Partial Anomalous Pulmonary Venous Connection Associated with Lutembacher's Syndrome

Abstract

Lutembacher's syndrome is a rare combination of congenital atrial septal defect and mitral stenosis (almost always rheumatic). The hemodynamic effect of this combination makes the clinical diagnosis difficult due to a wide variation of clinical presentation. Echocardiography and cardiac catheterization are useful tools for proper diagnosis and planning of the therapeutic strategy. We present a 28-year-old female with Lutembacher's syndrome associated with partial anomalous pulmonary venous connection (PAPVC), which was surgically corrected. To our knowledge, this combination has never been reported in the literature.

Transcatheter therapy of partial anomalous pulmonary venous connection with dual drainage and coarctation of the aorta in a single patient

“…Approximately 90% of all PAPVCs originate from the right lung, 7% originate from the left lung, and 3% of patients are found to have bilateral PAPVCs originating from both lungs connecting to the superior vena cava (SVC), the inferior vena cava (IVC), the right atrium, or the innominate vein. Partial anomalous pulmonary venous connections are frequently associated with atrial septal defects [1], [2], [3], and are rarely associated with other congenital abnormalities of the heart [4]. Echocardiography is the initial modality of choice for the noninvasive detection of PAPVC [1].…”

Lutembacher's syndrome associated with meningioma

“…The incidence of MS in patients with ASD is 4% and the incidence of ASD in patients with MS is 0.6%-0.7% ( 6 ). Of the different diagnostic modalities available in the armamentarium for Lutembacher's syndrome, echocardiography seems to be the most efficient ( 7 ).…”