PMC10020523_01
Case 1: echocardiography in the parasternal short axis view. Echocardiography at birth (A) and at the time of PH crisis (B) showing septal bowing to the left (white arrow) and a D-shaped left ventricle that indicates over-systemic pulmonary arterial pressure. On the day after the initiation of high-dose epoprostenol (C), left sided deviation of intraventricular septum (white arrowhead) ameliorated compared to at the time of PH crisis. At one year of age (D), the interventricular septal shift and left ventricle compression have improved.
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F1
Echocardiography at birth (Figure 1A) showed no structural anomaly, a flattened interventricular septum, and a bidirectional patent ductus arteriosus (PDA) shunt, which indicated severe PH. The echocardiogram (Figure 1B) demonstrated septal bowing to the left and a compressed left ventricle, which indicated PAP greater than systemic arterial pressure. Echocardiography on the day after the initiation of high-dose epoprostenol (Figure 1C) showed an improvement in the deviation of intraventricular septum compared to at the time of PH crisis. Echocardiography (Figure 1D) showed that the interventricular septal shift and LV compression improved, and right heart catheterization showed moderate pulmonary arterial hypertension (PAH) with a PAP of 52/26 mmHg (mean, 39 mmHg), a systolic pulmonary systemic arterial pressure ratio (Pp/Ps) of 0.71, and a PVR index of 4.4 Wood units-m2 on the intravenous treprostinil infusion (125 ng/kg/min), oral tadalafil, and macitentan at one year of age.
Case 1: axial CT images of lung window settings at 3 months. Axial CT images show heterogeneous ground-glass opacities in both lung fields.
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F2
Because severe PH associated with respiratory failure persisted, chest computed tomography (CT) was performed at 1 month of age, and it showed ground-glass opacities in both lung fields (Figure 2).
PMC10020523_02
PMC10020523_03
Case 2: axial CT images of lung window settings at 1 year of age. Axial CT images show parahilar consolidation and subpleural linear opacities of atelectasis or fibrosis with bronchodilation, predominantly in the right lung field.
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F3
Chest CT images (Figure 3) showed para-hilar consolidation and subpleural linear opacities predominantly in the right lung field, indicating atelectasis or fibrosis.
Case 2: echocardiography in the parasternal short-axis view. Echocardiography at the beginning of epoprostenol infusion (A) shows that the inverted interventricular septum (white arrow) compresses the left ventricle. At the time of epoprostenol infusion above 100 ng/kg/min (B), the interventricular septal shift (white arrowhead) and LV compression ameliorated.
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F4
Echocardiography after operation (Figure 4A) showed that the inverted intraventricular septum compressed the left ventricle, and the estimated systolic PAP (sPAP) based on Doppler assessment of the tricuspid regurgitant pressure gradient was around 95 mmHg. It was necessary to increase the epoprostenol from 36 ng/kg/min to 106 ng/kg/min over two months and add steroid for BPD, resulting in estimated sPAP of 50 mmHg and improvement of IVS shift towards LV on echocardiography (Figure 4B).