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April 11, 2021
Rheumatic Heart Disease Carey Franklin Coombs

Carey Franklin Coombs (1879-1932)

Rheumatic heart disease. New York, William Wood, 1924.

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Carey Franklin Coombs was a British physician with extensive clinical experience in the management of patients with rheumatic fever and its cardiac manifestations. He published the first comprehensive textbook on rheumatic heart disease in English language. The book is divided into 11 chapters with sections on etiology, physical signs and diagnosis, histologic studies of the heart, prognosis, treatment and prevention.

As previously mentioned, the etiology of rheumatic fever was a topic of controversy for several decades.  Coombs, however, agreed with Poynton and Paine who were the first to isolate the "diplococcus rheumaticus" as the causative agent of this disease. In 1926, Coombs and Poynton published an article supporting the streptococcus hypothesis. In their conclusion, they wrote "from work done since 1901, there is no rival to the streptococcic theory of acute rheumatism."

Regarding the general manifestations of acute rheumatism and its cardiac involvement, Coombs wrote:
"...Perhaps it would be more accurate  still to say that there is an infection which attacks sometimes the heart, sometimes the joints , sometimes the brain, and occasionally the subcutaneous tissue; that any one of these phenomena may be present by itself, or in combination with any one or more of the other three, and that the true criterion of the 'rheumatic' origin of any carditis is its conformation to a certain clinical and pathological type, rather than its association with any of the other phenomena alluded to above.

The chief characteristics of this kind of disease are: (1) It is a pancarditis incriminating the valves, the muscles, and the pericardium; (2) The type of inflammatory reaction is predominantly productive; (3) Its course in its earlier phase is one of recurring activity.  Each active period, though transient, leaves something permanent.  The active period injures the cardiac muscle pre-eminently, but the permanent lesions are those of the serous structures, and are of importance by virtue of the mechanical embarrassment which they inflict upon the working of the heart."

He also noted "...the joint lesions attract immediate attention by the pain which they cause; the movements of chorea are not likely to escape notice, but is the cardiac lesion which shorten life."

In this work, Coombs reported his experience in a series of 97 patients with rheumatic valve disease. He confirmed previous studies and showed that the left-sided valves were significantly more involved than the right-sided valves. In this study, mitral valve disease was noted in 100% of patients. Aortic and tricuspid valve lesions were noted in 59% and 36% of cases respectively. He described precisely the valvular lesions in the acute phase of the disease and noted annular dilatation of the atrioventricular valves as a very common findings. He also described in detail the histologic lesions observed during the acute phase: "The valve is swollen, there is a productive reaction centering on two [rheumatic] nodules, the surface endothelium is proliferating and necrotic, and on the necrotic area is a cap of fibrin."

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During the chronic phase of the disease he wrote:

"Of the permanent results of rheumatic endocarditis the mitral lesions are the most frequent and characteristics. It is as correct to regard mitral stenosis as being always rheumatic as it is to speak of tabes dorsalis as being without exception due to syphilis."

He also described valvular lesions such as commissural fusion, leaflets and chordal thickening and shortening very precisely and in detail. Finally he reported histological analysis of chronic valvular lesions including the formation of dense fibrous tissue and nodular calcifications.

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Coombs also mentioned that patients affected with rheumatic mitral valve disease were at risk of infective endocarditis. In that regard, he noted: "The valve which has been injured by a previous rheumatic infection is thereby laid more open to successful attack by some infective agent encountering it subsequently."

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Regarding the hemodynamic consequences of valvular lesions, Coombs correctly recognized the prognostic factor of left ventricular function in patients with mitral regurgitation.

The following brief quotation from the section on "disturbance of valvular function by rheumatic infection" is of great interest: 

"Post-rheumatic sclerosis of the valve limits cardiac efficiency when it produces:

1) Obstruction with or without constriction at the auriculo-ventricular openings, here the immediate stress is borne by the corresponding auricle: 

2) Incompetence with or without obstruction at the arterial orifices; in these it is the corresponding ventricle that bears the burden.  Actually the right heart suffers but little in this way, but the lesions of the left-sided valves are almost wholly responsible for the final breakdown in patients who have survived the acute stages of the disease."

Coombs also recognized the high incidence of arrhythmia in patients with rheumatic valve disease particularly paroxysmal tachycardia, atrial flutter and atrial fibrillation. He also understood that the distension of the left atrium was associated with blood stasis and thrombus formation which was at the origin of peripheral embolization.

Another point of interest in this work is Coombs' view on the surgical treatment of mitral stenosis. In 1923, Cutler and Levine reported the first successful case of mitral valvulotomy. Coombs in his monograph, published in 1924, made the following comments:

"Surgical treatment of valvular deformities is unknown except for the case recently recorded by Cutler and Levine of a patient with mitral stenosis.  It is not long enough since this was done to judge of results, but it is safe to say that the operation can never become a general method of treatment for a disease of which the mitral lesion is only one feature-to say nothing of the technical difficulties attending surgical approach to such a structure, or of the probability that the cicatrizing process responsible for the stenosis is still active and capable or reinstating the condition which the operation is supposed to destroy."

Coombs' opinion was shared by the majority of his contemporaries. The historical view on the surgical treatment of mitral stenosis during the early decades of the 20th century is further discussed in the Mitral Valve Surgery section."


Frederick John Poynton Alvin F Coburn