Lviv clinical bulletin 2016, 1(13): 43-57

https://doi.org/10.25040/lkv2016.01.043

The Modern View on the Problem of Respiratory Lesions in Patients with Liver Cirrhosis. Clinical Case Description

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M. Abrahamovych, S. Tolopko

Danylo Halytsky Lviv National Medical University

Introduction. The problem of liver disease is one of the most popular in modern gastroenterology, and liver cirrhosis (LC) is the main reason of hepatargy and hepatodepression. The imbalance of vasoactive substances on the one hand, and diffuse fibrosis with formed regeneration nodes on the other, lead to the increased portal vascular resistance and portal hypertension (PH) with the following disorders of systemic hemodynamics and circulation of almost all organs, including the lungs, resulting in hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH).

The aim of our work was to make an overview of the modern literature on the etiology, pathogenesis, clinical signs, principles of diagnosis and treatment of the lesions of the respiratory system in patients with LC and to describe the clinical cases from our own practice.

Materials and methods. The content analysis, the method of system and comparative analysis, the bibliosemantic method of study of actual scientific researches concerning HPS and PPH in patients with LC has been used.

Results. According to the classification, adopted in 2003 at the III International Conference in Vienna on the problems of primary pulmonary hypertension, PPH is defined as one of the forms of pulmonary hypertension, associated with PH, which is characterized by the increased pressure in the pulmonary artery more than 25.0 mm Hg resting and above 30.0 mm Hg – during physical activity, and increased pulmonary vascular resistance ≥120.0 dyn/sec/cm-5. Although the incidence of PPH is low, reported in various sources from 2.0 to 8.5 % in patients with PH, timely diagnosis of said condition and its correction is an actual problem of modern medicine, because of the extremely poor prognosis of the disease, when liver transplant is the only effective treatment, because of the absence of proven positive effects of pharmacological treatment.

To assess the different pathophysiological aspects of PPH it is important to combine the results of computer angiography of the lungs, magnetic resonance examination of the heart, echocardiography, ventilation-perfusion lung scanning, but the confirmation of the diagnosis is the method of right-heart catheterization with the measurement of pulmonary arterial pressure, cardiac output and pulmonary vascular resistance, that still remains the “gold diagnostic standard”.

HPS is an arterial oxygenation defect caused by the dilatation of intrapulmonary vessels or the occurrence of arteriovenous shunts, characterized by the reduction of oxygen partial pressure <70.0 mm Hg and increased alveolar- arterial gradient >20.0 mm Hg. It is believed, that the main role plays pulmonary hyperproduction of nitric oxide (NO) and carbon monoxide and, oddly enough, the E-1 by the increasing of the activity of NO-synthase, and thus NO production, as a result of the effect on EB-receptors. No less important are cytokines, such as the increase of TNFα, IL-1β synthesis and IL-10 decrease as a result of intestinal endotoxemia. There is a hypothesis of so-called alveolar-capillary oxygen imbalance, whereby inadequate hemoglobin oxygen saturation leads to systemic hypoxemia. Diagnosis of the HPS is based on complaints, physical examination results, pulse oximetry, the study of blood gases, lung CT, contrast transthoracic echocardiography, perfusion lung scans, using albumin, radiolabeled with 99Technetium. As for treatment, the most successful method is also a liver transplant, although some recommend medications. It is known, that methylene blue – guanylate cyclase and oxide synthase inhibitor – promotes rapid improvement of the patients after intravenous administration. You can also apply the common antidepressant from a group of selective serotonin reuptake inhibitors – paroxetine and also indomethacin, octreotide, which are potent inhibitors of NO synthesis, but their effectiveness is not yet proven. In the pilot study was noted the possibility of correction of pulmonary gas exchange by pentoxifylline, which is a blocker of TNFα. There have been attempts to use iloprost – selective vasodilator, that reduces pulmonary bypass and increases erythrocytes transit time in lungs.

Conclusions. Although at present there are no standard pharmacological treatment for patients with HPS and PPH, such patients require special attention, constant monitoring and prolonged oxygen therapy. Prognosis in such patients is poor, there is a high mortality, requiring their early diagnosis.

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