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Asian Cardiovasc Thorac Ann 2006;14:e68-e70
© 2006 Asia Publishing EXchange Ltd


CASE STUDIES

Valve Repair with Maze Procedure in Acromegaly

Koji Takeda, MD, Junjiro Kobayashi, MD, Hiroyuki Nakajima, MD, Hatsue Ishibashi-Ueda, MD1, Soichiro Kitamura, MD

Department of Cardiovascular Surgery
1 Department of Pathology National Cardiovascular Center Osaka, Japan

For reprint information contact: Junjiro Kobayashi, MD, Tel: 81 66 833 5012, Fax: 81 66 872 7486, Email: jkobayas{at}hsp.ncvc.go.jp, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita-city, Osaka 565-8565, Japan.


    ABSTRACT
 TOP
 ABSTRACT
 CASE REPORTS
 DISCUSSION
 REFERENCES
 
Acromegaly is characterized by a hypersecretion of growth hormone and is frequently associated with valvular abnormality and arrhythmia. The increased morbidity and mortality is primarily caused by stroke and cardiovascular disease. Therefore, valve reconstruction and sinus rhythm recovery will be particularly beneficial. We report herein the successful surgical treatment of two patients with combined valvular insufficiency and chronic atrial fibrillation in the setting of acromegaly.


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 CASE REPORTS
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CASE 1
A 60-year-old male presented with a long history of persistent atrial fibrillation (AF). Chest roentgenogram showed a cardiothoracic ratio of 60%. On the electrocardiogram, sustained AF with a F-wave of 0.1 mV in the V1 lead was found. Echocardiography revealed severe aortic regurgitation through the central area of the valve orifice (Figure 1aGo). Mitral regurgitation was also severe, and was due to both anterior and posterior leaflet prolapse, with the torn chordae. The left atrium was dilated to 61 mm in systolic dimension. Preoperative brain computed tomography revealed a pituitary tumor. Laboratory studies showed an elevated plasma concentration of growth hormone (GH) (23 ng·ml–1 vs. < 0.5 ng·ml–1). The patient underwent trans-sphenoidal adenotomy of the pituitary tumor to decrease the GH levels. After normalization of the GH level, the valve operation was carried out under standard cardiopulmonary bypass. The main cause of aortic regurgitation was a dilatation of the annulus to 30 mm in diameter. Aortic valve competence was achieved by commissural plication. In the mitral valve, chordal replacement was performed with three pairs of expanded polytetrafluoroethylene sutures, and a Duran ring (Medtronic, Inc, Minneapolis, MN, USA) was placed. Tricuspid annuloplasty was performed with DeVega’s method. Modified maze procedure was applied on the left and right atria using cryoablation.


Figure 1
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Figure 1a. Preoperative transesophageal echocardiogram (Case 1); aortic regurgitation was moderate, through the central area of the valve orifice.

 
CASE 2
A 79-year-old-female with acromegaly was admitted for congestive heart failure. Chest roentgenogram showed a cardiothoracic ratio of 68%. Electrocardiogram showed persistent AF. On echocardiography, severe mitral regurgitation with dilated left ventricle was detected (Figure 1bGo). Preoperative GH levels were controlled to within the normal range by the administration of bromocriptine. At operation, the anterior mitral leaflet was diffusely prolapsing and a chordae attached to the posterior leaflet in the posteromedial portion was torn. We carried out chordal replacement on the anterior mitral leaflet and quadrangular resection on the posterior leaflet, and a Carpentier-Edwards ring (Edwards Lifescience, Inc., Irvine, CA, USA) was applied. Subsequently, the cryo-maze procedure was performed. Pathological examination revealed a myxomatous change of the excised mitral leaflet (Figure 2Go).


Figure 1
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Figure 1b. Preoperative transthoracic echocardiogram (Case 2); severe mitral regurgitation due to diffuse leaflet prolapse. LV= left ventricle, Ao= aorta, LA= left atrium.

 

Figure 2
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Figure 2. Histological appearance of excised mitral leaflet showed metachromasia by Alcian blue stain (x 40).

 
In both patients, late echocardiography showed no recurrence of significant valvular regurgitation with sinus rhythm on electrocardiogram. No cardiovascular events occurred for 7 years in Case 1, and for 4 years in Case 2.


    DISCUSSION
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 ABSTRACT
 CASE REPORTS
 DISCUSSION
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In acromegaly, cardiovascular complications are relatively frequent. Chronic excess of GH has a significant association with the high incidences of hypertension, diabetes, and ventricular hypertrophy. Cardiovascular complications including stroke, coronary artery disease, congestive heart failure, and arrhythmia are the leading cause of the increased mortality and morbidity of acromegalic patients.1 Histologically, as seen in Case 2, GH stimulates mucopolysaccharide deposition, which results in myxomatous degeneration of cardiac valves.2,3 This would also play a considerable role in the development of ventricular hypertrophy, adversely affecting cardiac function and promoting cardiac arrhythmia.4 In patients with severely degenerated valves in acromegaly, valve replacement has been uniformly recommended because of the unreliable long-term durability after repair.3,5 However, since restitution of glucose tolerance and blood pressure along with rapid regression of ventricular hypertrophy have been observed after normalization of GH secretion by surgical or medical treatment,6 degeneration of the valvular tissue may not be necessarily progressive in acromegaly. Therefore, surgical repair for incompetent valves, concomitant with treatment to suppress GH, is considered to be feasible.

In our patients, the GH levels were stabilized initially. Subsequently, valve repair was performed successfully. In the follow-up period, the GH level remained adequately controlled. Postoperatively, the deformity of the valve structure was not at all progressive, and no valve related complications occurred for several years.

Increased blood coagulability due to hypersecretion of GH is considered to be one of the important risk factors for stroke.7 Moreover, in our patients, AF imposed an increased risk of stroke. Termination of AF combined with successful valve repair is necessary to reduce the risk of stroke and improve quality of life in terms of avoidance of postoperative anticoagulation. Nonetheless, because the tissues are fragile and liable to bleed in acromegaly,3 complicated maneuvers should be avoided. Our current modified maze procedure using cryoablation, as previously described, requires only a standard right-sided left atriotomy.8 This technique provides a sinus rhythm recovery rate that is comparable to the conventional procedure, while the additional procedural risk is minimal for a valvular heart operation.

We conclude that appropriate valve repair concomitant with the maze procedure is feasible and may provide considerable benefits owing to the elimination of prosthetic valves and AF. Clinically this may contribute to a better quality of life without anticoagulation, especially in medically well-controlled acromegaly.


    REFERENCES
 TOP
 ABSTRACT
 CASE REPORTS
 DISCUSSION
 REFERENCES
 

  1. Bengtsson BA, Eden S, Ernest I, Oden A, Sjogren B. Epidemiology and long-term survival in acromegaly. A study of 166 cases diagnosed between 1955 and 1984. Acta Med Scand 1988;223:327–35.[Medline]

  2. Ondreyco 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]

  3. Ohtsuka G, Aomi S, Koyanagi H, Tsukui H, Tomizawa Y, Hashimoto A, et al. Heart valve operation in acromegaly. Ann Thorac Surg 1997;64:390–3.[Abstract/Free Full Text]

  4. Colao A, Spinelli L, Marzullo P, Pivonello R, Petretta M, di Somma C, et al. High prevalence of cardiac valve disease in acromegaly: an observational, analytical, case-control study. J Clin Endocrinol Metab 2003;88:3196–201.[Abstract/Free Full Text]

  5. Cable DG, Dearani JA, O’Brien T, Orszulak TA, Puga FJ, Schaff HV. Surgical treatment of valvular heart disease in patients with acromegaly. J Heart Valve Dis 2000;9:828–31.[Medline]

  6. Lim MJ, Barkan AL, Buda AJ. Rapid reduction of left ventricular hypertrophy in acromegaly after suppression of growth hormone hypersecretion. Ann Intern Med 1992;117:719–26.[Abstract/Free Full Text]

  7. Landin-Wilhelmsen K, Tengborn L, Wilhelmsen L, Bengtsson BA. Elevated fibrinogen levels decrease following treatment of acromegaly. Clin Endocrinol (Oxf) 1997;46:69–74.[Medline]

  8. Nakajima H, Kobayashi J, Bando K, Niwaya K, Tagusari O, Sasako Y, et al. The effect of cryo-maze procedure on early and intermediate term outcome in mitral valve disease: case matched study. Circulation 2002;106(12 Suppl 1):I46–I50.





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Hiroyuki Nakajima
Soichiro Kitamura
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