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

Lambert, Virginie; Ladarre, Delphine; Fortas, Feriel; Durand, Philippe; Hervé, Pierre; Gonzalès, Emmanuel; Guérin, Florent; Savale, Laurent; Mclin, Valérie Anne; Ackermann, O.; Franchi‐Abella, Stéphanie

doi:10.1016/j.acvd.2020.10.003

Cited by 20 publications

(45 citation statements)

References 26 publications

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“…In our study, 75% (12/16) of patients had congenital anomalies, a rate in accordance with 65% (80/123) of patients with CPSS diagnosed prenatally ( 3 ). Congenital heart disease is the most common anomaly associated with CPSS (ranging from 17 to 30%) ( 1 – 3 , 5 , 7 – 9 ), which was discovered in 44% (7/16) patients in our study. In fact, these malformations, including CPSS, may be part of a syndrome or genetic abnormality or complex congenital malformative process such as Down syndrome, Trisomy 18, Turner syndrome, Noonan syndrome, and Bannayan–Zonana syndrome ( 1 – 3 ).…”

Section: Discussionmentioning

confidence: 48%

“…With the development of prenatal imaging, the number of CPSS diagnosed prenatally is increasing ( 3 , 5 ). In our study, three patients were prenatally diagnosed through US.…”

Section: Discussionmentioning

confidence: 99%

“…Francois et al ( 3 ) show that abnormal prenatal hemodynamics (pericardial effusion, dilated cardiomyopathy, and tricuspid insufficiency) is the most frequent complication (41%, 27/66) associated with CPSS. In addition, an abnormal intrahepatic duct or anechoic tubular, a dilated hepatic vein (HV), atypia of liver size, other prenatal hemodynamic abnormalities (e.g., oligohydramnios, polyhydramnios, elevated abdominal circumference during the third trimester), and CHD detected by prenatal US are important clues for identifying CPSS ( 3 , 5 , 6 ). In our study, two patients had oligohydramnios, and one patient presented with hepatomegaly, intrahepatic umbilical vein varix, hydropericardium, and polyhydramnios on prenatal US.…”

Section: Discussionmentioning

confidence: 99%

“…In fact, these malformations, including CPSS, may be part of a syndrome or genetic abnormality or complex congenital malformative process such as Down syndrome, Trisomy 18, Turner syndrome, Noonan syndrome, and Bannayan–Zonana syndrome ( 1 – 3 ). Lambert et al ( 5 ) found that left heterotaxy with complex cyanotic heart disease and polysplenia syndrome presented in 15% of patients with CPSS and at least one cardiovascular disorder, which is very rare in the general population ( 10 ). In view of this close association, screening for CPSS should be performed in neonates with CHD, especially those with multiple malformations.…”

Section: Discussionmentioning

confidence: 99%

“…However, when patients with CPSS develop severe complications or fail to close shunt spontaneously, we need to determine whether these clinical symptoms are entirely caused by CPSS or by other anomalies. Lambert et al ( 5 ) found that heart failure was the main symptom in CPSS children with cardiovascular disorders (especially neonates with hemodynamically significant CHD) during the first months of life. However, heart failure symptoms of these neonates gradually improved under conservative treatment and/or after cardiac surgery.…”

Section: Discussionmentioning

confidence: 99%

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Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period

Zhang

et al. 2021

Front. Pediatr.

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Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.

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Section: Discussionmentioning

confidence: 48%

“…With the development of prenatal imaging, the number of CPSS diagnosed prenatally is increasing ( 3 , 5 ). In our study, three patients were prenatally diagnosed through US.…”

Section: Discussionmentioning

confidence: 99%