The heart can also grow octopus, what is this operation?
An octopus grows on the heart?
The wonderful case was published in the top journal EHJ by the team of Professor Zhang Fengchun of Peking Union Medical College, a story about the growth of "octopus" on the heart.
Case profile
The patient, a 46-year-old woman, presents for more than 1 year of weight loss, fatigue, and exertional chest pain, accompanied by left upper extremity weakness and intermittent vertigo. Traces family history to the exclusion of smoking and other chronic diseases.
Laboratory tests suggest elevated highly sensitive C-reactive protein (CRP) (14.05 mg/L), moderately elevated erythrocyte sedimentation rate (ESR) (43 mm/h), and negative troponin (TnI) and autoimmune antibodies. Electrocardiogram (ECG) shows extensive depression of the ST segment and changes in T waves.
ECG shows that the ST band is widely depressed and the T waves change
Let's start with a brief analysis of the patient's complaints and the results of the existing tests.
Symptoms and ECG presentation suggest chronic and extensive myocardial ischemia and possible circulatory hypoperfusion, elevated erythrocyte sedimentation rate and CRP suggest that the cause may be associated with inflammation, and low markers of myocardial injury temporarily exclude myocardial injury or infarction.
Prolonged exhaustion and weight loss suggest cachexia or the possibility of chronic wasting disease.
Weakness and intermittent vertigo of the left upper extremity indicate inadequate systemic perfusion, and asymmetrical upper extremity symptoms are clues that cannot be ignored.
Finally, a negative autoantibody may be helpful for subsequent differential diagnosis.
The attending physician then decided to give the woman a CT angiography (CTA) to assess the coronary condition.
CTA shows multiple coronary aneurysms of the left main trunk (LM), left anterior descending branch (LAD), spiral branch (CX) and right coronary artery (RCA) with stenosis [Figure B-D], expansion of the ascending aorta and formation of left and right intercornal sinus aneurysms [Figure D arrow]
CTA this sweep, even looks a little scary... CTA suggests multiple coronary aneurysms, irregular dilations of the ascending aorta and three branches of the coronary artery, almost like an octopus lying on the patient's heart, and its muscles are particularly developed.
In the modeled image, the octopus legs are lying on their stomachs, section by section, the coronary arteries have undergone extensive dilation, which affects hemodynamics, the myocardial blood flow isperfusion leads to ischemia, and the coronary sinus aneurysm may further aggravate myocardial ischemia, which can explain the patient's chest pain and ECG performance.
The octopus head is also very large, and the ascending aorta has expanded, resulting in a decrease in systolic blood pressure due to a decrease in the elasticity and compliance of the ascending aorta, and a deficiency in systemic perfusion, which seems to be enough to explain the transient ischemic manifestations of the patient's brain.
However, the asymmetrical symptoms of the left upper limb are still very unnatural, why only affect the left hand, but the right hand is safe and sound?
Time to catch an octopus: Where did the "octopus" come from?
Diagnosis here, let's repeat the disk. The patient, a female, in her early 40s, has been presenting for more than 1 year of weight loss, fatigue and exertional chest pain, with left upper extremity weakness and intermittent vertigo. Coronary and ascending aorta form aneurysms and dilate widely, elevated erythrocyte sedimentation rate and CRP suggest an etiology associated with inflammation, and prolonged fatigue and weight loss suggest cachexia or chronic wasting disease.
To sum up: 40-year-old Chinese woman + subacute onset + elevated ESR/CRP + weight loss + extensive coronary and ascending aortic involvement + simply reduced systolic blood pressure can not explain the asymmetrical symptoms of the left upper extremity, see here, what is your diagnosis?
Let's think about it first
A. Giant cell arteritis, common in older white women (almost all > 50 years of age), typically large-vesselritis, most commonly involving the temporal arteries, with tenderness, decreased pulsation, or nodules, resulting in headache and movement disorders of the masseter and tongue muscles. The patient is a middle-aged woman, the lesion does not involve the temporal artery and the onset is before the age of 50 years, so it is not considered.
B. Behcet's disease, common in the Middle East and Asia young adults, involves a wide range of blood vessels, mainly to erode arterioles, small veins and microvascular, clinical typical manifestations of eye-mouth-genital triple, that is, recurrent oral ulcers, ophthalmic pigmentitis and genital ulcers, patients are mainly large vascular symptoms, so they are not considered.
C. Kawasaki disease, common in Asian children, mostly male, belongs to moderate large vasculitis, often involves coronary arteries, fever of more than 5 days and more than 4 mucosal manifestations and or evidence of coronary involvement can be diagnosed. In middle-aged women, although coronary involvement, mucosal manifestations and fever are absent, so they are excluded.
D. Aorticitis, known as "Oriental Beauty Disease", is common in Asian women, typical of macrovascularitis, often involving the aorta and its branches to form occlusion, resulting in differences in blood pressure in the extremities or pulselessness. The patient is a middle-aged Asian woman with coronary and ascending aorta involvement, extensive dilation and occlusion formation, with left upper extremity weakness and intermittent vertigo, Bingo~
To confirm the diagnosis, the attending physician continued to arrange cardiac magnetic resonance and delayed gadolinium-enhanced imaging (LGE-MRI) and positron emission tomography-computed tomography (PET/CT), which showed a mild decrease in the patient's left ventricular ejection fraction (54%), a weakening of the overall movement of the ventricular wall, and multiple thrombosis in the aneurysm (figure E arrow), LGE-MRI showing a delayed gadolinium imaging enhancement signal in the coronary artery wall (figure F, black arrow), and subcapital (figure F, white tip), Suggests fibrosis due to inflammation of the blood vessels. 18-FDG-PET/CT also showed high uptake of L, thoracic aorta, LM, and LAD, further supporting the diagnosis of aorticitis (Takayasu).
The octopus has finally caught, the next step should be charcoal grilled or teppanyaki, how to treat it?
The main treatment for Takayasu is glucocorticoids. The usual initial dose is prednisone at a daily dose of 45-60 mg, while monitoring the dynamics of inflammatory indicators (CRP and ESR), the dose can be gradually reduced with the decline of the inflammatory indicator, and if the disease worsens, the dose should be increased. There are currently ITAS 2010 scores available to help clinicians adjust medications [1].
In addition to glucocorticoids, immunosuppressants such as methotrexate, leflunomide, and mycophenolates have also been reported for the treatment of Takayasu. It can be used as an alternative when glucocorticoids are not sensitive. Once the disease progresses, irreversible arterial stenosis or large aneurysm develops, surgical intervention is required if necessary.
Takayasu also has a relatively good prognosis, with five-year survival shown to be 80 to 90 percent [2,3].
Patients eventually received a triptych of warfarin, clopidogrel, and aspirin, combined with immunosuppressants for vasculitis. There were no adverse events at the follow-up for 3 years [4].
Author summary
The causes of coronary artery aneurysms are very diverse, and it is often difficult to identify the specific cause when diagnosing the aneurysm itself.
In clinical work, as a specialist, it is more necessary to have a careful observation of symptoms, and not to stare at the single symptom of coronary involvement. Imagine if the attending physician had omitted the patient's left upper extremity weakness and elevated inflammatory indicators, perhaps the octopus that was dormant on the patient's heart would have come from nowhere and where it was going.
bibliography:
[1] Indian Rheumatology Vasculitis (IRAVAS) group, et al. Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010). Rheumatology (Oxford). 2013 Oct;52(10):1795-801. Epub 2013 Apr 16.
[2] Hall S, et al. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore). 1985;64(2):89.
[3] Ishikawa K. Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation. 1978;57(1):27.
[4] Peng L, et al. The octopus on a heart. Eur Heart J. 2019 Feb 14;40(7):635.
This article was first published: Cardiovascular Channel of the Medical Profession
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