Persistent Unexplained Dyspnea: A Case of Hepatopulmonary Syndrome

Campanile, Alfonso; Colombo, Alessandro; Pinto, Maurizio Del; Cavallini, Claudio

doi:10.1155/2017/1469893

Cited by 3 publications

(9 citation statements)

References 15 publications

(17 reference statements)

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“…There are multiple case reports featuring the different clinical features and diagnostic tests of HPS [9,10]. In our case, the interesting aspect is the young age of the patient with no co-morbid.…”

Section: Discussionmentioning

confidence: 82%

Hepatopulmonary Syndrome in a Thirteen Year Old Boy: A Case Report

Shahid

Din

Chaudhary

et al. 2019

Cureus

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Hepatopulmonary syndrome (HPS) is a late complication associated with liver cirrhosis. Recent studies have suggested that it can also occur in non-cirrhotic portal hypertension. The criteria that need to be met for making the diagnosis of HPS include chronic liver disease, impaired gaseous exchange leading to hypoxemia and intrapulmonary vascular dilation. The pathophysiology of this disease includes mediators like nitric oxide (NO), and endothelial growth factors which play an important role in intrapulmonary dilation of vessels. This, in turn, leads to ventilation-perfusion mismatch which is the main etiology of pulmonary symptoms associated with this syndrome. The classical symptoms include dyspnea, orthodeoxia and platypnea. Contrast echocardiography has emerged to be a very sensitive test for its diagnosis. The timing of appearance of microbubbles help to differentiate between intracardiac and intrapulmonary shunting, with the latter being the hallmark of HPS. The only curative treatment available is liver transplantation. Here we present a case of a 13-year-old patient who presented in Nishtar hospital in Multan, Pakistan. He initially presented with signs of liver cirrhosis but no definite cause could be found. The patient didn’t come for a follow-up after that. Now he presented with signs of HPS and acute decompensated liver disease. HPS was confirmed on contrast echocardiography. This is a rare presentation of HPS in this age group.

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Section: Discussionmentioning

confidence: 82%

Hepatopulmonary Syndrome in a Thirteen Year Old Boy: A Case Report

Shahid

Din

Chaudhary

et al. 2019

Cureus

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“…On average the prevalence is 25% [1,2]. It presents with exertional dyspnoea, platypnoea, orthodeoxia, cyanosis and digital clubbing [1][2][3]. The pathogenesis of HPS has not been clearly understood.…”

Section: Introductionmentioning

confidence: 99%

“…Failure of cirrhotic liver to clear the circulating pulmonary vasodilators may be a contributing factor. This leads to ventilation-perfusion mismatch in the lung, giving rise to the pulmonary symptoms [1,3]. The prognosis is poor when CLCD is complicated with HPS [1,3].…”

Section: Introductionmentioning

confidence: 99%

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Hepatopulmonary syndrome - An unusual complication of chronic liver cell disease

Senadheera,

Prasanna,

Karunarathne

et al. 2023

Anuradhapura Med. J.

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Intrapulmonary vascular dilatations in the presence of chronic liver cell disease (CLCD), portal hypertension or congenital portosystemic shunts result in hepatopulmonary syndrome (HPS). Cyanosis, platypnoea and orthodeoxia are the main clinical features and contrast echocardiography is used for its diagnosis. The only available curative therapy is liver transplantation. Here we report a case of 62-year-old lady with CLCD and portal hypertension, who was investigated for hypoxia, clubbing and cyanosis. The diagnosis of HPS was made with the aid of arterial blood gas analysis, saline bubble contrast echocardiography and computer tomographic pulmonary angiography.

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“…ст. (40,0 кПа)) может уменьшить гипоксию в некоторых случаях ГПС, поскольку оно обеспечивает давление, достаточное для частичного преодоления диффузионного ограничения, возникающего из-за расширенных легочных сосудов [9]. Однако PaO 2 не влияет на то, что гипоксия является результатом чрезмерного артериовенозного шунтирования.…”

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Hepatopulmonary Syndrome. Review

Potii¹,

Kiriienko²,

Глухова³

et al. 2021

Ukr. ž. med. bìol. sportu

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Liver cirrhosis is often accompanied by complications from the pulmonary system. These include hydrothorax, portopulmonary hypertension and hepatopulmonary syndrome. Hepatic hydrothorax affects about 6-10% of patients with end-stage disease, which results in the passage of ascetic fluid into the pleural space through diaphragm defects. The common cause of the hepatopulmonary syndrome and portopulmonary hypertension is portal hypertension and portosystemic shunting, indicating that vasoactive and angiogenetic factors originating from the liver normally control the pulmonary circulation. Portopulmonary hypertension is like pulmonary arterial hypertension, which develops against the background of portal hypertension as a result of chronic liver disease or without other causes of increased pressure in the pulmonary vessels. The prevalence of portopulmonary hypertension ranges from 2% to 8.5% among patients with portal hypertension and is associated with a poor prognosis. Hepatopulmonary syndrome is characterized by intrapulmonary dilatation of microvessels, which causes intrapulmonary shunting and leads to impaired gas exchange in liver diseases, and is associated with a decrease in the quality and duration of life in patients with cirrhosis. Nitric oxide overproduction and angiogenesis seem to be the hallmarks of a complicated pathogenetic mechanism, leading to intrapulmonary shunting and ventilation-perfusion mismatch. A classification of hepatopulmonary syndrome according to the severity of hypoxemia has been suggested. Hepatopulmonary syndrome includes a triad: hepatic dysfunction and / or portal hypertension, dilatation of intrapulmonary vessels, and increased alveolar-arterial oxygen gradient. The prevalence of hepatopulmonary syndrome varies depending on the study groups from 5% to 30%. The most common symptom of the complication is shortness of breath, but in most cases, hepatopulmonary syndrome is asymptomatic. A decrease in oxygen saturation less than 96% corresponds to a decrease in PaO2<70 mm Hg and testifies to the possible development of hepatopulmonary syndrome. In the case of a positive screening, the patient should undergo arterial blood gas analysis, which helps to determine PaO2 and alveolar to arterial oxygen gradient. Conclusion. Contrast-enhanced echocardiography with agitated saline is the gold standard in the diagnosis of intrapulmonary dilatation. The only effective treatment for hepatopulmonary syndrome is liver transplantation. Complete recovery of hepatopulmonary syndrome after liver transplantation is observed within a year in most patients with cirrhosis and hepatopulmonary syndrome

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Persistent Unexplained Dyspnea: A Case of Hepatopulmonary Syndrome

Cited by 3 publications

References 15 publications

Hepatopulmonary Syndrome in a Thirteen Year Old Boy: A Case Report

Hepatopulmonary Syndrome in a Thirteen Year Old Boy: A Case Report

Hepatopulmonary syndrome - An unusual complication of chronic liver cell disease

Hepatopulmonary Syndrome. Review

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