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%.

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.

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.

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 degrees 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.

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.

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.

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.