Portal Hypertension
Basics
Description
- Definition of portal hypertension (P-HTN): elevation of portal pressure (PP) >5 mm Hg
- Risk of developing complications occurs with “clinically significant P-HTN”: PP >10 mm Hg
- Significant risk of bleeding from esophageal varices (EV) with PP >12 mm Hg
- May be pre-, intra-, or posthepatic in origin
- A major cause of morbidity and mortality in children with chronic liver disease
Pathophysiology
- P-HTN is secondary to both increases in portal resistance and portal blood flow:
- Other contributing factors include hyperdynamic circulation, expanded intravascular volume, systemic arteriolar vasodilatation, decreased splanchnic arteriolar tone, and humoral factors (i.e., nitric oxide).
- Portosystemic collaterals develop to decompress the high PPs, leading to a number of sequelae, all with high morbidity and mortality risks, including:
- Variceal hemorrhage
- Splenomegaly/hypersplenism
- Ascites
- Spontaneous bacterial peritonitis
- Hepatorenal syndrome
- Hepatopulmonary syndrome
- Portopulmonary hypertension
- Hepatic encephalopathy
Etiology
- Prehepatic (presinusoidal) causes:
- Portal vein thrombosis (PVT) with cavernous transformation (increased risk with umbilical vein catheterization, sepsis, dehydration, hypercoagulable state)
- Splenic vein thrombosis
- Intrahepatic (sinusoidal) causes:
- Hepatocellular disorders: cirrhosis (secondary to chronic viral hepatitis [e.g., Hep B, Hep C, etc.], α1-antitrypsin deficiency, autoimmune hepatitis, Wilson disease, glycogen storage disease, tyrosinemia, etc.), schistosomiasis, peliosis hepatitis, vitamin A toxicity
- Biliary tract disorders: cirrhosis (secondary to biliary atresia, intrahepatic cholestasis syndromes, primary sclerosing cholangitis, cystic fibrosis, etc.), ductal plate malformation/congenital hepatic fibrosis, choledochal cyst
- Posthepatic (postsinusoidal) causes:
- Budd-Chiari syndrome: occlusion of suprahepatic inferior vena cava or hepatic veins by congenital web, tumor, or thrombus
- Congestive hepatopathy (cardiac: right-sided heart failure or hypertension, such as post-Fontan procedure; pulmonary hypertension)
- Sinusoidal obstruction syndrome (veno-occlusive disease of hepatic sinusoids and terminal venules)
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Citation
Cabana, Michael D., editor. "Portal Hypertension." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617257/all/Portal_Hypertension.
Portal Hypertension. In: Cabana MDM, ed. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617257/all/Portal_Hypertension. Accessed October 15, 2024.
Portal Hypertension. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult (8th ed.). Wolters Kluwer. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617257/all/Portal_Hypertension
Portal Hypertension [Internet]. In: Cabana MDM, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2024 October 15]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617257/all/Portal_Hypertension.
* Article titles in AMA citation format should be in sentence-case
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