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Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum: How to counsel the parents?


1 Pediatric Cardiology Unit, Department of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura, Egypt; Pediatric Cardiology Unit, Department of Pediatrics, King Abdul-Aziz University, Jeddah, Saudi Arabia
2 Pediatric Cardiology Unit, Department of Pediatrics, King Abdul-Aziz University, Jeddah, Saudi Arabia

Correspondence Address:
Dr. Shaimaa Rakha
Pediatric Cardiology Unit, Mansoura University Children Hospital, El Gomhouria St, Mansoura, Dakahlia Governorate 35516

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_101_19

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Year : 2020  |  Volume : 13  |  Issue : 2  |  Page : 136-140

 

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Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a different challenging process than with fetal Fallot type diagnosis. Moreover, 22q11 deletion has never been found as an association. Parents must also be aware of the expected early heart failure in cases with large patent ductus arteriosus and the need for its closure as early as possible.






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1 Pediatric Cardiology Unit, Department of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura, Egypt; Pediatric Cardiology Unit, Department of Pediatrics, King Abdul-Aziz University, Jeddah, Saudi Arabia
2 Pediatric Cardiology Unit, Department of Pediatrics, King Abdul-Aziz University, Jeddah, Saudi Arabia

Correspondence Address:
Dr. Shaimaa Rakha
Pediatric Cardiology Unit, Mansoura University Children Hospital, El Gomhouria St, Mansoura, Dakahlia Governorate 35516

Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_101_19

Rights and Permissions

Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a different challenging process than with fetal Fallot type diagnosis. Moreover, 22q11 deletion has never been found as an association. Parents must also be aware of the expected early heart failure in cases with large patent ductus arteriosus and the need for its closure as early as possible.






[FULL TEXT] [PDF]*


        
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