Lutembacher syndrome is defined as a combination of mitral stenosis and a left-to-right shunt at the atrial level. Typically, the left-to-right shunt is an atrial septal defect (ASD) of the ostium secundum variety. Both these defects, ASD and mitral stenosis, can be either congenital or acquired.
The definition of Lutembacher syndrome has undergone many changes. The earliest description in medical literature is found in a letter written by anatomist Johann Friedrich Meckel to Albrecht von Haller in 1750. In 1916, Lutembacher described his first case of this syndrome, involving a 61-year-old woman, and he attributed the mitral valvular lesion to congenital mitral stenosis. Because the mitral stenosis was, in fact, rheumatic in etiology, the syndrome was defined eventually as a combination of congenital ASD and acquired, almost always rheumatic, mitral stenosis.
In the current era of mitral valvuloplasty for acquired mitral stenosis, however, residual iatrogenic ASD secondary to transseptal puncture is more common than congenital ASD, as is the combination of ASD and mitral stenosis. Although this syndrome is generally defined as mitral stenosis in combination with ASD, some have argued to define the syndrome as a combination of ASD and any mitral valve lesion, ie, mitral stenosis, mitral insufficiency, or mixed lesion. Currently, any combination of ASD, congenital or iatrogenic, and mitral stenosis, congenital or acquired, is referred as Lutembacher syndrome.
Mitral stenosis can be either congenital, as initially described, or acquired in origin, most commonly due to rheumatic mitral valve disease. Isolated mitral stenosis is now known to be a rare congenital disorder, and most cases of mitral stenosis initially thought to be congenital were, in fact, caused by rheumatic mitral valve disease.
Similarly, understanding of the etiology of ASD as associated with Lutembacher syndrome has evolved over time. Initially, high left atrial pressure due to mitral stenosis was thought to stretch open the patent foramen ovale (PFO), causing left-to-right shunt and providing another outlet for the left atrium. Now ASD in this syndrome, like mitral stenosis, is recognized as being either congenital or acquired, as already described.
Acquired ASD is almost always iatrogenic, either intentional or as a complication of a percutaneous interventional procedure. The incidence of left-to-right atrial shunt following mitral valvuloplasty is estimated at 11-12%. Although most of these ASDs are small and hemodynamically insignificant, some can be large enough to have hemodynamic consequences, especially in patients who develop restenosis of the mitral valve.
The hemodynamic effects of this syndrome are a result of the interplay between the relative effects of ASD and mitral stenosis. In its initial description, the ASD was typically large in Lutembacher syndrome, thus providing another route for blood flow. Iatrogenic ASDs tend to be smaller but still may be hemodynamically significant. The direction of blood flow is determined largely by the compliance of left and right ventricles. Normally, the right ventricle is more compliant than the left ventricle.
As a result, in the presence of mitral stenosis, blood flows to the right atrium through the ASD instead of going backward into the pulmonary veins, thus avoiding pulmonary congestion. This happens at the cost of progressive dilatation and, ultimately, failure of the right ventricle and reduced blood flow to the left ventricle. Development of Eisenmenger syndrome or irreversible pulmonary vascular disease is very uncommon in the presence of large ASD and high left atrial pressure because of mitral stenosis.
The term reverse Lutembacher syndrome is sometimes used to describe those rare cases in which a predominant right-to-left shunt develops owing to development of severe tricuspid stenosis.
The true incidence of the syndrome is not clearly known. Although mitral stenosis is encountered in 4% of patients with an ASD, congenital mitral stenosis itself is very rare, accounting for only 0.6% of congenital heart disease cases at autopsy. The incidence of ASD in patients with mitral stenosis is 0.6-0.7%. In one US study, the combination was found in 5 of 25,000 autopsies. The syndrome was diagnosed more frequently in the past for the following reasons:
Without echocardiography, the combination of mid diastolic murmur, actually due to increased blood flow across the tricuspid valve, and systolic murmur of ASD led to a mistaken diagnosis of Lutembacher syndrome.
The prevalence of both rheumatic heart disease and mitral stenosis was higher in western developed countries before the antibiotic era. With the decline in the frequency of rheumatic fever, the prevalence of mitral stenosis has decreased and so has diagnosis of the syndrome. A history of rheumatic fever is frequently absent.
Even though ASD may be underdiagnosed in the United States, the combination of ASD and mitral stenosis may not be evident on physical examination and for that reason is best confirmed by echocardiography.
Although the exact prevalence of Lutembacher syndrome is not known, it is probably higher in areas where rheumatic heart disease is still common.
No definite data are available. Mortality and morbidity rates are related to the relative severity of the individual lesions.
No data are available regarding racial distribution of the condition.
Lutembacher syndrome is more common in females than males. Part of the reason is the higher incidence of both congenital ASD and rheumatic mitral stenosis in females.
This syndrome can present at any age. Cases have been diagnosed in the seventh decade of life. Lutembacher's original case was a 61-year-old woman who had been pregnant 7 times. In the current era of balloon mitral valvuloplasty and development of ASD, the age of presentation may change.
Patients may remain asymptomatic for many years. Symptoms are mainly due to the ASD, and signs and symptoms vary according to the size of the ASD. With a large ASD, symptoms of pulmonary congestion, typical of isolated mitral stenosis, do not appear until late in the course of the disease. Conversely, these symptoms may appear early if the patient has an associated small ASD or develops pulmonary hypertension for other reasons. Patients with large ASD and moderate-to-severe mitral stenosis have signs and symptoms due mainly to right ventricular overload and right-sided heart failure, while patients with a small ASD and moderate-to-severe mitral stenosis have signs and symptoms of pulmonary congestion typical of mitral stenosis.
The patient may or may not have a history of rheumatic fever.
Fatigue and reduced exercise tolerance result from decreased systemic blood flow. The presence of mitral stenosis and left-to-right blood flow in diastole through the ASD reduces the forward flow of blood into the left ventricle, thereby reducing systemic blood flow and leading to fatigue and poor exercise tolerance.
Palpitations are a common presenting symptom. Because of the augmented left-to-right shunt caused by higher left atrial pressure and mitral stenosis, both atria are dilated. This predisposes patients to atrial arrhythmias; atrial fibrillation is very common.
Weight gain, ankle edema, right upper quadrant pain, and ascites are seen more commonly in patients with large ASD. Such symptoms are manifestations of the development of right-sided heart failure. A chronically increased left-to-right blood flow at the atrial level can eventually lead to right-sided heart failure.
Paroxysmal nocturnal dyspnea, orthopnea, and hemoptysis are signs of pulmonary venous congestion. Such symptoms are caused by mitral stenosis and are seen less frequently in Lutembacher syndrome than in isolated mitral stenosis. They are more common in patients with small ASD and are probably more common in patients who develop reverse Lutembacher syndrome. In some patients with large pulmonary blood flow due to a large left-to-right shunt, orthopnea can develop because of decreased compliance of the lungs.
Physical examination reveals signs due to the ASD and mitral stenosis, which are modified because of the presence of both lesions in the same patient.
Rhythm regular or irregular -Atrial fibrillation most common arrhythmia
Jugular venous pulse
Distended jugular veins, even in the absence of right heart failure
Large a waves when sinus rhythm is present
Increased right ventricular pressure a more important determinant than equalization of atrial pressures in increasing jugular venous pressure
Left parasternal lift, caused by transmitted right ventricular and pulmonary artery impulse, is common.
Left ventricular impulse is unimpressive, owing to reduced filling of the left ventricle secondary to mitral stenosis.
A tapping apex impulse due to the palpable, loud first heart sound of mitral stenosis may be present.
A diastolic thrill at the apex is unusual.
Loud first heart sound, opening snap, and a mitral early-to-mid diastolic murmur are the classic auscultatory findings of mitral stenosis and are variably present.
Reduced transmitral pressure gradient resulting from decompression of the left atrium through the ASD and displacement of the left ventricular apex due to a large right ventricle attenuate these classic findings of mitral stenosis.
Development of pulmonary hypertension and, consequently, an increase in right atrial and left atrial pressures may increase transmitral pressure gradient and bring out these auscultatory findings, but this phenomenon is canceled by further dilatation of the right ventricle, thus obscuring the left ventricular apex.
The second heart sound (S2) may be widely split for 2 reasons. Increased right heart flow of ASD can result in late closure of the pulmonary component of the S2, and decreased left ventricular and aortic flow, secondary to mitral stenosis and ASD, can cause early closure of the aortic component of S2.
Additional heart sounds and murmurs
Third and fourth heart sounds of right ventricular origin may be audible at the left sternal border and are louder with inspiration.
Systolic murmurs are due to the following:
ASD along the upper left parasternal area - Typically a flow murmur due to increased flow across the pulmonic valve
Tricuspid regurgitation along the lower left parasternal area - Due to the displaced tricuspid valve secondary to right ventricular dilatation; common
Holosystolic murmur at the left parasternal area due to tricuspid regurgitation - Usually increases with inspiration (Carvallo sign), which differentiates it from ASD and mitral regurgitation
Mid diastolic murmurs are due to the following:
Increased flow across the tricuspid valve due to ASD or accompanying tricuspid stenosis, best heard at left lower sternal border or at apex for reasons already mentioned
Mitral stenosis, best heard with stethoscope bell at apex after exercise and with patient in left lateral position
Continuous murmur in the lower right sternal area is due to continuous shunting of blood across a small ASD in the presence of severe mitral stenosis. This is an unusual finding on physical examination.
Abdomen: Ascites and hepatomegaly may be noted in the presence of right heart failure.
Extremities: Ankle edema may be present in the presence of right-sided heart failure.
Mitral stenosis is mostly rheumatic in origin.
Congenital mitral stenosis is very rare.
ASD is either congenital or iatrogenic.
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).
Jumat, 08 Januari 2010
Diposkan oleh FX di 19.30
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