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[Absence of right and left atrioventricular connexion]
L Muñoz-Castellanos1, M Kuri Nivon, C A Vázquez Antona
1Departamento de embriología, Instituto Nacional de Cardiología, Ignacio Chávez, INCICH, Juan Badiano No. 1, 14080 México D.F.
Insights
This study details congenital heart defects involving absent atrioventricular (A-V) connections, revealing common anatomical features and associated anomalies. Findings correlate cardiac morphology with imaging, offering insights into developmental origins of these complex conditions.
Area of Science:
- Congenital heart disease
- Cardiac morphology
- Developmental cardiology
Context:
- Absence of atrioventricular (A-V) connection is a rare congenital anomaly.
- Understanding the spectrum of associated cardiac malformations is crucial for diagnosis and management.
- Morphological and imaging correlations are essential for characterizing these defects.
Purpose:
- To morphologically characterize hearts with absent atrioventricular (A-V) connections.
- To identify and classify associated ventriculoarterial connections and anomalies.
- To correlate anatomical findings with echocardiographic and cardioangiographic data.
Summary:
- Fifty-seven hearts with absent A-V connections were studied, revealing common features like deep A-V sulcus, ventricular septal defects, and atrial septal defects.
- Ventriculoarterial connections varied, with concordant connections in right absent A-V connection and discordant connections in left absent A-V connection.
- Associated anomalies included pulmonary stenosis, pulmonary atresia, aortic atresia, and double outlet from the ventricle.
- The study proposes that this cardiopathy results from unequal lateralization of common atrioventricular canal septation.
Impact:
- Precise correlations between cardiac morphology and imaging were established.
- Provides a detailed anatomical classification of hearts with absent A-V connections.
- Offers insights into the developmental etiology of these complex congenital heart defects.
- Enhances understanding for improved diagnostic accuracy and potential therapeutic strategies.
Abstract:
Fifty seven hearts with absence of atrioventricular (A-V) connection were studied morphologically to specify their types of ventriculoarterial connection and their associated anomalies; the anatomic features of the hearts were correlated with their echocardiographic and cardioangiographic images in order to establish their mutual correspondence. Fifty six hearts had situs solitus; fifty specimens had right absent A-V connection and six had left absent A-V connection. One had situs inversus. All the specimens had: A deep A-V sulcus at the site of the absent A-V connection, a dimple in the muscular floor of the involved atrium connected with the dilated and hypertrophic left ventricle, incomplete right ventricle without inlet portion, ventricular septal defect of variable dimensions (it was obliterated in two), atrial septal defect, the ventricular septum deviated from the crux cordis. The left absent A-V connection had ventricular inversion and discordant ventriculoarterial connection. In the right absent A-V connection the ventriculoarterial connections were concordant in thirty eight hearts, from which thirty four had pulmonary stenosis both infundibular and valvular (five had the tetrad of Fallot), two had pulmonary valve atresia and two had a dilated pulmonary artery; discordant in nine hearts, one with aortic atresia; double outlet, from the right ventricle in two, (one with the tetrad of Fallot) and from the left ventricle in one. The heart in situs inversus had ventricular inversion, right absent A-V connection (left-sided), single (right) ventricle and atresia of the left ventricle. The correlations between cardiac morphology and imaging were precise. Developmentally, this cardiopathy is the result of an ectopic unequally lateralized septation of the common atrioventricular canal, which separates two canals, one stenotic leading to atresia and the other which develops too wide.