Faculty Bibliography

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.

Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.