A 64-year-old man, weighing 47 kg and having a height of 154 cm, was scheduled for a pericardiectomy for treatment of constrictive pericarditis. When he was hospitalized with a two-week history of aggravating dyspnea, he had been placed on outpatient follow-up for pulmonary tuberculosis, previously diagnosed with dyspnea on exertion and pleural effusion. His past medical history included diabetes mellitus diagnosed 20 years ago and stent insertion in the left anterior descending (LAD) coronary artery eight years ago. Preoperative ECG revealed left atrial enlargement, and ST depression and flat T-wave on lead II, III and V4-6. Chest radiograph showed pulmonary edema and bilateral pleural effusion. Coronary computerized tomography demonstrated that the LAD stent was intact, but there was >50% narrowing of the left circumflex coronary artery, and pericardial thickening with adhesion to the right atrial and left ventricular lateral walls. Echocardiography showed a left ventricular ejection fraction of 73%, and mild pulmonary hypertension with a systolic right ventricular pressure of 38 mmHg.\nAn hour before surgery, premedication was done with IM morphine 3 mg. On arrival, continuous monitoring of ECG lead II and V5 was initiated, and the radial artery cannulation was done under local anesthesia for pressure monitoring. Vital signs before induction of anesthesia were HR 85/min, ABP 108/68 mmHg, and SpO2 98%. Anesthesia was induced with IV midazolam 3 mg and ketamine 20 mg. Rocuronium 50 mg was administered to facilitate endobronchial intubation with a 35 Fr left-sided double-lumen tube. After intubation, lungs were ventilated with a tidal volume of 8-10 ml/kg, adjusted to maintain end-tidal CO2 within 33-38 mmHg. Anesthesia was maintained with 1.0-1.5% sevoflurane in 60% oxygen in air and continuous infusion of remifentanil at 0.1-0.3µg/kg/min.\nAfter induction of anesthesia, the patient was turned to the head-down position with the head rotated leftward by approximately 60° to facilitate internal jugular catheterization. An 18-G puncture needle was inserted at the conjoined site of both bellies of the SCM, and was advanced toward the ipsilateral nipple until free regurgitation of venous, non-pulsatile blood flow was observed. During the first attempt, the puncture needle had to be withdrawn, as the J-tipped guide wire could be advanced <10 cm. After 5 min of manual compression, jugular venous puncture was retried in the same manner, and non-pulsatile, free-regurgitant blood flow was again obtained. In the second attempt, the J-tipped guide wire was inserted into the vessel without significant resistance, even though it was initially difficult to advance the wire and the puncture needle had to be withdrawn a few millimeters to obtain free regurgitant blood flow. Then, a 7-Fr double-lumen central venous catheter (Arrow Gard Blue, Arrow International, Inc., USA) was inserted using the Seldinger method and was suture-fixed at 16 cm in the skin after confirming free inflow of fluid. At the end of procedure, the guide wire withdrawn showed no deformations. Central venous pressure (CVP) was measured to be 20 mmHg with the patient in a supine position and thereafter was maintained within 30-40 mmHg in the right semi-lateral decubitus position at approximately 30° during one-lung ventilation for surgery. Such a high CVP was considered to be related to the constrictive pericarditis, as the pressure measurement was less than the mean arterial pressure of the radial artery (80-85 mmHg) and the waveform was flat without pulsations, being affected by right-side one-ling ventilation and surgical manipulation of the heart. During the catheterization, vital signs were stable with HR 83/min, BP 90-100/65-68 mmHg, and SpO2 99-100%. Arterial blood gas analysis was done after induction of anesthesia and revealed pH 7.486, PaO2 331 mmHg, and PaCO2 36.2 mmHg with FiO2 0.6.\nApproximately 50 minutes after the surgery began, the pressure waveform from the central venous catheter changed abruptly into what was seemingly an arterial waveform, with a mean pressure of 61 mmHg, almost same to the mean arterial pressure of 62 mmHg (). Simultaneously, blood regurgitated through the fluid line connected to the central venous catheter. Even though about 300 ml of fluid had already been administered, no hematoma or edema was shown in the puncture site. Arterial blood gas measurement was done on blood withdrawn from the central venous catheter, indicating a PaO2 of 438.7 mmHg with a FiO2 of 0.6, confirming that it was arterial blood. Furthermore, the catheter was apparent in the aortic arch on TEE (). Fluid infusion was immediately stopped, and the catheter was removed when the surgery was completed. After confirming that there was no bleeding or developing hematoma in the puncture site with >15 min of compression, the double-lumen tube was replaced with a single-lumen endotracheal tube. Thereafter, the patient was transferred to the intensive care unit with the endotracheal tube in situ. Extubation was done after seven days of mechanical ventilatory care for tachypnea and CO2 retention, and the patient was transferred to the general ward without central venous catheter-related complications on the 10th postoperative day.
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.