Adapted from information provided by physicians at PH centers

Julia H   35-year-old female with 3 children   Raising children and performing daily activities with no limitation   Chief complaint: exertional dyspnea
History   Hypothyroidism   No smoking, alcohol abuse, recreational drug use, or use of anorexigens   1 month earlier, preop CXR revealed enlarged pulmonary arteries Symptoms   2-year history of exertional dyspnea   Symptomatic when climbing stairs or walking on incline Medication   L-thyroxine 75 μg po QD
Physical examination
BP: 112/78 mm Hg   SpO2: 95% on room air   No JVD   Breath sounds clear	Normal 1st heart sound, but louder 2nd heart sound; no audible murmurs   No peripheral edema
Laboratory findings	Additional studies
Results within normal limits: —  LFTs —  Blood counts —  Thyroid function tests —  Chemistry   HIV negative   ANA normal	PFTs —  Mild restrictive defect —  DLCO: 50% of predicted —  ABG: pH 7.42 / 31 / SpO2 94%   6MWD —  389 meters   HRCT: No ILD or PE
Echocardiogram findings   Marked RV and atrial enlargement   Displaced intraventricular septum   Normal LV size and function   Estimated PASP 75 mm Hg   Negative saline contrast study
Other conditions ruled out based on diagnostic results   Interstitial lung disease   Pulmonary embolism	Reasons to investigate PAH   Enlarged pulmonary arteries   Isolated low DLCO   Louder second heart sound   History of exertional dyspnea   Hypothyroidism
Right heart catheterization findings
Hemodynamics Results Normal ranges1-3 Abnormal PAP (mm Hg): Mean Peak systolic End diastolic   68 105 47   8-20 15-30 4-12   mRAP (mm Hg) 12 1-5 mPCWP (mm Hg) 10 4-12 CO (L/min) [Fick] 4.2 4-8 CI: (L/min/m2) [Fick]   2.2   2.8-4.2   PVR*: Wood units dyn•sec/cm5   13.8 1104   0.3-1.6 20-130   *PVR = 80 × (mPAP – mPCWP)/CO.4 PVR provides a measure of the vascular resistance in the lungs and is an important prognostic indicator in PAH.5
Vasoreactivity negative —  Vasoreactivity testing will help discern whether a patient is responsive to treatment with CCBs. It will also offer prognostic information in positive responders. However, most patients with IPAH and PAH will not respond positively to vasoreactivity testing.5

Diagnosis: Idiopathic PAH, functional class II

Recommendation: Start Tracleer first

6MWD six-minute walk distance; ABG arterial blood gas; ANA antinuclear antibodies; BP blood pressure; CI cardiac index; CO cardiac output; CXR chest x-ray; DLCO diffusing capacity of lung for carbon monoxide; HRCT high-resolution computed tomography; HIV human immunodeficiency virus; ILD interstitial lung disease; IPAH idiopathic pulmonary arterial hypertension; JVD jugular vein distension; LFT liver function test; LV left ventricle; mPAP mean pulmonary arterial pressure; mPCWP mean pulmonary capillary wedge pressure; PASP pulmonary arterial systolic pressure; PE pulmonary embolism; PFT pulmonary function test; PH pulmonary hypertension; PVR pulmonary vascular resistance; QD once daily; RAP right atrial pressure; RV right ventricle; SpO₂ oxygen saturation on pulse oximetry; VSD ventricular septal defect.

Tracleer is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) 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 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 including BOXED WARNING.

Review EARLY study results

1. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(suppl S):S55-S66.
2. Davidson CJ, Bonow RO. Cardiac catheterization. In: Libby P, Bonow RO, Mann DL, Zipes DE. Braunwald's Heart Disease. 2 vols. 8th ed. Philadelphia, PA: Saunders Elsevier; 2008:439-463.
3. Cardiovascular Tests and Procedures: Cardiac Catheterization. Merck Manual Professional website. http://www.merck.com/ mmpe/sec07/ch070/ch070b.html. Accessed February 2, 2010.
4. Galiè N, Rubin LJ, Hoeper MM, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double blind, randomised controlled trial. Lancet. 2008;371:2093-2100.
5. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009;53:1573-1619.