Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries.

Abstract

SUMMARY Forty autopsy cases of complete transposition of the great arteries (TGA) and 22 autopsy cases of ventricular septal defect (VSD) were analyzed histologically for evidence of vascular damage due to pulmonary vascular disease (PVD). Positive correlations were generally observed between an index of pulmonary vascular disease (IPVD) and blood pressure of pulmonary circulation. No significant difference in IPVD was found between TGA and VSD in the first five months of life, when cases of each disease were compared at similar blood pressure levels. After that age, however, IPVD was much higher in TGA, and particularly severe PVD in this disease was DEVELOPMENT OF PULMONARY VASCULAR DISEASE (PVD) as a result of pulmonary hypertension in some congenital heart diseases is a major factor in the prognosis and postoperative course of patients. Although the role of pulmonary hypertension in inducing pulmonary vascular lesions is established in general terms, a number of authors have pointed out that complete transposition of the great arteries (TGA) in particular is characterized by early onset and development of severe vascular changes.'-3 The high vulnerability of the pulmonary artery in TGA suggests that factors other than elevated blood pressure also play a role in the production of vascular lesions. For this reason, a structural analysis of the arterial wall may contribute to the understanding of vascular lesions in this disease.Histopathological studies seem to have been limited to direct correlation of histological vascular lesions with clinical records of blood pressure.3 ' The response of the pulmonary artery to elevated blood pressure prior to the appearance of vascular injuries has not yet been sufficiently investigated. Histometrical and histological methods are used in this study to quantitate hypertrophy of the arterial muscular coat and to estimate the grade of vascular lesions. These methods are expected to facilitate the analysis of the behavior of the muscular coat to elevated blood pressure and the relation of pulmonary hypertension to vascular lesions. VSD. None were complicated by other serious cardiac malformations, except for three cases of associated coarctation of the aorta in the age group under 5 months. In three other cases, the patients had each undergone a Mustard procedure at least eight months before death. For comparative analysis, 22 autopsy cases of VSD (aged from 46 days to 24 years), and 33 autopsy cases of apparently normal cardiovascular systems and normal pulmonary circulation (aged from 10 months of gestation to 32 years) were studied. Premature infants were excluded from the control group. Pulmonary arterial pressure had been recorded during catheterization within two months prior to death in 23 cases of TGA and in all the cases of VSD. From the record the peak systolic pressure was estimated in each case and used for correlation with vascular lesions and medial hypertrophy (table 1). About 30 samples of lung were randomly excised from formalin-fixed specimens of both lungs ...

Prenatal Features of Ductus Arteriosus Constriction and Restrictive Foramen Ovale in d-Transposition of the Great Arteries

“…One could speculate that the common occurrence of persistent pulmonary hypertension and early progression of pulmonary vascular disease in the infants with TGA and intact ventricular septum may be related to the prenatal increase in pulmonary blood flow and prenatal alteration of the pulmonary vascular bed. [7][8][9][25][26][27] In our study, in fact, patient 1, with prenatal constriction of the ductus arteriosus, had such pulmonary vascular changes as previously described in TGA. 10 Furthermore, on review of the autopsy records at the Hospital for Sick Children, in the past 10 years, we found that 3 of 7 newborns with TGA/intact ventricular septum and preoperative death had established pulmonary vascular changes.…”

Pulmonary vascular disease in shunted and nonshunted patients with tetralogy of fallot.

“…The thickness of the media at the radius of 100 ,um in small pulmonary arteries was measured using a previously reported method of assessment (Yamaki and Tezuka 1976;Yamaki and Wagenvoort 1981), and given in terms of mean + s.D. Comparison of the thickness of the media was made among the normal control cases and shunted or nonshunted cases in various age groups and differences between mean values were analyzed using student t-test, and a p value less than 0.05 was accepted as the level of significance.…”

Significance of lung biopsy in determining surgical indication in cases of congenital cardiac anomalies with pulmonary hypertension.

“…The degree of medial hypertrophy of small pulmonary arteries was determined using Suwa's method (Suwa and Takahashi 1971;Yamaki and Tezuka 1976;Yamaki and Horiuchi 1979) and expressed as the thickness of the media, 100 µm in radius of small pulmonary arteries (DR=1ooµm). The severity of intimal alteration of pulmonary arteries was determined according to the index of pulmonary vascular disease (IPVD) reported previously Tezuka 1976, 1979;Yamaki et al 1980;Yamaki and Wagenvoort 1981).…”