RT Journal Article
SR Electronic
T1 Long-term outcomes in children with absent pulmonary valve syndrome: it is not just fixing the heart
JF Archives of Disease in Childhood
JO Arch Dis Child
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 877
OP 881
DO 10.1136/archdischild-2020-320219
VO 106
IS 9
A1 Katalin Torok
A1 Elizabeth Brettle
A1 Tarak Desai
A1 Paul Miller
A1 Natasha Khan
A1 John Stickey
A1 Prasad Nagakumar
A1 Anna Seale
YR 2021
UL http://adc.bmj.com/content/106/9/877.abstract
AB Objective Absent pulmonary valve syndrome (APV) is a rare condition usually associated with tetralogy of Fallot (TOF). Some infants develop respiratory failure from bronchial compression and the long-term neurodevelopmental outcome is unknown. We aimed to investigate the outcomes of APV and the need for long-term ventilation (LTV).Design, patients and setting Retrospective single-centre review of patients diagnosed with APV between 2007 and 2017.Outcome measures Survival, neurological disability and postoperative LTV (≥3 months of non-invasive or invasive respiratory support).Results Thirty patients were identified, 22 (73%) of whom were prenatally diagnosed. Pregnancy was discontinued in one patient, while in utero death occurred in three. One was lost to follow-up. Of the remaining 25 liveborn, 21 had the classic TOF/APV. One baby died immediately after birth, while two patients had palliative care due to severe airway compression and inability to wean ventilation support. Surgical repair was performed in 21 of the 25 (84%) liveborn, with one awaiting surgery. Of those undergoing surgery, two patients died: one during surgery and the other due to severe airway malacia 5 months postsurgery. In the surgical group survival from birth at 1 and 5 years was 89% (95% CI 75% to 100%). Six (30%) patients required LTV postoperatively; all had surgery within the first 6 months of life. Learning and/or other physical difficulties were evident in 63%.Conclusions Majority of patients with APV are diagnosed antenatally. A third of those operated required LTV and over half had learning and/or other physical difficulties. Prospective studies are needed to identify prenatal factors that predict postnatal outcomes so parents can be counselled appropriately.Data are available upon reasonable request.