Chapter 140

The superior vena cava (SVC) syndrome is a clinical disorder caused by compression or obstruction of the SVC. The signs and symptoms associated with this syndrome are swelling of the face, neck, and arms; cough; nasal congestion; visual disturbance; dyspnea; orthopnea; headaches; dizziness; and syncope. Serious symptoms secondary to severe laryngeal and cerebral venous congestion often require urgent intervention. The severity of the symptoms depends on the extent of obstruction and degree of collateral circulation. Generally speaking, a slowly progressive process has a well-developed collateral circulation, and the symptoms tend to be less severe.

SVC obstruction may be caused by malignant or benign disease, or it may have an iatrogenic etiology. Malignancy is the most common cause of the SVC syndrome.1–4 Benign processes include histoplasmosis, fibrosing mediastinitis, thrombosis, infection, aneurysm, benign tumors, and idiopathic etiologies.3–5 The iatrogenic causes include radiation therapy, IV catheters, and transvenous pacemaker leads. IV devices have been reported to be the leading cause of benign SVC syndrome.4

Most cases of SVC obstruction encountered by the thoracic surgeon are caused by malignant or benign mediastinal tumors. Mediastinal masses obstruct the SVC through extrinsic compression, intravascular invasion, or secondary thrombosis as a result of flow turbulence or the patient's hypercoagulatory status. Inciting malignancies include bronchogenic carcinoma, lymphoma, metastatic cancer, malignant germ cell tumor, and malignant thymic tumor, among others.1–4 Bronchogenic carcinoma is the most common cause of adult malignant SVC syndrome. Nieto and Doty reported that bronchogenic cancer accounts for 67–82% of all cases of SVC syndrome and lymphomas for 5–15%.2 In a more recent review, Rice and colleagues reported that bronchogenic carcinoma accounts for 46–78% of cases of SVC syndrome and lymphomas for 8%.4 Among bronchogenic carcinomas, squamous cell and small cell carcinomas are common.1,3 In children, lymphoma is the most common cause of SVC syndrome.6 Benign tumors that cause SVC obstruction include thymoma, teratoma, substernal thyroid goiter, cystic hygroma, and dermoid cyst, among others.5

The two circumstances in which surgical treatment of the SVC syndrome is considered include resection of an obstructing tumor and severe refractory syndrome. We describe the surgical management of SVC syndrome secondary to obstruction by malignant and benign mediastinal tumors, with particular focus on resection and reconstruction of the SVC with cardiopulmonary bypass (CPB).

The goal of surgical management of SVC obstruction is relief of the SVC syndrome, complete resection of the tumor, or both. Clarifying the goal is essential to affecting a reasonable surgical plan. The surgical indications for SVC obstruction of benign etiology are (1) severe SVC syndrome that cannot be alleviated by conservative therapy, (2) a tumor that is causing serious obstruction or compression of other organs such as a lung, trachea, heart, or aorta, and (3) a functional hormone-excreting tumor that is causing adverse symptoms that cannot be alleviated by conservative therapy.

Benign tumors tend to grow slowly, and ...

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