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 56-year-old woman with a medical history of multiple sclerosis, quadriplegia, sacral decubitus ulcer, chronic osteomyelitis, chronic deep vein thrombosis (DVT), chronic steroids use, and chronic Foley catheter use presented to the emergency department with severe sepsis due to multiple potential sources including urinary tract infection, soft tissue infection, or osteomyelitis. Vital signs were significant for blood pressure of 85/59 mmHg, heart rate of 63 beats per minute, respiratory rate of 33 breaths per minute, and oxygen saturation of 83% on room air. At the beginning, patient declined intubation so she was placed on 100% oxygen via nonrebreather mask. Arterial blood gas (ABG) on 100% oxygen was 7.29/49/55. Initial chest X-ray (CXR) showed small bilateral pleural effusions with suspected left lower lobe infiltrate. Despite intravenous (IV) fluid resuscitation, broad spectrum IV antibiotics, and stress dose steroids, patient became more hypotensive and IV norepinephrine was started through a right femoral CVL that was placed in the emergency department. Patient was started on heparin drip for suspected pulmonary embolism that was stopped later due to large bleeding from femoral line site and consideration of pneumonia as the cause of hypoxemia. Patient agreed later to be intubated and decision was made to remove femoral line and place a 7.0 Fr triple-lumen CVL through the left internal jugular vein (IJ) over a guidewire and under full ultrasound (U/S) guidance, which was placed easily with no complications. A CXR was obtained to confirm placement which showed that the catheter tip did not cross the midline; instead, it was extending towards the left of the aortic arch probably into an anomalous vein as read by the radiologists (). Ultrasound examination of the neck showed proper catheter placement in the left IJ. A pressure transducer was connected which showed a venous wave form with a pressure of 10 mmHg. ABG obtained from the catheter on continuous mechanical ventilation (FiO2 = 50%, positive end expiratory pressure = 5 CM H2O) showed PaO2 of 102 mmHg compared to 79 mmHg from radial arterial site. A transthoracic echocardiography, which was done to evaluate cardiac function, did not show evidence of atrial septal defect or any other cardiac malformation. Fluoroscopy study was used to place a left peripherally inserted central catheter (PICC) and to confirm the location of the left IJ CVL which demonstrated contrast injection in an anomalous pulmonary vein (). We concluded that the patient has partial anomalous pulmonary venous return (PAPVR) and the left IJ CVL was removed. The PICC line was used instead for central access.