Superior Vena Cava Syndrome after Bone Marrow Transplantation Caused by Aspergillosis: A Case Report

Aspergillosis is known for the variety of unusual presentations in immuno-suppressed patients. We report a patient in whom aspergillosis caused the superior vena cava (SVC) syndrome. A 37-year-old woman became febrile soon after bone marrow transplantation (BMT). Chest radiography demonstrated a 5-cm mass extending from the right lung apex to the right supraclavicular fossa beside her Hickman catheter. She then developed SVC syndrome, which progressed despite treatment. Despite recovery of the white blood cell count, the patient continued to deteriorate, became comatose, suffered a cardiac arrest and died 31 days after BMT. Autopsy revealed Aspergillus infection at the apex of the right lung associated with innominate artery thrombosis.     

Superior vena cava syndrome early after pacemaker implantation in a patient with lung carcinoma: A case report

Superior vena cava (SVC) syndrome is typically dramatic. Although the diagnosis is usually easy, elucidation of the etiology is difficult. We present a patient who developed SVC syndrome early after transvenous pacemaker implantation and who was subsequently diagnosed with lung carcinoma. The pathogenesis seems to be explained by a combination of two etiologies: lung carcinoma plus transvenous pacemaker implantation. We emphasize that common etiologies such as malignancy should be considered first when faced with SVC syndrome.     

Case of lung cancer with chylothorax]

A 42-year-old man had swelling in the right side of the neck, cough and chest pain. On admission, an abnormal shadow was detected in the right upper lung field and squamous cell carcinoma of the lung with superior vena cava (SVC) syndrome was diagnosed. Concurrent radiotherapy and systemic chemotherapy consisting of cisplatin and vinorelbine induced a partial response. At 15 months after diagnosis, he was re-admitted because of bilateral pleural effusion and facial edema due to relapse of SVC syndrome. Examination of the milky right pleural effusion revealed chylothorax (959mg/dl of beta-lipoprotein and 675mg/dl of triglyceride). The right effusion was finally controlled by pleurodesis with OK-432. Non-traumatic chylothorax is a rare complication of lung cancer.     

A case of mediastinal abscess due to Enterococcus faecalis accompanied with superior vena cava syndrome

Superior vena cava (SVC) syndrome caused by benign diseases is rare. We reported a case of mediastinal abscess due to Enterococcus faecalis (E. faecalis) accompanied with SVC syndrome and reviewed the literature on this particular condition. A 38-year-old female with swelling of the neck and dilatation of cervical vein was admitted to our hospital, being diagnosed as having a SVC syndrome. Chest roentgenogram revealed an enlargement of the right upper mediastinum and a massive infiltration in the right upper lung field. CT scan demonstrated a mass with central necrosis occupying the right upper mediastinum and stenosis of superior vena cava. Further conformation of the stenosis of vena cava was made by means of venography. E. faecalis was recovered from the pus aspirated from the mediastinal abscess, and a definitive diagnosis of SVC syndrome caused by mediastinal abscess due to E. faecalis was made. There has been no report, to our knowledge, on mediastinal abscess evolving SVC syndrome in which E. faecalis was identified as a causative organism. The present case indicates that a benign disease such as lung abscess should be considered to be a possible cause of SVC syndrome in addition to other frequent malignant diseases.     

Multimodality treatment of malignant superior vena caval syndrome.

Malignant superior vena caval (SVC) syndrome due to non-small cell lung cancer is invariably fatal, with most therapy directed toward palliating the manifestations of the disease. A cure, by means of any modality, is unusual. We report a patient with SVC syndrome secondary to documented ipsilateral peritracheal nodal involvement (stage IIIB disease) who underwent neoadjuvant chemoradiotherapy and resection. At surgery, his superior vena cava was not involved and his tumor had been downstaged to stage I (T1 nanoseconds). He remains alive and free of disease 60 months after surgery. Neoadjuvant chemoradiotherapy may be used to downstage malignant SVC syndrome to resectable lesions in good functional candidates.     

Bilateral pneumothoraces with multiple bullae in a patient with asymptomatic bronchiolitis obliterans 10 years after bone marrow transplantation

A 16-year-old boy developed bronchiolitis obliterans (BO) 10 years after BMT for myelodysplastic syndrome. Although the patient complained of almost no dyspnea on exertion, he had mild hypercapnea with a markedly reduced forced expiratory volume of 0.32 l. Chest X-rays showed occasional bilateral minimal pneumothoraces, which is in accordance with the existence of multiple small bullae found on the pleural surface at video-assisted thoracic surgery. Histologic examination of the biopsied lung revealed BO. This case indicates that BO in adolescence following BMT and possible chronic GVHD may be masked because of lung immaturity at BMT, and BO after BMT may be associated with multiple pleural bullae.     

Isolated aortic valvular and pulmonary involvement during essential eosinophilia

Endomyocardial fibrosis is the most classic and the most known complication of prolonged hypereosinophilic syndrome, whatever the cause. In Burkina Faso, this complication is most frequently encountered in idiopathic form of the syndrome. It commonly involves the apex of the ventricles with possible involvement of atrioventricular valves. The clinical picture is that of restrictive cardiomyopathy with poor prognosis. We report the case of a 22-year-old man with atypical cardiac involvement during idiopathic hypereosinophilic syndrome. Echocardiographic examination showed isolated aortic valve involvement. Left and right ventricular function was preserved. The apex of ventricles was free of lesion. Pulmonary CT-scan showed massive bilateral lung involvement. Treatment consisted of strict control of the eosinophilic process and pulmonary management. The patient suddenly died sudden pulmonary distress one month after first being seen.     

Leukaemic relapse of donor origin after allogeneic bone marrow transplantation from a donor who later developed bronchogenic carcinoma

Donor-derived leukaemia is exceptional after allogeneic bone marrow transplantation (BMT). A woman with chronic myeloid leukaemia received an allogeneic BMT from a human leucocyte antigen-identical brother. The donor, a 50-year-old non-smoker, died of squamous cell bronchogenic carcinoma 1 year later. At 4 years post BMT, the patient became BCR/ABL positive and relapsed with acute myeloid leukaemia, which was shown to be donor-derived cytogenetically and molecularly. Retrospective analysis showed that the donor-leukaemic clone had started to evolve as early as 6 months post BMT. Sequencing of p53 ruled out Li-Fraumeni syndrome. Predisposition to malignancy might be an underlying mechanism of donor-cell leukaemia.     

Transvenous defibrillator implantation in patients with persistent left superior vena cava and right superior vena cava atresia

In this report a transvenous cardioverter defibrillator implantationis described in two patients with a persistent left-sided superiorvena cava and right SVC atresia. In the first case, manoeuvringof the guide wire inserted through the left subclavian veininto the SVC proved impossible, revealing a left SVC originatingfrom the left brachiocephalic vein with an acute corner. Changingthe side of implantation and inserting a CPI Endotak catheterthrough the right subclavian vein, the lead was easily advancedthrough the left SVC into the coronary sinus and then into theright atrium with the tip abutting the lateral atrial wall.Subsequent manoeuvres allowed passage of the tip of the catheterinto the right ventricular apex with the proximal defibrillationcoil of the Endotak lead in the low left SVC, with its distallimit at the junction with the coronary sinus. A biphasic waveformsingle pathway RVleft SVC successfully defibrillated with astored energy of 5 J. In the second patient, implantation of a transvenous Medtronicsystem was possible from a left infraclavicular approach. Atripolar R V coil was inserted into the right ventricle viathe persistent left SVC and contiguous coronary sinus. Becauseof the acute angle required to enter the R V in this secondcase, the R V lead was looped in the right atrium in order toenter the RV in a satisfactory, albeit atypical RV location.This patient was successfully defibrillated with a 5 J monophasicwaveform delivered between the RV coil, a CSIleft SVC coil,and a subcutaneous patch. In conclusion, both of these patients illustrate the abilityto use transvenous ICDs successfully in patients with persistentleft superior vena cava although the implantation techniquedeviates substantially from traditional methods.     

Bilateral absence of the superior vena cava presenting with superior vena cava syndrome: A case report

Bilateral absence of the superior vena cava (SVC) is a very rare congenital vascular anomaly that is mainly asymptomatic. In this report, we describe an adult male patient with bilateral absence of the SVC presenting with SVC syndrome. Blood from the upper body returned to the right atrium via the superficial thoracoepigastric veins, the great saphenous veins, the common femoral veins, and the inferior vena cava.     

Successful allogeneic bone marrow transplantation for myelodysplastic syndrome complicated by severe pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the abnormal accumulation of alveolar surfactant protein in alveolar spaces. We report herein a rare case of myelodysplastic syndrome (MDS-RAEB) complicated by severe PAP, and successful allogeneic bone marrow transplantation (BMT) for both disorders. An unrelated BMT was planned for a 48-year-old male with advanced MDS-RAEB. Just before the initiation of the conditioning regimen for unrelated BMT in March 2007, he developed dyspnea. A diagnosis of PAP was made based on findings of chest X-ray, CT scanning, and the fluid obtained by bronchoalveolar lavage. To improve his dyspnea and improve BMT safety, whole lung lavage (WLL) was performed twice, with the partial improvement of PAP. Unrelated allogeneic BMT was performed in September 2007. We had to perform a third WLL because of the worsening of PAP on day 26 after BMT. Despite many infectious complications after BMT, GVHD was relatively mild. PAP had almost disappeared 6 months after BMT. He was well with favorable hematopoiesis 20 months after the BMT without any specific treatment. There has been no report of an MDS patient with PAP in whom 3 WLL procedures were performed before and after allogeneic BMT.     

Secondary Cardiac Lymphoma Presenting as Sick Sinus Syndrome and Atrial Fibrillation Which Required Leadless Pacemaker Implantation

Cardiac involvement of malignant lymphoma is relatively common, although such a phenomenon has subclinical manifestations that are difficult to detect. We herein describe a patient with atrial fibrillation and sick sinus syndrome as the main symptoms. Computed tomography showed a mass in the right atrium extending into the superior vena cava (SVC). We implanted the patient with a leadless pacemaker. Transvenous biopsy revealed a diffuse large B-cell lymphoma. The patient was treated successfully with chemotherapy including rituximab. This case suggested that cardiac lymphoma may cause sick sinus syndrome, and leadless pacemaker implantation is a safe treatment option in patients with partial SVC obstruction.     

Superior vena cava syndrome as a complication of transvenous permanent pacemaker implantation

Venous thrombosis induced by a transvenous permanent pacemaker is a common complication. However, superior vena cava (SVC) syndrome caused by pacemaker leads is only occasionally seen and its prevalence has been estimated to be less than 1 in 1000 pacemaker patients. Herein, we report a Taiwanese patient of high grade AV block, who presented with SVC syndrome 2 years after transvenous permanent pacemaker implantation. This case features fibrotic stenosis of the junction of right brachiocephalic trunk and SVC, and an extensive thrombus formation resulting in complete obliteration of the left brachiocephalic vein. The collateral circulation was so delicate that he still could lead a rather normal life, even if anticoagulant therapy proved to be ineffective from an angiographic point of view.     

人工血管置换上腔静脉在肺癌外科治疗中的应用

目的　探讨上腔静脉切除行人工血管置换在晚期肺癌外科治疗中的作用、技术方法及其预后。方法　全组共２６ 例,右侧中心型肺癌２１ 例、周围型肺癌５ 例。行肺癌原发病变、转移淋巴结及受侵及的上腔静脉切除,采用人工血管置换切除的上腔静脉。术后口服小剂量抗凝剂短期治疗,并进行长期的随访与观察。结果　全组无术中及术后早期死亡,无术后近期及远期上腔静脉及无名静脉梗阻症状的发生。随访２３ 例,１、３、５ 年生存率分别为８５％ （２２／２６） 、３９％ （７／１８） 、３６％ （４／１１） 。结论　人工血管置换受侵及的上腔静脉使部分晚期肺癌获得根治性切除,扩大了肺癌外科手术适应证,提高了晚期肺癌患者的生存率,可在晚期肺癌的外科治疗中推荐应用。     

Doppler superior vena cava flow evolution and respiratory variation in superior vena cava syndrome

BACKGROUND: Superior vena cava syndrome (SVCS) is a clinical expression of obstruction of blood flow through the superior vena cava. The patterns of the Doppler flow changes of superior vena cava (SVC), especially the respiratory effects on them have not yet been fully elucidated. This study was to examine SVC Doppler flow patterns and the respiratory effects on them in healthy subjects and patients with SVCS. METHODS: The SVC Doppler flow patterns of 18 normal human subjects and 22 patients with SVCS were analyzed at initial diagnosis and were followed up every 2 months for at least 11 months. RESULTS: Among the 22 patients, 5 patients with the tumor near the right atrium oppressing the inferior segment of the SVC had clear VR- and AR-waves, while in the other 17 patients the VR- and AR-waves disappeared or their outlines were vague. The respiratory variations of the S- and D-waves as a percentage change in inspiration compared to expiration in patient group were much lower than those in control group (S-wave: 1.67 +/- 3.32% vs. 15.65 +/- 16.15%, P = 0.0003; D-wave: 1.80 +/- 1.12% vs. 23.55 +/- 37%, P = 0.0087), which gradually became larger with treatment and showed no significant difference with those in control group after 7 months. CONCLUSIONS: The Doppler flows of the patients with SVCS correlate well with the images of CT scan of them. The respiratory variation of the S- and D-velocities could be used to evaluate the severity of SVC obstruction and its therapeutic effect.     

上腔静脉引流入左心房手术矫正成功一例

本文报道1例上腔静脉引流入左心房的手术矫正方法。先切开上腔静脉和右房上部,再切开房间隔,显露左房内的上腔静脉开口。补片上缘和腔静脉开口后缘相缝合,其他边缘和房间隔的切口缘相缝合,这样就将上腔静脉开口隔入右房。用猪心包补片加宽上腔静脉和右心房,以防上腔静脉开口堵塞。术后恢复满意。文中还讨论该病的诊断问题。     

Reflex ventilatory effects of KCl stimulation of lung receptors with sympathetic afferents

The purpose of this study was to determine the reflex ventilatory effects produced by lung receptors with sympathetic afferent nerves. Seven dogs were anesthetized with sodium pentobarbital, placed on a ventilator, and vagotomized. The chest was opened through a mid-sternal incision. Diaphragm EMG (D-EMG), right and left triangularis sterni EMG (TS-EMG), systemic arterial blood pressure (BP), and tracheal pressure were recorded before and after the application of 2 M KCl to the right or left lung near the venous hilum. The reflex effects produced by KCl applied to the superior vena cava (SVC) and of mechanical distortion of the lungs were also studied. KCl applied to the right or left lung or right or left lung distortion produced significant increases in peak TS-EMG ipsilateral to the applied stimulus with no significant effects on the contralateral TS-EMG. BP, inspiratory time, expiratory time, or peak D-EMG were not significantly affected, except for a decrease in inspiratory time when KCl was applied to the left lung and a decrease in BP with distortion of the left lung. KCl applied to the SVC produced a significant reflex increase in the right TS-EMG. The primary reflex response to chemical stimulation of lung receptors with sympathetic afferents was an increase in expiratory muscle activity. Additionally, mechanical stimulation may also activate these receptors. Furthermore, receptors which produce similar reflex responses are located on the SVC. That the reflex responses produced by intrathoracic receptors with sympathetic afferents were unilateral suggests that spinal mechanisms are involved.     

Bone marrow transplantation for MDS

Five patients with myelodysplastic syndrome (RA: 4 cases, RAEB in T: 1 case) were treated with myeloablative immunosuppressive therapy followed by bone marrow transplantation (BMT). Median age was 20-y-o (11-31-y-o). All patients were prepared with cyclophosphamide and total body irradiation. Engraftment was documented in all patients. One patients (case 4, 31-y-o female) died of brain hemorrhage due to the thrombocytopenia refractory to platelet transfusion because of anti-platelet antibody in 34 days after BMT. A patient with RAEB in T was also died of respiratory failure from interstitial pneumonia on Day 173. One patient (case 1, 22-y-o, female) progressively became granulocytopenic and thrombocytopenic status after BMT. She suffered from life-threatening infection and then received a second bone marrow cell infusion from the same donor without any preparative conditioning. These results suggest that BMT could be the treatment of choice for MDS, especially for the patients with RA who have poor prognostic factors including life-threatening cytopenia and or cytogenetical abnormalities.     

Electrical connection between the right atrium and the superior vena cava,and the extent of myocardial sleeve in a patient with atrial fibrillation originating from the superior vena cava

We describe a case in which the superior vena cava (SVC) was electrically isolated by an application of radiofrequency energy to a point with electrical connection between the right atrium and the SVC in a patient with atrial fibrillation (AF) originating from the SVC. The connection was located in the posteroseptal region between the right atrium and the myocardial sleeve extending into the SVC. Local AF occurred after radiofrequency ablation and rapid activities were recorded all around the SVC, whereas the atrium was still in sinus rhythm. It is suggested that focal AF in the SVC contributes not only to the initiation but also to the maintenance of AF, and the myocardial sleeve extends into the SVC through a connection point to cover the entire internal lumen.     

Allogeneic bone marrow transplantation after partial lobectomy for aspergillosis of the lung

A patient with acute myeloid leukemia (AML) who developed probable aspergillosis of the lung underwent partial lobectomy prior to allogeneic bone marrow transplantation (BMT). Diagnosis was proven by microscopic examination of the resected lung tissue. Local and systemic antifungal prophylaxis with oral amphotericin B plus itraconazole was given throughout the immediate post-transplant period and there was no evidence of recurrent mycosis. Fourteen months after BMT the patient is well, in complete remission and leading a normal life. Pre-transplant pulmonary aspergillosis need not therefore be a contraindication to successful BMT.