To evaluate outcomes in patients with Turner Syndrome, especially those with cardiac conditions, compared to those without Turner syndrome.

Retrospective cohort study utilising hospitalisation data from 2006 to 2012. Conditional logistic regression models are used to analyse outcomes of interest: all-cause mortality, increased length of stay, and discharge to home.

We identified 2978 women with Turner syndrome, matched to 11,912 controls by primary diagnosis.

Patients with Turner syndrome were more likely to experience inpatient mortality (odds ratio 1.44, 95% confidence interval 1.02–2.02, p = 0.04) and increased length of stay (OR 1.31, CI 1.18–1.46, p = 0.03) than primary diagnosis matched controls, after adjusting for age, race, insurance status, and Charlson comorbidity index. Patients with Turner syndrome were 32% less likely to be discharged to home (OR 0.68, CI 0.60–0.78, p < 0.001). When restricting the sample of patients to those admitted with a cardiac diagnosis, the likelihood of mortality (OR 3.10, CI 1.27–7.57, p = 0.01) and prolonged length of stay (OR 1.42, CI 1.03–1.95, p = 0.03) further increased, while the likelihood of discharge to home further decreased (OR 0.55, CI 0.38–0.80, p = 0.001) in Turner syndrome compared to primary diagnosis matched controls. Specifically, patients with congenital heart disease were more likely to have prolonged length of stay (OR: 1.53, CI 1.18–2.00, p = 0.002), but not increased mortality or decreased discharge to home.

Hospitalised women with Turner syndrome carry a higher risk of adverse outcomes even when presenting otherwise similarly as controls, an important consideration for those treating them in these settings.

Isolated aortic coarctation performed through a left thoracotomy resection and end-to-end anastomosis results in low mortality and morbidity rates. Recoarctation and late hypertension are among the most important complications after such repairs. In this study, we reviewed the results of children who underwent left-side thoracotomy to correct an isolated aortic coarctation.

A consecutive sample of 90 patients who underwent resection and extended end-to-end anastomosis through a left-side thoracotomy in our centre between 2011 and 2021 was retrospectively analysed. The patients' preoperative characteristics, operative data, and post-operative early and long-term results were examined

All patients underwent resection and extended end-to-end anastomosis. A pulmonary artery band was applied simultaneously to three (3.3%) patients, and an aberrant right subclavian artery division was applied to one (1.1%) patient. The mean cross-clamp time was 29.13 ± 6.97 minutes. Two (2.2%) patients required reoperation in the early period. Mortality was observed in one (1.1%) patient in the early period. Eight (8.8%) patients developed recoarctation, of whom four (4.4%) underwent reoperation and four (4.4%) underwent balloon angioplasty. Twenty-two (26.8%) patients received follow-up antihypertensive treatment. The mean follow-up period was 41.3 ± 22.8 months. No mortality was observed in the late period.

Isolated coarctation is successfully treated with left-side thoracotomy resection and an extended end-to-end anastomosis technique with low mortality, morbidity, and low long-term recoarctation rates. Long-term follow-up is required due to the risks of early and late post-operative recoarctation, which requires reintervention.

Operators are mindful of the balloon-to-aortic annulus ratio when performing balloon aortic valvuloplasty. The method of measurement of the aortic valve annulus has not been standardised.

Patients who underwent aortic valvuloplasty at two paediatric centres between 2007 and 2014 were included. The valve annulus measured by echocardiography and angiography was used to calculate the balloon-to-aortic annulus ratio and measurements were compared. The primary endpoint was an increase in aortic insufficiency by ≥2 degrees. Ninety-eight patients with a median age at valvuloplasty of 2.1 months (Interquartile range (IQR): 0.2–105.5) were included. The angiographic-based annulus was 8.2 mm (IQR: 6.8–16.0), which was greater than echocardiogram-based annulus of 7.5 mm (IQR: 6.1–14.8) (p < 0.001). This corresponded to a significantly lower angiographic balloon-to-aortic annulus ratio of 0.9 (IQR: 0.9–1.0), compared to an echocardiographic ratio of 1.1 (IQR: 1.0–1.1) (p < 0.001). The degree of discrepancy in measured diameter increased with smaller valve diameters (p = 0.041) and in neonates (p = 0.044). There was significant disagreement between angiographic and echocardiographic balloon-to-aortic annulus ratio measures regarding “High” ratio of >1.2, with angiographic ratio flagging only 2/12 (16.7%) of patients flagged by echocardiographic ratio as “High” (p = 0.012). Patients who had an increase in the degree of aortic insufficiency post valvuloplasty, only 3 (5.5%) had angiographic ratio > 1.1, while 21 (38%) had echocardiographic ratio >1.1 (p < 0.001). Patients with resultant ≥ moderate insufficiency more often had an echocardiographic ratio of >1.1 than angiographic ratio of >1.1 There was no association between increase in balloon-to-aortic annulus ratio and gradient reduction.

Angiographic measurement is associated with a greater measured aortic valve annulus and the development of aortic insufficiency. Operators should use caution when relying solely on angiographic measurement when performing balloon aortic valvuloplasty.