- Email: [email protected]
HYDATID CYST OF THE INTERVENTRICULAR SEPTUM OF THE HEART WITH RUPTURE INTO THE RIGHT VENTRICLE
HYDATID CYST OF THE INTERVENTRICULAR SEPTUM OF THE HEART WITH RUPTURE INTO THE RIGHT VENTRICLE Herndn Artucio, M.D., Jose L. Roglia, M.D., Raul Di Bello, M.D., Jorge Dubra, M.D., Agust'tn Gorlero, M.D., Jose Polero, M.D., and Hernan Artucio Urioste, M.D., Montevideo, Uruguay the first patient operated upon successfully by Long1 in 1932, operation has been performed on 50 cases of cardiac echinococcosis, consisting of cysts of the left ventricle, the pericardium, or the auricles. The following case is the first reported in world literature diagnosed and operated upon suc cessfully under open-heart surgery.
S
INCE
CASE REPORT .T. T. III., a 26-year-old male country dweller, was hospitalized at the Italian Hospital in Montevideo, Uruguay, on J a n . 5, 1961. Two months prior to admission he had experienced a sharp precordial pain radiating to the left axilla, accompanied by malaise. This episode lasted 20 minutes, followed by rest in bed for several hours. The patient remained symptomfree for one week, but on the eighth day he complained of a sudden, intense, constrictive, precordial pain accompanied by profuse perspiration, marked dyspnea, fever, pruritus, and collapse, with loss of consciousness for several minutes. The patient was taken to a county hospital where he was treated with oxygen, cardiotonics, and antibiotics. After marked im provement of his condition, ho was discharged from that hospital and referred to the Italian Hospital of our city for additional treatment and supplementary studies. On examination the patient was lucid, afebrile, and in excellent general condition. The apex beat was palpated at the fourth intercostal space inside the midclavicular line. On auscultation a split first sound was heard at the apical region; a split second sound was also heard at the pulmonary area. Blood pressure was 120/50 mm. Hg. The rest of the clinical examination was noncontributory. The hemogram showed 31 per cent eosinophils. The intradermal Casoni test and the Weinberg test were positive. On x-ray examination (Fig. 1), the heart appeared somewhat enlarged, with a normal shape. The right pulmonary hilus was enlarged. Arterial branches corresponding to the inferior region of the hilus appeared wide and irregular. I n the lateral infraclavicular region of the right lung there was an abnormal shadow suggesting an opened hydatid cyst. In the left lung there were two cystic shadows, one in the infraclavicular region and the other in the lateral basal region. Tomography confirmed these findings. Selective angiocardiography (Figs. 2 and 3 ) , with the catheter placed in the right ventricle, showed a filling defect suggesting an abnormal mass protruding into the cavity of the right ventricle and apparently based in the interventricular septum. The inferior From the Department ot Cardiologry of the Hospital Italiano, Montevideo, Uruguay. Supported in part by Research Grant B-3612 from the National Institutes of Health, U. S. Public Health Service. Received for publication Sept. 26, 1961. 110
Vol. 44, No. 1 July, 1962
HYDATID CYST
111
Fig. 1.—Chest roentgenogram which shows slight enlargement of the cardiac silhouette that retains its normal contour. Right hilus is enlarged with the vessels of its lower sector irregularly widened. In the axillary region of the right lung there is a shadow with the appearance of an opened hydatid cyst. In the left lung two cystic shadows are seen, one in the infraclavicular region and the other on the outer part of the lower third.
Pig. 2.—Selective angiocardiography. Frontal view: Dextrocardiogram showing a lacunar rounded image of irregular contour that occupies the left part of the right ventricle and that is surrounded by the contrast medium above, to the right, and below. Fig. 3.—Selective angiocardiography. Lateral view: Lacunar image implanted in the interventricular septum.
112
ARTTJCIO E T AL.
J. Thoracic and Cardiovas. Surg.
branches of the right pulmonary artery were not well filled with the contrast medium, as if they were partially obstructed by emboli of hydatid material. The electrocardiogram (Fig. 4) revealed the presence of a right bundle branch block. A roentgenogram of the right upper abdominal quadrant failed to show calcified hydatid cysts of the liver. There were no signs of echinococcosis in an x-ray study of the hip bones. Catheterization of the right cavities and pulmonary artery revealed normal pressure curves. Operation.—Right ventriculotomy under extracorporeal circulation revealed a hydatid cyst located in the middle p a r t of the ventricular septum, extending from its anterior to its posterior edge. The cyst was completely independent of the anterior wall of the right ventricle and protruded into the right ventricular cavity. The cyst had a perforation 7 to. 8 mm. in diameter in the region of its maximal prominence, through which one could see degenerated hydatid debris.
Fig. 4.—Electrocardiogram which shows a right bundle branch block. The cystic adventitia was widely opened and fragments of degenerated hydatid mem brane and daughter cysts were removed. The cystic cavity, whose diameter was 6 cm., was carefully swabbed with a saturated saline solution. By palpation it was verified t h a t the interventricular septum had a good thickness a t the region where the cyst was based. The cystic adventitia and the ventriculotomy were closed with a double over-and-over suture of 3-0 silk thread. The first 14 postoperative days were uneventful. The patient was up on the ninth day. On the fourteenth day, fever and right heart failure appeared. The heart failure was rapidly checked with cardiotonics and diuretics. The fever slowly disappeared with antibiotics, and the patient was discharged 35 days after operation. DISCUSSION
In the world literature approximately 40 cases of hydatid cyst of the inter ventricular septum of the heart are recorded. In all these the septal location of the parasite was established at autopsy. In only 3 cases was cardiac echinococ cosis diagnosed during life. Moreau and Boudin2 and Demin and Sumarokov3 report septal cysts which ruptured into the pericardium, producing echinococ cosis of the serosa. The diagnosis of hydatidosis of the heart in these cases was based on the roentgenologie deformities caused by the pericardial cysts. In the case of Blakely and Keid4 the cyst was located at the apex of the left ventricle and in the neighbouring portion of the septum; the diagnosis of hydatid cyst was based on the radiologic evidence of calcification of the heart. At operation, the cyst, which was growing out of the posterior and lower part of the ventricle,
Vol. 44, No. 1 July, 1962
H Y D A T I D CYST
113
was not removed owing to the extreme thinness of the myocardium. I n none of these 3 cases was the localization in the interventricular septum considered during life. In our observation, the presence of multiple and well-tolerated pulmonary shadows in both lungs of a patient living in a cattle-raising area, where human echinococcosis is quite prevalent, pointed to the primary diagnosis of multiple pulmonary echinococcosis.5 Our first impression was corroborated by positive Casoni and Weinberg tests. The enlarged cardiac shadow, with marked electroeardiographic changes, suggested that the multiple pulmonary cysts were metastatic 6 due to the rupture of a primary hydatid cyst located somewhere in the right heart chambers. The two episodes of precordial pain, with the accompanying symptoms of dyspnea, profuse perspiration, shock, etc., were considered to have been due to multiple hydatid pulmonary emboli originating in a ruptured primary hydatid cyst.7 The presence of an electrocardiogram which showed a pattern of right bundle branch block and the absence of localized deformities in the cardiac shadow prompted our conclusion that the primary hydatid cyst was located in the interventricular septum. In these respects it appeared similar to the cases of Barclay, 8 Buch,9 Herard, 10 Duroux and associates,11 and Maestrelli.12 In all these cases a primary hydatid cyst located in the interventricular septum rup tured into the right ventricular cavity, giving rise to metastatic pulmonary echinococcosis and, in some of them, to hydatid pulmonary embolization. A right bundle branch block was also present in the case reported by Duroux and associates11 and, probably, in the observation of Valdivieso and Sepulveda. 13 Nevertheless, we could not totally discard a possible localization of the primary cyst in the right ventricular wall, because it has been known that some cysts may invade the lumen of the right ventricle exclusively, without causing deformities in the cardiac contour. 14 It has been stated also that these in tramural cysts may cause right bundle branch block15 on the basis of local changes in the conducting system or because the embolic obstruction of the pulmonary artery creates a syndrome of chronic cor pulmonale. 14 ' 16 Angiocardiography confirmed the assumption of a septal localization of the cyst. The localization of the primary cyst having been established, it was decided to operate under extracorporeal circulation. With the removal of the primary cyst, we hoped to prevent further embolization of hydatid material to the lungs. Such hydatid emboli could cause death, 17 ' 18 give rise to the syndrome of chronic hydatid cor pulmonale 14 ' ir ' with its bad prognosis, or sow both lungs with new cysts. Operation was considered dangerous because (1) removal of the cyst could be followed by perforation of the interventricular septum, and (2) the cyst could involve the right ventricular wall complicating its surgical removal. 12 ' 1B Although cases in which the primary hydatid cyst occupied the whole thickness of the interventricular septum have been reported, 20 this is a quite unusual feature. In addition, it is known that no case of hydatid cyst of the interventricular septum has ruptured into both ventricles. The removal of the cyst was carried out with no technical difficulties. A good thickness of the interventricular septum was confirmed by palpation after the cyst was removed.
ARTUCIO E T AL.
114
J. Thoracic and Cardiovas. Surg.
The difficult problem of treatment of the multiple pulmonary cysts will be dealt with in the future. SUMMARY
The authors report a case of hydatid cyst of the interventricular septum of the heart which was suspected clinically, and confirmed by angiocardiography. The patient was successfully operated upon by ventrieulotomy under extracorporeal circulation. REFERENCES
1. Long, W. J . : Hydatid Cyst in the Loft Ventricular Wall of the Heart, M. J . Australia 12: 701, 1932. 2. Moreau, R., and Boudin, G.: Kystes hydatiques multiples du coeur, Arch. mal. coeur 34: 91, 1941. 3. Demin, A. A., and Sumarokov, A. V.: The Electrocardiographs Changes in Echinococcosis of the Heart, Soviet Med. 16: 81, 1952. 4. Blakely, E. R., and Reid, J. D . : Hydatid Disease: Report of an Unusual Case, New Zealand M. J . 50: 44, 1951. 5. Piaggio Blanco, R., and Garcia Capurro, F . : Equinococosis pulmonar, Buenos Aires, 1939, El Ateneo. 6. Boppe, M.: L'echinococcose pulmonaire metastatique, Thesis, Paris, 1916. 7. DeVe, F . : La rupture iterative des kystes hydatiques du coeur, Compt. rend. Soc. biol. Paris 79: 514, 1916. 8. Barclay, J . : Case of Hydatids of the Heart and Lungs, Glasgow M. J . 1: 426, 1867. 9. Buch: Echinococcosis of the Heart, Vrach. Gaz. 15: 829, 1908. 10. H e r a r d : Kystes hydatiques developp6s dans la cloison interventriculaire du coeur, Gaz. hebd. med. et chir. 7: 445, 1870. 11. Duroux, A., Tabusse, P., and Marty, J . : Une observation anatomoclinique d'echinococ cose pulmonaire m^tastasique d'origine cardiaque, J . franc, med. et chir. thorac. 5: 259, 1951. 12. Maestrelli: Storia clinica ed anatomica di un caso di echinococco dei pulmoni e del cuore e considerazioni varie, Gior. di med. mil. 19: 477, 1871. 13. Valdivieso, R., and Sepulveda, G.: Equinococosis del corazon, Rev. med. Chile 7 3 : 522, 1945. 14. Amarg6s, A., Meneridez, H., and Di Bello, R.: Cor pulmonale hidatidico, Dia med. 20: 600, 1948. 15. Piaggio Blanco, R.: Equinococosis pulmonar multiple, An. Fac. med. Montevideo 23: 135, 1938. 16. Mendendez, H., and Di Bello, R.: Corazon pulmonar cronico hidatico, An. Fac. med. Montevideo 40: 133, 1955. 17. Bingham, C.: Bursting of an Hydatid Cyst (Situated in the Septum Ventriculi of H e a r t ) , Australia M. J . 2: 362, 1880. 18. Rokitansky, K.: Kystes hydatiques dans la cloison des ventricules du coeur et dans le foie; mort subite in de Welling, L.: Des kystes hydatiques du coeur, Thesis. Paris, 1872 (case 15, p. 20). 19. Andreini, A.: Caso di echinococco del cuore umano, Boll. Soc. rom. per gli stud. zool. 6: 227, 1897. 20. Bado, J . L.: Quiste hidatico del corazon, Bol. Soc. Anat. pat. Montevideo 1: 115, 1930.
S
INCE
CASE REPORT .T. T. III., a 26-year-old male country dweller, was hospitalized at the Italian Hospital in Montevideo, Uruguay, on J a n . 5, 1961. Two months prior to admission he had experienced a sharp precordial pain radiating to the left axilla, accompanied by malaise. This episode lasted 20 minutes, followed by rest in bed for several hours. The patient remained symptomfree for one week, but on the eighth day he complained of a sudden, intense, constrictive, precordial pain accompanied by profuse perspiration, marked dyspnea, fever, pruritus, and collapse, with loss of consciousness for several minutes. The patient was taken to a county hospital where he was treated with oxygen, cardiotonics, and antibiotics. After marked im provement of his condition, ho was discharged from that hospital and referred to the Italian Hospital of our city for additional treatment and supplementary studies. On examination the patient was lucid, afebrile, and in excellent general condition. The apex beat was palpated at the fourth intercostal space inside the midclavicular line. On auscultation a split first sound was heard at the apical region; a split second sound was also heard at the pulmonary area. Blood pressure was 120/50 mm. Hg. The rest of the clinical examination was noncontributory. The hemogram showed 31 per cent eosinophils. The intradermal Casoni test and the Weinberg test were positive. On x-ray examination (Fig. 1), the heart appeared somewhat enlarged, with a normal shape. The right pulmonary hilus was enlarged. Arterial branches corresponding to the inferior region of the hilus appeared wide and irregular. I n the lateral infraclavicular region of the right lung there was an abnormal shadow suggesting an opened hydatid cyst. In the left lung there were two cystic shadows, one in the infraclavicular region and the other in the lateral basal region. Tomography confirmed these findings. Selective angiocardiography (Figs. 2 and 3 ) , with the catheter placed in the right ventricle, showed a filling defect suggesting an abnormal mass protruding into the cavity of the right ventricle and apparently based in the interventricular septum. The inferior From the Department ot Cardiologry of the Hospital Italiano, Montevideo, Uruguay. Supported in part by Research Grant B-3612 from the National Institutes of Health, U. S. Public Health Service. Received for publication Sept. 26, 1961. 110
Vol. 44, No. 1 July, 1962
HYDATID CYST
111
Fig. 1.—Chest roentgenogram which shows slight enlargement of the cardiac silhouette that retains its normal contour. Right hilus is enlarged with the vessels of its lower sector irregularly widened. In the axillary region of the right lung there is a shadow with the appearance of an opened hydatid cyst. In the left lung two cystic shadows are seen, one in the infraclavicular region and the other on the outer part of the lower third.
Pig. 2.—Selective angiocardiography. Frontal view: Dextrocardiogram showing a lacunar rounded image of irregular contour that occupies the left part of the right ventricle and that is surrounded by the contrast medium above, to the right, and below. Fig. 3.—Selective angiocardiography. Lateral view: Lacunar image implanted in the interventricular septum.
112
ARTTJCIO E T AL.
J. Thoracic and Cardiovas. Surg.
branches of the right pulmonary artery were not well filled with the contrast medium, as if they were partially obstructed by emboli of hydatid material. The electrocardiogram (Fig. 4) revealed the presence of a right bundle branch block. A roentgenogram of the right upper abdominal quadrant failed to show calcified hydatid cysts of the liver. There were no signs of echinococcosis in an x-ray study of the hip bones. Catheterization of the right cavities and pulmonary artery revealed normal pressure curves. Operation.—Right ventriculotomy under extracorporeal circulation revealed a hydatid cyst located in the middle p a r t of the ventricular septum, extending from its anterior to its posterior edge. The cyst was completely independent of the anterior wall of the right ventricle and protruded into the right ventricular cavity. The cyst had a perforation 7 to. 8 mm. in diameter in the region of its maximal prominence, through which one could see degenerated hydatid debris.
Fig. 4.—Electrocardiogram which shows a right bundle branch block. The cystic adventitia was widely opened and fragments of degenerated hydatid mem brane and daughter cysts were removed. The cystic cavity, whose diameter was 6 cm., was carefully swabbed with a saturated saline solution. By palpation it was verified t h a t the interventricular septum had a good thickness a t the region where the cyst was based. The cystic adventitia and the ventriculotomy were closed with a double over-and-over suture of 3-0 silk thread. The first 14 postoperative days were uneventful. The patient was up on the ninth day. On the fourteenth day, fever and right heart failure appeared. The heart failure was rapidly checked with cardiotonics and diuretics. The fever slowly disappeared with antibiotics, and the patient was discharged 35 days after operation. DISCUSSION
In the world literature approximately 40 cases of hydatid cyst of the inter ventricular septum of the heart are recorded. In all these the septal location of the parasite was established at autopsy. In only 3 cases was cardiac echinococ cosis diagnosed during life. Moreau and Boudin2 and Demin and Sumarokov3 report septal cysts which ruptured into the pericardium, producing echinococ cosis of the serosa. The diagnosis of hydatidosis of the heart in these cases was based on the roentgenologie deformities caused by the pericardial cysts. In the case of Blakely and Keid4 the cyst was located at the apex of the left ventricle and in the neighbouring portion of the septum; the diagnosis of hydatid cyst was based on the radiologic evidence of calcification of the heart. At operation, the cyst, which was growing out of the posterior and lower part of the ventricle,
Vol. 44, No. 1 July, 1962
H Y D A T I D CYST
113
was not removed owing to the extreme thinness of the myocardium. I n none of these 3 cases was the localization in the interventricular septum considered during life. In our observation, the presence of multiple and well-tolerated pulmonary shadows in both lungs of a patient living in a cattle-raising area, where human echinococcosis is quite prevalent, pointed to the primary diagnosis of multiple pulmonary echinococcosis.5 Our first impression was corroborated by positive Casoni and Weinberg tests. The enlarged cardiac shadow, with marked electroeardiographic changes, suggested that the multiple pulmonary cysts were metastatic 6 due to the rupture of a primary hydatid cyst located somewhere in the right heart chambers. The two episodes of precordial pain, with the accompanying symptoms of dyspnea, profuse perspiration, shock, etc., were considered to have been due to multiple hydatid pulmonary emboli originating in a ruptured primary hydatid cyst.7 The presence of an electrocardiogram which showed a pattern of right bundle branch block and the absence of localized deformities in the cardiac shadow prompted our conclusion that the primary hydatid cyst was located in the interventricular septum. In these respects it appeared similar to the cases of Barclay, 8 Buch,9 Herard, 10 Duroux and associates,11 and Maestrelli.12 In all these cases a primary hydatid cyst located in the interventricular septum rup tured into the right ventricular cavity, giving rise to metastatic pulmonary echinococcosis and, in some of them, to hydatid pulmonary embolization. A right bundle branch block was also present in the case reported by Duroux and associates11 and, probably, in the observation of Valdivieso and Sepulveda. 13 Nevertheless, we could not totally discard a possible localization of the primary cyst in the right ventricular wall, because it has been known that some cysts may invade the lumen of the right ventricle exclusively, without causing deformities in the cardiac contour. 14 It has been stated also that these in tramural cysts may cause right bundle branch block15 on the basis of local changes in the conducting system or because the embolic obstruction of the pulmonary artery creates a syndrome of chronic cor pulmonale. 14 ' 16 Angiocardiography confirmed the assumption of a septal localization of the cyst. The localization of the primary cyst having been established, it was decided to operate under extracorporeal circulation. With the removal of the primary cyst, we hoped to prevent further embolization of hydatid material to the lungs. Such hydatid emboli could cause death, 17 ' 18 give rise to the syndrome of chronic hydatid cor pulmonale 14 ' ir ' with its bad prognosis, or sow both lungs with new cysts. Operation was considered dangerous because (1) removal of the cyst could be followed by perforation of the interventricular septum, and (2) the cyst could involve the right ventricular wall complicating its surgical removal. 12 ' 1B Although cases in which the primary hydatid cyst occupied the whole thickness of the interventricular septum have been reported, 20 this is a quite unusual feature. In addition, it is known that no case of hydatid cyst of the interventricular septum has ruptured into both ventricles. The removal of the cyst was carried out with no technical difficulties. A good thickness of the interventricular septum was confirmed by palpation after the cyst was removed.
ARTUCIO E T AL.
114
J. Thoracic and Cardiovas. Surg.
The difficult problem of treatment of the multiple pulmonary cysts will be dealt with in the future. SUMMARY
The authors report a case of hydatid cyst of the interventricular septum of the heart which was suspected clinically, and confirmed by angiocardiography. The patient was successfully operated upon by ventrieulotomy under extracorporeal circulation. REFERENCES
1. Long, W. J . : Hydatid Cyst in the Loft Ventricular Wall of the Heart, M. J . Australia 12: 701, 1932. 2. Moreau, R., and Boudin, G.: Kystes hydatiques multiples du coeur, Arch. mal. coeur 34: 91, 1941. 3. Demin, A. A., and Sumarokov, A. V.: The Electrocardiographs Changes in Echinococcosis of the Heart, Soviet Med. 16: 81, 1952. 4. Blakely, E. R., and Reid, J. D . : Hydatid Disease: Report of an Unusual Case, New Zealand M. J . 50: 44, 1951. 5. Piaggio Blanco, R., and Garcia Capurro, F . : Equinococosis pulmonar, Buenos Aires, 1939, El Ateneo. 6. Boppe, M.: L'echinococcose pulmonaire metastatique, Thesis, Paris, 1916. 7. DeVe, F . : La rupture iterative des kystes hydatiques du coeur, Compt. rend. Soc. biol. Paris 79: 514, 1916. 8. Barclay, J . : Case of Hydatids of the Heart and Lungs, Glasgow M. J . 1: 426, 1867. 9. Buch: Echinococcosis of the Heart, Vrach. Gaz. 15: 829, 1908. 10. H e r a r d : Kystes hydatiques developp6s dans la cloison interventriculaire du coeur, Gaz. hebd. med. et chir. 7: 445, 1870. 11. Duroux, A., Tabusse, P., and Marty, J . : Une observation anatomoclinique d'echinococ cose pulmonaire m^tastasique d'origine cardiaque, J . franc, med. et chir. thorac. 5: 259, 1951. 12. Maestrelli: Storia clinica ed anatomica di un caso di echinococco dei pulmoni e del cuore e considerazioni varie, Gior. di med. mil. 19: 477, 1871. 13. Valdivieso, R., and Sepulveda, G.: Equinococosis del corazon, Rev. med. Chile 7 3 : 522, 1945. 14. Amarg6s, A., Meneridez, H., and Di Bello, R.: Cor pulmonale hidatidico, Dia med. 20: 600, 1948. 15. Piaggio Blanco, R.: Equinococosis pulmonar multiple, An. Fac. med. Montevideo 23: 135, 1938. 16. Mendendez, H., and Di Bello, R.: Corazon pulmonar cronico hidatico, An. Fac. med. Montevideo 40: 133, 1955. 17. Bingham, C.: Bursting of an Hydatid Cyst (Situated in the Septum Ventriculi of H e a r t ) , Australia M. J . 2: 362, 1880. 18. Rokitansky, K.: Kystes hydatiques dans la cloison des ventricules du coeur et dans le foie; mort subite in de Welling, L.: Des kystes hydatiques du coeur, Thesis. Paris, 1872 (case 15, p. 20). 19. Andreini, A.: Caso di echinococco del cuore umano, Boll. Soc. rom. per gli stud. zool. 6: 227, 1897. 20. Bado, J . L.: Quiste hidatico del corazon, Bol. Soc. Anat. pat. Montevideo 1: 115, 1930.