Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that results from incomplete resolution of pulmonary emboli. Failure of thrombi to resolve may be related to large or recurrent pulmonary emboli, insufficient anticoagulation, underlying autoimmune and haematological disorders, presence of lupus anticoagulant/antiphospholipid antibodies, ventriculo-atrial shunts, infected pacemaker leads and splenectomy.
Pulmonary endarterectomy (PEA) is potentially curative surgical procedure and remains the treatment of choice for eligible patients. However, residual pulmonary hypertension following surgery is common.
Methods and Results
Eight patients from the multidisciplinary PH clinic of the “ATTIKON” University Hospital, Athens, Greece, underwent PEA for CTEPH in PEA-specialized centers abroad, during the time period 2011-2016. There was no perioperative mortality and all patients improved their functional capacity during the follow-up period (1-6 years after surgery). The routine right heart catheterization one year after PEA, revealed that five patients had normal hemodynamics while three patients had residual PH requiring initiation of pulmonary vasodilator therapy.
Although the number of patients is small, our data confirm the excellent post-surgical outcome of patients undergone PEA and emphasize the need to use all available treatment modalities in patients with CTEPH.