Critical Congenital Heart Disease Research Articles

Impact of Newborn Screening Programme for Congenital Heart Disease in Shanghai: a five-year observational study in 801,831 newborns

Newborn Screening Programme for Congenital Heart Disease (CHD) in Shanghai has been in operation for over 5 years, and its feasibility and effectiveness still lack a systematic and comprehensive evaluation. This study aimed to detail the implementation of the programme and evaluate its results, benefits, and reliability in clinical practice. This study was an observational study involving all newborns received CHD screening in Shanghai from 2017 to 2021. Pulse oximetry (POX) plus cardiac murmur auscultation (namely the dual-index method) was used for CHD screening in newborns aged 6-72h. Newborns who screened positive was recommended for echocardiography, and those diagnosed with CHD would be planned for further evaluation and intervention. Data were aggregated by birth year and district of birth. Results of neonatal CHD screening, diagnosis and treatment, and temporal trends of infant mortality rate (IMR) and the proportion of under-five mortality (U5M) attributed to CHD were analysed. A retrospective cohort study was also conducted to assess the reliability of the dual-index method in clinical practice. In total, 801,831 (99.48%) newborns were screened for CHD, 16,489 (2.06%) were screened positive, and 3541 (21.47%) of the screened-positive newborns were identified with CHD. Seven hundred and fifty-two patients with CHD received surgical or interventional treatment with a successful rate of 94.81%. The period from 2015 to 2021 witnessed an approximately twofold decrease in IMR from 4.58‰ to 2.30‰, and a downtrend in the proportion of U5M attributed to CHD from 25.93% to 16.61%. High sensitivity and specificity of the dual-index method in clinical practice were observed for both critical (100.00% and 97.72%) and major CHD (98.47% and 97.76%). Newborn screening programme for CHD has been well implemented in Shanghai, and this programme is a successful public health intervention to reduce infant death. Our study provides encouraging evidence and experience for implementing newborn screening programme for CHD nationwide in China. This study was supported by the National Key Research and Development Programme of China (2021YFC2701004 and 2016YFC1000506), CAMS Innovation Fund for Medical Sciences (2019-I2M-5-002), and Three-Year Planning for Strengthening the Construction of Public Health System in Shanghai (No. GWIV-24).

Associated factors for prenatally diagnosed fetal congenital heart diseases

ObjectiveCurrent studies have suggested that fetal congenital heart diseases (CHDs) are caused by various factors. However, few data in this field is available in China.This study aimed to detect associated factors of prenatally diagnosed fetal CHD in a large sample in China.Study designPregnant women who underwent fetal echocardiography were recruited in our hospital between May 2018 and September 2019. The maternal sociodemographic and lifestyle characteristics and some fetal factors were obtained. We used forward stepwise logistic regression analysis to assess risk of fetal CHD associated with various factors.ResultsA total of 5024 subjects were enrolled, of whom 875 had CHD fetuses. Among the fetal CHD group (N = 875), critical CHDs account for 27%, of which Tetralogy of Fallot is the most (7.1%), followed by coarctation of aorta (4.0%), double-outlet right ventricle (2.9%). The forward stepwise logistic regression models revealed that history of spontaneous abortion (OR = 1.59, 95% CI 1.33–1.91, P = 0.000), upper respiratory tract infection during early pregnancy (OR = 1.30, 95% CI 1.04–1.62, P = 0.020), mental stress during early pregnancy (OR = 2.37, 95% CI 1.15–4.91, P = 0.020), single umbilical artery (OR = 2.30, 95% CI 1.18–4.51, P = 0.015), and paternal smoking (OR = 1.21, 95% CI 1.02–1.44, P = 0.027) are positively associated with an increased risk of fetal CHD.ConclusionWe identified several factors positively associated with fetal CHD. These findings suggest that it is important to strengthen healthcare and prenatal counseling for women with these factors.

Impact of prenatal diagnosis on outcomes of surgical correction in newborns with transposition of the great arteries comparing to other critical congenital heart defects

A review of the literature data describing the influence of prenatal diagnosis of critical congenital heart defects on perioperative and long-term results is presented, with a focus on the features of prenatal detection of transposition of the great arteries. This heart defect is a convenient object of studying due to the relative anatomical homogeneity of the nosological form; the birth of patients, as a rule, at full term; the rarity of the combination of this pathology with multiple congenital malformations and chromosomal abnormalities that can potentially worsen the results of treatment; the similarity of the principles of preoperative management in this category of patients in different clinics; the performing of arterial switch surgery in a strictly defined time frame (usually during the first month of life); the relative similarity of surgical techniques used. The relevance of this study is to identify patterns that make it possible to improve existing protocols for the treatment of newborns with transposition of the great arteries and create new algorithms for interaction between gynecologists, neonatologists, resuscitators, and cardiac surgeons.

Risk factors for critical and complex congenital heart diseases: Case-control study

BackgroundCongenital heart defects (CHD) are the most common type of congenital defects. Despite having much better-defined risk factors nowadays, the etiology of most CHDs remains unknown. In only 10 to 15 % of cases, a well-established causal relationship can be defined. ObjectiveTo identify risk factors for critical and/or complex congenital heart disease as well as to estimate the current prevalence of these conditions in newborns. MethodsThe present case-control study was matched by maternal age and newborn sex, performed between 2014 and 2016 by active search, involving interviews and the analysis of medical records, as well as clinical evaluation in newborns with critical or complex CHD. Our study was performed in the maternity and neonatology departments of four major public hospitals in the city of Salvador, Bahia-Brazil. ResultsSixty-one cases of critical and/or complex congenital heart disease were identified, with 122 controls selected at random from 20,917 live births during the study period. The prevalence of critical and/or complex CHD was 292 cases per 100,000 births. The most frequent kinds of complex CHD were coarctation of the aorta and interrupted aortic arch. Our reduced conditional logistic regression model identified the following risk factors: Number of pregnancies (odds ratio [OR]: 8.97 [1.75–46], p = 0.009), and known maternal (OR: 6.62 [1.23–35.81], p = 0.028) or paternal (OR: 8.88 [2.31–35.63] p = 0.002) family history of malformations. ConclusionWe observed a high prevalence of critical and complex CHD in the studied population. The final conditional logistic regression model identified a history of three or more pregnancies and a known paternal family history of malformations as independent risk factors for critical congenital heart disease.

Nurses’ Perceptions of a Novel Rooming-in Program for Infants With Critical Congenital Heart Disease

The transition to home for infants who require complex care can be overwhelming for caregivers. Infants with critical congenital heart disease (CCHD) require advanced care management, so their caregivers must acquire extensive training before the infants are discharged home. Rooming-in programs have improved patient outcomes in other settings, such as the postpartum period. However, little research has examined a rooming-in program in a pediatric cardiac acute care setting. To describe nurses' perceptions of a novel rooming-in program implemented in a pediatric cardiac acute care unit. A qualitative descriptive research design was used to describe nurses' perceptions of the rooming-in program for infants with CCHD. Three focus groups were conducted with a convenience sample of 13 registered nurses who cared for infants with CCHD during the rooming-in program. Four trained independent coders performed qualitative thematic analysis. Nurses provided critical insight into the rooming-in program. Three themes were identified: improved nursing and family outcomes, leading the way through collaboration, and room for improvement. Infants with CCHD have complex needs, and caregivers must acquire advanced skills to adequately care for these infants. This study is the first to explore nurses' perceptions of a rooming-in program for infants with CCHD. The findings could improve rooming-in programs in the pediatric acute care setting, which can translate to better patient outcomes.

Clinical Pearls.

Research has shown that unrestricted visitation is beneficial and contributes to high-quality family-centered care. However, with the onset of the COVID-19 pandemic most hospitals banned visitors. In the ensuing time, despite repeated evidence of the negative effects of restricted visitation on patients, caregivers, and families, many hospitals continue to enforce a restrictive policy.Marmo and colleagues examined current visitation policies of Magnet and Pathway to Excellence facilities that had unrestricted visitation policies in their adult intensive care units before the pandemic. They found that All participating nurse leaders thought that the decision to restrict during the pandemic was made by external systems with no input from nursing.None of the hospitals went back to an open visitation policy, and most study participants thought that open visitation polices would not return.Nurse leaders described restricted visitation causing harm to patients, families, and nurses.Although technology capabilities such as virtual visiting allowed family participation, these methods added further responsibility and strain to the nursing staff. The authors recommend that hospitals use the best evidence available and shared decision-making with all key stakeholders to develop safe visitation policies and practices.See Article, pp 31-41Infants with critical congenital heart disease (CCHD) often require complex medical care at home following surgery. The caregivers of these infants must learn a great deal of medical skills through extensive training, often in a short period. Many caregivers experience stress and feel unprepared for postdischarge parental care.Shackleford and colleagues describe nurses’ perceptions of a 24-hour rooming-in program for caregivers of infants with CCHD. They identified 3 themes: This study was the first to examine rooming-in with caregivers of patients with CCHD. Although areas of improvement were recognized, findings suggest that a rooming-in program for CCHD infant care can effectively support the quality of discharge education.See Article, pp 54-61Psychological distress of family caregivers of critically ill patients can result in post–intensive care syndrome– family after hospitalization. This distress also occurs during hospitalization and may include anxiety, lack of sleep, and decrease in sound decision-making. However, little research has explored the factors that affect caregiver stress during the hospitalization.Blok and colleagues interviewed adult family caregivers (most were adult children and patient spouses) in person in the intensive care unit to explore their sources of psychological distress. They found Overall, findings show that factors influencing family distress are wide-ranging and vary by level of caregiver anxiety. The authors suggest early identification and assessment of family factors to develop strategies for reducing distress.See Article, pp 21-30Delirium during hospitalization in the intensive care unit (ICU) contributes to adverse consequences such as prolonged hospital stay, increased hospitalization cost, and increased mortality risk. As several risk factors contribute to onset of delirium, prediction models have been developed. However, some models are more thoroughly validated than others, and the effect of mechanical ventilation on one of the models’ prognostic accuracy needs further study.Van Nieuw Amerongen and colleagues compared 3 prediction models—the PRE-DELIRIC, E-PRE-DELIRIC, and Lanzhou models—in adult ICU patients receiving and not receiving mechanical ventilation. They found Although there are limitations with any model, the findings support the use of a prognostic tool during the first 24 hours of ICU stay to support decision-making in delirium care.See Article, pp 43-50

Real-Time Remote-Mentored Echocardiography in Management of Newborns with Critical Congenital Heart Defects

Real-Time Remote-Mentored Echocardiography in Management of Newborns with Critical Congenital Heart Defects

Differences in Surgical Results according to the Prenatal Diagnosis of Critical Congenital Heart Disease

Differences in Surgical Results according to the Prenatal Diagnosis of Critical Congenital Heart Disease

Performance of Pulse Oximetry in Screening of Critical congenital Heart Diseases: Meta-analysis of meta-analytic studies and systematic review.

Performance of Pulse Oximetry in Screening of Critical congenital Heart Diseases: Meta-analysis of meta-analytic studies and systematic review.

Mapping the early life gut microbiome in neonates with critical congenital heart disease: multiomics insights and implications for host metabolic and immunological health

BackgroundThe early life gut microbiome is crucial in maintaining host metabolic and immune homeostasis. Though neonates with critical congenital heart disease (CCHD) are at substantial risks of malnutrition and immune imbalance, the microbial links to CCHD pathophysiology remain poorly understood. In this study, we aimed to investigate the gut microbiome in neonates with CCHD in association with metabolomic traits. Moreover, we explored the clinical implications of the host-microbe interactions in CCHD.MethodsDeep metagenomic sequencing and metabolomic profiling of paired fecal samples from 45 neonates with CCHD and 50 healthy controls were performed. The characteristics of gut microbiome were investigated in three dimensions (microbial abundance, functionality, and genetic variation). An in-depth analysis of gut virome was conducted to elucidate the ecological interaction between gut viral and bacterial communities. Correlations between multilevel microbial features and fecal metabolites were determined using integrated association analysis. Finally, we conducted a subgroup analysis to examine whether the interactions between gut microbiota and metabolites could mediate inflammatory responses and poor surgical prognosis.ResultsGut microbiota dysbiosis was observed in neonates with CCHD, characterized by the depletion of Bifidobacterium and overgrowth of Enterococcus, which was highly correlated with metabolomic perturbations. Genetic variations of Bifidobacterium and Enterococcus orchestrate the metabolomic perturbations in CCHD. A temperate core virome represented by Siphoviridae was identified to be implicated in shaping the gut bacterial composition by modifying microbial adaptation. The overgrowth of Enterococcus was correlated with systemic inflammation and poor surgical prognosis in subgroup analysis. Mediation analysis indicated that the overgrowth of Enterococcus could mediate gut barrier impairment and inflammatory responses in CCHD.ConclusionsWe demonstrate for the first time that an aberrant gut microbiome associated with metabolomic perturbations is implicated in immune imbalance and adverse clinical outcomes in neonates with CCHD. Our data support the importance of reconstituting optimal gut microbiome in maintaining host metabolic and immunological homeostasis in CCHD.4pXZJ27nHYVeyd4JvNJWQdVideo

Ambivalence is associated with decreased physical activity and cardiorespiratory fitness among adolescents with critical congenital heart disease

BackgroundAdolescents with congenital heart disease (CHD) are insufficiently physically active. Given that increasing physical activity may reduce their cardiovascular risk, it is important to identify correlates of this behavior. Perceived benefits of and barriers to physical activity are associated with physical activity engagement. Existing research has only considered these constructs separately. This population may be ambivalent toward physical activity (i.e., perceive both strong benefits and barriers). The association of ambivalence and physical activity related outcomes is unknown among this at-risk population. ObjectiveDetermine the association of ambivalence and sedentary behavior, moderate-to-vigorous physical activity (MVPA), and cardiorespiratory fitness (VO2Peak) among adolescents with CHD. MethodsThe present study is an analysis of data from an eligibility assessment for a randomized clinical trial of an intervention to promote MVPA among adolescents aged 15 to 18 years with moderate or complex CHD. Participants (N = 84) completed a survey assessing perceived benefits and barriers from which ambivalence toward physical activity was calculated, an exercise stress test to measure VO2Peak, and wore an accelerometer for one week to determine their engagement in sedentary behavior and MVPA. Linear regression analyses determined associations between ambivalence and physical activity related outcomes. ResultsGreater ambivalence toward physical activity was associated with increased sedentary behavior, decreased MVPA, and reduced VO2Peak, adjusting for demographic and clinical covariates. ConclusionsAmbivalence is associated with objectively measured physical activity (sedentary behavior, MVPA) and a biomarker of cardiovascular health (VO2Peak). Screening for ambivalence may help clinicians identify those most likely to benefit from physical activity-related education.

Current Postlaunch Implementation of State Mandates of Newborn Screening for Critical Congenital Heart Disease by Pulse Oximetry in U.S. States and Hospitals.

Our objective was to gauge adherence to nationally endorsed protocols in implementation of pulse oximetry (POx) screening for critical congenital heart disease (CCHD) in infants after mandate by all states and to assess associated characteristics. Between March and October 2019, an online questionnaire was administered to nurse supervisors who oversee personnel conducting POx screening. The questionnaire used eight questions regarding performance and interpretation of screening protocols to measure policy consistency, which is adherence to nationally endorsed protocols for POx screening developed by professional medical societies. Multilevel linear regression models evaluated associations between policy consistency and characteristics of hospitals and individuals, state of hospital location, early versus late mandate adopters, and state reporting requirements. Responses from 189 nurse supervisors spanning 38 states were analyzed. Only 17% received maximum points indicating full policy consistency, and 24% selected all four options for potential hypoxia that require a repeat screen. Notably, 33% did not recognize ≤90% SpO2 as an immediate failed screen and 31% responded that an infant with SpO2 of 89% in one extremity will be rescreened by nurses in an hour rather than receiving an immediate physician referral. Lower policy consistency was associated with lack of state reporting mandates (beta = -1.23 p = 0.01) and early adoption by states (beta = -1.01, p < 0.01). When presented with SpO2 screening values on a questionnaire, a low percentage of nurse supervisors selected responses that demonstrated adherence to nationally endorsed protocols for CCHD screening. Most notably, almost one-third of respondents did not recognize ≤90% SpO2 as a failed screen that requires immediate physician follow-up. In addition, states without reporting mandates and early adopter states were associated with low policy consistency. Implementing state reporting requirements might increase policy consistency, but some inconsistency may be the result of unique protocols in early adopter states that differ from nationally endorsed protocols. · Low adherence to nationally endorsed protocols.. · Inconsistent physician follow-up to hypoxia.. · Reporting improved consistency with national policy..

Pulse Oximetry Screening for Detecting Critical Congenital Heart Disease in Neonates.

Background Congenital heart disease(CHD) leads to significant morbidity in the neonatal population and is a crucial disorder behind early infancy death rates. Mosthave a critical congenital heart defect (Cr CHD) out of all the heart defects found in babies. A subgroup of cardiac anomalies needssurgery or catheter intervention during the neonatal period. Pulse oximetry is a good screening tool to detect cr CHDin neonates. This study aims to assess the effectiveness of pulse oximetry as a screening toolin a rural setting. Methods This was a hospital-based prospective observational study. All consecutively born neonates whose parents consented to the study were subjected to pulse oximetry on all four limbs. Their peripheral arterial oxygen saturation was measured on days one, two, and three of life, starting four hours after birth. Babies detected with cyanotic congenital heart disease (CCHD) before birth are not a part of this study. All those with arterial oxygen saturation of less than 95% or a difference of saturation of more than 3% in the upper and lower limbs were considered suspects for Cr CHD and subjected to echocardiography. Results Among 5874 neonates studied, researchers found 164 babies to have significant hypoxemia on pulse oximetry, and 44CHD were detected on echocardiography within the first three days of life (positive predictive value (PPV) 12.2%). The physician referred all of them to a higher center before further delay. Thirty-four babies with other congenital heart diseases were found using pulse oximetry examination. Also, 108cases of hypoxemia due to other causes were found and monitored. Conclusion Critical congenital heart diseases are a significant cause of death among neonates and require early diagnosis and emergent medical and surgical management. They are associated with hypoxemia, and this principle can be used to screen them using a pulse oximeter.

Ultrasonographic evaluation of endotracheal tube position in newborns with CHD.

In this prospective observational clinical study, we performed point-of-care ultrasound for endotracheal tube localisation in 65 intubated newborns with critical CHD. After routine radiography, each patient underwent point-of-care ultrasound examination with a portable ultrasonography device for endotracheal tube end-carina measurement. Endotracheal tube end-carina measurements on chest radiographs were compared with ultrasound images. The mean gestational age and birth weight were 37.8 ± 2.19 weeks and 2888 ± 595 g, respectively. Ultrasound images were obtained after an average of 2.08 ± 1.6 hours from the radiographs. The average ultrasound time allocated to each patient was 5 minutes. The mean endotracheal tube tip-to-carina distance on chest X-ray and ultrasound were optimally 1.33 ± 0.64 cm and 1.43 ± 0.67 cm, respectively. There was no significant difference between chest X-ray and ultrasound measurements in endotracheal tube end-carina distance values evaluated by the Bland-Altman method (mean difference 0.10 cm, p = 0.068). There was a linear correlation between the endotracheal tube tip-carina distance in ultrasound and radiography evaluation (r2 = 0.60, p < 0.001). It has been concluded that critical CHDs are frequently accompanied by vascular anomalies, and the endotracheal tube tip-carina distance measurement can be used by determining the carina section as a guide point in the ultrasonographic evaluation of the endotracheal tube location in this patient population.

Substance Exposure and Adverse Neonatal Outcomes: A Population-Based Cohort Study

To estimate the independent and combined effects of in utero exposures on birth outcomes in a rural population. The study used population-level data (2020-2022) from a state-wide surveillance tool (Working in Appalachia to identify at-risk infants, Critical congenital heart disease, and Hearing loss) in West Virginia. Outcomes included low birth weight, preterm birth, small for gestational age, and birth weight in grams. Exposure included a composite variable with 8 levels of 3 exposure (opioids, stimulants, and cannabis) categories. Analyses were adjusted for sociodemographic covariates using multiple logistic and linear regression analyses. Of the 34 412 singleton live births, 1 in 8 newborns (12.2%) had in utero exposure(s) to opioids, stimulants, and/or cannabis, 11.5% were preterm, 7.9% had low birthweight, 9.6% were small for gestational age, and mean birth weight was 3249±563.6g. Preterm birth was associated with stimulant alone exposure (aOR, 1.40; 95% CI, 1.03-1.89) and stimulant and cannabis concurrent exposure (aOR, 1.69; 95% CI, 1.16, 2.47). Low birthweight was associated with opioids alone (aOR, 1.34; 95% CI, 1.10, 1.63), cannabis alone (aOR, 1.31; 95% CI, 1.13 to -1.52), opioid and cannabis (aOR, 1.61; 95% CI, 1.12 to -2.31), and opioids, stimulants, and cannabis concurrent exposures (aOR, 2.27; 95% CI, 1.43-3.61). Five exposure categories were associated with lower birth weights (adjusted mean difference range. -72 to -211g). Small for gestational age was associated with opioids alone (aOR, 1.48; 95% CI, 1.24-1.78), cannabis alone (aOR, 1.49; 95% CI, 1.31-1.69), and opioids and cannabis concurrent exposures (aOR, 1.91; 95% CI, 1.36-2.67). We showed complex associations between in utero substance exposures, preterm birth, birth weight, and sociodemographic factors in a rural population. The results may inform policy efforts to improve maternal and child health in socioeconomically disadvantaged and underserved rural populations.

Wayfinding through the "ocean of the great unknown": how lactating parents establish a direct breastfeeding relationship with an infant with critical CHD.

Lactating parents of infants hospitalised for critical congenital heart disease (CHD) face significant barriers to direct breastfeeding. While experiences of directly breastfeeding other hospitalised neonates have been described, studies including infants with critical CHD are scarce. There is no evidence-based standard of direct breastfeeding care for these infants, and substantial practice variation exists. To explain how direct breastfeeding is established with an infant hospitalised for critical CHD, from lactating parents' perspectives. This study is a qualitative grounded dimensional analysis of interviews with 30 lactating parents of infants with critical CHD who directly breastfed within 3 years. Infants received care from 26 United States cardiac centres; 57% had single ventricle physiology. Analysis included open, axial, and selective coding; memoing; member checking; and explanatory matrices. Findings were represented by a conceptual model, "Wayfinding through the 'ocean of the great unknown'." The core process of Wayfinding involved a nonlinear trajectory requiring immense persistence in navigating obstacles, occurring in a context of life-and-death consequences for the infant. Wayfinding was characterised by three subprocesses: navigating the relationship with the healthcare team; protecting the direct breastfeeding relationship; and doing the long, hard work. Primary influencing conditions included relentless concern about weight gain, the infant's clinical course, and the parent's previous direct breastfeeding experience. For parents, engaging in the Wayfinding process to establish direct breastfeeding was feasible and meaningful - though challenging. The conceptual model of Wayfinding explains how direct breastfeeding can be established and provides a framework for research and practice.

The implications of an integrated management model of prenatal diagnosis/postnatal treatment for premature infants with critical congenital heart disease-a case-control study.

The high death rate and medical costs of critical congenital heart disease (CCHD) in preterm infants has resulted in significant burdens on both countries and individuals. It is unclear how this affects the mortality of the integrated management model of prenatal diagnosis/postnatal treatment. This study explored the effects of the delivery classification scale for fetal heart and postnatal infants' CCHD on prenatal and postnatal integrated treatment strategies to improve the effectiveness of disease management in CCHD. This study was a case-control study, which retrospectively analyzed the clinical data of 79 preterm infants (<37 weeks) who underwent prenatal diagnosis and postpartum treatment in Guangdong Provincial People' s Hospital (China) from June 2017 to June 2019. According to the diagnostic and exclusion criteria, the subjects were divided into prenatal and postpartum diagnostic groups. The clinical characteristics and survival outcomes of patients were collected and compared. The delivery classification scale was used for risk stratification and patient management. Among the 79 patients included in this study, 48 (60.76%) were diagnosed prenatally, and 31 (39.24%) were diagnosed postpartum. The prenatal diagnosis group was born slightly earlier during the gestation period [35.00 (33.29-35.86) vs. 35.57 (34.14-36.71) weeks, P<0.05], and their mothers were older (33.23±5.22 vs. 30.43±6.37 years, P<0.05). The difference in the admission age between the groups was statistically significant [0 (0-5.5) vs. 7 (5-16) days, P<0.001]. The median survival time of the prenatal diagnosis group was higher than the postnatal diagnosis group [48 months (95% CI: 40.78-57.29) vs. 39 months (95% CI: 34.41-44.32), P<0.05]. The 3-year survival rates of the classes I, II, and III were 92.31% (12/13), 59.09% (13/22), and 38.46% (5/13), respectively. The survival of class I as denoted in the delivery classification scale was better than classes II or III (class I vs. II, P<0.05; class I vs. III, P<0.05). Unexpectedly, the hospitalisation costs were lower and total in-hospital days were shorter in the postnatal diagnosis group. The results indicated that the integrated management of a prenatal diagnosis/postnatal treatment approach in premature infants may be effective. Furthermore, the delivery classification scale has a particular prognostic value for CCHD. The authors anticipate that their management model will be able to contribute to the shift from a reactive monodisciplinary system to a proactive, multidisciplinary and dynamic management paradigm in premature infants with CCHD in the near future.

Stenting of Ductus Arteriosus for Duct-Dependent Pulmonary Circulation: An Emergency and Life Saving Procedure

BACKGROUND: Critical congenital heart defect with ductal-dependent pulmonary flow presents as a life-threatening condition. The patency of ductus arteriosus is required to supply blood flow into the lung. Stent implantation into ductus arteriosus has been proposed as non-surgical management for critical congenital heart disease with duct-dependent. CASE DESCRIPTION: We present full-term newborns who had critical congenital heart disease with ductal-dependent pulmonary blood blow and successfully managed with ductal stenting. Patient A, a 13-days-old male newborn had Tetralogy of Fallot-Pulmonary Atresia, restrictive Ventricular Septal Defect (VSD) and patent ductus arteriosus (PDA). Patient B, a 5-days-old female newborn had Tricuspid Atresia, restrictive VSD, PDA, and multiple congenital anomaly. Patient C, a 2-days-old female newborn had PA-IVS, PDA with stretched Patent Foramen Ovale (PFO). Prostaglandin-E1 infusion was started at first and stopped 6 hours prior to the procedure. All patients underwent ductus arteriosus stenting via femoral artery approach with drug-eluting stent. Pulmonary vascular markings were increased and oxygen saturations were improved in all of the patient. 5-month follow up, patients were in stable condition and prepared for surgical correction. DISCUSSION: Ductal stenting in patient with duct-dependent pulmonary circulation appears to be an alternative to surgery. It provides bridging palliation until the time of definitive surgery. The effectiveness of the procedure was highlighted by the fact that all the patients showed a significant improvement in arterial saturation and pulmonary vascular marking. CONCLUSION: Stenting of ductus arteriosus is feasible and safe with great result. Early detection and timely management are imperative to save the life. KEYWORDS: critical congenital heart defect, ductus arteriosus, ductal stenting, ductal-dependent pulmonary blood flow

Prenatal diagnosis of critical congenital heart defects in Kazakhstan: a single-center 8 years’ experience

Purpose. To give a quantitative and qualitative assessment of the results of prenatal diagnosis of critical congenital heart defects in the Republic of Kazakhstan based on the treatment of newborns at the head cardiac surgery center.Methods. A retrospective analysis of the medical records of 511 newborns with critical congenital heart defects treated at the National Research Cardiac Surgery Center (NRCSC) in 2012–2019 was performed. The proportion of those operated on was 474/511 (92.8%). The studied parameters were the presence of prenatal diagnosis of critical congenital heart defects (yes/no); prenatal diagnosis formulation; postnatal diagnosis formulation; discrepancy between pre- and postnatal diagnoses (yes/no); for a prenatally diagnosed newborn — the gestational age at the time of the diagnosis and the region where the diagnosis was firstly made.Results. Prenatally, 297/511 (58.1%) newborns were diagnosed. The rate of discrepancies between pre- and postnatal diagnoses was 62/288 (21.5%). According to the timing of the prenatal diagnosis, newborns were distributed as follows: first trimester screening — 20/272 (7.4%), second trimester screening — 139/272 (51.1%), third trimester screening — 113/272 (41.5%). Up to 22 weeks of gestation, 71/272 (26.1%) patients were diagnosed. Among the newborns treated at the NRCSC, the proportion of those diagnosed prenatally in different regions of the Kazakhstan varies from 20% to 100%.Conclusions. 1) Prenatal diagnosis of critical congenital heart defects is carried out in all regions of Kazakhstan, providing an acceptable level of detection; 2) in half of the cases, critical congenital heart defects are diagnosed during the second screening, however, there is an experience of their accurate detection as early as during the first screening; 3) individually, doctors of ultrasound diagnostics in Kazakhstan apply an extended protocol for examining the fetal heart; 4) a significant proportion of fetuses diagnosed before the 22nd week of gestation shows the choice of families in favor of carrying of a pregnancy when a critical congenital heart defects is detected; 5) the greatest difficulty for prenatal diagnosis is presented by patients with total anomalous pulmonary veins return.

Critical Congenital Heart Disease Sadly Diagnosed at Autopsy in Calabar, South-South Nigeria

Congenital heart disease (CHD) is the most common type of birth defect causing more deaths in the first year of life than any other birth defect. Medical practice in a third-world country is faced with numerous challenges, created by poor health facilities and the unavailability of basic imaging studies at the community level where the majority of the poor resides. We present a case series of 4 neonates with a ratio of 1: 3 admitted into the neonatology unit of the University of Calabar Teaching Hospital, Calabar, Nigeria. Two of the patients were delivered at home by a traditional birth attendant via spontaneous vertex delivery and the rest two were booked cases in a private and obstetric unit of the University of Calabar Teaching Hospital. Their age ranged were 18 hours to 27 days of life and their common presentation were poor sucking, difficulty in breathing, central cyanosis and failure to thrive. A fetal echocardiogram was carried out for one of the cases and there was strong clinical suspicion of congenital heart disease but the precise type is unknown. The other three could not afford the necessary investigations required. They were all oxygen-dependent till death. Pathologic-anatomic findings showed a rare Critical congenital heart defect of the univentricular heart chamber of various types for the three cases and a case of TGA. The Immediate cause of death for all four cases was congestive cardiac failure.