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A series of 36 Black and Coloured patients, presenting during a 1-year period with life-endangering intrathoracic trauma secondary to assault, is presented. Penetrating wounds of the heart were documented in 32 of these patients, 6 of whom died almost immediately after having been brought into the Resuscitation Unit of Tygerberg Hospital, Parowvallei, CP. The remaining 26 patients had clinical features of cardiac tamponade and circulatory collapse, and in 4 of these patients an emergency thoracotomy was performed in the Resuscitation Unit as it was considered inadvisable to delay surgery until theatre had been arranged. Three of the latter 4 were discharged home completely recovered. Total peri-operative mortality was 13.3%, most deaths being due to lacerations of the left ventricle. Penetrating wounds of the ventricles accounted for some 85% of the total cardiac lacerations; other lacerations affected the pulmonary artery and its branches, the aorta, left atrium and internal thoracic artery. The incision most frequently employed at surgery was a median sternotomy (53%), followed by left thoracotomy (40%) and right thoracotomy (7%). Pre-operative emergency management based on pathophysiological principles is discussed. The fact that relatively inexperienced surgical registrars performed many of these operations with good results emphasizes the need for the establishment of resuscitation units in more peripheral hospitals where many of these patients could primarily be treated. These units would then be able to manage many of these patients, probably at an earlier stage than if they were referred to a teaching hospital such as Tygerberg.

A young Black woman presented with acute severe right ventricular and moderate left ventricular cardiac failure secondary to rupture of a sinus of Valsalva aneurysm originating from the right coronary sinus. The rupture into the right ventricle was accompanied by severe aortic valve insufficiency. The aortic valve showed congenital myxomatous degeneration with no evidence of infective endocarditis or syphilis. Right ventricular endomyocardial biopsy revealed no evidence of any specific cardiac disease such as amyloidosis. Successful closure of the fistula, together with aortic valve replacement and a De Vega tricuspid annuloplasty, was carried out. M-mode and two-dimensional echocardiography delineated the fistula, as well as the most unusual binding of tricuspid valve fluttering, which persisted after operation. Slight insufficiency of the Björk-Shiley aortic prosthesis was noted at postoperative cardiac catheterization. The patient was discharged from hospital asymptomatic and with no abnormal clinical features.

Two White male patients with temporary complete heart block (CHB) secondary to hyperkalaemia are presented. One, a 40-year-old man, developed CHB with ensuing shock within the first 24 hours of repeat aortic valve replacement for a paraprosthetic leak caused by previous endocarditis. This patient experienced iatrogenic hyperkalaemia. The second was an 81-year-old man who had chronic renal failure and presented with Stokes-Adams attacks. This patient was initially thought to have degenerative CHB and nearly underwent inadvertent permanent pacemaker insertion. Both patients were initially treated with emergency temporary cardiac pacing with subsequent successful management. Temporary CHB secondary to hyperkalaemia, from whatever cause, has very rarely been documented in the literature. A review of this potentially lethal complication is undertaken and the significance of unifascicular and bifascicular conduction block as a consequence of hyperkalaemia is discussed.

A young Coloured man whose only symptom was that of minimal dyspnoea on exertion, on examination had signs of infundibular stenosis which were confirmed by cardiac catheterization. Additional features were left anterior hemiblock and cardiomegaly as delineated radiologically. Cross-sectional echocardiography revealed a very large cystic mass located within the interventricular septum which encroached upon the right ventricular outflow tract. This cystic mass was further delineated by cardiac cine angiography. It is postulated that this mass was a primary cardiac echinococcal cyst and was directly responsible for the complications of left anterior hemiblock and the infundibular obstruction. The patient declined surgery and a definitive pathological diagnosis could therefore not be made. If this is a hydatid cyst then it is the second case reported in the literature diagnosed by two-dimensional echocardiography and in which left anterior hemiblock has been recorded. The clinical features, complications and surgical correction are briefly outlined.

An acute myocardial infarction in a young man when there was no fixed atherosclerotic lesion in the coronary arteries is reported. Soon after the myocardial infarction the patient was shown to have thrombosis in the anterior descending branch of the left coronary artery but this later disappeared. In view of recurrent angina pectoris, provocation tests were undertaken with ergometrine maleate, cold pressor and hyperventilation. Both the drug and the hyperventilation provoked coronary spasm accompanied by angina pectoris but no ECG evidence of ischaemia.

A very fit 28-year-old Coloured athlete presented with an acute transmural anteroseptal and non-transmural anterolateral myocardial infarction (MI). He had no significant risk factors for coronary artery disease apart from moderate cigarette smoking. Cardiac catheterization 2 months later demonstrated a significant area of myocardial damage as well as a large mural thrombus, but the coronary arteries appeared normal apart from a large irregular filling defect in the proximal left anterior descending (LAD) branch, apparently due to a thrombus. Cardiac catheterization a further 4 months later documented no further filling defect in the LAD branch and the coronary arteries appeared free of disease. Ergometrine maleate provocation on this occasion failed to demonstrate any coronary vasospasm. Possible pathophysiological mechanisms for the unexpected MI are outlined.

A 48-year-old man with symptoms of presyncope and congestive cardiac failure had hypertrophic cardiomyopathy (HCM) without obstruction. Complete heart block (CHB), a rare complication of this disease, was preceded by complete left bundle-branch block. Right ventricular (RV) heart failure was a dominant clinical feature but improved dramatically after temporary transvenous RV pacing prompting the insertion of a permanent RV inhibited pacemaker. Repeated ventricular fibrillation was successfully controlled by amiodarone. This is the seventh case of HCM complicated by CHB reported in the literature, and the first in which RV endomyocardial biopsies were undertaken. Two other patients reported in the literature had RV inhibited permanent pacemakers implanted, and a further 2 had atrioventricular sequential pacemakers.

A 27-year-old black woman with cardiac failure, angina pectoris and Raynaud's syndrome is presented. Skin biopsy and barium studies established the diagnosis of scleroderma (progressive systemic sclerosis (PSS)). Systemic lupus erythematosus (SLE) was strongly suggested by the results of immunological studies and increasing severity of renal failure. Because of the possibility of a cardiomyopathy, cardiac catheterization, selective coronary angiography and right ventricular endomyocardial biopsy were carried out but failed to show any histological features of either SLE or PSS. The patient went into progressive renal failure despite immunosuppressive therapy and plasmapheresis and died; consent for autopsy was refused. A final diagnosis of mixed connective tissue disease (MCTD) was made. The salient features of cardiac involvement in SLE, PSS and MCTD are outlined.

It is important to exclude coronary vasospasm, the mechanism responsible for so-called 'dynamic' coronary stenosis, when selecting of patients for percutaneous transluminal coronary angioplasty (PTCA). Although cine angiographic demonstration of this frequently fleeting entity can sometimes be difficult, a strong suspicion should be aroused by a carefully taken history. The danger of PTCA in these cases of Prinzmetal's variant angina, as well as the frustration often encountered in drug management, is high-lighted.

Death directly related to selective coronary arteriography in 5 patients with a history of unstable angina pectoris during the period 1975-1985 is reported. Four different cardiologists were involved. A feature common to all the cases was the presence of significant ostial stenosis of the left mainstem coronary artery (LMCA); 2 patients had haemodynamically important obstruction of a dominant right coronary artery (RCA) ostium, while 2 others had total occlusion in the proximal part of a dominant RCA. The RCA in the last case was angiographically normal and non-dominant. Collateral coronary blood flow was fairly sparse in most cases and in 4 left ventricular dysfunction of varying degree was present. All patients developed severe hypotension and electromechanical dissociation after arteriography while still in the cardiac catheterization laboratory. Resuscitation efforts were uniformly unsuccessful. Autopsy on 1 patient demonstrated extensive obstructive coronary atherosclerosis with a massive acute anterior myocardial infarction. Cardiac catheterization poses an extremely high risk for this subgroup of patients with LMCA disease, as does selective coronary arteriography. The possible role of catheter-provoked coronary vasospasm of the LMCA is suggested; a recently introduced soft-tipped cardiovascular catheter may be more appropriate in this setting.

A young Black man with many features of pseudoxanthoma elasticum (PXE), confirmed by skin biopsy, complained of classic angina pectoris, decreasing effort tolerance, and palpitations. Clinically he was in severe congestive cardiac failure which was confirmed by echocardiography and cardiac catheterization, investigations which indicated the presence of a 'congestive' cardiomyopathy. Selective coronary arteriography showed normal epicardial vessels. Antemortem endomyocardial biopsy in this condition is described for the first time in the literature. This showed abnormal light microscopic and electron microscopic features. It is postulated that the predominant cause of congestive cardiac failure and angina pectoris in this disease is a diffuse arteriopathy secondary to elastic fibre dysgenesis, involving the small intramural coronary vessels ('small-vessel disease'). Hitherto it has been accepted that the endocardial changes have been most important in the pathophysiology. A review of the literature as it applies to cardiac involvement in PXE is undertaken.

A young woman had a pulmonary arteriovenous fistula localized to the right lower lobe. The presence of a patent foramen ovale (atrial septal defect) detected preoperatively allowed for a unique investigation of this case. Her successful surgical management is outlined and the literature is reviewed.

A 36-year-old man with classic angina pectoris had marked ST-segment elevation (STE) in the inferior leads on stress-testing in the absence of chest pain. There was no evidence of previous myocardial infarction (MI). Selective coronary arteriography delineated severe obstructions in the right coronary artery (RCA) with additional left circumflex coronary artery (LCx) obstruction. Left ventricular cine-angiography established that there was normal contractility and confirmed the absence of past MI. Coronary artery bypass graft surgery to the RCA and LCx was unfortunately complicated by an acute transmural inferoposterolateral MI. Treadmill stress testing 6 weeks after surgery failed to demonstrate the preoperative ST-segment change. The patient may have developed exercise-induced coronary artery spasm superimposed on the severe proximal RCA stenosis; this in turn may have caused the inferior STE. Exercise-induced STE is reviewed.

The clinical presentation of a young hypertensive White man with acute high lateral non-transmural myocardial infarction (MI) is documented. This diagnosis was established on the grounds of a history of chest pain, elevated serial serum enzyme levels, technetium-99m pyrophosphate ('hot-spot') scintigraphy, exercise thallium-201 ('cold-spot') scanning, left ventricular cine angiography and selective coronary arteriography. Daily resting 12-lead ECGs failed to demonstrate unequivocal features of acute non-transmural subendocardial MI. The diagnostic difficulties facing the clinician in a case of acute MI associated with a non-diagnostic ECG are stressed, and the ECG features of acute subendocardial MI are reviewed.

In a 51-year-old medical colleague with symptomatic atherosclerotic coronary artery disease, coronary arteriography delineated significant left mainstem, left anterior descending and left circumflex coronary artery lesions and cine angiography demonstrated normal left ventricular contractility. Aortocoronary saphenous vein bypass grafting was successful. The postoperative appearance of QS waves on the ECG suggested the possible complication of an acute transmural anteroseptal and anterolateral myocardial infarction (MI). However, this possibility was excluded by resting technetium-99m and thallium-201 scintiscans, as well as by a technetium-99m-gated blood pool scintiscan. The occurrence of acute pericarditis approximately 2 weeks after surgery made clinical evaluation more difficult. The ECG may represent a pseudo-MI pattern, the patient having suffered a post-pericardiotomy syndrome. The importance of excluding postoperative acute MI is stressed. The causes of the appearance of new Q waves after aortocoronary saphenous vein bypass graft surgery are briefly outlined.

Over a period of 4 years Holter monitoring was performed on 607 patients in the Division of Cardiology at Tygerberg Hospital. Indications for monitoring were broadly grouped into four categories: (i) evaluation of symptoms suggestive of disorders of cardiac rhythm (210 patients); (ii) evaluation of arrhythmias associated with a specific underlying cardiac condition (139 patients); (iii) evaluation of a previously documented or suspected arrhythmia (233 patients); and (iv) miscellaneous reasons (25 patients). Findings are presented and aspects of Holter monitoring are discussed. Finally, some recommendations for improving the clinical value of our Holter analyses are made.

Two young Coloured men with proven syphilitic coronary ostial stenosis had severe angina pectoris unresponsive to conventional medication. One underwent an aortic valve replacement for severe aortic insufficiency associated with subtotal ostial occlusion of the right coronary artery (RCA), which was corrected by an aortocoronary bypass graft; the left coronary artery (LCA) ostium was normal and patent. The other patient had total occlusion of the LCA ostium which resulted in an extensive transmural anteroseptal and anterolateral myocardial infarction; the RCA ostium was unaffected and the aortic valve appeared normal. He was considered unsuitable for cardiac surgery and continued to receive anti-anginal drug therapy with quite satisfactory improvement in symptoms. Non-atheromatous coronary artery disease must always be sought for and excluded when a non-White patient presents with symptoms of ischaemic heart disease. Although atheromatous coronary artery involvement is becoming increasingly prevalent among 'westernized' Black and Coloured subjects, it is still relatively unusual in comparison with the extremely high incidence in the White population.

A 20-year-old Coloured man gave a history of atypical chest pain, palpitations after strenuous exercise and a single episode of post-exertional presyncope. The diagnosis of hypertrophic non-obstructive apical cardiomyopathy (HNOAC) was established by means of electrocardiography, echocardiography (both M-mode and two-dimensional) and left ventricular cineangiography. This variant of hypertrophic cardiomyopathy is most unusual and has been encountered most frequently in Japan, although a few cases have been diagnosed in the USA. The present case is the second reported from the Republic of South Africa. Important aspects of HNOAC are reviewed.

A 43-year-old coloured man had no risk factors for atheromatous coronary artery disease but suffered two acute myocardial infarctions (MIs) in rapid succession. Serological reactions for previous syphilitic (luetic) infection were positive. Hypertrophic cardiomyopathy (HCM) without obstruction was verified, although right ventricular endomyocardial biopsy specimens did not demonstrate histological features of this disease. Extensive MI was verified on left ventricular cine angiography. Selective coronary arteriography showed that the coronary arterial tree was diffusely aneurysmal in the absence of any obstruction. We postulate that syphilitic coronary arteritis, in the absence of the more pathognomonic coronary ostial stenotic lesions, was present and may have predisposed to coronary thrombus formation and repeated acute MI. Recurrent coronary vasospasm, associated with the HCM, cannot be excluded with certainty.

A young man had a congenital sinus of Valsalva aneurysm originating from the right coronary sinus, complicated by fistulas draining into both right atrium and right ventricle, as well as a congenitally abnormal aortic valve with mild aortic insufficiency. His dramatic clinical presentation, with the sudden appearance of severe biventricular cardiac failure unresponsive to intensive medical therapy, was an important clue to making the correct pre-operative diagnosis. The use of non-invasive techniques, such as phonocardiography and M-mode and two-dimensional echocardiography, is highlighted. Full cardiac catheterization was employed to define the cardiac pathophysiology. This was one of the few cases documented in which a catheter could be passed from the aorta into the right ventricle via the fistula connecting these two chambers. The fistulas were closed and the aortic valve replaced. Postoperative investigations confirmed the success of corrective surgery. As far as we are aware this is the first documented case of successful repair of a congenital sinus of Valsalva aneurysm rupturing into both the right atrium and right ventricle, accompanied by aortic insufficiency.

A fairly young man suddenly developed a large ventricular septal defect (VSD) after an extensive anterolateral and anteroseptal myocardial infarction. Because of congestive cardiac failure refractory to medical treatment he underwent simultaneous closure of the VSD and left ventricular aneurysmectomy 6 weeks after the myocardial infarction. Some 14 months after operation the patient is completely asymptomatic on the minimum of medical therapy and is able to live normally. The literature on these two combined lesions complicating acute myocardial infarction and their surgical correction was caused by coronary spasm of the Prinzmetal variety with underlying normal coronary arteries.

A 42-year-old White man suffered from recurrent symptomatic ventricular tachycardia but no angina pectoris. Cardiac catheterization demonstrated a normally contracting left ventricle and coronary angiography delineated significant atherosclerotic obstructions in the left circumflex (LC) coronary artery and the first diagonal branch of the left anterior descending (LAD) coronary artery. Coronary artery bypass graft (CABG) surgery was carried out on the anterolateral and mid-lateral branches of the LC coronary artery as well as the first diagonal branch of the LAD coronary artery. Frequent postoperative Holter monitoring as well as maximum-exercise stress testing has failed to show any recurrence of the ventricular arrhythmia, and the patient has remained asymptomatic and medical therapy has been discontinued. Some 30 months after operation left ventricular cine angiography demonstrated normal contractility. Selective coronary arteriography indicated that the CABG to the anterolateral branch of the LC coronary artery was occluded at its proximal aortic anastomosis. However, the CABGs to the midlateral branch of the LC and LAD coronary arteries were still patent. Repeat serial resting ECGs failed to show any evidence of postoperative myocardial infarction. It is concluded that CABG surgery was responsible for eliminating the episodes of life-threatening ventricular tachycardia, presumably by correcting myocardial ischaemia. The role of CABG surgery in the control of medically unresponsive and dangerous ventricular arrhythmias is reviewed.

A double-blind study was performed on 50 elderly patients undergoing hip-replacement surgery under general anaesthesia; 26 were given nifedipine and the remaining 24 placebo to determine effects on the continuously monitored (Holter) ECG during the 4 peri-operative days. Drugs were only administered during the latter 3 days of the observation period. Surgery was performed on the morning of the 3rd day. A striking feature was the high incidence of arrhythmias in both groups of patients, a finding previously documented in both 'normal' and elderly people. A decrease in ST-segment changes was expected in the nifedipine-treated patients. An unexpected finding, therefore, was the lack of protection against cardiac ischaemic changes in the nifedipine-treated patients compared with the placebo patients. Interpretation of the ST segment as seen in the Holter-monitored ECG remains controversial. We have no clear explanation for the lack of protection against ischaemic changes. The effects of profound vasodilatation produced by nifedipine in elderly patients subjected to major surgery, general anaesthesia including administration of enflurane, and a variable amount of blood loss in the postoperative period may be important factors. In conclusion, one should perhaps be cautious of nifedipine administration under these circumstances.

A review of the literature on primary cardiac amyloidosis is presented. Its differentiation from constrictive pericarditis, both on clinical and haemodynamic grounds, is outlined. The importance of antemortem endomyocardial biopsy in establishing a definitive diagnosis of this relatively rare condition is stressed. Finally, an updated overview of the classification and immunological pathogenesis of this disease is given.

A case of calcific constrictive pericarditis in a young White woman with a convincing history of previous acute rheumatic fever complicated by a possible valvular lesion is presented. Cardiac catheterization confirmed the suspicion of significant cardiac compression. Successful pericardiectomy was carried out, but microscopical examination of the excised pericardium failed to demonstrate a cause. In view of the strong past history of acute rheumatic fever and mild mitral insufficiency demonstrated at cardiac catheterization, the author proposes that the calcific constrictive pericarditis was of rheumatic origin. A review of the literature on the association between rheumatic infection and constrictive pericarditis follows.

A family with inherited cardiac and skeletal muscle disease was also found to have members with low intelligence. The effects of social and environmental conditions upon the behaviour of family members are described, with particular attention to the sociomedical problems created by the combination of a hereditary disease and low intelligence.

A 31-year-old doctor's wife suffered from severe unstable angina pectoris (AP) due to two large, heavily calcified masses involving the right coronary artery and the left anterior descending branch of the left coronary artery. The causes of the masses could not be determined with certainty, but in view of the history (which included the ingestion of large quantities of raw boerewors (traditional spiced sausage) and histopathological findings, we believe that they were coronary artery aneurysms which developed secondary to coronary arteritis many years previously. The possibility of echinococcal (hydatid) infection is also discussed. Cardiac surgery entailed total excision of both masses, together with sections of their accompanying coronary arteries which had become fibrotic as a result of the arteritis, and reestablishment of coronary blood flow by the insertion of two saphenous vein coronary artery bypass grafts. Her AP was dramatically relieved and she continues to be asymptomatic without taking anti-anginal drugs.

A family consisting of parents and their 6 sons were investigated to elucidate the relationship between a hypertrophic cardiomyopathy, musculoskeletal abnormalities and mental subnormality. The proband was diagnosed as having definite hypertrophic obstructive cardiomyopathy and the remaining family members were shown to have a spectrum of hypertropic non-obstructive cardiomyopathy. Mild muscle weakness was present in 3 sons. All the subjects except for 1 son showed definite signs of electromyographic abnormality, whereas sensory and motor conduction velocities were normal. All the EEGs except for that of the proband were normal. Testicular hypoplasia was present in 3 sons. The inheritance pattern appears to be polygenic autosomal recessive in type. Definite evidence of linkage between hypertrophic cardiomyopathy and HLA awaits further data.

An elderly White woman suffering from an acute transmural inferior myocardial infarction, with possible true posterior extension, is presented. Her holosystolic cardiac murmur and hypotension are attributed to rupture of the interventricular septum which occurred between the 2nd and 3rd days after infarction. Strong evidence for concomitant right ventricular infarction is put forward and therapy is discussed. As far as the authors can determine, this combination of cardiac lesions has not been documented ante mortem previously. The apparent beneficial use of intravenous hydrallazine, for the first time in right ventricular infarction, is discussed.

A 64-year-old white woman with moderately severe rheumatic mitral stenosis complicated by atrial fibrillation and recurrent systemic embolisation to the brain was found at operation to have a large 'free-floating' left atrial thrombus, as well as multiple left atrial appendage thrombi. These had not been detected by echocardiography. She also had significantly reduced left ventricular contractility on cine angiography, and right coronary artery atherosclerosis. She underwent successful mitral valve replacement and excision of the left atrial appendage.

A young man with primary cardiac amyloidosis presented clinically as a case of 'restrictive' cardiomyopathy with an initial diagnosis of constrictive pericarditis, probably of tuberculous origin. Cardiac catheterization showed features compatible with restrictive cardiomyopathy or constrictive pericarditis but pericardial biopsy was negative. Percutaneous right ventricular endomyocardial biopsies established amyloid infiltration of the myocardium, but rectal, tongue and liver biopsies were all negative for amyloidosis. Bence-Jones protein was absent from the urine. Serum electrophoresis, immunophoresis, and bone marrow examination all failed to detect possible multiple myelomatosis as a cause of secondary amyloidosis. Respiratory function tests showed a restrictive picture, probably secondary to chronic congestive cardiac failure. Thus, this case is the second example of primary cardiac amyloidosis in the world literature which has been dianosed by right ventricular endomyocardial biopsy. Phonocardiographic features of early aortic valve closure are reported for the first time in amyloid restrictive cardiomyopathy. Since tuberculous constrictive pericarditis is an important cause of cardiac disease in this country, with effective therapy, the authors stress more frequent use of endomyocardial biopsy as a safe diagnostic tool to exclude other similar disease states. The patient was offered the possibility of a cardiac transplantation in view of the localized nature of his disease, but refused. He now continues to respond unsatisfactorily to antifailure therapy with, presumably, a poor prognosis.

A 46-year-old black man employed in an explosives factory and in direct contact with industrial nitroglycerin complained of angina pectoris both at rest and with effort. Special investigations established the presence of dilated (congestive) cardiomyopathy (COCM), transmural anteroseptal and anterolateral myocardial infarction (MI), and total proximal occlusion of the left anterior descending branch of the left coronary artery. Apart from this total occlusion, the coronary arteries appeared free of disease on angiography. It is postulated that some of the episodes of angina pectoris at rest were related to industrial nitroglycerin withdrawal, and that the pathophysiological mechanism involved was coronary vasospasm leading to thrombus formation which culminated in MI, aggravating the congestive cardiac failure due to the COCM. It is less likely that this MI was caused by a coronary embolism originating from the mural thrombus in the left ventricle associated with COCM.

A 42-year-old symptomatic woman with possible adult Williams's syndrome (mental retardation, 'elfin facies' and supravalvular aortic stenosis (SVAS)) is documented. This patient displayed many unique features in addition to the severe SVAS (peak systolic gradient 96 mmHg): there was an associated persistent left superior vena cava draining into the coronary sinus, mitral valve prolapse (Barlow's syndrome) and complete right bundle-branch block on ECG. Peripheral pulmonary artery stenosis was absent. M-mode echocardiograhy in the adult with SVAS is described for the first time in the literature, as is the use of the 60 degrees cross-sectional scan. Both these non-invasive procedures proved of value in the diagnosis. The degree of subendocardial ischaemia, as determined by the 'endocardial viability ratio', was calculated and the possible mechanisms producing angina pectoris with 'coronary artery hypertension' in this condition are discussed. The literature on associated mitral valve abnormalities in SVAS is reviewed. A successful surgical result was obtained; the various forms of operation are outlined.

A case of 'primary' mitral valve prolapse is documented. The patient was admitted with right-sided hemiplegia of sudden onset, probably caused by a cerebral embolus from the mitral valve. He also had a painless transmural inferior myocardial infarction (MI) of indeterminate age which was diagnosed electrocardiographically and on left ventricular cine angiography. Since selective coronary arteriography delineated the absence of fixed obstructive atherosclerotic disease, and since coronary vasospasm could not be provoked with the ergonovine (ergometrine) maleate test, it is further postulated that a coronary embolus from the abnormal mitral valve apparatus was responsible for the painless MI. A percutaneous right ventricular endomyocardial biopsy specimen displayed findings not indicative of a 'cardiomyopathy'.

A 46-year-old White man had a 2-year history of frequent atypical chest pain associated with palpitations and presyncope. Clinical examination revealed an intermittent mid-systolic non-ejection click followed by a blowing mid-to-late systolic murmur indicative of the billowing mitral leaflet syndrome (BMLS) (primary mitral valve prolapse (MVP); Barlow's syndrome). This diagnosis was confirmed on cardiac catheterization, left ventricular cine angiography showing mild mitral insufficiency. M-mode and cross-sectional echocardiography documented the intermittent non-ejection click and mid-systolic apical murmur. Ambulatory Holter monitoring showed symptomatic inferolateral myocardial ischaemia, and maximal stress-testing elicited asymptomatic ischaemia in the same zone. Selective coronary angiography delineated a normal left coronary artery and an insignificant fixed obstructive lesion in the second part of the dominant right coronary artery (RCA). Provocation with ergometrine (ergonovine) maleate gave rise to severe coronary vasospasm superimposed on the insignificant lesion in the RCA. This spasm provoked myocardial ischaemia resulting in symptomatic ventricular fibrillation which was successfully reversed. The patient's symptoms have been fairly well controlled by nitrates and nifedipine. As far as I am aware this is the first documentation of coronary vasospasm in the BMLS. In this syndrome coronary artery spasm has often been postulated to be responsible for acute myocardial infarction with a normal appearance of the coronary arteries on angiography. This mechanism has also been incriminated in the genesis of ventricular arrhythmias in cases of primary MVP. These various contentious and important issues are reviewed.

A patient underwent aortocoronary saphenous bypass grafting to the left anterior descending coronary artery (LADA) and its first diagonal branch for the relief of severe angina pectoris. There was difficulty in exposure of the LADA, which was covered by a thick layer of epicardial adipose tissue. Postoperatively the patient continued to experience severe retrosternal pain which prompted early repeat coronary angiography. This revealed an aortocoronary vein fistula (AVF) secondary to the inadvertent anastomosis of one of the saphenous vein grafts to the left anterior descending coronary vein (LADV). It was only after this procedure that clinical examination revealed a grade 2/6 high-frequency continuous murmur best heard in the 2nd and 3rd left intercostal spaces. The persistance of severe angina pectoris at rest and on effort led to performance of a revision operation. The LADV was then transected and ligated, and an extended saphenous vein reimplanted into the LADA proper. This provided complete relief from angina. This is the first such case reported in South Africa and the sixth documented. In all the aortosaphenous vein grafts had been inserted into the LADV; all exhibited the classic continuous murmur postoperatively, apart from 1 patient who had an ejection systolic murmur. None of the other 5 patients complained of postoperative angina and only 3 were subjected to revision surgery. The literature is reviewed, with emphasis on the rationale for performing the first elective aortocoronary vein anastomosis in 1968. Clinical features of iatrogenic AVF are detailed and an attempt is made to assess when re-operation is indicated. It is easy to fail to notice this complication; however, its correction can have a dramatic effect on the patient's symptoms. It is the author's belief that many more such cases exist but are not being detected, probably because of lack of appreciation of the possibility of this iatrogenic condition occurring.

A middle-aged Coloured man had a 6-year history of chest pain induced by effort and also experienced at rest. Quite dramatic episodes of associated arrhythmias, specifically 'torsade de pointes' (atypical ventricular tachycardia) and syncope were experienced by the patient, despite the use of numerous anti-arrhythmic and anti-anginal agents. Transmural anteroseptal and non-transmural anterolateral myocardial infarctions were documented in the presence of a normal left coronary artery (LCA). Severe reversible vasospasm of the right coronary artery (RCA) was provoked with the use of ergonovine (ergometrine) maleate at cardiac catheterization. It is postulated that the cause of the previous myocardial infarctions was significant vasospasm of the LCA branches, and that he was subject to multiple coronary vasospasm, as was highlighted by the visualization of spasm superimposed on atheromatous plaque within the RCA. Furthermore, it is strongly suggested that the potentially lethal ventricular arrhythmias, including 'torsade de pointes', were a direct result of coronary vasospasm, which in turn gave rise to his presyncope and syncope attacks. No evidence of sinoatrial node disease could be found. The only risk factor for ischaemic heart disease which applied in his case was heavy cigarette smoking. Control of his disabling symptoms seems to have been achieved by the use of maintenance nifedipine (a calcium-blocking agent), long-acting nitrates (isosorbide dinitrate) and quinidine gluconate, confirming the probable vasospastic aetiology of the 'torsade de pointes'. At no stage was there dangerous prolongation of the QT interval, an oft-quoted prerequisite for this arrhythmia. Some of the more important aspects of coronary vasospasm are discussed; as far as I am aware this is the first patient documented in the literature with 'torsade de pointes' associated with angiographically demonstrated coronary artery spasm.

A review of the literature relating to endomyocardial biopsy (EMB) is presented. This is considered important at this time since EMB is being utilized with increasing frequency, particularly for the diagnosis of myocarditis. The development of the technique is briefly outlined. Emphasis is placed on the clinical application of EMB in the various primary cardiomyopathies (dilated, hypertrophic, restrictive, and obliterative), the infiltrative secondary cardiomyopathies (amyloidosis, sarcoidosis, hemochromatosis), myocarditis, as well as such conditions as adriamycin cardiotoxicity, cardiac transplant rejection, and Kawasaki disease. More controversial application of EMB in primary mitral valve prolapse (Barlow's syndrome), idiopathic ventricular arrhythmias, and the elucidation of the enigmatic finding of angina with angiographically normal coronary arteries is detailed. Experience with immunological and biochemical investigation of biopsy material, as well as with virus isolation and drug assays in the myocardium, is alluded to. Complications encountered with this procedure are also discussed, and its future role is contemplated.

A Black employee exposed to industrial nitroglycerin (NG) in an explosives factory presented with severe precordial pain. The clinical presentation was that of significant transient anteroseptal and anterolateral transmural myocardial ischaemia which responded promptly to sublingual isosorbide dinitrate. Despite being removed from exposure to industrial NG and receiving therapy with long-acting oral nitrates and calcium antagonists, the patient continued to experience repeated attacks of severe retrosternal pain, although transient myocardial ischaemia was not demonstrated electrocardiographically during these episodes. Cardiac catheterization revealed a normal myocardial haemodynamic system and selective coronary arteriography delineated coronary arteries free from any obstructive lesions. An ergonovine (ergometrine) maleate provocative test failed to elicit coronary artery spasm, although this was undertaken while the patient was on nitrate and calcium-blocker therapy. Clinical records of previous significant constrictive pericarditis (probably due to tuberculosis) with resultant abnormalities on the ECG complicated the diagnosis. Evaluation was further hindered by the known "variant pattern' seen on the ECGs of members of the Black population. We postulate that this patient's clinical features were a direct result of severe vasospasm affecting the left coronary artery; it is also strongly suggested that withdrawal from contact with industrial NG precipitated this potentially lethal coronary vasospasm. The role played by industrial NG in ischaemic heart disease is reviewed, as well as the importance of the "normal variant pattern' in the assessment of cardiac disease in Black patients. As far as we are aware this is the first time that the use of the ergonovine maleate provocative test has been documented in the industrial NG withdrawal syndrome.

Skeletal muscle morphology and mitochondrial oxidative phosphorylation capacity were examined in a family whose members showed very combinations of mental subnormality, cardiomyopathy and muscle weakness. Light and electron microscopic findings suggested a neuropathic process, while tests of mitochondrial function indicated a state of tight coupling of oxidative phosphorylation, a feature in marked contrast to those in biochemical studies so far reported.

A 32-year-old white woman presented with angina pectoris and an acute myocardial infarction (MI) complicated by congestive cardiac failure. Other symptoms and results of immunological investigation were highly suggestive of systemic lupus erythematosus (SLE). Thallium-201 scintigraphy confirmed an extensive MI, as initially suspected from an ECG. Cardiac catheterization delineated a poorly contracting left ventricle secondary to MI. Selective coronary angiography showed features suspicious of coronary arteritis involving the left anterior descending and left circumflex coronary arteries. Right ventricular endomyocardial biopsy failed to show any 'small-vessel disease', vasculitis or myocarditis. We suggest that the acute MI was caused by coronary arteritis due to SLE. Overview of the literature indicates that coronary arteritis is not as rare a complication of SLE as previously believed; however, acute MI is most unusual.

A case of acute transmural anterior myocardial infarction in a 45-year-old Black employee of an explosives factory during a period of withdrawal from industrial nitroglycerin is documented. Angiography revealed that the patient had normal coronary arteries. Coronary vasospasm could not be induced by the ergometrine (ergonovine) maleate provocation test. It is postulated that the infarction was directly attributable to coronary vasospasm provoked by the 'industrial nitroglycerin withdrawal syndrome', since there was no evidence of any other non-atheromatous aetiological factor. The authors believe this to be the first such case in a Black subject reported in the literature.

A middle-aged urbanised black man with unstable angina pectoris showed unusual findings on serial resting ECGs recorded during episodes of chest pain, as well as during symptom-free intervals. The 'normal variant pattern', known to occur in blacks, was recorded in the absence of angina; in a white patient with chest pain it would have been considered as being due to a possible hyperacute myocardial infarction. During repeated episodes of severe angina, 'pseudonormalisation' of the 'normal variant pattern' was seen but the pointer to myocardial ischaemia was the simultaneous occurrence of ST-segment depression, Selective coronary arteriography delineated critical lesions in both the left anterior descending and dominant left circumflex coronary arteries. Coronary artery bypass surgery was successful. The importance of recognition of the 'normal variant pattern' in the black population in which the incidence of atherosclerotic coronary artery disease is rising, is emphasised.

A 60-year-old woman with mild hypertension and presumed ischaemic heart disease was followed up over a very long period on account of angina pectoris. Acute myocardial infarction (MI) was suspected on the basis of the history, ECG findings and serum enzyme values, but disproved by radioisotope investigation. Echocardiography demonstrated features of hypertrophic obstructive cardiomyopathy (HOCM), a diagnosis supported by cardiac catheterization and endomyocardial biopsy (EMB). Histological features of HOCM were absent from left ventricular EMB specimens despite a significant intraventricular gradient, but the right ventricular EMB demonstrated extensive changes of HOCM despite a small intraventricular gradient. Cardiac catheterization excluded previous MI and coronary artery disease. A further interesting feature was the development of congestive cardiac failure, which necessitated modification of her drug therapy).