A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4-123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6-128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.
The use of hybrid techniques to avoid neonatal cardiopulmonary bypass in high-risk individuals is well reported in the setting of hypoplastic left heart syndrome. We describe the use of that technique as a bridging procedure in high-risk neonates with an interrupted aortic arch. We report three cases where hybrid branch pulmonary artery banding and ductal stent implantation has been successfully used to defer complete repair, allowing recovery, maturity, and weight gain. This strategy may be considered for patients deemed at high risk for primary neonatal repair.
Background Diuretics are used to manage congestive heart failure in infants with congenital heart disease. Adult data indicate that preoperative diuretic use increases the risk of cardiac surgery associated acute kidney injury (CS‐AKI). We have sought to understand if preoperative diuretics in infants increases the risk of CS‐AKI. Methods and Results This is a single‐center retrospective study of infants (1–12 months) who had CS requiring cardiopulmonary bypass between 2013 and 2018. The diagnosis and severity of CS‐AKI was defined according to the Kidney Disease Improving Global Outcomes guidelines. Three hundred patients were included (mean 6 months, SD 2.4, range 1.2–12.9 months). A total of 149 (49.7%) patients were diagnosed with CS‐AKI (stage 1: 80 [54%], stage 2: 57 [38%], stage 3: 12 [8%]). Logistic regression analysis showed preoperative diuretics were not associated with CS‐AKI (odds ratio [OR], 0.79; 95% CI, 0.43–1.44; P =0.45). A diagnosis of tetralogy of Fallot was an independent risk factor for CS‐AKI (OR, 3.49; 95% CI, 1.33–9.1, P =0.01). A diagnosis of tetralogy of Fallot (OR, 3.6; 95% CI, 1.28–10.22; P =0.02) and longer cardiopulmonary bypass (OR, 1.01; 95% CI, 1.0–1.02; P =0.04) time are risk factors for moderate to severe CS‐AKI. Conclusions Preoperative diuretic use does not contribute to the risk of CS‐AKI in infants early after surgery. A diagnosis of tetralogy of Fallot was the only risk factor for CS‐AKI identified using multivariate analysis in our cohort. Furthermore, a diagnosis of tetralogy of Fallot and longer cardiopulmonary bypass time are risk factors for moderate to severe CS‐AKI.
Introduction: The hybrid procedure (HP) - bilateral pulmonary artery bands+ductal stent - is an alternative palliation for high-risk hypoplastic left heart syndrome (HLHS) patients. Assessment and management of atrial restriction here is debated and we sought to identify useful echocardiographic parameters. Methods: Patients undergoing HP for HLHS were identified. Echocardiograms at birth, immediately after HP and pre-and post-atrial intervention were reviewed as well as time-matched echocardiograms in HP patients not requiring atrial intervention. Atrial septal parameters and systolic (Svel), diastolic (Dvel) and mean velocities were recorded on both bands and systolic to diastolic velocity ratio (Svel:Dvel), pulsatility index (PI) and velocity time integral (VTI) calculated. Results: Fifteen HP were compared to 5 controls. Age effect was not significant. There was a significant rise in peak Svel and changes in Dvel, Svel:Dvel and PI over time (<0.05). Although the pattern of change similar, the fall in Dvel and rise in Svel:Dvel and PI was more significant on the left. Compared to controls, transatrial gradient and VTI were significantly increased initially post-hybrid (median 0.4m/s vs 0.7m/s p=0.04; 29.1cm vs 64.6cm p=0.018) but not significantly different prior to septostomy when the only significant differences were seen with all left band parameters. Conclusions: After HP the transatrial gradient alone cannot be used. Significant changes in band Doppler parameters were not apparent in the control group but were principally seen in the left band with reduction in Dvel and increase in Svel:Dvel and PI in those undergoing an atrial intervention.
A 4-month-old previously healthy baby was found to be in congestive heart failure with LV dysfunction and a right aortic arch with severe coarctation, undetectable by blood pressure measurements. A cardiac CT and central blood pressure led to the diagnosis of a unique anatomic variant of aortic coarctation. Once diagnosed the patient underwent surgery with an uncomplicated recovery.
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