A 57-year-old Caucasian woman was recently diagnosed with advanced stage ovarian cancer. Otherwise healthy, she had initially consulted her general practitioner simply for abdominal distension. Further investigations [abdominal computed tomography (CT), serum CA125determination and laparoscopy] revealed a peritoneal carcinomatosis. The diagnosis of stage III (FIGO classification) ovarian cancer was established and the patient received a carboplatin-paclitaxel based chemotherapy regimen in a neoadjuvant setting. The indication of a debulking surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) was retained by our institutional tumor board. A CVL was inserted via the right jugular vein into the superior vena cava in prevision of surgery and the appropriate positioning of the catheter was verified by chest x-ray. An implantable central venous catheter (Port-a-Cath) was already in place (Fig. ).\nThe first 48 postoperative hours were marked by difficult pain management, hypotension, and transient hyperlactatemia responding to fluid replacement and norepinephrine. On postoperative day 3, she presented acute onset dyspnea when transferred from the bed to a chair, and a major drop in pulse oxygen saturation (from SpO2 96% to 83%) justified the administration of oxygen (5 L/min) via a nasal cannula. On physical examination, her body temperature was 37.4 °C, blood pressure 135/81 mmHg, heart rate 122 beats/min, respiratory rate 20/min. No chest pain was reported. Pulmonary examination revealed a bilateral reduction of basal breath sounds with dullness at percussion. Chest x-ray examination (Fig. ) showed bilateral pleural effusions that could largely explain patient’s dyspnea and oxygen desaturation. After switching from a nasal cannula to a nonrebreather mask (FiO2 0.40), the patient’s condition seemed to stabilize in supine position. A measurement of the arteriovenous oxygen difference was obtained via sampling through the arterial and central venous line after the patient was again lying in supine position (Table ).\nA sampling error or a wrong positioning of the recent CVL was suspected, but a sample taken from the Port-a-Cath confirmed the venous value. A transthoracic echocardiography (TTE) with bubble test failed to show a right-to-left shunt, atrial septum was intact. No pulmonary hypertension was detected and right ventricle was not dilated. A CT pulmonary angiography (Fig. ) showed large bilateral pleural effusions with atelectasis of both lower lobes, acute pulmonary embolism in the right middle and upper lobe pulmonary arteries, and ultimately an anatomical variant of the left upper pulmonary vein draining into the left innominate vein. Following bilateral chest drainage, the patient was treated with low molecular weight heparin twice daily. There was no evidence for deep venous thrombosis. This was followed by a significant clinical improvement, a disappearance of the platypnea–orthodeoxia complaints and a reduction of oxygen requirement over the following days.\nAt 1-year follow-up, TTE showed no right ventricular dilation and absence of pulmonary hypertension. From an oncological perspective, stability was also observed during her niraparib maintenance therapy, with absence of new lesions at a 1-year follow-up abdominal CT.
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.