Cardiac Surgery in Trisomy 13 and 18: A Guide to Clinical Decision-Making
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REVIEW ARTICLE
Cardiac Surgery in Trisomy 13 and 18: A Guide to Clinical Decision‑Making Horacio G. Carvajal1 · Connor P. Callahan2 · Jacob R. Miller1 · Bethany L. Rensink1 · Pirooz Eghtesady1 Received: 6 July 2020 / Accepted: 25 August 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract There has been substantial controversy regarding treatment of congenital heart defects in infants with trisomies 13 and 18. Most reports have focused on surgical outcomes versus expectant treatment, and rarely there has been an effort to consolidate existing evidence into a more coherent way to help clinicians with decision-making and counseling families. An extensive review of the existing literature on cardiac surgery in patients with these trisomies was conducted from 2004 to 2020. The effects of preoperative and perioperative factors on in-hospital and long-term mortality were analyzed, as well as possible predictors for postoperative chronic care needs such as tracheostomy and gastrostomy. Patients with minimal or no preoperative pulmonary hypertension and mechanical ventilation undergoing corrective surgery at a weight greater than 2.5 kg suffer from lower postoperative mortality. Infants with lower-complexity cardiac defects are likely to benefit the most from surgery, although their expected mortality is higher than that of infants without trisomy. Omphalocele confers an increased mortality risk regardless of cardiac surgery. Gastrointestinal comorbidities increased the risk of gastrostomy tube placement, while those with prolonged mechanical ventilation and respiratory comorbidities are more likely to require tracheostomy. Cardiac surgery is feasible in children with trisomies 13 and 18 and can provide improved long-term results. However, this is a clinically complex population, and both physicians and caretakers should be aware of the long-term challenges these patients face following surgery when discussing treatment options. Keywords Trisomy 13 · Trisomy 18 · Cardiac surgery · Risk factors · Outcomes
Introduction Trisomy 13 (T13) and trisomy 18 (T18) are the two most common aneuploidies after trisomy 21, with a reported prevalence of 1.42/10,000 and 3.19/10,000 pregnancies, respectively, in the United States [1, 2]. Both syndromes have been considered “universally lethal,” with around 70% of first-trimester pregnancies diagnosed with T18 and nearly 50% of those diagnosed with T13 resulting in fetal demise [3, 4]. Mortality remains high among live newborns, with a reported survival of 11.5% for T13 and 13.4% for T18 at * Pirooz Eghtesady [email protected] 1
Section of Pediatric Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine in St. Louis/St. Louis Children’s Hospital, Saint Louis, MO, USA
Department of Surgery, Barnes-Jewish Hospital, Washington University School of Medicine in St. Louis, Saint Louis, MO, USA
2
one year of age [5]. The primary causes of death are either respiratory or cardiovascular, although medical interventions h
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