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Total anomalous pulmonary venous connection

**{{{Name|Total anomalous pulmonary venous connection}}}**
[[Image:{{{Image}}}\|190px\|center\|]] {{{Caption\|}}}
ICD-10	Q26.2
ICD-O:	{{{ICDO}}}
ICD-9	747.41
OMIM	}}}
MedlinePlus	}}}
eMedicine	}}}
DiseasesDB	}}}

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 cyanoticcongenitalheart defect(CHD) in which all four pulmonary veinsare malpositionedand make anomalousconnections to the systemic venouscirculation. (Normally, pulmonary venous return carries oxygenated blood to the left atrium and to the rest of the body). A patent foramen ovaleshunt must be present in order to obtain systemic blood flow.

Inhaltsverzeichnis

1 Variations
2 Clinical manifestation
3 Treatment
4 External links

Variations

There are four variants:

Supracardiac (50%): blood drains to innominate veinor the superior vena cava
Cardiac (20%): blood drains into coronary sinusor directly into right atrium
Infradiaphragmatic (20%): blood drains into portalor 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.

Clinical manifestation

Clinically, the symptoms of TAPVC is similar to that of atrial septal defect (since both disorders involves the mixing of pulmonary blood into the right atrium):

right ventricular heave
fixed split S2
S3 gallop
systolic ejection murmurat left upper sternal border
cardiomegaly
right axis deviation on ECG
right ventricular hypertrophy
cyanosis, tachypnea, dyspneasince the overloaded pulmonary circuit can cause pulmonary edema

Treatment

In TAPVC without obstruction, surgical redirection can be performed within the first month of life. With obstruction, surgery should be undertaken emergently. PGE1should not be given because a patent ductus arteriosusadds even more volume into the already overloaded pulmonary flow.

External links

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

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Total+anomalous+pulmonary+venous+connection Wikipedia article Total anomalous pulmonary venous connection.

All text is available under the terms of the GNU Free Documentation License