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BACKGROUND: During perinatal transition in hypoplastic left heart syndrome (HLHS), reduced systemic blood flow (Qs) and cerebral blood flow and increased pulmonary blood flow (Qp) are observed, contributing to hemodynamic instability. The aim of the present study was to explore whether similar or discordant perinatal changes occur in critical pulmonary outflow tract obstruction (POFO) compared with HLHS and healthy control subjects. METHODS: Echocardiography was prospectively performed at 36 to 39 gestational weeks and then serially from 6 to 96 hours after birth, before cardiac intervention. Combined cardiac output (CCO), superior vena cava (SVC) flow rate, Qs and Qp, and resistance indices (RIs) in the middle cerebral artery (MCA), celiac artery, and superior mesenteric artery were compared among the three groups. RESULTS: In fetal POFO (n = 19) and HLHS (n = 31), CCO was comparable with that in control subjects (n = 21) because of elevated stroke volume, but CCO in POFO was lower compared with HLHS (P < .01). Compared with control subjects, POFO CCO was lower at 6 hours after delivery but comparable at 24 to 96 hours. In contrast, from 6 to 96 hours, the HLHS group had higher CCO than POFO and control subjects. Compared with control subjects, both neonates with POFO and those with HLHS had lower Qs and SVC flow (POFO at 24 hours, P < .001; HLHS 6-hour Qs and 6- to 24-hour SVC flow). Compared with control subjects, Qp was increased in POFO at 48 to 96 hours (P < .05) and in HLHS at all time points (P < .001). Compared with fetal MCA RI, postnatal MCA RI was acutely higher in both POFO and HLHS, whereas in control subjects, it tended to decrease postnatally. Celiac artery RI and superior mesenteric artery pulsatility index were higher in POFO and HLHS from 6 to 48 hours vs control subjects. CONCLUSIONS: POFO and HLHS demonstrate divergent acute hemodynamic changes in the early neonatal period, with early decreased CCO in POFO and increased CCO in HLHS. Both demonstrate early compromise in Qs and SVC (cerebral flow) and ongoing altered splanchnic flow.
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BACKGROUND: The perinatal transition is characterized by acute changes in cardiac loading. Compared with normal newborn combined cardiac output (CCO), single right ventricular (RV) output of neonates with hypoplastic left heart syndrome (HLHS) is markedly greater. The aim of this study was to examine the mechanisms of cardiac adaptation that facilitate this perinatal transition from late fetal to early neonatal life in HLHS. METHODS: Prospectively recruited pregnancies complicated by fetal HLHS (n = 35) and healthy control subjects (Ctrl; n = 17) underwent serial echocardiography in late gestation (38 ± 1 weeks) and 6, 24, and 48 hours after birth. Cardiac function was assessed using conventional, Doppler tissue, and speckle-tracking echocardiography. RESULTS: Term fetuses with HLHS had RV output comparable with Ctrl CCO via higher stroke volume. Compared with both left ventricular and RV indices of Ctrl, they exhibited globular and dilated right ventricles with reduced relative wall thickness (0.40 ± 0.08 vs 0.49 ± 0.10, P < .01), increased Tei index' (HLHS vs Ctrl left ventricle/Ctrl right ventricle: sphericity index, 0.9 ± 0.25 vs 0.5 ± 0.10/0.6 ± 0.11; RV area index, 28 ± 6 vs 15 ± 3/17 ± 5 cm2/m2; Tei index', 0.65 ± 0.11 vs 0.43 ± 0.07/0.45 ± 0.09; P < .0001 for all). Neonates with HLHS generated elevated RV cardiac output compared with Ctrl CCO via higher heart rate and stroke volume, with further RV dilatation, increased longitudinal systolic strain at 48 hours (-17 ± 4% vs -14 ± 3%/ 14 ± 5%) with reduced circumferential and rotational myocardial deformation and altered diastolic function. Neonates with HLHS also demonstrated right atrial enlargement with increased longitudinal strain: 6 hours (33 ± 12% vs 26 ± 6%), 24 hours (37 ± 15% vs 26 ± 13%), and 48 hours (38 ± 11% vs 24 ± 13%) (P < .0001). CONCLUSIONS: Term fetuses with HLHS exhibit altered RV geometry and RV systolic and diastolic functional parameters. After birth, further alterations in these cardiac parameters likely reflect adaptation to acutely altered RV loading from increasing cardiac output and pulmonary artery flow demands.
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OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.
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Consenso , Anomalia de Ebstein , Humanos , Recém-Nascido , Anomalia de Ebstein/cirurgia , Anomalia de Ebstein/fisiopatologia , Lactente , Medição de Risco , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fatores de Risco , Resultado do Tratamento , Cirurgia Torácica/normasRESUMO
BACKGROUND: Targeted neonatal echocardiography (TNE) and hemodynamic consultation have typically been performed by physicians. The Stollery Children's Hospital neonatal intensive care unit (NICU) expanded their TNE training program to include neonatal nurse practitioners (NNPs), the first in North America. PURPOSE: This study examines the thoughts and perceptions of clinicians about the incorporation of NNPs providing TNE and hemodynamic consultation and investigates key facilitators and challenges for consideration when planning future training, expansion of service in Edmonton, or beyond. METHODS: In this descriptive study using qualitative methodology, purposive sampling was used to invite NICU clinicians to participate. Using a semistructured topic guide, a focus group and 2 individual interviews were conducted. RESULTS: Participants were supportive of NNPs. Advantages included increased access to service, acquisition and retention of skills, provision of patient-centered care, and leveraged interpersonal relationships in the decision-making process. Key aspects of program expansion included climate and culture of the NICU, presence of adequate patient volume, and resources to support training. IMPLICATIONS FOR PRACTICE AND RESEARCH: Support across disciplines and the collaborative working nature of the NICU are key factors in the success of the program's development and implementation. Benefits of having NNPs on the TNE team were clearly expressed. Benefits to the health system included rapid access to hemodynamic information allowing for care based on specific pathophysiology and additional local capacity to perform TNE and reducing demand on other trained providers. Additional research could consider parental views of NNPs performing TNE and hemodynamic consultation as well as the accuracy of diagnosis between the NNPs and physician group.
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Atitude do Pessoal de Saúde , Ecocardiografia , Unidades de Terapia Intensiva Neonatal , Profissionais de Enfermagem , Humanos , Recém-Nascido , Ecocardiografia/métodos , Profissionais de Enfermagem/psicologia , Equipe de Assistência ao Paciente , Pesquisa Qualitativa , Feminino , Enfermagem Neonatal/métodos , Enfermagem Neonatal/educação , Masculino , Grupos FocaisRESUMO
BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS) and hypoplastic left heart syndrome (HLHS) encountered over the study period. Forty fetuses were considered high risk for acute cardiorespiratory instability: 15 with d-TGA/IVS and 7 with HLHS with restrictive atrial septum, 4 with absent pulmonary valve syndrome, 3 with obstructed anomalous pulmonary veins, 2 with severe Ebstein anomaly, 2 with thoracic/intracardiac tumors, and 7 others. Pediatric cardiac operating room delivery occurred for 33 but not for 7 (5 with d-TGA/IVS, 2 with HLHS with restrictive atrial septum). For high-risk cases, fetal echocardiography had a positive predictive value of 50% for intervention/extracorporeal membrane oxygenation/death at ≤2 hours and 70% at ≤24 hours. Of "low-risk" cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. Fetal echocardiography for predicting intervention/extracorporeal membrane oxygenation/death at ≤2 hours had a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 80% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively. CONCLUSIONS: Fetal echocardiography can predict the need for urgent intervention in a majority with d-TGA/IVS and HLHS and in half of the entire spectrum of high-risk cardiac disease.
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Cardiopatias Congênitas , Síndrome do Coração Esquerdo Hipoplásico , Transposição dos Grandes Vasos , Gravidez , Recém-Nascido , Feminino , Humanos , Criança , Salas Cirúrgicas , Coração Fetal/diagnóstico por imagem , Coração Fetal/cirurgia , Ultrassonografia Pré-Natal/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Estudos RetrospectivosRESUMO
OBJECTIVE: Pulmonary artery sling is a rare congenital anomaly accounting for 2% of all patients with vascular anomalies that cause airway obstruction. In the normal heart, the left (LPA) and right (RPA) pulmonary arteries arise in the intrapericardial space. However, in the pulmonary artery sling, the LPA trunk arises in the extrapericardial space from the posterior aspect of the mid RPA and courses posterior to the trachea causing tracheal compression and, at times, bronchial compression. While a full spectrum of congenital cardiac pathology can be identified before birth, only a few case reports document the prenatal diagnosis of an Left pulmonary artery sling (LPAS). METHOD: We retrospectively identified all cases of prenatal LPAS from three Canadian fetal cardiology centers (2015-2022). RESULTS: Using the 3-vessel-tracheal view via fetal echocardiography (FE), four fetuses from three pregnancies demonstrated abnormal origin of the LPA from RPA and echogenic trachea. In one of two affected monochorionic twins coronal imaging demonstrated a significant narrowing of the large airways consistent with significant airway obstruction. CONCLUSION: Prenatal detection of LPAS by FE is possible and should prompt an evaluation for airway obstruction in the coronal view. Investigating associated lesions and genetic testing are recommended for informed shared decision making.
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Artéria Pulmonar , Ultrassonografia Pré-Natal , Humanos , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Feminino , Gravidez , Estudos Retrospectivos , Adulto , Ecocardiografia/métodos , Obstrução das Vias Respiratórias/diagnóstico por imagem , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Malformações Vasculares/diagnóstico , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/complicaçõesRESUMO
Atrial septal defects (ASDs) are common in neonates. Although past studies suggest ASDs ≥ 3 mm in term neonates (TNs) are less likely to close, there is paucity of data regarding the natural history in preterm neonates (PNs), information that would inform surveillance. We sought to compare spontaneous closure rates and need for intervention for ASDs in TNs/near term (≥ 36 weeks) versus PNs (< 36 weeks). We included all TNs and PNs who underwent echocardiography at ≤ 1 month between 2010 and 2018 in our institution with an ASD ≥ 3 mm, without major congenital heart disease, and with repeat echocardiogram(s). Spontaneous resolution was defined as size diminution to < 3 mm or closure. We included 156 TNs (mean gestational age at birth 38.6 ± 1.4 weeks) and 156 PNs (29.6 ± 3.7 weeks) with a mean age at follow-up of 16 ± 19 and 15 ± 21 months, respectively (p = 0.76). Based on maximum color Doppler diameter, in TNs, ASD resolution occurred in 95% of small (3-5 mm), 87% of moderate (5.1-8 mm), and 60% of large (> 8 mm) defects; whereas, in PNs, resolution occurred in 79% of small, 76% of moderate, and 33% of large ASDs. There was a significant association between size and ASD resolution in TNs (p = 0.003), but not PNs (p = 0.17). Overall, ASD resolution rate was higher in TNs (89%) versus PNs (78%) (p = 0.009), and fewer TNs (1%) compared to PNs (7%) required ASD intervention (p = 0.02). Most ASDs identified in TNs and PNs spontaneously resolve. PNs, however, demonstrate lower ASD resolution and higher intervention rates within all size groups. These data should inform follow-up of affected neonates.
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Comunicação Interatrial , Recém-Nascido , Humanos , Comunicação Interatrial/diagnóstico por imagem , Ecocardiografia , Ultrassonografia Doppler em Cores , Resultado do Tratamento , Cateterismo CardíacoRESUMO
Several studies have suggested an inverse relationship between lower socioeconomic status (SES) and the incidence of congenital heart disease (CHD) among live births. We sought to examine this relationship further in a Canada-wide population study, exploring CHD subtypes, trends, and associated noncardiac abnormalities. Infants born in Canada (less Quebec) from 2008 to 2018 with CHD requiring intervention in the first year were identified using ICD-10 codes through the Canadian Institute for Health Information Discharge Abstract Database. Births of CHD patients were stratified by SES (census-based income quintiles) and compared against national birth proportions using X2 tests. Proportions with extracardiac defects (ED) and nonlethal genetic syndromes (GS) were also explored. From 2008 to 2018, 7711 infants born with CHD were included. The proportions of major CHD distributed across SES quintiles were 27.1%, 20.1%, 19.2%, 18.6%, and 15.0% from lowest to highest, with significant differences relative to national birth proportions (22.0%, 20.0%, 20.6%, 20.7%, and 16.7% from lowest (1) to highest (5)) (p < 0.0001). No temporal trends in the CHD proportions across SES categories were observed over the study period. The distribution across SES quintiles was different only for specific CHD subtypes (double-outlet right ventricle (n = 485, p = 0.03), hypoplastic left heart syndrome (n = 547, p = 0.006), heterotaxy (n = 224, p = 0.03), tetralogy of Fallot (n = 1007, p = 0.008), truncus arteriosus (n = 126, p < 0.0001), and ventricular septal defect (n = 1916, p < 0.0001)), with highest proportions observed in the lowest quintile. The proportion of the total population with ED but not GS was highest in lower SES quintiles (< 0.0001) commensurate with increased proportion of CHD. Our study suggests a negative association between SES and certain CHD lesions and ED.
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Cardiopatias Congênitas , Síndrome do Coração Esquerdo Hipoplásico , Lactente , Humanos , Incidência , Canadá/epidemiologia , Cardiopatias Congênitas/epidemiologia , Classe SocialRESUMO
BACKGROUND: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD. METHODS: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test. RESULTS: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m2 (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]). CONCLUSION: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.
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Anomalia de Ebstein , Cardiopatias Congênitas , Doenças das Valvas Cardíacas , Disfunção Ventricular Esquerda , Gravidez , Feminino , Humanos , Recém-Nascido , Anomalia de Ebstein/complicações , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/cirurgia , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologiaRESUMO
OBJECTIVE: This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti-SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second-degree atrioventricular block (AVB). METHODS: Women with anti-SSA/Ro autoantibodies by commercial testing were stratified into high and low anti-52-kD and/or 60-kD SSA/Ro titers applying at-risk thresholds defined by previous evaluation of AVB pregnancies. The high-titer group performed fetal heart rate and rhythm monitoring (FHRM) thrice daily and weekly/biweekly echocardiography from 17-26 weeks. Abnormal FHRM prompted urgent echocardiography to identify AVB. RESULTS: Anti-52-kD and/or 60-kD SSA/Ro met thresholds for monitoring in 261 of 413 participants (63%); for those, AVB frequency was 3.8%. No cases occurred with low titers. The incidence of AVB increased with higher levels, reaching 7.7% for those in the top quartile for anti-60-kD SSA/Ro, which increased to 27.3% in those with a previous child who had AVB. Based on levels from 15 participants with paired samples from both an AVB and a non-AVB pregnancy, healthy pregnancies were not explained by decreased titers. FHRM was considered abnormal in 45 of 30,920 recordings, 10 confirmed AVB by urgent echocardiogram, 7 being second-degree AVB, all <12 hours from normal FHRM and within another 0.75 to 4 hours to echocardiogram. The one participant with second/third-degree and two participants with third-degree AVB were diagnosed by urgent echocardiogram >17 to 72 hours from an FHRM. Surveillance echocardiograms detected no AVB when the preceding interval FHRM recordings were normal. CONCLUSION: High-titer antibodies are associated with an increased incidence of AVB. Anti-SSA/Ro titers remain stable over time and do not explain the discordant recurrence rates, suggesting that other factors are required. Fetal heart rate and rhythm (FHRM) with results confirmed by a pediatric cardiologist reliably detects conduction abnormalities, which may reduce the need for serial echocardiograms.
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Bloqueio Atrioventricular , Complicações na Gravidez , Criança , Gravidez , Humanos , Feminino , Bloqueio Atrioventricular/diagnóstico , Bloqueio Atrioventricular/epidemiologia , Autoanticorpos , Estudos Prospectivos , Anticorpos Antinucleares , Ecocardiografia/métodosRESUMO
Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21-27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.
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Bloqueio Atrioventricular , Cardiopatias Congênitas , Atresia Pulmonar , Transposição dos Grandes Vasos , Insuficiência da Valva Tricúspide , Feminino , Humanos , Gravidez , Lactente , Transposição das Grandes Artérias Corrigida Congenitamente , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/complicações , Insuficiência da Valva Tricúspide/complicações , Bloqueio Atrioventricular/complicações , Estudos Retrospectivos , Seguimentos , Diagnóstico Pré-Natal , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/complicações , Coração Fetal/diagnóstico por imagem , Coração Fetal/patologia , Arritmias Cardíacas/complicações , Morte FetalRESUMO
Major congenital heart disease (CHD) is associated with impaired neurodevelopment (ND), partly from prenatal insults. In this study we explore associations between 2nd and 3rd trimester umbilical (UA) and middle cerebral artery (MCA) pulsatility index (PI = systolic-diastolic velocities/mean velocity) in fetuses with major CHD and 2-year ND and growth outcomes. Eligible patients included those with a prenatal diagnosis of CHD from 2007 to 2017 without a genetic syndrome who underwent previously defined cardiac surgeries and 2-year biometric and ND assessments in our program. UA and MCA-PI Z-scores at fetal echocardiography were examined for relationships with 2-year Bayley Scales of Infant and Toddler Development and biometric Z-scores. Data from 147 children was analyzed. Second and 3rd trimester fetal echocardiograms were performed at 22.4 ± 3.7 and 34.7 ± 2.9 weeks (mean ± SD), respectively. Multivariable regression analysis showed an inverse relationship between 3rd trimester UA-PI for all CHD and cognitive - 1.98 (- 3.37, - 0.59), motor - 2.57 (- 4.15, - 0.99), and language - 1.67 (- 3.3, - 0.03) (effect size and 95th confidence interval) ND domains (p < 0.05), with the strongest relationships in the single ventricle and hypoplastic left heart syndrome subgroups. No association was found for 2nd trimester UA-PI or any trimester MCA-PI and ND or between UA or MCA-PI and 2-year growth parameters. Increased 3rd trimester UA-PI, reflecting an altered late gestation fetoplacental circulation, relates to worse 2-year ND in all domains.
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Cardiopatias Congênitas , Artérias Umbilicais , Feminino , Gravidez , Humanos , Terceiro Trimestre da Gravidez , Artérias Umbilicais/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Diagnóstico Pré-Natal , Segundo Trimestre da Gravidez , Feto , Ultrassonografia Pré-Natal , Idade Gestacional , Fluxo PulsátilAssuntos
Cardiopatias , Traqueia , Humanos , Feminino , Gravidez , Traqueia/diagnóstico por imagem , Ultrassonografia Pré-NatalRESUMO
Background Socioeconomic status (SES) impacts clinical outcomes associated with severe congenital heart disease (sCHD). We examined the impact of SES and remoteness of residence (RoR) on congenital heart disease (CHD) outcomes in Canada, a jurisdiction with universal health insurance. Methods and Results All infants born in Canada (excluding Quebec) from 2008 to 2018 and hospitalized with CHD requiring intervention in the first year were identified. Neighborhood level SES income quintiles were calculated, and RoR was categorized as residing <100 km, 100 to 299 km, or >300 km from the closest of 7 cardiac surgical programs. In-hospital mortality at <1 year was the primary outcome, adjusted for preterm birth, low birth weight, and extracardiac pathology. Among 7711 infants, 4485 (58.2%) had moderate CHD (mCHD) and 3226 (41.8%) had sCHD. Overall mortality rate was 10.5%, with higher rates in sCHD than mCHD (13.3% versus 8.5%, respectively). More CHD infants were in the lowest compared with the highest SES category (27.1% versus 15.0%, respectively). The distribution of CHD across RoR categories was 52.3%, 21.3%, and 26.4% for <100 km, 100 to 299 km, and >300 km, respectively. Although SES and RoR had no impact on sCHD mortality, infants with mCHD living >300 km had a higher risk of mortality relative to those living <100 km (adjusted odds ratio [aOR], 1.43 [95% CI, 1.11-1.84]). Infants with mCHD within the lowest SES quintile and living farthest away had the highest risk for mortality (aOR, 1.74 [95% CI, 1.08-2.81]). Conclusions In Canada, neither RoR nor SES had an impact on outcomes of infants with sCHD. Greater RoR, however, may contribute to higher risk of mortality among infants with mCHD.
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Cardiopatias Congênitas , Nascimento Prematuro , Canadá/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Características de Residência , Classe SocialRESUMO
BACKGROUND: Coronary artery fistulae (CAFs) are abnormal connections of a coronary artery to a cardiac chamber or vessel. There is a paucity of data regarding clinical outcomes, especially when detected prenatally. METHODS: This was a multicenter retrospective cohort study of all CAF cases from 2002 to 2016. Clinical characteristics and outcomes were compared between the prenatal and postnatal cohorts. A scoping literature review of prenatal CAFs was completed. RESULTS: CAFs were diagnosed prenatally in 12 (median, 23 weeks' gestation; interquartile range, 17-36 weeks' gestation) and postnatally in 94 (median, 2.8 years; interquartile range, 0-15 years) cases. Structural heart defects were present in five (42%) prenatal and 19 (20%) postnatal cases (P = .011) and genetic conditions in five (42%) and 14 (15%), respectively (P = .001). CAFs were considered large in 12 (100%) prenatal versus 14 (15%) postnatal cases (P < .001). The CAF distribution was similar between cohorts: 39 (67%) from the left and 19 (33%) from the right coronary artery, with the most common exit sites being the main pulmonary artery 54 (51%), right ventricle 30 (28%), and right atrium 12 (11%). Of prenatal cases, all large at presentation, none progressed, six (50%) resolved by birth, and one (8%) underwent elective neonatal ligation. Of postnatal cases, one presented in cardiogenic shock, and no other case had ventricular dysfunction, arrhythmias, or ischemic changes. Nine (10%) with large shunts underwent intervention (seven percutaneous, two surgical), of whom three were symptomatic. Two (17%) prenatal and two (2%) postnatal cases had coronary abnormalities, two with normal results on stress perfusion imaging. Postnatal death occurred in two (17%) prenatal and four (4%) postnatal cases (P = .05). Of the total 36 prenatal cases reported in the literature, including the 12 cases in the present series, 10 (28%) had clinical symptoms at birth, including three (8%) with cardiogenic shock, and 19 (53%) underwent intervention. CONCLUSIONS: Prenatally and postnatally encountered CAFs are associated with a good prognosis for most, with many not requiring intervention. Although half of the prenatal CAFs resolved prenatally, given the risk for cardiogenic shock at birth and heart failure in early infancy, appropriate perinatal planning and postnatal surveillance is warranted.
Assuntos
Doença da Artéria Coronariana , Fístula , Cardiopatias Congênitas , Recém-Nascido , Gravidez , Feminino , Humanos , Estudos Retrospectivos , Choque Cardiogênico , Ecocardiografia , Ultrassonografia Pré-Natal , Estudos Multicêntricos como AssuntoRESUMO
OBJECTIVE: The effect of expanded obstetrical ultrasound cardiac views on the diagnosis of fetal congenital heart disease (CHD) has not been fully examined at a population level. We hypothesized there has been a significant increase in the prenatal detection of CHD in Alberta, particularly for CHD associated with cardiac outflow tract and 3-vessel view abnormalities. METHODS: Using provincial databases, we retrospectively identified all fetuses and infants diagnosed between 2008 and 2018 in Alberta with major CHD requiring surgical intervention within the first postnatal year. We evaluated individual lesions and categorized CHDs into the following groups based on the obstetrical ultrasound cardiac views required for detection: (1) 4-chamber view (e.g., hypoplastic left heart syndrome, Ebstein's anomaly, single ventricle); (2) outflow tract view (e.g., tetralogy of Fallot, d-transposition, truncus arteriosus); (3) 3-vessel or other non-standard cardiac views (e.g., coarctation, anomalous pulmonary veins); and (4) isolated ventricular septal defects using any view. RESULTS: Of 1405 cases of major CHD, 814 (58%) were prenatally diagnosed. Over the study period, prenatal detection increased in all groups, with the greatest increase observed for groups 1 and 2 (75%-88%; P = 0.008 and 56%-79%; P = 0.0002, respectively). Although rates of prenatal detection also increased for groups 3 and 4 (27%-43%; P = 0.007 and 13%-30%; P = 0.04, respectively), fewer than half of the cases in each group were detected prenatally, even in more recent years. CONCLUSIONS: While rates of prenatal detection of CHD have significantly improved during the past decade, many defects with abnormal 3-vessel and non-standard views, as well as isolated ventricular septal defects, still go undetected.