anatomy Anatomy () is the branch of morphology concerned with the study of the internal structure of organisms and their parts. Anatomy is a branch of natural science that deals with the structural organization of living things. It is an old scien ...

, a persistent left

superior vena cava The superior vena cava (SVC) is the superior of the two venae cavae, the great venous trunks that return deoxygenated blood from the systemic circulation to the right atrium of the heart. It is a large-diameter (24 mm) short length vei ...

is the most common variation of the

thoracic The thorax (: thoraces or thoraxes) or chest is a part of the anatomy of mammals and other tetrapod animals located between the neck and the abdomen. In insects, crustaceans, and the extinct trilobites, the thorax is one of the three main ...

venous system Veins () are blood vessels in the circulatory system of humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are those of the pulmonary and fetal c ...

. It is present in between 0.3% and 0.5% of the population, and is an

embryologic Embryology (from Greek ἔμβρυον, ''embryon'', "the unborn, embryo"; and -λογία, ''-logia'') is the branch of animal biology that studies the prenatal development of gametes (sex cells), fertilization, and development of embryos and ...

remnant that results from a failure to

involute In mathematics, an involute (also known as an evolvent) is a particular type of curve that is dependent on another shape or curve. An involute of a curve is the Locus (mathematics), locus of a point on a piece of taut string as the string is eith ...

Presentation

In persistent left superior vena cava, the left

brachiocephalic vein The left and right brachiocephalic veins (previously called innominate veins) are major veins in the Thorax, upper chest, formed by the union of the ipsilateral internal jugular vein and subclavian vein (the so-called venous angle) behind the ster ...

does not develop fully and the left

upper limb The upper Limb (anatomy), limbs or upper extremities are the forelimbs of an upright posture, upright-postured tetrapod vertebrate, extending from the scapulae and clavicles down to and including the digit (anatomy), digits, including all the musc ...

and

head A head is the part of an organism which usually includes the ears, brain, forehead, cheeks, chin, eyes, nose, and mouth, each of which aid in various sensory functions such as sight, hearing, smell, and taste. Some very simple ani ...

and

neck The neck is the part of the body in many vertebrates that connects the head to the torso. It supports the weight of the head and protects the nerves that transmit sensory and motor information between the brain and the rest of the body. Addition ...

drain into the

right atrium The atrium (; : atria) is one of the two upper chambers in the heart that receives blood from the circulatory system. The blood in the atria is pumped into the heart ventricles through the atrioventricular mitral and tricuspid heart valves. ...

via the

coronary sinus The coronary sinus () is the largest vein of the heart. It drains over half of the deoxygenated blood from the heart muscle into the right atrium. It begins on the backside of the heart, in between the left atrium, and left ventricle; it begi ...

. In isolation, the variation is considered benign, but is very frequently associated with cardiac abnormalities (e.g.

ventricular septal defect A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. It's a common heart problem present at birth ( congenital heart defect). The extent of the opening may vary ...

atrioventricular septal defect Atrioventricular septal defect (AVSD) or atrioventricular canal defect (AVCD), also known as "common atrioventricular canal" or "endocardial cushion defect" (ECD), is characterized by a deficiency of the atrioventricular septum of the heart that c ...

) that have a significant mortality and

morbidity A disease is a particular abnormal condition that adversely affects the structure or function of all or part of an organism and is not immediately due to any external injury. Diseases are often known to be medical conditions that are asso ...

. It is more frequent in patients with

congenital heart defect A congenital heart defect (CHD), also known as a congenital heart anomaly, congenital cardiovascular malformation, and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital h ...

s. The (right)

is almost always unaffected by the presence of persistent left superior vena cava.

Diagnosis

If an anomaly is detected during a routine

ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...

, a

fetal A fetus or foetus (; : fetuses, foetuses, rarely feti or foeti) is the unborn offspring of a viviparous animal that develops from an embryo. Following the embryonic stage, the fetal stage of development takes place. Prenatal development is a ...

echocardiogram Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an echo ...

is performed to determine whether a fetus has the condition. Otherwise, it is often unnoticed unless an extenuating circumstance warrants further examination of the heart, usually much later in life. CT and

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...

scans in a parasagittal section may show a "pipe" sign where the left superior vena cava occurs.

Treatment

If no other cardiac abnormalities are present, persistent left superior vena cava will not be treated, as it is usually

asymptomatic Asymptomatic (or clinically silent) is an adjective categorising the medical conditions (i.e., injuries or diseases) that patients carry but without experiencing their symptoms, despite an explicit diagnosis (e.g., a positive medical test). P ...

and unharmful. If it drains into the

left atrium The atrium (; : atria) is one of the two upper chambers in the heart that receives blood from the circulatory system. The blood in the atria is pumped into the heart ventricles through the atrioventricular mitral and tricuspid heart valves. ...

, then

deoxygenated blood Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells. Blood is compo ...

enters the circulation to the body, and

cyanosis Cyanosis is the change of Tissue (biology), tissue color to a bluish-purple hue, as a result of decrease in the amount of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Cyanosis is apparent usually in the Tissue (bi ...

may occur.

References

External links

{{Congenital vascular defects Congenital vascular defects