Abstract
A 52-year-old woman is described in whom cardiac sarcoidosis was diagnosed clinically on the basis of high-grade heart block, recurring ventricular tachyarrhythmias, and acute cardiac decompensation. Confirmation of sarcoidosis was not determined until necropsy. When it causes cardiac dysfunction, sarcoidosis rarely causes dysfunction of another body organ, although small sarcoid granulomas may also be present in noncardiac organs or tissues. In the present patient, neurosarcoidosis accompanied the cardiac sarcoidosis, but it was the latter that produced the life-threatening symptoms and was fatal.
Sarcoidois is characterized by the presence of noncaseating giant-cell granulomas in more than one body organ. Although these granulomas may be present in virtually any organ, most commonly they occur in the lungs and in adjacent lymph nodes (1), but they are rare in the spinal cord. Symptoms usually appear gradually. Diagnosis often is made by chest radiograph showing hilar adenopathy with or without pulmonary parenchymal lesions before appearance of symptoms. Occasionally, sarcoidosis presents with symptoms related to the heart, and in such circumstances the manifestations usually are acute, consisting of a ventricular arrhythmia or a high grade of heart block or symptoms of heart failure (2). When sarcoidosis causes cardiac dysfunction, infrequently is there dysfunction of another body organ (2). To emphasize the fact that patients with cardiac sarcoidosis severe enough to cause cardiac dysfunction rarely have more than minimal involvement of noncardiac body organs, we describe the following case.
DESCRIPTION OF PATIENT
A 52-year-old nonsmoking, overweight African American female bus driver, who was born in February 1956, had been well until November 2007 when she noted right-sided mid back pain and, in December 2007, impaired vision in the right eye. The right optic disc was swollen and pale. The back pain made it difficult for her to dress herself, it was burning and shooting in nature, and it radiated to her right arm and breast. The impaired vision in the right eye caused blurriness and occasional double vision and was associated with pain with eye movement. Magnetic resonance imaging in January 2008 disclosed multiple small thoracic spine neural foramen tumors and abnormal contrast enhancement in the right retrobulbar fat and along the right optic nerve sheath, and a 15-mm “low-density” lesion in the left lower lobe of the lung was detected by computed tomography. The hilar and mediastinal structures were normal. Treadmill stress echocardiogram disclosed intermittent right bundle branch block throughout exercise and sinus bradycardia at rest, but no ST changes. A four-beat run of atrial tachycardia with aberrant conduction occurred during exercise. No wall-motion abnormalities were seen. Because of the eye problem, she was started on prednisone 80 mg daily and tapered to 10 mg for 7 months. She was off prednisone for the last 5 months of her life. Her vision improved in the right eye from 20/400 to 20/40 as a consequence of treatment.
Repeat chest radiograph in July 2008 disclosed no abnormalities. Echocardiogram disclosed normal-sized ventricular cavities and hyperdynamic left ventricular systolic function. Left ventricular diastolic function, however, was abnormal.
The eye problem cleared and, except for continued periodic right-sided back pain, occasional leg numbness, and some difficulty walking up stairs, she was active and continued working as a bus driver until December 26, 2008, when severe dyspnea appeared. She was found to have complete heart block, and a pacemaker was inserted. The dyspnea, however, persisted and within a week was accompanied by severe substernal chest pain radiating to both arms, dizziness, and worsening dyspnea. She was rehospitalized on January 6, 2009. The erythrocyte sedimentation rate was 76 mm in 1 hour; hematocrit, 31%; troponin, 2.92 ng/mL; and B-type natriuretic peptide, 1183 pg/mL. At cardiac catheterization, the epicardial coronary arteries were normal. The left ventricular ejection fraction was about 55%. Pressures (in mm Hg) were as follows: pulmonary artery, 45/26; pulmonary wedge a wave, 28, v wave, 33, and mean, 26; right atrial a wave, 18, v wave, 23, and mean, 15; left ventricle, 105/14; and aorta, 109/64. A left ventricular angiogram showed a mildly hypokinetic posterobasal wall and 2+/4+ mitral regurgitation. Ventricular tachycardia occurred during the procedure. An echocardiogram on January 16 showed depressed right and left ventricular systolic function (ejection fraction 45%), a restrictive left ventricular diastolic filling pattern, and severe mitral regurgitation. The heart failure progressively worsened, the blood pressure dropped, and ventricular tachycardia refractory to cardioversion occurred followed subsequently by fatal ventricular fibrillation on January 22, 2009.
At necropsy, the heart weighed 485 g. The epicardial coronary arteries were normal. Multiple lesions were present in the right and left ventricular free walls and in the ventricular septum (Figures 1 and 2). Additionally, the wall of the superior vena cava as it entered the right atrium and portions of the right atrial wall and atrial septum were infiltrated by similar lesions, which histologically were noncaseating giant-cell granulomas (Figure 3). Sarcoid granulomas in the right ventricular wall are shown in Figure 4 and in the left ventricular wall in Figure 5. The four cardiac valves were anatomically normal.
Figure 1.
The base of the heart, showing a large sarcoid nodule in the right ventricular free wall anteriorly (arrow).
Figure 2.
Cross-sections of the ventricular walls and cavities showing multiple sarcoid granulomas. One sarcoid focus involves a portion of the posteromedial papillary muscle of the left ventricle, and this deposit may have played a role in causing the mitral regurgitation.
Figure 3.
(a) The superior vena cava (SVC) entering the right atrium (RA), with the wall of this large vein replaced by sarcoid granulomas. (b) The more caudal portion of the RA and left atrium (LA), showing sarcoid granuloma in the right atrial wall posteriorly and in the atrial septum.
Figure 4.
Granulomas in the right ventricular wall. Hematoxylin and eosin stains each × 100. Stains for fungi and acid-fast organisms were negative.
Figure 5.
Sarcoid myocarditis in the left ventricular wall. Hematoxylin and eosin stains, (a) × 100, (b) × 400.
The lung parenchyma was normal grossly except for one lesion in the lower lobe of the left lung, and it too was a sarcoid granuloma (Figures 6 and 7). At least two hilar lymph nodes were infiltrated by similar granulomas: one was normal sized, and one about 4 times larger than normal (Figure 7a).
Figure 6.
(a) Longitudinal section of the lung showing a single sarcoid granuloma. (b) A close-up of the area in the square.
Figure 7.
Sarcoid granulomas in the (a) hilar lymph node and (b) lung. Hematoxylin and eosin stains, × 100 (a), × 400 (b).
The spinal cord tumors, seen by magnetic resonance, proved to be sarcoid granulomas (Figure 8). Similar granulomas were present in the single kidney (one had been removed earlier for donation to a family member for renal transplantation).
Figure 8.
Sarcoid granulomas in the thoracic spinal cord. Hematoxylin and eosin stains, (a) × 2, (b) × 100.
DISCUSSION
The patient described had classic features of cardiac sarcoidosis, namely a high degree of heart block (third degree), recurring ventricular arrhythmias, and both systolic and diastolic cardiac dysfunction resulting in severe heart failure. Because of this triad of cardiac manifestations, cardiac sarcoidosis was diagnosed clinically. Necropsy confirmed the diagnosis of sarcoidosis, which involved extensively the heart, thoracic spinal cord, and hilar lymph nodes (only one of which was enlarged), and minimally the lung and kidney. The cause of her back pain was sarcoid granulomas within the spinal cord. Although its cause about a year before death was never definitely determined, the visual disturbance prompted the initiation of prednisone therapy, which eliminated the eye problem and obviously is the treatment of choice for sarcoidosis.
A very unusual feature of the present case is the occurrence of sarcoid granulomas in the spinal cord, which led to some nonlimiting symptoms. Others also have reported neurosarcoid involvement in patients with cardiac sarcoidosis (3, 4). Saleh and colleagues (3) reviewed 172 patients with spinal cord sarcoidosis, and 7 (4%) had cardiac involvement. Perry and Vuitch (4), however, found no cases of neurosarcoidosis among 14 autopsied patients with fatal cardiac sarcoidosis.
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