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Familial Atrial Myxoma


Presentation

  • Abstract Familial atrial myxomas may present as a component of the Carney complex, an autosomal dominant multiple neoplasia and lentiginosis syndrome.[ncbi.nlm.nih.gov]
  • ATRIAL MYXOMA PRESENTING AS STROKE.[journalijar.com]
  • We present a rare case of autosomal dominant benign familial myxoma occurring in three generations of the family operated successfully at our institute.[infona.pl]
  • A bound-in CD-ROM contains all the images from the book, downloadable to PowerPoint presentations.[books.google.com]
  • Atrial myxomas presented at earlier age successively and in more aggressive form in these three generations.[medworm.com]
Pain
  • Physical examination findings included loss of reflexes, pain, and temperature below the T10 level with preservation of proprioception.[medworm.com]
  • A 22-year-old son complained of dyspnoea and chest pain. On evaluation, 5.6   2.6 cm right atrial myxoma attached to the roof of the right atrium was found, and the patient underwent myxoma excision in 2007.[infona.pl]
  • The most common symptoms of atrial myxoma include difficulty breathing, chest pain, a tight feeling in the chest, dizziness, and fainting. The severity of symptoms varies based on the size of the tumor, and smaller tumors are often asymptomatic.[diseaseinfosearch.org]
  • […] fever, fatigue, arthralgias, myalgias, weight loss), Raynaud’s phenomenon, skin rash, clubbing of digits; related to embolism—shortness of breath, orthopnoea, paroxysmal nocturnal dyspnoea, fainting, palpitations, dizziness and syncope, pleuritic chest pain[medical-dictionary.thefreedictionary.com]
Fatigue
  • The tumor is characterized by palpitations, disseminated neuritis, nausea, weight loss, fatigue, dyspnea, fever, and occasional sudden loss of consciousness. It is treated by surgical removal of the tumor.[medical-dictionary.thefreedictionary.com]
  • Constitutional disturbances, such as fatigue, fever, erythematous rash, arthralgia, myalgia, and weight loss, and laboratory abnormalities, such as anemia and elevations in the erythrocyte sedimentation rate and the serum C-reactive protein and globulin[histopathology-india.net]
  • Symptoms of right-sided heart failure Patients experience fatigue and peripheral edema. Abdominal distension due to ascites is rare; however, it is more common in slowly growing right-sided tumors.[odlarmed.com]
  • .  The classic clinical presentation of a myxoma is intracardiac obstruction with congestive heart failure (67%);  Signs of embolization (29%); systemic or constitutional symptoms of fever (19%); weight loss or fatigue (17%);  Immunologic manifestations[slideshare.net]
  • Common systemic symptoms are: fever, malaise, weight loss, fatigue, aneamia, elevated erythrocyte sedimentation rate (ESR).[romanianjournalcardiology.ro]
Weight Gain
  • That implies a range of other choices over the decades in order to raise the odds that you can in fact choose to remain active when older, such as avoiding weight gain.[medworm.com]
  • (Brookdale University Hospital and Medical Center, New York) and John T Fallon, MD, PhD (New York Medical College at Westchester Medical Center, New York): 75 year old woman with edema on bilateral extremities and weight gain; mass in right atrium Electron[pathologyoutlines.com]
  • Affected children may experience weight gain and growth delays.[rarediseases.org]
Pathologist
  • More than 90 preeminent surgical pathologists offer expert advice on the diagnostic evaluation of every type of specimen from every anatomic site. The Fourth Edition contains 3,494 full-color photographs, of which over 1,100 are new.[books.google.com]
Surgical Procedure
  • Table 2: Surgical procedure and morphology of myxoma Surgical procedure N Isolated Myxoma Resection 20 Myxoma Resection CABG 3 Myxoma Resection MV repair 1 Myxoma Resection MV replacement 1 Site & Size Left Atrium 16 Right Atrium 10 Mitral Valve Annulus[apicareonline.com]
Failure to Thrive
  • Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more.[books.google.com]
Chest Pain
  • A 22-year-old son complained of dyspnoea and chest pain. On evaluation, 5.6   2.6 cm right atrial myxoma attached to the roof of the right atrium was found, and the patient underwent myxoma excision in 2007.[infona.pl]
  • The most common symptoms of atrial myxoma include difficulty breathing, chest pain, a tight feeling in the chest, dizziness, and fainting. The severity of symptoms varies based on the size of the tumor, and smaller tumors are often asymptomatic.[diseaseinfosearch.org]
Palpitations
  • A 40-year-old female presented with chest pain and palpitation, was diagnosed with left atrial myxoma, and was operated in 1993. After 2 years of surgery, the patient developed recurrent myxoma, and excision was done in 1995.[infona.pl]
  • The tumor is characterized by palpitations, disseminated neuritis, nausea, weight loss, fatigue, dyspnea, fever, and occasional sudden loss of consciousness. It is treated by surgical removal of the tumor.[medical-dictionary.thefreedictionary.com]
  • Patients may also present with atypical chest pain, syncope, lethargy, malaise, palpitation, peripheral edema, pulmonary embolism, and hemoptysis.[jmedicalcasereports.biomedcentral.com]
Arthralgia
  • Prognosis Excellent after excision. atrial myxoma Cardiology The most common 1º cardiac neoplasm, age of onset 25-55 Clinical Symptoms may be obstructive–right-sided congestion—most are right-sided, ascites, constitutional–fever, fatigue, arthralgias,[medical-dictionary.thefreedictionary.com]
  • Other less frequent complaints include Raynaud’s phenomenon, arthralgias, myalgias, erythematous rash, clubbing of the digits. 18.  Possible etiologies of such varied complaints and symptoms include - tumor embolization with secondary myalgias arthralgias[slideshare.net]
  • Fever, weight loss, arthralgia, myalgia, Raynaud phenomenon, rash, and clubbing may occur. Cardiac findings include new-onset congestive heart failure, chest pain, and dyspnea that improves when supine.[rheumaknowledgy.com]
  • In this report, our patient denied fever, arthralgias, and anemia but complained of weight loss (2 kg in two months) as the only constitutional sign.[jmedicalcasereports.biomedcentral.com]
  • Constitutional disturbances, such as fatigue, fever, erythematous rash, arthralgia, myalgia, and weight loss, and laboratory abnormalities, such as anemia and elevations in the erythrocyte sedimentation rate and the serum C-reactive protein and globulin[histopathology-india.net]
Suggestibility
  • These findings support previous reports suggesting that atrial myxoma may be familial. Echocardiography can be used effectively in the diagnosis of atrial myxoma, detection of its possible recurrence, and screening other members of the family.[ncbi.nlm.nih.gov]
  • Access full-color images and more than 90 online-only topics at Expert Consult, as well as EBMs, Suggested Reading, Patient Teaching Guides, and additional algorithms.[books.google.com]
  • Cardiovascular and Thoracic Surgery, Mumbai, India Additional information Copyright owner: Indian Association of Cardiovascular-Thoracic Surgeons, 2014 Publication languages: English Data set: Springer Fields of science No field of science has been suggested[infona.pl]
Meningism
  • Ferri’s popular "5 books in 1" format provides quick guidance on short QT syndrome, microscopic polyangiitis, fungal meningitis, and much more. This medical reference makes the answers you need even easier to find - anytime, anywhere.[books.google.com]
  • When a lumbar puncture is being performed due to concern for meningitis, a CT and/or lumbar puncture should not delay antibiotic administration."[capriles-urgencias.com]
Flaccid Paralysis
  • Our patient is a 27-year-old man with X-linked agammaglobulinemia (XLA) first diagnosed when he presented at 11 months of age in the summer of 1989 with acute-onset bilateral flaccid paralysis and sensory loss in his lower extremities subsequent to varicella[medworm.com]

Workup

  • Much has changed in recent years when it comes to TIA risk stratification, workup and antiplatelet therapy.[capriles-urgencias.com]
  • […] regurgitaiton • Tricuspid stenosis • Tricuspid regurgitation • Pulmonary hypertension • Endocarditis • Vasculitis • Left atrial thrombus • Pulmonary embolism • Cerebrovascular accidents • Collagen-vascular disease • Carcinoid heart disease • Ebstein’s anomaly WORKUP[encyclopedia.lubopitko-bg.com]
  • Lab workup showed deranged PT/INR and severe factor 7 deficiencies.[apicareonline.com]

Treatment

  • Treatment for familial atrial myxoma depends on many factors such as how large the tumor is and its location in the body.[diseaseinfosearch.org]
  • This era was followed by improved environmental factors which operate to produce methods of medical treatment, introduction congenital cardiac malformations.[books.google.com]
  • According to new datasets, smaller tracts can be equally effective in the treatment and might offer the possibility to reduce bleeding, length of hospital stay, postoperative pain as well as overall ...[medworm.com]
  • A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions.[leehealth.org]
  • Treatment Treatments Although atrial myxomas are not cancerous, complications are common. If untreated, it can lead to an embolism (tumor cells breaking off and traveling with the bloodstream).[nhcs.com.sg]

Prognosis

  • Prognosis Excellent after excision. atrial myxoma Cardiology The most common 1º cardiac neoplasm, age of onset 25-55 Clinical Symptoms may be obstructive–right-sided congestion—most are right-sided, ascites, constitutional–fever, fatigue, arthralgias,[medical-dictionary.thefreedictionary.com]
  • Prognosis of Atrial Myxoma Although atrial myxomas have the potential to embolise and to grow elsewhere, they are not believed to be able to spread to other sites.[myvmc.com]
  • His daughters were at his bedside and his prognosis was bleak. A massive stroke on Monday left Nixon paralyzed on the right side, unable to speak and partially blind.[latimes.com]
  • Prognosis [ edit ] An embolized fragment of an atrial myxoma in the iliac bifurcation. Although a myxoma is not cancer, complications are common.[en.wikipedia.org]
  • Myxoma Prognosis While a myxoma is not cancer, complications commonly occur.[disabled-world.com]

Etiology

  • Their etiology is not precisely century with the first clinical recognition of known, so we have included chapters that dis symptoms and patterns for diagnosis of car cuss many aspects of congenital cardiac mal diovascular diseases.[books.google.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]
  • Raynaud phenomenon ETIOLOGY • Most cases (90%) of atrial myxomas are sporadic with no known cause. In the remaining 10% of cases a familial pattern occurs having an autosomal dominant transmission.[encyclopedia.lubopitko-bg.com]
  • Most cases of atrial myxoma are sporadic, and the exact etiology is unknown.[emedicine.medscape.com]
  • Causes Most cases of atrial myxoma are sporadic, and the exact etiology is unknown. Familial atrial myxomas have an autosomal dominant transmission. Carney syndrome is genetically heterogenous and is caused by a defect in more than one gene.[odlarmed.com]

Epidemiology

  • Myxomas have been demonstrated to produce numerous growth factors and cytokines, including vascular endothelial growth factor, resulting in angiogenesis and tumor growth and an increased expression of the inflammatory cytokine, interleukin-6. [6, 7, 8] Epidemiology[thehealthscience.com]
  • EPIDEMIOLOGY & DEMOGRAPHICS • Atrial myxomas account for 50% of all primary tumors of the heart. • Approximately 75% of myxomas arise from the left atrium in close relationship to the fossa ovalis.[encyclopedia.lubopitko-bg.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]
  • […] 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 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.[thehealthscience.com]
  • 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]

Prevention

  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Coated and drug-eluting stents seem to be promising concepts to prevent stent-related symptoms, but still have to be c...[medworm.com]
  • This may require emergency surgery to prevent sudden death. The tumor must be surgically removed and some patients may need their mitral valve replaced as well.[nhcs.com.sg]

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