Pulmonary Endarterectomy and Balloon Angioplasty in Children and Adolescents with Chronic Thromboembolic Pulmonary Hypertension.

Abstract

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.