Right Ventricular Thrombosis Combined With Fetal Death and Acrocyanosis in Pregnancy

  • Feridoun Sabzi Mail Department of Cardiac Surgery, Imam Ali Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran.
  • Aghigh Heidari Department of Anesthesiology, Imam Ali Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran.
Keywords:
Pregnancy, Thrombus, Thrombophilia

Abstract

Prepartum or postpartum right ventricular thrombosis (RVT) is an exceedingly rare and potentially lethal phenomenon in pregnancy. We here report a case of a pregnant patient with near term pregnancy admitted for dyspnea, amniotic fluids discharge and labor pain in a gynecology center that an eight-month dead fetus was diagnosed and delivered vaginally by induction. A post delivery period was complicated by aggravation of her dyspnea and pleuritic chest pain that she referred for further evaluation in our cardiac center. Physical exam revealed normal head and neck exam, and history taking revealed that her fetus had intra-uterine growth failure as reported by her gynecologist. Chest exam except to left lung crackle was normal. Lower and upper left extremities were normal. However, acrocyanosis was found in tips of 4 and 5th right-hand digits. Chest x-ray revealed some linear consolidation in left lower lung lobes, and the precordial exam was normal. ECG was normal. Post delivery transthoracic echocardiography (TEE) showed a 1.5×1.5 cm mobile right ventricular clot. C-T angiography revealed obstruction of left upper lung pulmonary artery branches. Complete thrombophilia assay showed the presence of high titer of antiphospholipid, anticardiolipin antibody, and β1 glycoprotein antibody. However, others test were normal. The patient was scheduled for cardiac surgery, and her hemodynamic was monitored by left radial artery line and central pressure venous line, and thrombus was removed from the right ventricle (RV), and subsequent anticoagulation therapy constituted. Six-month follow-up revealed no recurrence of thrombus and recovery of patient’s symptoms.

References

Tripodi A, de Groot PG, Pengo V. Antiphospholipid syndrome: Laboratory detection, mechanisms of action and treatment. J Intern Med 2011;270:110-22 .

Miyakis S, Lockshin MD, Atsumi T, Branch DW, BreyRL, Cervera R, et al. International consensus statement onan update of the classification criteria for definiteantiphospholipid syndrome (APS). J Thromb Haemost2006;4:295-306 .

Asherson RA, Cervera R. Microvascular andmicroangiopathic antiphospholipid-associated syndromes(“MAPS”): semantic or antisemantic? Autoimmu Rev2008;7:164-7 .

Merrill JT, Asherson RA. Catastrophic antiphospholipidsyndrome. Nature Clinical Practice Rheumatology.2006;2:81-9.

Nayfe R, Uthman I, Aoun J, Aldin ES, Merashli M,Khamashta MA. Seronegative antiphospholipidsyndrome. Rheumatology 2013;52:1358-67.

Shenkman B, Einav Y. Thrombotic thrombocytopenicpurpura and other thrombotic microangiopathic hemolyticanemias: Diagnosis and classification. Autoimmun Rev2014;13:584-6 .

Abildgaard U, Heimdal K. Pathogenesis of the syndromeof hemolysis, elevated liver enzymes, and low plateletcount (HELLP): a review. Eur J Obstet Gynecol ReprodBiol 2013;166:117-23 .

Noris M, Mescia F, Remuzzi G. STEC-HUS, atypicalHUS and TTP are all diseases of complement activation.Nat Rev Nephrol 2012;8:622-33.

Velicki L, Milosavljević A, Majin M, Vujin B, KovacevićP. Postpartal right ventricular thrombosis. Herz2008;33:532-4 .

Matos V, Pinheiro B, Maia P, Leite H, Coelho A,Fernandes A. A recurrent intracardiac mass in a pregnantwoman with the antiphospholipid antibody syndrome.Acta Med Port 1993;6:539-42.

Appenzeller S, Souza FHC, Wagner Silva de Souza A,Shoenfeld Y, de Carvalho JF. HELLP syndrome and itsrelationship with antiphospholipid syndrome andantiphospholipid antibodies. Semin Arthritis Rheum2011;41:517-23 .

Chong BH, Isaacs A. Heparin-induced thrombocytopenia:what clinicians need to know. Thromb Haemost2009;101:279-83 .

Taylor FB Jr, Toh CH, Hoots WK, Wada H, Levi M;Scientific Subcommittee on Disseminated IntravascularThrombosis and Haemostasis (ISTH). Towards definition,clinical and laboratory criteria, and a scoring system fordisseminated intravascular coagulation. Thromb Haemost2001;86:1327-30.

Mele C, Remuzzi G, Noris M. Hemolytic uremicsyndrome. Semin Immunopathol 2014;36:399-420.

Prechel M, Walenga JM. The laboratory diagnosis andclinical management of patients with heparin-inducedthrombocytopenia: an update. Semin Thromb Hemost2008;34:86-96 .

Sanna G, D'Cruz D, Cuadrado MJ. CerebralManifestations in the Antiphospholipid (Hughes)Syndrome. Rheum Dis Clin North Am 2006;32:465-90 .

Hatemi G, Silman A, Bang D, Bodaghi B, ChamberlainAM, Gul A, et al. EULAR recommendations for themanagement of Behçet disease. Annals of the RheumaticDiseases. 2008;67:1656-62 .

Silvestri E, Emmi G, Prisco D. Anti-TNF-α agents invascular Behçet's disease. Exp Rev Cardiovasc Ther2014;12:415-6 .

Stam J. Current concepts: thrombosis of the cerebral veinsand sinuses. N Engl J Med 2005;352:1791-8 .

Fanouriakis A, Boumpas DT, Bertsias GK. Pathogenesisand treatment of CNS lupus. Curr Opin Rheumatol2013;25:577-83.

Published
2017-09-16
How to Cite
1.
Sabzi F, Heidari A. Right Ventricular Thrombosis Combined With Fetal Death and Acrocyanosis in Pregnancy. Acta Med Iran. 55(7):464-469.
QRcode
Section
Case Report(s)