Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) in children is a rare condition among other forms of pulmonary hypertension. Diagnosis of CTEPH is very important for the patients because of a successful treatment, pulmonary endarterectomy(PE), is available. Here in we report a child who was recognized as CTEPH. Thus we want to emphasize the diagnostic milestones for children who have a suspicion for CTEPH.

Case

A 12 year girl who admitted to our hospital with complaints of easy fatiguability and chronic headeche that being two or three times a day for three years. A chronic thrombocytopenia was realized and probable casuses of this had been investigated for the etiology in her medical history. Altough making a comprehensive evaluation, the etiology of thrombocytopenia had been unexplained. Therefore, it had been accepted as idiopathic thrombocytopenia. On her physical examination, there was no murmur and the chest x-ray revealed no sign for heart disease. The ECG was completely normal. Right ventricular pressure was measured 56 mmHg and pulmonary artery diastolic pressure was 20 mmHg on echocardiography.An angiography was performed to identify possible causes of pulmonary hypertension.systolic, diastolic and mean pulmonary artery pressures were 50/24/34 mmHg respectively. Bilaterally pulmonary hypo-perfused areas was realized on angiography.Thus we thought that this is a PH which is originating from distal obstructive arteriopathy such as thromboembolic obstruction. The ventilation-perfusion scan was showing distinct characteristics of CTEPH. Partial mosaic perfusion defect was detected on HRCT bilaterally, according to the angiography. She was assessed for a tendency of thrombosis. Prot-C and Prot-S levels were measured lower than in normal population.

The patient has been started to receive a treatment of low-molecular-weight heparin (LMWH) for three months and decided to undergo to PE.

Case

A 12 year girl who admitted to our hospital with complaints of easy fatiguability and chronic headeche that being two or three times a day for three years. A chronic thrombocytopenia was realized and probable casuses of this had been investigated for the etiology in her medical history. Altough making a comprehensive evaluation, the etiology of thrombocytopenia had been unexplained. Therefore, it had been accepted as idiopathic thrombocytopenia. On her physical examination, there was no murmur and the chest x-ray revealed no sign for heart disease. The ECG was completely normal. Right ventricular pressure was measured 56 mmHg and pulmonary artery diastolic pressure was 20 mmHg on echocardiography.An angiography was performed to identify possible causes of pulmonary hypertension.systolic, diastolic and mean pulmonary artery pressures were 50/24/34 mmHg respectively. Bilaterally pulmonary hypo-perfused areas was realized on angiography.Thus we thought that this is a PH which is originating from distal obstructive arteriopathy such as thromboembolic obstruction. The ventilation-perfusion scan was showing distinct characteristics of CTEPH. Partial mosaic perfusion defect was detected on HRCT bilaterally, according to the angiography. She was assessed for a tendency of thrombosis. Prot-C and Prot-S levels were measured lower than in normal population.

The patient has been started to receive a treatment of low-molecular-weight heparin (LMWH) for three months and decided to undergo to PE.