A Fetal Presentation of a Ruptured Right Ventricular Diverticulum.

Ruptured diverticula and ventricular aneurysms are rare in the fetus, with a limited number of case reports published previously. Additional fetal complications secondary to these ventricular wall abnormalities can be seen. Interventional measures can be considered and attempted either in utero or postnatally to improve the chance of survival. We present a case of a ruptured diverticulum in a fetus and the clinical course.

Implementation of a statewide, multisite fetal tele-echocardiography program: evaluation of more than 1100 fetuses over 9 years.

OBJECTIVES: We sought to describe the implementation and effectiveness of a statewide fetal tele-echocardiography program serving a resource-limited population. STUDY DESIGN: In 2009, our heart center established six satellite clinics for fetal tele-echocardiography around the state. We retrospectively reviewed all fetal tele-echocardiograms performed through 2018. Yearly statewide prenatal detection rates of operable congenital heart disease were queried from the Society of Thoracic Surgeons database. RESULT: In 1164 fetuses, fetal tele-echocardiography identified all types of congenital heart disease, with a sensitivity of 74% and specificity of 97%. For the detection of ductal-dependent congenital heart disease, fetal tele-echocardiography was 100% sensitive and specific. Between 2009 and 2018, annual statewide prenatal detection rates of congenital heart disease requiring heart surgery in the first 6 months of life rose by 159% (17-44%; R2 = 0.88, p < 0.01). CONCLUSIONS: The present study provides a framework for an effective, large-scale fetal tele-echocardiography program.

Cerebral Hemodynamics Are Not Affected by the Size of the Patent Ductus Arteriosus.

INTRODUCTION: Although patent ductus arteriosus (PDA) has been implicated to play a role in the development of cerebral ischemia and intraventricular hemorrhage (IVH) through a cerebral steal phenomenon, there is conflicting data on the impact of PDA size on cerebral blood flow (CBF). Cerebral autoregulation is the brain's innate protective mechanism to maintain constant CBF despite changes in blood pressure, and it is unclear if it is influenced by PDA hemodynamics. OBJECTIVE: To delineate the relationship between PDA size and CBF velocity (CBFv) in premature infants. METHODS: 113 premature infants born at 23-29 weeks' gestation had echocardiograms performed during the first week after birth to evaluate for PDA. The infants were divided into 3 groups according to PDA size: none-to-small, moderate, or large. All infants had continuous recordings of umbilical artery blood pressure (ABP) and CBFv during the first week after birth. Critical closing pressure (CrCP) was calculated from ABP and CBFv tracings. Diastolic closing margin (DCM), defined as diastolic blood pressure minus CrCP, was calculated as a marker for the risk of developing IVH. RESULTS: Infants with a large PDA (n = 16) had the lowest ABP across all phases of the cardiac cycle (systole [p = 0.003], mean [p = 0.005], and diastole [p = 0.012]) compared to infants with a moderate (n = 19) or none-to-small PDA (n = 78). Despite blood pressure being different, systolic, mean, and diastolic CBFv were not different across groups. Cerebral autoregulation, as measured during systole, was intact regardless of the PDA size. CrCP and DCM were also not different across groups. CONCLUSIONS: In this cohort, CBFv and cerebral autoregulation during systole were not influenced by PDA size. Intact cerebral autoregulation may play a role in maintaining CBFv regardless of PDA size and differences in ABP.

Amplitude-integrated EEG in newborns with critical congenital heart disease predicts preoperative brain magnetic resonance imaging findings.

OBJECTIVE: The study aims are to evaluate cerebral background patterns using amplitude-integrated electroencephalography in newborns with critical congenital heart disease, determine if amplitude-integrated electroencephalography is predictive of preoperative brain injury, and assess the incidence of preoperative seizures. We hypothesize that amplitude-integrated electroencephalography will show abnormal background patterns in the early preoperative period in infants with congenital heart disease that have preoperative brain injury on magnetic resonance imaging. METHODS: Twenty-four newborns with congenital heart disease requiring surgery at younger than 30 days of age were prospectively enrolled within the first 3 days of age at a tertiary care pediatric hospital. Infants had amplitude-integrated electroencephalography for 24 hours beginning close to birth and preoperative brain magnetic resonance imaging. The amplitude-integrated electroencephalographies were read to determine if the background pattern was normal, mildly abnormal, or severely abnormal. The presence of seizures and sleep-wake cycling were noted. The preoperative brain magnetic resonance imaging scans were used for brain injury and brain atrophy assessment. RESULTS: Fifteen of 24 infants had abnormal amplitude-integrated electroencephalography at 0.71 (0-2) (mean [range]) days of age. In five infants, the background pattern was severely abnormal. (burst suppression and/or continuous low voltage). Of the 15 infants with abnormal amplitude-integrated electroencephalography, 9 (60%) had brain injury. One infant with brain injury had a seizure on amplitude-integrated electroencephalography. A severely abnormal background pattern on amplitude-integrated electroencephalography was associated with brain atrophy (P = 0.03) and absent sleep-wake cycling (P = 0.022). CONCLUSION: Background cerebral activity is abnormal on amplitude-integrated electroencephalography following birth in newborns with congenital heart disease who have findings of brain injury and/or brain atrophy on preoperative brain magnetic resonance imaging.

ß-Blockers and angiotensin converting enzyme inhibitors: comparison of effects on aortic growth in pediatric patients with Marfan syndrome.

OBJECTIVES: Angiotensin converting enzyme inhibitors (ACEI) have been shown to decrease aortic growth velocity (AGV) in Marfan syndrome (MFS). We sought to compare the effect of ß-blockers and ACEI on AGV in MFS. STUDY DESIGN: We retrospectively reviewed all data from all patients with MFS seen at Arkansas Children's Hospital between January 1, 1976 and January 1, 2013. Generalized least squares were used to evaluate AGV over time as a function of age, medication group, and the interaction between the 2. A mixed model was used to compare AGV between medication groups as a function of age, medication group (none, ß-blocker, ACEI), and the interaction between the 2. RESULTS: A total of 67 patients with confirmed MFS were identified (34/67, 51% female). Mean age at first encounter was 13 ± 10 years, with mean follow-up of 7.6 ± 5.8 years. There were 839 patient encounters with a median of 10 (range 2-42) encounters per patient. AGV was nearly normal in the ß-blocker group, and was less than either the ACEI or untreated groups. The AGV was higher than normal in ACEI and untreated groups (P < .001 for both). CONCLUSIONS: ß-blocker therapy results in near-normalization of AGV in MFS. ACEI did not decrease AGV in a clinically significant manner.

Increased maternal cytokine production and congenital heart defects.

Congenital heart defects (CHDs) are a major cause of infant mortality. Most CHDs are thought to result from genetic, lifestyle, and environmental factors that include maternal obesity, diabetes, toxicant exposure, and alterations in anti-oxidant capacity. Since these well-documented risk factors are also associated with immune dysregulation, we sought to compare the maternal immune response in mothers carrying a fetus with a CHD with those mothers whose pregnancies were not affected by any birth defect. We conducted a case-control study to examine the maternal cytokine profile using multiplex technology in pregnant mothers (subject mean=26 weeks' gestation). This investigation revealed that whole blood cultures derived from case mothers produced higher levels of certain cytokines and chemokines compared with cultures from control subjects when activated with mitogen. Cultures from case subjects produced higher levels of IL-10, IL-13, IL-4, IL-5, IL-17, and IL-6, when stimulated with mitogen compared with control subjects. Plasma levels of chemokine MIP-1α were higher in cases compared with controls. In contrast, C-reactive protein levels were not statistically different. These results demonstrate the need to further examine the maternal cytokine signature in CHD-affected pregnancies. This information could pave the way toward maternal immunotherapeutic intervention to prevent CHDs, and novel biomarker discovery to improve pre-natal diagnosis.

Transesophageal Doppler measurement of renal arterial blood flow velocities and indices in children.

BACKGROUND: Doppler-derived renal blood flow indices have been used to assess renal pathologies. However, transesophageal ultrasonography (TEE) has not been previously used to assess these renal variables in pediatric patients. In this study, we (a) assessed whether TEE allows adequate visualization of the renal parenchyma and renal artery, and (b) evaluated the concordance of TEE Doppler-derived renal blood flow measurements/indices compared with a standard transabdominal renal ultrasound (TAU) in children. METHODS: This prospective cohort study enrolled 28 healthy children between the ages of 1 and 17 years without known renal dysfunction who were undergoing atrial septal defect device closure in the cardiac catheterization laboratory. TEE was used to obtain Doppler renal artery blood velocities (peak systolic velocity, end-diastolic velocity, mean diastolic velocity, resistive index, and pulsatility index), and these values were compared with measurements obtained by TAU. Concordance correlation coefficient (CCC) was used to determine clinically significant agreement between the 2 methods. The Bland-Altman plots were used to determine whether these 2 methods agree sufficiently to be used interchangeably. Statistical significance was accepted at P ≤ 0.05. RESULTS: Obtaining 2-dimensional images of kidney parenchyma and Doppler-derived measurements using TEE in children is feasible. There was statistically significant agreement between the 2 methods for all measurements. The CCC between the 2 imaging techniques was 0.91 for the pulsatility index and 0.66 for the resistive index. These coefficients were sensitive to outliers. When the highest and lowest data points were removed from the analysis, the CCC between the 2 imaging techniques was 0.62 for the pulsatility index and 0.50 for the resistive index. The 95% confidence interval (CI) for pulsatility index was 0.35 to 0.98 and for resistive index was 0.21 to 0.89. The Bland-Altman plots indicate good agreement between the 2 methods; for the pulsatility index, the limits of agreement were -0.80 to 0.53. The correlation of the size of the measurement and the mean difference in methods (-0.14; 95% CI = -0.28, 0.01) was not statistically significant (r = 0.31, P = 0.17). For the resistive index, the limits of agreement were -0.22 to 0.12. The correlation of the size of the measurement and the mean difference in methods (-0.05; 95% CI = -0.09, -0.01) was not statistically significant (r = 0.10, P = 0.65). CONCLUSION: This study confirms the feasibility of obtaining 2-dimensional images of kidney parenchyma and Doppler-derived measurements using TEE in children. Angle-independent TEE Doppler-derived indices show significant concordance with those derived by TAU. Further studies are required to assess whether this correlation holds true in the presence of renal pathology. This technique has the potential to help modulate intraoperative interventions based on their impact on renal variables and may prove helpful in the perioperative period for children at risk of acute kidney injury.

Repair of an anomalous left coronary artery from the pulmonary artery in a premature neonate.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. It is typically diagnosed at a few months of age. The infant presents with cardiac failure secondary to left ventricular dysfunction from the coronary steal that develops as pulmonary vascular resistance (PVR) falls. With the diagnosis made, surgical repair is typically performed expeditiously in order to try and restore left ventricular perfusion and stop the ongoing coronary steal. We present an unusual case of a preterm infant undergoing a routine echocardiogram, who was incidentally found to have an ALCAPA. Management strategies between neonatology and cardiology may differ in this setting. In this case, elevated PVR helps to preserve myocardial perfusion prior to surgical repair. Therefore, common neonatal management strategies typically utilized for respiratory distress, elevated PVR, and cardiac dysfunction may have potential for detrimental effects on myocardial perfusion in this specific lesion. This case also emphasizes the importance of identifying the coronary origins as a routine part of a complete pediatric echocardiogram. It also presents a rarely encountered problem: when to repair an ALCAPA in an as yet asymptomatic patient. We believe this to be the youngest and smallest patient reported with the diagnosis and successful surgical correction of an ALCAPA.

Tricuspid annular and septal Doppler tissue velocities are reduced in pediatric heart transplant recipients without acute rejection.

BACKGROUND: We sought to determine any differences between myocardial velocities in healthy children and pediatric heart transplant recipients without acute allograft rejection and to study the clinical factors that could alter these velocities. METHODS: Fifty-eight pediatric heart transplant recipients without rejection and 27 healthy controls were prospectively enrolled. Doppler tissue imaging (DTI) was performed at the tricuspid annulus, septum, and mitral annulus. The influence of the following factors on DTI was assessed: time since transplant, extracorporeal membrane oxygenation, systemic hypertension, graft atherosclerosis, pulmonary hypertension, donor heart ischemic time, and previous rejection. RESULTS: The mean tricuspid annular and septal tissue velocities were significantly reduced in the transplant group compared with controls. The mitral annular velocities were similar in the two groups. Donor heart ischemic time and previous rejection significantly altered DTI velocities. CONCLUSION: Tricuspid annular and septal Doppler tissue velocities are significantly reduced in pediatric heart transplant recipients without allograft rejection and can be altered by prolonged donor heart ischemic time and previous rejection.

Usefulness of enalapril versus propranolol or atenolol for prevention of aortic dilation in patients with the Marfan syndrome.

Despite variable clinical results, beta blockers have become the primary therapy for prevention of aortic dilation in patients with the Marfan syndrome. This study examines the use of the angiotensin-converting enzyme inhibitor enalapril for treatment of these patients. We sought to examine the effects of enalapril versus beta-blocker therapy in patients with the Marfan syndrome and noted improved aortic distensibility (3.0 +/- 0.3 vs 1.9 +/- 0.4 cm2 dynes(-1); p <0.02) and a reduced aortic stiffness index (8.0 +/- 2.9 vs 18.4 +/- 3.8; p <0.05) in patients receiving enalapril compared with those receiving beta blockers. These favorable hemodynamic changes were associated with a smaller increase in aortic root diameter (0.1 +/- 1.0 vs 5.8 +/- 5.2 mm) and fewer clinical end points during follow-up.

Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death?

OBJECTIVES: We sought to assess outcomes in a series of young patients with Marfan syndrome and to define the prevalence of ventricular arrhythmias in this patient population. BACKGROUND: While sudden death is a well-recognized outcome in Marfan syndrome, ventricular arrhythmias are not well described. METHODS: Patients were followed with echocardiography, electrocardiography, and ambulatory electrocardiography. The prevalence and associated factors for ventricular dysrhythmias were defined. RESULTS: Seventy patients with Marfan syndrome diagnosed at birth to 52 years were followed for a period of up to 24 years. All patients had cardiovascular involvement and were started on medical therapy. No patient died from aortic dissection, while 4% died from arrhythmias. Ventricular arrhythmias were present in 21% and were associated with increased left ventricular size, mitral valve prolapse, and abnormalities of repolarization. CONCLUSIONS: Cardiac complications are rare in young patients with Marfan syndrome receiving medical therapy and close clinical follow-up. Sudden death still occurs, and appears more common in patients with a dilated left ventricle. Left ventricular dilation may predispose to alterations of repolarization and fatal ventricular arrhythmias.