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Cardiac Myxoma

Endocardium Myxoma

Cardiac myxomas are benign soft tissue tumors occurring more frequently in women than in men. Although they can be present at any age, they are most commonly seen in the 30 to 60 year age group. They can be asymptomatic or can cause an embolism, obstruction within the heart or other constitutional symptoms.


Presentation

Cardiac myxomas are the commonest amongst benign tumors of the heart [1]. Although they have been reported in individuals of all ages, they are usually seen between the third and the sixth decade of life. Sporadic myxomas occur more commonly in women and the female to male ratio has been reported to be 2.7:1 [2]. A majority of the myxomas occur on the left side of the heart while 25% occur on the right side [3].

A small proportion of the cardiac myxomas are familial in origin [4] and can either be part of a syndrome (Carney's complex with its subsets LAMB syndrome or NAME syndrome) [5] [6] [7] [8] or can be non-syndromic [9]. The familial Carney's complex is inherited in an autosomal dominant manner and characterized by skin pigmentation, cardiac and soft tissue myxomas, accompanied by endocrine dysfunction. Compared to sporadic myxomas, familial cardiac myxomas have been reported to occur at an earlier age, have less female preponderance and a higher incidence of right heart involvement and recurrence [4].

Affected individuals usually present with symptoms secondary to embolism, mechanical obstruction within the heart or with constitutional symptoms (classic Goodwin's triad). A minority of the patients with myxomas are asymptomatic [10] and they are detected incidentally. The site of the embolization depends on whether the myxoma is located on the right or the left side of the heart and the presence or absence of an intracardiac shunt. Other presenting features include those due to mitral regurgitation and conduction abnormalities like atrial fibrillation or flutter.

Fever of Unknown Origin
  • To the best of our knowledge, the first case of a cardiac myxoma infected with Staphylococcus lugdunensis is reported in a 54-year-old man with fever of unknown origin for 4 months.[ncbi.nlm.nih.gov]
High Fever
  • Abstract A 12-year-old girl with a high fever underwent echocardiography and was found to have a myxoma that arose from the atrial side of the anterior mitral valve leaflet. The tumor was successfully excised.[ncbi.nlm.nih.gov]
Chronic Abdominal Pain
  • We present a case of a 46-year-old man with chronic abdominal pain and discuss a number of diagnostic challenges that were confronted up until a definitive diagnosis of cardiac myxoma was made.[ncbi.nlm.nih.gov]
Acute Abdomen
  • After remaining asymptomatic for 3 weeks, she was readmitted with acute abdomen. Ultrasonography and magnetic resonance angiography detected intra-abdominal hemorrhaging and a saccular aneurysm of the abdominal aorta.[ncbi.nlm.nih.gov]
Heart Disease
  • Provides a clinically based approach to echocardiography, with an in-depth discussion of the main cardiac events seen in practice, including adult congenital heart disease.[books.google.de]
  • ., Braunwald’s heart disease A textbook of Cardiovascular medicine, tenth edition, Saunders, Philadelphia, 2015, 1865-6. In article [6] Kuon, E., Kreplin, M., Weiss, W., Dahm, J.[pubs.sciepub.com]
  • Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 95. Mckenna WJ, Elliott P. Diseases of the myocardium and endocardium. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed.[medlineplus.gov]
  • There was no history of headache, fever, head injury, seizures, or heart disease. He was on no medications. His birth history, growth, and development were normal.[pediatrics.aappublications.org]
  • Cardiac thrombi, which appear more frequently than cardiac myxomas are typically located more often in the LA or LAA and generally occur in patients with organic heart disease.[alliedacademies.org]
Diastolic Murmur
  • Typical examination findings include abnormal heart sounds , such as a rumbling diastolic murmur over the apex or a characteristic “ tumor plop.” The diagnosis is not easily established clinically because of the nonspecific nature of symptoms.[amboss.com]
  • These abnormalities may include a holosystolic murmur of mitral insufficiency, a systolic ejection murmur, a diastolic murmur of mitral stenosis, a loud S1 or S2, an S4, or a tumor plop [ 1 , 10 , 12 , 13 ] .[pubs.sciepub.com]
  • In cases where the tumour remains in the atrium throughout the cardiac cycle, a diastolic murmur and pressure tracings practically indistinguishable from those of mitral stenosis will likely be present.[atlasgeneticsoncology.org]
  • A tumor plop may be followed by a low pitched diastolic murmur. If the mitral valve inflow obstruction is significant enough, physical exam findings of congestive heart failure will be present. Treatment Treatment is universally surgical excision.[healio.com]
  • Also heard were an early diastolic heart sound ( tumour plop) and a mid-diastolic murmur at the apex. An ECG revealed evidence of left ventricular hypertrophy with repolarization abnormalities.[bjmp.org]
Cardiac Signs and Symptoms
  • Cardiac myxoma produces protean clinical manifestations that do not always include cardiac signs and symptoms. Neurologists may be called on for diagnostic consultation in patients who will prove to have cardiac myxoma.[neurology.org]
  • The tumor is usually benign, and the cardiac signs and symptoms produced are related to the size and location of the tumor. Primary cardiac tumors in children are rare. Myxomas are the third most common tissue type behind rhabdomyomas and fibromas.[pediatrics.aappublications.org]
Fracture
  • Radiographs showed an expansile lytic lesion with pathological fracture involving the neck and proximal shaft of the right femur.[ncbi.nlm.nih.gov]
Leg Pain
  • A 44-year-old man presented to us with abrupt right lower leg pain. A computed tomography angiogram revealed segmental obstruction of the right common femoral artery, and embolectomy was performed.[ncbi.nlm.nih.gov]
Lower Leg Pain
  • A 44-year-old man presented to us with abrupt right lower leg pain. A computed tomography angiogram revealed segmental obstruction of the right common femoral artery, and embolectomy was performed.[ncbi.nlm.nih.gov]
Gerstmann Syndrome
  • We describe a case of a 40-year-old female who presented with neurological signs and symptoms of Gerstmann's syndrome secondary to a left atrial myxoma.[ncbi.nlm.nih.gov]
Aura
  • We present a new case of cardiac myxoma triggering migraine auras. A 52-year-old woman was suffering from migraine attacks with aura. Magnetic resonance imaging (MRI) showed no abnormalities.[ncbi.nlm.nih.gov]

Workup

The workup of the patient depends on their manner of presentation. In all cases, a detailed history and physical examination are mandatory. If familial cardiac myxoma is suspected from family history then a detailed physical examination for other features of the syndrome should be performed.

Laboratory studies are not specific and findings can include hemolytic anemia (secondary to mechanical damage of erythrocytes), elevated levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and serum gamma globulin. Levels of serum interleukin- 6 may be elevated and they are used as a marker for determining recurrence.

Electrocardiography (ECG) is performed in all patients and may show atrial fibrillation, flutter or other conduction abnormalities. Two-dimensional echocardiography (2D ECHO) is used to visualize all the cardiac chambers and to evaluate the location, size, and mobility of the myxoma. Transesophageal echocardiography is a more sensitive test than the 2D ECHO and detects myxomas as small as 1 mm in diameter.

Chest radiographs are used to identify pulmonary edema, tumor calcification, and cardiomegaly while magnetic resonance imaging (MRI) detects tumor size, mobility and its site of attachment. Computed tomography (CT scan) is performed to differentiate between a myxoma and an intracardiac thrombus. While a thrombus is smaller than a myxoma, the myxoma has specific morphological characteristics (shape, mobility and ability to prolapse) which help to identify it [11].

PRKAR1A molecular genetic testing is recommended in all patients suspected to have Carney's complex and if positive, then their family members are also evaluated for cardiac myxomas [12]

Staphylococcus Aureus
  • Blood cultures were positive for methicillin-resistant Staphylococcus aureus. Transthoracic echocardiogram revealed enlarged left atrium filled with tumor with mobile structure protruding into the left ventricle during diastole.[ncbi.nlm.nih.gov]

Treatment

  • Treatment with intravenous recombinant tissue plasminogen activator (rtPA) and intra-arterial thrombolysis has been described previously but mechanical thrombectomy has not yet been reported, and treatment of myxoma-related ischemic stroke remains a clinical[ncbi.nlm.nih.gov]

Prognosis

  • In general, the prognosis of infected cardiac myxoma is relatively benign and the long-term survival is always promising.[ncbi.nlm.nih.gov]

Etiology

  • Abstract Most cases of atrial myxoma are sporadic, and the exact etiology is unknown. We examined if herpes simplex virus (HSV)-1 and HSV-2 antigens and/or DNA could be detected in a cohort of Iranian patients with cardiac myxomas.[ncbi.nlm.nih.gov]
  • Each chapter includes eight to ten gross and microscopic photographs with legends highlighting the pathologic features of the disease, as well as succinct text covering etiology, clinical aspects, and differential diagnosis.[books.google.de]
  • Etiology Clinical features General Clinical features caused directly by the tumor Symptoms caused by obstruction Dyspnea on exertion, paroxysmal nocturnal dyspnea , and/or orthopnea Dizziness or syncope Palpitations Auscultatory findings Low pitched,[amboss.com]
  • Etiology Sporadic – 90%. Familial – 10%. May form part of Carney's complex (Carney's, LAMB, NAME syndrome) or non-syndromic familial cardiac myxomas 10,11 . Pathophysiology Originate from the endocardium.[pie.med.utoronto.ca]
  • Patient was started on anticoagulation with heparin with suspicion of possible apical thrombus however possibility of different etiology of cardiac mass was not entirely excluded.[omicsonline.org]

Epidemiology

  • Epidemiology Most common primary cardiac neoplasm (usually benign) Most common location: left atrium ( 75% of all cases ) Sex: (3:1) Peak incidence : 40–60 years References: [1] [2] [3] Epidemiological data refers to the US, unless otherwise specified[amboss.com]
  • Epidemiology Cardiac myxomas can occur across all age groups, however, they occur with the greatest frequency amongst between the third and sixth decades of life.[atlasgeneticsoncology.org]
  • Epidemiological and pathological characteristics of cardiac tumors: A clinical study of 242 cases. Interact CardiovascThorac Surg 2007; 6: 636-639.[alliedacademies.org]
  • […] mutation was found in exon 2 in the causative gene of Carney complex, protein kinase A regulatory subunit 1 alpha ( PRKAR1A ). [10] At present, there does not appear to be an association between cardiac myxomas and herpes simplex types 1 and 2. [11] Epidemiology[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Originate from the endocardium. Composed of embryonic multipotential mesenchymal cells that persist during cardiac septation which can differentiate into angioblasts, fibroblasts, cartilage and myoblasts.[pie.med.utoronto.ca]
  • Molecular analysis of the cyclic AMP-dependent protein kinase A (PKA) regulatory subunit 1A (PRKAR1A) gene in patients with Carney complex and primary pigmented nodular adrenocortical disease (PPNAD) revels novel mutations and clues for pathophysiology[revespcardiol.org]
  • Pathophysiology Myxomas account for 40-50% of primary cardiac tumors. Approximately 90% are solitary and pedunculated, and 75-85% occur in the left atrial cavity. Up to 25% of cases are found in the right atrium. Most cases are sporadic.[emedicine.medscape.com]

Prevention

  • We describe a multidisciplinary approach applying cerebrovascular stent retriever devices in tumor embolectomy of the renal artery of a young patient with systemic morbidity, preventing safe laparotomy for open surgical tumor embolectomy.[ncbi.nlm.nih.gov]
  • Presently, the sporadic cases of Cardiac Myxoma cannot be prevented. Also, there are no specific methods or guidelines to prevent tumor formation, if it occurs in association with Carney complex, which is a genetic condition.[dovemed.com]
  • This may require emergency surgery to prevent sudden death. [7] See also [ edit ] Myxoma Interleukin 6 Papillary fibroelastoma Rhabdomyomas Cardiac sarcomas References [ edit ] a b c d e Hecht, Sisalee M. (2009-10-27).[en.wikipedia.org]

References

Article

  1. Bulkley BH, Hutchins GM. Atrial myxomas: a fifty-year review. Am Heart J. 1979;97:639–43.
  2. Zheng JJ, Geng XG, Wang HC. et al. Clinical and histopathological analysis of 66 cases with cardiac myxoma. Asian Pac J Cancer Prev. 2013;14(3):1743-6.
  3. Nath M P, Singh B, Chakrabarty A. Left atrial myxoma presenting as stroke: Case report and review of literature. The Indian Anaesthetists’ Forum 2011;1:1-6.
  4. Farah MG. Familial cardiac myxoma. A study of relatives of patients with myxoma. Chest 1994;105:65–8.
  5. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 1985;64:270–83.
  6. Carney JA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990;14:206–22.
  7. Yen RS, Allen B, Ott R, Brodsky M. The syndrome of right atrial myxoma, spotty skin pigmentation, and acromegaly. Am Heart J 1992;123:243–4.
  8. Krause S, Adler LN, Reddy PS, Magovern GJ. Intracardiac myxoma in siblings. Chest 1971;60:404–6.
  9. Dandolu BR, Iyer KS, Das B, Venugopal P. Non-syndrome familial atrial myxoma in two generations. J Thorac Cardiovasc Surg 1995;110:872–4.
  10. Ture P, Kurdi M, Hiregoudar S, Hegde G. Anesthetic management of a patient with asymptomatic atrial myxoma for hernia repair. Anaesth, Pain and intensive Care. 2016 Apr-Jun; 20(2): 246 -248
  11. Scheffel H, Baumueller S, Stolzmann P, Leschka S, Plass A, Alkadhi H. Atrial myxomas, and thrombi: comparison of imaging features on CT. AJR Am J Roentgenol. 2009 Mar;192(3):639-45.
  12. Hajj-Chahine J, Jayle C, Houmaida H, Corbi P. Utility of genetic testing in multisite myxoma to rule out Carney complex. Interact Cardiovasc Thorac Surg. 2011 Apr;12(4):624.

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Last updated: 2019-06-28 12:30