Page 129 - Journal of Structural Heart Disease Volume 5, Issue 4

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Meeting Abstracts
York City, USA. 8The Hospital for Sick Children, Toronto, Canada. 9Texas Children's Hospital, Houston, USA. 10Children's Hospital of Michigan, Detroit, USA. 11Children's Hospital Los Angeles, Los Angeles, USA
Background: Stent treatment for branch pulmonary arteries (BPAS) has been a standard of care for 3 decades, but is still considered "off-label use". This retrospective multi-institutional study was designed in collaboration with Congenital Cardiovascular Interventional Study Consortium, the FDA and industry (CardinalHealthTM) as a new pathway to pre-market approval (PMA) of an existing FDA-approved stent (Genesis XD).
Methods: Patients with two-ventricle physiology, weigh- ing ≥10 kg, who received a Genesis XD stent for unilateral BPAS from 1/2006-12/2014 were included. Patient profile, cath lab hemodynamic and angiographic data, pre, post and 1-year follow-up echocardiographic and advanced imaging data (CTA or MRI when available) were evalu- ated. Objective performance criteria (OPC) developed in collaboration with the FDA included primary end- points of technical success defined as one of the follow- ing: 1) Increase vessel minimum diameter by ≥ 50%, or 2) Decrease systolic gradient by ≥ 50%, or 3) Decrease right ventricle (RV)/systolic blood pressure (SBP) ratio to ≤ 50% and serious adverse events ≤ 9%. Secondary endpoint was preservation of improved diameter (≥50% of original ste- notic diameter) and physiology at 1-year follow-up (FU).
Results: The study included 112 pts (11 institutions) with mean age and wt of 17±9.2 yrs and 31.7±22.2 kg respec- tively. LPA stenosis was found in 72 (64%) and 104 (93%) had prior cardiac surgery. TOF, PA, and truncus arteriosus were the most common cardiac diagnoses (77/112). Stent implant was successful in 98.2% (110/112) but primary endpoints were achieved in 97.3% (109/112). One failed primary endpoint criteria with stented vessel diame- ter increasing only 38%. Mean minimal vessel diameter increased from 4.96±2.25mm to 10.82±3.16mm. RV/SBP ratio decreased from 52.5±16.4% to 43.8± 13.7%. Three (2.7%) serious adverse events were encountered (stent embolization in 2 requiring surgical repair and ventricular tachycardia in 1 with successful cardioversion and stent implantation). Adequate FU data was available in 92.9% (104/112) with inadequate data in 5. At 1-yr echo FU, mean vessel diameter was preserved at 9.55±2.56mm (N=68). Mean vessel gradient remained low at 17.4±10.34mmHg (N=40) (from 30.6±14.93mmHg pre-intervention) and RV/ SBP ratio remained low at 34.8±0.12% (N=28). Available CTA(N=4) and MRI(N=14) showed improvement of flow to affected lung from mean 23.5±7.5% to 37.6±8.2% at mean
FU of 15.7±18.8 months. Secondary endpoint success was seen in 90.2% (101/112) while 3 failed secondary endpoint criteria for success. Excluding the 5 with inadequate FU data, 94.4% (101/107) met both primary and secondary endpoint success. Further intervention was performed in 5 at mean FU of 25.3 months for “relative” obstruction due to normal somatic growth. Successful further dilation was achieved to match normal adjacent diameters in all. No stents fractures were observed during the study period.
Conclusion: Treatment of BPAS using the Genesis XD stent is safe and effective with preservation of vessel diameter and physiology in over 94% at 1 year FU. Further stent dila- tion to match normal somatic growth is possible. Based on these results, PMA application is currently in progress under a new FDA pathway for pediatric devices.
170. PARTIAL OCCLUSION OF A LARGE PDA IN THE SETTING OF SEVERE PULMONARY HYPERTENSION USING A MANUALLY FENESTRATED AND STENTED MUSCULAR VSD DEVICE.
Amr Matoq1, Gareth Morgan2, Dunbar Ivy2, Michael Ross2
1Nemours/Alfred I.duPont Hospital for Children, Wilmington, USA. 2Children's Hospital Colorado, Aurora, USA
Background: A large patent ductus arteriosus (PDA) both pressure loads and volume loads the pulmonary vascular bed and can lead to irreversible pulmonary vascular dis- ease (PVD). Managing a large PDA in the setting of sig- nificant long-standing pulmonary artery hypertension (PAH) is challenging. Elimination of the left to right shunt is important to protect the pulmonary vascular bed, but in the setting of irreversible PVD, closing the PDA can pre- cipitate low cardiac output and right ventricular failure. Fenestrated closure of atrial septal defects as well as inten- tional creation of a connection between the aorta and pulmonary arteries have been reported, however manual fenestration of PDA devices is rarely performed.
Objective: We present a case of PAH with bidirectional shunting at a large PDA, who underwent a manually-fenes- trated and stented muscular VSD device for partial closure of the PDA.
Case: A 10-year-old female presented with exercise intol- erance. Echocardiography showed a large PDA with bidirectional shunting and evidence of PAH. Cardiac cath- eterization confirmed the diagnosis, elevated pulmonary vascular resistance (PVR) that was not responsive to 100% FiO2 and inhaled nitric oxide. There was no change to pul- monary arterial pressures with PDA test occlusion. The patient was started on dual oral PAH therapy and oxygen.
Hijazi, Z
22nd Annual PICS/AICS Meeting