Surgical treatment for rhabdomyoma of the right atrium causing arrhythmias

Surgical treatment for rhabdomyoma of the right atrium causing arrhythmias

8 0 2 Brief communications tional myxoma cases, and these tumors should be examined for the presence of other biologically active peptides. REFERENCE...

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8 0 2 Brief communications

tional myxoma cases, and these tumors should be examined for the presence of other biologically active peptides. REFERENCES Effert VS, Domanig E: Diagnostik intra-aurikularer Tumoren und grosser Thromben mit dem UltraschallEchoverfahren. Dtsch Med Wochenschr 84:6-8, 1959 2 Saphir 0: Neoplasm of the Pericardium and Heart, SE Gould, ed., Springfield, 1953, Charles C Thomas, Publisher, pp 874-877 3 Likoff W, Geckeler GD, Gregory JE: Functional mitral stenosis produced by an intra-atrial tumor. Am Heart J 47:619-625, 1954 4 Heath D: Pathology of cardiac tumors. Am J Cardiol 21:315-327,1968 5 McAllister HA Jr, Fenoglio JJ Jr: Tumors of the Cardiovascular System, Second Series, Fascicle 15, Washington, D. C; 1977, Armed Forces Institute of Pathology 6 Ezekowitz MD, Smith EO, Rankin R, Harrison LH Jr, Krous HF: Left atrial mass. Diagnostic value of transesophageal 2-dimensional echocardiography and indium-III platelet scintigraphy. Am J Cardiol 51:1563-1564, 1983 7 Kabbani SS, Cooley DA: Atrial myxoma. Surgical considerations. J THORAC CARDIOVASC SURG 65:731-737, 1973 8 Donahoo JS, Weiss JL, Gardner TJ, Fortuin NJ, Brawley RK: Current management of atrial myxoma with emphasis on a new diagnostic technique. Am Surg 189:763-768, 1979 9 Pandian MR, Howat A, Said SI: Radio-immunoassay of VIP in blood and tissues, Vasoactive Intestinal Peptide, SI Said, Ed., New York, 1982, Raven Press, pp 35-50 10 Bulkley BH, Hutchins GM: Atrial myxomas. A fifty year review. Am Heart J 97:639-643, 1979 11 Sutton MG, Mercier L, Giuliani ER, Lee LT: Atrial myxomas. A review of clinical experience in 40 patients. Mayo Clin Proc 55:371-376, 1980 12 Selzer A, Sakai FJ, Popper RW: Protean clinical manifestations of primary tumors of the heart. Am J Med 52:9-18, 1972 13 Hattler BG, Fuchs JC, Cosson R, Sabiston DC Jr: Atrial myxoma. An evaluation of clinical and laboratory manifestations. Ann Thorac Surg 10:65-74, 1970 14 Ferrans VJ, Roberts WC: Structural features of cardiac myxomas. Histology histochemistry, and electron microscopy. Hum Pathol 4:111-46, 1973 15 Feldman PS, Horvath E, Kovacs K: An ultrastructural study of seven cardiac myxomas. Cancer 40:2216-2232, 1977 16 Said SI, Faloona GR: Elevated plasma and tissue levels of vasoactive intestinal polypeptide in the watery-diarrhea syndrome due to pancreatic, bronchogenic and other tumors. N Engl J Med 293:155-160, 1975

The Journal of Thoracic and Cardiovascular Surgery

Surgical treatment for rhabdomyoma of the right atrium causing arrhythmias Scott Goldman, M.D., Randall Lortscher, M.D., and George Pappas, M.D., Denver, Colo. From the Divisions of Cardiovascular Surgery and Cardiology, The Children's Hospital, Denver, Colo. Rhabdomyomas of the heart OCClD' as multiple lesions in 90% of patientsso affected, and 80 % of the patients die in tbe first year of life. A 21-mootb-old child presented with a large isolated right atrial rhabdomyoma ca~ing a supraventricular

tacbyarrbytlunia. Surgical excision cured the arrbythmia.

Rhabdomyoma of the heart involves multiple lesions

in 90% of the patients with this disease. I The natural history of untreated patients with rhabdomyoma is poor, with 80% dying in the first year of life.' We have recently treated an unusual case of a large isolated right atrial rhabdomyoma in a 21-month-old child. Case report. A 21-month-old girl was the fourth born ofa 28-year-old mother. The child's growth and development were normal until she fell approximately 3 feet and hit the right side of her head. She later began having right-sided seizures. The pulse rate was irregular at 150 beats/min. A chest x-ray film revealed mild cardiac enlargement and mildly increased pulmonary vascularity. Atrioventricular dissociation and an ectopic atrial focus were detected on the electrocardiogram. Fig. 1 is representative of the tracings obtained during 48 hours of telemetric monitoring in the hospital. The patient was treated with digoxin empirically. Electroencephalography revealed no spikes or seizure activity. A computed tomographic scan disclosed hypodense areas in the right parietal region, but typical findings of tuberous sclerosis were not present. A right atrial mass was detected by echocardiography (Fig. 2, A). All valves and other cardiac chambers were normal. A presumptive diagnosis of right atrial tumor was made. The child had no familial history or physical symptoms of tuberous sclerosis. The development of an acute upper respiratory tract illness during her first hospital admission delayed scheduled cardiac exploration for 1 month. Before the operation, a second computed tomographic scan revealed no change from the previous study. At cardiac exploration, a large tumor occupying one quarter of the volume of the right atrium was located in the superior lateral free wall. With the use of cardiopulmonary bypass and moderate hypothermia, the entire tumor was resected, including a free 5 mm margin of normal right atrium (Fig. 2, B) The right atrial defect was closed primarily. The resection involved the right lateral surface of the right atrium just distal to the orifice of the superior vena cava. The sinus node region was not disturbed, and the caval orifice remained intact after resection. Cardiac function was good at the termination of bypass. Sinus Address for reprints: Dr. George Pappas, The Children's Hospital, 1056 E. 19th Ave., Denver, Colo. 80218.

Volume 89 Number 5 May, 1985

Brief communications

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Fig 1. Lead II of the electrocardiogram (see text). rhythm was present and no arrhythmias occurred in the postoperative period. The child had a normal convalescence and was discharged 1 week after the operation. She remained asymptomatic and in sinus rhythm at 8 months postoperatively. Yearly follow-up with echocardiography is recommended to assess possible tumor recurrence. Discussion. Although rare, rhabdomyoma is the most common cardiac tumor in children.' It is thought to be a hamartoma as opposed to a true neoplasm and is associated with tuberous sclerosis in more than 50% of the patients.' The usual presentation is that of either severe hemodynamic obstruction in an infant or neonate, because of an intracavitary right or left ventricular tumor, or severe low cardiac output, because of multiple intramural tumors. I. 2. 5 Often these tumors cause recurrent tachyarrhythmias." The diagnosis of rhabdomyoma should be suspected in children with unexplained congestive heart failure, cardiomegaly, irregular heart borders, or arrhythmias. 1, 7 The two-dimensional echocardiogram is accurate in displaying the size and location of the tumor or tumors.v'? Cardiac catheterization and angiography may not add additional information and may induce severe arrhythmias. Surgical treatment should not be delayed in patients with evidence of inflow or outflow obstruction 1. 2, 11-13 or intractable arrhythmias." The operative procedure is best performed via a median sternotomy with total cardiopulmonary bypass and cardiac arrest. Complete removal of all tumors may not be possible, but good results may be obtained with removal of the obstructing mass 1• 2, 12, 13 or the arrhythmogenic focus." The supraventricular tachycardia may result from impingement of the tumor against the sinoatrial node or from arrhyth-

Fig 2. A, Two-dimensional echocardiogramdemonstrating a large right atrial rhabdomyoma (arrows). RA, Right atrium. Ao, Aorta. LV, Left ventricle. B, Intraoperative photograph demonstrating the tumor (T) that was attached to the lateral free wall of the right atrium. This was easily excised and was not attached to the orifice of the superior vena cava (SVC). mogenesis of the tumor area. Removal of the tumor cured the arrhythmia in our patient. The case reported here is similar to the case reported by Kilman, Craenen, and Hosier" and represents an unusual part of the spectrum of rhabdomyoma.

REFERENCES De Lorna JG, Villagra F, De Leon JP, Casanova M, Collado R, Brito JM: Rhabdomyoma of the heart. J Cardiovasc Surg 23:149-154, 1982 2 Como A, de Simone G, Catena G, Marcelletti C: Cardiac rhabdomyoma. Surgical treatment in the neonate. J THORAC CARDIOVASC SURG 87:725-731, 1984 3 Arciniegas E, Hakimi M, Farooki ZQ, Truccone NJ, Green EW: Primary cardiac tumors in children. J THORAC CARDIOVASC SURG 79:582-591, 1980 4 Sabiston DC, Hattler BG: Tumors of the heart, Gibbon's Surgery of the Chest, ed 4, Philadelphia, 1983, W. B. Saunders Company, pp 1585-1597

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5 Nadas AS, Ellison RC: Cardiac tumors in infancy. Am J Cardiol 21:363-366, 1968 6 Silverman NA: Primary cardiac tumors. Ann Surg 191:127-138, 1980 7 Van der Hauwaert LG: Cardiac tumours in infancy and childhood. Br Heart J 33:125-132, 1971 8 Riggs TW, Ilbawi M, De Leon S, Paul MH: Echocardiographic diagnosis of right ventricular rhabdomyoma in two infants. Pediatr Cardiol 3:31-34, 1982 9 Allen LD, Joseph MC, Tyran M: Clinical value of echocardiographic colour image processing in two cases of primary cardiac tumor. Br Heart J 49:154-156, 1983 10 Allen HD, Blieden LC, Stone FM, Bessinger FB Jr, Lucas RV Jr: Echocardiographic demonstration of a right ventricular tumor in a neonate. J Pediatr 84:854-856, 1974 11 Bini RM, Westaby S, Bargeron LM Jr, Pacifico AD, Kirklin JW: Investigation and management of primary cardiac tumors in infants and children. Pediatr Cardiol 2:351-357, 1983 12 Shaher RM, Mintzer J, Farina M, Alley R, Bishop M: Clinical presentation of rhabdomyoma of the heart in infancy and childhood. Am J Cardiol 30:95-103, 1972 13 Foster ED, Spooner EW, Farina MA, Shaher RM, Alley RD: Cardiac rhabdomyoma in the neonate surgical management. Ann Thorac Surg 37:244-253, 1984 14 Kilman JW, Craenen J, Hosier DM: Replacement of entire right atrial wall in an infant with a cardiac rhabdomyoma. J Pediatr Surg 8:317-321,1973

Transposition of posterior leaflet for treatment of ruptured main chordae of the anterior mitral leaflet Arrigo Lessana, M.D., Marcio Escorsin, M.D., Mauro Romano, M.D., Francois Ades, M.D., William Vergoni, M.D., Daniela Lorenzoni, M.D., Carlo Menozzi, M.D., and Igor Monducci, M.D.,

Paris, France, and Reggio Emilia, Italy Fromthe C.H.P.A.La Roseraie, 120Avenue de la Republique, 93300 Aubervilliers, Paris. France, and Ospedale Santa Maria Nuova, Unita Sanitaria Locale No.9, Reggio Emilia, Italy. A 13-year-old girl with mitral regurgitation resulting from rupture of multiple chordae of the anterior leaflet bad repair by transposition of a part of the posterior leaflet to the free edge of the anterior mitral cusp, Postoperative clinical hemodynamic, and angiographic studies showed perfect function of the mitral valve. This technique seems to be a good solution for mitral repair in the presence of ruptured anterior mitral chordae. Address for reprints: Arrigo Lessana, M.D., C.H.P.A. La Roseraie, 120, Avenue de la Republique, 93300 Aubervilliers, Paris, France.

The Journal of Thoracic and Cardiovascular Surgery

Fig. 1. A, Left ventricular angiograms with severe mitral regurgitation before repair. B, Postoperative angiogram showing mitral competence. Rupture of chordae of the anterior mitral leaflet is generally considered a contraindication for conservative procedures.':' Although chordal replacement has been attempted,' these techniques are used only rarely. Carpeatier' proposed another technique wherein chordae of the mural mitral leaflet are transposed to the anterior cusp. We report a case of multiple ruptured chordae of the anterior mitral leaflet in which a segment of the mural leaflet with its attached chordae was transplanted to the free edge of the prolapsed anterior cusp. Case report. A 13-year-old girl was admitted to our department for treatment of severe mitral regurgitation. Preoperatively, she was in New York Heart Association (NYHA) Functional Class II. A loud apical systolic murmur was present. An echocardiographic study showed a prolapsed anterior leaflet with a small mass, corresponding to the stump of one of the ruptured anterior chordae. Angiograms showed Stage 3 mitral regurgitation according to Bjork's classification" (Fig. I, A). The ejection fraction was 65%, wedge pressure 18 mm Hg, and cardiac index 3.8 L/min/m 2• Operation was performed with general hypothermia at 24° C, topical hypothermia, and crystalloid cardioplegia. The cross-clamp time was 38 minutes. Myocardial temperature was maintained at 10° C. The mitral valve anulus was found to be dilated, and the rupture of two chordae had caused prolapse of the anterior leaflet. Those ruptured chordae were 1 em apart; one was the principal chorda and the other, the paramedial.' Mitral repair was conducted as follows: Two