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Adolescents with congenital heart disease: psychopathological implications.

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


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.


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.

It is hard to define the role of chronic hypoxia in intellectual development. The hypothesis of a hypoxic effect on the central nervous system following cyanotic heart disease has been maintained only for specific populations; for example, patients with complications arising from cardiovascular surgery or postoperative intensive care. It is not clear whether the negative effect of hypoxia is greater in particular phases of central nervous system development, yet this information could be crucial for the timing of surgical intervention. Some evidence indicates that the earlier the surgical intervention, the more favorable the prognosis for future intellectual functioning. This can be attributed both to a reduction in hypoxia and to the global improvement in the child's physical condition.

Psychological factors, such as low self-esteem, anxiety, and impulsiveness, may influence cognitive functioning. Fatigability, parental anxiety (resulting in emotional overstimulation and limitations on life experiences), discontinuity in scholastic attendance, and reduction in social interaction may also negatively affect intellectual performance.


Is there psychopathological specificity to heart transplant in children and adolescents? Evaluation of transplant patients (congenital heart disease or cardiomyopathy) does not reveal distortions in cognitive or emotional development (Pericchi, 1994). However, in a subgroup of children, intellectual difficulties have been reported; an IQ below 100 is significantly more frequent in those with transplants than in the general population. Impairment in attention/concentration, verbal learning, and abstract reasoning have also been found (Nussbaum & Goldstein, 1992). Personality evaluation (Thematic Apperception Test) reveals an inhibition of emotions and marked anxiety. In adolescents, a depressive reaction is more common, along with loneliness, low self-esteem, and feelings of inadequacy. Feeling the need to make up the time lost to illness and to live life to the fullest are common in adolescent patients. Sometimes feelings regarding the heart donor, or a sense of having a "double identity" (typical in adults), are evident.

The presurgical phase is particularly delicate. Sometimes children and adolescents are kept uninformed, and may experience a breakdown when they become aware of the impending surgical intervention. For those who are well-informed, the presurgical phase is generally marked by a stronger, at times hypomanic, attitude. Soon after the intervention, however, depressive or persecutory symptoms may emerge, when medically necessitated limitations become apparent (Pericchi, 1994). Conversely, there may be a manic reaction. Later, upon reentering normal life (e.g., school, physical activity), increased anxiety will be evident, a mix of fear and desire.

There are also psychological repercussions for the parents of these children and adolescents, such as anxiety over possible death and feelings of being in debt to the heart donor (Pericchi, 1994). The parents, and sometimes the siblings, often have to define a new relationship with the transplant patient.


Parents of children with congenital heart disease may have negative reactions, ranging from guilt and feelings of inadequacy to resentment, neglect, and even abuse, with possibly severe implications for intellectual and emotional development. Early mother-infant interactions may be characterized by depressive feelings, anxiety and fear of imminent death. Many mothers feel responsible for their child's illness, projecting onto the sick heart all their suffering and sorrow, which may activate unconscious aggressive feelings ("I hate you because your illness makes me suffer"), covered by overprotective behaviors (Rausch de Traubenberg, 1973).

The first reaction to the diagnosis is often shock, discouragement, and depression. A phase of struggle against the disease follows, such as active collaboration with the doctor, or conversely a denial of the illness. With chronic disease, a reorganization of the family system around the illness is common. The child or adolescent is perceived as extremely fragile, and must be protected from every external danger. Oppositional and irritable adolescents may increase indulgent attitudes or, on the contrary, produce anger and resentment on the part of parents.

Parents may blame themselves for the birth of a sick child (Glaser et al., 1964), and have different ways of relieving the guilt and feelings of inadequacy. Overprotection by anxious parents is one such method. A new pregnancy may reassure them of the capacity to give birth to a healthy child. Similarly, emotional overinvestment in another child is a way of confirming the capacity to be good parents.

Involvement by parents in the treatment is often useful. Learning about the nature of the disease, medications and their side effects, and constraints on daily activities is beneficial and may help the family feel closer. The amount of involvement must be evaluated case by case. Except for crisis situations, group meetings with parents are a good setting for discussing hopes, fears, and anxieties.

The effect on siblings has been described for many severe illnesses (Lavigne & Ryan, 1979). They may believe that the illness was brought about by their feelings or behavior toward the sick sibling, causing guilt. Younger children are particularly at risk for developing separation anxiety. Long-term consequences for adolescents can include overmaturation, as well as clear psychopathology (e.g., depression, hysterical or psychosomatic symptoms). Siblings of sick children have been found to be at increased risk for psychopathological disorders: 27% of the siblings of children with cardiac diseases had unspecified behavioral problems, 13% had psychosomatic disorders, and 24% had both (Aply et al., 1967).


The psychological implications of severe illness in childhood and adolescence, including congenital heart disease, must be understood by all medical staff. The psychosocial needs of these children and their families should not be neglected during the treatment process.

Specific psychological interventions must be implemented by child and adolescent psychiatrists and psychologists. These are particularly important during crises, such as while waiting for the surgical intervention or transplant, when a clinical condition worsens or, in the worst case, at the time surrounding the death of a child or adolescent.


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Paola Brovedani, Ph.D., Institute of Developmental Neurology, Psychiatry, and Educational Psychology, University of Pisa-Stella Maris Scientific Institute, Italy.
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Author:Masi, Gabriele; Brovedani, Paola
Date:Mar 22, 1999
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