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).