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Journal of Thrombosis and Thrombolysis

, Volume 46, Issue 4, pp 516–520 | Cite as

Balloon pulmonary angioplasty is a promising option in thalassemic patients with inoperable chronic thromboembolic pulmonary hypertension

  • Panagiotis Karyofyllis
  • Dimitris Tsiapras
  • Varvara Papadopoulou
  • Michael D. Diamantidis
  • Paraskevi Fotiou
  • Eftychia Demerouti
  • Vassilis Voudris
Article

Abstract

Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. β-Thalassaemia, among other haematological disorders, develop PH which is not an infrequent finding and worsens the prognosis. Haemolysis, iron overload and hypercoagulable state are among the main pathogenetic mechanisms. Haemoglobinopathies and congenital haemolytic anaemia constitute a unique patients population more predisposed to developing chronic thromboembolic pulmonary hypertension (CTEPH). Although pulmonary endarterectomy is accepted as the best treatment for CTEPH, surgery in these patients poses significant practical challenges given the distinct nature of the disease. As drug therapy is not expected to offer relief from the mechanical obstructions of pulmonary arteries and the use of specific pulmonary arterial hypertension drugs is not established in thalassaemia patients, the novel technique of balloon pulmonary angioplasty (BPA) may emerge as a new therapeutic option for patients with inoperable CTEPH and thalassaemia. We are reporting the case of a thalassaemic patient with a history of splenectomy suffering from progressive severe pulmonary hypertension related to chronic thromboembolic disease, who was successfully treated by BPA with substantial improvement.

Keywords

Thalassemia Hemoglobinopathies Chronic thromboembolic pulmonary hypertension (CTEPH) Balloon pulmonary angioplasty (BPA) 

Notes

Compliance with ethical standards

Conflict of interest

Panagiotis Karyofyllis has received lecture fees from Actelion Pharmaceuticals Hellas, Bayer Hellas, and GlaxoSmithKline. Eftychia Demerouti has received lecture fees from Actelion Pharmaceuticals Hellas, Bayer Hellas, GlaxoSmithKline and MSD Hellas. All other authors declare that they have no conflict of interest.

References

  1. 1.
    Simonneau G, Gatzoulis MA, Adatia I et al (2013) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62:D34–D41CrossRefPubMedGoogle Scholar
  2. 2.
    Kato GJ, Taylor JGT (2010) Pleiotropic effects of intravascular haemolysis on vascular homeostasis. Br J Haematol 148:690–701CrossRefPubMedGoogle Scholar
  3. 3.
    Anthi A, Orfanos SE, Armaganidis A (2013) Pulmonary hypertension in β thalassaemia. Lancet Respir Med 1:488–496CrossRefPubMedGoogle Scholar
  4. 4.
    Fedullo PF, Auger WR, Kerr KM, Rubin LJ (2001) Chronic thromboembolic pulmonary hypertension. N Engl J Med 345:1465–1472CrossRefPubMedGoogle Scholar
  5. 5.
    Galiè N, Humbert M, Vachiery JL et al (2016) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37:67–119CrossRefPubMedGoogle Scholar
  6. 6.
    Jenkins D, Madani M, Fadel E, D’Armini AM, Mayer E (2017) Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 26:160111CrossRefPubMedGoogle Scholar
  7. 7.
    Pepke-Zaba J, Delcroix M, Lang I et al (2011) Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 124:1973–1981CrossRefPubMedGoogle Scholar
  8. 8.
    Aessopos A, Kati M, Farmakis D (2007) Heart disease in thalassemia intermedia: a review of the underlying pathophysiology. Haematologica 92:658–665CrossRefPubMedGoogle Scholar
  9. 9.
    Musallam KM, Taher AT (2011) Thrombosis in thalassemia: why are we so concerned? Hemoglobin 35:503–510CrossRefPubMedGoogle Scholar
  10. 10.
    Bonderman D, Jakowitsch J, Adlbrecht C et al (2005) Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. Thromb Haemost 93:512–516PubMedGoogle Scholar
  11. 11.
    Taher AT, Musallam KM, Karimi M et al (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood 115:1886–1892CrossRefPubMedGoogle Scholar
  12. 12.
    Scholzel BE, Post MC, Plokker HT, Snijder RJ (2012) Clinical worsening during long-term follow-up in inoperable chronic thromboembolic pulmonary hypertension. Lung 190:161–167CrossRefPubMedGoogle Scholar
  13. 13.
    Mahesh B, Besser M, Ravaglioli A et al (2016) Pulmonary endarterectomy is effective and safe in patients with haemoglobinopathies and abnormal red blood cells: the Papworth experience. Eur J Cardiothorac Surg 50:537–541CrossRefPubMedGoogle Scholar
  14. 14.
    Roggero S, Vullo S, Volpe G, Piga A, Albera C (2015) Management of pulmonary arterial hypertension associated to thalassemia: when pulmonary endarterectomy is the best therapeutical option? A case report. J Thromb Thrombolysis 39:139–143CrossRefPubMedGoogle Scholar
  15. 15.
    Taniguchi Y, Miyagawa K, Nakayama K et al (2014) Balloon pulmonary angioplasty: an additional treatment option to improve the prognosis of patients with chronic thromboembolic pulmonary hypertension. EuroIntervention 10:518–525CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Interventional CardiologyOnassis Cardiac Surgery CentreAthensGreece
  2. 2.Noninvasive Cardiology Diagnostic UnitOnassis Cardiac Surgery CentreAthensGreece
  3. 3.Thalassemia Unit, Department of HematologyGeneral Hospital of LarissaLarissaGreece

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