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Mitral and Tricuspid Valve Closure in Congenital Heart Disease SELWYN MILNER, M.B.B.CH., F.C.P.(S.A.), RICHARD A. MEYER, M.D., ALEX W. VENABLES, M.D., M.R.C.P., JOAN KORFHAGEN, ARDMS, AND SAMUEL KAPLAN, M.D. Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 SUMMARY Echocardiography was used to evaluate mitral and tricuspid valve closure in patients 1 day to 20 years of age. When possible, simultaneous phonocardiograms were obtained. The difference in time between the Q wave of the electrocardiogram and mitral closure and between Q and tricuspid closure was designated the delta value (A). Four groups of patients were assessed: 1) normals (40), secundum atrial septal defect (ASD) (10), mitral valve prolapse syndrome (Barlow's syndrome) (13), pulmonary hypertension (12), and pulmonic stenosis (6); 2) Ebstein's anomaly (10); 3) transposition of the great vessels (15); 4) right bundle branch block (RBBB) (25). Ten patients with surgically induced right bundle branch block were studied by phonocardiography alone. Group I had delta values of 50 msec or less (-5 to 50 milliseconds) and served as controls. Ebstein's anomaly showed prolongation of the delta value to 65 msec or greater in eight out of ten patients. Patients with transposition of the great vessels showed a striking difference from the preceding groups in that an average negative delta value was obtained. Twenty-two patients of group 4 (RBBB) had delta values within the norinal range. This study has shown that a delta value greater than 65 msec is suggestive of Ebstein's anomaly. In addition, if the delta value is negative, transposition of the great vessels can be suspected. IN THE NORMAL HEART mitral valve closure precedes that of the tricuspid valve. These valves may be subjected to unusual hemodynamics in certain forms of congenital heart disease (such as transposition of the great arteries) or may be structurally abnormal (as in Ebstein's anomaly of the tricuspid valve). Since their precise point of closure can be recorded echocardiographically, this study was undertaken to evaluate the diagnostic significance of abnormal atrioventricular valve closure time. The following groups of patients were assessed: Group 1. 1) Normals - 40 patients (3 days - 20 years of age); 2) Secundum atrial septal defect (ASD) - 10 patients (1 month - 17 years of age); 3) Mitral valve prolapse syndrome or Barlow's syndrome (with mild mitral insufficiency) - 13 patients (8 - 17 years of age); 4) Pulmonary hypertension - 12 patients (2 months - 21 years); 5) Pulmonic stenosis - 6 patients. Group 2: Ebstein's anomaly - 10 patients (1 day - 20 years of age). Group 3: Transposition of the great vessels - 15 patients (4 days - 17 years of age). Patients with associated defects before or after any form of surgery were included. Group 4: Complete right bundle branch block (RBBB) 25 patients (4 - 14 years of age). All patients with RBBB had a QRS duration equal to or greater than 110 msec. Fifteen with RBBB were examined ultrasonically. In six instances of surgically induced RBBB, echocardiograms were obtained pre and postoperatively. Three of the patients had naturally occurring RBBB, the etiology of which was unknown. Very few simultaneous phonocardiograms and echocardiograms could be obtained because the area required by the transducer and microphone frequently were the same. Therefore, phonocardiographic analysis of the major components of the first heart sound (Ml-Tl) was performed in an additional ten patients with surgically induced RBBB. Material and Methods Echocardiograms were obtained from the tricuspid and mitral valves with a Hoffrel 101 ultrasonoscope. Either a nonfocused 5 MHz ¼h inch or a focused 2.25 MHz ½/2 inch transducer was used depending upon the age of the patient and the ease of recording the structure sought. The echoes were recorded at 75 or 125 mm/sec with 40 msec time lines. In only two instances was a paper speed of 50 mm/sec used. When possible, a simultaneous external phonocardiogram, using a Cambridge type 72352 amplifier and type 53616 microphone, was obtained and recorded at a frequency and position that would best display components of the first heart sound. It was often difficult to record simultaneous phonocardiograms in younger patients since the microphone placement interfered with transducer position. All echocardiograms were obtained in the recumbent position with the patient breathing normally. The transducer was angled so that both anterior and posterior portions of the valve could be seen and their point of coaptation identified, which thus defined closure (fig. 1). Delta Value (A) The term delta value was used to designate the difference (in msec) between mitral closure (Mc) and tricuspid closure (Tc) as determined by echocardiography. This was determined as follows: The time taken from the Q wave of the ECG to tricuspid closure and from Q to mitral closure was measured to the nearest 5 msec (fig. 1). Three complexes with similar preceding R-R intervals were measured for each value and From the Department of Pediatrics, College of Medicine, University of Cincinnati, Children's Hospital, Cincinnati, Ohio. Supported by NIH Grant 5 TOI HL05728 and the American Heart Association, Southwestern Ohio Chapter. Address for reprints: Richard A. Meyer, M.D., Division of Cardiology, Children's Hospital Medical Center, Cincinnati, Ohio 45229. Received May 19, 1975; revision accepted for publication October 16, 1975. 513 514 CI RCULATION VOL. 53, No. 3, MARCH 1976 EKG EKG TV MV Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 PHONO I.z FIGURE 1. Left) Mitral closure (Mc) occurs when the anterior and posterior mitral leaflets coapt. Right) Similarly, tricuspid closure (Tc) occurs at the point of coaptation. The delta value (Tc-Mc) is 25 msec. the average taken for Q-Tc and Q-Mc. The difference between Q-Tc and Q-Mc was then compared in the various groups. The phase of respiration, per se, was not taken into account although it was related to some extent by using cycles with similar preceding R-R intervals. Parkinson-White (WPW) syndrome (case 5: table 2). It was possible to diagnose Ebstein's anomaly in an infant soon after birth by echocardiography because of prolongation of 60 r A Results 50 * Echocardiography Group 1 Control Group (fig. 2) The delta values of 40 normal subjects were plotted in three age groups; 0-2 years, 2-10 years, and 10-20 years. The mean value was 20 msec (SD =± 14) with a range from -5 to 50 msec. The mean delta value was not age or rate dependent although the lower values tended to be found in younger infants. Patients with right ventricular systolic hypertension (table 1) and Barlow's syndrome had values within this normal range, as did cases with ASD, except for one patient with a value of 60 msec. This group was considered normal and served as the control group. *0 - Group 2 - Ebstein's Anomaly (table 2; fig. 3) In eight of ten patients with this condition, the delta value was 65 msec or greater. This value was higher than any other group and was even seen in the presence of type B Wolff- 0 DO 40 0000*0 *o 30 Anm .. 0 30 -OAQ00 coo *nb (3 20 @00 *AA+ ec e00 ee l-z" Do 000 Doo3 10 _leA.a 0 eNormal (N740) mean = 20 msec. (±S D -14) - gao 6 A.AS D (n 10) mean = 25 msec. OM.VPS (n 13) meon 28 msec. z an oPHT (Nzl2) *A+ + +PS (N 6) - + S -10 L- 0-2 ___J 10-20 AGE (YEARS) 2-10 FIGURE 2. The delta values of the control group comprising normals, secundum atrial septal defect (ASD), mitral valve prolapse syndrome (M VPS), pulmonary hypertension (PHT) and pulmonic stenosis (PS). VALVE CLOSURE IN CONGENITAL HEART DISEASE/Milner et al. 515 TABTLE 1. Summoary of Echographic and Pressure Data in PatieCnts with RV Sylstolic Hypertcnsion Patient 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. Age (yr) 3 1 4/12 14 8 Ii1 14 1 10/12 iI 21 6/12 3 9/1.2 2 8/12 Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 17. 2/12 6/12 18. 60 60 80 45 40 60 70 65 75 70 85 20 65 75 VSI) VSI) Pl)A VSI1) V81) ASI) VS1) VS1-) 10/12 5 9/12 15 4/12 (msec) PS PS Ps PS PS PS 7/12 15. 16. (.ITc Dx DO(RV A qMe (msec) (Te-Me) (msec) 35 45 80 25 15 0 10Q3/6 40 40 .5 0 5 130/5 25 .15 41/4 70/5 30/7 20/10 41/14 50 60 75 10 50 60 25 76/9 137/6 110/0 105/5 [0/5 55 45 50 PA, RV, (mrm hfg) (mm Hg) 31/6 87/9 20/12 19/ 22/13 140/-2 12/6 40/4 10 0 10( 15 15 70/23 70/42 137/68 No record 95/25) 90/35 70 VSI) Truncus I 45 45 45 0 1.5 70/5 100/8 80/38 32 ventrictilar septal defect; PHT Group 3 - Transposition of the Great Vessels (fig. 4; table 3) These patients were strikingly different from previous groups. The average delta value for this group was -9 msec, which denoted earlier closure of the tricuspid valve (situated in the systemic ventricle) than the mitral valve. In 11/23 recordings the delta value was within the low normal range, and 6/15 patients had a normal delta value at some state during their course. Negative values seemed to occur irrespective of age or associated lesions. This finding, too, did not seem to be influenced by Mustard's operation. No patient in this group had tricuspid insufficiency following the Mustard repair. Group 4 - Right Bundle Branch Block (figs. 5 & 6) The delta values in patients with RBBB were within normal limits except for three patients who had values of 55, 60 and 65 msec. The six patients who had echocardiograms 1 2 3 4 5 6 7 8 9 Dclta Values (A) in Ebstein's Anoneady Commnent QPc-QQMc (msec) 80 Partial IRBBB 65 RtBBB, proloniged P-R 110 1BBB 80 IIBBB 75 W.P.W., Type B Mild disease 55 70 55 Mild disease 90 70 10 Average RBBB Inifant, died 75 Abbreviations: RISBII = Parkinson-White syndlrome. right bundle branch block; W.P.X. = Wolff- EEisennienger by previous cath; data not available Underwent/ PA banding = tricuispid closure; VSJ) the delta value (case 10), and this diagnosis was subsequently confirmed by autopsy. The two patients who had values of 55 msec had mild disease by clinical estimation. Case [)own's synd1orome, PIIT by clinical exam 92/53 = D)own's syndrome 70 60 92/5 = ItBBB 105 40 10 right ventricle; qTe Ml, IJV dysfunction Aorta 91/40 4'5 5-3 35 50 = TABILI: 2. 17 8 15 13 75 60 60 Comrnents 15 VS/1) Abbreviations: ASI) atrial sel)tal defecfl DOJV doiil)e otutlet right ventricle; LV left ventricle; M I artery pressture; I'S ptilinonary stenosis; PA = puilmonary artery; PA), patent dmiettus arteriostus; qoIM block; ISV MPA\, (mm Hg) initral insthfecieney; AlPA - mean pulmonary = initral closuire; Il5.1- = right bundlle branch puilmonary hypertension. before and after open heart operation with right ventriculotomy had prolongation of the delta value postoperatively as compared to preoperatively. Nevertheless, there was no increase in these values above normal. 1Phonocardiography In every case where it was possible to record a simultaneous phonocardiogram with the echocardiogram, Mc preceded the initial high frequency components of the first heart sound (Ml). Similarly, in instances where it was clear, Tc preceded TI (the second group of high frequency components of the first heart sound). However, the delta value by echocardiography and the Ml-TI duration, in YH EBSTEINS Q-T =135 mSec. Q- M =60 mSec. G- 75mSec. FIGURE 3. Simultaneous tricuspid and mitral valve echoes in Ebstein's anomaly. The delta value is 75 msec in the presence of a normal P-R interval and QRS duration. Mc= mitral closure; Tc= tricuspid closure. 5 16 CIRCULATION p * -1%, ~ VOL. 53, No. 3, MARCH 1976 p raA TV--10 Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 ineraX er te aelye riusidvlv cosr (c)pecde mralwavecosre q T0 7OmSec. c) hnc,th4dl0 q-mMcs8mSec. ale a F IGU RF 4. Tricusp id ( TV) and m it ral (M V) val ve ec h oes in a p a tien t with transpos itio n of th e grea t vessels. Th e R-R intervals were the same, yet tricuspid valve closure (Tc) preceded mitral valve closure (Mc), hence, the delta value was .-10 msec. every instance, were the same, thus showing a definite correlation of the major components of the first heart sound to valve closure. Analysis of the phonocardiograms of ten patients with RBBB showed that there was some prolongation of MI to TI duration compared to preoperative values (table 4). Discussion Controversy over the genesis of the first heart sound still exists.1 Recent work has shown a definite correlation T VTLE 3. Delta Values (msec) in Patients with 7Transposition of the Great Arteries Mtustard operation Post Case pre 1 2 -10 -20 3 4 0 10 5 2}0 Later Commnent Coaretation -20 -20 0 6 7 8 9 10 I11 12 13 -25 10( 10 15 -10 -3)0 10 15 -5 14 - :30 15 5 -15 PS (Blalock) -5,0 PfIT ASD, VSD -5 PI-IT Average -9.2 msec = atrial sep)tal (lefect; IPT1 = pulmonary hyperpul nonary stenosis; VSD ventricuilar septal defect. Abbreviations: ASD tension; I'S = between echocardiographic, phonocardiographic, cineangiographic and hemodynamic closure of the atrioventricular valves.2 I While recognizing that there are many methods to determine the precise timing of valve closure, we have taken echocardiographic closure of the valves to represent actual closure. Although delayed tricuspid closure has been demonstrated previously in Ebstein's anomaly,6 8 the role of RBBB has not been assessed extensively. All our patients with complete RBBB had delta values less than 65 msec and, with the exception of three, had values less than 50 msec (fig. 7). The nature of the delay in these three patients (55, 60 and 65 msec) is unclear; two of them were examined postoperatively but not preoperatively and may have had delayed delta values prior to surgery. The etiology of the high delta value in the patient with naturally occurring RBBB was also not apparent. The other two patients with naturally occurring RBBB had normal delta values. Furthermore, the five patients who had echocardiograms before and after their surgically induced RBBB had delta values less than 50 msec. Therefore, it would appear that RBBB has little effect on closure of the tricuspid valve and plays a minor role, if any, in the large delta value recorded in Ebstein's anomaly. Late closure of the tricuspid valve in Ebstein's anomaly has been ascribed to the large leaflets which somehow cause a mechanical delay in closure of the valve. Type B WPW9 does not influence the delay in Tc (table 2). As noted in our mild cases, the delta value may be in the high normal range. Thus, patients with Ebstein's anomaly may have normal delta values. Nevertheless, a delta value longer than 65 msec is strongly suggestive of the condition, since no other situation produced similar large delta values. t4-cHbM~ e VALVE CLOSURE IN CONGENITAL HEART DISEASE/Milner E 4 . t ) et al. 517 I 1 ~.. TV Mt2ccpr, ; . Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 Q-T =125 j45 mSec Q-Mc 80 FIGURE 5. Tricuspid (TV) and mitral (MV) valve echoes from a patient with surgically induced right bundle branch block. The QRS duration is 120 msec, but the delta value is only 45 msec. Mc = mitral closure; Tc = tricuspid closure. Rosenquist et al.12 recently described a spectrum of mitral valve disease in 71% of 163 specimens with transposition of the great vessels. Only 38 cases (23%) had entirely normal mitral valves. The abnormalities included normally formed valves with small mitral annuli (6%), normal valves and restricted free margins of the anterior leaflets (33%) and mitral valve anomalies (38%). The mitral valve anomalies included: 1) underdevelopment of space between papillary muscles; 2) underdevelopment of space between papillary muscles and ventricular wall; and 3) indentation of anterior leaflet and attachment to the ventricular septum. Three of these specimens were similar to those described by Layman and Edwards'0 and looked like Ebstein's anomaly. Thus, it would seem reasonable to speculate that the delayed closure of the mitral valve (resulting in negative delta values) may be related to an anatomically abnormal mitral valve. On the other hand, normal delta values would also be expected since a large percentage of these patients have normal mitral valves. Of great interest was the finding of predominantly negative delta values in patients with transposition of the great arteries. Only one other patient (a normal infant in the control group) had a negative delta value (fig. 7). One hypothesis to explain this finding may be that the mitral valve anomalies found in pathologic specimens with com- plete transposition of the great vessels" 12 in some manner affect the timing of closure of the valve. Layman and Edwards10 found anomalies of the mitral valve in 13 of 88 specimens (I15%) which consisted of: l) cleft anterior leaflet; 2) partial persistent atrioventricularis communis; 3) "6parachute" mitral valve; 4) abnormal adherence of the anterior leaflet to the ventricular septum; and 5) "filigree" of the mitral valve. Elliott et al.1" described an abnormality of the mitral valve in only one of 60 cases. However, in that specimen the basal aspect of the medial portion of each mitral cusp was attached to the ventricular wall in a manner similar to that observed in cases of Ebstein's malformation of the tricuspid valve. TAIBLE 4. Phonocardiogramrs of Patients with RBBIB Following Right Ventriculotomy 1Preop Case 1 2 3 4 5 6 7 8 9 10 Diagnosis VSJ) + PS TOF TOF + Waterston TOF TOF + Waterston TOF TOE TOF TOF VSI) iI I-Ti (msec) (mnsec) 30 45 35 30 50 40 60 65 50 55 100 75 QRS Postop l11 95 90 80 80 100 70 k2-P'2 (msec) Il-TI (msec) (msec) 55 60 45 45 45 55 40 45 40 35 45 130 120 110 130 150 110 110 115 170 115 60 40 35 40 75 100 100 35 70 106 30 60 35 piu1monary stenosis5; QRS QRS (luration.; TOF Abbreviations: HR heart rate; IPS septal defect; RIBBB= right bundle branch block. = QRS HR' 85 80 110 80 110 115 110 112 80 96 A2-1P2 (msec) 70 80 50 90 75 60 40 35 70 65 tetralogy of Fallot; VSD -ventricular CIRCULATION 518 VOL. 53, No. 3, MARCH 1976 8Cr 70F 60h 120 r R.B.B.B. * T.O.F. o VS.D. O OTHER E 105F 0 0 90 K 50F A q Tcq Mc 40F 75 30F + A~ + AL~ 60K*- 20h 11) q) (t) 10 .j Z.izr- 0 IZ)PRE-OP Ave. ORS dur.=71msec. POST-OP NO OP 15 Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 0 ++ ++ 0@0 _ I_ - mm - FIGURE 6. The delta values in patients with surgically induced right bundle branch block (RBBB) did not exceed the normal value (60 msec). One unoperated patient with RBBB had an unexplained value of 65 msec. Dur = duration; Mc = mitral closure; Tc = tricuspid closure; T. 0. F. = Tetralogy of Fallot; V.S. D. = ventricular septal defect. 1. Luisada AA, MacCanon DM, Kumar S, Feigen LP: Changing views on the mechanism of the first and second heart sounds. Am Heart J 88: 503, 1974 2. Lanaido S, Yellin EL, Miller H, Frater RWM: Temporal relations of the first heart sound to closure of the mitral valve. Circulation 47: 1006, 1973 3. Pohost GM, Dinsmore RE, Rubenstein JJ, O'Keefe DD, Grantham RN, Scully HE, Beierholm EA, Frederiksen JW, Weisfelot ML, Daggett 30 14. Ave. QRS dur. =l18msec. Another hypothesis is that the negative delta value in patients with transposition of the great vessels is related to a change in compliance of the ventricles. Since the resistance circuits to which the ventricles are exposed have been reversed (i.e., right ventricle is systemic, left ventricle is pulmonic), the compliance of the chambers is altered in such a way that the closure of the atrioventricular valves result in a negative delta value. Against this hypothesis are the positive delta values found in all our patients with systolic hypertension of the right ventricle (fig. 7). However, those patients are not identical to transposition patients because their left ventricles remain systemic. At this time, the mechanism of the negative delta value found in patients with transposition of the great vessels is still obscure. Nevertheless, a negative delta value supports the diagnosis of transposition. In addition, delta values greater than 65 msec strongly suggest Ebstein's anomaly of the tricuspid valve, since no other defect had a greater value. Hopefully, greater experience with echocardiographic determination of atrioventricular valve closures should not only provide insight into the etiology of abnormal delta values, but also provide a reliable means by which to investigate the relationship of atrioventricular valve closure to various hemodynamic states in disease as well as in the normal heart. References 45 _ ... - 1 ++ ++ 0 0 emum- - --------+_ CC0 -I5 - - - CO OD 0 n 0 0 GO -30 -- 45L _ CONTROL PRE POST RBBB EBSTEINS TGV FIGURE 7. The delta values of the four groups of patients are compared. The patients with transposition of the great vessels (TG V) are striking because their values are predominantly negative whereas the patients with right bundle branch block (RBBB) are normal and those with Ebstein's anomaly are the only values greater than 65 msec. Pre = preoperative; post = postoperative. 4. 5. 6. 7. 8. 9. 10. 11. 12. WM: The echocardiogram of the anterior leaflet of the mitral valve: Correlation with hemodynamic and cine roentgenographic studies in dogs. Circulation 51: 88, 1975 Rubenstein JJ, Pohost GM, Dinsmore RE, Harthorne JW: The echocardiographic determination of mitral valve opening and closure: Correlation with hemodynamic studies in man. Circulation 51: 98, 1975 Waider W, Craige E: The first heart sound and ejection sounds. Echocardiographic and phonocardiographic correlation with valvular events. Am J Cardiol 35: 346, 1975 Kotler MN, Tabatzik B: Recognition of Ebstein's anomaly by ultrasound technique. (abstr) Circulation 44 (suppl II): 11-34, 1971 Crews TL, Pridie RB, Benham R, Leatham A: Auscultatory and phonocardiographic findings in Ebstein's anomaly. Br Heart J 34: 681, 1972 Lundstrom NR: Echocardiography in the diagnosis of Ebstein's anomaly of the tricuspid valve. Circulation 47: 597, 1973 Tajik AJ, Gau T, Giuliani ER, Ritter DG, Schattenburg TT: Echocardiogram in Ebstein's anomaly with Wolff-Parkinson-White pre-excitation syndrome, Type B. Circulation 47: 813, 1973. Layman TE, Edwards JE: Anomalies of the cardiac valves associated with complete transposition of the great vessels. Am J Cardiol 19: 247, 1967 Elliott LP, Neufeld HN, Anderson RC, Adams P Jr, Edwards JE: Complete transposition of the great vessels. I. An anatomic study of sixty cases. Circulation 27: 1105, 1963 Rosenquist GC, Stark J, Taylor JFN: Congenital mitral valve disease in transposition of the great arteries. Circulation 51: 731, 1975 Mitral and tricuspid valve closure in congenital heart disease. S Milner, R A Meyer, A W Venables, J Korfhagen and S Kaplan Downloaded from http://circ.ahajournals.org/ by guest on June 16, 2017 Circulation. 1976;53:513-518 doi: 10.1161/01.CIR.53.3.513 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1976 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/53/3/513 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. 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