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1.
J Pediatr ; 273: 114120, 2024 Oct.
Article in English | MEDLINE | ID: mdl-38815740

ABSTRACT

OBJECTIVE: To characterize patterns in the geospatial distribution of pre- and postnatally diagnosed congenital heart disease (CHD) across 6 surgical centers. STUDY DESIGN: A retrospective, multicenter case series from the Fetal Heart Society identified patients at 6 centers from 2012 through 2016 with prenatally (PrND) or postnatally (PoND) diagnosed hypoplastic left heart syndrome (HLHS) or d-transposition of the great arteries (TGA). Geospatial analysis for clustering was done by the average nearest neighbor (ANN) tool or optimized hot spot tool, depending on spatial unit and data type. Both point location and county case rate per 10 000 live births were assessed for geographic clustering or dispersion. RESULTS: Of the 453 CHD cases, 26% were PoND (n = 117), and 74% were PrND (n = 336). PrND cases, in all but one center, displayed significant geographic clustering by the ANN. Conversely, PoND cases tended toward geographic dispersion. Dispersion of PoND HLHS occurred in 2 centers (ANN = 1.59, P < .001; and 1.47, P = .016), and PoND TGA occurred in 2 centers (ANN = 1.22, P < .05; and ANN = 1.73, P < .001). Hot spot analysis of all CHD cases (TGA and HLHS combined) revealed clustering near areas of high population density and the tertiary surgical center. Hot spot analysis of county-level case rate, accounting for population density, found variable clustering patterns. CONCLUSION: Geographic dispersion among postnatally detected CHD highlights the need for a wider reach of prenatal cardiac diagnosis tailored to the specific needs of a community. Geospatial analysis can support centers in improving the equitable delivery of prenatal care.


Subject(s)
Heart Defects, Congenital , Hypoplastic Left Heart Syndrome , Humans , Retrospective Studies , Female , Pregnancy , Hypoplastic Left Heart Syndrome/epidemiology , Hypoplastic Left Heart Syndrome/diagnosis , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/diagnosis , Infant, Newborn , Prenatal Diagnosis/statistics & numerical data , Prenatal Diagnosis/methods , United States/epidemiology , Transposition of Great Vessels/epidemiology , Transposition of Great Vessels/diagnosis , Male , Spatial Analysis , Societies, Medical
2.
Circulation ; 145(5): 345-356, 2022 02.
Article in English | MEDLINE | ID: mdl-34865500

ABSTRACT

BACKGROUND: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. METHODS: We retrospectively collected data on patients <21 years old presenting before July 4, 2021, with suspected myocarditis within 30 days of COVID-19 vaccination. Lake Louise criteria were used for cardiac MRI findings. Myocarditis cases were classified as confirmed or probable on the basis of the Centers for Disease Control and Prevention definitions. RESULTS: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1-20.3; interquartile range [IQR], 14.5-17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0-22; IQR, 1-3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0-10; IQR, 2-3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50-15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25-1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction <55% on echocardiogram. Of 97 patients who underwent cardiac MRI at a median 5 days (range, 0-88; IQR, 3-17) from symptom onset, 75 (77.3%) had abnormal findings: 74 (76.3%) had late gadolinium enhancement, 54 (55.7%) had myocardial edema, and 49 (50.5%) met Lake Louise criteria. Among 26 patients with left ventricular ejection fraction <55% on echocardiogram, all with follow-up had normalized function (n=25). CONCLUSIONS: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons <21 years have a mild clinical course with rapid resolution of symptoms. Abnormal findings on cardiac MRI were frequent. Future studies should evaluate risk factors, mechanisms, and long-term outcomes.


Subject(s)
COVID-19 Vaccines/adverse effects , COVID-19/prevention & control , Myocarditis/diagnostic imaging , Myocarditis/physiopathology , Adolescent , Child , Electrocardiography/methods , Female , Humans , Magnetic Resonance Imaging, Cine/methods , Male , Myocarditis/blood , Myocarditis/etiology , Retrospective Studies , Time Factors , Young Adult
3.
Cereb Cortex ; 32(13): 2858-2867, 2022 06 16.
Article in English | MEDLINE | ID: mdl-34882775

ABSTRACT

The subplate is a transient brain structure which plays a key role in the maturation of the cerebral cortex. Altered brain growth and cortical development have been suggested in fetuses with complex congenital heart disease (CHD) in the third trimester. However, at an earlier gestation, the putative role of the subplate in altered brain development in CHD fetuses is poorly understood. This study aims to examine subplate growth (i.e., volume and thickness) and its relationship to cortical sulcal development in CHD fetuses compared with healthy fetuses by using 3D reconstructed fetal magnetic resonance imaging. We studied 260 fetuses, including 100 CHD fetuses (22.3-32 gestational weeks) and 160 healthy fetuses (19.6-31.9 gestational weeks). Compared with healthy fetuses, CHD fetuses had 1) decreased global and regional subplate volumes and 2) decreased subplate thickness in the right hemisphere overall, in frontal and temporal lobes, and insula. Compared with fetuses with two-ventricle CHD, those with single-ventricle CHD had reduced subplate volume and thickness in right occipital and temporal lobes. Finally, impaired subplate growth was associated with disturbances in cortical sulcal development in CHD fetuses. These findings suggested a potential mechanistic pathway and early biomarker for the third-trimester failure of brain development in fetuses with complex CHD. SIGNIFICANCE STATEMENT: Our findings provide an early biomarker for brain maturational failure in fetuses with congenital heart disease, which may guide the development of future prenatal interventions aimed at reducing neurological compromise of prenatal origin in this high-risk population.


Subject(s)
Heart Defects, Congenital , Magnetic Resonance Imaging , Brain/diagnostic imaging , Female , Fetus/diagnostic imaging , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnostic imaging , Humans , Magnetic Resonance Imaging/methods , Pregnancy , Pregnancy Trimester, Second
4.
Circulation ; 143(21): 2049-2060, 2021 05 25.
Article in English | MEDLINE | ID: mdl-33993718

ABSTRACT

BACKGROUND: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. METHODS: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. RESULTS: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. CONCLUSIONS: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.


Subject(s)
Ethnicity/genetics , Hypoplastic Left Heart Syndrome/epidemiology , Racial Groups/genetics , Transposition of Great Vessels/epidemiology , Cohort Studies , Female , Geography , Humans , Male , Retrospective Studies , Social Class
5.
J Pediatr ; 240: 117-121, 2022 01.
Article in English | MEDLINE | ID: mdl-34480916

ABSTRACT

OBJECTIVE: To determine presence of spatial clustering or dispersion of pre and postnatally detected hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (TGA) cases. STUDY DESIGN: This retrospective study examined all patients with a prenatal or postnatal diagnosis of HLHS or TGA who had an initial visit or hospitalization at our tertiary care center over a 5-year period from 2012 to 2016 (n = 105). Using geographic information systems software, the nearest neighbor ratio (NNR) tool was used to determine whether statistically significant clustering or dispersion occurred. RESULTS: Geographic clustering was observed among prenatally diagnosed pooled cases of HLHS and TGA and all total cases (NNR = 0.73 and 0.66, respectively), but not postnatally detected cases (NNR = 1.08). Notably, there was significant dispersion of postnatally detected TGA cases (NNR = 1.22) There was no pattern for prenatally detected TGA or HLHS when analyzed individually. CONCLUSIONS: The spatial distribution of HLHS and TGA is not random; these conditions occur in geographic clusters. Clustering of all patients in the study population and dispersion of postnatal diagnosis of TGA represent opportunities for improved delivery of fetal cardiac care.


Subject(s)
Hypoplastic Left Heart Syndrome/epidemiology , Transposition of Great Vessels/epidemiology , District of Columbia/epidemiology , Female , Humans , Hypoplastic Left Heart Syndrome/diagnosis , Infant, Newborn , Male , Maryland/epidemiology , Pregnancy , Prenatal Diagnosis , Retrospective Studies , Transposition of Great Vessels/diagnosis , Virginia/epidemiology
6.
Am J Perinatol ; 39(S 01): S1-S6, 2022 12.
Article in English | MEDLINE | ID: mdl-36307093

ABSTRACT

The cardiac effects of novel coronavirus disease 2019 (COVID-19) infection on the pediatric heart has become an area of particular interest as elevated cardiac enzymes and abnormalities on echocardiogram and electrocardiogram were seen in a portion of children affected by the virus. In this article, we review the cardiac manifestations of acute COVID-19 infection, multisystem inflammatory syndrome in children, and postvaccine myocarditis. The limited research on the effects of COVID-19 on neonates and infants is also reported. KEY POINTS: · Cardiac involvement from MIS-C is much higher than the risk of COVID-19 vaccine-induced myocarditis.. · Neonates and infants have overall been less affected by COVID-19 than adults and older children.. · At this point in time, there is limited research on the cardiac effects of COVID-19 in neonates..


Subject(s)
COVID-19 , Myocarditis , Vaccines , Adult , Infant, Newborn , Child , Infant , Humans , Adolescent , COVID-19 Vaccines
7.
Cardiol Young ; : 1-3, 2022 Feb 02.
Article in English | MEDLINE | ID: mdl-35105393

ABSTRACT

Prematurity and low birthweight are associated with increased mortality in infants undergoing cardiac surgery. Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation carries one of the highest risks of mortality. We present a patient who was born at 28 weeks of gestation at 1.2 kg, with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, coronary artery atresia, and discontinuous pulmonary arteries, who successfully underwent palliation with a modified Blalock-Taussig shunt, pulmonary arterioplasty, and subsequently a bidirectional Glenn.

8.
Cardiol Young ; 32(8): 1268-1275, 2022 Aug.
Article in English | MEDLINE | ID: mdl-34588092

ABSTRACT

OBJECTIVES: Women carrying a fetus diagnosed with congenital heart disease often experience significant distress because of their medical diagnosis. Given the well-documented impact associated with elevated prenatal stress and critical importance of developing targeted interventions, this study aims to examine stressors, coping and resilience resources, and mental health treatment preferences in pregnant women receiving a congenital heart disease diagnosis to inform the development of a psychological intervention to reduce maternal distress prenatally. METHODS: Three groups of participants were included consisting of two pregnant women carrying a fetus with congenital heart disease, five women of children (4-16 months) with congenital heart disease, and five paediatric cardiology medical providers. Responses were gathered via semi-structured interviews and analysed using qualitative thematic analysis. RESULTS: Information regarding four broad areas were analysed of emotional distress during pregnancy; experience of initial diagnosis; coping and resilience; and perspectives on a mental health intervention in pregnancy. Anxiety regarding baby's future, guilt following diagnosis, and various coping strategies emerged as primary themes among the participant sample. Medical staff corroborated mothers' heightened anxiety and viewed a psychotherapeutic intervention during the prenatal period as essential and complimentary to standard of care. CONCLUSION: We identified salient themes and preferred components for a future psychological intervention delivered prenatally. PRACTICE IMPLICATIONS: Patients' and providers' perspectives regarding the nature of maternal distress, resilience and treatment preferences can inform the development of interventions to support the emotional well-being of pregnant women carrying a fetus with congenital heart disease to optimise care and potentially improve outcomes for fetal brain development.


Subject(s)
Heart Defects, Congenital , Mental Health , Anxiety/psychology , Child , Female , Heart Defects, Congenital/therapy , Humans , Pregnancy , Psychosocial Intervention , Stress, Psychological/psychology , Stress, Psychological/therapy
9.
Cardiol Young ; 32(5): 718-726, 2022 May.
Article in English | MEDLINE | ID: mdl-34348808

ABSTRACT

BACKGROUND: A novel paediatric disease, multi-system inflammatory syndrome in children, has emerged during the 2019 coronavirus disease pandemic. OBJECTIVES: To describe the short-term evolution of cardiac complications and associated risk factors in patients with multi-system inflammatory syndrome in children. METHODS: Retrospective single-centre study of confirmed multi-system inflammatory syndrome in children treated from 29 March, 2020 to 1 September, 2020. Cardiac complications during the acute phase were defined as decreased systolic function, coronary artery abnormalities, pericardial effusion, or mitral and/or tricuspid valve regurgitation. Patients with or without cardiac complications were compared with chi-square, Fisher's exact, and Wilcoxon rank sum. RESULTS: Thirty-nine children with median (interquartile range) age 7.8 (3.6-12.7) years were included. Nineteen (49%) patients developed cardiac complications including systolic dysfunction (33%), valvular regurgitation (31%), coronary artery abnormalities (18%), and pericardial effusion (5%). At the time of the most recent follow-up, at a median (interquartile range) of 49 (26-61) days, cardiac complications resolved in 16/19 (84%) patients. Two patients had persistent mild systolic dysfunction and one patient had persistent coronary artery abnormality. Children with cardiac complications were more likely to have higher N-terminal B-type natriuretic peptide (p = 0.01), higher white blood cell count (p = 0.01), higher neutrophil count (p = 0.02), severe lymphopenia (p = 0.05), use of milrinone (p = 0.03), and intensive care requirement (p = 0.04). CONCLUSION: Patients with multi-system inflammatory syndrome in children had a high rate of cardiac complications in the acute phase, with associated inflammatory markers. Although cardiac complications resolved in 84% of patients, further long-term studies are needed to assess if the cardiac abnormalities (transient or persistent) are associated with major cardiac events.


Subject(s)
COVID-19 , Cardiovascular Abnormalities , Coronary Artery Disease , Pericardial Effusion , COVID-19/complications , Child , Child, Preschool , Humans , Pericardial Effusion/etiology , Retrospective Studies , SARS-CoV-2 , Systemic Inflammatory Response Syndrome
10.
J Pediatr ; 237: 125-135.e18, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34181987

ABSTRACT

OBJECTIVE: To assess demographic, clinical, and biomarker features distinguishing patients with multisystem inflammatory syndrome in children (MIS-C); compare MIS-C sub-phenotypes; identify cytokine biosignatures; and characterize viral genome sequences. STUDY DESIGN: We performed a prospective observational cohort study of 124 children hospitalized and treated under the institutional MIS-C Task Force protocol from March to September 2020 at Children's National, a quaternary freestanding children's hospital in Washington, DC. Of this cohort, 63 of the patients had the diagnosis of MIS-C (39 confirmed, 24 probable) and 61 were from the same cohort of admitted patients who subsequently had an alternative diagnosis (controls). RESULTS: Median age and sex were similar between MIS-C and controls. Black (46%) and Latino (35%) children were over-represented in the MIS-C cohort, with Black children at greatest risk (OR 4.62, 95% CI 1.151-14.10; P = .007). Cardiac complications were more frequent in critically ill patients with MIS-C (55% vs 28%; P = .04) including systolic myocardial dysfunction (39% vs 3%; P = .001) and valvular regurgitation (33% vs 7%; P = .01). Median cycle threshold was 31.8 (27.95-35.1 IQR) in MIS-C cases, significantly greater (indicating lower viral load) than in primary severe acute respiratory syndrome coronavirus 2 infection. Cytokines soluble interleukin 2 receptor, interleukin [IL]-10, and IL-6 were greater in patients with MIS-C compared with controls. Cytokine analysis revealed subphenotype differences between critically ill vs noncritically ill (IL-2, soluble interleukin 2 receptor, IL-10, IL-6); polymerase chain reaction positive vs negative (tumor necrosis factor-α, IL-10, IL-6); and presence vs absence of cardiac abnormalities (IL-17). Phylogenetic analysis of viral genome sequences revealed predominance of GH clade originating in Europe, with no differences comparing patients with MIS-C with patients with primary coronavirus disease 19. Treatment was well tolerated, and no children died. CONCLUSIONS: This study establishes a well-characterized large cohort of MIS-C evaluated and treated following a standardized protocol and identifies key clinical, biomarker, cytokine, viral load, and sequencing features. Long-term follow-up will provide opportunity for future insights into MIS-C and its sequelae.


Subject(s)
COVID-19/immunology , Cardiovascular Diseases/etiology , Systemic Inflammatory Response Syndrome/immunology , Adolescent , Biomarkers/blood , COVID-19/blood , COVID-19/diagnosis , COVID-19/epidemiology , COVID-19 Nucleic Acid Testing , Cardiovascular Diseases/diagnosis , Cardiovascular Diseases/epidemiology , Case-Control Studies , Child , Child, Preschool , Diagnosis, Differential , Female , Humans , Infant , Male , Pandemics , Phenotype , Phylogeny , Prospective Studies , Risk Factors , SARS-CoV-2/immunology , Severity of Illness Index , Systemic Inflammatory Response Syndrome/blood , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/epidemiology
11.
J Paediatr Child Health ; 57(7): 1089-1095, 2021 07.
Article in English | MEDLINE | ID: mdl-33624369

ABSTRACT

AIM: Non-expert training in rheumatic heart disease (RHD) detection is a valuable strategy in resource-limited settings. Here we present an innovative handheld ultrasound application featuring views of correct probe position, imaging protocol and echocardiographic loops of RHD pathology versus normal, accessible during real-time scanning. METHODS: This prospective study was implemented into a pre-existing school health screening programme at an elementary school in Koror, Palau. Six learners with no prior ultrasound experience were taught a simplified screening protocol in which a mitral regurgitation jet ≥1.5 cm and/or presence of aortic insufficiency were considered a positive screen. All consented children underwent echocardiographic screening by experts with standard portable echocardiography. All positive cases and 25% of negative cases were referred for handheld scans by learners. RESULTS: A total of 26 (4.1%) children were diagnosed with borderline or definite RHD. Mean sensitivity and specificity compared to expert RHD diagnosis over all learners was 71% (standard deviation (SD) 11.3) and 92% (SD 4.9), respectively. For the three highest scanning learners, mean sensitivity was 88% (SD 4.9) and mean specificity was 90% (SD 5.7). For all definite RHD cases, sensitivity was 100% for all but one learner, who achieved sensitivity of 60%. The novel application was used in 229 of 624 (36%) of all handheld scans and 50 of 112 (45%) of expert-diagnosed positive scans, with protocol and reference features most frequently used. Utilisation of the novel application overall decreased per day of scanning per learner. CONCLUSION: Adjunctive handheld ultrasound technology may help ease non-experts into RHD screening.


Subject(s)
Rheumatic Heart Disease , Child , Humans , Mass Screening , Palau , Prevalence , Prospective Studies , Rheumatic Heart Disease/diagnostic imaging , Technology
12.
Telemed J E Health ; 27(11): 1235-1240, 2021 11.
Article in English | MEDLINE | ID: mdl-33513044

ABSTRACT

Background: This study aims to describe one center's experience in expanding a fetal telecardiology program through collaborative work with maternal fetal medicine (MFM) clinics with the goal of safely reaching mothers during the COVID-19 pandemic. We sought to define the extent of fetal telehealth conversion at a large fetal cardiac care center and evaluate the diagnostic accuracy for studies performed. Methods: At our center, fetal telemedicine expanded from one MFM site before the pandemic to four additional sites by May 2020. A retrospective review of fetal telecardiology visits between March 15 and July 15, 2020, was performed. The chart was reviewed for confirmation of diagnosis postnatally. Results: With pandemic onset, there was a large increase in the number of telemedicine visits with a total of 122 mothers seen between five MFM clinics. Fourteen mothers (11.5%) had abnormal fetal echocardiograms requiring additional follow-up, and seven mothers (5.8%) had a fetal echocardiogram suspicious for a critical congenital heart disease (CCHD). All the fetal echocardiograms suspicious for CCHD were confirmed on postnatal echocardiogram. To our knowledge, none of the normal fetal echocardiograms were found to have congenital heart disease postnatally. Conclusions: In response to the COVID-19 pandemic, we rapidly transitioned to fetal telecardiology using a variety of formats. This has reduced potential infectious exposure for pregnant mothers and minimized contact between physicians without compromising diagnostic accuracy. In our experience, the expansion of a telemedicine program requires strong initial infrastructure, prior relationships with MFM providers, and appropriate training among obstetric sonographers.


Subject(s)
COVID-19 , Pandemics , Female , Humans , Pregnancy , Prenatal Care , Retrospective Studies , SARS-CoV-2
13.
Prenat Diagn ; 40(5): 557-564, 2020 04.
Article in English | MEDLINE | ID: mdl-31913524

ABSTRACT

Assessing cardiac function and risk stratification in a fetal anti-Sjögren syndrome type A (SSA) or anti-Sjögren syndrome type B (SSB) complete atrioventricular block (CAVB) is challenging. We aimed to evaluate the cardiovascular profile score (CVP) and its components in surveillance of fetuses with autoimmune CAVB. METHODS: Retrospective cohort review of CAVB pregnancies, excluding fetuses with significant cardiac anomalies. RESULTS: CAVBs are in 17 fetuses, diagnosed at mean gestational age of 23 ± 5 weeks. Overall mortality is 18%: 1 termination, 1 fetal demise (intrauterine fetal demise [IUFD]), and 1 postnatal death. Both mortalities had intrauterine growth restriction; IUFD had placental infarction. Presenting CVP 8.7 ± 1. No fetus had CVP <7; the score correlated with increased risk of perinatal death. The 2 mortalities had initial CVP scores of 8 and 9; both increased to 10 on subsequent exams. 30% of fetuses had low middle cerebral artery pulsatility (MCA-PI) on the last study. All had high umbilical artery pulsatility (UA-PI) throughout gestation. The 2 deaths had the lowest MCA-PI. CONCLUSION: Despite low heart rates, high CVP scores in our cohort remained high and were not predictive of mortality. Abnormalities in MCA flow reflects fetal cerebral vasodilation that may indicate altered hemodynamics and be predictive of outcomes, but data is limited. Abnormal umbilical artery (UA) flow suggests that perinatal mortality may also be related to placental disease.


Subject(s)
Atrioventricular Block/diagnostic imaging , Autoimmune Diseases/diagnostic imaging , Fetal Diseases/diagnostic imaging , Perinatal Death , Antibodies, Antinuclear/immunology , Atrioventricular Block/immunology , Atrioventricular Block/physiopathology , Autoimmune Diseases/immunology , Autoimmune Diseases/physiopathology , Echocardiography , Female , Fetal Diseases/immunology , Fetal Diseases/physiopathology , Fetal Growth Retardation , Humans , Infarction , Lupus Erythematosus, Systemic , Middle Cerebral Artery/diagnostic imaging , Placenta , Pregnancy , Pregnancy Complications , Prognosis , Pulsatile Flow , Retrospective Studies , Sjogren's Syndrome , Ultrasonography, Prenatal
14.
Clin Auton Res ; 30(2): 165-172, 2020 04.
Article in English | MEDLINE | ID: mdl-31240423

ABSTRACT

PURPOSE: To compare early changes in autonomic nervous system (ANS) tone between newborns with complex congenital heart disease (CHD) and newborns without CHD. METHODS: We performed a case-control study of heart rate variability (HRV) in newborns with complex CHD [transposition of the great arteries (TGA) or hypoplastic left heart syndrome (HLHS)] and low-risk control newborns without CHD. Cases with CHD were admitted following birth to a pediatric cardiac intensive care unit and had archived continuous ECG data. Control infants were prospectively enrolled at birth. ECG data in cases and controls were analyzed for HRV in the time and frequency domains at 24 h of age. We analyzed the following HRV metrics: alpha short (αs), alpha long (αL), root mean square short and long (RMSs and RMSL), low-frequency (LF) power, normalized LF (nLF), high-frequency (HF) power, and normalized HF (nHF). We used ANOVA to compare HRV metrics between groups and to control for medication exposures. RESULTS: HRV data from 57 infants with CHD (TGA, n = 33 and HLHS, n = 24) and from 29 controls were analyzed. The HRV metrics αS, RMSL, LF, and nLF were significantly lower in infants with CHD than in the controls. Due to the effect of normalization, nHF was higher in CHD infants (P < 0.0001), although absolute HF was lower (P = 0.0461). After adjusting for medications, αS and nLF remained lower and nHF higher in newborns with CHD (P < 0.0005). CONCLUSIONS: Infants with complex CHD have depressed autonomic balance in the early postnatal period, which may complicate the fetal-neonatal transition.


Subject(s)
Electrocardiography/trends , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/physiopathology , Heart Rate/physiology , Case-Control Studies , Female , Humans , Infant, Newborn , Male , Prospective Studies , Retrospective Studies
15.
Pediatr Dermatol ; 37(3): 537-540, 2020 May.
Article in English | MEDLINE | ID: mdl-32110833

ABSTRACT

Propranolol is approved for treatment of infantile hemangiomas (IH). IH proliferation coincides with the time when most women are breastfeeding, yet there are no reports describing the clinical effects on infants treated with propranolol while being nursed by mothers on oral antihypertensive medications. We describe two cases of breastfed infants, with mothers taking multiple antihypertensives of different drug classes, who were initiated on propranolol for IH treatment and discuss the theoretical risk of propranolol use in this setting.


Subject(s)
Hemangioma , Skin Neoplasms , Adrenergic beta-Antagonists/therapeutic use , Antihypertensive Agents , Breast Feeding , Female , Humans , Infant , Mothers , Propranolol/therapeutic use , Treatment Outcome
16.
Fetal Diagn Ther ; 47(9): 711-716, 2020.
Article in English | MEDLINE | ID: mdl-32615554

ABSTRACT

INTRODUCTION: Early detection and monitoring for malignant arrhythmias is fundamental to prenatal care in long QT syndrome (LQTS). Recently, we studied the feasibility of isolating the fetal electrocardiogram (fECG) and measuring electrocardiographic intervals with a noninvasive fECG device using blind source separation with reference signal. Our aim was to evaluate the ability of fECG to diagnose LQTS. CASE PRESENTATIONS: We identified 3 cases of clinically suspected LQTS based on fetal echocardiogram (2 had sinus bradycardia, 1 had second-degree atrioventricular block with negative maternal anti-SSA/SSB antibody titers). With institutional review board approval, these patients were prospectively enrolled for fECG acquisition. Offline post-processing generated fECG waveforms and calculated QT intervals. Case 1 and 3 had a maternal history of LQTS. Two of the three fetuses with suspected LQTS had confirmed LQTS by postnatal ECG and genetic testing. FECG was able to identify a prolonged corrected QT interval in both cases. One of these also had fetal magnetocardiography (fMCG), which yielded similar findings to the fECG. The third fetus had a normal fECG; fMCG and postnatal ECG were also normal. CONCLUSIONS: In 3 cases, fECG findings corroborated the diagnosis of LQTS. Noninvasive fECG may offer a novel method for fECG that is portable and more clinically accessible.


Subject(s)
Electrocardiography/methods , Fetal Heart/physiopathology , Long QT Syndrome/diagnostic imaging , Magnetocardiography/methods , Adult , Female , Heart Rate, Fetal/physiology , Humans , Long QT Syndrome/physiopathology , Pregnancy , Prenatal Care , Prenatal Diagnosis , Young Adult
18.
Pediatr Cardiol ; 40(6): 1175-1182, 2019 Aug.
Article in English | MEDLINE | ID: mdl-31172229

ABSTRACT

Non-invasive fetal electrocardiography (ECG) is a promising method for evaluating fetal cardiac electrical activity. Despite advances in fetal ECG technology, its ability to provide reliable, interpretable results in a typical outpatient fetal cardiology setting remains unclear. We sought to determine the feasibility of measuring standard ECG intervals in an outpatient fetal cardiology practice using an abdominal fetal ECG device that employs blind source separation with reference, an innovative signal-processing technique for fetal ECG extraction. Women scheduled for clinically indicated outpatient fetal echocardiogram underwent 10 min of fetal ECG acquisition from the maternal abdomen using specialized gel electrodes. A bedside laptop computer performed fetal ECG extraction, allowing real-time visualization of fetal and maternal ECG signals. Offline post-processing of 1 min of recorded data yielded fetal P-wave duration, PR interval, QRS duration, RR interval, QT interval, and QTc. Fifty-five fetuses were studied with gestational age 18-37 weeks, including 13 with abnormal fetal echocardiogram findings and three sets of twins. Interpretable results were obtained in 91% of fetuses, including 85% during the vernix period and 100% of twin fetuses. PR interval and RR interval of 18-24 week gestation fetuses were significantly shorter than those with gestational age 25-31 and 32-37 weeks. Of the six fetuses with abnormal rhythms on fetal echocardiogram, fetal ECG tracing was interpretable in five and matched the rhythm noted on fetal echocardiogram. Abdominal fetal ECG acquisition is feasible for arrhythmia detection and ECG interval calculation in a routine clinical setting.


Subject(s)
Arrhythmias, Cardiac/diagnosis , Electrocardiography/methods , Heart Rate, Fetal , Prenatal Diagnosis/methods , Adult , Ambulatory Care Facilities , Electrocardiography/instrumentation , Feasibility Studies , Female , Gestational Age , Humans , Middle Aged , Pregnancy , Young Adult
19.
Cardiol Young ; 29(2): 216-218, 2019 Feb.
Article in English | MEDLINE | ID: mdl-30587271

ABSTRACT

Premature ductal constriction was diagnosed in a 30-week gestation fetus with hypoplastic left heart syndrome. The fetus developed right ventricular hypertrophy and mild tricuspid regurgitation. Foetal neuroimaging showed slowed growth of the brain and increased lactate. We describe the imaging and clinical findings. Fetuses with critical CHD and in utero ductal constriction are at increased risk of morbidity and mortality, and require appropriate counselling.


Subject(s)
Hypertrophy, Right Ventricular/diagnostic imaging , Hypoplastic Left Heart Syndrome/diagnostic imaging , Tricuspid Valve Insufficiency/diagnostic imaging , Adult , Brain/diagnostic imaging , Echocardiography , Fatal Outcome , Female , Fetal Heart/diagnostic imaging , Humans , Hypoplastic Left Heart Syndrome/surgery
20.
J Neurol Neurosurg Psychiatry ; 89(10): 1088-1100, 2018 10.
Article in English | MEDLINE | ID: mdl-29903905

ABSTRACT

The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). METHODS: Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements. RESULTS: Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist. CONCLUSIONS: In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.


Subject(s)
Headache/therapy , Pseudotumor Cerebri/therapy , Consensus , Headache/etiology , Humans , Pseudotumor Cerebri/complications
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