Case Presentation
A 27-year-old female patient was admitted to our clinic
complaining of easy fatigability and dyspnea that had
started six months ago. She also had been experiencing
multiple syncopal attacks, the last one happened two
days before her admission. Sixteen years before, she had
undergone patent ductus arteriosus closure. According
to the previous patient discharge form, her pulmonary
pressure had been within normal limits at the time
of preoperative diagnostic cardiac catheterization
(systolic, mean, and diastolic pressures were 30, 18 and
12 mmHg, respectively), and her postoperative period
had been uneventful. On her physical examination,
a 2-3/6 grade systolic murmur was heard along the
left lower parasternal border. An electrocardiogram
(ECG) showed a right ventricular hypertrophy and right
axis deviation. Transthoracic echocardiography (TTE)
revealed mild to moderate tricuspid regurgitation, severe
pulmonary hypertension (systolic pulmonary pressure:
98 mmHg calculated from peak tricuspid regurgitant
velocity), a D-shaped left ventricle, and enlarged right heart chambers. We did not find any residual ductal flow.
Other diagnostic evaluations examining rheumatologic,
hematologic, and pulmonary functions, including the
diffusion capacity of carbon monoxide, yielded no
evidence for other diseases that might cause PAH,
such as scleroderma or parenchymal lung diseases. Her
high-resolution chest computed tomography (CT) was
normal, except for mild enlargement of the pulmonary
truncus and the left and right main pulmonary arteries
(Figure
1). However, ventilation-perfusion scintigraphy
revealed hypoperfusion on her left lung, suggesting
chronic thromboemboli (Figure
2). Her six-minute walk
test was 300 m. We performed cardiac catheterization
and detected no residual shunt. Pulmonary angiography
revealed enlarged left and right main pulmonary arteries (Figure
3). Systolic, mean, and diastolic pressures for the
pulmonary artery, right ventricle, and right atrium were
99-61-42 mmHg, 90-30-0 mmHg, and 7-5-4 mmHg,
respectively, with normal left ventricular end-diastolic
pressure. A vasoreactivity test with iloprost was negative.
Based on this data, we prescribed bosentan 62.5 mg bid;
however, her transaminases increased at the two-week
control, and we shifted her medication to an iloprost
inhaler (nine times a day) and warfarin for a target an
international normalized ration (INR) level between
1.5-2.5. Her follow-up visit at week 12 revealed a drop
in pulmonary artery systolic pressure, which measured
73 mmHg in TTE. Furthermore, she improved from
functional class III to II.
Figure 1: High resolution computed tomographic view of the
patient.
Figure 2: Ventilation-perfusion scintigraphy of the patient.
Figure 3: Pulmonary angiography of the patient.