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HYDATID CYST OF THE RIGHT VENTRICLE OF THE HEART
HYDATID CYST OF THE RIGHT VENTRICLE OF THE HEART Raul Di Bello, M.D., ]uan Carlos Abo, M.D., Jorge Dubra, M.D., and Engel Garcia Diaz, M.D., Montevideo, Uruguay
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of only 34 cases of hydatid cysts of the right ventricle demonstrated at autopsy have been published to our knowledge. In the following case, surgical removal was successfully performed for the first time. EPORTS
CASE REPORT A woman, 34 years of age, showed a deformity of the left contour of the heart on a routine x-ray examination in October, 1962. In the 3 or 4 preceding years the patient had had occasional crises of left submammary pain propagated to the neck, back, left shoulder and arm down to the wrist, exacerbated by breathing and movements of the trunk; these seizures appeared at any moment and lasted for 1 or 2 days. I n the previous 2 or 3 years, she had also experienced crises of paroxysmal tachycardia that lasted some minutes. On examination the apex beat could be palpated only in the left lateral decubitus at the fifth intercostal space, slightly lateral to the midclavicular line. Dullness over the third, fourth, and fifth left intercostal spaces from the sternum to the anterior axillary line was found. There was tenderness over all the left phrenic points. On auscultation, heart sounds were normal. The rest of the clinical examination was noncontributory. The electrocardiogram only showed a T wave of low voltage in Dl and aV L . The posteroanterior roentgenogram of the chest showed a deformity of the left middle contour of the heart, just below the trunk of the pulmonary artery (Fig. 1 ) . In the left anterior oblique view there was an abnormal protuberance on the posterosuperior contour of the heart. In the lateral view, the abnormal image was superimposed upon the upper part of the heart; an teriorly, it did not reach the sternum, and it did not displace the esophagus backwards. Other laboratory findings were normal. Operation.—(Nov. 28, 1962). A thoracotomy was performed through the periosteal sheath of the left fifth rib. There were some adhesions in the left pleura and a lax, gen eralized, pericardial symphysis. The pericardium was incised immediately behind the phrenic nerve, which was found to be displaced by an egg-shaped hydatid cyst, measuring 6 by 4 cm., with a vertical major axis and situated in the anterior wall and upper part of the right ventricle, just to the right of the anterior interventricular groove. The cyst, as it developed within the thickness of the ventricular wall, had grown outward and had destroyed the over lying layer of muscle by compression and thus the most prominent part was whitish in color and composed of the adventitia without muscle fibers, whereas the peripheral zone was reddish because of the thin overlying heart muscle. When the cyst was opened, a semi-solid, sub stance emerged in which neither hydatid debris nor daughter vesicles were recognizable. The parietal pericardium that covered the cyst was abnormally vascularized. Careful exProm the Department of Cardiology of the Hospital Italiano, Montevideo, Uruguay. Supported in part by Research Grant AI-03612-03 from the National Institutes of Health, U. S. Public Health Service. Received for publication April 22, 1963. 522
Vol. 46, No. 4 October, 1963
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ploration of the pericardial recesses at the base of the heart revealed no additional cysts. After being cleansed with a 33 per cent sodium chloride solution, the emerging adventitia was excised and the cystic cavity was left open. The microscopic examination of the material obtained at the operation (Dr. J . F. Cassinelli) confirmed the hydatid nature of the lesion. Four months after the operation the pa tient is perfectly well.
Fig. 1.—Roentgenogram of the heart shows a deformity of the left contour just below the trunk of the pulmonary artery.
DISCUSSION
The presence of a deformity of the left contour of the roentgenological image of the heart in a patient coming from a region infested by the Taenia echinococcus immediately suggested the possible diagnosis of cardiac echinococcosis.1 In spite of the roentgenological location, the very slight and atypical abnormality of the electrocardiogram made its location in the left ventricle un likely as this always gives rise to an inverted, coronary type T wave. 2 ' 3 Only 2 case reports have been published of the cyst in this location and with a normal electrocardiogram, 4 ' 5 but it is almost certain that only standard leads were used. Nevertheless, in the case of Perez Pontana and Sapriza, 6 a cyst situated in the superficial layers of the myocardium of the left ventricle showed a T wave only slightly inverted and which became normal again shortly after sur gical removal of the cyst. Bearing this in mind, our patient was thought to have a cyst of the left ventricle initially implanted subepicardially, and which had later developed almost entirely extramyocardially. Other possibilities con-
524
D I B E L L O E T AL.
J. Thoracic and Cardiovas. Surg.
sidered were a hydatid graft in the pericardium 7 or a lesion of a different nature. The possibility of a location in the right ventricle was not taken into consideration because of its rarity, the absence of pulmonary manifestations subsequent to its frequent intracardiac rupture, 8 ' 9 and the location of the ab normal roentgenographic image, which was suggestive of a cyst of the left ven tricle. However, the cyst of the right ventricle reported by Piaggio Blanco10- " showed a roentgenological image with a similar location. According to our experience, a generalized symphysis of the pericardium signifies that the cyst has opened within the intrapericardial space, even in the absence of secondary echinococcosis of the pericardium 12 and of a clear his tory of acute pericarditis. In fact, in a review of the world literature, we have found generalized pericardial symphysis in 24 of 125 cases of primary myoeardial cyst without secondary pericardial echinococcosis (19.2 per cent). On the other hand, according to Lamelas and Olaran Osorio13 pericardial symphysis was only found in 1.7 per cent of 20,000 autopsies. These statistics suggest that the coexistence of a hydatid cyst of the myocardium with a generalized pericardial symphysis is generally not a mere coincidence but that the latter is related to the rupture of the cyst in the pericardium. In our case, there were no elements to suggest the intracardiac rupture of the cyst 14 ; at the opera tion a good thickness of ventricular wall was found beneath the bed of the cyst. This is an unusual case, as cysts of the right ventricle rupture into this cavity in 58.8 per cent of the cases and into the intrapericardial space exclusively in only 8.8 per cent. 15 ' 46 ' " This is a case similar to the more common forms of cysts of the left ventricle, for which reason it was treated surgically as such. We believe that in the eventuality of an intracardiac rupture of a cyst located in the right ventricular wall, the surgeon will be compelled to operate under extracorporeal circulation. The precordial pains felt by the patient were prob ably due to small, repeated ruptures of the cyst. SUMMARY
The authors present a case of hydatid cyst of the right ventricle successfully treated by operation. REFERENCES 1. Di Bello, E . : El diagnostieo y las formas clinicas de la hidatidosis cardiaca, An. Fac. med. Montevideo 40: 93, 1955. 2. Di Bello, B . : El electrocardiograma en la equinoeocosis cardiaca. Trabajo basado en 50 observaciones de la literatura mundial, An. Fac. med. Montevideo 4 1 : 16, 1956. 3. Di Bello, E.: Contribuci6n al estudio del quiste hidatico del ventriculo izquierdo. Trabajo basado en 94 observaciones de la literatura mundial, An. Fac. med. Montevideo 4 1 : 235, 1956. 4. Carrau, A., and Moreau, J . : Sobre hidatidosis cardiaca, Arch, pediat. Uruguay 1: 453, 1930. 5. Iakovlev, A. A., and Travianskaia, A. B . : Eoentgen Diagnosis of Cardiac Echinococcosis, Klin. med. CEuss.) 29: 49, 1951. 6. Perez Fontana, V., and Sapriza, J . P . : Quiste hidatico hialino del ventriculo izquierdo, Arch. int. Hidatid. 13: 157, 1953. 7. Di Bello, E., and Menendez, H . : E l injerto hidatico del pericardio, Arch. urug. med. 46: 167, 1955.
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8. Amarg6s, A., Menendez, H., and Di Bello, B . : Cor pulnionale hidatidico, Dia m6d. 20: 600, 1948. 9. Purriel, P., Muras, O., Tomalino, D., and Mendoza, D . : Equinococosis cardiaea. Siembra metastatica pulmonar. (Quiste hidatico de pared del ventriculo derecho abierto en cavidades derechas; obstrucci6n de la arteria pulmonar izquierda; equinococosis intraarterial y pulmonar), Torax 1: 223, 1952. 10. Piaggio Blanco, B . : Equinococosis pulmonar multiple, An. Fac. med. Montevideo 2 3 : 135, 1938. 11. Piaggio Blanco, B., and Garcia Capurro, F . : Equinococosis pulmonar, Buenos Aires, 1939, " E l Ateneo." 12. Di Bello, B., Bubio, B., Dighiero, J., Zubiaurre, L., and Cortes, B . : Pseudo-Aneurysmatic Form of Cardiac Eehinococcosis. Beport of a New Case and Beview of the Literature, J.
THORACIC SURG. 4 5 :
657,
1963.
13. Lamelas, J . A., and Olaran Osorio, B . : Enfermedades del pericardio, Barcelona, 1950, " E d i t o r i a l cientifico m e d i c a , " p. 266. 14. Di Bello, B., and Menendez, H . : Intracardiac Bupture of Hydatid Cysts of the Heart. A Study Based on 3 Personal Observations and 101 Cases in the World Literature. Circulation 27: 366, 1963. 15. Ballotta, F . : Cisti d ' echinococco del cuore, Arch. ital. anat. e istol. patol. 7: 435, 1936. 16. Bossi, E . : Echinococcosi del cuore e morte improwisa, Minerva med. 70: 24, 1950. 17. Lechnir: In Bourgeon, B . : Les kystes hydatiques du coeur, Thesis, Argel, 1940, p. 227.
R
of only 34 cases of hydatid cysts of the right ventricle demonstrated at autopsy have been published to our knowledge. In the following case, surgical removal was successfully performed for the first time. EPORTS
CASE REPORT A woman, 34 years of age, showed a deformity of the left contour of the heart on a routine x-ray examination in October, 1962. In the 3 or 4 preceding years the patient had had occasional crises of left submammary pain propagated to the neck, back, left shoulder and arm down to the wrist, exacerbated by breathing and movements of the trunk; these seizures appeared at any moment and lasted for 1 or 2 days. I n the previous 2 or 3 years, she had also experienced crises of paroxysmal tachycardia that lasted some minutes. On examination the apex beat could be palpated only in the left lateral decubitus at the fifth intercostal space, slightly lateral to the midclavicular line. Dullness over the third, fourth, and fifth left intercostal spaces from the sternum to the anterior axillary line was found. There was tenderness over all the left phrenic points. On auscultation, heart sounds were normal. The rest of the clinical examination was noncontributory. The electrocardiogram only showed a T wave of low voltage in Dl and aV L . The posteroanterior roentgenogram of the chest showed a deformity of the left middle contour of the heart, just below the trunk of the pulmonary artery (Fig. 1 ) . In the left anterior oblique view there was an abnormal protuberance on the posterosuperior contour of the heart. In the lateral view, the abnormal image was superimposed upon the upper part of the heart; an teriorly, it did not reach the sternum, and it did not displace the esophagus backwards. Other laboratory findings were normal. Operation.—(Nov. 28, 1962). A thoracotomy was performed through the periosteal sheath of the left fifth rib. There were some adhesions in the left pleura and a lax, gen eralized, pericardial symphysis. The pericardium was incised immediately behind the phrenic nerve, which was found to be displaced by an egg-shaped hydatid cyst, measuring 6 by 4 cm., with a vertical major axis and situated in the anterior wall and upper part of the right ventricle, just to the right of the anterior interventricular groove. The cyst, as it developed within the thickness of the ventricular wall, had grown outward and had destroyed the over lying layer of muscle by compression and thus the most prominent part was whitish in color and composed of the adventitia without muscle fibers, whereas the peripheral zone was reddish because of the thin overlying heart muscle. When the cyst was opened, a semi-solid, sub stance emerged in which neither hydatid debris nor daughter vesicles were recognizable. The parietal pericardium that covered the cyst was abnormally vascularized. Careful exProm the Department of Cardiology of the Hospital Italiano, Montevideo, Uruguay. Supported in part by Research Grant AI-03612-03 from the National Institutes of Health, U. S. Public Health Service. Received for publication April 22, 1963. 522
Vol. 46, No. 4 October, 1963
HYDATID CYST
523
ploration of the pericardial recesses at the base of the heart revealed no additional cysts. After being cleansed with a 33 per cent sodium chloride solution, the emerging adventitia was excised and the cystic cavity was left open. The microscopic examination of the material obtained at the operation (Dr. J . F. Cassinelli) confirmed the hydatid nature of the lesion. Four months after the operation the pa tient is perfectly well.
Fig. 1.—Roentgenogram of the heart shows a deformity of the left contour just below the trunk of the pulmonary artery.
DISCUSSION
The presence of a deformity of the left contour of the roentgenological image of the heart in a patient coming from a region infested by the Taenia echinococcus immediately suggested the possible diagnosis of cardiac echinococcosis.1 In spite of the roentgenological location, the very slight and atypical abnormality of the electrocardiogram made its location in the left ventricle un likely as this always gives rise to an inverted, coronary type T wave. 2 ' 3 Only 2 case reports have been published of the cyst in this location and with a normal electrocardiogram, 4 ' 5 but it is almost certain that only standard leads were used. Nevertheless, in the case of Perez Pontana and Sapriza, 6 a cyst situated in the superficial layers of the myocardium of the left ventricle showed a T wave only slightly inverted and which became normal again shortly after sur gical removal of the cyst. Bearing this in mind, our patient was thought to have a cyst of the left ventricle initially implanted subepicardially, and which had later developed almost entirely extramyocardially. Other possibilities con-
524
D I B E L L O E T AL.
J. Thoracic and Cardiovas. Surg.
sidered were a hydatid graft in the pericardium 7 or a lesion of a different nature. The possibility of a location in the right ventricle was not taken into consideration because of its rarity, the absence of pulmonary manifestations subsequent to its frequent intracardiac rupture, 8 ' 9 and the location of the ab normal roentgenographic image, which was suggestive of a cyst of the left ven tricle. However, the cyst of the right ventricle reported by Piaggio Blanco10- " showed a roentgenological image with a similar location. According to our experience, a generalized symphysis of the pericardium signifies that the cyst has opened within the intrapericardial space, even in the absence of secondary echinococcosis of the pericardium 12 and of a clear his tory of acute pericarditis. In fact, in a review of the world literature, we have found generalized pericardial symphysis in 24 of 125 cases of primary myoeardial cyst without secondary pericardial echinococcosis (19.2 per cent). On the other hand, according to Lamelas and Olaran Osorio13 pericardial symphysis was only found in 1.7 per cent of 20,000 autopsies. These statistics suggest that the coexistence of a hydatid cyst of the myocardium with a generalized pericardial symphysis is generally not a mere coincidence but that the latter is related to the rupture of the cyst in the pericardium. In our case, there were no elements to suggest the intracardiac rupture of the cyst 14 ; at the opera tion a good thickness of ventricular wall was found beneath the bed of the cyst. This is an unusual case, as cysts of the right ventricle rupture into this cavity in 58.8 per cent of the cases and into the intrapericardial space exclusively in only 8.8 per cent. 15 ' 46 ' " This is a case similar to the more common forms of cysts of the left ventricle, for which reason it was treated surgically as such. We believe that in the eventuality of an intracardiac rupture of a cyst located in the right ventricular wall, the surgeon will be compelled to operate under extracorporeal circulation. The precordial pains felt by the patient were prob ably due to small, repeated ruptures of the cyst. SUMMARY
The authors present a case of hydatid cyst of the right ventricle successfully treated by operation. REFERENCES 1. Di Bello, E . : El diagnostieo y las formas clinicas de la hidatidosis cardiaca, An. Fac. med. Montevideo 40: 93, 1955. 2. Di Bello, B . : El electrocardiograma en la equinoeocosis cardiaca. Trabajo basado en 50 observaciones de la literatura mundial, An. Fac. med. Montevideo 4 1 : 16, 1956. 3. Di Bello, E.: Contribuci6n al estudio del quiste hidatico del ventriculo izquierdo. Trabajo basado en 94 observaciones de la literatura mundial, An. Fac. med. Montevideo 4 1 : 235, 1956. 4. Carrau, A., and Moreau, J . : Sobre hidatidosis cardiaca, Arch, pediat. Uruguay 1: 453, 1930. 5. Iakovlev, A. A., and Travianskaia, A. B . : Eoentgen Diagnosis of Cardiac Echinococcosis, Klin. med. CEuss.) 29: 49, 1951. 6. Perez Fontana, V., and Sapriza, J . P . : Quiste hidatico hialino del ventriculo izquierdo, Arch. int. Hidatid. 13: 157, 1953. 7. Di Bello, E., and Menendez, H . : E l injerto hidatico del pericardio, Arch. urug. med. 46: 167, 1955.
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Vol. 46, No. 4 October, 1963
525
8. Amarg6s, A., Menendez, H., and Di Bello, B . : Cor pulnionale hidatidico, Dia m6d. 20: 600, 1948. 9. Purriel, P., Muras, O., Tomalino, D., and Mendoza, D . : Equinococosis cardiaea. Siembra metastatica pulmonar. (Quiste hidatico de pared del ventriculo derecho abierto en cavidades derechas; obstrucci6n de la arteria pulmonar izquierda; equinococosis intraarterial y pulmonar), Torax 1: 223, 1952. 10. Piaggio Blanco, B . : Equinococosis pulmonar multiple, An. Fac. med. Montevideo 2 3 : 135, 1938. 11. Piaggio Blanco, B., and Garcia Capurro, F . : Equinococosis pulmonar, Buenos Aires, 1939, " E l Ateneo." 12. Di Bello, B., Bubio, B., Dighiero, J., Zubiaurre, L., and Cortes, B . : Pseudo-Aneurysmatic Form of Cardiac Eehinococcosis. Beport of a New Case and Beview of the Literature, J.
THORACIC SURG. 4 5 :
657,
1963.
13. Lamelas, J . A., and Olaran Osorio, B . : Enfermedades del pericardio, Barcelona, 1950, " E d i t o r i a l cientifico m e d i c a , " p. 266. 14. Di Bello, B., and Menendez, H . : Intracardiac Bupture of Hydatid Cysts of the Heart. A Study Based on 3 Personal Observations and 101 Cases in the World Literature. Circulation 27: 366, 1963. 15. Ballotta, F . : Cisti d ' echinococco del cuore, Arch. ital. anat. e istol. patol. 7: 435, 1936. 16. Bossi, E . : Echinococcosi del cuore e morte improwisa, Minerva med. 70: 24, 1950. 17. Lechnir: In Bourgeon, B . : Les kystes hydatiques du coeur, Thesis, Argel, 1940, p. 227.