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Unusual presentation of endomyocardial fibrosis
409 3 Maniscalco B, Felner J, McCans J, Chiapella J. Echocardiographic abnormalities in systemic lupus erythematosus. Circulation 1975;52:211. 4 Elkayam U, Weiss S, Laniado S. Pericardial effusion and mitral valve involvement in systemic lupus erythematosus. Ann Rheum Dis 1977;36:349-353. 5 Kahan A, Amor B, De Vemejoul F, Saporta L. Libman-Sachs endocarditis: the diagnostic importance of two-dimensional echocardiography. Br J Rheumatol 1985:24:187-190.
International Elsevier
Journal
of Cardiology, 20 (1988) 409-412
IJC 07445
Unusual presentation of endomyocardial fibrosis V.N. Pyrgakis, L.M. Shapiro and R.M. Donaldson National Heart Hospital, London, U.K. (Received
5 February
1988; revision
accepted
29 February 1988)
A case of endomyocardial fibrosis in a Greek patient who had not visited the tropics is described. The patient suffered from an unusual form of the disease in that it was aggressive and recurrent (requiring three operative procedures) and presented with tricuspid stenosis, leading to syncope. Key
words: Endomyocardial
fibrosis; Right ventricular mass; Tricuspid stenosis
Case Report A 32-year-old man presented in March 1986 with low grade pyrexia, tiredness, exertional dyspnoea, loss of appetite and stabbing chest pain. He was admitted to a local hospital; an echocardiogram was performed which suggested a moderate sized pericardial effusion and a mass within the right ventricular cavity. At this stage he was transferred to the National Heart Hospital with a presumptive diagnosis of a right ventricular intracardiac tumour. On admission he was found to have a low grade fever (37.6 o C) and a tachycardia of 110 beats per minute. The pulse was of small volume and the blood pressure 120/90 mm Hg. The venous pressure was elevated to the angle of the jaw with a prominent “a” wave and dominant “x” descent. There was no palpable cardiac impulse and the heart was quiet. There was no clinical enlargement of lymph nodes, liver or spleen. Routine haematology and biochemistry studies were normal. The electrocardiogram showed right atrial enlargement
Correspondence U.K.
to: V.N. Pyrgakis
0167-5273/88/$03.50
M.D.,
National
Heart
0 1988 Elsevier Science Publishers
Hospital,
Westmoreland
B.V. (Biomedical
Division)
Street,
London
Wl,
Fig. 1. Cross-sectional echocardiogram, parastemal long axis view showing a normal left ventricle (LV) and atrium (LA), a large pericardial effusion (PE) and a mass (arrows) within the right ventricle (RV). The chest branch block with low voltage QRS complexes. An echocardiogram revealed a large pericardial effusion and a mobile mass within the right ventricle (Fig. 1). The mass filled approximately half of the cavity involving predominantly the apex and inflow tract of the ventricle. The right atrium was dilated. Although the left ventricle was displaced it appeared normal. An abdominal ultrasound and computer tomography scan were normal. Cardiac computer tomography scan showed a right ventricular wall mass contiguous with the anterior right ventricular wall and also the septal aspect of the right ventricle. Cardiac catheterisation showed the right atria1 pressure to be elevated with a mean of 16 mm Hg (a = 24, V = 22 with a dominant x descent) and the right ventricular pressure to be 46/12 with a tricuspid gradient on withdrawal of 12 mm Hg. The left sided pressures were normal. Right atria1 angiography showed atria1 enlargement and a large filling defect within the right ventricle involving the inflow, apex and outflow tracts (Fig. 2). A left ventriculogram was normal and a coronary arteriogram showed normal anatomy and no evidence of abnormal tumour circulation. and
incomplete
radiograph
was
right
bundle
normal.
Histological Examination. The mass consisted of thrombus only with foci of myxomatous degeneration; an area of thick endocardium was, however, included which showed the changes of endomyocardial fibrosis. The superficial area of thrombus was followed by a layer of dense collagen tissue and deep to that by loose connective tissue with vascular channels and inflammatory cells within it from which septae extended into the underlying myocardium. Several eosinophils with vacuolation were detected. After an uneventful recovery the patient was treated with warfarin for long-term anticoagulation. However, two months later he was readmitted for emergency surgery because of syncope resulting from recurrence of the right
411
Fig. 2. Right
atrial
angiogram (anterior-posterior projection) demonstrating large filling defect within the right ventricle (arrow).
atrial
enlargement
and a
ventricular mass which almost filled the entire right ventricular cavity. In addition to removal of the mass, a right Fontan procedure using pericardium was performed. Histology at the time was similar to that observed at the first operation. Three months later he presented with facial swelling and distended external and internal jugular veins without visible wave form. Echocardiography and magnetic resonance imaging showed that the right ventricle was almost completely filled by a mass with occlusion by thrombus of the superior vena cava without flow within it. A further thoractomy was undertaken which showed that the right ventricle was filled by organised thrombus and there was no line of demarcation between this and right ventricular myocardium. Because of the unsatisfactory nature of his cardiac status, he has been accepted for cardiac transplantation and is awaiting this procedure. Discussion Endomyocardial fibrosis results in restriction of ventricular filling produced initially by the endocardial fibrosis and later by progressive obliteration of ventricular cavity by fibrous masses and thrombi with contraction and scarring of the ventricular wall [l]. The clinical picture can vary greatly, depending upon the distribution of the lesions [2]. The patient reported in this paper had an aggressive and extensive form of endomyocardial fibrosis dominated by right atria1 and ventricular thrombosis and tricuspid stenosis. Tricuspid stenosis is a very rare sequela of this condition as tricuspid valve involvement usually leads to regurgitation [3]. The constitutional symptoms, which often remit prior to the onset of the cardiac disorder [l] persisted throughout this case reflecting the continuing aggressive nature of this patient’s
412
disease. The whole pattern of clinical presentation was very unusual and that explains why the diagnosis was established only after histology. The affected patient was a young man from the Northern part of Greece who had never travelled to the tropics; we are not aware of other cases of endomyocardial fibrosis described from this country.
Acknowledgements We are grateful to Dr. Eckhardt Olsen for the detailed histological findings, and to Mr. Donald Ross for the operative data.
References 1 Wenger N, Goodwin J, Roberts W. Restrictive obliterative cardiomyopathy. In: Hurst JW. ed. The heart, 6th ed. New York: McGraw-Hill. 1986;120. 2 Cherian G, Vijayaraghavan G, Krishnaswami DM, et al. Endomyocardial fibrosis: report on the hemodynamic data in 29 patients and review of the results of surgery. Am Heart J 1983;105. 3 Wyman A. Rankin R, King H. Loeffler’s endocarditis presenting as mitral and tricuspid stenosis. Am J Cardiol 1977;40:438.
International Elsevier
Journal of Cardiology, 20 (1988) 412-415
IJC 07446
Reversed splitting of the second heart sound due to tricuspid regurgitation Nicola H. Strickland and Derek G. Gibson Cardiac Department, (Received
5 February
Brompton Hospital, London,
1988; revision
accepted
U.K.
29 February
1988)
A previously unreported cause of reversed splitting of the second heart sound is described. This patient had tricuspid regurgitation which caused early closure of the pulmonary valve leading to reversed splitting. Left ventricular function and aortic valve closure were normal. Key
words: Second sound, reversed splitting; Tricuspid regurgitation
Correspondence
to: Dr. D.G. Gibson,
0167-5273/88/$03.50
0 1988 Elsevier
Cardiac
Dept.,
Brompton
Science Publishers
Hospital,
B.V. (Biomedical
London
SW3 ~HP, U.K.
Division)
International Elsevier
Journal
of Cardiology, 20 (1988) 409-412
IJC 07445
Unusual presentation of endomyocardial fibrosis V.N. Pyrgakis, L.M. Shapiro and R.M. Donaldson National Heart Hospital, London, U.K. (Received
5 February
1988; revision
accepted
29 February 1988)
A case of endomyocardial fibrosis in a Greek patient who had not visited the tropics is described. The patient suffered from an unusual form of the disease in that it was aggressive and recurrent (requiring three operative procedures) and presented with tricuspid stenosis, leading to syncope. Key
words: Endomyocardial
fibrosis; Right ventricular mass; Tricuspid stenosis
Case Report A 32-year-old man presented in March 1986 with low grade pyrexia, tiredness, exertional dyspnoea, loss of appetite and stabbing chest pain. He was admitted to a local hospital; an echocardiogram was performed which suggested a moderate sized pericardial effusion and a mass within the right ventricular cavity. At this stage he was transferred to the National Heart Hospital with a presumptive diagnosis of a right ventricular intracardiac tumour. On admission he was found to have a low grade fever (37.6 o C) and a tachycardia of 110 beats per minute. The pulse was of small volume and the blood pressure 120/90 mm Hg. The venous pressure was elevated to the angle of the jaw with a prominent “a” wave and dominant “x” descent. There was no palpable cardiac impulse and the heart was quiet. There was no clinical enlargement of lymph nodes, liver or spleen. Routine haematology and biochemistry studies were normal. The electrocardiogram showed right atrial enlargement
Correspondence U.K.
to: V.N. Pyrgakis
0167-5273/88/$03.50
M.D.,
National
Heart
0 1988 Elsevier Science Publishers
Hospital,
Westmoreland
B.V. (Biomedical
Division)
Street,
London
Wl,
Fig. 1. Cross-sectional echocardiogram, parastemal long axis view showing a normal left ventricle (LV) and atrium (LA), a large pericardial effusion (PE) and a mass (arrows) within the right ventricle (RV). The chest branch block with low voltage QRS complexes. An echocardiogram revealed a large pericardial effusion and a mobile mass within the right ventricle (Fig. 1). The mass filled approximately half of the cavity involving predominantly the apex and inflow tract of the ventricle. The right atrium was dilated. Although the left ventricle was displaced it appeared normal. An abdominal ultrasound and computer tomography scan were normal. Cardiac computer tomography scan showed a right ventricular wall mass contiguous with the anterior right ventricular wall and also the septal aspect of the right ventricle. Cardiac catheterisation showed the right atria1 pressure to be elevated with a mean of 16 mm Hg (a = 24, V = 22 with a dominant x descent) and the right ventricular pressure to be 46/12 with a tricuspid gradient on withdrawal of 12 mm Hg. The left sided pressures were normal. Right atria1 angiography showed atria1 enlargement and a large filling defect within the right ventricle involving the inflow, apex and outflow tracts (Fig. 2). A left ventriculogram was normal and a coronary arteriogram showed normal anatomy and no evidence of abnormal tumour circulation. and
incomplete
radiograph
was
right
bundle
normal.
Histological Examination. The mass consisted of thrombus only with foci of myxomatous degeneration; an area of thick endocardium was, however, included which showed the changes of endomyocardial fibrosis. The superficial area of thrombus was followed by a layer of dense collagen tissue and deep to that by loose connective tissue with vascular channels and inflammatory cells within it from which septae extended into the underlying myocardium. Several eosinophils with vacuolation were detected. After an uneventful recovery the patient was treated with warfarin for long-term anticoagulation. However, two months later he was readmitted for emergency surgery because of syncope resulting from recurrence of the right
411
Fig. 2. Right
atrial
angiogram (anterior-posterior projection) demonstrating large filling defect within the right ventricle (arrow).
atrial
enlargement
and a
ventricular mass which almost filled the entire right ventricular cavity. In addition to removal of the mass, a right Fontan procedure using pericardium was performed. Histology at the time was similar to that observed at the first operation. Three months later he presented with facial swelling and distended external and internal jugular veins without visible wave form. Echocardiography and magnetic resonance imaging showed that the right ventricle was almost completely filled by a mass with occlusion by thrombus of the superior vena cava without flow within it. A further thoractomy was undertaken which showed that the right ventricle was filled by organised thrombus and there was no line of demarcation between this and right ventricular myocardium. Because of the unsatisfactory nature of his cardiac status, he has been accepted for cardiac transplantation and is awaiting this procedure. Discussion Endomyocardial fibrosis results in restriction of ventricular filling produced initially by the endocardial fibrosis and later by progressive obliteration of ventricular cavity by fibrous masses and thrombi with contraction and scarring of the ventricular wall [l]. The clinical picture can vary greatly, depending upon the distribution of the lesions [2]. The patient reported in this paper had an aggressive and extensive form of endomyocardial fibrosis dominated by right atria1 and ventricular thrombosis and tricuspid stenosis. Tricuspid stenosis is a very rare sequela of this condition as tricuspid valve involvement usually leads to regurgitation [3]. The constitutional symptoms, which often remit prior to the onset of the cardiac disorder [l] persisted throughout this case reflecting the continuing aggressive nature of this patient’s
412
disease. The whole pattern of clinical presentation was very unusual and that explains why the diagnosis was established only after histology. The affected patient was a young man from the Northern part of Greece who had never travelled to the tropics; we are not aware of other cases of endomyocardial fibrosis described from this country.
Acknowledgements We are grateful to Dr. Eckhardt Olsen for the detailed histological findings, and to Mr. Donald Ross for the operative data.
References 1 Wenger N, Goodwin J, Roberts W. Restrictive obliterative cardiomyopathy. In: Hurst JW. ed. The heart, 6th ed. New York: McGraw-Hill. 1986;120. 2 Cherian G, Vijayaraghavan G, Krishnaswami DM, et al. Endomyocardial fibrosis: report on the hemodynamic data in 29 patients and review of the results of surgery. Am Heart J 1983;105. 3 Wyman A. Rankin R, King H. Loeffler’s endocarditis presenting as mitral and tricuspid stenosis. Am J Cardiol 1977;40:438.
International Elsevier
Journal of Cardiology, 20 (1988) 412-415
IJC 07446
Reversed splitting of the second heart sound due to tricuspid regurgitation Nicola H. Strickland and Derek G. Gibson Cardiac Department, (Received
5 February
Brompton Hospital, London,
1988; revision
accepted
U.K.
29 February
1988)
A previously unreported cause of reversed splitting of the second heart sound is described. This patient had tricuspid regurgitation which caused early closure of the pulmonary valve leading to reversed splitting. Left ventricular function and aortic valve closure were normal. Key
words: Second sound, reversed splitting; Tricuspid regurgitation
Correspondence
to: Dr. D.G. Gibson,
0167-5273/88/$03.50
0 1988 Elsevier
Cardiac
Dept.,
Brompton
Science Publishers
Hospital,
B.V. (Biomedical
London
SW3 ~HP, U.K.
Division)