Faculty Bibliography

The use of prostaglandin infusion to maintain patency of the ductus arteriosus in patients with critical coarctation of the aorta (CoA) to support systemic circulation is the standard of care. However, pulmonary overcirculation resulting from a patent ductus arteriosus in patients with critical CoA is not well described in the literature. We report two cases of critical CoA that required invasive measures to control pulmonary blood flow before surgical repair of the CoA. Both patients had signs of decreased oxygen delivery, hyperlactatemia, and systemic to pulmonary flow via the ductus arteriosus. One patient required surgical pulmonary artery banding and the second patient underwent pulmonary flow restrictor device placement for the control of pulmonary blood flow. A rapid improvement in oxygen delivery and normalization of lactate levels were observed after control of pulmonary overcirculation. Both patients underwent successful surgical repair of the coarctation A and were discharged home.

BACKGROUND/UNASSIGNED:Echocardiographic quantification of left ventricular (LV) volume and ejection fraction (EF) is widely used in the pediatric population. However, there is no consensus on the most accurate method of quantifying ventricular volumes and systolic function. PURPOSE/UNASSIGNED:The purpose of this study is to compare two commonly used echocardiographic methods for the evaluation of LV volume and quantification of EF, the five-sixth area-length (5/6 AL) and the modified biplane Simpson (BS), to cardiac magnetic resonance (CMR) imaging in children. METHODS/UNASSIGNED:CMR studies were paired with echocardiograms and retrospectively analyzed in children 18 years of age and younger. Studies performed more than 3 months between modalities, patients with congenital heart disease, and patients who had changes in medication regimen between corresponding CMR and echocardiograms were excluded. LV volumes and EF were calculated using the 5/6 AL and BS methods and compared to volumes and EF measured on corresponding CMR studies. Subgroup analyses were conducted based on LV function, pathology, and weight. RESULTS/UNASSIGNED:= 0.82). However, both echocardiographic methods overestimated LVEF and underestimated LV volumes when compared to CMR. CONCLUSION/UNASSIGNED:Left ventricular volumes and EF, as measured by echocardiography, correlate well with CMR measurements. Echocardiography underestimates LV systolic and diastolic volumes and overestimates LVEF. While echocardiography is a good surrogate for estimating LVEF, CMR should be considered in patients for whom accurate measurements are needed for critical clinical decision-making.

Congenital left ventricle to coronary sinus fistula is a rare entity. We report a case of an infant with prenatal finding of left ventricle to right atrial shunt. The anatomy was defined by multi-modality imaging. Baseline electrocardiogram was notable for a Wolff-Parkinson-White pattern. He underwent successful catheter device closure of the left ventricle to coronary sinus fistula. The patient developed supraventricular tachycardia and underwent successful ablation of the accessory pathway.

Surgical management of single ventricle with interrupted inferior vena cava and azygos continuation typically requires a Kawashima procedure with subsequent completion of Fontan. However, this group is at risk of development of pulmonary arteriovenous malformations. Evidence suggests preservation of hepatic venous flow into the pulmonary circulation can potentially delay this development. We hereby describe a method of preserving antegrade pulmonary blood flow during the Kawashima procedure in the setting of prior right ventricular outflow tract stents.

A patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea-orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions.

This report describes a young adult man presenting with subarachnoid hemorrhage secondary to an intracranial aneurysm who was found to have a short-segment type B interrupted aortic arch. We describe the clinical presentation, evaluation, and management of this patient and highlight imaging findings and percutaneous repair of the aneurysm and interrupted aortic arch. (Level of Difficulty: Intermediate.).

Background A right aortic arch is present in 18% of patients with congenital heart disease, and is usually associated with Tetralogy of Fallot or truncus arteriosus. Its incidence in D-TGA ranges from <1% to 12% in the literature. An isolated left subclavian, defined as a left subclavian artery that is disconnected from the aorta and arises from a branch pulmonary artery, is a rare right aortic arch anomaly. It is typically associated with Tetralogy of Fallot and truncus arteriosus, and exceedingly rare in cases of D-TGA. Case A full-term neonate with prenatal diagnosis of D-TGA with VSD was born at our institution. At birth, the baby was vigorous but with low oxygen saturation in the right hand (60s). The baby was started on continuous positive airway pressure at 100% FiO2 as well as a prostaglandin infusion and transferred to the NICU. Our initial echocardiogram confirmed the diagnosis of D-TGA with moderate-size muscular VSD as well as a right-sided aortic arch. On echocardiogram there appeared to be an aberrant left subclavian artery that could not be imaged definitively. The baby was taken to the catheterization lab the next day for balloon atrial septostomy and angiograms to better delineate arch anatomy. Angiography showed a left subclavian artery arising from the left pulmonary artery Decision-making In the case of our patient, the typical practice of measuring pre- and post-ductal saturations in the right and left hands, respectively, did not yield the "classic" picture of reverse differential cyanosis. We opted to monitor pre-ductal saturations on the right earlobe to better monitor saturations. Conclusion It is important to consider variability in arch sidedness and aortic arch anatomy when evaluating a newborn with D-TGA. We recommend checking for a pre-ductal saturation on one of the ears, if feasible, as this data is more representative of the arterial saturation of the cerebral blood vessels. Another important clinical consideration with an isolated subclavian artery with or without TGA is the subclavian steal phenomenon. When pulmonary vascular resistance drops, flow will preferentially go from the systemic circulation into the pulmonary circulation, reducing flow in vertebral arteries.
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