Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

Postgraduate Medical Journal (July 1978) 54, 477-484.

Treatment of congestive cardiomyopathy WALTER H. ABELMANN M.D. Department of Medicine; the Thorndike Laboratory, Harvard Medical School, and Beth Israel Hospital, Boston, Massachusetts 02215, U.S.A.

Summary responsible, and select the appropriate therapeutic Although the majority ofpatients with cardiomyopathy regime. A diagnosis of fever is generally considered are in the category of primary or idiopathic cardio- unacceptable. myopathy, for which therapy is symptomatic and The corresponding presenting symptom of con- non-specific, there are a number of secondary forms of gestive cardiomyopathy is congestive heart failure. cardiomyopathy for which specific therapy is available, What is the usual approach of the practising thus giving impetus to prompt and accurate diagnosis. physician to congestive heart failure? A prevalent Among inflammatory lesions, brucellosis, psittacosis approach consists of making the diagnosis of con- and toxoplasmosis are examples. Treatable metabolic gestive heart failure and selecting a therapeutic causes include thyrotoxicosis and thiamine deficiency, regime comprised of digitalis and diuretics. Striking the latter as well as calorie-protein relief of malnutrition are symptoms is usually obtained and, unless by copyright. also preventable. There is presumptive evidence that symptoms persist or recur, further diagnostic evalua- the cardiomyopathy of haemochromatosis is benefited tion and search for possible specific therapy often by repeated phlebotomies. Symptomatic relief of is not undertaken. Here, then, the very effectiveness obstructive cardiomyopathy is achieved by [- of therapy on the one hand and, on the other, the blockade, although resection of obstructing myo- complexity of diagnostic studies that may have to cardium still has a place. be employed to establish an aetiological diagnosis The therapeutic approach to the vast majority of become impediments to early diagnosis of the under- cases of congestive cardiomyopathy is non-specific, lying heart disease. Not only may this approach comprising controlled activity, sodium restriction, prevent the early application of specific therapy, but digitalis and diuretics. Vasodilators and, occasionally, it may also preclude steps toward secondary pre- http://pmj.bmj.com/ f-adrenergic blockade may be beneficial. Pacemakers vention. may be life-saving, whereas the place of anti-arrhyth- The recommended approach to a patient with mics remains uncertain. Transplantation warrants congestive heart failure must include (1) the accept- further application. Valve replacement has little to ance of the need to make every effort to establish an offer. early aetiological and anatomical as well as func- Primary prevention, comprising balanced nutrition, tional diagnosis, (2) the consideration that, with few

vaccines and genetic counselling, merits wider appli- exceptions (e.g. infectious or toxic myocarditis), on September 23, 2021 by guest. Protected cation. In individuals at risk or already afflicted, congestive heart failure is a manifestation of chronic programmes of secondary prevention should include heart disease, (3) the recognition that chronic heart good nutrition, abstinence from alcohol and protection disease warrants continuity of care to prevent further from drugs and toxins. cardiac damage, to maintain optimal balance be- tween demands put upon the heart and cardiac The approach to the patient with congestive cardio- reserve, and to facilitate rehabilitation of maximal myopathy self-care and function within family and society. When the contemporary, well educated physician Once congenital, valvular, hypertensive, ischaemic faces a patient with fever, he is quite likely to per- and pericardial heart disease have been ruled out, the form a thorough clinical and laboratory examination diagnosis of cardiomyopathy may be entertained. in order to find the cause of the fever, identify the The first question to be answered then must be 'are specific infectious organism or inflammatory process we dealing with a secondary cardiomyopathy?' Correspondence: Walter H. Abelmann, M.D., Cardio- Although the likelihood that one is dealing with a vascular Unit, Beth Israel Hospital, 330 Brookline Avenue, disease for which there is specific therapy is small, Boston, Massachusetts 02215 U.S.A. it always exists. Furthermore, the diagnosis of a 0032-5473/78/0700-0477 $02.00 © 1978 The Fellowship of Postgraduate Medicine Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

478 W. H. Abelmann specific disease often permits a more accurate by anaemia, as encountered, for instance, in sickle prognosis, a responsibility of the physician well cell disease or neoplastic disease. Generalized infec- recognized by Hippocrates. tions may be associated with tachycardia, increasing cardiac work. Renal disease may be accompanied The therapy ofsecondary cardiomyopathies by hypertension, confronting the left ventricle with Inasmuch as almost any recognized disease pro- an increased afterload. Similarly, acute or chronic cess may at one time or another involve the myo- pulmonary disease associated with pulmonary hyper- cardium, a full list of secondary cardiomyopathies tension may increase right ventricular afterload. would be very large indeed. When one reduces the A pertinent example here would be pulmonary list to diseases which have been associated with sarcoidosis. Thus, therapy of systemic disease under- heart failure and also are known to respond to lying a cardiomyopathy may benefit the heart by specific therapy, however, the list becomes manage- reducing the cardiovascular load, even when there able. is no direct effect of therapy upon the heart. Table I is an attempt to do just this. Recent advances allow one to expect that before long there The non-specific therapy of congestive heart failure may also be anti-viral agents available for treatment In general, congestive heart failure in patients of viral myocarditis if recognized early (Pavan- with cardiomyopathy responds to non-specific Langston, Buchanan and Alford, 1975). therapy, especially when acute or early in the course of the disease process (Goodwin, 1973). There is a TABLE 1. Secondary cardiomyopathies for which there is tendency for recurrent episodes of congestive failure special therapy to respond less and less well, especially if the recur- Infectious rence is a manifestation of the natural history of the Brucellosis Rickettsial infections disease rather than attributable to an acutely Histoplasmosis Toxoplasmosis supervening increased cardiac load or the patient's Pneumococcal pneumonia Trichinelliasis Mycoplasma pneumonia Tuberculosis non-compliance with the previously prescribedby copyright. Psittacosis therapeutic regimen. Inflammatory - Infiltrative Non-specific therapy of congestive heart failure Haemochromatosis associated with cardiomyopathy has four cardinal Lupus erythematosus Sarcoidosis objectives, to (1) strengthen the function ofthe pump; Endocrine - Metabolic (2) spare the pump by reducing the work load to the Calorie-protein malnutrition extent compatible with the body's need for blood Thiamine deficiency flow and perfusion pressure; (3) prevent secondary Thyrotoxicosis damage to the myocardium as well as other organ

systems; (4) promote optimal healing and recovery http://pmj.bmj.com/ One must now address a hidden assumption: of function of the myocardium. 'Myocardial disease or dysfunction, associated with a systemic disease for which there is specific therapy, I Control ofactivity itself is benefited by this therapy.' This remains It is well recognized that pathophysiological and largely an open question. The answer is surely in the clinical manifestations of myocardial failure are affirmative for some conditions, such as thyro- brought out or precipitated by increasing the work toxicosis. Increasing evidence also points to improve- of the heart. Although the increase in cardiac output

ment of congestive heart failure with treatment of associated with increased bodily activity or exercise on September 23, 2021 by guest. Protected haemochromatosis by repeated venesections (Easley, constitutes largely a volume or preload, and hence Schreiner and Yu, 1972; Skinner and Kenmure, is relatively well tolerated, it is often associated with 1973). an increased afterload as well. The increased venous One other consideration is worthy of mention. return and ventricular filling pressures result in The systemic disease or disorder underlying a ventricular dilatation and hence increased wall secondary cardiomyopathy may aggravate the tension. In the case of acute viral as well as trypano- associated cardiomyopathy by increasing cardiac somal myocarditis, it has been clearly shown experi- load. A few examples may serve to highlight this mentally that myocardial damage is increased by point. A patient with a viral or bacterial infection exercise (Gatmaitan, Chason and Lerner, 1970; may have fever, which by increasing metabolic rate Elson and Abelmann, 1965). and need for heat elimination increases cardiac Furthermore, the increased sympathetic tone output and hence preload. A patient with lupus ery- associated with congestive heart failure may raise thematosus and renal failure may have an increased arterial blood pressure not only at rest but especially blood volume, associated with increased cardiac during exercise. A good case can thus be made for output and hence preload. Preload is also increased resting the heart by restricting activity of patients Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

Treatment of congestive cardiomyopathy 479 with heart failure. Clinical experience indicates that be alert, however, to several risks associated with this indeed is a valuable therapeutic approach, diuretic therapy. One danger of diuretic therapy lies although therapeutic trials of rest alone have not in the possibility of excessive depletion of circulating been reported. The chief proponent of rest therapy, blood volume, resulting in a fall of ventricular filling George Burch, obtained considerably better results pressures below the level required to maintain an with hospital controlled bed rest in patients with adequate cardiac output. Another danger lies in the alcoholic cardiomyopathy than in patients with excessive depletion of tissue potassium and magne- other forms of cardiomyopathy, raising the question sium, which may enhance myocardial damage and of the role of environmental and nutritional effects delay healing; loss of these electrolytes may also of prolonged stays in hospital (Burch and Giles, lower the threshold of digitalis toxicity. These unde- 1972). Strict bed rest may not be required; actually, sirable effects of diuretic therapy are especially both heart size and stroke volume in the supine likely when the powerful diuretic agents ethacrynic posture are greater than in the upright posture. acid and furosemide are used. Therefore, whenever Furthermore, controlled activity may be tolerated possible, management of patients with congestive better psychologically than absolute bed rest. heart failure should prevent the advent of congestive Psychological stress may be associated with tachy- stages of a severity requiring these potent diuretics. cardia and/or increased peripheral resistance, in- diuretics, in association with moderate creasing cardiac work. Alcoholic cardiomyopathy restriction of dietary sodium, should be used to often responds well to abstinence and good nutrition. prevent accumulation of fluid and to maintain close Careful analysis of a patient's life style may reveal to dry weight. Potassium supplementation in the ways and means by which unnecessary physical form of fruit or potassium chloride should be exertion and stress may be avoided without totally remembered. Aldosterone inhibitors, such as incapacitating the individual. spironolactone or triamterene, are most effective when combined with . 2 Caloric restriction by copyright. Body weight is directly related to heart rate and 5 Oxygen therapy cardiac output. The demand for oxygen transport, Pulmonary oedema may result in decreased satura- and hence blood flow, for a given bodily activity is a tion of arterial blood for oxygen by decreasing direct function of body weight. Therefore, reduction pulmonary diffusion and increasing venous admix- of body weight may increase cardiac reserve and ture. In addition, low output cardiac failure is thus raise the threshold for symptoms of heart characterized by a wide arteriovenous oxygen failure. Weight reduction is mandatory for over- difference, resulting in further lowering of the weight patients and may be helpful in others. In the partial pressure of oxygen in the tissues, and especi- natural course of chronic cardiomyopathy, many

ally in the myocardium. The resulting tissue hypoxia http://pmj.bmj.com/ patients develop anorexia, lose weight, and may may enhance tissue damage in cases of toxic or actually improve symptomatically. One must be infectious cardiomyopathy, as has been shown in alert, however, for the development of cardiac experimental viral myocarditis (Pearce, 1960). cachexia, which itself may be deleterious. By means of intermittent inhalation of high con- 3 Sodium restriction centrations of oxygen, the oxygen-carrying capacity The value of dietary sodium restriction in the of blood, and hence tissue oxygenation, may be therapy ofcongestive heart failure is well established. significantly increased. Improved myocardial oxy- It is worth pointing out, however, that since the genation often reduces ventricular irritability. It is on September 23, 2021 by guest. Protected advent of potent diuretic agents, physicians tend to important to keep this in mind, inasmuch as the be less forceful in prescribing restriction of sodium biventricular failure of congestive cardiomyopathy intake. While this is understandable psychologically, often protects the lungs and is not accompanied by in that it substitutes giving for taking, it should be dyspnoea or orthopnoea, the usual indications for remembered that the value of sodium restriction lies oxygen therapy. in the reduction of need for potent diuretics. It should be pointed out that even mild anaemia causes a significant reduction in oxygen-carrying 4 Diuretics capacity and should be corrected. The aim of diuretic therapy is the excretion of sodium and water, effecting a reduction of circulating 6 Digitalis glycosides and other positive inotropic blood volume and hence preload. The resulting de- agents crease in ventricular volumes will also decrease wall Congestive cardiomyopathy represents the purest tension and afterload; the net effect will be improved form of failure of the muscle pump. This should be cardiac function. Ejection fraction, stroke volume the ideal situation, then, for effective use of agents and cardiac output may actually increase. One must with positive inotropic action, among which the Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

480 W. H. Abelmann

digitalis glycosides remain most important. Indeed, constitute an undesirable increase in left ventricular congestive cardiomyopathy is characterized by afterload, likely to precipitate or increase left ventri- marked depression of ventricular function, which, in cular failure. Furthermore, the peripheral vaso- general, responds favourably to digitalis glycosides dilatation associated with thiamine deficiency allows (Yankopoulos et al., 1968). Stroke volume tends to a prolonged state of high cardiac output without rise, even while ventricular filling pressure falls. failure, whereas treatment with thiamine, restoring Exceptions may be seen in patients with marked peripheral vascular tone, may precipitate acute endocardial fibrosis and occasionally in end-stages ventricular failure (Akbarian, Yankopoulos and of severe myocardial fibrosis, constituting what has Abelmann, 1966). The author and his colleagues have been called restrictive cardiomyopathy, the physio- also been aware that, in the absence of valvular heart logical equivalent of constrictive pericarditis. There disease or arrhythmia, thyrotoxicosis associated is, however, a tendency for progressive loss of myo- with peripheral vasodilatation tends to be quite well fibres and increase in fibrosis as the natural course tolerated by the heart, as is anaemia with its lowered of cardiomyopathy progresses, with the result that peripheral vascular resistance. the compliance of the myocardium decreases, the Nature's experiments have only recently been heart becomes stiffer, and digitalis glycosides become translated into the therapeutic approach of left less effective. ventricular unloading by lowering aortic impedance. The improved blood pressure, pulse pressure and Cohn and his colleagues first applied this therapeutic cardiac output caused by digitalization alone may approach to congestive heart failure secondary to initiate an effective diuresis, even when diuretic ischaemic heart disease as well as to cardiomyopathy agents have become ineffective. (Franciosa et al., 1972). A recent study of the cir- Clinical experience also holds that digitalis toxi- culatory response of twelve patients with congestive city may be more prevalent in patients with cardio- cardiomyopathy to the intravenous infusion of myopathy, especially in infiltratative forms and in sodium nitroprusside, at a rate of 51-100 ,ug/min,

amyloidosis. Controlled studies, however, are not exemplifies the striking effects of this method of un-by copyright. available. When either underdigitalization or over- loading the heart (Rossen, Alderman and Harrison, digitalization is suspected, plasma digoxin (or 1976). Systemic vascular resistance fell from 22-3 digitoxin) levels may be helpful in determining the to 13-9 resistance units, associated with a 48%Y proper dosage of the drug (Duhme, Greenblatt and increase in cardiac index from 2-1 to 3-1 I/min/m2, Koch-Weser, 1974). whereas the mean arterial pressure fell only 16%. from In acute, severe exacerbations of congestive heart 86 to 72 mmHg. These changes were associated with failure, especially when associated with hypotension, decreases in pulmonary arterial, pulmonary wedge more powerful positive inotropic agents such as and left ventricular end-diastolic pressures of 35,

, isoproterenol or have 38, and 37°/ respectively. These effects must be http://pmj.bmj.com/ been used and may be of value. This is especially the attributed primarily to the action of nitroprusside case if the episode has been precipitated by inter- as an arteriolar vasodilator, although this drug is current illness which is treatable or self-limited. The also known to decrease preload by dilating peri- newer agent appears to be especially pheral veins. Nitroprusside, however, must be given useful in patients with cardiomyopathy because of intravenously, and continuous monitoring of arterial its predominant positive inotropic activity without and left ventricular filling pressures is indicated. significant vascular, chronotropic or arrhythmo- Thus, its usefulness is restricted to intensive care genic actions (Akhtar et al., 1975). Loeb, Bredakis areas in hospitals. on September 23, 2021 by guest. Protected and Gunnar (1977) recently compared the acute More recently, it was shown by Franciosa, haemodynamic effects of dobutamine and dopamine Pierpont and Cohn (1977) that the same principles in thirteen patients with chronic low output failure, of reducing pre- and afterload may be applied to the secondary to cardiomyopathy in eight. At dosages ambulatory treatment of patients with congestive adjusted to achieve comparable increases in cardiac cardiomyopathy. A single oral dose of 50-100 mg output, dobutamine reduced left ventricular filling of hydrallazine was given to sixteen patients with pressure from 25 to 17 mmHg, while dopamine left ventricular failure, due to cardiomyopathy in increased it to 30 mmHg. Mikulic, Cohn and nine. The pulmonary wedge pressure was decreased Franciosa (1977) were able to show that the effect by 5*5 mmHg and the mean arterial pressure by of dobutamine could be enhanced by combining the 7-8 mmHg, while the cardiac index increased by drug with the vasodilator nitroprusside. 0 95 I/min/m2. Studies of central venous pressure, forearm venous capacitance and tone revealed no 7 Vasodilator therapy significant changes after hydrallazine, confirming It has long been known that increased peripheral that this drug acted primarily by reducing left vascular resistance and increased aortic impedance ventricular afterload. Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

Treatment of congestive cardiomyopathy 481

Massie et al. (1977) explored the combination of anti-arrhythmic agents in cardiomyopathy are avail- systolic unloading of the left ventricle by hydral- able - and it is conceivable that individual variations lazine with reduction of preload by means of nitrates, may preclude general conclusions - the choice of administered either sublingually or orally as sorbide drug for each patient must be made largely on an nitrate, or topically as glyceryl trinitrate ointment. empirical basis. Random sampling by examination Their group of twelve patients with severe chronic or routine ECG, however, should be replaced by heart failure included five with cardiomyopathy of long-term monitoring, preferably during the patient's 3-15 years' duration. They demonstrated that, routine day and night. whereas nitrates reduced the filling pressures of both Changing atrial and especially ventricular arrhy- ventricles without affecting cardiac output, hydral- thmias, as well as unstable conduction disturbances, lazine increased cardiac output without altering especially in a previously unknown patient, may filling pressures. Combining the two drugs resulted signal an active inflammatory cardiomyopathy and in a 36%4 decrease of left ventricular filling pressure constitute an indication for close surveillance and and a 58°/ increase in cardiac index. This and other monitoring such as is available in coronary care units. recent studies suggest that oral vasodilator therapy In cases of persistent sinus tachycardia, P3- may indeed represent a majoraddition to the manage- adrenergic blockade has been reported effective in ment of chronic congestive cardiomyopathy. The improving ventricular function as assessed non- study of Aronow et al. (1977), investigating the invasively, in reducing heart size, and in increasing vasodilator trimazosin, which acts on resistance as working capacity (Waagstein et al., 1975). well as capacitance vessels, merits special mention, In certain secondary cardiomyopathies, such as inasmuch as its sixteen cases included eight with sarcoidosis and Chagas' disease, the frequency of primary and one with alcoholic cardiomyopathy, sudden death in patients with conduction disturb- and because it was controlled, improved exercise ances represents an indication for early prophylactic duration was shown. permanent pacing (Duvernoy and Garcia, 1971). by copyright. 8 Antiarrhythmic agents andpacemakers 9 Anticoagulant therapy Sinus tachycardia, sinus bradycardia, atrial and Thrombo-embolic complications are frequent in ventricular arrhythmias, as well as disturbances of patients with chronic congestive cardiomyopathy. conduction are commonly encountered in patients A proved thrombo-embolic episode or demonstra- with congestive cardiomyopathy. Essentially no tion of intracardiac or peripheral venous thrombosis controlled studies of the prognostic significance of by means of angiography or radionuclide portrayal individual arrhythmias and of the therapeutic effi- constitute indications for chronic anticoagulation, cacy of individual antiarrhythmic agents are avail- usually with a coumarin derivative. In patients with able. Thus, in general, the therapeutic approaches intractable heart chronic, congestive failure, pro- http://pmj.bmj.com/ have been those used in the face of rheumatic or phylactic anticoagulation may be well advised. arteriosclerotic heart disease. A comprehensive review of these approaches does not fall within the 10 Corticosteroids scope of this review. However, a few comments are Despite experimental evidence of their deleterious in order. effect in viral and parasitic myocarditis (Kilbourne, The haemodynamic consequences of arrhythmias Wilson and Perrier, 1956), corticosteroids may have in patients with low output cardiac failure deserve a place in fulminant myocarditis, non-responsive

emphasis. As a result of the depressed myocardial to conventional therapy. However, the evidence is on September 23, 2021 by guest. Protected function, a high ventricular filling pressure is re- anecdotal, and corticosteriods should be tapered quired to maintain even a low stroke volume. These promptly as the patient improves. patients are thus especially vulnerable to reduction The main usefulness of these agents is in the in the diastolic filling period such as that caused by therapy of cardiomyopathy associated with collagen tachycardia or premature contractions. On the diseases known to be responsive, such as lupus other hand, stroke volumes are also limited, so that erythematosus or periarteritis nodosa, and perhaps in excessive bradycardia also reduces cardiac output. myocardial sarcoidosis, inasmuch as the myocardium One goal of antiarrhythmic therapy, then, is to tends to heal with fibrosis. assure optimal cardiac output for the given state of the myocardium. These considerations may con- 11 Surgical treatment ofmitral and tricuspid stitute the primary indications for drug therapy of insufficiency frequent premature beats or for cardioversion of Severe ventricular dilatation often causes incom- atrial tachy-arrhythmias. In cases of excessive brady- petence of the atrio-ventricular valves, resulting in cardia, an artificial pacemaker may be indicated. significant increases in preload, further cardio- Until controlled studies of the efficacy of individual megaly and increased wall stress. In the case of the Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

482 W. H. Abelmann mitral valve, significant regurgitation superimposed gery, and carries a poor prognosis. Patients with this on cardiomyopathy carries an especially poor prog- disorder would thus seem to be ideal candidates for nosis. Fortunately, the treatment modalities already cardiac transplantation. Indeed, many of the reci- discussed often effect a decrease in heart size and pients of heart transplants have been patients with reduction or even elimination of valvar regurgita- cardiomyopathy (Rider et al., 1975). It is quite tion. When this is not the case, patients may come likely that, when further advances in cardiac preser- under consideration for surgical replacement of the vation and in immuno-suppressive therapy warrant mitral and/or tricuspid valve. In the author's exper- expansion of cardiac transplantation centres, this ience, results have been uniformly disappointing. will become an even more important therapeutic These patients constitute formidable surgical risks approach to the patient with cardiomyopathy. and usually represent end-stages of myocardial disease. He has, however, seen moderately good 14 The needfor other therapeutic modalities results when pre-operative medical therapy was Clearly, therapy of congestive cardiomyopathy suboptimal and the operation brought the patient leaves much to be desired. Although this is in good under a strict therapeutic regimen. part a function of the disease itself, future develop- ments may increase therapeutic effectiveness. There 12 Circulatory assist devices is need for positive inotropic agents which are The most widely used circulatory assist device neither vasoconstricting nor arrhythmogenic, for is the intra-aortic balloon, which by means of antiarrhythmic agents without negative inotropic systolic unloading reduces left ventricular afterload effects, and for diuretics which do not deplete tissues and by means of diastolic augmentation increases of potassium or magnesium. Agents favouring pro- coronary blood flow. In low output states, significant tein synthesis might be useful. Controlled studies of increases in cardiac output at decreased ventricular prophylactic pacing and antiarrhythmic agents are filling pressures may be effected. This device often indicated, as is the evaluation of anti-inflammatory

is of clinical value in intractable cardiogenic shock. agents in the therapy of acute myocarditis. by copyright. Because it cannot ordinarily be used for more than a few days, it is of limited value in patients with Primary prevention cardiomyopathy. The device should, however, be Primary prevention of cardiomyopathy warrants considered in the therapy of acute fulminant, in- greater attention than it has been given. Hereditary tractable myocardial failure, when the process is myopathies with cardiac involvement and some thought to be self limited or reversible, e.g. in severe familial cardiomyopathies might be prevented by toxic or infectious myocarditis. The intra-aortic genetic counselling. Early detection by means of balloon has found its greatest application in the amniocentesis may provide another avenue for pre- cardiac surgical patient, whose acute or chronic vention (Mahoney et al., 1977). myocardial depression does not provide an adequate The introduction and wide application of vaccines http://pmj.bmj.com/ circulation after discontinuation ofcardio-pulmonary against smallpox, poliomyelitis, rubella, measles, by-pass (Buckley et al., 1973). In practice, these are influenza and diphtheria undoubtedly have prevented likely to be patients with ischaemic or valvular many acute and perhaps some chronic cardiomyo- heart disease. pathies. The recently developed vaccine against When the residual myocardial function is so poor pneumococcal pneumonia should also be of value that the support given by intra-aortic balloon pump in protecting the heart. Vaccines against Trypano-

does not suffice to maintain adequate tissue per- soma cruzi are being developed. The danger of myo- on September 23, 2021 by guest. Protected fusion, partial cardiac by-pass or an artificial heart cardial involvement with possible permanent late device may be considered (Litwak et al., 1976; effects should be a major impetus to the future Radvany et al., 1978). At this time, such devices development of vaccines against Coxsackie viruses. are largely experimental and suited only for Finally, the recognition of cardiotoxins and their temporary use. elimination or the control of their use are most A non-invasive approach to systolic unloading and important components of preventive cardiology. diastolic augmentation, known as external counter- Examples are radiation and cobalt. pulsation (Soroff et al., 1969), warrants further trials in patients with cardiomyopathy. Secondary prevention Earlier approaches to primary cardiomyopathy 13 Cardiac transplantation held that a patient so diagnosed was suffering from Primary congestive cardiomyopathy, by definition, a heart muscle disorder of a specific cause as yet is limited to the heart, occurs generally in young or undiscovered. Increasingly, we have come to recog- full adulthood, is characterized by limited response nize that even an individual patient's primary to medical therapy, is not subject to palliative sur- cardiomyopathy may be pluricausal, and that even Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

Treatment of congestive cardiomyopathy 483 in a secondary cardiomyopathy other aetiological habilitation to self-care, normal family life and even factors may be playing a contributory role. These gainful employment should be considered for all may be considered as risk factors. This concept patients. Although specific information is not as yet of cardiomyopathy permits a therapeutic approach available, it is not unreasonable to expect that in stressing the prevention of additional structural earlier stages of cardiomyopathy carefully designed and functional damage to the myocardium. physical exercise regimens may be of conditioning This approach to secondary prevention is presented value, reducing exercise heart rate and blood pressure, in Table 2. Only a few items tabulated here warrant as is the case in healthy subjects and in patients with additional comment. The acute and chronic depres- ischaemic heart disease (Wilhemsen et al., 1975). sant effect of alcohol upon myocardial function, as well as its enhancing effect upon an already existing Acknowledgments disease of heart muscle, have been so well demon- This work is supported, in part, by Grants HL 10539 and strated in both experimental animals and in man HL 5909 from the National Heart, Lung and Blood Institute, that its use should be severely restricted or pro- National Institutes of Health, Bethesda, Maryland. scribed altogether in patients with cardiomyopathy References (Regan, 1973; Gould et al., 1971). AKBARIAN, M., YANKOPOULOS, N.A. & ABELMANN, W.H. (1966) Hemodynamic studies in beriberi heart disease. American Journal of Medicine, 41, 197. TABLE 2. Secondary prevention of cardiomyopathy AKHTAR, N., MIKULIC, E., COHN, J.N. & CHAUDHRY, M.H. (1975) Hemodynamic effect of dobutamine in patients with 1. Assure balanced nutrition severe heart failure. American Journal of Cardiology, 36, Adequate proteins 202. Adequate vitamins ARONOW, W.S., GREENFIELD, R.S., ALIMADADIAN, H. & 2. Treat DANAHY, D.T. (1977) Effect of the vasodilator trimazosin Hypertension versus placebo on exercise performance in chronic left Anaemia 40, Infection ventricular failure. American Journal of Cardiology,

789. by copyright. 3. Prevent thrombo-embolic complication BUCKLEY, M.J., CRAVER, J.M., GOLD, H.K., MUNDTH, E.D., Anticoagulants DAGGETT, W.M., & AUSTEN, W.G. (1973) Intra-aortic 4. Avoid balloon pump assist for cardiogenic shock after cardio- Overweight pulmonary bypass. Circulation (Suppl. III), 90. Excessive activity BURCH, G.E. & GILES, T.D. (1972) Prolonged bed rest in the Pregnancy management of patients with cardiomyopathy. Cardio- Heat and humidity vascular Clinics, 4, 375. Potassium and magnesium loss DUHME, D.W., GREENBLATT, D.I. & KOCH-WESER, J. (1974) Alcohol Reduction of digoxin toxicity associated with measure- Cigarettes ment of serum levels: a report from the Boston Collabora- Drugs, esp. and tive Drug Surveillance Program. Annals of Internal Medicine, 80, 516. http://pmj.bmj.com/ Radiation DUVERNOY, W.F.C., & GARCIA, R. (1971) Sarcoidosis of the heart presenting with ventricular tachycardia and atrio- ventricular block. American Journal of Cardiology, 28, Cigarette smoking should be considered a myo- 348. EASLEY JR, R.M., SCHREINER JR, B.F. & Yu, P. (1972) cardial stress and avoided, in view of its chrono- Reversible cardiomyopathy associated with hemochro- tropic and positive inotropic effect, combined with matosis. New England Journal of Medicine, 287, 866. an increased afterload and decreased arterial satura- ELSON, S.H. & ABELMANN, W.H. (1965) Effects of muscular

tion for oxygen (Rabinowitz and Abelmann, 1977). activity upon the acute myocarditis of C3H mice infected on September 23, 2021 by guest. Protected It is also evident that, in order to with Trypanosoma cruzi. American Heart Journal, 69, render this 629. approach feasible and effective, early diagnosis of FRANCIOSA, J.A., GUIHA, N.H. LIMAS, C.J., RODRIGUERA, E. cardiomyopathy becomes most important. & COHN, J.N. (1972) Improved left ventricular function during nitroprusside infusion in acute myocardial infarc- Rehabilitation tion. Lancet, i, 650. FRANCIOSA, J.A., PIERPONT, G. & COHN, J.N. (1977) Hemo- Because of the generally guarded prognosis of dynamic improvement after oral hydralazine in left ventri- congestive cardiomyopathy, it is easy for physicians, cular failure. A comparison with nitroprusside infusion in patients and their families to accept a defeatist atti- 16 patients. Annals ofInternal Medicine, 86, 388. tude, if not with regard to therapy, certainly with GATMAITAN, B.G., CHASON, J.L. & LERNER, A.M. (1970) Augmentation of the virulence of murine Coxsackie regard to rehabilitation. The generally good re- virus B-3 myocardiopathy by exercise. Journal of Experi- sponse to therapy of early congestive failure must be mental Medicine, 131, 1121. kept in mind, and especially when heart failure has GOODWIN, J.F. (1973) Treatment of the cardiomyopathies. been precipitated by a treatable or preventable exces- American Journal of Cardiology, 32, 341. GOULD, L., ZAHIR, M., DEMARTINO, A. & GOMPRECHT, R.F. sive cardiac load or intercurrent illness, a cautiously (1971) Cardiac effects of a cocktail. Journal ofthe American optimistic attitude is warranted. A plan for re- Medical Association 218, 1799. Postgrad Med J: first published as 10.1136/pgmj.54.633.477 on 1 July 1978. Downloaded from

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