Development of a neonatal cardiac curriculum for neonatal-perinatal medicine fellowship training.

BACKGROUND: The topic of neonatal cardiovascular care in neonatal-perinatal medicine (NPM) fellowship training has continued to transform due to increased complexity of patients, development of specialized units, continued Accreditation Council for Graduate Medical Education requirements, and clinical practice variation across centers that care for neonates with congenital heart disease (CHD). METHODS: We developed a neonatal cardiac curriculum comprised of eight interactive sessions with novel active learning concepts specific to our NPM fellows. A self-assessment survey in comfort in managing infants with CHD and perceived competency in neonatal cardiology topics was performed by all neonatology fellows at baseline and after completion of the curriculum. The American Board of Pediatrics Subspecialty In-training Exam (SITE) scores for fellows were compared to that of the national average. RESULTS: The average comfort score (0-100) of the first-year fellows increased from 33 to 76, and that of the second and third-year fellows increased from 72 to 86, and 75 to 86, respectively. The first-year fellows improved their competency score by 44 points (3 standard deviations), the second-year fellows improved their score by about 26 points (one standard deviation), and there was an overall 9-point increase in the competency score of all fellows (one standard deviation). The average local SITE score was lower than the national average before the initiation of this curriculum, became nearly equal to the national average score at the end of the first year the curriculum was implemented, and has progressively become higher since then. CONCLUSION: Due to the variable clinical exposure and differing practice models of managing CHD a neonatal cardiac curriculum may be beneficial to NPM trainees.

Safety and Efficacy of Regadenoson for Pediatric Stress Perfusion Cardiac MRI with Quantification of Myocardial Blood Flow.

Myocardial stress perfusion magnetic resonance imaging is a non-invasive tool to assess for myocardial ischemia and viability. Pediatric myocardial stress perfusion MRI can be challenging due to multiple intravenous lines, sedation, inadequate breath holding, fast heart rates, and complex anatomy. We performed a retrospective analysis in 39 children to evaluate safety and efficacy of regadenoson, a coronary vasodilator administered via a single intravenous line (6−10 mcg/kg), with respiratory motion correction (MOCO) and semi-quantitative blood flow analysis. Stress response data and adverse events were recorded, and image quality compared between native and MOCO reconstructions, assessing for perfusion deficits. Semi-quantitative analysis compared myocardial perfusion reserve index (MPRI) between patients who had a focal perfusion defect, patients who had undergone an orthotopic heart transplant, and non-transplant patients with no focal defects. Stress perfusion was completed in 38/39 patients (median age 15 years with a 41 ± 27% rise in heart rate (p < 0.005). Fifteen out of thirty-eight had transient minor side effects with no major adverse events. MOCO image quality was better than non-MOCO (4.63 vs. 4.01 at rest, p < 0.005: 4.41 vs. 3.84 at stress, p < 0.005). Reversible perfusion defects were seen in 4/38 patients with lower segmental mean MPRI in the area of the perfusion defect, nearing statistical significance when compared to non-transplant patients with no defects (0.78 ± 0.22 vs. 0.99 ± 0.36, p = 0.07). The global MPRI of the 16 patients who had undergone orthotopic heart transplant was significantly lower than the non-transplant patients (0.75 ± 0.22 vs. 0.92 ± 0.23, p = 0.03). Regadenoson is a safe and effective coronary vasodilator for pediatric stress perfusion MRI with MOCO producing better image quality and allowing for semi-quantitative assessment of perfusion deficits that correlate with qualitative assessment.

Echocardiographic Diagnosis, Surgical Treatment, and Outcomes of Anomalous Left Coronary Artery from the Pulmonary Artery.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare condition that can be difficult to diagnose by echocardiography alone. The purpose of this study was to describe the clinical and echocardiographic presentation of ALCAPA, create a set of critical echocardiographic diagnostic criteria, and report outcomes. METHODS: A retrospective review was conducted of all patients diagnosed with isolated ALCAPA at two major cardiac centers between 1990 and 2015. RESULTS: Of the 37 patients identified, only 54% presented in infancy. The anomalous coronary artery (CA) origin was clearly imaged in only 54% of echocardiographic examinations. However, other consistently identified echocardiographic markers were found, including left CA flow reversal (91%), collateral CA flow (85%), right CA dilation (81%), abnormal pulmonary artery flow (79%), mitral regurgitation (74%), left ventricular dysfunction (66%) and endocardial fibroelastosis (57%). Presenting echocardiograms had five of seven markers in 85% of patients. Left ventricular dysfunction was the most common marker in infants (89% vs 38%, P = .005); older children were more likely to have collateral formation visualized by color Doppler (100% vs 75%, P = .04). Following surgery, there were no early surgical deaths. The median follow-up duration was 10.3 years. At last follow-up, 92% had normal left ventricular function, 3% had moderate or worse mitral regurgitation, and 17% had required reintervention. CONCLUSIONS: Echocardiographic markers can reliably identify ALCAPA; these markers vary with the age of presentation. Surgical outcomes are excellent, and most patients will recover left ventricular and mitral valve function.

Relation of Right Atrial Volume, Systemic Venous Dimensions, and Flow Patterns to Right Atrial Pressure in Infants and Children.

Echocardiographic assessment of right atrial (RA) volume, inferior vena cava (IVC) diameter, and hepatic vein flow velocity independently correlate with the RA pressure by direct catheter measurement in adults. We prospectively collected invasive RA pressure measurements and echocardiographic data in infants and young children with the goal of developing a predictive model to noninvasively determine normal RA pressure. All subjects had a central venous catheter through which RA pressure could be transduced. Specific inclusion criteria consisted of (1) biventricular heart, (2) absence of inotropes, (3) sinus rhythm, and (4) at least 24 hours from surgery. Two-dimensional echocardiography (2DE)-Doppler and 3DE-Doppler were used to measure RA volume, systemic venous diameters, and flow velocity. Regression equations of RA pressure with RA volume, systemic venous size, and flow velocity were explored. Of 46 studies, 43 (93%) had echocardiograms adequate for analysis. RA pressure did not correlate with body surface area or age (p = 0.69, p = 0.87). The mean indexed RA volume by 3DE-Doppler was significantly higher than by 2DE (p <0.005). On multivariable analysis, only IVC systolic flow velocity and systolic 2D Simpson's derived indexed RA volume demonstrated significant independent correlation with RA pressure, resulting in the equation: RA pressure (mm Hg) = 7.35 - 0.0025 × IVC systolic flow velocity (cm/s) + 0.119 × indexed RA volume by systolic 2D Simpson's (ml/m2). RA pressure did not show correlation with systemic venous diameters or systolic and diastolic flow velocities in the SVC and hepatic veins. In conclusion, regression incorporating 2DE-derived RA volume and IVC systolic flow velocity provided the best noninvasive estimate of normal RA pressure in infants and children. The model derived requires validation in an independent sample.