Tetralogy of Fallot with absent pulmonary valve: A single center retrospective review

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Context and Aim: Absent pulmonary valve syndrome (APVS) in association with tetralogy of Fallot (TOF) is a rare congenital heart disease characterized by the aneurysmal dilatation of pulmonary arteries and associated major airway anomalies. Postoperative outcomes have been suboptimal, especially concerning dependency on positive pressure ventilation and the need for repeated cardiac reinterventions. This study aims to describe the postoperative course of these patients and to identify the risk factors associated with poor outcomes. Methods and Results: A retrospective review of 38 patients who underwent surgery for TOF with APVS between 2001 and 2021 at a single cardiac center was conducted. There were 16 (42%) patients in the young infants group. They required longer postoperative mechanical ventilation, 77 (interquartile ranges [IQR] 22–306) versus 17 (IQR 6–67) hours for the older children ( P -0.009); longer inotropic support, 127 (103–285) versus 60 (20–108) hours for older children ( P -0.000); longer intensive care unit (ICU) stay, 12 (4–29) versus 4 (2–7) days ( P -0.01), and longer hospital stay 23 (14–64) versus 15 (11–20) days ( P -0.02). Seven (43%) of the younger infants’ group required readmission, whereas 4 (18%) of the older children needed to be readmitted ( P < 0.04). Bloodstream infections (BSIs) occurred more frequently in the infants’ group (31%) compared to older children (4.5%) ( P < 0.03). The need for redo surgeries and the incidence of hospital-acquired infections other than BSIs did not show differences between the groups. Conclusions: Age <3 months or weight <4 kg is associated with a more difficult postoperative course, characterized by longer ventilation days, a higher inotropic score, longer ICU and hospital stays, and more readmissions.

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