Post-surgical shunt hepatopulmonary syndrome in a case of non-cirrhotic portal hypertension

Hannam, Paul D.; Sandokji, Abdaul Kareem M.; Machan, Lindsay; Erb, Siegfried R.; Champion, P; Buczkowski, Andrzed K.; Scudamore, Charles H.; Steinbrecher, Urs P.; Chung, Stephen W.; Weiss, Alan; Yoshida, Eric M.

doi:10.1097/00042737-199912000-00015

Cited by 17 publications

(5 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Tercier et al [6] has documented that HPS was resolved by the shunt ligation in a type 2 Abernethy malformation patient with a 1-year history of hypoxemia. On the other hand, Hannam [21] has reported that shunt closure did not change the respiratory status in a 42-year-old patient who had undergone a decompressive surgical splenorenal shunt for noncirrhotic portal hypertension and presented with a 7-year history of cyanosis. We considered that the difference in efficacy of shunt ligation among the 3 cases should be attributed to the length of respiratory impairment, and dilatation of the pulmonary vessels could be irreversible with the long duration.…”

Section: Discussionmentioning

confidence: 98%

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation

Morikawa

Honna

Kuroda

et al. 2008

Journal of Pediatric Surgery

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 98%

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation

Morikawa

Honna

Kuroda

et al. 2008

Journal of Pediatric Surgery

View full text Add to dashboard Cite

“…We thought that patient 2, with Abernethy malformation type IIa, could develop sudden and marked portal hypertension with intestinal compromise if her large congenital shunt were suddenly ligated and portal blood flow forced through the hypoplastic portal vein 29. Additionally, the obliteration of a surgically constructed portosystemic shunt in a patient with noncirrhotic portal hypertension and HPS had failed to relieve hypoxia in a prior report 33. Tercier et al34 recently reported the successful treatment of HPS by ligation of the congenital shunt in a patient with Abernethy malformation type II, although this was not available at the time of our patient's assessment.…”

Section: Discussionmentioning

confidence: 99%

Pediatric hepatopulmonary syndrome is seen with polysplenia/interrupted inferior vena cava and without cirrhosis

Gupta¹,

Abramowsky²,

Pillen³

et al. 2007

Liver Transpl

View full text Add to dashboard Cite

Hepatopulmonary syndrome (HPS) is a triad of liver dysfunction, hypoxemia, and intrapulmonary vascular dilatation. We describe the prevalence and clinical features of HPS at a pediatric liver transplant center. Patients referred to Children's Healthcare of Atlanta/Emory University transplant program from February 1999 to May 2005 were reviewed. Oxygen saturation in room air was screened by percutaneous pulse oximetry. HPS cases were compared with similar age non-HPS recipients (n ϭ 38) to determine differences in clinical characteristics, Pediatric End-Stage Liver Disease (PELD) scores, and posttransplantation survival. Of 211 patients referred and 114 patients transplanted, 7 met criteria for HPS (3.3% and 6.1%, respectively). Patients with HPS had lower PELD score (Ϫ0.4 Ϯ 5.9 vs. 11 Ϯ 11; P ϭ 0.01) and total bilirubin (1.7 Ϯ 1.1 vs. 11.2 Ϯ 10.1; P ϭ 0.02) at the time of transplantation. Four of 7 patients with HPS had polysplenia/interrupted inferior vena cava (PS/IVC) compared with 0 of 38 age-matched controls (P ϭ 0.0002). Three patients with HPS did not have cirrhosis; 2 of these 3 had PS/IVC. All HPS cases normalized room air oxygen saturation by 6 months, and survival after transplantation in HPS cases was 100%. Marked hepatic synthetic or biochemical dysfunction may not be present, and cirrhosis is not a requirement for the development of HPS in children. HPS in children is frequently associated with PS/IVC. Histologic evidence of abnormal intrahepatic portal vein flow and the demonstration of portosystemic communications at any level should be sought in children presenting with unexplained intrapulmonary vascular dilatation. Liver transplantation for HPS in childhood may be appropriate even in the absence of cirrhosis. Liver Transpl 13:680-686, 2007. © 2007 AASLD.Received August 11, 2006; accepted January 1, 2007. Hepatopulmonary syndrome (HPS) consists of the triad of liver disease, hypoxemia, and intrapulmonary vascular dilatations (IPVD).1 The pathophysiology of HPS is not understood. A hepatically mediated imbalance between the pulmonary vasodilators and vasoconstrictors has been hypothesized to promote the development of IPVD in HPS.2 Putative humoral mediators may include nitric oxide and endothelin-1.

show abstract

“…6 HPS associated with noncirrhotic portal hypertension is uncommon. [7][8][9][10] In such patients, some degree of accompanying noncirrhotic liver injury may or may not exist. Resolution of HPS after OLT has been documented in that setting.…”

Section: Commentsmentioning

confidence: 99%

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Krowka

2001

Liver Transplantation

View full text Add to dashboard Cite

This report describes the unique development of pulmonary vascular dilatation and hypoxemia associated with portosystemic shunt in a pediatric liver transplant recipient. Ligation of the shunt resulted in resolution of hypoxemia. The outcome suggests that hepatic venous return to the pulmonary circulation is important in maintaining normal pulmonary vascular caliber.(2) Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. De BK, Sen S, Biswas PK, Sanyal R, Majumdar D, Biswas J. Gastroenterology 2000;118:192-196. (Reprinted with permission.) AbstractReports show that hepatopulmonary syndrome mostly occurs in the setting of advanced hepatic dysfunction, with the associated vasoactive substance imbalance believed to be responsible for its pathogenesis. However, hepatopulmonary syndrome has also been reported in cases of mild hepatic dysfunction or noncirrhotic portal hypertension, indicating that portal hypertension also plays a part in the pathogenesis. Liver transplantation remains the only therapeutic option of proven benefit. We describe 2 cases of hepatopulmonary syndrome in the setting of inferior vena cava (suprahepatic) obstruction, but with minimal hepatic dysfunction. After balloon cavoplasty, 1 patient showed, in addition to improvement of the features of hepatic outflow obstruction, significant reduction of dyspnea, cyanosis, and hypoxemia with arterial blood gas normalization within 2 weeks and intrapulmonary shunt reversal within 8 weeks. This implies that hemodynamic alterations (such as portal hypertension) independently contribute to the pathogenesis of hepatopulmonary syndrome in at least some of the cases. Therapies aimed at correcting these abnormal hemodynamics may be important in the treatment of this condition, especially when the hepatic functional status by itself does not warrant a liver transplant.

show abstract

Post-surgical shunt hepatopulmonary syndrome in a case of non-cirrhotic portal hypertension

Cited by 17 publications

References 0 publications

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation

Pediatric hepatopulmonary syndrome is seen with polysplenia/interrupted inferior vena cava and without cirrhosis

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Contact Info

Product

Resources

About