Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ KEY FEATURES +++ ESSENTIALS OF DIAGNOSIS ++ Progressive remodeling of the distal pulmonary arteries Dyspnea, malaise, chest pain, or exertional syncope Increased intensity of the pulmonic component of S2 ECG evidence of right ventricular (RV) hypertrophy On catheterization, end-expiratory mean pulmonary artery pressure (PAP) ≥ 25 mm Hg, pulmonary artery wedge pressure ≤ 15 mm Hg, and pulmonary vascular resistance (PVR) > 3 Wood units at rest +++ GENERAL CONSIDERATIONS ++ Uncommon disorder with an estimated prevalence of 15 to 50 per million population Characterized by elevated PAP and PVR, progressive right heart failure, and very poor survival Etiologies include inherited mutations, drug- or toxin-induced, or associated with connective tissue disease, congenital heart disease, portal hypertension, or HIV infection. Termed idiopathic pulmonary arterial hypertension (IPAH) when no causative factor is identified Bone morphogenetic protein receptor type II (BMPR2) gene mutations identified in 50% of familial cases and approximately 10% of sporadic cases Pathophysiology is pulmonary vasoconstriction, vascular remodeling, and in situ thrombosis Targeted treatment options are rapidly increasing, and overall prognosis is improving Pregnancy should be strongly discouraged because of high maternal and fetal mortality Noncardiac surgery is associated with high perioperative morbidity and mortality +++ CLINICAL PRESENTATION +++ SYMPTOMS AND SIGNS ++ Symptoms may be vague and subtle Dyspnea Exercise intolerance Exertional chest pain (angina) Dizziness and presyncope Syncope Palpitations Increased abdominal girth (ascites) Extremity swelling +++ PHYSICAL EXAM FINDINGS ++ Tachycardia Hypotension Hypoxia may or may not be present Normal or elevated jugular venous pressure with prominent a waves Left parasternal lift (enlarged RV) Increased intensity of P2 Holosystolic murmur of tricuspid regurgitation at the lower sternal border Diastolic murmur of pulmonic insufficiency at the left sternal border Hepatomegaly; pulsatile liver with severe tricuspid regurgitation Peripheral edema +++ DIFFERENTIAL DIAGNOSIS ++ Mitral stenosis Sleep apnea Chronic pulmonary embolism Autoimmune diseases such as scleroderma Left ventricular failure Congenital heart disease Pulmonary veno-occlusive disease +++ DIAGNOSTIC EVALUATION +++ LABORATORY TESTS ++ CBC (polycythemia suggests respiratory disease or congenital heart disease) Electrolytes, blood urea nitrogen, creatinine (prerenal azotemia suggests poor forward cardiac output or overdiuresis) Liver function tests (may be elevated with passive liver congestion from right heart failure) Serum brain natriuretic peptide and uric acid levels have prognostic value Tests to exclude secondary causes: antinuclear antibody, rheumatoid factor, HIV serology, thyroid function tests, urine toxicology for amphetamines/cocaine +++ ELECTROCARDIOGRAPHY ++ Sinus tachycardia Atrial flutter or fibrillation Right atrial enlargement RV hypertrophy Right bundle branch block +++ IMAGING STUDIES ++ Chest x-ray: RV enlargement Transthoracic echocardiography: – Findings associated with poor survival are right atrial enlargement, reduced RV function, and a pericardial effusion – Typical findings are ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.