Cardiac Tamponade and Superior Vena Cava Syndrome in Lung

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Cancer: A Case Report

A combination of pericardial effusion with cardiac tamponade and superior vena caval syndrome is an unusual first presentation of carcinoma of lung, although cardiac involvement is often a late finding in widespread malignancy. Clinical identification can be difficult antemortem. Accurate diagnosis and prompt intervention are necessary to prevent adverse outcomes. Decisions regarding treatment must take into account the clinical presentation and echocardiographic findings. Echocardiography-guided pericardiocentesis with catheter drainage and/or pericardial window is the primary treatment strategy of choice for most large or hemodynamically significant effusions. New cardiac symptoms or classic findings of cardiac tamponade should prompt aggressive investigation. We present a case of adenocarcinoma of the lung that initially presented as pericardial effusion with tamponade and superior vena cava syndrome. The patient had all the clinical features of tamponade such as pulsus paradoxus, tachycardia, elevated jugular venous pressure, hypotension, and electrical alternans on surface electrocardiography. The findings were confirmed on echocardiography and computed tomography of chest, both of which allowed for rapid confirmation of the presence of an effusion and compression of the superior vena cava. The existing literature on the subject is succinctly reviewed.

Introduction

Pericardial effusion with resultant impairment of the cardiac function is the most common clinical presentation (30%) in metastatic involvement of pericardium, but the majority of cases may go unrecognized.1 Cancers of the lung, breast; and melanoma and lymphoma are the most likely noncardiac tumors that involve the pericardium.2,3 Metastases to the heart and pericardium are discovered at autopsy in 10% to 12% of all patients with malignancy.2,3 The most common underlying malignancy is carcinoma of the lung, in part because of the proximity to the heart and its common prevalence.1 Autopsy series show that cardiac metastases is accounted by bronchogenic carcinoma (36%), non-solid primary malignancies (leukemia, lymphoma, and Kaposi sarcoma, 20%), carcinoma of the breast (7%), and carcinoma of the esophagus (6%).3 Earlier series suggested that breast cancer and melanoma were as common as lung cancer,4 but presently lung cancer is the most common primary tumor, and adenocarcinoma the most frequent cell type of cardiac metastases.3 Malignancy is the leading cause of cardiac tamponade in the developed nations, unlike infections (mainly tuberculosis) in other parts of the world.

The most common clinical presentation of neoplastic pericardial disease is shortness of breath, which is out of proportion to the imaging findings of pericardial effusion with or without associated pleural effusion. Apart from simple pericardial effusion, the patients with neoplastic pericardial disease can present with acute pericarditis, pericardial effusion, effusive-constrictive pericarditis, or cardiac tamponade.5 Cardiac tamponade is rarely the first manifestation of a metastatic neoplasm.6-10 Echocardiography is the imaging modality of choice to examine the pericardium, and computed tomography and magnetic resonance imaging offer additional information during the evaluation of metastatic disease.11,12 Death in these patients with cardiac or pericardial metastasis is predominantly the result of cardiac tamponade, in addition to congestive heart failure, or invasion of sinus node or coronary artery.13 Superior vena cava obstruction is an uncommon manifestation of carcinoma of the lung characterized by facial, and neck swelling and distended veins over the chest. Masses in the superior mediastinum cause extrinsic compression of the superior vena cava, resulting in the superior vena cava syndrome. We report a 42-year-old male active smoker with cardiac tamponade and superior vena cava syndrome at presentation due to stage 4 adenocarcinoma of the lung and succinctly review the subject.

Case Presentation

A 45-year-old male presented with acute onset of worsening shortness of breath at rest of 2 days' duration, swelling of the face and neck, nonproductive cough, and 10-kg weight loss over the preceding 6 months. He admitted a 60-pack-year history of cigarette smoking. On admission, his blood pressure was 99/72 mm Hg; heart rate 120 beats/min, with pulsus paradoxus of 25 mm Hg; respiratory rate of 22/minute; temperature 98F; and oxygen saturation of 98% on room air. The patient was in respiratory distress, with injected conjunctiva, very prominent neck veins, jugular venous pressure elevated at the angle of jaw, palpable bilateral posterior cervical and supraclavicular lymph nodes, a plethoric puffy face, and swelling of the neck and left arm. On chest examination there was redness of the anterior chest, the trachea was slightly deviated toward the right, with decreased breath sounds on the right and bilateral basal crepitations. The cardiac apex was not palpable and heart sounds were slightly distant. There was no pedal edema and abdominal and neurologic examinations were unremarkable.

The electrocardiogram showed sinus tachycardia, low voltage complexes, and evidence of electrical alternans. Chest radiography showed a prominent cardiac silhouette and bilateral pleural effusion (right > left). Findings on computed tomography of the neck and chest were bilateral pleural effusions, large pericardial effusion, impending superior vena caval obstruction, enlarged hilar lymph nodes, masses in anterior and middle mediastinum, and bilateral cervical lymphadenopathy (right > left) with fluid in the prevertebral space (Figures 1,2). Transthoracic echocardiography showed a large anterior and posterior echofree space, with diastolic collapse of the right ventricle, and swinging heart consistent with large pericardial effusion with tamponade. Left ventricular function was normal with no valvular abnormalities.

A diagnosis of cardiac tamponade with superior vena caval syndrome secondary to a possible malignancy was made. Subsequently an urgent pericardial window and right tube thoracostomy was performed. A total of 1,500 mL of serosanguinous fluid was drained. The patient felt better. Cytopathologic study results were consistent with adenocarcinoma of lung. Oncology service suggested palliative radiation therapy followed by chemotherapy in view of superior vena caval syndrome (stage IV non-small cell lung carcinoma). Over the next few weeks, the patient progressively deteriorated. A computed tomography chest obtained 3 months after radiation therapy and chemotherapy revealed a thickened and nodular appearance of the pericardium (Figure 3). The patient and family clearly understood the poor prognosis so preferred hospice care placement and had instructions for do not resuscitate orders. The patient died 5 months after initial presentation.

Figure 1. Axial-enhanced CT of the chest demonstrating extrinsic compression of the superior vena cava and mediastinal lymphadenopathy. In addition, pleural enhancement and bilateral large pleural effusions is visualized.

Figure 2. Axial-enhanced CT of the chest demonstrating large pericardial and bilateral pleural effusion. Enhancement of pericardium is seen.

Figure 3. Axial-enhanced CT of chest obtained 3 months post- radiation and chemotherapy, demonstrating thickened and nodular appearance of the pericardium with significant decrease in pericardial effusion and persistent of large pleural effusions.

Discussion

Involvement of the heart and pericardium is found at autopsy in 17% to 31% of patients who had carcinoma of the lung.3,14 Metastasis is usually by direct extension or by a combination of lymphatic and hematogenous dissemination.14 It represents Ml or stage IV disease in the American Thoracic Society's TNM staging system for carcinoma of lung. Cardiac tamponade is rarely a presenting manifestation of carcinoma of the lung. A literature review revealed approximately a total of 44 cases of cardiac tamponade as the first manifestation of malignancy, with 18 cases secondary to carcinoma of lung and 26 cases due to other extracardiac malignancies.6-10 The majority of the patients consisted of males and had underlying carcinoma of lung. Clinical features such as pulsus paradoxus, tachycardia, elevated jugular venous pressure, and hypotension are important clues to the diagnosis of cardiac tamponade. When there is a rapid accumulation of pericardial fluid, even as little as 250 mL can lead to tamponade.

Cardiac tamponade is a life-threatening condition. Accurate diagnosis and prompt intervention are necessary to prevent adverse outcomes. Echocardiography allows rapid confirmation of the presence or absence of an effusion, and enables assessment of its hemodynamic impact and is the most commonly used readily available noninvasive modality.11 Computed tomography and, more specifically, magnetic resonance imaging have emerged as useful diagnostic tools for assessing diseases of the pericardium.12 Advantage of computed tomography and magnetic resonance imaging is their ability to provide a large field of view, allowing evaluation of disease processes in the chest, with particular importance to assess the lung, pleura, mediastinum, and great vessels of the heart.12

Cardiac tamponade is an indication for urgent pericardiocentesis. Pericardiocentesi\s is useful as a diagnostic and therapeutic modality. Neoplastic effusions are either serosanguinous or hemorrhagic effusions.10 Yield of the cytologic studies of the pericardial fluid is seen in up to 80% to 90% of patients with malignant pericardial effusions.15 Decisions regarding treatment must take into account the clinical presentation and imaging findings.

Echocardiographic-guided pericardiocentesis with catheter drainage is the primary treatment strategy of choice for most large or hemodynamically significant effusions. In contemporary clinical practice, echocardiography is the gold standard for diagnosis of tamponade and is essential for directing treatment.11 A malignant pericardial effusion with tamponade is associated with a poor prognosis, but few therapeutic options include creation of a pericardial window, pericardial sclerosis, and radiation therapy.16- 18 External beam radiation therapy provides short-term relief (mean of 4 months).18 The diagnosis of malignant pericardial disease has ominous implications. The response to all therapeutic interventions is poor, with life expectancy in the range of weeks to months (especially non-hematologic cancers).5 The outlook for patients presenting with cardiac tamponade is even worse.

Superior vena cava syndrome secondary to malignant disease may manifest within weeks to months. Dyspnea is the most frequent symptom; in addition patients complain of facial swelling, facial plethora, and arm edema associated with venous distension in the neck and the chest wall with facial edema.19 Malignant tumors are responsible for 78% to 85% of cases of superior vena cava syndrome, lung cancer being most common, followed by lymphoma.19,20 Bronchogenic carcinoma and lymphoma account for 94% of cases.21 Approximately 2% to 4% of patients with bronchogenic carcinoma develop superior vena cava syndrome at some point in the disease course. It is common with small cell carcinoma due to its central nature, and superior vena cava syndrome tends to develop from either extrinsic compression or direct invasion of the superior vena cava by the primary neoplasm or by enlarged lymph nodes. Superior vena cava syndrome at presentation and associated with adenocarcinoma of the lung is a rarity. Approximately 60% of patients who present with superior vena cava syndrome related to malignancy are without a known diagnosis of neoplasm.20 The chest radiograph is uniformly abnormal in these patients (84%).19 Chest computed tomography is the preferred modality of imaging and magnetic resonance imaging may be useful. A histologic diagnosis is a prerequisite, so a biopsy is generally performed to confirm malignancy and plan therapy accordingly. Superior vena cava obstruction in patients with non- small cell lung cancer is a strong predictor of poor prognosis with a median survival of 5 months. In one review,22 only 60% of patients had relief of symptoms related to superior vena cava syndrome with chemotherapy, radiation therapy, or both.

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Ramesh M. Gowda, MD,* Ijaz A. Khan, MD, FACA,[dagger] Nirav J. Mehta, MD,[dagger] Mamatha R. Gowda, MD,* Phyllis Hyde, MD,* Balendu C. Vasavada, MD,* and Terrence J. Sacchi, MD,* Brooklyn, NY and Omaha, NE

Angiology 55:691-695, 2004

From the * Department of Medicine, Long Island College Hospital, Brooklyn, NY; and [dagger] Division of Cardiology, Creighton University School of Medicine, Omaha, NE

No financial support was received for this manuscript

Correspondence: Ijaz A. Khan, MD, FACA, Creighton University Cardiac Center, 3006 Webster Street, Omaha, NE 68131

E-mail: ikhan@cardiac.creighton.edu

2004 Westminster Publications, Inc., 708 Glen Cove Avenue, Glen Head, NY 11545, USA

Copyright Westminster Publications, Inc. Nov/Dec 2004

Source: Angiology