A 65-year-old Asian woman was admitted with exertional dyspnea. She had a medical history of hypertension, hyperlipidemia, and coronary artery disease that had required transcatheter intervention. Her family history also included coronary artery disease. Her social history was unremarkable with respect to environmental and workplace exposures, and she did not smoke or consume alcohol. A physical examination upon admission revealed no signs of cardiac congestion or neurological deficits. Her vital signs were a systemic blood pressure of 143/86 mmHg, pulse rate of 89 beats per minute, and a body temperature of 35.9 °C. Chest x-rays at the time of admission showed cardiomegaly with protrusion of the right first aortic arch, and electrocardiography showed an incomplete right bundle branch block of normal sinus rhythm. Coronary angiography performed because of her history of coronary artery disease revealed no significant coronary arterial stenosis. However, a concurrent pressure study revealed oxygen step-up in gas sampling between the SVC and right atrium (RA) with a pulmonary blood flow/systemic blood flow ratio (Qp/Qs) of 2.07, even though pulmonary artery pressure was normal in the pressure study and the atrial septum was intact on echocardiography. Contrast-enhanced computed tomography (CT) revealed that the RUPV flowed into the SVC (Fig. ) without any other congenital heart conditions, including an ASD and a dilated RA and right ventricle (RV). Isolated PAPVR was diagnosed on the basis of CT imaging findings, and surgery was indicated on the basis of the patient’s symptoms and the hemodynamic pressure findings.\nThe surgical approach was via a median sternotomy. The SVC was cannulated near the confluence of the innominate vein, sufficiently above the RUPV. A cardiopulmonary bypass was established, and cardiac arrest was applied. The RA was incised longitudinally. The intact atrial septum was incised in the cranial direction from the superior aspect of the fossa ovalis, and this new ASD formed a hole with an approximate diameter of 15 mm after the right and left atrial endocardia were closed using continuous 5-0 polypropylene sutures (Fig. a). An intra-atrial baffle comprising an autologous pericardial patch was sutured from the inferior border of the new ASD to the cavoatrial junction to separate the cavity between the RA and the SVC (Fig. b). The SVC was divided above the confluence of the RUPV, and the cardiac side of the SVC stump was sutured closed. A new RUPV outflow route to the left atrium (LA) was subsequently constructed through the surgically created ASD. A cavoatrial pathway between the cranial side of the SVC stump and the right atrial appendage (RAA) was reconstructed using a ringed extended polytetrafluoroethylene (EPTFE) prosthesis with a diameter of 16 mm.\nThe patient tolerated all procedures well and was discharged with normal sinus rhythm after recovery from temporary sinus bradycardia. Anticoagulation therapy with oral warfarin was postoperatively prescribed for 3 months, which is standard procedure after bioprosthetic valve replacement. Postoperative echocardiography showed flow from RUPV to the LA through the new ASD without a significant pressure gradient or congestion. Postoperative contrast-enhanced CT showed that the RUPV flowed into the LA through the new pathway without intracardiovascular thrombus (Fig. ). The patient remains free of dyspnea, arrhythmia, and thrombotic events, and echocardiography at 4-year follow-up did not reveal evidence of stenosis or obstruction of the reconstructed pathway.
A 66 year old man with underlying chronic kidney disease, presumed secondary to hypertension, presented to the emergency room with one-week history of weakness, fatigue and poor oral intake. The patient was hypotensive (systolic blood pressure of 60 mmHg) and had severe azotemia (serum creatinine of 4.9 mg/dl form baseline of 1.7 mg/dl). Septic shock, methicillin-sensitive Staphylococcus aureus bacteremia and oliguric acute kidney injury were diagnosed. The patient was adequately resuscitated with intravenous fluids, vasopressors and broad-spectrum antibiotics, later narrowed to cefazolin. The source of infection was unclear after broad investigation of body fluids and imaging studies. Despite an initial transient clinical improvement, the patient remained in shock with high vasopressor requirements, became anuric and developed acute mental status changes. As a consequence, the decision to start the patient on CRRT was made. We inserted a 13.5 French diameter temporary dual-lumen Quinton-Mahurkar catheter (Covidien, Inc.) into the left IJV under US guidance. Although the right IJ is preferred, the intensive care unit team had already inserted a triple-lumen central venous catheter in this site for hemodynamic monitoring and fluid and medication administration. The initial puncture with an 18-gauge needle into the left IJV using real-time US guidance was successful with non-pulsatile back-flow and venous (dark red) blood appearance. Subsequently, after removal of the ultrasound transducer, the rest of the procedure was performed without any resistance to advancing the guidewire and catheter by the Seldinger technique []. Good flow of dark red blood was obtained from both catheter lumens. However, the routine post-cannulation chest X-ray (Figure ) showed that the tip of the catheter was not crossing the midline to the right side and the tip appeared to be lying over the aortic arch suggesting inadvertent carotid artery cannulation. We then obtained blood gas analysis from the catheter which showed: pH of 7.34, partial pressure of oxygen (PO2) of 140 mmHg and partial pressure of carbon dioxide (PCO2) of 22 mmHg, also suggestive of arterial cannulation. The vascular surgery team was emergently consulted. A pressure transducer was attached to the catheter and showed venous wave forms. To definitively determine the location of the catheter, a Computed Tomography Angiography (CTA) of the head and neck was obtained. The CTA showed the Quinton-Mahurkar catheter extending into the left IJV and subsequently descending to terminate in an anomalous left upper lobe pulmonary vein, just inferior to its anomalous insertion in the left brachiocephalic (innominate) vein (Figures A and B). Interestingly, manipulation of the catheter under fluoroscopy by interventional radiology showed that the left brachiocephalic (innominate) vein failed to cross the midline to empty into the right atrium, confirming the venous anomaly. The catheter was therefore removed and a new similar catheter was placed in the right IJV. CRRT was then initiated and continued uneventfully until the patient’s critical condition improved.