Background Midaortic syndrome is often associated with refractory hypertension. Our aim was to better understand the short and medium-term outcomes in this patient population utilizing a multidisciplinary management approach. Methods We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years. Results Fifty-three patients presented at a median age of 6.7 (birth – 28.7) years. Thirty-five patients (66%) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58% at 1 year and 33% at 5 years. Freedom from reintervention after a surgical procedure was longer: 83% at 1 year and 72% at 10 years. At most recent follow-up, the majority of patients (69%) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4 – 21.1) years. The median number of anti-hypertensive medications was 1 (0 – 5). Conclusions A multidisciplinary management strategy, which couples comprehensive medical management with catheter-based and surgical interventions, can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome.

Although device closure of ASDs is associated with low morbidity and rare mortality, ongoing assessment of device safety profiles are warranted, and registries offer opportunities to facilitate the required surveillance.

Background-Melody Transcatheter Pulmonary Valve (TPV) replacement therapy represents an important advance in congenital cardiovascular interventions. The off-label extension of the Melody TPV to patients with nonconduit outflow tracts (right ventricular outflow tract [RVOT]) has the potential to vastly expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. However, knowledge on the performance of the Melody TPV in this setting is limited. Methods and Results-This is a multicenter, retrospective review of the Melody TPV when placed in nonconduit RVOTs, in which at least a portion of the circumference was composed of native tissue. Five centers contributed data on 31 patients. The median age at implantation was 24 years (range, 7-66). At a median follow-up of 15 months, all patients were alive. No patient had greater than mild TPV insufficiency, and the median maximum instantaneous gradients across the RVOT was 23 mm Hg. Stent fracture occurred in 32%. Eight patients developed more than mild TPV obstruction, of whom 6 were associated with identified stent fracture. Three patients developed blood stream infections. There were 5 reinterventions in 3 patients, including 3 repeat TPV implantations and 2 TPV explantations. Conclusions-Melody TPV implantation is feasible in selected patients with RVOTs comprised solely or predominantly native tissue and has the potential to expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. In early follow-up, valve competency seems preserved. The dominant mechanism of valve dysfunction seems to be related to stent fracture with recurrent obstruction. Additional data are necessary to better understand how to safely expand TPV therapy to this population. (Circ Cardiovasc Interv. 2014;7:374-380.)Key Word: pulmonary valve