Complaints in Adolescence: Clinical Considerations

 

Cyanotic

Acyanotic

Type

•Tetralogy of Fallot

•Transposition of great arteries

•Total anomalous pulmonary venous return

•Truncus arteriosus

•Tricuspid atresia

•Hypoplastic left heart syndrome

•Patent ductus arteriosus

•Coarctation of the aorta

•Atrial septal defects

•Ventricular septal defects

•Aortic stenosis

•Pulmonary stenosis

Physical symptoms

•Bluish discoloration of the lips, fingers, and toes

•Breathing problems (e.g., dyspnea/hyperventilation)

•Anxiety

•Syncope

•Chest pain

•Reduced appetite

•Puffy eyes or face

•Fatigue

•Delay in physical growth

•Clinical signs are not always apparent

•Increased pulmonary blood flow

•Increased cardiac workload

•Fatigue

•Chest pain

•Shortness of breath upon exertion

Limitations

•Physical limitations depend on severity of the defect and clinical presentation of the adolescent with CHD and will vary from teen to teen, but are more likely in those with cyanotic lesions





Treatment


If a lesion is considered of sufficient severity or risk, surgical repair is the most common treatment for CHD (Hoffman et al., 2004). In 2000, 80 % of those who underwent surgery survived to adulthood. This dramatic increase in survival rates has resulted in increased need for care of adolescents and adults with CHD. In fact, in 2000, nearly half of those alive with CHD were adults (Marelli, Mackie, Ionescu-Ihu, Rahme, & Pilote, 2007). Unfortunately, although mortality rates are substantially lower following surgical intervention, one out of every five CHD survivors will need an additional operation. Arrhythmias resulting in hospitalization are also experienced by adolescents and adults with CHD. Somerville (1997) reported on common nonmedical reasons adolescents and adults with CHD contact their cardiologist, including such psychological difficulties as anxiety and suicidal ideation.

In addition to surgical correction, some adolescents and teens with CHD take medication to improve or maintain optimal heart functioning. Adolescents may be prescribed none or several medications listed below to help palliate the effects of CHD (The Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology, 2003). Angiotensin-converting enzyme (ACE) inhibitors may be prescribed to induce relaxation of blood vessels and thus decrease the vascular resistance that the heart pumps against (i.e., afterload reduction). Other medications to reduce the effort the heart muscle include beta-adrenergic blocking agents (beta-blockers), calcium channel blockers, and vasodilators. Antiarrhythmics are often prescribed to regulate the rhythm of the heart and prevent potentially lethal ventricular arrhythmias. Another medication that helps regulate rate and rhythm and that also improves the strength of the heart muscle is digitalis. Anticoagulants or blood thinners keep the blood from clotting and thus prevent blood clots which may travel and result in heart attack or stroke. These are sometimes used in patients with man-made shunts or that have low-velocity blood flow. Diuretics may be used to reduce swelling or edema due to excess pulmonary blood flow or poor cardiac function. Finally, antibiotics are regularly prescribed to prevent infection (specifically endocarditis).


Physical Experience of Disease


When asked about his experience with CHD, the 116-year-old said, “When I was younger I guess I thought it didn’t really make a difference because no one cared—I didn’t bother to tell them. But no I mean, it’s a big factor because everyone is so critical and judgmental. Having a heart problem makes me more of a loner and unpopular because I’m not physical and stuff like that” (Tong et al., 1998, p. 306). His experience was marked by the physical limitations of his heart defect. This physical limitation resulted in social exclusion. This evaluation of the effects of CHD is not an isolated experience, but a shared experience of many with CHD. In fact, in a qualitative study of the challenges teens face coping with CHD, illness management (via medication adherence or limiting physical activity) and social integration were among the six most frequently mentioned challenges of living with CHD (Tong et al., 1998). Other challenges included seeking independence, developing strategies for coping with uncertainty, determining to whom and when to disclose medical status, and seeking normality. In empirical quantitative research, physical capacity is a predictor of psychosocial functioning among adolescents with CHD (Spurkland, Bjornstad, Lindberg, & Seem, 1993).

Physical activity may negatively affect someone with CHD through hemodynamic effects, fluid depletion, blood pressure disturbance, tachycardia, hypertrophy of the cardiac muscle, and arrhythmias which may result in sudden death. Physical limitations depend on severity of CHD, the type of defect, the surgical repair, and the impact of the activity on cardiac hemodynamics. According to the task force on the management of grown-up CHD of the European Society of Cardiology, those with Marfan’s syndrome, with aortic anomalies, taking oral anticoagulants, or with pacemakers are asked not to participate in impact sports (2003). In addition, many persons with CHD are encouraged to participate in social/leisure exercise, but not competitive or impact sports.


Psychosocial Functioning


Having CHD undoubtedly has the potential to affect the psychosocial functioning of adolescents. Foster et al. (2001) developed a long list of the tasks these teens face, including coping with body image and physical activity limitations, seeking peer acceptance, dealing with stigma, managing anxiety about medical procedures, learning to manage intellectual or learning difficulties, becoming more independent and responsible for their health care, and forming appropriate educational and vocational goals. These challenges are likely contributors to the reduced functioning in a variety of domains found in adolescents with CHD.

Poor emotional functioning has been found in many studies of those with CHD (Alpern, Uzark, & Dick, 1989; Aurer, Senturia, Shopper, & Biddy, 1971; Barrett, Van der Feen, Spieth, Berul, & DeMaso, 2001; DeMaso, Twente, Spratt, & O’Brien, 1995; 2000; Fricchione & Vlay, 1986; Green & Levitt, 1962; Kovacs et al., 2009; Linde, Rasof, & Dunn, 1966; Spurkland et al., 1993; Todaro, Fennell, Sears, Rodrigue, & Roche, 2000). Behavioral difficulties (especially internalizing problems) have been noted in 27 % of children with surgical repair of CHD at birth (Majnemer et al., 2008). Among teens with CHD there were a significantly greater number of patients who report anxiety and depression on the Child Behavior Check List than the reference group (Utens, Verhulst, Meijboom, Duivenvoorden, & Hess, 1993). In one sample of young adults with CHD, the prevalence of those with clinically significant depression or anxiety was 36.4 %. A significant minority had clinically significant depression (27.3 %) and a smaller, but still significant, group had clinically significant anxiety (9.1 %; Bromberg, Beasley, D’Angelo, Landzberg, & DeMaso, 2003). Adjustment difficulties have also been noted in these children (DeMaso et al., 1991; DeMaso, Beardslee, Silbert, & Fyler, 1990; 1991). Finally, health-related quality of life (QoL) has also been found to be significantly lower in children and adolescents with CHD according to patient and parent report on both generic and disease-specific indices of QoL (Kamphuis et al., 2004; Spijkerboer et al., 2006). Some of the key risk factors for poor emotional adjustment include CNS impairment, poor family functioning, more complex lesions (DeMaso, 2004), and older age (Kardsorp, Evaraerd, Kindt, & Mulder, 2007). Unfortunately, despite the large quantity of research studies on emotional difficulties in children and adolescents with CHD, these studies suffer from problems such as small sample size, heterogeneity of measures, and little consistency regarding type of lesion or age (DeMaso, 2004).

In addition to emotional issues, children and adolescents may also experience difficulties relating with peers. Majnemer and associates (2008) noted significantly lower than normative socialization in 13.3 % of children with CHD. Children and adolescents with CHD are rated as more withdrawn than their same-aged peers by teachers. Among adolescents and adults (aged 15–30) with CHD surveyed, social impairment was found in many domains including school functioning (19 %) and during free time activities (15 %). In addition, adolescents with CHD reported expectations of social impairment in sports (26 %), future career (11 %), and family planning (10 %) (Fekkes et al., 2001). Level of family strain related to the illness appears to be an important factor that impacts social functioning (Casey, Sykes, Craig, Power, & Mulholland, 1996). Other risk factors include low self-esteem, high levels of depression, lower IQ or cognitive impairment (DeMaso, 2004; Youssef, 1988), physical limitations, and altered physical appearance (DeMaso, 2004). Altered physical appearance may include blue lips, clubbed fingers and toes, or moon face (DeMaso, 2004).


Family Functioning


Having a child with a chronic illness like CHD can change family functioning, which may also impact the child’s ability to cope with and manage chronic disease. This is especially true during the transition to adolescence, which causes strain even in families that do not have a chronically ill member. Overall, families of children and adolescents with CHD report concerns about normality, when to disclose information about CHD to others, how to manage a chronic illness, social integration, and the impact of CHD on family coping (Sparacino et al., 1997). These concerns have been found to negatively impact family functioning in families coping with a CHD diagnosis (Peterson & Harbaugh, 1995; Van Horn, DeMaso, Gonzalez-Heydrich, & Dahlmeier Erickson, 2001). Mothers are especially distressed, with studies showing difficulties with adjustment, anxiety, and ­depression (DeMaso et al., 1990, 1991, 1995; Thompson, Gustafson, George, & Spock, 1994). Mothers report additional concerns about medical prognosis, QoL, psychosocial functioning, effects on the family, and impact on finances (Van Horn et al., 2001). Maternal adjustment to these additional stressors has been linked with child adjustment, mother-reported child behavior problems, and child-reported physical symptoms (DeMaso et al., 1990, 1991, 1995; Thompson et al., 1994). Other studies have also found maternal adjustment to be negatively correlated with increased daily stressors and increased palliative coping (e.g., self-blame, avoidance, emotion-focused, and wishful thinking; Davis, Brown, Bakeman, & Campbell, 1998). Risk factors for reduced parental QoL include child posttraumatic stress, high impact of CHD on family life, lower socioeconomic status, and foreign nationality (Landolt, 2011). Fathers tend to be less studied, so conclusions about the relative impact on mothers vs. fathers should be considered tentatively.


Neurological Functioning


In a meta-analysis of the neurocognitive consequences of CHD, Miatton and associates (2006) noted that the majority of children and adolescents with CHD do not have significantly lower IQ scores than healthy controls. However, certain groups have been found to have significantly lowered IQ scores, specifically those with hypoplastic left heart syndrome or who have had surgery for CHD, which are also likely markers of relative disease severity. In school functioning, those with cyanotic lesions, especially transposition of the great arteries, have more difficulties in arithmetic, spelling, and reading (Linde et al., 1966; Silbert, Wolff, Mayer, Rosenthal, & Nadas, 1969). Additional meta-analyses revealed attentional difficulties in children and adolescents with transposition of the great arteries, tetralogy of Fallot, or ventricular septal defects (Miatton, DeWolf, Francois, & Thiery, 2006). Most adolescents with CHD have normative memory functioning. On the other hand, those with single ventricle defects have significantly lowered abilities on learning and memory tasks. Meta-analysis also examined language development studies and found that children and adolescents with transposition of the great arteries, staged palliation survivors, and ventricular septal defects exhibit a language delay of 2–4 months (Miatton et al., 2006; Majnemer et al., 2008). Overall, in tests of linguistic skills, these groups also perform below average. Finally, tests of motor functioning reveal that those with transposition of the great arteries experience fine motor disturbance, those with hypoplastic left heart syndrome have weaker visual-motor integration, and those who have had surgery for CHD exhibit poor locomotor skills compared to same-aged peers. These findings highlight the importance of understanding the specific disease state children and adolescents with CHD suffer from, as well as the interventions they have undergone as a result.

Additionally, some preoperative factors are noted to contribute to increased risk for neurocognitive deficits (Miatton et al., 2006). Malformations and deletions of the 22q11.2 chromosome, polymorphisms of the apolipoprotein E, and Down syndrome have all been shown as risk factors for worse neurological outcomes following surgery. Additionally, among persons with CHD, there is a greater prevalence of structural brain abnormalities, for example ventriculomegaly, cerebral atrophy, periventricular leukomalacia, poor brain growth due to hemodynamic disturbance, embolic infarction, cerebrovascular thrombosis, and abscess formation. New lesions and worsening of preexisting lesions is a significant risk for children and adolescents with complex congenital heart disease following surgical intervention. There is also a negative correlation between age at time of surgery and IQ scores (O’Dougherty, Wright, Garmezy, Loewenson, & Torres, 1983; O’Dougherty, Wright, Loewenson, & Torres, 1985). Further, specific surgical techniques such as use of deep hypothermic circulatory arrest and hemodilution have been shown to result in lowered cognitive abilities (Majnemer et al., 2008). Finally, there are several other postoperative factors to consider when determining risk for neurocognitive deficits in those who have had surgery for CHD. Having a greater number of operations, a longer stay in the intensive care unit, and being older at age of testing have all been shown to be correlated with greater neurocognitive dysfunction (Majnemer et al., 2008).



Arrhythmias and Electrophysiological Disorders of the Heart



Etiology


Although rare, a variety of arrhythmias of the heart may surface and persist in adolescence. The American Heart Association (AHA, 2011) describes an arrhythmia as any change in the normal sequence of electrical impulses of the heart. Arrhythmias can fall into two categories: bradycardia, a rhythm that is too slow, and tachycardia, a rhythm that is too fast (above 100 beats per minute). Arrhythmias can be described as benign or life-threatening, and individuals who experience them may be symptomatic or asymptomatic. Further, arrhythmias can be secondary to structural problems of the heart or they can be primary, deriving from a faulty cardiac electrical system.

Quite often, arrhythmias occur in the context of those who have had repairs of CHD, wherein the altered structure of the heart interferes with the electrical impulses and pathways. It is estimated that more than 50 % of pediatric patients who experience symptomatic ventricular arrhythmias have proof of organic heart disease (Davis, Gow, McCrindle, & Hamilton, 1996; Paul, Marchal, & Garson, 1990). Thus, these individuals are at risk for tachycardia, leading to palpitations, fainting, or sudden cardiac death.

Alternatively, those with no structural damage or repair of the heart can experience problems with the electrical system thereof. For clarity, these primary electrical conditions will be referred to in this chapter as “electrophysiological disorders.” Disorders in which ventricular arrhythmias are the hallmark complication in a structurally normal heart include long QT syndrome (LQTS; Moss & Robinson, 1992), Brugada syndrome (Antzelevitch et al., 2002), catecholaminergic polymorphic ventricular tachycardia (CPVT; Francis, Sankar, Nair, & Priori, 2005), and Wolff–Parkinson–White syndrome (WPW; Gallagher, Gilbert, Svenson, Sealy, Kasell, Wallace, 1975). The exact prevalence of these disorders in the overall population is unknown, but overall, they are rare. For instance, LQTS is one of the more well known of these diseases, and its estimated prevalence is approximately 1 in 5,000 (Tester, Will, Haglund, & Ackerman, 2006).

To understand the etiology of electrophysiological disorders, one must first understand the electrical impulses of the heart. An action potential creates the electrical impulse of the heart, and this impulse is influenced by the opening and closing of the calcium, potassium, and sodium channels. DNA variations in these ion channels are most commonly implicated in arrhythmias in structurally normal hearts (Noseworthy & Newton-Cheh, 2008). In most cases, the aforementioned electrophysiological disorders are hereditary in nature, and within the affected family, each child has a 50 % chance of inheriting the disorder (Schimpf, Veltmann, Wolpert, & Borggrefe, 2009). To complicate matters, these disorders can exist in silent carriers of the DNA mutation, and further, affected individuals may or may not be symptomatic (Schimpf, Veltmann, Wolpert, & Borggrefe, 2009). Therefore genetic testing is often encouraged when electrophysiological disorders are suspected.

The most significant implication of arrhythmia is the potential for sudden cardiac arrest (SCA), leading to sudden cardiac death. SCA is the sudden, unexpected death from a cardiac cause, which occurs in a short period of time (minutes to 1 h) after the onset of symptoms (Zipes & Wellens, 1998). In the United States, it is estimated that 300,000 lives are lost due to SCA every year (Zheng, Croft, Giles, & Mensah, 2001). The majority of cases, 80 % of SCAs, are related to underlying coronary heart disease, while fewer, 10–15 %, are accounted for by non-ischemic processes, such as cardiomyopathy. Only 5 % of SCA cases are thought to be due to primary electrophysiological disorders existing in a structurally normal heart (Huikuri, Castellanos, & Myerburg, 2001). Unfortunately, in this minority of diseases, SCA may be the first and only manifestation of the disease, resulting in death.

Fatal arrhythmias may make their first appearance during strenuous physical activity. This is particularly troubling for adolescence because demanding exercise is often prevalent in the form of competitive sports (Schimpf, Veltmann, Wolpert, & Borggrefe, 2009). This is not to say that sports are the cause of increased rates of SCA; rather research has demonstrated that sports have elicited sudden death in those young persons who were predisposed to life-threatening ventricular arrhythmias during physical exercise (Corrado, Basso, Rizzolo, Schiavon, & Thiene, 2003). Screening through use of a 12-lead EKG is beneficial for athletes who may be at risk of SCA; however, there is a great deal of heterogeneity in screening practices, lending to continued fatalities (Corrado et al., 2005).


Treatment


Electrophysiological disorders often cannot be cured, rather managed through medication, ablation, and therapy from an implantable cardioverter defibrillator. Medications for persons with electrophysiological disorders and those prone to arrhythmias include beta-blockade and other antiarrhythmic medications, which are designed respectively to slow the rate of the heart and to prevent arrhythmias (Aleong, Milan, & Ellinor, 2007). While beta-blockade has mild risk, antiarrhythmic medications can elicit serious side effects, such as exacerbation of ventricular tachycardia, which is known as a pro-arrhythmic effect (Ali, 2008). Consequently, in some cases, the treatment of the underlying disease can increase risk of SCA.

Radiofrequency catheter ablation is another treatment option for those with some types of life-threatening arrhythmias. This is a procedure in which a catheter tip is positioned over the area of the heart that is responsible for triggering the tachycardia. The tissue is then heated with the catheter, thereby burning the myocardium and creating a scar, to prevent an electrical current from passing through or originating from this tissue (AHA, 2011a). This approach provides more permanent protection from arrhythmias. When there is a discrete arrhythmogenic focus, a recent meta-analysis demonstrated that ablation is feasible and safe in pediatric patients, and the success and low recurrence rates of tachycardia were encouraging after this procedure (Tomaske, Candina, Weiss, & Bauersfeld, 2011). However, for some of the arrhythmias which are known as channelopathies, such as LQTS or Brugada syndrome, catheter ablation is ineffective because all the ventricular myocardium is involved, rather than a small defined area. However, ongoing evaluation of the effectiveness of ablation procedures is ongoing in adults and children.

Those with risk of arrhythmias or primary electrophysiological disorders are often treated with an implantable cardioverter defibrillator (ICD) for either prophylactic or secondary prevention of SCA. The ICD is a device that operates by monitoring potentially lethal electrical rhythms of the heart and terminating them with an immediate high-voltage shock. Several large-scale clinical trials have demonstrated the mortality benefit of the ICD over antiarrhythmic medication. All-cause mortality has been reduced by 23–30 % in patients who are treated with the ICD in addition to antiarrhythmic medication (Bardy et al., 2005; Moss et al., 2002). Criteria for implantation often include survival of SCA, syncope, significant family history of sudden death, and/or diminished ejection fraction (Aleong et al., 2007).


Physical Experience of the Disease


Individuals with electrophysiological disorders may present with or without symptoms. When the heart beats too slow (bradycardia), the affected individual is prone to fatigue, loss of oxygen to the body and the brain, and syncope (near and actual fainting). In the case of tachycardia, which is the hallmark symptom of electrophysiological disorders, the most common warning signs are palpitations, light-headedness, dizziness, dyspnea (shortness of breath), and syncope, possibly leading to SCA and death (Schimpf, Veltmann, Wolpert, & Borggrefe, 2009). Those who are asymptomatic experience the same underlying pathology, but have no awareness of such, presenting the possibility of syncope and SCA, seemingly without forewarning. In these individuals, their first symptom may be their last.

In those with electrophysiological disorders, symptoms can be triggered by a plethora of stimuli. They include, but are not limited to, the following: strenuous exercise, auditory stimuli, sudden bursts of activity, swimming, sudden changes in temperature, caffeine, certain medications, and/or during sleep patterns (Schimpf et al., 2009). Not only do these triggers vary by disorder but also by genetic variation of each disorder. The complexity of the disorders and their variants can make it difficult for adolescents and their parents to know which triggers to avoid, and may lead some parents to take measures to overprotect their child from such stimuli.

The most significant limitation that those with electrophysiological disorders face is restraint from sports activity. Risk stratification for sports participation is complex and depends on the child’s disorder and presentation (Schimpf et al., 2009). It is difficult for medical providers themselves to know how to make the most accurate recommendations regarding sports (Heidbuchel et al., 2006). Several studies have demonstrated accord that those with LQTS, Brugada syndrome, and CPVT should not perform competitive sports (Heidbuchel et al., 2006). There is much more variation in medical providers’ approach toward acceptable leisure-time activities, lending to more uncertainty about limitations and the possibility of overprotection, which may or may not be necessary for the adolescent.


Psychosocial Adjustment


Children and adolescents with arrhythmias often face psychosocial struggles to adjust to their disease and treatment. These adjustment difficulties are made complex not only by the child’s reactions to his/her limitations but also by the parental reactions of overprotection and anxiety. Due to the low incidence of electrophysiological disorders, there is a paucity of research in the coping processes of these individuals, especially adolescents. This section will primarily focus on studies regarding the adjustment of individuals with one of the most common electrophysiological diseases, which is LQTS.

Due to the threat of sudden death, those with or at risk for arrhythmias often struggle with anxiety. Few studies actually focus on the psychosocial adjustment of the child with the arrhythmias; however, one study compared the adjustment of children with LQTS (mean age 13) and children with asthma (mean age  =  11; Giuffre, Gupta, Crawford, & Leung, 2008). With use of standardized measures of anxiety and behaviors, these researchers illustrated that children with both asthma and LQTS had extensive fears, yet the fears varied between the medical conditions. The fears of children with asthma were the following: medical fears, fear of danger/death, and fear of minor injury, whereas those with LQTS experienced fear of failure and criticism, and they tended to keep feelings in while minimizing their true feelings of anxiety. Also, children with LQTS showed more internalizing problems than their counterparts with asthma. This suggests that adolescents with LQTS may experience fears related to their disorder, but find difficulty in sharing their anxiety openly.

A qualitative study that included adults with LQTS likewise demonstrated that anxiety and worry often surface in the psychosocial adjustment of this population (Anderson, Oyen, Bjorvatn, & Gjengedal, 2008). Through interviews, these researchers found that anxiety can be related to the high level of uncertainty about when/why symptoms surface and the relatively little known about LQTS by medical providers. In those who have experienced and survived manifestations of their illness, anxiety seems related to those specific cardiac events that threatened their lives. For instance, the youngest person in the study at hand, a 23-year-old female, made the following statement related to her worry, “It comes when I’m about to go to rest, then… Well, it’s not every night, but quite a few nights of the week that I go to bed at night thinking: What was it like that night? What was it like when I passed out?” (Anderson et al., 2008, p. 492).

Next, treatments for electrophysiological disorders can be equally as distressing as the cardiac condition itself. Often a medication regimen is required, which can unfortunately cause side effects including fatigue, depression, or even exacerbation of ventricular tachycardia. Further, electrophysiological disorders are often treated with the ICD, and while it is a life-saving device, coping with the possibility of intense electrical shock to the body can cause anxiety. This will be addressed in the section titled Special Consideration: The Experience of Adolescents with Implantable Cardioverter Defibrillators.


Family Functioning


A broader set of research studies have been conducted on parents’ adjustment to having a child with an electrophysiological disorder. Naturally, having a child or adolescent with a disorder that could suddenly claim his or her life with little warning due to SCA increases the anxiety of parents and family members. Researchers found a significant fear of their child’s death in parents of children with LQTS (Farnsworth, Fosyth, Haglund, & Ackerman, 2006). One parent made the comment, “When I walk into their rooms in the morning to wake them, I am always aware in the back of my mind… will they be cold?” (Farnsworth et al., 2006, p.287).

Family function is often consequently centered on reducing the risk of sudden death of a child. Parents find themselves taking on the role of educator since these conditions are rare and relatively new to the scientific community. In one qualitative study of parents with a child with LQTS, a parent reported, “I educated the doctor; I talk to the older doctors who have never heard of it [LQTS]” and “I went to the school and talked to the teachers and told them if he passes out to call 911” (Farnsworth et al., 2006, p. 287).

Families often use avoidance as a coping mechanism when a family member suffers from an electrophysiological disorder. Certain electrophysiological problems can be triggered by arousal and strenuous activity; henceforth, parents attempt to reduce exposure to such stimuli (Farnsworth et al., 2006). This may be especially difficult when diagnosis presents in the adolescent time period, as it often does, since a child must suddenly stop physical activities that he or she had engaged in most of his or her young life. Parents may become overprotective, limiting activities that may not be necessary to stop. This overprotection may be allayed by treatment with the implantable cardioverter defibrillator, to be discussed later in this chapter.


Cardiomyopathy



Etiology


Cardiomyopathy is a term for a diseased state of the heart, in which there is a structural abnormality in the ventricular muscle fibers. There are four main forms of the disease, including dilated and hypertrophic cardiomyopathy, the most common forms, and restrictive and arrhythmogenic right ventricular cardiomyopathy, which occur less frequently in children. For relevance to adolescents, the focus in this chapter will be on dilated and hypertrophic cardiomyopathy. In their vital facts resource, the Children’s Cardiomyopathy Foundation (2008) has compiled research on statistics of the disease. The prevalence of cardiomyopathy is estimated at one in every 100,000 children under the age of 18 in the United States, yet this is thought to be an underestimation since many undiagnosed children can die suddenly with SCA. The majority of the time, the disease is detected when children are under the age of 12 months; however, the second largest age range of detection is adolescents aged 12–18 years. Cardiomyopathy can be a primary inherited problem, or it can be acquired from secondary causes, such as infections, cancer chemotherapy, low blood flow to the heart, oxygen, or high blood pressure. However, in 75 % of cases of the disease, the cause is unknown.

Those with cardiomyopathy are highly susceptible to life-threatening arrhythmias and heart failure. Cardiomyopathy is the most common cause of sudden cardiac death (including trained athletes; Maron, 2003). Also, cardiomyopathy can lead to heart failure, a state in which the heart is unable to pump blood in a substantial enough way to meet the body’s needs for oxygen and nutrients. Heart failure is a progressive disease, meaning that once symptoms thereof present, they must be actively monitored and managed to prevent further episodes of decline.


Treatment


There is no cure for cardiomyopathy; rather treatment is aimed at the management of symptoms. Treatment varies dependent upon the type of cardiomyopathy. In general, however, disease management often involves medications, including diuretics, inotropic agents, afterload-reducing agents, and beta-blockers (AHA, 2011b). Respectively, these medications serve to decrease excess fluid in the lungs or other organs, to help the heart contract more effectively, to relax the arteries and allow the blood to flow with more ease to the body, and to slow the heartbeat and decrease the effort needed for contraction of the heart muscle. Also, depending on the subtype and severity, medication management may include anticoagulation therapy to reduce the risk of blood clots and antiarrhythmic medications to reduce episodes of tachycardia, which can lead to SCA.

When individuals with hypertrophic cardiomyopathy develop symptoms of heart failure, alcohol septal ablation (ASA) and septal myomectomy may be recommended. Both procedures are designed to reduce the impact of heart failure symptoms by decreasing obstruction to blood flow out of the heart; however, they are not cures for cardiomyopathy or heart failure. Recent research was conducted with a patient sample with hypertrophic cardiomyopathy and concurrent significant psychological distress and compromised well-being. Participants were evaluated pre- and post-ASA, and post-scores demonstrated reductions in distress, and improvement in well-being and disease severity (Serber, Sears, & Nielsen, 2007).

Other forms of cardiomyopathy management include pacemaker implantation and ICD technology. Because individuals with cardiomyopathy are prone to life-threatening arrhythmias, ICD therapy is beneficial for protection against SCA and sudden death. Further, recent pacemaker technology has been updated with the biventricular pacing function. Biventricular pacing is used often in cases of dilated cardiomyopathy and when cardiomyopathy has progressed to heart failure. The biventricular pacemaker involves pacing in both the right and left ventricles to allow them to contract together and improve synchrony. Biventricular pacing has shown promising long-term benefits in those implanted with this form of pacemaker (Linde et al., 2002).

In the most severe forms of cardiomyopathy, heart transplantation is necessary to improve survival and QoL. Cardiomyopathy is one of the leading causes of heart transplantation in youth (AHA, 2011b). Survival rates are promising. When pediatric age groups and diagnoses are combined in analysis, the 1-year survival rate is 75–85 %. At 5 years, the survival rate is 65–75 %, while long-term survival rates continue to be monitored and are forecast to improve with better rejection surveillance and medications (Morrow, 2000). Despite the promise of heart transplantation, the procedure is related to its own complications, such as infection, organ rejection, coronary artery disease, and the side effects of medications, thereby creating its own unique disease state.

If a heart transplant is not available in adequate timing for the adolescent, ventricular assist devices may be helpful. Ventricular assist devices are mechanical devices that assist in the pumping of the heart for short periods of time. However, these devices are not intended for long-term use, and they prove beneficial primarily in those cases wherein the device is used as a bridge to actual heart transplantation (Sharma et al., 2006).


Physical Experience of the Disease


Adolescents with cardiomyopathy can vary in presentation from having no or mild symptoms to the more severe state, wherein cardiomyopathy has caused congestive heart failure and the symptoms thereof. In some cases, an adolescent may have no evident symptoms of cardiomyopathy and suddenly develop a life-threatening arrhythmia, prompting abrupt and immediate diagnosis and intervention. In other cases, the affected individual may have the mild symptoms of decreased exercise capacity or becoming easily fatigued. In the more severe state, such as when cardiomyopathy becomes suddenly and rapidly evident as the result of a viral infection, heart failure develops. The symptoms of heart failure include difficulty breathing, fatigue with non-strenuous activities, coughing, swelling, pale color, decreased urine output, and excessive sweating; unfortunately, if not promptly diagnosed, some of these symptoms may be mistaken as asthma (AHA, 2011b) and effective treatment may be delayed.

Medications used to manage cardiomyopathy often result in multiple side effects. Depending on the type of medication, the following are some of the complaints that may arise: dizziness, fatigue, excessive bruising or bleeding from minor injury, and lowered blood pressure/heart rate (AHA, 2011b). Medication management is more intense if heart failure occurs or transplantation is needed, resulting in more doctors’ appointments and, possibly, dietary restrictions.

Unfortunately, those with cardiomyopathy have many physical restrictions. Due to the risk of SCA/death and increased heart failure, adolescents with both hypertrophic and dilated cardiomyopathy are not permitted to play competitive sports, as defined as organized team activity involving training (AHA, 2011b). Leisure-time and less strenuous activity restrictions should be tailored by the treating cardiologist due to varying degrees of cardiomyopathy.

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Mar 10, 2017 | Posted by in PSYCHOLOGY | Comments Off on Complaints in Adolescence: Clinical Considerations

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