Interdisciplinary care of children with trisomy 13 and 18.

Children with trisomy 13 and 18 (previously deemed "incompatible with life") are living longer, warranting a comprehensive overview of their unique comorbidities and complex care needs. This Review Article provides a summation of the recent literature, informed by the study team's Interdisciplinary Trisomy Translational Program consisting of representatives from: cardiology, cardiothoracic surgery, neonatology, otolaryngology, intensive care, neurology, social work, chaplaincy, nursing, and palliative care. Medical interventions are discussed in the context of decisional-paradigms and whole-family considerations. The communication format, educational endeavors, and lessons learned from the study team's interdisciplinary care processes are shared with recognition of the potential for replication and implementation in other care settings.

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

OBJECTIVES: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort. DESIGN: Multicenter retrospective study. SETTING: Eleven pediatric hospitals within the United States. PATIENTS: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years. CONCLUSIONS: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Use of Amplatzer vascular plugs and Amplatzer duct occluder II additional sizes for occlusion of patent ductus arteriosus: A multi-institutional study.

BACKGROUND: Variable patent ductus arteriosus (PDA) morphology and the need to close PDAs in small size patients has led physicians to use Amplatzer Vascular Plugs (AVP) and recently available Amplatzer Duct Occluder II - Additional Sizes (ADO II AS). The purpose of this study was to analyze the safety, efficacy, and complication rates of the ADO II AS and AVPs, specifically AVP II. METHODS: All patients undergoing PDA closure with an AVP or ADO II AS from 2011 to 2016 were included. Clinical, echocardiographic, and angiographic data were collected and reviewed. RESULTS: Four hundred and sixty-nine patients were included. Median age was 27 months (0.75-852) and the median weight was 11.4 kg (1-92). There were 51 patients ≤5 kg. Type A PDA was most common in 48% (n = 225), followed by type E (27.5%, n = 129), type D (13%, n = 61), type C (10.2%, n = 48), and type B (1.3%, n = 6). Devices included AVP II (n = 421), ADO II AS (n = 30), and AVP IV (n = 18), left pulmonary artery stenosis occurred in 4.3% (n = 20). One patient required surgery for severe stenosis; the remaining cases were mild and required no intervention. Device embolization occurred in 3 patients (10%) with the ADO II AS. Successful device closure was achieved in 98.9% of cases. CONCLUSIONS: The AVP II was highly effective for closing PDAs in smaller babies with varying morphologies and is safe when used in small sized patients with relatively low risk of complications. More studies are warranted to clarify the risks of ADO II AS.

Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves: An International, Multicenter Registry Study.

BACKGROUND: Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports. METHODS AND RESULTS: An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type. CONCLUSIONS: TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses.

Traditional Long-Term Central Venous Catheters Versus Transhepatic Venous Catheters in Infants and Young Children.

OBJECTIVES: Children with congenital heart disease may require long-term central venous access for intensive care management; however, central venous access must also be preserved for future surgical and catheterization procedures. Transhepatic venous catheters may be an useful alternative. The objective of this study was to compare transhepatic venous catheters with traditional central venous catheters regarding complication rate and duration of catheter service. DESIGN: Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters. SETTING: Single freestanding pediatric hospital located in the central United States. PATIENTS: Pediatric patients with congenital heart disease who underwent placement of transhepatic venous catheter. INTERVENTIONS: Cohort's central venous catheter complication rates and duration of catheter service were compared with transhepatic venous catheter data. MEASUREMENTS AND MAIN RESULTS: Twelve patients had a total of 19 transhepatic venous lines. Transhepatic venous lines had a significantly longer duration of service than central venous lines (p = 0.001). No difference between the two groups was found in the number of documented thrombi, thrombolytic burden, or catheter sites requiring wound care consultation. A higher frequency of infection in transhepatic venous lines versus central venous lines was found, isolated to four transhepatic venous lines that had a total of nine infections. All but one was successfully managed without catheter removal. The difference in the proportion of infections to catheters in transhepatic venous lines versus central venous lines was significant (p = 0.0001), but no difference in the rate of infection-related catheter removal was found. CONCLUSIONS: Without compromising future central venous access sites, transhepatic venous lines had superior duration of service without increased thrombosis, thrombolytic use, or insertion site complications relative to central venous lines. Transhepatic venous catheters had a higher infection rate, and further investigation into the etiology is warranted.

Autoimmunity Against the Heart and Cardiac Myosin in Children With Myocarditis.

BACKGROUND: Host autoimmune activity in myocarditis has been proposed to play a role in development of cardiac disease, but evidence of autoimmunity and relationship to outcomes have not been evaluated in pediatric myocarditis. METHODS: We performed a multi-institutional study of children with clinical myocarditis. Newly diagnosed patients were followed for up to 12 months and previously diagnosed patients at a single follow-up for serum levels of autoantibodies to human cardiac myosin, beta-adrenergic receptors 1 and 2, muscarinic-2 receptors, and antibody-mediated protein kinase A (PKA) activation in heart cells in culture. Results were compared with those of healthy control children. RESULTS: Both previously diagnosed patient at follow-up (P = .0061) and newly diagnosed patients at presentation (P = .0127) had elevated cardiac myosin antibodies compared with control subjects. Antibody levels were not associated with recovery status at follow-up in either group. PKA activation was higher at presentation in the newly diagnosed patients who did not recovery normal function (P = .042). CONCLUSIONS: Children with myocarditis have evidence of autoantibodies against human cardiac myosin at diagnosis and follow-up compared with control subjects. Differences in antibody-mediated cell signaling may contribute to differences in patient outcomes, as suggested by elevated antibody-mediated PKA activation in heart cells by the serum from nonrecovered patients.

Non-infectious thrombosis of the melody® valve: A tale of two cities.

Bioprosthetic valve thrombosis is an uncommon complication. We report two young women with prior uncomplicated percutaneous pulmonary valve replacement (Melody® , Medtronic, Inc. Minneapolis, MN) who later developed symptomatic pulmonary emboli and pulmonary valve dysfunction without evidence of infection. Thrombophilia risk factors included oral contraceptive use and mild thrombophilia. Both experienced recovery of valve function following anticoagulation. Acute changes in Melody® valve function should prompt a thorough investigation for and treatment of potential thrombotic causes. © 2016 Wiley Periodicals, Inc.

High Frequency of Detection by PCR of Viral Nucleic Acid in The Blood of Infants Presenting with Clinical Myocarditis.

Specific viruses are associated with pediatric myocarditis, but the prevalence of viral DNAemia detected by blood polymerase chain reaction (PCR) is unknown. We evaluated the prevalence of known cardiotropic viruses (enterovirus, adenovirus, human herpesvirus 6, and parvovirus B19) in children with clinical myocarditis (n = 21). Results were compared to pediatric controls with similar viral PCR testing. The majority of positive PCR (89 %) was noted in children ≤12 months of age at diagnosis compared to older children. Infant myocarditis patients (8/10) had increased the prevalence of PCR positivity compared to infant pediatric controls (4/114) (p < 0.0001). Other than age, patient characteristics at diagnosis were similar between PCR-positive and PCR-negative patients. Both PCR-negative myocarditis infants had clinical recovery at follow-up. Of the PCR-positive myocarditis infants, 4 had clinical recovery, 2 developed chronic cardiomyopathy, 1 underwent heart transplant, and 1 died. Infants with clinical myocarditis have a high rate of blood viral positivity, which is higher compared to older children with myocarditis and healthy infant controls. Age-related differences in PCR positivity may be due to differences in host and/or virus characteristics. Our findings suggest that viral blood PCR may be a useful diagnostic tool and identify patients who would potentially benefit from virus-specific therapy.

Myhre syndrome: Clinical features and restrictive cardiopulmonary complications.

Myhre syndrome, a connective tissue disorder characterized by deafness, restricted joint movement, compact body habitus, and distinctive craniofacial and skeletal features, is caused by heterozygous mutations in SMAD4. Cardiac manifestations reported to date have included patent ductus arteriosus, septal defects, aortic coarctation and pericarditis. We present five previously unreported patients with Myhre syndrome. Despite varied clinical phenotypes all had significant cardiac and/or pulmonary pathology and abnormal wound healing. Included herein is the first report of cardiac transplantation in patients with Myhre syndrome. A progressive and markedly abnormal fibroproliferative response to surgical intervention is a newly delineated complication that occurred in all patients and contributes to our understanding of the natural history of this disorder. We recommend routine cardiopulmonary surveillance for patients with Myhre syndrome. Surgical intervention should be approached with extreme caution and with as little invasion as possible as the propensity to develop fibrosis/scar tissue is dramatic and can cause significant morbidity and mortality.

Transcatheter Closure of Coronary Artery Fistulae: Considerations and Approaches Based on Fistula Origin.

OBJECTIVES: To investigate technical approaches for transcatheter closure of coronary artery fistula based on anatomic type of the fistula. BACKGROUND: The variability in coronary artery fistulae (CAF) anatomy that necessitates different transcatheter closure (TCC) approaches has not been well documented. METHODS: Records of patients with CAF who underwent TCC at 2 centers were reviewed for technical details and procedural outcome. CAF were classified as proximal and distal. TCC approaches employed were arterio-venous or arterio-arterial loop, retrograde arterial, and antegrade venous. RESULTS: Eighteen patients with CAF, mean age 12.6 years (0.07-60), 11 male (61%), underwent TCC. All CAF drained predominantly into the right side of the heart. Types of CAF were proximal in 15 and distal in 3 patients. CAF calibers were large in 7, medium in 9, and small in 2 patients. The arterio-venous loop approach was used in the majority of the cases (11 patients) and the CAF size were medium to large. The retrograde arterial approach was used in 4; of these, 3 patients had small to medium sized CAF. In 2 patients with long tortuous CAF an antegrade venous approach was employed. TCC was successful in 17 of the 18 patients (94.4%). There were no peri-procedural deaths or vascular complications. CONCLUSIONS: This study documents transcatheter closure approaches for CAF and device selection based on fistula origin. The choices of TCC technique and device selection vary, and are primarily determined by the heterogeneous anatomic characteristics of the fistulae.

Patent ductus arteriosus closure using the Amplatzer® vascular plug II for all anatomic variants.

OBJECTIVES: To evaluate the safety and efficacy of the Amplatzer® Vascular Plug II (AVPII) for closure of the patent ductus arteriosus (PDA). BACKGROUND: The PDA has significant anatomic variation. No device has proven applicable to all PDAs. Previous case reports and small series have documented limited use of the AVPII for some PDA types. We describe the largest and most diverse experience using the AVPII. METHODS: A retrospective analysis of patients undergoing percutaneous PDA closure between 01/01/2009 and 05/01/2012 was performed. The PDA was characterized, measured, and the device chosen was listed. Deployment technique, complications and procedural results were recorded. RESULTS: Sixty-seven procedures were performed. The AVPII was utilized for 43 (64.2%), 15 (20.9%) had coils, 7 (10.4%) had the AGA duct occluder, and 3 (4.5%) were referred for surgery. The AVPII was placed in infants as young as 2 months and 4.2 kg. AVPII size ranged from 4 to 10 mm. All PDA types were closed. Retrograde and antegrade deployments were performed, using the outer disc as a "retention skirt" to secure the device and improve occlusion. Three patients were up-sized prior to release. All deployments were successful; 89% "in-lab" and 100% closure on postprocedural echocardiogram. There were no complications. CONCLUSIONS: We report the largest experience with the AVPII for PDA closure. The device was used in all morphologic types and small patients. It is low profile, easily repositioned, and had excellent results without complications. We contend that this is the most versatile device currently available.

Hybrid peratrial double device closure of a patent foramen ovale and sinus venosus defect in an infant with vein of Galen malformation.

Vein of Galen aneurysmal malformation is the most common cerebral arteriovenous malformation in children. Neurointerventional embolization has improved outcomes with this lesion. An association with cardiac defects has been recognized and can increase the risk of systemic embolic events during treatment. Cardiopulmonary bypass (CPB) to correct the cardiac defect before repair of the malformation may be associated with compromised cerebral perfusion and possible neurologic injury. We describe a successful hybrid peratrial closure of two intracardiac shunts (a patent foramen ovale and a sinus venosus ASD) before neurointervention, avoiding CPB, and eliminating the intracardiac embolic risk during treatment of the vein of Galen malformation.

Percutaneous Deactivation of Left Ventricular Assist Devices.

Left ventricular assist device (LVAD) deactivation may be considered in cases of left ventricular recovery, pump thrombosis, infection, and end-of-life palliation. Surgical pump explantation remains the principal method, but percutaneous deactivation presents a safe and effective alternative. We have developed a formal program for percutaneous LVAD deactivation within our advanced heart failure program including patient selection criteria, preprocedure testing, a procedural algorithm, and a postprocedure care plan. Patient selection for percutaneous LVAD deactivation required review by an interdisciplinary heart transplant team including reason for deactivation, cardiac function, surgical risk, and patient preference. All candidates underwent LVAD ramp studies with both transthoracic echocardiography and right heart catheterization assessment. Deactivation was performed under general anesthesia with transesophageal echocardiography guidance. Three Amplatzer Vascular Plug IIs (Abbott, St. Paul, MN) were deployed in the LVAD outflow cannula with the proximal edge of the third plug aligned with the aortic anastomosis of the graft as guided by angiography and 3-dimensional transesophageal echocardiography. In a separate procedure, the LVAD drive line was transected below the skin, which was closed surgically over the driveline stump. Anticoagulation was continued for at least 3 months. Since initiation in January 2017, our program has performed 7 percutaneous LVAD deactivation procedures. All procedures have been successful, 5 of the patients remain medically managed, and 2 have proceeded to heart transplant. Percutaneous LVAD deactivation provides an alternative to surgical explantation. A percutaneous LVAD deactivation program is an important component of an advanced heart failure program.

Mid-Term Valve-Related Outcomes After Transcatheter Tricuspid Valve-in-Valve or Valve-in-Ring Replacement.

BACKGROUND: Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis. OBJECTIVES: The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes. METHODS: Patients who underwent TTVR after prior surgical TV replacement or repair were collected through an international registry. Time-related outcomes were modeled and risk factors assessed. RESULTS: Data were collected for 306 patients who underwent TTVR from 2008 through 2017 at 80 centers; 52 patients (17%) had a prior history of endocarditis. Patients were followed for a median of 15.9 months after implantation (0.1 to 90 months), with 64% of patients estimated to be alive without TV reintervention or a valve-related event at 3 years. The cumulative 3-year incidence of death, reintervention, and valve-related adverse outcomes (endocarditis, thrombosis, or significant dysfunction) were 17%, 12%, and 8%, respectively. Endocarditis was diagnosed in 8 patients 2 to 29 months after TTVR, for an annualized incidence rate of 1.5% per patient-year (95% confidence interval: 0.45% to 2.5%). An additional 8 patients were diagnosed with clinically relevant valve thrombosis, 3 in the short term, 2 within 2 months, and 3 beyond 6 months. Only 2 of these 8 patients received anticoagulant therapy before thrombus detection (p = 0.13 vs. patients without thrombus). Prior endocarditis was not a risk factor for reintervention, endocarditis, or valve thrombosis, and there was no difference in valve-related outcomes according to TTVR valve type. CONCLUSIONS: TV dysfunction, endocarditis, and leaflet thrombosis were uncommon after TTVR. Patients with prior endocarditis were not at higher risk for endocarditis or other adverse outcomes after TTVR, and endocarditis occurred with similar frequency in different valve types. Though rare, leaflet thrombosis is an important adverse outcome, and further study is necessary to determine the appropriate level of prophylactic therapy after TTVR.

Covered CP Stent for Treatment of Right Ventricular Conduit Injury During Melody Transcatheter Pulmonary Valve Replacement.

BACKGROUND: High-pressure balloon and stent angioplasty are frequently necessary to prepare the dysfunctional right ventricular outflow tract conduit before transcatheter pulmonary valve replacement (TPVR). Conduit injury can result, which may be catastrophic to the patient or prevent successful TPVR. METHODS AND RESULTS: The PARCS trial (Pulmonary Artery Repair With Covered Stent) was a pivotal, prospective multicenter trial to evaluate the safety and efficacy of the NuMED Covered CP Stent (CCPS) for treatment of conduit injury occurring during TPVR. The study also evaluated immediate and short-term TPVR function in patients receiving covered stents. A total of 616 patients were consented; 120 (19.5%) had a wall injury identified and were treated with CCPS. Severe conduit injuries were uncommon (5%), but predictors for severe injury were not identified. Stenotic homografts had the highest incidence of injury (29%), compared with other conduit substrates. Among patients receiving CCPS implant, 96% required no further therapy for conduit injury, and 94% underwent TPVR at that procedure. Only 2 patients (1.6%) required urgent surgery for conduit injury, despite CCPS implant. There were few CCPS-related complications. TPVR function was similar between CCPS and non-CCPS groups at follow-up. CONCLUSIONS: Conduit injury during TPVR is common, although severe injury is rare. The CCPS was a safe and effective treatment for right ventricular outflow tract conduit injury during preparation for TPVR, allowing nearly all patients to complete the procedure without identifiable impact on valve performance. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov . Unique identifier: NCT01824160.

Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly.

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV. The most common indication for TVIV was the presence of moderate or severe tricuspid regurgitation (40%). Most patients received a Melody valve (64%). TVIV was ultimately successful in all patients, and there was no procedural mortality. Four patients (5%) developed acute valve thrombosis, 4 patients (5%) developed endocarditis, and 9 patients (11%) developed valve dysfunction not related to thrombosis or endocarditis. Eight patients (10%) underwent reintervention (2 transcatheter, 6 surgical) due to thrombosis (3), endocarditis (2), other valve dysfunction (2), and patient-prosthesis mismatch without valve dysfunction (1). Among 69 patients who were alive without reintervention at latest follow-up, 96% of those with NYHA status reported were class 1/2, a significant improvement from baseline (62% NYHA class 1/2, p <0.001). In conclusion, transcatheter TVIV offers a low-risk, minimally invasive alternative to surgical tricuspid valve re-replacement in patients with EA and a failing tricuspid valve bioprosthesis.

Multicenter Experience Evaluating Transcatheter Pulmonary Valve Replacement in Bovine Jugular Vein (Contegra) Right Ventricle to Pulmonary Artery Conduits.

BACKGROUND: Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit. METHODS AND RESULTS: This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mm Hg; P<0.001). At most recent follow-up, the maximum pulmonary valve gradient by echocardiogram remained significantly reduced relative to prevalve implant measurements (65.9 versus 27.3 mm Hg; P<0.001). The incidence of Melody transcatheter pulmonary valve stent fracture (3.4%) and infectious endocarditis (4.3%) were both low. Serious adverse events occurred in 3 patients. CONCLUSIONS: Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis.

Usefulness of cutting balloon angioplasty for pulmonary vein in-stent stenosis.

After radiofrequency ablation for atrial fibrillation, patients may develop pulmonary vein stenoses requiring stent angioplasty. The treatment options for when such patients develop in-stent stenoses are poorly defined. The investigators retrospectively reviewed their initial experience with cutting balloon angioplasty for pulmonary vein in-stent stenosis. Ten patients with 21 previously stented pulmonary veins returned to the catheterization laboratory for in-stent stenoses. Angioplasty of individual in-stent stenotic vessels were grouped into standard angioplasty alone (n = 6) and a combination of cutting balloon followed by standard angioplasty (n = 15). Although final mean lesion diameter was increased significantly in the 2 groups, restenosis occurred in 4 of 6 vessels in the group with angioplasty alone and 2 of 15 vessels in the cutting balloon group. In conclusion, cutting balloon angioplasty for pulmonary vein in-stent stenosis appears to improve the intermediate results of repeat angioplasty.

Progressive Aortic Dilation Associated With ACTA2 Mutations Presenting in Infancy.

Mutations in the gene ACTA2 are a recognized cause of aortic aneurysms with aortic dissection in adulthood. Recently, a specific mutation (Arg179His) in this gene has been associated with multisystem smooth muscle dysfunction presenting in childhood. We describe 3 patients with an R179H mutation, all of whom presented with an aneurysmal patent ductus arteriosus. Detailed information on the rate of aortic disease progression throughout childhood is provided. Death or need for ascending aortic replacement occurred in all patients. Genetic testing for ACTA2 mutations should be considered in all infants presenting with ductal aneurysms.

Can we talk? Reflections on effective communication between imager and interventionalist in congenital heart disease.

The rapid proliferation of catheter-mediated treatments for congenital heart defects has brought with it a critical need for cooperation and communication among the numerous physicians supporting these new and complex procedures. New interdependencies between physicians in specialties including cardiac imaging, interventional cardiology, pediatric cardiology, anesthesia, cardiothoracic surgery, and radiology have become apparent, as centers have strived to develop the best systems to foster success. Best practices for congenital heart disease interventions mandate confident and timely input from an individual with excellent adjunctive imaging skills and a thorough understanding of the devices and procedures being used. The imager and interventionalist must share an understanding of what each offers for the procedure, use a common terminology and spatial orientation system, and convey concise and accurate information about what is needed, what is seen, and what cannot be seen. The goal of this article is to review how the cardiovascular imaging specialists and interventionalists can work together effectively to plan and execute catheter interventions for congenital heart disease.