Virginie Lambert1, Delphine Ladarre2, Feriel Fortas3, Philippe Durand3, Pierre Hervé4, Emmanuel Gonzales5, Florent Guérin6, Laurent Savale7, Valérie A McLin8, Oanez Ackermann2, Stéphanie Franchi-Abella9. 1. Paediatric and Congenital Cardiology, Department of Cardiology, Institut Mutualiste Montsouris, 75014 Paris, France; Department of Paediatric Radiology, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France. Electronic address: v.lambert@cardiocongenitale.fr. 2. Department of Paediatric Hepatology and Liver Transplantation Unit, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France; Centre de Référence des Maladies Vasculaires du Foie (MVF), Filière de Santé des Maladies Rares du Foie de l'Enfant et de l'Adulte (FILFOIE), European Reference Network RARE-LIVER, FHU Hepatinov, Bicêtre Hospital, AP-HP, Paris-Saclay University Hospitals, 94275 Le Kremlin-Bicêtre cedex, France. 3. Department of Paediatric Intensive Care and Neonatal Medicine, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France. 4. Department of Paediatric Radiology, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France. 5. Department of Paediatric Hepatology and Liver Transplantation Unit, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France; Centre de Référence des Maladies Vasculaires du Foie (MVF), Filière de Santé des Maladies Rares du Foie de l'Enfant et de l'Adulte (FILFOIE), European Reference Network RARE-LIVER, FHU Hepatinov, Bicêtre Hospital, AP-HP, Paris-Saclay University Hospitals, 94275 Le Kremlin-Bicêtre cedex, France; INSERM UMR-S 1193, Paris-Saclay, 91400 Orsay, France. 6. Centre de Référence des Maladies Vasculaires du Foie (MVF), Filière de Santé des Maladies Rares du Foie de l'Enfant et de l'Adulte (FILFOIE), European Reference Network RARE-LIVER, FHU Hepatinov, Bicêtre Hospital, AP-HP, Paris-Saclay University Hospitals, 94275 Le Kremlin-Bicêtre cedex, France; Department of Paediatric Surgery, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France. 7. Department of Respiratory and Intensive Care Medicine, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France; INSERM UMR-999, Marie-Lannelongue Hospital, 92350 Le Plessis-Robinson, France. 8. Swiss Paediatric Liver Center, Department of Paediatrics, Gynecology and Obstetrics, European Reference Network RARE-LIVER, University Hospitals Geneva and University of Geneva Medical School, 1205 Geneva, Switzerland. 9. Department of Paediatric Radiology, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France; Department of Paediatric Hepatology and Liver Transplantation Unit, Bicêtre Hospital, AP-HP, Paris-Saclay, 94275 Le Kremlin-Bicêtre, France; Centre de Référence des Maladies Vasculaires du Foie (MVF), Filière de Santé des Maladies Rares du Foie de l'Enfant et de l'Adulte (FILFOIE), European Reference Network RARE-LIVER, FHU Hepatinov, Bicêtre Hospital, AP-HP, Paris-Saclay University Hospitals, 94275 Le Kremlin-Bicêtre cedex, France; BioMaps-UMR 8081, University Paris-Saclay, 91400 Orsay, France.
Abstract
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.
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.
Authors: Atessa Bahadori; Beatrice Kuhlmann; Dominique Debray; Stephanie Franchi-Abella; Julie Wacker; Maurice Beghetti; Barbara E Wildhaber; Valérie Anne McLin Journal: Children (Basel) Date: 2022-02-11