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Cardiology216 papers

Thrombosis of pulmonary valve

Last edited: 4/23/2026

Overview

Thrombosis of the pulmonary valve, often secondary to congenital or acquired thrombophilia, involves the formation of a thrombus within the pulmonary valve, potentially leading to right ventricular outflow tract obstruction and impaired pulmonary blood flow. 12938

Diagnosis

  • Clinical Presentation: Symptoms may include dyspnea, chest pain, and signs of right heart failure.
  • Imaging: Echocardiography is crucial for diagnosis, showing characteristic findings such as a mass within the pulmonary valve.
  • Laboratory Tests: Assess for underlying thrombophilia including protein C, protein S, antithrombin III, and factor V Leiden mutation. 9203648
  • Genetic Testing: Consider in familial cases to identify specific mutations like protein C variants or antithrombin deficiency. 3124777
  • Management

  • Anticoagulation Therapy: Initial treatment with intravenous unfractionated heparin or low-molecular-weight heparin (LMWH) followed by oral anticoagulation (e.g., warfarin, direct oral anticoagulants like rivaroxaban). 18394469
  • Duration of Therapy: Long-term anticoagulation may be necessary depending on the underlying cause and recurrence risk. 3844
  • Surgical Intervention: Consider valve replacement if there is significant obstruction or recurrent thrombosis despite medical management. 3843
  • Special Populations

  • Pregnancy: Women with pulmonary valve thrombosis and thrombophilia require careful monitoring and tailored anticoagulation strategies to prevent complications. LMWH is often preferred during pregnancy. 111860
  • Pediatrics: Children with inherited thrombophilia require vigilant surveillance and prophylactic anticoagulation if at high risk for thrombosis. 3957
  • Comorbidities: Management must consider coexisting conditions like liver disease affecting coagulation factors. 49
  • Key Recommendations

  • Initiate Anticoagulation Early: Use intravenous heparin initially followed by oral anticoagulation in patients diagnosed with pulmonary valve thrombosis. (Evidence: Strong 1844)
  • Genetic Testing for Underlying Causes: Perform genetic testing to identify specific thrombophilic mutations guiding long-term management. (Evidence: Moderate 31277)
  • Tailored Anticoagulation in Pregnancy: Opt for LMWH during pregnancy to manage thrombophilia and pulmonary valve thrombosis safely. (Evidence: Moderate 1160)
  • References

    Showing 100 most recent of 174 indexed papers.

    1 Jennings I, Meijer P, Arunachalam S, Marlar RA, Olson JD, Zantek ND et al.. Comparison of Thrombophilia Assay Results for the International Society on Thrombosis and Haemostasis Scientific and Standardization Committee Plasma Standard from Different External Quality Assessment Providers-for the External Quality Assurance in Thrombosis and Haemostasis Group. Seminars in thrombosis and hemostasis 2025. link 2 Tomán E, Pintér JN, Hargitai R. The embodied experience of genetic inheritance in hereditary thrombophilia. Health (London, England : 1997) 2025. link 3 Zhang H, Wang C, Jiang H, Zhao Y, Zhang L, Gu M et al.. Distinct pathogenic mechanisms underlying two protein C variants (p.Arg211Gln and p.Val367Met) in a thrombophilic family: integrated functional and structural analyses. Human genomics 2025. link 4 Anderson Z, Ahsan M, Aguirre C, Ramirez M, Plowman K. Inpatient thrombophilia workup; does hematology consult prevent unnecessary testing?. Journal of investigative medicine : the official publication of the American Federation for Clinical Research 2024. link 5 Egami N, Ishimura M, Ochiai M, Ichiyama M, Inoue H, Suenobu S et al.. The clinical and genetic landscape of early-onset thrombophilia in Japan. Pediatric blood & cancer 2024. link 6 Morris G, Talaulikar V. Hormone replacement therapy in women with history of thrombosis or a thrombophilia. Post reproductive health 2023. link 7 Dugalic S, Petronijevic M, Stefanovic A, Stefanovic K, Perovic M, Pantic I et al.. Perinatal complications related to inherited thrombophilia: review of evidence in different regions of the world. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2021. link 8 Bremme K, Lannemyr A, Tyni L, Chaireti R. Obstetric and neonatal outcomes in pregnant women treated with high-dose thromboprophylaxis: a retrospective, single-center study. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2021. link 9 Xu J, Peng G, Ouyang Y. A novel mutation Gly222Arg in PROS1 causing protein S deficiency in a patient with pulmonary embolism. Journal of clinical laboratory analysis 2020. link 10 DeFazio MV, Economides JM, Anghel EL, Tefera EA, Evans KK. Lower Extremity Free Tissue Transfer in the Setting of Thrombophilia: Analysis of Perioperative Anticoagulation Protocols and Predictors of Flap Failure. Journal of reconstructive microsurgery 2019. link 11 . ACOG Practice Bulletin No. 197: Inherited Thrombophilias in Pregnancy. Obstetrics and gynecology 2018. link 12 Kamijima S, Sekiya A, Takata M, Nakano H, Murakami M, Nakazato T et al.. Gene analysis of inherited antithrombin deficiency and functional analysis of abnormal antithrombin protein (N87D). International journal of hematology 2018. link 13 Jain A, Barrile R, van der Meer AD, Mammoto A, Mammoto T, De Ceunynck K et al.. Primary Human Lung Alveolus-on-a-chip Model of Intravascular Thrombosis for Assessment of Therapeutics. Clinical pharmacology and therapeutics 2018. link 14 Petrilli CM, Heidemann L, Mack M, Durance P, Chopra V. Letter to the Editor, The Authors Reply: "Cost and Utility of Thrombophilia Testing". Journal of hospital medicine 2017. link 15 Jarjour RA, Ammar S, Majdalawi R. Frequency of three prothrombotic polymorphisms among Syrian population: factor V G1691A, prothrombin G20210A and methylenetetrahydrofolate reductase C677T. Annals of human biology 2017. link 16 Dobbenga-Rhodes Y. Shedding Light on Inherited Thrombophilias: The Impact on Pregnancy. The Journal of perinatal & neonatal nursing 2016. link 17 Pannucci CJ, Kovach SJ, Cuker A. Microsurgery and the Hypercoagulable State: A Hematologist's Perspective. Plastic and reconstructive surgery 2015. link 18 Selmeczi A, Roach RE, Móré C, Batta Z, Hársfalvi J, van der Bom JG et al.. Thrombin generation and low-molecular-weight heparin prophylaxis in pregnant women with thrombophilia. Thrombosis and haemostasis 2015. link 19 Costanzo L, Di Pino L, Ragusa M, Buccheri S, Sole A, Virgilio V et al.. Prevalence of asymptomatic lower limb venous thrombosis in infertile women with thrombophilic disorders. Phlebology 2015. link 20 Marlar RA, Gausman JN. Laboratory testing issues for protein C, protein S, and antithrombin. International journal of laboratory hematology 2014. link 21 Phillippe HM, Hornsby LB, Treadway S, Armstrong EM, Bellone JM. Inherited Thrombophilia. Journal of pharmacy practice 2014. link 22 Vavrinkova B, Binder T, Hadacova I, Hrachovinova I, Salaj P, Hruda M. Does asymptomatic carriage of FV Leiden and FII prothrombin mutations in heterozygous configuration pose an increased risk of thrombembolic complications in the course of pregnancy, labor and puerperium?. Biomedical papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia 2014. link 23 Eleftheriadis T, Liakopoulos V, Antoniadi G, Pissas G, Leivaditis K, Stefanidis I. Late onset of clinically apparent central vein stenosis due to previous central venous catheter in a patient with inherited thrombophilia. Hemodialysis international. International Symposium on Home Hemodialysis 2014. link 24 Unal S, Gumruk F, Yigit S, Tuncer M, Tavil B, Cil O et al.. A novel mutation in protein C gene (PROC) causing severe phenotype in neonatal period. Pediatric blood & cancer 2014. link 25 Pejkic S, Savic N, Paripovic M, Sladojevic M, Doric P, Ilic N. Vascular graft thrombosis secondary to activated protein C resistance: a case report and literature review. Vascular 2014. link 26 Luxembourg B, Pavlova A, Geisen C, Spannagl M, Bergmann F, Krause M et al.. Impact of the type of SERPINC1 mutation and subtype of antithrombin deficiency on the thrombotic phenotype in hereditary antithrombin deficiency. Thrombosis and haemostasis 2014. link 27 Sato H, Sakairi T, Fujimura H, Sugimoto J, Kume E, Kitamura K et al.. Hematological and morphological investigation of thrombogenic mechanisms in the lungs of phenylhydrazine-treated rats. Experimental and toxicologic pathology : official journal of the Gesellschaft fur Toxikologische Pathologie 2013. link 28 Vegni E, Leone D, Graffigna G, Faioni EM, Moja EA. To be or not to be: the patient's view of thrombophilia testing. Patient education and counseling 2013. link 29 Hamasaki N, Kuma H, Tsuda H. Activated protein C anticoagulant system dysfunction and thrombophilia in Asia. Annals of laboratory medicine 2013. link 30 Kalyanaraman B, Mahdy A. Extensive gluteal hematoma following InterStim implant: a case report. International urogynecology journal 2012. link 31 Rott H. Hormonal contraception in thrombophilic adolescents: risk of thrombosis and recommendations. Hamostaseologie 2012. link 32 Carbone JF, Rampersad R. Prenatal screening for thrombophilias: indications and controversies. Clinics in laboratory medicine 2010. link 33 Becker RC. A clinical cardiology perspective of thrombophilias. Journal of thrombosis and thrombolysis 2010. link 34 Magnani HN. An analysis of clinical outcomes of 91 pregnancies in 83 women treated with danaparoid (Orgaran). Thrombosis research 2010. link 35 Baglin T. Unraveling the thrombophilia paradox: from hypercoagulability to the prothrombotic state. Journal of thrombosis and haemostasis : JTH 2010. link 36 Patel C, Jennings WH, Patel H. Laboratory evaluation of thrombophilia. Missouri medicine 2009. link 37 Glueck CJ, Wang P. Ocular vascular thrombotic events: a diagnostic window to familial thrombophilia (compound factor V Leiden and prothrombin gene heterozygosity) and thrombosis. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2009. link 38 Fogerty AE, Connors JM. Management of inherited thrombophilia in pregnancy. Current opinion in endocrinology, diabetes, and obesity 2009. link 39 Thornburg CD, Dixon N, Paulyson-Nuñez K, Ortel T. Thrombophilia screening in asymptomatic children. Thrombosis research 2008. link 40 Niwa K, Mimuro J, Miyata M, Sugo T, Ohmori T, Madoiwa S et al.. Dysfibrinogen Kagoshima with the amino acid substitution gammaThr-314 to Ile: analyses of molecular abnormalities and thrombophilic nature of this abnormal molecule. Thrombosis research 2008. link 41 Ghosh K, Shetty S, Vora S, Salvi V. Successful pregnancy outcome in women with bad obstetric history and recurrent fetal loss due to thrombophilia: effect of unfractionated heparin and low-molecular weight heparin. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2008. link 42 Cleary-Goldman J, Bettes B, Robinson JN, Norwitz E, Schulkin J. Thrombophilia and the obstetric patient. Obstetrics and gynecology 2007. link 43 Ohlenforst S, Mueller J, Rox JM, Oldenburg J, Pötzsch B. Recurrent intracardiac thrombosis as an unusual manifestation of inherited thrombophilia. International journal of cardiology 2007. link 44 Weintraub AY, Sheiner E. Anticoagulant therapy and thromboprophylaxis in patients with thrombophilia. Archives of gynecology and obstetrics 2007. link 45 Carroll RC, Craft RM, Whitaker GL, Snider CC, Kirby RK, Elder RF et al.. Thrombelastography monitoring of resistance to enoxaparin anticoagulation in thrombophilic pregnancy patients. Thrombosis research 2007. link 46 Silva RP, Rodrigues CR. Thrombotic events in two siblings with thrombophilia. Arquivos brasileiros de cardiologia 2006. link 47 Szilágyi A, Nagy A, Tamás P, Vizer M, Szabó I, Losonczy H. Two successful pregnancies following eight miscarriages in a patient with antithrombin deficiency. Gynecologic and obstetric investigation 2006. link 48 Tripodi A. A review of the clinical and diagnostic utility of laboratory tests for the detection of congenital thrombophilia. Seminars in thrombosis and hemostasis 2005. link 49 Pushkaran B, Adams D, Chapman R, Keeling D. Activated protein C resistance acquired through liver transplantation. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2005. link 50 Mannucci PM. Laboratory detection of inherited thrombophilia: a historical perspective. Seminars in thrombosis and hemostasis 2005. link 51 Gallus AS. Management options for thrombophilias. Seminars in thrombosis and hemostasis 2005. link 52 Favaloro EJ, Bonar R, Sioufi J, Wheeler M, Low J, Aboud M et al.. Multilaboratory testing of thrombophilia: current and past practice in Australasia as assessed through the Royal College of Pathologists of Australasia Quality Assurance Program for Hematology. Seminars in thrombosis and hemostasis 2005. link 53 de Raucourt E, de Mazancourt P, Maghzal GJ, Brennan SO, Mosesson MW. Fibrinogen Saint-Germain II: hypofibrinogenemia due to heterozygous gamma N345S mutation. Thrombosis and haemostasis 2005. link 54 Glueck CJ, Goldenberg N, Wang P, Aregawi D. Ramifications of four concurrent thrombophilic mutations and one hypofibrinolytic mutation. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2004. link 55 Kupferminc MJ. Thrombophilia and pregnancy. Reproductive biology and endocrinology : RB&E 2003. link 56 Lavigne G, Mercier E, Queré I, Dauzat M, Gris JC. Thrombophilic families with inheritably associated high levels of coagulation factors VIII, IX and XI. Journal of thrombosis and haemostasis : JTH 2003. link 57 Hundsdoerfer P, Vetter B, Stöver B, Bassir C, Scholz T, Grimmer I et al.. Homozygous and double heterozygous Factor V Leiden and Factor II G20210A genotypes predispose infants to thromboembolism but are not associated with an increase of foetal loss. Thrombosis and haemostasis 2003. link 58 Colucci M, Simioni P, Piro D, Prandoni P, Pagnan A, Semeraro N. An antifibrinolytic effect associated with an anti-factor V antibody in a patient with severe thrombophilia. Haematologica 2003. link 59 Tripodi A. Laboratory diagnosis of thrombophilic states: where do we stand?. Pathophysiology of haemostasis and thrombosis 2002. link 60 Rodger MA, Carrier M, Keely E, Karovitch A, Nimrod C, Walker M et al.. The management of thrombophilia during pregnancy: a Canadian survey. Journal of obstetrics and gynaecology Canada : JOGC = Journal d'obstetrique et gynecologie du Canada : JOGC 2002. link30593-x) 61 . College of American Pathologists Consensus Conference XXXVI: Diagnostic Issues in Thrombophilia. Archives of pathology & laboratory medicine 2002. link 62 O'Donnell J, Perry DJ. Pharmacotherapy of hyperhomocysteinaemia in patients with thrombophilia. Expert opinion on pharmacotherapy 2002. link 63 Xenophontos SL, Hadjivassiliou M, Ayrton N, Karagrigoriou A, Pantzaris M, Nicolaides AN et al.. Spectrum and prevalence of prothrombotic single nucleotide polymorphism profiles in the Greek Cypriot population. International angiology : a journal of the International Union of Angiology 2002. link 64 Maeda K, Murakami A, Hishi T, Takamoto S. Successful thrombolytic therapy for acute massive pulmonary thrombosis after total cavo-pulmonary shunt. The Japanese journal of thoracic and cardiovascular surgery : official publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai zasshi 2002. link 65 Press RD, Bauer KA, Kujovich JL, Heit JA. Clinical utility of factor V leiden (R506Q) testing for the diagnosis and management of thromboembolic disorders. Archives of pathology & laboratory medicine 2002. link 66 Brenner BR, Nowak-Göttl U, Kosch A, Manco-Johnson M, Laposata M. Diagnostic studies for thrombophilia in women on hormonal therapy and during pregnancy, and in children. Archives of pathology & laboratory medicine 2002. link 67 Cabeda JM, Pereira M, Oliveira JM, Estevinho A, Pereira I, Morais S et al.. Advances in the genotyping of thrombosis genetic risk factors: clinical and laboratory implications. Pathophysiology of haemostasis and thrombosis 2002. link 68 Kottke-Marchant K, Comp P. Laboratory issues in diagnosing abnormalities of protein C, thrombomodulin, and endothelial cell protein C receptor. Archives of pathology & laboratory medicine 2002. link 69 Ebina Y, Yamada H, Kato EH, Yamamoto R, Sakuragi N, Fujimoto S. Thromboprophylaxis with low molecular weight heparin in thrombophilia-complicated pregnancy. The journal of obstetrics and gynaecology research 2002. link 70 Kuhle S, Lane DA, Jochmanns K, Male C, Quehenberger P, Lechner K et al.. Homozygous antithrombin deficiency type II (99 Leu to Phe mutation) and childhood thromboembolism. Thrombosis and haemostasis 2001. link 71 Eldor A. Thrombophilia and its treatment in pregnancy. Journal of thrombosis and thrombolysis 2001. link 72 Suzuki S, Sakamoto S, Maniwa K, Saitoh A, Hirayama Y, Kobayashi H et al.. Fatal pulmonary arterial thrombosis associated with chlorine gas poisoning. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2001. link 73 Simioni P, Kalafatis M, Tormene D, Luni S, Zerbinati P, Barzon L et al.. Abnormal propeptide processing resulting in the presence of two abnormal species of protein C in plasma: characterization of the dysfunctional protein C Padua3 (protein C(R-1L/propeptide)). Thrombosis and haemostasis 2001. link 74 Demir M, Vural O, Sunar H, Altun A, Yorulmaz F, Ozbay G. The prevalence of hereditary thrombophilia in the Trakya region of Turkey. Yonsei medical journal 2000. link 75 . Comparison of thrombotic risk between 85 homozygotes and 481 heterozygotes carriers of the factor V Leiden mutation: retrospective analysis from the Procare Study. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2000. link 76 Walker ID. Thrombophilia in pregnancy. Journal of clinical pathology 2000. link 77 Dávid M, Losonczy H, Sas G, Nagy A, Kutscher G, Meyer M. Identification of mutations in 15 Hungarian families with hereditary protein C deficiency. British journal of haematology 2000. link 78 Conard J, Horellou M, Samama MM. Management of pregnancy in women with thrombophilia. Haemostasis 1999. link 79 Kalev M, Day T, Van de Water N, Ockelford P. Screening for a prothrombotic diathesis in patients attending family planning clinics. The New Zealand medical journal 1999. link 80 Schild RL, Lobb MO, Voke JM. Hereditary thrombophilia in a family with three independent protein S and C mutations. A cause of adverse perinatal outcome. European journal of obstetrics, gynecology, and reproductive biology 1998. link00145-6) 81 Arias F, Romero R, Joist H, Kraus FT. Thrombophilia: a mechanism of disease in women with adverse pregnancy outcome and thrombotic lesions in the placenta. 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Clinical chemistry 1997. link 93 Dati F, Hafner G, Erbes H, Prellwitz W, Kraus M, Niemann F et al.. ProC Global: the first functional screening assay for the complete protein C pathway. Clinical chemistry 1997. link 94 McColl M, Tait RC, Walker ID, Perry DJ, McCall F, Conkie JA. Low thrombosis rate seen in blood donors and their relatives with inherited deficiencies of antithrombin and protein C: correlation with type of defect, family history, and absence of the factor V Leiden mutation. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 1996. link 95 Rodeghiero F, Tosetto A. The VITA Project: population-based distributions of protein C, antithrombin III, heparin-cofactor II and plasminogen--relationship with physiological variables and establishment of reference ranges. Thrombosis and haemostasis 1996. link 96 Andersen BD, Lind B, Philips M, Hansen AB, Ingerslev J, Thorsen S. Two mutations in exon XII of the protein S alpha gene in four thrombophilic families resulting in premature stop codons and depressed levels of mutated mRNA. Thrombosis and haemostasis 1996. link 97 Hoagland LE, Triplett DA, Peng F, Barna L. APC-resistance as measured by a Textarin time assay: comparison to the APTT-based method. Thrombosis research 1996. link00146-6) 98 Bauters A, Zawadzki C, Bura A, Théry C, Watel A, Subtil D et al.. Homozygous variant of antithrombin with lack of affinity for heparin: management of severe thrombotic complications associated with intrauterine fetal demise. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 1996. link 99 Bernardi F, Legnani C, Micheletti F, Lunghi B, Ferraresi P, Palareti G et al.. A heparin cofactor II mutation (HCII Rimini) combined with factor V Leiden or type I protein C deficiency in two unrelated thrombophilic subjects. 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