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.

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.

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/UNASSIGNED:Acquired pulmonary vein stenosis (PVS) is an infrequent complication of atrial fibrillation ablation that is often misdiagnosed due to predominant respiratory symptoms. It can result in pulmonary venous hypertension, with varying presentations, ranging from shortness of breath to haemoptysis. Case summary/UNASSIGNED:We report two patients with a history of paroxysmal atrial fibrillation treated with radiofrequency ablation and pulmonary vein (PV) isolation, who subsequently developed PVS. Case 1 initially presented with indolent symptoms of shortness of breath and cough. He was initially diagnosed with and treated for pneumonia. In contrast, Case 2 presented with massive haemoptysis, requiring intubation and intensive care unit admission. Both patients were eventually diagnosed with PVS by computed tomography. They were treated with PV angioplasty and stenting. Discussion/UNASSIGNED:While previously limited to the congenital heart disease population, PVS is occurring more frequently now in adult patients as a complication of ablation procedures. It is most effectively treated with angioplasty and stent implantation but has a high rate of recurrence.

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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Partial anomalous pulmonary venous return (PAPVR) comprises a group of congenital cardiovascular anomalies associated with pulmonary venous flow directly or indirectly into the right atrium. Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins connected anomalously to the inferior vena cava. Surgery is the definitive treatment for scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left-to-right shunts, and enjoy a normal life expectancy without surgery. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome.