Volume
124
Number
4
REFERENCES
1.
2. 3. 4.
5.
6.
Jacobson RS, Rath CE, Perloff JK. Intravascular haemolysis and thrombocytopenia in left ventricular outflow obstruction. Br Heart J 1973;35:849-54. Zezulka A, Shapiro L, Singh S. Chronic haemolytic anaemia in hypertrophic cardiomyopathy. Br Heart J 1984;52:474-6. Marsh GW, Lewis SM. Cardiac hemolvtic anaemia. Semin Hematol 1969;6:133-49. Rosenthal DS, Braunwald E. Hematological-oncological disorders and heart disease. In: Braunwald E, ed. Heart diseases. A texbook of cardiovascular medicine. 3rd ed. Philadelphia: WB Saunders, 19881734-57. Feld H, Roth J. Severe haemolytic anaemia after replacement of the mitral valve by a St. Jude Medical prosthesis. Br Heart J 1989;62:475-6. Flamm MD, Harrison DC, Hancock EW. Muscular subaortic stenosis: prevention of outflow obstruction with propranolol. Circulation 1968;38:845-58.
Dynamic left ventricular outflow obstruction caused by cardiac echinococcosis A. M. Lanzoni, MD,” V. Barrios, MD,a J. L. Moya, MD,a A. Epeldegui, MD,h D. Celemin, MD,h C. Lafuente, MD,” and E. Asin-Cardiel, MD” Madrid, Spain
Hydatidosis is a human parasitic cystic infections that is caused by the larval stage of Echinococcus granulosus. Cardiac involvement is uncommon; it appears in only 0.4 % to 2 “2 of patients with echinococcosis.’ About 2 % to 9 % of the cysts are located in the interventricular septum (IVS).lt 2 This localization has been frequently associated with valvular dysfunction (mainly on the right side3) with varying degrees of atrioventricular2 and intraventricular block and with obstruction of the right ventricular outflow tract,l but it has also been described as a cause of dynamic obstruction of the left ventricle outflow tract.4 We report a case of cardiac hydatid cysts located in the IVS with protrusion into the left ventricular chamber that created an intraventricular dynamic obstruction. A 69-year-old man from a Spanish rural zone who smoked 20 cigarettes per day and had clinical criteria for chronic bronchitis, was admitted to our hospital with a 3-year history of syncopal episodes that were related to emotional stress. At the time of admission, blood pressure was lOO/SO mm Hg, pulmonary auscultation was fine, and cardiac auscultation disclosed a second widely split heart sound and a mild ejection systolic murmur at the left ster-
From the Departments of ‘Cardiology and “Cardiac Surgery, Hospital Ramm Y Cajal, Madrid. Reprint requests: Vivencio Barrios, MD, CiDuque de Alba 6,20 dcha, 28012. Madrid, Spain. 4/4/39802
Brief Communications
1083
nal border. No signs of cardiac failure were present, and results of the neurologic examination were normal. An electroencephalogram and a computed tomographic (CT) scan ruled out a neurologic origin for the syncopal episodes. The ECG revealed sinus rhythm, an undetermined QRS frontal axis, signs of biventricular enlargement, repolarization changes in right precordial leads, and an electrically inactive septal zone (Fig. 1). A chest x-ray film showed a cardiothoracic index of 0.6 and signs that were suggestive of chronic obstructive pulmonary disease. With a Holter monitor (Applied Cardiac Systems, Laguna Hills, Calif.), only isolated premature ventricular contractions were detect.ed. A Doppler two-dimensional echocardiogram demonstrated concentric hypertrophy of the left ventricular wall and a homogenous cystic mass with a diameter of 4.2 cm, uniform density, and smooth rims; it was located in the middle third of the IVS (Fig. 2) and protruded mainly into the left ventricular chamber, which created a maximal intraventricular gradient of 40 mm Hg according to Doppler echocardiography. The transmitral filling profile suggested impaired compliance. An abdominal ultrasonographic examination showed a cyst of 8.7 X 3 cm in diameter with internal echogenic zones in the posterior segments of the right hepatic lobe, which is a typical image of hyatid cyst. A thoracic-abdominal CT scan confirmed the presence of a hyatid cyst in the liver and an intracardiac cystic structure, but the CT scan could not precisely define the anatomic relationship of the cysts (Fig. 3). Cardiac catheterization showed a hypertrophic left ventricle and an intraventricular mass defect in the IVS, which caused an intraventricular peak systolic gradient of 25 mm Hg. Coronary arteries had no obstructive lesions. A blood test showed an erythrocyte sedimentation rate of 20 mm/hr and absence of eosinophilia. Results of an indirect hemagluttination test for echinococcus were positive (l/640). Surgical treatment was indicated, and 7 days before removal of the cyst, chemotherapy with albendazole was started at a dose of 5 mg/kg twice a day and continued during the postoperative period to complete the four cycles of treatment for the hepatic involvement. When cardiopulmonary bypass surgery was performed, the IVS was approached through the right ventricle. After the cyst was punctured and its contents drained, hypertonic glucose solution was instilled, and the hydatid cyst was removed. The septal defect was repaired with a low-porosity Dacron patch. Postoperative evolution was satisfactory, and after 7 months of follow-up the patient remains free of symptoms. Cardiac hydatid disease is a very infrequent entity. The cysts are commonly located in the free wall of the left ventricle (50 % to 77 % ). Localization in the IVS is unusual (2 0:) tOg”;)L2; pericardial involvement appears in 50 “C of cases. The cardiovascular manifestations of cardiac echinococcosis are: palpitations, syncope (as a result of conduction abnormalities, arrhythmias, or valvular or outflow tract obstructions), angina, pulmonary or systemic embolisms,3 or even sudden death in 20% of cases1 but in most of the cases, patients with cardiac hydatid cysts are free of symptoms. ECG could show varying degrees of atrioventricular”
1084
Brief Communications
American
October 1992 Heart Journal
Fig. 1. An ECG at the time of admission showed sinus rhythm, an undetermined QRS axis on the frontal plane, and a feature of QR on lead VI, which suggested an inactive septal zone. A great RS complex from lead V2-Vs, the Katz-Waschtel phenomenon, suggested biventricular enlargement.
Fig. 2. An echocardiogram with smooth rims located atrium; C, cyst.
(apical four-chamber view) demonstrated a homogeneous round cystic mass in the IVS. LV, Left ventricle; RV, right ventricle; RA, right atrium; LA, left
and intraventricular block,l complex premature ventricular contractions, nonspecific repolarization changes, or electrically inactive zones1 as in the case we report. Chest roentgenography can demonstrate signs that suggest the diagnosis, such as the presence of pulmonary cysts or the finding of a round mass with an “onion leaves” calcification on the cardiac silhouette when the cysts are located in the free wall of the left ventricle.’ However, two-dimensional echocardiography is the best diagnostic procedure for car-
diac hydatid disease because of this disease’s typical manifestations and scarce differential diagnosis, mainly when the cysts are located in the 1VS.l. s The diagnostic value of two-dimensional echocardiography is better than that of computed tomography and nuclear magnetic resonance imaging, so these methods should be reserved only for the study of extracardiac involvement of echinococcosis.5 Cardiac catheterization can be useful in defining the relationship of the cyst to the coronary arteries.’
Volume Number
124 4
Brief Communications
Fig. 3. A thoracic CT scan confirmed the presence of a round cystic cardiac inition of the localization and the anatomic relationship. C, Cyst. According to blood tests, eosinophilia is present in 20 9, of cases.’ The serologic diagnosis can be obtained by several methods (e.g., radioallergosorbent test, indirect hemaglutination reaction, basophil degranulation test, and latex agglutination test). Indirect hemagglutination, the test that was used in our patient, has a sensitivity of 82 0;) and a specificity of 91’; ; these rates are similar to those of the radioallergosorbent test, better than those of the latex agglutination test, and worse than rates derived from the basophil degranulation test. Nowadays, the use of larvicidal agents during surgery and preoperative Albendazole therapy are considered useful to reduce the incidence of recurrence, which may reach 10 5. There is no agreement with respect to the most adequate duration of preoperative treatment period for albendazole, but it has been suggested that this therapy should be continued for 4 weeks before surgery. It is commonly accepted that treatment of cardiac hydatid disease must be surgical in all cases2 because of the incidence of sudden death (20’0), rupture in the cardiac chambers (39 ?t8), or rupture into the pericardium (10% )l; however, to our knowledge, there are very few published cases of surgical removal of hydatid cysts located in the IVS.2 Only patients with recurrent or inoperable disease should be considered candidates for chemotherapy with albendazole; the recommended standard therapeutic regimen is four cycles of 30 days, each with a dose of 10 mg/kg per day and a rest period of 2 weeks between cycles, in an attempt to avoid toxicosis (18”;. of the patients have adverse effects),6 but the response to this treatment must be evaluated until at least 1 year after therapy is completed. REFERENCES
1. O’Connor F, Tellez G, Montero Hidatidosis cardiaca: a prop6sito quirhrgicamente. Rev Esp Cardiol
C, Nuiiez L, Figuera D. de 10 cases intervenidos 1988;41:97-102.
mass without
1085
a precise def-
2. Ottino G, Villani M, De Paulis R, Trucco G, Viara J. Restoration of atrioventricular conduction after surgical removal of a hydatid cyst of the intraventricular septum. J Thorac Cardiovasc Surg 1987;93:144-7. 3. Oliver JM, Sotillo J, Dominguez F, et al. Two dimensional echocardiographic features of echinococcosis of the heart and great blood vessels. Circulation 1988;78:327-37. 4. Russo G, Tamburino C, Cuscuni J, et al. Cardiac hydatid cyst with clinical features resembling subaortic stenosis. AM HEART J 1989;117:1385-7. 5. Desnos M, Brochet E, Cristofini P, et al. Polivisceral echinococcosis with cardiac involvement imaged by two dimensional echocardiography, computed tomography and nuclear magnetic resonance imaging. Am J Cardiol 1987;59:383-5. 6. Davis A, Dixon H, Pawlowski Z. Multicentre clinical trials of benzimidazole-carbamates in human cystic echinococcosis (phase 2). Bull WHO 1989;67:503-8.
Dynamic left ventricular outflow tract obstruction 4 years after aortic valve replacement Masataka Sata, MD, Shin-ichi Momomura, MD, Katsu Takenaka, MD, Teruhiko Aoyagi, MD, Toshiyuki Takahashi, MD, Takashi Serizawa, MD, and Tsuneaki Sugimoto, MD. Tokyo, Japan
Dynamic left ventricular outflow obstruction is characteristic of hypertrophic obstructive cardiomyopathy. InFrom the Second Department of Internal Medicine, Faculty of Medicine, University of Tokyo. Reprint requests: Masataka Sata, MD, Second Department of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-l Hongo, Bunkyoku, Tokyo, 113, Japan. 414/39893
124
Number
4
REFERENCES
1.
2. 3. 4.
5.
6.
Jacobson RS, Rath CE, Perloff JK. Intravascular haemolysis and thrombocytopenia in left ventricular outflow obstruction. Br Heart J 1973;35:849-54. Zezulka A, Shapiro L, Singh S. Chronic haemolytic anaemia in hypertrophic cardiomyopathy. Br Heart J 1984;52:474-6. Marsh GW, Lewis SM. Cardiac hemolvtic anaemia. Semin Hematol 1969;6:133-49. Rosenthal DS, Braunwald E. Hematological-oncological disorders and heart disease. In: Braunwald E, ed. Heart diseases. A texbook of cardiovascular medicine. 3rd ed. Philadelphia: WB Saunders, 19881734-57. Feld H, Roth J. Severe haemolytic anaemia after replacement of the mitral valve by a St. Jude Medical prosthesis. Br Heart J 1989;62:475-6. Flamm MD, Harrison DC, Hancock EW. Muscular subaortic stenosis: prevention of outflow obstruction with propranolol. Circulation 1968;38:845-58.
Dynamic left ventricular outflow obstruction caused by cardiac echinococcosis A. M. Lanzoni, MD,” V. Barrios, MD,a J. L. Moya, MD,a A. Epeldegui, MD,h D. Celemin, MD,h C. Lafuente, MD,” and E. Asin-Cardiel, MD” Madrid, Spain
Hydatidosis is a human parasitic cystic infections that is caused by the larval stage of Echinococcus granulosus. Cardiac involvement is uncommon; it appears in only 0.4 % to 2 “2 of patients with echinococcosis.’ About 2 % to 9 % of the cysts are located in the interventricular septum (IVS).lt 2 This localization has been frequently associated with valvular dysfunction (mainly on the right side3) with varying degrees of atrioventricular2 and intraventricular block and with obstruction of the right ventricular outflow tract,l but it has also been described as a cause of dynamic obstruction of the left ventricle outflow tract.4 We report a case of cardiac hydatid cysts located in the IVS with protrusion into the left ventricular chamber that created an intraventricular dynamic obstruction. A 69-year-old man from a Spanish rural zone who smoked 20 cigarettes per day and had clinical criteria for chronic bronchitis, was admitted to our hospital with a 3-year history of syncopal episodes that were related to emotional stress. At the time of admission, blood pressure was lOO/SO mm Hg, pulmonary auscultation was fine, and cardiac auscultation disclosed a second widely split heart sound and a mild ejection systolic murmur at the left ster-
From the Departments of ‘Cardiology and “Cardiac Surgery, Hospital Ramm Y Cajal, Madrid. Reprint requests: Vivencio Barrios, MD, CiDuque de Alba 6,20 dcha, 28012. Madrid, Spain. 4/4/39802
Brief Communications
1083
nal border. No signs of cardiac failure were present, and results of the neurologic examination were normal. An electroencephalogram and a computed tomographic (CT) scan ruled out a neurologic origin for the syncopal episodes. The ECG revealed sinus rhythm, an undetermined QRS frontal axis, signs of biventricular enlargement, repolarization changes in right precordial leads, and an electrically inactive septal zone (Fig. 1). A chest x-ray film showed a cardiothoracic index of 0.6 and signs that were suggestive of chronic obstructive pulmonary disease. With a Holter monitor (Applied Cardiac Systems, Laguna Hills, Calif.), only isolated premature ventricular contractions were detect.ed. A Doppler two-dimensional echocardiogram demonstrated concentric hypertrophy of the left ventricular wall and a homogenous cystic mass with a diameter of 4.2 cm, uniform density, and smooth rims; it was located in the middle third of the IVS (Fig. 2) and protruded mainly into the left ventricular chamber, which created a maximal intraventricular gradient of 40 mm Hg according to Doppler echocardiography. The transmitral filling profile suggested impaired compliance. An abdominal ultrasonographic examination showed a cyst of 8.7 X 3 cm in diameter with internal echogenic zones in the posterior segments of the right hepatic lobe, which is a typical image of hyatid cyst. A thoracic-abdominal CT scan confirmed the presence of a hyatid cyst in the liver and an intracardiac cystic structure, but the CT scan could not precisely define the anatomic relationship of the cysts (Fig. 3). Cardiac catheterization showed a hypertrophic left ventricle and an intraventricular mass defect in the IVS, which caused an intraventricular peak systolic gradient of 25 mm Hg. Coronary arteries had no obstructive lesions. A blood test showed an erythrocyte sedimentation rate of 20 mm/hr and absence of eosinophilia. Results of an indirect hemagluttination test for echinococcus were positive (l/640). Surgical treatment was indicated, and 7 days before removal of the cyst, chemotherapy with albendazole was started at a dose of 5 mg/kg twice a day and continued during the postoperative period to complete the four cycles of treatment for the hepatic involvement. When cardiopulmonary bypass surgery was performed, the IVS was approached through the right ventricle. After the cyst was punctured and its contents drained, hypertonic glucose solution was instilled, and the hydatid cyst was removed. The septal defect was repaired with a low-porosity Dacron patch. Postoperative evolution was satisfactory, and after 7 months of follow-up the patient remains free of symptoms. Cardiac hydatid disease is a very infrequent entity. The cysts are commonly located in the free wall of the left ventricle (50 % to 77 % ). Localization in the IVS is unusual (2 0:) tOg”;)L2; pericardial involvement appears in 50 “C of cases. The cardiovascular manifestations of cardiac echinococcosis are: palpitations, syncope (as a result of conduction abnormalities, arrhythmias, or valvular or outflow tract obstructions), angina, pulmonary or systemic embolisms,3 or even sudden death in 20% of cases1 but in most of the cases, patients with cardiac hydatid cysts are free of symptoms. ECG could show varying degrees of atrioventricular”
1084
Brief Communications
American
October 1992 Heart Journal
Fig. 1. An ECG at the time of admission showed sinus rhythm, an undetermined QRS axis on the frontal plane, and a feature of QR on lead VI, which suggested an inactive septal zone. A great RS complex from lead V2-Vs, the Katz-Waschtel phenomenon, suggested biventricular enlargement.
Fig. 2. An echocardiogram with smooth rims located atrium; C, cyst.
(apical four-chamber view) demonstrated a homogeneous round cystic mass in the IVS. LV, Left ventricle; RV, right ventricle; RA, right atrium; LA, left
and intraventricular block,l complex premature ventricular contractions, nonspecific repolarization changes, or electrically inactive zones1 as in the case we report. Chest roentgenography can demonstrate signs that suggest the diagnosis, such as the presence of pulmonary cysts or the finding of a round mass with an “onion leaves” calcification on the cardiac silhouette when the cysts are located in the free wall of the left ventricle.’ However, two-dimensional echocardiography is the best diagnostic procedure for car-
diac hydatid disease because of this disease’s typical manifestations and scarce differential diagnosis, mainly when the cysts are located in the 1VS.l. s The diagnostic value of two-dimensional echocardiography is better than that of computed tomography and nuclear magnetic resonance imaging, so these methods should be reserved only for the study of extracardiac involvement of echinococcosis.5 Cardiac catheterization can be useful in defining the relationship of the cyst to the coronary arteries.’
Volume Number
124 4
Brief Communications
Fig. 3. A thoracic CT scan confirmed the presence of a round cystic cardiac inition of the localization and the anatomic relationship. C, Cyst. According to blood tests, eosinophilia is present in 20 9, of cases.’ The serologic diagnosis can be obtained by several methods (e.g., radioallergosorbent test, indirect hemaglutination reaction, basophil degranulation test, and latex agglutination test). Indirect hemagglutination, the test that was used in our patient, has a sensitivity of 82 0;) and a specificity of 91’; ; these rates are similar to those of the radioallergosorbent test, better than those of the latex agglutination test, and worse than rates derived from the basophil degranulation test. Nowadays, the use of larvicidal agents during surgery and preoperative Albendazole therapy are considered useful to reduce the incidence of recurrence, which may reach 10 5. There is no agreement with respect to the most adequate duration of preoperative treatment period for albendazole, but it has been suggested that this therapy should be continued for 4 weeks before surgery. It is commonly accepted that treatment of cardiac hydatid disease must be surgical in all cases2 because of the incidence of sudden death (20’0), rupture in the cardiac chambers (39 ?t8), or rupture into the pericardium (10% )l; however, to our knowledge, there are very few published cases of surgical removal of hydatid cysts located in the IVS.2 Only patients with recurrent or inoperable disease should be considered candidates for chemotherapy with albendazole; the recommended standard therapeutic regimen is four cycles of 30 days, each with a dose of 10 mg/kg per day and a rest period of 2 weeks between cycles, in an attempt to avoid toxicosis (18”;. of the patients have adverse effects),6 but the response to this treatment must be evaluated until at least 1 year after therapy is completed. REFERENCES
1. O’Connor F, Tellez G, Montero Hidatidosis cardiaca: a prop6sito quirhrgicamente. Rev Esp Cardiol
C, Nuiiez L, Figuera D. de 10 cases intervenidos 1988;41:97-102.
mass without
1085
a precise def-
2. Ottino G, Villani M, De Paulis R, Trucco G, Viara J. Restoration of atrioventricular conduction after surgical removal of a hydatid cyst of the intraventricular septum. J Thorac Cardiovasc Surg 1987;93:144-7. 3. Oliver JM, Sotillo J, Dominguez F, et al. Two dimensional echocardiographic features of echinococcosis of the heart and great blood vessels. Circulation 1988;78:327-37. 4. Russo G, Tamburino C, Cuscuni J, et al. Cardiac hydatid cyst with clinical features resembling subaortic stenosis. AM HEART J 1989;117:1385-7. 5. Desnos M, Brochet E, Cristofini P, et al. Polivisceral echinococcosis with cardiac involvement imaged by two dimensional echocardiography, computed tomography and nuclear magnetic resonance imaging. Am J Cardiol 1987;59:383-5. 6. Davis A, Dixon H, Pawlowski Z. Multicentre clinical trials of benzimidazole-carbamates in human cystic echinococcosis (phase 2). Bull WHO 1989;67:503-8.
Dynamic left ventricular outflow tract obstruction 4 years after aortic valve replacement Masataka Sata, MD, Shin-ichi Momomura, MD, Katsu Takenaka, MD, Teruhiko Aoyagi, MD, Toshiyuki Takahashi, MD, Takashi Serizawa, MD, and Tsuneaki Sugimoto, MD. Tokyo, Japan
Dynamic left ventricular outflow obstruction is characteristic of hypertrophic obstructive cardiomyopathy. InFrom the Second Department of Internal Medicine, Faculty of Medicine, University of Tokyo. Reprint requests: Masataka Sata, MD, Second Department of Internal Medicine, Faculty of Medicine, University of Tokyo, 7-3-l Hongo, Bunkyoku, Tokyo, 113, Japan. 414/39893
