TABLE 1.

Patient characteristics

Characteristic
Female	11, 68.8%
Age at pulmonary hypertension diagnosis, y	50 (41–57)
Age at LT, y	51 (45–58)
Liver disease pathogenesis
Alcohol	7, 43.8%
Hepatitis C	7, 43.8%
Nonalcoholic steatohepatitis	1, 6.3%
Cryptogenic cirrhosis	2, 12.5%
Autoimmune hepatitis	1, 6.3%
Primary sclerosing cholangitis	1, 6.3%
Other	2, 12.5%
MELD-Na score at LT	18 (11–31)
Portopulmonary hypertension MELD exception	7, 43.8%
Initial pulmonary hemodynamics
Mean pulmonary artery pressure, mm Hg	48 (44–54)
Pulmonary artery wedge pressure, mm Hg (n = 15)	12 (8–17)
Transpulmonary gradient, mm Hg (n = 15)	36 (30–39)
Right atrial pressure, mm Hg (n = 15)	10 (5–18)
Cardiac output, L/min (n = 15)	5.8 (5.4–7.4)
Cardiac index, L/min/m2 (n = 15)	3.0 (2.8–3.6)
Pulmonary vascular resistance, dynes-s-cm−5 (n = 15)	455 (344–592)
Pretransplant pulmonary hemodynamics
Mean pulmonary artery pressure, mm Hg	38 (35–42)
Pulmonary artery wedge pressure, mm Hg (n = 15)	20 (14–22)
Transpulmonary gradient, mm Hg (n = 15)	20 (18–23)
Right atrial pressure, mm Hg (n = 15)	13 (10–14)
Cardiac output, L/min	8.5 (7.3–9.8)
Cardiac index, L/min/m2 (n = 14)	4.4 (4.1–5.2)
Pulmonary vascular resistance, dynes-s-cm−5 (n = 15)	191 (172–216)
Time between pretransplant right heart catheterization and liver transplant, d	40 (11–72)
PAH therapy at LT
Phosphodiesterase 5 inhibitor	10, 62.5%
Endothelin receptor antagonist	3, 18.8%
Parenteral prostacyclin therapy	8, 50%
Inhaled nitric oxide	1, 6.3%
Outcomes
Acute cardiopulmonary related mortality within 30 d of LT	1, 6.3%
1-y survival	11, 68.8%
Off PAH therapy at 1 y	7/11, 63.6%

Data reported as number, % or median (range). Individual patients may have had >1 liver disease etiology and may have been treated with >1 pulmonary arterial hypertension targeted therapy.

LT, liver transplantation; MELD-Na, model for end-stage liver disease sodium; PAH, pulmonary arterial hypertension.