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

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), nephrotic 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 pulmnonale, 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).

• Authors: O V Blagova, S L Dzemeshkevich, N L Kozlovskaia, A V Nedostup, N D Sarkisova, Iu 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 Iu Volkova
• Journal: Terapevticheskii arkhiv (Ter Arkh) Vol. 84 Issue 1 Pg. 41-7 ( 2012) ISSN: 0040-3660 [Print] Russia (Federation)
• PMID: 22616531 (Publication Type: Case Reports, English Abstract, Journal Article, Review)

Chemical References

• Anticoagulants
• Immunosuppressive Agents

Topics

• Anticoagulants (administration & dosage)
• Disease Progression
• Femoral Artery (physiopathology)
• Humans
• Immunosuppressive Agents (administration & dosage)
• Lumbosacral Region (injuries)
• Male
• Nephrotic Syndrome (etiology, physiopathology)
• Polymorphism, Genetic
• Prosthesis Implantation (instrumentation, methods)
• Pulmonary Artery (physiopathology)
• Pulmonary Embolism (etiology, physiopathology, therapy)
• Pulmonary Heart Disease (etiology, physiopathology)
• Remission Induction
• Renal Veins (physiopathology)
• Thrombectomy (methods)
• Thrombophilia (genetics, physiopathology, therapy)
• Thrombosis (etiology, physiopathology, therapy)
• Vena Cava Filters
• Vena Cava, Inferior (physiopathology, surgery)
• Wounds, Nonpenetrating (complications)
• Young Adult