https://www.amjmed.com/article/S0002-9343(11)00266-X/fulltext
A 35-year-old woman was referred to Accident and Emergency for an emergency opinion. She had developed ankle swelling 3 days before referral and had noted a marked decline in exercise tolerance and dyspnea for 2 weeks before referral. Her medical history included a single pregnancy and normal delivery 7 years previously, and a more recent diagnosis of inflammatory bowel disease.
She had consulted her general practitioner 2 years previously with a story of general ill health, principally mild fatigue, intermittent altered bowel habit, and occasional arthralgia. At this time, she was noted to have a microcytic anemia, increased platelet count, and increased C-reactive protein (
Table 1Table 1). In view of these findings and the history of altered bowel habit, she was referred for specialist gastroenterologic consultation. At this time, abdominal and other examination results were said to be normal, but the history and abnormal test results suggested the possibility of underlying inflammatory bowel disease, and a small bowel enema was performed. This was normal, and a colonoscopy was then performed that showed a few superficial ulcers in the rectum. Biopsies from the area showed mild edema only, with no features of Crohn's disease or ulcerative colitis. In view of the ongoing vague symptoms and deranged inflammatory markers, she underwent a trial of topical rectal steroids with no symptomatic or laboratory improvement. A referral was made to a gynecologist at this point, who confirmed normal pelvic organs, and an endometrial biopsy was recorded as being normal.
Table 1Inflammatory Markers During the Course of Clinical Supervision: Surgery Occurred in December 2009
| Date | 01/10/07 | 01/04/08 | 01/10/08 | 01/05/09 | 01/11/09 | 01/02/10 |
|---|
| C-reactive protein mg/L | 96 | 84 | 92 | 78 | 101 | <4 |
| Platelets | 524 | 520 | 496 | 477 | 561 | 233 |
| Plasma viscosity | 1.88 | 1.91 | 1.94 | 2.01 | 1.94 | 1.65 |
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She was reviewed 6 months before her acute presentation, at which time finger clubbing was noted; inflammatory markers continued to be increased along with a decrease in her serum albumin concentration (
Table 1Table 1). She was commenced on a 5-aminosalicylic acid derivative with some subjective improvement in her altered bowel habit but no improvement in her serum inflammatory markers.
At the time of her acute presentation, she was dyspneic at rest with a resting heart rate of 120 beats/min, and finger clubbing was present. She described dyspnea that varied, being worse supine or erect at different times. Blood pressure was 106/68 mm Hg, and her jugular venous pressure was elevated to 10 cm with prominent “v” waves. She had clinical evidence of cardiomegaly and a loud pansystolic murmur accompanied by a prominent “tumor plop.” Her chest was clear to auscultation, but she had peripheral edema. Urinalysis was clear, and her previous blood abnormalities persisted (
Table 1Table 1). In view of the clinical suspicion of a left atrial myxoma, transthoracic echocardiography was undertaken that confirmed the presence of a large pedunculated mass in the left atrium attached to the atrial septum (
Figure 1,
Figure 2). This was removed at surgery via a left atrial approach, and a septal defect closure was performed. She made a prompt recovery from surgery.
Figure 1
Standard 2-dimensional transthoracic long-axis parasternal view demonstrating a large left atrial myxoma prolapsing into the mitral valve apparatus.
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Figure 2
Standard 2-dimensional transthoracic apical 4-chamber view demonstrating a the left atrial myxoma within and almost completely filling the left atrium.
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At 6 months after surgery, her exercise tolerance is 2 miles on flat ground, and her inflammatory markers have become normal. The finger clubbing has resolved, and she has had no further bowel upset.
Cardiac tumors are uncommon with an incidence of 0.02% at autopsy.
1x1Reynen, K. Frequency of primary tumors of the heart. Am J Cardiol. 1996; 77: 107
Abstract |
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Google ScholarSee all References Atrial myxomas account for approximately half of all cardiac tumors in adults, the majority (>90%) arising in the left atrium. The clinical features of left atrial myxomas can be divided into those related to the presence of a left atrial mass, that is, obstruction to transmitral flow, embolization, and systemic features similar to those seen in systemic vasculitis or endocarditis.
2x2Shapiro, L.M. Cardiac tumours: diagnosis and management. Heart. 2001; 85: 218–222
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Patients with a left atrial myxoma typically will present with dyspnea, hemoptysis, or features of distant embolization,
3x3Leach, W.J. and Pollock, J.G. Saddle embolus as first presentation of left atrial myxoma in a 14-year-old girl. Scott Med J. 1988; 33: 340–341
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Google ScholarSee all References but fever, arthralgia, finger clubbing, and increased inflammatory markers also are typically found.
4x4Goodwin, J.F. Diagnosis of left atrial myxoma. Lancet. 1963; i: 464
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In this case, diagnostic confusion arose because cardiac manifestations of the left atrial myxoma were only apparent late in the course of her symptoms, whereas the systemic effects were prominent early on. The association of altered bowel habit, with increased inflammatory markers and finger clubbing, is common in the context of inflammatory bowel disease, and thus in this case apparently explicable.
However, fatal embolization from atrial myxoma can occur, as can long-term pulmonary hypertension and right ventricular failure, and it is important for clinicians to question the possibility of a second diagnosis if there are clear signs that the initial diagnosis, although plausible, does not respond to apparently adequate and appropriate therapy.
Finger clubbing is common, and the list of causes is not endless and can be easily remembered, particularly if it is learned by system (
Table 2Table 2).
Table 2Common Causes of Finger Clubbing Subdivided According to the System with the Underlying Disorder
| System | Potential Causes |
|---|
| Respiratory |
- Lung fibrosis
- Suppurative lung disease:
- Bronchiectasis
- Lung abscess
- Empyema
- Pulmonary tuberculosis
- Lung cancer (rarely small cell)
- Mesothelioma
- Pulmonary arteriovenous malformation
- Thymoma
|
| Cardiovascular |
- Subacute bacterial endocarditis
- Left atrial myxoma
- Cyanotic congenital heart disease
- Thoracic aortic aneurysm
|
| Gastrointestinal |
- Malabsorption, celiac disease
- Inflammatory bowel disease:
- Crohn's disease and ulcerative colitis
- Hepatic cirrhosis, especially primary biliary cirrhosis
- Esophageal cancer
|
| Other |
- Thyroid acropachy (mimics clubbing in patients with Graves' disease)
- Genetic
- Trauma (may be unilateral or spare some nails)
- Thalassemia
|
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In this case, application of the “cardiac causes of clubbing” immediately narrowed the diagnosis down to left atrial myxoma or subacute bacterial endocarditis, and the diagnosis was instantly confirmed by echocardiography. Successful treatment led to a satisfactory outcome, but the case might have ended disastrously if the diagnosis had been missed at this time, which highlights the vital roles played by a medical history, physical examination, and knowledge about the fingertips.
https://www.amjmed.com/article/S0002-9343(11)00266-X/fulltext
