Clinical and pathologic comparison of simple left-to-right shunt congenital heart disease and transposition of the great arteries with ventricular septal defect.

BACKGROUND

This study aimed to compare clinical and pathologic data for selected patients with congenital heart disease (CHD) and severe pulmonary hypertension (PH) treated with a diagnostic-treatment-and-repair strategy and to compare results for patients with pulmonary vascular disease (PVD) with simple left-to-right shunt CHD with patients with transposition of the great arteries (TGA) and ventricular septal defect (VSD).

METHODS

Group I comprised 38 patients with simple left-to-right shunt CHD and severe PH; group II included 11 older patients with TGA with VSD and severe PH; and group III comprised 6 autopsy cases of individuals with a normal circulation. The nature of the pulmonary arteries was determined by the Heath-Edwards classification system. All specimens were quantitatively analyzed.

RESULTS

Group I showed 31 patients with a change to grade I, 3 patients were grade II, 3 patients were grade III, and only 1 patient was grade IV. Group II showed 7 patients with a change to grade I, 2 patients were grade II, 1 patient was grade III, and only 1 patient was grade IV. The media wall thickness percentage (%MT), the media wall area percentage (%MS), and arteriole density were significantly higher in groups I and II than in group III. %MS was significantly higher in group II than in group I; no significant differences in %MT and arteriole density could be found between groups I and II.

CONCLUSIONS

The PVD in these selected patients with CHD and severe PH who were cared for with a diagnostic-treatment-and-repair strategy is generally reversible, and the changes in PVD in the patients with TGA and VSD were similar to those in the patients with simple left-to-right shunt CHD.