Afectación cardiaca en pacientes con enfermedad hepática avanzada, más allá del síndrome hepatopulmonar / Cardiac involvement in patients with end-stage liver disease, beyond the hepatopulmonary syndrome

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Síndrome hepatopulmonar y enfermedad pulmonar intersticial difusa: una asociación poco conocida / Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination

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Tromboembolismo intracardíaco durante trasplante hepático / Intracardiac tromboembolism during liver transplantation

Describimos un caso de trombosis cardíaca intraoperatoria durante una cirugía de trasplante ortotópico hepático que derivó en muerte intraoperatoria. Mediante ecocardiografía transesofágica, colocada durante la descompensación del paciente, se pudo determinar la causa del problema y observar con precisión el mecanismo de migración de trombos desde la circulación venosa hacia el corazón izquierdo

We describe a case of intraoperative cardiac trombosis during orthotopic liver transplant surgery that resulted in intraoperative death. By using transesophageal echocardiography, the cause of the descompensation of the patient could be determined and the mechanism of trombus migration from thrombi from the venous circulation to the left heart was accurately observed

Fístula hepatopulmonar: Una complicación rara del absceso hepático / Hepatopulmonary fistula: a rare complication of hepatic abscess

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Hepatopulmonary syndrome: which blood gas analysis criteria and position should we use for diagnosis?

Introduction: Different blood gas criteria have been used in the diagnosis of hepatopulmonary syndrome (HPS). Patients and methods: Arterial blood gases were prospectively evaluated in 194 cirrhotic candidates for liver transplantation (LT) in the supine and seated position. Three blood gas criteria were analyzed: classic (partial pressure of oxygen [PaO2] < 70 mmHg and/or alveolar-arterial gradient of oxygen [A-a PO2] ≥ 20 mmHg), modern (A-a PO2 ≥ 15 mmHg or ≥ 20 mmHg in patients over 64) and the A-a PO2 ≥ threshold value adjusted for age. Results: The prevalence of HPS in the supine and seated position was 27.8% and 23.2% (classic), 34% and 25.3% (modern) and 22.2% and 19% (adjusted for age), respectively. The proportion of severe and very severe cases increased in a seated position (11/49 [22.4%] vs 5/66 [7.6%], p = 0.02). No difference was observed in the pre-LT, post-LT and overall mortality in patients with HPS, regardless of the criteria used. Conclusion: Obtaining blood gas measurements in the supine position and the use of modern criteria are more sensitive for the diagnosis of HPS. Blood gas analysis with the patient seated detects a greater number of severe and very severe cases. The presence of HPS was not associated with an increase in mortality regardless of blood gas criterion used (AU)

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The utility of the macro-aggregated albumin lung perfusion scan in the diagnosis and prognosis of hepatopulmonary syndrome in cirrhotic patients candidates for liver transplantation

Background: The macro-aggregated albumin lung perfusion scan (99mTc-MAA) is a diagnostic method for hepatopulmonary syndrome (HPS). Aim: To determine the sensitivity of 99mTc-MAA in diagnosing HPS, to establish the utility of 99mTc-MAA in determining the influence of HPS on hypoxemia in patients with concomitant pulmonary disease and to determine the correlation between 99mTc-MAA values and other respiratory parameters. Methods: Data from 115 cirrhotic patients who were eligible for liver transplantation (LT) were prospectively analyzed. A transthoracic contrast echocardiography and 99mTc-MAA were performed in 85 patients, and 74 patients were diagnosed with HPS. Results: The overall sensitivity of 99mTc-MAA for the diagnosis of HPS was 18.9% (14/74) in all of the HPS cases and 66.7% (4/6) in the severe to very severe cases. In HPS patients who did not have lung disease, the degree of brain uptake of 99mTc-MAA was correlated with the alveolar-arterial oxygen gradient (A-a PO2) (r = 0.32, p < 0.05) and estimated oxygen shunt (r = 0.41, p < 0.05) and inversely correlated with partial pressure of arterial oxygen (PaO2) while breathing 100% O2 (r = -0.43, p < 0.05). The 99mTc-MAA was positive in 20.6% (7/36) of the patients with HPS and lung disease. The brain uptake of 99mTc-MAA was not associated with mortality and normalized in all cases six months after LT. Conclusions: The 99mTc-MAA is a low sensitivity test for the diagnosis of HPS that can be useful in patients who have concomitant lung disease and in severe to very severe cases of HPS. It was not related to mortality, and brain uptake normalized after LT (AU)

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Hepatopulmonary syndrome with large pulmonary arteriovenous malformations: CT findings with emphasis on its association with a mosaic pattern of the lung parenchyma / Síndrome hepatopulmonar con malformaciones arteriovenosas pulmonares grandes: hallazgos en TC con énfasis en el patrón en mosaico del parénquima pulmonar

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El síndrome platipnea-ortodeoxia: claves clínicas para su sospecha en urgencias / Platypnea-orthodeoxia syndrome in Spain

El síndrome de platipnea-ortodeoxia (SPO) se caracteriza por disnea e hipoxemia en ortostatismo. Su diagnóstico diferencial muestra causas cardiacas y extracardiacas responsables de distintos mecanismos fisiopatológicos. Se ha realizado una revisión desde enero de 2002 hasta junio de 2014 de los casos confirmados de SPO en el Hospital Universitario Miguel Servet de Zaragoza. Se encontraron un total de 4 casos: 3 de ellos secundarios a un shunt derecha-izquierda intracardiaco a través de un foramen oval permeable (FOP) y uno secundario a un shunt vascular pulmonar en el contexto de un síndrome hepatopulmonar. Se presentan dos de estos casos y se discuten las claves clínicas para su sospecha en urgencias (AU)

Platypnea orthodeoxia (PO) syndrome is characterized by dyspnea and orthostatic hypoxemia. Various cardiac and noncardiac conditions responsible for the pathophysiological mechanisms involved in the syndrome are found during differential diagnosis. We searched the records of Hospital Universitario Miguel Servet in Saragossa for confirmed cases of PO syndrome diagnosed between January 2002 and June 2014 and found 4 cases. Three were secondary to rightto-left shunt by way of a patent foramen ovale. The fourth was secondary to an intrapulmonary vascular shunt in the context of hepatopulmonary syndrome. A total of 129 titles on PO syndrome were indexed in MEDLINE between 1979 and April 2013; 77% were published in the last 10 years. PO syndrome is under diagnosed. The evident increase in incidence in recent years is probably attributable to better diagnostic techniques (AU)

Detección del shunt intrapulmonar en pacientes con cirrosis hepática mediante doppler transcraneal con contraste. Estudio de prevalencia, caracterización del patrón y validez diagnóstica / Detection of an intrapulmonary shunt in patients with liver cirrhosis through contrast-enhanced transcranial Doppler. A study of prevalence, pattern characterization, and diagnostic validity

INTRODUCCIÓN: Las dilataciones vasculares intrapulmonares (DVIPu) están consideradas una complicación de la cirrosis. La ecocardiografía con contraste (ETTc) es la técnica de elección para su diagnóstico. El objetivo de este estudio es demostrar que el doppler transcraneal con contraste (DTCc) puede servir para el diagnóstico de las DVIPu. MÉTODO: Se incluyeron consecutivamente pacientes evaluados para trasplante hepático. Estudio transversal con enmascaramiento de la prueba de referencia (ETTc) para quien interpreta la prueba a valorar (DTCc). Analizamos la exactitud de la prueba diagnóstica mediante los valores de sensibilidad, especificidad, valor predictivo positivo y negativo, y razón de verosimilitud. RESULTADOS: Por DTCc (n = 43) existía shunt derecha-izquierda en 23 pacientes (62,2%): 4 precoces, 2 indeterminados y 17 tardíos. Diecinueve (51,4%) casos fueron clasificados DVIPu. Con ETTc (n = 37) 10 estudios (27%) fueron negativos para shunt y 27 (73%) positivos, 21 (56,8%) fueron compatibles con DVIPu. Los pacientes con y sin DVIPu no diferían en edad, sexo, etiología, gravedad o índice de MELD, independientemente del método diagnóstico. En el estudio de validez diagnóstica (n = 37) del DTCc frente a la ETTc, el rendimiento diagnóstico fue AUC = 0,813% (IC 95%: 0,666-0,959; p = 0,001), sensibilidad: 76,2% (IC 95%: 54,9-89,4) y especificidad: 90% (IC 95%: 63,9-96,5). Razón de verosimilitud positiva: 6,095. CONCLUSIONES: Demostramos una alta prevalencia de DVIPu en candidatos a trasplante hepático. La probabilidad que tiene el DTCc en detectar DVIPu cuando se observa shunt derecha-izquierda tardío con recirculación es muy elevada, y con pocos falsos positivos. Al ser una técnica previamente no descrita en este contexto, deben llevarse a cabo estudios similares con fin comparativo

INTRODUCTION: Intrapulmonary vascular dilatations (IPVD) are considered a complication of cirrhosis. The technique of choice for their diagnosis is contrast-enhanced echocardiography (CEE). The aim of this study was to determine the usefulness of contrast-enhanced transcranial Doppler (CETD) in the diagnosis of IPVD. METHOD: We consecutively included patients evaluated for liver transplantation. A cross-sectional study was conducted. The investigator interpreting CETD was blind to the results of the gold standard (CEE). The accuracy of the diagnostic test was evaluated through sensitivity, specificity, positive and negative predictive values, and likelihood ratio. RESULTS: CETD (n = 43) showed a right-to-left shunt in 23 patients (62.2%): 4 early, 2 indeterminate and 17 late. Nineteen (51,4%) cases were classified as IPVD. With CEE (n = 37), 10 procedures (27%) were negative for shunt, 27 (73%) were positive, and 21 (56.8%) were compatible with IPVD. Patients with and without IPVD showed no differences in age, sex, etiology, severity, or MELD score, independently of the diagnostic test. In the diagnostic validity study (n = 37) of CETD versus CEE, the AUC for diagnostic yield was 0.813% (95% CI: 0.666-0.959; P = .001), sensitivity was 76.2% (95% CI: 54.9-89.4) and specificity was 90% (95% CI: 63.9-96.5). The positive likelihood ratio was 6.095. CONCLUSIONS: We found a high prevalence of IPVD in candidates for liver transplantation. When a late right-to-left shunt with recirculation is observed, CETD has a high probability of detecting IPVD, with few false-positive results. Because this technique has not previously been described in this indication, similar studies are needed for comparison

Platypnea-orthodeoxia syndrome: importance of patient position for correct diagnosis at the time of 99mTc-MAA injection / Síndrome platipnea-ortodeoxia: importancia de la posición del paciente en el momento de la inyección de 99mTc-MAA para un correcto diagnóstico

A 65-year-old male presented with unexplained hypoxia that became exacerbated by an upright posture (platypnea-orthodeoxia syndrome) secondary to hepatopulmonary syndrome (HPS). A 99mTc-macroaggregated albumin pulmonary perfusion scan revealed a right to left shunt of 29% in the sitting position, which had not been previously detected when the radiotracer injection was performed with the patient in supine position, nor was it diagnosed using another non-invasive imaging method (transthoracic contrast echocardiography and angio-CT). A transesophageal echocardiography was contraindicated due to the presence of esophageal varices. The administration of the radiopharmaceutical in sitting position for the study of the pulmonary perfusion allowed us to confirm the presence of the shunt and consider the patient a candidate for liver transplantation (AU)

Varón de 65 años de edad que presentó hipoxia sin explicación que se exacerbaba en sedestación (síndrome platipnea-ortodeoxia) secundaria a un síndrome hepatopulmonar (SHP). Una gammagrafía de perfusión pulmonar con macroagregados de albúmina 99mTc- reveló un cortocircuito derecha a izquierda, de 29% en la posición sentada que no se había detectado previamente cuando la inyección del radiotrazador se realizó con el paciente en posición supina, ni fue diagnosticado por otros métodos de imagen no invasivo (ecocardiografía transtorácica de contraste y la angio-TC). Una ecocardiografía transesofágica estaba contraindicada debido a la presencia de varices esofágicas. La administración del radiofármaco en sedestación nos permitió confirmar la presencia del cortocircuito y considerar al paciente candidato para trasplante hepático (AU)

Síndrome hepatopulmonar / Heptopulmonary síndrome

El síndrome hepatopulmonar está caracterizado por la existencia de enfermedad hepática, dilatación vascular pulmonar e hipoxemia arterial. Generalmente se asocia a una cirrosis hepática de cualquier origen aunque se ha descrito en otras enfermedades hepáticas, tanto agudas como crónicas, y no siempre asociada a la hipertensión portal. La ecocardiografía con contraste es el estándar de oro para el diagnóstico de las dilataciones vasculares pulmonares y fundamental por tanto para el diagnóstico del síndrome hepatopulmonar. Estas dilataciones reflejan cambios en la microvascularización pulmonar (vasodilatación, acúmulo intravascular de monocitos y angiogénesis) e inducen un desequilibrio en la relación ventilación/perfusión, o incluso verdaderos shunts, que finalmente desencadenan la hipoxemia. El síndrome hepatopulmonar empobrece el pronóstico y la calidad de vida de los pacientes y puede determinar la necesidad de un trasplante hepático que es el único tratamiento de eficacia demostrada. En el presente artículo se revisan los principales aspectos etiopatogénicos, fisiopatológicos, clínicos y terapéuticos de este síndrome

Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients’ prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome

Oxigenoterapia domiciliaria en la hipertensión pulmonar y patologias no respiratorias autores / No disponible

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Síndrome hepatopulmonar, una causa rara de hipoxemia / Hepatopulmonary syndrome -a rare cause of hypoxaemia

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Utilidad del MELD (Model for End-stage Liver Disease) para asignar prioridades en el trasplante hepático / The value of MELD in the allocation of priority for liver transplantation candidates

El trasplante hepático es el tratamiento de elección de las enfermedades hepáticas crónicas descompensadas. La disparidad en el número de órganos donantes y potenciales receptores condiciona una notable mortalidad pretrasplante que obliga a una óptima racionalización en la asignación de órganos. El MELD (Model for End-stage Liver Disease) es un índice pronóstico de mortalidad objetivo y fácilmente reproducible, basado en tres variables analíticas simples: la bilirrubina, la creatinina sérica y el cociente internacional normalizado del tiempo de protrombina (INR). La implementación del MELD como sistema de asignación de órganos ha disminuido la mortalidad en lista de espera sin afectar la supervivencia postrasplante. No obstante, el MELD tiene algunas limitaciones que obligan a seguir mejorando el sistema de asignación de prioridad en lista de espera de trasplante hepático (AU)

Liver transplantation is the most effective treatment for many patients with chronic end-stage liver disease. The discrepancy between the number of donor organs and potential recipients causes marked pre-transplantation mortality and consequently optimal rationalization of organ allocation is essential. The Model for End-Stage Liver Disease (MELD) is an objective and easily reproducible prognostic index of mortality based on three simple analytical variables: bilirubin and serum creatinine and the prothrombin time/International Normalized Ratio (INR) of protrombine time. The implementation of MELD as an organ allocation system has reduced mortality on the waiting list without affecting post-transplantation survival. Nevertheless, this model has some limitations and consequently further investigations should be performed to improve the organ allocation policy in liver transplantation (AU)

Paciente con síndrome de platipnea ortodesoxia / Patients with platypnea-orthodeoxia syndrome

Resumen. El síndrome platipnea-ortodesoxia (SPO) se caracteriza por disnea e hipoxemia en ortostatismo que remite al adoptar el decúbito supino. Las causas pueden ser cardíacas o pulmonares. Las cardíacas se deben a un circuito derecha- izquierda que se hace más evidente al incorporarse, cuando aumenta la cantidad de sangre desoxigenada que confluye a la circulación sistémica. Las causas pulmonares se deben a un circuito vascular (fístulas arteriovenosas pulmonares o síndrome hepatopulmonar), cuando en ortostatismo la gravedad aumenta el flujo en las bases pulmonares, o a un circuito parenquimatoso (enfisema pulmonar, neumonectomias, embolismos pulmonares o síndrome de distrés respiratorio del adulto), si la afectación es predominante en bases, al incrementarse el espacio muerto por hematosis ineficaz. El SPO es un fenómeno más común de lo que parece, y su diagnóstico ha aumentado, no sólo por la mejoría de las técnicas diagnósticas, sino porque se piensa en él y se incluye en los diagnósticos diferenciales (AU)

Abstract: Platypnea-orthodeoxia Syndrome (POS) consists in the presence, of dyspnea and hypoxemia in orthostatism that submit in recumbence. Its causes can be either cardiac or pulmonary. In the first case it is due to a right to left shunt which increases in orthostatism, causing more deoxygenated blood to enter the systemic circulation. If the origin is pulmonar it can happen in vascular shunt (arterious venous pulmonary fistulas and hepatopulmonary syndrome) where gravity increases the blood flow to the bases; or it can happen in parenchimal shunts (pulmonary emphysema, pneumonectomy, pulmonary embolism or adult respiratory distress syndrome) if the pulmonar bases are particularly affected increasing the dead space because of inefficient hematosis. This syndrome is more diagnosed due not only to the improvement of the techniques but also to its inclusion within the different and possible diagnosis (AU)

Mujer coinfectada por el VHC-VIH con disnea de esfuerzo de 6 meses de evolución / No disponible

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