A Brief Synopsis of AFP's "Pulmonary Arterial Hypertension: An Update on Diagnosis and Treatment"

Pulmonary Arterial Hypertension: An Update on Diagnosis and Treatment
RICHARDSTRINGHAM, MD, and NIPAR. SHAH,MD,University of Illinois at Chicago College of Medicine, Chicago, Illinois
Am Fam Physician. 2010 Aug 15;82(4):370-377
http://www.aafp.org/afp/2010/0815/p370.pdf
     Pulmonary arterial hypertension presents as dyspnea on exertion with chest pain, fatigue, and palpitations. It is often asymptomatic. Diagnosis is often delayed, and many times may be an incidental finding.  It has a large differential and may be overlooked. It is diagnosed by an elevated mean pulmonary arterial pressure greater than 25 mmHg at rest (normal is 15 mmHg). Causes can be idiopathic, heritable (BMPR2 gene), drug or toxin induced, or associated with HIV, systemic sclerosis, sickle cell anemia, heart disease, schistosomiasis, or portal hypertension. Physical examination findings include a right parasternal heave, tricuspid regurgitation, a loud second or third heart sound, JVD, ascites, or hepatomegaly. An echocardiogram may show RVH, septal systolic strain/ sprain,  or right axis deviation. A chest x ray may show bilateral hilar enlargement, right ventricular enlargement, or a prominent central pulmonary artery. Routine lab work includes CBC, chemistry, thyroid function, ANA, anti-Scl-70, anti-centromere, and anti-ribonucleoprotein. Right heart catheterization is used to confirm the diagnosis and evaluate the severity.
     Functional classification of PAH is based primarily on symptoms. Class I has no symptoms or limitations of physical activity. Class II has slight limitations of activity with symptoms. Class III has marked limitations with symptoms during less than normal activity. Class III patients are unable to carry out activity without symptoms.
     Standard treatment includes coumadin (INR 1.5-2.5), diuretics, oxygen and digoxin, although randomized trials are lacking. Newer treatment includes prostacyclins (epoprostenol, iloprost, and treprostinil), endothelin receptor antagonists (bosentan and ambrisentan) and phosphodiesterase type 5 inhibitors (sildenafil and tadalafil).  Combinations may be helpful.  Vaccines for pneumococcus and influenza should be given. There is no cure and prognosis is generally poor.