Quality of life and perceived health status in surviving adults with univentricular heart

Abstract

OBJECTIVETo evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics.DESIGN AND SETTINGRetrospective, cross sectional study conducted in a regional paediatric cardiology centre.PATIENTSThe health records of 89 survivors with univentricular heart (median age 21 years; range 17–49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed.RESULTSThe Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures.CONCLUSIONSDespite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.

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]. 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].…”

Narrative review of single ventricle: where are we after 40 years?

“…Years ago, there were only case reports of individuals with single ventricle physiology surviving to adulthood. In more recent years, a number of patients have reached adulthood without any type of even palliative surgery (2,(278)(279)(280)(281)(282), or after some form of palliation but without requiring a Fontan completion (193,(283)(284)(285)(286). Attempts should be made to identify the morphologic and pathophysiological characteristics of these patients, identify the more favorable patterns, and compare their survival and quality of life with those who underwent all conventional surgical stages.…”

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

“…8,[22][23][24][25][26][27][28][29][30][31][32][33] Eleven studies found no increase in risk of internalising problems as compared to controls. [34][35][36][37][38][39][40][41][42][43][44] Anxiety symptoms were measured in 23 studies with 12 showing a higher risk of anxiety disorders compared to single ventricle heart disease compared to controls. One study showed as much as a fivefold increase in the rate of lifetime anxiety diagnosis in patients with single ventricle heart disease compared to referents (35% vs. 7%).…”