April 2023 Medical Image of the Month: Atrial Myxoma in the setting of Raynaud’s Phenomenon: Early Echocardiography and Management of Thrombotic Disease

Figure 1. ECG demonstrating sinus bradycardia and T-wave inversion in lead III and aVF.

Figure 2.  Transthoracic echo apical four-chamber view (zoomed) demonstrating 2.3 x 2.6 cm echogenic mass of the left atrium. LV = left ventricle. RA = right atrium. LA = left atrium.

Figure 3.  Transesophageal echo, midesophageal long axis view demonstrating 2.5 x 1.71 cm echogenic left atrial mass attached to upper dome of the left atrium. LA = left atrium. LV = left ventricle.

A 43-year-old woman presents to the Emergency Department (ED) with right-sided weakness and numbness for several hours. Medical history is significant for Raynaud’s Phenomenon (RP), initially presenting six months prior to presentation, manifesting as intermittent episodes of painless discoloration of multiple fingers.  Cardiac exam was unremarkable with regular rhythm and no discernable murmur. Neurological exam demonstrated right arm pronator drift. Other examination findings were unremarkable. Labs demonstrated a troponin of 0.00 ng/mL, C-reactive protein of 2.28 mg/dL, and an erythrocyte sedimentation rate of 40 mm/hr. The electrocardiogram (ECG) demonstrated sinus bradycardia and notable for T-wave inversion in lead III and aVF, but without any ST-segment deviations (Figure 1).  Magnetic Resonance Imagining (MRI) of the brain demonstrated acute ischemic left frontal, left parietal, and right parietal infarcts along with mild subcortical left parietal infarct, concerning for venous or watershed distal embolic arterial infarct. MRI Angiogram of the brain showing diminutive bilateral, lateral transverse dural venous sinuses, consistent with thrombus. The patient’s neurological deficits resolved within five hours of ED arrival. Given the background diagnoses of RP and new thrombosis, a complete autoimmune and hypercoagulability workup was pursued and was otherwise negative.

As part of acute stroke work-up, the patient also underwent transthoracic echocardiogram (TTE) with a bubble study, which was significant for left atrial (LA) echogenic intracardiac structure attached to the superior part of the LA (Figure 2). Transesophageal Echocardiogram (TEE) was performed which demonstrated a large, 2.5 x 1.71 cm mass, consistent with an atrial myxoma, not appearing to involve the interatrial septum but instead thought to originate from the upper dome of the atrium immediately adjacent to the pulmonary veins (Figure 3). Patient was also evaluated by neurology and started on anticoagulation with parental continuous unfractionated heparin infusion given the dural venous sinus thrombosis and a possible hypercoagulable state due to the underlying myxoma. Patient underwent surgical resection of the atrial mass Histopathological examination of the resected mass was consistent with the diagnosis of atrial myxoma.

Although atrial myxomas are the most common primary cardiac tumor, clinical presentation ranges from incidental imaging findings to profound life-threading cardiovascular manifestations (1). This range of presentation is closely associated with size, mobility, and location (2). Pinede et al. studied 112 cases of atrial myxomas and reported that signs of cardiac obstruction were the primary manifestation of LA myxoma. Approximately, 67% of patients presented with signs of cardiac obstruction, such as heart failure, syncope, or myocardial infarction, while embolic signs were only present in 29% of patients. Systemic signs including fever and weight loss were only reported in 34% of patients with only 5% of patients having associated connective tissue disease (3). Rarely, RP has been described as the primary presenting symptom of atrial myxoma (4,5), underscoring the utility of maintaining a high degree of suspicion when symptomatology coexists.

RP is a vascular response to stress or cold temperature that appears as color changes in the digits (6). Although primary RP has no known underlying etiology, it is more commonly seen in female patients with a history of smoking, migraine headaches, and cardiovascular disease (6). This is in contrast to secondary RP, which presents in patient with an underlying autoimmune rheumatic disease including, but not limited, to Systemic Sclerosis, Mixed Connective Tissue Disease, Systemic Lupus Erythematosus, Sjogren’s Syndrome, and hematologic disorders such as Cryoglobulinemia, Cold Agglutinins Disease, and Paraproteinemia (7).

Atrial myxoma may rarely make its initial appearance under the guise of RP (4). This phenomenon is likely attributable to overproduction of IL-6 by the myxoma (9-11). Our patient presented with RP six months prior to her presentation to the ED with right-sided weakness and numbness and a complete autoimmune and hypercoagulability workup was negative; this may suggest that the underlying pathophysiology of her RP is the associated overproduction of IL-6 by the atrial myxoma.

TTE may be considered in the initial diagnostic evaluation of a patient presenting with RP without additional findings suggestive of secondary etiologies. Given that myxomas are typically localized within the atrial lumen, transthoracic echocardiography is a highly sensitive modality for diagnosis, whereas CT and MRI may also help in diagnostics in uncertain cases. Once suspicion of a cardiac myxoma has been supported by imaging modalities, surgical removal of the tumor should be performed as soon as possible due to the risk of myxoma associated embolic episodes (5).  Post intervention, long term prognosis is excellent with an approximated 5% rate of recurrence (3). Long-term follow-up with serial TTE are recommended, particularly in younger patients (3) but there is no specific guideline regarding the frequency of TTE surveillance post atrial myxoma resection.

Ali A. Mahdi MD, Chris Allahverdian MD, Vishal Patel MD, Serap Sobnosky MD

Dignity Health, St. Mary Medical Center, Department of Internal Medicine, Long Beach, CA

References

1. Roberts WC. Primary and secondary neoplasms of the heart. Am J Cardiol. 1997 Sep 1;80(5):671-82. [CrossRef] [PubMed]
2. Zaher MF, Bajaj S, Habib M, Doss E, Habib M, Bikkina M, Shamoon F, Hoyek WN. A giant left atrial myxoma. Case Rep Med. 2014;2014:819052. [CrossRef]
3. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). 2001 May;80(3):159-72. [CrossRef] [PubMed]
4. Skanse B, Berg No, Westfelt L. Atrial myxoma with Raynaud's phenomenon as the initial symptom. Acta Med Scand. 1959 Jul 25;164:321-4. [CrossRef] [PubMed]
5. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996 Jan 1;77(1):107. [CrossRef] [PubMed]
6. Abdulla, M. C., & Alungal, J. (2015). Atrial myxoma in a primigravida presenting as Raynaud’s phenomenon. Rheumatology Reports, 7(1). [CrossRef]
7. Prete M, Favoino E, Giacomelli R, et al. Evaluation of the influence of social, demographic, environmental, work-related factors and/or lifestyle habits on Raynaud's phenomenon: a case-control study. Clin Exp Med. 2020 Feb;20(1):31-37. [CrossRef] [PubMed]
8. Khouri C, Blaise S, Carpentier P, Villier C, Cracowski JL, Roustit M. Drug-induced Raynaud's phenomenon: beyond β-adrenoceptor blockers. Br J Clin Pharmacol. 2016 Jul;82(1):6-16. [CrossRef] [PubMed]
9. Jourdan M, Bataille R, Seguin J, Zhang XG, Chaptal PA, Klein B. Constitutive production of interleukin-6 and immunologic features in cardiac myxomas. Arthritis Rheum. 1990 Mar;33(3):398-402. [CrossRef] [PubMed]
10. Saji T, Yanagawa E, Matsuura H, Yamamoto S, Ishikita T, Matsuo N, Yoshirwara K, Takanashi Y. Increased serum interleukin-6 in cardiac myxoma. Am Heart J. 1991 Aug;122(2):579-80. [CrossRef] [PubMed]
11. Parissis JT, Mentzikof D, Georgopoulou M, Gikopoulos M, Kanapitsas A, Merkouris K, Kefalas C. Correlation of interleukin-6 gene expression to immunologic features in patients with cardiac myxomas. J Interferon Cytokine Res. 1996 Aug;16(8):589-93. [CrossRef] [PubMed]

Cite as: Mahdi AA, Allahverdian C, Patel V, Sobnosky S. April 2023 Medical Image of the Month: Atrial Myxoma in the setting of Raynaud’s Phenomenon: Early Echocardiography and Management of Thrombotic Disease. Southwest J Pulm Crit Care Sleep. 2023;26(4):56-58. doi:https://doi.org/10.13175/swjpccs006-23 PDF

Medical Image of the Month: Idiopathic Right Lower Lobe Pulmonary Vein Thrombus

Figure 1. CT angiogram chest sagittal view: showing low density filling defect consistent with pulmonary vein thrombus (yellow arrow).

Figure 2. A: CT angiogram chest axial view showing right lower lobe pulmonary vein thrombus. B: the vein (red arrow) is well differentiated by his lower contrast than the adjacent artery (blue arrows).

A 62-year-old man with a medical history notable only for a seasonal allergy, presented to the emergency department with complaints of shortness of breath with productive cough for 2 months which were worsening for the last 2 weeks. CTA chest revealed low density filling defect in the RLL vein consistent with RLL vein thrombus (Figures 1 and 2). After a comprehensive work up to rule out malignancy and hypercoagulable disorders, a diagnosis of idiopathic pulmonary vein thrombosis was made. The patient received heparin and was discharged with rivaroxaban.

Pulmonary vein thrombosis is a rare disease but can be fatal, usually patient presents with non-specific symptoms such as cough and shortness of breath (1). The etiology in most of cases is hypercoagulable disorders, malignancies, atrial fibrillation, post lung operations such as lobectomy and lung transplantation, or could be idiopathic as in our patient.

Timothy Jon Rolle MD¹ and Mohammad Abdelaziz Mahmoud MD, DO^2
1Department of Radiology and the ²Internal Medicine Residency

Midwestern University Arizona College of Osteopathic Medicine

Canyon Vista Medical Center
Tucson, AZ USA

Reference

1. Chaaya G, Vishnubhotla P. Pulmonary vein thrombosis: a recent systematic review. Cureus. 2017 Jan 23;9(1):e993. [CrossRef] [PubMed]

Cite as: Rolle TJ, Mahmoud MA. Medical image of the month: idiopathic right lower lobe pulmonary vein thrombus. Southwest J Pulm Crit Care. 2020;20(1):7-8. doi: https://doi.org/10.13175/swjpcc048-19 PDF

Medical Image of the Week: Paget-Schroetter Syndrome

Figure 1. Venous contrast study demonstrating thrombosis and flow obstruction at the thoracic outlet (arrow). 

A 22-year-old right-handed man developed acute swelling of his right upper extremity following a weekend of vigorous physical military training. There was no associated pain or numbness. Physical examination demonstrated edema of the right arm. Radial and ulnar pulses were intact, and neurological exam was normal. Venous doppler examination demonstrated thrombus in the subclavian-axillary venous system. A confirmatory venous contrast study was performed (Figure 1), followed by catheter directed lysis.  Effort related thrombosis of the subclavian vein secondary to mechanical compression at the thoracic outlet is known as Paget-Schroetter Syndrome (1). Current treatment commonly includes catheter directed clot lysis and an arbitrary three-month period of systemic anticoagulation, followed by surgical resection of the first rib (2). Post-operative balloon angioplasty of the involved venous segment improves long-term results (3). This patient underwent successful lysis, was discharged on oral rivaroxaban, and has been referred to thoracic surgery for consideration of rib resection.

Charles Van Hook MD and Ken Hirasaki MD

Longmont United Hospital

Longmont, Colorado USA

References

1. Kucher N. Deep-vein thrombosis of the upper extremities. N Engl J Med. 2011;364:861-9.[CrossRef] [PubMed]
2. Engelberger RP, Kucher N. Management of deep vein thrombosis of the upper extremity. Circulation. 2012;126:768-73. [CrossRef] [PubMed]
3. Illif KA, Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg. 2010;51:1538-47. [CrossRef] [PubMed]

Cite as: Van Hook C, Hirasaki K. Medical image of the week: Paget-Schroetter syndrome. Southwest J Pulm Crit Care. 2018;16(3):156. doi: https://doi.org/10.13175/swjpcc031-18 PDF 

Medial Image of the Week: Purpura Fulminans

Figure 1.  A: Purpura fulminans. B: Close up view of the left lower extremity.

A 54-year-old man with coronary artery disease, fibromyalgia and chronic sacral ulcers was brought to the emergency department due to acute changes in mentation and hypotension. He suffered a cardiac arrest shortly after arrival to the emergency department during emergent airway management. After successful resuscitation, he was admitted to the medical intensive care unit and treated for septic shock with fluid resuscitation, vasopressors and broad spectrum antibiotics. Laboratory results were significant for disseminated intravascular coagulopathy (DIC)- thrombocytopenia, decreased fibrinogen and elevated PT, PTT and D-dimer levels. Profound metabolic acidosis and lactate elevation was also seen. Blood Cultures later revealed a multi-drug resistant E. coli bacteremia. Images of the lower extremities (Figure 1) were obtained at initial assessment and are consistent with purpura fulminans. He did not survive the stay.

Purpura fulminans, also referred to as skin mottling, is an evolving skin condition which is characterized by an acutely worsening reticular pattern of ecchymosis, tissue thrombosis and eventual hemorrhagic skin necrosis. Traditionally associated with either an inherited and/or acquired protein C deficiency, it is more commonly seen in DIC. It is generally considered a poor prognostic indicator when associated with DIC. In our patient, the DIC was secondary to septic shock. When encountered in this clinical scenario it should be considered an acute life threatening emergency.

Emilio Perez Power MD¹, Norman Beatty MD¹ and Bhupinder Natt MD²

¹Department of Internal Medicine and ²Division of Pulmonary, Allergy, Critical Care and Sleep

Banner-University Medical Center South Campus

University of Arizona

Tucson, AZ USA

Cite as: Power EP, Beatty N, Natt B. Medical image of the week: purpura fulminans. Southwest J Pulm Crit Care. 2016;13(6):307-8. doi: https://doi.org/10.13175/swjpcc129-16 PDF

Medical Image of the Week: Superior Vena Cava Syndrome

Figure 1. Thoracic CT in soft tissue windows. Panels A, B, C and G show extensive collateral circulation along the right chest wall especially subcutaneous vessels and subcapsular hepatic vessels during contrast injection in the right arm (arrows). There are also prominent right hepatic and capsular enhancing vessels with vascular shunt within the posterior subcapsular right hepatic lobe. Panels D, E, F and I show extensive collateral circulation on the left side when the contrast is injected (on a different admission) in the left arm (arrows). Panel H shows absent blood flow in the totally thrombosed SVC stent.

Superior vena cava (SVC) syndrome results from obstruction of blood flow in the SVC. Most cases are secondary to malignancy, the most common being lung cancer or non-Hodgkin lymphoma. Other less encountered etiologies include fibrosing mediastinitis and thrombosis associated with intravascular devices (1,2). Here, we present a case of advanced lung cancer undergoing chemo-radiation therapy who presented with typical symptoms of SVC syndrome including progressive shortness of breath and facial swelling/ fullness over weeks to months. His chest CT scan showed SVC obstruction due to his tumor mass (Figure 1). The patient underwent stenting and improved partially for sometime. However, he returned again with worsening symptoms over a few weeks with discovery of SVC in-stent thrombosis. He was started on therapeutic enoxaparin and his symptoms improved partially with time.

Huthayfa Ateeli, MBBS¹, Kawanjit Sekhon, MD² and Dena K. L'Heureux, MD³.

¹Department of Medicine, Division of Pulmonary, Critical Care, Sleep and Allergy Medicine; ²Department of Medicine, Internal Medicine Residency Program, Main Campus; and ³Department of Medicine, Division of Pulmonary, Critical Care, Sleep and Allergy Medicine, University of Arizona, Southern Arizona VA Health Care System, Tucson, AZ USA

References

1. Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007 May 3;356(18):1862-9. [CrossRef] [PubMed] 
2. Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore). 2006 Jan;85(1):37-42. [CrossRef] [PubMed] 

Cite as: Ateeli H, Sekhon K, L'Heureux DK. Medical image of the week: superior vena cava syndrome. Southwest J Pulm Crit Care. 2016;13(2):99-100. doi: http://dx.doi.org/10.13175/swjpcc065-16 PDF

Medical Image of the Week: Vascular Occlusion

Figure 1. Left arm angiogram showing complete occlusion of ulnar artery (red arrow).

Figure 2. Angiogram showing left radial artery complete occlusion (red arrow).

Figure 3. Left hand with necrotic fingers.

The patient is a 39 year-old woman with no significant past medical history presenting with progressive left hand pain for five days. The patient denied a history of Raynaud’s phenomenon or clotting disorders. She had no radial pulse on presentation and angiogram showed severe complete occlusion of the radial and ulnar arteries (Figures 1 and 2). She had an initial partial response with intra-arterial verapamil and nitroglycerin but her hand ischemia did not improve on heparin or with intra-arterial tissue plasminogen activator. Autoimmune and coagulation work-ups were negative. Her left hand finger necrosis at time of discharge is shown (Figure 3).  Further evaluation is ongoing for coagulation disorders.

Allison Shapiro MD, Carmen Luraschi-Monjagatta MD, Matthew Schreiber MD.

Department of Internal Medicine, Pulmonary and Critical Care, University of Nevada School of Medicine, Las Vegas, NV

Reference as: Shapiro A, Luraschi-Mongagatta C, Schreiber M. Medical image of the week: vascular occlusion. Southwest J Pulm Crit Care. 2014;9(1):36-7. doi: http://dx.doi.org/10.13175/swjpcc093-14 PDF

Medical Image of the Week: May-Thurner Syndrome

Figure 1. Figure A: Venogram showing extensive thrombosis of the left common iliac vein. Thrombus appearing as filling defects (arrows). Patient is in prone position. Figure B: Venogram after catheter-directed pharmaco-mechanical thrombectomy and stent placement (2). Inferior vena cava filter in place (1). Patient is in prone position. Figure C: Venogram after catheter-directed pharmaco-mechanical thrombectomy and stent (arrow) placement showing improved venous flow. Patient is in prone position.

A 20-year-old Caucasian female presented with 7-day history of pain and swelling of the left lower extremity. She had no significant past medical history. Her only medication at the time of presentation was oral contraceptive pills. She denied smoking cigarettes. She denied shortness of breath, recent travel, surgery or miscarriage. She did not have any family history of clotting problems. She was hemodynamically stable. Physical examination was significant for swelling of the left lower extremity up to mid-thigh level. Duplex ultrasonography of the extremity showed extensive thrombosis of the left ilio-femoral, common femoral and popliteal veins. A retrievable inferior vena cava filter was placed. Subsequently, she underwent catheter directed thrombolysis and percutaneous mechanical thrombectomy.  Venogram, after the procedure showed resolution of most of the clot burden. It also revealed a band-like stenosis at the location where the right iliac artery is expected to cross the left iliac vein, consistent with May-Thurner syndrome. Endovascular stenting was done. Following the placement of stent venous flow improved significantly. The inferior vena cava filter was removed about a week later. She completed 6 months of anticoagulation with warfarin. Screening for hypercoagulable state, including protein C and S level, antithrombin III level, homocysteine level, anti-phospholipid antibody, factor V Leiden mutation and prothrombin gene mutation was negative.

May-Thurner syndrome is an anatomical variation of the left common iliac vein that increases the risk of deep venous thrombosis of the left lower extremity. It is caused by the compression of the left iliac vein by the right iliac artery against the fifth lumbar vertebra, where it crosses over the vein. Chronic pulsation of the artery against the vein causes vascular thickening. Patients are usually females and commonly present in their second to fourth decades of life. The estimated prevalence is about 22% in the general population. So, it should be suspected when younger females present with extensive, proximal deep venous thrombosis of the left lower extremity. Patients are at increased risk of recurrent thrombosis which can be prevented by correction of the anatomical lesion.

Sathish Krishnan MD, Malav Parikh MD, Dima Dandachi MD

Department of Internal Medicine

Saint Francis Hospital

Evanston, IL

References

1. Kalu S, Shah P, Natarajan A, Nwankwo N, Mustafa U, Hussain N.. May-Thurner Syndrome: A Case Report and Review of the Literature. Case Rep Vasc Med. 2013; 2013:740182. [CrossRef] [PubMed]
2. Peters M, Syed RK, Katz M, Moscona J, Press C, Nijjar V, Bisharat M, Baldwin D. May-Thurner syndrome: a not so uncommon cause of a common condition. Proc (Bayl Univ Med Cent) ; 25(3):231-3. [PubMed]

Reference as: Krishnan S, Parikh M, Dandachi D. Medical image of the week: May-Thurner syndrome. Southwest J Pulm Crit Care. 2014;9(1):25-26. doi: http://dx.doi.org/10.13175/swjpcc066-14 PDF