Chronic Thromboembolic Pulmonary Hypertension in a Child With Sickle Cell Disease

Overview

Journal Front Pediatr

Specialty Pediatrics

Date 2020 Aug 28

PMID 32850520

Citations 4

Authors

Robert Spencer

Gerson Valencia Villeda

Koji Takeda

Erika B Rosenzweig

Affiliations

Soon will be listed here.

Abstract

Chronic thromboembolic pulmonary hypertension is a potentially curable form of pre-capillary pulmonary hypertension (PH) resulting from incomplete resolution of pulmonary thromboemboli. We describe an 11-year-old boy with homozygous sickle cell disease with an indwelling catheter found to have severe PH on routine screening echocardiography. The diagnosis was confirmed by CT, ventilation-perfusion scintigraphy, and right heart catheterization. The patient was medically managed until undergoing pulmonary thromboendarterectomy with resolution of his PH. This case highlights the need for pediatric providers to be aware of this underdiagnosed form of PH, particularly for patients at high risk.

Citing Articles

Evolution and New Perspectives of Balloon Pulmonary Angioplasty in CTEPH.

Larsen J, Lakhter V, Nasri A, Bashir R J Clin Med. 2025; 14(3).

PMID: 39941370 PMC: 11818377. DOI: 10.3390/jcm14030699.

Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy: An Official American Thoracic Society Clinical Practice Guideline.

Hayes Jr D, Jennerich A, Coleman R, Abston E, Adamson G, Berger J Am J Respir Crit Care Med. 2024; 211(2):157-173.

PMID: 39531626 PMC: 11812548. DOI: 10.1164/rccm.202410-1901ST.

Diagnosis and management of chronic thromboembolic pulmonary hypertension (CTEPH) in sickle cell disease: A review.

Hersi K, Ramani G, Law J, Sadek A, Vaidya A, Gladwin M Pulm Circ. 2024; 14(2):e12362.

PMID: 38803827 PMC: 11128985. DOI: 10.1002/pul2.12362.

Case report: Central venous catheter thrombosis complicated by chronic thromboembolic disease/pulmonary hypertension in two children requiring parenteral nutrition.

Hanuna M, Pattathu J, Buech J, Kamla C, Kneidinger N, Behr J Front Cardiovasc Med. 2023; 10:1198204.

PMID: 37363098 PMC: 10285210. DOI: 10.3389/fcvm.2023.1198204.

Nuclear Imaging in Pediatric Cardiology: Principles and Applications.

Venet M, Friedberg M, Mertens L, Baranger J, Jalal Z, Tlili G Front Pediatr. 2022; 10:909994.

PMID: 35874576 PMC: 9301385. DOI: 10.3389/fped.2022.909994.

References

Ghofrani H, DArmini A, Grimminger F, Hoeper M, Jansa P, Kim N . Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013; 369(4):319-29. DOI: 10.1056/NEJMoa1209657. View

Lang I, Meyer B, Ogo T, Matsubara H, Kurzyna M, Ghofrani H . Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017; 26(143). PMC: 9489135. DOI: 10.1183/16000617.0119-2016. View

Jenkins D, Mayer E, Screaton N, Madani M . State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management. Eur Respir Rev. 2012; 21(123):32-9. PMC: 9487476. DOI: 10.1183/09059180.00009211. View

Kumbasar U, Aypar E, Karagoz T, Demircin M, Dogan R . Pulmonary thromboendarterectomy in pediatric patients: Report of three cases. Turk J Pediatr. 2019; 60(5):604-607. DOI: 10.24953/turkjped.2018.05.023. View

Levy M, Moshous D, Szezepanski I, Galmiche L, Castelle M, Lesage F . Pulmonary hypertension after bone marrow transplantation in children. Eur Respir J. 2019; 54(5). DOI: 10.1183/13993003.00612-2019. View

Gall H, Hoeper M, Richter M, Cacheris W, Hinzmann B, Mayer E . An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan. Eur Respir Rev. 2017; 26(143). PMC: 9488926. DOI: 10.1183/16000617.0121-2016. View

Lang I, Pesavento R, Bonderman D, Yuan J . Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2012; 41(2):462-8. DOI: 10.1183/09031936.00049312. View

Lim M, Ataga K, Key N . Hemostatic abnormalities in sickle cell disease. Curr Opin Hematol. 2013; 20(5):472-7. DOI: 10.1097/MOH.0b013e328363442f. View

Weir N, Conrey A, Lewis D, Mehari A . Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series. Pulm Circ. 2018; 8(4):2045894018791802. PMC: 6083805. DOI: 10.1177/2045894018791802. View

10.

Gordeuk V, Castro O, Machado R . Pathophysiology and treatment of pulmonary hypertension in sickle cell disease. Blood. 2016; 127(7):820-8. PMC: 4760088. DOI: 10.1182/blood-2015-08-618561. View

11.

Madani M, Auger W, Pretorius V, Sakakibara N, Kerr K, Kim N . Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients. Ann Thorac Surg. 2012; 94(1):97-103. DOI: 10.1016/j.athoracsur.2012.04.004. View

12.

Klepetko W, Mayer E, Sandoval J, Trulock E, Vachiery J, Dartevelle P . Interventional and surgical modalities of treatment for pulmonary arterial hypertension. J Am Coll Cardiol. 2004; 43(12 Suppl S):73S-80S. DOI: 10.1016/j.jacc.2004.02.039. View

13.

Yung G, Channick R, Fedullo P, Auger W, Kerr K, Jamieson S . Successful pulmonary thromboendarterectomy in two patients with sickle cell disease. Am J Respir Crit Care Med. 1998; 157(5 Pt 1):1690-3. DOI: 10.1164/ajrccm.157.5.9710032. View

14.

Parent F, Bachir D, Inamo J, Lionnet F, Driss F, Loko G . A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011; 365(1):44-53. DOI: 10.1056/NEJMoa1005565. View

15.

Ende-Verhaar Y, Cannegieter S, Vonk Noordegraaf A, Delcroix M, Pruszczyk P, Mairuhu A . Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature. Eur Respir J. 2017; 49(2). DOI: 10.1183/13993003.01792-2016. View

16.

Madani M, Wittine L, Auger W, Fedullo P, Kerr K, Kim N . Chronic thromboembolic pulmonary hypertension in pediatric patients. J Thorac Cardiovasc Surg. 2010; 141(3):624-30. DOI: 10.1016/j.jtcvs.2010.07.010. View

17.

Pengo V, Lensing A, Prins M, Marchiori A, Davidson B, Tiozzo F . Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004; 350(22):2257-64. DOI: 10.1056/NEJMoa032274. View

18.

Jais X, Ioos V, Jardim C, Sitbon O, Parent F, Hamid A . Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax. 2005; 60(12):1031-4. PMC: 1747270. DOI: 10.1136/thx.2004.038083. View

19.

Verbelen T, Cools B, Fejzic Z, Van den Eynde R, Maleux G, Delcroix M . Pulmonary endarterectomy in a 12-year-old boy with multiple comorbidities. Pulm Circ. 2020; 9(4):2045894019886249. PMC: 7119433. DOI: 10.1177/2045894019886249. View

20.

Anthi A, Machado R, Jison M, Taveira-DaSilva A, Rubin L, Hunter L . Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Am J Respir Crit Care Med. 2007; 175(12):1272-9. PMC: 2176091. DOI: 10.1164/rccm.200610-1498OC. View