Atrial myxomas are the most common primary heart tumors. Because of nonspecific symptoms, early diagnosis may be a challenge. Left atrial myxoma may or may not produce characteristic findings on auscultation. Two-dimensional echocardiography is the diagnostic procedure of choice. Most atrial myxomas are benign and can be removed by surgical resection.
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. Approximately 10% are familial and are transmitted in an autosomal dominant mode. Multiple tumors occur in approximately 50% of familial cases and are more frequently located in the ventricle (13% vs 2% in sporadic cases).
Myxomas are polypoid, round, or oval. They are gelatinous with a smooth or lobulated surface and usually are white, yellowish, or brown. The most common site of attachment is at the border of the fossa ovalis in the left atrium, although myxomas can also originate from the posterior atrial wall, the anterior atrial wall, or the atrial appendage. The mobility of the tumor depends upon the extent of attachment to the interatrial septum and the length of the stalk.
Although atrial myxomas are typically benign, local recurrence due to inadequate resection or malignant change has been reported. Occasionally, atrial myxomas recur at a distant site because of intravascular tumor embolization. The risk of recurrence is higher in the familial myxoma syndrome.1
Symptoms are produced by mechanical interference with cardiac function or embolization. Being intravascular and friable, myxomas account for most cases of tumor embolism. Embolism occurs in about 30-40% of patients. The site of embolism is dependent upon the location (left or right atrium) and the presence of an intracardiac shunt.
Jong-Won Ha and associates reported a more frequent occurrence of systemic embolism in polypoid tumors as compared to round (58% vs 0%).2 Also, polypoid tumors more frequently prolapse into the ventricle. Prolapse of a tumor through the mitral or tricuspid valve may result in the destruction of the annulus or valve leaflets. In one study, 19% of the patients had atrial fibrillation associated with large atrial myxoma. Left atrial myxomas produce symptoms when they reach about 70 g. Right atrial myxomas grow to approximately twice this size before becoming symptomatic. Tumors vary widely in size, ranging from 1-15 cm in diameter. Rate of growth is not exactly known. In one case report, right atrial myxoma had a growth rate of 1.36 x 0.03 cm/month.
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-6.3,4,5
Based upon the data of 22 large autopsy series, the prevalence of primary cardiac tumors is approximately 0.02% (200 tumors per million autopsies). About 75% of primary tumors are benign, and 50% of benign tumors are myxomas, resulting in 75 cases of myxoma per million autopsies.
Surgical incidence in the Republic of Ireland from 1977-1991 was 0.50 atrial myxomas per million population per year.
Sudden death may occur in 15% patients with atrial myxoma. Death is typically caused by coronary or systemic embolization or by obstruction of blood flow at the mitral or tricuspid valve.
Morbidity is related to symptoms produced by tumor embolism, heart failure, mechanical valvular obstruction, and various constitutional symptoms.
Approximately 75% of sporadic myxomas occur in females.
Female sex predominance is less pronounced in familial atrial myxomas.
Myxomas have been reported in patients aged 3-83 years.
The mean age for sporadic cases is 56 years. In a retrospective review of 171 patients from India, the mean age of presentation was 37.1 years. Most of these patients were symptomatic; dyspnea was the most common symptom.6
The mean age for familial cases is 25 years.
Symptoms range from nonspecific and constitutional to sudden cardiac death. In about 20% of cases, myxoma may be asymptomatic and discovered as an incidental finding. Signs and symptoms of mitral stenosis, endocarditis, mitral regurgitation, and collagen vascular disease can simulate those of atrial myxoma. A high index of suspicion aids in diagnosis.
Symptoms of left-sided heart failure
Dyspnea on exertion (75%) that may progress to orthopnea, paroxysmal nocturnal dyspnea, and pulmonary edema is observed.
Symptoms are caused by obstruction at the mitral valve orifice. Valve damage may result in mitral regurgitation.
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.
These symptoms are also observed in the later stage of progressive heart failure associated with left atrial myxomas.
This is experienced by approximately 20% of patients.
The most frequent cause in patients with left atrial myxomas is obstruction of the mitral valve.
Symptoms may change as the patient changes positions.
Symptoms related to embolization
Systemic or pulmonary embolization may occur from left- or right-sided tumors.
Left-sided symptoms are produced from the infarction or hemorrhage of viscera.
Embolization to the central nervous system may result in transient ischemic attack, stroke, or seizure. In an analysis of 113 cases of atrial myxoma with neurologic presentation, 83% of patients presented with ischemic stroke, most often in multiple sites (43%). Twelve percent of patients presented with seizures. In a retrospective review of 74 patients with atrial myxoma, 12% had neurologic manifestations.7 Cerebral infarction was present in 89% of the cases and most myxomas (89%) demonstrated a mobile component on transesophageal echocardiography. Other complications include myxoma-induced cerebral aneurysm and myxomatous metastasis that can mimic vasculitis or endocarditis.
Involvement of the retinal arteries may result in vision loss.
Systemic embolization that causes occlusion of any artery, including coronary, aortic, renal, visceral, or peripheral, may result in infarction or ischemia of the corresponding organ.
On the right side, embolization results in pulmonary embolism and infarction.
Multiple, recurrent small emboli may result in pulmonary hypertension and cor pulmonale.
Presence of an intracardiac shunt (atrial septal defect or patent foramen ovale) may result in a paradoxical embolism.
Constitutional symptoms that include fever, weight loss, arthralgias, and Raynaud phenomenon are observed in 50% of patients. These symptoms may be related to overproduction of interleukin-6.
Hemoptysis due to pulmonary edema or infarction is observed in up to 15% of patients.
Chest pain is infrequent. If it occurs, it may be due to coronary embolization.
Jugular venous pressure may be elevated, and a prominent A wave may be present.
A loud S1 is caused by a delay in mitral valve closure due to the prolapse of the tumor into the mitral valve orifice (mimicking mitral stenosis).
P2 may be delayed. Its intensity may be normal or increased, depending on the presence of pulmonary hypertension.
In many cases, an early diastolic sound, called a tumor plop, is heard. This sound is produced by the impact of the tumor against the endocardial wall or when its excursion is halted.
An S3 or S4 may be audible.
A diastolic atrial rumble may be heard if the tumor is obstructing the mitral valve.
If there is valve damage from the tumor, mitral regurgitation may cause a systolic murmur at the apex.
A right atrial tumor may cause a diastolic rumble or holosystolic murmur due to tricuspid regurgitation.
General examination may reveal fever, cyanosis, digital clubbing, rash, or petechiae.
Patients with familial myxoma may have a variety of features called syndrome myxoma or Carney syndrome8 , as follows:
Myxomas in breast, skin, thyroid gland, or neural tissue
Spotty pigmentation such as lentigines (ie, flat brown discoloration of skin), pigmented nevi, or both
Endocrine hyperactivity such as Cushing syndrome
Multiple cerebral fusiform aneurysms may be seen in patients with Carney syndrome.9
Other described syndromes associated with atrial myxomas include the following:
NAME syndrome features nevi, atrial myxoma, myxoid neurofibroma, and ephelides (ie, freckles [tanned macules found on the skin]).
LAMB syndrome features lentigines, atrial myxoma, and blue nevi.
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. Abnormalities in the short arm of chromosome 2 (Carney) and chromosome 12 (Ki-ras oncogene) have been described. In a recent case report, a frame shift mutation was found in exon 2 in the causative gene of Carney complex, protein kinase A regulatory subunit 1 alpha (PRKAR1A).
Abu Zubair meriwayatkan dari Jabir bin Abdullah bahwa Nabi Muhammad SAW bersabda:
"Setiap penyakit ada obatnya. Jika obat yang tepat diberikan dengan izin Allah, penyakit itu akan sembuh".
(HR. Muslim, Ahmad dan Hakim).
Kamis, 24 Desember 2009
Diposkan oleh FX di 08.13
The Holy Al-Qur'an (English version)
- Surah 1 - Al Fatiha THE OPENING
- Surah 2 - Al Baqarah THE HEIFER
- Surah 3 - Ali 'Imran - THE FAMILY OF 'IMRAN
- Surah 4 - Al-Nisa' THE WOMEN
- Surah 5 - Al Ma'idah THE REPAST
- Surah 6 - Al An'am THE CATTLE
- Surah 7 - Al A'raf THE HEIGHTS
- Surah 8 - Al Anfal THE SPOILS OF WAR
- Surah 9 - Al Tawbah THE REPENTANCE
- Surah 10 - Yunus JONAH
- Surah 11 - Hud THE PROPHET HUD
- Surah 12 - Yusuf JOSEPH
- Surah 13 - Al Ra'd THE THUNDER
- Surah 14 - Ibrahim ABRAHAM
- Surah 15 - Al Hijr THE ROCKY TRACT
- Surah 16 - Al Nahl BEES
- Surah 17 - Al Isra' THE NIGHT JOURNEY
- Surah 18 - Al Kahf THE CAVE
- Surah 19 - Maryam MARY
- Surah 20 - TA HA
- Surah 21 - Al Anbiya THE PROPHETS
- Surah 22 - Al Hajj THE PILGRIMAGE
- Surah 23 - Al Mu'minun THE BELIEVERS
- Surah 24 - Al Nur THE LIGHT
- Surah 25 - Al Furqan THE CRITERION
- Surah 26 - Al Shu'ara' THE POETS
- Surah 27 - Al Naml THE ANTS
- Surah 28 - Al Qasas THE NARRATIONS
- Surah 29 - Al 'Ankabut THE SPIDER
- Surah 30 - Al Rum THE ROMANS
- Surah 31 - Luqman LUQMAN
- Surah 32 - Al Sajdah THE PROSTRATION
- Surah 33 - Al Ahzab THE CONFEDERATES
- Surah 34 - Saba' SHEBA
- Surah 35 - Fatir THE ORIGINATOR OF CREATION
- Surah 36 - Ya Sin YA SIN
- Surah 37 - Al Saffat THOSE RANGED IN RANKS
- Surah 38 - Sad SAD
- Surah 39 - Al Zumar CROWDS
- Surah 40 - Ghafir FORGIVER
- Surah 41 - Fussilat EXPOUNDED
- Surah 42 - Al Shura CONSULTATION
- Surah 43 - Al Zukhruf THE GOLD ADORNMENTS
- Surah 44 - Al Dukhan THE SMOKE
- Surah 45 - Al Jathiyah THE KNEELING DOWN
- Surah 46 - Al Ahqaf WINDING SAND-TRACTS
- Surah 47 - Muhammad MUHAMMAD
- Surah 48 - Al Fath THE VICTORY
- Surah 49 - Al Hujurat THE CHAMBERS
- Surah 50 - Qaf QAF
- Surah 51 - Al Dhariyat THE WINDS THAT SCATTER
- Surah 52 - Al Tur THE MOUNT
- Surah 53 - Al Najm THE STAR
- Surah 54 - Al Qamar THE MOON
- Surah 55 - Al Rahman THE MOST GRACIOUS
- Surah 56 - Al Waq'iah THE INEVITABLE
- Surah 57 - Al Hadid IRON
- Surah 58 - Al Mujadilah THE WOMAN WHO PLEADS
- Surah 59 - Al Hashr THE MUSTERING
- Surah 60 - Al Mumtahinah THAT WHICH EXAMINES
- Surah 61 - Al Saff THE BATTLE ARRAY
- Surah 62 - Al Jumu'ah FRIDAY
- Surah 63 - Al Munafiqun THE HYPOCRITES
- Surah 64 - Al Taghabun THE MUTUAL LOSS AND GAIN
- Surah 65 - Al Talaq DIVORCE
- Surah 66 - Al Tahrim PROHIBITION
- Surah 67 - Al Mulk THE DOMINION
- Surah 68 - Al Qalam THE PEN
- Surah 69 - Al Haqqah THE SURE REALITY
- Surah 70 - Al Ma'arij THE WAYS OF ASCENT
- Surah 71 - Nuh NOAH
- Surah 72 - Al Jinn THE SPIRITS
- Surah 73 - Al Muzzammil THE ENFOLDED ONE
- Surah 74 - Al Muddaththir THE ONE WRAPPED UP
- Surah 75 - Al Qiyamah THE RESURRECTION
- Surah 76 - Al Insan MAN
- Surah 77 - Al Mursalat THOSE SENT FORTH
- Surah 78 - Al Naba' THE GREAT NEWS
- Surah 79 - Al Nazi'at THOSE WHO TEAR OUT
- Surah 80 - 'Abasa HE FROWNED
- Surah 81 - Al Takwir THE FOLDING UP
- Surah 82 - Al Infitar THE CLEAVING ASUNDER
- Surah 83 - Al Mutaffifin THE DEALERS IN FRAUD
- Surah 84 - Al Inshiqaq THE RENDING ASUNDER
- Surah 85 - Al Buruj THE CONSTELLATIONS
- Surah 86 - Al Tariq THE NIGHT STAR
- Surah 87 - Al A'la THE MOST HIGH
- Surah 88 - Al Ghashiyah THE OVERWHELMING EVENT
- Surah 89 - Al Fajr THE DAWN
- Surah 90 - Al Balad THE CITY
- Surah 91 - Al Shams THE SUN
- Surah 92 - Al Layl THE NIGHT
- Surah 93 - Al Duha THE GLORIOUS MORNING LIGHT
- Surah 94 - Al Sharh THE EXPANSION OF THE BREAST
- Surah 95 - Al Tin THE FIG
- Surah 96 - Al Alaq THE CLINGING CLOT
- Surah 97 - Al Qadr THE NIGHT OF POWER
- Surah 98 - Al Bayyinah THE CLEAR EVIDENCE
- Surah 99 - Al Zalzalah THE EARTHQUAKE
- Surah 100 - Al 'Adiyat THOSE THAT RUN
- Surah 101 - Al Qari'ah THE GREAT CALAMITY
- Surah 102 - Al Takathur THE PILING UP
- Surah 103 - Al 'Asr TIME THROUGH THE AGES
- Surah 104 - Al Humazah THE SCANDALMONGER
- Surah 105 - Al Fil THE ELEPHANT
- Surah 106 - Quraysh THE TRIBE OF QURAYSH
- Surah 107 - Al Ma'un THE NEIGHBOURLY ASSISTANCE
- Surah 108 - Al Kawthar THE ABUNDANCE
- Surah 109 - Al Kafirun THOSE WHO REJECT FAITH
- Surah 110 - Al Nasr THE HELP
- Surah 111 - Al Masad THE PLAITED ROPE
- Surah 112 - Al Ikhlas THE PURITY OF FAITH
- Surah 113 - Al Falaq THE DAYBREAK
- Surah 114 - Al Nas MANKIND
- Acute Coronary Syndromes
- Angina Pectoris
- Anomalous Left Coronary Artery From the Pulmonary Artery
- Aortic Coarctation
- Aortic Dissection
- Aortic Regurgitation
- Aortic Stenosis
- Aortic Stenosis, Subaortic
- Aortic Stenosis, Supravalvar
- Ashman Phenomenon
- Atrial Fibrillation
- Atrial Flutter
- Atrial Myxoma
- Atrial Septal Defect
- Atrial Tachycardia
- Atrioventricular Block
- Atrioventricular Dissociation
- Atrioventricular Nodal Reentry Tachycardia (AVNRT)
- Benign Cardiac Tumors
- Brugada Syndrome
- Complications of Myocardial Infarction
- Coronary Artery Atherosclerosis
- Coronary Artery Vasospasm
- Digitalis Toxicity
- Dissection, Aortic
- Ebstein Anomaly
- Eisenmenger Syndrome
- First-Degree Atrioventricular Block
- HACEK Group Infections (Infective Endocarditis)
- Heart Failure - Decompensatio Cordis
- Holiday Heart Syndrome
- Hypertensive Heart Disease
- Junctional Rhythm
- Loeffler Endocarditis
- Long QT Syndrome
- Lutembacher Syndrome
- Mitral Regurgitation
- Mitral Stenosis
- Mitral Valve Prolapse
- Myocardial Infarction
- Myocardial Rupture
- Paroxysmal Supraventricular Tachycardia
- Patent Ductus Arteriosus
- Patent Foramen Ovale
- Pericardial Effusion
- Pericarditis Acute
- Pericarditis, Constrictive
- Pericarditis, Constrictive-Effusive
- Pulmonic Regurgitation
- Pulmonic Stenosis
- Right Ventricular Infarction
- Saphenous Vein Graft Aneurysms
- Second-Degree Atrioventricular Block
- Sinus of Valsalva Aneurysm
- Sudden Cardiac Death
- Tetralogy of Fallot
- Third-Degree Atrioventricular Block
- Torsade de Pointes
- Tricuspid Regurgitation
- Tricuspid Stenosis
- Unstable Angina
- Ventricular Fibrillation
- Ventricular Septal Defect
- Ventricular Tachycardia
- Wolff-Parkinson-White Syndrome