Using electronic health record data to link families: an illustrative example using intergenerational patterns of obesity.

OBJECTIVE: Electronic health record (EHR) data are a valuable resource for population health research but lack critical information such as relationships between individuals. Emergency contacts in EHRs can be used to link family members, creating a population that is more representative of a community than traditional family cohorts. MATERIALS AND METHODS: We revised a published algorithm: relationship inference from the electronic health record (RIFTEHR). Our version, Pythonic RIFTEHR (P-RIFTEHR), identifies a patient's emergency contacts, matches them to existing patients (when available) using network graphs, checks for conflicts, and infers new relationships. P-RIFTEHR was run on December 15, 2021 in the Northwestern Medicine Electronic Data Warehouse (NMEDW) on approximately 2.95 million individuals and was validated using the existing link between children born at NM hospitals and their mothers. As proof-of-concept, we modeled the association between parent and child obesity using logistic regression. RESULTS: The P-RIFTEHR algorithm matched 1 157 454 individuals in 448 278 families. The median family size was 2, the largest was 32 persons, and 247 families spanned 4 generations or more. Validation of the mother-child pairs resulted in 95.1% sensitivity. Children were 2 times more likely to be obese if a parent is obese (OR: 2.30; 95% CI, 2.23-2.37). CONCLUSION: P-RIFTEHR can identify familiar relationships in a large, diverse population in an integrated health system. Estimates of parent-child inheritability of obesity using family structures identified by the algorithm were consistent with previously published estimates from traditional cohort studies.

The incidence of recurrent laryngeal nerve injury resulting in vocal cord paralysis following interventional congenital catheterisation procedures.

BACKGROUND: Recurrent laryngeal nerve injury leading to vocal cord paralysis is a known complication of cardiothoracic surgery. Its occurrence during interventional catheterisation procedures has been documented in case reports, but there have been no studies to determine an incidence. OBJECTIVE: To establish the incidence of left recurrent laryngeal nerve injury leading to vocal cord paralysis after left pulmonary artery stenting, patent ductus arteriosus device closure and the combination of the procedures either consecutively or simultaneously. METHODS: Members of the Congenital Cardiovascular Interventional Study Consortium were asked to perform a retrospective analysis to identify cases of recurrent laryngeal nerve injury after the aforementioned procedures. Twelve institutions participated in the analysis. They also contributed the total number of each procedure performed at their respective institutions for statistical purposes. RESULTS: Of the 1337 patients who underwent left pulmonary artery stent placement, six patients (0.45%) had confirmed vocal cord paralysis. 4001 patients underwent patent ductus arteriosus device closure, and two patients (0.05%) developed left vocal cord paralysis. Patients who underwent both left pulmonary artery stent placement and patent ductus arteriosus device closure had the highest incidence of vocal cord paralysis which occurred in 4 of the 26 patients (15.4%). Overall, 92% of affected patients in our study population had resolution of symptoms. CONCLUSION: Recurrent laryngeal nerve injury is a rare complication of left pulmonary artery stent placement or patent ductus arteriosus device closure. However, the incidence is highest in patients undergoing both procedures either consecutively or simultaneously. Additional research is necessary to determine contributing factors that might reduce the risk of recurrent laryngeal nerve injury.

Diversity of Cardiac and Gastrointestinal Presentations of Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19: A Case Series.

COVID-19 is generally a benign or asymptomatic infection in children, but can occasionally be severe or fatal. Delayed presentation of COVID-19 with hyperinflammation and multi-organ involvement was recently recognized, designated the Multisystem Inflammatory Syndrome in Children (MIS-C). Six children with MIS-C with molecular and serologic evidence of SARS-CoV-2 infection were admitted to our hospital between May 5, 2020 and June 25, 2020. All had fever and weakness; 4/6 presented with gastrointestinal symptoms. Two children had features of complete Kawasaki disease, 3 had incomplete Kawasaki disease, while 1 had terminal ileitis with delayed onset of circulatory shock. Treatment consisted of intravenous immunoglobulin and aspirin for Kawasaki-like disease. Remdesivir, corticosteroids, and infliximab were used when indicated. Median hospitalization was 7 days. Immediate treatment resulted in rapid clinical improvement. In children presenting with hyperinflammatory syndromes without cardiac manifestations, testing for SARS-CoV-2 RNA and antibodies, with close cardiac monitoring should be pursued due to the manifold presentations of SARS-CoV-2 infection in children.

Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

Diagnosis and Management of the Unligated Vertical Vein in Repaired Total Anomalous Pulmonary Venous Connection.

During initial repair of supracardiac total anomalous pulmonary venous connection (TAPVC), the vertical vein (VV) is sometimes left patent (not ligated or divided) in the hope that this strategy may reduce the likelihood or severity of postoperative pulmonary hypertensive crises. We report a case of a 35-year-old pregnant patient with previously repaired supracardiac TAPVC who presented with atrial arrhythmia and right heart dilation. A cardiac magnetic resonance imaging study confirmed the diagnosis of patency of the vertical vein and right heart dilation. The VV was occluded with a catheter-delivered vascular occlusion device through a percutaneous approach, resulting in resolution of right heart dilation and arrhythmia. This case highlights the role of cross-sectional imaging as an adjunct to echocardiography in adults with repaired congenital heart disease.

Association between patient age at implant and outcomes after transcatheter pulmonary valve replacement in the multicenter Melody valve trials.

OBJECTIVES: To investigate whether age and valve size at implant contribute to outcomes after Melody transcatheter pulmonary valve replacement (TPVR). BACKGROUND: Patient age and valve size at implant contribute to longevity of surgical pulmonary valves. METHODS: All patients discharged with a Melody valve in the pulmonary position, as part of three prospective Melody valve multicenter studies, comprised the study cohort. Acute and time-related outcomes were analyzed according to age: children (≤12 years), adolescents (13-18 years), young adults (19-29 years), and older adults (≥30 years). RESULTS: Successful Melody valve implantation occurred in 49 children, 107 adolescents, 96 young adults, and 57 older adults. Pediatric patients (≤18 years) were more likely to have TPVR for conduit stenosis than adults (62% vs. 44%); children had the smallest conduits. After TPVR, pediatric and adult patients had similar decreases in right ventricular (RV) size by MRI, but adults had improved percentage predicted peak VO2 (58% preimplant to 64% postimplant, p = .02) and FEV1 (69% pre to 71% post, p = .005). Younger age was associated with shorter freedom from RVOT dysfunction, reintervention, and explant. Children had the shortest freedom from endocarditis (p = .041), but all other groups had 5-year freedom from endocarditis of ≥90%. CONCLUSIONS: Younger age was associated with shorter time to RVOT dysfunction, reintervention, and explant after Melody TPVR. Patients ≥13 years of age were at low risk for endocarditis and explant to 5 years. A better understanding of time-related outcomes by age will aid in the comparison of therapeutic options for TPVR candidates. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT00740870 (NCT00740870), https://clinicaltrials.gov/ct2/show/NCT01186692 (NCT01186692), and https://clinicaltrials.gov/ct2/show/NCT00688571 (NCT00688571).

Closure of insufficient, native right ventricular outflow tract with AMPLATZER™ muscular ventricular septal defect occluder in a patient with tetralogy of Fallot post-Melody® valve.

AMPLATZER™ muscular ventricular septal defect occluder is used commonly for off-label purposes. We describe an unusual case of a patient with tetralogy of Fallot who underwent repair with a right ventricle to pulmonary artery homograft due to abnormal coronary artery pattern. During the initial surgery, the native right ventricular outflow tract was left open. At 30 years of age, he was symptomatic due to severe native right ventricular outflow tract insufficiency. Cardiac MRI confirmed a dilated right ventricle and pulmonary insufficiency through the native right ventricular outflow tract, despite no significant homograft insufficiency due to previous Melody® valve placement. The right ventricular outflow tract was closed successfully using an 18 mm AMPLATZER™ muscular ventricular septal defect occluder. At 5-year follow-up, there is no native right ventricular outflow tract insufficiency and no additional arrhythmia. We suggest that percutaneous closure of the insufficient; native right ventricular outflow tract using a septal occluder is an alternative to surgical management.

Implantation of the Melody transcatheter pulmonary valve PB1016 in patients with dysfunctional right ventricular outflow tract conduits.

OBJECTIVES: This study describes procedural and 1-year outcomes of the 16 mm Melody PB1016 valve in patients with dysfunctional RVOT conduits. BACKGROUND: The Melody PB1016 is a standard Melody valve produced from a 16 mm bovine jugular vein and is intended for deployment up to 20 mm. METHODS: This is a prospective, non-randomized, multicenter study of the procedural and short-term outcomes of Melody PB1016 TPV replacement within dysfunctional RVOT conduits. Data from eight centers were included in the analysis. RESULTS: During the study period, 39 patients underwent attempted Melody TPVR. Of the 39 patients, 30 underwent successful Melody TPVR. The majority of patients underwent placement of one or more stents prior to TPVR. There was a significant reduction in peak conduit pressure gradient following TPVR (38 mmHg vs. 11 mmHg, P < 0.001). There were three cases of confined conduit tears successfully treated with covered stents or the valve itself. Repeat catheterization was performed in one patient for early re-obstruction that was successfully treated with balloon valvuloplasty. At recent follow-up, there were no cases of more than mild valve regurgitation and the mean pulmonary valve gradient by echocardiogram remained reduced relative to pre-TPVR implant measurements (33.5 mmHg vs. 15.2 mmHg). There were no cases of valve stent fracture or endocarditis reported at the 1-year follow-up. CONCLUSIONS: Our analysis of TPVR with the PB1016 valve in RVOT conduits showed it to be safe and effective and can be performed in a wide range of conduit sizes with preserved valve function. ClinicalTrials.gov Identifier: NCT02347189.

Catheter-directed therapy for acute pulmonary embolism in children.

BACKGROUND: Acute pulmonary embolism is a life-threatening condition and rarely occurs in children. In adults, catheter-directed therapy emerges as a potentially safer and effective therapeutic option. However, there is a paucity of data on the safety and efficacy of catheter-directed therapy for pulmonary embolism in children. We report a single-centred experience of catheter-directed therapy for acute pulmonary embolism in children. METHODS: This is a retrospective study of children who had no CHD and underwent catheter-directed therapy at Detroit Medical Center during a 12-year period from 2005 to 2017. Demographic and clinical data associated with pulmonary embolism were collected along with the outcome. RESULTS: A total of nine patients of median age 16 years with the range from 12 to 20 received catheter-directed therapy for sub-massive (n = 6) and massive pulmonary embolism (n = 3). Among nine patients, one patient received Angiojet thrombectomy and balloon angioplasty, whereas eight patients received catheter-directed thrombolysis using tissue plasminogen activator through infusion catheters (n = 3) or EkoSonic ultrasound-accelerated thrombolysis system (n = 5). In four out of five patients treated with EkoSonic, significant clinical improvement was noticed within 24 hours. Among seven patients who survived, two patients had minor gastrointestinal bleeding with median hospital stay of 8 days with the range from 5 to 24 days, and two patients with massive pulmonary embolism died possibly due to delayed institution of catheter-directed therapy. CONCLUSION: Catheter-directed therapy with/without EkoSonic is an emerging alternative therapy for sub-massive and massive pulmonary embolism in children. A timely institution of catheter-directed therapy appeared important to improve the outcome.

Reducing radiation exposure by lowering frame rate in children undergoing cardiac catheterization: A quality improvement study.

INTRODUCTION: Reduction of radiation dosage in the pediatric cardiac catheterization laboratory (PCL) is important to reduce the risk of its stochastic effect in children with congenital heart disease. Lowering the frame rate would reduce radiation dosage possibly at the expense of image quality, potentially resulting in higher fluoroscopic time and procedural complication rate. METHODS: The data were retrospectively analyzed in three eras: era 1 (n = 234), cineangiography 30 frames/sec (f/s) and fluoroscopy 15 pulse/sec (p/s); era 2 (n = 381), cineangiography 30 f/s and fluoroscopy 6 p/s; and era 3 (n = 328), cineangiography 15 f/s and fluoroscopy 6 p/s. Also, three operators blinded to the frame rate setting evaluated cineangiography image quality. In this study, the impact of lowering the default frame rates on radiation dosage, fluoroscopic time, contrast volume, diagnostic image quality, and complication rates in the PCL was assessed. RESULTS: Overall radiation dosage progressively declined during these eras (70.0 vs 64.1 vs 36.6 µGym2 /kg, P < .001) without a difference in significant adverse event rates. There was no significant increase in either fluoroscopy time or contrast volume. There was no difference in the diagnostic image quality between cineangiography 30 and 15 f/s. Lowering the default frame/pulse rates of both fluoroscopy and cineangiography significantly decreased the overall radiation dosage in the PCL. Importantly, fluoroscopy time, contrast volume, and complication rates did not increase while maintaining diagnostic image quality. CONCLUSION: This quality improvement project proved successful in lowering radiation dosage without compromising the efficacy and safety of catheterizations.

Transcatheter pulmonary valve replacement using the melody valve for treatment of dysfunctional surgical bioprostheses: A multicenter study.

BACKGROUND: Stented bioprosthetic valves (BPVs) are commonly used for surgical pulmonary valve (PV) replacement in postoperative congenital heart disease, but develop structural failure in a time-related fashion. The Melody transcatheter PV (TPV) (Medtronic, Minneapolis, Minn) has been used to treat BPV dysfunction, but there have been few studies in this population. METHODS: We performed a retrospective, multicenter study to evaluate Melody valve function in patients who underwent TPV replacement (TPVR) into a dysfunctional pulmonary BPV. RESULTS: One hundred patients who underwent TPVR at 10 centers between January 2010 and June 2015 were enrolled. The median patient age was 22 years (range, 5-79 years), and 32 patients were age <18 years. The underlying diagnosis was tetralogy of Fallot in 80 patients, and moderate or severe pulmonary regurgitation (PR) was present in 84%. The TPV was implanted into various types of BPVs, with a median size of 23 mm (range, 19-33 mm). At hospital discharge, PR was mild or less in all but 1 patient, and the mean Doppler right ventricular outflow tract (RVOT) gradient was reduced from a mean of 29.3 ± 12.0 mm Hg to 16.2 ± 6.9 mm Hg (median, 29 mm Hg to 16 mm Hg; P < .001). During follow-up (median, 12.4 months), no patients underwent reintervention on the TPV. Endocarditis was diagnosed in 1 patient who was managed medically without intervention. The mean RVOT gradient at the most recent follow-up was ≤35 mm Hg in all patients, and was similar to that at early postimplantation. PR was more than mild in only 1 patient. Hemodynamic outcomes did not differ between patients with small BPVs (≤23 mm) and those with large BPVs (≥25 mm). CONCLUSIONS: TPVR restores competence and relieves the obstruction of dysfunctional surgical BPVs, with excellent early results in both small and large BPVs, highlighting the potential for TPVR to extend the life of existing BPVs in adults and children. Collaboration between surgeons and cardiologists is important to determine the optimal lifetime management, combining surgical PV replacement and TPVR in this population.

Bilateral branch pulmonary artery valve implantation in repaired tetralogy of fallot.

BACKGROUND: Transcatheter, bilateral branch pulmonary artery (PA) valve implantation is a novel treatment for patients with severe pulmonary insufficiency and oversized right ventricle (RV) outflow tract. There is scarce data on efficacy and safety of this approach. METHODS: This was a retrospective study of 8 patients with repaired tetralogy of fallot (TOF) who underwent bilateral branch PA valve implantation. Demographics, echocardiography, cardiac catheterization, and axial imaging data were reviewed. Variables were compared by a paired sample t-test. RESULTS: All patients were adult sized (weight 43-99 kg) with oversized RV outflow tract not suitable for conventional transcatheter pulmonary valve implantation. Staged bare metal PA stenting followed by valve implantation (interval 3-5 months) was technically successful in 7 patients with one stent embolization. In another patient, proximal stent migration prevented placement of bilateral pulmonary valve stents. There were a total of 14 valved branch PA stents placed (Melody valve n = 9, Sapien XT n = 2, Sapien 3 n = 3). In the 7 patients undergoing successful branch pulmonary valve placement, at median follow up of 10 months (range 3 months to 6 years), 13 (93%) valves had none/trivial insufficiency on echocardiography. Prevalve and postvalve implantation cardiac magnetic resonance imaging in five patients showed significant reduction of indexed RV end-diastolic volume (152 ± 27 to 105 ± 15 mL/m2 , P < .001). CONCLUSIONS: Transcatheter, bilateral branch PA valve implantation was technically feasible with satisfactory efficacy and safety in patients with repaired TOF, severe pulmonary insufficiency, and oversized RV outflow tracts. Elimination of pulmonary insufficiency with this method resulted in reduced RV end-diastolic volume. This approach can be offered as an alternative to surgery, particularly in patients considered high risk for standard surgical placement and who are not candidates for the newer self-expanding valve prosthesis for placement in RV outflow tracts larger than 30 mm diameter.

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.

Parental anxiety among children undergoing cardiac catheterisation.

BACKGROUND: Parents may experience anxiety and stress when their children undergo cardiac catheterisation. The goal of this study was to assess the level of anxiety in parents of children undergoing cardiac catheterisation and to identify factors that were associated with level of anxiety. METHODS: This was a cross-sectional survey of parents of children who underwent cardiac catheterisation. Anxiety levels were measured using a validated self-report questionnaire - State-Trait Anxiety Inventory, which generates state anxiety scores on the current state of anxiety and trait anxiety scores on the stable aspects of anxiety proneness. One sample t-test was used to compare the data with normative data. Multiple linear regression was used to assess the factors associated with the state score. RESULTS: A total of 113 parents completed the survey. The mean age of parents was 34.0±7.7 years and the mean age of children undergoing catheterisation was 6.7±5.7 years. Compared with normative data, mean state score was significantly higher in our cohort (p<0.05) despite no difference in the trait score. Final multivariate model showed that the state score was significantly associated with child age group (<1 year [coefficient ß 7.2] and 10-18 years [6.3], compared to 1 to <10 years of age [reference]) and history of previous catheterisation (-5.2) (p<0.05). CONCLUSIONS: Concurrent state anxiety level was high among parents of children undergoing cardiac catheterisation, whereas trait anxiety level was not. Higher anxiety was experienced by parents of infants and adolescents without a history of previous catheterisation.

Closure of Secundum Atrial Septal Defects With the AMPLATZER Septal Occluder: A Prospective, Multicenter, Post-Approval Study.

BACKGROUND: Prospective data on the medium-term safety and effectiveness of the AMPLATZER Septal Occluder in clinical practice are not available. The objective of this study was to prospectively evaluate the risk of hemodynamic compromise and obtain medium-term survival data on patients implanted with the AMPLATZER Septal Occluder for percutaneous closure of secundum atrial septal defects. METHODS AND RESULTS: Subjects were enrolled prospectively at 50 US sites and followed for 2 years. Between 2008 and 2012, atrial septal defect closure with the AMPLATZER Septal Occluder was attempted in 1000 patients (aged 0.3-83.6 years, mean 21±22 years). Procedural closure occurred in 97.9%, with 1-month and 2-year closure 98.5% and 97.9%, respectively. Hemodynamic compromise occurred in 6 subjects (0.65%), because of dysrhythmia in 2, device embolization in 1, and cardiac erosion in 3. The rate of cardiac erosion was 0.3% (average 83, range 12-171 days from implant). CONCLUSIONS: Closure of atrial septal defect with the AMPLATZER Septal Occluder is safe and effective. The rate of hemodynamic compromise and cardiac erosion is rare. The risk factors for cardiac erosion after device closure are not yet clear. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00650936.

Impact of Transcatheter Intervention on Myocardial Deformation in Patients with Coarctation of the Aorta.

Myocardial deformation measured by speckle tracking echocardiography can detect subtle abnormalities of left ventricular function before an obvious abnormality in traditional echocardiographic parameters such as ejection fraction is seen. We hypothesized that patients with coarctation of the aorta (CoA) may have impaired myocardial deformation that may persist even after successful transcatheter intervention. This is a retrospective study to assess the myocardial deformation in patients undergoing transcatheter intervention of CoA. The data were compared with age-matched normal controls. Echocardiographic parameters were obtained before, immediately and at median 6 months (range 3-8 months) after transcatheter intervention. Myocardial deformation indices were obtained off-line using Tomtec 2D Cardiac Performance Analysis Software. Repeated measure ANOVA was used to compare the indices between three time points. Independent sample t test or Chi-square test was used to compare data between groups. Twenty-four patients (age 13.5 ± 7.7 years) underwent successful transcatheter CoA intervention (stent 19, balloon angioplasty five) improving CoA peak gradient in the catheterization laboratory from 27.1 ± 11.3 to 4.5 ± 3.0 mmHg (p < 0.001). To compare with normal controls (n = 25, age 14.5 ± 1.9 years), fraction shortening was significantly higher in pre-intervention CoA patients (40.4 ± 7.1 vs. 33.8 ± 2.4 %, p < 0.001). In contrast, CoA patients had significantly abnormal left ventricular longitudinal strain compared to normal controls (-14.9 ± 2.6 vs. -20.5 ± 1.8 %, p < 0.001). In CoA patients, left ventricular longitudinal strain improved immediately and 3-8 months after intervention (-18.0 ± 2.9 and -17.6 ± 2.9 %, p < 0.007) but continued to be abnormal compared to normal controls. Patients with CoA had impaired myocardial deformation compared to normal controls. Myocardial mechanics improved but did not normalize even after successful transcatheter intervention on CoA in the short term.

Effect of Hybrid stage 1 procedure on ventricular function in infants with hypoplastic left heart syndrome.

OBJECTIVE: The effect of Hybrid stage 1 palliation for hypoplastic left heart syndrome on right ventricular function is unknown. We sought to compare right ventricular function in normal neonates and those with hypoplastic left heart syndrome before Hybrid palliation and to assess the effect of Hybrid palliation on right ventricular function, using the right ventricular myocardial performance index and the ratio of systolic and diastolic durations. METHODS: We carried out a retrospective review of echocardiographic data on 23 infants with hypoplastic left heart syndrome who underwent Hybrid palliation and 35 normal controls. Data were acquired before Hybrid and after Hybrid palliation - post 1, 0-4 days; post 2, 1 week; post 3, 2-3 weeks; post 4, 1-1.5 months following Hybrid palliation. RESULTS: Myocardial performance index and ratio of systolic and diastolic durations were higher in the pre-Hybrid hypoplastic left heart syndrome group (n=23) - 0.47±0.16 versus 0.25±0.07, p<0.001; 1.59±0.44 versus 1.09±0.14, p<0.0001 - compared with controls (n=35). There was no significant change in the myocardial performance index at any of the post-Hybrid time points. Ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid - post 3: 2.08±0.62 and post 4: 2.21±0.45 versus pre: 1.59±0.44, p=0.043 and 0.003. There were no significant differences in parameters between sub-groups of infants who died (n=10) and survivors (n=13). CONCLUSIONS: Right ventricular myocardial performance index and ratio of systolic and diastolic durations were significantly higher in infants with hypoplastic left heart syndrome before intervention compared with controls. The ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid palliation. Our data suggest that infants with hypoplastic left heart syndrome have right ventricular dysfunction before intervention, which worsens over 2 weeks after Hybrid palliation.

Usefulness of vascular stenting with and without transvenous pacing leads for vena caval obstruction among children and adults with repaired congenital heart disease.

Vena caval obstruction (VCO) is a common complication after vascular manipulation for congenital heart disease. Long-term efficacy of stent therapy for relief of VCO and long-term stent patency with and without intrastent transvenous pacing leads (TPLs) is not well described. This was a retrospective review of patients treated for VCO, including those who received intrastent TPLs, between 1995 and 2012. Patient demographics, diagnoses, vascular pressure gradients, and vessel diameters were analyzed. Forty-one patients (mean age 23.5 ± 10.3 years) with and without congenital heart disease underwent stent implantation, 26 of whom also received intrastent TPLs. Short-term stent implantation success in relieving obstructions was 93%. Poststent vascular pressure gradients and percentage vascular narrowing significantly improved (from 6.2 ± 4.5 to 1.1 ± 1.6 mm Hg and from 63.1 ± 19.5% to 18.0 ± 17.1%, respectively, p <0.05). On follow-up in 38 of 41 patients from 0.2 to 18 years (median 6.0), all survived; 6 (14%) required stent reintervention. Freedom from reintervention was 87% at 15 years. Patients with short-term procedural failure were at higher risk for stent reintervention. Among 27 patients with intrastent TPLs, freedom from reintervention was 96%. In 26 patients with follow-up catheterization, intrastent intimal proliferation was not significantly associated with TPL but was higher in the superior vena cava-innominate vein junction compared with other stent locations (p <0.05). In conclusion, stent therapy for VCO can be successfully and safely performed with good long-term results. Pre-pacing lead stent placement for VCO is effective in allowing TPL placement with encouraging long-term patency.

Transcutaneous removal of an intravenous catheter fragment using a spider FX™ Embolic Protection device.

Long-term use of intravenous catheters can lead to catheter fracture and embolization of fragments. Transcutaneous retrieval of these catheter fragments can be challenging because of their fragility. We report an 8-year-old boy with Hemophilia disease who underwent removal of intravenous Port catheter after 7 years of use, resulting in embolization of fractured catheter fragments into the distal pulmonary arteries. The snare technique to pull the snared fragment into a sheath was unsuccessful, and it leads to further breakdown due to its fragility. An alternative technique using a combination of a snare kit and a Spider FX™ Embolic Protection Device was employed. This technique allowed the fragments to be secured proximally with the basket device and distally with the snare. The unit was then pulled through a sheath and removed from the body. To our knowledge, Spider FX™ Device has not been used in this way before.

One-year follow-up of the Melody transcatheter pulmonary valve multicenter post-approval study.

OBJECTIVES: This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial. BACKGROUND: TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years. The Melody TPV received U.S. Food and Drug Administration approval in 2010 as a Humanitarian Use Device. METHODS: Patients with dysfunctional RVOT conduits were entered in this prospective, nonrandomized study at 10 centers. The primary endpoint was acceptable hemodynamic function at 6 months post-implantation, defined as a composite of RVOT echocardiographic mean gradient ≤30 mm Hg, pulmonary regurgitation less than moderate as measured by echocardiography, and freedom from conduit reintervention and reoperation. RESULTS: Cardiac catheterization was performed in 120 patients for potential implantation of the Melody TPV; of these, 100 patients were implanted, with a 98.0% procedural success rate. There were no procedure-related deaths. Acceptable hemodynamic function at 6 months was achieved in 96.7% of patients with evaluable data (87.9% of the entire implanted cohort), with results maintained through 1 year. No patient had moderate or severe pulmonary regurgitation after implantation. No patient required catheter reintervention in the first year after implantation, and 2 patients required reoperation for conduit replacement. The rate of freedom from TPV dysfunction was 96.9% at 1 year. CONCLUSIONS: This first prospective, real-world experience with the Melody TPV in the United States demonstrates continued high procedural success, excellent short-term TPV function, and low reintervention and reoperation rates at 1 year. (Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692).