Lutembacher’s Syndrome Accompanied by Partial Anomalies of Pulmonary Veins in a 70-Year-Old Male: A Case of Congenital Heart Disease in the Elderly

Lutembacher’s syndrome is a rare disease defined as the combination of atrial septal defect and mitral stenosis, which relieves the progression of symptoms resulting from mitral stenosis. We present a case of a previously asymptomatic 70-year-old man having Lutembacher’s syndrome with sinus venosus type of interatrial defect associated with partial anomalous pulmonary venous drainage. Our patient’s only complaint was of mild exertional dyspnea, which started six months prior to referral. Clinical suspicion of the combination of defects arose during transthoracic echocardiography. The patient underwent transesophageal echocardiography and multi-slice computed tomography for defect confirmation. The combination of Lutembacher’s syndrome with this complex deformity of pulmonary venous drainage has rarely been described. Such complex congenital defects with multifaceted physiological repercussions can go largely undiagnosed for the patients affected for most of their lifetimes. LEARNING POINTS: Despite the complexity of Lutembacher’s syndrome, including the involvement of several deformities and pathological mechanisms of mitral stenosis, sinus venosus type of interatrial defects with partial anomalous return in the pulmonary veins, the multiplicity of mechanisms involved contribute to altered hemodynamics. Multiple intersecting mechanisms can further contribute to delaying congenital heart disease presentation. Congenital heart disease remains a plausible differential in the evaluation of elderly patients presenting with exertional dyspnea. Adult congenital heart defects can have very late onset for presentation, especially with physiological processes that paradoxically reduce the severity and progression of symptoms.