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Superior vena cava syndrome-induced hemoptysis

Published:September 21, 2022DOI:https://doi.org/10.1016/j.amjms.2022.07.021

      Abstract

      Superior vena cava (SVC) syndrome resulting from obstruction of the blood flow to the superior vena cava is rarely reported to present with life-threatening hemoptysis. The pathogenesis and the underlying mechanism are still not well described in the literature. We report a unique case of a 27-year-old man known to have end-stage kidney disease (ESKD) on hemodialysis that presented with shortness of breath and life-threatening hemoptysis that developed during the dialysis session. Computerized tomography with contrast (CTPA) confirmed the presence of a large, calcified thrombus within the SVC along with the formation of multiple collaterals which was diagnostic for SVC syndrome. Attempts for revascularization and stenting failed, and the patient had a prolonged and stormy course while admitted, including difficult alternative dialysis access that unfortunately resulted in death eventually. Here we are highlighting the importance of recognition of hemoptysis as a presentation of SVC syndrome by explaining the underlying pathogenesis and possible management options.

      Introduction

      Superior vena cava (SVC) syndrome is defined as a group of signs and symptoms caused by blood flow obstruction through the super vena cava.
      • Seligson MT
      • Surowiec SM.
      Superior Vena Cava Syndrome. [Updated 2020 Jul 13].
      SVC syndrome has been classically described as an oncological emergency, however, benign causes such as indwelling intravenous dialysis catheters have been evolving in recent years.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      ,
      • De Potter B
      • Huyskens J
      • Hiddinga B
      • et al.
      Imaging of urgencies and emergencies in the lung cancer patient.
      Hemoptysis is a rare presentation of SVC syndrome that is still not quite well described in the literature.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      In this article, we report a rare, and rather complicated case of hemoptysis caused by SVC obstruction in a young patient with end-stage kidney disease (ESKD) on intermittent hemodialysis. We are highlighting the presentation, pathogenesis, investigations, and further management of hemoptysis in a patient with SVC syndrome along with a concise review of the literature.

      Case presentation

      A 27-year-old man, known to have type 2 diabetes mellitus, on insulin and with end-stage kidney disease (ESKD) secondary to chronic vesicoureteral reflux and obstructive uropathy due to neurogenic bladder resulting from spina bifida since childhood. He had been on dialysis since the age of 12 years. He had thrice-weekly intermittent dialysis sessions through a left side brachiocephalic arteriovenous fistula (AVF), but he had a previous history of multiple failed temporary veinous accesses complicated by central venous thromboses including right side femoral vein access, left side femoral vein access and left and right internal jugular veins accesses.
      The patient was referred to our tertiary hospital from the local dialysis unit with heavy repetitive episodes of hemoptysis that started after completing one hour of the duration of the dialysis session. The patient had a history of chronic swelling of the face and neck associated with a headache for a few months that was not investigated before. No previous history of hemoptysis or hematemesis was noted; however, the patient had a chronic history of cough and progressive shortness of breath. No other history of visible dilated veins. At the initial presentation to the emergency department, the patient had a blood pressure of 112/80 mmHg that dropped later to 89/45, heart rate of 130 bpm, SPO2 100% in room air, however, he was in distress not able to maintain his airways thus he was sedated with fentanyl and midazolam, intubated, and ventilated with PSMV with FIO2 of 0.5 and Positive end-expiratory pressure (PEEP) of 6 mmHg. After stabilization, the initial assessment showed facial and neck swelling with prominent dilatation of veins, and upper limbs edema. The other systemic examination was unremarkable.
      Further investigations showed hemoglobin of 12.2 g/dL, platelets of 236 × 1012, white blood cells of 5.7 × 1012. The coagulation profile showed an international randomized ratio (INR) of 1.04 and Activated Partial Thromboplastin Time (APTT) of 29.8 seconds. Renal function test showed urea of 16.3 and creatinine of 749, no electrolytes imbalances. Liver function test showed ALT of 41 and bilirubin of 4. Chest x-ray showed bilateral diffuse tiny nodular opacities in both lung fields more on the left, features suggestive of alveolar hemorrhage. After stabilization and admission to the intensive care unit, a CT scan with IV contrast done showed no features of pulmonary embolism (Fig. 1), and bilateral multi-lobar ground-glass opacities, most likely to represent alveolar hemorrhage, no active extravasation of the contrast seen, superior vena cava obstructed with reflux of contrast into the inferior vena cava and hepatic veins which were dilated and torturous extending to the sub-capsular area of the liver and the chest wall vessels (Fig. 2). Bilateral subcostal and posterior chest wall veins along with azygous veins were dilated as well (Fig. 3). Features were suggestive of longstanding chronic SVC obstruction. Echocardiography (ECHO) showed normal left and right ventricular size, systolic and diastolic function, ejection fracture estimated to be >60%, and no significant valvopathy. Calcific mass was seen at the inlet of SVC with color turbulence seen in it.
      Fig 1:
      Fig. 1CT pulmonary angiography (CTPA) showed no pulmonary embolism.
      Fig 2:
      Fig. 2CT pulmonary angiography (CTPA) showed a long segment of chronic superior vena cava (SVC) occlusion and multiple large intercostal collateral veins.
      Fig 3:
      Fig. 3Multiple large intercostal collateral veins on the right side.
      After a multidisciplinary team discussion with surgical services, the patient was determined to be not fit for surgery initially due to hemodynamic instability. He continued to improve and the bleeding from the endotracheal (ET) tube eventually subsided after reducing the rate of the dialysis through the AVF to 150ml/min. He was extubated and remained hemodynamically well, however during a subsequent session of dialysis the patient developed another episode of massive hemoptysis that required re-intubation after two days. He underwent venography and attempt of recanalization and possible embolization through interventional radiology with access from the right internal jugular vein that showed complete occlusion of the SVC and dilated large azygous vein (Fig. 4), several attempts to cross the lesions were failed. Attempts for access from the left and right femoral and brachial veins showed that all lower deep and central veins were occluded as well (Fig. 5, Fig. 6, Fig. 7). Rheumatological workup such as antinuclear antibodies (ANA), Anti-neutrophilic cytoplasmic autoantibodies (ANCA) and Anti-Double Stranded DNA (Anti-DsDNA), bleeding disorders workup and tuberculosis test as a cause of hemoptysis resulted negative. He required a total of 4 packed red blood cell transfusions over the admission.
      Fig 4:
      Fig. 4Central venogram from jugular vein access showed complete occlusion of the SVC and a large azygos vein.
      Fig 5:
      Fig. 5Central venogram from right brachial vein access showed complete occlusion of right central veins.
      Fig 6:
      Fig. 6Central venogram from left brachial vein access showed complete occlusion of right central veins.
      Fig 7:
      Fig. 7Central venogram from femoral vein access showed complete occlusion of lower deep and central venous systems.
      The patient had continuous minimal bleeding from the ET tube despite trails of furosemide and hydrocortisone. The bleeding eventually responded well to a reduction of dialysis blood flow to 150ml/hr. The major obstacle was decreasing the venous hypertension of the upper limb by changing the course of the dialysis to a circulation that is drained by the IVC however all central veins were occluded and no further veinous access for dialysis could be obtained. Thus, the patient decided to be started on acute peritoneal dialysis instead. However, he developed gram-positive cocci bacteremia from peripheral and central blood cultures, which were identified after death as Enterococci fascium sensitive to ampicillin, along with ventilator-associated pneumonia with Klebsiella pneumonia sensitive to co-trimoxazole, gentamycin, and ciprofloxacin and multi-drug resistant Acinetobacter baumannii. The patient remained hypotensive regardless of noradrenalin of 1 mcg/kg/min and adrenalin 0.5 mcg/kg/min, he developed massive bleeding from the ET tube, the lungs were very stiff to ventilate despite changing the ET tube. He continued to have poor compliance, the low tidal volume was <100 ml and he was desaturating with respiratory acidosis, then arrested and passed away.

      Discussion

      The superior vena cava (SVC) is a 6-8 cm long central vein that is formed by the joining of the left and right brachiocephalic veins that drain the upper parts of the body, including the head and neck along with upper limbs.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      If obstructed, several veins act as a diversion to bypass the obstruction and drain the upper body venous blood. The azygous vein connects the superior vena cava with the inferior vena cava (IVC) by passing anteriorly to the vertebral bodies.
      • Godwin JD
      • Chen JT.
      Thoracic venous anatomy.
      Both the azygous and hemiazygos systems drain veins from the right and left lower bronchia, respectively. Blockage within the SVC can lead to dilatation of the azygous and hemiazygos due to venous hypertension that eventually leads to the engorgement of the other veins drained by these major vessels.
      • Baile EM.
      The anatomy and physiology of the bronchial circulation.
      In case of chronic obstruction or blockage of both SVC and the azygous vein, reversal of blood flow occurs from the brachiocephalic vein through the internal thoracic vein to the inferior epigastric vein that drains to the external iliac then to IVC.
      • Lochridge SK
      • Knibbe WP
      • Doty DB.
      Obstruction of the superior vena cava.
      The lateral thoracic veins also can drain blood to the thoraco-epigastric veins that drain to the femoral veins.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      ,
      • Godwin JD
      • Chen JT.
      Thoracic venous anatomy.
      In 1757, William Hunter described SVC obstruction caused by syphilitic aortic aneurysm for the first time.
      • Higdon ML
      • Higdon JA.
      Treatment of oncologic emergencies.
      SVC syndrome is defined as a group of signs and symptoms caused by partial or complete obstruction of the blood flow in the SVC and its branches.
      • Seligson MT
      • Surowiec SM.
      Superior Vena Cava Syndrome. [Updated 2020 Jul 13].
      Classically, SVC syndrome has been described as an oncological emergency as malignant tumors are known to cause about 90% of all cases, where 95% of them are caused by lung cancer or lymphoma.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      However, benign causes are growing recently, and it has been associated with 1:650 to 1:3100 cases of SVC syndrome,
      • De Potter B
      • Huyskens J
      • Hiddinga B
      • et al.
      Imaging of urgencies and emergencies in the lung cancer patient.
      ,
      • Friedman T
      • Quencer KB
      • Kishore SA
      • et al.
      Malignant venous obstruction: superior vena cava syndrome and beyond.
      perhaps due to the increasing number of patients with end-stage kidney disease (ESKD) on hemodialysis. Superior vena cava syndrome caused by indwelling dialysis venous catheters has become more recognized. Qanadli and colleagues studied benign causes of SVC obstructions and found that 67% of all patients had obstructions caused by intravenous dialysis catheters. However, none of them had hemoptysis on initial presentation like our patient.
      • Qanadli SD
      • El Hajjam M
      • Mignon F
      • et al.
      Subacute and chronic benign superior vena cava obstructions: endovascular treatment with self-expanding metallic stents.
      Other intravenous foreign bodies, such as pacemakers and defibrillator wires, can also predispose patients to SVC syndrome.
      • Cohen R
      • Mena D
      • Carbajal-Mendoza R
      • et al.
      Superior vena cava syndrome: a medical emergency?.
      Extracorporeal membrane oxygenation (ECMO) catheters have been reported by Zreik and colleagues to cause SVC obstruction in 7 of 60 neonates with ECMO. Other benign causes may include infections such as tuberculosis or syphilitic aortic aneurysm.
      • Cohen R
      • Mena D
      • Carbajal-Mendoza R
      • et al.
      Superior vena cava syndrome: a medical emergency?.
      In our case, the patient had multiple insertions of dialysis catheters in the past, making it the most likely cause of his presentation, which was also supported by the presence of multiple lower limb thrombosed central veins.
      SVC syndrome can be caused generally by external compression by tumors in malignant causes or intraluminal obstruction by thrombosis, which is the most common process in benign processes. Qanadli et al have proposed multiple factors involving the development of thromboses in the SVC in patients with indwelling venous catheters including repetitive mechanical traumas to the vessel walls by the foreign objects or chemical trauma caused by the turbulence of blood flow and the material infused through these catheters or the mechanical compression exerted on the blood vessel by the venous catheter.
      • Qanadli SD
      • El Hajjam M
      • Mignon F
      • et al.
      Subacute and chronic benign superior vena cava obstructions: endovascular treatment with self-expanding metallic stents.
      The venous system having lower pressure leaves it more vulnerable to the impairment of the blood flow and damage to the thin walls, in which integrity can be disturbed easily.
      • Kalinin RE
      • Suchkov IA
      • Shitov II
      • et al.
      Venous thromboembolic complications in patients with cardiovascular implantable electronic devices.
      ,
      • Ghorbani H
      • Vakili Sadeghi M
      • Hejazian T
      • Sharbatdaran M
      Superior vena cava syndrome as a paraneoplastic manifestation of soft tissue sarcoma.
      ,
      • Labriola L
      • Seront B
      • Crott R
      • et al.
      Superior vena cava stenosis in haemodialysis patients with a tunnelled cuffed catheter: prevalence and risk factors.
      Chronic obstructions are more tolerated usually due to the gradual onset of the symptoms due to the formation of collaterals compared to the more abrupt onset in acute cases.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      The SVC obstruction causes venous hypertension, and eventually, reversal of blood flow through the collaterals.
      • Lochridge SK
      • Knibbe WP
      • Doty DB.
      Obstruction of the superior vena cava.
      A previous review that analyzed the presentations of SVC syndrome found that the most common presentation was neck swelling in almost all cases reported. Dyspnea was the second most common presentation in about 83% of all patients.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      This can be explained by various mechanisms including a reduction in the venous return to the heart, and venous hypertension impairs the lymphatic drainage of the lungs which can lead to increased incidence of pleural effusions.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      A previously reported case of SVC syndrome presented with shortness of breath and found to have a pulmonary embolism (PE) rises the need to suspect PE as a cause of dyspnea in these patients.
      • Chin C-G
      • Yeh J-S
      • Lin Y-K
      • et al.
      Superior vena cava syndrome complicated with acute pulmonary thromboembolism in a patient with lung cancer.
      Other presentations might include upper limb swelling (38-75%), facial swelling (48-82%), cough (22-58%), dilated chest wall veins in 38% of cases, and in more rare occasions weight loss, phrenic nerve palsy, plethora, and dysphagia.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      Cyanosis has been also reported as a presentation of SVC syndrome which highlights the diversity of the presentation that patients can present with.
      • Rafiq I
      • Morgan G
      • Silva Vieira M
      • et al.
      Chronic occlusion of the superior vena cava resulting in cyanosis in an adult: unusual case highlighting the value of collaboration between adult and congenital cardiology services.
      Venous hypertension can lead to dilatation of the azygous, brachiocephalic, and subclavian veins along with the subsequent territories supplied. The proximal and middle esophagus is drained by those systems; thus, SVC syndrome has been reported to be associated with upper gastrointestinal bleeding from proximal (downhill) esophageal varices in a similar fashion with portal hypertension.
      • Pillai U
      • Roopkiranjot K
      • Lakshminarayan N
      • Balabhadrapatruni K
      • Gebregeorgis W
      • Kissner P.
      Downhill varices secondary to HeRO graft-related SVC syndrome.
      The distal part of the esophagus is drained by the splanchnic circulation, thus distal (uphill) esophageal varices are usually caused by portal hypertension.
      • Gopaluni S
      • Warwicker P.
      Superior vena cava obstruction presenting with epistaxis, haemoptysis and gastro-intestinal haemorrhage in two men receiving haemodialysis with central venous catheters: two case reports.
      The first case of proximal variceal bleeding has been described in 1964 by Felson and Lessure.
      • Froilán C
      • Adán L
      • Suárez JM
      • et al.
      Therapeutic approach to “downhill” varices bleeding.
      In a previous non-bleeding screen of patients with SVC obstruction caused by benign pathologies, 30% of patients had proximal esophageal varices.
      • Siegel Y
      • Schallert E
      • Kuker R.
      Downhill esophageal varices: a prevalent complication of superior vena cava obstruction from benign and malignant causes.
      Hemoptysis in patients with SVC has been rarely described before in the literature. Nguyen et al reported a case of SVC syndrome with hemoptysis and dependent periorbital edema, however, unlike our patient the obstruction was caused by malignancy.
      • Nguyen AL
      • Belderbos H
      • van Harten JG
      • et al.
      Superior vena cava syndrome presenting as position-dependent periorbital oedema.
      In an analysis of 12 SVC syndrome cases caused by benign pathologies, none of them reported hemoptysis.
      • Qanadli SD
      • El Hajjam M
      • Mignon F
      • et al.
      Subacute and chronic benign superior vena cava obstructions: endovascular treatment with self-expanding metallic stents.
      Gopaluni and Warwicker reported a case of benign SVC obstruction with hemoptysis in an ESKD patient with a dialysis catheter in the internal jugular vein, similar to our patient the patient died from the complications, which brings into question the mortality of this patient group.
      • Gopaluni S
      • Warwicker P.
      Superior vena cava obstruction presenting with epistaxis, haemoptysis and gastro-intestinal haemorrhage in two men receiving haemodialysis with central venous catheters: two case reports.
      The exact mechanism of the hemoptysis has not been previously well described, however, Tannu and colleagues proposed a similar mechanism in patients with mitral stenosis where they documented to have bronchial varices.
      FELSON B, LESSUREAP
      “Downhill” varices of the esophagus.
      ,
      • Tannu M
      • Greer M
      • Rim J
      • et al.
      Hemoptysis secondary to superior vena cava syndrome.
      In mitral stenosis, the back pressure and the increase in the left atrial pressure can lead to bronchial varices that can easily bleed if irritated. As described before, part of the bronchial veins can be drained by branches of the azygous and hemiazygos veins, that if dilated can potentially lead to similar bronchial varices. Unfortunately, these types of patients face a major obstacle if intubated, as the high Positive End Expiratory Pressure (PEEP) can cause a reduction in the venous return which is already compromised by the SVC obstruction and exaggerate the hemodynamic instability.
      • Schwartz R
      • Myerson RM
      • Lawrence T
      • et al.
      Mitral stenosis, massive pulmonary hemorrhage, and emergency valve replacement.
      Conventional x-ray radiographs can give limited insight into the underlying vascular pathology. However, it has been stated before that patients with SVC obstruction could have findings of enlarged right hilum and pleural effusion.
      • Nguyen AL
      • Belderbos H
      • van Harten JG
      • et al.
      Superior vena cava syndrome presenting as position-dependent periorbital oedema.
      Computerized tomography (CT) findings of filling defect of the superior vena cava and presence of collateral have shown a sensitivity of 96% and specificity of 92%.
      • Labriola L
      • Seront B
      • Crott R
      • et al.
      Superior vena cava stenosis in haemodialysis patients with a tunnelled cuffed catheter: prevalence and risk factors.
      ,
      • Kim HJ
      • Kim HS
      • Chung SH.
      CT diagnosis of superior vena cava syndrome: importance of collateral vessels.
      Contrast magnetic resonance imaging (MRI) has shown an outstanding specificity and sensitivity of almost 100%.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      In a study by Casullo and Belley, they analyzed 16 SVC syndrome patients diagnosed with CT scans found that 44% of patients have SVC due to dialysis access, about 56% of patients had thromboses in the SVC only, and 25% of patients had SVC obstruction below the azygous veins.
      • Casullo J
      • Belley G.
      CT of the bronchopulmonary veins in patients with superior vena cava or left brachiocephalic vein obstruction.
      A dilated azygous vein is also a known finding in CT scans for patients with SVC obstruction.
      • Cohen R
      • Mena D
      • Carbajal-Mendoza R
      • et al.
      Superior vena cava syndrome: a medical emergency?.
      The obstruction can be classified using the Stanford classifications into four types based on the percentage of blockage and the territories affected. Type I where there is 90% stenosis or more and patent azygous, type II is a near-complete obstruction of the SVC with antegrade flow of blood in the azygous veins, type III is near-complete obstruction of SVC with reversal of blood flow in the azygous vein, and type IV is complete obstruction of the SVC along with its territories.
      • Stanford W
      • Jolles H
      • Ell S
      • et al.
      Superior vena cava obstruction: a venographic classification.
      The treatment of SVC obstruction due to malignant causes is well established compared to the benign causes.
      • Hooker JB
      • Hawkins BM
      • Abu-Fadel MS.
      Endovascular stenting in 2 patients with benign superior vena cava syndrome.
      In benign SVC syndrome, there is no strong evidence to support the use of the steroids, and probably the evidence has been extrapolated from the ability of steroids to reduce edema and swelling, hence reducing the symptoms of the airway and soft tissue edema and provide symptomatic relief.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      In cases of intravenous catheter causing a thrombosis, withdrawal of the provocative agent is recommended initially.
      • Gopaluni S
      • Warwicker P.
      Superior vena cava obstruction presenting with epistaxis, haemoptysis and gastro-intestinal haemorrhage in two men receiving haemodialysis with central venous catheters: two case reports.
      The use of diuretics in SVC syndrome was first tested by Lewis in 1960 in a limited number of patients who showed initial symptomatic relief. Currently, there are no trials that have evaluated the efficacy and no recommendations advocating the use of diuretics in SVC syndrome.
      • Higdon ML
      • Higdon JA.
      Treatment of oncologic emergencies.
      ,
      • Wan JF
      • Bezjak A.
      Superior vena cava syndrome.
      Unfortunately, there are no guidelines that outline the need, duration, and follow-up duration for anticoagulation in such patients.
      • Sfyroeras GS
      • Antonopoulos CN
      • Mantas G
      • et al.
      A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology.
      Unlike benign obstructions, novel anticoagulants (NOACs) have been proven to be as effective as vitamin K antagonists in cancer patients.
      • Lyman GH
      • Bohlke K
      • Khorana AA
      • et al.
      Venous thromboembolism prophylaxis and treatment in patients with cancer: american society of clinical oncology clinical practice guideline update 2014.
      The decision to anticoagulate is quite challenging especially in patients with gastrointestinal bleeding or hemoptysis associated with the obstruction. A previous review that evaluated open versus endovascular treatment of SVC obstructions highlighted the variety of regimens those patients received, which included single antiplatelets, dual antiplatelet alone, dual, or single antiplatelet with anticoagulation and anticoagulation alone, but no comparison between the outcome has been made.
      • Sfyroeras GS
      • Antonopoulos CN
      • Mantas G
      • et al.
      A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology.
      This calls for further evaluation and well-formulated evidence regarding anticoagulation in SVC syndrome.
      Perhaps the first revascularization attempt was performed in the 1980s when successful bypass of the SVC using femoral vein grafts.
      • Alaa M
      • El mezaien M
      • Amin M
      Superior Vena Cava syndrome: a systematic review.
      The first percutaneous balloon angioplasty was performed by Rocchini et al in SVC in a child.
      • Carcao MD
      • Connolly BL
      • Chait P
      • et al.
      Central venous catheter-related thrombosis presenting as superior vena cava syndrome in a haemophilic patient with inhibitors.
      By 1987, an expandable stent was used successfully for the first time to stent SVC obstruction. Currently, some authors suggest the use of a Kishi score of 4 more as an indication for stenting in SVC syndrome.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      Reviews and metanalyses have shown that up to 75% of patients had immediate relief of symptoms post stenting. The recurrence of symptoms happened in 40% of patients with only 3-4% overall mortality.
      • Uberoi R.
      Quality assurance guidelines for superior vena cava stenting in malignant disease.
      ,
      • Rachapalli V
      • Boucher L-M.
      Superior vena cava syndrome: role of the interventionalist.
      However, the hemodynamics including the pulmonary wedge pressure and cardiac output were noted to improve to baseline immediately after the procedure in another study.
      • Yamagami T
      • Nakamura T
      • Kato T
      • et al.
      Hemodynamic changes after self-expandable metallic stent therapy for vena cava syndrome.
      It has been estimated that about 24% is the one-year overall survival post-procedure and the five-year overall survival is 9% for all causes of SVC syndrome.
      • Straka C
      • Ying J
      • Kong F-M
      • et al.
      Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
      Phase III of a recent Japanese clinical trial comparing SVC stenting versus other non-surgical treatments of SVC syndrome found that SVC stenting is superior to any other approaches.
      • Takeuchi Y
      • Arai Y
      • Sone M
      • et al.
      Evaluation of stent placement for vena cava syndrome: phase II trial and phase III randomized controlled trial.
      Analysis of 136 patients with endovascular treatment and 87 patients with surgical management of benign causes of SVC syndrome, the endovascular approach had a 95.9% success rate compared to the 100% success rate of open approaches. Both approaches had no 30-day mortality noted, and the recurrence of symptoms in both endovascular and open approaches was found to be 97.3% and 93.5%, respectively.
      • Sfyroeras GS
      • Antonopoulos CN
      • Mantas G
      • et al.
      A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology.
      It is hypothesized that a compromised circulation at the level of venous systems, capillaries, and arterioles can eventually result in bronchial artery proliferation and hypertrophy in chronic lung diseases, which can lead to hemoptysis. It is estimated that 90% of massive hemoptysis cases, in general, are caused by bronchial artery bleeding. Despite the immediate efficacy of bronchial artery embolization with a success rate between 73-98%, still studies have shown a high risk of rebleeding at longer terms. The evidence of the efficacy of bronchial artery embolization is however less established in SVC obstruction cases.
      • Yoon W
      • Kim J
      • Kim Y
      Bronchial and Nonbronchial Systemic Artery Embolization for Life-threatening Hemoptysis: A Comprehensive Review.
      Endovascular treatment complications occur in approximately 3.6% of patients, which include stent migration, SVC rapture, penetration to the pericardium, puncture site local complications, and to a less extent third-degree heart block.
      • Sfyroeras GS
      • Antonopoulos CN
      • Mantas G
      • et al.
      A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology.
      Acute pulmonary edema and acute onset heart failure have also been recognized before as a complication of revascularization especially in benign chronic obstructions.
      • Dasgupta R
      • Hussein E
      • Shah H
      • et al.
      Erosion of superior vena caval stent into trachea, an unusual cause of hemoptysis.
      In more rare instances, fatal pulmonary embolism after angioplasty and stenting has been described before.
      • Breault S
      • Doenz F
      • Jouannic A-M
      • Qanadli SD.
      Percutaneous endovascular management of chronic superior vena cava syndrome of benign causes : long-term follow-up.
      In our patient, the vascular access for dialysis was a challenge. Dialysis of 200ml/min through the upper limb arteriovenous fistula provoked the hemoptysis episodes since the blood from the dialysis machine was pushed into an already engorged and terminally blocked venous system. Additionally, our patient had all central veins including the lower limbs blocked by thromboses from previous dialysis catheters inserted. In hemodialysis patients, vascular access is a known cause of mortality and morbidity.
      • Windus DW.
      Permanent vascular access: a nephrologist's view.
      Another less recognized dialysis access is the trans-lumbar inferior vena cava (IVC) catheter that was described by Kenny and colleagues.
      • Kenney PR
      • Dorfman GS
      • Denny DFJ.
      Percutaneous inferior vena cava cannulation for long-term parenteral nutrition.
      The trans-lumbar IVC lines have been used before by oncologists and hematologists in bone marrow transplantation, parenteral feeding, and administration of medications for a prolonged duration.
      • Gupta A
      • Karak PK
      • Saddekni S.
      Translumbar inferior vena cava catheter for long-term hemodialysis.
      A retrospective study of 12 patients with trans-lumbar IVC dialysis catheters showed overall average patency was 315.5 catheter-day. Complications were exit site infections in 8.3% of patients and catheter-related bloodstream infection (CRBSI) in 33.3% where 75% of them had staphylococcus species.
      • F Moura
      • Guedes FL
      • Dantas Y
      • et al.
      Translumbar hemodialysis long-term catheters: an alternative for vascular access failure.
      Another retrospective study of 26 patients found that all patients were able to achieve a good flow of more than 300ml/min with no immediate complications. A similar rate of CRBSI was found in this study as well (36%).
      • Liu F
      • Bennett S
      • Arrigain S
      • et al.
      Patency and complications of translumbar dialysis catheters.
      Trans-lumbar IVC dialysis could be an excellent option if expertise is available to perform such procedures.

      Conclusions

      SVC syndrome is a well-recognized oncological emergency, however benign causes such as indwelling intravenous dialysis catheter have been evolving recently. Hemoptysis is one of the least recognized manifestations of SVC obstruction that can be fatal and challenging to treat. Prompt early recognition and effective treatment in such patients groups can prevent further morbidity and hinder mortality. This case report highlighted important valuable lessons.

      References

        • Seligson MT
        • Surowiec SM.
        Superior Vena Cava Syndrome. [Updated 2020 Jul 13].
        StatPearls [Internet]. StatPearls Publishing, Treasure Island (FL)2021 (Jan-. Available from:)
        • Alaa M
        • El mezaien M
        • Amin M
        Superior Vena Cava syndrome: a systematic review.
        J Egypt Soc Cardiothor Surg. 2009; 34
        • De Potter B
        • Huyskens J
        • Hiddinga B
        • et al.
        Imaging of urgencies and emergencies in the lung cancer patient.
        Insights Imaging. 2018; 9: 463-476
        • Straka C
        • Ying J
        • Kong F-M
        • et al.
        Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome.
        Springerplus. 2016; 5: 229
        • Godwin JD
        • Chen JT.
        Thoracic venous anatomy.
        AJR Am J Roentgenol. 1986; 147: 674-684
        • Baile EM.
        The anatomy and physiology of the bronchial circulation.
        J Aerosol Med. 1996; 9: 1-6
        • Lochridge SK
        • Knibbe WP
        • Doty DB.
        Obstruction of the superior vena cava.
        Surgery. 1979; 85: 14-24
        • Higdon ML
        • Higdon JA.
        Treatment of oncologic emergencies.
        Am Fam Physician. 2006; 74: 1873-1880
        • Friedman T
        • Quencer KB
        • Kishore SA
        • et al.
        Malignant venous obstruction: superior vena cava syndrome and beyond.
        Semin Intervent Radiol. 2017 Dec; 34: 398-408
        • Qanadli SD
        • El Hajjam M
        • Mignon F
        • et al.
        Subacute and chronic benign superior vena cava obstructions: endovascular treatment with self-expanding metallic stents.
        AJR Am J Roentgenol. 1999; 173: 159-164
        • Cohen R
        • Mena D
        • Carbajal-Mendoza R
        • et al.
        Superior vena cava syndrome: a medical emergency?.
        Int J Angiol. 2008; 17: 43-46
        • Kalinin RE
        • Suchkov IA
        • Shitov II
        • et al.
        Venous thromboembolic complications in patients with cardiovascular implantable electronic devices.
        Angiol Sosud Khir. 2017; 23: 69-74
        • Ghorbani H
        • Vakili Sadeghi M
        • Hejazian T
        • Sharbatdaran M
        Superior vena cava syndrome as a paraneoplastic manifestation of soft tissue sarcoma.
        Hematol Transfus Cell Ther. 2018; 40: 75-78
        • Labriola L
        • Seront B
        • Crott R
        • et al.
        Superior vena cava stenosis in haemodialysis patients with a tunnelled cuffed catheter: prevalence and risk factors.
        Nephrol Dial Transplant. 2018; 33: 2227-2233
        • Chin C-G
        • Yeh J-S
        • Lin Y-K
        • et al.
        Superior vena cava syndrome complicated with acute pulmonary thromboembolism in a patient with lung cancer.
        J Cardiol Cases. 2018; 17: 9-11
        • Rafiq I
        • Morgan G
        • Silva Vieira M
        • et al.
        Chronic occlusion of the superior vena cava resulting in cyanosis in an adult: unusual case highlighting the value of collaboration between adult and congenital cardiology services.
        Circ Cardiovasc Interv. 2015; 8e002163
        • Pillai U
        • Roopkiranjot K
        • Lakshminarayan N
        • Balabhadrapatruni K
        • Gebregeorgis W
        • Kissner P.
        Downhill varices secondary to HeRO graft-related SVC syndrome.
        Semin Dial. 2013; 26: E47-E49
        • Gopaluni S
        • Warwicker P.
        Superior vena cava obstruction presenting with epistaxis, haemoptysis and gastro-intestinal haemorrhage in two men receiving haemodialysis with central venous catheters: two case reports.
        J Med Case Rep. 2009; 3: 6180
        • Froilán C
        • Adán L
        • Suárez JM
        • et al.
        Therapeutic approach to “downhill” varices bleeding.
        Gastrointest Endosc. 2008; 68: 1010-1012
        • Siegel Y
        • Schallert E
        • Kuker R.
        Downhill esophageal varices: a prevalent complication of superior vena cava obstruction from benign and malignant causes.
        J Comput Assist Tomogr. 2015; 39: 149-152
        • Nguyen AL
        • Belderbos H
        • van Harten JG
        • et al.
        Superior vena cava syndrome presenting as position-dependent periorbital oedema.
        BMJ Case Rep. 2018; 2018
        • FELSON B, LESSUREAP
        “Downhill” varices of the esophagus.
        Dis Chest. 1964; 46: 740-746
        • Tannu M
        • Greer M
        • Rim J
        • et al.
        Hemoptysis secondary to superior vena cava syndrome.
        Chest. 2019; 156: A1431
        • Schwartz R
        • Myerson RM
        • Lawrence T
        • et al.
        Mitral stenosis, massive pulmonary hemorrhage, and emergency valve replacement.
        N Engl J Med. 1966; 275: 755-758
        • Kim HJ
        • Kim HS
        • Chung SH.
        CT diagnosis of superior vena cava syndrome: importance of collateral vessels.
        AJR Am J Roentgenol. 1993; 161: 539-542
        • Casullo J
        • Belley G.
        CT of the bronchopulmonary veins in patients with superior vena cava or left brachiocephalic vein obstruction.
        AJR Am J Roentgenol. 2014; 203: 594-600
        • Stanford W
        • Jolles H
        • Ell S
        • et al.
        Superior vena cava obstruction: a venographic classification.
        AJR Am J Roentgenol. 1987; 148: 259-262
        • Hooker JB
        • Hawkins BM
        • Abu-Fadel MS.
        Endovascular stenting in 2 patients with benign superior vena cava syndrome.
        Tex Heart Inst J. 2018; 45: 264-269
        • Wan JF
        • Bezjak A.
        Superior vena cava syndrome.
        Hematol Oncol Clin North Am. 2010; 24: 501-513
        • Sfyroeras GS
        • Antonopoulos CN
        • Mantas G
        • et al.
        A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology.
        Eur J Vasc Endovasc Surg. 2017; 53: 238-254
        • Lyman GH
        • Bohlke K
        • Khorana AA
        • et al.
        Venous thromboembolism prophylaxis and treatment in patients with cancer: american society of clinical oncology clinical practice guideline update 2014.
        J Clin Oncol. 2015; 33: 654-656
        • Carcao MD
        • Connolly BL
        • Chait P
        • et al.
        Central venous catheter-related thrombosis presenting as superior vena cava syndrome in a haemophilic patient with inhibitors.
        Haemophilia. 2003; 9: 578-583
        • Uberoi R.
        Quality assurance guidelines for superior vena cava stenting in malignant disease.
        Cardiovasc Intervent Radiol. 2006; 29: 319-322
        • Rachapalli V
        • Boucher L-M.
        Superior vena cava syndrome: role of the interventionalist.
        Can Assoc Radiol J. 2014; 65: 168-176
        • Yamagami T
        • Nakamura T
        • Kato T
        • et al.
        Hemodynamic changes after self-expandable metallic stent therapy for vena cava syndrome.
        AJR Am J Roentgenol. 2002; 178: 635-639
        • Takeuchi Y
        • Arai Y
        • Sone M
        • et al.
        Evaluation of stent placement for vena cava syndrome: phase II trial and phase III randomized controlled trial.
        Support Care Cancer. 2019; 27: 1081-1088
        • Yoon W
        • Kim J
        • Kim Y
        Bronchial and Nonbronchial Systemic Artery Embolization for Life-threatening Hemoptysis: A Comprehensive Review.
        Radiographics. 2002; 22: 1395-1409https://doi.org/10.1148/rg.226015180
        • Dasgupta R
        • Hussein E
        • Shah H
        • et al.
        Erosion of superior vena caval stent into trachea, an unusual cause of hemoptysis.
        J Bronchology Interv Pulmonol. 2021; 28: e28-e31
        • Breault S
        • Doenz F
        • Jouannic A-M
        • Qanadli SD.
        Percutaneous endovascular management of chronic superior vena cava syndrome of benign causes : long-term follow-up.
        Eur Radiol. 2017; 27: 97-104
        • Windus DW.
        Permanent vascular access: a nephrologist's view.
        Am J Kidney Dis. 1993; 21: 457-471
        • Kenney PR
        • Dorfman GS
        • Denny DFJ.
        Percutaneous inferior vena cava cannulation for long-term parenteral nutrition.
        Surgery. 1985; 97: 602-605
        • Gupta A
        • Karak PK
        • Saddekni S.
        Translumbar inferior vena cava catheter for long-term hemodialysis.
        J Am Soc Nephrol. 1995; 5: 2094-2097
        • F Moura
        • Guedes FL
        • Dantas Y
        • et al.
        Translumbar hemodialysis long-term catheters: an alternative for vascular access failure.
        J Bras Nefrol. 2019; 41: 89-94
        • Liu F
        • Bennett S
        • Arrigain S
        • et al.
        Patency and complications of translumbar dialysis catheters.
        Semin Dial. 2015; 28: E41-E47