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A case report of embolic retinal artery occlusion: the lost measurement of healthcare quality.


Rheumatic heart disease is the most common cause of acquired heart disease in developing countries. In sub-Saharan Africa, rheumatic heart disease is a major cause of disability, morbidity, and premature death; with a reported prevalence of 5.7 per 1,000. (1) On the other hand, rheumatic heart disease is uncommonly seen in western developed countries, though occasional outbreaks of rheumatic heart disease have been reported and are thought to be secondary to immigration from area with high prevalence of the disease or due to emergence of virulent streptococcal strains. (2) Systemic embolism is occasionally a complication and initial presentation of mitral stenosis from rheumatic heart disease. (3)

In this article, we report an unusual presentation of rheumatic heart disease in a patient who was born and lived all of her life in Louisiana.


A 43-year-old woman, with no known past medical history of medical diseases, was referred to investigate the cause of left eye central retinal artery occlusion that manifested as sudden loss of vision a week earlier.

The patient denied pain, trauma, swelling, or prior similar episodes. She denied any neurological, cardiac, or constitutional symptoms. There was no history of arthritis or rashes. The patient was born and had been living in an inner city of Louisiana. Her past medical history was only significant for six pregnancies (four live births and two miscarriages); her last delivery was two years earlier by a cesarean section. She had history of 10 pack-years of smoking, moderate alcohol intake, and occasional marijuana use but no intravenous drug use. Family history was irrelevant. Physical examination on admission showed normal vital signs. Head and neck examination was unremarkable.

Cardiac examination revealed an apex in the fifth inter-costal space inside the midclavicular line, a mild parasternal right ventricular heave. Auscultation revealed a rumbling mid-diastolic murmur grade 3/6 over the apex with soft pansyslolic murmur radiating to the axilla, and the pulmonary component of the second sound was accentuated. Chest, abdomen, and complete neurological examinations were normal. There was no cyanosis, clubbing, or lower extremities edema.

Left eye fundus examination showed macular cherry red spot with surrounding retinal edema, arterial attenuation, and optic nerve pallor consistent with central retinal artery occlusion (Figure 1, 2). Fluorescent angiography of the left retina showed vessel attenuation and slightly delayed perfusion of the retinal artery (Figure 3).




Laboratory tests showed normal serum electrolytes, glucose, uric acid, and kidney function tests. Liver function tests were normal, but total protein was 5.9 g/dL and albumin was 2.7 g/dL. Blood picture showed normal total and differential white cell count and platelet count. Her hemoglobin was 10.4 g/dL, but she had normal mean corpuscular volume and hemoglobin concentration. Prothrombin time, partial thromboplastin time, anti-thrombin III, functional protein C%, functional protein S%, activated protein C resistance ratio, cardiolipin immunoglobulin A, cardiolipin immunoglobulin G, cardiolipin immunoglobulin M, and homocysteine were within the normal reference range.

Electrocardiogram showed normal sinus rhythm and no ventricular hypertrophy or ischemia. Chest X-ray showed no acute cardiopulmonary processes. Computed tomography of the head was unremarkable.

Doppler echocardiography showed left atrial dilatation (diameter = 47mm). There were normal left ventricular cavity dimensions, wall thickness, regional movement, and global systolic function. There was mild tricuspid valve regurgitation, with estimated right ventricular systolic pressure of 50 mmHg. Mitral valve and sub-valvular apparatus were calcified, thickened, and severely stenotic; mean trans-mitral pressure gradient was 13 mmHg. There was also severe mitral regurgitation.

Transesophageal echocardiography confirmed the above findings. Left atrial appendage showed no thrombus, and contrast bubble study showed no inter-atrial shunt.

Patient underwent mitral valve replacement surgery; her operative findings were consistent with the echoardiographic findings.


Rheumatic heart disease is an uncommon cause of acquired heart disease in developed countries. Approximately 60% of patients with rheumatic valve disease do not give a history of rheumatic fever or chorea, and approximately 50% of patients with acute rheumatic carditis do not eventually have clinical valvular heart disease. In temperate climates and developed countries, there is usually a long interval (averaging 10 to 20 years) between an episode of rheumatic carditis and the clinical presentation of symptomatic mitral stenosis. The latent period between acute rheumatic fever and symptomatic mitral stenosis is variable and appears to be related to the presence of repeated streptococcal infection. (3)

Systemic embolism is a known complication of mitral stenosis. Atrial fibrillation, increasing age of the patient, increasing left atrial size, and a previous history of embolism are associated with an increased incidence of systemic embolism. However, there is no simple correlation between the incidence of embolism and the degree of mitral stenosis. Indeed, embolism may be the first symptom of mitral stenosis. (3) Approximately, half of all clinically apparent embolism occurs in cerebral vessels; it may affect other arteries, resulting in occlusion of extremity arterial supply, aortic bifurcation, and visceral or coronary arteries. (3) Isolated central retinal artery occlusion is a rare, yet very devastating, complication of mitral stenosis. Few case reports and series, which date back more than 40 years, have been published reporting this rare complication. (4-9) The diagnoses in most of these reports were made clinically by the characteristic clinical findings. The patient in this case report was completely asymptomatic until her presentation with acute central retinal artery occlusion, despite the fact of having six pregnancies and four deliveries in the past. Moreover, although the patient was born and lived all of her life in an inner city in Louisiana, had many encounters with healthcare providers--including multiple hospitalizations and surgical procedures, such as cesarean section--the obvious characteristic auscultatory findings of mitral stenosis were not recognized. This case clearly shows that rheumatic heart disease, though uncommon, is still prevalent among individuals who lived all of their life in the United States and not only among new immigrants.

This case also raises the issue of the importance of physical examination of patients during routine healthcare encounters. The value of annual physical examination for generally healthy subjects without complaints is debated. (10,11) Reviewing this patient's medical records shows that she made many routine visits to healthcare facilities where she received medical care. She was up-to-date on routine immunization (as indicted by the serum level of antibodies to infectious agents, such as mumps and rubella); and she had several Pap smears, mammograms, and fasting lipid panels. Discovery of her problem during her prenatal visits, healthcare encounters, previous admissions, and deliveries would have probably saved her from vision loss in her left eye. The value of physical examination gradually declined in North American preventive task forces, which have moved from advocating annual physical examination to recommending selected preventive services in the context of visits for other reasons such as Papanicolaou test (Pap smear) or screening investigations. (12) On the other hand, the last decade has witnessed the rise of several national healthcare quality measures in both inpatient and outpatient settings. (13,14) Conformance with such measures may be used to rate healthcare quality. (14) None of these guidelines aimed to quantify adequacy of physical examination. Careful auscultation of the heart was not listed as one of the quality measures for preventive services of heart disease. (13) The main caveat in the healthcare quality provided for this patient was the failure to recognize the abnormal physical examination signs indicative of a valvular heart disease, which resulted in a devastating consequence. By current criteria of some national healthcare quality measures, (13) her primary care facility would be adherent to preventive health guidelines, including the recommended "quality measures" for prevention of cardiovascular disease. Occasionally, attention to follow national "quality measures," which may have public visibility, may divert attention from individual patients' problems or population-specific challenges. (15) Possibly, it is time to revisit the issue of importance of an annual physical examination that offers the opportunity to plug some cracks in our healthcare system. (12) In the 21st century, the need for careful attentive physical examination is as much as it was in the year 1861, when the British physician Horace Dobell first advocated the importance of routine physical examination. (16)

In conclusion, this case illustrates that severe rheumatic heart disease still exists in the western developed world, may have devastating complications and result in permanent disability, and that careful physical examination is of paramount importance for adequate healthcare delivery.


The authors are grateful to Dr. Eva Mansi, M.Sc (ophthal), FRCS (Glasg) for helpful comments and guidance with figures and legends.


The views expressed herein are those of the authors and do not reflect the official policy or position of the Department of the Army, Department of Defense, or the U.S. Government. One of the authors is an employee of the U.S. Government. This work was prepared as part of his official duties and, as such, there is no copyright to be transferred.


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(7.) Kulshreshtha OP, Jain MR, Singh MM, et al. Embolism of central retinal artery originating from mitral valves (mitral stenosis) (case report). J All India Ophthalmol Soc. 1969;17:270-2.

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(11.) Charap MH. The periodic health examination: genesis of a myth. Ann Intern Med. 1981;95:733-5.

(12.) Laine C. The annual physical examination: needless ritual or necessary routine? Ann Intern Med. 2002;136:701-3.

(13.) National Healthcare Quality & Disparities Reports; NHQRDRNet. [cited 2011 June 15, 2011]; Report from Agency for healthcare Research and Quality. Available from: nhqrdr/jsp/nhqrdr.jsp#snhere#snhere

(14.) 2011 Physician Quality Reporting System (Physician Quality Reporting) Measures List. In: CMS, editor. Version 5.2 ed; 2011.

(15.) Mansi IA. The effect of financial incentives on hospitals that serve poor patients. Ann Intern Med. 2011;154:370; author reply--1.

(16.) Tidmarsh CJ. Periodical Health Examinations. Can Med Assoc J. 1928;18:697-700.

Michel Tanios, MD, MRCP; Hayam Shaker, MD; Ishak A. Mansi, MD, FACP

Dr. Tanios is with the Louisiana State University Health Sciences Center in Shreveport's Department of Medicine. Dr. Shaker is a Staff Physician at Pardee Hospital and Assistant Professor of Medicine at University of North Carolina at Chapel Hill. Dr. Mansi is Staff Internist at Brooke Army Medical Center in San Antonio, Texas, and Clinical Professor of Medicine at the University of Texas Health Science Center in San Antonio.
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Author:Tanios, Michel; Shaker, Hayam; Mansi, Ishak A.
Publication:The Journal of the Louisiana State Medical Society
Article Type:Case study
Geographic Code:1USA
Date:Mar 1, 2012
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