Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre

Arch Cardiovasc Dis. 2021 Mar;114(3):221-231. doi: 10.1016/j.acvd.2020.10.003. Epub 2020 Dec 3.


Background: Congenital portosystemic shunts are rare vascular malformations that may have an impact on the heart-lung system. Associated congenital and/or acquired heart diseases are poorly reported.

Aims: To analyse cardiovascular disorders within a large congenital portosystemic shunt population, and develop a diagnostic strategy.

Methods: Among the 168 consecutive fetuses and children referred for congenital portosystemic shunt (1996-2019), patients presenting with at least one cardiovascular disorder, including congenital heart disease, heart failure, portopulmonary hypertension and/or hepatopulmonary syndrome, were reviewed retrospectively. Cardiovascular disorders were detected using echocardiography and one or more of the following: right-sided heart catheterization; contrast-enhanced transthoracic echocardiography; or lung perfusion radionuclide scan.

Results: Overall, 46/168 patients with a congenital portosystemic shunt (27.4%) had one or more clinically significant cardiovascular disorders. Congenital heart disease was present in 28 patients, including six with left heterotaxy. Heart failure was present in six fetuses and 21 neonates (eight without congenital heart disease, and 13 with congenital heart disease). In neonates without congenital heart disease, heart function recovered by the age of 3years. Portopulmonary hypertension was identified in 11 patients (mean age at diagnosis: 9years); it was fatal in one patient, and remained stable in five of six patients after congenital portosystemic shunt closure. In six patients, hepatopulmonary syndrome presented as hypoxia (mean age at diagnosis: 5.3years), which reversed after congenital portosystemic shunt closure.

Conclusions: Evaluation and monitoring of the cardiopulmonary status of patients with a congenital portosystemic shunt is mandatory to detect and prevent cardiovascular complications. Furthermore, congenital portosystemic shunts must be sought in patients with unexplained cardiovascular disorders, especially when malformations are present.

Keywords: Congenital heart defects; Congenital portosystemic shunt; Heart failure; Hepatopulmonary syndrome; Hypertension pulmonaire; Insuffisance cardiaque; Malformations cardiaques congénitales; Pulmonary hypertension; Shunt portosystémique congénital; Syndrome hépatopulmonaire.

MeSH terms

  • Adolescent
  • Cardiovascular Diseases / diagnostic imaging
  • Cardiovascular Diseases / etiology*
  • Cardiovascular Diseases / physiopathology
  • Child
  • Child, Preschool
  • Coronary Circulation
  • Female
  • Hemodynamics*
  • Hepatopulmonary Syndrome / etiology
  • Hepatopulmonary Syndrome / physiopathology
  • Humans
  • Hypertension, Portal / etiology
  • Hypertension, Portal / physiopathology
  • Hypoxia / etiology
  • Hypoxia / physiopathology
  • Infant
  • Infant, Newborn
  • Liver Circulation
  • Male
  • Portal Vein / abnormalities*
  • Portal Vein / diagnostic imaging
  • Portal Vein / physiopathology
  • Portal Vein / surgery
  • Prognosis
  • Pulmonary Arterial Hypertension / etiology
  • Pulmonary Arterial Hypertension / physiopathology
  • Pulmonary Circulation
  • Referral and Consultation
  • Registries
  • Retrospective Studies
  • Tertiary Care Centers
  • Vascular Malformations / complications*
  • Vascular Malformations / diagnostic imaging
  • Vascular Malformations / physiopathology
  • Vascular Malformations / surgery

Supplementary concepts

  • Patent Ductus Venosus