Primary pulmonary hypertension is a disease for which there is no single best therapy. Rather, it is a process that progresses inexorably to disability and death, for which there are a variety of palliative therapies, all with significant side effects, and none curative. Nevertheless, it is clear that the available therapies improve the quality of life and prolong life; failure to offer therapy for patients with this disease in the current era is indefensible. As primary pulmonary hypertension progresses, one must chose from among the available therapies the regimen that provides the most benefit for the patient with the least associated morbidity. Organ replacement is appropriate only after all other available therapies have been exhausted. The recommended hierarchy of therapy is 1) anticongestive therapy, anticoagulation, and supplemental oxygen, 2) calcium channel blockade, 3) continuous intravenous prostacyclin, 4) beta-receptor agonists for cardiac support, and 5) lung transplantation. Newer therapies, described in this review, soon will be incorporated into this hierarchy.

We sought to compare the responses of patients with pulmonary hypertension from primary and secondary causes (PPH and SPH, respectively) to inhaled nitric oxide (iNO) in the cardiac catheterization laboratory.

Pulmonary hypertension can lead to right ventricular pressure overload and failure. Although vasodilators are effective as therapy in patients with PPH, less is known about their role in adults with SPH. Inhaled nitric oxide can accurately predict the response to other vasodilators in PPH and could be similarly utilized in SPH.

Forty-two patients (26 to 77 years old) with pulmonary hypertension during cardiac catheterization received iNO. Demographic and hemodynamic data were collected. Their response to iNO was defined by a decrease of > or =20% in mean pulmonary artery (PA) pressure or pulmonary vascular resistance (PVR).

Mean PA pressures and PVR were lower during nitric oxide (NO) inhalation in all patients with pulmonary hypertension. Seventy-eight percent of patients with PPH and 83% of patients with SPH were responders to iNO. A trend was seen toward a greater response with larger doses of NO in patients with SPH. Nitric oxide was a more sensitive predictor of response (79%), compared with inhaled oxygen (64%), and was well tolerated, with no evidence of systemic effects. Elevation in right ventricular end-diastolic pressure appeared to predict poor vasodilatory response to iNO.

Nitric oxide is a safe and effective screening agent for pulmonary vasoreactivity. Regardless of etiology of pulmonary hypertension, pulmonary vasoreactivity is frequently demonstrated with the use of NO. Right ventricular diastolic dysfunction may predict a poor vasodilator response.

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.

Pulmonary hypertension is a potentially lethal complication of end-stage liver disease with a prevalence of 2%. In the setting of liver transplantation, the prevalence may be as high as 12%. Given the potential importance of this syndrome to the transplantation community, the purpose of this review is to summarize the current state of understanding of portopulmonary hypertension and to suggest potential management strategies for (1) liver transplant candidates with suspected pulmonary hypertension and (2) intraoperative pulmonary hypertension following liver allograft reperfusion.

Pulmonary hypertension is rarely described in association with Sjögren's syndrome. The authors report the case of a patient in which pulmonary hypertension was the inaugural clinical manifestation of primary Sjögren's syndrome. Clinical assessment, differential diagnosis, etiopathological implications, and therapeutic approach are discussed.

Pulmonary hypertension is a potentially lethal complication of end-stage liver disease with a prevalence of 2%. In the setting of liver transplantation, the prevalence may be as high as 12%. Given the potential importance of this syndrome to the transplantation community, the purpose of this review is to summarize the current state of understanding of portopulmonary hypertension and to suggest potential management strategies for (1) liver transplant candidates with suspected pulmonary hypertension and (2) intraoperative pulmonary hypertension following liver allograft reperfusion.

Pulmonary hypertension develops in approximately 2% of patients with portal hypertension. Diagnosis is often difficult and requires a high degree of clinical suspicion. Treatment of patients with portal and pulmonary hypertension is limited, and mean survival following diagnosis is approximately 15 months. The effect of liver transplantation on the natural history of disease is discussed.