Ann Thorac Surg 2009;87:290-292. doi:10.1016/j.athoracsur.2008.05.057
© 2009 The Society of Thoracic Surgeons
Case Reports
Aortic Valve Replacement for Aortic Regurgitation in a Patient With Left Ventricular Noncompaction
Satoshi Ohki, MD*,
Yukinori Moriyama, MD,
Jun Mohara, MD,
Chieri Kimura, MD,
Naoyuki Sata, MD,
Kenkichi Miyahara, MD
Department of Cardiovascular Surgery and Cardiology, Shinkyo Hospital, Kagoshima, Japan
Accepted for publication May 21, 2008.
* Address correspondence to Dr Ohki, Department of Cardiovascular Surgery, Shinkyo Hospital, 3-41-1 Usuki, Kagoshima, 890-0073, Japan (Email: ooki-ths{at}umin.ac.jp).
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Abstract
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We present a case of a 61-year-old man who underwent aortic valve replacement for aortic regurgitation complicated with left ventricular noncompaction. The pathogenesis of this condition remains unknown. In advanced form, left ventricular noncompaction produces marked disability and carries a poor prognosis. We underscore that familiarity with this disease entity will help to stimulate early diagnosis and timely treatment when necessary.
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Introduction
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Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy believed to be a result of an arrest in cardiac development in early embryogenesis. With the recent progress and spread of cardiac imaging technique, the chance to diagnose this disease entity is apparently increasing [1]. In pediatric patients, LVNC is often associated with other cardiac and extracardiac abnormalities and carries a high mortality as a result of heart failure or sudden cardiac death [2]. On the other hand, the prognosis of adult patients with LVNC is still controversial [3]. A morphological distinction between them is still unclear. Herein we report a case of aortic valve replacement for aortic regurgitation in a patient with LVNC.
A 61-year-old man came to our institution because of increasing shortness of breath. He had no family history of cardiovascular disease or sudden cardiac death. An echocardiogram and subsequent left heart catheterization demonstrated severe degree of aortic regurgitation with a bicuspid aortic valve. Characteristically, left ventricular trabeculations and intertrabecular recesses were prominent. There was thin, compacted myocardium on the epicardial side of the left ventricular wall and thicker, noncompacted myocardium on the endocardial side (Fig 1). The ratio of noncompacted to compacted thickness was approximately 2.0 to 3.0, which met the diagnostic criteria of LVNC [4]. Left ventricular end-diastolic diameter was 61 mm, and left ventricular ejection fraction was 55%. There was no other associated congenital anomaly. Thallium-201 myocardial imaging performed at rest disclosed a hypoperfusion area in the left ventricle corresponding to the zones where noncompacted ventricular myocardium can be localized. No significant disease was detected by a preoperative coronary angiography. Left ventricular dysfunction was deemed secondary to aortic valve regurgitation in addition to LVNC. The patient was referred for surgical management of his bicuspid aortic valve.
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Fig 1. Echocardiographic appearance of the patient: in the short-axis view of the left ventricle. Arrows denote noncompacted area showing prominent trabeculations and deep intertrabecular recesses.
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The aortic valve was replaced with an ATS 23-mm mechanical valve (ATS Medical Inc, Minneapolis, MN) in a supra-annular position. After excision of the bicuspid aortic leaflets, prominent left ventricular trabeculations and intertrabecular recesses were observed through an endoscope (Fig 2). The patient's postoperative course was straightforward, and he was discharged home on postoperative day 18. A follow-up echocardiogram 1 year after surgery showed almost normal cardiac function with a left ventricular end-diastolic diameter of 55 mm and an ejection fraction of 60%.
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Fig 2. Intraoperative endoscopic view of prominent trabeculations through the left ventricular outflow tract.
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Comment
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Left ventricular noncompaction is assumed to have a higher prevalence than previously believed [1], although surgical cases have been rarely reported. We believe that there was only one surgical case previously reported in the English literature, in which left ventricular restoration surgery was effective for end-stage dilated cardiomyopathy caused by LVNC [5]. In our patient, LVNC was complicated with a severe degree of aortic regurgitation due to bicuspid aortic valve, but cardiac function was still good enough to receive a valve replacement. Hence, we performed an elective surgery on this patient without any complication.
Left ventricular noncompaction is a rare congenital dysfunction of ventricular morphogenesis resulting from arrested muscle fiber compaction in early embryogenesis [1]. Initially, LVNC was reported to carry a high mortality [6, 7]. In the meantime, a fairly large number of cases have been published with much better prognosis [1]. Especially, asymptomatic patients had clearly better prognosis than symptomatic patients [3]. However, a morphological distinction of noncompacted myocardium among these patients is still unclear. Moreover, the pathogenesis of this condition remains unknown.
In the present case, aortic valve regurgitation was diagnosed before cardiac function became deteriorated, and a valve replacement was performed according to the usual operative indication. In view of his improved cardiac performance after operation, even patients with LVNC should receive cardiac repair procedure while they are viable candidates for surgery. However, left ventricular dysfunction can occur any time after initial presentation, emphasizing that the patients with LVNC should have lifelong follow-up with at least an annual echocardiography.
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References
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- Stöllberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction J Am Soc Echocardiogr 2004;17:91-100.[Medline]
- Burke A, Mont E, Kutys R, et al. Left ventricular noncompaction: a pathological study of 14 cases Human Pathology 2005;36:403-411.[Medline]
- Ritter M, Oechslin EN, Sutsch G, et al. Isolated noncompaction of the myocardium in adults Mayo Clin Proc 1997;72:26-31.[Abstract/Free Full Text]
- Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated noncompaction: a step towards classification as a distinct cardiomyopathy Heart 2001;86:666-671.[Abstract/Free Full Text]
- Shimamoto T, Murai A, Yamanaka K, et al. Left ventricular restoration surgery for isolated left ventricular noncompaction: report of the first successful case J Thorac Cardiovasc Surg 2007;134:246-247.[Free Full Text]
- Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium: a study of eight cases Circulation 1990;82:507-513.[Abstract/Free Full Text]
- Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis J Am Coll Cardiol 2000;36:493-500.[Abstract/Free Full Text]
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Abstract
Introduction
