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Case report 2: necropsy lung histology (hematoxylin and eosin staining) demonstrates the characteristic histologic features of ACD/MPV: thickened alveolar septae with scarce dilated pulmonary capillaries located away from the alveolar epithelium, with absence of the usual alveolar-capillary barrier ( ); medial hypertrophy of small pulmonary arteries and muscularization of distal arterioles ( ); congested pulmonary veins malpositioned, adjacent to pulmonary arteries in the same adventitial sheath (*); lymphangiectasis not present.
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The lung histology was diagnostic of ACD/MPV (Figure 1).
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Case report 3: (a) chest radiograph with pulmonary ground glass opacities and right pleural effusion. (b) Chest radiograph showing pneumomediastinum. (c) Percutaneous nephrostomy using water-soluble contrast revealing very severe bilateral hydronephrosis.
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Echocardiographic evaluation revealed a severe PPHN with a normal cardiac anatomy and chest radiographs showed ground glass opacities and right pleural effusion (Figure 2(a)). Pneumomediastinum with spontaneous resolution was observed, (Figure 2(b)). To complement the study, an ultrasound-guided percutaneous nephrostomy using water-soluble contrast was preformed, revealing a very severe bilateral hydronephrosis with obstruction to contrast flow from the renal pelvis to the proximal ureter, suggestive of ureteropelvic junction obstruction, (Figure 2(c)).