Background: Congenital hepatic arteriovenous malformations complicated by high-output heart failure and hematologic derangements are associated with up to 90% mortality. Prenatal diagnosis alerts the pediatrician to the need for early neonatal intervention.
Case: A fetal hepatic arteriovenous malformation with associated high-output cardiac failure was diagnosed at 29 weeks' gestation using real-time and color flow Doppler sonography. Hydrocortisone injected directly into the umbilical vein and the amniotic sac resulted in appreciable improvement in hemodynamic and hematologic indices. The pregnancy ended in a preterm delivery at 31 weeks with no evidence of heart failure at birth.
Conclusion: The compromised preterm fetus with a hepatic arteriovenous malformation can be treated in utero, avoiding early emergency delivery.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/0029-7844(94)00227-5 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Cholangitis and Congestive Heart Failure Secondary to Biliary Hemorrhage in Hereditary Hemorrhagic Telangiectasia.
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 PDFLife-Threatening Esophageal Variceal Hemorrhage in a 7-Year-Old Boy with Massive Portal Vein Enlargement Due to Congenital Arterioportal Fistula.
Am 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 PDFLung transplantation for diffuse pulmonary arteriovenous malformations associated with juvenile polyposis-hereditary hemorrhagic telangiectasia overlap syndrome: a case report.
Gen 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.
Spontaneous Ischemic Cholecystitis in a Patient with Hereditary Hemorrhagic Telangiectasia (HHT).
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 PDFGlenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations.
Physiol 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.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!