Korean J Pathol.  2002 Dec;36(6):412-415.

Plexogenic Pulmonary Arteriopathy in Congenital Heart Disease: A Report of Two Cases

• Ha SY
• Park KY
• Cho HY
• Oh YH
• Chung JG
• Chung DH
• Kim CY
• Kim HK

Abstract

Hypertensive pulmonary vascular disease can develop in those cases of congenital cardiac shunt in which critical levels of pulmonary artery pressure and flow are reached and exceeded. We have experienced two cases of plexogenic arteriopathy in complex congenital heart disease and tried to evaluate of distribution of arterial lesions by total mapping of the explanted lung. Case 1 and 2 were 12-year-old boy and 36 year-old man. They were treated with combined heart-lung transplantation. Mapping of the both lungs was done, and graded according to Heath and Edward's grading scheme. The elastic pulmonary artery was tortuous, dilated and aortic configuration. Both lungs showed mostly grade 3. Plexiform lesion or veinlike branches of hypertrophied muscular arteries arosed in a lateral branch of a muscular artery that might be proximal to an area of occlusion. Comprising the right and left lung, the right was more severe than the left. By getting closer to the distal part, the grade tended to increase to 4 to 5. By analyzing the pulmonary lobe, severe pulmonary hypertension of grade 4 or 5 was comparatively disseminated throughout the right lung. On the other hand, in the left lung, the grade of the lower lobe was higher than that of the upper lobe, and within the upper lobe, there was a tendency for the grade of inferior segment to be higher than that of the corresponding apical segment.

Keyword

Hypertension; Pulmonary; Congenital Heart Disease

MeSH Terms

Adult
Arteries
Child
Hand
Heart Defects, Congenital*
Heart-Lung Transplantation
Humans
Hypertension
Hypertension, Pulmonary
Lung
Male
Pulmonary Artery
Vascular Diseases