Endomyocardial Biopsy in Diagnosis of Cardiomyopathies

British Heart_Journal, I97I, 33, 822-832. Endomyocardial biopsy in diagnosis of cardiomyopathies K. Somers,' M. S. R. Hutt,2 A. K. Patel,3 and P. G. D'Arbela From the Department of Cardiology and Department of Pathology, Makerere Medical School, Kampala, Uganda The Konno bioptome, adapted to the intracardiac catheter, has been used in 64 patients in the differential diagnosis ofcardiomyopathies, as an additional diagnostic methodduring standardright heart catheterization and angiocardiography. In the specific instance of endomyocardial fibrosis the Konno biopsy enables histological diagnosis in life. Not surprisingly, in advanced cases dense collagen scar is the usualfeature in endomyocardialfibrosis. The Konno method is a real advance in the evaluation and differential diagnosis of heart disease of obscure origin. The technique of biopsy has proved easy to acquire and it is without hazard. Illustrative data from 5 cases of endomyocardialfibrosis and 2 cases of 'congestive cardiomyopathy' are detailed. Though considerable progress has been during life, with careful histological and achieved in the diagnosis of heart diseases, histochemical studies, offers prospects of there remains a group of heart diseases pri- information on pathogenesis and pathology marily affecting the myocardium the nature of and perhaps a long-term possibility of treat- which is obscure, and in which diagnosis is ment and prevention in areas where the not easily reached in life. In such cases there cardiomyopathies are common. is usually no obvious antecedent illness or Hitherto, procedures for obtaining biopsy associated lesion. The patients present with specimens of the myocardium have involved acute or chronic cardiac failure and con- thoracotomy and pericardiotomy or the use siderable cardiac enlargement may be present. of percutaneous needle biopsy techniques. A diversity of conditions occurs in this group Weinberg, Fell, and Lynfield (I958) used a of myocardial diseases generally labelled small thoracotomy incision carried out under 'cardiomyopathy' and defined by Goodwin local anaesthesia for myocardial and peri- (I964) as, 'an acute, subacute or chronic dis- cardial biopsy in the elucidation ofaetiological order of the heart muscle of unknown or diagnosis in cases of myocarditis and peri- obscure aetiology, often with associated endo- carditis. The need to obviate a surgical cardial or sometimes with pericardial involve- incision led to the development of needle ment but not atherosclerotic in origin'. techniques in the diagnosis of cardiac path- Increasing awareness of the occurrence of the ology. Sutton and Sutton (I960) devised a cardiomyopathies, clinical recognition of percutaneous needle technique using a thin- primary myocardial disease, and experience walled, modified Silverman needle with a at necropsy in various centres in the world notched cutting tip. By preference the site indicate an international prevalence (Fejfar, used was that of percutaneous left ventricular I968). The exact histopathological lesion in catheterization (Brock, Milstein, and Ross, the myocardium, if determined at all, is I956). A few years later Soubihe (I963) intro- usually not ascertained before necropsy duced a needle with a non-cutting tip. After examination. A biopsy of the myocardium penetration of the heart a lateral window Received 7 December 1970. allowed trapping of tissue, subsequently cut 1 Correspondence and requests for reprints to Pro- with an inner trocar. A needle with a similar fessor K. Somers, Makerere University Medical principle of entrapment and cutting was School, P.O. Box 7072, Kampala, Uganda. widely used by Decourt and his colleagues 2 Present address: Department of Morbid Anatomy, (I963) in Brazil. Success with needle tech- St. Thomas' Hospital, London S.E.i. 3 Present address: Veterans Administration Hospital, niques confirmed the value of the information 2500 Overlook Terrace, Madison, Wisconsin 53705, obtained from histological studies in primary U.S.A. myocardial disorders. However, the amount of Endomyocardial biopsy 823 tissue obtainable by percutaneous needle scars composed of collagen and elastica. Charac- biopsy is small, and the sample is very local- teristic foci of collagen necrosis may be found in ized and ordinarily does not contain endo- the scar tissue. The macroscopical and microscopical appearances of the heart in endomyo- cardium. To meet these challenges Konno cardial fibrosis indicate that it is a distinct developed an instrument, the Konno biop- pathological entity which can be distinguished tome,1 adapted to the intracardiac catheter, from other forms of cardiomyopathy (Connor et which ensured biopsy material from both al., I967, I968). Clinical suspicion of endo- endocardium and the myocardium and in myocardial fibrosis can be corroborated at cardiac larger amounts than obtained from needle catheterization from analysis of pressure tracings biopsy. Konno's encouraging results (Konno and angiocardiograms (Somers, D'Arbela, and and Sakakibara, I963; Sekiguchi and Konno, Patel, I97I). In the routine catheter investigation I969) led us to explore the usefulness of his of endomyocardial fibrosis we have used the in the of the cardio- Konno bioptome as an additional diagnostic technique diagnosis method and in staging the disease. myopathies in Uganda with special reference As for the 'congestive cardiomyopathies' to endomyocardial fibrosis. ('idiopathic cardiomegaly'), these form a distinct group with considerable cardiac enlargement, Subjects presenting in cardiac failure, and unassociated In Ugandan experience there are 2 major groups with any special diagnostic features. Mitral and of cardiomyopathy, endomyocardial fibrosis and tricuspid incompetence may be present. Response the 'congestive cardiomyopathies' ('idiopathic to treatment of the cardiac failure is usually cardiomegaly') (Table i). Differential diagnosis unsatisfactory. At necropsy, there is bilateral between them and rheumatic heart disease can ventricular dilatation and hypertrophy, and the often be difficult, especially when associated with heart usually weighs over 400 g, occasionally as atrioventricular valvular incompetence. much as 700 g. On microscopical examination the Endomyocardial fibrosis is characterized by myofibres show hypertrophy and there is a mural endocardial lesions in 3 major sites, the variable increase in the connective tissue between apex of the right ventricle, the apex of the left the fibrils. The muscle fibres vary considerably in ventricle, and the posterior wall of the left size and often have large nuclei (Edington and ventricle including the posterior cusp of the mitral Hutt, I968). We have also used Konno's tech- valve. At necropsy, biventricular disease is present nique of biopsy for evaluating diagnosis in this in 50 per cent of cases though often the lesion on group of cardiomyopathies and in differentiation one side is more severe (Edington and Hutt, I968). from endomyocardial fibrosis. The clinical picture is dominated by severe cardiac failure (Somers and Fowler, 1968; Somers, Brenton, and Sood, I968b), and differential Konno biopsy method diagnosis from constrictive pericarditis, a dis- For very young children and the occasional order amenable to operation, can often be difficult apprehensive patient, basal sedation was achieved (Somers et al., i968a, b). Studies of the histo- with diazepam ('Valium') 5 mg orally or by pathology of endomyocardial fibrosis suggest that intravenous injection, repeated if required. No the lesion starts in the connective tissues of the other premedication was used in any patient. The endocardium and adjacent myocardium. This right saphenous vein was exposed under local leads to alteration of the overlying endothelium anaesthesia for standard right heart catheteriza- with deposition of platelets and fibrin and subse- tion. Retrograde left heart catheterization was quent organization. In the later stages, the carried out via the right femoral artery. The involved areas are transformed into hard white Konno bioptome (Fig. Ia and b), previously sterilized in the folded position fixed with a tape, was introduced and advanced under fluoroscopic TABLE I Incidence of cardiac disorders control to the right ventricle (Fig. 2). The Cardiac Clinic, Mulago Hospital to bioptome was gently advanced to a point of I963 I969 contact with the ventricular wall which was No. Per cent recognized by a sensation of resistance. At this stage connexion with an exploring electrode, using Rheumatic heart disease 524 3I-8 a sterilized clamp connexion attached to the 'Congestive cardiomyopathy' 20I 12-2 proximal end of the ensured an Endomyocardial fibrosis I96 ii-8 bioptome, intra- Aortitis 72 4-4 cardiac electrocardiographic record indicating Pericardial disease 50 3 0 chamber position and also contact with the 'Atherosclerotic heart disease' 45 2.7 ventricular wall (Emslie-Smith and Somers, I968). Cor pulmonale 36 2-2 Still under fluoroscopic control, the screw at the Congenital heart disease 349 212 operator's end controlling the biting mouth of the Miscellaneous/unclassified 177 I0-7 bioptome was loosened and while pressure was Total I,650 100 still maintained at the contact area, the now opened biting end was brought to close and the 1 Supplied by Tokyo Hospital Supply Inc., C.P.O. screw tightened again (Fig. ib). At this stage there Box 1997, Tokyo, Japan. may be one or two ventricular ectopic beats 824 Somers, Hutt, Patel, and D'Arbela II mE (a) (b) FIG. I (a) The Konno endomyocardial bioptome.

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