Tricuspid atresia

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Tricuspid atresia
Other namesTri atresia [1]
Tricuspid atresia.svg
Anterior (frontal) view of the opened heart in tricuspid atresia with ASD+VSD. White arrows indicate blood flow. (Atresic tricuspid valve labeled at bottom left.)
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Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. [2] Therefore, there is an absence of right atrioventricular connection. [2] 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. [3]

Contents

In most cases of tricuspid atresia, additional defects exist to allow exchange of blood between the loops of systematic circulation and pulmonary circulation, filling in the role of the missing atrioventricular connection. An atrial septal defect (ASD) must be present to fill the left atrium and the left ventricle with blood. [4] Since there is a lack of a right ventricle, there must also be a way to pump blood into the pulmonary artery. This can be accomplished by a ventricular septal defect (VSD) connecting the left ventricle to the pulmonary artery or by a patent ductus arteriosus (PDA) connecting the aorta to the pulmonary artery. In the latter case, prostaglandin E1 is used to maintain the PDA connection until emergency corrective surgery can be completed. As oxygenated blood is mixed with deoxygenated blood in both cases, there is a reduction in the oxygen-carrying capacity. [4]

It is also possible for tricuspid atresia to appear without the life-saving defects. In this case, the systemic and pulmonary circulations would be cut off from each other and no useful breathing can occur. An experimental procedure called fetal balloon atrial septostomy can be used to artificially create the required defect in utero. [5]

Presentation

Cause

Tricuspid atresia is caused by complete absence of the tricuspid valve. [2] The underlying cause of this absence remains unknown. [3] This prevents direct blood flow between the right atrium and the right ventricle. [2] This usually causes the foramen ovale to remain open after birth, leading to atrial septal defect. [4]

Pathophysiology

As there is no communication between the right atrium and the right ventricle, there must be an atrial septal defect to allow blood to flow into the left cardiac chambers. Due to the lack of blood flow into the right ventricle, it will be hypoplastic. In most cases, there will also be a ventricular septal defect allowing some blood into the pulmonary circulation. Due to the lack of blood flow into the pulmonary circulation, there is poor oxygenation of blood, leading to progressively worsening cyanosis. [6]

Diagnosis

The majority of cases can be diagnosed prenatally during a routine anomaly scan. If evidence of a congenital heart disease is found, the diagnosis can be confirmed by a foetal echocardiogram.

If it is not diagnosed prenatally, it may be diagnosed shortly after birth with physical examination, which would reveal cyanosis and murmur. Further evidence for the diagnosis can be obtained with an electrocardiogram and a chest radiograph. ECG will typically show a left axis deviation, while the chest X-ray may show pulmonary oligaemia or hyperaemia. The definitive investigation is, as in all congenital heart diseases, an echocardiogram, although the aforementioned tests along with clinical features might be sufficient for most cases. [6]

Treatment

Treatment is based on:

Epidemiology

Tricuspid atresia is the third most common critical congenital heart defect. [2] It is estimated to cause between 1% and 3% of all congenital heart defects. [8]

Related Research Articles

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

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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.

<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.

<span class="mw-page-title-main">Fontan procedure</span> Surgical procedure used in children with univentricular hearts

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<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

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

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<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:

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<span class="mw-page-title-main">Anomalous pulmonary venous connection</span> Medical condition

Anomalous pulmonary venous connection is a congenital heart defect of the pulmonary veins. It can be a total anomalous pulmonary venous connection, wherein all four pulmonary veins are incorrectly positioned, or a partial anomalous pulmonary venous connection, wherein only some of the pulmonary veins are incorrectly positioned.

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

Hypoplastic right heart syndrome (HRHS) 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.

<span class="mw-page-title-main">Pulmonary atresia with ventricular septal defect</span> Type of congenital heart defect

Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract.

Raghib syndrome is rare a congenital heart defect where the left superior vena cava (LSVC) is draining into the left atrium in addition to an absent coronary sinus and an atrial septal defect. This can be considered a dangerous heart condition because it puts the individual at a high risk of stroke. Other defects that are often associated with Raghib syndrome can include ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. While this is considered an extremely rare developmental complex, cases regarding a persistent left superior vena cava (PLSVC) are relatively common among congenital heart defects. It is also important to note that the PLSVC often drains into the right atrium, and only drains into the left atrium in approximately 10 to 20% of individuals with the defect.

References

  1. ↑ "Tricuspid atresia: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 28 May 2019.
  2. 1 2 3 4 5 Murthy, Raghav; Nigro, John; Karamlou, Tara (2019-01-01), Ungerleider, Ross M.; Meliones, Jon N.; Nelson McMillan, Kristen; Cooper, David S. (eds.), "65 - Tricuspid Atresia" , Critical Heart Disease in Infants and Children (Third Edition), Philadelphia: Elsevier, pp. 765–777.e3, doi:10.1016/b978-1-4557-0760-7.00065-6, ISBN   978-1-4557-0760-7, S2CID   214741527 , retrieved 2020-11-27
  3. 1 2 "Congenital Heart Defects — Facts about Tricuspid Atresia | CDC". 2019-01-22.
  4. 1 2 3 4 Lok, Josephine M.; Spevak, Philip J.; Nichols, David G. (2006-01-01), Nichols, David G.; Ungerleider, Ross M.; Spevak, Philip J.; Greeley, William J. (eds.), "Chapter 39 - Tricuspid Atresia", Critical Heart Disease in Infants and Children (Second Edition), Philadelphia: Mosby, pp. 799–822, ISBN   978-0-323-01281-2 , retrieved 2020-12-05
  5. ↑ Plackett, Benjamin (25 November 2021). "The surgical solution to congenital heart defects". Nature. 599 (7886): S21. doi: 10.1038/d41586-021-03517-z .
  6. 1 2 3 Sumal, Anoop S.; Kyriacou, Harry; Mostafa, Ahmed M.H.A.M. (2020). "Tricuspid atresia: Where are we now?". Journal of Cardiac Surgery. 35 (7): 1609–1617. doi: 10.1111/jocs.14673 . PMID   32484582. S2CID   219172518.
  7. ↑ Aykanat, Alper; Yavuz, Taner; Özalkaya, Elif; Topçuoğlu, Sevilay; Ovalı, Fahri; Karatekin, Güner (2016). "Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease". Pediatric Cardiology. 37 (1): 131–134. doi:10.1007/s00246-015-1251-0. PMID   26260095. S2CID   12433727.
  8. ↑ Hoffman Julien I.E; Kaplan Samuel (2002-06-19). "The incidence of congenital heart disease". Journal of the American College of Cardiology. 39 (12): 1890–1900. doi: 10.1016/S0735-1097(02)01886-7 . PMID   12084585.
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