SHA24/021003

What is it ? Is it the disease(s) or the sequalea ?

Mushabab Al-Murayeh, MBBS(hon.), FRCPC, FACC Consultant Cardiologist Clinical Director of Cardiac Services Armed Forces Hospital Southern Region Khamis Mushayt

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Clinical Presentation 19 yrs old male Saudi. c/o SOB for last 7 months increased lately, low grade fever, sweating and weight loss.

Not smoker and no chronic chest problems or muscloskeletal complaints.

Physical examination Looking fair, some facial acne. Normal BP, atrerial pulsations .

O2 Sat in RA= 90%.

First degree fingers clubbing . Body wt = 52 kg, BMI= 18 Skin, joints and lymph nodes; NAD Chest, heart, abd. & CNS exam; unremarkable.

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Chest X-Ray & ECG

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Echocardiography

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

What is the provisional diagnosis?

1. Primary cardiac malignancy (Sarcoma)?

2. Metastasis?

3. Benign tumor (fibroma, myxoma, lipoma)?

4. Cardiac Lymphoma?

5. Thrombus ?

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Course of disease(s) The patient had further investigations done in the other center, cleared from oncology for RV resection While being investigated he had two attacks of PE documented by spiral CT. The histopathology exam showed nonspecific inflammation. He was discharged on Warfarin.

3 month latter he presented to our center with fever for 5 days and SOB. Work up ruled out endocarditis was negative. TTE showed small residual flat layer of RV mass . CT was performed to assess for new PE. After a short course of empirical antibiotics , he was discharged on warfarin.

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

CT

7/2012

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

CT report: a small hypodense filling defect is seen at the lower lobe division of the rt pulm artery indicating pulmonary embolism. A hypodense filing defect is seen in right ventricle likely representing thrombus.

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Course of disease(s) The patient had a new complain for buccal ulcer, he also reported similar complaint before.

He also reported genital ?ulcer lesion and recurrent eye redness over the last year.

After rheumatology consultation Bechet’s Disese was diagnosed. He was started on Azathioprine 50 mg BD and prednisolone 5 mg OD for 2 months.

The azathioprine was increased to 100 mg BD for the last 2 months.

5 mon latter the patient presented again to our center with chest pain and shortness of breathing.

He is also on Warfarin, with good INR follow up.

ESR = 47 . CRP = 96 . D-Dimer = 1.24

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

The International Study Group guidelines for diagnosis of Behรงet's disease: 1. The patient must have oral aphthous ulcers (any shape, size, or number at least 3 times in any 12 months period) 2. Any 2 out of the following 4 hallmark symptoms: a) genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis. b) Skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in postadolescents not on corticosteroids) c) Eye inflammation (iris, uveitis, retinal vasculitis, vitrous cells) d) Pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick).

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

20 13

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

July 2012 February 13 -16, 2013 . SHA24-GHA10

Jan 2013 Armed Forces Hospital Southern Region, Cardiac Services

1. Shall we proceed for mass resection again?

2. Other lines of therapy could be applied. ? meds?

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

TTE after Second Resection of RV mass:

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Cardiac involvement in Behçet’s disease Symptomatic cardiac disease is uncommon. Pericarditis, myocarditis, endocarditis, endomyocardial fibrosis.

Intracardiac thrombosis.

Coronary arteritis ± MI. coronary artery aneurysms, atrial septal aneurysm, conduction system disturbances, ventricular arrhythmias, MVP, and valvular insufficiency.

No acceleration of atherosclerosis.

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

Behcet’s disease current treatment is aimed at:



Easing the symptoms,



Reducing inflammation,



Controlling the immune system.

High dose Corticosteroid therapy (1 mg/kg/d oral prednisone) is indicated for severe disease manifestations February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

The message of this presentation !

Behçet’s syndrome to be kept in mind in the differential diagnosis of intracardiac thrombi of young adults that have no apparent explanation.

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services

February 13 -16, 2013 . SHA24-GHA10

Armed Forces Hospital Southern Region, Cardiac Services