Objectives To assess whether severity of congenital diaphragmatic hernia (CDH)

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Objective: To evaluate pre-intervention echocardiographic parameters of cardiac function in fetuses who survive without hydrops as compared to fetuses who develop hydrops or perinatal death in the setting of sacrococcygeal teratoma (SCT) and twin-reversed arterial perfusion sequence (TRAP). Methods: Clinical, echocardiographic and sonographic data of fetuses with SCT or TRAP during 1999–2009 were reviewed retrospectively. Measurements of cardiothoracic ratio (CTR), cardiac dimension Z-scores, combined ventricular output (CVO), valvular regurgitation, and cardiovascular profile scores (CVPS) were obtained. Results: In total, 19 fetuses (11 SCT, 8 TRAP) met the inclusion criteria and 26 detailed fetal echocardiographic studies were reviewed. Outcome was poor in 7 pregnancies (group A) and good in 12 (group B). Group A had worse CVPS (8.5 vs. 10, p < 0.01) and higher CTR (0.37 vs. 0.30, p = 0.04). At least one of the following was present in each group A fetus: CTR >0.5, CVO >550 ml/min/kg, tricuspid or mitral regurgitation, or mitral valve Z-score >2. No group B fetus had any of these abnormalities. No fetus in either group had abnormal venous Doppler waveforms at presentation. Conclusions: Fetal echocardiography can identify abnormalities of cardiac size and systolic, but not diastolic, function in all fetuses who subsequently died or developed hydrops.

Background: Small left heart structures are observed in fetuses with left-sided congenital diaphragmatic hernia (CDH). Fetoscopic tracheal occlusion (FETO) in mid-gestation promotes lung growth in fetuses with CDH, however cardiac effects of FETO are poorly described. We studied the effects of FETO on cardiac structure size at birth, hypothesizing that left heart structures would be larger in neonates who had undergone fetal intervention. Methods/Results: We performed retrospective measurements of atrioventricular and semilunar valve and pulmonary artery diameters, ventricular lengths, left ventricular end-diastolic volume indexed (LVEDVi) to body surface area. 35 patients were studied (9 FETO, 26 controls). All fetuses had liver herniation and a lung-to-head ratio <1 at fetal presentation. At birth the intervention group had larger LVEDVi (16.8 vs. 12.76 ml/m², p < 0.05), LV length Z-score (-2.05 vs. -4, p < 0.01), LV:RV length ratio (1.43 vs. 1.04, p < 0.05), LPA diameter Z-score (+1.71 vs. -1.04, p < 0.05), and better growth of aortic valve (-2.18 FETO, -3.3 controls, p < 0.01). There was a trend toward higher LV output in the FETO group. Conclusions: Left heart structures and LPA were larger postnatally in patients with CDH who underwent FETO than in those who did not. Hemodynamic alterations are introduced with tracheal occlusion that are associated with alterations in ventricular loading and may influence growth.

Objectives: Myopericarditis is a newly described entity that is primarily a pericardial syndrome with elevated cardiac enzymes that has been seen with increasing frequency in our pediatric intensive care unit. For this reason, we performed a retrospective cohort analysis with a goal of establishing differences between myopericarditis and myocarditis that could lead to proper diagnosis on emergency department presentation.Methods: A database query was performed and identified patients over a 9-year period, and clinical data, laboratory data, and cardiac studies were extracted and analyzed from the electronic health record.Results: A total of 36 patients were identified with the discharge diagnosis of myopericarditis and 22 with myocarditis. The median age for myopericarditis patients was 16.2 years, and 97% were male. The median initial troponin was 7.1 ng/mL, the peak was at 16.6 ng/mL, and 58% had ST changes on electrocardiogram. The median length of stay for myopericarditis patients was 1.7 days, and 50% were discharged home on nonsteroidal anti-inflammatory medication. Compared with myocarditis, myopericarditis patients were older, had a higher incidence of chest pain, and were less likely to have fever, vomiting, abdominal pain, upper respiratory infection symptoms, chest radiograph abnormalities, or T-wave inversion (P < 0.05). Myopericarditis patients also had lower Pediatric Risk of Mortality version 3 scores, B-type natriuretic peptide levels, and higher left ventricular ejection fractions on admission (67% vs 41%; P < 0.05). A classification model incorporating initial left ventricular ejection fraction, B-type natriuretic peptide, electrocardiogram, and chest radiograph findings distinguished myopericarditis from myocarditis with correct classification in 95% of patients.Conclusions: Myopericarditis is a relatively common cause of chest pain for patients admitted to the pediatric intensive care unit, presents differently than true myocarditis, and carries a good prognosis.

ObjectivesIn fetuses with left-sided congenital diaphragmatic hernia (CDH), left heart structures may appear small, but usually normalize after birth in the absence of structural cardiac anomalies. To decrease the possibility of an erroneous diagnosis of structural heart disease, we identify normal values for left heart structures in the presence of left CDH and secondarily investigate the relationship of left heart size and survival to neonatal hospital discharge.MethodsLeft heart structures [mitral valve (MV) and aortic valve (AoV) annulus diameter, left ventricle (LV) length and width] were measured by fetal echocardiogram in fetuses with left CDH and no congenital heart disease. We generated linear regression models to establish the relationship of gestational age for each left heart structure using data from fetuses who survived after birth. We calculated z-scores (normalized to gestational age), and assessed the relationship of survival to the size of each structure.ResultsOne hundred forty-two fetuses underwent fetal echocardiogram (median 25 weeks' gestation, IQR 23, 27 weeks). Left heart structures were deemed small when using published normative data from unaffected fetuses (z-scores: MV −1.09 ± 1.35, AoV −2.12 ± 1.16, LV length −1.36 ± 1.24, LV width −4.79 ± 0.79). CDH-specific models derived from log-transformed values yielded left-shifted distributions, reflecting the small structures (mean z-score lower by: MV 0.99 ± 0.30, AoV 2.04 ± 0.38, LV length 1.30 ± 0.36, and LV width 4.69 ± 0.28; p &lt; 0.0001 for all comparisons). Non-survivors had worse z-scores than survivors for all measurements, but this did not reach statistical significance.ConclusionsLog-transformed linear models generated new normative data for fetal left heart structures in left CDH, which may be used to allay antenatal concerns regarding structural left heart anomalies. There were no significant differences in z-scores between survivors and non-survivors, suggesting that in the absence of true structural disease, cardiac evaluation is not predictive in isolation and that causes of mortality are likely multifactorial in this population.

We present two cases of early detection of anencephaly and encephalocele at the routine first trimester examination.

An 18-year-old male with complex single-ventricle physiology status post-3-stage palliation developed a large Norwood aneurysm (77 × 67 mm). The patient underwent a successful surgical reconstruction. Care providers must be aware of this rare complication and provide appropriate surveillance.