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1 The favourable haemodynamic effects of captopril in patients with congestive heart failure have been reported. 2 We have treated 25 patients with severe chronic congestive heart failure with captopril in doses of 75-450 mg daily. Before entering the study all patients remained in New York Heart Association functional class IV despite high- dose diuretic and vasodilator therapy. 3 Mean cardiothoracic ratio was 60%, and all patients had a shortening fraction of 18% or less on echocardiography (normal 25 to 40%). 4 Five patients died within one month of captopril and five between four and seven months, three of whom had improved to class IIM and one to IIS before death. 5 Of the 15 survivors one was referred for a heart transplant when he had improved to class IIM. The remaining 14 patients were followed for 8-16 months. Ten improved to New York Heart Association class I or IIS and four to class IIM or III. Diuretic requirements were decreased considerably in all 14. Side effects were common but captopril did not have to be withdrawn. Captopril is a highly effective drug in the treatment of patients with congestive heart failure refractory to currently accepted therapy. [ABSTRACT FROM AUTHOR]

Left atrial thrombi are not infrequent in patients with mitral stenosis. Occasionally, free-floating thrombi have been reported in these patients. We report on the echocardiographic and Doppler findings in a patient with mitral stenosis.

Objective: To describe the demographic, pathologic, and hemodynamic profiles of patients with severe rheumatic mitral valve disease in a developing country and to assess their relation to uncontrolled rheumatic disease activity., Design: Retrospective, cross-sectional, cohort study., Setting: Tertiary medical center in Soweto, South Africa., Patients: 714 of 737 consecutive black patients, 4 to 73 years old, with pure mitral regurgitation, pure mitral stenosis, or mixed mitral disease who had mitral valve surgery and in whom preoperative and surgical data were concordant., Measurements: Valve lesions were evaluated on the basis of clinical, echocardiographic, hemodynamic, and surgical pathologic data. Active rheumatic carditis was diagnosed according to clinical evidence for concurrent acute rheumatic fever (Jones criteria), macroscopic appearances at surgery, and histologic findings., Results: 219 patients had pure mitral regurgitation, 275 had pure mitral stenosis, and 220 had mixed lesions. Ongoing rheumatic activity was diagnosed in 106 patients with pure regurgitation (47%) and in only 5 patients with pure stenosis (2%). Pure regurgitation was the most common lesion in the first and second decades; the relative prevalence of pure stenosis increased with age. Purely regurgitant valves had pliable, unscarred leaflets (95%), dilated mitral annuli (95%), elongated chordae tendineae (92%), and anterior leaflet prolapse (81%). In contrast, purely stenotic valves had fused leaflet commissures (100%) and rigid leaflets (38%) but no evidence of prolapse., Conclusions: The spectrum of rheumatic mitral valve disease that is hemodynamically severe in developing countries differs from that currently reported in the United States. Severe, pure rheumatic mitral regurgitation is as prevalent as pure stenosis but has an entirely different time course, surgical anatomy, and relation to disease activity, suggesting a separate pathophysiologic mechanism.

Fulminating active rheumatic carditis has been observed for over three decades in this environment with no recent alteration in either the incidence or the pattern of presentation. Heart failure (in this context defined as 'an inadequate circulation at rest together with a raised pulmonary venous pressure, with or without an associated high systemic venous pressure in the absence of haemodynamically significant tricuspid valve disease or pericardial effusion') is prevalent but occurs only when a haemodynamically important left-sided valve lesion supervenes. Regurgitation is the predominant valve lesion and involves principally the mitral valve. Mitral annular dilatation is marked and predisposes to lengthening--or rupture--of chordae tendineae and prolapse of the anterior leaflet. The resultant cardiac work-overload apparently perpetuates or aggravates the rheumatic activity. Heart failure, as defined, whether caused by or associated with active rheumatic carditis, makes surgical management of the valve lesion mandatory as a life-saving measure. Aggressive medical therapy for heart failure, which should include vasodilator drugs and especially angiotensin-converting enzyme inhibitors, provides temporary improvement only. Contrary to ongoing doctrine, treatment with steroid drugs in this context is neither life-saving nor beneficial.

The mechanism of severe mitral regurgitation (MR) due to active rheumatic carditis is ill defined. This study involved 73 patients, aged 7 to 27 years (mean 13), with severe MR and active rheumatic carditis who were subjected to surgery. Sixty-one were studied retrospectively (group 1) and 12 prospectively (group 2). Active rheumatic carditis was diagnosed according to the modified Jones' criteria, morphologic appearances of the heart at operation and histology of the valve. All patients had preoperative 2-dimensional echocardiographic and intraoperative assessment of the mitral valve apparatus. The presence of mitral valve prolapse--defined as failure of leaflet edge coaptation resulting in systolic displacement of the free edge of the involved leaflet toward the left atrium--was determined in all patients. Mitral anular diameter and maximal systolic chordal length were measured at 2-dimensional echocardiography in group 2 patients and compared to values obtained from matched control subjects. Anular and chordal dimensions in 6 of the group 2 patients were correlated with precise measurements obtained at surgery. Mitral valve prolapse involving the anterior leaflet was detected on echocardiography and confirmed at surgery in 69 patients (94%). Mitral anular dilatation was observed at operation in 70 patients (96%). Maximal anular diameter was significantly greater (p less than 0.0001) than in matched control subjects (37 +/- 4 vs 23 +/- 2 mm). The mean anular dimension measured at surgery (36 +/- 3 mm) was similar to that obtained by echocardiography and individual values using the 2 methods correlated well (r = 0.93). Chordal elongation was observed in 66 patients at operation (90%).(ABSTRACT TRUNCATED AT 250 WORDS)

In 1972 we conducted a survey of 12,050 urban Black schoolchildren and detected 168 (prevalence rate of 14 per 1,000) with a non-ejection systolic click (NESC), a late systolic murmur, or both. The etiology of the mitral valve abnormality was unknown but we considered that a significant proportion might have early rheumatic heart disease. The auscultatory features four years later of 139 of the original 168 subjects as well as those of 139 age- and sex-matched controls are presented in this study. No cardiac abnormality was detected in as many as 55 of the subjects. Five children now had pansystolic murmurs but the mitral regurgitation was assessed as mild in four. Twenty-five (17.9 per cent) of the controls, 23 of whom had NESCs, had auscultatory features compatible with mitral valve prolapse. These findings do not support our earlier suggestion that a large number of the 1972 subjects have mild rheumatic heart disease. The results are in accord with other studies which have indicated that auscultatory features compatible with mitral valve prolapse are common in "normals" and also that the prognosis of the specific "billowing mitral leaflet syndrome" is generally benign.

Mitral valve aneurysm is an uncommon complication of infective endocarditis. This report describes a patient with severe regurgitation due to perforations in a mitral aneurysm who required mitral valve replacement 9 years after a staphylococcal infection was superimposed on a billowing mitral leaflet. The unusual auscultatory signs and angiographic appearance could have led to diagnosis of the aneurysm.

Nineteen adolescent or adult patients with secundum atrial septal defect (ASD) underwent pulmonary arteriography to evaluate the presence of proximal pulmonary arterial (PA) thrombosis. This procedure demonstrated proximal PA thrombosis in 8 patients (group 2). These patients had a distinctive hemodynamic profile, consisting primarily of significant PA hypertension. None of the 11 patients with normal angiograms (group 1) had severe PA hypertension (p less than 0.0001). Proximal PA thrombosis appears to be the major factor in the development and progression of PA hypertension in adult patients with ostium secundum ASD. Pulmonary angiography should be undertaken in all adult patients with ostium secundum ASD who have at least moderate PA hypertension. Long-term anticoagulation is advocated for patients with PA thrombosis irrespective of a decision for surgical intervention.

The interrelationships between body weight, insulin secretion and serum lipids were studied in 40 young white patients (mean age 37 years) with established ischemic heart disease (IHD), living in Johannesburg. None was severely obese, hypertensive or overtly diabetic. In general, strong positive correlations were found between body weight and insulin concentrations and between insulin levels and fasting serum triglycerides. However, insulin levels were relatively low in 4 patients with marked hypertriglyceridemia (above 350 mg/dl). These data are consistent with the postulate that insulin promotes (hepatic) triglyceride synthesis, but when there is gross hypertriglyceridemia peripheral triglyceride clearance becomes defective. Insignificant correlations were observed between body weight and serum lipids and between cholesterol and other metabolic variables. We conclude that there is a sequential link between increasing body weight, insulin secretion and triglyceride levels in young patients with IHD, but that cholesterolemia is independent of this axis.

The timing of surgery in patients with severe aortic regurgitation and left ventricular (LV) failure, particularly when associated with active infective endocarditis (IE), is of the utmost importance. From July 1982 to May 1984, 34 patients, aged 15 to 60 years, with severe aortic regurgitation underwent immediate (within 24 hours of diagnosis) aortic valve surgery. All patients were in New York Heart Association class IV for LV failure. Eighteen patients had right-sided heart failure. Decision for immediate surgery was based on the echocardiographic demonstration of diastolic closure of the mitral valve or of vegetations on the aortic valve. Premature closure of the mitral valve was demonstrated echocardiographically in 17 patients, 13 of whom had diastolic crossover of LV and left atrial pressure tracings recorded at surgery. IE of the aortic valve was confirmed at surgery in 29 patients, 27 of whom had vegetations on echocardiography. Seven patients required replacement of both aortic and mitral valves. Antibiotic therapy for IE was started immediately after blood cultures were taken and continued for 4 to 6 weeks postoperatively. The mortality rate within 30 days of surgery was 6% for the group as a whole and 7% for those with IE. Mean follow-up period for the 32 survivors was 10.6 months. There were 2 late deaths. No patient had periprosthetic regurgitation or persistence of endocarditis. Procrastination in referral for surgery of these extremely ill patients is not justified and is likely to be associated with higher risks of morbidity and mortality.

The findings in four young patients with the billowing mitral leaflet syndrome who presented with evidence of acute myocardial infarction are reported. Because technically adequate coronary arteriograms demonstrated patent vessels and the electrocardiograms initially showed pronounced elevation of the ST segments as occurs in Prinzmetal's angina, it is postulated that spasm of normal coronary arteries was the operative factor. Scrutiny of those cases of clinically apparent ischemic heart disease with normal coronary angiograms is suggested to establish whether there is in fact a causal relationship with the billowing mitral leaflet syndrome.

A survey to determine the prevalence of rheumatic heart disease (R.H.D.) in Black children was conducted in the creeches and primary schools of the South Western Townships of Johannesburg (Soweto). A total of 12 050 Black children were examined by 10 cardiologists in May to October 1972. The overal prevalence rate of R.H.D. was 6.9 per 1000, with a peak rate of 19.2 per 1000 in children of the seventh school grade. The maximal age incidence was 15-18 years and there was a female preponderance of 1 6:1. A rise in prevalence occurred with increasing family size. Most children (92%) were asymptomatic, and in 82.5% R.H.D. was diagnosed for the first time during the school survey. The commonest valve lesion was mitral regurgitation, which was present in 93% and occurred as an isolated lesion in 47.5%. Lancefield's group A beta-haemolytic streptococcus was isolated from the throats of 52 per 1000 Soweto children. The auscultatory features of a non-ejection systolic click and late systolic murmur were prevalent (13.9 per 1000) and had several epidemiological factors in common with R.H.D. A comprehensive preventative campaign is urgently needed in South Africa, directed at both primary and secondary prophylaxis of R.H.D. The socioeconomic status of the community must be improved if optimal prevention is to be achieved.

Fifty patients in severe congestive heart failure (CHF) were treated with captopril (Capoten; Squibb), an oral angiotensin-converting enzyme inhibitor, over a 2-year period (range 3-24 months, mean 8,6 +/- 7,7 months). At entry, all patients were in New York Heart Association (NYHA) functional class IV despite high-dose diuretic and conventional vasodilator therapy. The overall cumulative survival at 6 and 12 months was 64% and 53% respectively. There were 22 deaths (18 during captopril therapy) including 8 sudden deaths. At 2-year follow-up (mean 14,6 +/- 6,9 months), there were 25 survivors on captopril; 18 in NYHA class I or IIS and 7 in class IIM or III. Diuretic requirements were decreased considerably in all. Side-effects were common but transient and in no case did captopril have to be withdrawn. We confirm our earlier conclusion that captopril has long-term beneficial effects and is a highly effective drug in the treatment of patients with CHF refractory to currently accepted therapy. Sudden death despite satisfactory clinical improvement continues to cause concern. Precautions which may reduce or avoid these are briefly discussed.

Thirteen patients developed syncope and a prolonged QTc interval while taking therapeutic doses of sotalol. Polymorphous ventricular tachycardia was observed in 12 patients, and criteria typical of torsade de pointes were present in 10. In 12 patients sotalol had been given with hydrochlorothiazide in a combined preparation, Sotazide, but with inadequate or no potassium supplementation. Serum potassium concentrations were reduced in eight patients. Four patients were taking other drugs known to prolong the QT interval, including disopyramide (three patients) and tricyclic antidepressants (two patients). The QT interval returned to normal in all patients after withdrawal of the drugs and correction of the hypokalaemia. Thus even in low dosage sotalol may be hazardous in the presence of hypokalaemia or when combined with drugs that also prolong the QT interval. The use of sotalol concurrently with potassium losing diuretics, such as the combined preparation Sotazide, may expose the patient to unnecessary risk and should be avoided unless the class III antiarrhythmic action of this unique beta adrenoreceptor blocking agent is also required.

Acute severe aortic regurgitation (AR) is characterized by a steep increase in left ventricular end-diastolic pressure, exceeding left atrial pressure and occasionally equilibrating with aortic diastolic pressure. These pressure phenomena correlate with the M-mode echocardiographic (echo) findings of diastolic closure of the mitral valve (DCMV) and diastolic opening of the aortic valve (DOAV). Six men, aged 23 to 48 years, with recent-onset, severe AR were evaluated by M-mode echo and pressures at cardiac catheterization. DCMV was seen in all 6 patients and DOAV in 4. Near-constant time intervals from the preceding R wave of the electrocardiogram to DCMV and DOAV were seen, even in 3 patients with varying RR intervals (1 with atrial fibrillation and 2 with asynchronous and demand atrial pacing). When the RR intervals were shorter than these intervals, DCMV and DOAV did not occur. Increasing the heart rate in the 2 patients by atrial pacing resulted in a marked decrease in left ventricular end-diastolic pressure. In conclusion, DCMV and DOAV have a near constant relation to the preceding R wave of the electrocardiogram, DCMV with competence of the mitral valve must be present for DOAV to occur, DOAV indicates that the diastolic blood pressure equals left ventricular end-diastolic pressure, and heart rate is an important factor influencing the hemodynamic and echocardiographic parameters in recent-onset, severe AR.

Ten patients with the billowing mitral leaflet syndrome complicated by infective endocarditis are reported. Two patients had a non-ejection systolic click and 8 had both a non-ejection systolic click and a late systolic murmur. These auscultatory features were difficult to detect in 4 instances in that they were intermittent, soft, or brought out only with postural change. Seven patients were unaware of their cardiac lesions. A low grade pyrexia was present in all 10 patients. Four patients presented with clinical features caused by reversible neurological lesions. Blood cultures were positive in all patients, with Staphylococcus albus the infecting organism in 6. Antibiotic therapy was successful with significant mitral regurgitation supervening in only one instance. The importance of the billowing leaflet as a potential site of infective endocarditis is emphasized. It seems that antibiotic prophylaxis is indicated at times of increased risk of infection in subjects with a non-ejection systolic click or a late systolic murmur.

Normal auscultatory findings were studied during a heart survey in which 12 050 Black schoolchildren, aged 2 to 18 years, were examined by cardiologists. Physiological third heart sounds were detected in 96 per cent of children, innocent systolic murmurs in 72 per cent, and innocent mid-diastolic murmurs in 0.27 per cent. The term 'innocent systolic murmur" was used for vibratory systolic murmurs (70%) and pulmonary ejection systolic murmurs (4.2%) but distinct separation of these two murmurs was often difficult. Vibratory systolic murmurs were present throughout the age range. Important features in differentiating innocent systolic murmurs from those caused by mild organic heart disease included the intonation, site of maximal intensity, timing in systole, and behaviour with postural change. Innocent mid-diastolic murmurs are short murmurs occurring immediately after the third heart sound in children, with no supportive evidence of organic heart disease.

Nonejection clicks and associated mitral systolic murmurs are common in routine cardiologic practice and can result from multiple etiologic factors affecting the complex mitral valve mechanism. Such factors include a specific syndrome the essential feature of which is that the mitral leaflets or part thereof, primarily the posterior one, are voluminous. The syndrome has stimulated widespread interest and study during the last decade and various descriptive terms, including the "billowing mitral leaflet syndrome" (BMLS), have been applied to it. A familial occurrence of the BMLS may be detected and symptoms include chest pain, palpitations, syncope, and anxiety. Arrhythmias, conduction defects, and ECG abnormalities which mimic occlusive coronary artery disease are important features which remain ill understood. It is suggested that there is a possible relationship between the so-called "athlete's heart" and the BMLS. We also postulate that the entity of acute myocardial infarction without demonstrable occlusive coronary artery disease is, in at least some instances, a complication of the BMLS-possibly on the basis of coronary spasm. More severe mitral regurgitation, infective endocarditis, or, rarely, sudden death may supervene in the BMLS but we conclude, from published data and our own experience, that the prognosis is generally good.

The syndrome of primary mitral leaflet billow, with or without prolapse, is associated with myxomatous degeneration of the mitral valve apparatus, mainly the posterior leaflet, and the syndrome may be familial. It manifests clinically with an isolated nonejection systolic click (billow), a murmur of mitral regurgitation that is usually late systolic (prolapse), or a combination of murmur and click. Echocardiography identifies and assesses the extent of the billowing of mitral leaflet bodies but there are no specific echocardiographic criteria that can differentiate normal from pathological billowing. Similarly, a prolapsed leaflet is not detected echocardiographically when there is localized and mild failure of leaflet edge apposition but a more severely prolapsed, or flail, leaflet can be demonstrated and confirmed by that technique. Symptoms of the syndrome include anxiety, chest pain and palpitations. The resting electrocardiogram may show ST segment and T wave abnormalities. The majority of patients have a benign course and require reassurance only. Complications include systemic emboli, infective endocarditis, progression to severe mitral regurgitation, arrhythmias and, rarely, sudden death. Patients with prolapse of a leaflet edge are more likely to develop complications than those with only billowing of the leaflet bodies. Surgery, preferably valvuloplasty, is required for severe regurgitation and may also be indicated for potentially lethal tachyarrhythmias unresponsive to medical therapy. Mitral leaflet billow and prolapse may be secondary to, or associated with, many conditions. The prognosis is then principally that of the underlying disease of which ischemic heart disease and hypertrophic cardiomyopathy are the most important.

A patient with congenital complete absence of the left pericardium had a late systolic murmur and an intermittent non-ejection systolic click. A post-exercise ECG showed ST-segment depression and T-wave inversion, compatible with that described in the billowing mitral leaflet syndrome. Left ventricular cine-angiography confirmed prolapse of the mitral valve. It is suggested that an associated billowing mitral leaflet syndrome may be responsible for chest pain, variable auscultatory features and abnormal ECGs in some patients with absent left pericardium.

The auscultatory signs in 90 subjects with hypertrophic obstructive cardiomyopathy are described. The late-onset ejection systolic murmur and its responses to vaso-active manoeuvres reflect a volume-dependent outflow tract obstruction. Late vibrations of the systolic murmur, not uncommonly recorded at the apex, are due to associated mitral incompetence. Non-ejection systolic clicks may occur, and the likely explanation is inequality of the functional length of the mitral chordae tendineae secondary to asymmetrical myocardial hypertrophy. The second heart sound is often abnormal, usually with delay in the aortic component. Some correlation was demonstrated between the relative degrees of left and right ventricular outflow obstruction and the pattern of splitting of the second heart sound. Reversed or partially reversed splitting is usually associated with a more severe left ventricular outflow obstruction. Ejection systolic clicks and early diastolic murmurs occur infrequently, but are not incompatible with the diagnosis of hypertrophic obstructive cardiomyopathy.

A survey was conducted on 12 050 Black schoolchildren, aged 2 to 18 years, in the South Western Townships of Johannesburg (Soweto), and the prevalence of non-ejection systolic clicks and late systolic murmurs was determined. One or both of these auscultatory findings were detected in 168 children, yielding a prevalence rate of 13-99 per 1000 in the school population. A female preponderance of 1-9:1 was present and there was a strong linear increase in prevalence with age, with a peak rate of 29-41 per 1000 in 17-year-old children. A non-ejection click was the only abnormal auscultatory finding in 123 children (73%) and a mitral systolic murmur in 8 (5%), whereas in 37 (22%) both these findings were present. Of the latter 37 children, the murmur was late systolic in 32; in 5 it was early systolic. Auscultation in different postures was important in the detection of both non-ejection clicks and mitral systolic murmurs. Experience in the detection of these auscultatory findings influenced the frequency with which they were heard. Electrocardiographic abnormalities compatible with those previously described in the billowing mitral leaflet syndrome were present in 11 of 158 children. The aetiology of these auscultatory findings in this community remains unknown. In the same survey, a high prevalence rate of rheumatic heart disease was recorded and the epidemiology of the non-ejection clicks and these mitral systolic murmurs showed similarties to that of rheumatic heart disease. Though the specific billowing mitral leaflet syndrome almost certainly accounts for some of these auscultatory findings, a significant proportion may have early rheumatic heart disease. Further elucidation of this problem is necessary.