%0 Journal Article %A Andrei Seferian %A Xavier Jaïs %A David Montani %A Sven Günther %A Delphine Natali %A Laurent Savale %A Florence Parent %A Dermot Steven O'Callaghan %A Olivier Sitbon %A Marc Humbert %A Gerald Simonneau %T Chronic thromboembolic pulmonary hypertension associated with Klippel Trenaunay syndrome: A retrospective series of 5 patients %D 2011 %J European Respiratory Journal %P p2351 %V 38 %N Suppl 55 %X Introduction: Klippel Trenaunay syndrome (KTS) is a rare congenital disorder characterized by the triad: (1) cutaneous capillary malformations; (2) soft tissue and bone hypertrophy; (3) multiple vascular malformations at arterial, venous and lymphatic level. KTS is associated with recurrent venous thromboembolism (VTE), that may lead to chronic thromboembolic pulmonary hypertension (CTEPH).Patients and methods: We retrospectively reviewed clinical and haemodynamical characteristics of 5 patients with CTEPH associated with KTS referred to our center between 1993 and 2010.Results: Four patients had a previous history of VTE. At diagnosis, 1 patient was in NYHA functional class (FC) II and 4 patients were in NYHA FC III. Pulmonary haemodynamics were: mean pulmonary artery pressure (mPAP)= 56.4±9.3 mmHg, cardiac index (CI)= 2.74±0.89 l/min/m2 and total pulmonary resistance (TPR)= 20.2±12.4 Wood units. One patient had proximal CTEPH, underwent a pulmonary endarterectomy and normalized his pulmonary pressures after surgery. The other 4 patients had inoperable CTEPH due to distal lesions and were treated with specific pulmonary arterial hypertension (PAH) therapy. Two patients were clinically and haemodynamically improved by oral therapy with a mean reduction of 22% of the mPAP and 60% of the TPR after a mean follow-up of 50 months. One patient died after 34 months due to recurrent VTE complications and one patient needed a heart-lung transplantation after 15 years of follow up.Conclusion: CTEPH is a rare but severe complication of KTS. Careful monitoring of patients with KTS in a multidisciplinary setting is thus appropriate. %U