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Transthoracic echocardiography is the most common noninvasive method of evaluating pulmonary hypertension (PH) in infants. Identification of reliable, quantitative indices of myocardial function may enhance the diagnostic value of echocardiography in this population. We hypothesized that pulsed wave tissue Doppler imaging (TDI) and tricuspid annular plane systolic excursion (TAPSE) would be reproducible measurements and would suggest decreased ventricular function, in infants with PH. This retrospective case-control study involved subjects diagnosed clinically and echocardiographically with PH at <12 months of age, matched with controls by age and gestational age (GA). TAPSE was measured by M-mode in the apical 4-chamber view. TDI velocities were averaged from three consecutive cardiac cycles. Observers were blinded to patient identity and clinical status. Fifty-seven subjects, including 35 term or near-term infants ≥35 weeks GA [mean GA 39 weeks (±1.7), median age 1 day (range 0-2)] and 22 preterm infants [median GA 25.4 weeks (24.1-26.6), age 66 days (4-128)], were matched with 57 controls. Subjects with PH had lower TAPSE (term p < 0.001, preterm p = 0.03) and TAPSE indexed to body surface area (term p < 0.001, preterm p = 0.005). Mitral annular, septal, and tricuspid annular systolic (S') and early diastolic (E') TDI velocities were also decreased compared to controls (all p < 0.05). Intraclass correlation demonstrated 84-99% agreement between observers in measuring TDI and 92% for TAPSE. Intraobserver reliability for these measures was 98-99% and 96%, respectively. We concluded that TDI and TAPSE are reproducible indices of myocardial function and may serve as useful adjuncts to standard echocardiographic measures in infants with PH. [ABSTRACT FROM AUTHOR]

Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While "tight" rings often produce symptoms and require surgery, "loose" rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients. An anonymous web-based survey was distributed to several listservs for pediatric cardiologists and pediatric cardiothoracic surgeons. Survey questions targeted respondent practice characteristics, testing obtained, and indications for testing or surgical referral. In total 61 responses were received, predominantly from pediatric cardiologists (95%) in the United States (97%). About 60% of clinicians reported frequently diagnosing patients with vascular rings by fetal echocardiogram, with only about 20% diagnosing them frequently on evaluation of symptoms. Computed tomography angiography and echocardiogram were the most common imaging modalities employed. Most clinicians obtained cross-sectional imaging at the time of diagnosis and referred to surgery once patients had at least occasional symptoms. Respondents demonstrated a low degree of agreement (Krippendorf's alpha 0.48). Few statistically significant patterns were identified between respondents based on their practice characteristics. This study identified significant variation between clinicians regarding the evaluation and management of vascular rings. Further research or expert opinions may help to standardize practice, saving costs and improving the quality of care for affected patients. [ABSTRACT FROM AUTHOR]

Evidence suggests an association between left heart obstructive lesions and dilated coronary sinus (DCS), but this has not been studied in fetuses. A retrospective review of fetal echocardiograms (FE) over an 8-year period was conducted, and patients with DCS were identified and confirmed postnatally. There were 5840 FE performed on 4920 women during this period. Of 49 patients with DCS, 22 had normal intracardiac anatomy and 27 patients had congenital heart disease (CHD) yielding an incidence of 4.6 % in the presence of CHD (27/584). Of 27 patients with DCS and CHD, approximately a third had either hypoplastic left ventricles and/or coarctations (10/27, 37 %). The incidence of left heart obstructive lesions was much higher in the presence of a DCS (37 % vs 45/557, 8 %, p < 0.0001). The odds ratio of left heart hypoplasia in fetuses with CHD and a DCS was 6.6 (95 % CI 2.8-15.3). Comparison of patients with postnatally confirmed coarctation with those with normal intracardiac anatomy with DCS, revealed that in the former, the right ventricle ( p = 0.005), pulmonic valve annulus ( p = 0.0001) and the tricuspid inflow were larger ( p = 0.001) compared to corresponding left-sided structures. The size of the DCS was not significantly different between the two groups, but in the former, the DCS was more closely related to the posterior leaflet of the mitral valve and caused a significant diminution of the mitral inflow. Our study suggests a strong association, possibly causal, between left heart obstructive lesions and DCS in utero. [ABSTRACT FROM AUTHOR]

The purpose of this study was to determine the yield of fetal echocardiography based on indications for referral and to establish the incidence of structural heart disease noted in a diverse urban population. A retrospective review of our Pediatric Echocardiography Laboratory database was undertaken from March 2010 to June 2014. Demographic information, indications for referral and prenatal diagnoses were recorded. The diagnosed cardiac anomalies were classified into structural and rhythm abnormalities and the former was divided into major and minor anomalies. We compared the number of fetal echocardiograms performed during this four-year period to a similar four-year period, a decade earlier. Also, we compared the number of cardiac admissions to the neonatal intensive care unit in these two periods. 3623 fetal echocardiograms were performed on 2953 patients between 2010 and 2014. Fetal indications accounted for 70% of referrals. Overall, yield of congenital heart disease was 11.5%; 6.8% from maternal and 13.4% from fetal indications. Abnormal obstetrical fetal ultrasound was associated with congenital heart disease in 13.9% of cases. Of 336 fetuses with congenital heart disease, 119 were major and 47 were critical. 80% of all congenital heart diseases (273 of 336) and 90% of major and critical heart disease were found in those referred for fetal indications. Approximately half (24 of 47, 51%) of the critical congenital heart disease involved defects with varying degrees of left heart hypoplasia. Compared to a previous period, there was an increase in FE and neonatal intensive care unit admissions with cardiac diagnoses. However, critical congenital heart disease detected postnatally fell from 28.8 to 12.8% (P = 0.001). Yield of fetal echocardiograms is higher for fetal versus maternal indications. At our center, increase in prenatal detection of critical congenital heart disease may have resulted in reduction in congenital heart disease diagnosed in the neonatal intensive care unit. [ABSTRACT FROM AUTHOR]