Klippel-Trenaunay Syndrome with Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty

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Angiomatous lesions may be origin of even asymptomatic thromboembolic events. We report an older patient with Klippel-Trenaunay-Syndrome who presented with huge angiomatous lesions of the left upper extremity, slowly progressive dyspnea due to chronic thromboembolic pulmonary hypertension (CTEPH). Since birth his left hand, arm and shoulder were covered by venous convolutes. CT-scan showed angiomatous lesions of the thoracic wall and signs of pulmonary hypertension. Accordingly, right heart enlargement with D-sign was found in echocardiography. Right heart catheterization confirmed pulmonary hypertension with a mean PAP of 43 mmHg and a PVR of 7.5 WU. Pulmonary angiogram showed peripheral obstructions confirming the diagnosis of CTEPH. Therapeutic anticoagulation was established. As pulmonary endarterectomy was not feasible, medical treatment with riociguat was initiated and the patient underwent 5 sessions of BPA addressing 10 pulmonary segments. This resulted in significant improvement of dyspnea, right heart function, parenchymal perfusion, as well as reduction of PVR to 5 WU. We present this case because in KTS patients thromboembolism and development of CTEPH has to be kept in mind. Therapeutic options are PEA surgery, interventional with BPA, and medical treatment.

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