Prevalence of spontaneous portosystemic shunts in patients with portopulmonary hypertension and effect on treatment.

BACKGROUND & AIMS: We documented the frequency of large spontaneous portosystemic shunts in patients with moderate or severe portopulmonary hypertension (POPH) and determined the association between large shunts and response to treatment.

METHODS

We performed a retrospective case-control study of data from patients with mild (mean pulmonary artery pressure [MPAP], 25-35 mm Hg; n = 18), moderate (MPAP, 35-50 mm Hg; n = 45), and severe POPH (MPAP, >50 mm Hg; n = 16). Data were compared with those from controls (normal echocardiography with estimated right ventricular systolic pressure, <35 mm Hg; n = 122). Spontaneous portosystemic shunts greater than 10 mm in diameter, identified by computed tomography or magnetic resonance, were classified as large. Response to treatment at 6 months was defined by right ventricular systolic pressure or MPAP as significant (<35 mm Hg), partial (35-50 mm Hg), or no response (>50 mm Hg).

RESULTS

The frequency of spontaneous shunts did not differ significantly between groups of subjects with severe (n = 14 of 16), moderate (n = 38 of 45), or mild POPH (n = 11 of 18) or normal echocardiograms (controls, n = 86 of 122) (P = .77). Large shunts were associated with severe (14 of 16) and moderate POPH (32 of 45), compared with mild POPH (6 of 18) or controls (30 of 122) (P < .01). In 13 patients with severe POPH, large shunts were associated with lack of response to treatment in 90% (8 of 9) or partial response in 50% (2 of 4). Among 27 patients with moderate POPH, large shunts were associated with no response to treatment in 13 of 19 (68%) and a partial response in 2 of 6 (33%).

CONCLUSIONS

Large spontaneous portosystemic shunts are associated significantly with moderate and severe POPH, and with lack of response to treatment.