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A 56-year-old female presented to the Emergency Department with palpitations and shortness
of breath. The patient had a history of hypertension, diabetes mellitus, and hyperthyroidism.
The patient's body temperature was 36.7°C and blood pressure was 125/80 mmHg. On electrocardiogram,
negative T-waves were observed in the right precordial leads. Laboratory analysis
revealed low haemoglobin value (11.4 g/dL; reference, 12–14 g/dL), high fasting blood
glucose (185 mg/dL; reference, 74–106 mg/dL), and elevated D-dimer level (788 ng/mL;
reference, <280 ng/mL). Other laboratory parameters, including C-reactive protein
and high-sensitivity troponin I, were within normal limits. A chest radiograph revealed
cardiomegaly, pulmonary artery dilatation, and pulmonary vessel cephalisation (Figure 1A). The patient underwent pulmonary computed tomography angiography (CTA) with suspicion
of a pulmonary embolism, and the CTA showed dilatation of the right heart chambers
and pulmonary arteries without pulmonary embolism. Moreover, CTA revealed a persistent
left superior vena cava (PLSVC) draining into the left atrium, dilatation of the coronary
sinus (CS), and complete communication between the CS and left atria, consistent with
unroofed CS (UCS) (Figures 1B-F). Transthoracic echocardiography (TTE) revealed left ventricular systolic dysfunction
(ejection fraction of 48%) and severe dilatation in the right heart chambers, with
mid-systolic notching in the pulmonary artery. Moreover, TTE revealed a tricuspid
annular systolic excursion of 10 mm, systolic pulmonary artery pressure of 75 mmHg,
and inferior vena cava size 24 mm and collapsibility <50%. The patient underwent right
heart catheterisation, and the pulmonary artery pressure was 44 mmHg. The patient
was followed up with medical treatment with a diagnosis of pulmonary hypertension.
Figure 1(A) Posteroanterior chest radiograph showing cardiomegaly, pulmonary artery dilatation,
and pulmonary vessel cephalisation (arrowheads). (B–E) Consecutive axial pulmonary computed tomography angiography images showing persistent
left superior vena cava draining into the left atrium (arrowheads) and unroofed coronary
sinus (arrows). (F) Multiplanar reformatted computed tomography angiography of the coronary sinus showing
dilatation of the coronary sinus and unroofed coronary sinus (arrows). Also, note
the persistent left superior vena cava (arrowheads) draining into the left atrium.