RESUMEN
Aim and Background: hepatopulmonary syndrome [HPS] is defined ccs triad of chronic liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. Presence of HPS in pediatric age group is an indication liver transplantation. We investigated the presence of HPS in 25 children [13 boys and 12 girls], age ranged from 2-15 years, with cirrhosis [8 Child-Pugh class A, 10 class B, and 7 class C]
Methods: they underwent detailed clinical evaluation, x-ray chest, ECG, and contrast enhanced transthoracic Doppler Echocardiographic examination using saline, results were compared to a control group of 8 healthy matched children
Results: children with cirrhosis had increased heart rate, decreased, mean arterial blood pressure, peak velocity of aortic blood flow, and increased cardiac output that were statistically significant [p<0.03, p<0.003, p< 0 .002, and p<0.03] when compared to controls. Contrast enhanced Echo was positive in 4 patience with arterial P alfa O2 < 70 mmHg [Child-Pugh grade CI: HPS +ve group. and was negative in all other patients whom arterial P alfa O2 < 70 mmHg [Child-Pugh class A=8, class B=10. class C=3]: HPS -ve group. When compared to controls, HPS +ve group have statistically significant increase in Echo-derived aortic diameter [p<0.004] and cardiac output [p< 0.006]. In addition, there was significant difference as regards age [p< 0.01], Echo-derived aortic diameter [p> 0.004] and cardiac output [p> 0.001] between HPS group and rest of patients
Conclusion: children with cirrhosis have hyper-dynamic circulatory status ;as evidenced by decreased mean arterial: blood pressure and increased cardiac output. Contrast enhanced Echocardiography is a sensitive as well as specific tool to diagnose HPS in children. HPS in children is associated with advancement in age and in Child-Pugh classification