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Replacement of entire right atrial wall in an infant with a cardiac rhabdomyoma
Replacement of Entire Right Atrial Wall in an Infant With a Cardiac Rhabdomyoma By James W. Kilman, Josepha Craenen, and Don M. Hosier
A R D I A C T U M O R S are exceedingly rare in infancy and in general are benign. R h a b d o m y o m a is probably the most c o m m o n cardiac tumor in infancy and m a y be regardecl as a truly congenital tumor since it has been f o u n d in the hearts of stillborn infants. I The tumor is usually multiple, 2 and is sometimes associated with tuberous sclerosis. These infants frequently present with supraventricular arrhythmias of u n k n o w n etiology and can have associated central venous anomalies. 3 W e recently treated an infant with a single r h a b d o m y o m a of the right atrium who presented with a severe tachycardia. Surgical excision of the tumor was carried out and the entire right atrial wall was replaced b y pericardia. This appears to be the y o u n g e s t infant to have an intracavitary tumor excised from the heart and the only k n o w n survivor of a complete excision of a r h a b d o m y o m a of the heart.
C
CASE REPORT A 9-too-old white female was the first born of art 18-yr-old mother who was a known user of LSD during her pregnancy. The baby had normal growth and development until approximately 1 wk prior to admission. The infant became quite irritable and did n o t feed as well as before. She had a heart rate of 200/rain at a routine well-baby visit and was referred to the hospital for evaluation. A chest film revealed mild cardiac hypertrophy and the electrocardiogram showed a bizarre supraventricular tachycardia with unusual "P" waves (Fig. 1). There was a wandering pacemaker and the deep, enlarged "P" waves were thought to be due to right atrial enlargement. She was treated with digoxin and quinidine until the arrhythmia was controlled before cardiac catheterization which showed a large mass in the right atrium projecting from the lateral wall. This mass allowed only a small passage from the superior and inferior cava to the right ventricle (Fig. 2A). A presumptive diagnosis of a right atrial tumor was made. Intravenous pyelogram was normal, as was an electroencephelogram. There was no family history of tuberous sclerosis. On mediastinal exploration, a large tumor occupying the entire right atrium proved to be a benign rhabdomyoma. There was no tumor involving any other chamber. Using total cardiopulmonary bypass, the entire tumor and most of the right atrial wall from the orifice of the superior vena cava to the orifice of the inferior vena cava
From the Department of Thoracic Surgery and Division of Pediatric Cardiology, Department of Pediatrics, and the Ohio State University College of Medicine, The Columbus Children's Hospital, Columbus, Ohio. Presented at the annual meeting of the Surgical Section of the American Academy of Pediatrics, New York, N.Y., October 14-15, 1972. James W. Kilman, M.D.: Professor of Surgery, Department of Surgery, Ohio State University College of Medicine, Columbus, Ohio. Josepha Craenen, M.D.: Assistant Professor of Pediatrics, Department of Pediatrics, Ohio State University College of Medicine, Columbus, Ohio. Don M. Hosier, M.D. : Professor of Pediatrics, Department of Pediatrics, Ohio State University College of Medicine, Columbus, Ohio. Address for reprint requests: ]ames W. Kilman, M.D., The Columbus Children's Hospital, 56I South I7th Street, Columbus, Ohio 43205. (~ 1973 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 8, No. 2 (April), 1973
317
318
K I L M A N , CRAENEN, A N D HOSIER
to the annulus of the tricuspid valve below the right coronary artery to the intraatrial groove was excised. Autogenous pericardium was used to reconstruct a new right atrium. Cardiac action was good after bypass ceased. Sinus rhythm was present and no arrhythmias occurred in the postoperative period. A follow-up cineangiogram of the
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Fig. 1. (A) E l e c t r o c a r d i o g r a m at the t i m e of a d m i s s i o n to the hospital but f o l l o w i n g t r e a t m e n t with d i g o x i n and q u i n i d i n e . (B) E l e c t r o c a r d i o g r a m f o l l o w i n g resection of t u m o r a n d r e c o n s t r u c t i o n of right atrial c h a m b e r with a u t o g e n o u s p e r i c a r d i u m .
REPLACEMENT OF ATRIAL WALL
319
Fig. 2. (A) Cineangiogram of the heart showing mass les=on in right atrium. (B) Cineangiogram of the heart after resection of the tumor and reconstruction of the right atrium with autogenous pericardium.
right atrium showed a good-sized tight atrium and the patient has been asymptomatic for I yr following surgery (Fig. 2B). She has been on no medication and has remained in sinus rhythm. The rhabdomyoma was 2~/2 cm by 6 cm and demonstrated classic spider cells histologically (Fig. 3A,B).
DISCUSSION Cardiac tumors in infancy are rare but are not so uncommon that they should not be considered in the differential diagnosis of the infant with unusual cardiac findings. The intramural or the intracavitary tumors seem most likely to produce the main hemodynamic symptoms. ~ The patient with a cardiac rhabdomyoma has a predeliction to arrhythmias and since these tumors are usually associated with tuberous sclerosis giving central nervous system symptoms, and/or skin ancl kidney tumors, the diagnosis should be considered in these patients when appropriate. Most of the reported cardiac rhabdomyomas have been found at postmortem examination in stillborn, newborn, and neonatal patients and only two reports of a surgical approach to these tumors have been found. Golding and Reed5 reported a 14-mo-old patient with a ventricular septal defect and infundibular pulmonic stenosis who underwent successful open-heart surgery. The muscle fragments removed during the infundibular resection revealed rhabdomyoma. Postoperative correlation with previous central nervous system symptoms m a d e the diagnosis of tuberous sclerosis likely. Kuehl et al. 8 reported an unsuccessful attempt to remove a rhabdomyoma causing subaortic stenosis in a newborn infant. The operation perhaps would have been unsuccessful even if the obstruction had been removed, since multiple tumors were found in the heart at the time of postmortem examination. In a recent review of cardiac tumors in infancy and childhood by Van der Hauwaert, v concerning material from 15 European cardiac centers, 22 tumors were recorded, No well-documented malignant primary cardiac tumor was found in an infant or a child. These "benign" cardiac tumors were the cause of death in 11 of the patients, usually by encroachment into cardiac cavities
320
KILMAN, CRAENEN, AND HOSIER
or by resulting arrhythmias. Eleven children had surgical removal of their tumors and eight had excellent results. Six children under 2 yr of age had rhabdomyomas found at postmortem examination; none of these patients had been considered a candidate for surgery. Three of the patients had single tumors present in the heart that conceivably could have been removed. Open-heart surgery for the neonate and infant is now well enough established to justify a more aggressive approach when the infant exhibits unusual cardiac signs a n d symptoms. 8-11 A complete angiographic study should be made and exploration done if any evidence of intracavitary or intramural mass is demonstrated. Tumors should be excised if possible since malignant tumors are rare and the usual benign tumors do not invade cardiac muscle but only displace structures. Right and left atrial walls may be replaced or reconstructed by autogenous pericardium since this material is readily available and funCtions well as a reservoir for carrying blood. Benign tumors may cause death as these space-occupying lesions produce obstructive symptoms or arrhythmias. Operative removal may well result in a cure.
3
4
Fig. 3. (A) Rhabdomyoma removed from the right atrium. Cut surface of the tumor is demonstrated. (B) Microscopic section of the rhabdomyoma demonstrating the classic "spider" cell. PAS stain. X 600.
REPLACEMENT OF ATRIAL WALL
321
T h e association of t u b e r o u s sclerosis w i t h cardiac r h a b d o m y o m a s m a y b e p r e s e n t in 4 0 % - 5 0 % of the patients a n d s h o u l d be diligently searched f o r in the patient a n d in his family, b u t this s h o u l d n o t deter the surgical a t t a c k o n the cardiac tumor. SUMMARY
A 9 - m o - o l d white female with severe s u p r a v e n t r i c u l a r t a c h y c a r d i a o n cinea n g i o g r a p h i c studies p r o v e d to h a v e a s p a c e - o c c u p y i n g m a s s in t h e r i g h t atrium. A r h a b d o m y o m a o f the right a t r i u m w a s r e m o v e d a n d t h e entire r i g h t atrial w a l l w a s r e c o n s t r u c t e d w i t h a u t o g e n o u s pericardium. T h e i n f a n t is a s y m p t o m a t i c I y r following surgery. REFERENCES
1. Bigelow, N. H., Klinger, S., and Wright, A . W.: Primary tumors of heart in infancy and early childhood. Cancer 7:549, 1954. 2. Goodwin, J. F.: The spectrum of cardiac tumors. Am. J. Cardiol. 21:307, 1968. 3. Griffiths, G. C.: A review of primary tumors of the heart. Prog. Cardiovasc. Dis. 7:465, 1965.
4. Nedas, A. S., and Ellison, R. C.: Cardiac tumors in infancy. Am. ]. Cardiol. 21:363, 1968. 5. Golding, R., and Reed, G.: Rhabdomyoma of the heart. N. Engl. J. Med. 276: 957, 1967. 6. Kuehl, K. S., Perry, L. W., Chandra, R., and Scott, L. P.: Left ventricular rhabdomyoma: A rare cause of subaortic stenosis in the newborn infant. Pediatrics 46: 464, 1970.
7. Van der Hauwaert, L. : Cardiac t u m o r s in infancy and childhood. Br. Heart J. 33:125, 1971. 8. Geha, A. S., Weidman, W. H., S0ule, E. H., and McGoon, D. C.: Intramural ventricular cardiac flbroma--successful removal in two cases and review o f the |iterature. Circulation 36:427, 1967. 9. Gerbode, F., Kerth, W. J., and Hill, D.: Surgical management of tumors o f the heart. Surgery 61:94, 1967. 10. Lincoln, J. C. R., Tynan, M. J., and Waterston, D. J. : Successful excision of an endocardial fibroma of the left ~entrlcle in a lO-month old infant. J. Thorac. Cardiovasc. Sur~. 56:63, 1968. 11. White, J. J., Kaback, M. M., and HaUer, J. A.: Diagnosis and excision of an intrapericardial teratoma in an infant. J. Thorac. Cardiovasc. Surg. 55:704, 1968.
A R D I A C T U M O R S are exceedingly rare in infancy and in general are benign. R h a b d o m y o m a is probably the most c o m m o n cardiac tumor in infancy and m a y be regardecl as a truly congenital tumor since it has been f o u n d in the hearts of stillborn infants. I The tumor is usually multiple, 2 and is sometimes associated with tuberous sclerosis. These infants frequently present with supraventricular arrhythmias of u n k n o w n etiology and can have associated central venous anomalies. 3 W e recently treated an infant with a single r h a b d o m y o m a of the right atrium who presented with a severe tachycardia. Surgical excision of the tumor was carried out and the entire right atrial wall was replaced b y pericardia. This appears to be the y o u n g e s t infant to have an intracavitary tumor excised from the heart and the only k n o w n survivor of a complete excision of a r h a b d o m y o m a of the heart.
C
CASE REPORT A 9-too-old white female was the first born of art 18-yr-old mother who was a known user of LSD during her pregnancy. The baby had normal growth and development until approximately 1 wk prior to admission. The infant became quite irritable and did n o t feed as well as before. She had a heart rate of 200/rain at a routine well-baby visit and was referred to the hospital for evaluation. A chest film revealed mild cardiac hypertrophy and the electrocardiogram showed a bizarre supraventricular tachycardia with unusual "P" waves (Fig. 1). There was a wandering pacemaker and the deep, enlarged "P" waves were thought to be due to right atrial enlargement. She was treated with digoxin and quinidine until the arrhythmia was controlled before cardiac catheterization which showed a large mass in the right atrium projecting from the lateral wall. This mass allowed only a small passage from the superior and inferior cava to the right ventricle (Fig. 2A). A presumptive diagnosis of a right atrial tumor was made. Intravenous pyelogram was normal, as was an electroencephelogram. There was no family history of tuberous sclerosis. On mediastinal exploration, a large tumor occupying the entire right atrium proved to be a benign rhabdomyoma. There was no tumor involving any other chamber. Using total cardiopulmonary bypass, the entire tumor and most of the right atrial wall from the orifice of the superior vena cava to the orifice of the inferior vena cava
From the Department of Thoracic Surgery and Division of Pediatric Cardiology, Department of Pediatrics, and the Ohio State University College of Medicine, The Columbus Children's Hospital, Columbus, Ohio. Presented at the annual meeting of the Surgical Section of the American Academy of Pediatrics, New York, N.Y., October 14-15, 1972. James W. Kilman, M.D.: Professor of Surgery, Department of Surgery, Ohio State University College of Medicine, Columbus, Ohio. Josepha Craenen, M.D.: Assistant Professor of Pediatrics, Department of Pediatrics, Ohio State University College of Medicine, Columbus, Ohio. Don M. Hosier, M.D. : Professor of Pediatrics, Department of Pediatrics, Ohio State University College of Medicine, Columbus, Ohio. Address for reprint requests: ]ames W. Kilman, M.D., The Columbus Children's Hospital, 56I South I7th Street, Columbus, Ohio 43205. (~ 1973 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 8, No. 2 (April), 1973
317
318
K I L M A N , CRAENEN, A N D HOSIER
to the annulus of the tricuspid valve below the right coronary artery to the intraatrial groove was excised. Autogenous pericardium was used to reconstruct a new right atrium. Cardiac action was good after bypass ceased. Sinus rhythm was present and no arrhythmias occurred in the postoperative period. A follow-up cineangiogram of the
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Fig. 1. (A) E l e c t r o c a r d i o g r a m at the t i m e of a d m i s s i o n to the hospital but f o l l o w i n g t r e a t m e n t with d i g o x i n and q u i n i d i n e . (B) E l e c t r o c a r d i o g r a m f o l l o w i n g resection of t u m o r a n d r e c o n s t r u c t i o n of right atrial c h a m b e r with a u t o g e n o u s p e r i c a r d i u m .
REPLACEMENT OF ATRIAL WALL
319
Fig. 2. (A) Cineangiogram of the heart showing mass les=on in right atrium. (B) Cineangiogram of the heart after resection of the tumor and reconstruction of the right atrium with autogenous pericardium.
right atrium showed a good-sized tight atrium and the patient has been asymptomatic for I yr following surgery (Fig. 2B). She has been on no medication and has remained in sinus rhythm. The rhabdomyoma was 2~/2 cm by 6 cm and demonstrated classic spider cells histologically (Fig. 3A,B).
DISCUSSION Cardiac tumors in infancy are rare but are not so uncommon that they should not be considered in the differential diagnosis of the infant with unusual cardiac findings. The intramural or the intracavitary tumors seem most likely to produce the main hemodynamic symptoms. ~ The patient with a cardiac rhabdomyoma has a predeliction to arrhythmias and since these tumors are usually associated with tuberous sclerosis giving central nervous system symptoms, and/or skin ancl kidney tumors, the diagnosis should be considered in these patients when appropriate. Most of the reported cardiac rhabdomyomas have been found at postmortem examination in stillborn, newborn, and neonatal patients and only two reports of a surgical approach to these tumors have been found. Golding and Reed5 reported a 14-mo-old patient with a ventricular septal defect and infundibular pulmonic stenosis who underwent successful open-heart surgery. The muscle fragments removed during the infundibular resection revealed rhabdomyoma. Postoperative correlation with previous central nervous system symptoms m a d e the diagnosis of tuberous sclerosis likely. Kuehl et al. 8 reported an unsuccessful attempt to remove a rhabdomyoma causing subaortic stenosis in a newborn infant. The operation perhaps would have been unsuccessful even if the obstruction had been removed, since multiple tumors were found in the heart at the time of postmortem examination. In a recent review of cardiac tumors in infancy and childhood by Van der Hauwaert, v concerning material from 15 European cardiac centers, 22 tumors were recorded, No well-documented malignant primary cardiac tumor was found in an infant or a child. These "benign" cardiac tumors were the cause of death in 11 of the patients, usually by encroachment into cardiac cavities
320
KILMAN, CRAENEN, AND HOSIER
or by resulting arrhythmias. Eleven children had surgical removal of their tumors and eight had excellent results. Six children under 2 yr of age had rhabdomyomas found at postmortem examination; none of these patients had been considered a candidate for surgery. Three of the patients had single tumors present in the heart that conceivably could have been removed. Open-heart surgery for the neonate and infant is now well enough established to justify a more aggressive approach when the infant exhibits unusual cardiac signs a n d symptoms. 8-11 A complete angiographic study should be made and exploration done if any evidence of intracavitary or intramural mass is demonstrated. Tumors should be excised if possible since malignant tumors are rare and the usual benign tumors do not invade cardiac muscle but only displace structures. Right and left atrial walls may be replaced or reconstructed by autogenous pericardium since this material is readily available and funCtions well as a reservoir for carrying blood. Benign tumors may cause death as these space-occupying lesions produce obstructive symptoms or arrhythmias. Operative removal may well result in a cure.
3
4
Fig. 3. (A) Rhabdomyoma removed from the right atrium. Cut surface of the tumor is demonstrated. (B) Microscopic section of the rhabdomyoma demonstrating the classic "spider" cell. PAS stain. X 600.
REPLACEMENT OF ATRIAL WALL
321
T h e association of t u b e r o u s sclerosis w i t h cardiac r h a b d o m y o m a s m a y b e p r e s e n t in 4 0 % - 5 0 % of the patients a n d s h o u l d be diligently searched f o r in the patient a n d in his family, b u t this s h o u l d n o t deter the surgical a t t a c k o n the cardiac tumor. SUMMARY
A 9 - m o - o l d white female with severe s u p r a v e n t r i c u l a r t a c h y c a r d i a o n cinea n g i o g r a p h i c studies p r o v e d to h a v e a s p a c e - o c c u p y i n g m a s s in t h e r i g h t atrium. A r h a b d o m y o m a o f the right a t r i u m w a s r e m o v e d a n d t h e entire r i g h t atrial w a l l w a s r e c o n s t r u c t e d w i t h a u t o g e n o u s pericardium. T h e i n f a n t is a s y m p t o m a t i c I y r following surgery. REFERENCES
1. Bigelow, N. H., Klinger, S., and Wright, A . W.: Primary tumors of heart in infancy and early childhood. Cancer 7:549, 1954. 2. Goodwin, J. F.: The spectrum of cardiac tumors. Am. J. Cardiol. 21:307, 1968. 3. Griffiths, G. C.: A review of primary tumors of the heart. Prog. Cardiovasc. Dis. 7:465, 1965.
4. Nedas, A. S., and Ellison, R. C.: Cardiac tumors in infancy. Am. ]. Cardiol. 21:363, 1968. 5. Golding, R., and Reed, G.: Rhabdomyoma of the heart. N. Engl. J. Med. 276: 957, 1967. 6. Kuehl, K. S., Perry, L. W., Chandra, R., and Scott, L. P.: Left ventricular rhabdomyoma: A rare cause of subaortic stenosis in the newborn infant. Pediatrics 46: 464, 1970.
7. Van der Hauwaert, L. : Cardiac t u m o r s in infancy and childhood. Br. Heart J. 33:125, 1971. 8. Geha, A. S., Weidman, W. H., S0ule, E. H., and McGoon, D. C.: Intramural ventricular cardiac flbroma--successful removal in two cases and review o f the |iterature. Circulation 36:427, 1967. 9. Gerbode, F., Kerth, W. J., and Hill, D.: Surgical management of tumors o f the heart. Surgery 61:94, 1967. 10. Lincoln, J. C. R., Tynan, M. J., and Waterston, D. J. : Successful excision of an endocardial fibroma of the left ~entrlcle in a lO-month old infant. J. Thorac. Cardiovasc. Sur~. 56:63, 1968. 11. White, J. J., Kaback, M. M., and HaUer, J. A.: Diagnosis and excision of an intrapericardial teratoma in an infant. J. Thorac. Cardiovasc. Surg. 55:704, 1968.