Aortic valve replacement in patients with severe aortic valve incompetence associated with rheumatoid spondylitis

Aortic Valve Replacement in Patients with Severe Aortic Valve Incompetence Associated with Rheumatoid Spondylitis

RICHARD BEN

D. SPANGLER,

D. McCALLISTER,

DWIGHT C. McGOON, Rochester,

MD MD,

FACC

MD

Minnesota

Long-term results of aortic valve replacement in 5 patients with severe aortic valve incompetence associated with rheumatoid spondylitis are reviewed. Aortic valve surgery can be recommended for patients of this type, and satisfactory clinical improvement can be anticipated in spite of the presence of problems unique to their disease. There was 1 surgical death (1960) and 1 late sudden death (after 41/2 years of a good clinical result). The 3 other patients who survived operation have had excellent long-term clinical results (31/2, 41/2 and 4 years).

The use of prosthetic valves and better surgical techniques has greatly improved the prognosis for many patients with severe hemodynamic insufficiency of the aortic valve. Aortic insufficiency in most instances results from rheumatic valvulitis but, as is increasingly evident,, many cases result from systemic disorders of connective tissue including ankylosing spondylitis. This report describes our experience with the surgical treatment of 5 patients with intractable congestive heart failure secondary to severe aortic valve insufficiency associated with ankylosing spondylitis.

Methods Five patients with aortic valve insufficiency associated with rheumatoid spondylitis underwent aortic valve replacement at the Mayo Clinic between 1960 and 1965. A single-unit tricuspid Teflon8 valve, replacing the total valve, was used in 1 case’; a St.arr-Edwards ball valve was used in the other 4 cases. The subsequent clinical courses of 3 patients were observed by examination at the Mayo Clinic. Information about, the 2 other patients was obtained from their physicians. The patients were evaluated immediately postoperatively, during recovery from the surgical procedure, and at intervals up to 4 years postoperatively. Patients able to resume normal activity without evidence of cardiac disability were classified as well or in satisfactory condition. From the Section of Medicine and Surgery, Mayo Clinic and Mayo Foundation: Mayo Graduate School of Medicine, University of Minnesota, Rochester, Minn. Manuscript received August 14, 1969, accepted January 5, 1970. Address for reprints: Section of Publications, Mayo Clinic, Rochester, Minn. 55901.

130

Results Clinical

Features

All 5 patients were men (Table I) and were 31 to 56 years old (mean 44 years) at the time of operation. All had complained of pain, tenderness or limitation of motion involving the cervical, thoracic or lumbar region of the spinal column; 4 of the 5 patients

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TABLE Clinical

VALVE

REPLACEMENT

I Features

in 5 Cases

of Aortic

Valve

Incompetence

Associated

with

Rheumatoid

Spondylitis

Date of Onset Case no. 1

Age (yr) 31

Rheumatoid Spondylitis

Aortic Insufficiency 1965

1963

Congestive Heart failure 1965

Angina None

2

56

1932

1961

1963

1962

3

47

1943

1960

1963

None

4

51

?

1962

1962

1962

5

38

1954

1959

1960

1960

* 314 = 3 on a scale of 1 to 4. A-V = atrioventricular; LA = left atrial;

QRS axis,+60”;LVH; 1” A-V block (P-R=0.28 set)

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Date of Surgery

Cardiomegaly; LV enlargement, 3/4*; LA enlargement, 2/4; no intracardiac calcification; aortic root normal; pulmonary vascular markings increased

U/4/65

Results of Surgery Alive and asymptomatic, 5/10/69 (3% yr)

(1 to 2+) Cardiomegaly; LV. 7/10/64 Alive and well, enlargement, 3/4; LA 12/7/68 (4% yr) enlargement, 2/4; single fleck of intracardiac calcium (undetermined location); pulmonary vascular markings normal QRS axis, -15”; Cardiomegaly; LV 5/17/65 Alive and well LVH; 1” A-V block enlargement, 3/4; 5/5/69 (4 yr) no LA enlargement; dilatation of aortic root, l/4; no intracardiac calcification; pulmonary vascular markings normal Cardiomegaly; LV QRS axis, -20”; 3/13/64 Alive and well LVH; 1” A-V block enlargement, 3/4; 8/27/68; died (P-R=0.24 set) LA enlarge10/8/68 (4% yr) with intermittent ment, l/4; RV 2” block enlargement, 2/4; dilatation of ascending aorta, 2+; pulmonary vascular markings normal QRS axis, +60”; Marked cardiomegaly 11/21/60 Died during LVH; 1” A-V block with prominence of operation (P-R =0.24 set) LV; no cardiac fluoroscopy QRS axis, 0”; LVH; 1” A-V block (P-R=0.24 set)

LV = left ventricular;

also had peripheral joint involvement. One patient had had recurrent, iritis. The duration of arthritic symptoms ranged from 2 to 32 years (mean 15l,z years) before surgical intervention in the 4 patients in whom the onset could be determined. Congestive heart failurc evidenced by a history of orthopnea, paroxysmal nocturnal dyslrnca or edema had occurred in all 5 patients from-2 to 29 months (mean 14?h; months) before operation. Three patients had angina pectoris. Peripheral signs of increased aortic runoff were noted in all 5 patients, and all were noted to have a decrr-

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Roentgenographic and Fluoroscopic Findings

Electrocardiographic Findings

LVH = left ventricular

hypertrophy;

RV = right ventricular.

scendo diastolic murmur (grades l/6 to 4/6) at the base and precordial area. A diastolic murmur of the Austin-Flint variety was described in 4 patients. An nortic systolic ejection murmur (grades l/6 to 4/6) was noted in each of the 5 patients. Laboratory Features The results of the serologic test for syphilis (VDRL) , hemoglobin determination, leukocyte count and urinalysis were normal in all patients. The sedimentation rate was increased in 4 patients (74, 52,

131

SPANGLER

TABLE

ET

AL.

II

Incidence of Aortic Valve Rheumatoid Spondylitis

Incompetence

in

Patients

with

Patients with Aortic Incompetence Series

_

Total Patients

no.

%

6 4

1.7 3.1 3.0 1.4 1.8

~~

Bernstein4 Blumberg & Raga# Toone et al.20 Davidson et al.15 Total

352 128 265 1,000 1,745

a 14 32

72 and 79 mm in 1 hour, Westcrgren). Rheumatoid factor was not detected in the 3 patients tested. Tests for lupus erythe!natosus cells were negative in 3 patients. Roentgenographic examination of the spinal column, including sacroiliac joints, revealed characteristic changes of rheumatoid spondylitis in the 5 patients. Examination of the heart hy standard anteroposterior chest X-ray films and fluoroscopy showed cardiomegaly with left ventricular hypertrophy in all 5 patients. Dilation of the aortic root was observed in 2 patients. Calcification of the aortic annulus or valve was not observed at fluoroscopy in 4 patients; the fifth patient was noted to have a single fleck of calcium near the aortic root, but its exact location could not be determined. Electrocardiographic evidence of left ventricular hypertrophy was described in all 5 patients. Four patients had first degree atriovcntricular block, and 1 patient had intermittent first and second degree atriovcntricular block before valve replacement. Results of Operation Excellent results were obtained in 4 of the 5 patients undergoing surgery. One patient died from ventricular fibrillation immediately after valve replacement with the tricuspid Teflon prosthesis. The 4 surviving patients experienced relief of their cardiac symptoms and have returned to normal or nearnormal activity. Patient 2 had transient’ symptoms of aphasia and hemiparesis 10 months postoperatively, after the cessation of anticoagulant therapy, but there was complete recovery with no residual neurologic deficit, and warfariin (Coumadin@) therapy was reinstituted. One patient (Case 4) whose condition improved after surgery died unexpectedly of unknown cause 55 months postoperatively. Patient 4 had a marked reduction in cardiac size and decrease of QRS voltage in the electrocardiogram postoperatively. In Patients 1 and 3 the thoracic roentgenogram showed a modest decrease in

$32

cardiac size, and the electrocardiogram showed a considerable decrease in evidences of left ventricular hypertrophy. Rocntgenographic and electrocardiographic follow-up examination on the remaining patient who survived surgery (Case 2) has not been available.

Discussion Aortic valvular disease in ankylosing spondyliin 1936, was the first to describe tis: Mallory,2 cardiac lesions in association with ankylosing spondylitis. In the ensuing years progress in differentiating the various cardiac lesions that occur as a complication of rheumatoid arthritis, ankylosing spondylitis or rheumatic fever was slow. Cardiac involvement in a patient with preexisting articular disease was thought to represent the superimposition of rheumatic fever on the underlying rheumatoid disease.:‘z4 Some American investigator@ considered ankylosing spondylitis to be an atypical expression of rheumatoid arthritis. The reports of Baggenstoss and Roscnber@l” shed light on the pathology of the cardiac IcFions characteristic of these diseases. Subsequent microscopic, immunologic and clinical studies showed characteristics further distinguishing these disorders. However, as recently emphasized by Weintraub and Zvaiflcr,” in patients with inflammatory joint disease, valvular and myocardial involvement, may occur in a remarkable spectrum of connective tissue disorders, as well as granulomatous processes, and overlap is demonstrable. Bauer and associatcsG,’ were the first to distinguish between the aortitis and the aortic valvulitis associated with clinical evidence of aortic regurgitation in patients with ankylosing spondylitis. Their observation was subsequently confirmed by other invcstigators.1Z-2* In the present series, marked shortening of the aortic valve leaflets was observed at the time of operation in all 5 patients and was the cause of the free aortic valve incompetence. The aortic ring was described as thickened and fibrous in 4 patients, in 2 the aortic tissue was noted to he brittle and in 1 the aortic ring was dilated. Incidence of aortic insufficiency: The reported incidence of sortie insufficiency in ankylosing spondylitis has varied in several large series (Table II). In contrast to the detectable incidence of aortic in&iciency in the various clinical series, Davidson and associates’” found isolated aortic valvulitis on microscopic study of autopsy specimens of 50 percent of 25 patients who had had ankylosing spondylitis. In 5 percent of another series of 100 consecutive patients who had aortic valve replacement for aortic lesion was considered to insufficiency, I4 the valvular

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bc a complication of ankylosing spondylitis. The 5 patients repotted herein represent 0.7 percent of 696 patieiits in whom aortic valvuloplasty or prosthetic replacement was performed at the Mayo’ Clinic between 1960 and 1965.

Associated

myocardial

and

vascular

involve-

paticntr with aortic insufficiency asment : Ilost soriatcd with ankylosing spondylitis have a benign clinical course. Yet, as illustrated by Patient 1, rapidly progressive heart failure may develop, and thcrc may 1~ an urgent need for aortic valve replacetncnt. Xot only is the myocardium subjected to the hcmodynamic hurdcn itnposcd by the incompetent aortic valve, hut tltc, myocardium itself may bc involved in an inflammatory rheumatic process. O’blitcrativc endangiiti:: of the intramural coronary vessels, pcrivascular infiltratio11 by ly1nphocytcs and plasma cells, and destruction and rcplaccment rSI the conduction system fibers by fibrous scar tissue have been documcntcd niicroscopic~~lly,~~~~z~ Reviews by Bernstein and Broclt”*4 and others’4-““i have discussed disturbances in conduction, ranging in severity frohl the frcqutnt occurrence of first degree atrioventricular block to less frequent reports of left bundle branch block and complete heart block. Dvspite the presence of atriovcntricular disturbances in carh of our 5 patients prcopcratively, intracardiac pacing was not requirctl either immediately postopcrativcly or in the long-term follow-up period. Other authoW-17 have commented on possible correlations among the development of aortic valve insufficiency in patients with ankylosing spondylitis, the presence and duration of peripheral joint involvcment and the presence of extra-articular manifcstations such as recurrent iritis. Peripheral joint involvement was observed in 4 of our 5 patients, yet neither iritis nor the duration of arthritic symptoms appeared to be of prognostic value in our experience.

VALVE

REPLACEMENT

joints may bc present in certain cases; pulmonary function should be tested preoperativtly and, if there is an abnormality, Il?eticulous attention is necessary during the postoperative period to prcvcnt hypoventilation atelcctaxis or pneumonia. (2) The chance of gastrointestinal hcmorrhagc after tht stress of operation may bc incrcascd bccaust of long-term use of salicylates or corticoids. (3 I A history of steroid thcrapp indicates a ljotcntial need for supplemental corticoids as part of t,lie prcopcr.titivr preparation and the postoperative support. (41 There may be mcchanical difficulties in intuhating, prrforming trncheostomy or CVCII in positioning thr patient on the operating tahlc because of dorsal kyphosis or cervical subluxation ithc former was a problc1n in 1 of our patients). (51 Possible aort,ic tlisscction or dchiecencc of the valvular prosthesis because of activc aortitis is of concern. 16, Potential hazards with control of anticoagulant therapy may bc prcscnt if the patient requirrs large doses of ~ctlicylntc~s or phenylhutazone (Rutazolidin@i .“? Dcspitr these problems, our results indicate that in patirnts with nortic incompctcncc associated with ankylosing spondylitis aortic valve rcplacrment may hc accomplished with a favorahlc mortality rate, and excellent clinical improrcment of the patient may be anticipated. AIalrtte ant1 associ:ltrsZX recently reported successful aortic valet rc~plxctment in 3 patirnts with rheut11atoid sponclylitis and aortic insufficiency ; these patients w’crc well 1, 2 and 4 years postoperativrly. Thr single d~attr in our series occurred during the earliN cxprricnrr with valve replacrment ( 1960 I, at a time n-hen the surgical mortality rate after total aortic x11\-r rc>placelnrnt using a Teflon prosthesis approached 5.5 percrnt.’ Therefore, we conclude that criteria for the sclcction of patients for aortic valve rcplaccinc~nt should not cxclutle those with ankylosing spondylitis.

Special surgical problems in patients with ahkylosing spondylitis : In addition to the usual pre-

Acknowledgment

oT)erative nlanagcment details, there are several spccial problems relevant to thtt patient with ankylosing spondylitis. il j Restrictive ventilatory disease due to immobility of the costovertebral and costosternal

References &Goon DC, Moffitt EA: Total prosthetic reconstruction of the aortic valve. J Thorac Cardiovasc Surg 46:162-173, 1963 MalloryTB: Discussion. New Eng J Med 214:697-698, 1936 Bernstein L, Broth OJ: Cardiac complications in spondylarthritis ankylopoietica. Acta Med Stand 135:185-194, 1949 Bernstein L: Cardiac complications in spondylarthritis ankylopoietica. Rheumatism (London) 7:18-23, 1951

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1970

Discussion. 5. Baker WH: Amer Pratt Digest Treat 6:1236-1239, 1955 Systemic manifestations of rheu6. bauer W, ClarkWS: matoid arthritis. Trans Ass Amer Physicians 61:339-342, 1948 7. Bauer W, Clark WS, Kulka JP: Aortitis and aottic endocarditis, an unrecognized manifestation of rHeumatoid arthritis. Ann Rh&m Dis 10:470-471, 1951 8. Clark WS, Kulka JP, Bauer W: Rheumatoid aortitis with aortic regurgitation: an unusual manifestation of

133

SPANGLER

9.

10.

11.

12.

13. 14. 15.

16.

17.

18.

134

ET AL.

rheumatoid arthritis (including spondylitis). Amer J Med 22:580-592, 1957 Baggenstoss AH, Rosenberg EF: Cardiac lesions associated with chronic infectious arthritis. Arch Intern Med (Chicago) 67:241-258, 1941 Baggenstoss AH, Rosenberg EF: Unusual cardiac lesions associated with chronic multiple rheumatoid arthritis. Arch Path (Chicago) 37:54-60, 1944 Weintraub AM, Zvaifler NJ: The occurrence of valvular and myocardial disease in patients with chronic joint deformity: a spectrum. Amer J Med 35:145-162, 1963 Ansell BM, Bywaters EGL, Doniach I: The aortic lesion of ankylosing spondylitis. Brit Heart J 20:507-515, 1958 Crow RS: Aortic incompetence in ankylosing spondy litis. Brit Med J 2:271-273, 1960 Heart lesions in rheumatoid disease. Cruickshank B: J Path Bact 76:223-240, 1958 Davideon P, Baggenstoss AH, Slocumb CH, et al: Cardiac and aortic lesions in rheumatoid spondylitis. Proc Staff Meet Mayo Clin 38:427-435, 1963 Goehrs HR, Baggenstoss AH, Slocumb CH: Cardiac lesiohs in rheumatoid arthritis. Arthritis Rheum 3:298-308, 1960 Graham DC, Smythe HA: The carditis and aortitis of ankylosing spondylitis. Bull Rheum Dis 9:171-174, 1958 Schilder DP, Harvey WP, Hufnagel CA: Rheumatoid spondylitis and aortic insufficiency. New Eng J Med 255:11-17, 1956

19. Storstein

20.

21.

22.

23.

24.

25.

26.

27. 28.

Rheumatoid spondylitis and 0. Waaler E: aortic insufficiency. Acta Med Stand 165:125-130, 1959 Toone EC Jr, Pierce EL, Hennigar GR: Aortitis and aortic regurgitation associated with rheumatoid spondylitis. Amer J Med 26:255-263, 1959 Valaitis J, Pilz CG, Montgomery MM: Aortitis with aortic valve insufficiency in rheumatoid arthritis. Arch Path (Chicago) 63:207-212, 1957 The natural history of rheuBlumberg B, Ragan C: matoid spondylitis. Medicine (Balt) 35:1-31, 1956 Sobin LH, Hagstrom JWC: Lesions of cardiac conduction tissue in r.heumatoid aortitis. JAMA 180:1-5,1962 Weed CL, Kulander BG, Mazzarella JA, et al: Heart block in ankylosing spondylitis. Arch Intern Med (Chicago) 117:800-806, 1966 Complete atrioventricular block Hoffman FG, Leight L: associated with rheumatoid disease. Amer J Cardiol 16:585-592, 1965 Julkunen H, Luomanmlki K: Complete heart block in rheumatoid (ankylosing) spondylitis. Acta Med Stand 176:401-405, 1964 Kazmier FJ, Spittell JA Jr: Coumarin drug interactions. Unpublished data Malette WG, Eisenman B, Danielson GK, et al: Rheumatoid spondylitis and aortic insufficiency: An operable combination. J Thorac Cardiovasc Surg 57:471-474, 1969

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