Late correction of tetralogy of Fallot in children.

Paul P Heinisch,Alexander Kadner,Gabor Erdoes,Damian Hutter,Laetitia Guarino,Maris Bartkevics,Balthasar Eberle,Jaafar Rhissass,Jean-Pierre Pfammatter,Thierry Carrel,Carlos Royo

doi:10.4414/smw.2019.20096

Abstract

To report our experience of late correction after infancy in patients with tetralogy of Fallot (ToF). Observational single-centre retrospective analysis of the surgical techniques and perioperative development of patients from developing countries undergoing total surgical correction of ToF after infancy, between 1 November 2011 and 30 November 2016. Variables are presented as numbers with percentages or as mean ± standard deviation. Due to the setting of the humanitarian programme, clinical and echocardiographic follow-up procedures could be conducted for only one month postoperatively. Twenty-five children (mean age: 70.8 ± 42 months, range 23-163; 44% female) underwent total surgical correction of ToF. Two patients (0.8%) initially received a Blalock-Taussig shunt and underwent subsequent correction 24 and 108 months later, respectively. Preoperative mean right ventricular/pulmonary artery (RV/PA) gradient was 84 ± 32 mm Hg, with a Nakata index of 164 ± 71 mm2/m2. Major aortopulmonary collateral arteries (MAPCAs) were observed in eight children (32%), six (26%) of whom underwent transcatheter closure before surgery. 24 children (96%) underwent a valve-sparing pulmonary valve repair and one patient received a transannular patch (TAP). There were no cases which saw major adverse cardiac and cerebrovascular events (MACCE). Mean duration of mechanical ventilation was 28 ± 19.6 hours (range 7-76). Pre-discharge echocardiography demonstrated a mean RV/PA gradient of 25 ± 5.7 mm Hg, with left ventricular ejection fraction >60% in all cases. Overall length of hospital stay was 11.7 ± 4.5 days. There were no in-hospital mortality cases. Late surgical correction of ToF can be safely performed and produce highly satisfying early postoperative results comparable to those of classical “timely” correction. A valve-sparing technique can be applied in the majority of children.

Highlights

Surgical correction of Tetralogy of Fallot (ToF) is performed during infancy
Repair of tetralogy of Fallot (ToF) has many advantages: it reduces the duration of hypoxaemia and its negative sequelae, such as the development of cyanotic nephropathy, preserves myocardial function, allows early normalisation of pulmonary flow, which in turn stimulates angiogenesis in the pulmonary vascular bed and supports lung growth [5]
Due to limited data on late total repair, we investigated the surgical techniques, perioperative development and short-term outcomes following late corrective surgery for ToF in children

Summary

Introduction

Surgical correction of Tetralogy of Fallot (ToF) is performed during infancy. The majority of paediatric cardiac centres aim to achieve total correction by the age of nine months [1,2,3]. According to recent analysis of the Society of Thoracic Surgeons’ congenital database, the perioperative mortality rate lies at 1.3% [4]. Repair of ToF has many advantages: it reduces the duration of hypoxaemia and its negative sequelae, such as the development of cyanotic nephropathy, preserves myocardial function, allows early normalisation of pulmonary flow, which in turn stimulates angiogenesis in the pulmonary vascular bed and supports lung growth [5]. Chronic hypoxaemia, especially during the first year of life, may result in cognitive and developmental delay.

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Conclusion