Prenatal physiologic findings and postnatal advanced imaging in the management of absent pulmonary valve syndrome with intact ventricular septum

2018 ◽  
Vol 35 (10) ◽  
pp. 1695-1697 ◽  
Author(s):  
Emilia Maria C. Cadiz ◽  
Michele M. Pasierb ◽  
Stephen J. Dolgner ◽  
Bhawna Arya
Keyword(s):  
Pulmonary Valve ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
Advanced Imaging ◽  
Absent Pulmonary Valve Syndrome

Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome

2004 ◽  
Vol 14 (4) ◽  
pp. 402-408 ◽  
Author(s):  
Nili Zucker ◽  
Ilya Rozin ◽  
Aviva Levitas ◽  
Eli Zalzstein
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Clinical Presentation ◽  
Pulmonary Valve ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
History Of ◽  
Absent Pulmonary Valve Syndrome

So-called ‘absent pulmonary valve syndrome’ is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.

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 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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