Annuloplasty in Children and Young Adolescents with Severe Rheumatic Mitral Insufficiency

Transcription

1 Annuloplasty in Children and Young Adolescents with Severe Rheumatic Mitral Insufficiency By HOWARD H. KLOTH, M.D., GEORGE E. REED, M.D., DAVID A. TICE, M.D., EUGENIE F. DOYLE, M.D., BRIAN KIELY, M.D., AND MARIO SPAGNUOLO, M.D. Additional Indexing Words: Acute rheumatic fever Hemodynamic findings Cardiac size SUMMARY Eleven patients aged 8 to 15 years underwent measured asymmetrical annuloplasty for severe mitral regurgitation in the years 1961 through They had had a total of 20 attacks of acute rheumatic fever. The intervals between the last attack of acute rheumatic fever and operation ranged from 2 to 8 years. The criteria for surgery were congestive failure and progressive cardiac enlargement. Using the hydraulic formula of Gorlin, a mitral annuloplasty was tailored to the size of each patient so that insufficiency was eliminated without producing hemodynamically significant stenosis. In this group of 11 children there has been one death. The majority of our 11 patients reacquired murmurs of mitral regurgitation. Satisfactory results, however, are not dependent on complete hemodynamic correction. All patients have improved remarkably and have sustained this improvement up to 7 years. These results suggest that mitral annuloplasty should be the operation of choice in children with severe mitral regurgitation. PROSTHETIC REPLACEMENT of diseased heart valves frequently substitutes other disease processes for the primary one, and is irreversible since any existing valvular tissue has to be excised. Because of these disadvantages we have attempted to repair, rather than to replace, the diseased mitral valves of young patients with severe rheumatic mitral insufficiency. Acceptance of this operation, known as annuloplasty, has been de- From the Irvington House Institute and the Department of Medicine, Surgery, and Pediatrics, New York University School of Medicine, New York, New York. Investigation was supported in part by U. S. Public Health Research Grant CDO0030 and by a New York State Fellowship in Rheumatic Fever (Dr. Kloth). Paper was presented at the meeting of the American Academy of Pediatrics, Cardiology Section, Washington, D.C., October, Electrocardiograms layed because the results obtained with some techniques have been disappointingly associated with early recurrence of regurgitation' and significant operative mortality.2 5 In addition, physicians have been reluctant to recommend any operative procedure in children and young adolescents with rheumatic heart disease and congestive heart failure since they are assumed to have chronic rheumatic activity which would increase operative risks.6 The following report deals with the results obtained with measured asymmetrical annuloplasty in a young population group. Population and Methods Eleven patients aged 8 to 15 years underwent measured asymmetrical amnuloplasty for severe mitral regurgitation in the years 1961 through 1966 (table 1). Eight of these patients were followed at Irvington House and three at Bellevue Hospital. They had had a total of 20 attacks of acute rheumatic fever, all of which were observed

2 104 KLOTH ET AL. B a. NORMAL b. DILATED c. CORRECTED Figure 1 Schematic representation of normal, dilated, and corrected annulus, drawn to scale. Two measured sections of the annulus (arcs A-B and A'-B') are used to construct an annulus of 6 cm, as in drawing C. This produces an orifice area in excess of 2.8 sq cm. Population Data Table 1 in one or the other hospital. The intervals between the last attack of acute rheumatic fever and operation ranged from 2 to 8 years. All patients had been symptomatic for several years (table 2); the most common preoperative symptoms were fatigue and exertional dyspnea. A grade IV murmur of mitral insufficiency and moderate to marked cardiomegaly were uniformly present. In addition, 10 of the 11 patients had a mid-diastolic apical rumble and loud third heart sound presumably from excessive diastolic flow through the mitral orifice. Three patients had clinical evidence of tricuspid regurgitation. Three patients were in atrial fibrillation, and another had multiple attacks of supra- Figure 2 Pilot sutures of 3-0 silk have been placed through the annulus on each side of the commissures. These provide visual landmarks and anchorage for ease in placement of definitive sutures. Acute rheumatic fever Interval between last Patients Age at operation attack (yr) and operation Sex No. of attacks (yr) J.H. 8 F P.O. 9 M M.C. 12 F 2 8 E.G. 12 F 1 5 Y.B. 12 F 3 2 J.A. 12 M 2 2 M.H. 13 F L.H. 15 F 1 8 R.I. 15 F 2 7 B.M. 15 F 2 2 B.A. 15 F 1 5 Csrculation, Volume XXXVlII, July 1968

3 ANNULOPLASTY (f 10- z W 8-10S PRE-OPERATIVE POST-OPERATIVE C 6- m2 ID z FATIGUE EXERTIONAL DYSPNEA ORTHOPNEA CHF DYSPNEA AT REST INTRACTABLE AF DIGITALIS DURETICS Figure 3 Number of patients with specified manifestations and therapy before and after surgery. ventricular tachycardia. All patients had had symptoms or signs of cardiac failure and had been digitalized. Four required additional diuretic therapy. All patients were restricted in their daily activities. Electrocardiograms disclosed that, in all nine patients in whom P waves could be identified at some time before operation, left atrial hypertrophy was present. This was defined as a P wave of 0.12 msec or greater in lead II, or diphasic P wave in lead VI with the downstroke greater than 2 mm.7 Cardiac catheterizations, which included right and left-sided study and angiograms in the posteroanterior and right and left anterior oblique positions, were performed in all patients before operation, and in eight patients 1 to 3 years after operation. As noted in table 3, seven patients had pulmonary artery systolic pressures over 35 mm Hg at rest. Of these, four had pressures over 50 mm. Six of 10 patients had elevated left atrial or wedge mean pressures, and eight of nine patients had abnormally large V waves. In general, cardiac index tended to be normal and the A-V (arteriovenous) oxygen difference tended to be high. Left ventricular angiograms were performed on 10 patients; nine of these had 4+ mitral insufficiency, the other had 3+ insufficiency.8 Patient B.M. had angiographic evidence of tricuspid insufficiency. In addition, patient Y.B. had a grade IV/VI decrescendo diastolic blowing murmur at the left sternal border which proved to be pulmonary incompetence, not aortic insufficiency. No other valvular lesions were found. The criteria for surgery were congestive failure and progressive cardiac enlargement.9 Using the hydraulic formula of Gorlin and Gorlin,'0 a mitral annuloplasty was tailored to the size of each pa- Results Following surgery, as shown in figure 3, the symptoms and signs of failure diminished GRADE' GRADE GRADE NO tient so that insufficiency was eliminated without producing hemodynamically significant stenosis. An orifice of approximately 6 cm in circumference (2.8 sq cm in area) is usually sufficient for this purpose (figs. 1 and 2) in adults. A smaller orifice is created by calculation and measurement in children." Patients were followed at Irvington House Clinic or at Bellevue Pediatric Cardiac Clinic at monthly intervals. Electrocardiograms and chest x-rays were repeated at 6-month or yearly intervals. PRE- OPERATIVE 2 3 POST- OPERATIVE YEARS Figure 4 Loudness of the murmur of mitral insufficiency (on a 1 to 6 scale) before surgery and at varied postoperative intervals for each of the ten surviving patients.

4 106 KLOTH ET AL. Clinical Data Before Operation Table 2 Symptoms Paroxysmal Exertional Dyspnea nocturnal Patient Fatigue Palpitations dyspnea at rest dyspnea Orthopnea J.H P.O M.C E.G Y.B J.A M.H L.H R.I B.M B.A *Other than tricuspid incompetence which disappeared postoperatively and was considered to have been functional, Cardiac Catheterization Data Table 3 LA or wedge Interval between PA pressure LA or wedge surgery and re-cath pressure (mean) V wave Patient (mo) Preop Postop Preop Postop Preop Postop J.H.t 85/35-19W P.O /14 30/14 18W 24W M.C /6 24/4 7W 6W 12W 4W E.G. - 20/8 12LA 20LA Y.B /20 32/16 24LA 15W 44LA 21W J.A /47 35/15 23W 15W 31W 16W 25LA 41LA M.H. 28/13 11W 17W L.H. 40/15 18W - 25W R.I /12 16/3 20W 2W 30W 5W B.M /34 30/18 20W 13W 25W 14W B.A /7 32/7 5LA 5LA 12W 9W 7W 12LA Abbreviations: Preop = preoperative; postop = postoperative; PA = pulmonary artery; LA = left atrium; W = wedge regurgitation; AS = aortic stenosis; MS = mitral stenosis: AA = ascending aorta; -= not performed; 0 = negative. *LV not entered due to arterial spasm. I'Expired. strikingly; only one patient still expenences easy fatigability. Atrial fibrillation (AF) converted spontaneously in one patient while quinidine easily obtained a sinus rhythm in another. The patient with preoperative supraventricular tachycardia has been continued on quinidine and has had no attacks in the last seven months. Only three patients still continue to take digoxin, and use of diuretics has been stopped in all. Five patients have no murmurs; in the remaining five patients the murmurs have decreased significantly in intensity (fig. 4). The mid-diastolic rumble heard preoperatively in nine of the 10 surviving patients is no longer present. The findings of tricuspid regurgitation in three pa-

5 ANNULOPLASTY 107 Signs Murmur Pulmonary Hepatic Mid-diastolic Clinical Hepatic Venous of mitral apical tricuspid Atrial Therapy edema enlargement tenderness distention regurgitation rumble regurgitation* fibrillation Digitalis Diuretics O o o o O o o O o o no associated valvular lesions were present. LV Cardiac A-VO2 Angiographic data ipressure index difference MR TR AR, AS, MS Preop Postop Preop Postop Preop Postop Preop Postop Preop Postop Preop Postop 100/ /10 100/ /76 140/ AA 93/ /12 92/ /25 120/ / / AA 110/70 96/ * - * AA AA 85/8 80/ /8 104/ pressure; LV = left ventricle; A-V = arteriovenous; MR = mitral regurgitation; TR = tricuspid regurgitation; AR = aortic tients and the pulmonary incompetence noted in one have also disappeared. X-rays Figure 5 shows the last preoperative and latest postoperative chest films. Cardiac size decreased significantly in all patients and dramatically in some, with the left atrium Circglation, Volume XXXVIII, July 1968 showing the most striking change. The oblique and lateral views (not shown) confirm these findings. Electrocardiography Two of the three patients in atrial fibrillation converted to normal sinus rhythm. Of the nine patients who had left atrial hyper-

6 KLOTH ET AL. 108 Figure 5 Posteroanterior chest x-rays of the 10 surviving patients. The paired films represent the last x-rays taken before surgery and the most recent postoperative film (respectively, 5, 7, 13, 14, 14, 16, 22, 26, 31, and 60 months after surgery). Pre-op = months preoperative; mos. post-op = months postoperative.

7 ANNULOPLASTY 2C 109 daries of hypertrophy as defined previously. In addition, there has been a general trend toward decreasing voltages of S and R waves in the postoperative period EG YB 10- kh BA I W W~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ IYR. BEFORE ANNULOPLASTY IYR. AFTER 2 YRS. AFTER Figure 6 Mean wedge pressures determined at preoperative and postoperative catheterizations. trophy, seven now have normal P waves; the remaining two patients have had significant reduction in the height and duration of the P wave but continue to fall within the boun- Hemodynamic Studies Left atrial or pulmonary capillary pressure fell to normal or near normal in those patients in whom elevation was present preoperatively (fig. 6). Those patients with normal pressures before operation maintained normal pressures after operation (table 3). Pulmonary artery pressure showed a similar pattern (fig. 7). Selective left ventricular cineangiocardiography was performed in six patients postoperatively. In one patient the catheter could not be advanced into the left ventricle because of severe arterial spasm according to the criteria of Gray and associates8; 801 T SYSTOLIC 70 60r I BM YB RI MEAN DIASTOLIC PO IYR. BEFORE ANNULOPLASTY I YR. AFTER 2 YRS. AFTER Figure 7 Systolic, diastolic, and mean pulmonary postoperative catheterizations. artery pressures determined at preoperative and

8 110 IYR. 8EFORE AWLPATY YR. AFTER 2 YS. ATER Figure 8 Degree of mitral regurgitation determined by selective left ventricular cineangiocardiography. all patients had significant reduction in the degree of mitral regurgitation (fig. 8). Patient Y.B. still has grade 3+ regurgitation, but by all other criteria-decreased pulmonary artery and wedge pressure, smaller wedge V wave, and disappearance of pulmonary incompetence-an excellent result has been achieved. Discussion A comparison of results of the various open heart procedures must include evaluation of operative mortality and morbidity, the degree of success in correcting the hemodynamic abnormalities, and the long-term clinical response. In this group of 11 children there has been one death: the second child operated on died as a result of air embolism. Operative mortality in the entire group of children and adults who have undergone annuloplasty for rheumatic mitral regurgitation in our institutions12 has been 4.3% (three of 70 patients) compared to a mortality of 5 to 15% reported for prosthetic replacement There have been no deaths among the last 60 patients undergoing annuloplasty. As demonstrated by the parameters of clinical evaluation, ECG, x-rays, and cardiac catheterization, all patients have improved remarkably and have sustained this improvement up to 7 years. This parallels the results of Anderson,1" Steinmetz,2 Logan,17 Aldridge,18 Harris,19 and their associates in older patients, and of Kahn and associates20 in children. The majority of our 11 KLOTH ET AL. patients reacquired murmurs of mitral regurgitation. Satisfactory results, however, are not dependent upon complete hemodynamic correction, as shown by our series and by Kahn and associates'20 series in which 18 of 19 patients with rheumatic or congenital mitral valve insufficiency had significant clinical improvement despite the recurrence of significant murmurs. The possible need for reoperation at a later date should not deter the choice of annuloplasty since in fact replacement of the original prosthetic valve, due to wear, malfunction, or the need for a larger valve in the growing child, is an even more likely prospect. Although prosthetic valves frequently function without regurgitation, they present several major disadvantages. Embolization is not uncommon. Bjork and Malers'4 noted embolism in 10 of 19 cases. Ellis and associates' followed 81 patients for periods of up to 5 years and noted that 13 had developed emboli, as did eight of 76 patients reported on by Morrow and co-workers.'5 Of the 24 patients in this last-mentioned group who expired, eight had massive thrombosis of the mitral Starr valve. Mitral valve replacement appears to give rise to emboli more frequently than aortic replacement, and permanent anticoagulation, which is now used routinely in all patients with prosthetic valves, adds the risk of bleeding and does not eliminate the danger of embolization." 14, 15 Another less common disadvantage of prosthetic replacement is that bacterial endocarditis occurring on the silastic material used and in suture lines is frequently refractory to medical therapy and is commonly fatal None of these disadvantages occurs with annuloplasties. The reluctance to operate on children with rheumatic heart disease stems in part from the view that rheumatic activity persists throughout childhood and that congestive failure during this time is always due to acute rheumatic fever. This view seemed to be supported by the persistence of Aschoff bodies in the myocardium for many years following an acute episode of rheumatic fever.23 Recently, however, it has been shown that only a few pa-

9 ANNULOPLASTY tients have "chronic" rheumatic activity, and that congestive heart failure can appear in children with rheumatic heart disease who do not have evidence of rheumatic activity.24 Children in congestive failure, therefore, should not be considered any greater surgical risks than adults if there are no clinical and laboratory signs of rheumatic activity. Children appear to differ from adults in only one parameter postoperatively. Serial x-rays suggest that there is a much greater degree of reduction in left atrial enlargement in children following surgery.'2 This may be related to the length of time that chamber enlargement has existed. Logan and co-workers have suggested this possibility, inferring that long-standing left atrial dilatation leads to destruction of muscular tissue and fibrotic replacement. The latter factor limits reduction in left atrial size when regurgitation is decreased. This suggests that surgery might well be offered to children prior to the development of congestive failure and massive left atrial enlargement in an effort to change the natural history of the disease process. These results suggest that mitral annuloplasty should be the operation of choice in children with severe mitral regurgitation. The operative mortality is low; there is minimal postoperative morbidity and no need for anticoagulants. The ultimate impact on the natural history of mitral insufficiency requires longer follow-up. References 1. ELLIS, F. H., JR., CALLAHAN, J. A., MCGOON, D. C., AND KIRKLIN, J. W.: Results of open operation for acquired mitral valve disease: Comparison of replacement versus reconstructive procedures. New Eng J Med 272: 869, STEINMETZ, G. P., JR., ANDERSON, A. M., COBB, L. A., BRUCE, R. A., AND MERENDINO, K. A.: Posterior medial annuloplasty for acquired mitral insufficiency: Methods and results. Progr Cardiov Dis 5: 280, BIGELOW, W. G., KYPERS, P. J., HEINBECKER, R. O., AND GUNTON, R. W.: Clinical assessment of the efficiency and durability of direct vision annuloplasty. Ann Surg 154: 320, LINDE, L., HARPER, J., CHUANG, K., AND MULDER, D.: Mitral valve replacement in ill children. J Thorac Cardiov Surg 49: 475, LEVY, M. J., VARCO, R. C., LILLEHEI, C. W., AND EDWARDS, J. E.: Mitral insufficiency in infants, children and adolescents. J Thorac Cardiov Surg 45: 434, COLLINs, H. A., DANIEL, R. A., AND SCOTT, H. W.: Surgery for mitral valve disease during childhood and adolescence. J Thorac Cardiov Surg 51: 639, AREVALO, A., SPAGNUOLO, M., AND FEINSTEIN, A.: Simple electrocardiographic interpretation of left atrial enlargement. JAMA 135: 358, GRAY, I. R., JOSHIPU-RA, C. S., AND MACKINNON, J.: Retrograde left ventricular cardioangiography in the diagnosis of mitral regurgitation. Brit Heart J 25: 145, YUAN, S. H., DOYLE, E. F., PISACANO, J. C., AND REED, G. E.: Severe rheumatic mitral insufficiency in childhood amenable to surgery. Pediatrics 33: 571, GORLIN, R., AND GORLIN, S. G.: Hydraulic formulae for calculation of the area of the stenotic mitral valve, other cardiac valves and central circulatory shunts. Amer Heart J 41: 1, REED, G. E., TIcE, D. A., AND CLAUss, R. H.: Asymmetric exaggerated mitral annuloplasty: Repair of mitral insufficiency with hemodynamic predictability. J Thorac Cardiov Surg 49: 752, KLOTH, H. H., REED, G., REPPERT, E., AND SPAGNUOLO, M.: Mitral annuloplasty for the correction of severe mitral insufficiency. Unpublished data. 13. MoRRIs, K.: Open heart surgery for mitral incompetence. New Zeal Med J (suppl. 64): 45, BJORK, V. 0., AND MALERS, E.: Annuloplastic procedures for mitral insufficiency. J Thorac Cardiov Surg 48: 251, MoRRow, A. G., OLDHAM, H., NEWLAND, E., RONALD, C., AND BRAUNWALD, E.: Prosthetic replacement of the mitral valve. Circulation 55: 962, ANDERSON, A. M., COBB, L. A., BRUCE, R. A., AND MERENDINO, K. A.: Evaluation of mitral annuloplasty for mitral regurgitation. Circulation 26: 26, LOGAN, A., TURNER, R. W. D., AND KITCHIN, A. H.: Surgical treatment of mitral incompetence. Brit Heart J 29: 1, ALDRIDGE, H., LIPTON, E., AND BIGELOW, W.: Annuloplasty for mitral insufficiency. Circulation 34: 337, HARRIS, L. C., NGHIEM, Q. X., AND SCHREIBER, M. H.: Rheumatic mitral insufficiency in children, course, prognosis and effect of mitral

10 112 valve replacement. Amer J Cardiol 17: 194, KAHN, D. B., STERN, A. M., SIGMANN, J. M., KIRSH, M. M., LENNOX, S., AND SLOAN, H.: Long term results of valvuloplasty for mitral insufficiency in children. J Thorac Cardiov Surg 53: 1, ROBERTS, W. C., AND MoRRow, A. G.: Bacterial endocarditis involving prosthetic valve. Arch Path (Chicago) 82: 164, COHN, L. H., ROBERTS, W. C., ROCKOFF, S. D., KLOTH ET AL. AND MoRRow, A. G.: Bacterial endocarditis following aortic valve replacement: Clinical and pathological correlation. Circulation 33: 209, TARANTA, A., SPAGNUOLO, M., AND FEINSTEIN, A.: Chronic rheumatic fever. Ann Intern Med 56: 357, SPAGNUOLO, M., AND FEINSTEIN, A.: Congestive heart failure and rheumatic activity in young patients with heart disease. Pediatrics 33: 653, Atrioventricular Valves-Harvey, 1628 There are three forked portals in the entry of the vena cava and arteria venosa, lest that when the blood is most driven out it should fall back, and for that cause they are not in all creatures, and in those in which they are, they do not seem to be made by the same diligence of Nature, but in some they are shut more exactly, in others more carelessly and negligently; therefore in the left ventricle, that for the greater impulsion there may be a closer stoppage, there are only two like a Mitre, having tendons reaching out far, even to the conus of it, through its middle, that they may be most exactly shut... therefore these portals do much surpass in bigness, strength, and exact shutting, those which are placed in the right.-the Anatomical Exercises of Dr. William Harvey: De Motu Cordis 1628; De Circulatione Sanguinis 1649 (first English text). Edited by GEOFFREY KEYNES. London, The Nonesuch Press, 1653, p. 109.