Total anomalous pulmonary venous connection

	Total anomalous pulmonary venous connection Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Total anomalous pulmonary venous connection from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X Ray
MRI
CT
Echocardiography
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Prevention
Case Studies
Case #1
Total anomalous pulmonary venous connection On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Total anomalous pulmonary venous connection All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Total anomalous pulmonary venous connection
CDC on Total anomalous pulmonary venous connection
Total anomalous pulmonary venous connection in the news
Blogs on Total anomalous pulmonary venous connection
Directions to Hospitals Treating Type page name here
Risk calculators and risk factors for Total anomalous pulmonary venous connection

	Total anomalous pulmonary venous connection;
	Common pulmonary vein
ICD-10	Q26.2
ICD-9	747.41

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]

Overview

Total anomalous pulmonary venous connection (TAPVC), also known as total anomalous pulmonary venous drainage(TAPVD) and total anamalous pulmonary venous return(TAPVR), is a rare cyanotic congenital heart defect (CHD) in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation. (Normally, pulmonary venous return carries oxygenated blood to the left atrium and to the rest of the body). A patent foramen ovale or an atrial septal defect must be present in order to allow systemic blood flow.

Variations

There are four variants:

Supracardiac (50%): blood drains to one of the innominate veins (brachiocephalic veins) or the superior vena cava
Cardiac (20%): blood drains into coronary sinus or directly into right atrium
Infradiaphragmatic (20%): blood drains into portal or hepatic veins
Mixed (10%)

TAPVC can occur with obstruction, which occurs when the anomalous vein enters a vessel at an acute angle and can cause pulmonary venous hypertension and cyanosis because blood cannot easily enter the new vein as easily.

Diagnosis

Symptoms

cyanosis, tachypnea, dyspnea since the overloaded pulmonary circuit can cause pulmonary edema

Physical Examination

right ventricular heave
fixed split S2
S3 gallop
systolic ejection murmur at left upper sternal border
cardiomegaly

Electrocardiographic Findings

right axis deviation on ECG
right ventricular hypertrophy

Cardiac Catheterization

Treatment

In TAPVC without obstruction, surgical redirection can be performed within the first month of life. With obstruction, surgery should be undertaken emergently. PGE1 should not be given because a patent ductus arteriosus adds even more volume into the already overloaded pulmonary flow.

References

External links

Total Anomalous Pulmonary Venous Return information from Seattle Children's Hospital Heart Center

Additional Reading

Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843

Template:WH Template:WS