Hepatopulmonary syndrome has low prevalence of pulmonary vascular abnormalities on chest computed tomography.

PURPOSE: Hepatopulmonary syndrome (HPS) is defined as an arterial oxygenation defect induced by intrapulmonary vascular dilatations associated with hepatic disease. This study aimed to assess the prevalence of type 1 and 2 pulmonary vascular abnormalities on chest computed tomography (CT) in patients with cirrhosis and HPS and to characterize intra- and interobserver reliability. MATERIALS AND METHODS: Two thoracic radiologists retrospectively evaluated chest CT scans from 38 cirrhosis patients with HPS. They classified the pulmonary vascular abnormalities as type 1 (multiple dilated distal pulmonary arteries), type 2(nodular dilatation or individual pulmonary arterial malformation), or absence of abnormality. Furthermore, they measured the diameters of the central pulmonary arteries and subsegmental pulmonary arteries and bronchi. We analyzed the prevalence, intraobserver reliability, and interobserver reliability of abnormal CT findings related to HPS, and the correlation of these findings with partial arterial oxygen pressure (PaO2). RESULTS: The overall prevalence of pulmonary vascular abnormalities was 28.9% (95% confidence intervals: 15.4%, 45.9%). Moreover, 26.3% of patients had type 1 abnormality (13.4%, 43.1%) and 2.6% of patients had type 2 abnormality (0.0%, 13.8%). The intraobserver reliability kappa value was 0.666 (0.40, 0.91) and the interobserver kappa value was 0.443 (0.12, 0.77). There was no correlation between pulmonary vascular abnormalities on CT and PaO2 values. CONCLUSIONS: The prevalence of pulmonary vascular abnormalities on chest CT of patients with cirrhosis and HPS is low and not correlated with PaO2. These findings question the usefulness of chest CT for the evaluation of patients with cirrhosis and HPS.

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

BACKGROUND Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS). OBJECTIVES To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis. METHODS We evaluated patients with HSS with or without concomitant liver cirrhosis. All patients underwent laboratory testing, ultrasound, endoscopy, contrast echocardiography, and arterial blood gas analysis. FINDINGS Of the 121 patients with HSS, 64 were also diagnosed with liver cirrhosis. HPS was diagnosed in 42 patients (35%) and was more frequent among patients with concomitant liver cirrhosis than in those without cirrhosis (42% vs. 26%), but the difference was not significant (p = 0.069). HPS was more common in those with spider naevi, Child-Pugh classes B or C and high model for end stage liver disease (MELD) scores (p < 0.05 each). MAIN CONCLUSIONS The prevalence of HPS was 35% in this study. The occurrence of liver cirrhosis concomitantly with HSS may have influenced the frequency of patients presenting with HPS.

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis.

BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS). OBJECTIVES: To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis. METHODS: We evaluated patients with HSS with or without concomitant liver cirrhosis. All patients underwent laboratory testing, ultrasound, endoscopy, contrast echocardiography, and arterial blood gas analysis. FINDINGS: Of the 121 patients with HSS, 64 were also diagnosed with liver cirrhosis. HPS was diagnosed in 42 patients (35%) and was more frequent among patients with concomitant liver cirrhosis than in those without cirrhosis (42% vs. 26%), but the difference was not significant (p = 0.069). HPS was more common in those with spider naevi, Child-Pugh classes B or C and high model for end stage liver disease (MELD) scores (p < 0.05 each). MAIN CONCLUSIONS: The prevalence of HPS was 35% in this study. The occurrence of liver cirrhosis concomitantly with HSS may have influenced the frequency of patients presenting with HPS.

Exercise capacity of cirrhotic patients with hepatopulmonary syndrome.

INTRODUCTION: Hepatopulmonary syndrome (HPS) is characterized by a clinical triad of liver disease and/or portal hypertension, intrapulmonary vascular dilatation and abnormal arterial oxygenation. These conditions can worsen muscle strength, exercise capacity and functionality in the affected population. OBJECTIVE: The objective of this study was to compare exercise capacity, functional condition and respiratory muscle strength in cirrhotic patients diagnosed with HPS and cirrhotic patients without this diagnosis. MATERIAL AND METHODS: This cross-sectional study used a convenience sample consisting of 178 patients (92 patients with HPS and 86 patients without HPS) with a diagnosis of liver cirrhosis caused by either alcohol consumption or the hepatitis C virus (HCV). Peak oxygen consumption (VO2 peak) was used to verify exercise capacity, the six-minute walk test (6MWT) was used to test functionality, and manovacuometry was used to evaluate the strength of the respiratory muscles. The Kolmogorov-Smirnov test and Student's t-test were used for the statistical analysis. The data were analyzed using SPSS 16.00, and p < 0.05 was considered significant. RESULTS: The group of patients with the diagnosis of HPS exhibited a lower VO2 peak (14.2 ± 2.3 vs. 17.6 ± 2.6, p < 0.001), shorter distance walked in the 6MWT (340.8 ± 50.9 vs. 416.5 ± 91.4, p < 0.001), lower maximal inspiratory pressure (-49.1 ± 9.8 vs. -74.2 ± 13.9, p = 0.001) and lower maximum expiratory pressure (60.1 ± 12.2 vs. 76.8 ± 14.7, p = 0.001). CONCLUSION: The group of cirrhotic patients diagnosed with HPS exhibited lower values for VO2 peak, distance walked in the 6MWT and respiratory muscle strength than the cirrhotic patients not diagnosed with HPS.

Hypoxia among patients on the liver-transplant waiting list.

BACKGROUND: Hepatopulmonary syndrome is formed by a triad of liver disease, intrapulmonary vascular dilatation and changes in blood gases. This condition is present in 4-32% of patients with cirrhosis. AIM: To analyze the blood gas changes data of patients in liver-transplant waiting list. METHOD: Clinical data of 279 patients in liver transplantation waiting list in May 2013 were studied. Overall patient was analyzed by the demographic aspects, laboratorial and image findings on exams that determine lung disease (hypoxemia) in these cirrhotic patients. The mean values and standard deviations were used to examine normally distributed variables. RESULTS: There was a high prevalence of male patients (68%); the mean age was 51(± 5,89) years, and the predominant reason for listing was hepatitis C cirrhosis. The MELD score mean was 16 ± 5,89, without prioritization or special situation. The most common blood type was O in 129 cases (46%) and the mean of body max index was 25,94 ± 4,58. Regarding arterial blood gas tests was observed 214 patients with PaO2 <90 mmHg, 80 with PaO2 <80 mmHg and 39 with PaO2 <50 mmHg. In relation to O2 saturation, 50 patients had <90%, 33 <80% and 10 <50%. CONCLUSION: Was observed a high rate of hypoxemia in patients on waiting list liver transplant. Due to the high severity and morbidity, is suggested better monitoring and therapeutic support to hypoxemic patients on liver transplant waiting list.

Hypoxia among patients on the liver-transplant waiting list / Hipoxia entre os pacientes na lista de espera para transplante de figado

Background: Hepatopulmonary syndrome is formed by a triad of liver disease, intrapulmonary vascular dilatation and changes in blood gases. This condition is present in 4-32% of patients with cirrhosis. Aim : To analyze the blood gas changes data of patients in liver-transplant waiting list. Method: Clinical data of 279 patients in liver transplantation waiting list in May 2013 were studied. Overall patient was analyzed by the demographic aspects, laboratorial and image findings on exams that determine lung disease (hypoxemia) in these cirrhotic patients. The mean values and standard deviations were used to examine normally distributed variables. Results: There was a high prevalence of male patients (68%); the mean age was 51(±5,89) years, and the predominant reason for listing was hepatitis C cirrhosis. The MELD score mean was 16±5,89, without prioritization or special situation. The most common blood type was O in 129 cases (46%) and the mean of body max index was 25,94±4,58. Regarding arterial blood gas tests was observed 214 patients with PaO2 <90 mmHg, 80 with PaO2 <80 mmHg and 39 with PaO2 <50 mmHg. In relation to O2 saturation, 50 patients had <90%, 33 <80% and 10 <50%. Conclusion: Was observed a high rate of hypoxemia in patients on waiting list liver transplant. Due to the high severity and morbidity, is suggested better monitoring and therapeutic support to hypoxemic patients on liver transplant waiting list. .

Racional: A síndrome hepatopulmonar é formada por tríade clínica com doença do fígado, dilatação vascular intrapulmonar e alterações nos gases sanguíneos. Esta condição está presente em 4-32% dos pacientes com cirrose. Objetivo : Analisar as alterações gasométricas nos pacientes em lista de espera de transplante de fígado. Método: Foram estudados dados clínicos de 279 pacientes na lista de espera para transplante hepático em maio de 2013. Foram analisados aspectos demográficos, gasometria arterial e achados de imagem que determinam a doença pulmonar (hipoxemia) nestes pacientes cirróticos. Os valores médios e desvios-padrão foram utilizados para examinar as variáveis ​​normalmente distribuídas. Resultados: Houve alta prevalência de homens (68%); a idade média foi de 51 (±5,89) anos; e a razão predominante para listar para o transplante foi cirrose pelo vírus C. O MELD médio foi de 16±5,89, sem priorização ou situação especial. O tipo de sangue mais comum foi O, 129 casos (46%) e a média do índice de massa corporal foi 25,94±4,58. Com relação aos exames de gasometria arterial, observou-se 214 pacientes com PaO2 <90 mmHg, 80 com PaO2 <80 mmHg e 39 com PaO2 <50 mmHg, e em relação à saturação de O2, 50 pacientes <90%, 33 pacientes <80% e 10 pacientes <50%. Conclusão: Observou-se alta taxa de hipoxemia nos pacientes em lista de transplante de fígado; devido à elevada gravidade e morbidade, sugere-se melhor seguimento e suporte terapêutico aos doentes hipoxêmicos na lista de espera para o transplante de fígado. .

Are the spider angiomas skin markers of hepatopulmonary syndrome?

CONTEXT: Hepatopathies can significantly influence both veins and arteries, these changes may cause some cutaneous stigmas, such as spider angioma (SA) and some systemic vascular changes, such as those observed in hepatopulmonary syndrome (HPS). Based on this common pathophysiological root we can assume that the SA can be skin markers of HPS. OBJECTIVE: The objective of this study is to assess whether there is a relationship between the presence of SA and HPS. METHODS: Records of 40 patients with liver cirrhosis who underwent contrast echocardiography were evaluated, in which we researched the description of SA, physical examination, and other clinical and laboratory data. For diagnosis of HPS we use these signs of the disease: presence of liver disease (cirrhosis in the case), abnormalities in gas exchange by arterial blood gases, and evidence of pulmonary vasodilations by the contrast echocardiography. RESULTS: The SA were found in 21/40 (52.5%) patients and hepatopulmonary syndrome in 9/40 (22.5%). The HPS was observed in 8/21 (38.1%) of patients with SA and 1/19 (5.3%) patients were without this sign (P<0.01). We found no statistically significant difference between the SA and the presence of HPS with sex or age. Patients with SA had a higher hypoxemia [PaO2 84.8 ± 11.5 mmHg and 19.8 ± 14.7 mmHg alveolar-arterial gradient of oxygen (AAG)] than those without SA (PaO2 90.8 ± 10.7 mmHg and 10.9 ± 11.7 AAG mmHg) (P<0.05). CONCLUSION: Our findings show a correlation between the presence of SA and HPS, suggesting that the SA may be cutaneous markers of HPS.

Are the spider nngiomas skin markers of hepatopulmonary syndrome? / Sao as aranhas vasculares marcadores cutaneos da sindrome hepatopulmonar?

Context Hepatopathies can significantly influence both veins and arteries, these changes may cause some cutaneous stigmas, such as spider angioma (SA) and some systemic vascular changes, such as those observed in hepatopulmonary syndrome (HPS). Based on this common pathophysiological root we can assume that the SA can be skin markers of HPS. Objective The objective of this study is to assess whether there is a relationship between the presence of SA and HPS. Methods Records of 40 patients with liver cirrhosis who underwent contrast echocardiography were evaluated, in which we researched the description of SA, physical examination, and other clinical and laboratory data. For diagnosis of HPS we use these signs of the disease: presence of liver disease (cirrhosis in the case), abnormalities in gas exchange by arterial blood gases, and evidence of pulmonary vasodilations by the contrast echocardiography. Results The SA were found in 21/40 (52.5%) patients and hepatopulmonary syndrome in 9/40 (22.5%). The HPS was observed in 8/21 (38.1%) of patients with SA and 1/19 (5.3%) patients were without this sign (P<0.01). We found no statistically significant difference between the SA and the presence of HPS with sex or age. Patients with SA had a higher hypoxemia [PaO2 84.8 ± 11.5 mmHg and 19.8 ± 14.7 mmHg alveolar-arterial gradient of oxygen (AAG)] than those without SA (PaO2 90.8 ± 10.7 mmHg and 10.9 ± 11.7 AAG mmHg) (P<0.05). Conclusion Our findings show a correlation between the presence of SA and HPS, suggesting that the SA may be cutaneous markers of HPS. .

Contexto As hepatopatias podem influenciar de forma considerável tanto as veias quanto as artérias, dessas alterações podem surgir alguns estigmas cutâneos, como as aranhas vasculares (AV) e algumas alterações vasculares sistêmicas, como as observadas na Síndrome Hepatopulmonar (SHP). Baseados nessa possível raiz fisiopatogênica comum, podemos supor que as AV sejam marcadores cutâneos da SHP. Objetivo Avaliar se há relação entre a presença das AV e a SHP. Métodos Foram avaliados os prontuários de 40 pacientes com cirrose hepática submetidos a ecocardiografia com contraste (ECC), nos quais pesquisamos a descrição de AV, no exame físico, e outros dados clínicos e laboratoriais. Para diagnóstico da SHP utilizamos os seguintes critério: presença de hepatopatia (no caso cirrose), de anormalidades nas trocas gasosas pela gasometria arterial, e evidências de vasodilatações pulmonares pela ecocardiografia com contraste. Resultados As AV foram encontradas em 21/40 (52,5%) pacientes e a síndrome hepatopulmonar em 9/40 (22,5%). A SHP foi observada em 8/21 (38,1%) dos pacientes com AV e em 1/19 (5,3%) dos pacientes sem esse sinal (P<0,01). Não encontramos diferença estatisticamente significativa entre a presença das AV e da SHP com sexo ou faixa etária. Pacientes com AV apresentaram maior hipoxemia (PaO2 84,8 ± 11,5 mmHg e GAA 19,8 ± 14,7 mmHg) que os sem AV (PaO2 90,8 ± 10,7 mmHg e GAA 10,9 ± 11,7 mmHg) (P<0,05). Conclusão Nossos achados mostram correlação entre a presença das AV e a SHP, sugerindo que as AV possam ser marcadores cutâneos da SHP. .

Transplante hepático para síndromes hepatopulmonar e de obstrução sinusoidal / Liver transplantation for hepatopulmonary and sinusoidal obstruction syndrome

INTRODUÇÃO: A síndrome hepatopulmonar é complicação das doenças hepáticas que afeta a vascularização pulmonar, causando comprometimento do sistema respiratório e acompanha-se de elevada morbidade. O transplante hepático é o tratamento ideal para esses casos. O objetivo desse relato é descrever o caso de um paciente que apresentava síndrome hepatopulmonar avançada, devida à síndrome da obstrução sinusoidal.RELATO DO CASO: Homem de 23 anos foi encaminhado com o diagnóstico de síndrome hepatopulmonar avançada e cirrose hepática sem etiologia idefinida. Exames de imagem foram compatíveis com shunt intrapulmonar, porém sem alterações da vascularização pulmonar factíveis de serem embolizadas. A endoscopia digestiva alta demonstrou varizes esofágicas de pequeno calibre. Exames laboratoriais para função hepática estavam alterados. O paciente foi submetido a transplante hepático falecendo 14 dias após o procedimento devido a complicações relacionadas à sepse e insuficiência ventilatória refratária.CONCLUSÃO: Na vigência de síndrome hepatopulmonar o único tratamento efetivo é o transplante hepático, para os casos em que não houver área de shunt factível de ser realizada a embolização seletiva.

INTRODUCTION: The hepatopulmonary syndrome is a complication of liver diseases that affects the pulmonary vascular system compromising respiratory function. High morbidity rates are associated with this syndrome. Liver transplantation is the treatment of choice. The aim of this report is to present the case of a patient who sustained advanced hepatopulmonary syndrome resulting from the sinusoidal obstruction syndrome.CASE REPORT: A 23-year-old male was referred with the diagnosis of advanced hepatopulmonary syndrome and liver cirrhosis of undefined etiology. Imaging studies were consistent with intrapulmonary shunt yet without alterations in pulmonary vascularization amenable to embolization. Upper digestive endoscopy demonstrated small-caliber esophagian varices. Laboratory tests for liver function showed alterations. The patient underwent liver transplantation and died 14 days after, due to sepsis-related complications and refractory ventilatory failure.CONCLUSION: In cases when it is not feasible to perform selective embolization in the shunt area, the only effective treatment for hepatopulmonary syndrome is liver transplantation.

[Experimental models for assessment of pulmonary alterations in hepatopulmonary syndrome]. / Modelos experimentais para avaliação das alterações pulmonares na síndrome hepatopulmonar.

OBJECTIVE: The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). METHODS: Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. RESULTS: There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 +/- 43 vs. 500.5 +/- 90.3 IU/L); alanine aminotransferase (78.75 +/- 37.7 vs. 162.75 +/- 35.4 IU/L); alkaline phosphatase (160 +/- 20.45 vs. 373.25 +/- 45.44 IU/L); arterial oxygen tension (85.25 +/- 8.1 vs. 49.9 +/- 22.5 mmHg); and oxygen saturation (95 +/- 0.7 vs. 73.3 +/- 12.07%). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 +/- 0.3 vs. 2.01 +/- 0.9 nmol/mg protein); chemiluminescence (16008.41 +/- 1171.45 vs. 20250.36 +/- 827.82 cps/mg protein); and SOD (6.66 +/- 1.34 vs. 16.06 +/- 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. CONCLUSIONS: The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.

Modelos experimentais para avaliação das alterações pulmonares na síndrome hepatopulmonar / Experimental models for assessment of pulmonary alterations in hepatopulmonary syndrome

OBJETIVO: O objetivo deste trabalho foi avaliar o melhor modelo experimental para observar alterações pulmonares que caracterizam a síndrome hepatopulmonar (SHP). MÉTODOS: Ratos machos Wistar, com peso médio de 250 g foram usados em quatro modelos experimentais: tetracloreto de carbono inalatório; tetracloreto de carbono intraperitoneal; ligadura parcial de veia porta; e ligadura de ducto biliar (LDB). Em todos os grupos os animais foram divididos em controle e experimental. Foram avaliadas as seguintes variáveis: transaminases; gasometria; lipoperoxidação por substâncias que reagem ao ácido tiobarbitúrico (TBARS) e por quimiluminescência; e atividade antioxidante da enzima superóxido dismutase (SOD). Foi feito também o exame anatomopatológico do pulmão. RESULTADOS: Observou-se diferenças significativas entre os grupos LDB controle e experimental: aspartato amino transferase (105,3 ± 43 vs. 500,5 ± 90,3 UI/L); alanino aminotransferase (78,75 ± 37,7 vs. 162,75 ± 35,4 UI/L); fosfatase alcalina (160 ± 20,45 vs. 373,25 ± 45,44 UI/L); pressão parcial de oxigênio (85,25 ± 8,1 vs. 49,9 ± 22,5 mmHg); e saturação de hemoglobina (95 ± 0,7 vs. 73,3 ± 12,07 por cento). A lipoperoxidação e a atividade antioxidante também demonstrou diferenças entre os dois grupos LDB (controle vs. experimental): TBARS (0,87 ± 0,3 vs. 2,01 ± 0,9 nmol/mg proteína); quimiluminescência (16008,41 ± 1171,45 vs. 20250,36 ± 827,82 cps/mg proteína); e SOD (6,66 ± 1,34 vs. 16,06 ± 2,67 UI/mg proteína). No exame anatomopatológico observou-se vasodilatação pulmonar no modelo de LDB. CONCLUSÕES: Os dados sugerem que o modelo de LDB pode ser usado para outros estudos envolvendo alterações hepáticas e suas relações com o estresse oxidativo e a SHP.

OBJECTIVE: The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). METHODS: Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. RESULTS: There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 ± 43 vs. 500.5 ± 90.3 IU/L); alanine aminotransferase (78.75 ± 37.7 vs. 162.75 ± 35.4 IU/L); alkaline phosphatase (160 ± 20.45 vs. 373.25 ± 45.44 IU/L); arterial oxygen tension (85.25 ± 8.1 vs. 49.9 ± 22.5 mmHg); and oxygen saturation (95 ± 0.7 vs. 73.3 ± 12.07 percent). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 ± 0.3 vs. 2.01 ± 0.9 nmol/mg protein); chemiluminescence (16008.41 ± 1171.45 vs. 20250.36 ± 827.82 cps/mg protein); and SOD (6.66 ± 1.34 vs. 16.06 ± 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. CONCLUSIONS: The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.

Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies.

The association of nodular regenerative hyperplasia with celiac disease is not as well established as it is with hepatopulmonary syndrome and portopulmonary hypertension. IgA anticardiolipin antibodies were reported recently in celiac patients with nodular regenerative hyperplasia. The subject of this study was the description of pulmonary abnormalities and IgA anticardiolipin antibodies in celiac patients with noncirrhotic portal hypertension. Five patients with portal hypertension were investigated to diagnose its etiology. Celiac disease was diagnosed by means of autoantibody reactivity and duodenal biopsies. Liver histology revealed nodular regenerative hyperplasia in four patients and suggested its presence in 1 case. Two cyanotic patients had severe hypoxemia with a confirmed diagnosis of hepatopulmonary syndrome. Another case exhibited features of hepatopulmonary syndrome with increased levels of arterial pulmonary pressure. The remaining 2 cases had slight abnormalities of arterial oxygenation. Three patients had reactivity to IgA anticardiolipin antibodies. The concomitance of celiac disease and nodular regenerative hyperplasia, two infrequent conditions, raises suspicion of there being a nonfortuitous coincidence. Pulmonary abnormalities, and especially hepatopulmonary syndrome, are described for the first time in association with celiac disease and nodular regenerative hyperplasia.

[Platypnea-orthodeoxia syndrome]. / Sindrome platipnea-ortodeoxia.

Platypnea-orthodeoxia is an uncommon syndrome of dyspnea and hypoxemia induced by upright posture, which is subsequently relieved by recumbency. Traditionally, this condition has been reported in association with pulmonary, hepatic and cardiac diseases, but the mechanism is different in each situation. In presence of an atrial septal defect, a right to left cardiac shunt resulting as a consequence of redirection of the inferior vena cava flow towards the atrial septum and results in postural hypoxemia. In pulmonary shunts, as in hepatopulmonary syndrome and a-v pulmonary fistulas, the mechanism of hypoxemia is related to the preferential circulation to basal areas of both lungs in the upright position. On the other hand, lung diseases affecting basal areas and increasing the alveolar pressure are related to the development of parenchimal pulmonary shunts with hypoxemia during postural changes, since the dead space, the functional shunt and the hypoxic vascular pulmonary constriction are affected. The autonomic neuropathy may attenuate normal sympathetic vasoconstrictor responses during postural changes, resulting in ventilation-perfusion mismatching and hypoxemia. Clinical and therapeutic aspects of this sindrome are provided.

Sindrome platipnea-ortodeoxia / Platypnea-orthodeoxia syndrome

El síndrome platipnea-ortodeoxia (SPO) está caracterizado por disnea e hipoxemia en posición erecta que mejoran en el decúbito supino. Para que se genere concurren dos condiciones, una basal (anatómica) y otra funcional que manifiesta la hipoxemia en posición erecta. El SPO es causado por alteraciones a nivel cardíaco y/o pulmonar y los mecanismos responsables son diferentes. En el shunt derecha-izquierda intracardíaco a través de una comunicación interauricular, el mecanismo de la hipoxemia postural es el redireccionamiento del flujo de la vena cava inferior hacia el septum interauricular por distorsión de las relaciones anatómicas. En el shunt vascular pulmonar, referido en el síndrome hepatopulmonar y en las fístulas a-v pulmonares, la hipoxemia es por derivación del flujo sanguíneo en forma preferencial a las regiones basales por el efecto gravitacional. El shunt pulmonar parenquimatoso es referido en enfermedades con compromiso predominante en las bases y aumento de la presión alveolar. La hipoxemia postural es generada por el aumento del espacio muerto, la exageración del shunt funcional en las bases pulmonares y la alteración del reflejo vasoconstrictor hipóxico. En la disautonomía, el reflejo vasoconstrictor con el cambio de postura está anulado, generando hipoxemia por incremento del espacio muerto en áreas inferiores a la apical. Se hacen consideraciones sobre el diagnóstico y tratamiento del síndrome.

Sindrome platipnea-ortodeoxia / Platypnea-orthodeoxia syndrome

El síndrome platipnea-ortodeoxia (SPO) está caracterizado por disnea e hipoxemia en posición erecta que mejoran en el decúbito supino. Para que se genere concurren dos condiciones, una basal (anatómica) y otra funcional que manifiesta la hipoxemia en posición erecta. El SPO es causado por alteraciones a nivel cardíaco y/o pulmonar y los mecanismos responsables son diferentes. En el shunt derecha-izquierda intracardíaco a través de una comunicación interauricular, el mecanismo de la hipoxemia postural es el redireccionamiento del flujo de la vena cava inferior hacia el septum interauricular por distorsión de las relaciones anatómicas. En el shunt vascular pulmonar, referido en el síndrome hepatopulmonar y en las fístulas a-v pulmonares, la hipoxemia es por derivación del flujo sanguíneo en forma preferencial a las regiones basales por el efecto gravitacional. El shunt pulmonar parenquimatoso es referido en enfermedades con compromiso predominante en las bases y aumento de la presión alveolar. La hipoxemia postural es generada por el aumento del espacio muerto, la exageración del shunt funcional en las bases pulmonares y la alteración del reflejo vasoconstrictor hipóxico. En la disautonomía, el reflejo vasoconstrictor con el cambio de postura está anulado, generando hipoxemia por incremento del espacio muerto en áreas inferiores a la apical. Se hacen consideraciones sobre el diagnóstico y tratamiento del síndrome. (AU)