Psychological functioning in paediatric patients with single ventricle heart disease: a systematic review

Abstract

Background: Patients with single ventricle heart disease are living into adulthood due to medical and surgical advancements but have significant physical comorbidities and an increased risk for psychological comorbidities compared to healthy subjects or those with other CHD diagnoses. This study aimed to systematically review psychological functioning in paediatric single ventricle heart disease. Methods: Literature was searched using PubMed, Embase, PsycInfo, CINAHL Complete and Scopus. Peer-reviewed articles that included patients ages 0–25 years with single ventricle heart disease, and quantitative measures of psychological outcomes were included. Meta-analysis using a fixed-effect model was conducted for internalising and externalising t-scores, utilised by the Achenbach Child Behavior Checklist. Results: Twenty-nine records met the criteria for inclusion. 13/24 studies demonstrated increased risk for internalising disorders, such as anxiety/depression; 16/22 studies demonstrated risk for externalising disorders, such as attention or behavioural problems. Meta-analysis of four studies revealed that paediatric single ventricle heart disease patients had no significant difference in internalising and externalising t-scores compared to normative values. Conclusions: The current review demonstrates the need for further studies to better understand psychological functioning in patients with single ventricle heart disease, with a majority of studies showing increased risk for psychological problems despite no difference seen in a small meta-analysis. This summary of the literature underscores the need for regular psychological screening, earlier intervention and integrated mental health therapies in paediatric single ventricle heart disease.

The Functioning of the Frog’s Single Ventricle

“…However, this finding need be validated in a larger sample. Similarities between the anatomical and physiological findings in frogs and other amphibians and the few reported cases with single ventricle who survived until adult age without surgery have already been proposed [12][13][14][15][16][17]40,41,54]. The presence of intact interatrial septum, preventing any blood mixing at atrial level, may explain why, in the absence of complete mixing in the single ventricular chamber, the amphibians can live unrestrictive long life.…”

“…The life expectancy of children born with "functionally" uni-ventricular hearts is severely reduced, as well as the long-term outcomes are negatively affected by heart failure and cyanosis, substantially compromising the quality of life [1][2][3][4][5][6][7][8][9][10][11][12][13]. Reports of patients with single ventricle surviving to the adult life either without any surgical treatment or after limited palliative procedures without undergoing Fontan completion are exceptionally rare [12,[14][15][16][17][18][19][20][21][22][23].…”

“…The life expectancy of children born with "functionally" uni-ventricular hearts is severely reduced, as well as the long-term outcomes are negatively affected by heart failure and cyanosis, substantially compromising the quality of life [1][2][3][4][5][6][7][8][9][10][11][12][13]. Reports of patients with single ventricle surviving to the adult life either without any surgical treatment or after limited palliative procedures without undergoing Fontan completion are exceptionally rare [12,[14][15][16][17][18][19][20][21][22][23]. In patients born with this type of complex congenital heart defect at least 2-3 staged operations are needed to achieve Fontan circulation, with the single ventricular chamber pumping the oxygenated blood to the body (systemic circulation), while the desaturated blood returning from the superior and inferior vena cava by pressure difference passively flows directly through the lungs (pulmonary circulation) [1][2][3][4][5][6][7][8][9][10][11][12][13].…”

“…Reports of patients with single ventricle surviving to the adult life either without any surgical treatment or after limited palliative procedures without undergoing Fontan completion are exceptionally rare [12,[14][15][16][17][18][19][20][21][22][23]. In patients born with this type of complex congenital heart defect at least 2-3 staged operations are needed to achieve Fontan circulation, with the single ventricular chamber pumping the oxygenated blood to the body (systemic circulation), while the desaturated blood returning from the superior and inferior vena cava by pressure difference passively flows directly through the lungs (pulmonary circulation) [1][2][3][4][5][6][7][8][9][10][11][12][13]. Abnormally high systemic venous pressure is a consequence of the arrangement of this type of circulation and the chronic elevation of systemic venous pressure may cause liver failure, renal failure, protein-losing enteropathy and plastic bronchitis [4][5][6][7][8][9][10][11]13,24,25].…”

Recommendations for Centers Performing Pediatric Heart Surgery in the United States

“…These recommendations are due to recent data showing that patients with CHD regardless of age or disease severity appear to have a significantly higher burden of anxiety and depression and that caregivers can also have significant mental health needs both pre-and postoperatively. [135][136][137] It is recommended that across centers screening and referral for services are available whether in person or via telemedicine. Essential Care Centers may also partner with Comprehensive Care Centers for provision of these services (Table 13).…”