Distinctive Clinical Features of Portopulmonary Hypertension

Kuo, Paul C.; Plotkin, Jeffrey S.; Johnson, Lynt B.; Howell, Charles D.; Laurin, Jacqueline; Bartlett, Stephen T.; Rubin, Lewis J.

doi:10.1378/chest.112.4.980

Cited by 146 publications

(105 citation statements)

References 7 publications

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“…This is in contrast to the previous series in which the most common diagnoses were alcoholic cirrhosis and hepatitis C. It is also in contrast to the description of PPHTN by Kuo et al, 1 who, in characterizing the condition, reported a series of 30 subjects, with the largest diagnostic group having alcoholic cirrhosis (13 subjects). It is notable, however, that despite the difference in diagnostic groupings, the baseline hemodynamics and blood gas analyses in our patients are very similar to those reported by Kuo et al, 1 with increased MPAP and PVR in the setting of accentuated respiratory alkalosis and decreased PO 2 compared with liver failure controls. Until a clear understanding of the pathophysiologic characteristics of PPHTN emerges, the possibility of a link between autoimmune liver disease and pulmonary vasoreactivity is extremely speculative.…”

Section: Discussioncontrasting

confidence: 86%

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Inhaled nitric oxide reduces pulmonary artery pressures in portopulmonary hypertension

et al. 1999

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Pulmonary artery hypertension in association with liver failure (portopulmonary hypertension [PPHTN]) is a significant barrier to liver transplantation because patients with this condition have a very high mortality when transplantation is undertaken. Inhaled nitric oxide (NO), a potent pulmonary vasodilator, reduces pulmonary artery pressure (PAP) in some patients with primary pulmonary hypertension, but its effect in patients with PPHTN is controversial. We investigated the hemodynamic effects of inhaled NO in 6 patients with PPHTN. Five of 6 patients responded to NO inhalation with decreases in PAP and pulmonary vascular resistance of greater than 10%; these decreases were statistically significant at NO concentrations of 10 and 30 ppm. Cardiac output did not significantly change. We conclude that inhalation of NO reduces PAPs in some patients with PPHTN.

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Section: Discussioncontrasting

confidence: 86%

“…1 It occurs in 0.2% to 4% of these patients. [2][3][4] When patients with PPHTN undergo liver transplantation, perioperative mortality is high.…”

Section: P Ortopulmonary Hypertension (Pphtn) Is Amentioning

confidence: 99%

Inhaled nitric oxide reduces pulmonary artery pressures in portopulmonary hypertension

et al. 1999

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“…The high incidence of abnormal CXR results in patients with ESLD is caused by volume overload. 18,23 PCO 2 and PO 2 values, despite being less in the PPHTN group, did not reach statistical significance. This contrasts to the findings of the study of Kuo et al, 18 in which significantly lower PCO 2 and PO 2 values were found in the patients with PPHTN.…”

Section: Discussionmentioning

confidence: 69%

“…Compared with primary pulmonary HTN, there are significant differences with regard to pathophysiological characteristics, 5 pathological characteristics, 16,17 and clinical and hemodynamic variables. 5,16,18 The diagnosis of this condition is often challenging because patients with ESLD have a multitude of clinical symptoms, which may confuse the clinician. The symptoms of ESLD and pulmonary HTN may be indistinguishable.…”

Section: Discussionmentioning

confidence: 99%

Clinical predictors of pulmonary hypertension in patients undergoing liver transplant evaluation

et al. 2000

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Clinical prediction of portopulmonary hypertension (PPHTN) is critical in the preoperative evaluation of candidates for orthotopic liver transplantation (OLT) because of its association with significant morbidity and mortality. To determine the clinical, laboratory, and echocardiographic predictors of PPHTN, we retrospectively evaluated 55 candidates before OLT. From those, 8 candidates had pulmonary hypertension ([HTN] group A) and 47 candidates did not (group B). Pulmonary HTN was defined as a mean pulmonary artery pressure (PAP) of 25 mm Hg or greater and either elevated pulmonary vascular resistance or normal pulmonary artery wedge pressure. The significant predictors of PPHTN were (1) systemic arterial HTN (63% in group A v 9% in group B; P Ͻ .001), (2) loud pulmonary component of the second heart sound (38% v 2%; P ϭ .001), (3) right ventricular (RV) heave (38% v 4%; P ϭ .002), (4) RV dilatation by echocardiogram (63% v 0%; P Ͻ .001), (5) RV hypertrophy by echocardiogram (38% v 0%; P ϭ .001), and (6) echocardiogram-estimated systolic PAP (SPAP) greater than 40 mm Hg (63% v 2%; P Ͻ .001). The sensitivity of these variables for the detection of pulmonary HTN ranges from 37% to 63%, and their specificity from 91% to 100%. We conclude that several clinical and echocardiographic features are significantly associated with pulmonary HTN in patients with cirrhosis. In particular, echocardiogram-estimated SPAP greater than 40 mm Hg is strongly associated with pulmonary HTN and is specific. These predictors, however, are not sensitive enough to identify all the patients with PPHTN. Therefore, the evaluation of a combination of these variables may be useful for the preoperative identification of pulmonary HTN in liver transplant candidates.

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“…This is in contrast to a published series of PPHTN, in which the most frequent primary diagnosis was alcoholic cirrhosis. 11 This may imply a common link between the cause of the primary disease and the occurrence of splenomegaly after epoprostenol therapy.…”

Section: Discussionmentioning

confidence: 99%

Progressive splenomegaly after epoprostenol therapy in portopulmonary hypertension

et al. 1999

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Patients with end-stage liver failure, portal hypertension, and associated pulmonary artery hypertension (portopulmonary hypertension [PPHTN]) have a high mortality when undergoing liver transplantation. Successful transplantation in these patients may depend on efforts to reduce pulmonary artery pressure (PAP). To this end, a number of centers are using a continuous intravenous (IV) infusion of epoprostenol, which has been shown to improve symptoms, extend life span, and reduce PAP in patients with primary pulmonary hypertension. We report four cases in which treatment of patients with PPHTN with continuous IV epoprostenol was followed by the development of progressive splenomegaly, with worsening thrombocytopenia and leukopenia. This finding may limit the usefulness of epoprostenol in PPHTN and influence the timing of transplantation in such patients.

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Distinctive Clinical Features of Portopulmonary Hypertension

Cited by 146 publications

References 7 publications

Inhaled nitric oxide reduces pulmonary artery pressures in portopulmonary hypertension

Inhaled nitric oxide reduces pulmonary artery pressures in portopulmonary hypertension

Clinical predictors of pulmonary hypertension in patients undergoing liver transplant evaluation

Progressive splenomegaly after epoprostenol therapy in portopulmonary hypertension

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