Lutembacher's syndrome

Jump to navigation Jump to search

WikiDoc Resources for Lutembacher's syndrome

Articles

Most recent articles on Lutembacher's syndrome

Most cited articles on Lutembacher's syndrome

Review articles on Lutembacher's syndrome

Articles on Lutembacher's syndrome in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Lutembacher's syndrome

Images of Lutembacher's syndrome

Photos of Lutembacher's syndrome

Podcasts & MP3s on Lutembacher's syndrome

Videos on Lutembacher's syndrome

Evidence Based Medicine

Cochrane Collaboration on Lutembacher's syndrome

Bandolier on Lutembacher's syndrome

TRIP on Lutembacher's syndrome

Clinical Trials

Ongoing Trials on Lutembacher's syndrome at Clinical Trials.gov

Trial results on Lutembacher's syndrome

Clinical Trials on Lutembacher's syndrome at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Lutembacher's syndrome

NICE Guidance on Lutembacher's syndrome

NHS PRODIGY Guidance

FDA on Lutembacher's syndrome

CDC on Lutembacher's syndrome

Books

Books on Lutembacher's syndrome

News

Lutembacher's syndrome in the news

Be alerted to news on Lutembacher's syndrome

News trends on Lutembacher's syndrome

Commentary

Blogs on Lutembacher's syndrome

Definitions

Definitions of Lutembacher's syndrome

Patient Resources / Community

Patient resources on Lutembacher's syndrome

Discussion groups on Lutembacher's syndrome

Patient Handouts on Lutembacher's syndrome

Directions to Hospitals Treating Lutembacher's syndrome

Risk calculators and risk factors for Lutembacher's syndrome

Healthcare Provider Resources

Symptoms of Lutembacher's syndrome

Causes & Risk Factors for Lutembacher's syndrome

Diagnostic studies for Lutembacher's syndrome

Treatment of Lutembacher's syndrome

Continuing Medical Education (CME)

CME Programs on Lutembacher's syndrome

International

Lutembacher's syndrome en Espanol

Lutembacher's syndrome en Francais

Business

Lutembacher's syndrome in the Marketplace

Patents on Lutembacher's syndrome

Experimental / Informatics

List of terms related to Lutembacher's syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-in-Chief: Ayokunle Olubaniyi, M.B,B.S

Overview

Lutembacher's syndrome is a rare form of congenital heart disease. It refers to a combination of congenital atrial septal defect, or even a patent foramen ovale (PFO) complicated by an acquired mitral stenosis.[1] The atrial septal defect is usually a specific type called a secundum atrial septal defect. This syndrome was named after René Lutembacher, a french cardiologist, who described the syndrome in 1916.[2] Iatrogenic Lutembacher's syndrome has also been reported.[3]

Pathophysiology

The presence of both ASD and mitral stenosis occuring together, usually modify the clinical and hemodynamic manifestation of each other. The presence of an ASD creates a second exit (left-to-right shunt) for the blood in the left atrium; consequently reducing the hemodynamic effects of a severe mitral stenosis. In the same fashion, the pressure in the left atrium, pulmonary veins and the pulmonary capillaries decrease if the ASD is large. Therefore, the typical presentation of mitral stenosis as a result of pulmonary venous congestion such as orthopnea, paroxsymal nocturnal dyspnea, hemoptysis and pulmonary edema are attenuated or diminished, and are often substituted by symptoms of low volume output such as weakness and fatigue.[4]

Epidemiology and Demographics

This is a very rare disease. The incidence is 0.001/1000000[5]. This syndrome is more frequently seen in adults because the mitral stenosis is usually an acquired valvulopathy of rheumatic origin. It is also more commonly observed in female patients because both ASD and MS are more prevalent in this gender.[4]

Complications and Prognosis

Complications are usually related to a late diagnosis. They include pulmonary hypertension, heart failure and infective endocarditis. Early diagnosis and surgical treatment has a good prognostic value.

Diagnosis

History and Symptoms

The presentation depends on the size of ASD, extent of mitral stenosis, compliance of the right ventricle and degree of changes in the pulmonary circulation.[4] Symptoms can be due to:

Physical Examination

The physical findings in an adult with Lutembacher's syndrome depends on:

Heart

Inspection

Palpation

  • Right ventricular impulse: An increased left-to-right atrial shunt can cause a hyperdynamic right ventricular impulse or heave. The heave can be best palpated at the left sternal border or the subxiphoid area.
  • Pulmonary artery pulsations: Pulsatile, enlarged pulmonary artery pulsation can be felt at the second left intercostal space. These are more pronounced in patients with large left-to-right shunts. Patients with obstruction to right ventricular outflow have a less dynamic right ventricular impulse and may present with more of a tapping or thrusting quality.

Auscultation

The classic presentation of pure mitral stenosis such as the loud first heart sound, opening snap, mid-diastolic rumble with presystolic accentuation are not usually heard.[4] A continuous murmur may be present in some cases of Lutembacher's syndrome with small ASD and a tight mitral stenosis because of the high left atrium-to-low right atrial pressure difference across the ASD, which persists during the entire cardiac cycle.[4] A loud pulmonic mid-systollic murmur and a holosystollic murmur due to the presence of a tricuspid regurgitation may also be present in these patients.[4]

Electrocardiogram

EKG features may include a normal sinus rhythm, normal PR interval/normal QTc, right bundle branch block (RBBB), bi-atrial enlargement, left ventricular hypertrophy.

Chest X-Ray

CXR findings on an anteroposterior view of the chest x-ray in Lutembacher's syndrome may include:

1) Prominent pulmonary artery, increased pulmonary vascular markings.

2) Cardiomegaly due to right atrial and ventricular enlargement.

Echocardiography

This is often required in order to make a diagnosis. It can be used to assess the degree of severity of the condition through the estimation of the left ventricular ejection fraction. Features may include left ventricular hypertrophy, bi-atrial enlargement and valvular thickenings and calcifications.

Treatment

Percutaneous approach

Traditionally, this condition has been treated surgically.[6] Nowadays, it can be treated with percutaneous transcathetar mitral commissurotomy (PTMC) using the Inoue balloon.[7] The Inoue technique has become the procedure of choice around the world with excellent long term results.[8] The ASD was closed with an Amplatzer atrial septal occluder under transthoracic echocardiogram (TTE) guidance without general anesthesia.[7] By combining these two techniques in the same patient at the same cardiac catheterization, percutaneous management of Lutembacher syndrome can obviate the morbidity and mortality associated with cardiac surgery, the psychological trauma of a thoracotomy scar, the prolonged hospital stay followed by another prolonged period of home convalescence and the possibility of repeat thoracotomy for mitral restenosis.[6]

Surgical approach

The mitral valve can either be repaired or replaced. Mitral valve repair is the treatment of choice for patients with both secundum ASD and mitral valve disease.[9] When valve replacement is the only alternative, selection of prosthetic valves and maintenance of cardiac output by temporary cardiac pacing are important considerations.[9]

References

  1. Goldman 2011, pp. 400
  2. Shen XQ, Zhou SH, Zhou T, Qi SS, Fang ZF, Lv XL (2005). "Transcatheter treatment of Lutembacher syndrome". Chin Med J (Engl). 118 (21): 1843–5. PMID 16336826.
  3. Sadaniantz A, Luttmann C, Shulman RS, Block PC, Schachne J, Thompson PD (1990). "Acquired Lutembacher syndrome or mitral stenosis and acquired atrial septal defect after transseptal mitral valvuloplasty". Cathet Cardiovasc Diagn. 21 (1): 7–9. PMID 2208272.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 Olivares-Reyes A, Al-Kamme A (2005). "Lutembacher's syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement". J Am Soc Echocardiogr. 18 (10): 1105. doi:10.1016/j.echo.2005.01.017. PMID 16198889.
  5. Berry NS, Bauman JL, Gallastegui JL, Bauma W, Beckman KJ, Hariman RJ (1988). "Analysis of antiarrhythmic drug concentrations determined during electrophysiologic drug testing in patients with inducible tachycardias". Am J Cardiol. 61 (11): 922–4. PMID 3354470.
  6. 6.0 6.1 Cheng TO (1999). "Coexistent atrial septal defect and mitral stenosis (Lutembacher syndrome): An ideal combination for percutaneous treatment". Catheter Cardiovasc Interv. 48 (2): 205–6. PMID 10506781.
  7. 7.0 7.1 Shabbir M, Ahmed W, Akhtar K (2008). "Transcatheter treatment of Lutembacher's syndrome". J Coll Physicians Surg Pak. 18 (2): 105–6. doi:02.2008/JCPSP.105106 Check |doi= value (help). PMID 18454897.
  8. Chen CR, Cheng TO (1995). "Percutaneous balloon mitral valvuloplasty by the Inoue technique: a multicenter study of 4832 patients in China". Am Heart J. 129 (6): 1197–203. PMID 7754954.
  9. 9.0 9.1 Shigenobu M, Sano S (1994). "Surgical indications and treatment of mitral valve disease associated with secundum atrial septal defect with special reference to left ventricular geometry and function". J Cardiovasc Surg (Torino). 35 (6): 469–74. PMID 7698959.


Template:WikiDoc Sources