Case Report
This is the case of a 21 years-old male who was diagnosed with β-thalassemia major at the age of 8 months. He underwent splenectomy at the age of 10 years, and has been receiving monthly blood transfusions ever since. Three months prior to admission he presented to a peripheral emergency department with a sudden episode of dyspnea and central cyanosis, and was suspected to be infected with covid-19. Chest CT scan was made and ruled out any interstitial lung involvement, and revealed the presence of bilateral pulmonary emboli. Anticoagulation therapy with warfarin was started, but on follow-up the patient developed progressive exertional dyspnea, deteriorating to NYHA functional class III.
The patient was referred to our institution because of worsening symptoms. Physical examination showed clinical evidence of right heart failure and pulmonary hypertension, with a mild drop in arterial oxygen saturation (93%). Echocardiography revealed a dilated right atrium (RA) which contained a large 47×35×16 mm hyperechogenic mobile mass attached to the RA free wall near the orifice of the inferior vena cava. The right ventricle (RV) was severely dilated and dysfunctional, with tricuspid annular plane systolic excursion (TAPSE) of 13 mm and severe tricuspid regurgitation. Estimated systolic pulmonary arterial pressure (PAP) was 85 mmHg. Lung perfusion scintigraphy was not available at our center. Chest CT angiography showed bilateral defects in the pulmonary arteries consistent with bilateral chronic pulmonary thromboemboli (fig. 1).
On the basis of this evaluation, the diagnosis of CTEPH was established and PTE was performed, the technique of which was reported previously [5]. Total circulatory arrest time was 45 minutes and total cardiopulmonary bypass perfusion time was 94 minutes. A large organized clot was removed from the RA, in addition to removing the thickened intima and organized thrombi from lobar and segmental pulmonary arteries of the left middle and lower lobes, as well as from all major branches on the right.
Early recovery following surgery was uneventful, and the patient was discharged from ICU on the 4th post-operative day. Pre-discharge echocardiogram confirmed satisfactory hemodynamic results, with an improved RV function and an estimated PAP of 45 mmHg. The patient was discharged home 19 days after surgery on warfarin but no requirement for supplemental oxygen. Three months after discharge, significant improvements in clinical status (NYHA class I) and oxygen saturation (99%) were noted despite a degree of residual pulmonary hypertension (PAP of 42 mmHg).