Cases from the University of Texas at Austin


Case 1: Pericardial tamponade due to multiple myeloma

By Anam Shaikh, MD, ACP Resident/Fellow Member, and Alejandro Moreno, MBBS, MPH, JD, FACP

The patient

A 75-year-old man presented with 1 month of increasing dyspnea and peripheral edema. He had been diagnosed with an isolated plasmacytoma of the left orbit 2 years previously, which was treated with dexamethasone and radiation therapy. One year ago, the patient relapsed, with involvement of the sternum, vertebrae, and the lumbar paraspinal soft tissues. He had completed a course of bortezomib and dexamethasone 6 months prior to admission with good response; however, he did not complete radiation therapy due to incarceration.

EKG demonstrating electrical alternans, suggestive of tamponade physiology
EKG demonstrating electrical alternans, suggestive of tamponade physiology.

On physical exam, the patient appeared to be in severe distress; his respiratory rate was 19 breaths/min, his heart rate was 138 beats/min, and blood pressure was 117/79 mm Hg (below his hypertensive baseline). The patient had elevated jugular venous pressure (JVP), distant heart sounds, distended neck veins, and 2+ lower-extremity edema. Lungs were clear to auscultation. Laboratory testing revealed mild pancytopenia and abnormal renal function. A chest X-ray showed an enlarged cardiac silhouette with vascular cephalization, but no marked pulmonary edema. The electrocardiogram (EKG) was noteworthy for sinus rhythm with low voltage and electrical alternans (Figure 1).

Transthoracic echocardiogram showing large pericardial effusion (arrows)
Transthoracic echocardiogram showing large pericardial effusion (arrows).
Pericardial fluid cytology (Quik-Diff stain) showing atypical plasmacytoid cells (arrows)
Pericardial fluid cytology (Quik-Diff stain) showing atypical plasmacytoid cells (arrows).

Urgent transthoracic echocardiography confirmed the presence of a large pericardial effusion with evidence of tamponade (Figure 2). Pericardiocentesis yielded 1,700 mL of serosanguineous fluid. CT scanning showed a bulky right-sided pelvic mass, right hydronephrosis, and diffuse pericardial nodularities. Cytology of the pericardial fluid showed atypical plasmacytoid cells (Figure 3). The patient received dexamethasone, 40 mg, for 5 days and was discharged with follow-up to start second-line chemotherapy and radiation.

The diagnosis

This patient had a malignant pericardial effusion with tamponade as the result of recurrent multiple myeloma. The presence of Beck's triad (hypotension, jugular venous distention, and muffled heart sounds) on presentation was suggestive of cardiac tamponade. Forty percent of all pericardial effusions are due to a malignancy, with lung, breast, lymphoma, and leukemia accounting for 75% of malignant effusions. Pericardial involvement occurs in fewer than 1% of patients with multiple myeloma. A majority of patients with pericardial involvement in multiple myeloma (60%) are women, are older than 65 years, and present with tamponade. These patients are also known to have reduced survival, which on average is 13 weeks after the effusion is discovered.

The immediate treatment of the cardiac tamponade is the same as for other etiologies: emergent pericardiocentesis. The surgical creation of a pericardial window is also an effective management option for malignant pericardial effusion, although for many patients, severity of illness and poor prognosis may preclude this intervention.

Pearls

  • Forty percent of all pericardial effusions are malignant, with lung and breast cancer, leukemia, and lymphoma accounting for most cases.
  • A majority of patients with pericardial involvement in multiple myeloma are women, are older than 65 years, and present with tamponade.

Case 2: Postpartum spontaneous coronary artery dissection

By Sandia Iskandar, MD, ACP Member; Sameeksha Bhama, MD, ACP Resident/Fellow Member; Caitlin Geisler, MD; and Alejandro Moreno, MBBS, MPH, JD, FACP

The patient

EKG showing diffuse T-wave inversion and ST-segment changes in the precordial leads
EKG showing diffuse T-wave inversion and ST-segment changes in the precordial leads.

A 37-year-old woman presented with sudden onset of substernal chest pain 8 days after an uncomplicated cesarean delivery. The physical examination was unremarkable: JVP was not elevated, there was no heart murmur, point of maximal impulse was nondisplaced, and peripheral edema was absent. Her initial EKG was normal, and the troponin I level was mildly elevated at 0.07 ng/mL (normal value <0.05 ng/mL). Following admission, the patient's chest pain persisted and her subsequent troponin level was elevated at 40.62 ng/mL. A repeated EKG revealed new T-wave inversions and ST-segment changes in the precordial leads (Figure 4).

Angiography demonstrating right coronary dissection (arrows)
Angiography demonstrating right coronary dissection (arrows).

She received standard medical treatment for non-ST-segment elevation myocardial infarction (MI). Cardiac catheterization showed a spontaneous right coronary artery dissection with extension to the posterior-lateral branch, a mild left anterior descending artery stenosis, and an ejection fraction of 50% with apical wall motion abnormality (Figure 5). No percutaneous coronary intervention (PCI) was performed since the patient was hemodynamically stable, and there was Thrombolysis In Myocardial Infarction (TIMI) grade 3 flow throughout the right coronary artery. She received a heparin infusion for 48 hours, followed by aspirin and clopidogrel, as well as a statin and a beta-blocker.

The diagnosis

This patient had a postpartum spontaneous coronary artery dissection (SCAD), a rare condition that can present as acute MI during the peripartum period. It is also associated with fibromuscular dysplasia and inflammatory autoimmune diseases. Most women with peripartum SCAD do not have atherosclerosis or other traditional risk factors for cardiovascular disease. It is estimated that 1 in 16,000 pregnancies is complicated by acute MI. Of all pregnancy-associated SCADs, more than 80% occur in the postpartum period. SCAD is diagnosed by angiography. In women, an estimated 80% of reported SCAD cases have occurred in the left anterior descending artery, whereas right coronary artery compromise is more common in men.

There is no well-established standardized treatment of SCAD, but case series report good outcomes with medical management, such as aspirin, beta-blocker, statin, and angiotensin-converting enzyme inhibitor, even in patients with multivessel involvement. Revascularization with PCI or coronary artery bypass graft is usually reserved for those with recurrent ischemia, left main coronary artery involvement, or hemodynamic instability. The success rate of stent deployment into the dissected area is 65%, although extension of the dissection can occur in 25% of cases. Durable results with PCI have been achieved in only about 30% of cases; close follow-up is important, as the 10-year recurrence rate approaches 20%. Patients with pregnancy-associated SCAD are counseled to avoid future pregnancy.

Pearls

  • Pregnancy-associated SCAD is rare, but when it occurs it is usually in the postpartum period and involves the left anterior descending coronary artery.
  • Medical management is recommended for SCAD unless there is evidence of recurrent ischemia, left main coronary artery involvement, or hemodynamic instability that prompts intervention.

Case 3: Neurogenic T-wave inversions

By Saranjit Kaur, MD, ACP Resident/Fellow Member, and Alejandro Moreno, MBBS, MPH, JD, FACP

The patient

A 40-year-old woman with no significant chronic medical comorbidity was admitted for an elective intracranial aneurysm clipping. Eighteen hours into the postoperative period, the neurosurgical intensive care unit staff noted new T-wave inversions on telemetry. The patient did not have traditional cardiovascular risk factors. On physical examination, she was obtunded without any focal neurological signs; the patient had received small doses of opiates for pain, but no sedatives. Blood pressure was noted to increase from 130/85 mm Hg to 165/95 mm Hg, while heart rate decreased from 85 beats/min to 60 beats/min. No changes in the respiratory rate or oxygen saturation were noted. Cardiac examination was normal. There was no JVD or crackles.

EKG showing diffuse T-wave inversions
EKG showing diffuse T-wave inversions.
EKG showing resolution of T-wave inversions
EKG showing resolution of T-wave inversions.

A 12-lead EKG revealed diffuse T-wave inversions without any other abnormality (Figure 6); no prior EKG was available for comparison. Laboratory testing was unremarkable, including a troponin I level of 0.06 ng/mL. A CT of the head without contrast revealed cerebral edema, an intracranial hematoma, and ventricular effacement. The patient received mannitol and underwent emergent surgical decompression. A postoperative echocardiogram showed normal chamber size and valves and normal wall thickness with an ejection fraction of 55%. An EKG repeated 1 day later demonstrated resolution of the T-wave abnormalities (Figure 7). The patient underwent coronary angiography prior to discharge, which demonstrated nonobstructive coronary artery disease without need for intervention.

The diagnosis

This patient was noted to have diffuse T-wave abnormalities on EKG that were neurogenic in origin. Repolarization abnormalities in neurosurgical patients present a broad differential diagnosis, including the acute intracranial event itself, acute myocardial ischemia, or electrolyte abnormalities. EKG abnormalities can be found in up to 35% of patients with central nervous conditions, such as tumors with intracranial hypertension, intracranial bleeds, strokes, and head trauma. Common EKG abnormalities include sinus bradycardia, T-wave inversions, QT interval abnormalities, and U-waves. Central nervous system-mediated cardiac injury is thought to be primarily due to stress-related cardiomyopathy through 1 of 2 proposed mechanisms: elevated serum catecholamine levels or excitation of the limbic system (amygdala and hippocampus). Animal studies have demonstrated permanent myocardial damage via parenteral administration of catecholamines, likely as a result of the direct toxic effect on myocardium. Alternatively, prolonged activation of the autonomic nervous system has been reported to have a cumulative effect on myocardium.

Pearls

  • Intracranial events such as head trauma, increased intracranial pressure from tumors, hemorrhages, and strokes may cause EKG abnormalities such as T-wave inversions, sinus bradycardia, QT abnormalities, and U-waves.
  • EKG changes associated with central nervous system conditions appear to be the result of either direct myocardial injury from elevated catecholamine levels or direct stimulation of the limbic system.

Case 4: Dysphagia lusoria and Kommerell's diverticulum

By Ryan Sandoval, MD, ACP Resident/Fellow Member; Bakht Cheema, MD, ACP Resident/Fellow Member; and Alejandro Moreno, MBBS, MPH, JD, FACP

The patient

A 66-year-old woman presented to the hospital with progressive dysphagia and dysphonia for 2 months. Her dysphagia was exclusive to solid food and localized to the throat. She reported occasionally regurgitating food immediately after attempting to swallow it. The patient's family also believed that the pitch of her voice had changed. She reported no weight loss, enlarged lymph nodes, fever, chills, shortness of breath, choking, or cough. Her medical history was significant for papillary thyroid carcinoma with a total thyroidectomy 10 years prior. The patient did not smoke.

Physical examination revealed no tonsillar, cervical, supraclavicular, or axillary lymph node enlargement. There were no indurations or masses over the neck or supraclavicular areas; the thyroidectomy scar was discrete, mobile, and not indurated. No stridor was present. The radial, brachial, and ulnar pulses were symmetric and they did not change after maneuvering the arms and the neck. There were no axillary, supraclavicular, or neck bruits.

Portable chest X-ray showing fullness over the aortic arch (arrow)
Portable chest X-ray showing fullness over the aortic arch (arrow).
CT angiogram showing Kommerell's diverticulum (arrow head) and aberrant subclavian artery (arrows)
CT angiogram showing Kommerell's diverticulum (arrow head) and aberrant subclavian artery (arrows).

A chest X-ray showed fullness on the right side of the aortic arch (Figure 8) and a neck CT scan showed only old surgical changes. Chest CT angiography demonstrated an aberrant subclavian artery arising from an aneurysmal aortic outcropping (Figure 9). After a consultation with vascular surgery, the patient opted for dietary modification (soft foods) to manage her dysphagia.

The diagnosis

Dysphagia is a common condition that may have a variety of mechanical, neoplastic, or neurologic causes. This patient's diagnosis is dysphagia lusoria, a rare mechanical compression of the trachea or esophagus due to an aberrant subclavian artery. The prevalence of an aberrant right subclavian artery is estimated to be less than 2%. An aberrant subclavian artery originates at an aneurysmal aortic outcropping, known as a Kommerell's diverticulum, and it may follow 1 of 3 anatomical paths: behind the esophagus toward the contralateral arm (80%), between the esophagus and the trachea (15%), or anterior to the trachea (5%).

Most adult patients (95%) are asymptomatic; however, a progressive dilatation of the Kommerell's diverticulum may lead to compression of the trachea or esophagus causing dysphagia, dyspnea, wheezing, cough, or chest pain. The use of advanced imaging has increased the number of incidentally diagnosed Kommerell's diverticula. Although the best diagnostic modality to identify this condition is CT angiography, superior mediastinal widening can be seen on chest X-ray. Fluoroscopy during a barium swallow may also demonstrate the pulsatile nature of the indentation in the esophagus. Treatment options vary depending on the individual anatomy and include open surgical repair, endovascular stent placement, or both modalities performed simultaneously. Surgery is advisable when the diverticulum exceeds 3 cm or the adjacent aorta measures more than 5 cm in diameter.

Pearls

  • Dysphagia lusoria is a rare mechanical compression of the trachea or esophagus due to an aberrant subclavian artery.
  • Depending on the anatomy of the Kommerell's diverticulum, open vascular surgery, endovascular stent placement, or both are treatment options.