A 52-Year-Old Woman with Headache and Bradycardia
Total Page：16
File Type：pdf, Size：1020Kb
Journal of the Louisiana State Medical Society CLINICAL CASE OF THE MONTH A 52-Year-Old Woman With Headache and Bradycardia Christopher Burkenstock, MD; Murtuza Ali, MD; Tracy Dewenter, MD; Fred Lopez, MD CLINICAL VIGNETTE anterior fascicular block, as well as T wave abnormalities suggestive of possible ischemia (Figure 1). Chest X-ray dem- A 52-year-old woman presented to the emergency onstrated hilar fullness but was otherwise within normal department with a chief complaint of headache for one limits. Non-contrast computed tomography scan (CT) of day. She reported a “left frontal headache,” which she the head demonstrated a hypodensity in the calcarine re- described as “pressure-like,” non-radiating, and 9 out of 10 gion of the left occipital cortex; magnetic resonance imaging in intensity, beginning on the evening prior to presentation. (MRI) study of the brain demonstrated a hyperintensity on The patient denied any aggravating or relieving factors. As- diffusion-weighted imaging suggestive of an acute ischemic sociated symptoms included blurred vision and a left facial event. A CT of the thorax with contrast confirmed bilateral paresthesia. The patient also endorsed nasal congestion and hilar lymphadenopathy. Endobronchial ultrasound with several episodes of palpitations over the few months prior to fine needle aspiration biopsy identified multiple small non- presentation but denied photophobia, meningismus, facial caseating epithelioid granulomas in several mediastinal asymmetry, weakness, and dysarthria. She did admit to pre- lymph nodes (Figure 2), and competing etiologies of granu- vious episodes of headache, which were similar in nature, lomatous inflammation were ruled out with special stains though not as severe or progressive, and never associated and cultures. A transthoracic echocardiogram demonstrated with visual changes or paresthesia. grossly normal chamber size and proximal aorta left ven- The patient reported a history of diabetes mellitus, tricular ejection fraction of >55%, mild apical hypokinesis, hyperlipidemia, and hypertension - all of which were elevated pulmonary arterial systolic pressures of 42 mmHg, poorly controlled due to medication non-adherence. Her mild tricuspid and mitral regurgitation, and a moderately home medications included fexofenadine, ibuprofen, and sized intracavitary mass attached to the apicoposterior acetaminophen. The patient quit smoking one year ago and wall of the left ventricle (Figure 3), consistent with a left reported occasional alcohol use but denied illicit drug use. ventricular thrombus. Cardiac technetium (99mTc) sestamibi Vital signs in the emergency department demonstrated a perfusion scan demonstrated a paucity of photon uptake temperature of 98.9oF, heart rate of 47 beats per minute, in the anterior wall of the left ventricle, consistent with a respiratory rate of 20 per minute, blood pressure of 158/71 perfusion defect of scar or stunned myocardium. Cardiac mmHg, oxygen saturation of 98% on room air, and a BMI of MRI demonstrated normal atrial and ventricular volumes; 26 kg/m.2 Physical examination revealed a well-nourished normal left and right ventricular ejection fraction; and no female who was fully oriented. No cervical, axillary, or evidence of delayed hyperenhancement, thereby, suggest- inguinal lymphadenopathy was appreciated. She was bra- ing a lack of active myocardial granulomatous inflamma- dycardic without murmurs or gallops. Her lungs were clear tion. The patient was initiated on oral steroids and referred to auscultation without wheezes or crackles. Neurologi- for sarcoidosis management. In addition, a dual chamber cally, she demonstrated no motor or sensory deficits in her implantable cardioverter defibrillator was placed and was extremities. There was normal coordination and no ataxia. functioning well following discharge. Her face was symmetric, and cranial nerve examination demonstrated a right homonymous hemianopsia. INTRODUCTION Initial laboratories demonstrated normal blood cell lines and chemistries, with the exception of hyperglycemia Sarcoidosis is an idiopathic disease of chronic granu- (250 mg/dL). An EKG demonstrated sinus rhythm with lomatous inflammation. There is a bimodal distribution complete heart block, right bundle branch block, and left (peaks between the ages of 20-29 and 50 years of age), and 228 J La State Med Soc VOL 164 July/August 2012 Figure 1: EKG on admission demonstrating normal sinus rhythm with complete heart block. Ventricular escape rhythm has right bundle branch and left anterior fascicular morphology. A premature ventricular complex is noted toward the end of the EKG (second beat from the end). prevalence approaches 10-40 per 100,000.1 There is also a granulomatous inflammatory response. Mononuclear a propensity towards African Americans (10-17:1) and phagocytes and Type 1 helper CD4 T cells predominate in women.1 Sarcoidosis predominantly affects the lung paren- the early granulomatous infiltrates, with cytokine expres- chyma and mediastinal lymph nodes; patients often present sion predominated by interleukin-2 and interferon-v, in with chronic respiratory complaints, including dyspnea addition to interleukin-6.8 Late granulomas demonstrate a on exertion, cough, and chest pain.2 Although pulmonary predominance of Type 2 helper CD4 T cells and fibroblasts complaints are most common, sarcoidosis may affect virtu- with production of interleukin-10 and transforming growth ally any organ system. factor-B, which inhibits the Th1 response while promoting Extrapulmonary manifestations of sarcoidosis include fibrosis and scar formation.8 neurologic complaints, ocular pain and pruritus, cutane- In cardiac sarcoidosis, the characteristic non-caseating ous manifestations (such as lupus pernio and erythema granuloma may be found in the left ventricular free wall, nodosum), hepatosplenomegaly, polyarthralgia (Lofgren’s ventricular septum, right ventricular wall, papillary muscle, syndrome), constitutional symptoms (fatigue, anorexia, and either the right or left atrium.9 When left unchecked, weight loss, fever), and cardiac abnormalities.2 Cardiac the natural history of the disease follows a progression sarcoidosis is a subset of sarcoidosis that may precede, follow, or be concurrent with involvement of other organs. Upon autopsy, about 25% of patients with sarcoidosis are found to have cardiac involvement, while only about 5% of patients have clinically apparent cardiac disease.3 When present, cardiac sarcoidosis portends a poorer prognosis.4-5 PATHOPHYSIOLOGY The etiology of sarcoidosis is unknown, and by defini- tion, is considered an idiopathic inflammatory condition. There are multiple hypotheses involving the confluence of genetic susceptibility with antigenic triggers from par- ticular exposures (including environmental, occupational, and infectious).6 Agricultural occupations with exposure to insecticides and bioaerosols have been implicated, as have microbes such as Mycobacteria, Borrelia, Rickettsia, and herpes virus.6-7 The classic lesion of sarcoidosis is the non-caseating Figure 2: Mediastinal lymph node fine needle aspiration epithelioid granuloma. Its genesis is thought to be the re- biopsy reveals a small epithelioid granuloma, consisting of sult of a process of immune dysregulation, which requires a loose syncytial aggregate of epithelioid histiocytes with an antigenic exposure, cell-mediated immune response elongated nuclei. against that antigen, and effector cells, which facilitate J La State Med Soc VOL 164 July/August 2012 229 Journal of the Louisiana State Medical Society pre-syncope to syncope to sudden death. Cardiac sarcoidosis should be considered when a patient with a confirmed conduction defect is far younger than the age range which is classically associated with that defect, especially in the setting of extra- cardiac sarcoidosis.12 Ventricular arrhythmias are the second most common manifestations in cardiac sarcoidosis. The granulomas act as foci for abnormal automaticity and reentry (via dispersion of activation and recov- ery) thereby leading to arrhythmia. This process occurs both in the setting of active disease, as well as inactive disease (e.g. fibrosis and scar).13 When the patient is symptomatic, ventricular tachycar- dia and premature ventricular contraction are second only to complete heart block in terms of incidence at about 23%.14 Ventricular arrhythmias and complete heart block combined account for 25-65% of sudden deaths in patients with cardiac sarcoidosis.13 Atrial arrhythmias are rarer than ventricular Figure 3: Echocardiogram demonstrating well-circumscribed mass at the arrhythmias at about 15-17%. They are more likely left ventricular apex consistent with thrombus (T). to be the result of atrial enlargement and the effects of pulmonary sarcoidosis than the direct result of granulomatous infiltration or scar.9 When present, from edema to granulomatous inflammation to fibrosis, and paroxysmal atrial tachycardia, atrial fibrillation, and atrial finally, scar formation.9 flutter are most common. Genetic factors appear to play a role in the pathophysiol- Heart failure may present as either left ventricular ogy of sarcoidosis. It appears that some individuals may be systolic or diastolic dysfunction and may take the form of more likely to respond to an antigenic trigger with a more restrictive or dilated cardiomyopathy from granulomatous robust cellular immune response leading to granuloma infiltration of the myocardium. Heart failure accounts for formation. The familial