- Email: [email protected]
Complex arrhythmias in a patient with predominantly right ventricular cardiomyopathy
268
InternationalJournal
ojCardiologv,
19 (1988) 268-271 Elsevier
IJC 06905
Complex arrhythmias in a patient with predominantly right ventricular cardiomyopathy Bortolo Martini, Andrea Nava and Gian Franc0 Buja Department
of Cardiology, Padua Medical School, Italy
(Received 15 July 1987; revision accepted 3 November 1987)
A 23-year-old man presented with ventricular tachycardia. The electrocardiogram revealed right bundle branch block plus right axis deviation. It also showed frequent episodes of asystole, 2: 1 sinuatrial block and couplets of left bundle branch block morphology. Electrophysiologic study demonstrated sinuatrial entrance block, with an HV interval of 65 msec. It was aIso possible to induce sustained ventricular tachycardia of left bundle branch block pattern with normal QRS axis. Subsequent investigations were consistent with the diagnosis of right ventricular cardiomyopathy with mild left ventricular involvement. Key words: syndrome
Arrhythmogenic
right ventricular
dysplasia;
Ventricular
tachycardia;
Sick sinus
Introduction The careful clinical investigation of young patients affected by complex arrhythmias and without evident structural heart disease is failing to substantiate the concept that these are idiopathic. Instead, it is often possible to document concealed pathology in the right ventricle. We describe such a case. Case Report A 23-year-old man was admitted to our hospital because of palpitations. His mother had died suddenly in her forties and he had suffered a syncopal episode 2 years before, while eating. The electrocardiogram showed a ventricular tachycardia with a rate of 270 beats per minute and with QRS morphology indicative of right bundle branch block and right axis deviation (Fig. 1). The arrhythmia did not respond to vagal maneuvres, to verapamil or to intravenous propafenone, but was converted to sinus rhythm by direct current shock. Chest X-rays and blood tests were all normal. During the following days the patient presented frequent asymptomatic episodes of asystole, up to 6 seconds in duration, and episodes of 2 : 1
Correspondence to: Dr. Bortolo Martini, via Gioberti 9, 36016 Thiene (VI). Italy. Supported by: Target Project “Juvenile Sudden Death”, Regione Veneto, Italy.
0167-5273/88/$03.50
0 1988 Elsevier Science Publishers B.V. (Biomedical Division)
269
ig. 1. A. Twelve-lead electrocardiogram during spontaneous ventricular tachycardia, with a pattern of ght bundle branch block and right axis deviation QRS morphology. B. Basic 12-lead electrocardiogram laracterized only by a right axis deviation on frontal plane. C. Sustained ventricular tachycardia. educed during electrophysiologic study, with a pattern of left bundle branch block and normal right xis, QRS pattern. The tachycardia was induced by a single ventricular extrastimulus, with a coupling lterval of 220 msec. D. Spontaneous episode of asystole. The patient also had isolated and paired premature ventricular contractions of left bundle branch block morphology during exercise stress test.
Fig. 2. Ten-degree oblique right anterior view of right ventricle (diastolic frame). The right ventricle is enlarged (150 ml/m* : normal value = 81 f 12.3 ml/m2) with normal ejection fraction (59%; normal value 61+_ 8%). The diaphragmatic region is hypokinetic and a localized area of systolic dyskinesia is seen on the lateral wall.
270 sinuatrial block (Fig. 1). An exercise stress test documented a normal tolerance to effort and frequent premature ventricular contractions and couplets during recovery. These were of left bundle branch block morphology. Cross-sectional echocardiography and angiocardiography showed the right ventricle to be enlarged (150 ml/ m* : normal value = 81 &-12.3 ml/m*) with an ejection fraction of 59% (normal value 61 f 8%). The diaphragmatic region was hypokinetic and a localized area of systolic dyskinesia was detectable on the lateral wall (Fig. 2). The left ventricle was of normal volume and had a normal ejection fraction (83 ml/m2 and 69%, respectively). The anterolateral wall and the apex showed mild dyskinesia. The coronary arteries were normal. Electrophysiological studies following our described protocol [l], showed sinuatrial entrance block, a prolonged HV interval (65 msec) and sustained ventricular tachycardia of left bundle branch block pattern with normal QRS axis (Fig. 1). Endomyocardial biopsy at the right ventricle showed marked interstitial fibrosis. A permanent pacemaker was implanted and the patient was treated with amiodarone 200 mg per day. After 9 months he is totally asymptomatic. Holter monitoring now shows only short episodes of atria1 fibrillation. Discussion This patient, in spite of the poor history and clinical data, had an unusual association of sick sinus syndrome, intraventricular conduction disturbance and spontaneous or induced ventricular arrhythmias of both right and left bundle branch block morphology. Taken together, this was suggestive of significant myocardial disease. Angiographic and histologic data were consistent with right ventricular pathology. It is not easy to classify this predominantly right ventricular cardiomyopathy but, according to its clinical and investigational results, the disease could be a concealed form of arrhythmogenic right ventricular dysplasia [1,2]. The classical form of this disease is characterized by cardiomegaly (caused mostly by right ventricular dilation with reduced ejection fraction), inverted T waves, post-excitation waves and right heart failure. It can be familial but its most characteristic feature is tachycardia with left bundle branch block and right axis deviation. Polymorphism and right bundle branch block have also been described, in a very small number of patients [l-3]. In most of the latter, left ventricular or generalized chamber involvement have been documented. This can explain the right bundle branch block pattern of some ventricular arrhythmias, as well as the electrocardiographic features of sick sinus syndrome, atria1 arrhythmias and conduction disturbances, found in these patients [2-41. The frequency of the arrhythmias listed above is directly related to the severity of the disease, but recent reports frequently describe “concealed” forms of the condition in patients with ventricular arrhythmias and otherwise normal clinical findings [1,2]. The clinical diagnosis in these patients is difficult. Nonetheless, echoand angiographic evidence of predominant right ventricular structural and/or wall motion abnormalities, along with evidence of fibrosis or fibroadiposis, from the biopsy are wellrecognized findings in young patients affected by rhythm disturbance who die suddenly [l,S].
Acknowledgments The authors acknowledge Dr. Bruno Canciani, Professor Gaetano Thiene and Professor Francesco Cucchini, for their important contribution to the investigation of the patient and preparation of the manuscript.
211
References 1 Nava A, Scognamiglio R, Thiene G, et al. A polymorphic form of familial arrhythmogenic right ventricular dysplasia. Am J Cardiol 1987;59:1405-1409. 2 Fauchier JP, Desveaux B, Cosnay P, Raynaud P, Philippe L, Itti R. Troubles du rythme ventriculaire complexes du sujet jeune apparemment win. Arch Ma1 Coeur 1985;78:1333-1343. 3 Fontaine G, Frank R, Guiraudon G, et al. Signification des troubles de conduction intraventriculaires observes dans la dysplasie ventriculaire droite arythmogene. Arch Ma1 Coeur 1984;77:872-879. 4 Webb JG, Kerr CR, Huckell VF, Mizgala HF, Ricci DR. Left ventricular abnormalities in arrhythmogenie right ventricular dysplasia. Am J Cardiol 1986;58:568-570. 5 Thiene G, Nava A, Corrado D, Rossi L, Pennelh N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988;318:129-139.
InternationalJournal
ojCardiologv,
19 (1988) 268-271 Elsevier
IJC 06905
Complex arrhythmias in a patient with predominantly right ventricular cardiomyopathy Bortolo Martini, Andrea Nava and Gian Franc0 Buja Department
of Cardiology, Padua Medical School, Italy
(Received 15 July 1987; revision accepted 3 November 1987)
A 23-year-old man presented with ventricular tachycardia. The electrocardiogram revealed right bundle branch block plus right axis deviation. It also showed frequent episodes of asystole, 2: 1 sinuatrial block and couplets of left bundle branch block morphology. Electrophysiologic study demonstrated sinuatrial entrance block, with an HV interval of 65 msec. It was aIso possible to induce sustained ventricular tachycardia of left bundle branch block pattern with normal QRS axis. Subsequent investigations were consistent with the diagnosis of right ventricular cardiomyopathy with mild left ventricular involvement. Key words: syndrome
Arrhythmogenic
right ventricular
dysplasia;
Ventricular
tachycardia;
Sick sinus
Introduction The careful clinical investigation of young patients affected by complex arrhythmias and without evident structural heart disease is failing to substantiate the concept that these are idiopathic. Instead, it is often possible to document concealed pathology in the right ventricle. We describe such a case. Case Report A 23-year-old man was admitted to our hospital because of palpitations. His mother had died suddenly in her forties and he had suffered a syncopal episode 2 years before, while eating. The electrocardiogram showed a ventricular tachycardia with a rate of 270 beats per minute and with QRS morphology indicative of right bundle branch block and right axis deviation (Fig. 1). The arrhythmia did not respond to vagal maneuvres, to verapamil or to intravenous propafenone, but was converted to sinus rhythm by direct current shock. Chest X-rays and blood tests were all normal. During the following days the patient presented frequent asymptomatic episodes of asystole, up to 6 seconds in duration, and episodes of 2 : 1
Correspondence to: Dr. Bortolo Martini, via Gioberti 9, 36016 Thiene (VI). Italy. Supported by: Target Project “Juvenile Sudden Death”, Regione Veneto, Italy.
0167-5273/88/$03.50
0 1988 Elsevier Science Publishers B.V. (Biomedical Division)
269
ig. 1. A. Twelve-lead electrocardiogram during spontaneous ventricular tachycardia, with a pattern of ght bundle branch block and right axis deviation QRS morphology. B. Basic 12-lead electrocardiogram laracterized only by a right axis deviation on frontal plane. C. Sustained ventricular tachycardia. educed during electrophysiologic study, with a pattern of left bundle branch block and normal right xis, QRS pattern. The tachycardia was induced by a single ventricular extrastimulus, with a coupling lterval of 220 msec. D. Spontaneous episode of asystole. The patient also had isolated and paired premature ventricular contractions of left bundle branch block morphology during exercise stress test.
Fig. 2. Ten-degree oblique right anterior view of right ventricle (diastolic frame). The right ventricle is enlarged (150 ml/m* : normal value = 81 f 12.3 ml/m2) with normal ejection fraction (59%; normal value 61+_ 8%). The diaphragmatic region is hypokinetic and a localized area of systolic dyskinesia is seen on the lateral wall.
270 sinuatrial block (Fig. 1). An exercise stress test documented a normal tolerance to effort and frequent premature ventricular contractions and couplets during recovery. These were of left bundle branch block morphology. Cross-sectional echocardiography and angiocardiography showed the right ventricle to be enlarged (150 ml/ m* : normal value = 81 &-12.3 ml/m*) with an ejection fraction of 59% (normal value 61 f 8%). The diaphragmatic region was hypokinetic and a localized area of systolic dyskinesia was detectable on the lateral wall (Fig. 2). The left ventricle was of normal volume and had a normal ejection fraction (83 ml/m2 and 69%, respectively). The anterolateral wall and the apex showed mild dyskinesia. The coronary arteries were normal. Electrophysiological studies following our described protocol [l], showed sinuatrial entrance block, a prolonged HV interval (65 msec) and sustained ventricular tachycardia of left bundle branch block pattern with normal QRS axis (Fig. 1). Endomyocardial biopsy at the right ventricle showed marked interstitial fibrosis. A permanent pacemaker was implanted and the patient was treated with amiodarone 200 mg per day. After 9 months he is totally asymptomatic. Holter monitoring now shows only short episodes of atria1 fibrillation. Discussion This patient, in spite of the poor history and clinical data, had an unusual association of sick sinus syndrome, intraventricular conduction disturbance and spontaneous or induced ventricular arrhythmias of both right and left bundle branch block morphology. Taken together, this was suggestive of significant myocardial disease. Angiographic and histologic data were consistent with right ventricular pathology. It is not easy to classify this predominantly right ventricular cardiomyopathy but, according to its clinical and investigational results, the disease could be a concealed form of arrhythmogenic right ventricular dysplasia [1,2]. The classical form of this disease is characterized by cardiomegaly (caused mostly by right ventricular dilation with reduced ejection fraction), inverted T waves, post-excitation waves and right heart failure. It can be familial but its most characteristic feature is tachycardia with left bundle branch block and right axis deviation. Polymorphism and right bundle branch block have also been described, in a very small number of patients [l-3]. In most of the latter, left ventricular or generalized chamber involvement have been documented. This can explain the right bundle branch block pattern of some ventricular arrhythmias, as well as the electrocardiographic features of sick sinus syndrome, atria1 arrhythmias and conduction disturbances, found in these patients [2-41. The frequency of the arrhythmias listed above is directly related to the severity of the disease, but recent reports frequently describe “concealed” forms of the condition in patients with ventricular arrhythmias and otherwise normal clinical findings [1,2]. The clinical diagnosis in these patients is difficult. Nonetheless, echoand angiographic evidence of predominant right ventricular structural and/or wall motion abnormalities, along with evidence of fibrosis or fibroadiposis, from the biopsy are wellrecognized findings in young patients affected by rhythm disturbance who die suddenly [l,S].
Acknowledgments The authors acknowledge Dr. Bruno Canciani, Professor Gaetano Thiene and Professor Francesco Cucchini, for their important contribution to the investigation of the patient and preparation of the manuscript.
211
References 1 Nava A, Scognamiglio R, Thiene G, et al. A polymorphic form of familial arrhythmogenic right ventricular dysplasia. Am J Cardiol 1987;59:1405-1409. 2 Fauchier JP, Desveaux B, Cosnay P, Raynaud P, Philippe L, Itti R. Troubles du rythme ventriculaire complexes du sujet jeune apparemment win. Arch Ma1 Coeur 1985;78:1333-1343. 3 Fontaine G, Frank R, Guiraudon G, et al. Signification des troubles de conduction intraventriculaires observes dans la dysplasie ventriculaire droite arythmogene. Arch Ma1 Coeur 1984;77:872-879. 4 Webb JG, Kerr CR, Huckell VF, Mizgala HF, Ricci DR. Left ventricular abnormalities in arrhythmogenie right ventricular dysplasia. Am J Cardiol 1986;58:568-570. 5 Thiene G, Nava A, Corrado D, Rossi L, Pennelh N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988;318:129-139.