Catheterization and Cardiovascular Diagnosis 277-85 (1976)
Case Reports
PROLAPSING MITRAL VALVE LEAFLET SYNDROME. A Spectrum That Includes Cleft Posterior Mitral Valve Nicholas 2. Kerin, M.D., Josef Edelstein, M.D., and George Louridas, M.D. Two patients with a prolapse and cleit posterior mltral leaflet were studied. The first case had an associated ostium secundum type atrial septal defect. In both cases, the pansystolic regurgitation of contrast material during anglography correspondedto the pansystolic configuration of the murmur. In each instance, the systolic murmurs displayed a late systolic accentuationduringthe maximal prolapseof the mRral valve. The echocardiographic studies demonstrated only a late systolic prolapse which in both patients correspondedangiocardiographicallyto the maximum buckling of the pansystolic prolapse. Echocardiographlc and angiocardiographic features of cleft posterior mltral valve leaflet are discussed. Key words: billowing mitral valve, midsystolic click and late systollc murmur syndrome, midsystolic click, atrial septal defect, mitral regurgitation, cleft posterlor mitral valve
Since its first description by Barlow and Bosman in 1966 ( I ) , the “prolapsing” mitral valve or “billowing” mitral valve has been a subject of great interest ( 2 4 ) . The association between prolapsing mitral valve and congenital heart disease has seldom been reported (5-7). We have found only one previous report describing a cleft of the posterior leaflet of the mitral valve in combination with a billowing mitral valve (8). This report discusses two cases of prolapsing mitral valve (PMV) associated with a cleft in the posterior leaflet of the mitral valve. MATERIALS AND METHODS
This report includes two cases of prolapsing mitral leaflet syndrome with associated cleft in the posterior leaflet of the mitral valve. Both patients satisfied the following criteria earlier described by Gooch et al. (9): (1) symptoms of chest pain From the Irving 6. Hexter Center, Mount Sinai Hospital, Cleveland, Ohio Dr. Kerin is now at the Sinai Hospital of Detroit. 6767 West Outer Drive. Detroit. Michigan 48235. Address reprint requests to this address. Received Aug. 1 . 1975; revision accepted Oct. 6. 1975
77
@ 1976 Alan R. Liss, Inc., 150 Fifth Avenue, New York,
N.Y. 10011
78
Kerin, Edelstein, and Louridas
and palpitation; (2) arrhythmias, atrial or ventricular demonstrable on electrocardiogram at rest or immediately after exercise; (3) T-wave abnormalities with a broad QRS-T angle; (4) auscultatory findings of midsystolic, late systolic, or pansystolic apical murmur with or without a systolic click. Clinical studies included physical examination and electrocardiographic, echocardiographic , phonocardiographic, and roentgenographic examinations. The phonocardiographic recordings were made using the Cambridge photographic recorder. Cambridge-Leatham microphones were used for recording of the heart sounds, with a medium frequency range. Echocardiograms were obtained by means of a Smith-Kline Ekoline 20 Ultrasonoscope with a 2.25 MH transducer of 0.5 in. diameter. They were displayed on the oscilloscope of the Cambridge Multichannel Recorder simultaneously with an electrocardiogram for timing purposes. Exercise test was performed by means of a motorized treadmill. Each subject walked at progressively greater loads for 4 min at each level until 85% of the age-predicted heart rate was obtained. Right and left heart catheterization was performed in both cases. Left ventriculography was performed with the patient in the right anterior oblique position, and recordings were made on 35 mm film at a speed of 60 fps. Selective coronary cineartenograms were done by the Judkins (first case) and Sones (second case) technique. In the second case, pacing was performed with a no. 7 Bipolar Gorlin catheter from the junction of the superior vena cava and right atrium. Leads I, aVF, and V4 were used to monitor the electrocardiographic response. Multistage atrial paciang was initiated at a rate of 110 b e a t s h i n and additional rates of 134. 142, and 150 beatdmin were used for periods of 2 min each. Electrocardiographic recordings, measurements of left ventricular pressure, and dp/dt were done following each pacing stage. FINDINGS
Case 1 A 51-year-old female was admitted to the Mount Sinai Hospital of Cleveland in July 1972 in right-sided heart failure. There was no past history of rheumatic fever. Since 1968, she complained of atypical chest pain and exertional dyspnea. The pain was not related to exercise and was variable in duration, lasting from a few minutes to half an hour. During June and July of 1972, she noted progressive increase of dyspnea and peripheral edema. Despite adequate treatment with Digoxin and Lasix, the signs of right-sided heart failure worsened. Physical examination revealed an underdeveloped and gracile lady. Her blood pressure was 170/90 mmHg, and her pulse rate was 100/min. The neck veins were distended 5 cm above the clavicle with the patient at 45" and showed marked V waves which were higher than the A waves. A few bronchial rales were heard at the lung bases. No left ventricular impulse was palpable, but a substantial systolic lift was present along the left sternal edge. The first heart sound (S 1) was accentuated at the apex. In the 3rd left intercostal space (LICS) an early diamond-shaped murmur, initiated by a pulmonic ejection click, was present. Neither the murmur nor the narrowly split second heart sound exhibited respiratory variation. At the apex, the anterior axillary line (AAL), and 4th LICS, a grade IV/VI pansystolic murmur with late systolic accentuation and a very prominent midsystolic click (MSC) were
Cleft Posterior Mitral Valve
79
audible. The liver was slightly enlarged due to passive congestion. The electrocardiogram showed sinus rhythm with a frontal QRS axis of plus 90"and an incomplete RBBB. The phonocardiographic (PCG) recordings revealed the described auscultatory findings. The A2 to P2 interval was rlOlmsec, and both components were about equal (Fig. 1). An echocardiogram recorded on July 6, 1973, disclosed the typical late systolic buckling of the posterior and anterior leaflets of the mitral valve. The PCG showed that the MSC preceded the prolapse. During systole, the mitral valve revealed multiple parallel lines. The remainder of the echocardiographic examination revealed a paradoxical septa1 movement and an increased right ventricular dimension of 3 cm (Fig. 2). Cardiac catheterization, which was performed on July 16, 1973, demonstrated a left to right shunt at the atrial level of 2.23 L/min (Table I). The left ventriculogram revealed an inadequate long axis shortening (10). The contraction pattern was normal with symmetrical shortening of the small axis. Throughout systole, a prolapse of the posterior and medial scallops of the posterior leaflet, as well as of the anterior leaflet, was present. In mid- and late systole, the prolapse reached its maximum. A radiolucent area within the prolapse (posterior-medial scallop) marked the location of a cleft. Through the center of this radiolucent area, a reflux of contrast material was seen regurgitating into the left atrium (LA) in systole (Fig. 3). A no. 8 F Rodriguez catheter was advanced from the right atrium to the LA
PEC
,
Fig. 1. Surface phonocardiograms(PCG) were recorded from the 3rd left intercostal space at the left sternal border (LICS) (top tracing) and the anterior axillary line (AAL) (bottom tracing). Right jugular phiebogram is displayed between the two PCG tracings and its components are labeled. An ejection murmur having the characteristicsof a functional murmur is seen in the top tracing, preceded by a pulmonic ejection click (PEC). A midsystolic click (MSC) and a pansystolic murmur (PSM) havlng a late systolic accentuatlon were recorded in AAL (bottom tracing) in a high frequency range.
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Fig. 2. Ultrasound recording of the mitral valve. The septum displayed paradoxical movement. Duringsystole, there was a late systolic prolapse involving both leaflets with multiple duplication of mitral leaflets echoes. The right ventricle (RV) was dilated. RS, right septum; LS, left septum; LV, left ventricle; AMV, anterior mitral valve; PMV, posterior mitral leaflet.
TABLE I. Cardiac Catheterization Data
Site
02
Sat. %
Superior vena cava
I5
Inferior vena cava Mid-right atrium Low-right atrium Right ventricle Pulmonary artery Left atrium
76.5 83 86.5 84 79.5 94
Pressure, mmHg Q p = 5.43 ml/rnin/M2 m=4
Qs = 3.2 ml/min/M2 L-R shunt = 2.23 L/min
65/25 44/18 m = 20 m=2
through a patent ostium secundum defect. An LA injection demonstrated a relatively large atrial septa1 defect (ASD). The coronary cineangiograms demonstrated a dominant, normal right coronary artery. The main left coronary artery (LCA) was of normal length and divided into the left anterior descending artery (LAD) and a small circumflex artery (Cx). The LAD was smooth in outline and gave off two diagonal branches. The LCA and its divisions were completely free of obstructive disease. N o intercoronary circulation was seen.
Cleft Posterior Mitral Valve
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Fig. 3. Left ventricuiogram of patient no. 1, done in a 25” RAO position showing a prolapse of the posterior and anterior mitral leaflets. A nonopaque “notch” marked the location of a cleft in the posteromediai scallop.
On Sept. 9, 1973, the patient underwent open heart surgery, and a medium-sized ostium secundum defect was found. Examination of the mitral valve revealed a prolapse of both leaflets, with a rupture of one chorda tendinea. The chordae sustaining both leaflets were long and lax. In the posterior leaflet, there was a small cleft which permitted a moderate degree of regurgitation. The ASD was sutured, and the mitral insufficiency was corrected by narrowing the valve ring with one mattress suture at its medial commissural area and repairing the cleft with two interrupted sutures. The surgical procedure was well tolerated and the postoperative course was benign. One year after surgical repair the patient has continued to d o well. Case 2 A 53-year-old male was admitted to the hospital on Jan. 9. 1974. with a history of chest pain. In 1969. he first noticed short episodes of nonradiating retrosternal pain accompanied by dyspnea on exertion. Very rarely did he experience pain at rest. Labile hypertension was discovered at the same time. There was a significant family history of arterial hypertension. The physical examination showed a normally developed male with a blood pressure of 170/108 mmHg and a regular pulse of 82/min. The heart was not enlarged by palpation or percussion. On auscultation, a grade 2-3/6, high-pitched pansystolic murmur with late systolic accentuation was audible along the LSB and at the apex. No systolic click was present. The SI was accentuated at the apex. The second heart sound was physiologically split. The remainder of the physical examination
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was unremarkable. The X ray of the chest was normal. The electrocardiogram showed sinus rhythm and nonspecific ST-T changes in the inferior leads. Occasional ventricular premature beats were registered. Echocardiography showed prolapse of the posterior leaflet of the mitral valve in late systole. No duplication of the systolic image of the mitral leaflets was present (Fig. 4). The remainder of the echocardiographic examination revealed normal septal motion and normal right ventricular and left ventricular dimensions. On Nov. 16, 1973, cardiac catheterization revealed normal right atrial, right ventricular, pulmonary arterial, and pulmonary capillary wedge pressures. Left ventricular end diastolic pressure (LVED) was 14 mmHg. Neither angina pectoris nor ST-T changes were elicited during atrial pacing. The resting LVED of 14 mmHg declined to 4 mmHg at the maximum pacing rate. When pacing was discontinued, the LVED rose to 19 mmHg. The dp/dt of the LV declined from 4,480 mmHg/s to 3,400 mmHg/s at the highest pacing rate of 150 b e a t s h i n . After left ventricular angiography (LVA), LVED rose to 21 mmHg. The LVA revealed a slightly increased end systolic volume. The mitral valve showed pansystolic prolapse of the medial scallops of the posterior leaflet. A radiolucent area in the center of the prolapse marked the location of the cleft (Fig. 5). Through the center of the cleft, a fine whiff of contrast material regurgitated in systole into the left atrium. In diastole, the V-shaped defect was well delineated. Coronary cineangiography revealed a normal. short LCA which bifurcated into the LAD and Cx artery. The Cx artery showed a preponderant distribution. This artery was very tortuous but free from obstructive disease. The LAD was a large, smooth vessel and gave off two diagonal branches. The LCA and its divisions were completely free from arteriosclerotic changes. The RCA was a small nondominant artery, which, after a short course, divided into two small terminal right ventricular branches. Just above the bifurcation there was a focal lesion that obstructed about 50% of the lumen. No intercoronary circulation was seen. DISCUSSION
The syndrome produced by prolapse of the posterior leaflet of the mitral valve has been the subject of many comprehensive reports (1-6). The etiological basis has been shown to be heterogeneous. In a series of 200 cases (7), only 22 cases were associated with congenital heart abnormalities. These included secundum atrial septal defect in 17 cases, one Eisenmenger syndrome in a patient with a ventricular septal defect, and four cases of patent ductus arteriosus. The combination of prolapse of the posterior leaflet of the mitral valve and cleft posterior mitral leaflet was only recently reported (8). Cleft Mitral Valve
It is rare to find clefts in the anterior o r posterior mitral leaflets. The scarceness of this lesion was attested to by Davachi et al. (1 I ) who reported no instance among 55 patients with mitral valve abnormalities. Endocardia1 cushion defect consists of a broad spectrum of anomalies ranging from ostium primum defects to a complete persistent atrioventricular canal. Partial or complete cleft of the anterior leaflet of the mitral valve was a frequent finding in combination with a complete or incomplete form of endocardial cushion defect (12). Only a few cases have been reported
Cleft Posterior Mitral Valve
83
in which a cleft anterior mitral valve leaflet was found in combination with secundum type of ASD (8, 13-15). The combination of ostium secundum defect with a cleft posterior leaflet has rarely been described (8, 16-19). The association of PPML with ostium secundum defect was found to be very common. In a recent article, this combination was encountered in 9 out of 43 patients (20). The uniqueness of our first case is in the association of ASD, PPML, and a cleft posterior leaflet, which has only been reported once previously (8). At operation, a moderate ostium secundum type ASD was found. The posterior leaflet of the mitral
I $
I
I
I
i
Fig. 4. Echocardlogramof the mitrai valve revealinga latesystolic prolapseof the posterior leaflet (arrows). The septum displayed normal movement
us
LS
ED
Fig. 5. Left ventricular angiogram of patient no. 2, done In a 30”RAO position, showing a prolapse of the posteromedlai scallop. The arrow points to the prolapsed mitrai leaflet. The nonopaque “notch” is markedwith broken lines. Inearly syrtole(ES), a whiff of contrast material regurgitated into the left atrium through the cleft. in mid- (MS) and late systole (LS), there was less regurgitation seen. The same nonopaque notch was well seen In the end diastolic (ED) frame.
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Kerin, Edelstein, and Louridas
valve was cleft in its lateral position, and the chordae sustaining both leaflets were long and lax. with rupture of one of the chordae supporting the posterior leaflet. It is apparent in this case that the prolapse of the posterior and anterior leaflets was due to multiple defects involving the subvalvular mitral apparatus. These abnormalities involving the mitral valve (deficiency of valve tissue) and subvalvular mitral apparatus (lax and ruptured chordae) were responsible for the regurgitation. An abnormal left axis deviation, the electrocardiographic hallmark of ostium primum defect, was not present in our first case. Echocardiography.This procedure has provided a useful noninvasive technique for the detection of mitral valve prolapse. Echocardiography revealed a typical posterior displacement of both mitral leaflets in the first case and only the posterior leaflet in the second case. I t has been suggested that multiple duplication of the systolic image of the mitral leaflet was caused by redundancies and irregularities of the leaflets as they approximate during systole (21). These multiple systolic echoes were thought to represent clefts in the valve (21). While Case I (Fig. 2) revealed multiple mitral systolic echoes, Case 2 did not (Fig. 4). It is difficult to interpret this sign, since many echocardiograms from normal patients may reveal systolic duplication of the mitral leaflets. Angiocardiography.Only left ventriculography may give a definite assessment of the mitral valve and permit differentiation of patients with endocardia1 cushion defects from those with ostium secundum ASD with mitral valve cleft ( 1 1). The anatomical basis of the angiocardiographic findings is the abnormal insertion of the chordae to the deficient valve tissue (cleft). The “nonopaque notch,” which marks the location of the cleft, is a result of the thickening of the free edge of the cleft at the site of insertion of the chordae tendinae (12,22). The nonopacified defect in the prolapse valve may be seen in both systole and diastole (Figs. 3 3 . The defect may opacify during systole if the leaflets do not coapt and if the contrast regurgitates into the left atrium (Fig. 5A). Differentiation of a prolapsing cleft mitral valve leaflet from normal indentations located at the junctions of the mitral valve scallops is based upon the central position of the cleft within the scallop and possible entrapment of opaque material in both sides of the cleft within the scallop during diastole (Fig. 5). In both cases, the pansystolic prolapse demonstrated by angiography corresponded to the pansystolic configuration of the murmur. The systolic murmurs showed a late systolic accentuation during the maximal prolapse of the mitral valve. Echocardiographically , only this maximal late systolic prolapse was recorded. Etiology. Several etiological theories for the prolapsing mitral valve have been entertained, including rheumatic (23), ischemic (24, 25), or traumatic (6). None of these causes were present in our patients, thus suggesting a congenital origin of the mitral valve abnormalities. Our patients document the presence of prolapsing mitral valve in association with a cleft posterior mitral valve in both cases and a secundum type atrial septa1 defect in one of them. This combination adds further support to a congenital origin of the mitral valve abnormality. ACKNOWLEDGMENTS
We heartily thank Dr. Stephen Wiener for his help in the preparation of this manuscript.
Cleft Posterior Mitral Valve
85
REFERENCES 1 . Barlow JB and Bosman CK: Aneurysmal protrusion of the posterior leaflet of the mitral valve. An
auscultatory-electrocardiographic syndrome. Am Heart J 71: 166-178. 1966. 2. Bittar N and Sosa JA: The billowing mitral valve leaflet. Report on 14 patients. Circulation 38:763-770, 1968. 3 . Pocock WA and Barlow JB: Etiology and electrocardiographic features of the billowing posterior mitral leaflet syndrome. Analysisofafurther 130patients with a late systolicmurrnuror nonejection systolic click. Am J Med 51:731-739, 1971. 4. Hancock EW and Cohn K: The syndrome associated with midsystolic click and late systolic murmur. Am J Med 41:183-196. 1%7. 5. Barlow JB: Conjoint clinic on the clinical significance of late systolic murmurs and nonejection systolic clicks. J Chronic Dis 18:665473. 1965. 6 . Barlow JB, Bosman CK. Pocock WA and Marchant P Late systolic murmurs and nonejection (“mid-late”) systolic clicks. An analysis of 90 patients. Brit Heart J 30:203-218. 1968. 7. Popock AW and Barlow JB: A n association between the billowingposterior mitral leaflet syndrome and congenital heart disease. particularly atrial septal defect. Am Heart J 81:72&722, 1971. 8. Goodman JD and Hancock WE: Secundum atrail defect associated with a cleft mitral valve. Brit Heart J 35:1315-1320, 1973. 9. Gooch AS. Vicencio F, Maranhao V and Goldberg H: Arrhythmias and left ventricular asynergy in the prolapsing mitral valve leaflet syndrome. Am J Cardiol 29:611-620, 1972. 10. Scampardonis G, Yang SS, Maranhao V et al: Left ventricular abnormalities in prolapsed mitral leaflet syndrome. Circulation 48:287. 1973. 11. Davachi F. Moller JH and Edwards JE: Disease of the mitral valve in infancy. Circulation 43565579. 1971. 12. Baron GM. Wolf SB, Steinfeld Land Van Mierop LHS: Endocardial cushion defects. AmJ Cardiol 13:162-175, 1964. 13. Salomon J. Aygen M and Levy MJ: Secundum type atrial septal defect with cleft mitral valve. Chest 58540-542, 1970. 14. Pifare‘ R, Dieter RA. Hoffman FG and Neville WE: Atrial secundum septal defect and cleft mitral valve. Ann Thorac Surg 6:373-376. 1968. 15. Billig DM, Hallman GL. Bloodwell RD and Cooley DA: Surgical treatment ofatrial septal defects in patients with angina pectoris. Ann Thorac Surg 5:566-568. 1968. 16. McEnany MT, English AT and Ross ND: The congenitally cleft posterior mitral valve leaflet. Ann Thorac Surg 16:281-292. 1973. 17. Edwards J E and Burchell HB: Pathologic anatomy of rnitral insuficiency. R o c Staff Meet. Mayo Clinic 33:497. 1958. 18. Berghuis J L , Kirklin JW. Edwards J E and Titus JL: The surgical anatomy of isolated congenital mitral insufficiency. J Thorac Cardiovasc Surg 47:791-798. 1964. 19. Flege JB. Vlad P and Ehrenhaft JL: Congenital mitral incompetence. J Thorac Cardiovasc Surg 53:138-144. 1967. 20. Betriu A, Wigle ED, Felderhof HC and McLoughlin MJ: Prolapse of the posterior leaflet of the mitral valve associated with atrial septal defect. AmJ Cardiol 33:126, 1974. 21. Lunstrom N and Edler I: Ultrasound cardiology in infants and children. Acta Paediatr Scand 60:200-300. 1967. 22. Girod D, Raghib G, Wang Y. Adams P and Amplatz K: Angiocardiographic characteristic of persistent common atrioventricular canal. Radiology 85:442447, 1965. 23. Barlow JB, Pocock WA. Marchand D et al. The significance of late systolic murmurs. Am Heart J 66:443, 1963. 24. Zoneraich S, Zoneraich 0 and Gupta MP: Double mid-systolic clicks in ischemic heart syndromes. Chest 63:815, 1973. 25. Steelman RB. White RS. Hill J C et al: Mid-systolic clicks in arteriosclerotic heart disease. A new facet in the clinical syndrome of papillary muscle dysfunction. Circulation 44:503, 1971.
Case Reports
PROLAPSING MITRAL VALVE LEAFLET SYNDROME. A Spectrum That Includes Cleft Posterior Mitral Valve Nicholas 2. Kerin, M.D., Josef Edelstein, M.D., and George Louridas, M.D. Two patients with a prolapse and cleit posterior mltral leaflet were studied. The first case had an associated ostium secundum type atrial septal defect. In both cases, the pansystolic regurgitation of contrast material during anglography correspondedto the pansystolic configuration of the murmur. In each instance, the systolic murmurs displayed a late systolic accentuationduringthe maximal prolapseof the mRral valve. The echocardiographic studies demonstrated only a late systolic prolapse which in both patients correspondedangiocardiographicallyto the maximum buckling of the pansystolic prolapse. Echocardiographlc and angiocardiographic features of cleft posterior mltral valve leaflet are discussed. Key words: billowing mitral valve, midsystolic click and late systollc murmur syndrome, midsystolic click, atrial septal defect, mitral regurgitation, cleft posterlor mitral valve
Since its first description by Barlow and Bosman in 1966 ( I ) , the “prolapsing” mitral valve or “billowing” mitral valve has been a subject of great interest ( 2 4 ) . The association between prolapsing mitral valve and congenital heart disease has seldom been reported (5-7). We have found only one previous report describing a cleft of the posterior leaflet of the mitral valve in combination with a billowing mitral valve (8). This report discusses two cases of prolapsing mitral valve (PMV) associated with a cleft in the posterior leaflet of the mitral valve. MATERIALS AND METHODS
This report includes two cases of prolapsing mitral leaflet syndrome with associated cleft in the posterior leaflet of the mitral valve. Both patients satisfied the following criteria earlier described by Gooch et al. (9): (1) symptoms of chest pain From the Irving 6. Hexter Center, Mount Sinai Hospital, Cleveland, Ohio Dr. Kerin is now at the Sinai Hospital of Detroit. 6767 West Outer Drive. Detroit. Michigan 48235. Address reprint requests to this address. Received Aug. 1 . 1975; revision accepted Oct. 6. 1975
77
@ 1976 Alan R. Liss, Inc., 150 Fifth Avenue, New York,
N.Y. 10011
78
Kerin, Edelstein, and Louridas
and palpitation; (2) arrhythmias, atrial or ventricular demonstrable on electrocardiogram at rest or immediately after exercise; (3) T-wave abnormalities with a broad QRS-T angle; (4) auscultatory findings of midsystolic, late systolic, or pansystolic apical murmur with or without a systolic click. Clinical studies included physical examination and electrocardiographic, echocardiographic , phonocardiographic, and roentgenographic examinations. The phonocardiographic recordings were made using the Cambridge photographic recorder. Cambridge-Leatham microphones were used for recording of the heart sounds, with a medium frequency range. Echocardiograms were obtained by means of a Smith-Kline Ekoline 20 Ultrasonoscope with a 2.25 MH transducer of 0.5 in. diameter. They were displayed on the oscilloscope of the Cambridge Multichannel Recorder simultaneously with an electrocardiogram for timing purposes. Exercise test was performed by means of a motorized treadmill. Each subject walked at progressively greater loads for 4 min at each level until 85% of the age-predicted heart rate was obtained. Right and left heart catheterization was performed in both cases. Left ventriculography was performed with the patient in the right anterior oblique position, and recordings were made on 35 mm film at a speed of 60 fps. Selective coronary cineartenograms were done by the Judkins (first case) and Sones (second case) technique. In the second case, pacing was performed with a no. 7 Bipolar Gorlin catheter from the junction of the superior vena cava and right atrium. Leads I, aVF, and V4 were used to monitor the electrocardiographic response. Multistage atrial paciang was initiated at a rate of 110 b e a t s h i n and additional rates of 134. 142, and 150 beatdmin were used for periods of 2 min each. Electrocardiographic recordings, measurements of left ventricular pressure, and dp/dt were done following each pacing stage. FINDINGS
Case 1 A 51-year-old female was admitted to the Mount Sinai Hospital of Cleveland in July 1972 in right-sided heart failure. There was no past history of rheumatic fever. Since 1968, she complained of atypical chest pain and exertional dyspnea. The pain was not related to exercise and was variable in duration, lasting from a few minutes to half an hour. During June and July of 1972, she noted progressive increase of dyspnea and peripheral edema. Despite adequate treatment with Digoxin and Lasix, the signs of right-sided heart failure worsened. Physical examination revealed an underdeveloped and gracile lady. Her blood pressure was 170/90 mmHg, and her pulse rate was 100/min. The neck veins were distended 5 cm above the clavicle with the patient at 45" and showed marked V waves which were higher than the A waves. A few bronchial rales were heard at the lung bases. No left ventricular impulse was palpable, but a substantial systolic lift was present along the left sternal edge. The first heart sound (S 1) was accentuated at the apex. In the 3rd left intercostal space (LICS) an early diamond-shaped murmur, initiated by a pulmonic ejection click, was present. Neither the murmur nor the narrowly split second heart sound exhibited respiratory variation. At the apex, the anterior axillary line (AAL), and 4th LICS, a grade IV/VI pansystolic murmur with late systolic accentuation and a very prominent midsystolic click (MSC) were
Cleft Posterior Mitral Valve
79
audible. The liver was slightly enlarged due to passive congestion. The electrocardiogram showed sinus rhythm with a frontal QRS axis of plus 90"and an incomplete RBBB. The phonocardiographic (PCG) recordings revealed the described auscultatory findings. The A2 to P2 interval was rlOlmsec, and both components were about equal (Fig. 1). An echocardiogram recorded on July 6, 1973, disclosed the typical late systolic buckling of the posterior and anterior leaflets of the mitral valve. The PCG showed that the MSC preceded the prolapse. During systole, the mitral valve revealed multiple parallel lines. The remainder of the echocardiographic examination revealed a paradoxical septa1 movement and an increased right ventricular dimension of 3 cm (Fig. 2). Cardiac catheterization, which was performed on July 16, 1973, demonstrated a left to right shunt at the atrial level of 2.23 L/min (Table I). The left ventriculogram revealed an inadequate long axis shortening (10). The contraction pattern was normal with symmetrical shortening of the small axis. Throughout systole, a prolapse of the posterior and medial scallops of the posterior leaflet, as well as of the anterior leaflet, was present. In mid- and late systole, the prolapse reached its maximum. A radiolucent area within the prolapse (posterior-medial scallop) marked the location of a cleft. Through the center of this radiolucent area, a reflux of contrast material was seen regurgitating into the left atrium (LA) in systole (Fig. 3). A no. 8 F Rodriguez catheter was advanced from the right atrium to the LA
PEC
,
Fig. 1. Surface phonocardiograms(PCG) were recorded from the 3rd left intercostal space at the left sternal border (LICS) (top tracing) and the anterior axillary line (AAL) (bottom tracing). Right jugular phiebogram is displayed between the two PCG tracings and its components are labeled. An ejection murmur having the characteristicsof a functional murmur is seen in the top tracing, preceded by a pulmonic ejection click (PEC). A midsystolic click (MSC) and a pansystolic murmur (PSM) havlng a late systolic accentuatlon were recorded in AAL (bottom tracing) in a high frequency range.
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Kerin, Edelstein, and Louridw
Fig. 2. Ultrasound recording of the mitral valve. The septum displayed paradoxical movement. Duringsystole, there was a late systolic prolapse involving both leaflets with multiple duplication of mitral leaflets echoes. The right ventricle (RV) was dilated. RS, right septum; LS, left septum; LV, left ventricle; AMV, anterior mitral valve; PMV, posterior mitral leaflet.
TABLE I. Cardiac Catheterization Data
Site
02
Sat. %
Superior vena cava
I5
Inferior vena cava Mid-right atrium Low-right atrium Right ventricle Pulmonary artery Left atrium
76.5 83 86.5 84 79.5 94
Pressure, mmHg Q p = 5.43 ml/rnin/M2 m=4
Qs = 3.2 ml/min/M2 L-R shunt = 2.23 L/min
65/25 44/18 m = 20 m=2
through a patent ostium secundum defect. An LA injection demonstrated a relatively large atrial septa1 defect (ASD). The coronary cineangiograms demonstrated a dominant, normal right coronary artery. The main left coronary artery (LCA) was of normal length and divided into the left anterior descending artery (LAD) and a small circumflex artery (Cx). The LAD was smooth in outline and gave off two diagonal branches. The LCA and its divisions were completely free of obstructive disease. N o intercoronary circulation was seen.
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Fig. 3. Left ventricuiogram of patient no. 1, done in a 25” RAO position showing a prolapse of the posterior and anterior mitral leaflets. A nonopaque “notch” marked the location of a cleft in the posteromediai scallop.
On Sept. 9, 1973, the patient underwent open heart surgery, and a medium-sized ostium secundum defect was found. Examination of the mitral valve revealed a prolapse of both leaflets, with a rupture of one chorda tendinea. The chordae sustaining both leaflets were long and lax. In the posterior leaflet, there was a small cleft which permitted a moderate degree of regurgitation. The ASD was sutured, and the mitral insufficiency was corrected by narrowing the valve ring with one mattress suture at its medial commissural area and repairing the cleft with two interrupted sutures. The surgical procedure was well tolerated and the postoperative course was benign. One year after surgical repair the patient has continued to d o well. Case 2 A 53-year-old male was admitted to the hospital on Jan. 9. 1974. with a history of chest pain. In 1969. he first noticed short episodes of nonradiating retrosternal pain accompanied by dyspnea on exertion. Very rarely did he experience pain at rest. Labile hypertension was discovered at the same time. There was a significant family history of arterial hypertension. The physical examination showed a normally developed male with a blood pressure of 170/108 mmHg and a regular pulse of 82/min. The heart was not enlarged by palpation or percussion. On auscultation, a grade 2-3/6, high-pitched pansystolic murmur with late systolic accentuation was audible along the LSB and at the apex. No systolic click was present. The SI was accentuated at the apex. The second heart sound was physiologically split. The remainder of the physical examination
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was unremarkable. The X ray of the chest was normal. The electrocardiogram showed sinus rhythm and nonspecific ST-T changes in the inferior leads. Occasional ventricular premature beats were registered. Echocardiography showed prolapse of the posterior leaflet of the mitral valve in late systole. No duplication of the systolic image of the mitral leaflets was present (Fig. 4). The remainder of the echocardiographic examination revealed normal septal motion and normal right ventricular and left ventricular dimensions. On Nov. 16, 1973, cardiac catheterization revealed normal right atrial, right ventricular, pulmonary arterial, and pulmonary capillary wedge pressures. Left ventricular end diastolic pressure (LVED) was 14 mmHg. Neither angina pectoris nor ST-T changes were elicited during atrial pacing. The resting LVED of 14 mmHg declined to 4 mmHg at the maximum pacing rate. When pacing was discontinued, the LVED rose to 19 mmHg. The dp/dt of the LV declined from 4,480 mmHg/s to 3,400 mmHg/s at the highest pacing rate of 150 b e a t s h i n . After left ventricular angiography (LVA), LVED rose to 21 mmHg. The LVA revealed a slightly increased end systolic volume. The mitral valve showed pansystolic prolapse of the medial scallops of the posterior leaflet. A radiolucent area in the center of the prolapse marked the location of the cleft (Fig. 5). Through the center of the cleft, a fine whiff of contrast material regurgitated in systole into the left atrium. In diastole, the V-shaped defect was well delineated. Coronary cineangiography revealed a normal. short LCA which bifurcated into the LAD and Cx artery. The Cx artery showed a preponderant distribution. This artery was very tortuous but free from obstructive disease. The LAD was a large, smooth vessel and gave off two diagonal branches. The LCA and its divisions were completely free from arteriosclerotic changes. The RCA was a small nondominant artery, which, after a short course, divided into two small terminal right ventricular branches. Just above the bifurcation there was a focal lesion that obstructed about 50% of the lumen. No intercoronary circulation was seen. DISCUSSION
The syndrome produced by prolapse of the posterior leaflet of the mitral valve has been the subject of many comprehensive reports (1-6). The etiological basis has been shown to be heterogeneous. In a series of 200 cases (7), only 22 cases were associated with congenital heart abnormalities. These included secundum atrial septal defect in 17 cases, one Eisenmenger syndrome in a patient with a ventricular septal defect, and four cases of patent ductus arteriosus. The combination of prolapse of the posterior leaflet of the mitral valve and cleft posterior mitral leaflet was only recently reported (8). Cleft Mitral Valve
It is rare to find clefts in the anterior o r posterior mitral leaflets. The scarceness of this lesion was attested to by Davachi et al. (1 I ) who reported no instance among 55 patients with mitral valve abnormalities. Endocardia1 cushion defect consists of a broad spectrum of anomalies ranging from ostium primum defects to a complete persistent atrioventricular canal. Partial or complete cleft of the anterior leaflet of the mitral valve was a frequent finding in combination with a complete or incomplete form of endocardial cushion defect (12). Only a few cases have been reported
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in which a cleft anterior mitral valve leaflet was found in combination with secundum type of ASD (8, 13-15). The combination of ostium secundum defect with a cleft posterior leaflet has rarely been described (8, 16-19). The association of PPML with ostium secundum defect was found to be very common. In a recent article, this combination was encountered in 9 out of 43 patients (20). The uniqueness of our first case is in the association of ASD, PPML, and a cleft posterior leaflet, which has only been reported once previously (8). At operation, a moderate ostium secundum type ASD was found. The posterior leaflet of the mitral
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I
I
I
i
Fig. 4. Echocardlogramof the mitrai valve revealinga latesystolic prolapseof the posterior leaflet (arrows). The septum displayed normal movement
us
LS
ED
Fig. 5. Left ventricular angiogram of patient no. 2, done In a 30”RAO position, showing a prolapse of the posteromedlai scallop. The arrow points to the prolapsed mitrai leaflet. The nonopaque “notch” is markedwith broken lines. Inearly syrtole(ES), a whiff of contrast material regurgitated into the left atrium through the cleft. in mid- (MS) and late systole (LS), there was less regurgitation seen. The same nonopaque notch was well seen In the end diastolic (ED) frame.
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Kerin, Edelstein, and Louridas
valve was cleft in its lateral position, and the chordae sustaining both leaflets were long and lax. with rupture of one of the chordae supporting the posterior leaflet. It is apparent in this case that the prolapse of the posterior and anterior leaflets was due to multiple defects involving the subvalvular mitral apparatus. These abnormalities involving the mitral valve (deficiency of valve tissue) and subvalvular mitral apparatus (lax and ruptured chordae) were responsible for the regurgitation. An abnormal left axis deviation, the electrocardiographic hallmark of ostium primum defect, was not present in our first case. Echocardiography.This procedure has provided a useful noninvasive technique for the detection of mitral valve prolapse. Echocardiography revealed a typical posterior displacement of both mitral leaflets in the first case and only the posterior leaflet in the second case. I t has been suggested that multiple duplication of the systolic image of the mitral leaflet was caused by redundancies and irregularities of the leaflets as they approximate during systole (21). These multiple systolic echoes were thought to represent clefts in the valve (21). While Case I (Fig. 2) revealed multiple mitral systolic echoes, Case 2 did not (Fig. 4). It is difficult to interpret this sign, since many echocardiograms from normal patients may reveal systolic duplication of the mitral leaflets. Angiocardiography.Only left ventriculography may give a definite assessment of the mitral valve and permit differentiation of patients with endocardia1 cushion defects from those with ostium secundum ASD with mitral valve cleft ( 1 1). The anatomical basis of the angiocardiographic findings is the abnormal insertion of the chordae to the deficient valve tissue (cleft). The “nonopaque notch,” which marks the location of the cleft, is a result of the thickening of the free edge of the cleft at the site of insertion of the chordae tendinae (12,22). The nonopacified defect in the prolapse valve may be seen in both systole and diastole (Figs. 3 3 . The defect may opacify during systole if the leaflets do not coapt and if the contrast regurgitates into the left atrium (Fig. 5A). Differentiation of a prolapsing cleft mitral valve leaflet from normal indentations located at the junctions of the mitral valve scallops is based upon the central position of the cleft within the scallop and possible entrapment of opaque material in both sides of the cleft within the scallop during diastole (Fig. 5). In both cases, the pansystolic prolapse demonstrated by angiography corresponded to the pansystolic configuration of the murmur. The systolic murmurs showed a late systolic accentuation during the maximal prolapse of the mitral valve. Echocardiographically , only this maximal late systolic prolapse was recorded. Etiology. Several etiological theories for the prolapsing mitral valve have been entertained, including rheumatic (23), ischemic (24, 25), or traumatic (6). None of these causes were present in our patients, thus suggesting a congenital origin of the mitral valve abnormalities. Our patients document the presence of prolapsing mitral valve in association with a cleft posterior mitral valve in both cases and a secundum type atrial septa1 defect in one of them. This combination adds further support to a congenital origin of the mitral valve abnormality. ACKNOWLEDGMENTS
We heartily thank Dr. Stephen Wiener for his help in the preparation of this manuscript.
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