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Index of Pulmonary Vascular Disease (IPVD) of Yamaki and Tezuka for a Patient with Congenital Heart Disease and Pulmonary Hypertension

Purpose: To determine the index of pulmonary vascular disease (IPVD) for a patient with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH).

Description

Yamaki and Tezuka developed an index of pulmonary vascular disease (IPVD) to describe vascular changes in pulmonary arteries seen in patients with congenital heart disease and pulmonary hypertension. The IPVD is a weighted average of the histologic changes seen in pulmonary arteries included in a lung biopsy. The authors are from Tohoku University in Sendai, Japan.


Patient selection: congenital heart disease (CHD) with pulmonary arterial hypertension (PAH)

 

Parameters:

(1) grading of changes seen in the cross-section of an individual pulmonary artery

(2) the number of pulmonary arteries show each of the 4 grades

(3) the total number of pulmonary arteries examined

 

Changes in the Intima

Changes in Media

Grade

none

none

1

cellular reaction

none

2

fibrous and fibroelastic proliferation

none or minor

3

NA

partial or total destruction

4

 

IPVD =

= ((number of arteries showing Grade 1) + (2 * (number of arteries showing Grade 2)) + (3 * (number of arteries showing Grade 3)) + (4 * (number of arteries showing Grade 4))) / (total number of arteries examined)

 

Interpretation:

• minimum IPVD: 1

• maximum IPVD: 4

• The higher the IPVD the more severe the vascular changes.

• The higher the pulmonary hypertension the higher the IPVD.

 

Limitations:

• Determining the IPVD requires a representative lung biopsy large enough to provide an adequate number of pulmonary arteries.


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Specialty

Objective

ICD-10