Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital defect of the pulmonary veins.

Total anomalous pulmonary venous connection

''Total anomalous pulmonary venous connection'', also known as total ''anomalous pulmonary venous return'', is a rare

cyanotic Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...

congenital A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...

heart defect in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous

circulation Circulation may refer to: Science and technology * Atmospheric circulation, the large-scale movement of air * Circulation (physics), the path integral of the fluid velocity around a closed curve in a fluid flow field * Circulatory system, a bio ...

. (Normally, pulmonary veins return oxygenated blood from the lungs to the left atrium where it can then be pumped to the rest of the body). A patent

foramen ovale There are multiple structures in the human body with the name foramen ovale (plural: ''foramina ovalia''; Latin for "oval hole"): * Foramen ovale (heart), in the fetal heart, a shunt from the right atrium to left atrium * Foramen ovale (skull), at ...

, patent ductus arteriosus or an atrial septal defect ''must'' be present, or else the condition is fatal due to a lack of systemic blood flow. In some cases, it can be detected prenatally. There are four variants: Supracardiac (50%): blood drains to one of the

innominate veins The left and right brachiocephalic veins (previously called innominate veins) are major veins in the upper chest, formed by the union of each corresponding internal jugular vein and subclavian vein. This is at the level of the sternoclavicular jo ...

(brachiocephalic veins) or the superior vena cava; Cardiac (20%), where blood drains into coronary sinus or directly into right atrium; Infradiaphragmatic (20%), where blood drains into portal or hepatic veins; and a mixed (10%) variant. 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 enter the new vein as easily.

Signs and symptoms

* right ventricular heave * Loud S1 * fixed split S2 *

S3 gallop The third heart sound or S3 is a rare extra heart sound that occurs soon after the normal two "lub-dub" heart sounds (S1 and S2). S3 is associated with heart failure. Physiology It occurs at the beginning of the middle third of diastole, approxima ...

* systolic ejection murmur at left upper sternal border * cardiomegaly * right axis deviation on ECG * Snowman sign or 'figure of 8 configuration' on chest radiograph * right ventricular hypertrophy *

cyanosis Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...

, tachypnea, dyspnea since the overloaded pulmonary circuit can cause pulmonary edema * ''Cottage-loaf sign'', that is,

chest X-ray A chest radiograph, called a chest X-ray (CXR), or chest film, is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common film taken in med ...

appearance similar to a cottage loaf, also known as the 'snow man' sign or 'figure of 8' sign.

Treatment

In TAPVC without obstruction, surgical redirection can be performed within the first month of life. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed. With obstruction, surgery should be undertaken emergently. PGE1 should be given because a patent ductus arteriosus allows oxygenated blood to go from the circulation of the right heart to the systemic circulation.

Partial anomalous pulmonary venous connection

A ''Partial anomalous pulmonary venous connection'' (or ''Partial anomalous pulmonary venous drainage'' or ''Partial anomalous pulmonary venous return'') is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins. It is less severe than

total anomalous pulmonary venous connection Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital defect of the pulmonary veins. Total anomalous pulmonary venous connection ''Total anomalous pulmonary venous con ...

which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. In less severe cases, with smaller amounts of blood flow, diagnosis may be delayed until adulthood, when it can be confused with other causes of pulmonary hypertension. There is also evidence that a significant number of mild cases are never diagnosed, or diagnosed incidentally. It is associated with other vascular anomalies, and some genetic syndromes such as Turner syndrome.

Diagnosis

It can be diagnosed with

CT scan A computed tomography scan (CT scan; formerly called computed axial tomography scan or CAT scan) is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers ...

, angiography, transesophageal

echocardiography An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in t ...

, or

cardiac MRI Cardiac magnetic resonance imaging (cardiac MRI), also known as cardiovascular MRI, is a magnetic resonance imaging (MRI) medical imaging, technology used for non-invasive assessment of the function and structure of the cardiovascular system. Condi ...

. Unfortunately, less invasive and expensive testing, such as transthoracic echocardiography and CT scanning are generally less sensitive.

Treatment

It is sometimes treated with surgery, which involves rerouting blood from the right atrium into the left atrium with a patch or use of the Warden procedure. However, interest is increasing in catheter-based interventional approaches, as well as medical therapy for less severe cases.

References

External links

{{Congenital vascular defects Congenital vascular defects