Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS).

Hypoplastic left heart syndrome (HLHS) is an inborn complex heart malformation. A multi-stage treatment is initiated in a neonatal period with a Norwood surgery. The next step is Glenn surgery - a bidirectional superior cavo-pulmonary anastomosis. At the last stage anastomosis of inferior vena cava (IVC) with the right pulmonary artery (RPA) is formed as a result of a Fontan surgery. The aim of this study was to assess lung perfusion in patients with HLHS after completion of a surgical therapy, using a scintigraphic method. In 92 patients with HLHS a planar lung scintigraphy in anterior and posterior projections after administration of 99mTc-macroaggregates in activity 18-111MBq was carried out twice (in several day intervals). At first, a radiopharmaceutical was administered to the right extremity in order to assess the lung distribution of blood flowing through the anastomosis of superior vena cava (SVC) with RPA. In the next study, after administration of the tracer to the right lower extremity, the distribution of blood flowing through the anastomosis of IVC with RPA was assessed. The relative percentage of each lung in the total lung perfusion was calculated on a Xeleris workstation using the "Lung perfusion analysis" program. Lung perfusion was considered close to symmetrical when the proportion was in the range of 40-60%. In spite of the fact that mean relative values of distribution of blood flowing through the anastomosis of SVC with RPA to the left lung (LL) and right lung (RL) in the entire study group did not differ significantly: LLmean = 47%; RLmean = 53%, p = 0.14, relative values of perfusion of both lungs were differentiated - in 26% of patients LL was better perfused, in 38% RL was better perfused and in 36% a perfusion of both lungs was similar. The analysis of blood distribution by anastomosis of IVC with RPA showed that the mean relative perfusion of RL was significantly higher than that of LL (70% vs. 30%, p < 0.0000001). No signs of pulmonary emboli were detected. In 13% of studied patients, uptake of the radiopharmaceutical in kidneys was shown indicating the presence of shunt "from right to left". After completion of surgical treatment of patients with HLHS, differentiated blood supply of the lungs was observed through SVC with RPA anastomosis and a tendency to higher blood supply of RL than LL by anastomosis of IVC with RPA. No signs of pulmonary embolism were detected. The study revealed a "right to left" shunt in some patients.