G. A. Mayman

Diagnosis of Absent Ductus Venosus in a Population Referred for Fetal Echocardiography Association With a Persistent Portosystemic Shunt Requiring Postnatal Device Occlusion

The purpose of this series was to assess the incidence, anatomic variants, and implications of an absent ductus venosus (ADV) in patients referred for fetal echocardiography.

Vascular Ring Diagnosis and Management: Notable Trends Over 25 Years.

Background: Vascular rings (VRs) are recognized as uncommon but not rare cardiovascular malformations. Methods: We analyzed data from all patients born in Southern Nevada, who underwent diagnosis and management of VR from 1990 to 2015, Results: From 1990 to 2015, a total of 92 patients were diagnosed prenatally and postnatally. Of the 92 patients, 73 (79%) had right aortic arch and aberrant left subclavian artery (RAA-ALS) with a left ductus arteriosus or ligamentum, 17 (19%) had a double aortic arch (DAA), and 2 (2%) had a pulmonary artery sling. Of the 92 patients, 75 had an isolated VR and 17 VR had significant additional congenital heart disease (CHD). Of the 75 patients with an isolated VR, 52 underwent surgical repair, and the most common surgical diagnosis was DAA in 6 (66%) of 9 for the period 1990 to 2005 versus less common in 9 (21%) of 43 during the period 2006 to 2015, P < .05. The isolated VR repair age significantly negatively correlated with increasing time from 1990 to 2015, R = −0.7 (P < .0001). Of the 75 isolated VR, 23 remain asymptomatic. The 17 VR with CHD were treated during infant palliation or intracardiac repair. Of the total 92 VR, 60 were born after a 2004 community introduction of the three-vessel fetal echocardiography view, from then the prenatal-detection rate has significantly increased—2004 to 2006, 0 (0%) of 9; 2007 to 2009, 1 (9%) of 11; 2010 to 2012, 11 (55%) of 20; and 2013 to 1015, 14 (70%) of 20 (P < .0001). Conclusion: Over 25 years in Southern Nevada, VR prenatal diagnosis has increased, isolated VR age at surgery has decreased, and the percentage of those with RAA-ALS has increased.

Percutaneous fenestration closure with problematic residual native atrial septum

A variety of techniques have been utilized to close Fontan fenestrations. Among 20 patients who underwent a cardiac catheterization for fenestration closure, 3 patients had residual native atrial septum, forming an additional intermediate chamber on the pulmonary venous side of the fenestration. Three different methods were used to close these fenestrations.

Simplified Pediatric Electrocardiogram Interpretation

We describe a simplified method for interpreting a pediatric electrocardiogram (EKG). The method uses 4 steps and requires only a few memorized rules, and it can aid health care providers who do not have immediate access to pediatric cardiology services. Most pediatric EKGs are normal. However, both abnormal and normal EKGs should be sent to a pediatric cardiologist for later, confirmatory interpretation.

Overlapping Covered Stents to Exclude a Postcoarctation Stenting Aortic Aneurysm

An 18-year-old boy who underwent coil occlusion of a patent ductus arteriosus and stenting for aortic coarctation at 13 years of age had evidence of re-stenosis. Cardiac catheterization and angiography showed a 25-mmHg gradient across the stent and a large aneurysm originating at midstent level. The aneurysm measured 2 cm in width and 4.3 cm in length. At a subsequent catheterization procedure, two 4.5-cm-long overlapping Cheatham platinum covered stents were implanted, completely excluding the aneurysm. Computed tomographic (CT) angiography 2 years later showed no evidence of endoleak or re-stenosis.

A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: Preliminary findings

OBJECTIVE We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan. METHODS Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square root of the number of years post-Fontan. Further, we analyzed correlations between Fontan hepatic index values and fibrosis scores from hepatic biopsy. RESULTS We identified a total of 79 post-Fontan patients that underwent cardiac catheterization and liver biopsy. Of the 79 patients, 53 met inclusion criteria, and 32 consented to undergo hepatic-ultrasound elastography. Of the 32 that underwent elastography, data from 30 patients was used for analysis. We found no statistically significant differences in demographics, laboratory values, or cardiac catheterization data between the 30 included patients and the 21 that did not participate. Utilizing data from the 30 included patients, we found a strong, highly statistically significant correlation between the Fontan hepatic index values and total fibrosis scores (R = 0.8, P < .00001). However, the cohort size prevented reliable discriminating cut-off values for the range of total fibrosis scores. CONCLUSIONS In a small cohort of patients post-Fontan, preliminary findings suggest that the composite Fontan hepatic index might be a clinically useful, noninvasive method of serially monitoring for hepatic fibrosis. Further studies, with large patient cohorts, are necessary to validate our findings and develop clinically useful discriminatory cutoff values.

Right aortic arch with situs solitus frequently heralds a vascular ring

OBJECTIVE We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring. METHODS From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch and situs solitus. From the 50 patients, 6 did not meet inclusion criteria for further analysis. RESULTS Of the 44 remaining, 33 (75%) had a vascular ring. Of the 33 with a vascular ring, 26 (79%) occurred with an isolated right aortic arch, and 7 (21%) had an associated cardiovascular malformation. Of the total 44 patients with a right aortic arch in situs solitus, 34 (79%) were diagnosed prenatally. CONCLUSIONS In conclusion, we found a right aortic arch in situs solitus was often associated with a vascular ring. Further, to the best of our knowledge, no previous general population study has demonstrated an equal or higher right aortic arch, prenatal detection rate of 79%.

B-natriuretic Peptide: A Helpful Clinical Marker After Norwood I

Amplified cardiac B-natriuretic peptide (BNP) expression results from ventricular volume or pressure overload. Clinicians have used BNP levels when evaluating cardiac performance in patients with varied clinical conditions. We report a case in which BNP levels helped guide early catheterization intervention in a patient after stage 1 Norwood palliation.

Expedited Oxygen Wean After Coil Embolization of Systemic-to-Pulmonary Collaterals in a Premature Infant With Bronchopulmonary Dysplasia

Systemic-to-pulmonary collaterals (SPCs) occur in a variety of pathologic conditions, including congenital heart diseases, chronic pulmonary infections, lung tumors, pulmonary emboli, and bronchopulmonary dysplasia. 1,2 Systemic-to-pulmonary collaterals can also occur in premature infants without cardiac or lung disease; such collateral vessels appear to be transient.3 Occasionally, SPCs result in left-to-right shunts that warrant intervention. 2,4,5 We describe the case of a preterm infant whom we weaned from chronic supplemental oxygen after coil embolization of 2 persistent SPCs.

SYSTEMIC TO PULMONARY COLLATERALS IN A PREMATURE INFANT WITH BRONCHOPULMONARY DYSPLASIA.: 153

Introduction Systemic to pulmonary artery collaterals (SPCs) occur in a variety of pathologic conditions, including congenital heart disease, chronic pulmonary infections, lung tumors, pulmonary emboli, and bronchopulmonary dysplasia. SPCs may result in symptomatic left-to-right shunts. We describe a preterm infant with two SPCs who we weaned from chronic supplemental oxygen following coil embolization of persistent SPCs. Case A 580 g female infant product of a twin pregnancy was born by cesarean section at 28 weeks9 gestation. The patient was hospitalized for a total of 116 days. On day 13 of life an echocardiogram demonstrated a patent ductus arteriosus and left ventricular volume overload, requiring surgical ligation. After surgery the infant continued to experience pulmonary congestion and ventilator dependency. Extubation was successful at 51 days of age, but the patient continued to receive oxygen via nasal canula. On day 98, the echocardiogram demonstrated SPCs, and a cardiac catheterization was performed. The aortogram showed two small to moderate collaterals from the descending aorta to the right lung, each measuring approximately 1.5 mm in diameter. Both were occluded by coil embolization. Nine days after the procedure, the infant was weaned to room air. Conclusion SPCs may contribute to prolonged ventilatory support in premature infants. SPCs can be demonstrated by echocardiographic color Doppler interrogation of the aortic arch and the proximal descending aorta. Coil embolization by interventional cardiac catheterization is indicated in symptomatic patients. Careful echocardiographic evaluation is necessary to rule out SPCs in premature infants with a prolonged supplemental oxygen requirement.