Post-Thromboembolic pulmonary hypertension: a complex case

Introduction Patients with post–thromboembolic pulmonary hypertension (CTEPH) achieve functional recovery after pulmonary endarterectomy (PEA). 40% of them are inoperable (1,2). Clinical case 66–year–old man admitted in 2012 to our ward for dyspnea on exertion and high echocardiographic probability of pulmonary hypertension (PI). Medical history: smoking; thalassemia trait; COPD; splenectomy at 13; diagnosis of chronic myeloproliferative syndrome. BP: 120/70 mmHg. NYHA class III. Blood tests: Hb: 9.4 g/dl; MCV: 70; HCT: 30%; PLT: 511; WBC: 14.84 (Mo 13%).NT–pro BNP:933. Autoantibodies and tests –HIV: negative. EGA in AAPaO2: 53 mmHg, PaCO2: 32 mmHg, SO2: 87%. PFR: FEV1: 68% FVC:74%, FEV1/FVC:72.DLCO:75%.6MWT:450m,Borg:0/3;Baseline SO2: 86%,final SO2 74%. On ECG: sinus r. at 64 beats/min. Echocardiogram: left sections within limits; right ventricle:DTD:30mm; RV/LV< 1; right atrial area: 19cm2, PAPs: 70+5 mmHg (Vel: 4.1 m/s), AcT: 90 msec. TAPSE: 16mm. Performs pulmonary CT scan with contrast medium: negative for pulmonary embolism; some 1 cm bubbles of emphysema in the subpleural area of the right middle lobe; right heart catheterization: PAD: 13mm; PAPM: 50mm; IC: 4.1l /min/m2; RVPtot:11WU;RVP:art:5.2WU;WP:11mmHg.Haematological diagnosis of thalassemia intermedia and exclusion of myeloproliferative syndrome/P lung scintigraphy is positive for multiple bilateral perfusion defects. The possibility of PEA for peripheral lesions at the referral Centre, therapy with warfarin (TAO), riociguat and bronchodilators was started., discontinued therapy with TAO. Finding of recurrence of pulmonary micro embolism on scintigraphy. At the end of the intravitreal treatments with resumption of TAO, at control catheterization evidence of PAD:5mm PAPm19mmHg;WP:9mmHg;IC: 3.4l/min/m2; RVPart:2.9 WU;NYHA:II. Discussion Not all CTEPH from thalassemia intermedia can perform PEA (3). Hypercoagulability is an important pathogenetic mechanism in the genesis of PH (4) and comorbidities have a significant role in the prognosis and response to medical therapy. COPD is the comorbidity that favors hypercoagulability, worsens pulmonary ventilation and gaseous exchanges (5). Conclusions In thalassemia intermedia with inoperable CTEPH, hypercoagulability is the condition that most influences the prognosis and efficacy of vasodilator therapy.