Diagnosis workup – SSPH Algorhytm


General guidelines for the evaluation of pulmonary hypertension

Since the suspicion of PH may arise in various ways, the sequence of tests may vary. However, the diagnosis of PAH requires that certain data support a specific diagnosis. In addition, the diagnosis of idiopathic pulmonary arterial hypertension is one of excluding all other reasonable possibilities. Pivotal tests are those that are essential to establishing a diagnosis of any type of PAH either by identification of criteria of associated disease or exclusion of diagnoses other than IPAH. All pivotal tests are required for a definitive diagnosis and baseline characterization. An abnormality of one assessment (such as obstructive pulmonary disease on PFTs), does not preclude that another abnormality (chronic thromboembolic disease on VQ scan and pulmonary angiogram) is contributing or predominant. Contingent tests are recommended to elucidate or confirm results of the pivotal tests, and need only be performed in the appropriate clinical context.

The combination of pivotal and appropriate contingent tests contribute to assessment of the differential diagnoses in the right-hand column. It should be recognized that definitive diagnosis may require additional specific evaluations not necessarily included in this general guideline. 6MWT indicates 6-minute walk test; ABGs, arterial blood gases; ANA, antinuclear antibody serology; CHD, congenital heart disease; CPET, cardiopulmonary exercise test; CT, computerized tomography; CTD, connective tissue disease; CXR, chest X-ray; ECG, electrocardiogram; HIV, human immunodeficiency virus screening; Htn, hypertension; LFT, liver function test; PE, pulmonary embolism; PFT, pulmonary function test; PH, pulmonary hypertension; RA, rheumatoid arthritis; RAE, right atrial enlargement; RH Cath, right heart catheterization; RVE, right ventricular enlargement; RVSP, right ventricular systolic pressure; SLE, systemic lupus erythematosus; TEE, transesophageal echocardiography; VHD, valvular heart disease; and VQ Scan, ventilation-perfusion scintigram.

(ref. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. JACC 2009;53;1573-1619)