• Congential Heart Disease Clinical Interest Section

    Fall 2014 Journal Scan

    Presented by V. Vivian Dimas, MD, FSCAI and Daniel S. Levi, MD, FSCAI

    Risk of coronary artery compression among patients referred for transcatheter pulmonary valve implantation: a multicenter experience.

    Morray BH, McElhinney DB, Cheatham JP, Zahn EM, Berman DP, Sullivan PM, Lock JE, Jones TK.
    Circ Cardiovasc Interv. 2013 Oct 1;6(5): 535-42.

    • Author’s Conclusions: In a multicenter study which included 407 cases in 404 patients, there was a 5% rate of coronary artery compression detected by coronary artery angiography during right ventricular outflow tract angiography. None of these 21 patients underwent Melody valve implantation and there were no “clinically apparent” coronary artery compressions after transcatheter valve implantation in the 85% of these patients who did receive a valve. Coronary artery compression was higher in patients with abnormal coronary artery patterns. (DSL)

    • Interpretation: This is large series of patients in whom patients were catheterized for potential Melody valve implantation.  Only 5% of the total population of patients had positive coronary artery compression but 71% of these patients had abnormal coronary patterns. Only 17% of the original group of 404 patients had abnormal coronary patterns. Prior to transcatheter pulmonary valve implantation, we must have an increased index of suspicion for coronary compression in patients with abnormal coronary patterns especially as found in Tetralogy and transposition patients. (DSL)

    Device closure of Secundum Atrial Septal Defects in Infants Weighing Less Than 8 kg

    Ram N. Bishnoi, Allen D. Everett, Richard E. Ringel, Carl Y. Owada, Ralf J. Holzer, Joanne L. Chisolm, Wolfgang A. Radtke, D. Scott Lim, John F. Rhodes Jr, John D. Coulson.
    Pediatr Cardiol. 2014 Oct; 35: 1124-1131.

    • Summary: Retrospective review of data from MAGIC (Mid-Atlantic Group of Interventional Cardiology) Registry. The authors address the technical aspects of device closure using both the Amplatzer Septal Occluder (ASO) and Gore Helex Septal Occluder (GSO) in infants weighing less than 8 kg. A total of 68 patients included with weight range of 2.3 to 7.8 kg with a mean of 5.5 ± 1.6 kg ages 1 to 24 months (mean 8.6 ± 4.7). Indications were failure to thrive, significant right sided enlargement, hemodynamically significant shunts, poor clinical status. Sixty patients had additional comorbidities classified as both cardiac and non-cardiac. Closure was attempted in all patients (65 ASO, 3 GSO) and was successful in 66/ 68. No major procedural adverse events were reported to the Registry. There were 6 late deaths reported that were deemed not related to device closure. A trivial residual shunt was noted in 2 patients at 6 months with no residual shunting noted in the cohort at 1 year. (VVD)

    • Authors’ Conclusions: Infants weighing less than 8 kg who undergo percutaneous device closure of an ASD are likely to have multiple comorbidities. Acutely, the ASO can be safely and effectively implanted to close ASDs in infants weighing less than 8 kg. The procedures are associated with a high success rate and a low procedural complication rate. Near-to-intermediate-term results demonstrate significant clinical improvement overall. Too few patients received the GSO to draw any conclusions regarding use of this device for this patient population. (VVD)

    Radiation Dose Benchmarks During Cardiac Catheterization for Congenital Heart Disease in the United States

    Sunil J. Ghelani, Andrew C. Glatz, Stuthi David, Ryan Leahy, Russel Hirsch, Laurie B. Armsby, Sara M. Trucco, Ralf J. Holzer, Lisa Bergersen.
    J Am Coll Cardiol Interv. 2014 Sep; 7(9): 1060-9.

    • Summary: The aim of this study was to define age-stratified, procedure-specific benchmark radiation dose levels during interventional catheterization for congenital heart disease for 6 specific interventional procedures: 1) patent ductus arteriosus closure; 2) atrial septal defect closure; 3) pulmonary valvuloplasty; 4) aortic valvuloplasty; 5) treatment of coarctation of aorta; and 6) transcatheter pulmonary valve placement. Data were obtained retrospectively from 7 laboratories participating in the Congenital Cardiac Catheterization Project on Outcomes (C3PO) collaborative to attempt to set standards for radiation exposure by age and procedure type in the pediatric interventional catheterization lab. Total air kerma, dose area product, and total fluoroscopy time were obtained to assess radiation dose. Highest doses were noted with transcatheter pulmonary valve replacement. (VVD)

    • Authors’ Conclusions: Fluoroscopy time alone is not an adequate measure for monitoring radiation exposure and correlated poorly with DAP and Ka,r. Radiation exposure varies widely among types of interventional procedures and age groups. These data will facilitate application of the ACC-endorsed pediatric radiation dose metric, namely, the proportion of cases receiving radiation in excess of the 95th percentile. (VVD)

    Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome: Postnatal Outcomes of the First 100 Patients

    Lindsay R. Frued, Doff B. McElhinney, Audrey C. Marshall, Gerald R. Marx, Kevin G. Friedman, Pedro J. del Nido, Sitaram M. Emani, Terra Lafranchi, Virginia Silva, Louise E. Wilkins-Haug, Carol B. Benson, James E. Lock, Wayne Tworetzky.
    Circulation. 2014 Aug 19; 130: 638-45.

    • Summary: One hundred patients underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis. The procedure was technically successful in 77 cases with 35 of those achieving biventricular circulation. There were 11 fetal deaths. Eighty-eight survived to delivery. Fifty-seven were managed as univentricular circulation and 31 as biventricular circulation initially. Ultimately, 38 achieved biventricular circulation (31 from birth, 7 converted after initial univentricular palliation). During follow up, a total of 14 deaths occurred, 3 in the biventricular group (1 from injuries suffered in an automobile accident and 2 following conversion from uni- to biventricular circulation). At least one (up to 7) subsequent interventional procedure was performed in 37/38 of patients in the biventricular group. Over half of the biventricular group (55%) required at least 1 surgical procedure, with valve replacement being the most common. (VVD)

    • Authors’ Conclusions: Short and intermediate term survival among patients who underwent fetal aortic valvuloplasty is encouraging. However biventricular patients still carried a substantial burden of cardiac disease. It remains to be seen which type of circulation and ventricle is better in the long term—a univentricular circulation with a systemically functioning right ventricle or a biventricular circulation with a rehabilitated left ventricle. (VVD)

    Implantation and Preliminary Follow Up of the Bard Valeo Stent in Pulmonary Artery Stenosis

    Vikram Kudumula, Patrick Noona, Demetris Taliotis, Christopher Duke.
    Catheter Cardiovasc Interv. 2014 Aug 1; 84(2): 197-03.

    • Summary: The Bard Valeo stent is a low profile premounted stent that can be post-dilated to 20 mm in diameter on bench testing. It is unclear if it has adequate radial strength to maintain patency in pulmonary artery stenoses. Twelve stents were implanted in 10 patients. No technical difficulties reported with stent delivery to target vessel. Balloon expansion noted to be asymmetric in 5 cases but did not result in stent displacement from target site. The stent was distorted in one patient with resistant stenosis. No stent fractures were noted at short and medium term follow up (0 to 30 months follow up). (VVD)

    • Authors’ Conclusions: The Valeo stent is low profile, conformable, and easy to deliver, even in small children. It has adequate radial strength to stent pulmonary artery stenoses with minimal recoil. It maintains its geometry on medium term follow up in compliant lesions. Resistant stenoses may distort the stent. (VVD)

    Results of the U.S. Food and Drug Administration Continued Access Clinical Trial of the GORE HELEX Septal Occluder for Secundum Atrial Septal Defect

    Alexander J. Javois, Jonathan J. Rome, Thomas K. Jones, Evan M. Zahn, Craig E. Fleishman, Ricardo H. Pignatelli, Larry A. Latson, for the Gore HLEX Continued Access Study Group.
    J Am Coll Cardiol Interv. 2014 Aug; 7(8): 905-12.

    • Summary: This report describes the immediate, 1-, and 5-year follow-up results of the U.S. Food and Drug Administration Continued Access clinical trial of the GORE HELEX Septal Occluder (W. L. Gore & Associates, Inc., Flagstaff, Arizona) for closure of secundum atrial septal defect. A total of 137 devices were implanted in 137 patients. Eleven patients were lost to follow up at 1 year. A significant number of patients were lost to follow up. At 5 years, 83/95 patients who agreed to long term follow up completed the evaluation. Five major adverse events occurred (2 embolizations, 2 wire frame fractures requiring removal, 1 unrelated death). Frame fracture increased significantly with increasing device size (26.7% of 30 mm devices, 45.5% of 35 mm devices). Clinically insignificant leaks were also more frequent with increasing device size at 5 year follow up (21.1% of 20 mm devices, 26.7% of 25 mm devices, 26.3% of 30 mm devices and 57.1% of 35 mm devices). (VVD)

    • Authors’ Conclusions: More than 96% of patients with successful implantation met the primary endpoint of clinical success with no more than a trivial residual shunt, normalization of right ventricular size, and no major adverse event. In particular, there were no erosions, perforations, allergic reactions, or arrhythmias requiring treatment. Small residual leaks are more likely to occur with device size–to–defect size ratios <2:1 and in patients with defects >18 mm. Wire frame fractures were seen in 11.7% of patients, but all were asymptomatic, with 92% being detected only by radiographic evaluation including fluoroscopy. A relatively high incidence of clinically insignificant residual leak (or inability to rule out a trivial leak) was seen at 1 year and persisted at 5-year follow-up. Immediate, 1-, and 5-year follow-up of the multicenter trial indicates that the device is a safe and effective alternative to the repair of an ostium secundum ASD. (VVD)

    Technical Challenges of Atrial Septum Stent Placement in the Fetus with Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

    Kalish BT, Tworetzky W, Benson CB, Wilkins-Haug L, Mizrahi-Arnaund A, McElhinney DB, Lock J, Marshall AC.
    Catheter Cardiovascular Interv. 2014 Jul 1;84(1): 77-85.

    • Summary: Single center experience with prenatal atrial septal stent placement for fetuses with hypoplastic left heart syndrome and intact atrial septum. Nine fetuses (24-31 weeks gestation) underwent attempted atrial septal stent placement. Stent was planned in 7 fetuses with successful placement in 5. In the remaining 4 fetuses, 3 underwent balloon atrial septoplasty successfully at the same procedure. Procedural adverse events include bradycardia, hemopericardium and technical stent complications. There was 1 fetal demise. There were no maternal complications. The remaining 8 fetuses survived to delivery with 4 dying in the neonatal period. Two survived to Stage I palliation, one underwent both atrial stent placement and aortic valvuloplasty in utero and subsequently was able to achieve biventricular circulation. (DSL)

    • Authors’ Conclusions: Ultrasound-guided atrial septal stent placement is feasible in some fetuses with HLHS/IAS. Visualization of the septum and catheter tip is critical to technical success. Technical complications were largely the result of poor intra-cardiac visualization of the balloon prior to stent deployment. (DSL)

      Stent placement in HLHS with Intact atrial septum is technically feasible. Of the nine fetuses in which this was attempted, four cases were successful and three of these patients survive to either Glenn shunt or a bidirectional repair. To minimize technical complications it is critical to have good visualization of the balloon prior to stent deployment. (VVD)
    • Interpretation: This is a revolutionary approach to a disease that has >80% mortality at many experience tertiary care centers.This procedure is clearly technically challenging and requires close collaboration between cardiologists, perinatologists and obstetricians. Although the complication rate is high and included stent embolization, hemopericardium and fetal demise and arrhythmias, this approach is justified given the three survivors and the poor prognosis in the absence of fetal intervention. (VVD)

    Use and Performance of the Melody Transcatheter Pulmonary Valve in Native and Postsurgical, Nonconduit Right Ventricular Outflow Tracts

    Meadows JJ, Moore PM, Berman DP, Cheatham JP, Cheatham SL, Porras D, Gillespie MJ, Rome JJ, Zahn EM, McElhinney DB.
    Circ Cardiovasc Interv. 2014 Jun; 7: 374-80.

    • Summary: Multicenter, retrospective review of the Melody TPV placed in nonconduit RVOTs, in which at least a portion of the circumference was composed of native tissue. Thirty one patients underwent implantation at a median age of 24 years (range, 7–66). Pre-stenting was performed in 22 of 31 patients (single stent in 21, 2 stents in 1). Two serious procedural complications occurred: contained RVOT tear which was treated with a covered stent and creation of iatrogenic aortopulmonary connection requiring closure at the same catheterization. Stent fracture occurred in 32%. Eight patients developed more than mild valve 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. (VVD)

    • Authors’ 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. (VVD)