Congenital heart disease patients are at risk of developing PAH1

• Approximately 34% of patients with unclosed atrial septal defects and 12% of patients with closed atrial septal defects may have PAH2
• Approximately 50% of patients with large (>1.5 cm) ventricular septal defects may develop Eisenmenger syndrome, the most advanced form of PAH associated with congenital heart disease3

Defined as a reversal of the left-to-right shunt resulting from severe pulmonary hypertension4 One of the most debilitating of all congenital heart diseases5 Accompanied by compromised hemodynamics Marked by increased endothelin expression*1 *Statement is based on observations reported from in vitro or animal trials. The clinical significance in humans is unknown.

Tracleer is indicated for the treatment of pulmonary arterial hypertension (PAH) in patients with WHO Class II-IV symptoms, to improve exercise ability and decrease the rate of clinical worsening. Patients with WHO Class II symptoms showed reduction in the rate of clinical deterioration and a trend for improvement in walk distance. Physicians should consider whether these potential benefits are sufficient to offset the risk of liver injury in WHO Class II patients, which may preclude future use as their disease progresses.

IMPORTANT SAFETY INFORMATION

Because of the associated risks, Tracleer may be prescribed only through the Tracleer Access Program. Potential for serious liver injury (including, after prolonged treatment, rare cases of liver failure and unexplained hepatic cirrhosis in a setting of close monitoring)—Liver monitoring of all patients is essential prior to initiation of treatment and monthly thereafter. High potential for major birth defects—Pregnancy must be excluded and prevented through the use of reliable forms of birth control; monthly pregnancy tests should be obtained.
Contraindicated for use with cyclosporine A and glyburide.

Please see full prescribing information.

REFERENCES

1. Benza RL, Rayburn BK, Tallaj JA, et al. Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest. 2006;129:1009–1015.

2. Engelfriet PM, Duffels MGJ, Möller T, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682-687.

3. Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(suppl S):5S-12S.

4. Christensen DD, McConnell ME, Book WM, Mahle WT. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am J Cardiol. 2004;94:261–263.

5. Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828–835.

• HEALTHCARE PROFESSIONALS

• About PAH

• Patients at Risk for PAH

• About Tracleer

• 
  Tracleer in FC II —
  EARLY Study

• Prescribing Tracleer

• PAH Resource Links

• PATIENTS

• About Actelion