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Congenital diverticulum of the right ventricle: Report of two cases associated with other congenital heart defects
Volume
117
Number
4
8.
9.
10.
Brief
plications involving the Ionescu-Shiley pericardial valve in the mitral position. J Am Co11 Cardiol 1984;3:328-33. Nagata S, Park Y-D, Nagae K, Beppu S, Kawaxoe K, Fujita T, Sakakibara H, Nimura Y. Echocardiographic features of bioprosthetic valve endocarditis. Br Heart J 1984;51:263-6. Alam M, Rosman HS, Lakier JB, Kemp S, Khaja F, Hautamaki K, Magilligan DJ Jr, Stein PD. Doppler and echocardiographic features of normal and dysfunctioning bioprosthetic valves. J Am Co11 Cardiol 1987;10:851-8, Figure 3. Pechacek LW, Solana LG, DeCastro CM, Edelman SK, Garcia E, Hall RJ. Noninvasive assessment of the IonescuShiley pericardial xenograft in the mitral position: preliminary experience. Tex Heart Inst J 1982;9:275-84.
Patrick Hallali, MD, Bernard Iung, MD, Alain Davido, MD, Jean-Paul Binet, MD, Henri Leriche, MD, and Pierre Corone, MD. France
Congenital cardiac diverticula, first describedby O’Bryan, are rare, their incidence being estimated at 0.013%.l The present report concernstwo casesof congenital muscular diverticula arising from the baseof the right ventricle, one associatedwith tetralogy of Fallot, the other with ventricular septal defect. Case No. 1. A Z-month-old boy wasreferred for the first time becauseof cyanosis while crying. Clinical examination was consistent with the diagnosis of tetralogy of Fallot, but chest x-ray films showedcardiomegaly (cardiothoracic ratio 0.73) with significant prominence of the right cardiac border. Electrocardiogram (ECG) showed right bundle branch block. Echocardiography revealed a large perimembranousseptal defect associatedwith pulFrom the Department of Medicine and the Department of Cardiac Lannelongue, Le Plessis-Robinson. Reprint requests: Patrick Hallali, Mbdecine, 83 Blvd. de l’H8pital,
Table
and Cardiology, HBpital la Pitie, Paris; Surgery, Centre Chirurgical MarieMD, HBpital 75651 Paris
de la Pitie, 13, France.
Consultation
stenosis, suggesting a diagnosis of tetralogy of Fallot. The radiologic and electrical findings, however, were atypical. Cyanosis became more marked over the subsequent17 months, at which time catheterization and angiocardiography were performed (Table I), confirming an atypical tetralogy of Fallot with a large perimembranousventricular septal defect with a right-to-left shunt, a tight infundibular and valvular pulmonary stenosis,and the existence of a large diverticulum arising from the base of the right ventricle anterior to the tricuspid ring compressing the right atrium and displacing the right coronary artery, two branches of which encircled the baseof
monary
de
and the intraluminal pressureswere equal (Fig. 1). After a further period of 2 years, increasing cyanosisand polycythemia indicated that surgical treatment was necessary. At operation (Fig. Z), the diverticulum was large (diameter 50 mm) and wassituated on the anterior wall of the right ventricle in front of the tricuspid annulus, opposite the ventricular septal defect. Surgical repair included ventricular septal defect closurewith a patch, pulmonary valvotomy, infundibular enlargementwith a patch, resection of most of the diverticulum leaving only 15 mm, and closure with a Teflon patch. The microscopic examination of the resectedspecimenrevealed an absent epicardium replaced by a fibrous shell, a hypertrophic myocardium with an atypical plexiform structure causing a deformity of the endocardium, and a fibrous and thickened endocardium without thrombosis. The postoperative course was uneventful and at 6 months postoperatively the child was asymptomatic and acyanotic. Case No. 2. A 3-week-old boy with a cardiac murmur wasreferred becauseof failure to thrive. Clinical examination suggestedthe presenceof a ventricular septal defect. Chest x-ray films demonstrated cardiomegaly (cardiothoracic ratio 0.64) with increasedpulmonary vascular markings. ECG showedright bundle branch block. Echocardiography showed a large ventricular septal defect in the muscular portion of the septum. The findings at cardiac catheterization at the age of 5 months are detailed in Table I. Angiography confirmed a large ventricular septal defect and demonstrated a large contractile and trabeculated diverticulum of the anterolateral wall of the right
I. Cardiac catheterization data Case No. 2
CaseNo. 1 Pressure (mm Hg) Right atrium (mean) Right ventricle Pulmonary artery Pulmonary artery wedge (mean) Left atrium Left ventricle
6 8612-g la/8 iz 8 8413-8
957
the diverticulum. Contraction of the wall of the diverticulum was vigorous and synchronous with the right ventricle
Congenital diverticulum of the right ventricle: Report of two cases associated with other congenital heart defects
Paris and Le Plessis-Robinson,
Communications
Oxygen saturation (%o) 60 63 58 97 89
Pressure (mm Hd 4 8010 78128 = 12 82/O
Oxygen saturation (%) 54 89 89 96
April
958
Brief
Communications
Fig. 1. Right ventricular angiogram of caseNo. 1. Left anterior oblique view showing the diverticulum arising from the baseof the right ventricle. Contrast material is injected selectively into the diverticulum. The opacified blood flows sparingly into the right ventricle and opacifies the left ventricle and the aorta through the ventricular septal defect (right-to-left shunt). AO, Aorta; D, diverticulum; RV, right ventricle; LV, left ventricle.
Fig. 2. Operative view of caseNo. 1. The diverticulum is shown by the finger of the surgeon.It appearsat the base of the anterior wall of the right ventricle.
ventricle (Fig. 3). At the age of 6 months, a deteriorating clinical status in spite of treatment with digitalis and a diuretic indicated the need for surgical intervention. At operation, the diverticulum (size 25 x 15 mm) was connected to the right ventricle by a single opening. It was neither incisednor resected.The ventricular septal defect
Amer#can
Heart
1989 Journal
Fig. 3. Right ventricular angiogramof caseNo. 2. Frontal view showingthe diverticulum connectedto the infundibulum. Contrast material is injected into the right ventricle. D, Diverticulum; IF, pulmonary infundibulum; PT, pulmonary trunk.
was closed by a Weavnit patch. There was a marked postoperative clinical improvement that was correlated with a decreasein the cardiothoracic ratio to 0.58. Comment. It is important to define the terminology of cardiac sacculardeformities. Two different entities can be described. In the first are aneurysms that consist of fibrous saccular lesions with paradoxical wall motion, with or without myocardial fibers. True aneurysm contains thinned myocardium intermingled with fibrous tissue.The classicalcasedescribed by Chessleret a1.3is the subvalvular aneurysm of the left ventricle in the Bantu. On the other hand are cardiac diverticula, the wall of which contain three intact layers (endocardium, myocardium, and pericardium) and which contract normally.2 Most often they are apical, as in the syndrome described by Cantrell et a1.,4which combines mid-line thoracoabdominal lesionsand congenital heart disease. Muscular diverticula without extracardiac anomalies are infrequent. They may arisefrom the left, most often at the apex, or from the right ventricle. In the latter, they are often associatedwith congenital cyanotic heart disease. Involvement of both ventricles is rare.” Hoeffel” reported in 1974 from a survey of the literature 29 cases of congenital ventricular diverticula without extracardiac anomalies. In 12 cases,the right ventricle was involved and eight of these were associatedwith cyanotic congenital heart disease(half being tetralogy of Fallot). In our two cases,the diagnosiswasvery difficult prior to angiography, asin the casesdescribedin the literature.2,“-’
Volume Number
117 4
Some anomalies may alert the physician; for example, unusual enlargementof the right heart border on radiologic examination.sp7 ECG showedright bundle branch block in our two cases,although the classicalfinding in tetralogy of Fallot as well as in large ventricular septal defect is either right ventricular hypertrophy or biventricular hypertrophy. Right bundle branch block may suggest unusual enlargement or dilatation of the right ventricle, indicating the existenceof the diverticuium. Such findings have been reported2 in other casesof diverticulum of the right ventricle. Although echocardiography can help diagnose diverticula of the left ventricle,9 we have not found echocardiographicdescriptions of right ventricular diverticula. In our two cases,echocardiography wasnot helpful in the diagnosis,probably becauseof the location of the diverticula. Angiography usually establishesthe diagnosis, as it demonstrates the site of the diverticulum, its relationship to the tricuspid ring and the right coron&y artery, and its contractile nature.6,7 In 9 of the 12 casesreported by Hoeffel,2 the outcome was unfavorable but seemedto be related more to the associatedheart diseasethan to the complications due to the diverticulum itself.6l7It seemsthat isolateddiverticula are less prone to complications such as emboli, heart failure, and diverticular rupture becauseof their thickness and their contractile nature.8 In casesof isolated diverticula or incidental occurrencewith congenital heart disease, the indication for surgery is based on the presence or absence of coexisting anomalies.*There is no uniform surgical approach toward diverticula. They can be left in place or resected at the time of coincidental surgery, asin our first case and the case of Carter and associates.? The choice depends on the site of the diverticulum, its anatomic connections, and the importance and the quality of the remaining right ventricular myocardium. In our secondcase, the surgeon left the diverticulum in place becauseof its small size and the poor quantity of right ventricle myocardium remaining after resection of the diverticulum. Histologically, endocardial fibrosis has beenreported6v7severaltimes in musculardiverticulum of the right ventricle. The myocardium of the diverticulum may be normal7 or may be thickened with fibroelastosis.G There is no definite embryologic hypothesis in the etiology of diverticula without extracardiac anomalies. The possiblerole of a shunt through a ventricular septal defect during organogenesisand the role of stretching or ballooning mechanismson the embryonic ventricle have been proposed.6*10Experiments of Dor and Coronel” on chicken heart embryogenesisshowedthat the embryonic heart, encountering an obstacle such as a fibrous band, can develop a musculardiverticulum. Theseobstaclesmay disappear during development and therefore may not be found at operation. This hypothesis underlines the possible role of environmental factors in the etiology of congenital heart disease. REFERENCES
1. Keith JD, Rowe RW, Vlad P. Incidence of various congenital heart defects. In: Keith JD, ed. Heart disease in infancy and
Brief Communications 959 childhood. 3rd ed. New-York Toronto: Mac Millan Publishing Co, 19’78:4-6. 2. Hoeffel JC. Les diverticules ventriculaires cong&nitaux du coeur. Med Infantil 1974;81:863-86. 3. Chesler E, Tucker RB, Barlow JB. Subvalvular and apical left ventricular aneurysms in the Bantu as a source of systemic emboli. Circulation 1967;35:1156-62. 4. Cantrell JR, Haller AJ, Ravich MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602-14. 5. Cumming GR. Congenital diverticulum of the right ventricle. Am J Cardiol 1969;23:294-7. 6. Bharati S, Rowen M, Camarata SJ, Ostermiller WE, Singer M, Lev M. Diverticulum of the right ventricle. Arch Path01 1975;99:383-6. 7. Carter JB, Van Tassel RA, Moller JH, Amplatz K, Edwards JE. Congenital diverticulum of the right ventricle. Association with pulmonary stenosis and ventricular septal defect. Am J Cardiol 1971;28:478-82. 8. Baltaxe HA, Wilson WJ, Amiel M. Diverticulosis of the left ventricle. AJR 1979;133:257-61. 9. Mardini MK. Congenital diverticulum of the left ventricle. Report of two unusual cases. Br Heart J 1984;51;321-6. 10. Dor X, Corone P. Embryologie normale et g&&e des cardiopathies congbnitales. Encycl Med Chir Paris: Coeur-vaisseaux, 1981:llOOlc 20, 14-15.
Ebstein’s anomaly with ventricular tachycardia: Evidence for the arrhythmogenic role of the atrialized ventricle Huey-Ming Lo, MD, Fang-Yue Lin, MD,* Yuh-Shiun Jong, MD, Yting-Zu Tseng, MD, and Teh-Lu Wu, MD. Taipei, Taiwan, Republic of China In patients with Ebstein’s anomaly, paroxysmal supraventricular arrhythmias have generally been thought to be a problem, but recently ventricular tachycardia (VT) has been reported’ to be responsiblefor sudden death, especially in postoperative patients. This is the first caseof VT responsiblefor symptomsin unoperated Ebstein’s anomaly, and the atrialized ventricle was found to be the arrhythmogenic site of the VT, which was successfully ablated with cryosurgery. A 49-year-old man patient washospitalized on September 30, 1987, becauseof frequent attacks of palpitation that he had experiencedsince the ageof 20 years. He had previously been admitted to another hospital six times during the period of 1970to 1986 for the sameproblem, and in each casethe electrocardiogram (ECG) discloseda wide QRS tachycardia (Fig. 1) that was refractory to carotid sinus massage and intravenous injections of
From the Department of Medicine, Taiwan Provincial Tao-Yuan General Hospital, and the Departments of Medicine and Surgery,* College of Medicine, National Taiwan University. Reprint
requests:
Huey-Ming
Lo, MD, Department
of Medicine,
Taiwan
Provincial Tao-Yuan General Hospital, 1492 Chung-Shan Road, TaoYuan, Taiwan, Republic of China.
117
Number
4
8.
9.
10.
Brief
plications involving the Ionescu-Shiley pericardial valve in the mitral position. J Am Co11 Cardiol 1984;3:328-33. Nagata S, Park Y-D, Nagae K, Beppu S, Kawaxoe K, Fujita T, Sakakibara H, Nimura Y. Echocardiographic features of bioprosthetic valve endocarditis. Br Heart J 1984;51:263-6. Alam M, Rosman HS, Lakier JB, Kemp S, Khaja F, Hautamaki K, Magilligan DJ Jr, Stein PD. Doppler and echocardiographic features of normal and dysfunctioning bioprosthetic valves. J Am Co11 Cardiol 1987;10:851-8, Figure 3. Pechacek LW, Solana LG, DeCastro CM, Edelman SK, Garcia E, Hall RJ. Noninvasive assessment of the IonescuShiley pericardial xenograft in the mitral position: preliminary experience. Tex Heart Inst J 1982;9:275-84.
Patrick Hallali, MD, Bernard Iung, MD, Alain Davido, MD, Jean-Paul Binet, MD, Henri Leriche, MD, and Pierre Corone, MD. France
Congenital cardiac diverticula, first describedby O’Bryan, are rare, their incidence being estimated at 0.013%.l The present report concernstwo casesof congenital muscular diverticula arising from the baseof the right ventricle, one associatedwith tetralogy of Fallot, the other with ventricular septal defect. Case No. 1. A Z-month-old boy wasreferred for the first time becauseof cyanosis while crying. Clinical examination was consistent with the diagnosis of tetralogy of Fallot, but chest x-ray films showedcardiomegaly (cardiothoracic ratio 0.73) with significant prominence of the right cardiac border. Electrocardiogram (ECG) showed right bundle branch block. Echocardiography revealed a large perimembranousseptal defect associatedwith pulFrom the Department of Medicine and the Department of Cardiac Lannelongue, Le Plessis-Robinson. Reprint requests: Patrick Hallali, Mbdecine, 83 Blvd. de l’H8pital,
Table
and Cardiology, HBpital la Pitie, Paris; Surgery, Centre Chirurgical MarieMD, HBpital 75651 Paris
de la Pitie, 13, France.
Consultation
stenosis, suggesting a diagnosis of tetralogy of Fallot. The radiologic and electrical findings, however, were atypical. Cyanosis became more marked over the subsequent17 months, at which time catheterization and angiocardiography were performed (Table I), confirming an atypical tetralogy of Fallot with a large perimembranousventricular septal defect with a right-to-left shunt, a tight infundibular and valvular pulmonary stenosis,and the existence of a large diverticulum arising from the base of the right ventricle anterior to the tricuspid ring compressing the right atrium and displacing the right coronary artery, two branches of which encircled the baseof
monary
de
and the intraluminal pressureswere equal (Fig. 1). After a further period of 2 years, increasing cyanosisand polycythemia indicated that surgical treatment was necessary. At operation (Fig. Z), the diverticulum was large (diameter 50 mm) and wassituated on the anterior wall of the right ventricle in front of the tricuspid annulus, opposite the ventricular septal defect. Surgical repair included ventricular septal defect closurewith a patch, pulmonary valvotomy, infundibular enlargementwith a patch, resection of most of the diverticulum leaving only 15 mm, and closure with a Teflon patch. The microscopic examination of the resectedspecimenrevealed an absent epicardium replaced by a fibrous shell, a hypertrophic myocardium with an atypical plexiform structure causing a deformity of the endocardium, and a fibrous and thickened endocardium without thrombosis. The postoperative course was uneventful and at 6 months postoperatively the child was asymptomatic and acyanotic. Case No. 2. A 3-week-old boy with a cardiac murmur wasreferred becauseof failure to thrive. Clinical examination suggestedthe presenceof a ventricular septal defect. Chest x-ray films demonstrated cardiomegaly (cardiothoracic ratio 0.64) with increasedpulmonary vascular markings. ECG showedright bundle branch block. Echocardiography showed a large ventricular septal defect in the muscular portion of the septum. The findings at cardiac catheterization at the age of 5 months are detailed in Table I. Angiography confirmed a large ventricular septal defect and demonstrated a large contractile and trabeculated diverticulum of the anterolateral wall of the right
I. Cardiac catheterization data Case No. 2
CaseNo. 1 Pressure (mm Hg) Right atrium (mean) Right ventricle Pulmonary artery Pulmonary artery wedge (mean) Left atrium Left ventricle
6 8612-g la/8 iz 8 8413-8
957
the diverticulum. Contraction of the wall of the diverticulum was vigorous and synchronous with the right ventricle
Congenital diverticulum of the right ventricle: Report of two cases associated with other congenital heart defects
Paris and Le Plessis-Robinson,
Communications
Oxygen saturation (%o) 60 63 58 97 89
Pressure (mm Hd 4 8010 78128 = 12 82/O
Oxygen saturation (%) 54 89 89 96
April
958
Brief
Communications
Fig. 1. Right ventricular angiogram of caseNo. 1. Left anterior oblique view showing the diverticulum arising from the baseof the right ventricle. Contrast material is injected selectively into the diverticulum. The opacified blood flows sparingly into the right ventricle and opacifies the left ventricle and the aorta through the ventricular septal defect (right-to-left shunt). AO, Aorta; D, diverticulum; RV, right ventricle; LV, left ventricle.
Fig. 2. Operative view of caseNo. 1. The diverticulum is shown by the finger of the surgeon.It appearsat the base of the anterior wall of the right ventricle.
ventricle (Fig. 3). At the age of 6 months, a deteriorating clinical status in spite of treatment with digitalis and a diuretic indicated the need for surgical intervention. At operation, the diverticulum (size 25 x 15 mm) was connected to the right ventricle by a single opening. It was neither incisednor resected.The ventricular septal defect
Amer#can
Heart
1989 Journal
Fig. 3. Right ventricular angiogramof caseNo. 2. Frontal view showingthe diverticulum connectedto the infundibulum. Contrast material is injected into the right ventricle. D, Diverticulum; IF, pulmonary infundibulum; PT, pulmonary trunk.
was closed by a Weavnit patch. There was a marked postoperative clinical improvement that was correlated with a decreasein the cardiothoracic ratio to 0.58. Comment. It is important to define the terminology of cardiac sacculardeformities. Two different entities can be described. In the first are aneurysms that consist of fibrous saccular lesions with paradoxical wall motion, with or without myocardial fibers. True aneurysm contains thinned myocardium intermingled with fibrous tissue.The classicalcasedescribed by Chessleret a1.3is the subvalvular aneurysm of the left ventricle in the Bantu. On the other hand are cardiac diverticula, the wall of which contain three intact layers (endocardium, myocardium, and pericardium) and which contract normally.2 Most often they are apical, as in the syndrome described by Cantrell et a1.,4which combines mid-line thoracoabdominal lesionsand congenital heart disease. Muscular diverticula without extracardiac anomalies are infrequent. They may arisefrom the left, most often at the apex, or from the right ventricle. In the latter, they are often associatedwith congenital cyanotic heart disease. Involvement of both ventricles is rare.” Hoeffel” reported in 1974 from a survey of the literature 29 cases of congenital ventricular diverticula without extracardiac anomalies. In 12 cases,the right ventricle was involved and eight of these were associatedwith cyanotic congenital heart disease(half being tetralogy of Fallot). In our two cases,the diagnosiswasvery difficult prior to angiography, asin the casesdescribedin the literature.2,“-’
Volume Number
117 4
Some anomalies may alert the physician; for example, unusual enlargementof the right heart border on radiologic examination.sp7 ECG showedright bundle branch block in our two cases,although the classicalfinding in tetralogy of Fallot as well as in large ventricular septal defect is either right ventricular hypertrophy or biventricular hypertrophy. Right bundle branch block may suggest unusual enlargement or dilatation of the right ventricle, indicating the existenceof the diverticuium. Such findings have been reported2 in other casesof diverticulum of the right ventricle. Although echocardiography can help diagnose diverticula of the left ventricle,9 we have not found echocardiographicdescriptions of right ventricular diverticula. In our two cases,echocardiography wasnot helpful in the diagnosis,probably becauseof the location of the diverticula. Angiography usually establishesthe diagnosis, as it demonstrates the site of the diverticulum, its relationship to the tricuspid ring and the right coron&y artery, and its contractile nature.6,7 In 9 of the 12 casesreported by Hoeffel,2 the outcome was unfavorable but seemedto be related more to the associatedheart diseasethan to the complications due to the diverticulum itself.6l7It seemsthat isolateddiverticula are less prone to complications such as emboli, heart failure, and diverticular rupture becauseof their thickness and their contractile nature.8 In casesof isolated diverticula or incidental occurrencewith congenital heart disease, the indication for surgery is based on the presence or absence of coexisting anomalies.*There is no uniform surgical approach toward diverticula. They can be left in place or resected at the time of coincidental surgery, asin our first case and the case of Carter and associates.? The choice depends on the site of the diverticulum, its anatomic connections, and the importance and the quality of the remaining right ventricular myocardium. In our secondcase, the surgeon left the diverticulum in place becauseof its small size and the poor quantity of right ventricle myocardium remaining after resection of the diverticulum. Histologically, endocardial fibrosis has beenreported6v7severaltimes in musculardiverticulum of the right ventricle. The myocardium of the diverticulum may be normal7 or may be thickened with fibroelastosis.G There is no definite embryologic hypothesis in the etiology of diverticula without extracardiac anomalies. The possiblerole of a shunt through a ventricular septal defect during organogenesisand the role of stretching or ballooning mechanismson the embryonic ventricle have been proposed.6*10Experiments of Dor and Coronel” on chicken heart embryogenesisshowedthat the embryonic heart, encountering an obstacle such as a fibrous band, can develop a musculardiverticulum. Theseobstaclesmay disappear during development and therefore may not be found at operation. This hypothesis underlines the possible role of environmental factors in the etiology of congenital heart disease. REFERENCES
1. Keith JD, Rowe RW, Vlad P. Incidence of various congenital heart defects. In: Keith JD, ed. Heart disease in infancy and
Brief Communications 959 childhood. 3rd ed. New-York Toronto: Mac Millan Publishing Co, 19’78:4-6. 2. Hoeffel JC. Les diverticules ventriculaires cong&nitaux du coeur. Med Infantil 1974;81:863-86. 3. Chesler E, Tucker RB, Barlow JB. Subvalvular and apical left ventricular aneurysms in the Bantu as a source of systemic emboli. Circulation 1967;35:1156-62. 4. Cantrell JR, Haller AJ, Ravich MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602-14. 5. Cumming GR. Congenital diverticulum of the right ventricle. Am J Cardiol 1969;23:294-7. 6. Bharati S, Rowen M, Camarata SJ, Ostermiller WE, Singer M, Lev M. Diverticulum of the right ventricle. Arch Path01 1975;99:383-6. 7. Carter JB, Van Tassel RA, Moller JH, Amplatz K, Edwards JE. Congenital diverticulum of the right ventricle. Association with pulmonary stenosis and ventricular septal defect. Am J Cardiol 1971;28:478-82. 8. Baltaxe HA, Wilson WJ, Amiel M. Diverticulosis of the left ventricle. AJR 1979;133:257-61. 9. Mardini MK. Congenital diverticulum of the left ventricle. Report of two unusual cases. Br Heart J 1984;51;321-6. 10. Dor X, Corone P. Embryologie normale et g&&e des cardiopathies congbnitales. Encycl Med Chir Paris: Coeur-vaisseaux, 1981:llOOlc 20, 14-15.
Ebstein’s anomaly with ventricular tachycardia: Evidence for the arrhythmogenic role of the atrialized ventricle Huey-Ming Lo, MD, Fang-Yue Lin, MD,* Yuh-Shiun Jong, MD, Yting-Zu Tseng, MD, and Teh-Lu Wu, MD. Taipei, Taiwan, Republic of China In patients with Ebstein’s anomaly, paroxysmal supraventricular arrhythmias have generally been thought to be a problem, but recently ventricular tachycardia (VT) has been reported’ to be responsiblefor sudden death, especially in postoperative patients. This is the first caseof VT responsiblefor symptomsin unoperated Ebstein’s anomaly, and the atrialized ventricle was found to be the arrhythmogenic site of the VT, which was successfully ablated with cryosurgery. A 49-year-old man patient washospitalized on September 30, 1987, becauseof frequent attacks of palpitation that he had experiencedsince the ageof 20 years. He had previously been admitted to another hospital six times during the period of 1970to 1986 for the sameproblem, and in each casethe electrocardiogram (ECG) discloseda wide QRS tachycardia (Fig. 1) that was refractory to carotid sinus massage and intravenous injections of
From the Department of Medicine, Taiwan Provincial Tao-Yuan General Hospital, and the Departments of Medicine and Surgery,* College of Medicine, National Taiwan University. Reprint
requests:
Huey-Ming
Lo, MD, Department
of Medicine,
Taiwan
Provincial Tao-Yuan General Hospital, 1492 Chung-Shan Road, TaoYuan, Taiwan, Republic of China.