Partial anomalous pulmonary venous connection
- David R Fulton, MD
David R Fulton, MD
- Section Editor — Pediatric Cardiology
- Associate Professor of Pediatrics
- Harvard Medical School
- Brian Soriano, MD
Brian Soriano, MD
- Associate Professor of Pediatrics & Adjunct Associate Professor of Radiology
- Heart Center, Seattle Children’s Hospital
- University of Washington School of Medicine
Partial anomalous pulmonary venous connection (PAPVC), also known as partial anomalous pulmonary venous return, encompasses a specific group of congenital cardiovascular anomalies that are caused by the abnormal return of one or more, but not all, of the pulmonary veins directly to the right atrium or indirectly through a variety of venous connections from the anomalous pulmonary vein.
The anatomic abnormalities that result in partial anomalous pulmonary connection (PAPVC), and the diagnosis and management of PAPVC will be reviewed here.
The overall incidence of partial anomalous pulmonary venous connection (PAPVC) is estimated to be 0.7 percent of the population . However, as this rate is based upon autopsy data, the true prevalence of PAPVC may actually be higher [2-5]. Multiple case series report PAPVC as an incidental finding, which in most of these instances is asymptomatic.
Although PAPVC can present as an isolated structural abnormality, it commonly occurs with other cardiac abnormalities, most often an atrial septal defect. In addition, patients with Turner syndrome are at increased risk for PAPVC [6,7]. (See "Clinical manifestations and diagnosis of Turner syndrome".)
There is a wide range of pulmonary vein arrangements that return blood from the lung to the heart.
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- ANATOMIC VARIATIONS
- Partial anomalous pulmonary venous connections
- - Scimitar syndrome
- Partial anomalous pulmonary venous drainage
- - Sinus venosus defects
- - Malposition of the septum primum
- CLINICAL MANIFESTATIONS
- Associated cardiac and pulmonary defects
- - Atrial septal defect
- - Presentation of scimitar syndrome
- Isolated PAPVC
- Physical examination
- Initial tests
- - Chest radiography
- Magnetic resonance imaging
- Computed tomography
- Cardiac catheterization
- - Electrocardiogram
- Scimitar syndrome
- Other causes
- SUMMARY AND RECOMMENDATIONS