VENTRICULAR SEPTAL DEFECT WITH AORTIC INSUFFICIENCY

VENTRICULAR SEPTAL DEFECT WITH AORTIC INSUFFICIENCY

VENTRICULAR SEPTAL DEFECT W I T H AORTIC INSUFFICIENCY A Method of Management George Robinson, M.D., Stanley C. Fell, M.D., and Belle E. Jacobson, M.D...

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VENTRICULAR SEPTAL DEFECT W I T H AORTIC INSUFFICIENCY A Method of Management George Robinson, M.D., Stanley C. Fell, M.D., and Belle E. Jacobson, M.D., New York, N. Y.

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HE surgical treatment of ventricular septal defect associated with aortic insufficiency due to prolapse of an aortic cusp warrants further elucidation. This syndrome must be differentiated from a variety of other congenital, ac­ quired, or mixed forms of heart disease. Scott10 summarized the previously reported cases and discussed the differential diagnosis of this lesion. The most frequent error in diagnosis has been the confusion of this lesion with patent ductus arteriosus. 6 ' 12 Other lesions from which it must be differentiated include ruptured aneurysm of a sinus of Valsalva, aorticopulmonary septal defect, ven­ tricular septal defect with or without patent ductus arteriosus, and ventricular septal defect with acquired aortic insufficiency or bicuspid aortic valve.10' " Ledbetter 9 established the diagnosis in one case by dye dilution techniques, employing right-heart catheterization, direct puncture of the left ventricle, and retrograde catheterization of the aorta. Retrograde aortography has also been employed to confirm the diagnosis. 11 The anatomy of this combined lesion is not complex, but variations in its components are to be expected. The defect in the ventricular septum occurs in the membranous portion. There is ordinarily not a large interventricular com­ munication, and the size of this communication is determined partially by the degree to which the unsupported aortic cusp is adherent to the superior, anterior, and posterior margins of the defect. Further, the magnitude of the shunt is in part determined by the extent to which the aneurysmally dilated aortic valve cusp descends in diastole and occludes the interventricular communication. Aor­ tic regurgitation results when there is failure of apposition of the corpora arantii and lunulae of the three aortic leaflets, as one cusp sags unsupported in diastole to a level below its neighbors. In previously reported cases,10' u the right cusp prolapsed most frequently; prolapse of the noncoronary cusp was much less common, and prolapse of both right and noncoronary cusps was rare. In the case detailed below, intraoperative visualization of the lesion through a right ventriculotomy prompted the use of the method described. From the Surgical and Medical Divisions, Monteflore Hospital, New York City. Received for publication July 17, 1961.

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ROBINSON, FELL, JACOBSON

786 CASE REPORT

F . H., a 12-year-old Negro boy, was first seen at the Montefiore Hospital Cardiac Clinic on Feb. 11, 1958. I n early infancy, a congenital cataract of the right eye with microphthalmus and a heart murmur were noted. There were no symptoms of diminished cardiac reserve except easy fatigability. Two siblings and a twin had no evidence of congenital heart disease. Physical examination revealed a well-developed thin child with microphthalmus and a cataract of the right eye. At various times the recorded blood pressures ranged from 90/40 to 135/60 mm. Hg. A prominent apical impulse was present one centimeter to the left of the

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Fig. 1.—A, Preoperative phonocardlogram. Symbols: I = second sound. II = second sound. Ill = third sound. S.M. = systolic murmur. D.M. = diastolic murmur. S.C. = systolic click. I.C.S. = intercostal space. L.S.B. = left sternal border. M.C.L. = midclavicular line. B, Postoperative phonocardiogram. Note the decrease in intensity and duration of the systolic, murmur which now terminates in midsystole. The diastolic murmur and the systolic click have disappeared. midclavicular line in the fifth intercostal space. A systolic thrill was palpable in the second and third intercostal spaces on the left, and a Grade 4/6 harsh, high-pitched pansystolic mur­ mur was best heard at the left sternal border in the third intercostal space. A moderately loud blowing murmur was heard early in diastole in the second right intercostal space and along the left sternal border in the third intercostal space (Fig. 1, A). Serial electrocardiograms showed a tall R. and a deep S in V, and V2, consistent with left ventricular hypertrophy. Fluorscopy demonstrated a widened aorta with expansile pulsa­ tions, and moderate enlargement of both ventricles and the left atrium. Roentgenograms of

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the chest showed moderate cardiac enlargement, predominantly of the left ventricle, with increased vascularity of the lung fields. The clinical impression was ventricular septal defect associated with aortic valvular insufficiency or patent ductus arteriosus. Right-heart catheterization was performed on Sept. 25, 1958 (Table I ) . The findings were those of a small ven­ tricular septal defect without evidence of a patent ductus. A marginally broadened brachial artery pulse-pressure was noted, with a normal upstroke time.

TABLE I.

CARDIAC CATHETERIZATION DATA

Superior vena cava Inferior vena cava Right atrium Right ventricle

71 70 71.5 75

Pulmonary artery

75

Brachial artery

92

Brachial artery, upstroke time Data Obtained at Right atrium Right ventricle Pulmonary artery Left atrium Left ventricle Before repair After repair Femoral artery Before repair After repair

PRESSURE (MM. H g )

0 2 SATURATION (PER CENT)

SITE

1 mm. (mean) 35-40 0 35-42 10-12 125-140 62-68 715-925 mm. Hg/sec.

Operation 80 87 90 98 150/0 120/0 130/50 130/80

Operation was performed on June 3, 1960, utilizing a median sternotomy. The right ventricle was markedly enlarged, and there was moderate enlargement of the left ventricle, pulmonary artery, and aorta. The right aortic sinus of Valsalva was dilated, and thrills were palpable over the right ventricle, pulmonary artery, and aorta. The pressures and oxygen saturations recorded a t operation (Table I ) revealed a significant shunt at the ventricular level. The blood pressure in the femoral artery was 130/50 mm. Hg. After the usual cannulations had been performed, extracorporeal circulation was established, utilizing a modified DeWall oxygenator and a Sigmamotor pump at a flow rate of 66 c.c. per kilogram of body weight per minute. A 2 cm. incision was made in the outflow tract of the right ventricle. The aorta was not cross-clamped. Continuous and alarming loss of bright and dark blood suggested that a patent ductus arteriosus had been overlooked. Digital exploration of the interior of the pulmonary artery did not disclose a ductus. The ventriculotomy was quickly closed and the perfusion terminated. Intrapericardial exploration of the ductus area confirmed the absence of a patent ductus arteriosus. Extracorporeal circulation was re-established, and, with the ascending aorta crossclamped, the ventriculotomy was reopened. The septal defect was visualized high in the mem­ branous septum. I t was approximately 2.5 cm. in diameter, and the right aortic cusp was adherent to its margins for approximately two thirds of its circumference; thus, the interventricular communication was a small crescentie orifice inferiorly. The superior border of the defect consisted of white fibrous tissue which appeared to be a portion of the annulus of both the pulmonic and aortic valves. With release of the aortic clamp, the aneurysmally dilated right aortic cusp herniated into the right ventricle (Wg. 2 ) , and the field was rapidly flooded

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J. Thoracic and Cardiovas. Surg.

by a regurgitant flow of aortic blood. The ventricular septal defect was repaired with a prosthesis of compressed Ivalon sponge, which served also to buttress the right aortic cusp (Fig. 3 ) . Extreme care was required a t the superior margin of the defect to avoid perforation of the pulmonie and adjacent aortic valve cusps. During the performance of the repair it was necessary to cross-clamp the aorta intermit­ tently for periods of 5 to 10 minutes. Following the insertion of the prosthesis, and prior to closure of the ventriculotomy, the aortic clamp was released. There was no evidence of left ventricular dilatation or excessive leakage through the prosthesis to suggest the presence of residual aortic insufficiency. At the conclusion of cardiopulmonary bypass, lasting 26 minutes, heart action was excellent, despite the long intervals of anoxic arrest. The blood pressure in the femoral artery remained stable at 130/80 mm. Hg. The p a t i e n t ' s postoperative course was uneventful and he was discharged on the four­ teenth postoperative day.

Fig. 2.—The septal defect as visualized via right ventriculotomy. Upon release of the aortic clamp the right aortic cusp dilates and prolapses through the ventricular septal defect. Note the relationship of the septal defect to the pulmonie valve. Inset: The mechanism of aortic insufficiency due to cusp prolapse is illustrated diagrammatically. Arrow indicates the shunting of blood through the interventricular communication.

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Phonocardiograms taken on the twelfth postoperative day demonstrated a marked de­ crease in intensity and duration of the systolic murmur, and absence of the diastolic murmur and systolic click (see Fig. 1, B). Seven months after operation, the phonocardiogram was unchanged, except that a faint, well-localized diastolic murmur can be recorded only after diligent search. Blood pressures range between 100-110/60-70 mm. Hg. The child is more active and no longer experiences undue fatigue. DISCUSSION

The anatomy of the defect observed at operation deserves further descrip­ tion. It is important to differentiate sharply between two descriptive terms: inter ventricular communication and ventricular septal defect. The former refers

Fig. 3.—A prosthesis has been circuraferentially sutured to the true margin of the ventricular septal defect. Inset: Sectional view demonstrates effective buttressing of the right aortic cusp by the prosthesis. With the cusp retained within the aortic lumen there is adequate approximation of the leaflets in diastole.

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ROBINSON, FELL, JACOBSON

J. Thoracic and Cardiovas. Surg.

to the crescentic opening between the chambers which has its margins formed by aortic cusp superiorly and by ventricular septum elsewhere. The term ventricular septal defect refers to the total zone of deficient membranous septum. This zone was largely occluded by the adherent aortic valve cusp and further occluded in diastole by descent of the cusp into the communication. Closure of the interventricular communication may be effected by suture of the inferior surface of the prolapsed cusp to the adjacent septum; this entails the risks of cusp perforation and further valve deformity. A suture repair anchoring the lower border of the defect to the superior margin, formed by the annulus of the aortic and pulmonie valves, would close the ventricular septal defect. This repair would be under tension and would also deform the prolapsed aortic cusp. 7 ' 8 Starr and co-workers11 reported a case in which there was successful treat­ ment by suture repair of the ventricular septal defect via right ventriculotomy, with subsequent transaortic commissural suture and cusp suspension. Gold­ man, 4 ' 5 discussing a similar problem in an adult without associated ventricular septal defect, stated that he had utilized segments of autogenous artery to splint the prolapsed cusp to its normal neighbors. Bahnson, 1 ' 2 however, states that he has not achieved successful results utilizing commissural coaptation in cases of aortic insufficiency associated with ventricular septal defect. He prefers to shorten the free edge of the prolapsed cusp by reefing, but notes that it is diffi­ cult to determine how much reefing is required. Garamella and associates3 reported the first successful transaortic correc­ tion of this syndrome in a patient in whom the ventricular septal defect was not diagnosed on preoperative catheterization, since the prolapsed right aortic cusp effectively occluded the interventricular communication. These authors state, however, that their successful result might be fortuitous, and suggest separate exposures in the aorta and right ventricle when the septal defect is difficult to repair from above. In the presently reported case, transaortic repair of both the ventricular septal defect and the prolapsed cusp would have been difficult because of the paucity of tissue in the superior margin of the defect, despite complete mobiliza­ tion of the cusp. Although aortic insufficiency was undoubtedly not severe, under the circumstances of cardiopulmonary bypass and right ventriculotomy the operative field was inundated with regurgitant flow. The prosthesis applied to the true margins of the defect served both to support the aortic cusp and correct the prolapse, which is a basic mechanism in this type of aortic insuf­ ficiency.1' e It is interesting to note that Starr 11 observed that digital support of the aortic valve ameliorated aortic regurgitation in an unsuccessful attempt at correction of this combined lesion. Closure may not be dynamically insufficiency, age through

of the ventricular septal defect employing a prosthesis as described suitable in instances of this syndrome with aortic regurgitation more severe than was encountered in this case. Residual aortic as demonstrated by left ventricular dilatation and excessive leak­ the prosthesis upon release of the aortic clamp, as well as unsatis-

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factory elevation of the diastolie pressure upon cessation of cardiopulmonary bypass, would indicate the need for aortotomy and cusp plication or suspension. It is suggested, however, that initial application of a prosthetic support may be advantageous for the following reasons: corrective suturing of the delicate aortic cusp may be minimized, the duration of the aortotomy and associated problems of myocardial perfusion would be shortened, and the prosthetic sup­ port may serve to lessen the stress exerted upon a sutured leaflet during diastole. SUMMARY

The case of a patient with aortic valvular insufficiency associated with ven­ tricular septal defect is presented, and the operative findings are described. The application of a prosthesis to the ventricular septal defect served to correct the aortic insufficiency by supporting the cusp and preventing its prolapse. In situations in which this method does not afford complete correction, cusp sus­ pension or shortening may be performed through a supplementary aortotomy. The assistance of Dr. Doris J . W. Escher, Director of the Cardiac Catheterization Laboratory, is gratefully acknowledged. REFERENCES 1. Bahnson, H. T.: Discussion of Kirklin, J . W., McGoon, D. C. and DuShane, J . W.: Surgical Treatment of Ventricular Septal Defect, J . THORACIC SURQ. 40: 805, 1960. 2. Bahnson, H. T.: Personal communication. 3. Garamella, J . J., Cruz, A. B., Heupel, W. H., Dahl, J. C , Jensen, N. K., and Berman, R.: Ventricular Septal Defect With Aortic Insufficiency. Successful Surgical Correction by the Transaortic Approach, Am. J . Cardiol. 5: 266, 1960. 4. Goldman, A.: Discussion of Harken, D. E., Soroff, H. S., Taylor, W. J., Lefemine, A. A., Gupta, S. K. and Lunzer, S.: Partial and Complete Prosthesis in Aortic Insufficiency, J. THORACIC SURG. 40:

804,

1960.

5. Goldman, A.: Personal communication. 6. Gross, R. E . : The Patent Ductus Arteriosus. Observations on Diagnosis and Therapy in Five Hundred and Twenty Five Surgically Treated Cases, Am. J . Med. 12: 472, 1952. 7. Kirklin, J . W.: Discussion of Kirklin, J. W., McGoon, D. C , and DuShane, J . W.: Sur­ gical Treatment of Ventricular Septal Defect, J . THORACIC SURG. 40: 807, 1960. 8. Kirklin, J. W.: Personal communication. 9. Ledbetter, M. K., and Daugherty, G. W.: Ventricular Septal Defect With Aortic Regurgitation, Proc. Staff Meet. Mayo Clin. 33: 600, 1958. 10. Scott, R. C , McGuire, J., Kaplan, S., Fowler, N. O., Green, R. S., Gordon, L. Z., Shabetai, R., and Davolos, D. D.: The Syndrome of Ventricular Septal Defect With Aortic Insufficiency, Am. J . Cardiol. 2: 530, 1958. 11. Starr, A., Menashe, V. and Dotter, C.: Surgical Correction of Aortic Insufficiency As­ sociated With Ventricular Septal Defect, Surg. Gynec. & Obst. I l l : 71, 1960. 12. Winehell, P., and Bashour, F . : Ventricular Septal Defect With Aortic Incompetence Simulating Patent Ductus Arteriosus, Am. J . Med. 20: 361, 1956.