EDITORIALS

ROBERT M. JERESATY

Hartford, Connecticut

The mitral valve prolapse-click syndrome continues to arouse considerable interest in view of its preva- lence and the features (chest pain, ST-T wave changes, arrhythmias and sudden death) it shares with coronary artery disease.1-3 Sudden death, a common manifestation of coronary artery disease, is fortunately uncommon in this syndrome. The report by Ritchie et al.* in this issue of the Journal docu- ments a probable mechanism for sudden death in this syndrome, namely, ventricular tachycardia and fibrillation. To my knowledge only seven cases of sudden death, including two of mine, have previously been reported. 1-3,5-7 I now have data on five more cases, three through the courtesy of Drs. Jesse E. Ed- wards and Richard Guthries and the following two additional cases of my own (total of four cases in a consecutive series of 240 patients).

Case 1: A 40 year old man died suddenly at his work desk on April 11,1973. I had last seen him in 1964 for chest pain, palpitations, lightheadedness and attacks of presyn- cope. He subsequently had heart failure and was given digi- talis. A pansystolic murmur with late systolic accentuation was heard, but no click was noted. The electrocardiogram revealed atria1 fibrillation, multiple premature ventricular beats, normal Q-T interval, T wave inversion in leads I, II, aVF, Vd to Ve and left ventricular hypertrophy. Chest X-ray films showed mild to moderate cardiomegaly. At car- diac catheterization, the left ventricular end-diastolic pres- sure was 14 mm Hg. Left ventriculography showed massive mitral valve prolapse (Fig. 21 in Ref. 3). No autopsy was performed.

Case 2: A 39 year old woman was found unconscious on June 27, 1974 by her husband, who initiated cardiopulmo- nary resuscitation. In the emergency room, she was found to have ventricular fibrillation and, although conversion to normal sinus rhythm was accomplished, she died 2 days later of irreversible brain damage. She had been essentially asymptomatic according to her husband and her only med- ication had been Lasix,@ self-administered for “fluid reten- tion.” The serum potassium level on admission was 3 mEq/ liter. Multiple systolic clicks and a grade 3/6 late systolic

murmur were heard and recorded in 1965. The electrocar- diogram had revealed normal sinus rhythm, no premature ventricular beats, a normal Q-T interval and T wave inver- sion in leads II, III and aVF. Chest X-ray films disclosed a normal heart size. Cardiac catheterization had revealed a left ventricular end-diastolic pressure of 8 mm Hg. Left cineventriculography showed mitral valve prolapse and mild mitral insufficiency. At autopsy both mitral leaflets were redundant, hooded and myxomatous. Her two chil- dren, aged 16 and 14 years, respectively, had acoustically “silent” mitral valve prolapse proved by echocardiogra- phy.’

Clinical features: Nine of the 12 patients who died sud- denly were women, thus reflecting the female preponder- ance in this syndrome. Their ages ranged from 20 to 63 years (mean 39). Only two patients had chest pain; five had dyspnea and six had syncopal or presyncopal episodes. Only one patient had an isolated click described as a “very faint systolic click in the base.“8 A late systolic murmur, heard in six patients, was preceded by a click in five; a pan- systolic murmur, heard in four, had late systolic accentua- tion in two.

Electrocardiograms were available in 10 patients and showed ST-T wave changes in the inferior leads in 6, of whom 5 had similar changes in the left precordial leads; atria1 fibrillation was noted in three, and the Q-T interval was prolonged in 2. lv6 Chest X-ray films available in eight patients revealed mild to moderate cardiomegaly in four.

Of four patients who underwent cardiac catheterization only-one was found to have an elevated end-diastolic pres- sure. Of nine whose coronary arteries were examined at au- topsy, only two had coronary artery disease, and in both it was mild. Left ventriculography performed in seven pa- tients demonstrated mitral valve prolapse in all; the pro- lapse was massive in six of the seven. Mild to moderate mi- tral regurgitation was demonstrated in six.

Autopsy examination of the mitral valve was performed in 10 patients and showed redundant, hooded and myxo- matous leaflets in all but one case in which the autopsy was “performed elsewhere.“l Both leaflets were involved in seven cases and the posterior leaflet alone in two. No myo- cardial abnormalities were noted except for friction lesions in two patients8 and interstitial fibrosis in the papillary muscles or the inferobasilar subendocardial areas in three

From the Section of Cardiology, Saint Francis Hospital, and the Uni- patients.5xs versity of Connecticut School of Medicine, Hartford, Conn. From a review of these 12 cases of fatal mitral valve pro-

Address for reprints: Robert M. Jeresaty, MD, Section of Cardiolo- lapse a composite picture of the possible candidate for gy, Saint Francis Hospital, 114 Woodland St., Hartford, Conn. sudden death emerges: a 40 year old woman with a history 06105. of syncopal or presyncopal episodes and dyspnea who has a

Sudden Death in the Mitral Valve Prolapse-Click Syndrome

February 1976 The American Journal of CARDIOLOGY Volume 37 317

EDITORIALS

late systolic murmur preceded by a click or a pansystolic murmur with late systolic accentuation. The electrocardio- gram shows ST-T wave changes in the inferior and left pre- cordial leads and multiple premature ventricular beats. The left ventriculogram displays massive mitral valve pro- lapse with involvement of both leaflets and minimal to moderate mitral insufficiency.

Treatment: Ventricular fibrillation is the probable mechanism of sudden death in this syndrome.3-6 Antiar- rhythmic therapy has been advocated in the management of mitral valve prolapse associated with ventricular irrita- bility. 2,3 Propranolol may be the drug of choice because, in addition to its intrinsic value as an antiarrhythmic agent, it decreases left ventricular contractility, increases left ven- tricular volume and may therefore lessen the myocardial ischemia resulting from excessive traction on the papillary muscles.3~‘0 Quinidine and Dilantin@ have also been used.

In the case reported by Ritchie et a1.4 overdrive pacing was successfully used to prevent arrhythmia in a 56 year old man who had repeated attacks of ventricular fibrilla- tion refractory to antiarrhythmic drug therapy. However, one of Edward’s patient9 died 1 day after her discharge from the hospital where a pacemaker had been implanted. Mitral valve replacement for prevention of life-threatening

arrhythmia was performed in two patients who had ven- tricular fibrillationlo . including a patient of Dr. Paul N. Yu (case not yet reported). It would be almost impossible to document the beneficial effect of this drastic therapy on this rare complication of the mitral valve prolapse syn- drome. It should be considered only in patients with recur- rent episodes of ventricular tachycardia and fibrillation un- responsive to antiarrhythmic therapy and pacing. The risk of sudden death, although remote, may have to be taken into account in weighing surgical indications in patients with mitral valve prolapse and severe mitral regurgitation; it may tilt the balance in favor of surgery in this difficult decision.

In evaluating the cause of sudden death, both general and forensic pathologists “should maintain a high index of suspicion that the death may be related to the ballooning posterior leaflets syndrome.” 7 A review of my series would confirm the assessment of Allen et al.‘l of the benign na- ture of this syndrome. Their series is, unfortunately, dilut- ed by the inclusion of isolated late systolic murmur, a non- specific finding. W1 The risk of sudden death is remote in this syndrome and therefore should not be mentioned to the patient or his family for fear of inducing undue anxiety and neurosis.

References

1. Hancock EW, Cohn K: The syndrome associated with midsys- 6. Shappell SD, Marshall CE, Brown RE, et al: Sudden death and tolic click and late systolic murmur. Am J Med 41:183-196, the familial occurrence of mid-systolic click, late systolic mur- 1966 mur syndrome. Circulation 48: 1128-I 134, 1973

2. Marchand P: Late systolic murmurs and non-ejection (“mid- 7. Marshall CE, Shappell SD: Sudden death and the ballooning late”) systolic clicks: an analysis of 90 patients. Br Heart J 30: posterior leaflet syndrome. Arch Pathol 98:134-138, 1974 203-218, 1968 8. Edwards JE, Guthrie R: Personal communication, June 17,

3. Jeresaty RM: Mitral valve prolapse-click syqdrome. Prog Car- 1974 diovasc Dis 15:623-652, 1973

4. Ritchle JL, Hammermelster KE, Kennedy JW: Refractory ven- 9. Jeresaty RM, Landry AB, Liss JP: Silent mitral valve prolapse:

analysis of 32 cases (abstr). Am J Cardio135: 146, 1975 tr/cular tachycardia and fibrillation in a patient with the prolaps- IO. Cobbs BW, King SB: Mechanism of abnormal ventriculogram ing mitral leaflet syndrome: successful control with overdrive associated with prolapsing mitral valve (abstr). Circulation 49, pacing. Am J Cardiol 37:315-317, 1976 50: Suppl Ill: 111-7, 1974

5. Trent JK, Adelman AC, Wigle ED, et al: Morphology of a pro- Il. Allen H, Harris A, Leatham A: Significance and prognosis of an lapsed posterior mitral valve leaflet. Am Heart J 79x539-543, isolated late systolic murmur: a nine to 22 year follow-up. Br 1970 Heart J 36525-532. 1974

318 February 1976 The American Journal of CARDIOLOGY Volume 37