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.
This case is being reported after obtaining written consent from the patient.\nA 38-year-old woman was admitted with per rectal bleeding and abdominal pain. She had a history of Crohn's disease. She had undergone four previous bowel resections during the last 15 years. Total parenteral nutrition was planned through a CV catheter, and a referral was made to the on-call anesthesia resident for gaining CV access. Preprocedural examination showed normal vital signs. The patient's coagulation profile was normal. Patient had been receiving azathioprine, zopiclone, tramadol, and amitriptyline. She had history of the previous catheterization of both the right and left internal jugular veins during the last 5 years.\nWith noninvasive monitoring of blood pressure and pulse oximetry established, right IJV catheterization was attempted in the High Dependency Unit (HDU) using a triple lumen catheter (16 cm 7.5 Fr Multicath 3 L Expert/VYGON GmbH and Co., Germany). Due to technical reasons, ultrasound (US) could not be used, and the right IJV was accessed by the standard landmark approach at the apex of two heads of sternomastoids using the Seldinger's technique. The right IJV was first accessed with a 22G finder needle. The vein was then cannulated with the 70 mm larger needle and nonpulsatile dark blood was aspirated freely. The J-tip guide-wire was then passed successfully through the needle, and the dilator was advanced without any resistance over the guide-wire and was then removed. The catheter was threaded over the guide-wire up to the 7 cm mark, when some resistance was encountered. Further attempts to thread the catheter were abandoned as aspiration revealed bright red and pulsating blood giving rise to suspicions of an arterial placement. Immediately, the catheter along with the guide-wire was removed, and firm pressure applied over the site. 4 h later, IJV cannulation was performed on the same side by identifying the IJV with a finder needle and then successfully inserting the catheter without any resistance. Dark venous blood was aspirated freely from all the three ports of the catheter. A chest X-ray was taken, and satisfactory position of the catheter tip was confirmed. 2 h later, the patient woke up from sleep complaining of pain from the right shoulder to the elbow and also a dead feeling in the right arm. Physical examination revealed a cold and tender right arm, with a delayed capillary refill and absent radial, brachial and axillary pulses on the right side. Doppler signals were also negative for axillary, brachial and radial pulsations on this side. The radial and brachial pulsations on the other side were normal. A partial or complete arterial occlusion due to spasm or thrombosis was suspected, and the patient was transferred to the regional vascular center for further management. At the regional center, the IJV catheter was removed and anticoagulation by low molecular weight heparin was commenced. Ascending aortography with selective catheterization of the brachiocephalic trunk was performed subsequently, which confirmed an intraluminal thrombus in the proximal right SCA []. The patient was started on a heparin infusion. Thrombolysis was not thought to be appropriate in view of the risk of bleeding at the CV access site, active rectal bleeding and Crohn's disease. Subsequently, right SCA thrombectomy with graft was performed under general anesthesia. A Fogarty catheter was passed easily, and a large volume of clot extracted. Postoperatively positive Doppler signals for arterial flow were obtained. She was transferred to the ward after 3 days of monitoring in the intensive care unit and was discharged home a week after surgery.