Atrioventricular conduction tissues in univentricular hearts of left ventricular type with absent right atrioventricular connection ('tricuspid atresia').

AbstractWe describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.

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The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialised conduction system

Cardiology in the Young ◽

10.1017/s1047951106000382 ◽

2006 ◽

Vol 16 (5) ◽

pp. 437-454 ◽

Cited By ~ 62

Author(s):

Audrey Smith ◽

Siew Yen Ho ◽

Robert H. Anderson ◽

M. Gwen Connell ◽

Robert Arnold ◽

...

Keyword(s):

Surgical Management ◽

Light Microscope ◽

Individual Case ◽

Conduction System ◽

Atrioventricular Conduction ◽

Cardiac Morphology ◽

Cardiac Malformations ◽

Segmental Approach ◽

Conduction Tissues

Congenital cardiac malformations which include isomerism of the atrial appendages are amongst the most challenging of problems for diagnosis and also for medical and surgical management. The nomenclature for pathological description is controversial, but difficulties can be overcome by the use of a segmental approach. Such an approach sets out the morphology and the topology of the chambers of the heart, together with the types and modes of the atrioventricular, ventriculo-arterial, and venous connections. We have applied this method to a study of 35 hearts known to have isomerism of the atrial appendages. We have already published accounts of 27 of these cases, but these were reviewed for this study in the light of our increased awareness of the implications of isomerism, and 8 new cases were added. After examining, or re-examining, the morphology of every heart in detail, we grouped them together according to their ventricular topology and modes of atrioventricular connection. Then we studied the course of the specialised conduction system, by the use of the light microscope, first in each individual case, and then together in their groups. We conclude that the pathways for atrioventricular conduction in hearts with isomerism of the atrial appendages are conditioned both by ventricular topology, and by the atrioventricular connections. Based on our experience, we have been able to establish guidelines that direct the clinician to the likely location of the conduction tissues.

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The morphology and diagnosis of atrioventricular septal defects

Cardiology in the Young ◽

10.1017/s1047951100010362 ◽

1991 ◽

Vol 1 (4) ◽

pp. 290-305 ◽

Cited By ~ 32

Author(s):

Robert H. Anderson ◽

Edward J. Baker ◽

Siew Yen Ho ◽

Michael L. Rigby ◽

Tjark Ebels

Keyword(s):

Ventricular Outflow Tract ◽

Left Ventricular ◽

Outflow Tract ◽

Normal Heart ◽

Atrioventricular Septal Defects ◽

Septal Defects ◽

Left Ventricular Outflow ◽

Normal Mitral Valve ◽

Atrioventricular Septation ◽

Conduction Tissues

SummaryIn this review, we discuss and describe those features which distinguish hearts with abnormal atrioventricular septation from the normal heart. The hearts, best described as atrioventricular septal defects, are unified by having a common atrioventricular junction guarded by a valve having five leaflets. The left component has three leaflets and cannot be interpreted in terms of a cleft in a normal mitral valve. The papillary muscles supporting this valve are also markedly dissimilar from the arrangement seen in normal hearts. The subaortic outflow tract is displaced superiorly compared to the normal heart, and is no longer wedged between the left valve and the septum. There is marked discrepancy in the inlet and outlet lengths of the ventricular mass, these dimensions being equal in hearts with normal atrioventricular septation. Although having the above features in common, atrioventricular septal defects show anatomic variations related to the arrangement of the bridging leaflets and their relationship to the septal structures. There may be a common valvar orifice or separate right and left orifices. The anatomic potential for shunting may be at atrial or ventricular levels, or both. Rarely, the septal structures may be intact. Other important features include ventricular dominance, the left ventricular outflow tract, and the disposition of the atrioventricular conduction tissues.

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Identification of the atrioventricular conduction axis and its positional relationship with anatomical landmarks of a heart with tricuspid atresia

JTCVS Open ◽

10.1016/j.xjon.2021.09.048 ◽

2021 ◽

Author(s):

Yuson Wada ◽

Hironori Matsuhisa ◽

Yoshihiro Oshima ◽

Naoki Yoshimura ◽

Yuson Wada ◽

...

Keyword(s):

Tricuspid Atresia ◽

Atrioventricular Conduction ◽

Anatomical Landmarks

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Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

Pediatric and Developmental Pathology ◽

10.1007/s100249910050 ◽

2000 ◽

Vol 3 (4) ◽

pp. 353-366 ◽

Cited By ~ 20

Author(s):

Silvio Litovsky ◽

Michael Choy ◽

Jeanny Park ◽

Mark Parrish ◽

Brenda Waters ◽

...

Keyword(s):

Right Ventricular ◽

Pulmonary Valve ◽

Left Ventricular ◽

Tricuspid Atresia ◽

Ventricular Free Wall ◽

Ventricular Septum ◽

Free Wall ◽

Absent Pulmonary Valve ◽

Right Ventricular Free Wall ◽

The Right

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.

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