‘Absent’ pulmonary valve with intact ventricular septum mimicking tricuspid valve atresia: Prenatal diagnosis and postnatal course

2019 ◽  
Vol 45 (3) ◽  
pp. 714-718
Author(s):  
Francesca Monacci ◽  
Tommaso Bondi ◽  
Clementina Canessa ◽  
Enrico Chiappa
Keyword(s):  
Prenatal Diagnosis ◽  
Tricuspid Valve ◽  
Pulmonary Valve ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve

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.

Pulmonary atresia with intact ventricular septum: a multicenter analysis of 21 prenatal diagnosis cases

2017 ◽  
Author(s):  
M.V. Medvedev , M.V. Kubrina , O.L. Galkina et all
Keyword(s):  
Prenatal Diagnosis ◽  
Aortic Arch ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Right Atrial ◽  
Intact Ventricular Septum ◽  
Multicenter Analysis ◽  
The Right ◽  
Atrial Isomerism

A retrospective analysis of 21 cases of prenatal diagnosed of pulmonary atresia with intact ventricular septum (PA-IVS) is presented. In the study of the four chambers view of the fetal heart the right ventricle was hypoplastic in 16 (76.1 %), normal in 5 (23.8 %) cases. Tricuspid atresia identified in 8 (38 %) cases. The dysplasia of the tricuspid valve identified in 5 (23.8 %) cases. In 2 (8.7 %) cases PA-IVS were in combination with atrioventricular defects. The changes of the tricuspid valve were not identified in 6 (28.5 %) cases. In 100 % cases reduced pulmonary artery with retrograde flow in the pulmonary valve was registrated. Hydrops fetal is detected in 2 (9.5 %) cases. The left aortic arch was in 17 (80.9 %) cases and right aortic arch – 4 (19.1 %). The average gestational age at prenatal diagnosis was 22,6 weeks (range 11–32 weeks). Early diagnosis is represented by one case in 11 weeks. Cardiac defects were registered in 6 (28.5 %) cases and extracardiac defects in 5 (23.8 %) cases. Ventriculocoronary arterial communications were found in 2 (9.5 %) cases of PA-IVS. In 3 (13 %) cases were in combination with heterotaxy syndrome. In 2 (8.7 %) cases PA-IVS were in combination with left atrial isomerism and 1 (4.8 %) with right atrial isomerism. Outcomes in fetuses with PA-IVC: termination of pregnancy — 13 (61.9 %), fetal death — 2 (9.5 %), neonatal death — 5 (23.8 %) cases, only 1 (4.8 %) survivor.

Absent Pulmonary Valve with Intact Ventricular Septum Presenting as Cardiorespiratory Failure at Birth

1997 ◽  
Vol 18 (2) ◽  
pp. 136-138 ◽  
Author(s):  
H. Horigome ◽  
Y. Sakakibara ◽  
N. Atsumi ◽  
T. Miyamoto ◽  
H. Sato
Keyword(s):  
Pulmonary Valve ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
Cardiorespiratory Failure

Absent pulmonary valve or pulmonary atresia with intact ventricular septum: Which is it?

2020 ◽  
Vol 37 (11) ◽  
pp. 1869-1872
Author(s):  
Erin Nealon ◽  
Darren Berman ◽  
Daniel Rowland ◽  
Brian Boe ◽  
Eric Lloyd ◽  
...  
Keyword(s):  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve

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.

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