Abstract
Hepatopulmonary syndrome is a liver disease complication characterized by the clinical triad of an advanced chronic liver disease, a pulmonary vascular dilatation, and a reduced arterial oxygenation (hypoxemia: PaO2 less than 70 mm Hg at rest) in the absence of intrinsic cardiopulmonary disease. Precapillary vasodilatation, intrapulmonary angiogenesis and hyperdynamic circulation lead to arteriovenous shunting and reduced gas diffusion. In case of detecting macroscopic shunts, the pulmonary artery branch embolization could be performed. However, the hepatopulmonary syndrome can be cured nowadays by means of liver transplantation only. A 5-year survival in these patients is about 70%. The observed mortality is the highest during the surgery or early after, especially in patients with PaO2 lower than 50 mm Hg. It ranges from 8.5 to 29%. A prolonged oxygenation support is often required after liver transplantation using invasive or non-invasive ventilation, or even extracorporeal membrane oxygenation. In this review, we have focused on the recent advances in this field as described in available literature and have presented a case report of successful liver transplantation in a patient with a severe hepatopulmonary syndrome.
Highlights
Hepatopulmonary syndrome is a liver disease complication characterized by the clinical triad of an advanced chronic liver disease, a pulmonary vascular dilatation, and a reduced arterial oxygenation in the absence of intrinsic cardiopulmonary disease
Precapillary vasodilatation, intrapulmonary angiogenesis and hyperdynamic circulation lead to arteriovenous shunting and reduced gas diffusion
A prolonged oxygenation support is often required after liver transplantation using invasive or non-invasive ventilation, or even extracorporeal membrane oxygenation
Summary
Одышка встречается у всех пациентов с ГПС, однако она может быть следствием асцита и гидроторакса, развивающихся при декомпенсации цирроза печени. При ГПС контраст проходит через расширенные легочные капилляры (нормальный диаметр капилляров легких составляет 8 мкм). При этом необходимо проводить дифференциальную диагностику между внутрисердечным и внутрилегочным шунтированием крови: у пациентов с внутрисердечным шунтированием контрастное вещество попадает в левые камеры через 1–2 сердечных цикла (раннее шунтирование), в то время как у больных с ГПС пузырьки появляются в левом предсердии только через 4–8 циклов (позднее шунтирование) [34, 35]. Описаний клинических случаев использования эмболизации легочных артерий при ГПС недостаточно, чтобы рекомендовать методику для применения в качестве рутинного метода лечения ГПС как первого, так и второго типа [40, 51, 52]
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