Total anomalous pulmonary venous connection (TAPVC), also known as total anomalous pulmonary venous drainage (TAPVD) and total anomalous pulmonary venous return (TAPVR), is a rare cyanotic

Cyanotic heart defect

A cyanotic heart defect is a group-type of congenital heart defects . The patient appears blue , due to deoxygenated blood bypassing the lungs and entering the systemic circulation...

 congenital heart defect

Congenital heart defect

A congenital heart defect is a defect in the structure of the heart and great vessels which is present at birth. Many types of heart defects exist, most of which either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Other...

 (CHD) in which all four pulmonary vein

Pulmonary vein

The pulmonary veins are large blood vessels that carry blood from the lungs to the left atrium of the heart. In humans there are four pulmonary veins, two from each lung...

s are malpositioned and make anomalous connections to the systemic venous circulation

Circulatory system

The circulatory system is an organ system that passes nutrients , gases, hormones, blood cells, etc...

.(Normally, pulmonary venous return carries 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

Foramen ovale (heart)

In the fetal heart, the foramen ovale , also ostium secundum of Born or falx septi, allows blood to enter the left atrium from the right atrium. It is one of two fetal cardiac shunts, the other being the ductus arteriosus...

 or an atrial septal defect

Atrial septal defect

Atrial septal defect is a form of congenital heart defect that enables blood flow between the left and right atria via the interatrial septum. The interatrial septum is the tissue that divides the right and left atria...

 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.

Variations

There are four variants:

• Supracardiac (50%): blood drains to one of the innominate veins (brachiocephalic veins) or the superior vena cava
  Superior vena cava
  The superior vena cava is truly superior, a large diameter, yet short, vein that carries deoxygenated blood from the upper half of the body to the heart's right atrium...
  
  
• Cardiac (20%): blood drains into coronary sinus
  Coronary sinus
  The coronary sinus is a collection of veins joined together to form a large vessel that collects blood from the myocardium of the heart. It is present in all mammals, including humans...
  
   or directly into right atrium
• Infradiaphragmatic (20%): blood drains into portal or hepatic vein
  Hepatic vein
  In human anatomy, the hepatic veins are the blood vessels that drain de-oxygenated blood from the liver and blood cleaned by the liver into the inferior vena cava....
  
  s
• 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 enter the new vein as easily.

Clinical manifestation

• right ventricular heave
• fixed split S2
  Split S2
  A split S2 is a finding upon auscultation of the S2 heart sound.It is caused when the closure of the aortic valve and the closure of the pulmonary valve are not synchronized.-Physiologic split:...
  
  
• S3 gallop
• systolic ejection murmur
  Heart murmur
  Murmurs are extra heart sounds that are produced as a result of turbulent blood flow that is sufficient to produce audible noise. Most murmurs can only be heard with the assistance of a stethoscope ....
  
   at left upper sternal border
• cardiomegaly
  Cardiomegaly
  Cardiomegaly is a medical condition wherein the heart is enlarged. It is generally categorized in the following manner:* Cardiomegaly due to dilation* Cardiomegaly due to ventricular hypertrophy** Left ventricular hypertrophy...
  
  
• right axis deviation on ECG
• Snowman sign or `figure of 8 configuration` on chest radiograph
• right ventricular hypertrophy
  Right ventricular hypertrophy
  Right ventricular hypertrophy is a form of ventricular hypertrophy affecting the right ventricle.Blood travels through the right ventricle to the lungs via the pulmonary arteries. If conditions occur which decrease pulmonary circulation, meaning blood does not flow well from the heart to the...
  
  
• cyanosis
  Cyanosis
  Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...
  
  , tachypnea
  Tachypnea
  Tachypnea means rapid breathing. Any rate between 12-20 breaths per minute is normal. Tachypnea is a respiration rate greater than 20 breaths per minute. - Distinction from other breathing terms :...
  
  , dyspnea
  Dyspnea
  Dyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...
  
   since the overloaded pulmonary circuit can cause pulmonary edema

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

Patent ductus arteriosus

Patent ductus arteriosus is a congenital disorder in the heart wherein a neonate's ductus arteriosus fails to close after birth. Early symptoms are uncommon, but in the first year of life include increased work of breathing and poor weight gain...

allows oxygenated blood to go from the circulation of the right heart to the systemic circulation.

External links

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