Table 2.
—PH ICD-9-CM Codes vs Chart Review Diagnoses
| Chart Review |
|||
| Code, Year | No. | Expert-Reviewer Diagnosis | No. |
| ICD-9 code, 1999 | |||
| 416.0 Idiopathic PAH | 30 | IPAH | 2 (probable, 0; possible, 2) |
| Unclear | 3 | ||
| Not IPAH | 25 | ||
| PAH | 9 | ||
| Congenital heart disease | 6 | ||
| Connective tissue disease | 2 | ||
| Cirrhosis | 1 | ||
| Left-sided heart disease | 12 | ||
| Lung disease | 3 | ||
| Chronic pulmonary emboli | 1 | ||
| 416.8 Other chronic pulmonary heart disease | 2 | Not IPAH | 2 |
| Lung disease | 2 | ||
| 416.9 Pulmonary heart disease unspecified | 2 | Not IPAH | 2 |
| Left-sided heart disease | 1 | ||
| Lung disease | 1 | ||
| ICD-9 code, 2002 | |||
| 416.0 Idiopathic PAH | 3 | IPAH | 1 (probable, 0; possible, 1) |
| Not IPAH | 2 | ||
| Lung disease | 1 | ||
| Sarcoid | 1 | ||
| 416.8 Other chronic pulmonary heart disease | 22 | IPAH | 1 (probable, 0; possible, 1) |
| Unclear | 6 | ||
| Not IPAH | 15 | ||
| PAH | 3 | ||
| Congenital heart disease | 3 | ||
| Left heart disease | 5 | ||
| Lung disease | 5 | ||
| Chronic pulmonary emboli | 1 | ||
| Sarcoid | 1 | ||
| 416.9 Pulmonary heart disease unspecified | 2 | Not IPAH | 2 |
| PAH | 1 | ||
| Congenital heart disease | 1 | ||
| Lung disease | 1 | ||
Most patients coded as IPAH were found to have comorbidities, suggesting PH associated with another condition, including left-sided heart disease (n = 12), lung disease (n = 4), and congenital heart disease (n = 6), among others, with the years 1999 and 2002 combined. PAH = pulmonary arterial hypertension. See Table 1 legend for expansion of other abbreviations.