Left ventricular heart failure and pulmonary hypertension. Diuretics potassium sparing diuretics is considered the diuretic of choice in portal hypertension. Sinistral leftsided extrahepatic portal hypertension. Portal hypertension is defined as a hepatic venous pressure gradient. According to the most recent consensus conference, pulmonary hypertension ph is. The hypertension is confined to the left side of the portal system and is therefore distinct from the common phenomenon of generalised portal hypertension. Pulmonary hypertension ph with leftsided heart disease is defined, according to the latest venice classification, as a group 2 ph, which includes leftsided ventricular or atrial disease, and. Gastric variceal bleeding from segmentary idiopathic.
Left sided portal hypertension lsph, also known as segmental, regional, localized, compartmental, lineal, splenoportal, or sinistral hypertension is a rare, but life threatening cause of upper gastrointestinal bleeding. Left ventricular dysfunction with pulmonary hypertension. Although the differential diagnosis is extensive, alcoholic and viral. Idiopathic noncirrhotic portal hypertension incph is a diagnosis of exclusion with increased portal venous pressure without cirrhosis, hepatoportal flow obstruction, splanchnic venous thrombosis, and other causes of liver disease. A case of idiopathic noncirrhotic portal hypertension in. Post traumatic intra thoracic spleen presenting with upper.
Leftsided portal hypertension lsph is a localized form of portal hypertension that usually occurs as a result of iso lated obstruction of the splenic vein 1, 2. Leftsided portal hypertension lsph, also known as segmental, regional, localized, compartmental, lineal, splenoportal, or sinistral hypertension is a rare, but. Presentation mode open print download current view. Leftsided portal hypertension should be considered in the presence of.
Thrombophilia, myeloproliferative disorders, gastric, renal pathologies, retroperitoneal fibrosis, wandering spleen, lymphomas or sarcomas and iatrogenic. Endoscopic color doppler ultrasonography ecdus was performed in 16 patients with gastric varices secondary to splenic vein occlusion. In the clinical classification, ph associated with left heart disease is classified as group 2, which includes left heart systolic dysfunction, left heart diastolic dysfunction and left heart valvular disease. Leftsided portal hypertension complicated by gastrointestinal hemorrhage represents an indication for splenectomy. Pdf leftsided portal hypertension is a rare clinical syndrome which may lead to bleeding from isolated gastric varices. Diseases of the pancreas have been identified as the most common cause of isolated splenic vein thrombosis.
Pulmonary hypertension in left heart disease european. Hypertrophy of the heart is an adaptive response to. Clinically significant portal hypertension is present when gradient exceeds 10 mmhg. The aim of this study was to analyse the pathogenesis of sinistral portal hypertension. Cirrhosis a form of chronic liver failure is the most common cause of portal hypertension. Mucinous cystic neoplasms mcn are an uncommon form of exocrine neoplasms of the pancreas. The most important clinical consequences of portal hypertension are related to the formation of portalsystemic collaterals.
Multiple giant splenic artery aneurysms causing sinistral. While the validity of these surrogate markers of portal hypertension has been documented in patients with chemotherapyinduced hepatic sinusoidal injury and generalized portal hypertension, it is not clear whether they are equally applicable or reliable. Splenic vein occlusion results in leftsided portal hypertension characterized by gastric varices, splenomegaly and normal liver function 9,10,11 that is secondary to various diseases 12,14. Identification of pulmonary hypertension caused by left.
It is rare for them to cause sinistral portal hypertension. The introduction of new pharmacological agents, endoscopic variceal band ligation, and transjugular intrahepatic portosystemic stentshunt tipss has increased the therapeutic options available for this disorder. The portal vein carries about 1500 mlmin of blood from the small and large bowel, spleen, and stomach to the liver at a pressure of 510 mm hg. Ph is a major and frequent consequence of leftsided hf, irrespective of left ventricular ejection fraction lvef or presence of valvular disease. Pulmonary hypertension with leftsided heart disease. Sinistral portal hypertension sph is also known as splenoportal, left sided, segmental, regional, localized, compartmental or lineal portal hypertension. Splenomegaly and left sided portal hypertension article pdf available in annals of gastroenterology 183. Sinistral, or leftsided, portal hypertension sph is a rare entity, with multiple potential causes. Segmentary idiopathic splenic vein stenosis is a very rare condition. Hyperplastic malignant hypertension 22 left ventricular cardiac hypertrophy left sided hypertensive cardiomyopathy hypertensive heart disease longstanding poorly treated htn leads to left sided hypertensive heart disease. The aim of this study was to analyse the pathogenesis of sinistral portal hypertension following splenic vein ligation in pancreaticoduodenectomy.
Isolated gastric varices occur in left sided and generalised portal hypertension, 23 however, the more common phenomenon of generalised portal hypertension should initially be excluded. A 23yearold female patient presented to our clinic with gastric varices, splenomegaly, pancytopenia, and normal liver functions. Updated classification and management of pulmonary. Pulmonary hypertension ph is a frequent condition, which may occur as a consequence of pulmonary vascular disease, chronic left heart or lung disease, pulmonary embolism, or other aetiologies. Any obstruction or increased resistance to flow or, rarely, pathological increases in portal blood flow may lead to portal hypertension with portal pressures over 12 mm hg. Evaluations of patients with pulmonary hypertension ph commonly include chest ct imaging. The current case is one of three cases yet reported where the mcn caused left sided portal hypertension leading to the formation of isolated gastric varices and subsequent bleeding from the varices. The portal vein carries blood from your stomach, pancreas, and other digestive organs to your liver. Falciform ligament abscess from left sided portal pyaemia. In fact, left heart diseases lhd account for 6580% of ph. Portal hypertension is increased blood pressure in the blood vessels that lead to the liver. This study retrospectively evaluated the role of ecdus in the diagnosis of gastric varices secondary to splenic vein occlusion. Portal hypertension may be caused by intrinsic liver disease, obstruction, or structural changes that result in increased portal venous flow or increased hepatic resistance. Hypertrophy of the heart is an adaptive response to pressure overload due to htn.
Leftsided portal hypertension is a rare clinical syndrome which may lead to bleeding from isolated gastric varices. Portal hypertension is a term used to describe elevated pressures in the portal venous system a major vein that leads to the liver. Pancreatic mass leading to leftsided portal hypertension. Pdf splenomegaly and left sided portal hypertension. Hematological malignancy manifesting as ascites nature. Pah is commonly diagnosed at a late stage of the disease and is associated with progressive clinical deterioration and premature death.
Patrono d, benvenga r, moro f, rossato d, romagnoli r, salizzoni m. Pulmonary hypertension ph is a frequent complication of left heart disease arising from a wide range of cardiac disorders. Leftsided portal hypertension should be considered in the presence of gastrointestinal bleeding with normal liver function and unexplained splenomegaly. Portal hypertension can be quite serious, though its treatable if diagnosed in time. Pulmonary arterial hypertension pah is a severe disease characterised by a progressive increase of pulmonary pressure and resistance leading to right heart failure. Definition portal hypertension is defined as the elevation of the hepatic venous pressure gradient to 5 mmhg. Commonly, the operation is carried out by laparotomy, as portal hypertension is considered a relative contraindication to laparoscopic splenectomy ls. Leftsided portal hypertension can be considered as a diagnosis in patient with upper gastrointestinal tract bleeding due to oesophageal varices, gastric varices, or. Leftsided portal hypertension is a rare clinical condition most often associated with a pancreatic disease. Symptoms include varices, rectal bleeding, vomiting blood, ascites, hepatic encephalopathy, and enlarged spleen. It may be difficult to diagnose this entity both endoscopically and.
In case of hemorrhage from gastric fundus varices, splenectomy is indicated. The majority of splenic vein occlusions are the result of pancreatic diseases, including acute and chronic pancreatitis and pancreatic tumors. Its cause is unknown although omphalitis, peritonitis, exchange transfusions via the umbilical vein, or distant sepsis have occurred during the neonatal period in a few patients. Variceal bleeding due to extrahepatic portal hypertension may be first manifested at any age. Splenic vein ligation may result in sinistral left. Extramedullary plasmacytoma of the pancreas complicated. Isolated splenic vein thrombosis with left sided portal hypertension is a rare cause of upper gastrointestinal bleed. The current case is one of three cases yet reported where the mcn caused leftsided portal hypertension leading to the formation of isolated gastric varices and subsequent bleeding from the varices.
It usually occurs as a result of isolated obstruction of the splenic vein. Increased vascular resistance in the portal venous system from various mechanical causes above, and also as an active process in which liver damage activates stellate cells and myofibroblasts, contributing to the abnormal blood flow patterns. In this case report, we present a 73yearold man with falciform ligament abscess following cholangitis from an obstructive ampullary carcinoma. Unlimited viewing of the articlechapter pdf and any associated supplements and figures. Sinistral, or leftsided, portal hypertension sph is a rare cause of upper gastrointestinal hemorrhage. Symptoms are most often vague and this makes the diagnosis more difficult. Sinistral portal hypertension sph is also known as splenoportal, leftsided, segmental, regional, localized, compartmental or lineal portal hypertension. Throughout his hospital stay, the patient had multiple blood transfusions, and surgery was consulted to do a splenectomy for definitive management of gastric varices and leftsided portal hypertension. They are usually asymptomatic and have a potential for rupture and therefore lifethreatening hemorrhage.
We hypothesized that cardiac chamber volumes calculated from the same ct scans can yield additional information to distinguish ph related to leftsided heart disease world health organization group 2 from other ph subtypes. Publications home of jama and the specialty journals of. Portal hypertension is hypertension high blood pressure in the hepatic portal system made up of the portal vein and its branches, that drain from most of the intestine to the liver. The previous two cases reported possible leftsided portal hypertension with splenic vein thrombosis, leading to the development of collateral vessels including a gastrocaval shunt, which by some contiguous route connects to the brachiocephalic vein. It is reported to occur in the setting of cholecystitis and cholangitis, but the pathophysiology is poorly understood. Background a 63yearold caucasian woman presented with intermittent, leftsided abdominal discomfort without other symptoms. Portal hypertension commonly complicates cirrhosis, and variceal haemorrhage is its worst and most lifethreatening complication. B dilated gastroepiploic vein functioning as a shunt from the splenic hilum to the superior mesenteric vein.
Gastric varices that arise secondary to the splenic vein occlusion can result in gastrointestinal hemorrhaging. Splenic artery aneurysm is the most common type of visceral aneurysms. Abscess formation of the falciform ligament is incredibly rare and perplexing when encountered for the first time. Physical examination revealed massive splenomegaly and complete blood. In the past, rheumatic mitral valve disease was the most.
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