Lung biopsy findings in previously inoperable patients with severe pulmonary hypertension associated with congenital heart disease

Background

Congenital heart disease with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary vascular disease (PVD), has been successfully corrected at our institution recently. Whether the PVD is reversible remains unknown. This study aimed to examine the nature of the pulmonary arterial vessels in these selective patients.

Methods

All patients with congenital heart disease and severe pulmonary hypertension (PH) were selected using Diagnostic-treatment to undergo radical repair (n = 49). Lung biopsy specimens were obtained during operation. The nature of PVD was determined by Heath-Edwards classification system. All specimens were quantitatively analyzed by calculating percentage media wall area, percentage media wall thickness and arteriole density.

Results

Transcutaneous oxygen saturation of all selected patients increased significantly after Diagnostic-treatment (P < 0.001). There were no operative deaths. Mean pulmonary artery pressure and pulmonary vascular resistance regressed significantly postoperatively (P < 0.001). The incidence of postoperative PH was 59.2% (29/49). Of 49 selected patients with severe PH, 38 (77.6%) showed grade I change, 5 (10.2%) showed grade II change, 4 (8.2%) showed grade III change and only 2 (4%) showed grade IV change with plexiform lesion. The percentage media wall area, percentage media wall thickness and arteriole density were significantly increased in patients associated with PH than in normal subjects (P < 0.001). Follow-up data showed the reversal of PVD in these 2 patients with plexiform lesions.

Conclusions

The PVD in these selective patients with congenital heart disease and severe PH using a Diagnostic-treatment-and-Repair strategy is generally reversible and these patients are operable in current era.