Combined Percutaneous Procedure in Patient with Lutembacher Syndrome: A Case Report and Real-World Experience Review

Phan, Quang Tan; Nguyen, Hieu Lan; Le, Tai Duc; Lee, WangSoo; Won, Hoyoun; Shin, Seung Yong; Sharmin, Saima; Nguyen, Truong Q.; Kim, Sang–Wook

doi:10.14740/cr776w

Cited by 9 publications

(11 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Tradicionalmente, el tratamiento estándar ha sido la reparación quirúrgica a corazón abierto 12 , procedimiento que se llevó a cabo en nuestra paciente con adecuada evolución posquirúrgica, permaneciendo asintomática desde el punto de vista cardiovascular durante su seguimiento. No obstante, gracias al avance de las técnicas de intervención percutánea, el cierre del defecto interauricular con dispositivo y la valvuloplastia mitral percutánea con balón se han convertido en una opción de tratamiento menos invasiva 10,12 . El diagnóstico oportuno y el tratamiento quirúrgico temprano desempeñan un papel importante en el pronóstico, ya que evitan la progresión a insuficiencia cardiaca y síndrome de Eisenmenger.…”

Section: Discussionunclassified

Presentación inusual del síndrome de Lutembacher

Mosquera¹,

Aristizábal²,

Pinto³

2023

RCCAR

View full text Add to dashboard Cite

Introducción: El síndrome de Lutembacher corresponde a la asociación de un defecto interauricular (congénito o iatrogénico) e insuficiencia o estenosis mitral (congénita o adquirida). La etiología reumática es la causa más frecuente del compromiso mitral. Tiene una prevalencia reportada de 0.001 por cada 1.000.000 habitantes. Caso clínico: Paciente femenina de 6 años con cuadro de 10 meses de palpitaciones asociadas a dolor torácico. Se realizó un electrogardiograma en el que se evidenció bloqueo incompleto de la rama derecha del haz de His y prolongación del intervalo PR, además de un ecocardiograma en el que se visualizó una comunicación interauricular tipo ostium secundum no restrictiva de 28 mm, con cortocircuito de izquierda a derecha, dilatación del ventrículo derecho, prolapso de válvula mitral, valvas engrosadas e insuficiencia mitral moderada a grave. Se realizó plastia de válvula mitral y cierre quirúrgico de la comunicación interauricular, sin complicaciones. Durante el seguimiento se encuentra asintomática desde el punto de vista cardiovascular, en manejo farmacológico. Conclusiones: La asociación Lutembacher tiene una prevalencia de 0.001/1.000.000 habitantes; la mayoría tiene etiología reumática. La paciente no tiene historia de fiebre reumática y sería la paciente más joven reportada en la literatura con síndrome de Lutembacher.

show abstract

Section: Discussionunclassified

Presentación inusual del síndrome de Lutembacher

Mosquera¹,

Aristizábal²,

Pinto³

2023

RCCAR

View full text Add to dashboard Cite

show abstract

“…There is neither sufficient evidence nor recommendation on how to treat LS, and its management mainly followed the guideline‐directed therapy of MS or ASD. When MS surgery therapy is indicated, both abnormalities were reported to be corrected simultaneously via open‐heart MV repair/replacement and ASD repair, or transcatheter balloon mitral valvuloplasty and ASD device closure 3 . However, no experience existed about how to manage patients with large ASD but mild MS that was insufficient for surgery.…”

Section: Discussionmentioning

confidence: 99%

“…The MS enhances the left‐to‐right shunt, predisposing patients to right heart dilation and pulmonary artery hypertension. On the other hand, a nonrestrictive ASD serves as another exit of the left atrium (LA) which could decompress the LA result in less turbulent flow through the mitral valve (MV) 2,3 . Therefore, the symptom and progression of MS were supposed to be relived and delayed.…”

Section: Introductionmentioning

confidence: 99%

Rapid progression of mitral stenosis after atrial septal defect closure in a Lutembacher's syndrome patient

Yang

Kang

Wei

2022

Journal of Cardiac Surgery

View full text Add to dashboard Cite

The combination of the atrial septal defect (ASD) and mitral stenosis (MS) is an unusual clinical entity called Lutembacher's syndrome. The hemodynamic interaction between the two cardiac malformations modifies the disease progression of each other. The symptom and progression of MS were thought to be slowed because of the existence of a left‐to‐right shunt that relived the blood flow through the mitral orifice. There is no consensus about caring this patient population for now. Here, we present a 58‐year‐old female with mild MS and coexistent ASD experiencing rapid progression of mitral valve lesions after percutaneous ASD closure. This case might identify the effect of ASD on delaying MS progression. From this point of view, MS and coexisting ASD should be evaluated and treated as a whole.

show abstract

“…Recently, the percutaneous transcatheter intervention has become the most widely accepted therapy, using a balloon mitral valvuloplasty for MS and the Amplatzer atrial septal occluder for closure of an ASD [10]. It is preferred over surgical correction in terms of decreased morbidity and length of hospital stay [11]. Late presentation where PAH and severe TR (functional TR develops due to volume overloaded right side and severe PAH) have already started to set in, the percutaneous intervention has limited utility.…”

Section: Discussionmentioning

confidence: 99%

Surgical Management of Lutembacher’s Syndrome in Congestive Cardiac Failure: A Case Report & Review of Literature

Yadav

Pathak

Gupta

2022

clin. med. & helt. res. jour.

View full text Add to dashboard Cite

Introduction: Lutembacher’s syndrome is a rare clinical entity. In 1916, French physician Rene Lutembacher described his first case of 61 years old woman with a combination of congenital ASD with acquired MS. But he attributed this mitral lesion to congenital mitral stenosis. The definition of this syndrome named after him has undergone many changes since then. The current consensus defines Lutembacher Syndrome (LS) as any combination of ASD (congenital or iatrogenic) and MS (congenital or acquired). The clinical presentation of LS is a result of the interplay between the size of ASD, the severity of MS, pulmonary vascular resistance and RV compliance. Financial constraints also add to the delayed diagnosis and adequate treatment in our part of the world. Here we have discussed a case of LS with severe TR and moderate PAH in congestive cardiac failure which was successfully treated by surgery thereby demonstrating cost-effective and successful surgical management of a rare cardiac disease where vascular resistance, compliance, size and severity of lesions interplay causing cardiac failure. Case report: A 42 years old thin built, poorly nourished housewife presented with chest discomfort and fatigue for the last 4 years. She was diagnosed with an atrial septal defect four years back but could not receive appropriate treatment due to financial constraints. For the last 15 days, she had breathlessness even at rest and paroxysmal episodes that interrupted her sleep. Icterus and pedal edema was also present. As we evaluated the patient, her clinical examination could not be entirely explained by ASD alone. Soon, our suspicion of LS was confirmed by TTE and the diagnosis of Lutembacher Syndrome with Severe Tricuspid Regurgitation with Moderate PAH was made. As the patient presented to us with atrial fibrillation & heart failure, she was admitted for optimization. The patient was then successfully managed by MVR [#27 SJM, metallic] with pericardial patch closure of ASD with Devaga’s Tricuspid Valve annuloplasty and discharged on a postop day 11. Conclusions: Early diagnosis and surgical treatment bear a good prognosis and prolong survival. If a patient is diagnosed late, pulmonary hypertension and heart failure set in and the outcome becomes poor. The higher cost of surgical intervention and low socio-economic distribution of the disease in developing countries like India should not challenge the patient’s right to receive the standard treatment.

show abstract

Combined Percutaneous Procedure in Patient with Lutembacher Syndrome: A Case Report and Real-World Experience Review

Cited by 9 publications

References 27 publications

Presentación inusual del síndrome de Lutembacher

Presentación inusual del síndrome de Lutembacher

Rapid progression of mitral stenosis after atrial septal defect closure in a Lutembacher's syndrome patient

Surgical Management of Lutembacher’s Syndrome in Congestive Cardiac Failure: A Case Report & Review of Literature

Contact Info

Product

Resources

About