William T
 | 31-year-old male |
| | Unmarried |
| | Active throughout childhood but limited ability to participate in competitive sports |
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Weight: 214 lb
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Height: 5'11"
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History
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Diagnosed with VSD at 1 year of age due to signs of left-sided congenital heart
failure
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VSD surgically repaired at 16 months of age
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Symptoms
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At age 28 years, developed palpitations and irregular wide tachycardia >200 bpm
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Exertional dyspnea
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Lower extremity edema
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Orthopnea at rest
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Reasons to investigate PAH
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Tachycardia
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Repaired VSD
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Progressive exertional dyspnea and orthopnea at rest
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Physical examination
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Vital signs: BP 100/62 mm Hg; HR 74 bpm; RR 24 bpm; O2 sat 93% (room
air)
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CV: RV systolic heave at left sternal border and left midprecordium, palpable pulmonary
valve closure, normal S1, loud P2, 3/6 holosystolic murmur loudest at left midsternal
and lower sternal borders, RV S4 gallop
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Lungs: clear to auscultation bilaterally, no wheezes or crackles
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Neck: JVP 13 cm with visible a and v waves
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Chest: well-healed midline sternotomy scar
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Abdomen: hepatomegaly with pulsatile liver
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Extremities:
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2+ bilateral pitting edema to the knees
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No clubbing or cyanosis
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Normal pulses
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Laboratory findings
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CBC unremarkable
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Chem-7 normal
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LFTs normal
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BNP: 1200 pg/mL
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Other Findings
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6MWD: 205 m
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12-lead ECG demonstrated sinus rhythm with first-degree AV block, right axis deviation,
RBBB with RVH, and associated ST- and T-wave abnormalities, LA abnormality
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Chest x-ray findings
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Cardiomegaly, with evidence of RA and RV enlargement
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No significant increase in lung vascularity, suggesting absence of significant left-to-right
shunt
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Echocardiogram findings
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Small, D-shaped LV with preserved systolic function
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No residual VSD shunt
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Severely dilated RA and RV
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Moderately high-pressure tricuspid regurgitation of >4 m/sec
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Dilated IVC with minimal change on inspiration (3.6 cm; normal <2.5 cm)
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Consistent with elevated RAP of
15 to 20 mm
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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.
