Unlabelled: This report presents the case of a 42-year-old man with liver cirrhosis who presents with breathlessness. Initial investigations are unable to explain his persistent hypoxia and a diagnosis of hepatopulmonary syndrome is considered. Saline contrast echocardiography is utilised in confirming the diagnosis. Details of this case as well as practicalities in performing and interpreting saline contrast echocardiography are reviewed.
Learning Points: Key features of hepatopulmonary syndrome are liver disease, hypoxia and pulmonary vascular dilatations.Saline contrast echocardiography is a simple inexpensive procedure to perform and key to confirming the diagnosis of hepatopulmonary syndrome. Detection can be improved by performing the scan in the stand-up position.Agitated saline contrast studies are more commonly performed to identify intra-cardiac shunts. Timing of contrast arrival in the left heart chambers is key to differentiating intra-cardiac shunting from extra-cardiac pulmonary transit.
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http://dx.doi.org/10.1530/ERP-14-0100 | DOI Listing |
Pediatr Nephrol
January 2025
Department of Paediatric Nephrology, The Royal Children's Hospital, Melbourne, Australia.
Hepatopulmonary syndrome (HPS) is a life-threatening complication of chronic liver disease (CLD) that currently can be managed only by liver transplant. Though uncommon, some children with kidney disease have coexistent CLD and hence are at risk of developing HPS. Paediatric cases of HPS are rarely described in the nephrology literature.
View Article and Find Full Text PDFHeliyon
February 2024
Department of Anesthesia, Southwest Hospital, The Third Military Medical University, Chongqing 400038, China.
Hepatopulmonary syndrome (HPS) is a severe lung injury caused by chronic liver disease, with limited understanding of the disease pathology. Exosomes are important mediators of intercellular communication that modulates various cellular functions by transferring a variety of intracellular components to target cells. Our recent studies have indicated that a new long noncoding RNA (lncRNA), PICALM-AU1, is mainly expressed in cholangiocytes, and is dramatically induced in the liver during HPS.
View Article and Find Full Text PDFIndian J Nucl Med
November 2024
Department of Nuclear Medicine, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
Hepatopulmonary syndrome (HPS) is a rare pulmonary vascular complication of chronic liver disease characterized by dilatation of pulmonary capillaries leading to vascular shunting and systemic hypoxemia. Diagnosis of HPS requires documentation of intrapulmonary vasodilation (IPVD), the two most common imaging studies performed for the detection of IPVD include transthoracic contrast echocardiography (TTCE) and 99m-Tc-macroaggregated albumin scintigraphy (99mTc-MAA scan). TTCE has high sensitivity and thus, is the preferred initial investigation, while 99mTc-MAA scan is highly specific and plays an adjuvant role in diagnosis.
View Article and Find Full Text PDFIntern Med
January 2025
Department of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Japan.
A 32-year-old man presented with cirrhosis. At 8 years of age, he underwent resection of a craniopharyngioma, which resulted in panhypopituitarism. He underwent self-interrupted hormone replacement therapy at 20 years of age.
View Article and Find Full Text PDFArab J Gastroenterol
January 2025
Department of Radiology, West China Hospital, Sichuan University, Chengdu, PR China. Electronic address:
Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare anatomic vascular malformation. Patients with Abernethy malformation may present with abdominal pain, abnormal liver function tests, hepatopulmonary syndrome, pulmonary hypertension, and/or portosystemic encephalopathy. Accurate identification of the shunt and portal vein and effective management of complications is vital in these patients.
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