PMC9788526_01
PMC9788526_02
PMC9788526_03
Echocardiography on day 2 and Chest CT on day 7 in PICU at the age of 6 months. Several Echo variables and ratios indicate severe PH on the second PICU day in acute cardiorespiratory decompensation. Panel (A) shows severe RV hypertrophy and RV dilation. Panel (B) shows increased RV/LV end-systolic diameter ratio of 1.5. Panel (C) shows chest CT with atelectasis of the right upper lobe and mild mosaic perfusion. No signs of cysts or bullae were identified.
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F1
The patient underwent a chest-computed tomography (CT) already on day 9 in the PICU at the age of 6 months, in order to evaluate the pulmonary parenchyma and vessels (Figure 1C).
PMC9788526_04
PMC9788526_05
PMC9788526_06
Echocardiography on day 2 and Chest CT on day 7 in PICU at the age of 6 months. Several Echo variables and ratios indicate severe PH on the second PICU day in acute cardiorespiratory decompensation. Panel (A) shows severe RV hypertrophy and RV dilation. Panel (B) shows increased RV/LV end-systolic diameter ratio of 1.5. Panel (C) shows chest CT with atelectasis of the right upper lobe and mild mosaic perfusion. No signs of cysts or bullae were identified.
fcvm-09-984698-g001.jpg
PMC9788526_06_fcvm-09-984698-g001.jpg
F1
The patient underwent a chest-computed tomography (CT) already on day 9 in the PICU at the age of 6 months, in order to evaluate the pulmonary parenchyma and vessels (Figure 1C).
(A,B) Cardiac catheterization 3 months after PICU discharge at the age of 9 months. Right-Left heart catheterization 3 months after acute cardiorespiratory decompensation and initiation of triple oral PH-targeted medication (Sildenafil, Macitentan, Selexipag). Panel (A) shows angiography of the dilated proximal pulmonary artery: PVRi 4.1 WU m2; mPAP 23 mmHg; mSAP 48 mmHg; mPAP/mSAP 0.48 (<half systemic PAP). Panel (B) shows angiography of the left upper pulmonary vein with the stenosis at entry to LA with LUPV pressure of 21-22 mmHg and dP mean LUPV to LA of 12 mmHg.
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F2
A total of 3 months after PICU discharge and initiation of triple combination antihypertensive PH-targeted drug therapy, we performed right-left heart catheterization (RLHC) (Figure 2) at the age of 9 months.
PMC9788526_07
Lung biopsy 5 months after PICU discharge at the age of 11 months. Histology shows pulmonary capillary hemangiomatosis (PCH) without pulmonary venous involvement and mild pulmonary interstitial glycogenosis (PIG) with small foci. Severe bronchopulmonary dysplasia (BPD) is evident as alveolar simplification/hypoalveolarisation. Scale bar = 100 mum.
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F3
Small areas of the biopsy showed mild pulmonary interstitial glycogenosis (PIG) with cytoplasmic accumulation of glycogen in the interstitial cells (Figure 3).
PMC9788526_08
PMC9788526_09