Congenital heart disease-associated liver disease: a narrative review

Abstract

Congenital heart diseases (CHD) can be associated with liver dysfunction. The cause for liver impairment can result out of a wide spectrum of different causes, including liver congestion, hypoxemia or low cardiac output. Fortunately, most CHD show a good long-term outcome from a cardiac perspective, but great attention should be paid on non-cardiac health problems that develop frequently in patients suffering from CHD. The treatment of liver dysfunction in CHD requires a close multidisciplinary management in a vulnerable patient collective. Unfortunately, structured recommendations on the management of liver dysfunction in patients with CHD are scarce. The objective of this review is to provide insights on the pathophysiology and etiologies of liver dysfunction as one of the most relevant non-cardiac problems related to CHD. Furthermore, we advise here on the management of liver disease in CHD with special attention on assessment of liver dysfunction, management of portal hypertension as well as on surveillance and management of hepatocellular carcinoma (HCC). A multidisciplinary perspective may help to optimize morbidity and mortality in the long-term course in these patients. However, as evidence is low in many aspects, we encourage the scientific community to perform prospective studies to gain more insights in the treatment of liver dysfunction in patients with CHD.

Caring for the Critically Ill Adult Congenital Heart Disease Patient

“…Patients with congenital heart disease often have comorbid liver dysfunction due to venous congestion, decreased cardiac output, hypoxemia, or ischemic injury to the hepatic parenchyma. This is especially common in patients with right-sided lesions (such as ToF or Ebstein anomaly), or in single ventricle physiology palliated with Fontan circulation [ 27 ]. For patients who underwent surgery before 1992, viral hepatitis infection from a blood transfusion should also be considered an etiology of liver disease [ 28 ].…”

Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management

“…In blood expectoration cases, the differentiation of haemoptysis from haematemesis is highlighted; especially in patients with PAH-CHD and known liver dysfunction or cirrhosis as a result of progressive right heart failure [36,43]. Preceding nausea, dark colored sputum simulating coffee ground in the absence of frothiness, and the presence of food particles compose supporting evidence of hematemesis and require vigilance [44].…”

Somatic GATA4 mutation contributes to tetralogy of Fallot

“…As a global pediatric concern, congenital heart defects comprise a wide spectrum of cardiovascular developmental defects, which are categorized into >25 distinct clinical subtypes, including tetralogy of Fallot (TOF) ( 1 ). Although certain minor congenital heart defects spontaneously resolve, severe congenital heart disease may lead to poor health and quality of life ( 5-8 ), diminished physical exercise capacity ( 9-13 ), impaired neurodevelopment (the most prevalent extracardiac manifestation in patients with a congenital heart defect) and brain damage ( 14-18 ), thromboembolic complications ( 19-21 ), acute renal injury and chronic kidney disease ( 22-24 ), hepatic dysfunction ( 25 ), pulmonary arterial hypertension ( 26-28 ), infective endocarditis ( 29-31 ), congestive cardiac failure ( 32-34 ), miscellaneous cardiac dysrhythmia ( 35-37 ) and cardiovascular demise ( 38-40 ). Improvement has been made in cardiovascular surgery and transcatheter interventional treatment, which has allowed >90% of children with congenital heart defects to survive to adulthood; adults living with various congenital heart defects outnumber children affected by congenital heart defects ( 41-43 ).…”