Ruben J. Acherman

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.

Cardiovascular Effects of Low-Dose Dexamethasone in Very Low Birth Weight Neonates with Refractory Hypotension

Background: Administration of hydrocortisone and relatively high doses of dexamethasone increase blood pressure in volume- and pressor-resistant hypotensive preterm infants. However, little is known about the temporal relationship of dexamethasone administration and the improvement in blood pressure and the weaning of pressors/inotropes. Furthermore, there are no sufficient data available on whether a smaller dose of dexamethasone would also be effective in treating refractory hypotension. Objective: To study the cardiovascular responses to low-dose dexamethasone in very low birth weight neonates with volume- and pressor-resistant hypotension. Methods: Retrospective database review. Twenty-four preterm neonates (gestational age 26 (23–34) weeks; birth weight 801 (457–1,180) g; postnatal age 2 (1–24) days, medians (ranges)) who remained hypotensive despite volume administration and combined dopamine and dobutamine treatment at ≧30 µg/kg/min received dexamethasone 0.1 mg/kg followed by 0.05 mg/kg intravenously every 12 h for 5 additional doses if still on pressors ≧8 µg/kg/min. Results: Two hours after the first dose of dexamethasone the mean blood pressure increased from 30 ± 5 to 34 ± 6 mm Hg (p < 0.001) and remained elevated at 4, 6, 12, and 24 h after treatment was started (p < 0.001). Six hours after the initial dose of dexamethasone the pressor/inotrope requirement decreased from 34 ± 9 to 24 ± 13 µg/kg/min (p = 0.001) and continued to decrease at 12 and 24 h (p < 0.001). Urine output also increased significantly during the first 6 h after dexamethasone (p < 0.001). Conclusions: Low-dose dexamethasone rapidly increases blood pressure and decreases pressor requirements in very low birth weight neonates with volume- and pressor-resistant hypotension.

Systemic to Pulmonary Collaterals in Very Low Birth Weight Infants: Color Doppler Detection of Systemic to Pulmonary Connections During Neonatal and Early Infancy Period

Objective. Angiographic visualization of systemic to pulmonary collaterals (SPC) has been documented in premature infants needing prolonged ventilatory support. Noninvasive identification of such communications in premature infants was reported recently. The purpose of this study was to describe: 1) incidence, 2) clinical findings and implications, and 3) short-term follow-up of SPC diagnosed by echocardiography in very low birth weight (VLBW) infants admitted to the neonatal intensive care unit. Methods. From December 1, 1994 to August 31, 1996, 196 infants with birth weight <1500 g were admitted to the neonatal intensive care unit; 133 of them received serial echocardiographic evaluations at 1 to 2 days, at 2 weeks, and at 1, 2, and 3 months of life. Follow-up echocardiograms were scheduled at 6 months and 1 year of age for patients with SPC persisting at 3 months of age. Results. SPC were demonstrated in 88 patients (66%) at 1 to 90 days of life (mean 28 days). In most cases, the SPC originated at the distal aortic arch or the proximal descending aorta. Ten patients (11%) were treated for congestive heart failure. The symptoms improved and anticongestive therapy was discontinued in 9. One patient with persistent congestive heart failure underwent therapeutic cardiac catheterization and 1 prominent SPC was embolized. Conclusions. The incidence of SPC in VLBW infants is much higher than previously reported. We postulate that SPC are bronchopulmonary communications that enlarge and/or proliferate in response to a given stimulus. These communications are associated with increased time on positive pressure ventilation and length of stay in the hospital. SPC may lead to pulmonary edema and should be searched for in VLBW infants with a more complicated course. Echocardiographic examination with color Doppler performed in premature infants to evaluate left to right shunts should include careful search for systemic to pulmonary collaterals. echocardiography, systemic to pulmonary collaterals, aortopulmonary collaterals, prematurity, pulmonary edema.

Aorta Larger Than Pulmonary Artery in the Fetal 3-Vessel View

Objective. Fetal great artery asymmetry may accompany congenital heart disease. The fetal echocardiographic 3‐vessel view (3VV) allows assessment of the superior vena cava, ascending aorta (AA), and main pulmonary artery (MPA). Our aim was to determine the association of congenital heart disease in fetuses with an AA/MPA ratio of greater than 1. Methods. We electronically searched our fetal echocardiographic database for studies performed between March 2002 and January 2008 that showed 3VVs with AA/MPA ratios of greater than 1 and correlated the findings with the presence of congenital heart disease. Results. In 2797 fetal echocardiograms, we identified 31 fetuses with normal 4‐chamber views showing AA/MPA ratios of greater than 1 in the 3VV. Of 31 fetuses, 25 (81%) had tetralogy of Fallot (ToF) or a ToF variant, and 6 (19%) had an aortic valve abnormality or isolated dilatation of the AA. Conclusions. Screening obstetric fetal sonography showing a 3VV AA/MPA ratio of greater than 1 suggests congenital heart disease and indicates the need for comprehensive fetal echocardiography.

A rare presentation of Pompe disease with massive hypertrophic cardiomyopathy at birth.

Abstract We report a term infant with Pompe disease presenting in the immediate newborn period. The infant was born at 40 weeks gestation, weighing 3600 g to a 32 year-old black female. Infant presented at delivery with massive hypertrophic cardiomyopathy and pulmonary hypertension. Diagnosis was confirmed by low α-glucosidase activity. The histopathology and electron microscopic findings were consistent with Pompe disease. This is the second reported case of Pompe disease presenting at delivery.

Argatroban Anticoagulation for Cardiac Surgery with Cardiopulmonary Bypass in an Infant with Double Outlet Right Ventricle and a History of Heparin-induced Thrombocytopenia

Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. Anticoagulation in pediatric patients with HIT is challenging as there are no approved heparin substitutes. We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.

Acute myocardial infarction in a 12-year-old as a complication of hyperosmolar diabetic ketoacidosis.

Objective This report describes the evolution of an acute myocardial infarction in a 12-yr-old male with severe hyperosmolarity secondary to diabetic ketoacidosis. Design Case report. Setting Intensive care unit of a university teaching hospital. Patient A 12-yr-old male with new-onset diabetic ketoacidosis and an abnormal electrocardiogram. Measurements and Main Results The evolution of an acute myocardial infarction in this patient is described. This includes serial electrocardiographic, echocardiographic, and enzymatic changes. Early recognition of this complication of diabetic ketoacidosis prompted immediate initiation of antithrombotic therapy and possible prevention of infarct extension. Conclusion This report demonstrates the need for accurate electrocardiographic monitoring in these patients, both for arrhythmias as well as ischemic changes and the potential for myocardial injury caused by hyperosmolarity.

Hypoalbuminemia, The etiology of antenatally diagnosed pericardial effusion in rhesus-hemolytic anemia

gested that intrauterine breathing represents, at least in part, “practice” for extrauterine life,’ it is tempting to speculate that fetal tachypnea may reflect unconscious struggling for oxygen of the asphyxiated fetus. l t may be that transient episodes of tachypnea precede the more commonly observed state of diminished or absent respiratory motion that characterizes the severely hypoxic fetus. Until additional observations clarify the significance of intrauterine tachypnea, we suggest this phenomenon be regarded as a possible manifestation of severe asphyxia and impending fetal death. When this finding is observed, particularly in association with an\ other abnormal antepartum monitoring test, consideration should be given to prompt delivery of the fetus.

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.

Stenosis of Alternative Umbilical Venous Pathways in Absence of the Ductus Venosus

Objective. We evaluated fetuses with absence of the ductus venosus (ADV) and restricted alternative umbilical venous pathways. Methods. We identified 3 cases that fit our objective. The angles of insonation for spectral Doppler ultrasound interrogation were less than 20° in all cases. We used commercially available ultrasound systems with a curved array transducer. Results. In all 3 cases, we noted mild cardiac volume overload without fetal hydrops. Conclusions. We speculate that the fetus with ADV and a restrictive alternative umbilical venous pathway may have a more benign clinical course than fetuses previously reported with unrestricted alternative pathways.