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Rhabdomyoma of the Myocardium
Myocardium Rhabdomyoma

Presentation

The dermatologic lesions and hypomelanotic macules are usually present at birth. [7] Our patient had no evidence of tuberous sclerosis. [jcnonweb.com]

Clinical presentation: Clinical profile varies from still-birth to intrauterine myocardial infarct (due to coronary artery compression). [histopathology-india.net]

The patient presented with a systolic murmur (graded 3/6) with a palpable thrill, no cyanosis, and no edema. [jtd.amegroups.com]

Some patients may present with only renal and pulmonary manifestations while in some skin or neurological manifestations might be absent. However, in our case, the patient had skin and neurological features as the presenting complaints. [mjdrdypu.org]

In more than half of the cases, it spontaneously remits after weaning. 5 We present the case of a patient with an echocardiographic diagnosis of suspected multiple rhabdomyomas with spontaneous, complete early remission. [revespcardiol.org]

Entire Body System

  • Congestive Heart Failure

    Complications development of cardiac arrhythmias intracavitary growth may cause ventricular outflow tract obstruction valvular compromise disruption of intracardiac blood flows leading to congestive heart failure and hydrops For the in utero sonographic [radiopaedia.org]

    Congestive heart failure and a low cardiac output can occur when the tumor extensively involves the myocardium or the ventricular papillary muscles. Cyanosis and decreased peripheral pulses can result. [emedicine.medscape.com]

    […] id=506 Artificial cardiac Pacemaker Sonography of the heart (echocardiography) was done in this patient of congestive cardiac failure (CCF) or congestive heart failure (CHF). [ultrasound-images.com]

    Clinical symptoms in the neonate include severe congestive heart failure and cyanosis. 52, 54, 56, 63, 64 Significant pericardial effusions have also been associated with fibromas. 29 Myxoma Cardiac myxomas are the most common primary cardiac tumor in [radiologykey.com]

    Serial ultrasonic examination should be offered to identify signs of congestive heart failure or rhythm disturbances. [jcnonweb.com]

  • Sepsis

    Investigations at admission revealed normal sepsis screen. Chest X-ray was suggestive of gross cardiomegaly with a cardiothoracic ratio of 90%. The left lung was not visualized [Figure 1]. [jcnonweb.com]

  • Disability

    It is also a major cause of disability. There are many different forms of heart disease. The most common cause of heart disease is narrowing or blockage of the coronary arteries, the blood vessels that supply blood to the heart itself. [icdlist.com]

Gastrointestinal

  • Diarrhea

    Some of the clinical difficulty and progression of cardiac electrical instability could be attributed to extracardiac problems such as dehydration (vomiting, diarrhea) or fever(23). The lesions are in a dynamic state of development/ progression(4). [conganat.org]

Cardiovascular

  • Heart Disease

    heart disease (Medical Encyclopedia) Being active when you have heart disease (Medical Encyclopedia) Electrocardiogram (Medical Encyclopedia) Exercise stress test (Medical Encyclopedia) Heart disease - risk factors (Medical Encyclopedia) Heart disease [icdlist.com]

    Because tumors in the heart can mimic other heart diseases, they are difficult to diagnose accurately. [cedars-sinai.edu]

    Patten BM: Development of the heart, in Gould SE (ed): Pathology of the Heart. Springfield, Ill, Charles C. Thomas, 1968. 3. Volodaver Z, Neufeld HN, Edwards JE: Coronary Arterial Variations in the Normal Heart and in Congenital Heart Disease. [sonoworld.com]

    […] myxoma, gross Heart, atrial myxoma, microscopic Heart, epicardium, metastases, gross Heart, myocardium, metastatic melanoma, microscopic Congenital Heart Disease Congenital heart disease, table Heart, probe patent foramen ovale, and paradoxical embolus [library.med.utah.edu]

    Neoplastic heart disease. In: Foster A, Alexander RW, O'Roule RA, Cober R, King SB, Weller HJ, editors. Hurst's the heart. 10 th ed, Vol. 2. New York: McGraw Hill; 2001.10. [jcnonweb.com]

  • Heart Failure

    They are often asymptomatic but can cause heart failure, arrhythmias and obstruction. In these cases, they must be operated upon. [em-consulte.com]

    Symptoms of a heart tumor caused by the spread of a malignancy from another part of the body include: Sudden enlargement of the heart, Bizarre changes in the shape of the heart on a chest x-ray A blockage Irregular heart rhythms Unexplained heart failure [cedars-sinai.edu]

    Cardiac rhabdomyoma is frequently multiple and asymptomatic, but the tumour may cause cardiomegaly, heart failure by causing outflow obstruction, arrhythmias, thromboembolic disease or sudden unexpected death. [histopathology-india.net]

    Complications development of cardiac arrhythmias intracavitary growth may cause ventricular outflow tract obstruction valvular compromise disruption of intracardiac blood flows leading to congestive heart failure and hydrops For the in utero sonographic [radiopaedia.org]

    Only one case with signs of heart failure was subjected to surgical resection with good results. [bmccardiovascdisord.biomedcentral.com]

  • Heart Murmur

    Cardiac auscultation detected a systolic heart murmur in the LSE retaining a second tone. The rest of the physical examination was not significant. ECG was in sinus rhythm with no significant findings. [revespcardiol.org]

    RV overload signs Multiple rhabdomyomas No 30 Fetal Heart murmur Normal Multiple rhabdomyomas No 31 7 days Heart murmur Normal Multiple rhabdomyomas No 32 21 days No symptoms RV hypertrophy Multiple rhabdomyomas No 33 Fetal Heart murmur Ventricular ectopic [bmccardiovascdisord.biomedcentral.com]

    Interference in the way the heart works by the tumor can create symptoms similar to valve disorders caused by rheumatic disease including lung congestion and heart murmurs. [cedars-sinai.edu]

    Symptoms (Wolff-Parkinson-White syndrome, heart failure or arrhythmias) were present in 48 of the 134 patients in whom symptoms were mentioned (35.8%) while 86/134 patients (64.2%) were asymptomatic (nothing, heart murmur or irregular heartbeats). [em-consulte.com]

  • Cardiomegaly

    Cardiac rhabdomyoma is frequently multiple and asymptomatic, but the tumour may cause cardiomegaly, heart failure by causing outflow obstruction, arrhythmias, thromboembolic disease or sudden unexpected death. [histopathology-india.net]

    Chest X-ray was suggestive of gross cardiomegaly with a cardiothoracic ratio of 90%. The left lung was not visualized [Figure 1]. ECG was suggestive of regular sinus rhythm and right ventricular hypertrophy. [jcnonweb.com]

Musculoskeletal

  • Arthralgia

    Depending on location and morphology, cardiac tumors can produce four types of clinical manifestations: Systemic – constitutional (fever, arthralgias, weight loss, fatigue) and paraneoplastic syndromes (primary cardiac tumors). [wikiecho.org]

Fetus

  • Hydrops Fetalis

    Cardiac rhabdomyomas may have detrimental effects on the fetus or newborn from a cardiovascular standpoint as there is a risk of arrhythmias, outflow tract obstruction, and non-immune hydrops fetalis. [thieme-connect.com]

    Tumors obstructing the right-side inflow or the outflow of the ventricles can lead to decreased cardiac output, atrial and caval hypertension, hydrops fetalis, and death. Arrhythmias, both ventricular and atrial, are not uncommon. [emedicine.medscape.com]

    Rarely, it may be a sporadic event. 28 The clinical and hemodynamic findings of cardiac rhabdomyomas are related to the number, size, and position of the tumors. 29 In the fetus, if supraventricular tachycardia occurs, nonimmune hydrops fetalis and polyhydramnios [radiologykey.com]

    Rehuban KS, McDaniel NL, Feldman PS, Mayes DC, Rodgers BM: Intrapericardial teratoma causing nonimmune hydrops fetalis and pericardial tamponade: a case report. Pediatr Cardiol 1991;12:54-56 55. [fac.org.ar]

Workup

Other ECG Findings

  • Ischemic Changes

    Irreversible ischemic changes occur during fatal episodes of paroxysmal atrial tachycardia(14). [conganat.org]

Treatment

MRI T1 : isointense to adjacent myocardium T2: hyperintense to adjacent myocardium Treatment and prognosis In most cases, no treatment is required, and these lesions regress spontaneously. [radiopaedia.org]

Treatment of benign tumors is usually surgical resection; tumors may recur. Treatment of metastatic cancer depends on tumor type and origin; prognosis is generally poor. Primary cardiac tumors are found in 1/2000 people at autopsy. [msdmanuals.com]

Patients with inoperable cardiac rhabdomyomas and with symptoms may be candidates for everolimus treatment. [pediatrics.aappublications.org]

Michler RE, Goldstein DJ: Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 1997;24:534-539 81. [fac.org.ar]

Without treatment, patients have a life expectancy of only a few months ( 7, 16, 17 ). [aerzteblatt.de]

Prognosis

The overall prognosis is dependent on the number, size and location of the lesions as well as the presence or absence of associated anomalies. [radiopaedia.org]

Benign cardiac tumors in childhood have an excellent prognosis when completely excised and appear to have a good short-time prognosis even when excision is incomplete. [9] Indications of surgery include presence of significant symptoms in combination [jcnonweb.com]

It should also be noted that symptomatic patients generally have a poorer prognosis than those who are asymptomatic [ 5 ]. A review of all papers on cardiac rhabdomyomas published up to 1990 is available [ 11 ]. [em-consulte.com]

Treatment of metastatic cancer depends on tumor type and origin; prognosis is generally poor. Primary cardiac tumors are found in 1/2000 people at autopsy. Metastatic tumors are 30 to 40 times more common. [msdmanuals.com]

Etiology

The etiology of rhabdomyoma is unknown. Studies have delineated at least two genetic loci on the long arm of chromosome 9 (9q34) and the long arm of chromosome 11 (11q23). [5] Rhabdomyoma resembles a hamartoma derived from embryonal myoblasts. [jcnonweb.com]

(Etiology) The exact cause and mechanism of formation of Cardiac Rhabdomyosarcoma is unknown. [dovemed.com]

III) Cardiomyopathy The clinical and pathological alterations described have benn frequently described as a CARDIOMYOPATHY, the etiology of which is unclear(3,13). [conganat.org]

Epidemiology

This timely volume covers history, epidemiology, demographics, clinical diagnosis, pathology, imaging by echo, CT and MRI of both benign and malignant cardiac tumors, either primary or secondary. [books.google.it]

[Crossref] Torimitsu S, Nemoto T, Wakayama M, Okubo Y, Yokose T, et al. (2012) Literature survey on epidemiology and pathology of cardiac fibroma. Eur J Med Res 17: 5. [oatext.com]

[…] atrium and ventricle Figure 4 Positron emission tomography/computed tomography of nodular ecurrent sarcoma Figure 5 Explanted heart after ex-situ resection of a sarcoma and implantation of a mitral valve before cardiac reimplantation Benign tumors Myxoma Epidemiology [aerzteblatt.de]

Pathophysiology

Criterion for fetal primary spongiform Cardiomyopathy: restrictive pathophysiology. Obstet Gynecol 2002 May; 99 (5 pt 2); 882-5. 7. Stamm E and Drose J. The Fetal Heart, in Rumack C, Wilson S, Charboneau J. Diagnostic Ultrasound. 2nd edition. [sonoworld.com]

Prevention

In the Anura (frogs and toads) the whole ventricle is filled with a spongy network which prevents the arterial and venous blood from the two auricles mixing to any great extent. [thefullwiki.org]

For a patient with obvious symptoms, a resection of the tumor to release the symptoms and prevent further complications and risk of sudden death should be considered since there is a high probability of arrhythmias and ventricular tachycardia [4]. [oatext.com]

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