Pulmonary Endarterectomy and Balloon Angioplasty in Children and Adolescents with Chronic Thromboembolic Pulmonary Hypertension.
DOI:
https://doi.org/10.34257/LJMHR229558UKAbstract
Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension (PH) that can lead to progressive right heart failure and death. Pulmonary thromboendarterectomy (PTE) is the treatment of choice resulting in significant improvements in functional status, cardiopulmonary hemodynamics, and survival [1] [ 2]. Pulmonary emboli and associated CTEPH is unusual in the pediatric population and mostly reveal an underlying thrombophilic state [3].
Methods: Bakulev National Medical Research Center (BNMRC) for Cardiovascular Surgery, CTEPH database identified patients 18 years or younger at the time of PTE surgery or balloon pulmonary angioplasty (BPA) performance. Medical charts were reviewed for hemodynamics, thromboembolic risk factors, postoperative complications and outcomes.
Results: PTE in pediatric patients resulted in improved functional status and significantly improved cardiopulmonary hemodynamics: mean pulmonary arterial pressure (mPAP) decreased from 44.0 mm Hg (37.8-47.5) to 19.0 mm Hg (17.8-29.0) (P = 0.008), pulmonary vascular resistance (PVR) decreased from 560 dynes*s*cm−5 (393.8-785.8) to 147 dynes*s*cm−5 (129.5-245.0) (P = 0.008), brain natriuretic peptide (NT-proBNP) also decreased from 315.0 pg/ml (163.2-432.5) to 41.0 pg/ml (23.0-69.8) (P =0.008). BPA also improve cardiopulmonary hemodynamics. There were no deaths during PTE or 30 days after surgery, as and after BPA. Long-term survival (5+years) was achieved in 100%. In the long-term we did not observe rethrombosis in our patients.
Conclusions: This study demonstrates that PTE and BPA in pediatric patients with CTEPH is well tolerated with improved postoperative hemodynamics, functional status, minimal postoperative complications, and without perioperative mortality.
References
Humbert, Kovacs, Hoeper, Badagliacca, Berger, Brida (2022) 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. 43, 3618-3731. https://doi.org/10.1093/eurheartj/ehac237
Delcroix, Torbicki, Gopalan, Sitbon, Klok, Lang (2021) ERS statement on chronic thromboembolic pulmonary hypertension. 57, 2002828. https://doi.org/10.1183/13993003.02828-2020
Kumbasar, Aypar, Karago, Demircin, Doğan (2018) Pulmonary thromboendarterectomy in pediatric patients: report of three cases. 60, 604–607. https://doi.org/10.24953/turkjped.2018.05.023
Van Ommen, Heijboer, Büller, Hirasing, Heijmans, Peters (2001) Venous thromboembolism in childhood: a prospective two-year registry in The Netherlands. 139, 676–81. https://doi.org/10.1067/mpd.2001.118192
Goldenberg, Bernard (2008) Venous thromboembolism in children. 55, 305–22. https://doi.org/10.1016/j.pcl.2008.01.003
Verbelen, Cools, Fejzic, Van Den Eynde, Maleux, Delcroix (2019) Pulmonary endarterectomy in a 12-year-old boy with multiple Comorbidities. 9(4), 1–4. https://doi.org/10.1177/2045894019886249
Stein, Kayali, Olson (2004) Incidence of venous thromboembolism in infants and children: Data from the National Hospital Discharge Survey. 145, 563-565. https://doi.org/10.1016/j.jpeds.2004.06.021
Carpenter, Richardson, Hall (2018) Increasing rate of pulmonary embolism diagnosed in hospitalized children in the United States from 2001 to 2014. 2(12), 1403-1408. https://doi.org/10.1182/bloodadvances.2017013292
Lambert, Durand, Devictor, Planche, Serraf (1999) Unilateral right pulmonary thromboendarteretomy for chronic embolism: a successful procedure in an infant. 118, 953–954. https://doi.org/10.1016/s0022-5223(99)70067-x
Madani, Wittine, Auger, Fedullo, Kerr, Kim (2011) Chronic pulmonary thromboembolic disease. 141, 624–630. https://doi.org/10.1016/j.jtcvs.2010.07.010
de Perrot, Gopalan, Jenkins, Lang, Fadel, Delcroix (2021) Evaluation and management of patients with chronic thromboembolic pulmonary hypertension—consensus statement from the ISHLT. 40(11), 1301–26. https://doi.org/10.1016/j.healun.2021.07.020
Berger, Beghetti, Humpl, Raskob, Ivy, Jing (2012) Clinical features of paediatric pulmonary hypertension: a registry study. 379(9815), 537–46. https://doi.org/10.1016/S0140-6736(11)61621-8
Spencer, Valencia Villeda, Takeda, Rosenzweig (2020) Chronic thromboembolic pulmonary hypertension in a child with sickle cell disease. 8, 363. https://doi.org/10.3389/fped.2020.00363
Humpl, Honjo, Temple, de Perrot (2019) Pulmonary endarterectomy in a toddler with chronic thromboembolic pulmonary hypertension after Denver shunt. 157(6), e409–10. https://doi.org/10.1016/j.jtcvs.2019.02.085
Osakwe, Das (2022) Successful Pulmonary Endarterectomy after Acute Pulmonary Embolism and Reversal of Acute Cor Pulmonale in an 11-Year-Old Boy with Nephrotic Syndrome. 9, 1444. https://doi.org/10.3390/children9101444
Tormene, Gavasso, Rossetto, Simioni (2006) Thrombosis and thrombophilia in children: a systematic review. 32, 724-8. https://doi.org/10.1055/s-2006-951457
van Loon, Roofhooft, van Osch-Gevers, Delhaas, Strengers, Blom (2009) Clinical characterization of pediatric pulmonary hypertension: complex presentation and diagnosis. 155, 176-82. https://doi.org/10.1016/j.jpeds.2009.02.036
Hanuna, Pattathu, Buech, Kamla, Kneidinger, Behr (2023) Case report: Central venous catheter thrombosis complicated by chronic thromboembolic disease/pulmonary hypertension in two children requiring parenteral nutrition. 10, 1198204. https://doi.org/10.3389/fcvm.2023.1198204
Diepenbruck, Dalla‐Pozza, Pattathu, Haas, Jakob (2021) Successful intravascular pulmonary lithotripsy in a child with chronic thromboembolic pulmonary hypertension. 56, 1690‐1693. https://doi.org/10.1002/ppul.25338
Gorbachevsky, Bockeria, Averina, Rakhmonov, Sabitov, Diasamidze, Golukhova (2025) 70 Cases of Pulmonary Thromboendarterectomy without Circulatory Arrest. 15(2). https://doi.org/10.4236/wjcs.2025.152005
Gorbachevsky, Petrosyan, Rakhmonov, Sagymbaev, Averina, Diasamidze, Golukhova (2026) Combined Balloon Pulmonary Angioplasty and Pulmonary Endarterectomy in Patients with Chronic Thromboembolic Pulmonary Hypertension. 16(2), 18-34. https://doi.org/10.4236/wjcs.2026.162004
Thistlethwaite, Mo, Madani, Deutsch, Blanchard, Kapelanski, Jamieson (2002) Operative Classification of Thromboembolic Disease Determines Outcome after Pulmonary Endarterectomy. 124, 1203–1211. https://doi.org/10.1067/mtc.2002.127313
Jamieson, Kapelanski, Sakakibara, Manecke, Thistlethwaite, Kerr (2003) Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. 76(5), 1457-62; discussion 1462-4. https://doi.org/10.1016/s00034975(03)00828-2
Gerotziafas (2004) Risk factors for venous thromboembolism in children. 23, 195-205.
Babyn, Gahunia, Massicotte (2005) Pulmonary thromboembolism in children. 35, 258-74. https://doi.org/10.1007/s00247-004-1353-y
Kim, Delcroix, Jais, Madani, Matsubara, Mayer (2019) Chronic thromboembolic pulmonary hypertension. 53(1), 1801915. https://doi.org/10.1183/13993003.01915-2018
Madani, Auger, Pretorius, Sakakibara, Kerr, Kim (2012) Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients. 94(1), 97-103; discussion 103. https://doi.org/10.1016/j.athoracsur.2012.04.004
Jenkins, Madani, Fadel, D'Armini, Mayer (2017) Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension. 26(143), 160111. https://doi.org/10.1183/16000617.0111-2016
Tromeur, Jaïs, Mercier, Couturaud, Montani, Savale (2018) Factors predicting outcome after pulmonary endarterectomy. 13(6), e0198198. https://doi.org/10.1371/journal.pone.0198198
Huisman, Büller, Ten Cate, van Royen, Vreeken, Kersten (1989) Unexpected high prevalence of silent pulmonary embolism in patients with deep venous thrombosis. 95, 498–502. https://doi.org/10.1378/chest.95.3.498
Monagle, Adams, Mahoney, Ali, Barnard, Bernstein (2000) Outcome of pediatric thromboembolic disease: a report from the Canadian Childhood Thrombophilia Registry. 47, 763–6. https://doi.org/10.1203/00006450-200006000-00013
Andrew, David, Adams, Ali, Anderson, Barnard (1994) Venous thromboembolic complications (VTE) in children: first analyses of the Canadian Registry of VTE. 83, 1251–7. https://doi.org/10.1182/blood.V83.5.1251.1251
Zaidi, Hutchins, Rajpurkar (2017) Pulmonary embolism in children. 5, 170. https://doi.org/10.3389/fped.2017.00170
Jain, Sheikh, Bajaj, Townsend, Krasuski, Secemsky (2023) Periprocedural complications with balloon pulmonary angioplasty: analysis of global studies. 16, 976–983. https://doi.org/10.1016/j.jcin.2023.01.361
Hyder, Chatterjee, Aggarwal (2022) Percutaneous treatments for pulmonary hypertension: reviewing the growing procedural role for interventional cardiology. 11, 293–305. https://doi.org/10.1016/j.iccl.2022.01.006
Provost, Mornex, Nasser, Zeghmar, Traclet, Ahmad (2023) Changes in the management of chronic thromboembolic pulmonary hypertension over a 10-year period, in a French expert regional competence centre. 84, 101021. https://doi.org/10.1016/j.resmer.2023.101021
Shimahara, Suzuki, Fujiyoshi, Honda, Koizumi, Yamashita (2023) Balloon pulmonary angioplasty followed by pulmonary endarterectomy: Combination treatment for high-surgical-risk patients with chronic thromboembolic pulmonary hypertension. https://doi.org/10.1093/icvts/ivad031
Jevnikar, Solinas, Brenot, Lechartier, Kularatne, Montani (2023) Sequential multimodal therapy in chronic thromboembolic pulmonary hypertension with mixed anatomical lesions: a proof of concept. 62(5), 2300517. https://doi.org/10.1183/13993003.00517-2023
Broch, Murbraech, Ragnarsson, Gude, Andersen, Fiane (2016) Echocardiographic evidence of right ventricular functional improvement after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. 35(1), 80-86. https://doi.org/10.1016/j.healun.2015.08.007
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