Congenital portosystemic shunts (CPSS) are rare and underdiagnosed abdominal vascular malformations. Their relationship with cardiovascular (CV) disorders are poorly reported. Herein, we describe CV disorders associated with CPSS in a large cohort to propose adequate diagnosis and management. Among 168 children referred for a CPSS (1996–2019), those presenting at least one clinically significant CV disease including congenital heart diseases (CHD), heart failure (HF), pulmonary hypertension (PH) and/or hepatopulmonary syndrome (HPS) were included. CV disorders were detected using echocardiography complemented by right-sided heart catheterization and/or lung perfusion radionuclide when appropriate. 46/168 children with CPSS (27,4%) were included. In 36 patients, endovascular or surgical CPSS closure was required, including 24 for CV indications. CHD was present in 28 patients (16,6%); 7 had heterotaxy (4,1%). HF occurred in 21 neonates, diagnosed prenatally in 7, including 13 with CHD. HF decreased after cardiac surgery in 10 with CHD but also spontaneously before the age of 3 years in 8 except one without CHD. In 11 patients (mean age: 11±5.2 years) CPSS were diagnosed as part of a work up for fixed PH (mean pressures 46±15.1mmHg, pulmonary vascular resistances 7±2.5UW/m²). One of them died before CPSS closure, while PH remained stable despite CPSS closure in 10 patients, of which 2 then required lung transplantation. In 6 patients (median age: 5.5 years), hypoxia revealed HPS which resolved within 2 years of CPSS closure. CV disorders are frequently associated with CPSS including CHD with/without heterotaxy. HF is found in fetuses and neonates, regressing in most cases. Later, HPS and PH may develop. After CPSS closure, HPS is reversible while PH may only stabilize. Perinatal HF, complex CHD, heterotaxy, unusual course of CHD, HPS and PH should raise the possibility of CPSS. Conversely CV complications should be searched for in patients with CPSS.