Top

Pediatric Surgery International

Published in:

01-10-2017 | Original Article

Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia

Authors: Takehisa Ueno, Ryuta Saka, Yuichi Takama, Hiroaki Yamanaka, Yuko Tazuke, Kazuhiko Bessho, Hiroomi Okuyama

Published in: Pediatric Surgery International | Issue 10/2017

Abstract

Purpose

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients.

Methods

BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests. Lung perfusion scintigraphy and cardiac ultrasound were performed to diagnose HPS. Cardiac catheterization was conducted to diagnose PoPH.

Results

The study population consisted of 88 BA patients. The median follow-up duration was 11.6 years (range 0.8–26.0 years). Six patients (6.8%) developed HPS and three patients (3.4%) developed PoPH. The median age of onset of HPS was significantly younger than that of PoPH (HPS: 4 years, PoPH: 15 years, P < 0.019). Two patients (66%) with PoPH died, while all patients with HPS survivied.

Conclusion

The onset of HPS was significantly earlier than that of PoPH. The mortality rate was high in patients with PoPH. Teenagers with BA should receive routine cardiac echocardiograms to detect PH in its early stages.

Okazaki T, Kobayashi H, Yamataka A et al (1999) Long-term postsurgical outcome of biliary atresia. J Pediatr Surg 34(2):312–315CrossRefPubMed

Castro M, Krowka MJ (1996) Hepatopulmonary syndrome. A pulmonary vascular complication of liver disease. Clin Chest Med 17(1):35–48CrossRefPubMed

Budhiraja R, Hassoun PM (2003) Portopulmonary hypertension: a tale of two circulations. Chest 123(2):562–576CrossRefPubMed

Rodriguez-Roisin R, Krowka MJ, Herve P et al (2004) Pulmonary–hepatic vascular disorders (PHD). Eur Respir J 24(5):861–880CrossRefPubMed

Mandell MS, Groves BM (1996) Pulmonary hypertension in chronic liver disease. Clin Chest Med 17(1):17–33CrossRefPubMed

Lee S, Park H, Moon SB et al (2013) Long-term results of biliary atresia in the era of liver transplantation. Pediatr Surg Int 29(12):1297–1301CrossRefPubMed

Gallo A, Esquivel CO (2013) Current options for management of biliary atresia. Pediatr Transplant 17(2):95–98CrossRefPubMed

Ogawa E, Hori T, Doi H et al (2014) Living-donor liver transplantation for congenital biliary atresia with porto-pulmonary hypertension and moderate or severe pulmonary arterial hypertension: Kyoto University experience. Clin Transplant 28(9):1031–1040CrossRefPubMed

Umeda A, Tagawa M, Kohsaka T et al (2006) Hepatopulmonary syndrome can show spontaneous resolution: possible mechanism of portopulmonary hypertension overlap? Respirology 11(1):120–123CrossRefPubMed

10.

Urashima Y, Tojimbara T, Nakajima I et al (2004) Living related liver transplantation for biliary atresia with portopulmonary hypertension: case report. Transplant Proc 36(8):2237–2238CrossRefPubMed

11.

Sasaki T, Hasegawa T, Kimura T et al (2000) Development of intrapulmonary arteriovenous shunting in postoperative biliary atresia: evaluation by contrast-enhanced echocardiography. J Pediatr Surg 35(11):1647–1650CrossRefPubMed

12.

Soh H, Hasegawa T, Sasaki T et al (1999) Pulmonary hypertension associated with postoperative biliary atresia: report of two cases. J Pediatr Surg 34(12):1779–1781CrossRefPubMed

13.

Hoeper MM, Krowka MJ, Strassburg CP (2004) Portopulmonary hypertension and hepatopulmonary syndrome. Lancet 363(9419):1461–1468CrossRefPubMed

14.

Zopey R, Susanto I, Barjaktarevic I et al (2013) Transition from hepatopulmonary syndrome to portopulmonary hypertension: a case series of 3 patients. Case Rep Pulmonol 2013:561870PubMedPubMedCentral

15.

Ioachimescu OC, Mehta AC, Stoller JK (2007) Hepatopulmonary syndrome following portopulmonary hypertension. Eur Respir J 29(6):1277–1280CrossRefPubMed

16.

Martinez-Palli G, Barbera JA, Taura P et al (1999) Severe portopulmonary hypertension after liver transplantation in a patient with preexisting hepatopulmonary syndrome. J Hepatol 31(6):1075–1079CrossRefPubMed

17.

Mal H, Burgiere O, Durand F et al (1999) Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis. J Hepatol 31(2):360–364CrossRefPubMed

18.

Pham DM, Subramanian R, Parekh S (2010) Coexisting hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation. J Clin Gastroenterol 44(7):e136–e140PubMed

19.

Matsumoto H, Uemasu J, Kitano M et al (1994) Clinical significance of plasma endothelin-1 in patients with chronic liver disease. Dig Dis Sci 39(12):2665–2670CrossRefPubMed

20.

Uchihara M, Izumi N, Sato C et al (1992) Clinical significance of elevated plasma endothelin concentration in patients with cirrhosis. Hepatology 16(1):95–99CrossRefPubMed

21.

Hasegawa T, Kimura T, Sasaki T et al (2001) Plasma endothelin-1 level as a marker reflecting the severity of portal hypertension in biliary atresia. J Pediatr Surg 36(11):1609–1612CrossRefPubMed

22.

Swanson KL, Wiesner RH, Nyberg SL et al (2008) Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups. Am J Transplant 8(11):2445–2453CrossRefPubMed

Title: Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia
Authors: Takehisa Ueno
Ryuta Saka
Yuichi Takama
Hiroaki Yamanaka
Yuko Tazuke
Kazuhiko Bessho
Hiroomi Okuyama
Publication date: 01-10-2017
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Surgery International / Issue 10/2017
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-017-4136-x

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia

Abstract

Purpose

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 10/2017

Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan

An iPhone application using a novel stool color detection algorithm for biliary atresia screening

Management of congenital tracheal stenosis in the neonatal period

Enhancing recovery after minimally invasive repair of pectus excavatum

Comparison of outcomes of thoracoscopic primary repair of gross type C esophageal atresia performed by qualified and non-qualified surgeons

Changes in quality of life from infancy to school age after esophagoesophagostomy for tracheoesophageal fistula: thoracotomy versus thoracoscopy