Hybrid Palliation for Ductal-Dependent Systemic Circulation.

We reviewed our hybrid palliation experience for 91 neonates, with ductal-dependent systemic circulation, born between August 2007 and October 2015. For analysis, we stratified the 91 patients by a risk factor (RF) score and divided them into three groups: (1) high-risk two-functional ventricles (2V) median RF score of 3 (N = 20); (2) low-risk one-functional ventricle (1V) RF score 0-1 (N = 32); and (3) high-risk 1V RF score ≥2 (N = 39). Midterm survival (median 4 years) by group was: (1) 95 %, (2) 91 %, and (3) 15 %, (p = 0.001). In conclusion, hybrid palliation was associated with excellent midterm results for high-risk 2V and low-risk 1V patients with ductal-dependent systemic circulation. In contrast, high-risk 1V patients had significantly worse outcomes.

Moving towards universal prenatal detection of critical congenital heart disease in southern Nevada: a community-wide program.

This study compares the current, prenatal detection rate for critical congenital heart disease in Southern Nevada with the previously reported rate, after developing and expanding a comprehensive, community-wide fetal cardiology program. For the current-period analysis, we inquired our database and electronic health records for patients born in Clark County, Nevada, with critical congenital heart disease between May 2012 and April 2014, and we compared the results with the previous period between May 2003 and April 2006. The major components of the community-wide program include fetal congenital heart disease screening via general obstetric ultrasound studies performed in obstetrician's offices, radiology imaging centers, or maternal-fetal medicine specialty practices; subsequent referral for comprehensive fetal echocardiography performed in maternal-fetal medicine offices under the on-site supervision by fetal cardiologists; and recurring community educational programs teaching the 5-axial plane, fetal echocardiographic screening protocol to general obstetric sonographers and instructing perinatal sonographers in advanced imaging topics. For the current period, the prenatal detection rate for critical congenital heart disease in Southern Nevada was 71 versus 36% for the previous period (p < 0.001). The temporal improvement in prenatal detection of critical congenital heart disease may be related to our expanded decentralized, community-wide fetal cardiology program, and our experiences may be applicable to other metropolitan areas.

Dextrocardia: practical clinical points and comments on terminology.

Dextrocardia is defined by the authors as a right-sided heart with a base-apex axis directed rightward, resulting from a variation in cardiac development, and not used as a general term indicating any heart in the right chest. Dextrocardia occurs in approximately 0.01% of live births and can be discovered in various clinical settings and at various patient ages. The authors review their experience with dextrocardia, discuss useful clinical points that aid in evaluating complex anatomy, recount the history of dextrocardia terminology, and note the current inconsistent nomenclature.

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.

Hair-grooming syncope in children.

OBJECTIVES: This report describes children undergoing pediatric cardiology evaluation for syncope in which a hair-grooming trigger was determined as the stimulus. METHODS: A review of our database revealed 1525 patients with syncope seen by our program of whom 111 had a hair-grooming trigger determined as a cause. RESULTS: Of the 111 patients, 78% were girls. We found characteristic difference between boys and girls with boys experiencing syncope more during hair cutting whereas girls experienced syncope more during hair combing and brushing. Electrocardiograms and echocardiograms were performed as part of syncope evaluation and no significant abnormalities were found in either test in this patient group. CONCLUSIONS: This is the largest reported group of children presenting with syncope that had a hair-grooming trigger. Our data also include the first series of boys with the condition. The hair-grooming trigger appears to stimulate a benign form of neurocardiogenic reflex syncope.

Prenatal detection of congenital heart disease in southern Nevada: the need for universal fetal cardiac evaluation.

OBJECTIVE: 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. METHODS: We electronically searched our research department-maintained computer database for patients with serious congenital heart disease born in Clark County between May 2003 and April 2006. We excluded patients that did not have at least 1 local prenatal ultrasound examination. All pre-natal ultrasound studies were performed in obstetric offices, radiology imaging centers, or maternal-fetal medicine specialty practices. Fetal echocardiography was performed in maternal-fetal medicine specialists' offices under the supervision of a fetal cardiologist. Pediatric cardiologists performed all postnatal echocardiographic examinations. RESULTS: During the study period, we diagnosed serious congenital heart malformations in 161 patients among a total estimated 77,000 births (2/1000). Of the 161 patients, 58 (36%) had a prenatal diagnosis, and 103 (64%) had an exclusively postnatal diagnosis. CONCLUSIONS: Standard prenatal ultrasound fails to show congenital heart disease in most fetuses.

Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion.

OBJECTIVE: 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. METHODS: We searched our fetal cardiology database for diagnoses of ADV from May 2003 to December 2006. RESULTS: During the study period, we performed 1328 fetal echocardiographic examinations in 990 fetuses. We found 6 cases of ADV (6/1000). Indications for fetal echocardiography were cardiomegaly, dilated umbilical or systemic veins, and extracardiac abnormalities. We identified 5 anatomic variants of ADV. In 2 patients, the umbilical vein connected to the systemic venous circulation by way of the portal sinus: via an abnormal venous channel from the portal sinus to the right atrium (case 1) and presumably via hepatic sinusoids to the hepatic veins (case 2). In the remaining 4 patients, the umbilical vein bypassed the portal sinus and the liver and connected to the systemic venous circulation via an abnormal venous channel: from the umbilical vein to the right atrium (case 3), from the umbilical vein to the inferior vena cava (cases 4 and 5), and from the umbilical vein to the right iliac vein (case 6). All patients survived; 2 required cardiovascular intervention. No intervention was required in 3 patients. CONCLUSIONS: An ADV should be ruled out in a fetus with unexplained cardiomegaly or dilatation of the umbilical vein, systemic veins, or portal sinus. To our knowledge, prenatal diagnosis of an ADV with an abnormal communication between the portal sinus and the right atrium has not been reported previously. The portosystemic communication persisted after birth and required device occlusion.