Pulmonary hypertension and pulmonary heart disease

The documentation and coding of pulmonary hypertension and pulmonary heart disease (also known as cor pulmonale) to accurately reflect the patient's condition are surprisingly challenging.

Photo by Thinkstock
Photo by Thinkstock

Documentation requirements for correct coding of these conditions are more specific than what is needed to describe and communicate clinically. If the documentation does not allow correct code assignment, the severity and significance of the patient's condition may be inaccurately captured for quality metrics and reimbursement, including Medicare's pay-for-performance programs.

Pulmonary hypertension

Pulmonary hypertension is defined by a mean pulmonary artery pressure of 25 mm Hg or more. It is classified as primary (without apparent cause) or secondary (due to another condition). Causes include chronic pulmonary disease, pulmonary vascular disease including pulmonary embolism, and left-heart disease (Table 1). It is often associated with chronic hypoxic states like chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea.

Pulmonary heart disease

Pulmonary heart disease, or cor pulmonale, describes right-heart dysfunction due to pulmonary hypertension. Elevated pulmonary artery pressure increases right ventricular workload with resulting myocardial hypertrophy and diastolic dysfunction ultimately leading to diastolic right-heart failure. Pulmonary heart disease, as used in this column, is intended to include “cor pulmonale” since the terms are equivalent.

Chronic pulmonary heart disease becomes “acute” whenever decompensation occurs, often the result of an exacerbation of an associated pulmonary condition such as COPD. In the absence of pre-existing chronic pulmonary heart disease, pulmonary embolism may cause acute pulmonary heart disease, a grave prognostic sign.

Clinical findings associated with pulmonary heart disease include jugular venous distension, prominent v-wave in the jugular venous pulsation, narrow S-2 split or no split (slight delay in closure of the pulmonic valve), and, in advanced stages, peripheral edema or ascites. Doppler echocardiography may demonstrate a number of findings (Table 2), but right-heart strain suggests an acute decompensation.

Documentation and coding challenges

Precise documentation terminology is required for correct code assignment and severity of illness classification of a patient's condition. Reimbursement determinations, quality metrics, and other data analysis depend on the codes assigned.

With pulmonary heart disease, documentation of acuity is crucial. A diagnosis of acute pulmonary heart disease (or acute cor pulmonale) is assigned a high level of severity. Also, the term “pulmonary hypertension with acute right-heart [or ventricular] strain [or failure]” is classified as acute pulmonary heart disease having high severity.

Keep in mind that patients with chronic pulmonary heart disease due to COPD or other chronic lung diseases often experience acute pulmonary heart disease in association with an exacerbation of their pulmonary condition—an important diagnostic and documentation consideration.

Chronic pulmonary heart disease contributes little or nothing to severity classification, but if there is co-existing right-heart failure, documentation of diastolic heart failure does contribute to severity. Heart failure in chronic pulmonary heart disease is rarely systolic in nature.

The diagnosis of pulmonary hypertension, whether acute or chronic, has little, if any, impact on severity classification. Other terms that do not express significant severity include right ventricular hypertrophy, failure, or dysfunction; right-heart failure; elevated right ventricular pressure; and right-heart strain.


It is essential to identify and document acute pulmonary heart disease (acute cor pulmonale) whenever it occurs. Documentation of “pulmonary hypertension with acute right-heart [or ventricular] strain [or failure]” is also classified as acute pulmonary heart disease.

Pulmonary embolism may cause acute pulmonary heart disease. Consider the possibility of an “acute” decompensation of pre-existing chronic pulmonary heart disease, especially in patients with an exacerbation of COPD and other chronic lung diseases. Likewise, ask yourself and document if any patient with pulmonary hypertension has “acute right-heart strain” or other evidence of acute pulmonary heart disease.

For correct coding, a specific diagnosis must be stated by a clinician in the record; inpatient codes cannot be assigned based on reports only.