pulmonary thrombosis in Eisenmenger ASD: A case report

Transcription

1 In situ RV thrombus with diffuse pulmonary thrombosis in Eisenmenger ASD: A case report You-Chun Huang,MD; Wei- Hsien Yin, MD,PHD. Cheng-Hsin general hospital,taiwan. 1

2 Present illness A 38-year-old female experienced progressive breathless, orthopnea that had developed in recent weeks History of atrial septal defect (secundum type) and diagnosed of pulmonary hypertension 10 years ago (PA=98mmHg, Qp/Qs=1.4) and she refused surgical correction, and lost follow-up since then. Denied any systemic disease or major surgery before No habit of smoking, alcohol drinking 2

3 Physical examination Chronic-ill looking, malnutrition. Height: 154cm, BW: 41Kgw BP = 156/104 mmhg. BT= HR=125/min O 2 saturation 75-80%, room air Neck: 4.5 cm above Luis angle at degree of 45 Chest: clear breathing sounds. Heart: Regular and rapid heart beats. RV heave on LSB with palpable pulmonary trunk. Grade III Pansystolic tricuspid murmur Extremity: Clubbing finger with cyanosis 3

4 B/R: Laboratory data WBC=7.8x10 3 /ul, RBC=7.8 x10 6 /ul,hgb=15.6 g/dl, HCT=53%, PLT= 263x10 3 /ul Biochemistry: Uric acid=8.4mg/dl, albumin=2.3 g/dl ABG (room air) ph= 7.45, pco 2 = 24.5mmHg,pO 2 = 43.8mmHg HCO - 3 = 16.6mmol/L, Sat O 2 =81% 4

5 EKG 2000 EKG

6 Chest X-ray There are increased cardiothoracic ratio with enlarged bilateral pulmonary vascular shadow and rapid tapering vasculature. Supraaortic vessels are remarkable 6

7 Transthoracic echocardiography Transthoracic echocardiography showed dilated RA and RV. Global severe hypokinesis of right ventricle (RVEF=20%) and preserved LV systolic function. 7

8 Transthoracic echocardiography Moderate PR and severe TR, RVSP=87mmHg 8

9 Transthoracic echocardiography h An ASD with bidirectional shunt 9

10 3D- Transthoracic echocardiography A huge ASD in 3D image 10

11 Computerized tomographic (CT) scan of chest A marked dilated pulmonary trunk (5.5cm) and bilateral pulmonary arteries. Eccentric thrombus in bilateral MPA, interlobar and segmental arteries. Presence of contrast in left atrium via atrial septal defect with 1.36cm 2 defect between RA and LA. (1.2 cm ) 11

12 Transthoracic echocardiography 2-D echography showed dilated RV, MPA and two organized thrombi in MPA 12

13 Transthoracic echocardiography A laminated calcified thrombus attached to RV wall 13

14 Transthoracic echocardiography A size of 3.0 cm 2 thrombus attached to RV wall 14

15 Diagnosis Secundum- type atrial septal defect with Eisenmenger syndrome complicated diffuse pulmonary thrombosis and in situ RV thrombus. 15

16 Management Thrombolytic therapy (Actilyse 10mg + 10mg + 30mg) LMWH use (Fragmin 5000U sc q12h) Plan to Heart-lung transplantation 16

17 Discussion 17

18 Eisenmenger syndrome Eisenmenger syndrome : Late-complication of pre-existing existing intracardiac shunt and consequence of pulmonary hypertension High incidence of PA thrombosis in Eisenmenger syndrome (20%) -Silversides et al.,2003 and Craig S. et al.,

19 Pulmonary thrombosis in Eisenmenger syndrome Mechanism Past -- Silversides et al., 2003 Pulmonary vasculature injury Structural and functional change Clotting and coagulation factors deficiency Sluggish flow promoting cell aggregation In recent study--craig S et al., 2007 Rather than degree of cyanosis or coagulation abnormalities. Older age Biventricular i dysfunction Slow pulmonary artery blood flow 19

20 Pulmonary thrombosis with RV thrombus RV thrombi was 4% to 18% in patients with pulmonary embolism These migrating g thrombi were all acute and thrombogenic associated with diverse clotting factors deficiency. Diffuse pulmonary thrombosis with concomitant in situ thrombus in right ventricle is rare. Few cases ever were seen in Systemic disease: Antiphospholipid h id syndrome, Behcet s disease Emphysema with secondary pulmonary hypertension 20

21 Management For pulmonary embolism Anticoagulation is recommended in thrombus identified without intracardiac lesions Prophylactic use in pulmonary hypertenion -Silversides et al.,2003 Coumadin is choice for pulmonary thromboembolism -Ridker PM et al.,2003 For Eisenmenger syndrome Anticoagulation (dilemma) Hemoptysis about 20% in Eisenmenger syndrome - Diller GP et al.,

22 Management In situ RV thrombus and pulmonary thrombosis in Eisenmenger syndrome No optimal therapy. For floating right heart thrombus with pulmonary embolism Thrombolytic therapy- Justin et al., 2005, Chartier et al., 1999, Ferrari et al., 2005 Heparin alone for floating right heart thrombus Insufficient - Torbichi A et al.,

23 Management Thromboendarterectomy t Percutaneous thrombectomy Single or bilateral lung transplantation + ASD closure 70-80% 1 year survival rate, long term is less poor Normal LV systolic function. No CAD No left-side severe VHD Simple intracardiac shunt RV systolic function > 10% Heart-Lung transplantation 60-80% 1 year survival rate, better long-term 23

24 Conclusion In situ RV thrombus is rare and more life-threatening.. What is the definite mechanism? Role of coagulation or clotting factors? Optimal treatment?-----need more study. 24