Dextrocardia

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Dextrocardia
Situs inversus chest Nevit.jpg
Chest X ray of a person with dextrocardia situs inversus showing the cardiac apex pointing towards right
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Dextrocardia (from Latin dextro, meaning "right hand side," and Greek kardia, meaning "heart") is a rare congenital condition in which the apex of the heart is located on the right side of the body, rather than the more typical placement towards the left. [1] There are two main types of dextrocardia: dextrocardia of embryonic arrest (also known as isolated dextrocardia)[ citation needed ] and dextrocardia situs inversus . Dextrocardia situs inversus is further divided.

Contents

Classification

Dextrocardia of embryonic arrest

In this form of dextrocardia, the heart is simply placed further right in the thorax than is normal. It is commonly associated with severe defects of the heart and related abnormalities including pulmonary hypoplasia. [2]

Dextrocardia situs solitus

Dextrocardia refers to a heart positioned in the right side of the chest. Situs solitus describes viscera that are in the normal position, with the stomach on the left side. [3]

Dextrocardia situs inversus

Dextrocardia situs inversus refers to the heart being a mirror image situated on the right side. For all visceral organs to be mirrored, the correct term is dextrocardia situs inversus totalis. [4] [5]

Although statistically people with dextrocardia do not have any medical problems from the disorder, they may be prone to a number of bowel, esophageal, bronchial and cardiovascular disorders (such as double outlet right ventricle, endocardial cushion defect and pulmonary stenosis). [6] Certain cardiovascular and pulmonary disorders related to dextrocardia can be life-threatening if left unchecked.[ citation needed ]

Kartagener syndrome may also be present in patients with dextrocardia but this must be in the setting of situs inversus and may include male infertility.[ medical citation needed ]

Dextrocardia with situs ambiguus

In contrast to dextrocardia situs inversus which is only rarely associated with congenital heart disease, dextrocardia situs ambiguus is often associated with intracardiac anomalies. Dextrocardia situs ambiguus presents a surgical challenge not per se due to associated cardiac malformation, but because achieving adequate exposure is difficult. Right sided structures such as right atrium, right ventricle and tricuspid valve are oriented posteriorly in dextrocardia situs ambiguus (in contrast to dextrocardia with situs inversus). This presents a challenge to the surgeons operating on the right-sided cardiac structures in a case of dextrocardia situs ambiguus. [7]

Diagnosis

Medical diagnosis of the two forms of congenital dextrocardia can be made by ECG [2] or imaging.

Technical dextrocardia

Technical dextrocardia refers to an ECG reading that has no basis in the patient's anatomy. This apparent presentation is typically caused by the accidental lead placement of the left and right arm electrodes. Usually, this would show as an extreme axis deviation.[ citation needed ]

Management

ECG leads must be placed in reversed positions on a person with dextrocardia. In addition, when defibrillating someone with dextrocardia, the pads should be placed in reverse positions. [8] That is, instead of upper right and lower left, pads should be placed upper left and lower right. [9]

When heart transplantation is required in a person with situs inversus, reconstruction of the venous pathways to accommodate a normal donor heart is a major, but not insurmountable, challenge. [10]

Epidemiology

Dextrocardia is estimated to occur in approximately 1 in 12,019 pregnancies. [11]

A Japanese study of 1,753 fetal cardiac echocardiograms over five years revealed only two cases. [12]

Related Research Articles

<span class="mw-page-title-main">Tetralogy of Fallot</span> Type of congenital heart defect

Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:

<span class="mw-page-title-main">Situs inversus</span> Condition in which organs are reversed

Situs inversus is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement of internal organs is known as situs solitus. Although cardiac problems are more common, many people with situs inversus have no medical symptoms or complications resulting from the condition, and until the advent of modern medicine, it was usually undiagnosed.

<span class="mw-page-title-main">Atrial septal defect</span> Human heart defect present at birth

Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO). It is common in patients with a congenital atrial septal aneurysm (ASA).

<span class="mw-page-title-main">Congenital heart defect</span> Defect in the structure of the heart that is present at birth

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 heart defect is classed as a cardiovascular disease. Signs and symptoms depend on the specific type of defect. Symptoms can vary from none to life-threatening. When present, symptoms are variable and may include rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not cause chest pain. Most congenital heart defects are not associated with other diseases. A complication of CHD is heart failure.

<span class="mw-page-title-main">Ventricular septal defect</span> Medical condition

A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle. The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.

Situs ambiguus is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen. Clinically heterotaxy spectrum generally refers to any defect of Left-right asymmetry and arrangement of the visceral organs; however, classical heterotaxy requires multiple organs to be affected. This does not include the congenital defect situs inversus, which results when arrangement of all the organs in the abdomen and chest are mirrored, so the positions are opposite the normal placement. Situs inversus is the mirror image of situs solitus, which is normal asymmetric distribution of the abdominothoracic visceral organs. Situs ambiguus can also be subdivided into left-isomerism and right isomerism based on the defects observed in the spleen, lungs and atria of the heart.

<span class="mw-page-title-main">Hypoplastic left heart syndrome</span> Type of congenital heart defect

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital heart disease. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart. Several structures can be affected including the left ventricle, aorta, aortic valve, or mitral valve all resulting in decreased systemic blood flow.

<span class="mw-page-title-main">Transposition of the great vessels</span> Group of congenital heart defects

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.

<span class="mw-page-title-main">Pulmonary atresia</span> 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.

<span class="mw-page-title-main">Tricuspid atresia</span> 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. The causes of tricuspid atresia are unknown.

<span class="mw-page-title-main">Valvular heart disease</span> Disease in the valves of the heart

Valvular heart disease is any cardiovascular disease process involving one or more of the four valves of the heart. These conditions occur largely as a consequence of aging, but may also be the result of congenital (inborn) abnormalities or specific disease or physiologic processes including rheumatic heart disease and pregnancy.

<span class="mw-page-title-main">Atrioventricular septal defect</span> Medical condition

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 creates connections between all four of its chambers. It is a very specific combination of 3 defects:

<span class="mw-page-title-main">Situs solitus</span>

Situs solitus is the medical term referring to the normal position of thoracic and abdominal organs. Anatomically, this means that the heart is on the left with the pulmonary atrium on the right and the systemic atrium on the left along with the cardiac apex. Right-sided organs are the liver, the gall bladder and a trilobed lung as well as the inferior vena cava, while left-sided organs are the stomach, single spleen, a bilobed lung, and the aorta.

A right-to-left shunt is a cardiac shunt which allows blood to flow from the right heart to the left heart. This terminology is used both for the abnormal state in humans and for normal physiological shunts in reptiles.

<span class="mw-page-title-main">Right ventricular hypertrophy</span> Medical condition

Right ventricular hypertrophy (RVH) is a condition defined by an abnormal enlargement of the cardiac muscle surrounding the right ventricle. The right ventricle is one of the four chambers of the heart. It is located towards the lower-end of the heart and it receives blood from the right atrium and pumps blood into the lungs.

<span class="mw-page-title-main">Lutembacher's syndrome</span> Medical condition

Lutembacher's syndrome is a very rare form of congenital heart disease that affects one of the chambers of the heart as well as a valve. It is commonly known as both congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). Congenital atrial septal defect refers to a hole being in the septum or wall that separates the two atria; this condition is usually seen in fetuses and infants. Mitral stenosis refers to mitral valve leaflets sticking to each other making the opening for blood to pass from the atrium to the ventricles very small. With the valve being so small, blood has difficulty passing from the left atrium into the left ventricle. Septal defects that may occur with Lutembacher's syndrome include: Ostium primum atrial septal defect or ostium secundum which is more prevalent.

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.

<span class="mw-page-title-main">Bidirectional Glenn procedure</span>

The bidirectional Glenn (BDG) shunt, or bidirectional cavopulmonary anastomosis, is a surgical technique used in pediatric cardiac surgery procedure used to temporarily improve blood oxygenation for patients with a congenital cardiac defect resulting in a single functional ventricle. Creation of a bidirectional shunt reduces the amount of blood volume that the heart needs to pump at the time of surgical repair with the Fontan procedure.

<span class="mw-page-title-main">Hypoplastic right heart syndrome</span> Type of congenital heart disease

Hypoplastic right heart syndrome is a congenital heart defect in which the structures on the right side of the heart, particularly the right ventricle, are underdeveloped. This defect causes inadequate blood flow to the lungs, and thus a cyanotic infant.

Isolated levocardia is a rare type of organs' situs inversus in which the heart is still in normal position but other abdominal viscera are transposed. Isolated levocardia may occur with heart defects and patients without having operations have low life expectancy: only about 5% to 13% of patients survive more than 5 years. Therefore, even though the risk of cardiac surgeries is high, once patients are diagnosed, operations are suggested to be held as soon as possible. Isolated levocardia is congenital. So far, there is not sufficient evidence to prove that chromosome abnormalities will result in isolated levocardia, and the cause of isolated levocardia is still unknown.

References

  1. Stephenson, Susan (2012). Diagnostic Medical Sonography Obstetrics and Gynecology. 351 West Camden Street Baltimore, MD 21201: Lippincott Williams & Wilkins. p. 582.{{cite book}}: CS1 maint: location (link)
  2. 1 2 M. E. Abbott & J. C. Meakins (1915). "On the differentiation of two forms of congenital dextrocardia". Bulletin of the International Association of Medical Museums (5): 134–138.
  3. Leung, A. K.; Robson, W. L. (2006). "Dextrocardia with sinus solitus". Canadian Medical Association Journal. 175 (3): 244. doi:10.1503/cmaj.060721. PMC   1513419 . PMID   16880440.
  4. "Dextrocardia with Situs Inversus".
  5. Doshi, Ashish (5 May 2022). "Dextrocardia". Johns Hopkins Medicine. Johns Hopkins University. Retrieved 21 August 2022.
  6. MedlinePlus Medical Dictionary. "Dextrocardia".
  7. Mahajan, Sachin; Khanna, Sudhansoo; Kumar, Bhupesh; Halder, Vikram (2020). "Dextrocardia, situs solitus, inlet ventricular septal defect (VSD), and iatrogenic tricuspid regurgitation (TR)―A surgical perspective". Journal of Cardiac Surgery. 35 (6): 1383–1386. doi: 10.1111/jocs.14587 . ISSN   1540-8191. PMID   32349171. S2CID   217585359.
  8. Bindra, S. MD; Tabibiazar, R. MD; Mazar, M MD & Dave, R MD (2011). "Clinical Vignette: Dextrocardia with Situs Inversus: Through the Looking Glass with an ECG". Proceedings of UCLA Healthcare. Department of Medicine, UCLA. 15. Archived from the original on 2014-06-06. Retrieved 2013-05-14.
  9. Eddy, S. "Dextrocardia and Proper Lead Placement".
  10. Deuse, Tobias; Reitz, Bruce A. (September 2009). "Heart-Lung Transplantation In Situs Inversus Totalis". The Annals of Thoracic Surgery. 88 (3): 1002–1003. doi: 10.1016/j.athoracsur.2009.01.060 . PMID   19699943.
  11. Bohun CM, Potts JE, Casey BM, Sandor GG (July 2007). "A population-based study of cardiac malformations and outcomes associated with dextrocardia". Am. J. Cardiol. 100 (2): 305–9. doi:10.1016/j.amjcard.2007.02.095. PMID   17631088.
  12. Sato, T.; et al. (2014). "Clinical Course and Prognosis of Minor Abnormal Sonographic Findings in Fetal Echocardiography: Five Years of Experience at a Single Institute". Pediatric Cardiology and Cardiac Surgery (in Japanese and English). 30 (5): 563–568. doi: 10.9794/jspccs.30.563 .