Faculty Bibliography

BACKGROUND:Patent foramen ovale (PFO) is a common finding that occurs in approximately 25% of the population and, in the absence of clinical sequelae, is considered benign. However, PFO can potentially become clinically significant and result in systemic embolization, strokes, or hypoxemia. During pregnancy, hemodynamic cardiovascular changes may unmask previously undetected congenital heart anomalies or exacerbate existing ones, affecting both mother and fetus. CASE SUMMARY/METHODS:A healthy 33-year-old woman developed symptomatic hypoxemia on minimal exertion during pregnancy secondary to newly diagnosed right-to-left intracardiac shunting through a PFO. We posit that the physiological changes of pregnancy enhanced this abnormal streaming through the PFO without evidence of pulmonary hypertension. The patient had a successful percutaneous occlusion of the PFO with a 25-mm Amplatzer cribriform septal occluder device at 18 weeks' gestation, with appropriate fetal protection. CONCLUSIONS:Pregnancy may produce or accentuate acute hypoxemia due to right-to-left shunting through a PFO. The PFO may be safely treated with percutaneous device closure to resolve hypoxemia-related symptoms and avert risks of hypoxia to the developing fetus.

BACKGROUND:There are multiple potential etiologies for right-to-left shunting across a large atrial septal defect (ASD). CASE SUMMARY/METHODS:A 34-year-old woman who presented with exertional dyspnea was found to be cyanotic due to right-to-left shunting across a secundum ASD and hypoplastic right ventricle (RV) due to an abnormal interventricular septum. Balloon occlusion of the ASD resulted in a significant drop in systemic cardiac output, so the patient underwent bidirectional Glenn shunt to both unload and partially bypass a hypoplastic RV, allowing for ASD closure. DISCUSSION/CONCLUSIONS:The bidirectional Glenn shunt is primarily used in the staged palliation of single-ventricle physiology in pediatric patients, and its use in adults is rare. In this case, its use allowed for hemodynamic tolerance of ASD closure with a hypoplastic RV. TAKE-HOME MESSAGE/CONCLUSIONS:This case exemplifies the complex hemodynamics that goes into consideration of ASD closure with an abnormal RV.

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.