Ventricular Septa1 Defect and Aortic Insufficiency

Transcription

1 THE ANNALS OF THORACIC SURGERY Journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association VOLUME 17 NUMBER 3 * MARCH 1974 Ventricular Septa1 Defect and Aortic Insufficiency Surgical Considerations and Results of Operation Lt Col Peter M. Sanfelippo, MC, USAF, James W. DuShane, M.D., Dwight C. McGoon, M.D., and Gordon K. Danielson, M.D. ABSTRACT Ventricular septal defect (VSD) is uncommonly associated with valvular aortic insufficiency (AI). From 1957 through 1971, 51 patients with this combination underwent operation at the Mayo Clinic; these patients made up 2.2% of all patients with VSD repaired during that period. The most frequent aortic valve abnormality was a prolapsing right cusp. Surgical management of A1 consisted of: (1) no procedure on the aortic valve in 25 patients, (2) plastic repair of the aortic valve in 13 patients, and (3) partial or total replacement of the aortic valve in 13 patients; in the group with severe A1 prior to operation, those treated by replacement did better than those treated by repair. Early closure is indicated for VSD associated with mild AI. With severe AI, VSD closure and valve plication are preferred, although valve replacement may be necessary either at the primary operation or at some future date. In adults, replacement with a prosthesis is indicated for severe AI. V entricular septal defect (VSD), the most common congenital cardiac anomaly [9] and the most frequently encountered lesion requiring surgical treatment in early life [ 13, is uncommonly associated with aortic valve insufficiency (AI). The combination of VSD with AI, however, poses a special problem in management, as it is often necessary to repair the A1 as well as the VSD to achieve a satisfactory result. The large number of From the Departments of Surgery and Pediatrics, Mayo Clinic and Mayo Foundation, Rochester, Minn. The views expressed herein are those of the authors and do not necessarily reflect the views of the Air University, the United States Air Force, or the Department of Defense. The authors appreciate the opportunity to include in this study operations performed at the Mayo Clinic by Drs. Robert B. Wallace, John W. Kirklin, and F. Henry Ellis, Jr. Accepted for publication Oct. 26, 19i3. Address reprint requests to Section of Publications, Mayo Clinic, 200 First St. S.W., Rochester. Minn

2 SANFELIPPO ET AL. operative procedures suggested in the literature for correction of A1 [3, 10, 11, 17-19] attests to the difficulty of managing the combination of VSD with AI. This report summarizes our current management and results of operation in patients with VSD and associated AI. Clinical Material From 1957 through December, 1971, 51 patients with VSD and A1 underwent repair of their lesions at the Mayo Clinic. This group constituted 2.2% of the 2,283 patients with VSD who were operated upon during the same interval. All 51 patients had primary A1 in association with a VSD located beneath the aortic valve. Not included were 26 with secondary A1 caused by bacterial endocarditis, rheumatic valvulitis, closed chest trauma, or operative trauma to the aortic valve during repair of a VSD. Data on the first 19 patients in this series have been reported previously [3]. Of the 51 patients, 28 were male and 23 were female; their ages at operation ranged from 5 to 53 years with a median of 15 years. Fourteen patients had undergone a previous cardiovascular operation; 7 of the 14 had had explorations carried out for a presumed patent ductus arteriosus (6 at other institutions and 1 at the Mayo Clinic); 2 had had an unsuccessful attempt at closure of a VSD at another institution; 1 had had a Brock procedure for valvular pulmonary stenosis; and 4, who also had significant pulmonary stenosis (tetralogy of Fallot), had undergone systemic-to-pulmonary arterial shunting (3 Blalock-Taussig and 1 Potts-Smith-Gibson anastomosis). The diagnosis of VSD had usually been made at a much younger age (birth to 10 years, median 5 months) than had the diagnosis of A1 (2 to 53 years, median 14 years). The symptoms and characteristics of the cardiac murmurs are shown in Table 1, and the hemodynamic features are summarized in Table 2. Although it is difficult to evaluate the degree of A1 TABLE 1. PREOPERATIVE CLINICAL FEATURES OF 51 PATIENTS WITH VENTRICULAR SEPTAL DEFECT AND AORTIC INSUFFICIENCY Clinical Feature Symptoms None Weight < 10th percentile D yspnea Cyanosis Fatigue Congestive failure Respiratory infections Murmurs Systolic & diastolic Continuous Systolic only No. of Patients THE ANNALS OF THORACIC SURGERY

3 Ventricular Septa1 Defect and Aortic Znsuficiency clinically, for the purposes of this study the A1 was considered swere if the systolic blood pressure was greater than twice the diastolic blot Id pressure (38 of 51 patients). Twenty-two of the patients had associated pulmonary stenosis (valvular, subvalvular, or both). Whenever possible, patients were reexamined at the Mayo Clinic one or more times after operation. Follow-up of the patients whs could not return for reexamination was obtained through a detailed questionnaire completed by the patient (or his parent). A second questionnaire, different from that sent to the patient, went to each patient's personal physician. Results Operation was performed using extracorporeal circulation and, usually, moderate hypothermia (30" to 32 C.). Often the perfusate was cooled to lower temperatures before cross-clamping the aorta, and then the temperature was raised to 30"C., permitting the cardiac ischemia time to be somewhat longer than the 15 minutes usually allowed. In 20 patients who required extended operative time on the aortic valve, perfusion of the left and right coronary arteries was instituted. The mean duration of bypass for all patients was 93 minutes. Other associated anomalies repaired at operation are shown in Table 3. The estimated diameters of the VSDs ranged from 0.3 to 3.0 cm. with a median of 1.2 cm. Ten lesions were supracristal (subpulmonary or located directly beneath the pulmonary valve), and 39 were infracristal (located in TABLE 2. PREOPERATIVE HEMODYNAMIC FEATURES OF 51 PATIENTS WITH VENTRICULAR SEPTAL DEFECT AND AORTIC INSUFFICIENCY No. of Feature Patients Value VSD Severe AI' QVQs 21 Range Mean 1.9 PPPS 23 Range Mean 0.31 RP/& 14 Range Mean 0.16 Severe AI' RV-PA gradient (mm. Hg) 18 Range Mean 59 VSD & pulmonary stenosis 'Defined as systolic blood pressure more than twice the diastolic. VSD = ventricular septa1 defect; A1 = aortic insufficiency; Qp = pulmonary flow; Qs = systemic flow; Pp = pulmonary pressure; Ps = systemic pressure; Rp = pulmonary resistance; Rs = systemic resistance. VOL. 17, NO. 3, MARCH,

4 SANFELIPPO ET AL. TABLE 3. ASSOCIATED LESIONS REPAIRED IN 24 PATIENTS WITH VENTRICULAR SEPTAL DEFECT AND AORTIC INSUFFICIENCY Associated Lesion No. of Patients Valvular & subvalvular 22 pulmonary stenosis Patent ductus arteriosus 2 Atrial septa1 defect 2 Aortic sinus fistula 4 Subvalvular aortic stenosis 1 the membranous septum); for 2 lesions the locations were not specified. Exposure of the VSD was accomplished through a right ventriculotomy in all patients except for 2 in whom it was visualized through the aorta. Closure of the VSD was effected with a patch in 27 patients and by direct suture in 24. At operation it was found that the A1 was due to deformity or prolapse of an aortic cusp in 24 patients (right cusp in 22, noncoronary cusp in 1, noncoronary and left cusp in 1). A dilated annulus or diffuse involvement of all three cusps produced a central leak in 8 other patients. In 19 patients the cause of the A1 was not indicated or could not be determined because the aorta was not opened. The prolapsed cusp, when present, was usually described as being abnormal, with rolled edges, central cusp thickening, and elongation of the free edge. Four patients had calcification of the prolapsed cusp. Operative management of the A1 is shown in Table 4. Various types of plastic repairs were attempted in 13 patients: plication of the redundant cusp near the commissures (7 patients), plication of the leading edge and pericardial patch enlargement of the cusp (2 patients), patch enlargement alone (2 patients), plication of the leading edge of the cusp at its central portion (1 patient), and excision of a wedge of the cusp with repair (1 patient). Early Results. Five patients died after the operation; only 1 of the deaths was directly related to uncorrected AI. None of the 17 patients operated upon during the most recent 5-year period of the study died. Severe A1 was corrected in all patients who underwent replacement of the aortic valve except in 2 of the 5 patients who received Teflon leaflets. A disappointingly large number of patients (7 of 13) who had plastic repair of their valves continued to have severe A1 after operation, with no differences in results according to the type of plastic repair. Approximately the same percentage of patients (6 of 12) had severe residual A1 when no procedure was done for the aortic valve. Late Results. Follow-up information of as long as 14 years was available for all the surviving patients; the median follow-up was 6 years. There 216 THE ANNALS OF THORACIC SURGERY

5 LE 4. AORTIC VALVE PROCEDURES IN 51 PATIENTS WITH VENTRICULAR SEPTAL DEFECT AND AORTIC INSUFFICIENCY Severe AI' In Survivorsb No. of Age (Yr4 No. of Early Late Reoperation ocedure Patients Range - Median Survivors Preop. Postop. Postop. for A /12 4/12 0 repair /12 8/12 3 cement I /11 1/11 4 2' on leaflets ograft on valve r-edwards lve otal /35 13/35 7 stolic blood pressure two times diastolic blood pressure. atients surviving operation and postoperative hospitalization. ate development of A1 due to deterioration of prosthesis in 1 patient. = aortic insufficiency. 1" 1" 0

6 SANFELIPPO ET AL. were 6 late deaths. One patient died of pulmonary vascular obstructive disease 1 months after operation. Four late deaths were related to reoperation for severe AI; 1 patient had received a Teflon leaflet, and the other 3 had undergone plastic repair of the aortic valve. The sixth patient, who had late failure of a Teflon leaflet which was successfully replaced by a Starr- Edwards prosthesis, developed acute bacterial endocarditis on the prosthesis 8 years after operation. He had been in functional Class I (N.Y.H.A.) prior to his terminal illness. Two additional patients treated by valve replacement required reoperation because of later deterioration of the prosthesis. In 1, a Starr-Edwards prosthesis was required 8 years after placement of a Teflon prosthetic valve, and the patient was well 3 years later; in the other, a new homograft valve was required 4 years after placement of an aortic homograft, and the patient was well 2 years later. Thirteen patients had severe A1 but were able to carry on their normal activities and were considered functionally improved over their preoperative status; only 2 of the 13 were on digitalis therapy. Twenty-seven patients (including the 2 previously mentioned patients who underwent reoperation for late prosthetic failure) were free of severe AI; 1, a 53-year-old man, continued with digitalis therapy after the operation although he had no clinical evidence of failure. The other 26 patients were given no cardiac medications. The number of patients with late A1 is shown in Table 4. Eleven patients underwent postoperative cardiac catheterization to document clinically suspected abnormalities. Three had residual or recurrent VSDs with a left-to-right shunt of 11, 22, and 40%, respectively. Two had undergone suture closure of the VSD in our early experience, and the third had had a patch closure. The patient with the largest shunt underwent reoperation for closure of the VSD and repair of the residual AI. The data available from these studies did not include quantitation of the AI. Comment As summarized by Van Praagh and McNamara [22], competence of the aortic valve is dependent on three factors: (1) normal support from above (commissures), (2) normal support at the leaflet level (leaflet apposition during diastole), and (3) normal support from below (conal septum). Although A1 appears to be acquired postnatally [9, 121, its anatomical basis is thought to be one or more congenital malformations involving the three factors related to aortic leaflet support [22]. Absent or defective develop ment of the conal septal portion of the crista supraventricularis is suspected to be a major cause of AI, particularly in the supracristal type of VSD [22]. Others have commented on the close relationship among aneurysms of the sinus of Valsalva, supracristal VSD, and A1 [15, 201. Ventricular septal defects may be large or small, and some larger ones 218 THE ANNALS OF THORACIC SURGERY

7 Ventricular Septa1 Deject and Aortic Znsuficiency may be functionally small because of prolapse or herniation of the aortic leaflet into or through the VSD during diastole. A characteristic counterclockwise rotation of the aortic valve of about 20 to 40 degrees (looking from the aorta toward the ventricle), so that the anterior commissure between the right and noncoronary cusps points more to the left, has been described pathologically [12] and at operation, particularly in patients with a supracristal defect [lo, 161. The overall incidence of A1 associated with VSD in our surgical series was 3.4y0 (77 of 2,283 patients), but the incidence of primary AI, as it was defined for this study, was 2.2ojb (51 patients). These figures are comparable to the 2 to 5% reported in the literature [2, 9, 121. The clinical aspects of VSD and A1 have been well described in previous reports [8, 9, 121. Of interest is the presence of a continuous murmur in 12 of the 51 patients in the present series. In 7 patients this led to an erroneous diagnosis of patent ductus arteriosus and to thoracotomy. Similar experiences have been reported by others [13, 16, 171. The roentgenographic aspects of VSD associated with A1 have been reviewed by Deutsch and colleagues [2]. The late appearance of the A1 murmur in our series confirms the findings of others [9, 12, 131 and suggests that valve incompetence is a progressive feature. This would lend support to the argument proposed by some [5, 9, 201 for early repair of the VSD so that the onset of significant A1 may be prevented or delayed. Closure of a VSD diminishes valve incompetence in some patients [5, 13, 14, 201, presumably by supporting a prolapsing right coronary cusp. It has been suggested that closure of the VSD without a patch will increase AI, so all VSDs should be closed with a patch [23]; but the opposite viewpoint also has been expressed [221. Improvement of A1 has followed both suture and patch closure of the VSD in our series and in those of others [5, 13, 14, 201. Improvement of A1 cannot be ensured in most instances of VSD closure, but it is interesting that hemodynamically severe A1 (systolic blood pressure of more than double the diastolic blood pressure) was converted to moderate or mild A1 in 6 of 12 patients in the present series who had closure of their VSD alone (3 with a patch and 3 by direct suture). Some investigators have suggested that supracristal VSD with A1 may be cured by VSD closure alone but that in infracristal VSD with AI, VSD closure alone does not cure the A1 [22]. This has not been our experience. Hemodynamically severe A1 was converted to moderate or mild A1 in 6 of 12 survivors with infracristal VSD when only the VSD was closed. Furthermore, aortic valve repair or replacement was considered necessary for all 7 patients with severe A1 and supracristal VSD. Treasure and colleagues [ZO] described 3 patients out of 9 with infracristal VSD and A1 who lost all evidence of A1 with closure of the VSD alone, and Weldon [23] reported that A1 was a much greater problem with the supracristal variety of VSD than with the infracristal type. Therefore, significant A1 probably should be managed VOL. 17, NO. 3, MARCH,

8 SANFELIPPO ET AL. at operation according to factors other than whether or not the VSD is supracristal or infracristal. Various operative approaches have been suggested for repair of VSD associated with AI. For most of the patients in our series, a right ventriculotomy was made high in the outflow tract, and this was combined with an aortotomy when an aortic valve procedure was contemplated. Whenever significant subvalvular pulmonary stenosis is present, a ventriculotomy offers the best exposure for complete relief of stenosis and closure of the VSD. Also, when repair of A1 is planned using one of the valvuloplasty techniques, both incisions should be made. The aortic cusps should be repaired first and the effectiveness of the valvuloplasty evaluated by inspecting the aortic valve through the VSD. Residual insufficiency may be corrected by reopening the aorta and performing further valvuloplasty [18] or valve replacement. The VSD is then closed as the final step in the repair. A single transaortic exposure of both defects has been proposed by several workers [4, 6, 7, 161. Only 2 patients in our series had their lesions repaired in this manner. This approach is best used in older patients in whom the aortic valve annulus is large enough to permit adequate visualization and closure of the VSD, and it may be preferable in patients who have recurrent VSD or in whom the coronary anatomy is obscured by adhesions because they have had other cardiac procedures. Two of the earliest innovations for repair of A1 were plication of the elongated leading edge of the right coronary cusp [I71 and suturing the leaflet to the aortic wall or to adjacent cusps [19]. Other modifications of cusp plication and valvuloplasty have been suggested [3, 10, 11, 14, 181. Plication procedures have given some good results, but the incidence of immediate and late A1 has been disappointing in many series [3, 6, 13, 16, 201. Good results using various plication repairs have been reported by some [18, 211. Another suggested approach for repair of VSD with A1 is that of utilizing the prolapsed right cusp to close the VSD, followed by insertion of an aortic valve homograft [6, 161. We have not used the right cusp to close the VSD, but we have used aortic homografts in 2 patients. One of the homografts failed, necessitating replacement with another homograft 4y2 years postoperatively. In our early experience, procedures designed to shorten the leading edge of the prolapsed aortic cusp were not consistently satisfactory, whether the procedure consisted of taking the tucks in the leading edge or suturing the leaflet to the aortic wall or to adjacent cusps. Particularly unsatisfactory were those procedures designed to lengthen the distance between the leading edge of the right coronary cusp and its base. Either aortic valve incompetence persisted after these plastic procedures or it recurred early in the postoperative period. In 2 of 3 patients who underwent reoperation it appeared that the sutures had torn out of the involved tissues. This led to a policy of 220 THE ANNALS OF THORACIC SURGERY

9 Ventricular Septa1 Defect and Aortic Znsuficiency deferring operation in growing children unless significant symptoms were present. Operation was advised when the patient was about 12 to 14 years old, with the plan being to replace the aortic valve if the A1 was severe [3]. One of the hazards of allowing a serious hemodynamic defect to persist, however, is that severe cardiac hypertrophy and dilatation are produced, resulting in impaired performance of the myocardium that may persist after subsequent repair of the lesions. A dogmatic decision regarding the best management of these patients cannot be proposed on the basis of this study alone. Our approach, however, recognizes that valve repair is preferable to valve replacement, particularly in younger patients, if hemodynamically good results can be obtained. Currently our approach would follow these guidelines: In the presence of VSD with a hemodynamically significant left-to-right shunt, repair of the VSD is indicated and repair of the A1 is done only if it is more than mild. In some patients the A1 may improve or at least not get worse. In the presence of moderate or severe AI, one of the currently accepted plication techniques should be considered. If satisfactory aortic valve competence cannot be restored, valve replacement should be performed in preference to leaving significant incompetence. With plication techniques, severe incompetence can recur late. If it does, reoperation with valve replacement is indicated. In adults, the aortic valve probably should be replaced primarily with a prosthesis if the A1 is severe. In the case of VSD without a significant left-toright shunt, operation may be deferred if (1) the A1 is not severe, (2) the patient is asymptomatic, (3) the heart is small, and (4) there is no significant pulmonary outflow obstruction. References 1. Ching, E., DuShane, J. W., McGoon, D. C., and Danielson, G. K. Total correction of cardiac anomalies in infancy using extracorporeal circulation: Surgical consideration and results of operation. J. Thorac. Cardiovasc. Surg. 62:117, Deutsch, V., Blieden, L. C., Kraus, Y., Yahini, J. H., and Neufeld, H. N. Ventricular septal defect associated with aortic insufficiency. Am. J. Roentgenol. Radium Ther. Nucl. Med. 106:32, Ellis, F. H., Jr., Ongley, P. A., and Kirklin, J. W. Ventricular septal defect with aortic valvular incompetence: Surgical considerations. Circulation 27:789, Garamella, J. J., Cruz, A. B., Heupel, W. H., Dahl, J. C., Tensen, N. K., and Berman. R. Ventricular septal defect with aortic insufficiency: Successful surgical correction of both defects by the transaortic approach. Am. J. Cardiol. 5: 266, Glasser, S. P., Cheitlin, M. D., McCarty, R. J., Haas, J. H., Hall, R. J., and Mullins, C. E. Thirty-two cases of interventricular septal defect and aortic insufficiency: Clinical, hemodynamic and surgical features (abstract). Am. J. Cardiol. 29: Gonzalez-Lavin, L., and Barratt-Boyes, B. G. Surgical considerations in the treatment of ventricular septal defect associated with aortic valvular incompetence. J. Thorac. Cardiovasc. Surg. 57:422, 1969.

10 SANFELIPPO ET AL. 7. Hufnagel, C. A., and Conrad, P. W. Transaortic repair of ventricular septal defects. Am. J. Surg. 110:448, Keck, E. W. 0.. Ongley, P. A., Kincaid, 0. W., and Swan, H. J. C. Ventricular septal defect with aortic insufficiency: A clinical and hemodynamic study of 18 proved cases. Circulation 27:203, Keith, J. D., Rowe, R. D., and Vlad, P. Heart Disease in Infancy and Childhood (2d ed.). New York: Macmillan, Pp and Appendix V. 10..Murphy, D. A., and Pokier, N. A technique of aortic valvuloplasty for aortic insufficiency associated with ventricular septal defect. J. Thorac. Cardiovasc. Surg. 64:800, Mustard, W. T. The operative closure of ventricular septal defects in childhood. Postgrad. Med. J. 37:653, Nadas, A. S., Thilenius, 0. G., LaFarge, C. G., and Hauck, A. J. Ventricular septal defect with aortic regurgitation: Medical and pathologic aspects. Circulation 29:862, Plauth, W. H., Jr., Braunwald, E., Rockoff, S. D., Mason, D. T., and Morrow, A. G. Ventricular septal defect and aortic regurgitation: Clinical, heme dynamic and surgical considerations. Am. J. Med. 39:552, Robinson, G., Fell, S. C., and Jacobson, B. E. Ventricular septal defect with aortic insufficiency: A method of management. J. Thoruc. Cardiovasc. Surg. 43:785, Sakakibara, S., and Konno, S. Congenital aneurysm of the sinus of Valsalva associated with ventricular septal defect: Anatomical aspects. Am. Heart J. 75:595, Somerville, J., Brandao, A., and Ross, D. N. Aortic regurgitation with ventricular septal defect: Surgical management and clinical features. Circulation 41317, Spencer, F. C., Bahnson, H. T., and Neill, C. A. The treatment of aortic regurgitation associated with a ventricular septal defect. J. Thorac. Cardiovasc. Surg. 43:222, Spencer, F. C., Doyle, E. F., Danilowicz, D. A., Bahnson, H. T., and Weldon, C. S. Long-term evaluation of aortic valvuloplasty for aortic insufficiency and ventricular septal defect. J. Thorac. Cardiovasc. Surg. 65: 15, Starr, A., Menashe, V., and Dotter, C. Surgical correction of aortic insufficiency associated with ventricular septal defect. Surg. Gynecol. Obstet. 111:71, Treasure, R. L., Hopeman, A. R., Jahnke, E. J., Green, D. C., and Czamecki, S. W. Ventricular septal defect with aortic insufficiency. Ann. Thorac. Surg. 12:411, Trusler, G. A. Discussion of Treasure et al. [20], p Van Praagh, R., and McNamara, J. J. Anatomic types of ventricular septal defect with aortic insufficiency: Diagnostic and surgical considerations. Am. Heart J. 75:604, Weldon, C. S. Discussion of Spencer et al. [18], p THE ANNALS OF THORACIC SURGERY