, there was a statistical difference in baseline scores in the cardiac module between patients (median = 79.6, IQR = 69.7–87.4) and caregivers (median = 73.6, IQR = 62.6–84.3), p = 0.019. At one-year of follow-up, scores for the general module between patients (median = 72.8, IQR = 59.2–85.9) and caregivers (median = 69.9, IQR = 58.1–83.7) were not statistically different (p = 0.332). Finally, a significant difference was found for cardiac module scores between patient (median = 75.0, IQR = 67.1–87.1) and caregivers (median = 73.1, IQR = 59.5–83.8), p = 0.034. Conclusions QoL in children with I-CHD can be compromised. However, children have a better perception of their QoL when compared with their caregivers’ assessments. To provide high-quality care, besides a thorough clinical evaluation, QoL directly elicited by the child should be an essential aspect in the integral management of I-CHD.

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

Archives of Disease in Childhood ◽

10.1136/archdischild-2020-319936 ◽

2021 ◽

pp. archdischild-2020-319936

Author(s):

Gunnar Wik ◽

Jarle Jortveit ◽

Vasileios Sitras ◽

Gaute Døhlen ◽

Arild E Rønnestad ◽

...

Keyword(s):

Oxygen Saturation ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Low Oxygen ◽

Clinical Registry ◽

Cardiac Intervention ◽

Cardiac Defects ◽

Staged Surgery

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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5206SICD registry in European paediatric and adult patients with congenital heart defects: preliminary results of the SIDECAR project

European Heart Journal ◽

10.1093/eurheartj/ehz746.0064 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

M S Silvetti ◽

L Kornyei ◽

J Kwiatkowska ◽

I Tamburri ◽

F A Saputo ◽

...

Keyword(s):

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Young Patients ◽

Adult Patients ◽

Icd Implantation ◽

Preliminary Results ◽

Inappropriate Shocks ◽

European Paediatric

Abstract Aim Use of the subcutaneous implantable cardioverter-defibrillator (S-ICD) to prevent sudden cardiac death is increasing. Few data exist on S-ICD in young patients. We report preliminary data from a multicenter European registry of paediatric and young adult patients who underwent S-ICD implantation Methods Observational, prospective, non-randomized, standard-of-care study on S-ICD implantation/follow-up in young patients with inherited arrhythmias (IA), cardiomyopathies, and congenital heart defects (CHD). Results 29 patients (12 CHD, 14 Cardiomyopathies, and 3 IA), mean age 17±6 years, 13 of them <18 years, with body mass index (BMI) 23±4, underwent S-ICD implantation (primary prevention 66%). The first 8 patients underwent a three-incision procedure, the following 21 (72%) a two-incision procedure. No intraoperative complications occurred. Over 19 months median follow-up (25th–75th percentiles, 5–37) 5 patients (17%) received appropriate and 2 (7%) inappropriate shocks. Four patients (14%) had device-related complications requiring surgical intervention: three skin erosions at the superior parasternal incision, one pocket infection. A higher risk of complications was seen in patients who underwent standard procedures [hazard ratio (HR) 14.7, 95% confidence interval (CI) 2.34 to 93.03; P=0.001] and those with BMI <20 (HR 11.06, 95% CI 1.01–121.07; P=0.008). Conclusion These preliminary results of a multicenter European paediatric registry suggest that S-ICD is safe and effective with low rates of inappropriate shocks. Improvement of implantation techniques seems associated with better outcome.

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