Cardiovascular System - Tricuspid Atresia
|Embryology - 20 Apr 2019 Expand to Translate|
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- 1 LA89.1 Tricuspid Atresia
LA89.1 Tricuspid Atresia
|LA89.1 Tricuspid atresia|
Complete lack of formation of the tricuspid valve which results in an hypoplastic right ventricle. The pulmonary circulation can be maintained via a VSD, and an ASD is necessary for survival. Results in cyanosis and tachypnoea. Treatment is initially via administration of prostaglandins followed by surgery to place a shunt to maintain the pulmonary circulation.
Some Recent Findings
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|The image shows a congenitally malformed heart in which both the right and left atrioventricular valves (RAVV, LAVV) are connected with the dominant left ventricle. The right ventricle is incomplete, and is supplied through a ventricular septal defect. Note that, in this heart, the aorta arises from the incomplete right ventricle, and the pulmonary trunk from the dominant left ventricle. This is the arrangement usually described as “transposition”, but better accounted for in terms of discordant ventriculo-arterial connections.||The image shows the lesion known as classical tricuspid atresia. There has been failure of expansion of the atrioventricular junctions, so that the floor of the right is separated from the roof of the right ventricle by the right atrioventricular groove (dashed black lines). Only the dominant left ventricle has an inlet, with the blood entering the incomplete right ventricle through the ventricular septal defect (star).||The image shows a heart dissected to reveal the structure of the incomplete right ventricle. In this heart, as in most example of tricuspid atresia, it gives rise to the pulmonary trunk. The ventricular septal defect (VSD) is restrictive in this heart.
Images from Anderson (2016)
||The first western clinical description of ventricular septal defects was made by Henri Roger in 1879, which later became known as maladie de Roger.
His description was based upon 6 acyanotic patients and autopsy finding of a child with ventricular septal defect.
(Heath-Edward grade) A pathological grading system for pulmonary artery structural changes that occur with congenital cardiac septal defects. The classification is named after the two original paper authors Donald HEATH and Jessee EDWARDS and grades from I to VI with increasing severity of the arterial changes.
- Grade I - hypertrophy of the media of small muscular arteries and arterioles.
- Grade II - intimal cellular proliferation in addition to medial hypertrophy.
- Grade III - advanced medial thickening with hypertrophy and hyperplasia including progressive intimal proliferation and concentric fibrosis. Results in an obliteration of the arterioles and small arteries.
- Grade IV - "plexiform lesions" of the muscular pulmonary arteries and arterioles with a plexiform network of capillary-like channels within a dilated segment.
- Grade V - complex plexiform, angiomatous and cavernous lesions and hyalinization of intimal fibrosis.
- Grade VI - necrotizing arteritis.
Heart defects and preterm birth are the most common causes of neonatal and infant death. The long-term development of the heart combined with extensive remodelling and post-natal changes in circulation lead to an abundance of abnormalities associated with this system.
A UK study literature showed that preterm infants have more than twice as many cardiovascular malformations (5.1 / 1000 term infants and 12.5 / 1000 preterm infants) as do infants born at term and that 16% of all infants with cardiovascular malformations are preterm. (0.4% of live births occur at greater than 28 weeks of gestation, 0.9% at 28 to 31 weeks, and 6% at 32 to 36 weeks. Overall, 7.3% of live-born infants are preterm)
"Baltimore-Washington Infant Study data on live-born cases and controls (1981-1989) was reanalyzed for potential environmental and genetic risk-factor associations in complete atrioventricular septal defects AVSD (n = 213), with separate comparisons to the atrial (n = 75) and the ventricular (n = 32) forms of partial AVSD. ...Maternal diabetes constituted a potentially preventable risk factor for the most severe, complete form of AVSD." 
In addition, there are in several congenital abnormalities that exist in adults (bicuspid aortic valve, mitral valve prolapse, and partial anomalous pulmonary venous connection) which may not be clinically recognized.
- Anderson RH. Teratogenecity in the setting of cardiac development and maldevelopment. (2016)
- <pubmed>20895864</pubmed>| PMC2104017 | PDF
Search Pubmed: Ventricular Septal Defect
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Cite this page: Hill, M.A. (2019, April 20) Embryology Cardiovascular System - Tricuspid Atresia. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Cardiovascular_System_-_Tricuspid_Atresia
- © Dr Mark Hill 2019, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G