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 65-yr-old woman with an underlying chronic kidney disease was presented to the emergency room (ER) with uremia because of sepsis accompanied by oliguria and severe metabolic acidosis on August 29, 2008. To begin emergency HD, we attempted to insert a 7.5 French (Fr) catheter into the right IJV under ultrasound guidance. The initial puncture with an 18-gauge needle into the right IJV using real-time US guidance by the short-axis method was successful with no pulsatile arterial back flow. Thereafter, the rest of the procedure with removal of the ultrasound transducer was performed without any resistance to advancing the guide wire and HD catheter by the Seldinger technique. No immediate complications such as rapidly expanding hematoma, pain, dyspnea, shock, or signs of neurologic deficits were noticed during insertion of the THC. However, a routine post-cannulation chest X-ray suggested that the catheter tip had been misplaced into the right subclavian artery (SCA) (), and subsequent computed tomographic (CT) angiography with contrast enhancement revealed that the catheter had indeed passed through the right IJV and been misplaced into the right SCA (). We assumed that the puncture needle had initially been inserted into the right IJV, but had been unintentionally advanced into the right SCA after the absence of arterial back flow had been established and before inserting the guide wire.\nThe HD catheter into the right SCA was left in place without an immediate catheter removal and direct external compression, because of the uncontrollable arterial bleeding that might result from application of the pull/pressure technique. Following consultation with a vascular surgeon, the surgical exploration in the operating room revealed that the HD catheter had transected the right IJV and the right SCA. The catheter was removed and the SCA injury was surgically repaired without complications. An angiogram under real-time fluoroscopy revealed no further bleeding or fistula between right IJV and right SCA (). Also, subsequent evaluation of neurological status following open surgical repair and angiography for any intra-arterial catheter-related thrombi or emboli, revealed no neurologic deficit. The patient underwent an uneventful hospital course with antibiotic therapy and intermittent hemodialysis through the THC into the right femoral vein, as required. HD was stopped on the 12th hospital day.