Effect of Maternal Preeclampsia on Cardiac Structure and Function in Very Low Birth Weight Infants.

OBJECTIVE: We aimed to determine whether exposure to severe maternal preeclampsia (PE) in very low birth weight (VLBW) infants is associated with hypertrophic cardiac changes and altered hemodynamics. STUDY DESIGN: Case-control study of VLBW infants born at Los Angeles General Medical Center from May 2015 to August 2023, who had an echocardiogram within the first 7 days of life. Cases were infants exposed to maternal PE and controls were infants not exposed to maternal PE matched by birth weight (BW) 1:1. Laboratory, placental pathology results, hemodynamic data and clinical outcomes were collected and compared between cases and control infants. RESULTS: A total of 43 cases matched by BW with control infants were studied. There were no significant anatomical cardiac changes by echocardiography between cases and control infants. Cases had significantly higher blood pressure within the first 72 hours of life and lower ejection fraction (EF), fractional shortening, and peak systolic flow velocity through their patent ductus arteriosus (PDA) within the first week of life. Cases were more likely to be smaller despite being born at a later gestational age (GA), as well as small for GA with placental weight less than 10th percentile compared to control infants. CONCLUSION: Our findings indicate that infants born to mothers with PE have higher systemic vascular resistance as evidenced by elevated blood pressure, and lower EF and shortening fraction and higher pulmonary vascular resistance as evidenced by lower peak flow velocity through the PDA. We did not observe hypertrophic cardiac changes in exposed infants. These findings should be considered in clinical decision-making during management of these infants. KEY POINTS: · VLBW infants exposed to severe PE have higher rate of Small for gestational age and smaller placentas.. · VLBW infants exposed to severe PE have higher systemic vascular resistance during transitional period and lower EF and fractional shortening.. · VLBW infants exposed to severe PE have higher pulmonary vascular resistance..

Outcomes of patent foramen ovale greater than 3 mm at birth in extremely low birthweight infants.

BACKGROUND: Foramen ovale (FO) is an obligate fetal shunt that typically resolves after birth, although patency throughout life is not uncommon. The natural history of patent FO (PFO) is known in term infants, but less is known about its course in extremely preterm infants. We describe the echocardiographic changes in FO size from birth to discharge in extremely low birth weight (ELBW) infants in this retrospective study. METHODS: Cohort was divided based on size of FO at birth. Size of FO at discharge was measured and evaluated relative to postnatal weight gain. Demographics and clinical outcomes were compared between the two groups. RESULTS: Of the 54 ELBW infants, 50 were born with FO less than 3 mm in diameter (small), and 4 were born with FO greater than 3 mm (large). Of small defects, the majority (44/50, 88%) did not increase in size with weight gain, and minority (6/50, 12%) increased in size, and three of these 6 patients, FO grew to be slightly larger than 3 mm. In contrast, all large defects (4 of 4, 100%) nearly doubled in size with postnatal growth. These 4 ELBW infants with enlargement of FO had a flap valve evident on echocardiogram obtained prior to discharge, and subsequently closed on outpatient follow-up echocardiograms, although time to resolution was variable (6 months - 3 years). One infant had presumptive resolution because of the presence of flap valve. CONCLUSION: No maternal or neonatal demographic characteristics were predictive of FO enlargement, although, demonstrable flap valve on discharge echocardiogram correlated with resolution of FO on outpatient follow-up echocardiogram. Therefore, based on our data, we recommend that ELBW infants born with large FO should have echocardiographic re-evaluation of the atrial septal opening prior to discharge, to specify the presence of a flap valve or lack thereof, which is an important detail that can help a neonatologist determine the need for outpatient cardiac follow-up.

Effectiveness of Simulation Training in Acquiring Echocardiography Skills among Neonatology Care Providers.

OBJECTIVE: The objective of this study was to test the hypothesis that simulation training improves echocardiography skills. STUDY DESIGN: In this prospective study, 43 participants (16 neonatologists, 26 neonatology fellows, and 1 nurse practitioner) were tested on the simulator after 4 hours of didactic sessions on topographical cardiac anatomy and standard echocardiographic views. Participants were given 20 minutes to obtain and save the standard views. After the simulation hands-on training for 8 hours over 3 days, they were tested again. Each image was scored from 0 to 3 based on the image quality. Pre- and postsimulator training data were compared using both automated and visual scoring methods. RESULTS: After the hands-on simulator training, the automated median (interquartile) score for the quality of acquired images increased from 36 (22, 43) to 55 (48, 58), p < 0.0001. The increase was similar using visual scoring. The number of views with acceptable or good image quality (scores of 2 or 3) increased from 11 (6, 16) to 20 (17, 21), p < 0.0001. The neonatology fellows and faculty, as well as those with or without prior echocardiography experience, demonstrated significant improvement. CONCLUSIONS: Echocardiography simulation is an effective tool in improving echocardiography skills among neonatology care providers. KEY POINTS: · Simulation is effective in acquiring echocardiography skills among neonatology care providers.. · Simulation improves image acquisition in those with and without prior echocardiography experience.. · Effectiveness of simulation in retaining the acquired echocardiography skill should be investigated..

Aortopulmonary window, aortic arch interruption, and anomalous origin of the right pulmonary artery in a neonate with Goldenhar syndrome.

The combination of aortopulmonary window, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the ascending aorta is a rare and complex congenital cardiac malformation. Despite good prenatal care in our case, this cardiac anomaly was not detected prior to birth. Untreated infants who do not undergo surgical correction have a mortality rate of 70% in their first year, and 30% will die within the first 3 months of life.

Myocarditis in drug rash with eosinophilia and systemic symptoms.

Drug rash with eosinophilia and systemic symptoms is a drug hypersensitivity reaction. Hepatitis and nephritis are the most common visceral manifestations. Myocarditis is important to recognise, given the high mortality rate. We describe a child with drug rash with eosinophilia and systemic symptoms and discuss the role of N-terminal pro-hormone of basic natriuretic peptide in early recognition of associated myocarditis.

Pulmonary hemorrhage in extremely low birth weight infants: Significance of the size of left to right shunting through a valve incompetent patent foramen ovale.

OBJECTIVE: Pulmonary hemorrhage is a rare but severe complication of extremely low birth weight (ELBW) infants. The association of hemodynamically significant patent ductus arteriosus (hsPDA) and the diameter of the foramen ovale (FO) with pulmonary hemorrhage has not been reported. STUDY DESIGN: Case control study of ELBW infants with and without pulmonary hemorrhage. Each ELBW infant with an echocardiogram within 48 h of pulmonary hemorrhage was analyzed. RESULT: 16 infants with pulmonary hemorrhage were matched with 32 controls by birth weight and gestational age. Echocardiogram showed hsPDA in all infants and those with pulmonary hemorrhage had significantly smaller patent FO [PFO] (1 vs 2.4 mm, p < 0.01) (OR 0.007; 95% CI 0.00007, 0.67 p = 0.03). Incidence of pulmonary hemorrhage was 8.9%. CONCLUSION: ELBW infants with hsPDA who experienced pulmonary hemorrhage had a significantly restricted or closed FO. Evaluation of FO should be considered with serial echocardiograms when evaluating for hsPDA.

Supraventricular tachycardias, conduction disease, and cardiomyopathy in 3 families with the same rare variant in TNNI3K (p.Glu768Lys).

BACKGROUND: Rare genetic variants in TNNI3K encoding troponin-I interacting kinase have been linked to a distinct syndrome consisting primarily of supraventricular tachycardias and variably expressed conduction disturbance and dilated cardiomyopathy in 2 families. OBJECTIVE: The purpose of this study was to identify new genetic variants associated with inherited supraventricular tachycardias, cardiac conduction disease, and cardiomyopathy. METHODS: We conducted next generation sequencing in 3 independent multigenerational families with atrial/junctional tachycardia with or without conduction disturbance, dilated cardiomyopathy, and sudden death. We also assessed the effect of identified variant on protein autophosphorylation. RESULTS: In this study, we uncovered the same ultra-rare genetic variant in TNNI3K (c.2302G>A, p.Glu768Lys), which co-segregated with disease features in all affected individuals (n = 23) from all 3 families. TNNI3K harboring the TNNI3K-p.Glu768Lys variant displayed enhanced kinase activity, in line with expectations from previous mouse studies that demonstrated increased conduction indices and procardiomyopathic effects with increased levels of Tnni3k. CONCLUSION: This study corroborates further the causal link between rare genetic variation in TNNI3K and this distinct complex phenotype, and points to enhanced kinase activity of TNNI3K as the underlying pathobiological mechanism.

Short-term outcomes following implementation of a dedicated young adult congenital heart disease transition program.

OBJECTIVE: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease. DESIGN, SETTING, AND PATIENTS: We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January 2012 to December 2015 within a single academic institution that delivered pediatric and adult care at separate children's and adult hospitals, respectively. INTERVENTION AND OUTCOME MEASURES: Demographic characteristics including congenital heart disease severity, gender, age, presence of comorbidities, presence of cardiac implantable electronic devices, and type of insurance were correlated to success of transfer. Rate of successful transfer was evaluated, and multivariate analysis was performed to determine which demographic variables were favorably associated with transfer. RESULTS: Thirty-nine percent of patients successfully transferred from pediatric to adult services during the study period. Severe congenital heart disease (OR 4.44, 95% CI 1.25-15.79, P = .02) and presence of a cardiac implantable electronic device (OR 4.93, 95% CI 1.18-20.58, P = .03) correlated with transfer. Trends favoring successful transfer with presence of comorbidities and private insurance were also noted. CONCLUSIONS: Despite a dedicated transition clinic, successful transfer rates remained relatively low though comparable to previously published rates. Severity of disease and presence of implantable devices correlated with successful transfer. Other obstacles to transfer remain and require combined efforts from pediatric and adult care systems, insurance carriers, and policy makers to improve transfer outcomes.