A 32-year-old woman presents to the hospital with chest pain. She states that the pain is sharp, worse with deep inspiration, and is somewhat relieved by leaning forward. She is febrile and a friction rub is heard on examination. An ECG is shown below.
The patient is offered treatment but refuses all medications. Which of the following is the most common complication if this disease remains untreated?
Recurrent pericarditis. The chest pain and ECG are typical of acute pericarditis, which commonly presents with fever, pleuritic chest pain, new onset pericardial effusion, and diffuse concave ST elevations on ECG. Most cases have an infectious etiology, including Coxsackie viruses, HIV, influenza, S. aureus, S. pneumoniae, tuberculosis, and various fungi. Other important causes include cancer, autoimmune diseases, post-MI or cardiac surgery, radiation therapy, and uremia. All patients should be treated with NSAIDs and colchicine to improve symptoms and prevent complications. Patients who are not treated are much more likely to develop recurrent pericarditis, which is defined as a recurrence of symptoms after the inciting event (e.g., virus) has passed. (A) Pericardial effusions commonly accompany acute pericarditis; however, cardiac tamponade is a rare complication. (C) Constrictive pericarditis is a possible outcome of any cause of acute pericarditis; however, it is not the most common complication. (D) Free wall rupture is a complication of acute myocardial infarction. (E) Pericarditis affects the pericardium although the myocardium is sometimes affected as well. However, the rate of valve involvement and complications is low.
A young man with Marfan syndrome is brought in by paramedics with sharp chest pain that radiates to his back. Shortly after the pain started, he developed left-sided weakness. The chest pain progressed and an ambulance was called. The initial blood pressure recorded by paramedics on the right arm was 78/54 mmHg, but a reading from the left arm shows 114/74 mmHg. An ECG is performed and shows ST elevations in the anterolateral leads. There is widening of the mediastinum on chest x-ray.
Which of the following is the most likely underlying diagnosis?
Aortic dissection. The most important risk factor for aortic dissection in the general population is hypertension; however, there is a high incidence in patients with connective tissue disease (e.g., Marfan and Ehlers–Danlos syndromes). Sharp chest pain radiating to the back is the first clue to this diagnosis. Other symptoms may occur based on which arteries are occluded by the dissected flap. This patient has a dissection of the ascending aorta, since he has symptoms/signs involving the left coronary artery (ST elevations in the anterolateral distribution) and brachiocephalic artery (affecting the right coronary artery leading to symptoms of stroke and the right subclavian artery leading to a decrease in systolic blood pressure >20 mmHg in the right arm compared to the left). Other potential manifestations not seen in this patient are cardiac tamponade and Horner syndrome (from compression of the superior cervical ganglion). Dissections involving the proximal aorta require immediate surgical intervention.
(A, C, D) All of these diagnoses are a result of the patient’s aortic dissection, but are not the underlying (primary) diagnosis. ACS may occur during an aortic dissection as a result of involvement of one or more coronary arteries, and stroke can occur with involvement of the carotid arteries. Aortic regurgitation is common in patients with Marfan syndrome, and this can also occur as a result of a dissection in the ascending aorta. In either situation, it is not the primary diagnosis.
A 62-year-old Caucasian man with a history of diabetes and a myocardial infarction 2 years ago is hospitalized for acute decompensated heart failure. He is diuresed with improvement in symptoms and is discharged on appropriate medications. He is seen in clinic 4 months later with complaints of worsening symptoms over the last month. Previously he became mildly short of breath after significant exertion, but he now reports severe shortness of breath after walking only 50 m. He is asymptomatic only at rest. His current medications include aspirin, captopril, carvedilol, furosemide, atorvastatin, omega-3 fatty acids, and pantoprazole. An ECG performed in the office shows Q waves in leads V3 and V4, with a normal QRS duration. An echocardiogram performed 1 week ago showed an ejection fraction of 35%.
The patient has been encouraged to quit smoking and drinking alcohol, and to eat a low-sodium diet. What additional therapy is recommended at this time?
: Spironolactone. This question tests the reader’s understanding of chronic heart failure treatment. The patient displays New York Heart Association (NYHA) class III symptoms (no symptoms at rest, but symptoms with minimal exertion), and therefore needs an escalation in his treatment. He is on an appropriate initial regimen, which includes a loop diuretic, an ACE inhibitor, and a β-blocker. Angiotensin receptor blockers (ARBs) are appropriate alternatives to ACE inhibitors, but should not be used in conjunction with ACE inhibitors. The three β-blockers shown to have a survival benefit in heart failure are carvedilol, metoprolol, and bisoprolol, so one of these agents is usually used. This patient is symptomatic and needs the next step in therapy, which is an aldosterone antagonist (e.g., spironolactone). These agents reduce mortality in patients with NYHA class III/IV with a reduced ejection fraction (≤35%), or in patients with heart failure that have suffered a previous myocardial infarction. Notable side effects of spironolactone include hyperkalemia, gynecomastia, and agranulocytosis. If the patient is still symptomatic, digoxin may be prescribed although there is no mortality benefit with this agent. The patient should also consider an ICD given his history of myocardial infarction and reduced ejection fraction. ICDs have been shown to reduce mortality, both for primary prevention and for secondary prevention of fatal arrhythmias.
(A) The patient is already on an ACE inhibitor, and there is no benefit to adding an angiotensin receptor blocker. (C) Cardiac resynchronization therapy (CRT) may be considered in heart failure patients with dyssynchrony between the left and right ventricles, and therefore criteria for CRT includes a QRS duration >120 ms. This patient does not meet criteria. (D) Hydralazine and nitrates are used as alternatives to ACE inhibitors and ARBs if the patient cannot tolerate one of these agents. Hydralazine and nitrates are especially beneficial for African Americans, but this patient is Caucasian.
A 42-year-old man presents to the hospital with worsening leg swelling and exertional dyspnea. He denies any recent illness, fevers, weight loss, cough, chest pain, or abdominal pain. His medical history is significant for Hodgkin lymphoma at the age of 20, and he has been in remission after treatment with chemotherapy and radiation. He does not smoke or drink alcohol, and he denies any recent travel or new environmental exposures. His vitals are normal, and there is no scleral icterus or cervical lymphadenopathy. The jugular venous pressure is elevated and increases further during inspiration. Heart sounds are distant with no S3, and the lungs are clear bilaterally. There is hepatomegaly and pitting edema of the lower extremities. A chest x-ray shows a normal heart border without any pulmonary infiltrates. An echocardiogram is performed that shows normal wall thickness with no significant pericardial effusion.
Which of the following is the most likely diagnosis?
Constrictive pericarditis. Patients with previous radiation therapy to the chest are at risk for constrictive pericarditis, which is a result of thickening and decreased compliance of the pericardium. Any cause of acute pericarditis can progress to constrictive pericarditis. These patients present with symptoms of right-sided heart failure and Kussmaul sign, which is a paradoxical increase in jugular venous pressure during inspiration. (C) Kussmaul sign can also be seen in restrictive cardiomyopathy; however, the echocardiogram did not show an increase in myocardial wall thickness and therefore the more likely diagnosis is constrictive pericarditis. (A) Pulmonary hypertension may cause right-sided heart failure but would not present with Kussmaul sign. (B) There are many causes of dilated cardiomyopathy, including radiation exposure; however, Kussmaul sign should lead the reader to think of constrictive pericarditis. In addition, the chest x-ray did not show an enlarged heart.
A 73-year-old man with severe aortic stenosis presents to the hospital after passing a moderate amount of blood per rectum. Over the past few years, he has noticed easy bruising and frequent nose bleeds. He has no history of a bleeding disorder, and has no family history of such conditions. He does not take any medications. His vitals are normal, and the examination is unremarkable except for the murmur of aortic stenosis. Laboratory values are drawn and show a hemoglobin level of 11 g/dL and a normal prothrombin time (PT) and partial thromboplastin time (PTT). Factor VIII levels are decreased.
What is the most likely explanation for the episodes of bleeding in this patient?
Defect in platelet adhesion. Patients with severe aortic stenosis are at risk for von Willebrand disease (vWD). This is caused by a qualitative deficiency of von Willebrand factor (vWF), which functions in platelet adhesion during coagulation. The name of this condition is Heyde syndrome, and the most dangerous complication is bleeding in the GI tract from angiodysplasias. In severe aortic stenosis, the shear forces on the blood passing through the stenotic valve cause proteolysis of vWF, making it ineffective. vWF also functions to carry factor VIII in the plasma, which is degraded rapidly if not bound to vWF. Therefore, patients with vWD will present with bleeding from skin and mucosal surfaces and low levels of factor VIII; PTT may or may not be prolonged, and PT will be normal. Diagnosis can be made by first measuring vWF antigen (quantitative assay), vWF activity (ristocetin cofactor, a qualitative assay), and factor VIII levels. Confirmation can be made with more specialized tests, such as vWF multimer analysis.
(A) Although these patients will have factor VIII deficiency, PTT is not always prolonged and the cause of their prolonged bleeding is due to platelet adhesion, not clotting factor deficiency. (B) Some patients may have mildly decreased platelet levels, but the abnormal bleeding is due to problems in platelet adhesion (not platelet count). (D) Inhibition of antithrombin III is the mechanism of heparin. (E) There is nothing to suggest malnutrition in this patient, and the patient is not taking warfarin, which inhibits vitamin K epoxide reductase and decreases functional vitamin K, which is responsible for activating clotting factors II, VII, IX, and X.
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