Surgical Outcomes for Patients with Turner Syndrome.

To the Editor, We read with great interest the report by Cramer et al. [3] on the spectrum of congenital heart disease (CHD) and outcomes after surgical repair in children with Turner syndrome (TS). In their retrospective single-center review, the authors showed that although patients with TS undergoing aortic arch repair have a longer operative and postoperative course, their outcomes appear similar to those of non-TS subjects. Recent papers [1, 8] have identified a vasculopathy in TS patients involving intimal and medial thickening of large arteries that can lead to progressive aortic dilation, dissection, and even rupture. These arterial characteristics can explain the friability of the aortic wall, with higher risk of hemorrhages at surgery [2, 9] and after stent implantation [4] as described in the literature. It is noteworthy that although these findings constitute a potential increased risk of surgical complications, no patients reported by Cramer et al. [3] died due to the intervention, and only one patient required early reintervention for recoarctation. In line with these results, we observed the same outcome in our small cohort of six TS patients who underwent surgical coarctation repair. No surgical mortality or morbidity occurred, and during the long-term follow-up period, none of the patients had a residual systolic pressure gradient higher than 10 mmHg detected by serial echocardiography. Currently, overall data [2, 3, 9] suggest that the association with TS is not a specific risk factor for mortality among patients who have undergone surgery for aortic coarctation. However, specific caution should be observed relative to the possibility of perioperative bleeding and “a more challenging early postoperative course” [3]. Moreover, as reported in our recent study [10], it is important to consider that these patients can have not only cardiovascular malformations but also an impaired cardiopulmonary response to exercise and impaired systolic and diastolic function of the left ventricle. These further findings can be considered a specific cardiac phenotype of TS subjects and may represent additional risk factors for perioperative complications or for postoperative morbidity. Studies increasingly report on perioperative outcomes for patients with CHD and genetic syndromes such as trisomy 21 and del 22q11.2 with the aim of detecting the impact not only of specific anatomic cardiac patterns but also of extracardiac anomalies on the intensive care unit stay and surgical results [6, 7]. We underscore the necessity of recognizing specific surgical risk factors for syndromic patients with CHD that can lead to the preparation of specific diagnostic and perioperative protocols to reduce operative mortality and morbidity. The stratification of risks based on genotyping is an encouraging perspective in cardiovascular medicine, and the shift from a genotype–phenotype correlation toward a genotype prognosis paradigm currently is available also in pediatric cardiology [5].