Intimal cushion

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The intimal cushion is smooth muscle in the center of arteries that paradoxically keep the artery patent by serving as valves. It is present in Helicine arteries of penis and in the patent ductus arteriosus. [1]

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Patent ductus arteriosus Condition wherein the ductus arteriosus fails to close after birth

Patent ductus arteriosus (PDA) is a medical condition in which the ductus arteriosus fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs by flowing from the aorta, which has a higher pressure, to the pulmonary artery. Symptoms are uncommon at birth and shortly thereafter, but later in the first year of life there is often the onset of an increased work of breathing and failure to gain weight at a normal rate. With time, an uncorrected PDA usually leads to pulmonary hypertension followed by right-sided heart failure.

Ligamentum arteriosum

The ligamentum arteriosum, also known as the Ligament of Botallo or Harvey's ligament, is a small ligament attaching the aorta to the pulmonary artery. It serves no function in adults but is the remnant of the ductus arteriosus formed within three weeks after birth.

dextro-Transposition of the great arteries Medical condition

dextro-Transposition of the great arteries, is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.

Ductus arteriosus Blood vessel connecting the pulmonary artery to the proximal descending aorta

The ductus arteriosus, also called the ductus Botalli, named after the Italian physiologist Leonardo Botallo, is a blood vessel in the developing fetus connecting the trunk of the pulmonary artery to the proximal descending aorta. It allows most of the blood from the right ventricle to bypass the fetus's fluid-filled non-functioning lungs. Upon closure at birth, it becomes the ligamentum arteriosum.

A cyanotic heart defect is any congenital heart defect (CHD) that occurs due to deoxygenated blood bypassing the lungs and entering the systemic circulation, or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is caused by structural defects of the heart such as right-to-left or bidirectional shunting, malposition of the great arteries, or any condition which increases pulmonary vascular resistance. The result may be the development of collateral circulation.

Coarctation of the aorta Medical condition

Coarctation of the aorta, also called aortic narrowing, is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus inserts. The word coarctation means "pressing or drawing together; narrowing". Coarctations are most common in the aortic arch. The arch may be small in babies with coarctations. Other heart defects may also occur when coarctation is present, typically occurring on the left side of the heart. When a patient has a coarctation, the left ventricle has to work harder. Since the aorta is narrowed, the left ventricle must generate a much higher pressure than normal in order to force enough blood through the aorta to deliver blood to the lower part of the body. If the narrowing is severe enough, the left ventricle may not be strong enough to push blood through the coarctation, thus resulting in a lack of blood to the lower half of the body. Physiologically its complete form is manifested as interrupted aortic arch.

Transposition of the great vessels Group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta

Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital heart diseases involving only the primary arteries belong to a sub-group called transposition of the great arteries (TGA), which is considered the most common congenital heart lesion that presents in neonates.

Ductus venosus

In the fetus, the ductus venosus shunts a portion of umbilical vein blood flow directly to the inferior vena cava. Thus, it allows oxygenated blood from the placenta to bypass the liver. Compared to the 50% shunting of umbilical blood through the ductus venosus found in animal experiments, the degree of shunting in the human fetus under physiological conditions is considerably less, 30% at 20 weeks, which decreases to 18% at 32 weeks, suggesting a higher priority of the fetal liver than previously realized. In conjunction with the other fetal shunts, the foramen ovale and ductus arteriosus, it plays a critical role in preferentially shunting oxygenated blood to the fetal brain. It is a part of fetal circulation.

Persistent truncus arteriosus Medical condition

Persistent truncus arteriosus (PTA), often referred to simply as Truncus Arteriosus, is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. This results in one arterial trunk arising from the heart and providing mixed blood to the coronary arteries, pulmonary arteries, and systemic circulation. For the International Classification of Diseases (ICD-11), the International Paediatric and Congenital Cardiac Code (IPCCC) was developed to standardize the nomenclature of congenital heart disease. Under this system, English is now the official language, and persistent truncus arteriosus should properly be termed Common arterial trunk.

Tricuspid atresia Medical condition

Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the systemic circulation to be filled with relatively deoxygenated blood. Because of this, hypoxia occurs, so other defects must occur to maintain blood flow. Because of the lack of an atrioventricular connection, an atrial septal defect (ASD) must be present to fill the left atrium and the left ventricle with blood. Since there is a lack of a right ventricle, there must be a way to pump blood into the pulmonary arteries, and this is accomplished by a ventricular septal defect (VSD). The causes of tricuspid atresia are unknown.

Fossa ovalis (heart)

The fossa ovalis is a depression in the right atrium of the heart, at the level of the interatrial septum, the wall between right and left atrium. The fossa ovalis is the remnant of a thin fibrous sheet that covered the foramen ovale during fetal development.

Foramen ovale (heart)

In the fetal heart, the foramen ovale, also foramen Botalli, or the ostium secundum of Born, 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. Another similar adaptation in the fetus is the ductus venosus. In most individuals, the foramen ovale closes at birth. It later forms the fossa ovalis.

Fetal circulation

In animals that give live birth, the fetal circulation is the circulatory system of a fetus. The term usually encompasses the entire fetoplacental circulation, which includes the umbilical cord and the blood vessels within the placenta that carry fetal blood.

Levo-Transposition of the great arteries is an acyanotic congenital heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed.

Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood.

Aortic arches

The aortic arches or pharyngeal arch arteries are a series of six paired embryological vascular structures which give rise to the great arteries of the neck and head. They are ventral to the dorsal aorta and arise from the aortic sac.

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch is often associated with DiGeorge syndrome.

Umbilical line

An umbilical line is a catheter that is inserted into one of the two arteries or the vein of the umbilical cord. Generally the UAC/UVC is used in Neonatal Intensive Care Units (NICU) as it provides quick access to the central circulation of premature infants. UAC/UVC lines can be placed at the time of birth and allow medical staff to quickly infuse fluids, inotropic drugs, and blood if required. It is sometimes used in term or near-term newborns in whom the umbilical cord stump is still connected to the circulatory system. Medications, fluids, and blood can be given through this catheter and it allows monitoring of blood gasses and withdrawing of blood samples. Transumbilical catheter intervention is also a method of gaining access to the heart, for example to surgically correct a patent ductus arteriosus.

Continuous murmurs

Heart murmurs are most frequently organized by timing, into systolic heart murmurs and diastolic heart murmurs. However, continuous murmurs can not be directly placed into either category.

Char syndrome is an autosomal dominant congenital disease caused by mutations in TFAP2B gene which affects the development of the bones of the face as well as the heart and limbs. During embryo development, TFAP2B regulates the production of the protein AP-2β, a transcription factor that is active in the neural crest and helps regulate genes that control cell division and apoptosis. There are at least 10 mutations of this gene that have been identified in people presenting Char syndrome, which alters specific regions of the gene preventing production of the transcription factor and disrupting normal development of embryo structures. People with this condition present a very distinct facial appearance with flattened cheek bones, flat and broad tip nose, shortened distance between the nose and upper lip, triangular-shaped mouth with tick lips and strabismus. It is also characterized by a patent ductus arteriosus, which is the failure to close the ductus that connects the aorta and pulmonary artery during pre-birth life and may cause many symptoms including breathing issues and heart failure. Abnormalities of hand and finger development have also been reported in people with this condition, including short or absent fifth finger. Other abnormal findings include supernumerary nipples. These conditions often affect multiple members of a family and there are no reports of non-genetic factors that might be related with incidence of this syndrome. It was first described by Florence Char in 1978.

References

  1. The paradoxical patent ductus arteriosus [ dead link ]