Mitral Valve Prolapse and Psychiatric Complications: A Case Report C. Stavrakaki,' E. Williams2, A. Boisjoli,' P. Vlad,3 H. Chasse,2 Department of Family and Child, Ottawa General Hospital. 2 Department of Psychiatry, Royal Ottawa Hospital. 3 Department of Pediatrics, Children's Hospital of Eastern Ontario. Accepted: January 30, 1991
This case study describes a 9-year-old-girl presenting with symptoms of anxiety and depression who was found to have mitral valve prolapse syndrome. The relationship of mitral valve prolapse and anxiety and depression has been studied and a review of the literature is presented. The family history of heart and psychiatric problems is explored. The importance of the bio-psycho-social approach is stressed as well as the suggestion that mitral valve prolapse be included as part of the differential diagnosis for anxiety.
Keywords: Anxiety, Mitral Valve Prolapse, Child, Depression The history of mitral valve prolapse dates to 1871 when Da Costa described the "irritable heart." Over the years there have been case descriptions using different nomenclatures e.g., "soldier's heart and the effort syndromes," "anxiety neurosis," "ausculatory-electrocardiographic syndrome," "pseudo neurotic" and "prolapse of the mitral valve." The names reflected a cardiac problem and/or neurosis. Over the years diagnostic techniques have also changed. Wooley (1976) established a possible link between past diagnoses and the present diagnosis of mitral valve prolapse. The relationship between anxiety and mitral valve prolapse has received a great deal of attention from researchers. Some studies support a link between the two (Kantor et al 1980; Venkatesh et al 1980), whereas others do not (Hickey et al 1983; Mavissakalian et al 1983). What complicates the comparison of studies is that different criteria and different diagnostic methods are used in making a diagnosis of mitral valve prolapse. Studies have shown that criteria variance used by different cardiologists in examining the same test results greatly affected the diagnosis and therefore affected the results of mitral valve prolapse and anxiety studies (Dager et al 1988; Gorman et al 1981; 1986). Address reprint requests to: Esther Williams, Royal Ottawa Hospital, 1145 Carling Avenue, Ottawa, Ontario K I Z 7K4.
J Psychiatr Neurosci, VoL 16, No. 1, 1991
Mitral valve prolapse syndrome is characterized by a midsystolic prolapse of one of the mitral valve leaflets, with or without regurgitation. It is frequently associated with a midsystolic click and/or late systolic murmur. The diagnosis of this syndrome is sometimes difficult. Auscultatory abnormalities such as nonejection clicks, a late systolic murmur or both are not always audible. A majority of patients with mitral valve prolapse are asymptomatic (Davies and Montague 1989; Devereaux et al 1989; Gingell and Vlad 1978). Bisset et al (1980) found that only one of 119 children with mitral valve prolapse had negative ausculatory findings. In 19 patients of echo-confirmed mitral valve prolapse, Moodie (1982) found 68% had both a midsystolic click and a systolic murmur, 18% had only a murmur, 5% only a click, and 11 % were "silent." When ultrasound techniques such as M-mode echocardiography and two dimensional echocardiography are used, certain views or incorrect beam angulation may present a false picture (Gorman et al 1981; Hickey et al 1983). While Doppler echocardiographic techniques have been found to be very sensitive in detecting mitral regurgitation, a large proportion of presumably normal populations appear to experience trace regurgitation on Doppler examination, indicating the technique may be overly sensitive as a diagnostic tool. However, it is effective in assessing the severity of mitral insufficiency. The use of catecholamine activity, as measured by urinary levels of 3-methoxy-4 hydroxyphenylglycol (MHPG), has been studied and found not to be a useful marker (Dager
46
Journal of Psychiatry & Neuroscience
1988). Serum catecholamine levels have also been used to aid in diagnosis. Part of the problem may be that symptoms do not always become apparent until a later age. Davies and Montague (1989) contend that the presence of reproducibly audible mid-to-late systolic murmur, loudest at the apex and with characteristic changes in response to standing and squatting manoeuvres, establishes the diagnosis of mitral valve prolapse, especially in patients under 40. They note that isolated clicks at the cardiac apex have few causes other than mitral valve prolapse, and that a typical response of the clicks to the manoeuvres confirms the diagnosis. A combination of clinical exam including family history interview, echocardiographic findings and serum catecholamine levels could provide more accurate results. The lack of a "gold standard" by which to define mitral valve prolapse has been a problem in diagnosing this disorder. Many articles note a specific phenotype of a tall, slim female as a characteristic sign of possible mitral valve prolapse (Gingell and Vlad 1978). Bony abnormalities of the thorax such as pectus excavatum, scoliosis, or "straight back syndrome" are often seen in patients with mitral valve prolapse. Trying to understand the relationship between anxiety and mitral valve prolapse is further complicated because they share many of the same symptoms, e.g., heart palpitations, occasions of rapid heart beat or fluttering, anxiety, fear and dread, easy fatigability, hyperventilation, dyspnea, and chest discomfort (Jeresaty 1979; Spitzer and Williams 1987). Szmuilowicz and Flannery (1980) found that patients with primary mitral valve prolapse were more likely perceived by their doctors as being anxious or depressed than other groups. Both mitral valve prolapse and anxiety have been found to be more prevalent in females than males, and both have a familial prevalence and appear to be inherited in an autosomal dominant mode (Devereaux and Kramer-Fox 1989; Jeresaty 1979; Pini et al 1988). Mitral valve prolapse and panic disorder are often found in first degree female relatives (Crowe et al 1980; Devereaux and Kramer-Fox 1989). Some resolve the dilemma by noting that since anxiety is prevalent in the population and mitral valve prolapse is also prevalent in the population, it is not surprising to find them occurring together (Davis et al 1981). Mitral valve prolapse is the most frequently diagnosed valvular abnormality in the general population (Dager et al 1988). The Framingham study (Savage et al 1983) is the largest epidemiological study of cardiac risk factors in a population aged 20-80 years of age. It reported a 5 percent overall prevalence rate of mitral valve prolapse based on echocardiographic diagnostic techniques. The prevalence in males was 2 to 4 percent regardless of age. The prevalence in females was 17 percent in the 20-29 age group and declined with age to 1.4 percent among women over 80. In childhood and adolescence the prevalence of mitral valve prolapse is also approximately 1 percent. Panic disorder occurs in up to 10 percent of the population (Crowe et al 1980). Studies of prevalence of mitral valve prolapse and anxiety mostly study the link between mitral valve
VoL 16, No. 1, 1991
prolapse and panic disorder. Prevalence of mitral valve prolapse and anxiety in combination ranged from 0 - 59 percent, depending on the population studied, diagnostic techniques and criteria used (Dager et al 1988; Hickey et al 1983). Generally there was higher prevalence of mitral valve prolapse among patients with panic attacks as compared to patients with generalized anxiety disorder. A cause-effect relationship has also been postulated (Pariser et al 1978; Van Valkenberg 1986). It is hypothesized that some patients with a vulnerability of mitral valve prolapse as a result of anxiety induced stress or, possibly, the arrhythmias and palpitations felt by the patient with mitral valve prolapse, might make the patient anxious (Kantor 1980). Gorman et al (1981) speculate that the reason for a high incidence of mitral valve prolapse in panic disorder patients is that both are manifestations of a basic generalized disturbance of the autonomic nervous system. Casat (1987) intimates that a link between the two disorders may be found in a dysfunctional catecholaminergic or adrenergic system. Few case reports have been presented previously in professional journals which illustrate a link between psychiatric disturbance and mitral valve prolapse. Weinstein et al (1982) reported the case of a woman admitted to hospital with physical complaints similar to those found in a severe anxiety attack who upon physical exam and echocardiogram was found to have mitral valve prolapse syndrome. A psychiatric consult was also obtained and an MMPI administered. She was found to be depressed and anxious due to factors in her life situation. More recently, Casat et al (1987) published the case of a 12-year-oldgirl presenting with separation anxiety and found to have mitral valve prolapse and speculated on a common adrenergic dysfunction. While many studies discuss a link between anxiety and mitral valve prolapse, only two were found which link depression and mitral valve prolapse (Giannini et al 1984; Szmuilowicz and Flannery 1980). Stavrakaki and Vargo (1986) have reviewed many articles which discuss the relationship between anxiety and depression. There are very few studies which discuss mitral valve prolapse and anxiety and depression in children. The following case report profiles the youngest patient yet reported in the literature diagnosed with co-existing mitral valve prolapse and anxiety and depression in conjunction with mildly elevated serum catecholamines. CASE REPORT J.A. was not quite 9-years-old when her parents sought the help of a psychiatrist for school refusal. At this time, her mother was beginning to look for work outside the home and her father was travelling on business. After some supportive psychotherapy she returned to school. She saw the psychiatrist on a weekly basis for 5 months. Almost a year later, she complained of abdominal pain while in the library and was sent home from school. She began again
March 1991
Mitral Valve Prolapse and Psychiatric Complications
to refuse to attend school. The psychiatrist diagnosed school refusal with depression and prescribed imipramine 10 mg b.i.d. Two weeks later, her condition had worsened and the dosage was increased to 50 mg h.s. Her parents said this only made her more hyper. Once in school, she refused to attend class and refused to eat if forced to attend school. She lost 5-10 lbs. One day she told a police officer, visiting the school, of her suicidal intent. She was then taken immediately to the Emergency Department of the psychiatric hospital. She presented as a pale, thin little girl with little energy. She was tense and apprehensive and complaining of stomach aches and headaches. Verbal response was curt and monosyllabic. Her appetite was poor. Her physical examination revealed a heart rate of 140. The apical beat was forceful and the precordium was hyperdynamic. The blood pressure was 100/60. There was a high pitch systolic murmur over the mitral valve. Her ECG showed right axis deviation. Because of these abnormalities, antidepressants were stopped and she was referred for a complete cardiac evaluation. Before the cardiac evaluation, a short battery of self-report questionnaires were administered - the shortened version of the Piers-Harris Self Esteem Inventory (Friedman et al 1977/78), the Children's Depression Inventory (Kovacs 1981) and the Revised Children's Manifest Anxiety Scale (Reynolds and Richmond 1978). Results of the Piers-Harris showed good self-esteem. On the CDI, the items marked indicated feelings of anxiety rather than depression; she did not meet criteria for depression on the CDI (score > 19). On the RCMAS she scored in the above average range but did not meet criteria for anxiety. However, when subscales for physiological anxiety; worry/oversensitivity; social concerns/concentration and a lie subscale were scored, the most significant was an 87% score for the worry/oversensitivity subscale when compared with other girls the same age. This subscale has been found to be the most significant for distinguishing the anxiety group from those with other disorders (Mattison et al 1988). The items marked indicated symptoms of worry and fatigue which are found in both mitral valve prolapse syndrome and anxiety. These self-report instruments were not used as a diagnostic tool but as questionnaires that would provide additional information. Upon admission to a children's hospital for a cardiac evaluation, it was found that she had a rapid heart rate and a grade 3/6 late systolic murmur and multiple systolic clicks, heard best at the left sternal border and the apex. An echocardiogram was consistent with mitral valve prolapse. The cardiologist suspected the presence of catecholamine excess and felt that both her physical and psychiatric symptoms may be related to that. Serum catecholamine determinations eventually revealed a mild elevation in spite of the absence of high blood pressure. Epinephrine was 0.69 nmol/L (N = 0.11 - 0.52) and norepinephrine was 3.74 nmol/L (N = 1.27 - 2.8 1). During this examination, the patient told the cardiologist she had a 15 month history of episodic heart palpitations and other symptoms. She described a history of spontaneous
47
onset of a fast heart rate or the feeling that her heart was coming into her throat. This was at times combined with complaints of stomach aches, flushing, nausea, vertigo, hyperventilation and paraesthesia - all of varying intensity and in varying combinations. These were reported to occur 5 times a day. She said these episodes usually were brought on with activity and relieved after 5 to 10 minutes of rest. About the same time these symptoms appeared, she began refusing to attend school. It is interesting to note that these cardiac symptoms were revealed to no one until questioned by the pediatric cardiologist. Oral propranolol was begun at 10 mg t.i.d. and increased to 20 mg p.o. t.i.d. Tachycardia settled nicely and within a week the heart rate stablized to 80 beats per minute. With this treatment, her parents noted that she seemed less depressed and anxious. Holter monitoring and follow-up electrocardiograms showed inverted T-waves which reverted to normal on the inderal. While in hospital, she showed signs of separation anxiety. She had a few episodes of crying when family members left after a visit. There is a history of heart problems as well as a history of emotional problems on both sides of the family (Figure I - Genogram). In fact, the patient's mother has a less severe case of mitral valve prolapse and has had episodes of anxiety and depression since childhood. Every spring, the mother experiences seasonal depression. Coincidentally, spring has also been the time of year that J.A.'s school refusal has been the most severe. Figure 1
tralogy of Fallot
Sere Mtral Vale
Prolapse AnxIety & DepressIon
Identified Patient
History of cardiac and emotional problems in family of young girl with MVP, anxiety and depression J.A. was seen two weeks after her discharge from hospital.
She looked less tense and worried. She spoke easily and was generally more pleasant and cheerful. She had gained 6 lbs in two weeks, had rosy cheeks and a pulse of 78/ minute. She was less depressed and no suicidal intention was expressed. School attendance had resumed. She was continuing with propranolol and supportive psychotherapy. She made a remarkable recovery after diagnosis and proper treatment.
DISCUSSION The relationship of psychiatric problems and cardiac symptoms, as believed to relate to mitral valve prolapse, has been discussed in the literature for the past 100 years
48
Journal of Psychiatry & Neuroscience
and yet much remains to be learned. This case report illustrates the importance of considering the mental, physical and environmental aspects when formulating a diagnosis and treatment plan. In this case the first psychiatrist treated the problem as presented - school refusal. When this proved ineffective, a physical exam revealed a possible heart problem and a cardiac consult was requested. The cardiologist saw a problem with catecholamine excess associated with mitral valve prolapse and recognized the possibility that the psychiatric disorder was secondary to this. In fact, the patient also dichotomized her problems. She presented her mental status information to the psychiatrist and her physical complaints related to her heart to the cardiologist. It was only when a physical examination was done during the admission to the emergency room of a psychiatric hospital that the combination of physical and emotional disturbances were noted. This case study supports the suggestion of Shapiro and McFerran (1981) that mitral valve prolapse be included as part of the Diagnostic and Statistical Manual of Mental Disorders (DSM) differential diagnosis for anxiety, including "Anxiety in Childhood." They documented 14 cases (ages 10-55) of undiagnosed mitral valve prolapse and found a decrease in symptoms when diagnosis was confirmed. In this casejust presented, the change in this child was dramatic once the diagnosis of mitral valve prolapse with anxiety and depression was made and appropriate treatment initiated. Of great interest is the association of psychiatric disorders with high blood levels of catecholamines. This is a phenomenon also noted in a young child with phaeochromocytoma, another condition with over-production of adrenaline-like substances. (P. Vlad, personal communication November 22, 1989). In this case study, when catecholamine secretion was blocked with medication, behavior improved dramatically. This case illustrates how the mind and body are inextricably linked and the importance of treating the whole person. A thin girl who presents with symptoms of anxiety and family history of heart problems should be examined for possible mitral valve prolapse with appropriate referral to cardiology, if indicated, to confirm the severity of the problem. Even though both mitral valve prolapse and anxiety have a high prevalence in the general population, and both commonly occur in the same family, and both have similar symptoms, the treatment may not be the same. Mitral valve prolapse may require separate treatment which may favorably affect the anxiety symptoms.
REFERENCES Bisset GS, Schwartz DC, Meyer RA, James FW, Kaplan S (1980) Clinical spectrum and long term follow-up of isolated mitral valve prolapse in 1 19 children. Circulation 62:423-429.
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Casat CD, Ross BA, Scardina R, Sarno C, Smith KE (1987) Separation anxiety and mitral valve prolapse in a 12-yearold girl. J Am Acad Child Adolesc Psychiatry 26:(3)444446. Da Costa JM (1871) On irritable heart; a clinical study of a form of functional cardiac disorder and its consequences. Am J Med Sci 61:17-52. Dager SR, Saal AK, Comess KA, Dunner DL (1988) Mitral valve prolapse and the anxiety disorders. Hosp Community Psychiatry 39:(5)517-529. Davies NJ, Montague TJ (1989) Mitral valve prolapse: "The cardiac disease of the decade" revisited. Annuals RCPC 22:(5)307-310. Davis JM, Suhayl N, Spira N, Vogel BA (1981) Anxiety: Differential diagnosis and treatment from a biologic perspective. J Clin Psychiatry 42:( 11)Sec. 2:4-13. Devereaux RB, Kramer-Fox R (1989) Gender differences in mitral valve prolapse. Cardiovasc Clin 19:(3)243-258. Devereaux RB, Kramer-Fox R, Brown WT, Shear MK, Hartman N, Digfield P, Lutas EM, Spitzer MC, Litwin SD (1986) Relation between clinical features of the mitral prolapse syndrome and echocardiographically documented mitral valve prolapse. JAm Coil Cardiol8:(4)763772. Friedman RJ, Metzis S, Butler LF, Blanchard JP (1977/ 78) Development and Evaluation of School Based Assessment and Treatment Approaches for Depressed Children. Part Il. Ontario Institute for Studies in Education, Toronto. Giannini AJ, Price WA, Loiselle RH (1984) Prevalence of mitral valve prolapse in bipolar affective disorder. Am J Psychiatry 141:(8)991-992. Gingell RL, Vlad P (1978) Mitral valve prolapse. In: Heart Disease in Infancy and Childhood, Third Edition, Keith JD, Rowe RD, Vlad P (eds). New York: MacMillan Publishing Co., 810-827. Gorman JM, Fyer AF, Glicklick J, King DL, Klein DF (1981) Mitral valve prolapse and panic disorders: Effect of imipramine. In: Anxiety: New Research and Changing Concepts. Klein DF, Rabkin J (eds). New York: Raven Press, 317-326. Gorman JM, Shear MK, Devereaux RB, King DL, Klein DF (1986) Prevalence of mitral valve prolapse in panic disorder: Effect of echocardiographic criteria. Psychosom Med 48:(3-4)167-171. Hickey AJ, Andrews G, Wilcken D (1983) Independence of mitral valve prolapse and neurosis. Br Heart J 50:333336. Jeresaty RM (1979) Mitral Valve Prolapse. New York: Raven Press. Kantor JS, Zitrin CM, Zeldis SM (1980) Mitral valve prolapse syndrome in agoraphobic patients. Am J Psychiatry 137:(4)467-469. Kovacs M (1981) The Children's Depression Inventory, unpublished manuscript, University of Pittsburgh.
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Mattison RE, Bagnato SJ, Brubaker BH (1988) Diagnostic utility of the Revised Children's Manifest Anxiety Scale in children with DSM-III Anxiety Disorders. J Anxiety Disord 2:147-155. Mavissakalian M, Salerni R, Thompson ME, Michelson L (1983) Mitral valve prolapse and agoraphobia. Am J Psychiatry 140:(12)1612-1614. Moodie DS (1982) Mitral valve prolapse in children and adolescents. Cleve Clin Q 49:181 - 189. Pariser SF, Pinta ER, Jones BA (1978) Mitral valve prolapse syndrome and anxiety neurosis/panic disorder. Am J Psychiatry 135:(2)246-247. Pini R, Greppi B, Kramer-Fox R, Roman MJ, Devereux RB (1988) Mitral valve dimensions and motion and familial transmission of mitral valve prolapse with and without mitral valve billowing. J Am Coll Cardiol 12:(6)1423- 1431. Reynolds CR, Richmond BP (1978) What I think and feel: A revised measure of children's manifest anxiety. J Abnorm Child Psychol 6:(2)271-280.24. Savage DD, Garrison RJ, Devereaux RB, Castelli WP, Anderson SJ, Levy D, MacNamara PM, Stokes J III, Cannel WB, Feinleib M (1983) Mitral valve prolapse in the general population, I: epidemiological features: the Framingham study. Am Heart J 106:(3)571-576.
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Shapiro J, McFerran RJ (1981) Psychological aspects of mitral valve prolapse. Am Fam Physician 24:(4) 100-102. Spitzer RL, Williams JBW (1987) Diagnostic and Statistical Manual of Mental Disorders, third edition-revised, DSMIII-R, Washington D.C.: The American Psychiatric Association, 58-65, 235-254. Stavrakaki C, Vargo B (1986) The relationship of anxiety and depression: A review of the literature. Br J Psychiatry 149:7-16. Szmuilowicz J, Flannery JG (1980) Mitral valve prolapse syndrome and psychological disturbance. Psychosomatics 21:(5)419-421. Van Valkenburg C (1986) Anxiety symptoms In: The Medical Basis of Psychiatry. Winokur G, Clayton P (eds). Toronto: WB Saunders, 402-403. Venkatesh A, Pauls DL, Crowe R, Noyes R, Van Valkenberg C, Martins JB, Kerber RE (1980) Mitral valve prolapse in anxiety neurosis (panic disorder). Am Heart J 100:(3)302-305. Weinstein G, Allen G, Ford CV (1982) Anxiety and mitral valve prolapse syndrome. J Clin Psychiatry 43:(1)33-34. Wooley CF (1976) Where are the diseases of yesteryear? Circulation 53:(5)749-75 1.
This case study describes a 9-year-old-girl presenting with symptoms of anxiety and depression who was found to have mitral valve prolapse syndrome. The relationship of mitral valve prolapse and anxiety and depression has been studied and a review of the literature is presented. The family history of heart and psychiatric problems is explored. The importance of the bio-psycho-social approach is stressed as well as the suggestion that mitral valve prolapse be included as part of the differential diagnosis for anxiety.
Keywords: Anxiety, Mitral Valve Prolapse, Child, Depression The history of mitral valve prolapse dates to 1871 when Da Costa described the "irritable heart." Over the years there have been case descriptions using different nomenclatures e.g., "soldier's heart and the effort syndromes," "anxiety neurosis," "ausculatory-electrocardiographic syndrome," "pseudo neurotic" and "prolapse of the mitral valve." The names reflected a cardiac problem and/or neurosis. Over the years diagnostic techniques have also changed. Wooley (1976) established a possible link between past diagnoses and the present diagnosis of mitral valve prolapse. The relationship between anxiety and mitral valve prolapse has received a great deal of attention from researchers. Some studies support a link between the two (Kantor et al 1980; Venkatesh et al 1980), whereas others do not (Hickey et al 1983; Mavissakalian et al 1983). What complicates the comparison of studies is that different criteria and different diagnostic methods are used in making a diagnosis of mitral valve prolapse. Studies have shown that criteria variance used by different cardiologists in examining the same test results greatly affected the diagnosis and therefore affected the results of mitral valve prolapse and anxiety studies (Dager et al 1988; Gorman et al 1981; 1986). Address reprint requests to: Esther Williams, Royal Ottawa Hospital, 1145 Carling Avenue, Ottawa, Ontario K I Z 7K4.
J Psychiatr Neurosci, VoL 16, No. 1, 1991
Mitral valve prolapse syndrome is characterized by a midsystolic prolapse of one of the mitral valve leaflets, with or without regurgitation. It is frequently associated with a midsystolic click and/or late systolic murmur. The diagnosis of this syndrome is sometimes difficult. Auscultatory abnormalities such as nonejection clicks, a late systolic murmur or both are not always audible. A majority of patients with mitral valve prolapse are asymptomatic (Davies and Montague 1989; Devereaux et al 1989; Gingell and Vlad 1978). Bisset et al (1980) found that only one of 119 children with mitral valve prolapse had negative ausculatory findings. In 19 patients of echo-confirmed mitral valve prolapse, Moodie (1982) found 68% had both a midsystolic click and a systolic murmur, 18% had only a murmur, 5% only a click, and 11 % were "silent." When ultrasound techniques such as M-mode echocardiography and two dimensional echocardiography are used, certain views or incorrect beam angulation may present a false picture (Gorman et al 1981; Hickey et al 1983). While Doppler echocardiographic techniques have been found to be very sensitive in detecting mitral regurgitation, a large proportion of presumably normal populations appear to experience trace regurgitation on Doppler examination, indicating the technique may be overly sensitive as a diagnostic tool. However, it is effective in assessing the severity of mitral insufficiency. The use of catecholamine activity, as measured by urinary levels of 3-methoxy-4 hydroxyphenylglycol (MHPG), has been studied and found not to be a useful marker (Dager
46
Journal of Psychiatry & Neuroscience
1988). Serum catecholamine levels have also been used to aid in diagnosis. Part of the problem may be that symptoms do not always become apparent until a later age. Davies and Montague (1989) contend that the presence of reproducibly audible mid-to-late systolic murmur, loudest at the apex and with characteristic changes in response to standing and squatting manoeuvres, establishes the diagnosis of mitral valve prolapse, especially in patients under 40. They note that isolated clicks at the cardiac apex have few causes other than mitral valve prolapse, and that a typical response of the clicks to the manoeuvres confirms the diagnosis. A combination of clinical exam including family history interview, echocardiographic findings and serum catecholamine levels could provide more accurate results. The lack of a "gold standard" by which to define mitral valve prolapse has been a problem in diagnosing this disorder. Many articles note a specific phenotype of a tall, slim female as a characteristic sign of possible mitral valve prolapse (Gingell and Vlad 1978). Bony abnormalities of the thorax such as pectus excavatum, scoliosis, or "straight back syndrome" are often seen in patients with mitral valve prolapse. Trying to understand the relationship between anxiety and mitral valve prolapse is further complicated because they share many of the same symptoms, e.g., heart palpitations, occasions of rapid heart beat or fluttering, anxiety, fear and dread, easy fatigability, hyperventilation, dyspnea, and chest discomfort (Jeresaty 1979; Spitzer and Williams 1987). Szmuilowicz and Flannery (1980) found that patients with primary mitral valve prolapse were more likely perceived by their doctors as being anxious or depressed than other groups. Both mitral valve prolapse and anxiety have been found to be more prevalent in females than males, and both have a familial prevalence and appear to be inherited in an autosomal dominant mode (Devereaux and Kramer-Fox 1989; Jeresaty 1979; Pini et al 1988). Mitral valve prolapse and panic disorder are often found in first degree female relatives (Crowe et al 1980; Devereaux and Kramer-Fox 1989). Some resolve the dilemma by noting that since anxiety is prevalent in the population and mitral valve prolapse is also prevalent in the population, it is not surprising to find them occurring together (Davis et al 1981). Mitral valve prolapse is the most frequently diagnosed valvular abnormality in the general population (Dager et al 1988). The Framingham study (Savage et al 1983) is the largest epidemiological study of cardiac risk factors in a population aged 20-80 years of age. It reported a 5 percent overall prevalence rate of mitral valve prolapse based on echocardiographic diagnostic techniques. The prevalence in males was 2 to 4 percent regardless of age. The prevalence in females was 17 percent in the 20-29 age group and declined with age to 1.4 percent among women over 80. In childhood and adolescence the prevalence of mitral valve prolapse is also approximately 1 percent. Panic disorder occurs in up to 10 percent of the population (Crowe et al 1980). Studies of prevalence of mitral valve prolapse and anxiety mostly study the link between mitral valve
VoL 16, No. 1, 1991
prolapse and panic disorder. Prevalence of mitral valve prolapse and anxiety in combination ranged from 0 - 59 percent, depending on the population studied, diagnostic techniques and criteria used (Dager et al 1988; Hickey et al 1983). Generally there was higher prevalence of mitral valve prolapse among patients with panic attacks as compared to patients with generalized anxiety disorder. A cause-effect relationship has also been postulated (Pariser et al 1978; Van Valkenberg 1986). It is hypothesized that some patients with a vulnerability of mitral valve prolapse as a result of anxiety induced stress or, possibly, the arrhythmias and palpitations felt by the patient with mitral valve prolapse, might make the patient anxious (Kantor 1980). Gorman et al (1981) speculate that the reason for a high incidence of mitral valve prolapse in panic disorder patients is that both are manifestations of a basic generalized disturbance of the autonomic nervous system. Casat (1987) intimates that a link between the two disorders may be found in a dysfunctional catecholaminergic or adrenergic system. Few case reports have been presented previously in professional journals which illustrate a link between psychiatric disturbance and mitral valve prolapse. Weinstein et al (1982) reported the case of a woman admitted to hospital with physical complaints similar to those found in a severe anxiety attack who upon physical exam and echocardiogram was found to have mitral valve prolapse syndrome. A psychiatric consult was also obtained and an MMPI administered. She was found to be depressed and anxious due to factors in her life situation. More recently, Casat et al (1987) published the case of a 12-year-oldgirl presenting with separation anxiety and found to have mitral valve prolapse and speculated on a common adrenergic dysfunction. While many studies discuss a link between anxiety and mitral valve prolapse, only two were found which link depression and mitral valve prolapse (Giannini et al 1984; Szmuilowicz and Flannery 1980). Stavrakaki and Vargo (1986) have reviewed many articles which discuss the relationship between anxiety and depression. There are very few studies which discuss mitral valve prolapse and anxiety and depression in children. The following case report profiles the youngest patient yet reported in the literature diagnosed with co-existing mitral valve prolapse and anxiety and depression in conjunction with mildly elevated serum catecholamines. CASE REPORT J.A. was not quite 9-years-old when her parents sought the help of a psychiatrist for school refusal. At this time, her mother was beginning to look for work outside the home and her father was travelling on business. After some supportive psychotherapy she returned to school. She saw the psychiatrist on a weekly basis for 5 months. Almost a year later, she complained of abdominal pain while in the library and was sent home from school. She began again
March 1991
Mitral Valve Prolapse and Psychiatric Complications
to refuse to attend school. The psychiatrist diagnosed school refusal with depression and prescribed imipramine 10 mg b.i.d. Two weeks later, her condition had worsened and the dosage was increased to 50 mg h.s. Her parents said this only made her more hyper. Once in school, she refused to attend class and refused to eat if forced to attend school. She lost 5-10 lbs. One day she told a police officer, visiting the school, of her suicidal intent. She was then taken immediately to the Emergency Department of the psychiatric hospital. She presented as a pale, thin little girl with little energy. She was tense and apprehensive and complaining of stomach aches and headaches. Verbal response was curt and monosyllabic. Her appetite was poor. Her physical examination revealed a heart rate of 140. The apical beat was forceful and the precordium was hyperdynamic. The blood pressure was 100/60. There was a high pitch systolic murmur over the mitral valve. Her ECG showed right axis deviation. Because of these abnormalities, antidepressants were stopped and she was referred for a complete cardiac evaluation. Before the cardiac evaluation, a short battery of self-report questionnaires were administered - the shortened version of the Piers-Harris Self Esteem Inventory (Friedman et al 1977/78), the Children's Depression Inventory (Kovacs 1981) and the Revised Children's Manifest Anxiety Scale (Reynolds and Richmond 1978). Results of the Piers-Harris showed good self-esteem. On the CDI, the items marked indicated feelings of anxiety rather than depression; she did not meet criteria for depression on the CDI (score > 19). On the RCMAS she scored in the above average range but did not meet criteria for anxiety. However, when subscales for physiological anxiety; worry/oversensitivity; social concerns/concentration and a lie subscale were scored, the most significant was an 87% score for the worry/oversensitivity subscale when compared with other girls the same age. This subscale has been found to be the most significant for distinguishing the anxiety group from those with other disorders (Mattison et al 1988). The items marked indicated symptoms of worry and fatigue which are found in both mitral valve prolapse syndrome and anxiety. These self-report instruments were not used as a diagnostic tool but as questionnaires that would provide additional information. Upon admission to a children's hospital for a cardiac evaluation, it was found that she had a rapid heart rate and a grade 3/6 late systolic murmur and multiple systolic clicks, heard best at the left sternal border and the apex. An echocardiogram was consistent with mitral valve prolapse. The cardiologist suspected the presence of catecholamine excess and felt that both her physical and psychiatric symptoms may be related to that. Serum catecholamine determinations eventually revealed a mild elevation in spite of the absence of high blood pressure. Epinephrine was 0.69 nmol/L (N = 0.11 - 0.52) and norepinephrine was 3.74 nmol/L (N = 1.27 - 2.8 1). During this examination, the patient told the cardiologist she had a 15 month history of episodic heart palpitations and other symptoms. She described a history of spontaneous
47
onset of a fast heart rate or the feeling that her heart was coming into her throat. This was at times combined with complaints of stomach aches, flushing, nausea, vertigo, hyperventilation and paraesthesia - all of varying intensity and in varying combinations. These were reported to occur 5 times a day. She said these episodes usually were brought on with activity and relieved after 5 to 10 minutes of rest. About the same time these symptoms appeared, she began refusing to attend school. It is interesting to note that these cardiac symptoms were revealed to no one until questioned by the pediatric cardiologist. Oral propranolol was begun at 10 mg t.i.d. and increased to 20 mg p.o. t.i.d. Tachycardia settled nicely and within a week the heart rate stablized to 80 beats per minute. With this treatment, her parents noted that she seemed less depressed and anxious. Holter monitoring and follow-up electrocardiograms showed inverted T-waves which reverted to normal on the inderal. While in hospital, she showed signs of separation anxiety. She had a few episodes of crying when family members left after a visit. There is a history of heart problems as well as a history of emotional problems on both sides of the family (Figure I - Genogram). In fact, the patient's mother has a less severe case of mitral valve prolapse and has had episodes of anxiety and depression since childhood. Every spring, the mother experiences seasonal depression. Coincidentally, spring has also been the time of year that J.A.'s school refusal has been the most severe. Figure 1
tralogy of Fallot
Sere Mtral Vale
Prolapse AnxIety & DepressIon
Identified Patient
History of cardiac and emotional problems in family of young girl with MVP, anxiety and depression J.A. was seen two weeks after her discharge from hospital.
She looked less tense and worried. She spoke easily and was generally more pleasant and cheerful. She had gained 6 lbs in two weeks, had rosy cheeks and a pulse of 78/ minute. She was less depressed and no suicidal intention was expressed. School attendance had resumed. She was continuing with propranolol and supportive psychotherapy. She made a remarkable recovery after diagnosis and proper treatment.
DISCUSSION The relationship of psychiatric problems and cardiac symptoms, as believed to relate to mitral valve prolapse, has been discussed in the literature for the past 100 years
48
Journal of Psychiatry & Neuroscience
and yet much remains to be learned. This case report illustrates the importance of considering the mental, physical and environmental aspects when formulating a diagnosis and treatment plan. In this case the first psychiatrist treated the problem as presented - school refusal. When this proved ineffective, a physical exam revealed a possible heart problem and a cardiac consult was requested. The cardiologist saw a problem with catecholamine excess associated with mitral valve prolapse and recognized the possibility that the psychiatric disorder was secondary to this. In fact, the patient also dichotomized her problems. She presented her mental status information to the psychiatrist and her physical complaints related to her heart to the cardiologist. It was only when a physical examination was done during the admission to the emergency room of a psychiatric hospital that the combination of physical and emotional disturbances were noted. This case study supports the suggestion of Shapiro and McFerran (1981) that mitral valve prolapse be included as part of the Diagnostic and Statistical Manual of Mental Disorders (DSM) differential diagnosis for anxiety, including "Anxiety in Childhood." They documented 14 cases (ages 10-55) of undiagnosed mitral valve prolapse and found a decrease in symptoms when diagnosis was confirmed. In this casejust presented, the change in this child was dramatic once the diagnosis of mitral valve prolapse with anxiety and depression was made and appropriate treatment initiated. Of great interest is the association of psychiatric disorders with high blood levels of catecholamines. This is a phenomenon also noted in a young child with phaeochromocytoma, another condition with over-production of adrenaline-like substances. (P. Vlad, personal communication November 22, 1989). In this case study, when catecholamine secretion was blocked with medication, behavior improved dramatically. This case illustrates how the mind and body are inextricably linked and the importance of treating the whole person. A thin girl who presents with symptoms of anxiety and family history of heart problems should be examined for possible mitral valve prolapse with appropriate referral to cardiology, if indicated, to confirm the severity of the problem. Even though both mitral valve prolapse and anxiety have a high prevalence in the general population, and both commonly occur in the same family, and both have similar symptoms, the treatment may not be the same. Mitral valve prolapse may require separate treatment which may favorably affect the anxiety symptoms.
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