Congenital hepatic arteriovenous malformations (HAVM) are rare and serious with a known high mortality rate. They are mostly described in the term population and, if symptomatic, present with early-onset congestive heart failure. To our knowledge, there have been no published cases of an affected very preterm infant. Prenatal diagnosis and preprocedural planning are paramount in these critically ill infants. Strategies for management of HAVM differ depending on the size of both the infant and HAVM as well as severity of symptoms. Management may include supportive pharmacologic therapy, transcatheter embolization, and surgical resection or ligation. In this report, we present the case of a 28-week gestation infant with high-output cardiac failure secondary to prenatally diagnosed hepatic arteriovenous malformation. On day of life 3, the infant underwent successful transcatheter occlusion. However, despite maximal medical management and partial embolization of his HAVM, he did not survive.
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http://dx.doi.org/10.1177/2333794X231207347 | DOI Listing |
Cureus
December 2024
Hepatology, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, JPN.
This case report discusses the case of a 74-year-old man who was diagnosed with hereditary hemorrhagic telangiectasia (HHT). The patient initially presented with right upper quadrant abdominal pain and was later diagnosed with cholangitis. Subsequently, heart failure was identified due to hepatic arteriovenous malformations.
View Article and Find Full Text PDFAm J Case Rep
December 2024
I Department of Radiology and Diagnostic Imaging, Norbert Barlicki Memorial Teaching Hospital No. 1, Medical University of Łódź, Łódź, Poland.
BACKGROUND Arterioportal fistulas (APFs) are abnormal connections between the arterial and portal venous systems, leading to portal hypertension (PH) and symptoms such as gastrointestinal bleeding, splenomegaly, and hepatic pain. Symptoms typically appear by the age of 2 years in about 75% of cases. CASE REPORT A 7-year-old boy with an asymptomatic APF developed life-threatening complications following a Clostridium difficile infection.
View Article and Find Full Text PDFGen Thorac Cardiovasc Surg Cases
December 2024
Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan.
Background: Lung transplantation is a viable lifesaving option for patients with diffuse pulmonary arteriovenous malformations (AVMs). We present a case of diffuse pulmonary AVMs associated with juvenile polyposis and hereditary hemorrhagic telangiectasia (JP-HHT) that was successfully managed by lung transplantation.
Case Presentation: A 19-year-old woman developed severe hypoxemia due to pulmonary AVMs diagnosed at 4 years of age.
J Clin Med
November 2024
Department of Digestive Surgery and Emergency Surgery, Edouard Herriot Hospital, Hospices Civils de Lyon, 69002 Lyon, France.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by abnormal blood vessel formation, leading to recurrent epistaxis, cutaneous and mucosal telangiectases, and visceral arteriovenous malformations (AVMs). Hepatic involvement may result in complications such as high-output heart failure, portal hypertension, and biliary ischemia. We report an uncommon case of ischemic cholecystitis in a patient with HHT.
View Article and Find Full Text PDFPhysiol Rep
November 2024
Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children's Wisconsin, Herma Heart Institute, Milwaukee, Wisconsin, USA.
Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease. Single ventricle PAVMs have been recognized for over 50 years but remain poorly understood. To improve our understanding, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points.
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