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Ann Thorac Surg 1997;64:390-393
© 1997 The Society of Thoracic Surgeons

Original Articles: Cardiovascular

Heart Valve Operation in Acromegaly

Goro Ohtsuka, MD, Shigeyuki Aomi, MD, Hitoshi Koyanagi, MD, Hiroyuki Tsukui, MD, Yasuko Tomizawa, MD, Akimasa Hashimoto, MD, Yasunari Sakomura, MD

Departments of Cardiovascular Surgery and Department of Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan

Accepted for publication January 7, 1997.


   Abstract
Top
 Footnotes
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 References
 
Background. Intractable congestive heart failure is known as a serious complication of acromegaly, but valvular heart disease rarely occurs in acromegalic patients. We experienced 5 surgical cases of valvular heart disease associated with acromegaly. We describe the features of those cases in this report.

Methods. The patient characteristics and operative and pathologic findings were retrospectively studied.

Results. There were 4 men and 1 woman. Age at operation was 59 ± 5.5 years. Cardiac lesions consisted of 1 case of aortic regurgitation associated with mitral regurgitation, 1 of aortic regurgitation, and 3 of mitral regurgitation. Operative procedures consisted of 1 double valve replacement (aortic and mitral valve replacement), 1 aortic valve replacement, and 3 mitral valve replacements. The causes of aortic valvular regurgitation were aortic valvular degeneration and aortic annular dilatation. The causes of mitral regurgitation were chordal rupture and mitral valvular degeneration. Histopathologic examination of the excised valves showed mucopolysaccharide deposits and myxomatous degeneration of the leaflets. The myocardium showed fibrosis of interstitial spaces and endocardium, and disarrangement of muscle fibers.

Conclusions. We report 5 successful surgical cases of valvular heart disease associated with acromegaly. Earlier operation is recommended for such cases because of acromegalic cardiomyopathy.


   Introduction
Top
 Footnotes
 Abstract
 Introduction
Patients and Methods
 Results
 Comment
 References
 
Cardiac hypertrophy, cardiomyopathy, and congestive heart failure are often associated with acromegaly, and cause intractable congestive heart failure and disturbance of the conduction system. When valvular heart diseases occur in association with acromegalic heart muscle disease, management is extremely complicated. However, there are few reports concerned with valvular heart disease in acromegaly. In this study, we report the features of 5 successful surgical cases of valvular heart disease associated with acromegaly. According to our experiences, we describe the four major problems in acromegalic heart valve surgery: intractable congestive heart failure, arrhythmia and conduction disturbances, fragility of tissues, and control of blood growth hormone levels.


   Patients and Methods
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 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 References
 
Five patients underwent operation for valvular heart disease associated with acromegaly (0.2% of total heart valve operations at our institute in the same period). The preoperative duration of acromegaly (from clinical onset of acromegaly to operation), duration of valvular heart disease (from clinical onset of valvular heart disease to operation), surgical procedure, postoperative course including circulatory support and catecholamine administration, long-term result, and pathologic findings of excised valves and myocardium were investigated. All values were expressed as mean ± standard deviation.


   Results
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 Footnotes
 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 References
 
Age at operation, sex, diagnoses of heart disease, duration of valvular disease, complication of diabetes mellitus and hypertension, treatment for acromegaly, and growth hormone level at the onset of acromegaly and at the time of operation for heart valve disease are given in Table 1Go. Patients 1, 2, 3, and 4 had acute left heart failure and needed emergency administration of diuretics and digitalis. Echocardiography showed they had valvular disease already. Patient 5 suffered from congestive heart failure and was also treated with diuretics and digitalis; at that time, echocardiography did not point out valvular disease. However, left heart failure developed in all patients after a short period in spite of medical treatment. Hypertension (systolic blood pressure >140 mm Hg, diastolic blood pressure >90 mm Hg, or both) was present in 4 patients.


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  		Table 1. . Patient Characteristics
 
Growth hormone levels at the diagnosis of acromegaly were all above the normal range, which is less than 5.0 ng/mL. As treatment for acromegaly, transsphenoidal adenotomy of a pituitary tumor was performed in patient 1 (2 years before the cardiac operation) and patient 4 (6 weeks before the cardiac operation), and bromocriptine was given to 3 patients including patient 1 because of high growth hormone level after adenotomy (in patient 4, bromocriptine was given after the heart valve operation). After treatment for acromegaly, the level of growth hormone declined; however, in 2 patients (patients 1 and 5), it remained extraordinarily high, greater than 20 ng/mL. New York Heart Association functional class of all patients was class II or III before the cardiac operation.

Cardiothoracic ratio, the findings of electrocardiography and ambulatory 24-hour electrocardiography, left ventricular end-diastolic volume index and ejection fraction, operative procedures, and late results are given in Table 2Go. Ambulatory 24-hour electrocardiography revealed 4 patients had premature ventricular contractions and 2 had paroxysmal atrial fibrillation. The causes of mitral valve regurgitation were chordal rupture of a mural leaflet and mitral valvular degeneration. The causes of aortic valvular regurgitation were aortic valvular degeneration and aortic valvular annular dilatation without valvular sclerosis and commissural fusion suggesting rheumatic fever. In all cases the aortic and mitral annulus were so fragile that precise pledgeted mattress sutures were employed to suture the prostheses in place.


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  		Table 2. . Cardiac Characteristics
 
Weaning from cardiopulmonary bypass was uneventful after 30 minutes of bypass assist; however, in patient 5, it was difficult to obtain hemostasis because of the fragile aortic wall. All patients survived the operation. Long-time results showed one late death 7 years after operation due to ileus in patient 2 and one pacemaker implantation in patient 1 because of bradycardia.

Histopathologic examination of the excised valves revealed mucopolysaccharide deposits on the leaflets (Fig 1Go). The myocardium of both atria and ventricles showed fibrosis of interstitial spaces and endocardium, and disarrangement and enlargement of muscle fibers. There was no evidence of cell infiltration. The aortic wall of patient 5 showed medial fibrosis (Fig 2Go).



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  		Fig 1. . (A) Excised aortic valve. (B) Cross-section of aortic leaflet shows rolled edge with myxomatous transformation rich in mucopolysaccharides. (Alcian blue; x40 before 52% reduction.) (C) Excised mitral valve has elongated chordae. (D) Microscopic appearance of mitral leaflet also shows myxomatous degeneration. (Alcian blue; x40 before 52% reduction.) All specimens were taken from the same patient (patient 5).

 


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  		Fig 2. . (A) Left atrium shows marked thickening of endocardium. (Hematoxylin and eosin; x40 before 52% reduction.) (B) Left ventricle shows interstitial fibrosis, enlarged myocardium, and thickened endocardium. (Hematoxylin and eosin; x200 before 52% reduction.) (C) Microscopic appearance of right ventricle shows slightly hypertrophied myocardium and disarrangement of myocytes. (Hematoxylin and eosin; x200 before 52% reduction.) (D) Ascending aorta shows moderate medial fibrosis. (Masson's trichrome; x40 before 52% reduction.) All specimens were taken from patient 5.

 

   Comment
Top
 Footnotes
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
References
 
It is well known that acromegaly is often associated with cardiovascular morbidity [1], and about one third of patients with acromegaly have cardiac diseases [2, 3]. Acromegalic patients, however, rarely show valvular heart disease [2–9]. A few reports have described surgical cases [5–7, 9].

Heart valve operations in acromegaly are associated with four major problems: (1) intractable congestive heart failure, (2) arrhythmia and conduction disturbances, (3) fragility of tissues, especially great vessels, valvular rings and valvular apparatus, and (4) control of blood growth hormone level.

Intractable Congestive Failure
Nearly all patients with acromegaly, especially after the fifth decade, have cardiomegaly [10]. This increase in heart size appears related to the duration of the disease and unrelated to the presence of hypertension or coronary artery disease [4], or growth hormone level [10]. In patients with disease duration of 10 or more years since the clinical onset of acromegaly, 93.8% showed cardiomegaly; however, in those with duration less than 10 years, 63.6% showed cardiomegaly [4]. The left ventricular function in acromegalic patients shows no change in systolic function but deterioration of diastolic function [1, 2, 11]. Also in our 5 patients, left ventricular ejection fraction was maintained at 0.58 ± 0.10; however, congestive heart failure persisted postoperatively, so catecholamine infusion was necessary for more than 1 week in 3 patients. These 3 patients had suffered from acromegaly much longer (12, 17, and 23 years) than the other 2 patients (5 years each). Histopathologic studies have shown that cardiac abnormalities are frequent in acromegaly; these are called acromegalic heart muscle disease [1–5, 10]. Their features are myocardial cell hypertrophy, interstitial fibrosis, and cellular infiltration consistent with myocarditis. All of our patients showed histologic degeneration of the myocardium compatible with acromegaly.

Arrhythmia and Conduction Disturbances
Interstitial fibrosis of the myocardium may affect the cardiac conduction system [3, 12]. In our experience, 4 of 5 patients had arrhythmia preoperatively, and the other 1 had arrhythmia postoperatively. Patient 1 underwent pacemaker implantation for bradycardia 8 years after mitral valve replacement.

Fragility of Tissues, Especially Great Vessels and the Valvular Rings and Apparatus
Intraoperative findings showed enlargement of great vessels and the heart as part of generalized organomegaly. They were slightly fragile and liable to bleed. The heart valves excised at operation showed degeneration with mucopolysaccharide deposition, and the valvular rings and their apparatus were also fragile as reported by Read and associates [10] and Ondeyco and colleagues [13]. We therefore decided to perform valve replacement rather than plasty [9] to assure the success of the operation, expecting a better long-term result.

Control of Blood Growth Hormone Level
Because of the unfavorable effect of excess growth hormone on cardiac function and metabolic activity, and because of anticoagulant therapy after cardiac operations, treatment for acromegaly, especially surgical treatment, should be carried out before a heart valve operation.

Conclusion
We report 5 successful surgical cases of valvular heart disease associated with acromegaly. Earlier operation is recommended for such cases because of acromegalic cardiomyopathy.


   Footnotes
Top
 Footnotes
Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 References
 
Address reprint requests to Dr Koyanagi, Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1, Kawada-cho, Shinjuku-ku, Tokyo, 162, Japan.


   References
Top
 Footnotes
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 References
 

  1. Morvan D, Komajda M, Grimaldi A, Turpin G, Grosgogeat Y. Cardiac hypertrophy and function in asymptomatic acromegaly. Eur Heart J 1991;12:666–72.[Medline]
  2. Rodrigues EA, Caruana MP, Lahiri A, Nabarro JDN, Jacobs HS, Raftery EB. Subclinical cardiac disfunction in acromegaly. Br Heart J 1989;62:185–94.[Abstract/Free Full Text]
  3. Hayward RP, Emanuel RW, Nabarro JDN. Acromegalic heart disease: influence of treatment of the acromegaly on the heart. Q J Med 1987;62:41–58.[Medline]
  4. Jonas EA, Aloia JF, Lane FJ. Evidence of subclinical heart muscle dysfunction in acromegaly. Chest 1975;67:190–4.[Abstract/Free Full Text]
  5. Lie JT, Grossman SJ. Pathology of the heart in acromegaly: anatomic findings in 27 autopsied patients. Am Heart J 1980;100:41–52.[Medline]
  6. Kawada M, Iida Y, Koyanagi H, et al. Successful mitral valve replacement for non-rheumatic mitral regurgitation associated with active acromegaly. Shinzo 1985;17:673–80.
  7. Tomizawa Y, Endo M, Kawada M, Fukuchi S, Soejima K, Koyanagi H. Successful aortic valve replacement for aortic regurgitation due to enlargement of aortic annulus associated with acromegaly: a case report. J Jpn Thorac Surg 1985;33:2015–9.
  8. Kaku T, Nakashima Y, Ichiyasu H, Soejima M, Baba K, Kuroiwa A. Ruptured chordae tendineae in acromegaly. An autopsy case. Jpn Heart J 1991;32:521–7.[Medline]
  9. Sasahashi N, Kuji T, Shin'oka T, Suehiro M, Tomino T. Valvuloplasty of mitral regurgitation associated with acromegaly: report of a case. Kyoubu Geka 1992;45:363–6.
  10. Read RC, White HJ, Paracios E. Floppy valve syndrome. A possible expression of pituitary and mucopolysaccharide dysfunction. Surg Clin N Am 1967;47:1427–35.[Medline]
  11. Berotoni PD, Morandi G. Impaired left ventricular diastolic function in acromegaly: an echocardiographic study. Acta Cardiol 1987;42:1–10.[Medline]
  12. Baldwin A, Cundy T, Butler J, Timmis AD. Progression of cardiovascular disease in acromegalic patients treated by external pituitary irradiation. Acta Endocrinol 1985;108:26–30.
  13. Ondeyco SM, Lewis HD Jr, Hartman CR. Myxomatous degeneration and cystic medial necrosis associated with acromegaly. Arch Intern Med 1980;140:547–9.[Abstract/Free Full Text]



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