Successful treatment of massive thrombosis of the vena cava inferior with nephrоtic syndrome and chronic bilateral pulmonary artery thromboembolism in a patient with genetic thrombophilia


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Abstract

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrоtic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed. Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter. After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier).

About the authors

Ol'ga Vladimirovna Blagova

Email: blagova@mail.ru

S L Dzemeshkevich

N L Kozlovskaya

A V Nedostup

N D Sarkisova

Yu V Frolova

V V Raskin

A S Dzemeshkevich

S A Abugov

O G Skipenko

E M Shilov

V P Sedov

N V Gagarina

V E Sinitsyn

E A Mershina

E Yu Volkova

O V Blagova

I.M. Sechenov Moscow Medical University, Moscow

I.M. Sechenov Moscow Medical University, Moscow

S L Dzemeshkevich

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

N L Kozlovskaya

I.M. Sechenov Moscow Medical University, Moscow

I.M. Sechenov Moscow Medical University, Moscow

A V Nedostup

I.M. Sechenov Moscow Medical University, Moscow

I.M. Sechenov Moscow Medical University, Moscow

N D Sarkisova

I.M. Sechenov Moscow Medical University, Moscow

I.M. Sechenov Moscow Medical University, Moscow

Yu V Frolova

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

V V Raskin

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

A S Dzemeshkevich

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

S A Abugov

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

O G Skipenko

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

E M Shilov

I.M. Sechenov Moscow Medical University, Moscow

I.M. Sechenov Moscow Medical University, Moscow

V P Sedov

I.M. Sechenov Moscow Medical University, Moscow

I.M. Sechenov Moscow Medical University, Moscow

N V Gagarina

B.V. Petrovsky Russian Research Surgical Center, Moscow

B.V. Petrovsky Russian Research Surgical Center, Moscow

V E Sinitsyn

Treatment and Rehabilitation Center, Moscow

Treatment and Rehabilitation Center, Moscow

E A Mershina

Treatment and Rehabilitation Center, Moscow

Treatment and Rehabilitation Center, Moscow

E Yu Volkova

Municipal hospital, Dubna

Municipal hospital, Dubna

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