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Membranous subaortic stenosis is a CHD with high recurrence-rate despite surgical treatment. This study investigated the outcome of operated patients and possible predictors for recurrence.
Methods:
Retrospective review of all patients (n = 38) ≤ 18 years of age operated for membranous subaortic stenosis between 1994–2019 at Sahlgrenska University Hospital. The primary outcomes were recurrence, reintervention, and mortality. Predictors of recurrence and reintervention were secondary outcomes.
Results:
Median age (range) at diagnosis, initial intervention, and last follow-up were 2.3 (0.003–17.2), 5.3 (0.03–17.5) and 17.5 (3.6–20.4) years, respectively. Median follow-up time was 9.9 (0.01–19.5) years. 61% were males, and 53% had other associated CHD. 19 patients (56%) developed recurrence and 7 (21%) underwent reintervention. One patient died peri-operatively. Age <5 years at first intervention increased the likelihood of reintervention. Postoperative peak/mean gradients were higher in patients with disease recurrence.
The median echocardiographic peak-/mean gradients at initial diagnosis, pre-, postoperative, and at last follow-up were 61/36, 83/50, 16/8, and 19/17 mmHg respectively (p < 0.0001 pre/post). Pre-/postoperative peak gradients were linearly correlated, decreasing by 80% pre-/postoperatively (p < 0.01). Presence of symptoms and the preoperative peak gradient were positively associated (p < 0.001) with a peak gradient threshold value of > 90 mmHg. The distance between the subaortic stenosis membrane and the aortic valve was inversely correlated to the preoperative peak-gradient (p < 0.01).
Conclusions:
Reintervention following surgical intervention of membranous subaortic stenosis is common. A positive correlation exists between high pre- and postoperative peak-gradient. A low postoperative peak gradient may be important in avoiding recurrence.
Edited by
James Ip, Great Ormond Street Hospital for Children, London,Grant Stuart, Great Ormond Street Hospital for Children, London,Isabeau Walker, Great Ormond Street Hospital for Children, London,Ian James, Great Ormond Street Hospital for Children, London
The majority of paediatric surgery carried out is for minor procedures in fit and healthy children and can be performed as day-case procedures. A large quantity of this work is performed in non-specialist hospitals. Children and families need to be able to access high-quality services close to home, and delivering care locally, where possible to do so safely, can add to the patient and parent experience. A non-specialist centre should have arrangements and local guidance for treating and managing simple surgical emergencies in the paediatric population, along with the ability to resuscitate and stabilise critically ill children of all ages before transfer to a tertiary specialist centre for either paediatric critical care or surgery. Continual education and training within regional networks are vital in maintaining skills and confidence of staff in non-specialist centres, and standardisation along with protocols is helpful in the anaesthetic management, analgesic plan, preassessment and critical transfers of children in a non-specialist centre.
This chapter covers selected topics which illustrate the basis for modern general paediatric anaesthesia practices. Many paediatric procedures can be undertaken on a day case basis which has tremendous benefits for children and families. Clear written, verbal and pictorial information concerning fasting, surgery, anaesthesia, analgesia and postoperative care are an essential part of obtaining informed consent from parents and children. The use of topical local anaesthesia of the skin with EMLA cream or amethocaine gel allows painless venous access after 60 and 45 minutes. Sevoflurane is now the most often used agent for induction in children because it is more pleasant and less irritant than other volatile agents. Regional anaesthesia produces excellent postoperative analgesia and attenuation of the stress response in infants and children. Although formal evidence-based systematic reviews or meta-analyses are relatively few, there is now a fairly robust basis for modern paediatric anaesthesia techniques and practices.
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