What are the ISHLT criteria for referral and listing for lung transplantation in patients with pulmonary arterial hypertension?

Updated: Aug 19, 2019
  • Author: Bryan A Whitson, MD, PhD; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
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The median survival for patients with primary pulmonary hypertension is 2.8 years. The indicators of poor survival are NYHA functional class III or IV, elevated mean right atrial pressure, elevated mean pulmonary arterial pressure and decreased cardiac index, and reduced diffusion. Mean pulmonary arterial pressure greater than 85 mm Hg is associated with a median survival of less than 12 months. A response to vasodilator therapy is associated with improved survival.

Present treatment of choice for NYHA class III and IV patients with pulmonary hypertension is long-term prostacyclin therapy, especially if they fail to demonstrate vasoreactivity during formal vasodilator trial. Prostacyclin has demonstrated improved survival, improved exercise capacity, and better quality of life. [19, 20] Transplantation is indicated only if the patient cannot tolerate or fails prostacyclin therapy. In patients who have developed severe right heart failure, the right heart pressures and functions return to near normal values following lung transplantation alone.

ISHLT criteria for referral for transplantation in patients with pulmonary hypertension are as follows [16] :

  • NYHA functional class III or IV symptoms during escalating therapy

  • Rapidly progressive disease (assuming weight and rehabilitation concerns not present)

  • Use of parenteral targeted pulmonary arterial hypertension (PAH) therapy regardless of symptoms or NYHA Functional Class

  • Known or suspected pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis

ISHLT criteria for listing for transplantation in patients with pulmonary hypertension are as follows [16] :

  • NYHA functional class III or IV despite a trial of at least 3 months of combination therapy including prostanoids

  • Cardiac index < 2 L/min/m2

  • Mean right atrial pressure >15 mm Hg

  • 6-minute walk test < 350 m

  • Development of significant hemoptysis, pericardial effusion, or signs of progressive right heart failure 

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