Two successful pregnancies in a patient with advanced liver cirrhosis and hepatopulmonary syndrome.
Ella Veitsman, Mordechai Yigla, Israel Thaler, Yaacov Baruch
Abstract
Ella Veitsman, Mordechai Yigla, Israel Thaler, Yaacov Baruch
Abstract
Hepatopulmonary syndrome (HPS) is characterized by the presence of chronic liver disease, intrapulmonary vascular dilatation, and arterial hypoxemia.1 The pathophysiology of the pulmonary features involve ventilation-perfusion mismatch, oxygen diffusion limitation, and varying degrees of intrapulmonary shunting. The natural history of HPS is poorly understood to date, and significant mortality, despite therapeutic attempts to correct hypoxemia, has been reported.1 The role of liver transplantation in this condition and the timing of transplantation is still controversial. Nevertheless, the progressive worsening of hypoxemia, irrespective of Child-Pugh class, may be considered an indication for liver transplantation.2,3 Pregnancy in patients with liver cirrhosis is not common. There is significant perinatal mortality, and the effect of pregnancy on the underlying liver disease remains uncertain. Pregnancy in a patient with lung disease, however, is known to have an adverse effect on both the woman and the fetus. In this case report, we describe the successful completion of 2 pregnancies in a woman with autoimmune type I hepatitis and Child-Pugh class B cirrhosis complicated by HPS.
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Hepatopulmonary syndrome (HPS) is characterized by the presence of chronic liver disease, intrapulmonary vascular dilatation, and arterial hypoxemia.1 The pathophysiology of the pulmonary features involve ventilation-perfusion mismatch, oxygen diffusion limitation, and varying degrees of intrapulmonary shunting. The natural history of HPS is poorly understood to date, and significant mortality, despite therapeutic attempts to correct hypoxemia, has been reported.1 The role of liver transplantation in this condition and the timing of transplantation is still controversial. Nevertheless, the progressive worsening of hypoxemia, irrespective of Child-Pugh class, may be considered an indication for liver transplantation.2,3 Pregnancy in patients with liver cirrhosis is not common. There is significant perinatal mortality, and the effect of pregnancy on the underlying liver disease remains uncertain. Pregnancy in a patient with lung disease, however, is known to have an adverse effect on both the woman and the fetus. In this case report, we describe the successful completion of 2 pregnancies in a woman with autoimmune type I hepatitis and Child-Pugh class B cirrhosis complicated by HPS.
Key concepts: Hepatopulmonary syndrome, Medicine, Hypoxemia, Cirrhosis, Liver transplantation, Liver disease, Pregnancy, Chronic liver disease