Robert C. Rollins

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.

Hemidiaphragm plication after repair of congenital heart defects in children : Quantitative return of diaphragm function over time

OBJECTIVE Phrenic nerve injury resulting in hemidiaphragm paresis leads to morbidity in children undergoing repair of congenital heart defects. Previous studies have documented short-term benefits of diaphragm plication, but little is known about the return of diaphragm function. METHODS We reviewed 46 consecutive patients undergoing hemidiaphragm plication after repair of congenital heart defects. The function of plicated diaphragms was measured at follow-up fluoroscopy using excursion of the unplicated side as a control. RESULTS The median age at the procedure resulting in phrenic nerve injury was 6.4 months (0-62 months). Among the 46 patients, 29 (63%) and 17 (37%) had repair for single and 2-ventricle defects, respectively. Hemidiaphragm paresis occurred on the left side in 32 patients (70%). Phrenic nerve injury was documented at a median of 8 days (1-84 days) after operation. The median time from diagnosis to plication was 2 days (0-21 days). Five patients required prolonged ventilation after plication. One patient died 10 weeks later, and 4 patients required tracheostomy. The remaining 41 patients were extubated within 2 days (0-19 days). In 17 patients, fluoroscopy assessing diaphragm motion was performed at a mean interval of 16.4 months after plication. Excursion of the plicated diaphragm was 77% of the contralateral side. There was a trend toward improved function over time. CONCLUSIONS Hemidiaphragm paresis results in significant morbidity after repair of congenital heart defects. Early diagnosis and plication result in timely extubation. The plicated diaphragm demonstrates return of function that may improve over time. This is the first study to numerically quantitate the degree of diaphragm recovery.

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.

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.

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.

Doppler fetal mechanical PR interval prolongation with positive maternal anti-RNP but negative SSA/Ro and SSB/La auto-antibodies.

Cutaneous neonatal lupus can occur with negative SSA/Ro or SSB/La but positive non-SSA/SSB ribonucleoprotein (RNP) maternal autoantibodies (Provost et al., 1987). No fetal-cardiac manifestations, however, have been reported in association with isolated maternal RNP autoantibodies. We report the first association of transient first-degree atrioventricular block (AVB) in a fetus exposed to maternal anti-RNP in the absence of SSA/Ro-SSB/La autoantibodies.

All Cardiac Right Ventricular Outpouches Are Not Created Equal

Congenital right ventricular diverticula and aneurysms are rare. Clinically, a diverticulum is difficult to distinguish from an aneurysm. Four diverse right ventricular cameral defects or right ventricular outpouches (RVOs) are described together with the management of each. Surgery may be necessary if an RVO has thin walls. However, if an RVO is composed of uniform thick contractile walls, conservative follow-up care likely is appropriate.

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.