Adolescents with congenital heart disease: psychopathological implications

Adolescence, Spring, 1999 by Gabriele Masi, Paola Brovedani

An illness at an early age, especially if it is congenital, life threatening, of uncertain prognosis, and requires frequent therapeutic interventions, is an extremely significant life event. Yet, the psychological implications of the disease for the child and the family are often neglected. The aim of this paper is to describe the effects of congenital heart disease on adolescents' emotional development and on the quality of familial relations. Greater knowledge, in turn, can help offset psychopathological risks

EMOTIONAL DEVELOPMENT

The findings on the emotional development of children and adolescents with cardiopathy are diverse (Glaser, Harrison, & Lynn, 1964; Linde, Rasof, & Dunn, 1967; Kramer et al., 1989; Yang, Liu, & Townes, 1994). The experience of disease can provoke different reactions, such as regression, loneliness and feelings of inadequacy, disruption of the sense of self, and thought patterns that center on dying and death (Ajuriaguerra & Marcelli, 1982; Ferrari, 1989). A "vulnerable child syndrome" has been proposed as a possible consequence of severe illness (Green & Solnit, 1964). These children manifest a strong bond with the mother and intense separation anxiety, which can endure through adolescence. Thus, the disease may become a main actor in the psychic play.

In adolescence, the main goal is to achieve autonomy through separation and individuation. Some ill adolescents accept the dependence that accompanies drug therapies and other medical interventions. Others, however, may rebel against all forms of restrictions, real or imagined, with defiant and risky behaviors. For example, compliance with insulin therapy is a problem for 50% of diabetic adolescents, and about 30% are noncompliant (Kovacs et al., 1992).

While parents may impose physical constraints on the ill adolescent, they may be overindulgent in other areas for fear that any form of frustration may have lethal effects. The adolescent may then oscillate between passive adaptation and tyrannical behaviors, impulsiveness and feelings of inferiority (Kramer et al., 1989).

Anxiety and depression can alter the quality of thought processes in children and adolescents faced with prolonged hospitalizations, lowering academic achievement and self-esteem. In addition to the effects on intellectual functioning, fantasy and emotional life may be inhibited.

When a child or an adolescent is faced with a potentially life-threatening experience, the specter of death can become overwhelming (Rimbault, 1976). The representation of death depends on the level of psychic maturation. In adolescence, it has less to do with a sense of nothingness and fear of the unknown (as it does for adults) and more to do with a sense of abandonment and loneliness.

Adolescents may have difficulty comprehending a severe congenital disease. Their theories about the origin of the disease are often centered on themes of guilt: the illness is a punishment for bad actions or thoughts. These themes may be shared by other members of the family.

Body image, which is a fundamental element of self-identity, may be distorted. The disease seems to foster a "constricted" view of the physical self, as shown in drawings of the human figure by children and adolescents with cardiopathy (Green & Levitt, 1962). This is particularly evident when a surgical procedure is necessary, as in the case of open-heart surgery or heart transplant. In adolescence, disruptions in the normal development of body image can lower self-esteem and promote depressive symptoms.

INTELLECTUAL DEVELOPMENT

Research on the cognitive development of children with cardiopathy has distinguished between cyanotic and acyanotic conditions. Many studies report cognitive difficulties in children with congenital heart disease and a positive correlation between level of cognitive functioning and clinical severity of cardiopathy (Silbert et al., 1969; Linde et al., 1967; Rausch de Traubenberg, 1973; Kramer et al., 1989; Yang et al., 1994). Children younger than three years of age with cyanotic cardiopathy perform worse than age-matched controls in gross motor, adaptive, social, and fine motor areas (Gesell Developmental Schedules), with the lowest score reported for gross motor abilities (Linde et al., 1967). There is a delay in motor development (perhaps because of the early limitations in physical activity), while language development is normal. In children with acyanotic heart disease, only gross motor performance is below average.

Children older than three years of age with cyanotic and acyanotic heart disease have an intelligence quotient (IQ) in the normal range, yet the IQ of the cyanotics is significantly lower than that of acyanotics for both the Stanford-Binet (Linde et al., 1967; Silbert et al., 1969) and the Wechsler scales (Kramer et al., 1989). Cyanotics are more delayed than noncyanotics without heart failure; noncyanotic children with heart failure occupy an intermediate position (Silbert et al., 1969). Differences in intellectual and perceptual performance between cyanotic and noncyanotic children are statistically significant irrespective of their physical activity (Silbert et al., 1969). A positive correlation has been reported for a hematological variable (arterial oxygen saturation), developmental milestones, and IQ: the lower the oxygen saturation, the lower the IQ and the more impaired are gross motor abilities.