Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension

Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension. Frank BS, Schäfer M, Grenolds A, Ivy DD, Abman SH, Darst JR. J Pediatr. 2019 May;208:127-133. doi: 10.1016/j.jpeds.2018.12.004. Epub 2019 Mar 11. PMID: 30871795   Take Home Points:   Positive response to AVT but not baseline hemodynamics is associated with better long-term clinical outcomes in premature infants with BPD-associated PH. Cardiac catheterization plays an important diagnostic and therapeutic role in the management of this patient population.   Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch:  Bronchopulmonary dysplasia (BPD) associated pulmonary hypertension (PH) contributes to significant long-term mortality and morbidity in premature infants. The optimal strategies to diagnose and treat this patient population are poorly defined. The authors sought to evaluate the role of acute vasoreactivity testing (AVT) in the assessment of premature infants with BPD-associated PH.   From 2009-2017 twenty-six premature infants (mean birth weight 580g, gestational age 26 weeks) with PH underwent cardiac catheterization and made up the study cohort; those with complex congenital heart disease were excluded. At the time of catheterization the mean age was 156 days and weight 4.1kg. Patients first underwent a baseline assessment on room air (or as close as possible) and then on 100% FiO2 and 20-40ppm iNO. Baseline hemodynamics reflected the presence of PH; 7 patients had pulmonary vein stenosis identified (not suspected pre-cath imaging in 4); and 6 patients had interventions performed (pulmonary vein dilation [4]; ASD closure [1]; PDA closure [1]). There was no relationship between baseline pulmonary hemodynamics and clinical outcomes.   Nine patients had a positive response to AVT (based on the Barst criteria) with responders having a higher birth weight (690 v 540 g), gestational age (28 vs. 26 weeks) and baseline PVRi (6.7 vs. 4 iWu). Only one of the responders (11%) had the combined outcome of death or tracheostomy vs. 6 of non-responders (35%). Kaplan-Meier event-free survival comparison is shown below. There were 4 procedural complications: 3 minor (vascular thrombosis [2]; atrial flutter requiring cardioversion [1]) and 1 major (ventricular tachycardia requiring cardioversion).   The authors conclude that while baseline hemodynamics did not predict long-term clinical outcomes, positive response to AVT was associated with a lower long-term risk of tracheostomy or death. While the risk of cardiac catheterization in patients PH is known to be significant the authors report only 1 major adverse event with no mortality and report that many patients had a change in the clinical management based on the catheterization. The findings presented in this manuscript raise important questions about the diagnosis and management of patients with BPD-associated PH. Should more patients with BPD-associated PH undergo invasive evaluation with AVT? Should non-responders be treated more aggressively given their higher long-term risk? As the survival of premature infants continues to improve it will be increasingly important to answer these (and other) questions.    


Comparison of drug eluting versus bare metal stents for pulmonary vein stenosis in childhood

Comparison of drug eluting versus bare metal stents for pulmonary vein stenosis in childhood. Khan A, Qureshi AM, Justino H. Catheter Cardiovasc Interv. 2019 May 8. doi: 10.1002/ccd.28328. [Epub ahead of print] PMID: 31067002 Similar articles Select item 31066199   Take Home Points: Pulmonary vein stenosis is a challenging disease process to manage with poor outcomes when treated surgically and with transcatheter therapies. Drug eluting stents show promise in treatment of pulmonary vein stenosis with less lumen loss than standard bare metal stents in short term follow up despite being used in higher risk patients. Long term results of pulmonary vein stenosis treated with drug eluting stents remains unknown. Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Interventional Cardiology Journal Watch:  Pulmonary vein stenosis (PVS) remains a challenging lesion to manage with significant morbidity and mortality despite advances and transcatheter and surgical treatments.  Transcatheter intervention with balloon angioplasty, cutting balloon angioplasty and bare-metal stent (BMS) implantation has been shown to be acutely successful but have poor medium and long-term outcomes (large modern era series with 5-year survival of 30-50%), along with high rates of restenosis.  Surgical treatment is typically used in patients with ostial lesions and lesions proximal to the first order branching.  Again, there is a high rate of restenosis and 5-year survival of approximately 50%.  Use of the sutureless technique has improved surgical outcomes.  However, lesions beyond the first order division of the pulmonary veins are typically beyond surgeon's reach and hence not amenable to this technique.  Additionally, the sutureless technique tends to be more successful in patients with postoperative pulmonary vein stenosis than in primary pulmonary vein stenosis. Given these poor outcomes, drug eluting stents (DES) are being evaluated as a potential strategy to decrease restenosis and potential loss of pulmonary veins.   Khan et al report on their retrospective single center review of all patients who underwent stent implantation for PVS (native and post-surgical) over a 24 year period. A total of 66 patients underwent stent implantation. 37 patients had 58 lesions treated with 62 BMS and 41 patients had 105 lesions treated with 111 DES. 12 patients were treated with both BMS and DES. Patients in the DES group were significantly younger (16.2 +/- 18.8 months versus 2.9 +/- 3.5 years) and smaller (7.4 +/- 3.9 kg versus 11.8 +/- 8.7 kg) than those in the BMS group. Lesion diameter increased from 2.9 +/- 1.7 mm to 7 +/- 2 mm with BMS implantation and 1.3 +/- 1 mm to 3.8 +/- 0.6 mm with DES implantation (significantly more increase with BMS). Mean gradient decreased from 10.7 +/- 6.5 mmHg to 2.1 +/- 2.8 mmHg in the BMS group and 12.6 +/- 6.2 mmHg to 2.8 +/- 2.7 mmHg in the DES group (no statistically significant difference in pre or post gradient between groups). Follow up cardiac catheterization was performed on 44/58 lesions treated with BMS at an interval of 6.4 +/- 6.4 months and 86/105 lesions treated with DES at an interval of 6.8 +/- 7.4 months. Absolute lumen loss (mm), fractional lumen loss (% lumen diameter change from time of implantation to follow up catheterization), absolute lumen loss rate (mm/month) and fractional lumen loss rate (%/month) were all significantly lower in the DES group as shown in the table below.   Patients were divided into two groups in an attempt to determine era and treatment strategy effect. The conventional group received BMS only throughout their treatment course, with their first stent implantation prior to 2008. The novel group included all remaining patients. In this group, DES were implanted whenever possible (lesion diameter < 4mm as this was the largest DES available) and BMS were implanted only when DES was not possible (lesion >4 mm). The table below shows outcomes for the two groups. The authors note that their center trended to more transcatheter rather than surgical intervention in these patients over time. Patients in the novel therapy group tended to be younger, have more native than post-operative PVS, and have more veins involved. Despite this, 2 year survival was 74.7% in the novel therapy group and 53.1% in the conventional group.   The authors note that the mechanism of PVS appears to be related to myofibroblast proliferation. DES allow for the benefit of local drug delivery to prevent neo-intimal proliferation. They have been shown to decrease in stent stenosis in adults who undergo stent implantation for coronary artery disease. Systemic drug levels from DES have been evaluated in patients who undergo DES implantation in the ductus arteriosus (PDA stenting for ductal dependent pulmonary blood flow). Sirolimus levels were found to be in an immunosuppressive range but there were no serious infectious complications. Analysis of patient specific outcomes is challenging as there may be a significant era effect (there was not access to DES in earlier patients). However, patients receiving drug eluting stents were younger, smaller, and had more veins involved indicating a much more aggressive disease process. Also, the diameter at first implant was smaller in the DES group than BMS group making the risk of neointimal proliferation higher. Despite these factors, lesions treated with DES had significantly less lumen loss than those with BMS at the first follow up catheterization. The authors conclude that this must be due to the local delivery of the antiproliferative agents. The study is limited by the fact that it does not take into account additional treatment strategies applied to the later (novel) cohort of patients including more aggressive recanalization of atretic veins, aggressive treatment of restoring complete patency during a single procedure, and scheduled follow up catheterization every 6 months to evaluate for in-stent stenosis and disease progression. Longer term outcomes of patients treated with DES remains unknown.  


Fenestration in the Fontan circulation as a strategy for chronic cardioprotection

Fenestration in the Fontan circulation as a strategy for chronic cardioprotection. Saiki H, Kuwata S, Iwamoto Y, Ishido H, Taketazu M, Masutani S, Nishida T, Senzaki H. Heart. 2019 Mar 2. pii: heartjnl-2018-314183. doi: 10.1136/heartjnl-2018-314183. [Epub ahead of print] PMID: 30826770   Take Home Points:   Fenestration patency may confer hemodynamic advantages such as lower HR, lower CVP and a better preload reserve. Providers should carefully consider the risks and benefits of elective fenestration occlusion prior to proceeding with closure. Further study is indicated as to the long term risks and benefits of fenestration patency.   Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch:  Fenestration of the Fontan connection was initially developed as a strategy to minimize morbidity in the immediate post-Fontan period. The long-term benefits of a Fontan fenestration are not well defined, and some physicians argue for elective fenestration closure to improve systemic oxygenation. The authors hypothesized that a patent fenestration offers hemodynamic advantages and sought to assess the hemodynamic implications of a fenestration utilizing catheter-based, load-independent measures at baseline, with dobutamine stress testing (DST), and rapid atrial pacing.   Thirty-five consecutive Fontan patients undergoing cardiac catheterization were prospectively enrolled to undergo invasive testing – 24 with a patent fenestration (PF) and 11 with a naturally closed fenestration (NCF). The baseline demographics of the 2 groups were similar. Patients with a PF had a lower baseline HR, CVP, PVRi, Qp:Qs ratio, and SaO2; while the cardiac index was similar between the groups.   After baseline assessment, patients underwent various physiologic manipulations (DST +/- transient fenestration occlusion (TFO) and rapid atrial pacing +/- TFO). Among patients who underwent DST, ventricular systolic and diastolic stiffening was significantly greater in those with NCF. Compared to baseline, TFO decreased the ventricular diastolic area, stroke volume, and cardiac index, while increasing afterload and ventricular chamber stiffness. Eight patients with PF had atrial pacing performed both at baseline and with TFO. At higher HRs there was a reduced CI and increased afterload – suggesting that the beneficial effects of a fenestration diminish at higher HRs.   The authors conclude that a chronically persistent fenestration secures preload reserve, reduces afterload and suppresses the adrenergic response; it also results in a lower HR at baseline. The number of patients in this study is small but the implications are potentially significant. If a patent fenestration can be confirmed to have hemodynamic benefits acutely and then demonstrated to decrease long term Fontan morbidity then fenestration patency would become a priority for long term Fontan management.  


Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization

Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization. Adamson GT, Peng LF, Feinstein JA, Yarlagadda VV, Lin A, Wise-Faberowski L, McElhinney DB. Catheter Cardiovasc Interv. 2019 Oct 4. doi: 10.1002/ccd.28508. [Epub ahead of print] PMID: 31584246 Similar articles Select item 31584303   Take Home Points: Patients with Alagille syndrome have a significant risk of pulmonary hemorrhage with both diagnostic and interventional cardiac catheterizations. A diagnosis of tetralogy of Fallot, higher RV to aorta pressure ratio, and PA angioplasty are associated with an increased risk of pulmonary hemorrhage. The exact etiology of this increased risk remains unknown and warrants further investigation. Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch:  Children with Alagille syndrome often have hemorrhagic complications during invasive procedures and are also at risk for spontaneous bleeds (particularly intracranial hemorrhage). This is thought to be a result of abnormalities in angiogenesis, hemostasis, and/or platelet function.  Some have suggested that the bleeding risk is associated with the severity of congenital cardiovascular phenotype. This bleeding risk contributes significantly to morbidity and mortality in this patient population. The large majority (90%) of children with Alagille syndrome have congenital heart disease. This typically affects the branch pulmonary arteries (PA), though the disease severity is highly variable. It can range from mild branch PA stenosis to severe diffuse PA hypoplasia. Tetralogy of Fallot (TOF) is seen in ~ 15% of children [including TOF with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs)].  Patients with Alagille syndrome who have the most severe cardiovascular disease are frequently referred to the cardiac catheterization lab for diagnostic catheterizations to assist in surgical planning or for interventional procedures (pulmonary angioplasty and/or stent implantation).  Pulmonary hemorrhage is a known complication of branch PA intervention in general and has anecdotally been seen in higher frequencies in patients with Alagille syndrome.   Adamson et al report on their retrospective single center experience with 30 patients with Alagille syndrome who underwent a total of 87 cardiac catheterizations (median 2 per patient) over an 8 year period (2010-2018). The most common diagnosis was isolated branch PA stenosis/hypoplasia in 15 patients (50%) followed by TOF/PA/MAPCAs in 10 patients (33%), TOF in 4 patients (13%), and pulmonary atresia with an intact ventricular septum (PA/IVS) and MAPCAs in a single patient. Surgical intervention had been performed prior to the cardiac catheterization in 61% of cases. The cardiac catheterizations included branch PA interventions in 37% of the cases and were diagnostic cardiac catheterizations in the remaining 73%. All patients were systemically heparinized with an activated coagulation time maintained above 200 seconds.   Pulmonary hemorrhage occurred in 26/87 cardiac catheterizations (30%) and in 14/30 patients (46%). The majority of these (n=17, 65%) were mild hemorrhage (defined as mechanical ventilation <24 hours, no treatment other than a single packed red blood cell transfusion). There were 4 (15%) moderate hemorrhages (24-72 hours mechanical ventilation or >72 hours noninvasive positive pressure ventilation), and 5 (19%) severe hemorrhages (>72 hours mechanical ventilation, use of inotropic support, ECMO, or death). Only two of the hemorrhages were clearly due to a vessel tear from an intervention, though the source of bleeding was not always able to be identified on review of the medical records. Two hemorrhages were managed with venoarterial ECMO support without any major ECMO complications or deaths.  No patients required intravascular closure devices or covered stents to treat the hemorrhage.  All 9 moderate and severe hemorrhages occurred in 5 patients with TOF, three of whom had TOF/PA/MAPCAs.   The authors highlight that pulmonary hemorrhage occurred in approximately one third of procedures, including 44% of interventional and 22% of diagnostic cardiac catheterizations (incidence in patients without Alagille syndrome undergoing PA angioplasty 12-14% in two recent studies).  Fortunately, the majority of these were mild.  Two patient's required ECMO support but there were no deaths.  Higher RV to aorta pressure ratio, intervention on the branch PAs, and a diagnosis of TOF were associated with pulmonary hemorrhage, with severe hemorrhage occurring primarily in patients with TOF/PA/MAPCAs. The authors note that the patient population they care for is not representative of a typical center’s given their expertise in surgical branch pulmonary arterioplasty (~12% of patients with Alagille syndrome have TOF with 1/3 of those TOF/PA/MAPCAs, nearly 50% with TOF and 2/3 of these with TOF/PA/MAPCAs in this cohort). Additionally, they may be less aggressive intervening in the cath lab given their centers preference for surgical intervention when possible and thus may be underestimating the risk of pulmonary hemorrhage.  


Use of 65 cm large caliber Dryseal sheaths to facilitate delivery of the Edwards SAPIEN valve to dysfunctional right ventricular outflow tracts

Use of 65 cm large caliber Dryseal sheaths to facilitate delivery of the Edwards SAPIEN valve to dysfunctional right ventricular outflow tracts. Kenny D, Morgan GJ, Murphy M, AlAlwi K, Giugno L, Zablah J, Carminati M, Walsh K. Catheter Cardiovasc Interv. 2019 Aug 13. doi: 10.1002/ccd.28409. [Epub ahead of print] PMID: 31408262   Take Home Points: Implantation of the Sapien XT or S3 valve carries a significant (6-7.5%) risk of tricuspid valve injury. Use of the large diameter 65cm DrySeal sheath facilitates advancement of the Sapien valve to the right ventricular outflow tract and may decrease the incidence of tricuspid valve injury.   Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch:  The Sapien XT and S3 valves have been a welcome addition to the interventional armamentarium for the treatment of dysfunctional right ventricular outflow tracts (RVOT) allowing for treatment of a larger variety of anatomies. However, as the Sapien system was designed for trans-catheter aortic valve implant, the valve is uncovered to allow for a smaller delivery profile and has been associated with a significant risk (6-7.5%) of tricuspid valve injury during delivery to the RVOT. The authors sought to describe their experience using the large diameter 65cm Gore Dryseal Sheath to facilitate delivery of the Sapien XT and Sapien 3 valves to dysfunctional RVOTs.   Thirty patients at 3 large congenital heart centers underwent Sapien 3 (29) or XT (1) implants using the Gore Dryseal sheath. Sheath sizes ranged from 22F to 26 F (see table below for sizing). The valve was mounted directly onto the balloon in 29 cases. Seventeen of the patients were female with a median age of 17.5 years (8-72) and median weight of 60 kg (23.8-128). Most patients had a native RVOT (20/30) with 7 having a bioprosthetic valve and 3 having a conduit.   The valve was delivered successfully in all patients with 1 requiring a second valve and ultimately requiring surgery secondary to an RVOT gradient related to a complex RVOT membrane. No patients had increase in tricuspid valve regurgitation severity related to valve delivery. There were no vascular injuries even though 2 patients who were just over 30kg had a 26F sheath successfully used. All patients had the venotomy closed by a suture placed at the beginning of the procedure or using a figure-of-eight stitch. The short term outcomes were favorable with low post-procedure RVOT gradients and no significant pulmonary valve insufficiency.   The authors conclude that use of the large diameter 65 cm Gore Dryseal sheath facilitates delivery of the Sapien 3 and XT valves to dysfunctional RVOTs with no obvious tricuspid valve injury related to valve delivery. One of the main disadvantages to use of the Sapien valves in dysfunctional RVOTs is the risk of tricuspid valve injury, but using the technique described by Kenny et al, this risk can likely be eliminated making use of the Sapien valves much more appealing.


A Low Residual Pressure Gradient Yields Excellent Long-Term Outcome After Percutaneous Pulmonary Valve Implantation

A Low Residual Pressure Gradient Yields Excellent Long-Term Outcome After Percutaneous Pulmonary Valve Implantation. Georgiev S, Ewert P, Tanase D, Hess J, Hager A, Cleuziou J, Meierhofer C, Eicken A. JACC Cardiovasc Interv. 2019 Jun 10. pii: S1936-8798(19)30846-5. doi: 10.1016/j.jcin.2019.03.037. [Epub ahead of print] PMID: 31202951 Similar articles Select item 31103536   Take Home Points: Transcatheter pulmonary valve implantation has excellent long-term outcomes with surgery-free survival 92% at 5 years and 83% at 10 years. Significant risk factor for death and valve failure include an RV-PA gradient >15 mmHg. All efforts to adequate prepare the RVOT and minimize residual gradient leads to significant improvement in survival free of repeat interventions. Commentary from Dr. Wendy Whiteside (Ann Arbor MI), section editor of Congenital Heart Disease Interventions Journal Watch: Since the introduction of percutaneous pulmonary valve implantation (PPVI) in 2000, the use and treatment indications of transcatheter valves has broadened substantially, and in many centers, has become the treatment of choice for rehabilitation of right ventricular outflow tract (RVOT) dysfunction. While there has been significant data to support the short and intermediate outcomes of PPVI, long term outcome data is somewhat limited. Georgiev add to this, a large, prospectively collected dataset of their single center experience with meticulous follow-up of up to 11 years. Between 2006-2017, 236 patients underwent PPVI at the German Heart Center Munich. Follow-up data were available for an impressive 96% of patients (226 patients) over a median follow-up time of 3.9 years (range 2 months to 11 years). Median age at implant was 18 years, median weight 59 kg, with 14% (32 patients) weighing <30 kg. The majority of patients had surgically placed conduits or bioprosthetic valves with 26 patients (11%) having a native RVOT. Given the later introduction of the Sapiens valve, the majority of patients received Melody valves (220 patients, 92.8%) with only 16 patients (6.8%) receiving a Sapiens valve. Pre-stenting was performed in the vast majority of patients (96%) and post-dilation of the implanted valve with high-pressure balloons was performed in 63% of patients. Seven patients died during the study period—2 patient deaths were directly related to the procedure (uncontained conduit rupture and coronary artery compression) with 4 patients dying at home 3-7 years post-procedure.  Overall survival was 96% at 5 years, and 93% at 10 years. Surgery free survival was 92% at 5 years and 83% at 10 years. At the end of the study period 219 patients (93%) were alive with the initially implanted valve. The remaining 17 patients reached the endpoint of valve failure—10 patients because of stenosis due to infective endocarditis and 7 due to progressive stenosis without infective endocarditis. Of these patients, 11 received new surgical valves and 6 received a second transcatheter valve-in-valve procedure. Significant risk factors for reaching the endpoints of the study (including death or valve failure) were analyzed and the best outcome was seen in patients treated with pre-stenting and who were left with a residual RVOT gradient <15 mmHg.  Survival free from intervention in patients who had pre-stenting and a post-interventional residual gradients <15 mmHg was significantly better than those without (88% vs 56%, see Figure).  In follow-up MRI imaging, RV dimensions and function improved after 6 months and remained stable, and exercise capacity improved slightly after new valve implantation and remained stable at 5-year follow-up. This study supports the excellent outcomes following PPVI, even with longer term follow-up up to 11 years. Reinterventions are uncommon and are primarily due to valve stenosis, which occurs both with and without infective endocarditis.  This study also supports what many have done in practice—meticulously preparing the landing zone prior to valve implantation with pre-stenting and attention to minimizing residual stenosis—and shows significantly improved outcomes in patients left with RVOT gradient <15 mmHg.    


Leaflet morphology classification of the Melody Transcatheter Pulmonary Valve

Leaflet morphology classification of the Melody Transcatheter Pulmonary Valve. Boe BA, Cheatham SL, Armstrong AK, Berman DP, Chisolm JL, Cheatham JP. Congenit Heart Dis. 2019 Mar;14(2):297-304. doi: 10.1111/chd.12728. Epub 2018 Dec 13. PMID: 30548926 Similar articles Select item 30506893   Take Home Points: The Melody transcatheter pulmonary valve is made from a bovine jugular vein sutured onto a stent. There are variations in the valve morphology. Midterm outcomes for all valve morphologies are excellent. However, longer term outcomes and differences in outcomes of valve function and complications based on valve morphology remain unknown. Commentary by Dr. Ryan Romans (Kansas City), section editor of Interventional Cardiology Journal Watch:  The Melody Transcatheter Pulmonary Valve (TPV) was first implanted as a treatment for right ventricular outflow tract (RVOT) conduit dysfunction in 2000 and in the United States in 2007. Since then, use of the Melody valve has expanded to include placement in bioprosthetic valves in the tricuspid (off label) and pulmonary positions (valve in valve) and in native RVOTs (such as tetralogy of Fallot s/p repair with a transannular patch, also off-label). The Melody valve is constructed from a bovine jugular vein sutured within a platinum/iridium stent. The bovine jugular vein has thin leaflets with deep commissures making it ideal for implantation over a wide range of geometric configurations and implanted diameters. All valves are rigorously tested prior to release for clinical use and all bicuspid valves are discarded. However, variations in valve morphology have been noted but never described.   Boe et al performed a single center retrospective study to evaluate the different Melody TPV morphologies. Their institution routinely captures intraprocedural down-the-barrel videos and photographs of the Melody TPV to assess leaflet appearance. A random cohort of Melody TPV images were reviewed and used to create a morphologic classification system as shown below.   All of the Melody valves were then blindly reviewed and classified by all 5 implanters, with the final classification assigned based on the consensus (classification assigned by 3 or more reviewers). Over a 5-year period, a total of 62 valves were implanted in 61 patients. Type A morphology was seen in 48% of valves, type B in 32%, type C in 16%, and type D in 5%. Follow up data at 6-months was available for 41/55 Melody valve implantations (7 patients were excluded for outcomes analysis due to the valve being implanted in a non-pulmonary position). Acceptable hemodynamic function (defined in the Melody Post-Approval Study as a mean RVOT gradient of 30 mmHg or less and mild regurgitation or less) at 6 months post implantation was seen in 40/41 valves. Over a median follow-up period of 1.5 years (range 0-4.4), only 2 of the Melody valves developed greater than mild regurgitation, with both valves being type A. Complications were seen in 9 valves implanted in 8 patients during follow up. There were 3 cases of endocarditis (all requiring Melody valve explantation), two valves with frame fracture, one case of refractory arrhythmias (patient with multiple pre-stents) requiring surgical pulmonary valve replacement, two patients requiring conduit replacement due to Melody valve stenosis, and one death. 4 of the complications occurred with type A valves and 5 with type B.   This is the first description of Melody TPV morphology. This study was not sufficiently powered to adequately assess outcomes between valve morphologies. However, all valve types had excellent midterm function and low complication rates. Future prospective studies may offer better understanding of outcomes based on valve morphology.  


Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory

Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory. O'Byrne ML, Millenson ME, Steven JM, Gillespie MJ, Dori Y, Glatz AC, Rome JJ. JACC Cardiovasc Interv. 2019 May 13;12(9):835-843. doi: 10.1016/j.jcin.2019.01.224. Epub 2019 Apr 10. PMID: 30981573 Similar articles Select item 30981569 Comment from Dr. Wendy Whiteside (Michigan), section editor of Congenital Heart Disease Interventions Journal Watch:  Procedural sedation, without the use of an anesthesiologist, has historically been used commonly within the pediatric cardiac catheterization lab.  More recently, however, there has been a shift towards the use of anesthesiologists in these cases, with the intention of providing improved patient safety with a dedicated provider in charge of patient sedation/anesthetic.  With this transition has come an increasing use of general anesthesia (GA) in the pediatric catheterization lab.  The safety of procedural sedation versus general anesthesia has not, however, been critically evaluated in this setting. O’Byrne et al therefore present a single-center retrospective cohort study comparing the relative safety, cost, and case times of catheterization procedures performed using operator-directed sedation (ODS) compared with general anesthesia administered by a cardiac anesthesiologist.  ODS consisted of either IV sedating agents or local analgesia administered by a registered nurse under the supervision of the interventional cardiologist.  Over a 6.5 year period from 2011-2017, they studied 4,424 cases in 2,547 patients.  Of these, 27% of cases were performed with ODS (93% performed with IV sedation and 7% with local anesthetic alone).  Major adverse events (MAE) were higher in GA cases compared with ODS cases (6.6% vs 3.4%; p<0.001), a difference which remained in propensity score-adjusted models, with odds of a MAE lower in cases performed using ODS compared with GA (0.66, 95% CI 0.4-0.9, p=0.03).  Applying the CRISP score retrospectively to their cases, they determined that only a minority (10.2%) of procedures using ODS would have aligned with the 2016 expert consensus recommendations and would have been appropriate to be performed without an anesthesiologist.  Despite this, when the examined observed-to-expected MAE ratios for cases stratified by CRISP score and mode of sedation, they found that this deviation from recommendations was not associated with a change in the observed-to-expected ratio for MAE.  Total room time, total sheath time, and room exit time (time from hemostasis to room exit) were also significantly shorter in the ODS group.  Again using propensity score adjusted models, ODS was associated with decreased hospital and professional charges compared with GA with an estimated savings in hospital costs of 2017 US dollars of $2,238 per procedure. Clearly, as the authors state, these results are certainly not meant to imply that involvement of an anesthesiologist increases the risk of a cath procedure, however they do suggest that a careful review of each individual case may be able to identify cases in whom ODS may be safe and effective and can thereby reduce resource use (both cost and case times).  The safety and comfort of either of these techniques are certainly very center- and provider-specific and available resources in a particular center may very well dictate their distribution of sedation vs GA. There can be an argument made to utilizing sedation over GA in the cath lab, particularly in diagnostic cases, where free breathing with procedural sedation will provide a more accurate assessment of baseline hemodynamics.  GA, however, may provide a better steady-state, with less patient movement, that may be advantageous for particular procedures, especially in light of the more complex procedures being performed in more hemodynamically vulnerable patients in current cardiac catheterization labs. While involvement of anesthesiologists has become more common in catheterization labs through the country, this should not automatically imply the use of GA.  There should be careful consideration of the most appropriate sedation/anesthesia for a particular case, particularly in light of increased safety and efficiency of sedation over GA.  


Drug-Eluting Stents Compared With Bare Metal Stents for Stenting the Ductus Arteriosus in Infants With Ductal-Dependent Pulmonary Blood Flow

Drug-Eluting Stents Compared With Bare Metal Stents for Stenting the Ductus Arteriosus in Infants With Ductal-Dependent Pulmonary Blood Flow. Aggarwal V, Dhillon GS, Penny DJ, Gowda ST, Qureshi AM. Am J Cardiol. 2019 Sep 15;124(6):952-959. doi: 10.1016/j.amjcard.2019.06.014. Epub 2019 Jun 26. PMID: 31350000     Take-Home Points: Drug eluting coronary artery stents (DES) have been theorized to have a lower incidence of neo-intimal proliferation (compared with bare metal stents) when implanted in the ductus arteriosus. DES were found to have significantly less luminal loss and fewer unplanned interventions when compared to BMS. Commentary from Dr. Konstantin Averin (Edmonton, Canada), catheterization section editor of Pediatric Cardiology Journal Watch:  Stenting of the ductus arteriosus (DA) is rapidly emerging as an equivalent (or superior) option for augmenting pulmonary blood flow in infants with ductal-dependent pulmonary blood flow (PBF). Drug eluting coronary artery stents (DES) have been theorized to have a lower incidence of neo-intimal proliferation but data comparing them to bare metal stents (BMS) in this patient population are lacking. The authors sought to compare the safety and efficacy of DES to BMS in neonates undergoing DA stenting for ductal-dependent PBF. Over a 14 year period (2004-2018) 71 infants with confluent central pulmonary arteries underwent ductal stenting for ductal-dependent PBF (46 BMS and 25 DES) with stent selection being uniform during the study period. The baseline characteristics of the cohort as a whole were unremarkable and were similar between the 2 groups (BMS v DES). Freedom from ≥ 50% luminal loss was significantly higher in the DES group (see Kaplan Meier curve below) with the odds ratio for a 50% loss in luminal diameter (as assessed via a subsequent angiographic evaluation) being 1.6 (95% CI 1.2-2.3) for BMS v DES. Sixteen infants (23%) underwent unplanned re-interventions to treat cyanosis, with a significantly higher percentage in the BMS group (28%) compared to the DES group (12%), p=0.02.   There was no mortality difference between the groups. Given concerns about serum drug levels in infants with DES, the authors compared rates of infection between the 2 groups and found the rate of bacterial infection to be higher in the DES group (8% v 2%) but this did not reach statistical significance.   The authors conclude that luminal loss was significantly less and unplanned interventions less common in patients with DES compared to BMS. While the authors did not measure serum drug levels, even if they had been elevated there do not appear to have been significant clinical sequela related to this (consistent with prior reports). Intuition would suggest that anti-proliferative agents would mitigate neo-intimal proliferation of ductal tissue and this report begins to lend credence to that theory. Given the data presented in this report, providers should strongly consider use of DES (vs BMS) for ductal stenting.  


Wire-frame integrity of patch-like Gore devices following atrial septal defect closure.

Wire-frame integrity of patch-like Gore devices following atrial septal defect closure. Kubicki R, Fingerhut K, Uhl M, Hummel J, Höhn R, Reineker K, Fleck T, Stiller B, Grohmann J. Catheter Cardiovasc Interv. 2019 Jan 24. doi: 10.1002/ccd.28103. [Epub ahead of print] PMID: 30680882 Similar articles Select item 30545978   Take Home Points The Gore Cardioform Septal Occluder is safe and effective in closing secundum atrial septal defects. The rate of wire frame fractures is similar to the previous Gore device (Helex septal occluder) at 6.8%. In this small cohort, there were no clinical sequelae of wire frame fracture, though longer term follow up in a larger group are needed. Commentary from Dr. Ryan Romans (Kansas City), section editor of Congenital Interventional Journal Watch:  Transcatheter closure of secundum atrial septal defects (ASDs) is now first-line therapy in amenable defects. The Gore Cardioform Septal Occluder (GSO) is one of the most commonly used devices to close secundum atrial septal defects. It also has FDA approval for closure of patent foramen ovale’s. Use of this device has increased given the concerns of erosion with the Amplatzer septal occluder.  These are the only two devices approved for ASD closure in the United States. The first generation device from Gore (Helex Septal Occluder, now discontinued) had a wire-frame fracture (WFF) incidence of 6-12%.  The GSO features a sturdier frame design with 5 wires (instead of a single wire) and increased strength at larger diameters. This study evaluated the incidence of WFF in the GSO.   Kubicki et al describe their single center, retrospective analysis of consecutive patients who underwent successful ASD closure with a GSO over a 6 year period. Routine post-procedure EKGs and echocardiograms were also evaluated with particular attention on echocardiogram paid to residual shunts, spatial position, and alignment toward the atrial septum. A chest x-ray was performed at least one year following GSO implantation to evaluate for the presence of WFFs. The x-rays were reviewed by an interventional cardiologist and pediatric radiologist. WFFs were classified as detailed in the table below.   A total of 91 patients met inclusion criteria, with 4 patients lost to follow up. The median patient age at time of ASD closure was 5 years old (range 2-18 years) with a median weight of 20.2 kg (range 11-95). There were no serious adverse events at the time of implantation or at follow up. One patient developed new onset first degree AV block and three patients had transient supraventricular ectopy immediately after device implantation. All patients were asymptomatic at their most recent clinic visit, with a median follow up time of 42.5 months. Complete defect closure was confirmed in 96.7% of patients, with the remaining patients having only a trivial residual defect. Of the 87 patients that were seen in follow up, 80 had a chest X-ray performed, with 74/80 of adequate diagnostic quality to assess for WFF. WFF was detected in 5/74 patients (6.8%), with only types A and C seen (this may be slightly underestimated as fluoroscopy is more sensitive in detecting WFF than X-ray). No device had more than one fracture. There was no wire frame fragment embolization, device migration, clinical sequelae, or change in echocardiographic appearance (of the device or the appearance of new residual shunts). No predisposing factors (such as device size or deficient retro-aortic rim) were identified.   This small series shows that the GSO is safe and effective for ASD closure in mid-term follow up. Wire frame fractures are seen but do not affect device function or cause clinical sequelae (though this should be interpreted with caution given the small sample size). If there is any concern about the device by transthoracic echocardiography, further investigation (chest X-ray or fluoroscopy) should be pursued.


Pulmonary artery interventions after the arterial switch operation: Unique and significant risks.

Pulmonary artery interventions after the arterial switch operation: Unique and significant risks. Lee J, Abdullah Shahbah D, El-Said H, Rios R, Ratnayaka K, Moore J. Congenit Heart Dis. 2019 Jan 8. doi: 10.1111/chd.12726. [Epub ahead of print] PMID: 30620141 Similar articles Select item 30624624   Take Home Points: Patients s/p ASO for d-TGA (and its variants) commonly develop the need for late re-intervention on the pulmonary arteries. Due to the orientation of the branch PAs (LeCompte position) to the aorta and re-implanted coronary arteries, these patients are at higher risk for development of adverse events related to PA angioplasty/stenting procedures—most notably they are at risk for AP fistula formation and coronary artery compression with PA stent/attempted PA stent placement. Vigilance to coronary arteries (including intraprocedural dynamic balloon testing) is important even with branch PA intervention. Risk factors for AP fistula formation may include overdilation of existing PA stents, stent fracture, and presence of bilateral PA stents Comment from Dr. Wendy Whiteside (Michigan), section editor of Congenital Heart Disease Interventions Journal Watch:  Many patients who undergo the arterial switch operation (ASO) for d-Transposition of the great arteries eventually require reintervention, the most common of which are for pulmonary artery (PA) angioplasty and/or stenting.  There has been an increasing appreciation for potential complications related to stenting branch PA in these patients s/p ASO, with the PAs in a LeCompte position. The branch PAs in this anatomy may be on stretch as they drape over the aorta and the re-implanted coronary arteries may be in close proximity to the MPA or its branches.  This may put patients at risk for development of aorto-pulmonary fistula (at the site of branch PA stent placement) or coronary compression.   Lee et all report their single center experience over a 10-year period (2006-2016) of catheterization procedures performed in patients s/p ASO.  During this time period 47 patients had diagnostic or interventional cath procedures performed; in 29 patients, 37 procedures were performed for pulmonary artery stenosis. Of the 37 procedures, 16 were for angioplasty of PA branches, 21 for stenting of PA branches, 16 for angioplasty of main PA, and 1 for percutaneous pulmonary valve replacement. Median age at the time of c All AP fistulae were treated by covered stent implantation. ath was 11.3 years and time from ASO to catheterization procedure was 10.7 years.  Coronary artery angiography or, more recently, 3D rotational angiography, were performed routinely at this center before intervention in these patients to delineate the proximity of the coronary arteries and bronchi to the targets of stenting. There were 5 major adverse events (14%) including 3 AP fistula and 1 left main coronary artery compression.  There were 6 additional episodes (16%) of intended stent procedures that were aborted (or modified) due to threatened left main coronary artery compression—4 episodes due to close proximity to the LPA and 2 to the supra-valvar MPA area.  All patients with coronary compression had typical coronary artery anatomy. There were no deaths or urgent surgeries. These rates of serious adverse events in patients s/p ASO were significantly higher than would be expected for the larger group of PA angioplasty/stent procedures as a whole (2-3% by IMPACT and C3PO data, respectively). In the 3 patients with AP fistula, 2 were from the LPA and 1 from the RPA and all patients had stent fractures noted at the fistula site.  All stent fractures occurred at time of post-dilation of existing stents.  In one patient, the fistulae developed immediately, with post-angioplasty angiography revealing the shunt, and in the other 2 cases, it was appreciated at a later time with development of congestive heart failure (unknown period of time later) or new continuous murmur (noted 2 years post-stent dilation).  All AP fistulae were successfully treated by covered stent implantation.   While formal risk factor analysis was not performed, the authors suggest that factors leading to fistula formation included re-dilation of stents to larger than recommended diameters, stent fracture, and the close relationship of the stent to the pulsatile, high-pressure aorta.  Interestingly, all patients who developed fistulae had bilateral PA stents in place, suggesting that decreased compliance of the stented PAs against the aorta may contribute added risk. While this is a small series, this and other studies suggest an increased risk of both complications of AP fistula formation and of coronary artery compression when performing PA work in patients s/p ASO.  While we have all become very accustomed to testing coronary arteries prior to transcatheter pulmonary valve implantation and RVOT interventions, it would be prudent to assess coronaries also in these patients s/p ASO, both via cross-sectional imaging prior to the procedure and with possible dynamic testing during the procedure. Additionally, when intervening on LeCompted branch PAs, primary covered stent implantation may be worthwhile to prevent fistula formation, particularly in those with high risk features.  There should be continued vigilance for development of new continuous murmurs/heart failure over time that may be indicative of latera fistula development.


Modified Lung Ultrasound Examinations in Assessment and Monitoring of Positive End-Expiratory Pressure-Induced Lung Reaeration in Young Children With Congenital Heart Disease Under General Anesthesia.

Wu L, Hou Q, Bai J, Zhang J, Sun L, Tan R, Zhang M, Zheng J. Pediatr Crit Care Med. 2019 May;20(5):442-449. doi: 10.1097/PCC.0000000000001865. PMID:  31058784 Similar articles   Take Home Points: The most prevalent region of post-intubation atelectasis in...

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