Refractory ventricular arrhythmias in a patient with mitral valve prolapse. Successful control with mitral valve replacement

J. ELECTROCARDIOLOGY 11 (3), 1978, 289-295

Case Studies MARTIN M. LEWINTER, M.D., EDITOR

Refractory Ventricular Arrhythmias in a Patient with Mitral Valve Prolapse. Successful Control with Mitral Valve Replacement BY ANDREW ROSS, M.D., JAMES A. DEWEESE, M.D., AND PAUL N. YU, M.D.

SUMMARY A 35-year-old w o m a n with proven mitral valve prolapse developed life threatening ventricular a r r h y t h m i a s which were refractory to medical therapy. She had one episode of "cardiac arrest," presumably due to ventrieular tachycardia or possibly ventricular fibrillation, and was s u c c e s s f u l l y r e s u s c i t a t e d with closed chest compression. Mitral valve replacement resulted in dramatic control of the ventrieular arrhythmias. Over a period o f three years following the operation, she has been able to resume an active life with occasional v e n t r i e u l a r p r e m a t u r e beats and no f u r t h e r episodes of ventricular tachyarrhythmias. Mitral valve prolapse - - systolic click, late systolic m u r m u r syndrome - - continues to a r o u s e m u c h i n t e r e s t in v i e w of its freq u e n c y , 1,2 p o t e n t i a l i t y for s u d d e n d e a t h , bacterial endocarditis, r u p t u r e of chordae t e n d i n a e a n d m i t r a l r e g u r g i t a t i o n , s The necessity for m i t r a l valve r e p l a c e m e n t in those patients with marked mitral regurgitation adds another dimension to this disease. 4 The incidence of atrial and ventricular arr h y t h m i a s is s t r i k i n g l y h i g h . 5-9 L i f e threatening arrhythmias, however, in view of the relative frequency of mitral valve prolapse in the general population, are rather uncommon, lO In a recent review of the literature of those patients reported to have died of sudden death in association with the click m u r m u r syndrome, 1~ J e r e s a t y was able to report 12 patients, of whom four were from a consecutive series of 240 patients from his own group. From the Cardiology Unit, Department of Medicine and the Cardiothoracic Division, Department of Surgery, University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital, Rochester, New York. This research was supported in part by Research Training Grant #HL-05500 from the National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland. The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. w solely to indicate this fact. Reprint requests to: Paul N. Yu, M.D., University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY 14642

Clinical features which might differentiate the subgroup of high risk individuals have not yet been elucidated, although inferior ST and T wave changes have been suggested as one c h a r a c t e r i s t i c finding which is associated with high risk of sudden death, s'9 Overdrive pacing 11 has been used as a successful mode of t h e r a p y in one patient with r e f r a c t o r y v e n t r i c u l a r a r r h y t h m i a s . In another case reported by Cobbs, mitral valve replacement was necessary for suppression of life threatening arrhythmias. 12 We present the case of a woman who had refractory and life t h r e a t e n i n g ventricular t a c h y a r r h y t h m i a s which were finally controlled after mitral valve replacement. Over a period of three and a half years after the operation, there has been no recurrence of ventricular arrhythmias and she has been able to lead a normal and active life.

CASE REPORT B.L., a 35-year-old woman from Pittsburgh, Pennsylvania, was first seen in November, 1966, at the age of 24 years with a six-week history of recurrent attacks of lightheadedhess, palpitations and sharp anterior precordial chest pain. Physical examination revealed a tall ast h e n i c w o m a n , h e i g h t 175 cm, w i t h o u t s u b l u x a t i o n of lens, h i g h a r c h e d p a l a t e , arachnodactyly, abnormality of sternum, or hyperextensibility of joints. Blood pressure was 120/70, standing 110/80. There was a grade I/VI systolic ejection m u r m u r , best heard at the lower left sternal border with minimal radiation. No click was demo n s t r a t e d either at r e s t or with isometric hand grip. Electrocardiogram (ECG) showed normal sinus rhythm with a rate of 76 beats per minute and no ST-T wave changes were seen in the inferior leads. The QT interval was normal. The r h y t h m strip showed frequent paired ventricular premature contractions. It was noted t h a t the propensity for ventricular p r e m a t u r e contractions became more marked on the assumption of the upright position. She was treated with a course of Propranolol, 20 mg t.i.d., which was increased over the ensuing months to 40 mg q.i.d.

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Fig. 1. A rhythm strip recorded paired ventricular premature contractions9The QT interval was prolonged, probably due to quinidine effect. Propranolol therapy resulted in marked reduction in the number of ventricular premature contractions seen on the ECG and also in the episodes of lightheadedness. The patient, however, continued to complain of attacks of precordial chest pain. On re-examination in March, 1967, a systolic click was heard. Over the ensuing six years, as her palpitations and precordial chest pain decreased in frequency, she was able to discontinue the propranolol without adverse effects. In March, 1973, because of increasing episodes of lightheadedness associated with ventricular premature contractions recorded on the ECG, propranolol therapy with 20 mg q.i.d, was reinstituted. Despite increasing dosage to 240 mg per day, her activity became increasingly restricted. In November, 1973, while walking up stairs, she experienced the acute onset of severe crushing precordial pain lasting two to three hours, associated with irregularity of heart beats. In J a n u a r y , 1974, she was admitted to

another hospital because of severe precordial pain and palpitations. There was no evidence of acute myocardial infarction. With bed rest the precordial pain subsided, although she continued to have frequent atrial and ventricular a r r h y t h m i a s . An echocardiogram showed the classical pattern of mitral valve prolapse. Quinidine sulphate, 200 mg q four hours was prescribed and subsequently increased to 400 mg q four hours for the control of c o n t i n u i n g a t r i a l a n d v e n t r i c u l a r arrhythmias. Little relief resulted from the medication and she returned to Rochester in July, 1974 for evaluation. A 12-lead ECG recorded prior to the routine examination demonstrated a heart rate of about 60 per minute, markedly prolonged QT interval and infrequent vent r i c u l a r p r e m a t u r e contractions (Fig. 1). While performing isometric hand grip during examination of the heart in the Outpatient Clinic, she acutely collapsed and became unconscious, during which time no heart sounds d. E L E C T R O C A R D I O L O G Y , VOL. 11, NO. 3, 1978

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Fig. 2. Pre-operative 6-hour Holter recording showing sinus rhythm with frequent, multifocal ventricular premature beats. Twelve episodes of ventricular tachycardia were noted during the recording. could be heard and the pulses were imperceptible. The patient was diagnosed to have had a "cardiac arrest." She was successfully resuscitated with closed chest compression with return of heart sounds and arterial pulsations. Unfortunately, because of the emergency situation, no ECG was taken to document the nature of "cardiac arrest," but based upon the clinical manifestations and subsequent recording of s e r i o u s v e n t r i c u l a r t a c h y a r rhythmias, it was felt that she probably had an episode of v e n t r i c u l a r t a c h y c a r d i a or possibly ventricular fibrillation. She was admitted to the Coronary Care Unit where a 12-lead electrocardiogram showed many ventricular premature beats but no evidence of acute myocardial infarction. The serial serum enzyme c h a n g e s were non-diagnostic. A 6-hour Holter recording (Fig. 2) showed frequent multifocal ventricular premature beats, which were seen to fall near the T wave of the previous sinus beats. Twelve transient episodes of ventricular tachycardia with ventricular rate ranging between 104 and 130 beats per minute were noted during the recording. During each episode she complained of palpitations and weakness. Subsequently, she continued to have ventricular tachyarrhythmias associated with marked reduction of blood pressure (systolic blood pressure as J. ELECTROCARDIOLOGY, VOL. 11, NO. 3, 1978

low as 80 mm Hg) and cold extremities, despite sequential administration of oral propranolol, i n t r a v e n o u s lidocaine and oral quinidine. The doses of each drug were as follows: propranolol 40 mg q4h, lidocaine up to 4 mg/minute after a loading dose of 75 mg, and quinidine 400 mg q4h. Using a USCI pacing catheter, overdrive suppression of the cardiac arrhythmias with a pacing rate up to 140 per minute was attempted for a period of 15 m i n u t e s . Despite adequate capture, pacing from either right atrium or right ventricle did not abolish or suppress the frequent ventricular premature beats. An echocardiogram substantiated the clinical diagnosis of mitral valve prolapse with characteristic posterior midsystolic displacement of the mitral valve (Fig. 3). In order to confirm the presence of mitral valve prolapse, to rule out significant coronary disease, and to evaluate left ventricular function, left heart catheterization with left ventriculography in the RAO and LAO projections and coronary angiography were performed. The left ventricular pressure was 120/7 mm Hg. The left ventricular angiogram revealed marked prolapse and ballooning of the mitral valve leaflets into the left atrium (Fig. 4). The predominant prolapse involved

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MV

ECG Fig. 3. M-mode echocardiogram of the mitral valve showing classical mitral valve prolapse, arrow. MV = mitral valve; ECG = electrocardiographic lead II. the posterior leaflet, although in the RAO projection, a moderate degree of prolapse of the anterior leaflet was also observed. The ventricular contractility was normal and ejection fraction rate was estimated to be 70%. Selective coronary angiograms demonstrated no lesions in the left anterior descending and right coronary arteries. The circumflex branch of the left coronary artery was not visualized, while the right coronary artery was noted to be large and to supply the posterior aspect of the left ventricle. In view of the continuing multifocal ventricular arrhythmias and runs of ventricular tachycardia resulting in marked reduction of systemic arterial pressure despite the use of various antiarrhythmic agents, mitral valve replacement with a #75 Cross-Jones mitral valve prosthesis was performed on August 24, 1974. At operation the mitral leaflets and papillary muscles were excised, and both leaflets showed myxomatous degeneration and extensive fibrosis in the posterior papillary muscle. The patient's postoperative course was uneventful except for an episode of minor cardiac failure requiring the administration of digoxin and diuretics. An ECG showed inversion of T wave in the precordial leads. A 6-hour Holter recording obtained prior to discharge r e v e a l e d sinus r h y t h m with inf r e q u e n t p r e m a t u r e v e n t r i c u l a r b e a t s recorded over a period of six hours (Fig. 5). The QT interval was prolonged, probably due to quinidine effect. From September through December, 1974, serial 12-lead ECGs showed T wave inversion

in all precordial and limb leads with the exception of AVR. In January, 1975, the ECG returned to a normal pattern. Subsequently, both the patient's m o t h e r and her only brother were found to have mitral valve prolapse based upon echocardiographic findings. From 1975 to the early part of 1977, she was followed in the Outpatient Clinic on several occasions. She has felt well and has experienced no more irregularity of heart beats. No v e n t r i c u l a r p r e m a t u r e b e a t s were recorded on the rhythm strips. In May, 1977, a 48-hour Holter recording obtained in Pittsburgh, Pennsylvania showed sinus r h y t h m with the presence of occasional ventricular premature beats. In March, 1978, she remained asymptomatic and was able to do all her housework without symptoms.

DISCUSSION In those patients with mitral valve prolapse who have died suddenly, ventricular fibrillation is the most probable cause, s'11'13'14 Antiarrhythmic therapy has been advocated in the m a n a g e m e n t of mitral valve prolapse associated with v e n t r i c u l a r arrhythmias23'15 Propranolol has been used in a n u m b e r of cases. In addition to its intrinsic value as an a n t i - a r r h y t h m i c agent, it m a y reduce the amount of prolapse by decreasing left ventricular contractility and increasing left yentricular volume. There is a possibility that it may lessen the myocardial ischemia resulting J. ELECTROCARDIOLOGY, VOL. 11, NO. 3, 1978

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Fig. 4. Left ventricular angiocardiogram in the RAO (upper panel) and LAO (lower panel) projections. The arrow points to the prolapsing mitral valve. Both angiograms are mid-systolic frames.

from excessive traction on the papillary muscles.12-14 The incidence of non-response to conventional anti-arrhythmic agents is completely unknown. Mitral valve replacement has been r e c o m m e n d e d to a b o l i s h t h e c a r d i a c arrhythmias, as demonstrated in this case and reported elsewhere. TM However, as J e r e s a t y 1~ pointed out, ~it would be almost impossible to document the beneficial effects of this drastic t h e r a p y (mitral valve replacement) on this rare complication of the mitral valve prolapse syndrome." The episode of "cardiac arrest" which developed in the patient while being examined was most likely due to ventricular tachycardia or possibly v e n t r i c u l a r fibrillation, alJ. ELECTROCARDIOLOGY, VOL 11, NO. 3, 1978

though no electrocardiographic confirmation was available. It is possible that she might have had a severe vasovagal reaction, b u t the findings of imperceptible heart sounds and pulse rate and unobtainable blood pressure strongly suggests serious hemodynamic derangement as a consequence of serious ventricular arrhythmias. In addition, the continuation of ventricular arrhythmias after the "cardiac arrest" despite what was considered maximal anti-arrhythmic medication is similar to the case reported by Ritchie, et al. 11 Overdrive pacing was attempted but, despite capture, little effect was noted on the frequency of the ventricular premature beats. W h e t h e r the a b s e n c e of the circumflex b r a n c h of the left coronary a r t e r y w a s a

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Fig. 5. Post-operative 6-hour Holter recording showing sinus rhythm. A total of 12 ventricular premature beats were recorded. causative factor in precipitating the ventricular tachyarrhythmia, as has been suggested by Gentzler, 17 remins unresolved. The reduction in frequency of the ventricular premature beats following operation would appear not to substantiate this as an important factor in this patient. The mechanism whereby mitral valve replacement produced successful reduction in the number of ventricular premature beats remains to be determined. We regret that no sophisticated electrophysiological studies were conducted. Cobb and King TM suggested, based on postoperative study of their patient, that mitral valve prolapse produces abnormal stresses on the supporting papillary muscle and underlying ventricular wall, producing ventricular arrhythmias. The normalization of the post-operative ventriculogram from the preoperative localized inferior wall dyskinesis consonant with reduction of ventricular premature beats was felt to substantiate this point.

To the best of our knowledge, this was the second case report in which refractory ventricular arrhythmias in a patient with mitral valve prolapse were successfully controlled by valve replacement. Fortunately, there is ordinarily no urgent need for surgical intervention to control various types of cardiac arrhythmias in patients with mitral valve prolapse. Nevertheless, mitral valve replacement should be seriously considered as a therapeutic measure to control refractory life threatening ventricular arrhythmias in a patient with such a condition. Acknowledgements: The authors are indebted to Dr. Edward I. Curtiss of Presbyterian-University Hospital, Pittsburgh, Pennsylvania for the report of the 48-hour Holter recording obtained in May, 1977. We gratefully acknowledge the help of Dr. Navin Nanda in the interpretation of the echocardiogram and in the preparation of one of the illustrations. The secretarial assistance J. ELECTROCARDIOLOGY, VOL. 11, NO. 3, 1978

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of Mrs. S u m m e r King, Miss Ann Groff and Mrs. B eth Nestorowycz is deeply appreciated.

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arrhythmias in syndrome of balloon deformity of mitral valve. Definition of possible high risk group. Br Heart J 38:1053, 1976 10. JERESATY, R M: Sudden death in the mitral valve prolapse-click syndrome. Am J Cardiol 37:317, 1976 11. R1TCHIE,J L, HAMMERMEISTER,K E AND KENNEDY, J W: Refractory ventricular tachycardia and fibrillation in a patient with the prolapsing mitral leaflet syndrome: Successful control with overdrive pacing. Am J Cardiol 37:314, 1976 12. COBBS,B W AND KING, S B: Ventricular buckling: A factor in the abnormal ventriculogram and peculiar hemodynamics associated with mitral valve prolapse. Am H eart J 93:741, 1977 13. JERESATY, R M: Mitral valve prolapse-click syndrome. Prog Cardiovasc Dis 15:623, 1973 14. LEWINTER,M M, HOFFMAN,J R, SHELL,W E, KARLINER, J S AND O'ROURKE, R A: Phenyleprine-induced atypical chest pain in patients with prolapsing mitral valve leaflets. Am J Cardiol 34:12, 1974 15. SHAPPELL,S D, MARSHALL,C E, BROWN, R E ANDBRUCE,T A: Sudden death and the familial occurrence of mid-systolic click, late systolic murmur syndrome. Circulation 48:1128, 1973 16. MARCHAND,P: Late systolic murmurs and non-ejection Cmid-late") systolic clicks: An analysis of 90 patients. Br Heart J 30"203, 1968 17. GENTZLER,R D, GAULT, J H, LIEDTKE, A J, MCCANN, W D, MANN, R H AND HUNTER,A S: Congenital absence of the left circumflex coronary artery in the systolic click syndrome. Circulation 52:490, 1975