eISSN: 2449-6731
ISSN: 2449-6723
Prenatal Cardiology
Current issue Archive About the journal Editorial board Abstracting and indexing Contact Instructions for authors Ethical standards and procedures
Editorial System
Submit your Manuscript
Original paper

Absent pulmonary valve syndrome – special prenatal care and early surgery in obstetric/cardiac center - a new hope for postnatal survivors?

Katarzyna Zych-Krekora
Michał Krekora
Marek Kopala
Maria Respondek-Liberska
1, 4

  1. Prenatal Cardiology Department Research Institute Polish Mother’s Memorial Hospital in Łódź, Poland
  2. Obstetrics and Gynecology Department, Research Institute Polish Mother’s Memorial Hospital in Łódź, Poland
  3. Cardiosurgery Department , Research Institute Polish Mother’s Memorial Hospital in Łódź, Poland
  4. Fetal Malformations Department, Medical University of Lodz
Prenat Cardio 2017 Jan; 7(1):19-25
Online publish date: 2019/07/16
View full text Get citation
APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 – 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality.

Material and methods
In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome.

Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05).

There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.


Absent pulmonary valve syndrome, survivability, surgery, prenatal echocardiography, monitoring

Quick links
© 2024 Termedia Sp. z o.o.
Developed by Bentus.