Subannular aortic aneurysms in whites

J

THoRAc CARDIOVASC SURG

92:950-952, 1986

Subannular aortic aneurysms in whites Report of two cases and review of literature Subannular aortic aneurysms are a rare entity occurring predominantly in young black men. Five white patients have been reported who underwent surgical correction, but long-term survival occurred in only two. We report two white men, 36 and 45 years old, who survived aortic valve replacement and direct suture of subannular aneurysms, with no symptoms at 29 and 42 postoperative months.

Renato A. K. Kalil, M.D., Roque Falleiro, M.D., Domingos Vitola, M.D., Paulo R. Prates, M.D., Joao Ricardo Sanfanna, M.D., Fernando A. Lucchese, M.D., and Ivo A. Nesralla, M.D., Porto Alegre, Brazil

Although subannular aortic aneurysm seems to have been first noted in Corvisart in 1813, the term "annular subvalvular left ventricular aneurysm" was introduced by Abrams and associates' in 1962, with a report of 12 cases observed in Nigeria. This is a rare malformation.s 3 more frequent in blacks, with few reports in whites.'" It has been almost exclusively described in the African population. The majority of these aneurysms originate below the mitral valve anulus and measure 3 to 8 em in diameter.v' A few are located in a subaortic position. By extension, they can cause valvular regurgitation, pericarditis, ventricular arrhythmias, and myocardial ischemia by compression of a coronary artery.v' The cause remains uncertain, but several hypotheses have been suggested.l? including tuberculous, ischemic, traumatic, and congenital origins. Congenital origin is most widely accepted. ',2.5 The rarity of this entity in whites and the paucity of reports of successful surgical repair have motivated our report of clinical findings and long-term results in two white patients. Case reports CASE 1. A 36-year-old white man with a 9 month history of dyspnea was hospitalized for investigation of congestive heart

From Instituto de Cardiologia do Rio Grande do Sul/Fundacao Universitaria de Cardiologia, Porto Alegre, Brazil. Received for publication Sept. 9, 1985. Accepted for publication Jan. 14, 1986. Address for reprints: Dr. Renato A. K. Kalil, Instituto de Cardiologia do RS, Avenida Princesa Isabel 395, 90.000 Porto Alegre RS, Brazil.

950

failure (New York Heart Association Class IV), There was no evidence of prior infective endocarditis. Physical examination revealed good general appearance, no fever, and appropriate hydration. Ictus cordis was localized to the seventh intercostal space, anterior axillary line, with an impulsive feature. Heart rate was 90 beats/min with normal sinus rhythm. There was an ejection systolic Grade 2/6 murmur and an aspiration diastolic murmur in the aortic area. There were no abnormalities in the abdomen or extremities. The electrocardiogram had a pattern of left ventricular overload and secondary ST-T changes. A chest roentgenogram showed increased heart volume, aortic dilation, and pulmonary congestion, Echocardiography disclosed a calcified aortic valve, fine diastolic vibrations of the anterior mitral leaflet, increased left atrial and left ventricular volumes, with an ejection fraction of 0.45. Cardiac catheterization revealed left ventricular systolic pressure of 132 mm Hg and aortic systolic pressure of 126 mm Hg. Left ventricular end-diastolic pressure was 36 mm Hg (Table I). Cineangiography showed the aortic valve to be thick, with severe regurgitation and increased left ventricular volume. At operation the heart was markedly enlarged and the aortic valve was bicuspid with calcified leaflets. There was an aneurysm in the area of confluence of the noncoronary aortic, left coronary aortic, and anterior mitral leaflets, with posterior extension to the atria. A bioprosthetic valve was implanted in the aortic position, and the aneurysmal cavity was occluded with buttressed mattress sutures of polyester and Teflon felt. The postoperative course was uneventful, and the patient left the hospital on the fifteenth day using digoxin and a thiazide diuretic. Forty-two months after the operation he continues to take digoxin and thiazide diuretic and is free of symptoms. CASE 2. A 46-year-pld white man had dyspnea for 2 years. There was no evidence of prior infective endocarditis. He came to our institution with dyspnea at rest for 2 weeks. Physical examination revealed an unremarkable general state, with pale mucosa and body temperature of 37.8° C. The ictus cordis was nonpalpable. Pulmonary sounds were low in the left base, with dullness on percussion and no thrill on palpation. Heart rhythm was regular, and there was a Grade 3/6 systolic

Volume 92 Number 5 November 1986

Subannular aortic aneurysms

95I

Table I. Cardiac catheterization data Chamber

Right atrium

Wave

Patient J

a x m

4

6

"7

6

42 8 41 20

44 EDP

Pulmonary wedge

II

10

Right ventricle Pulmonary artery

Patient 2

s d m a v x m

Left ventricle EDP

Aorta d m

II

44' 25 30 31

28

CIleUrysm below right co~ary SinuS

26 28 17

20 23 110 36 90 52

2T

222 22 90 60 70

72

Legend: All results given in millimeters of mercury (mm Hg). EDP, Enddiastolic pressure. s, Systolic. d, Diastolic.

Fig. I. Representation of subannular aortic aneurysm below right and left coronary sinuses. These aneurysms may also develop below noncoronary sinus and present more than one cavity. RV, Right ventricle, LV, Left ventricle, RC, Right coronary. Ao, Aorta. LC, Left coronary.

Table II. Reported cases of subannular left ventricular aneurysms in 12 white patients Author

Pocock et al." Waldhausen et al.' Collins et al.12 MacYaugh et al." Puig- Massana et al.J3 Olowoyeye et al." Lavee et al.' Brito et al.14 This report

1965 1966 1969 1969 1971 1980 1981 1984 1985

Patient

Position

Operation

I I I I I I I

Aortic Mitral Aortic Mitral Aortic Aortic Aortic Mitral Aortic

No Yes Yes Yes Yes Yes Yes Yes (3 yr) Yes

3 2

ejection murmur in the aortic area. The electrocardiogram showed left ventricular hypertrophy, and the chest roentgenogram a left-sided pleural effusion and signs of left ventricular failure. Cardiac catheterization demonstrated a transvalvular aortic gradient of 132 mm Hg and a pulmonary wedge pressure of 21 mm Hg (Table I). Transoperative examination revealed an enlarged heart with severe left ventricular concentric hypertrophy, a bicuspid and calcified aortic valve, and two saccular aneurysms of about 1.2 em diameter between the right coronary and noncoronary leaflets that protruded to the left atrium and right ventricular infundibulum, respectively. A mechanical prosthesis was implanted at the aortic position, with suture through the aneurysmal edges and aortic anulus to occlude the aneurysms. During the immediate postoperative period atrial fibrilation was the only significant event, and the patient was discharged on the eighth day using amiodarone and anticoagulants. Twenty-nine months after the operation he continues to take thiazide diuretic and anticoagulant and is free of symptoms.

Outcome

Alive/6 wk postop. dead/2 mo postop. Alive/22 mo postop. Alive/Iu mo postop.

Alive/2 mo postop. Alive (3 patients)? Alive/29 to 42 mo postop.

Discussion Excluding those caused by infection" and trauma,' subannular aortic aneurysms are probably congenital.': 2. 5 This hypothesis is supported by the association of a bicuspid aortic valve in our patients, which is the most common congenital heart disease (I % to 2% of the general population),'? and by the report of such an aneurysm in an infant. t 1 Pathogenesis of the aneurysms should involve a discontinuity between the myocardium and the aortic anulus, with their wall being formed by thickened myocardium'. Subannular aortic aneurysms are usually situated below the right coronary and the noncoronary leaflets, next to the ventricular septum (Fig. 1).9, t2 They are often smaller than subannular mitral aneurysms and have only one cavity. However, in one of our two patients the aneurysm had two cavities,

9 5 2 Kalil et al.

and in the other patient it was situated between the left coronary and the noncoronary leaflets. Usually small, they are generally not suspected on clinical investigation.': 7.12 The age and sex of our patients are in accord with the literature': 21 to 45 years, and male. Signs of heart failure are common," 5. 12. 13 as we observed in our two patients. Thoracic pain and ventricular distortion with valvular incompetence, as in Case 1, were also observed by other investigators.v"" Subannular aortic aneurysms are rarely observed in white persons. Only 10 patients have been reported'<" since the first description by Abrams and associates. I in 1962 (Table 11), and in five (44.4%) of these the aneurysm was subaortic.4-6.12.13 Although generally in the submitral position, with addition of our cases to the literature a subaortic origin is somewhat more common (58.3%) in whites. Until now, 15 patients with subannular aortic aneurysms had been reported, excluding Arbulu and Thorns" report of 14 patients with subaortic aneurysms caused by infective endocarditis. In Brazil, four patients with subannular aneurysms have been reported, three of them white, and all with aneurysms in the submitral position. Three were operated on; one died, and the others were free of symptoms in a follow-up of 40 days to 6 years." Some authors>? advocate a Teflon patch to close the aneurysmal orifice,': 9 although Arbulu and Thorns," Olowoyeye and colleagues," and we have successfully employed mattress sutures for several cases. Collins; Matloff, and Harken'? reported the first white case treated by operation. This patient eventually died in the second postoperative month. The patient reported by Olowoyeye's group had no symptoms 10 months after the operation. REFERENCES Abrams DG, Barton CJ, Cockshott WP, Edington GM, Weaver EJM: Annular subvalvular left ventricular aneurysms. Q J Med 31:345-360, 1962 2 Chesler E, Joffe N, Schamroth L, Meyers A: Annular subvalvular left ventricular aneurysms in the South African bantu. Circulation 32:43-51, 1965 3 Chesler E, Mitha AS, Edwards JE: Congenital aneurysms

The Journal of Thoracic and Cardiovascular Surgery

adjacent to the anuli of the aortic and/or mitral valves. Chest 82:334-337, 1982 4 Pocock WA, Cockshott WP, Ball PJ, Steiner RE: Left ventricular aneurysms of uncertain ethiology. Br Heart J 27: 184-192, 1965 5 Lavee J, Milo S, Pelled B, Tov AS, Newfeld HN, Goor DA: Successful surgical repair of subaortic annular aneurysm, discrete membranous subaortic stenosis, and valvular aortic stenosis and incompetence. Case report and review of the literature. J THORAC CARDIOVASC SURG 82:790-793,1981 6 Olowoyeye JO, Thadani U, Charrette EJP, Salerno TA, Parker TA: Subaortic annular left ventricular aneurysm. An unusual cause of aortic regurgitation. Cathet Cardiavase Diagn 6:285-291, 1980 7 Poltera AA, Jones AW: Subvalvular left ventricular aneurysms. A report of 5 Ugandan cases. Br Heart J 35:1085-1091, 1973 8 Waldhausen JA, Petry EL, Kurlander GJ: Successful repair of subvalvular annular aneurysm of the left ventricle. N Engl J Med 275:984-987, 1966 9 Arbulu A, Thoms NW: Subannular aneurysms associated with acute, massive aortic insufficiency. J THORAC CARDJOVASC SURG 68:380-383, 1974 10 Roberts WC: The congenitally biscupid aortic valves. A study of 85 autopsy cases. Am J Cardiol 26:72-83, 1970 II Folger GM, Stanton PE: Annular subvalvular left ventricular aneurysm in a North American infant. Am Heart J 81:392-397, 1971 12 Collins JJ Jr, Matloff JM, Harken DE: Surgical repair of subaortic annular aneurysm. J THORAC CARDIOVASC SURG 57:764-769, 1969 13 Puig-Massana M, Murtra M, Castells E, Revvelta JM: Repair and long-term follow-up of a subaortic annular aneurysm. J THORAC CARDIOVASC SURG 61:397-402, 1971 14 Brito JC, Carvalho H, Feitosa G, Nery AC, Ribeiro A, Figueiredo G, Leite ML, Brandiio S, Ferreira A, Eloy R, Tadeu E, Ribeiro N: Aneurisma sub-valvar mitral de ventriculo esquerdo--"aneurisma Africano." Aprescntacao de 4 casos (abstr.). Arq Bras Cardiol43:Suppl 1:30, 1984 15 MacVaugh H, Joyner CR, Pierce WS, Johnson J: Repair of subvalvular left ventricular aneurysm occurring as a complication of mitral valve replacement. J THORAC CARDIOVASC SURG 58:291-295, 1969