Pulmonary Hypertension Classification Review

April 26th, 2022 - Wyn Staheli, Director of Content
Categories:   Coding  

As of October 2017, there were several revisions to pulmonary hypertension coding. Although these codes have been around for quite some time, it would be good to review the rationale behind the reasons for the changes. The following information comes from the request for the code revisions (emphasis added).

Pulmonary hypertension (PH) is clinically classified into five groups, based on categories that share similar pathological findings, hemodynamic characteristics and management.

Group 1: Pulmonary Arterial Hypertension (PAH)
PAH is the most widely recognized category of PH, and includes the previously designated Primary Pulmonary Hypertension (PPH). PAH includes idiopathic PAH (IPAH) without an identifiable family history or risk factor, and heritable PAH such as that due to mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene. PAH also includes a number of secondary causes of PH: drug- and toxin-induced PH, and PH associated with other chronic conditions such as HIV infection, and congenital heart diseases. PAH due to congenital heart disease can be related to defects that cause a left to right shunt. However, over time with PAH, a right to left shunt may develop, in what is referred to as Eisenmenger's syndrome.

Group 2: PH due to left heart disease
This subgroup may be due to left heart failure (systolic or diastolic), or left heart valvular disease that may produce increase in left atrial pressure. Some patients with left heart valvular disease or left heart dysfunction can develop PH as severe as that seen in PAH.

Group 3: PH due to lung diseases and/or hypoxia
In this subgroup, the predominant cause of PH is alveolar hypoxia as a result of lung disease, impaired control of breathing, or residence at high altitude. Among those with pulmonary fibrosis and emphysema, the prevalence of PH is almost 50 percent.

Group 4: Chronic Thromboembolic PH (CTEPH)
Obstruction of pulmonary arterial vessels by thromboemboli, tumors, or foreign bodies can lead to CTEPH.

Group 5: PH with unclear multifactorial mechanisms
This group includes multiple forms of PH for which the etiology is unclear or multifactorial. The subgroups include hematologic disorders such as myeloproliferative disorders and splenectomy; systemic disorders such as sarcoidosis and pulmonary Langerhans cell histiocytosis; metabolic disorders such as glycogen storage disease, Gaucher disease and thyroid disorders; and other conditions that lead to PH.

This information can help provide valuable decision-making information on code selection.


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