Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle

2019 ◽  
Vol 30 (1) ◽  
pp. 126-128
Author(s):  
Cheul Lee ◽  
Kyung Min Kim ◽  
Jae Young Lee ◽  
Jihong Yoon
Keyword(s):  
Right Ventricle ◽  
Pulmonary Valve ◽  
Tricuspid Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
Cavopulmonary Anastomosis ◽  
Complete Exclusion ◽  
Bidirectional Cavopulmonary Anastomosis ◽  
The Right

AbstractTricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

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

2000 ◽  
Vol 3 (4) ◽  
pp. 353-366 ◽  
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.

Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum

2016 ◽  
Vol 27 (2) ◽  
pp. 391-393
Author(s):  
Yasunobu Miki ◽  
Toshikatsu Tanaka ◽  
Yoshihiro Oshima
Keyword(s):  
Pulmonary Artery ◽  
Pulmonary Valve ◽  
Main Pulmonary Artery ◽  
Pulmonary Regurgitation ◽  
Right Ventricular Outflow Tract ◽  
Tricuspid Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
Bilateral Pulmonary Artery Banding

AbstractAbsent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock–Taussig shunt at 11 days of age.

scholarly journals Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum

1991 ◽  
Vol 101 (2) ◽  
pp. 222-229 ◽  
Author(s):  
William G. Williams ◽  
Patricia Burrows ◽  
Robert M. Freedom ◽  
George A. Trusler ◽  
John G. Coles ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
The Right
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