RESUMO
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.
Assuntos
Bloqueio Atrioventricular , Complicações na Gravidez , Criança , Gravidez , Humanos , Feminino , Bloqueio Atrioventricular/diagnóstico , Bloqueio Atrioventricular/epidemiologia , Autoanticorpos , Estudos Prospectivos , Anticorpos Antinucleares , Ecocardiografia/métodosRESUMO
Persistent pressure overload can cause cardiac hypertrophy and progressive heart failure (HF). The authors developed a pressure-overload HF model of juvenile mice to study the cardiac response to pressure overload that may be applicable to clinical processes in children. Severe thoracic aortic banding (sTAB) was performed using a 28-gauge needle for 40 juvenile (age, 3 weeks) and 47 adult (age, 6 weeks) C57BL/6 male mice. To monitor the structural and functional changes, M-mode echocardiography was performed for conscious mice that had undergone sTAB and sham operation. Cardiac hypertrophy, dilation, and HF occurred in both juvenile and adult mice after sTAB. Compared with adults, juvenile HF is characterized by greater impairment of ventricular contractility and less hypertrophy. In addition, juvenile mice had significantly higher rates of survival than adult mice during the early postoperative weeks. Consistent with clinical HF seen in children, juvenile banded mice demonstrated a lower growth rate than either adult banded mice or juvenile control mice that had sham operations. The authors first developed a juvenile murine model of pressure-overload HF. Learning the unique characteristics of pressure-overload HF in juveniles should aid in understanding age-specific pathologic changes for HF development in children.
Assuntos
Pressão Sanguínea , Cardiomegalia/patologia , Modelos Animais de Doenças , Insuficiência Cardíaca/patologia , Contração Miocárdica , Fatores Etários , Animais , Velocidade do Fluxo Sanguíneo , Cardiomegalia/diagnóstico por imagem , Progressão da Doença , Ecocardiografia , Insuficiência Cardíaca/diagnóstico por imagem , Humanos , Estimativa de Kaplan-Meier , Masculino , Camundongos , Miocárdio , Toracotomia , Estados UnidosRESUMO
Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21â¯=â¯71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (pâ¯=â¯0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (pâ¯=â¯0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.
Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Canal Arterial/fisiopatologia , Anomalia de Ebstein/tratamento farmacológico , Terapias Fetais/métodos , Idade Gestacional , Insuficiência da Valva Pulmonar/tratamento farmacológico , Insuficiência da Valva Tricúspide/tratamento farmacológico , Valva Tricúspide/anormalidades , Constrição , Canal Arterial/diagnóstico por imagem , Duração da Terapia , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Feminino , Coração Fetal , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/tratamento farmacológico , Cardiopatias Congênitas/fisiopatologia , Humanos , Ibuprofeno/uso terapêutico , Indometacina/uso terapêutico , Nascido Vivo , Troca Materno-Fetal , Mortalidade Perinatal , Gravidez , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/fisiopatologia , Ultrassonografia Doppler , Ultrassonografia Pré-NatalRESUMO
Congenital diaphragmatic hernia (CDH) presenting beyond the neonatal period is commonly perceived to be rare. With reported frequencies of 2.6% to 20% of all CDH, it may be an overlooked cause of mortality. Variable symptomatology makes its diagnosis challenging. We report the sudden death of a 3-month-old patient shortly after hospital discharge following congenital heart surgery. Autopsy findings associated the patient's demise with migrated abdominal contents in the chest through a Bochdalek hernia defect. No indications of CDH existed before hospital discharge. Relevant issues pertaining to congenital heart disease, CDH, and importance of autopsy in this context are discussed.
Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Cardiopatias Congênitas/cirurgia , Hérnias Diafragmáticas Congênitas , Autopsia , Evolução Fatal , Feminino , Hérnia Diafragmática/etiologia , Hérnia Diafragmática/mortalidade , Humanos , Lactente , Morte Súbita do LactenteRESUMO
BACKGROUND: Doppler tissue imaging may help identify children with dyssynchrony who could benefit from resynchronization therapy. However, few studies have quantified dyssynchrony measures in children; no study has investigated the relationship among age, heart rate, and dyssynchrony measures in children; and no study has quantified cross-correlation delay in children. The aim of this study was to test the hypotheses that measures of left ventricular dyssynchrony would correlate with age, primarily because of the correlation between heart rate and age, and that children with cardiomyopathy would have left ventricular dyssynchrony. METHODS: Sixty healthy children and 11 children with dilated cardiomyopathy were prospectively enrolled. Seven dyssynchrony measures were quantified: septal-to-lateral delay, peak velocity difference, the standard deviations of times to peak in 12 segments in systole and diastole, and cross-correlation delay in systole, diastole, and the whole cycle. RESULTS: The seven dyssynchrony measures were either not correlated with age or only weakly correlated with age after correcting for heart rate using Bazett's formula. Septal-to-lateral delay, peak velocity difference, and the standard deviation of times to peak in 12 segments in systole showed dyssynchrony in 57% to 85% of normal controls, compared with 20% for cross-correlation delay in the whole cycle and 3% for the standard deviation of times to peak in 12 segments in diastole. Cross-correlation delay in systole, cross-correlation delay in diastole, cross-correlation delay in the whole cycle, and the standard deviation of times to peak in 12 segments in diastole were elevated in children with dilated cardiomyopathy compared with controls. CONCLUSIONS: Echocardiographic dyssynchrony measures should be corrected for heart rate using Bazett's formula in children. Time-to-peak Doppler tissue imaging dyssynchrony measures classify many healthy children as having abnormalities with the timing of left ventricular contraction, which suggests that the methodology is not accurate in children. In preliminary studies, cross-correlation dyssynchrony measures show elevated systolic and diastolic measures of dyssynchrony in children with dilated cardiomyopathy compared with controls, which deserves further investigation to help identify children who may benefit from resynchronization therapy.