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Chest X-ray P/A view showing bulged pulmonary conus, cardiomegaly, and prominent right pulmonary artery.
Source publication
Lutembacher’s syndrome is a rare cardiovascular defect comprising of mitral stenosis and atrial septal defect. A combination of acquired mitral stenosis and congenital atrial septal defect is the most well-recognized pattern. As atrial septal defect acts as a pressure relieving gateway, signs and symptoms of mitral stenosis may be attenuated and/or...
Citations
... 3 They can also present with signs and symptoms of right heart failure because of pulmonary hypertension, such as jugular engorgement, systolic murmur in the pulmonic valve area, and systolic murmur in the tricuspid valve area. 3,5 Patients will ultimately develop Eisenmenger syndrome (ES) because of increased pulmonary vascular resistance. The initial left-to-right shunt will become a right-toleft shunt, resulting in significant hypoxemia and cyanosis, and worsening their prognosis. ...
... En 1987, Bashi et al. reportaron en una serie de casos que hasta un 40% de los pacientes con síndrome de Lutembacher tenían antecedente de fiebre reumática 8 . La estenosis mitral congénita es una entidad rara, que representa solo el 0.6% de las cardiopatías congénitas 9 . En pacientes con defectos interauriculares, hasta el 4% tienen una estenosis mitral asociada, mientras que la incidencia de defectos interauriculares en estenosis mitral es de solo 0.6 a 0.7% 2 . ...
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.
... Lutembacher syndrome (LS) is a rare cardiac clinical condition characterised by any combination of atrial septal defect (ASD) (congenital or iatrogenic) and mitral stenosis (MS) (congenital or acquired) [1,2]. However the combination of a congenital ASD (usually secundum type) and acquired MS (usually rheumatic) is the common well recognized pattern [2]. ...
... Lutembacher syndrome (LS) is a rare cardiac clinical condition characterised by any combination of atrial septal defect (ASD) (congenital or iatrogenic) and mitral stenosis (MS) (congenital or acquired) [1,2]. However the combination of a congenital ASD (usually secundum type) and acquired MS (usually rheumatic) is the common well recognized pattern [2]. Although LS might be common in some part of the world but it is not commonly seen in this environment probably due to limited appropriate equipment and inadequate skilled personnel to make the diagnosis when present. ...
... Although LS might be common in some part of the world but it is not commonly seen in this environment probably due to limited appropriate equipment and inadequate skilled personnel to make the diagnosis when present. Generally, LS is a very rare clinical entity with very few case reports across the globe [2,3]. We present a case of a young adult male with this rare heart disease at Obafemi Awolowo University Teaching Hospitals complex (OAUTHC), ile-ife located in South Western region of Nigeria. ...
Lutembacher syndrome (LS) is a rare syndrome comprising a combination of atrial septal defect (ASD) and mitral stenosis. We present the case of a 28-year-old man, who presented with progressively worsening dyspnea of 2 months associated with orthopnea, paroxysmal nocturnal dyspnea, bilateral leg swelling and productive cough. Chest X-ray revealed plethoric lung fields with prominent pulmonary conus and cardiomegaly. Transthoracic echocardiography revealed a large ostium secundum ASD with left to right shunt, mild mitral stenosis, severe mitral and tricuspid regurgitations and pulmonary hypertension. A diagnosis of Lutembacher syndrome in heart failure with pulmonary hypertension was made. The patient was managed conservatively, but declined surgery primarily because of financial reasons. This rare case of LS presenting with heart failure and complicated by pulmonary hypertension is the first reported case in our centre and our region. The patient's inability to afford the cost of definitive care posed a significant problem in his management.
A woman in her 30s presented with progressive worsening of dyspnoea for 6 months. On evaluation, she was diagnosed with severe rheumatic mitral stenosis (mitral valve area of 0.6 cm ² ) and a large ostium secundum atrial septal defect (21 mm) with a left to right shunt and severe pulmonary artery hypertension. She was diagnosed with Lutembacher syndrome and was evaluated for suitability of a percutaneous approach. She was subjected to a combined procedure of percutaneous transluminal mitral commissurotomy followed by device closure of the atrial septal defect. The patient tolerated the procedure, remained haemodynamically stable and was discharged after 4 days. This procedure can prevent the morbidity and mortality associated with anaesthesia and cardiac surgery and the psychological trauma of a thoracotomy scar particularly in a female patient, as well as obviate the need for prolonged hospital stay.
Purpose of review:
Despite numerous attempts, none of a wide variety of tested drugs achieved meaningful improvement in the outcomes of heart failure with preserved ejection fraction (HFpEF), making new therapeutic strategies a major unmet medical need. The medical device industry embraced the challenge, developing novel technologies directed to face specific aspects of the pathophysiology of HFpEF. This review focuses on some of the most promising technologies attaining meaningful clinical progress recently in the field of HFpEF therapy.
Recent findings:
Implantable pulmonary artery pressure, monitoring for optimization of medical therapy, proved to be beneficial in heart failure admissions in a large postmarketing clinical study. Investigational devices, such as inter-atrial shunts and transvenous phrenic nerve stimulators for the treatment of central sleep apnea with Cheyne-Stokes breathing, are currently being evaluated in HFpEF cohorts in recent trials.
Summary:
Device-based therapies for HFpEF demonstrated encouraging safety and efficacy results in various stages of the disease. Further efforts are needed to ensure that these devices will reach clinical use and contribute to the management of HFpEF patients.
