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J Thorac Cardiovasc Surg 2007;134:1070-1072
© 2007 The American Association for Thoracic Surgery

Brief Communication

An innovative technique for the relief of right ventricular trabecular cavity obliteration in endomyocardial fibrosis

Ana Olga Mocumbi, MDa, Daniel Sidi, MD, PhDb, Pascal Vouhe, MD, PhDa, Magdi Yacoub, FRSa,*

a Imperial College, London, United Kingdom, and Instituto do Coração, Maputo, Mozambique
b Hopital Necker Enfants Malades, Paris, France, and Instituto do Coração, Maputo, Mozambique.

Received for publication April 2, 2007; accepted for publication April 20, 2007.

* Address for reprints: Magdi Yacoub, FRS, Heart Science Centre–Harefield, Middlesex UB9 6JH, United Kingdom. (Email: m.yacoub{at}imperial.ac.uk).

Endomyocardial fibrosis (EMF) mainly affects persons from Africa, South America, and Asia.1Go The pathogenesis of this condition remains unknown. In advanced forms, EMF produces marked disability and carries a poor prognosis. Although there is consensus about the potential value of surgical intervention in symptomatic patients, there is still debate regarding the exact timing and the surgical technique to be used.2,3Go

EMF is characterized by endocardial fibrosis affecting the inflow tract and the apex of 1 or both ventricles, commonly involving the atrioventricular valves. The right ventricle is affected in most cases.4Go In severe forms, marked reduction of ventricular volume is thought to be due to the presence of a plug of fibrous tissue involving both the trabecular part and the apex.5Go

We here describe a new mechanism for apical obliteration of the right ventricle in EMF. The concept was used to evolve and apply a new surgical technique to increase ventricular volume, improve contractile function by releasing the myocardium and making use of viable myocardium in the obliterated area, and correct the tricuspid regurgitation.

This technique was conceived after detailed echocardiographic examination showed a layer of endocardial fibrosis forming an artificial floor to the right ventricle and isolating the trabecular part from the rest of the ventricular cavity (Figure 1, A).


Figure 1
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  		Figure 1. A, Echocardiogram showing obliteration of the trabecular part of the right ventricle in a case of bilateral endomyocardial fibrosis. Notice fibrosis in the apex of the left ventricle and dilatation of both atria. B, Through sharp dissection, a plane of cleavage is created, allowing the separation of the fibrous tissue from the myocardium. Excision of the fibrotic tissue is done as a block, revealing a healthy myocardium underneath it.

 
After establishing cardiopulmonary bypass with moderate hypothermia, cold blood antegrade cardioplegia was used for myocardial protection. A wide longitudinal right atriotomy was performed to expose the tricuspid valve, which was usually markedly dilated, allowing wide access to the right ventricle. The distribution and extent of pathologic involvement of the tricuspid valve apparatus and right ventricular cavity were carefully defined.

In patients with obliteration of the trabecular part of the right ventricle described in this article, white glistering fibrous tissue 2 to 3 mm in thickness was found lining the inflow tract and extended down into the cavity of the right ventricle to the level of the junction of the inflow and trabecular parts. The fibrotic process produced a relatively thin layer that formed an "artificial floor" to the right ventricle, separating the inflow cavity from the obliterated muscular trabecular part. The fibrotic process spared the tricuspid leaflets and chordae.

Endocardial resection was started near the tricuspid annulus by retracting the leaflets of the valve. If the latter was fused in some areas, it was mobilized if at all possible. After the development of a cleavage plane by means of sharp dissection, a combination of sharp and blunt dissection was used to excise the thick, fibrous endocardial lining. This process was continued into the ventricular cavity, ensuring preservation and mobilization of the tricuspid valve chordae and papillary muscles. The membrane covering the entry into the trabecular part was removed (Figure 1, B), exposing the fused muscular tissue underneath it. This was followed by recreating a cavity inside the trabecular part by mainly separating the fused trabeculae but, if necessary, also excising some muscular tissue, taking care not to perforate the ventricular wall.

In patients with complete fusion of the leaflets and chordae to the mural fibrosis, freeing of the tricuspid valve apparatus was performed. Reconstruction of the tricuspid valve with 2 bands of polytetrafluroethylene* tubes was used in all patients.

Clinical Summary

Between February 2003 and June 2006, 4 patients with right ventricular trabecular cavity obliteration were treated by means of this operation. The clinical characterization of these patients and the procedures used are summarized in Table 1. There was no early or late mortality. Pericardial tamponade occurred in 2 patients. Patients were kept on low doses of diuretics, aspirin, and angiotensin-converting enzyme inhibitors for 6 months. At a mean follow-up of 18 months (range, 9–48 months), all patients are asymptomatic. A two-dimensional echocardiogram showed an increase in right ventricular cavity dimensions to nearly normal levels with improvement in systolic function, acceptable compliance in all, and mild tricuspid regurgitation in 1 patient. Magnetic resonance imaging analysis in 1 patient confirmed these results.


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  		Table 1 Clinical and preoperative data of the 4 patients operated on with the technique
 
Discussion

A new mechanism for right ventricular trabecular cavity obliteration in EMF is described. A surgical technique for its relief was developed and used with very encouraging results in terms of restoration of both structural and functional changes of the right ventricle. There was no evidence of recurrence over the relatively short follow-up period. We hope that familiarity with this technique will help to stimulate early diagnosis and timely treatment of EMF before shrinkage of the right ventricle occurs and thus have a favorable effect on the prognosis of this potentially fatal disease.

Acknowledgments

We thank the Magdi Yacoub Institute and the Chain of Hope–UK for their financial support of surgical missions to Mozambique and Drs Beatriz Ferreira and Gavin Wright for their help.

Footnotes

* Gore-Tex tube, registered trademark of W. L. Gore & Associates, Inc, Newark, Del. Back

References

  1. Somers K. Restrictive cardiomyopathies. In: Pongpanich B, Sueblinvong V, Vongptrateep C, editors. Pediatric cardiology international congress series 906. Amsterdam: Excerpta Medica; 1990.
  2. Valiathan MS, Balakrishnan KG, Sankarkumar R, Kartha CC. Surgical treatment of endomyocardial fibrosis. Ann Thorac Surg 1987;43:68-73.[Abstract/Free Full Text]
  3. Moraes F, Lapa C, Hazing S, Tenorio E, Gomes C, Moraes CR. Surgery for endomyocardial fibrosis revisited. Eur J Cardiothorac Surg 1999;15:309-313.[Abstract/Free Full Text]
  4. Somers K, Patel AK, D’Arbela PG. The natural history of African endomyocardial fibrosis. In: Hayase S, Murao S, editors. International Congress Series 470. Amsterdam: Excerpta Medica; 1978.
  5. Connor DH, Somers K, Hutt MS, Manion W, D’Arbela PG. Endomyocardial fibrosis in Uganda (Davies’ disease). Part I. Am Heart J 1967;74:687-709.[Medline]



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