Crisscross heart

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Crisscross heart
Other namesCriss-cross atrioventricular relationships [1]
Specialty Cardiology

Crisscross heart is a type of congenital heart defect where the right atrium is closely associated with the left ventricle in space, and the left atrium is closely associated with the right ventricle. [2]

Contents

Although it is classified as a defect, the criss-cross is more of a spatial anomaly than a functional one, and it is possible for the heart to have relatively normal functioning. The ventricles are rotated either clockwise or counterclockwise resulting in the twisting of their connection. [3] The actual blood flow stream through the ventricles is not interrupted.

Symptoms and signs

Crisscross heart is a very rare congenital heart defect, and results in many different symptoms, even though the heart still has the ability to perform its major function of pumping blood throughout the body. Individuals who have this disease will experience cyanosis which is a blue tint to the skin because of inadequate blood flow to the body, this symptom will be seen especially around the mouth. Other symptoms include pallor, extreme dyspnea, pulmonary valve stenosis, cardiac murmurs [4] and a deviated ventricular septum. [5] Pallor can be described as a pale color of the skin, and dyspnea is difficulty breathing. Pulmonary valve stenosis is the narrowing of the pulmonary valve which leads to decreased blood flow to the pulmonary artery. Cardiac murmurs are sounds that can be heard when using a stethoscope that make a swooshing noise rather than a normal “lub-dup”. Lastly a deviated ventricular septum is when there is a hole between the ventricle walls resulting in blood between the ventricles flowing freely between each other. [6]

Anatomy

In an anatomically correct heart the right atrium and right ventricle are working together to supply blood to the pulmonary artery, similarly to how the left atrium and the left ventricle work simultaneously to supply blood to the aorta. During the process of the heart contracting and releasing the right atrium and left atrium contract at the same time, while the left ventricle and right ventricle relax. In opposition, when the left atrium and right atrium are relaxed the left ventricle and right ventricle contract pushing blood to either the aorta or pulmonary artery. In an anatomically correct heart the atria are smaller than the ventricles. The ventricles include more muscle in order to push high quantities of blood throughout the body. Normal blood flow throughout the heart begins at the superior vena cava coming from the upper half of the body and the inferior vena cava coming from the lower half of the body. Next blood will be in the right atrium and will flow uninterrupted through the tricuspid valve through to the right ventricle. The blood from the right ventricle should go to the pulmonary artery via the pulmonary valve. The blood from the pulmonary vein enters the left atrium, then flows through the mitral valve to the left ventricle. After the left ventricle is filled with blood the aortic valve opens allowing blood to go through, which the blood then enters the aorta and goes to the rest of the body. [7]

Diagnosis

Crisscross heart can be diagnosed by an echocardiogram, angiocardiogram, and a cardiac MRI. To diagnose crisscross heart during embryonic development a sonogram will be used, and it is very important identify the disease prenatally so the child can be treated immediately for better cardiac function in adulthood. [8]

Treatment

Without treatment crisscross heart can be fatal. The treatment is aimed to fix a deviated septum and pulmonary valve stenosis rather than the rotated heart itself.[ citation needed ]

Related Research Articles

Heart Muscular organ responsible for pumping blood through the circulatory system in most animals

The heart is a muscular organ in most animals, which pumps blood through the blood vessels of the circulatory system. The pumped blood carries oxygen and nutrients to the body, while carrying metabolic waste such as carbon dioxide to the lungs. In humans, the heart is approximately the size of a closed fist and is located between the lungs, in the middle compartment of the chest.

Coronary circulation

Coronary circulation is the circulation of blood in the blood vessels that supply the heart muscle (myocardium). Coronary arteries supply oxygenated blood to the heart muscle. Cardiac veins then drain away the blood after it has been deoxygenated. Because the rest of the body, and most especially the brain, needs a steady supply of oxygenated blood that is free of all but the slightest interruptions, the heart is required to function continuously. Therefore its circulation is of major importance not only to its own tissues but to the entire body and even the level of consciousness of the brain from moment to moment. Interruptions of coronary circulation quickly cause heart attacks, in which the heart muscle is damaged by oxygen starvation. Such interruptions are usually caused by coronary ischemia linked to coronary artery disease, and sometimes to embolism from other causes like obstruction in blood flow through vessels.

Heart valve A flap of tissue that prevent backflow of blood around the heart

A heart valve is a one-way valve that normally allows blood to flow in only one direction through the heart. Four valves are usually present in a mammalian heart and together they determine the pathway of blood flow through the heart. A heart valve opens or closes incumbent on differential blood pressure on each side.

Systole

The systole is the part of the cardiac cycle during which some chambers of the heart muscle contract after refilling with blood. The term originates, via New Latin, from Ancient Greek συστολή (sustolē), from συστέλλειν, and is similar to the use of the English term to squeeze.

Ventricle (heart) Chamber of the heart

A ventricle is one of two large chambers toward the bottom of the heart that collect and expel blood received from an atrium towards the peripheral beds within the body and lungs. The atrium primes the pump.

Afterload Pressure in the wall of the left ventricle during ejection

Afterload is the pressure that the heart must work against to eject blood during systole. Afterload is proportional to the average arterial pressure. As aortic and pulmonary pressures increase, the afterload increases on the left and right ventricles respectively. Afterload changes to adapt to the continually changing demands on an animal's cardiovascular system. Afterload is proportional to mean systolic blood pressure and is measured in millimeters of mercury.

Atrium (heart) Upper chamber in the heart for blood to enter through

The atrium or auricle is the upper chamber through which blood enters the ventricles of the heart. There are two atria in the human heart – the left atrium receives blood from the pulmonary (lung) circulation, and the right atrium receives blood from the venae cavae. The atria receive blood while relaxed (diastole), then contract (systole) to move blood to the ventricles. All animals with a closed circulatory system have at least one atrium. Humans have two atria.

The Rastelli procedure is an open heart surgical procedure developed by Italian physician and cardiac surgery researcher, Giancarlo Rastelli in 1967 at the Mayo Clinic, Ajmer and involves using a pulmonary or aortic homograft conduit to relieve pulmonary obstruction in double outlet right ventricle with pulmonary stenosis.

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.

A transthoracic echocardiogram (TTE) is the most common type of echocardiogram, which is a still or moving image of the internal parts of the heart using ultrasound. In this case, the probe is placed on the chest or abdomen of the subject to get various views of the heart. It is used as a non-invasive assessment of the overall health of the heart, including a patient's heart valves and degree of heart muscle contraction. The images are displayed on a monitor for real-time viewing and then recorded.

Pulmonary atresia Medical condition

Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close

Cardiac cycle

The cardiac cycle is the performance of the human heart from the beginning of one heartbeat to the beginning of the next. It consists of two periods: one during which the heart muscle relaxes and refills with blood, called diastole, following a period of robust contraction and pumping of blood, called systole. After emptying, the heart immediately relaxes and expands to receive another influx of blood returning from the lungs and other systems of the body, before again contracting to pump blood to the lungs and those systems. A normally performing heart must be fully expanded before it can efficiently pump again. Assuming a healthy heart and a typical rate of 70 to 75 beats per minute, each cardiac cycle, or heartbeat, takes about 0.8 seconds to complete the cycle. There are two atrial and two ventricle chambers of the heart; they are paired as the left heart and the right heart—that is, the left atrium with the left ventricle, the right atrium with the right ventricle—and they work in concert to repeat the cardiac cycle continuously,. At the start of the cycle, during ventricular diastole–early, the heart relaxes and expands while receiving blood into both ventricles through both atria; then, near the end of ventricular diastole–late, the two atria begin to contract, and each atrium pumps blood into the ventricle below it. During ventricular systole the ventricles are contracting and vigorously pulsing two separated blood supplies from the heart—one to the lungs and one to all other body organs and systems—while the two atria are relaxed. This precise coordination ensures that blood is efficiently collected and circulated throughout the body.

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.

A cardiac shunt is a pattern of blood flow in the heart that deviates from the normal circuit of the circulatory system. It may be described as right-left, left-right or bidirectional, or as systemic-to-pulmonary or pulmonary-to-systemic. The direction may be controlled by left and/or right heart pressure, a biological or artificial heart valve or both. The presence of a shunt may also affect left and/or right heart pressure either beneficially or detrimentally.

Double inlet left ventricle Medical condition

A double inlet left ventricle (DILV) or "single ventricle", is a congenital heart defect appearing in 5 in 100,000 newborns, where both the left atrium and the right atrium feed into the left ventricle. The right ventricle is hypoplastic or does not exist.

The following outline is provided as an overview of and topical guide to cardiology, the branch of medicine dealing with disorders of the human heart. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in cardiology are called cardiologists.

Hypoplastic right heart syndrome is a congenital heart defect in which the right atrium and right ventricle are underdeveloped. This defect causes inadequate blood flow to the lungs and thus, a blue or cyanotic infant.

Heart development

Heart development refers to the prenatal development of the heart. This begins with the formation of two endocardial tubes which merge to form the tubular heart, also called the primitive heart tube. The heart is the first functional organ in vertebrate embryos.

Bernheim syndrome

Bernheim Syndrome is a presumed disorder whereby the right ventricle is severely compressed due to a shift in the ventricular septal wall of the heart leading to heart failure. It was first described by Hippolyte Bernheim in 1910. Today it is questioned whether or not Bernheim Syndrome is its own syndrome or a side effect of other cardiac conditions such as left ventricular heart failure whereby the left ventricle is substantially enlarged which encroaches on the space of the right ventricle.

References

  1. RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Criss cross heart". www.orpha.net. Retrieved 27 May 2019.
  2. Fontes VF, de Souza JA, Pontes Jùnior SC (March 1990). "Criss-cross heart with intact ventricular septum". International Journal of Cardiology. 26 (3): 382–5. doi:10.1016/0167-5273(90)90102-B. PMID   2312210.
  3. Angelini P (2010). "Left ventricle on top versus right ventricle on top in superoinferior ventricles: what are we talking about?". Texas Heart Institute Journal. 37 (4): 442–4. PMC   2929877 . PMID   20844618.
  4. "Gale - Product Login". go.galegroup.com. Retrieved 2018-10-25.
  5. RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Criss cross heart". www.orpha.net. Retrieved 2018-10-25.
  6. Taksande AM (April 2013). "Echocardiographic recognition of a criss-cross heart with double outlet right ventricle". Images in Paediatric Cardiology. 15 (2): 3–7. PMC   3669543 . PMID   23847656.
  7. Philadelphia, The Children's Hospital of (2014-08-10). "How the Normal Heart Works". www.chop.edu. Retrieved 2018-10-25.
  8. Li S, Luo G, Norwitz ER, Wang C, Ouyang S, Yao Y, Wen H, Chen C, Fu Q, Xia X, Bi J, Zhu J (February 2013). "Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases". Journal of Perinatology. 33 (2): 98–102. doi: 10.1038/jp.2012.56 . PMID   22555779.

Further reading

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External resources