C. F. Luna

Prenatal Detection of Congenital Heart Disease in Southern Nevada The Need for Universal Fetal Cardiac Evaluation

Congenital cardiac malformations are common developmental anomalies. In the United States, congenital heart disease is the number one cause of infant mortality from congenital malformations. Prenatal diagnosis of congenital heart defects aids treatment coordination. Our aim was to evaluate prenatal detection of serious congenital heart malformations in Clark County, Nevada.

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.

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.

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.

Enlarged Left Vitelline Vein Remnant as a Cause of Cyanosis after the Fontan Procedure: Resolution with an Amplatzer Vascular Plug

A 6-year-old girl with heterotaxy and a functional single ventricle had persistent cyanosis 4 years after a fenestrated Fontan procedure. Cardiac catheterization revealed a large venous fistula from a left-sided hepatic vein to the coronary sinus, resulting in desaturation. The anomalous vein was occluded with an Amplatzer vascular plug.

FETAL BRADYCARDIA. A PRACTICAL APPROACH

Fetal bradycardia may herald fetal demise. This article highlights arrhythmic fetal bradycardia rather than bradycardia caused by perinatal distress. We briefly examine the embryonic conduction systems development and physiology and we review the classification, aetiology, evaluation, and approach to fetal bradycardia. Our aim is to provide the clinician with practical information about fetal bradycardia that enlightens causative conditions and aids management.

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%.

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.

172 BRAIN NATRIURETIC PEPTIDE: A SENSITIVE MONITOR IN PATIENTS WITH HYPOPLASTIC LEFT HEART SYNDROME.

Introduction Amplified cardiac B-natriuretic peptide (BNP) expression results from ventricular volume or pressure overload. The literature reports limited experience with serial BNP levels in pediatric patients. We report a small series of patients with hypoplastic left heart syndrome (HLH) undergoing serial outpatient BNP monitoring. Objective To identify the clinical usefulness of rapid, serial BNP-level monitoring in patients with complex congenital heart disease. Methods Rapidly available fingerstick BNP levels were obtained in our in-office certified outpatient clinical laboratory. Results take less than 20 minutes. We analyzed 36 BNP values obtained in 12 patients with HLH. Results Age ranged from 2 months to 8 years. Patients were status post stage I, II, or III HLH Norwood palliation, and all were on combination medical therapy, including angiotensin-converting enzyme inhibitors, digoxin, diuretics, and aspirin or Coumadin. Serial BNP levels were normal (20 ± 6 pg/dL) in 8 patients > 4 weeks after cardiac surgery. BNP levels were elevated (range 103-839 pg/dL) during serial evaluation in four patients, two with systemic pressure overload (one with systemic hypertension and one with residual coarctation) and two with volume overload (atrioventricular valve regurgitation [AVR]). Elevated BNP values decreased with adjustments to medical therapy in the patients with hypertension and AVR and by interventional cardiac catheterization in the patient with residual coarctation. Conclusions BNP levels were normal in hemodynamically stable HLH patients. Abnormal BNP levels were a guide to clinical management. Prolonged pathologic loading conditions, especially in those patients with single right ventricles, may lead to ventricular dysfunction, congestive heart failure, and death. Predicting the potential for ventricular dysfunction is preferable to detecting its presence. Validation of the clinical usefulness of rapid, serial BNP level monitoring in patients with complex congenital heart disease warrants further study.

IMPROVEMENT IN EXERCISE CAPACITY IN OVERWEIGHT CHILDREN TREATED IN A LIFESTYLE MODIFICATION PROGRAM.: 299

Background Physical inactivity and decreased exercise capacity have been associated with long-term poor prognosis in terms of morbidity and mortality in overweight children. Objectives To assess changes in exercise capacity in overweight children attending a 12-week lifestyle modification program. Methods This study includes data from 121 children with body mass index (BMI) ≥ 95th percentile who complete a 12-week lifestyle modification program. The program consists of nutrition counseling and medically supervised exercise. BMIs were assessed using the 2000 CDC growth charts. Exercise capacity was estimated by the amount of exercise load (Rockport walking test) and the energy cost of exercise (metabolic equivalents [METs]). All measurements were taken at entry and at the end of the program. Each individual served as his or her own control. Results The group was composed of 50 girls and 73 boys, mean age 11.2 years (range 6-17 years) and ethnic distribution of Hispanic, 43%; Caucasian, 33%; African American, 7%; and other races, 13%. By the end of the program there was significant increase in the Rockport test (19.5 ± 11.9 vs 29.2 ± 9.3, p Conclusions This group of children was successful in achieving a significant improvement in their exercise capacity and BMI Z-score, after participation in a 12-week lifestyle modification program.