Not a Simple Cyst: Hepatic Cyst Causing Intra-cardiac Shunting Relieved by Percutaneous Drainage
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Hepatic cysts are a relatively benign and asymptomatic group of disorders, which are usually found incidentally during imaging studies. We present a case of a large hepatic cyst causing intra-cardiac shunting during a workup of hypoxemia. A 68 year old male, with recently diagnosed marginal zone lymphoma of the lung, presented to the ED for dyspnea on exertion and hypoxia requiring 100% FiO2 by high-flow nasal cannula. ABG showed severe A-a gradient with PaO2 of 42 and SaO2 of 82, concerning for cardiac shunt. Transesophageal echocardiogram showed right to left inter-atrial shunt, across a patent foramen ovale (PFO), following injection of agitated saline. Further workup with CT Chest PE protocol ruled out pulmonary embolus and right-sided cardiac catheterization did not show evidence of pulmonary hypertension. Interestingly, the patient would desaturate when laying on his right side. The CT Chest and echocardiogram caught a portion of a hepatic cyst near the right atrium. A dedicated abdominal CT scan showed multiple complex cystic lesions within the liver, largest in the dome and right lobe of liver measuring 21.7x14.8 cm. The lesion in the dome of the liver caused mass effect on the right atrium. Differential of the cystic lesions were benign hepatic cysts, hydatid disease or biliary cyst adenomas. Cardiology attempted catheter-based closure of the PFO, but were unsuccessful due to the high-pressure gradient. Hepatobiliary surgery consult did not suggest resection given medical comorbidities; thus, percutaneous drainage was recommended. There was hesitation to drain the hepatic cysts percutaneously due to the possibility of echinococcus. The patient had a travel history outside of the United States and was recently exposed to farm animals. Echinococcus serum antibody IgG was tested and returned negative. Percutaneous drain was placed in the largest cyst by Interventional Radiology with 600 mL of brown serosanguinous fluid drained upon insertion. Patient had immediate symptomatic relief and oxygen requirements were weaned to room air within hours. There have been cases where large hepatic cysts caused compression of the right atrium as well as causing cardiac arrhythmias, which resolved with surgical cyst resection. There has been one other reported case of a hepatic cyst causing intra-cardiac shunting across a PFO with symptomatic relief through surgical cyst resection. We present a case in which a large hepatic cyst causing cardiopulmonary abnormalities was successfully treated with non-surgical and minimally invasive techniques.Figure 1Figure 2Figure 3Keywords:
Hepatopulmonary syndrome
Transesophageal echocardiogram
Shunting
We report a 38-year-old patient with hepatopulmonary syndrome related to posthepatitis C liver cirrhosis. After liver transplantation, hypoxemia initially worsened markedly, then resolved completely 14 months after transplantation. Liver transplantation may be a reliable treatment of hepatopulmonary syndrome, but clinicians should be aware that the correction of hypoxemia may be delayed and that transient deterioration is possible.
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Forty-two patients with various pulmonary abnormalities had anatomic right-to-left shunting determined during perfusion lung scintigraphy. In no instance was an abnormal shunt detected despite some severe pulmonary vascular or airway abnormalities. Twelve patients had arterial blood gas determinations performed within 24 hours of scintigraphy; several were hypoxemic despite the lack of abnormal shunting. Ventilation-perfusion mismatching or physiologic shunting was the probable cause of hypoxemia in those cases. Anatomic shunting undoubtedly occurs in some pulmonary abnormalities but should not be evoked offhandedly as a proximate cause of hypoxemia in all cases.
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Hepatopulmonary syndrome
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Hepatopulmonary syndrome
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Background. The hepatopulmonary syndrome with profound hypoxemia is a rare but severe complication for children with liver cirrhosis. It can be reversed by liver transplantation (LT), which is now regarded as a good indication. However, previous reports have described cases of transient or fatal deteriorations of intrapulmonary shunting after pediatric liver transplantation with dramatically worsening hypoxemia. Methods and Results. A similar case during and after LT in a 4-year-old girl with severe hepatopulmonary syndrome is described with prompt reversal of hypoxemia by inhaled nitric oxide, which was discontinued definitely until day 14 after LT. Conclusions. During or after LT, worsening hypoxemia may be improved by using inhaled nitric oxide in pediatric patients undergoing liver transplantation for liver cirrhosis and hepatopulmonary syndrome. The mechanisms are unclear, but may involve mismatching lung ventilation-perfusion. However, additional clinical reports are necessary before accepting these results.
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Hepatopulmonary syndrome
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Racional - A síndrome hepatopulmonar caracteriza-se pela presença de hipoxemia arterial resultante de dilatações vasculares intra-pulmonares em portadores de doença hepática crônica e/ou hipertensão portal. A síndrome, que resulta em hipoxemia arterial por vezes grave, pode alcançar completa resolução após o transplante hepático. Objetivo - Determinar a prevalência da síndrome hepatopulmonar em portadores de cirrose hepática, candidatos a transplante de fígado no Serviço de Transplante Hepático do Hospital de Clinicas da Universidade Federal do Paraná, Curitiba, PR. Pacientes e Métodos - Cinqüenta e quatro pacientes adultos (idade > ou = 18 anos) com cirrose hepática avançada foram submetidos a análise dos gases arteriais em ar ambiente na posição sentada. Os pacientes com hipoxemia arterial (PaO2 < 70 mm Hg) foram avaliados com ecocardiografia com microbolhas e estudo da função pulmonar. Resultados - Sete dos 54 pacientes (12,9%) apresentaram hipoxemia arterial em ar ambiente, embora apenas um deles referisse dispnéia aos esforços. Os sete pacientes apresentavam disfunção hepática variável de acordo com a classificação de Child (A = 1, B = 4, C = 2). Em todos os sete pacientes a ecocardiografia com microbolhas foi positiva, caracterizando a presença de síndrome hepatopulmonar. O estudo da função pulmonar não revelou anormalidades nos sete pacientes hipoxêmicos. Conclusão - A prevalência da síndrome hepatopulmonar em candidatos a transplante hepático é elevada. Essa síndrome nem sempre se acompanha de sintomas respiratórios, devendo ser rastreada rotineiramente no processo de seleção dos candidatos a transplante hepático.
Hepatopulmonary syndrome
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Hepatopulmonary syndrome
Hypoxia
Liver function
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Hepatopulmonary syndrome
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Hypoxemia is common in cirrhotic patients and, when obvious pulmonary or cardiac causes are discarded, it is attributed to the so-called hepatopulmonary syndrome. The aim of this work was to assess the frequency of hypoxemia and orthodeoxia and its relationship with the degree of liver failure, in cirrhotic alcoholic patients. We studied 30 alcoholic cirrhotics. In all, arterial blood gases were measured in supine and standing positions, in 26 a chest X ray examination was done and in 20 a spirometry. Twelve patients had a subnormal PaO2 and this parameter fell more than 105 when assuming the standing position in one of these. The same reduction was observed in two subjects with normal supine PaO2. In the chest X ray examinations, pleural effusions were observed in five hypoxemic subjects and four with normal PaO2. Likewise minimal athelectasis was found in six and seven subjects and intestinal infiltrates in one of the two subjects. A significant association between hypoxemia and Pugh score was observed. Similarly, subjects with hypoxemia is frequent in alcoholic cirrhotic patients and, since it is not associated to obvious pulmonary causes, it may be attributed to the hepatopulmonary syndrome.
Hepatopulmonary syndrome
Supine position
Arterial blood
Arterial oxygen tension
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