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Year : 2019  |  Volume : 9  |  Issue : 2  |  Page : 105-107

An unusual presentation of acute pulmonary embolism secondary to upper extremity deep vein thrombosis

Department of Cardiology, Lakshmi Hospital, Palakkad, Kerala, India

Date of Web Publication23-Sep-2019

Correspondence Address:
Dr. Khadhar Mohamed Sarjun Basha
Department of Cardiology, Lakshmi Hospital, Palakkad - 678 013, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JICC.JICC_14_19

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Acute pulmonary embolism (PE) secondary to congenital venous malformation of the upper limb is a rare clinical entity. Clinical manifestation may range from vague shoulder discomfort to features of superior vena cava or thoracic outlet syndrome. We report a case of a 43-year-old adult patient who was referred as acute coronary syndrome and on evaluation was diagnosed as acute PE secondary to upper extremity deep vein thrombosis. Magnetic resonance venogram of the right upper limb revealed congenital venous malformation with thrombus as the cause for deep vein thrombosis.

Keywords: Deep vein thrombosis, pulmonary embolism, venous malformation

How to cite this article:
Sarjun Basha KM, Jayagopal P B. An unusual presentation of acute pulmonary embolism secondary to upper extremity deep vein thrombosis. J Indian coll cardiol 2019;9:105-7

How to cite this URL:
Sarjun Basha KM, Jayagopal P B. An unusual presentation of acute pulmonary embolism secondary to upper extremity deep vein thrombosis. J Indian coll cardiol [serial online] 2019 [cited 2021 May 17];9:105-7. Available from:

  Introduction Top

Acute pulmonary embolism (PE) secondary to congenital venous malformation of the upper limb is a rare entity. PE secondary to upper extremity deep vein thrombosis (UEDVT) occurs in one-third of patients.[1] We report an adult patient who was referred as acute coronary syndrome (ACS) and on subsequent evaluation proved to be acute PE secondary to UEDVT.

  Case Report Top

A 43-year-old male was presented with a history of sudden-onset retrosternal chest pain associated with sweating and breathlessness. He was detected to have tachycardia, hypotension, ischemic changes in the electrocardiogram (ECG), and elevated troponin and referred as ACS for further management. On physical examination, he was obese and tachypneic. Pulse rate was 170–180/min, irregularly irregular; blood pressure was 90/70 mmHg. Cardiovascular system examination revealed tachycardia with variable first heart sound. Respiratory system was normal, and the abdomen was soft with no focal neurological deficits. The ECG showed atrial fibrillation (AF) with fast ventricular rate, and chest X-ray revealed cardiomegaly with congested lung fields. Two-dimensional echocardiogram showed normal left ventricular systolic function, dilated right atrium, right ventricle (RV), mild-to-moderate RV dysfunction, and moderate pulmonary hypertension. Cordarone 150 mg intravenous (IV) bolus was administered and it reverted AF to sinus rhythm. Due to persistent chest pain, emergency coronary angiogram was done which revealed ectatic coronaries with slow flow. He continued to have hypotension needing inotropic support and had episodes of breathlessness and sweating. In view of dilated right heart on echo, possibility of PE was considered.

D-dimer sent was >15,000 ng/ml. Hemogram, renal function test, liver function test, and thyroid function tests were normal. A search for a focus of DVT showed normal lower limbs and a hugely dilated swelling in the right arm [Figure 1]. On detailed interrogation, it was clear that this was present ever since he could remember with episodes of pain and swelling which he attributed to his occupation of auto rickshaw driving. He had past history of episodes of acute- onset breathlessness and giddiness whenever he use to squeeze the swelling due to pain. Palpation of the swelling at bedside suddenly made him breathless with tachycardia and profuse sweating. Thus, a clinical diagnosis of acute PE from upper limb was made. Venous Doppler of the right upper limb was suggestive of venous malformation in the right arm vein with thrombus. Computed tomogram pulmonary angiography (CTPA) revealed pulmonary emboli involving right and left pulmonary artery [Figure 2]c. Magnetic resonance venogram of the right upper limb was suggestive of multifocal low-flow venous malformation in the right arm with subcutaneous, intramuscular components and ectatic brachial vein and basilic vein segments in the mid-arm with thrombus in ecstatic basilic vein [Figure 2]a and [Figure 2]b. The patient was treated with unfractionated heparin 5000 units IV bolus followed by 1000 units/h and titrated to activated partial thromboplastin time 2–3 times of control. Oral anticoagulant warfarin was started and continued to keep the international normalized ratio (INR) between 2 and 3. He had no further episodes of breathlessness and remained clinically stable. He is planned for elective surgical ligation and exclusion of venous malformation of the right arm.
Figure 1: Clinical image showing swelling (⟶) in the right arm

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Figure 2: Magnetic resonance venogram – (a) axial view, (b) coronal contrast view showing venous malformation (△) with thrombus (*) in the ectatic dilatation in basilic vein. Computed tomography pulmonary angiogram (CTPA) (c) showing multifocal thrombi (*) in the pulmonary arteries

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  Discussion Top

PE occurs in one-third of patients with UEDVT.[1] Based on pathogenesis, UEDVT can be classified as primary or secondary. Primary UEDVT is rare and occurs due to idiopathic UEDVT or Paget–Schroetter syndrome. Secondary UEDVT developing in patients with central venous catheters, pacemaker leads, or malignancy contributes to many cases of UEDVT. Paget–Schroetter syndrome occurs due to microtrauma to the vessel intima usually after strenuous activity and leads to activation of coagulation cascade with subsequent thrombosis. Idiopathic UEDVT mostly is associated with occult cancers. The presenting symptoms and signs may range from vague shoulder discomfort to manifestations of superior vena cava (SVC) syndrome or thoracic outlet syndrome. Our patient presented with acute PE with swelling of the right arm. The common differential diagnosis would be lymphedema, extravascular compression of blood vessels, and muscle injury. Hence, imaging is always needed to confirm the diagnosis.

The initial test used for diagnosing UEDVT is Doppler ultrasound which is noninvasive with high sensitivity and specificity for peripheral UEDVT[1] but not for central thrombus that is directly below the clavicle. Contrast venography provides excellent anatomy of the veins but with the drawback of contrast-induced reaction.

Magnetic resonance angiography (MRA) is a more accurate noninvasive method that detects central thrombus and provides detailed evaluation of central collaterals and blood flow. Hence, it is most commonly preferred for diagnosing UEDVT, especially when contrast venography is impossible. Our patient initially underwent Doppler ultrasound of the right upper extremity which revealed venous malformation in arm vein with thrombus followed by CTPA which revealed evidence of PE. MRA, however, defined the underlying anatomic abnormality responsible for UEDVT.

Treatment of UEDVT ranges from initial medical stabilization followed by corrective treatment of underlying disorder to prevent recurrence. Anticoagulation is the mainstay of therapy which maintains patency of venous collaterals and also reduces thrombus propagation even if the clot does not completely resolve. Oral Vitamin K antagonist with goal INR of 2.0–3.0 or newer oral anticoagulant may be started. Unfractionated heparin is used as a bridge to oral anticoagulation. Thrombolysis restores early patency of veins and reduces the risk of complication, namely postthrombotic syndrome.[2],[3],[4] Catheter-directed thrombolysis reduces the risk of serious bleeding when compared with systemic thrombolysis with higher rates of clot resolution. However, thrombolysis is successful only within 14 days of symptoms onset.

Percutaneous mechanical thrombectomy is often used in combination with thrombolytic therapy which reduces the dose of thrombolytic therapy by extracting large quantities of thrombus. Surgery involves correction of cause responsible for vein compression, namely resection of part of the first rib or clavicle in thoracic outlet syndrome. Our patient is planned for corrective surgery for venous malformation. Venography done following surgery can assess residual stricture which can be treated with balloon venoplasty, and if it fails, vein stenting can be done. SVC filter can be considered in patients with contraindication to anticoagulation or who develops PE despite adequate anticoagulation although the risk of SVC filter outweighs the benefits. This case is reported in view of the unusual rare presentation and to highlight the importance of detailed history-taking and meticulous clinical examination.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Prandoni P, Polistena P, Bernardi E, Cogo A, Casara D, Verlato F, et al. Upper-extremity deep vein thrombosis. Risk factors, diagnosis, and complications. Arch Intern Med 1997;157:57-62.  Back to cited text no. 1
Hicken GJ, Ameli FM. Management of subclavian-axillary vein thrombosis: A review. Can J Surg 1998;41:13-25.  Back to cited text no. 2
Aburahma AF, Sadler DL, Robinson PA. Axillary subclavian vein thrombosis. Changing patterns of etiology, diagnostic, and therapeutic modalities. Am Surg 1991;57:101-7.  Back to cited text no. 3
Urschel HC Jr., Razzuk MA. Paget-Schroetter syndrome: What is the best management? Ann Thorac Surg 2000;69:1663-8.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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