RESUMO
Prenatal diagnosis of critical congenital heart disease (CCHD) has improved over time, and previous studies have identified CCHD subtype and socioeconomic status as factors influencing rates of prenatal diagnosis. Our objective of this single-center study was to compare prenatal diagnosis rates of newborns with CCHD admitted for cardiac intervention from the COVID-19 pandemic period (March 2020 to March 2021) to the pre-pandemic period and identify factors associated with the lack of CCHD prenatal diagnosis. The overall rate of CCHD and rates of the various CCHD diagnoses were calculated and compared with historical data collection periods (2009-2012 and 2013-2016). Compared with the 2009-2012 pre-pandemic period, patients had 2.17 times higher odds of having a prenatal diagnosis of CCHD during the pandemic period controlling for lesion type (aOR = 2.17, 95% CI 1.36-3.48, p = 0.001). Single ventricle lesions (aOR 6.74 [4.64-9.80], p < 0.001) and outflow tract anomalies (aOR 2.20 [1.56-3.12], p < 0.001) had the highest odds of prenatal diagnosis compared with the remaining lesions. Patients with outflow tract anomalies had higher odds for prenatal detection in the pandemic period compared with during the 2009-2012 pre-pandemic period (aOR 2.01 [1.06-3.78], p = 0.031). In conclusion, prenatal detection of CCHD among newborns presenting for cardiac intervention appeared to have improved during the pandemic period.
Assuntos
COVID-19 , Cardiopatias Congênitas , Diagnóstico Pré-Natal , Humanos , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/diagnóstico , COVID-19/epidemiologia , COVID-19/diagnóstico , Feminino , Recém-Nascido , Gravidez , Diagnóstico Pré-Natal/estatística & dados numéricos , Diagnóstico Pré-Natal/métodos , Masculino , SARS-CoV-2 , Estudos Retrospectivos , PandemiasRESUMO
OBJECTIVE: To determine whether weight gain velocity (g/kg/day) 30 days after the initiation of feeds after cardiac surgery and other clinical outcomes improve in infants with single ventricle physiology fed an exclusive human milk diet compared with a mixed human and bovine diet. STUDY DESIGN: In this multicenter, randomized, single blinded, controlled trial, term neonates 7 days of age or younger with single ventricle physiology and anticipated cardiac surgical palliation within 30 days of birth were enrolled at 10 US centers. Both groups received human milk if fed preoperatively. During the 30 days after feeds were started postoperatively, infants in the intervention group received human milk fortified once enteral intake reached 60 mL/kg/day with a human milk-based fortifier designed for term neonates. The control group received standard fortification with formula once enteral intake reached 100 mL/kg/day. Perioperative feeding and parenteral nutrition study algorithms were followed. RESULTS: We enrolled 107 neonates (exclusive human milk = 55, control = 52). Baseline demographics and characteristics were similar between the groups. The median weight gain velocity at study completion was higher in exclusive human milk vs control group (12 g/day [IQR, 5-18 g/day] vs 8 g/day [IQR, 0.4-14 g/day], respectively; P = .03). Other growth measures were similar between groups. Necrotizing enterocolitis of all Bell stages was higher in the control group (15.4 % vs 3.6%, respectively; P = .04). The incidence of other major morbidities, surgical complications, length of hospital stay, and hospital mortality were similar between the groups. CONCLUSIONS: Neonates with single ventricle physiology have improved short-term growth and decreased risk of NEC when receiving an exclusive human milk diet after stage 1 surgical palliation. TRIAL REGISTRATION: This trial is registered with ClinicalTrials.gov (www. CLINICALTRIALS: gov, Trial ID: NCT02860702).
Assuntos
Enterocolite Necrosante , Leite Humano , Lactente , Recém-Nascido , Humanos , Animais , Bovinos , Recém-Nascido Prematuro , Recém-Nascido de muito Baixo Peso , Dieta , Enterocolite Necrosante/epidemiologia , Aumento de PesoRESUMO
Congenital complete heart block (CCHB) affects 1 in 20,000 newborns. This study evaluates fetal and neonatal risk factors predictive of neonatal pacemaker placement in antibody-mediated complete heart block. The Children's Hospital Los Angeles institutional fetal, pacemaker, and medical record databases were queried for confirmed SSA/SSB cases of CCHB between January 2004 and July 2019. Cases excluded were those with a diagnosis beyond the neonatal period, diagnosis of a channelopathy, or if maternal antibody status was unknown. We recorded the gestational age (GA), birth weight (BW), fetal heart rates (FHRs) of the last echocardiogram before delivery, specific neonatal ECG and echocardiogram findings, age at pacemaker placement, and mortality. Of 43 neonates identified with CCHB, 27 had confirmed maternal antibody exposure. Variables associated with neonatal pacemaker implantation were FHRs < 50 bpm (p = 0.005), neonatal heart rates < 52 bpm (p = 0.015), and neonatal left ventricular fractional shortening (FS) percentages < 34% (p = 0.03). On multivariate analysis, FHR remained significant (p = 0.03) and demonstrated an increased risk of neonatal pacemaker placement by an odds ratio of 12.5 (95% CI 1.3-116, p = 0.05). The median GA at which the FHR was obtained was 34 weeks (IQR 26-35 weeks). Neonatal pacemaker placement was highly associated with a FHR < 50 bpm, neonatal HR < 52 bpm, and neonatal FS < 34%. FHRs at 34 weeks GA (IQR 26-35 weeks) correlated well with postnatal heart rates and were predictive of neonatal pacemaker placement.
Assuntos
Bloqueio Atrioventricular , Marca-Passo Artificial , Bloqueio Atrioventricular/terapia , Criança , Feminino , Frequência Cardíaca Fetal , Humanos , Recém-Nascido , Gravidez , Terceiro Trimestre da Gravidez , Cuidado Pré-NatalRESUMO
BACKGROUND: Chagas disease is a pathogenic parasitic infection with approximately 8 million cases worldwide and greater than 300,000 cases in the United States (U.S.). Chagas disease can lead to chronic cardiomyopathy and cardiac complications, with variable cardiac presentations in pediatrics making it difficult to recognize. The purpose of our study is to better understand current knowledge and experience with Chagas related heart disease among pediatric cardiologists in the U.S. METHODS: We prospectively disseminated a 19-question survey to pediatric cardiologists via 3 pediatric cardiology listservs. The survey included questions about demographics, Chagas disease presentation and experience. RESULTS: Of 139 responses, 119 cardiologists treat pediatric patients in the U.S. and were included. Most providers (87%) had not seen a case of Chagas disease in their practice; however, 72% also had never tested for it. The majority of knowledge-based questions about Chagas disease cardiac presentations were answered incorrectly, and 85% of providers expressed discomfort with recognizing cardiac presentations in children. Most respondents selected that they would not include Chagas disease on their differential diagnosis for presentations such as conduction anomalies, myocarditis and/or apical aneurysms, but would be more likely to include it if found in a Latin American immigrant. Of respondents, 87% agreed that they would be likely to attend a Chagas disease-related lecture. CONCLUSIONS: Pediatric cardiologists in the U.S. have seen very few cases of Chagas disease, albeit most have not sent testing or included it in their differential diagnosis. Most individuals agreed that education on Chagas disease would be worth-while.
Assuntos
Cardiologistas , Doença de Chagas , Competência Clínica , Conhecimentos, Atitudes e Prática em Saúde , Pediatras , Idade de Início , Doença de Chagas/diagnóstico , Doença de Chagas/epidemiologia , Doença de Chagas/fisiopatologia , Doença de Chagas/terapia , Humanos , Estudos Prospectivos , Inquéritos e Questionários , Estados UnidosRESUMO
INTRODUCTION: This study was designed to evaluate ventricular size, shape, and function in recipient twins following laser therapy for twin-twin transfusion syndrome (TTTS), using novel speckle-tracking techniques. METHODS: This retrospective study enrolled patients that underwent fetal laser surgery for TTTS and had fetal echocardiograms (FE) performed pre- and post-operatively (op), with adequate resolution in the 4-chamber view for analysis, using a speckle-tracking software, to compute the size, shape, and function of both the right (RV) and left (LV) ventricles. Values were indexed to published normal values. Pre- and post-laser Z-score values for each of the measurements were compared using the Student's t-test, with significance defined as P < 0.05. RESULTS: Fifteen TTTS candidate pregnancies that underwent laser therapy between 2010 and 2017, with adequate pre- and post-op FE, were selected for the analysis. Post-op FE at 28.5 ± 8.3 days showed a significant decrease in RV base dimension, increased LV base dimension, and improvements in many functional measurements: LV global and free wall strain, LV fractional area change, LV basal-apical fractional change, and LV and RV 24-segment fractional shortening (FS) of the basal segments. CONCLUSIONS: Cardiac remodeling, following laser surgery in TTTS recipient twins, was demonstrated in the basal portion of both the RV and LV with improved biventricular function.
Assuntos
Ecocardiografia/métodos , Coração Fetal/diagnóstico por imagem , Transfusão Feto-Fetal/cirurgia , Remodelação Ventricular , Adulto , Ecocardiografia/estatística & dados numéricos , Feminino , Coração Fetal/fisiologia , Humanos , Terapia a Laser , Gravidez , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVES: Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) carry high perinatal mortality. Past studies have focused on cardiac predictors of mortality; we sought to describe the fetal echo (FE) extracardiac Dopplers in this cohort and determine their association with perinatal mortality. METHOD: Fetuses with EA/TVD at 23 centers from 2005-2011 were included for retrospective study. Doppler pattern and velocity of the umbilical artery (UA), umbilical vein (UV), ductus venosus (DV), and middle cerebral artery (MCA) were collected. Bivariate and multivariate analyzes were performed. The primary outcome measure was perinatal mortality, defined as fetal demise or neonatal death. RESULTS: Of 190 cases that met eligibility criteria, alterations were seen in 50% of UA, 16% of UV, 48% of DV, and 8% of MCA Doppler indices on the last FE (median 27.4 weeks). Independent predictors of perinatal mortality included abnormal UA Doppler pattern of absence or reversed end diastolic flow (OR 9.7) and UV velocity z score <1 (OR 2.5), in addition to diagnosis <32 weeks (OR 4.2) and tricuspid valve (TV) annulus z score ≥6 (OR 5.3). CONCLUSION: Abnormal UA Doppler pattern and decreased UV velocity are independent predictors of perinatal mortality in EA/TVD fetuses and should be used to refine mortality risk and guide perinatal management.
Assuntos
Anomalia de Ebstein/mortalidade , Mortalidade Infantil/tendências , Insuficiência da Valva Tricúspide/mortalidade , Ultrassonografia Doppler/normas , Estudos de Coortes , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/diagnóstico por imagem , Feminino , Feto/anormalidades , Feto/diagnóstico por imagem , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Gravidez , Resultado da Gravidez/epidemiologia , Estudos Retrospectivos , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Ultrassonografia Doppler/métodos , Ultrassonografia Doppler/estatística & dados numéricosRESUMO
OBJECTIVES: We aimed to evaluate the effect of technical aspects of fetal aortic valvuloplasty (FAV) on procedural risks and pregnancy outcomes. BACKGROUND: FAV is performed in cases of severe mid-gestation aortic stenosis with the goal of preventing hypoplastic left heart syndrome (HLHS). METHODS: The International Fetal Cardiac Intervention Registry was queried for fetuses who underwent FAV from 2002 to 2018, excluding one high-volume center. RESULTS: The 108 fetuses had an attempted cardiac puncture (mean gestational age [GA] 26.1 ± 3.3 weeks). 83.3% of attempted interventions were technically successful (increased forward flow/new aortic insufficiency). The interventional cannula was larger than 19 g in 70.4%. More than one cardiac puncture was performed in 25.0%. Intraprocedural complications occurred in 48.1%, including bradycardia (34.1%), pericardial (22.2%) or pleural effusion (2.7%) requiring drainage, and balloon rupture (5.6%). Death within 48 hr occurred in 16.7% of fetuses. Of the 81 patients born alive, 59 were discharged home, 34 of whom had biventricular circulation. More than one cardiac puncture was associated with higher complication rates (p < .001). Larger cannula size was associated with higher pericardial effusion rates (p = .044). On multivariate analysis, technical success (odds ratio [OR] = 10.9, 95% confidence interval [CI] = 2.2-53.5, p = .003) and later GA at intervention (OR = 1.5, 95% CI = 1.2-1.9, p = .002) were associated with increased odds of live birth. CONCLUSIONS: FAV is an often successful but high-risk procedure. Multiple cardiac punctures are associated with increased complication and fetal mortality rates. Later GA at intervention and technical success were independently associated with increased odds of live birth. However, performing the procedure later in gestation may miss the window to prevent progression to HLHS.
Assuntos
Estenose da Valva Aórtica/terapia , Valvuloplastia com Balão , Cateterismo Cardíaco , Terapias Fetais , Síndrome do Coração Esquerdo Hipoplásico/prevenção & controle , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/mortalidade , Estenose da Valva Aórtica/fisiopatologia , Valvuloplastia com Balão/efeitos adversos , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/mortalidade , Europa (Continente) , Feminino , Morte Fetal/etiologia , Terapias Fetais/efeitos adversos , Terapias Fetais/mortalidade , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Nascido Vivo , América do Norte , Gravidez , Sistema de Registros , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Resultado do Tratamento , Ultrassonografia Pré-NatalRESUMO
The objective of this analysis was to update trends in LOS and costs by survivorship and ECMO use among neonates with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation surgery using 2016 data from the Healthcare Cost and Utilization Project Kids' Inpatient Database. We identified neonates ≤ 28 days old with HLHS undergoing Stage 1 surgery, defined as a Norwood procedure with modified Blalock-Taussig (BT) shunt, Sano modification, or both. Multivariable regression with year random effects was used to compare LOS and costs by hospital region, case volume, survivorship, and ECMO vs. no ECMO. An E-value analysis, an approach for conducting sensitivity analysis for unmeasured confounding, was performed to determine if unmeasured confounding contributed to the observed effects. Significant differences in total costs, LOS, and mortality were noted by hospital region, ECMO use, and sub-analyses of case volume. However, other than ECMO use and mortality, the maximum E-value confidence interval bound was 1.71, suggesting that these differences would disappear with an unmeasured confounder 1.71 times more associated with both the outcome and exposure (e.g., socioeconomic factors, environment, etc.) Our findings confirm previous literature demonstrating significant resource utilization among Norwood patients, particularly those undergoing ECMO use. Based on our E-value analysis, differences by hospital region and case volume can be explained by moderate unobserved confounding, rather than a reflection of the quality of care provided. Future analyses on surgical quality must account for unobserved factors to provide meaningful information for quality improvement.
Assuntos
Custos Hospitalares/estatística & dados numéricos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Tempo de Internação/estatística & dados numéricos , Procedimentos de Norwood/mortalidade , Bases de Dados Factuais , Oxigenação por Membrana Extracorpórea/economia , Oxigenação por Membrana Extracorpórea/mortalidade , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/economia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Masculino , Procedimentos de Norwood/economia , Cuidados Paliativos/economia , Cuidados Paliativos/estatística & dados numéricos , Melhoria de Qualidade , Estudos Retrospectivos , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: Patients with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS) have wide variation in right ventricle (RV) size, systolic function, and diastolic function at birth. Establishment of antegrade pulmonary blood flow creates the potential for RV dilation from chronic pulmonary insufficiency. Future surgical decisions are based on RV size and function, largely supported by longitudinal studies of patients with Tetralogy of Fallot (TOF). Given potential differences in RV physiology and lack of similar data in PAIVS/CPS, the objective of this study was to determine differences in RV size, systolic function, and diastolic function between patients with PAIVS/CPS versus TOF. METHODS: We retrospectively collected cardiovascular magnetic resonance (CMR) data in 27 patients with PAIVS/CPS (ages 13.3 ± 8.8 years) and 78 with TOF (11.4 ± 5.4 years). RV volumes, ejection fraction (EF), regurgitant fraction, end-diastolic forward flow across the pulmonary valve, and right atrial cross-sectional area were calculated. RESULTS: There was no difference between the groups in RV end-diastolic volume (RVEDVi), RVEF, or pulmonary regurgitation. RVEF tended to decrease in TOF when RVEDVi exceeded 164 ml/m2. In PAIVS/CPS, RVEDVi less frequently reached 164 ml/m2 and was not associated with RVEF. There was worse RV diastolic dysfunction in PAIVS/CPS, with 1.5 times larger right atrial area and two times higher pulmonary end-diastolic forward flow (p < 0.0001). CONCLUSIONS: Patients with PAIVS/CPS have similar RV size, systolic function, and pulmonary regurgitation as TOF. However, impaired RV diastolic function may limit extremes of RV dilatation and impact long-term management of PAIVS/CPS.
Assuntos
Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Atresia Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/fisiopatologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia/métodos , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Atresia Pulmonar/complicações , Atresia Pulmonar/cirurgia , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Volume Sistólico , Tetralogia de Fallot/cirurgia , Resultado do Tratamento , Adulto JovemRESUMO
The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Fetal pulmonary and vertical vein Dopplers were analyzed as predictors of PPVO. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy. Of the prenatally diagnosed patients, 63% also had heterotaxy. TAPVR repair was performed in 135 cases and survival to discharge was 82% (112/137). Heterotaxy was the only independent predictor of mortality on multiple regression analysis [OR 5.5 (CI 1.3-16.7), p = 0.02]. PPVO was associated with preoperative acidosis, need for inhaled nitric oxide, and more emergent surgery, but not postoperative mortality. Fetal vertical vein Doppler peak velocity > 0.74 m/s mmHg predicted PPVO (93% sensitivity; 83% specificity) while pulmonary vein Doppler did not. TAPVR has severe neonatal morbidity and mortality with low prenatal diagnosis rates in the absence of heterotaxy. Patients with obstructed TAPVR had greater preoperative morbidity, but only heterotaxy was independently associated with increased postoperative mortality. Vertical vein velocity helped prenatally identify those at risk of PPVO.
Assuntos
Procedimentos Cirúrgicos Cardiovasculares/métodos , Síndrome de Heterotaxia/complicações , Pneumopatia Veno-Oclusiva/complicações , Síndrome de Cimitarra/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Procedimentos Cirúrgicos Cardiovasculares/efeitos adversos , Procedimentos Cirúrgicos Cardiovasculares/mortalidade , Ecocardiografia Doppler/métodos , Feminino , Síndrome de Heterotaxia/diagnóstico , Síndrome de Heterotaxia/epidemiologia , Humanos , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Gravidez , Prognóstico , Circulação Pulmonar , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Pneumopatia Veno-Oclusiva/diagnóstico por imagem , Pneumopatia Veno-Oclusiva/cirurgia , Estudos Retrospectivos , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/cirurgia , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Fetal echocardiography facilitates the prenatal diagnosis of infants with congenital heart disease (CHD) and through sequential examinations, allows assessment of fetal hemodynamics and cardiovascular status from the time of diagnosis to delivery. Fetal cardiologists have created diagnostic protocols aimed at risk stratifying severity and potential postnatal compromise in fetuses with CHD, and identifying those who may require special intervention at birth or within the first days of life. In this article, we review fetal cardiovascular physiology, the progression of CHD in utero and fetal echocardiographic findings used for risk stratification of newborns with CHD, as well as some of the basic principles of planning for the neonatal resuscitation and initial transitional care of these complex newborns.
Assuntos
Ecocardiografia/métodos , Coração Fetal/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/terapia , Assistência Perinatal/métodos , Ultrassonografia Pré-Natal/métodos , Salas de Parto , Feminino , Hemodinâmica , Humanos , Recém-Nascido , GravidezRESUMO
OBJECTIVES: To evaluate risk for congenital heart disease (CHD) in recipient twins with circular shunt physiology (CSP). METHODS: This prospective study enrolled twin-twin transfusion syndrome (TTTS) cases from 2006 to 2015. Fetal echocardiography (FE) was performed before laser surgery when cardiac involvement was suspected. Diagnosis of recipient twin CSP required tricuspid and pulmonary regurgitation, right ventricular dysfunction, and flow reversal in the ductus arteriosus. Outcomes were assessed at 30 days after birth. RESULTS: Of the 496 TTTS pregnancies, 20 (4%) met the criteria for CSP. Among those born alive, who had documented cardiac outcomes (n = 457), patients with CSP were more likely to have CHD, specifically right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 22 of 439 [5.0%], odd ratio [OR] 7.29, 95% confidence interval [CI] 2.05-24.72, P = .0025). Of the recipient twins with preoperative FE (n = 259, 52%) who were born alive and had documented cardiac outcomes (n = 242), those with CSP were still more likely to have right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 14 of 224 [6.3%], OR 5.77, CI 1.54-20.92, P = .0077). With both analyses, twins with CSP had higher Quintero stage, but similar patient characteristics and 30-day mortality compared with those without CSP. Subgroup analyses of the CSP cohort identified no differences in preoperative characteristics or FE findings predictive of CHD. CONCLUSIONS: Recipient twins with preoperative CSP were at increased risk for postnatal right ventricular outflow tract obstruction, but appeared to have comparable survival after fetal laser surgery despite these dramatic pathophysiological prenatal findings. Preoperative FE in TTTS remains important for prediction of postnatal CHD.
Assuntos
Coração Fetal/fisiopatologia , Transfusão Feto-Fetal/complicações , Cardiopatias Congênitas/complicações , Terapia a Laser , Cuidados Pré-Operatórios/métodos , Obstrução do Fluxo Ventricular Externo/complicações , Feminino , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Recém-Nascido , Gravidez , Estudos Prospectivos , Risco , Análise de Sobrevida , Gêmeos , Ultrassonografia Pré-Natal/métodosRESUMO
BACKGROUND: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. METHODS AND RESULTS: Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). CONCLUSION: In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.
Assuntos
Anomalia de Ebstein/mortalidade , Valva Tricúspide/anormalidades , Aborto Eugênico , Adulto , Peso ao Nascer , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Síndrome de Down/complicações , Síndrome de Down/mortalidade , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/embriologia , Anomalia de Ebstein/cirurgia , Feminino , Idade Gestacional , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/embriologia , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Mortalidade Hospitalar , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/mortalidade , Masculino , Cuidados Paliativos , Derrame Pericárdico/etiologia , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Valva Tricúspide/fisiopatologia , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/cirurgia , Ultrassonografia Pré-Natal , Adulto JovemRESUMO
In this study, we present a case of prenatally diagnosed tetralogy of Fallot with a double aortic arch, correlating images from fetal echocardiography, transthoracic echocardiography, and cardiac MRI.
Assuntos
Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Diagnóstico Pré-Natal , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Adulto , Ecocardiografia Doppler , Feminino , Feto/diagnóstico por imagem , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Gravidez , Nascimento a TermoRESUMO
OBJECTIVE: Twin survivors of twin-twin transfusion syndrome (TTTS) may be at risk for early onset of cardiovascular disease. The aim of this study was to determine prevalence and risk factors for elevated blood pressure (BP) among children treated with selective laser photocoagulation of communicating vessels. STUDY DESIGN: Data were prospectively collected from surviving children treated for TTTS with laser surgery from 2008 through 2010. Systolic BP (SBP) and diastolic BP (DBP) were obtained from 91 child survivors at age 24 months (±6 weeks) and evaluated based on age, sex, and height percentile. BP percentiles were calculated for each patient and categorized as normal (<95%) or abnormal (>95%). Clinical variables were evaluated using multilevel regression models to evaluate risk factors for elevated BP. RESULTS: BP was categorized as normal in 38% and abnormal in 62% of twin survivors based on percentile for sex, age, and height; a comparable distribution was found for DBP elevation. There were no differences between donor and recipient twins for absolute SBP and DBP or BP classification. In a multivariate analysis, significant risk factors for higher SBP included prematurity (ß -0.54; 95% confidence interval [CI], -0.99 to -0.09; P = .02), higher weight percentile (ß 0.24; 95% CI, 0.05-0.42; P = .01), and presence of cardiac disease (ß 0.50; 95% CI, 0.10-0.89; P = .01). Prematurity was also a significant risk for abnormal DBP (odds ratio, 0.89; 95% CI, 0.80-1.00; P = .05). CONCLUSION: Child survivors of TTTS had elevated SBP and DBP measurements at 2 years of age, with no differences seen between former donor and recipient twins. Prematurity may be a risk factor for elevated BP measurements in this population. Future studies are warranted to ascertain whether these cardiovascular findings persist over time.
Assuntos
Terapias Fetais , Transfusão Feto-Fetal/cirurgia , Hipertensão/etiologia , Fotocoagulação a Laser , Complicações Pós-Operatórias , Pré-Escolar , Feminino , Transfusão Feto-Fetal/complicações , Seguimentos , Humanos , Hipertensão/diagnóstico , Hipertensão/epidemiologia , Modelos Lineares , Modelos Logísticos , Masculino , Análise Multivariada , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Gravidez , Prevalência , Estudos Prospectivos , Fatores de RiscoRESUMO
OBJECTIVE: The aim of this study was to evaluate outcomes for neonates with critical congenital heart disease (CHD) requiring emergent neonatal cardiac intervention (ENCI). METHODS: Neonates < 30 days of age that underwent ENCI at <48 h of age were retrospectively enrolled over a 2-year period. RESULTS: Forty-seven neonates met inclusion criteria for ENCI comprising nine cardiac defects that underwent 25 catheterizations and 22 cardiothoracic surgeries. The main groups were d-transposition of the great arteries (DTGA) and total anomalous pulmonary venous return (TAPVR). Prenatal detection was 38% overall: higher for single ventricle (86%) and heterotaxy (75%) than for DTGA (28%) or TAPVR (13%). Mortality was 11.1% (2/18) in prenatally diagnosed versus 13.8% (4/29) in postnatally diagnosed neonates (p = 0.86). Prenatal detection was associated with shorter mean hospital stay: 16.8 versus 30.3 days (p = 0.03). Prenatally diagnosed patients had lower preoperative inotropic scores (p = 0.02), less acidosis (pH; p = 0.09), but decreased likelihood of spontaneous labor (p = 0.01), lower gestational age (p = 0.01), and lower birth weight (p = 0.01). CONCLUSIONS: Fewer deaths occurred in neonates with prenatal detection of their critical CHD requiring ENCI. However, there was no statistical difference in survival demonstrated for prenatally diagnosed neonates in this small cohort. Prenatal detection did improve preoperative clinical status and shorten hospital length of stay.
Assuntos
Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Diagnóstico Pré-Natal/estatística & dados numéricos , Serviços Médicos de Emergência , Cardiopatias Congênitas/terapia , Humanos , Recém-Nascido , Terapia Intensiva Neonatal/estatística & dados numéricos , Los Angeles/epidemiologia , Estudos RetrospectivosRESUMO
OBJECTIVE: We sought to evaluate the impact of mode of delivery (MOD) on early outcome for neonates diagnosed prenatally with major forms of congenital heart disease (CHD). METHODS: We retrospectively studied infants admitted, over a 2-year period, to a single institution for cardiac intervention. Infants were grouped on the basis of timing of diagnosis (prenatal/postnatal) and MOD--planned (induced labor or planned cesarean delivery) versus spontaneous labor. Multivariate logistic regression was used to evaluate independent predictors for MOD and early outcomes. RESULTS: Of 329 patients, 45% received a prenatal diagnosis of CHD. A prenatal diagnosis of CHD increased the likelihood for planned delivery [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.6 to 4.5, p < 0.001]. Newborns prenatally diagnosed with CHD were more likely to have been delivered between 8 am and 6 pm, Monday through Friday (OR 2.3, 95% CI 1.1 to 4.8, p = 0.019). However, MOD had no statistical impact on Apgar score, duration of pre-operative intubation, and survival to surgery or to discharge. The Risk Adjustment for Congenital Heart Surgery 1 surgical mortality score was the only independent predictor of hospital mortality. CONCLUSIONS: In our experience, although a prenatal diagnosis of CHD decreased the likelihood of spontaneous labor, MOD had no demonstrable impact on neonatal outcome.
Assuntos
Parto Obstétrico/estatística & dados numéricos , Cardiopatias Congênitas/diagnóstico , Diagnóstico Pré-Natal/estatística & dados numéricos , California/epidemiologia , Feminino , Cardiopatias Congênitas/mortalidade , Humanos , Recém-Nascido , Recém-Nascido Pequeno para a Idade Gestacional , Gravidez , Estudos RetrospectivosRESUMO
Large placental chorioangiomas (>4 cm) can precipitate severe polyhydramnios, fetal anemia, growth restriction, high-output cardiac failure, hydrops, and fetal demise. We report a case of a large chorioangioma that was treated in a stepwise fashion with amnioreduction to ameliorate maternal discomfort, followed by fetoscopic laser ablation of the feeding vessels after rapid evolution of heart failure. Although amnioreduction was helpful in improving maternal symptoms, we suspect that the drop in intrauterine pressure from the amniotic fluid decompression may have resulted in increased tumor perfusion, thereby promoting fetal deterioration due to a 'steal' phenomenon. Close scrutiny of the fetal status via ultrasound is required, particularly if amnioreduction is necessary, and definitive treatment should be considered once early signs of heart failure develop. Fetoscopic laser ablation of the feeding vessels is a feasible definitive treatment of a large chorioangioma in cases in which perfusion of the tumor is via a superficial nonterminus umbilical artery. Future reports should describe the anatomy of the blood supply to the chorioangioma, in the hopes that further guidelines for surgical candidacy may be established.
Assuntos
Descompressão Cirúrgica/efeitos adversos , Sofrimento Fetal/cirurgia , Fetoscopia , Hemangioma/cirurgia , Fotocoagulação , Doenças Placentárias/cirurgia , Adulto , Débito Cardíaco Elevado/etiologia , Débito Cardíaco Elevado/cirurgia , Feminino , Sofrimento Fetal/diagnóstico por imagem , Sofrimento Fetal/etiologia , Idade Gestacional , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/cirurgia , Hemangioma/irrigação sanguínea , Hemangioma/complicações , Hemangioma/diagnóstico por imagem , Humanos , Nascido Vivo , Doenças Placentárias/diagnóstico por imagem , Poli-Hidrâmnios/etiologia , Poli-Hidrâmnios/cirurgia , Gravidez , Resultado do Tratamento , Ultrassonografia Doppler , Ultrassonografia Pré-Natal/métodosRESUMO
OBJECTIVE: To assess postnatal prevalence of congenital heart disease (CHD) in surviving twins treated for twin-twin transfusion syndrome (TTTS) with laser surgery. METHODS: Patients who underwent laser surgery for TTTS at a single institution were prospectively enrolled between January 2009 and May 2010. Preoperative fetal echocardiography was performed. Perinatal outcomes were obtained via postnatal echocardiograms and review of medical records. CHD was defined as unequivocal structural cardiac pathology; patent foramen ovale and patent ductus arteriosus were excluded. RESULTS: Fifty pregnancies with TTTS were enrolled, with the following Quintero stage distribution: stage I, 10%; stage II, 22%; stage III, 50%; and stage IV, 18%. Of the 88 fetuses born alive, 84 patients (95.5%) completed postnatal cardiac evaluation. Overall postnatal prevalence of CHD was 15.5% (13 of 84): 15.6% of recipient twins and 15.4% of donor twins. Three patients required cardiac intervention during infancy. Among liveborn twins with CHD, the overall prenatal detection rate was 30.8% (4/13), comprising 16.7% (1/6) of donors compared with 42.9% (3/7) of recipients. CONCLUSION: Following successful laser surgery for TTTS, an increased and comparable postnatal prevalence of CHD occurs among both former recipients and donors.
Assuntos
Transfusão Feto-Fetal/complicações , Transfusão Feto-Fetal/cirurgia , Cardiopatias Congênitas/etiologia , Fotocoagulação a Laser/métodos , Gêmeos , Adulto , California/epidemiologia , Ecocardiografia/métodos , Feminino , Transfusão Feto-Fetal/mortalidade , Idade Gestacional , Cardiopatias Congênitas/mortalidade , Humanos , Recém-Nascido , Gravidez , Resultado da Gravidez , Gravidez Múltipla , Prevalência , Estudos Prospectivos , Taxa de Sobrevida , Ultrassonografia Pré-NatalRESUMO
Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow-up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1-30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.