Skip to main content
Erschienen in: Heart and Vessels 2/2021

25.08.2020 | Original Article

Portosystemic venous shunt in the patients with Fontan circulation

verfasst von: Hazumu Nagata, Ichiro Sakamoto, Shouji Fukuoka, Ayako Ishikita, Kiyoshi Uike, Yusaku Nagatomo, Yuichiro Hirata, Kenichiro Yamamura, Shouichi Ohga

Erschienen in: Heart and Vessels | Ausgabe 2/2021

Einloggen, um Zugang zu erhalten

Abstract

Portosystemic venous shunt (PSVS) is a vascular anomaly between the portal and systemic veins, resulting in several critical complications. Although PSVS is often associated with congenital heart diseases, the clinical association between Fontan circulation and PSVS has not been elucidated. This study aimed to investigate the clinical features of Fontan patients with PSVS. Two hundred thirteen patients who underwent Fontan procedure are being followed up at Adult Congenital Heart Disease clinic in Kyushu University Hospital. Among them, 139 adult patients underwent cardiac catheterization between January 1, 2011 and September 30, 2019. Medical records were reviewed to investigate the laboratory, echocardiography, and cardiac catheterization findings, as well as clinical manifestations and outcomes. Eleven Fontan patients received the diagnosis of PSVS. The median age at cardiac catheterization was 25 (range 18–45) years. Fontan operation was performed using extracardiac conduit or lateral tunnel 22 (16–35) years previously. Ten patients presented with chronic heart failure [New York Heart Association class 2 (n = 5) and 3 (n = 5)]. The median level of peripheral oxygen saturation was 87 (70–95)%. Cardiac catheterization showed increased cardiac index [5.3 (2.72–14.3) L/min/m2] with or without high central venous pressure [18 (9–25) mmHg]. Although the pulmonary vascular resistance was within the normal range, the systemic vascular resistance was decreased [7.08 (1.74–18.6) Wood units]. Fontan patients complicated with PSVS had increased cardiac output. The presence of PSVS in Fontan circulation might be associated with unfavorable long-term outcome.
Literatur
1.
Zurück zum Zitat Blanc T, Guerin F, Franchi-Abella S, Jacquemin E, Pariente D, Soubrane O, Branchereau S, Gauthier F (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260:188–198CrossRef Blanc T, Guerin F, Franchi-Abella S, Jacquemin E, Pariente D, Soubrane O, Branchereau S, Gauthier F (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260:188–198CrossRef
2.
Zurück zum Zitat Uike K, Nagata H, Hirata Y, Yamamura K, Terashi E, Matsuura T, Morihana E, Ohkubo K, Ishii K, Sakai Y, Taguchi T, Ohga S (2018) Effective shunt closure for pulmonary hypertension and liver dysfunction in congenital portosystemic venous shunt. Pediatr Pulmonol 53:505–511CrossRef Uike K, Nagata H, Hirata Y, Yamamura K, Terashi E, Matsuura T, Morihana E, Ohkubo K, Ishii K, Sakai Y, Taguchi T, Ohga S (2018) Effective shunt closure for pulmonary hypertension and liver dysfunction in congenital portosystemic venous shunt. Pediatr Pulmonol 53:505–511CrossRef
3.
Zurück zum Zitat Ohuchi H (2017) Where is the “optimal” Fontan hemodynamics? Korean Circ J 47:842–857CrossRef Ohuchi H (2017) Where is the “optimal” Fontan hemodynamics? Korean Circ J 47:842–857CrossRef
4.
Zurück zum Zitat Gewillig M, Goldberg DJ (2014) Failure of the fontan circulation. Heart Fail Clin 10:105–116CrossRef Gewillig M, Goldberg DJ (2014) Failure of the fontan circulation. Heart Fail Clin 10:105–116CrossRef
5.
Zurück zum Zitat Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287CrossRef Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287CrossRef
6.
Zurück zum Zitat Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guerin F, International Registry of Congenital Portosystemic Shunt member (2018) Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY) 43:2023–2036CrossRef Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guerin F, International Registry of Congenital Portosystemic Shunt member (2018) Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY) 43:2023–2036CrossRef
7.
Zurück zum Zitat Papamichail M, Pizanias M, Heaton N (2018) Congenital portosystemic venous shunt. Eur J Pediatr 177:285–294CrossRef Papamichail M, Pizanias M, Heaton N (2018) Congenital portosystemic venous shunt. Eur J Pediatr 177:285–294CrossRef
8.
Zurück zum Zitat McElhinney DB, Marx GR, Newburger JW (2011) Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review. Congenit Heart Dis 6:28–40CrossRef McElhinney DB, Marx GR, Newburger JW (2011) Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review. Congenit Heart Dis 6:28–40CrossRef
9.
Zurück zum Zitat Maruyama H, Kondo T, Kiyono S, Sekimoto T, Takahashi M, Yokosuka O (2015) Influence of splenorenal shunt on long-term outcomes in cirrhosis. Scand J Gastroenterol 50:593–600CrossRef Maruyama H, Kondo T, Kiyono S, Sekimoto T, Takahashi M, Yokosuka O (2015) Influence of splenorenal shunt on long-term outcomes in cirrhosis. Scand J Gastroenterol 50:593–600CrossRef
10.
Zurück zum Zitat Qi X, Qi X, Zhang Y, Shao X, Wu C, Wang Y, Wang R, Zhang X, Deng H, Hou F, Li J, Guo X (2017) Prevalence and clinical characteristics of spontaneous splenorenal shunt in liver cirrhosis: a retrospective observational study based on contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) scans. Med Sci Monit 23:2527–2534CrossRef Qi X, Qi X, Zhang Y, Shao X, Wu C, Wang Y, Wang R, Zhang X, Deng H, Hou F, Li J, Guo X (2017) Prevalence and clinical characteristics of spontaneous splenorenal shunt in liver cirrhosis: a retrospective observational study based on contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) scans. Med Sci Monit 23:2527–2534CrossRef
11.
Zurück zum Zitat Hsieh JS, Wang JY, Huang CJ, Chen FM, Huang TJ (2004) Effect of spontaneous portosystemic shunts on hemorrhage from esophagogastric varices. World J Surg 28:23–28CrossRef Hsieh JS, Wang JY, Huang CJ, Chen FM, Huang TJ (2004) Effect of spontaneous portosystemic shunts on hemorrhage from esophagogastric varices. World J Surg 28:23–28CrossRef
12.
Zurück zum Zitat Gordon-Walker TT, Bove K, Veldtman G (2019) Fontan-associated liver disease: a review. J Cardiol 74:223–232CrossRef Gordon-Walker TT, Bove K, Veldtman G (2019) Fontan-associated liver disease: a review. J Cardiol 74:223–232CrossRef
13.
Zurück zum Zitat Terashi E, Kodama Y, Kuraoka A, Ishikawa Y, Nakamura M, Sagawa K, Ishikawa S (2019) Usefulness of liver stiffness on ultrasound shear-wave elastography for the evaluation of central venous pressure in children with heart diseases. Circ J 83:1338–1341CrossRef Terashi E, Kodama Y, Kuraoka A, Ishikawa Y, Nakamura M, Sagawa K, Ishikawa S (2019) Usefulness of liver stiffness on ultrasound shear-wave elastography for the evaluation of central venous pressure in children with heart diseases. Circ J 83:1338–1341CrossRef
14.
Zurück zum Zitat McKie PM, McCully RB, Kamath PS, Bower TC, McKusick MA, Lapyere AC, Pereira NL (2012) Amelioration of high cardiac output and pulmonary hypertension by occlusion of congenital porto-systemic shunt. Circulation 126:2533–2534CrossRef McKie PM, McCully RB, Kamath PS, Bower TC, McKusick MA, Lapyere AC, Pereira NL (2012) Amelioration of high cardiac output and pulmonary hypertension by occlusion of congenital porto-systemic shunt. Circulation 126:2533–2534CrossRef
15.
Zurück zum Zitat Ohuchi H, Miyazaki A, Negishi J, Hayama Y, Nakai M, Nishimura K, Ichikawa H, Shiraishi I, Yamada O (2017) Hemodynamic determinants of mortality after Fontan operation. Am Heart J 189:9–18CrossRef Ohuchi H, Miyazaki A, Negishi J, Hayama Y, Nakai M, Nishimura K, Ichikawa H, Shiraishi I, Yamada O (2017) Hemodynamic determinants of mortality after Fontan operation. Am Heart J 189:9–18CrossRef
16.
Zurück zum Zitat Miranda WR, Borlaug BA, Hagler DJ, Connolly HM, Egbe AC (2019) Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis. Eur J Heart Fail 21:803–809CrossRef Miranda WR, Borlaug BA, Hagler DJ, Connolly HM, Egbe AC (2019) Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis. Eur J Heart Fail 21:803–809CrossRef
Metadaten
Titel
Portosystemic venous shunt in the patients with Fontan circulation
verfasst von
Hazumu Nagata
Ichiro Sakamoto
Shouji Fukuoka
Ayako Ishikita
Kiyoshi Uike
Yusaku Nagatomo
Yuichiro Hirata
Kenichiro Yamamura
Shouichi Ohga
Publikationsdatum
25.08.2020
Verlag
Springer Japan
Erschienen in
Heart and Vessels / Ausgabe 2/2021
Print ISSN: 0910-8327
Elektronische ISSN: 1615-2573
DOI
https://doi.org/10.1007/s00380-020-01685-y

Weitere Artikel der Ausgabe 2/2021

Heart and Vessels 2/2021 Zur Ausgabe

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.