- Email: [email protected]
Natural history of cardiac rhabdomyoma in infancy and childhood
Natural History of Cardiac Rhabdomyoma Infancy and Childhood
in
John F. Smythe, MD, John D. Dyck, MD, Jeffrey F. Smallhorn, MD, and Robert M. Freedom, MD
Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis is still considered to be poor and surgery continues to be indicated. The experience with rhabdomyoma diagnosed in live infants over a 20-year period was reviewed. Diagnosis by angiography or echocardiography was accepted only if multiple tumors were present or if tuberous sclerosis was also diagnosed. Nine patients (3 diagnosed prenatally and the remaining 6 at age <8 months) were identified as having a total of 24 tumors. Measurements in 2 planes demonstrated at least some evidence of regression in 24 patients (lOO%), with 20 of 24 having complete resolution. One patient required delayed surgery for excision of a subaortic ridge that appeared at the site of a resolved tumor. Our findings suggest that pediatric cardiac rhabdomyoma is most often a benign condition in which spontaneous regression is the rule. Surgery is recommended only for patients with refractory dysrhythmias or severe hemodynamic compromise. (Am J Cardiol 61990;66:1247-1
From the Divisions of &rdio!ogy and Pathology, The Hospital For Sick Children, Department of Medicine, University of Toronto, Toronto, Canada. Manuscript received January 26, 1990; revised manuscript received and accepted July 9, 1990. Address for reprints: John D. Dyck, MD, The Hospital For Sick Children, 555 University Avenue, Toronto, Ontario, Canada, M5G 1X8.
ardiac rhabdomyoma, while rare at all ages,is the most common primary cardiac tumor in infancy and childhood.’ This tumor first described in 18622has been demonstratedto have a clear association with the neurocutaneous syndrome known as tuberous sclerosis.3The majority of the reports dealing with this tumor are autopsy series.4,5The few reports of this tumor in live patients suggestthat the outlook is poor and that death within the first 5 years of life is usua1.6-8A number of investigators have therefore suggested that an aggressivesurgical approach to this tumor is indicated.5,g-11Recently, published reports have described spontaneous regression of cardiac rhabdomyomas.l U* To date, the natural history of this unusual cardiac problem remains unclear. We review our experienceover a XI-year period with cardiac rhabdomyoma diagnosed in living patients during infancy and childhood. ETHDDS
The hospital records, angiograms and 2-dimensional ultrasound evaluations of all patients entered cardiac record with the diagnosis of cardiac myoma during life, between the years 1969 and 1989, were reviewed. To be certain that all patients selected did indeed have this tumor the following criteria for acceptanceinto the study were established:demonstration of the presence of multiple intracardiac masses with characteristic echocardiographic or angiographic appearance,in the absenceof known malignant disease;or existence of X or more tumors in association with the neurocutaneous syndrome of tuberous sclerosis. These criteria were based on the information that fibromas and myxomas (other primary cardiac tumors occasionally seen in childhood) are invariably solitary tumors,t3 and on the very strong association between rhabdomyomas and tuberous sclerosis3J3 We believe that these criteria ensured that all patients entered into the study had rhabdomyomas exclusively. Angiograms and ultrasound evaluations were reviewed separately by 2 cardiologists with training in these imaging techniques. Note was made of tumor position in the casesof multiple tumors. Follow-up studies of the identified tumors were classified on the basis of size as demonstrating a given tumor to be (1) increased, (2) unchanged, (3) partially resolved, and (4) completely resolved.Tumors that did not undergo complete resolution had their areas in 2 planes measured with a Sony@Offline Ultrasound Analysis System to objectively confirm the observations of the cardiologists. In all
THE AMERICAN JOURNAL OF CARDIOLOGY
NOVEMBER 15, 1990
TABLE
I Patient Characteristics
and Tumor Outcome Tumor Location
Case
Sex/Age
Presentation
1
M/7 mo F/2 days M/3 mo F/2 days M/3 days F/prenat. M/prenat. F/l day F/prenat.
Arrhythmia Arrhythmia Murmur Murmur Murmur Screening U/S Arrhythmia Murmur FHxTS
2 3 4 5 6 7 8 9
Regression denotes complete resolution. FH = family history; IVS = interventricularseptum: TS = tuberous sclerosis: U/S = ultrasound.
LV = left ventricle:
RV
RA
LV
IVS
Follow-Up (regression)
Status (clinical)
l/l 1
l/l
Alive/TS Alive/TS Alive/TS Alive/TS Alive Alive/TS Alive/TS Sub AS-Sx Alive/TS
1 1
1 1 2
prenat.
2
= prenatal:
casesthere was complete concordance between the observations of the 2 cardiologists and between the cardiologists and the analysis system, In an effort to determine if the true incidence of rhabdomyoma in infancy and childhood was changing, a review of autopsy records at the Hospital for Sick Children was conducted for 2 consecutive periods. The number of caseshaving this tumor identified at autopsy between 1919 and 1969 was compared with the number identified between 1969 and 1989. The number for each period was compared with the total number of patients seen at the Hospital for Sick Children, Toronto, over the same time periods. RESULTS Eleven patients (6 men and 5 women) who fulfilled the diagnostic criteria were identified over the 20-year period from January 1969 to January 1989. Of these, 1 patient died at 1 day of age of unrelated anomalies. Nine of the remaining 10 have been followed for at least 1 year and constitute our study population. Seven
1
2/2
1 1 2 2 2 1 RA = right atrium:
l/l 1 2
l/2
1
4/5 5/5 4/5
1
l/2
RV = right ventricle:
Sub As-% 91subaortic
stenosis-surgical
resection:
patients (78%) were followed with the diagnosis of tuberous sclerosis.In 3 patients the initial diagnostic study was angiographic, with all other studies being echocardiographic. Table I summarizes the pertinent clinical data and investigations on all patients. Three patients were initially identified at the time of fetal cardiac ultrasound examination (Table I). These examinations had been performed because of fetal tachycardia, family history of tuberous sclerosis,and an abnormality detected on a level 2 obstetric ultrasound evaluation. Among the remaining 6 patients detected after birth, 2 presented with dysrhythmias, 2 with asymptomatic cardiac murmurs and 2 with cardiac murmurs in the setting of tuberous sclerosis. Mean period of follow-up was 7 years (range 1.9 to 15.3). Spontaneous regression,either complete or partial, was observedin all cases.Figure 1 is a representative example (case5). Of a total of 24 tumors identified, 20 underwent complete regression,and 3 partial regression, which by area measurement exceeded50%. One tumor (Table I, case9) at 2 years of follow-up had decreasedin size by only 30% according to area measurements. One patient (case 8) developeda significant subaortic fibrous diaphragm at the precise site of a resolved rhabdomyoma. The surgical resection specimen failed to demonstrate any evidenceof residual tumor. None of the other patients required surgical intervention and all are currently alive without cardiac symptomatology. Between 1919 and 1969 a total number of 6 casesof rhabdomyoma were diagnosedat autopsy, whereasbetween 1969 and 1989 a single case was diagnosed at autopsy. The incidence of cardiac rhabdomyoma has therefore not undergone a significant change between these 2 time intervals. DISCUSSION
FIGURE 1. A and 6, long-axis and 4-chamber views, respectively, of patient 5 at age 16 months. C and D, same patient at 57 months. A0 = am LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle; Turn = tumor.
1248
THE AMERICAN JOURNAL OF CARDIOLOGY
VOLUME 66
Histologic proof of the diagnosisof rhabdomyoma is no longer considered necessaryin the presenceof characteristic echocardiographic findings.14Rhabdomyomas are usually multiple and frequently lobulated. They arise most often in the interventricular septum but some 30% will have atria1 wall involvement.4These features plus the strong associationwith tuberous sclerosisfacilitate noninvasive diagnosis.l4 The advent and widespread
use of 2-dimensional echocardiography8,14,16 (supplant- clearly advisable in the absence of life-threatening ing angiography as the principal diagnostic method) symptoms. and, more recently, the broad application of the ultrasound technique to screening of fetal cardiac anatom l&l6 had undoubtedly led to the earlier and more frequent diagnosis of cardiac rhabdomyoma in asymp- REFERENCES tomatic cases.Certainly, the casesreported here were 1. Nadas A, Ellison R. Cardiac tumours in infancy. Am J Cardioi 1968;21:363366. far lesssymptomatic at the time of diagnosis than those 2. Von Recklinghausen F. Verhandlungen der Gesellschaft fur Geburtshulfe reported by others.‘JjT*Review of our records and autop- Herr V. Recklinghausen legt der Gesellschaft ein Herz voneinen Neugeborenen. Geburtsk 1862;20:1-2. sy material suggeststhat the true incidence of cardiac Monatsschr 3. Shaher R, Mintzer J, Farina M, Alley R, Bishop M. Clinical presentation of rhabdomyoma is probably not changing but that the de- rhabdomyoma of the heart of infancy and childhood. Am J Cardiol1972;30:95tection rate for this tumor must be increasing. It is only 103. J, McAllister H, Ferrans V. Cardiac rhabdomyoma: a clinicopathorecently, therefore, that an accurate appraisal of the 4.logicFenogiio and electron microscopic study. Am J Cardiol 1976;38:241-25 1. natural history of this lesion can be made. The value of 5. Arciniegas E, Hakimi M, Farooki I, Truccone N, Green E. Primary cardiac magnetic resonance imaging in assessingintracardiac tumors in children. J Thorac Cardiouasc Surg 198&79:5X2-591. Simcha A, Wells B, Tynan M, Wateston, DJ. Primary cardiac tumours in masseshas been reported,17and this method may also 6.childhood. Arch Dis Child 1971;46:508-514. assistin charting the course of these lesions. 7. Bloor CM. Cardiac Pathology. Philadelphia: Lippincott, 1974;400. S. Bini R, Westaby S, Bargeron L, Pacifico A, Kirklin J. Investigation and Symptoms resulting from cardiac rhabdomyomas management of primary cardiac tumors in infants and children. J Am Co/l are largely a consequenceof tumor size or location Cardiol 1983;2:351-7. within the heart. Inflow or outflow tract obstruction 9. Homer S, Forbes N, Stewart S. Rhabdomyoma of the heart: a diagnostic and challenge. Ann Thorac Surg 1980;29:373-377. may lead to myocardial dysfunction with resultant con- therapeutic 10. Reecc I, Co&y D, Frazier 0, Hallman G, Powers P, Mantero C. Cardiac gestion or cyanosis. Refractory cardiac dysrhythmias Tumors. J Thorac Cardiovasc Surg 1984;88:439-446. and systemic embolization are other events that will II. Khattar H, Guerin R, Fouron J, Stanley P, Kratz C, Davignon A. Les cardiaques chez I’enfant. Rapport de 3 observations avec evolution sponbring patients to medical attention. It is apparent that tumeurs tanement favorable. Arch Ma1 Coeur 1975;68:419-429. symptomatic patients may have a poor outlook without 12. Isnard-Baladi J, Iselin M, Veneziea R. Potier J, Focault J. Tumeurs cardia revelation neonatate. Arch Mrd Coeur 1985;78:785-789. intervention, which has prompted the advocacy of early aques 13. McAllister H, Fenoglio J. Tumors of the cardiovascular system. Atlas of and aggressivesurgery by some workers.5+‘1 Tumour Pathology, vol 15. Washington D.C., Armed Forces Institute of PatholoIn 1975, spontaneousregressionof a cardiac rhabdo- gy, 1978:25-31. 14. Alkalay A, Ferry D, Lin 8, Fink B, Pomerance J. Spontaneous regression of myoma was reported.lI Subsequently, others have advo- cardiac rhabdomycnna in tuberous sclerosis. Clin Ped 1987;26:532-535. cated an expectant approach to management.I4 Never- 15. Marx G, Bicrman F, Matthews H, Williams R. Two dimensional echocardiotheless,the strength of the casefor or against a%ressive graphic diagnosis of intracardiac masses in infancy. J Am Coil Cwdiol management remains unresolved. Our study certainly 1984;3:827-832. 16. Gresser C, Shime J, Rakowski H, Smallhorn J, Hui A, Berg J. Fetal cardiac demonstratesthat spontaneoustumor regressionis over- tumor: a prenatal echocardiographic marker for tuberous sclerosis. Am J Obstet 1987;156:689-690. whelmingly the rule and that in the absence of life- Gynecol 17. Amparo E, Higgins C, Farmer D, Gamsu G, McNamara M. Gated MRI of threatening complications conservative management is cardiac and paracardiac masses. AJR 1984;143:1151-1156. 18. Smith H, Watson G, Pate1 R, Super M. Cardiac rhabdomyomata in tuberous probably indicated. their course and diagnostic value. Arch Dis Child 1989;64:196-200, A review of published reports revealed 15 casesof sclerosis: 1% Foster E, Spooner E, Farina M, Shaher R, Alley R. Cardiac rhabdomyoma in rhabdomyoma with at least some evidence of tumor re- the neonate: surgical management. Ann Thorac Surg 1984;37:249-253. gression.‘,l2,14,18-25 N evertheless,the question of wheth- 20. Gillor A, Stock 6, Schuster D. Diagnostik und Behandlung der Kardialen Rhabdomyome. Monatsschr Kinderheilkd 1986;134:445-449. er aggressivesurgical management is indicated in all 21. Stijns M, Lintermans J, Tremouroux M, Vliers A. Spontaneous disappearcases remains unresolved. Our study documents that ance of aortic subvalvar obstruction in young infants with tuberous sclerosis. Cardiol 1982;3:88-89. with a large m.umberof patients followed in a single Pediatr 22. Casta A. Tuberous sclerosis and Wolff-Parkinson-White syndrome. J Pediatr center, cardiac rhabdomyoma, while occasionally life- 1986;109:399. threatening, is increasingly diagnosed in patients with 23. Journel H, Rousscy M, Plais M, Milan J, Almange C, LeMarec B. Prenatal of famililial tuberous sclerosis following detection of cardiac rhabdofew or no symptoms.This suggeststhat the tumor may diagnosis myoma by ultrasound. Prenat Diagn 1986;6:283-289. in fact have a greater incidence than previously appreci- 24. Pernot C, Hoeffel JC., Touche M, Worms A. Les tumeurs primitives du cower ated. Furthermore, spontaneousregression of most, if spontanement regressives chez i’enfant. A propos d’une observation. Coeur 1979;10:303-307. not all, tumors over several years can be anticipated, to 25. Ross RD, Paridon SE, Humes RA, Karpawich PP, Pinsky. Spontaneous the extent that an expectant approach to managementis regression of cardiac rhabdomyomas. Am J Cardio/ 1989,64:416.
THE AMERICAN JOURNAL OF CARDIOLOGY
NOVEMBER 15, 1990
1249
in
John F. Smythe, MD, John D. Dyck, MD, Jeffrey F. Smallhorn, MD, and Robert M. Freedom, MD
Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis is still considered to be poor and surgery continues to be indicated. The experience with rhabdomyoma diagnosed in live infants over a 20-year period was reviewed. Diagnosis by angiography or echocardiography was accepted only if multiple tumors were present or if tuberous sclerosis was also diagnosed. Nine patients (3 diagnosed prenatally and the remaining 6 at age <8 months) were identified as having a total of 24 tumors. Measurements in 2 planes demonstrated at least some evidence of regression in 24 patients (lOO%), with 20 of 24 having complete resolution. One patient required delayed surgery for excision of a subaortic ridge that appeared at the site of a resolved tumor. Our findings suggest that pediatric cardiac rhabdomyoma is most often a benign condition in which spontaneous regression is the rule. Surgery is recommended only for patients with refractory dysrhythmias or severe hemodynamic compromise. (Am J Cardiol 61990;66:1247-1
From the Divisions of &rdio!ogy and Pathology, The Hospital For Sick Children, Department of Medicine, University of Toronto, Toronto, Canada. Manuscript received January 26, 1990; revised manuscript received and accepted July 9, 1990. Address for reprints: John D. Dyck, MD, The Hospital For Sick Children, 555 University Avenue, Toronto, Ontario, Canada, M5G 1X8.
ardiac rhabdomyoma, while rare at all ages,is the most common primary cardiac tumor in infancy and childhood.’ This tumor first described in 18622has been demonstratedto have a clear association with the neurocutaneous syndrome known as tuberous sclerosis.3The majority of the reports dealing with this tumor are autopsy series.4,5The few reports of this tumor in live patients suggestthat the outlook is poor and that death within the first 5 years of life is usua1.6-8A number of investigators have therefore suggested that an aggressivesurgical approach to this tumor is indicated.5,g-11Recently, published reports have described spontaneous regression of cardiac rhabdomyomas.l U* To date, the natural history of this unusual cardiac problem remains unclear. We review our experienceover a XI-year period with cardiac rhabdomyoma diagnosed in living patients during infancy and childhood. ETHDDS
The hospital records, angiograms and 2-dimensional ultrasound evaluations of all patients entered cardiac record with the diagnosis of cardiac myoma during life, between the years 1969 and 1989, were reviewed. To be certain that all patients selected did indeed have this tumor the following criteria for acceptanceinto the study were established:demonstration of the presence of multiple intracardiac masses with characteristic echocardiographic or angiographic appearance,in the absenceof known malignant disease;or existence of X or more tumors in association with the neurocutaneous syndrome of tuberous sclerosis. These criteria were based on the information that fibromas and myxomas (other primary cardiac tumors occasionally seen in childhood) are invariably solitary tumors,t3 and on the very strong association between rhabdomyomas and tuberous sclerosis3J3 We believe that these criteria ensured that all patients entered into the study had rhabdomyomas exclusively. Angiograms and ultrasound evaluations were reviewed separately by 2 cardiologists with training in these imaging techniques. Note was made of tumor position in the casesof multiple tumors. Follow-up studies of the identified tumors were classified on the basis of size as demonstrating a given tumor to be (1) increased, (2) unchanged, (3) partially resolved, and (4) completely resolved.Tumors that did not undergo complete resolution had their areas in 2 planes measured with a Sony@Offline Ultrasound Analysis System to objectively confirm the observations of the cardiologists. In all
THE AMERICAN JOURNAL OF CARDIOLOGY
NOVEMBER 15, 1990
TABLE
I Patient Characteristics
and Tumor Outcome Tumor Location
Case
Sex/Age
Presentation
1
M/7 mo F/2 days M/3 mo F/2 days M/3 days F/prenat. M/prenat. F/l day F/prenat.
Arrhythmia Arrhythmia Murmur Murmur Murmur Screening U/S Arrhythmia Murmur FHxTS
2 3 4 5 6 7 8 9
Regression denotes complete resolution. FH = family history; IVS = interventricularseptum: TS = tuberous sclerosis: U/S = ultrasound.
LV = left ventricle:
RV
RA
LV
IVS
Follow-Up (regression)
Status (clinical)
l/l 1
l/l
Alive/TS Alive/TS Alive/TS Alive/TS Alive Alive/TS Alive/TS Sub AS-Sx Alive/TS
1 1
1 1 2
prenat.
2
= prenatal:
casesthere was complete concordance between the observations of the 2 cardiologists and between the cardiologists and the analysis system, In an effort to determine if the true incidence of rhabdomyoma in infancy and childhood was changing, a review of autopsy records at the Hospital for Sick Children was conducted for 2 consecutive periods. The number of caseshaving this tumor identified at autopsy between 1919 and 1969 was compared with the number identified between 1969 and 1989. The number for each period was compared with the total number of patients seen at the Hospital for Sick Children, Toronto, over the same time periods. RESULTS Eleven patients (6 men and 5 women) who fulfilled the diagnostic criteria were identified over the 20-year period from January 1969 to January 1989. Of these, 1 patient died at 1 day of age of unrelated anomalies. Nine of the remaining 10 have been followed for at least 1 year and constitute our study population. Seven
1
2/2
1 1 2 2 2 1 RA = right atrium:
l/l 1 2
l/2
1
4/5 5/5 4/5
1
l/2
RV = right ventricle:
Sub As-% 91subaortic
stenosis-surgical
resection:
patients (78%) were followed with the diagnosis of tuberous sclerosis.In 3 patients the initial diagnostic study was angiographic, with all other studies being echocardiographic. Table I summarizes the pertinent clinical data and investigations on all patients. Three patients were initially identified at the time of fetal cardiac ultrasound examination (Table I). These examinations had been performed because of fetal tachycardia, family history of tuberous sclerosis,and an abnormality detected on a level 2 obstetric ultrasound evaluation. Among the remaining 6 patients detected after birth, 2 presented with dysrhythmias, 2 with asymptomatic cardiac murmurs and 2 with cardiac murmurs in the setting of tuberous sclerosis. Mean period of follow-up was 7 years (range 1.9 to 15.3). Spontaneous regression,either complete or partial, was observedin all cases.Figure 1 is a representative example (case5). Of a total of 24 tumors identified, 20 underwent complete regression,and 3 partial regression, which by area measurement exceeded50%. One tumor (Table I, case9) at 2 years of follow-up had decreasedin size by only 30% according to area measurements. One patient (case 8) developeda significant subaortic fibrous diaphragm at the precise site of a resolved rhabdomyoma. The surgical resection specimen failed to demonstrate any evidenceof residual tumor. None of the other patients required surgical intervention and all are currently alive without cardiac symptomatology. Between 1919 and 1969 a total number of 6 casesof rhabdomyoma were diagnosedat autopsy, whereasbetween 1969 and 1989 a single case was diagnosed at autopsy. The incidence of cardiac rhabdomyoma has therefore not undergone a significant change between these 2 time intervals. DISCUSSION
FIGURE 1. A and 6, long-axis and 4-chamber views, respectively, of patient 5 at age 16 months. C and D, same patient at 57 months. A0 = am LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle; Turn = tumor.
1248
THE AMERICAN JOURNAL OF CARDIOLOGY
VOLUME 66
Histologic proof of the diagnosisof rhabdomyoma is no longer considered necessaryin the presenceof characteristic echocardiographic findings.14Rhabdomyomas are usually multiple and frequently lobulated. They arise most often in the interventricular septum but some 30% will have atria1 wall involvement.4These features plus the strong associationwith tuberous sclerosisfacilitate noninvasive diagnosis.l4 The advent and widespread
use of 2-dimensional echocardiography8,14,16 (supplant- clearly advisable in the absence of life-threatening ing angiography as the principal diagnostic method) symptoms. and, more recently, the broad application of the ultrasound technique to screening of fetal cardiac anatom l&l6 had undoubtedly led to the earlier and more frequent diagnosis of cardiac rhabdomyoma in asymp- REFERENCES tomatic cases.Certainly, the casesreported here were 1. Nadas A, Ellison R. Cardiac tumours in infancy. Am J Cardioi 1968;21:363366. far lesssymptomatic at the time of diagnosis than those 2. Von Recklinghausen F. Verhandlungen der Gesellschaft fur Geburtshulfe reported by others.‘JjT*Review of our records and autop- Herr V. Recklinghausen legt der Gesellschaft ein Herz voneinen Neugeborenen. Geburtsk 1862;20:1-2. sy material suggeststhat the true incidence of cardiac Monatsschr 3. Shaher R, Mintzer J, Farina M, Alley R, Bishop M. Clinical presentation of rhabdomyoma is probably not changing but that the de- rhabdomyoma of the heart of infancy and childhood. Am J Cardiol1972;30:95tection rate for this tumor must be increasing. It is only 103. J, McAllister H, Ferrans V. Cardiac rhabdomyoma: a clinicopathorecently, therefore, that an accurate appraisal of the 4.logicFenogiio and electron microscopic study. Am J Cardiol 1976;38:241-25 1. natural history of this lesion can be made. The value of 5. Arciniegas E, Hakimi M, Farooki I, Truccone N, Green E. Primary cardiac magnetic resonance imaging in assessingintracardiac tumors in children. J Thorac Cardiouasc Surg 198&79:5X2-591. Simcha A, Wells B, Tynan M, Wateston, DJ. Primary cardiac tumours in masseshas been reported,17and this method may also 6.childhood. Arch Dis Child 1971;46:508-514. assistin charting the course of these lesions. 7. Bloor CM. Cardiac Pathology. Philadelphia: Lippincott, 1974;400. S. Bini R, Westaby S, Bargeron L, Pacifico A, Kirklin J. Investigation and Symptoms resulting from cardiac rhabdomyomas management of primary cardiac tumors in infants and children. J Am Co/l are largely a consequenceof tumor size or location Cardiol 1983;2:351-7. within the heart. Inflow or outflow tract obstruction 9. Homer S, Forbes N, Stewart S. Rhabdomyoma of the heart: a diagnostic and challenge. Ann Thorac Surg 1980;29:373-377. may lead to myocardial dysfunction with resultant con- therapeutic 10. Reecc I, Co&y D, Frazier 0, Hallman G, Powers P, Mantero C. Cardiac gestion or cyanosis. Refractory cardiac dysrhythmias Tumors. J Thorac Cardiovasc Surg 1984;88:439-446. and systemic embolization are other events that will II. Khattar H, Guerin R, Fouron J, Stanley P, Kratz C, Davignon A. Les cardiaques chez I’enfant. Rapport de 3 observations avec evolution sponbring patients to medical attention. It is apparent that tumeurs tanement favorable. Arch Ma1 Coeur 1975;68:419-429. symptomatic patients may have a poor outlook without 12. Isnard-Baladi J, Iselin M, Veneziea R. Potier J, Focault J. Tumeurs cardia revelation neonatate. Arch Mrd Coeur 1985;78:785-789. intervention, which has prompted the advocacy of early aques 13. McAllister H, Fenoglio J. Tumors of the cardiovascular system. Atlas of and aggressivesurgery by some workers.5+‘1 Tumour Pathology, vol 15. Washington D.C., Armed Forces Institute of PatholoIn 1975, spontaneousregressionof a cardiac rhabdo- gy, 1978:25-31. 14. Alkalay A, Ferry D, Lin 8, Fink B, Pomerance J. Spontaneous regression of myoma was reported.lI Subsequently, others have advo- cardiac rhabdomycnna in tuberous sclerosis. Clin Ped 1987;26:532-535. cated an expectant approach to management.I4 Never- 15. Marx G, Bicrman F, Matthews H, Williams R. Two dimensional echocardiotheless,the strength of the casefor or against a%ressive graphic diagnosis of intracardiac masses in infancy. J Am Coil Cwdiol management remains unresolved. Our study certainly 1984;3:827-832. 16. Gresser C, Shime J, Rakowski H, Smallhorn J, Hui A, Berg J. Fetal cardiac demonstratesthat spontaneoustumor regressionis over- tumor: a prenatal echocardiographic marker for tuberous sclerosis. Am J Obstet 1987;156:689-690. whelmingly the rule and that in the absence of life- Gynecol 17. Amparo E, Higgins C, Farmer D, Gamsu G, McNamara M. Gated MRI of threatening complications conservative management is cardiac and paracardiac masses. AJR 1984;143:1151-1156. 18. Smith H, Watson G, Pate1 R, Super M. Cardiac rhabdomyomata in tuberous probably indicated. their course and diagnostic value. Arch Dis Child 1989;64:196-200, A review of published reports revealed 15 casesof sclerosis: 1% Foster E, Spooner E, Farina M, Shaher R, Alley R. Cardiac rhabdomyoma in rhabdomyoma with at least some evidence of tumor re- the neonate: surgical management. Ann Thorac Surg 1984;37:249-253. gression.‘,l2,14,18-25 N evertheless,the question of wheth- 20. Gillor A, Stock 6, Schuster D. Diagnostik und Behandlung der Kardialen Rhabdomyome. Monatsschr Kinderheilkd 1986;134:445-449. er aggressivesurgical management is indicated in all 21. Stijns M, Lintermans J, Tremouroux M, Vliers A. Spontaneous disappearcases remains unresolved. Our study documents that ance of aortic subvalvar obstruction in young infants with tuberous sclerosis. Cardiol 1982;3:88-89. with a large m.umberof patients followed in a single Pediatr 22. Casta A. Tuberous sclerosis and Wolff-Parkinson-White syndrome. J Pediatr center, cardiac rhabdomyoma, while occasionally life- 1986;109:399. threatening, is increasingly diagnosed in patients with 23. Journel H, Rousscy M, Plais M, Milan J, Almange C, LeMarec B. Prenatal of famililial tuberous sclerosis following detection of cardiac rhabdofew or no symptoms.This suggeststhat the tumor may diagnosis myoma by ultrasound. Prenat Diagn 1986;6:283-289. in fact have a greater incidence than previously appreci- 24. Pernot C, Hoeffel JC., Touche M, Worms A. Les tumeurs primitives du cower ated. Furthermore, spontaneousregression of most, if spontanement regressives chez i’enfant. A propos d’une observation. Coeur 1979;10:303-307. not all, tumors over several years can be anticipated, to 25. Ross RD, Paridon SE, Humes RA, Karpawich PP, Pinsky. Spontaneous the extent that an expectant approach to managementis regression of cardiac rhabdomyomas. Am J Cardio/ 1989,64:416.
THE AMERICAN JOURNAL OF CARDIOLOGY
NOVEMBER 15, 1990
1249