Pediatr Cardiol 12:57-59, 1991

Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991

U n u s u a l E v o l u t i o n o f a P e r i c a r d i a i M a s s D i a g n o s e d by F e t a l Echocardiography Nabil-Maurice H. Guirgis, j Annabelle B. Azancot, ~ and Philippe Blot 2 ~Service d'Explorations Fonctionnelles et le 2Departement de Gynecologie et d ' O b s t e t r i q u e , Hopital Robert Debre, Paris, France

SUMMARY. An intrapericardial mass with a pericardial effusion was diagnosed by ultrasound examination in the fetus of a 29-year-old gravida 1 para 0 woman at 32 weeks' gestation. Serial in utero examinations showed disappearance of the pericardial effusion at 35.5 weeks' gestation, while the mass remained unchanged. Vaginal delivery ensued at 39 weeks. Clinical and echocardiographic examination of the newborn revealed normal cardiac anatomy with no abnormal mass or pericardial effusion. Follow-up examinations confirmed these normal data. KEY WORDS: Fetal echocardiography--Pericardial mass--Pericardial effusion

Case Report A 29-year-old gravida 1 para 0 w o m a n was referred to our department for prenatal echocardiograpby. Her medical history revealed Charcot-Marie-Tooth disease. U l t r a s o u n d e x a m i n a t i o n at 32 weeks' gestation d e m o n s t r a t e d a m a s s i v e circumferential pericardial effusion (Fig. 1) associated with an intrapericardial m a s s measuring 1.2 • 1.2 cm. The m a s s was adherent posteriorly to the left atrioventricular groove, being related to the left atrium and the left ventricle (Fig. 2). The m a s s produced a nonh o m o g e n e o u s echo with a hyperechogenic contour, associated with irregular strands between the two layers of the pericardium. There was no Doppler echocardiographic evidence of obstruction to either right or left ventricular inflow, nor signs of valvular regurgitation. The morphology of the heart and great vessels was normal with no signs of heart failure, a r r h y t h m i a s , pleural effusion, tissue edema, or ascites. The patient was hospitalized in the Gynaecology and Obstetrical D e p a r t m e n t and a cesarean section was scheduled if any signs of fetal intolerance occurred. Fetal heart rate was monitored daily. Ecbocardiographic e x a m i n a t i o n s s h o w e d no modification of either the m a s s or the effusion until 35.5 weeks" gestation when the pericardial effusion started to regress. The echocardiographic follow-up examinations continued to show regression of this effusion and its disappearance: however, no modification of the size of the m a s s was noticed. The fetal h e m o d y n a m i c s and growth were not altered. The last echocardiographic examination was performed 8 days before delivery. The infant was born at a gestational term of 39 weeks by s p o n t a n e o u s vaginal delivery. His weight was 4100 g, height 55 cm, and crown perimeter 37 cm. The A p g a r score was 8 at I min,

Address offprint requests to: Dr. Annabelle B. Azancot, Service d'Explorations Fonctionnelles, Hopital Robert Debre, 75019 Paris, France.

and 10 at 5 rain. The clinical e x a m i n a t i o n and routine blood analysis were normal. The electrocardiogram, chest x-ray, and e c h o c a r d i o g r a m were also normal with no evidence of effusion or m a s s within the pericardium or heart. The child was e x a m i n e d again at the age of 3 and 6 m o n t h s . The clinical e x a m i n a t i o n s h o w e d a healthy infant with a normal growth curve. Other investigations included a full blood picture, platelet count, s e r u m electrolytes, immunoglobulin level, c a t e c h o l a m i n e s in blood and urine, and abdominal x-rays, abdominal e c h o g r a p h y , echocardiography, and finally an e l e c t r o m y o g r a p h y . All results were normal.

Discussion Fetal echocardiography has become a valuable and reliable technique for prenatal diagnosis of structural heart defects. Since the development of fetal echocardiography, pericardial masses diagnosed in utero have been reported and confirmed postnatally as benign teratoma [4] and cavernous hemangioma [8]. These tumors usually manifest themselves in the newborn period or in early infancy by cardiopulmonary distress and huge cardiomegaly. Thus, our first most likely diagnosis was an intrapericardial teratoma. The majority of intrapericardial teratomas are anterior and rightward, being attached to the ascending aorta by a broad pedicle. They are usually wedged between the aortic root and superior vena cava, both of which they may compress severely. They may also impinge upon the right atrium, right ventricle, and pulmonary artery displacing the heart posteriorly and to the left [1].

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Fig. 1. Four-chamber view showing circumferential pericardial effusion. EP PER, pericardial effusion: OD, right atrium: OG, left atrium: VD, right ventricle: VG, left ventricle. Fig. 2. The intrapericardial mass {arrows) is shown at the left atrioventricular groove. The left ventricular inflow appears delineating the left ventricular cavity. EP PERIC, pericardial elfusion; VG, left ventricle.

The localization of the mass in our case was not suggestive o f a pericardial teratoma, although very rarely the latter has been reported as occurring in the left side and compressing the left atrium and the left ventricle to the right 11]. In a recent report, W e b e r et al. [9] described a case of a massive benign intrapericardial teratoma in a newborn that had a normal prenatal echocardiogram at 20 weeks' gestation. These authors suggested that the time period in which this abnormal group of cells undergoes rapid growth and differentiation is s o m e w h e r e between 20 and 40 weeks of gestation. Our case is special because of the prenatal regression o f the pericardial mass and the pericardial effusion. Regression of cardiac tumors were first reported by Khattar et al. in 1973 [6]: two cases were r h a b d o m y o m a s and one case was a fibroma. A similar regression o f probably a fibromyoma o f the interventricular septum was reported by Pernot el al. [7]. These authors suggested that nonspecific de-

Pediatric Cardiology Vol. 12, No. 1, 1991

generative p h e n o m e n a : h e m o r r h a g i c centers, together with areas of hydropic degeneration and areas of calcifications, caused the regression of these tumors. In our case, the association of this pericardial mass with the circumferential pericardial effusion suggested the diagnosis of a pericardial teratoma. H o w e v e r , the localization of this mass and its d i s a p p e a r a n c e - - n o t reported in the literature for pericardiai t e r a t o m a s - - m a k e this diagnosis unlikely. Other tumors have been reported intrapericardially, such as bronchogenic cysts and cystic tumors of bronchial origin [3]. These cysts cannot be differentiated from pericardial teratomas except by microscopic examination which shows only tissues of respiratory and gastrointestinal origin. Our case could be an intrapericardial bronchogenic cyst. H o w e v e r , to our knowledge, no cases of involution o f bronchogenic cysts have been published previously. Neoplasms o f neural crest origin, such as paraganglioma (chemodectoma) arising in the chromaffin and c h e m o r e c e p t o r tissues, can be located in the heart and even within the pericardium [2] and such tumors are not known to regress. Echocardiographic diagnosis of an intrapericardial thrombus associated with pericardial effusion has been reported, Our case could be an intrapericardial thrombus associated with a fetal pericardial effusion of unknown etiology. H o w e v e r , to our knowledge, no similar case has been reported by

Guirgis et al,: Pericardial Mass

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prenatal echocardiography. The m o t h e r ' s disease, C h a r c o t - M a r i e - T o o t h , is a h e r e d i t a r y n e u r o m u s c u lar d i s e a s e that c a n yield c a r d i a c r h y t h m or conduction abnormalities. However, intrapericardial m a s s e s have not b e e n d e s c r i b e d in this s y n d r o m e

Aeknowled~,ment. We wish to thank Miss Fran~oise Fontaine for

[5].

I. Arciniegas E, Hakimi M. Farooki ZQ, Green EW (1980) lnIrapericardial teratoma in infancy. J Thorac Cardiovasc Surg 79:306-311 2. Besterman E, Bromley LL, Peart WS (1974) An intrapericardial phaeochromocytoma. Br Heart J 36:318-320 3. Dabbs CH, Berg R, Peirce EC (1957) lntrapericardial bronchogenic cysts. Report of two cases and probable embryologic explanation. J Thorac Surg 34:718-735 4. De Geeter B, Kretz JG, Nisand 1, Eisenmann B, Kieny MTH, Kieny R (1983) lntrapericardial teratoma in a newborn infant: Use of fetal echocardiography. Ann Thorac Sttr3r 35: 664- 666 5. lsner JM. Hawley RJ, Weintraub AM, Engel WK (1979) Cardiac findings in Charcot Marie Tooth disease: A prospective study for 68 patients. Arch Intern Med 139:1161-1165 6. Khattar H, Guerin R, Fouron JC, Stanley P, Kratz C, Davignon A (1975) Los tumeurs cardiaques chez I'enfant. Rapport de trois observations avec 6volution spontan6ment favorable. Arch Mal Coeur 4:419-429 7. Pernot C. Hoeffel JC, Touche M, Worms AM (1979) Les tumeurs primitives du coeur spontan6ment r6gressives chez l'enfant. Apropos d'une observation. Coeur 10:303-314 8. Riggs T, Sholl JS, llbawi M, Gardner T (1984) In utero diagnosis of a pericardial tumour with successful surgical repair. Pediatr Cardiol 5:23-26 9. Weber HS, Kleinman CS, Hellenbrand WE, Kopf GS, Copel J (1988) Development of a benign intrapericardial turnout between 20 and 40 weeks of gestation. Pediatr Cardiol 9:153156

Clinical Implications

A l t h o u g h the n a t u r e of the fetal p a t h o l o g y r e m a i n s u n s o l v e d , we s p e c u l a t e that the i n t r a p e r i c a r d i a l m a s s r e g r e s s e d d u r i n g the last w e e k of p r e g n a n c y or that it r u p t u r e d d u r i n g the u t e r i n e c o n t r a c t i o n s o f labor. O u r s t u d y is o f i n t e r e s t b e c a u s e p r e n a t a l e c h o c a r d i o g r a p h y a l l o w e d close f o l l o w - u p of the fetal t o l e r a n c e of a m a s s i v e pericardial e f f u s i o n a n d o b s e r v a t i o n of the n a t u r a l e v o l u t i o n of a n i n t r a p e r i cardial mass. In this case p r e n a t a l e c h o c a r d i o graphy fulfilled its m a i n role, in g u i d i n g o b s t e t r i c m a n a g e m e n t , a v o i d i n g h a s t y d e c i s i o n s , a n d in assuring i m m e d i a t e n e o n a t a l care. T w o c o n c l u s i o n s might be d r a w n from this case. First, the p r o g n o s i s of a fetal i n l r a p e r i c a r d i a l m a s s a s s o c i a t e d with a pericardial e f f u s i o n should not be c o n s i d e r e d as p o o r from the start. S e c o n d , the d e v e l o p m e n t o f p r e n a t a l e c h o c a r d i o g r a p h y c o n t r i b u t e s to a b e t t e r u n d e r s t a n d i n g o f the n a t u r a l h i s t o r y o f the fetal pathology, and it m a y be able, in the f u t u r e , to a n s w e r q u e s t i o n s that r e m a i n o b s c u r e to us t o d a y .

typing and editorial assistance.

References