PMC3198627_01
Catheterization study showing mild RPA origin stenosis
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F1
PAH (RV systolic pressure - 55 / 8 mmHg, with a corresponding systemic pressure of 82 / 43 mmHg) with no gradient across the RVOT. Pulmonary artery angiogram showed mild RPA origin stenosis [Figure 1] and free pulmonary regurgitation.
Catheterization study showing LSVC opening to CS
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F2
LSVC draining to the LA was ruled out [Figure 2].
(a and b) Histological section showing prominence of the intracinar arterioles with medial hypertrophy corresponding to Grade I pulmonary hypertension (Heath-Edwards grading system), (H and E x100 (a), x200 (b). (c) Elastic von-Gieson's (EVG) stain highlighting the prominent intracinar arterioles (EVG x100). (d and e) Muscle hypertrophy along with proliferation of intimal cells in the arterioles corresponding to Grade II pulmonary hypertension (Masson's trichrome x100 (d), x400 (e)
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F3
The subsequent postmortem lung biopsy revealed features of pulmonary hypertension, with medial hypertrophy and intimal proliferation in the intra-acinar arterioles corresponding to grade II of the Heath-Edwards grading system of pulmonary hypertension [Figure 3].