Surgical Treatment of Lutembacher Syndrome with a Huge Right Atrium: A Case Report
DOI:
https://doi.org/10.1532/hsf.3641Keywords:
Lutembacher’s syndrome, right atrium, atrial septal defect, mitral stenosisAbstract
Background: Lutembacher’s syndrome (LS) is a rare cardiovascular anomaly that is defined as any combination of congenital or iatrogenic atrial septal defect (ASD) with congenital or acquired mitral stenosis (MS). The clinical features and hemodynamic effects of LS depend on the balance between ASD and MS.
Case Report: In this case report, we describe a rare case of LS with a huge right atrium in a 39-year-old male patient who was admitted to the hospital with worsening fatigue and breathlessness on exertion. Clinical examination revealed central cyanosis, raised jugular venous pressure bilaterally, clear breath sounds bilaterally with no dry and wet rales, hyperdynamic apex beat, and dull heart sounds. His vital signs on admission included blood pressure of 90/60 mmHg, irregular pulse of 76 beats/min, and oxygen saturation of 90.4%. Echocardiography revealed moderate to severe MS with ASD (ostium secundum). The patient’s condition deteriorated after initial medical management, and he underwent open heart surgery for mitral valve replacement, ASD repair, tricuspid annuloplasty, and right atrial volume reduction.
Conclusions: This case report describes the successful surgical management of this rare condition.
References
Alam MM, Khaled MFI. An incidental diagnosis of rheumatic mitral stenosis and secundum atrial septal defect (Lutembacher’s syndrome) in a young woman. Case Rep Cardiol 2019;2019:9402987.
Aminde LN, Dzudie A, Takah NF, Ngu KB, Sliwa K, Kengne AP. Current diagnostic and treatment strategies for Lutembacher syndrome: The pivotal role of echocardiography. Cardiovasc Diagn Ther 2015;5:122-132.
Blok IM, van Riel A, van Dijk APJ, Mulder BJM, Bouma BJ. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement?. Int J Cardiol 2017;227:51-52.
Henry WL, Morganroth J, Pearlman AS, et al. Relation between echocardiographically determined left atrial size and atrial fibrillation. Circulation 1976;53:273-279.
Khanna SN, Paul M, Bhan S, Sharma KK. Surgical correction of Lutembacher’s syndrome in a patient with severe pulmonary artery hypertension. J Card Surg 2014;29:569-571.
Mahajan K, Oliver TI. Lutembacher Syndrome. Treasure Island (FL), StatPearls Publishing; 2020.
Mathur R, Sanghvi S, Baroopal A. Percutaneous transcatheter treatment of Lutembacher syndrome. J Assoc Physicians India 2018;66:100-101.
Tezcan M, Isilak Z, Atalay M, Uz O. Echocardiographic assessment of Lutembacher syndrome. Kardiol Pol 2014;72:660.
Varsha AV, George G, Sahajanandan R. Lutembacher syndrome: Dilemma of doing a tricuspid annuloplasty. Ann Card Anaesth 2017;20:456-458.
