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Right arrow Transplantation - heart

Ann Thorac Surg 2004;77:1806-1808
© 2004 The Society of Thoracic Surgeons

Case report

Heart transplantation in a patient with isolated noncompaction of the left ventricular myocardium

Sotiris C. Stamou, MDa,b, Edward A. Lefrak, MDa,b*, Freydoon C. Athari, MDa,b, Nelson A. Burton, MDa,b, Paul S. Massimiano, MDa,b

a Section of Cardiac Surgery, Inova Fairfax Hospital, Falls Church, Virginia, USA
b Department of Pathology, Inova Fairfax Hospital, Falls Church, Virginia, USA

Accepted for publication June 3, 2003.

* Address reprint requests to Dr Lefrak, Section of Cardiac Surgery, Inova Fairfax Hospital, 3301 Woodburn Rd, Suite 301, Annadale, VA 22003, USA
e-mail: ealefrak{at}aol.com


   Abstract
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 Abstract
Introduction
 Comment
 References
 
We describe a patient with isolated noncompaction of the left ventricle who presented with worsening congestive heart failure and was successfully treated with heart transplantation. The prognosis for these patients is poor because of accelerated event rates of fatal arrhythmias, thromboemboli, and profound left ventricular decompensation. Only 7 patients with isolated noncompaction of the left ventricle have been reported to have undergone heart transplantation. Herein we describe a patient with isolated noncompaction of the left ventricle who underwent successful heart transplantation.


   Introduction
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 Abstract
 Introduction
Comment
 References
 
Noncompaction of the ventricular myocardium is a rare disorder characterized by arrest of normal endomyocardial embryogenesis that results in the persistence of intertrabecular recesses and the development of a spongy myocardium [1]. The myocardial abnormality has been described more often in conjunction with congenital defects; however, the isolated form is an established cause of profound heart failure [2, 3]. Herein we describe a patient with isolated noncompaction of the left ventricle in whom severe congestive heart failure developed. He was treated successfully with heart transplantation.

An 18-year-old man was transferred to Inova Fairfax Hospital on intravenous dobutamine support for evaluation of severe signs and symptoms of congestive heart failure. He had been well throughout his childhood, but 1 month previously he experienced weakness, cough, and abdominal pain. A chest roentgenogram revealed cardiomegaly and pulmonary venous congestion. An echocardiogram demonstrated dilated cardiomyopathy with an ejection fraction less than 10%, mild mitral regurgitation, and moderate tricuspid insufficiency. Intravenous dobutamine treatment was instituted because of marked hypotension.

A repeat echocardiogram at Inova Fairfax Hospital revealed severely depressed left ventricular function (on dobutamine), estimated pulmonary artery pressure of 70/40 mm Hg, and excessive trabeculations, with deep recesses in the left ventricular wall indicative of the noncompaction deformity (Fig 1). The patient's congestive heart failure was treated vigorously with pharmacologic therapy, but his condition progressively deteriorated. He required intravenous milrinone to stabilize his condition. However, he did not experience any other organ failure throughout the hospital stay. He remained in the hospital on continuous intravenous inotropic support from August 30, 2000, until January 18, 2001, when he underwent orthotopic heart transplantation.



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  		Fig 1. Echocardiographic features of noncompaction of the left ventricular myocardium. (A) Four-chamber-view 2-dimensional echocardiography obtained at the time of presentation. Note multiple prominent trabeculations in the apex and mid ventricle, with deep intertrabecular recesses. (B) Short-axis-view 2-dimensional echocardiography that demonstrates the spongy myocardial appearance resulting from abundant myocardial trabeculations and intertrabecular recesses.

 
The explanted heart weighed 426 g. The coronary arteries were normal. There was endocardial thickening of the left ventricle, with some areas of ischemic change and discordance of myocardial fibers. Additional examination of the heart at the Armed Forces Institute of Pathology confirmed the presence of marked trabeculation and sinusoidal invaginations consistent with the diagnosis of ventricular noncompaction, or spongy myocardium (Figs 2, 3). The patient was discharged from the hospital and has remained well and fully active for the subsequent 2.5 years.



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  		Fig 2. Trabeculation and sinusoidal invaginations of the left ventricle consistent with ventricular noncompaction.

 


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  		Fig 3. Sections of the native explanted heart showing extensive endocardial thickening, discordance of myocardial fibers, and trabeculation of the myocardium within the left ventricle.

 

   Comment
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 Abstract
 Introduction
 Comment
References
 
Noncompaction of the ventricular myocardium, or spongy myocardium, is a congenital abnormality characterized by arrest in the compaction of the ventricular myocardium; it normally occurs between weeks 5 and 8 of fetal life [4]. The process of compaction progresses from the epicardium to endocardium and from the base of the heart to the apex. Noncompaction of the ventricular myocardium results in the persistence of multiple ventricular trabeculations, which usually affect the left ventricular myocardium [5, 6].

Clinical symptoms depend on the extent of noncompacted cardiac segments [7]. Patients with ventricular noncompaction usually present with heart failure resulting from systolic and diastolic dysfunction, primarily of the left ventricle. Systolic dysfunction is most probably the result of relative ischemia of the myocardium due to the mismatch of myocardial oxygen supply and demand [4]. Mechanisms implicated in the diastolic dysfunction include a combination of abnormal ventricular relaxation and restriction to ventricular filling secondary to the prominence of intracavitary trabeculations [4]. The age at onset of heart failure is variable. It ranges from infancy to old age, possibly because of the variable degree of myocardial trabeculations and the effects of chronic myocardial ischemia. Other clinical manifestations include atrial and ventricular tachyarrhythmias [5, 6], conduction abnormalities (mainly bundle branch block) [8], and thromboembolic events. Familial occurrence has been documented [5, 6, 9].

The typical image on the echocardiogram in isolated noncompaction is a 2-layered hypokinetic left ventricular myocardium. The epicardial portion is thin and compacted, whereas the endocardial layer is considerably thicker and not compacted [10]. Color Doppler imaging reveals the deep recesses filled with blood. The ventricular wall appears thickened, especially near the apex [4, 5, 9–11]. When the diagnosis is not certain, transesophageal echocardiography may be helpful because it enhances the assessment of left ventricular wall structure and imaging of the spongiform character seen in the myocardium [7].

Other modalities that have been used for the diagnosis of noncompaction of the left ventricular myocardium include magnetic resonance imaging and left ventriculography [12]. Endomyocardial biopsy may also be used for the diagnosis of noncompaction of the ventricular myocardium.

The prognosis of patients with ventricular noncompaction is poor. Mortality in adults during the first 4 years after development of symptoms exceeds 35%, of which half of the deaths are sudden [6]. In a recent study, 34 patients were followed up. At the end of 44 months, 8 patients had died, and 4 patients had undergone cardiac transplantation [6]. The most frequent causes of death in nontransplanted patients were ventricular tachycardia (41%) and thromboembolic events (24%) [6]. Ritter and colleagues [6], in a 6-year follow-up, reported that 59% of patients with isolated noncompaction of the left ventricle either died or underwent heart transplantation. A review of the literature identified only 7 patients with isolated noncompaction of the left ventricle who underwent cardiac transplantation. Our patient is fully active in both school and work 2.5 years after heart transplantation.

This case report is an interesting case presentation and literature review about an unusual condition and an exceedingly rare indication for heart transplantation. Cardiomyopathy due to isolated noncompaction of the left ventricle is a rare cause of severe heart failure. Awareness of the unusual myocardial malformation is critical to attain prompt diagnosis with either echocardiography or magnetic resonance imaging.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Hook S., Ratliff N.B., Rosenkranz E., Sterba R. Isolated noncompaction of the ventricular myocardium. Pediatr Cardiol 1996;17:43-45.[Medline]
  2. Chenard J., Samson M., Beaulieu M. Embryonal sinusoids in the myocardium: report of a case successfully treated surgically. Can Med Assoc J 1965;92:1356-1359.
  3. Jenni R., Goebel N., Tartini R., Schneider J., Arbenz U., Oelz O. Persisting myocardial sinusoids of both ventricles as an isolated anomaly: echocardiographic, angiographic, and pathologic anatomical findings. Cardiovasc Intervent Radiol 1986;9:127-131.[Medline]
  4. Agmon Y., Connolly H.M., Olson L.J., Khandheria B.K., Seward J.B. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr 1999;12:859-863.[Medline]
  5. Chin T.K., Perloff J.K., Williams R.G., Jue K., Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990;82:507-513.[Abstract/Free Full Text]
  6. Ritter M., Oechslin E., Sutsch G., Attenhofer C., Schneider J., Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997;72:26-31.[Medline]
  7. Lubiszewska B., Hoffman P., Rufyllo W. Isolated noncompaction of the ventricular myocardium: case report and review of literature. Pol Heart J 2001;55:443-448.
  8. Robida A., Hajar H.A. Ventricular conduction defect in isolated noncompaction of the ventricular myocardium. Pediatr Cardiol 1996;17:189-191.[Medline]
  9. Ichida F., Hamamichi Y., Miyawaki T., et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol 1999;34:233-240.[Abstract/Free Full Text]
  10. Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., Jenni R. 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]
  11. Shah C.P., Nagi K.S., Thakur R.K., Boughner D.R., Xie B. Spongy left ventricular myocardium in an adult. Tex Heart Inst J 1998;25:150-151.[Medline]
  12. Conraads V., Paelinck B., Vorlat A., Goethals M., Jacobs W., Vrints C. Isolated non-compaction of the left ventricle: a rare indication for transplantation. J Heart Lung Transplant 2001;20:904-907.[Medline]



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