Ann Thorac Surg 2007;84:e12-e13
© 2007 The Society of Thoracic Surgeons
Case Reports
Successful Surgical Treatment for Dilated Cardiomyopathy With Cardiac Sarcoidosis
Masahiro Daimon, MD, PhD*,
Tomoyasu Sasaki, MD,
Eiki Woo, MD,
Hideki Ozawa, MD,
Tomoshige Morimoto, MD,
Takahiro Katsumata, MD, PhD
Department of Thoracic and Cardiovascular Surgery, Osaka Medical College, Daigakumachi, Takatsuki, Osaka, Japan
Accepted for publication April 13, 2007.
* Address correspondence to Dr Daimon, Department of Thoracic and Cardiovascular Surgery, Osaka Medical College, 2-7, Daigakumachi, Takatsuki, Osaka, 569-8686, Japan (Email: m-daimon{at}jg7.so-net.ne.jp).
 |
Abstract
|
|---|
We report a surgical case of severe left ventricular dysfunction due to cardiac sarcoidosis. A 45-year-old man who underwent pacemaker implantation for complete atrioventricular block was admitted to the hospital because of dyspnea due to congestive heart failure. Echocardiography revealed a left ventricular ejection fraction of 11%, with severe mitral insufficiency and thinning of the ventricular septum. He was successfully treated by anteroseptal ventricular exclusion, mitral and tricuspid annuloplasty, and bi-ventricular pacing. Postoperative pathologic study revealed noncaseating granulomas. The patient was referred to a cardiologist for further treatment with prednisone.
|
Introduction
|
|---|
Cardiac sarcoidosis is a rare cause of cardiomyopathy that can lead to clinical heart failure. Cardiac dysfunction is often severe and progressive. Sudden death is the most feared common manifestation of sarcoidosis. Corticosteroids may be helpful in patients with conduction disturbances and myocardial dysfunction of sarcoidosis. Transplantation has been performed in patients with severe heart failure.
In this report we describe our surgical treatment of severe dilated cardiomyopathy and mitral insufficiency in a patient with cardiac sarcoidosis.
A 45-year-old man (height, 172 cm; weight, 56 kg) who underwent DDD pacemaker implantation for complete atrioventricular block 8 years previously was admitted to the hospital because of dyspnea due to congestive heart failure. On admission, heart rate was 98 beats per minute and regular, and a grade 2 pansystolic murmur was audible. Echocardiography revealed a left ventricular ejection fraction of 11%, with severe mitral insufficiency and thinning of the ventricular septum (Fig 1). The left ventricular diastolic dimension was over 80 mm. Brain natriuretic peptide levels were 1,250 pg/mL. Coronary angiography was normal. Chest computed tomography showed hilar lymphadenopathy. Cardiac sarcoidosis was suspected from his past and present medical history and the results of these examinations.
View larger version (102K):
[in this window]
[in a new window]
|
Fig 1. Transesophageal echocardiographic scan showed left ventricular dilatation and thinning of the interventricular septum.
|
|
Under mild hypothermic cardiopulmonary bypass and cardioplegic arrest, the mitral valve was repaired by means of an undersized annuloplasty using a 24-mm annuloplasty ring (Carpentier-Edwards Physio Annuloplasty Ring [Edwards Lifesciences, Irvine, CA]). A DeVega-like suture tricuspid annuloplasty was performed. After de-clamping of the aorta, the anteroseptal ventricular exclusion procedure was used to reduce the ventricular volume. The anterior wall of the left ventricle was incised 1 cm apart from and parallel to the left anterior descending artery. The incision was extended 7 cm distally to the second diagonal branch. The interventricular septum was diffusely scarred. An oval Dacron patch (Boston Scientific, Natick, MA) tailored to 2 x 7 cm was secured onto this opening and eliminated the scarred part of the septum (Fig 2). The incision was closed in a double layer. To synchronize the left and right ventricle, a ventricular pacing lead was fixed in the epicardium side of the anterior papillary muscle. Cardiopulmonary bypass was successfully weaned under the support of inotropic agents, intra-aortic balloon pumping, and bi-ventricular pacing with a heart rate of 100 beats per minute.
Pathologic study of the specimen of the left ventricular septum and the free wall revealed noncaseating granulomas consistent with cardiac sarcoidosis. The patient was referred to a cardiologist for further treatment with prednisone.
Echocardiography after the operation showed an ejection fraction of over 20% with no mitral regurgitation. The brain natriuretic peptide decreased to 335 pg/mL. The New York Heart Association functional class improved from IV to II, and the patient was found to be clinically well at 12 months postoperatively.
|
Comment
|
|---|
Myocardial sarcoidosis may result in congestive heart failure and ventricular arrhythmias. The typical pathologic finding in sarcoidosis is the presence of noncaseating granulomas. They infiltrate the myocardium and may eventually become fibrotic scars. Myocardial biopsy may be positive in only approximately half of the patients [1]. The treatment of myocardial sarcoidosis is difficult. Permanent pacing or an implantable cardioverter-defibrillator may be considered in patients with arrhythmias [2, 3]. Corticosteroids may be of some benefit in treating the arrhythmias and myocardial dysfunction. Heart transplantation has been performed in selected patients with end-stage heart failure [2, 4]. However, recurrence of sarcoidosis in the transplanted heart has been reported [5].
Anteroseptal ventricular exclusion is a method adapted for dysfunction of the interventricular septum and anterior wall. The object is to make the left ventricle closer to a spheroidal shape. Operative results have gradually improved, and recent reports suggest that the operative death is approximately 6% for elective operations [6]. As in this case, the left ventricular free wall and the interventricular septum are the most common sites of cardiac sarcoidosis. Thus anteroseptal ventricular exclusion for left ventricular volume reduction is useful for the treatment of cardiac sarcoidosis [7]. Although in this case, global left ventricular contractility was severely reduced, the myocardial systolic function of the second diagonal branch domain was slightly better than the interventricular septum. Furthermore, severe fibrotic scars were mainly seen in the interventricular septum. Therefore we maintained the lateral ventricular wall as much as possible. There are currently no adequate guidelines for ventricular reduction in global dysfunction without left ventricular aneurysm. In existing reports, New York Heart Association functional class III or IV adult patients with nonischemic cardiomyopathy and left ventricular end-diastolic dimension over 70 mm are candidates for ventricular reduction. These criteria overlap with those for cardiac transplantation.
Mitral insufficiency often presents at the end stage of dilated cardiomyopathy. Dilatation of the left ventricle and mitral annulus reduces the coaptation zone of the mitral leaflets. Once mitral insufficiency has developed, the prognosis is poor [8]. Thus it is important to maintain the left ventricular function to eliminate mitral insufficiency. The acceptable size of the mitral annuloplasty is still controversial. We usually use annuloplasty rings of 24 mm or 26 mm for undersized mitral annuloplasty because the septal-lateral dimension is significantly reduced and a better coaptation zone is obtained.
We selected bi-ventricular pacing with cardiac resynchronized therapy because this patient underwent right heart pacing prior to the operation. The effect of cardiac resynchronized therapy was uncertain in this case, but possible treatments should be chosen for improvement of the left ventricular function.
In conclusion, we report a successful case of left ventricular volume reduction, undersized mitral annuloplasty, and bi-ventricular pacing for the treatment of end-stage heart failure due to cardiac sarcoidosis. However, the long-term outcome of this treatment remains unknown, so careful follow-up is needed.
|
Acknowledgments
|
|---|
We thank Dr Fumio Terasaki, Departament of Internal Medicine (III), Osaka Medical College, for the instruction of the pathologic findings and careful follow-up of this patient.
|
References
|
|---|
- Sharma OP, Maheshwari A, Thaker K. Myocardial sarcoidosis Chest 1993;103:253-258.[Medline]
- Sharma OP. Myocardial sarcoidosisA wolf in sheep’s clothing. Chest 1994;106:988-990.[Medline]
- Paz HL, McCormick DJ, Kutalek SP, Patchefsky A. The automated implantable cardiac defibrillatorProphylaxis in cardiac sarcoidosis. Chest 1994;106:1603-1607.[Medline]
- Scott J, Higenbottam T. Transplantation of the lungs and heart and lung for patients with severe pulmonary complications from sarcoidosis Sarcoidosis 1990;7:9-11.[Medline]
- Yager JE, Hernandez AF, Steenbergen C, et al. Recurrence of cardiac sarcoidosis in a heart transplant recipient J Heart Lung Transplant 2005;24:1988-1990.[Medline]
- Suma H, Isomura T, Horii T, Nomura F. Septal anterior ventricular exclusion procedure for idiopathic dilated cardiomyopathy Ann Thorac Surg 2006;82:1344-1348.[Abstract/Free Full Text]
- Buckberg GD, Coghlan HC, Torrent-Guasp F. The structure and function of the helical heart and its buttress wrapping. VI. Geometric concepts of heart failure and use for structural correction Semin Thorac Cardiovasc Surg 2001;13:386-401.[Medline]
- Robbins JD, Maniar PB, Cotts W, Parker MA, Bonow RO, Gheorghiade M. Prevalence and severity of mitral regurgitation in chronic systolic heart failure Am J Cardiol 2003;91:360-362.[Medline]
This article has been cited by other articles:
|
|
|
|
 
T. Shimamoto, T. Nishina, A. Marui, and M. Komeda
Dual left ventricular restorations in a patient with cardiac sarcoidosis.
J. Thorac. Cardiovasc. Surg.,
May 1, 2009;
137(5):
1286 - 1288.
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
