Percutaneous Balloon-Expandable Stent Implantation to Treat Transverse Aortic Arch Obstruction: Medium- to Long-Term Outcomes of a Retrospective Multicenter Study.

BACKGROUND: Transverse aortic arch obstruction is a challenging lesion for which stent implantation provides a potentially important alternate therapy. The objectives were to evaluate the technical, procedural, and medium-to-long-term clinical outcomes of percutaneous stent implantation of transverse aortic arch obstruction. METHODS: This is a retrospective, multicenter study of transverse aortic arch stent implantation. Univariable and multivariable analyses were performed. RESULTS: Index catheterization included 187 stent implants in 146 patients. The median age is 14.3 years (interquartile range, 9.3-19), weight is 53 kg (30-69), and follow-up is 53 months (12-120). The most common stent design was open cell (n=90, 48%). Stents overlapped 142 arch vessels (37 carotid arteries) in 118 (81%) cases. Technical and procedural success rates were 100% and 88%, respectively. Lower weight (P=0.018), body surface area (P=0.013), and minimum-to-descending aortic diameter ratio (P<0.001) were associated with higher baseline aortic gradient. The residual gradient was inversely associated with implant and final dilation diameters (P<0.001). The combined incidence of aortic injury and stent-related complications was 14%. There were no reports of abnormal brain scans or stroke. Blood pressure cuff gradient, echocardiographic arch velocity, and hypertension rates improved within 1-year follow-up with increased antihypertensive medication use. Reintervention was reported in 60 (41%) patients at a median of 84 (22-148) months to first reintervention. On multivariable logistic regression, residual aortic gradient >10 mm Hg was associated with increased odds of reintervention at all time points when controlling for each final dilation diameter, weight, and minimum-to-descending aortic diameter ratio. CONCLUSIONS: Transverse aortic arch stent implantation has high rates of technical, procedural, and medium-to-long-term clinical success. Aortic gradient >10 mm Hg is associated with increased odds of reintervention at 1-year and most recent follow-ups. Open cell stent design was frequently used for its advantages in conformability, perfusion of arch vessels, low fracture rate, and the ability to perform effective angioplasty of side cells.

Utility of Follow-Up Echocardiograms in Uncomplicated PDA Device Closures Performed During Infancy.

INTRODUCTION: Guidelines recommend lifelong follow-up with transthoracic echocardiograms (TTE) for patients who had a patent ductus arteriosus (PDA) device closure via catheterization. The goal of this study was to determine the utility of follow-up TTE in patients who underwent an uncomplicated PDA device closure during infancy. METHODS: Chart review was performed on patients who had a PDA closure at not more than 1 year of age between January 1, 2002 and June 1, 2020. Patients were excluded if they had other congenital heart disease, did not have a follow-up TTE at least 3 months after procedure, or had a velocity greater than 2.0 m/s in the left pulmonary artery (LPA) or descending aorta (DAo) on the first TTE at least 3 months after device placement. Time points included the first TTE after the procedure, first TTE at least 3 months after procedure, and the most recent TTE. RESULTS: Total of 147 infants met the inclusion criteria. Age and weight at initial procedure were 141 ± 217 days and 4.2 ± 2.8 kg. There was no significant difference in DAo velocity between initial and most recent TTE. LPA velocity and left ventricular diastolic Z score significantly decreased between initial and most recent TTE. Seventy-eight patients had repeat echocardiograms more than 1 year after PDA procedure with no change in clinical management. No patient underwent an intervention on the LPA or DAo for stenosis. CONCLUSION: In patients who underwent an uncomplicated PDA closure during infancy, TTE parameters either stayed stable or improved over time. These findings need to be corroborated in larger studies with longer follow-up. If verified, the long-term TTE guidelines may need to be simplified for this patient population.

Utility of Follow-Up Echocardiograms in Uncomplicated PDA Device Closures Performed After Infancy.

INTRODUCTION: Guidelines recommend lifelong follow-up with transthoracic echocardiograms (TTE) for patients who had a patent ductus arteriosus (PDA) device closure via catheterization. The goal of this study was to determine the utility of follow-up TTE in patients who underwent an uncomplicated PDA device closure after infancy. METHODS: Chart review was performed on patients who had a PDA closure ≥ 1 year of age between 1/1/2002 and 6/1/2020. Patients were excluded if they had other congenital heart disease, did not have a follow-up TTE ≥ 6 months after procedure, had a residual PDA or velocity > 2.0 m/s in the left pulmonary artery (LPA) or descending aorta (DAo) on the first TTE ≥ 6 months after device placement. Time points included the initial TTE after the procedure, first TTE ≥ 6 months after procedure, and the most recent TTE. RESULTS: A total of 189 patients met the study criteria. The median age and weight at initial procedure were 2.7 (1.0-64.7) years and 12.5 (3.4-69.2) kg. Most recent TTE was performed 2.0 (0.4-17.0) years after PDA closure. There were no significant differences in fractional shortening (36.4 ± 5.0% vs. 36.9 ± 5.6%) or LPA velocity (1.1 ± 0.4 m/s vs. 1.1 ± 0.4 m/s) from initial to most recent TTE, respectively. Left ventricular internal diastolic diameter Z-score significantly decreased (1.4 ± 1.8 vs. - 0.01 ± 1.2, p < 0.01) and DAo peak velocity significantly increased (1.2 ± 0.3 m/s vs. 1.3 ± 0.3 m/s, p = 0.02) from initial to most recent TTE, respectively. No patient died or underwent an intervention on the LPA or DAo for stenosis. Seventy-five patients had a total of 208 repeat TTE > 1 year after PDA procedure with no change in clinical management. CONCLUSIONS: In patients who underwent an uncomplicated PDA closure after infancy, TTE parameters improved or stayed within normal limits on the most recent TTE. Repeat lifetime TTEs after 1-year post-device placement in this population may not necessarily be needed if there are no clinical concerns.

Incidence of and Risk Factors for Aortic Arch Interventions After the Comprehensive Stage II Procedure for Hypoplastic Left Heart Syndrome.

Minimal data exist about the incidence and risk factors for arch intervention after comprehensive stage II (CSII). Goal of this study was to document incidence of arch interventions after CSII and determine if any differences existed between those who underwent an arch intervention (aiCSII) versus those did not have an intervention. Single-center retrospective chart review of all hypoplastic left heart syndrome patients who underwent a CSII between 6/1/2005 and 2/1/2020 was performed. Univariate analysis was conducted in addition to principal components analysis (PCA). One hundred patients were evaluated. Sixteen patients underwent 24 arch interventions. Age at initial arch reintervention was 1.3 ± 1.2 years (median 1.0 years, range 0.5-2.2 years). Univariate analysis showed that the aiCSII group were more likely to be female, to have had a retrograde arch intervention post-hybrid procedure, and to be younger at time of CSII. On echocardiograms, aiCSII group had significantly higher pre-CSII patent ductus arteriosus velocities, arch velocities on their 1st post-operative and discharge study post-CSII, and arch velocities pre-Fontan. Gradients were higher in the aiCSII via pre-Fontan catheterization. With PCA, echocardiographic and catheterization data remained significantly associated with aiCSII versus those who did not undergo an arch intervention (OR = 4.5 (1.9, 19.8), p = 0.008). Incidence of arch intervention post-CSII was 16%. Echocardiographic arch velocities during the CSII hospitalization were the strongest predictors for subsequent aortic arch interventions. Further studies are needed to determine any modifiable variables that may reduce the incidence of arch interventions.

Implantable pulmonary artery pressure monitoring device in patients with palliated congenital heart disease: Technical considerations and procedural outcomes.

OBJECTIVE: We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF). BACKGROUND: Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population. METHODS: We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated. RESULTS: CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval. CONCLUSIONS: Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics.

Follow-up after Percutaneous Patent Ductus Arteriosus Occlusion in Lower Weight Infants.

OBJECTIVES: To describe longer term outcomes for infants <6 kg undergoing percutaneous occlusion of the patent ductus arteriosus (PDA). STUDY DESIGN: This was a retrospective cohort study of infants <6 kg who underwent isolated percutaneous closure of the PDA at a single, tertiary center (2003-2017). Cardiopulmonary outcomes and device-related complications (eg, left pulmonary artery obstruction) were examined for differences across weight thresholds (very low weight, <3 kg; low weight, 3-<6 kg). We assessed composite measures of respiratory status during and beyond the initial hospitalization using linear mixed effects models. RESULTS: In this cohort of lower weight infants, 92 of 106 percutaneous occlusion procedures were successful. Median age and weight at procedure were 3.0 months (range, 0.5-11.1 months) and 3.7 kg (range, 1.4-5.9 kg), respectively. Among infants with pulmonary artery obstruction on initial postprocedural echocardiograms (n = 20 [22%]), obstruction persisted through hospital discharge in 3 infants. No measured variables were associated with device-related complications. Rates of oxygenation failure (28% vs 8%; P < .01) and decreased left ventricular systolic function (29% vs 5%; P < .01) were higher among very low weight than low weight infants. Pulmonary scores decreased (indicating improved respiratory status) following percutaneous PDA closure. CONCLUSIONS: Percutaneous PDA occlusion among lower weight infants is associated with potential longer term improvements in respiratory health. Risks of device-related complications and adverse cardiopulmonary outcomes, particularly among very low weight infants, underscore the need for continued device modification. Before widespread use, clinical trials comparing percutaneous occlusion vs alternative treatments are needed.

Creation of a novel algorithm to identify patients with Becker and Duchenne muscular dystrophy within an administrative database and application of the algorithm to assess cardiovascular morbidity.

BACKGROUND: Outcome analyses in large administrative databases are ideal for rare diseases such as Becker and Duchenne muscular dystrophy. Unfortunately, Becker and Duchenne do not yet have specific International Classification of Disease-9/-10 codes. We hypothesised that an algorithm could accurately identify these patients within administrative data and improve assessment of cardiovascular morbidity. METHODS: Hospital discharges (n=13,189) for patients with muscular dystrophy classified by International Classification of Disease-9 code: 359.1 were identified from the Pediatric Health Information System database. An identification algorithm was created and then validated at three institutions. Multi-variable generalised linear mixed-effects models were used to estimate the associations of length of stay, hospitalisation cost, and 14-day readmission with age, encounter severity, and respiratory disease accounting for clustering within the hospital. RESULTS: The identification algorithm improved identification of patients with Becker and Duchenne from 55% (code 359.1 alone) to 77%. On bi-variate analysis, left ventricular dysfunction and arrhythmia were associated with increased cost of hospitalisation, length of stay, and mortality (p<0.001). After adjustment, Becker and Duchenne patients with left ventricular dysfunction and arrhythmia had increased length of stay with rate ratio 1.4 and 1.2 (p<0.001 and p=0.004) and increased cost of hospitalization with rate ratio 1.4 and 1.4 (both p<0.001). CONCLUSIONS: Our algorithm accurately identifies patients with Becker and Duchenne and can be used for future analysis of administrative data. Our analysis demonstrates the significant effects of cardiovascular disease on length of stay and hospitalisation cost in patients with Becker and Duchenne. Better recognition of the contribution of cardiovascular disease during hospitalisation with earlier more intensive evaluation and therapy may help improve outcomes in this patient population.

Leaflet morphology classification of the Melody Transcatheter Pulmonary Valve.

OBJECTIVE: We sought to describe the leaflet morphology variation in the Melody Transcatheter Pulmonary Valve (TPV) and evaluate associated outcomes. The Melody TPV is constructed from harvested bovine jugular venous valves which have been rigorously tested. Natural anatomic leaflet variations are seen in the Melody TPV but have not been evaluated. DESIGN: A Melody TPV leaflet morphology classification system was devised after reviewing a subset of photographed and implanted TPVs. All images were blindly reviewed by implanters and classified. Midterm hemodynamic outcomes and complications of the Melody TPVs were compared by leaflet morphology. RESULTS: Photographed Melody TPVs implanted between 2011 and 2016 (n = 62) were categorized into the following leaflet morphology types: A-symmetric trileaflet (47%); B-asymmetric trileaflet with a single small leaflet (32%); C-asymmetric trileaflet with a single large leaflet (16%); D-rudimentary leaflet with near bicuspid appearance (5%). Acceptable hemodynamic function at 6 months postimplantation was seen in 97.5% of valves. Over a median follow-up of 1.5 years (range 0-4.4 years), two TPVs (Type A) had > mild regurgitation. Nine TPVs developed complications (endocarditis, 3; stent fracture, 2; refractory arrhythmia, 1; conduit replacement, 2; death, 1), of which 6 required reintervention. There was no significant difference in outcomes based on Melody TPV leaflet morphology type. CONCLUSIONS: The Melody TPV can be classified into one of four categories based on leaflet morphology. Study outcomes were not associated with leaflet morphology. Further documentation and evaluation of Melody TPV morphology may lead to better understanding of this technology.

Cerebral Blood Flow Following Hybrid Stage I Palliation in Infants with Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) palliation may result in altered cerebral blood flow with subsequent neurodevelopmental implications. The purpose of the study was to assess blood flow in the middle cerebral artery (MCA) and investigate the relationship with early neurodevelopmental outcomes in infants with HLHS after hybrid stage I. Transcranial Doppler (TCD) was performed to obtain peak systolic, end-diastolic, and mean velocities, as well as pulsatility index of the MCA in infants with HLHS (n = 18) at baseline and at 2, 4, and 6 months of age. Developmental assessment was performed at 6 months of age. Results of TCD and development were compared to healthy control subjects (n = 6) and normative data. Overall, peak systolic velocity (p = 0.0031), end-diastolic velocity (p < 0.0001), and mean velocity (p < 0.0001) were significantly lower and pulsatility index (p = 0.0011) significantly higher in the HLHS group compared to the control group. A significant increase in change over time was noted for peak systolic velocity (p < 0.0016) and mean velocity (p < 0.0046). There was no significant correlation between TCD variables and development scores. TCD values in 5-6 months old infants with HLHS who undergo hybrid stage I palliation had consistently lower blood flow velocities than control infants; however, pulsatility index was slightly higher. No correlation between TCD measurements and measures of cognitive, language, and motor skills were noted.

Intravascular Ultrasound Characterization of a Tissue-Engineered Vascular Graft in an Ovine Model.

Patients who undergo implantation of a tissue-engineered vascular graft (TEVG) for congenital cardiac anomalies are monitored with echocardiography, followed by magnetic resonance imaging or angiography when indicated. While these methods provide data regarding the lumen, minimal information regarding neotissue formation is obtained. Intravascular ultrasound (IVUS) has previously been used in a variety of conditions to evaluate the vessel wall. The purpose of this study was to evaluate the utility of IVUS for evaluation of TEVGs in our ovine model. Eight sheep underwent implantation of TEVGs either unseeded or seeded with bone marrow-derived mononuclear cells. Angiography, IVUS, and histology were directly compared. Endothelium, tunica media, and graft were identifiable on IVUS and histology at multiple time points. There was strong agreement between IVUS and angiography for evaluation of luminal diameter. IVUS offers a valuable tool to evaluate the changes within TEVGs, and clinical translation of this application is warranted.

Accuracy of Imaging Modalities in Detection of Baffle Leaks in Patients Following Atrial Switch Operation.

AIMS: Patients with dextro-transposition of the great arteries (d-TGA) status post atrial switch operation are vulnerable to complications such as baffle leaks. The best noninvasive imaging modality to detect baffle leaks is unknown. The purpose of this study was to determine the sensitivity and specificity of different noninvasive imaging modalities in the detection of baffle leaks in this population. METHODS AND RESULTS: A single center retrospective chart review of atrial switch patients was performed. Sensitivity, specificity, negative predictive value, and positive predictive value for detecting leaks were calculated for transthoracic echocardiogram (TTE) with and without agitated saline, transesophageal echocardiogram (TEE) with and without agitated saline, and cardiac magnetic resonance imaging (cMRI). Studies were included if performed within 1 year of catheterization. Angiography via catheterization was used as the gold standard for the detection of baffle leaks. Fifty-eight atrial switch patients (54 Mustards: four Sennings) from a single pediatric center, undergoing 76 catheterizations, were analyzed. Thirty-nine catheterizations documented a baffle leak. Overall combination of sensitivity and specificity was better in agitated saline studies (TTE: sensitivity 71.4%, specificity 100%; TEE: sensitivity 100%, specificity 92.3%) versus nonagitated studies (TTE: sensitivity 50.0%, specificity 100%; TEE: sensitivity 83.3%, specificity 77.8%; or cMRI: sensitivity 66.7%, specificity 100%). CONCLUSION: TTE or TEE with agitated saline is superior to cardiac magnetic resonance imaging or nonagitated saline TTE and TEE in detecting baffle leaks in atrial switch patients. Agitated saline studies should be performed in this population when looking for baffle leaks.

Mustard baffle obstruction and leak - How successful are percutaneous interventions in adults?

Atrial switch operations for D-Transposition of the great arteries (D-TGA) were performed until the late 20th century. These patients have substantial rates of re-operation, particularly for baffle related complications. This study sought to analyze the efficacy of percutaneous transcatheter intervention (PTI) for baffle leak and/or stenosis in adult atrial switch patients. Adult patients with a prior atrial switch operation who underwent heart catheterization (2002-2014) at a tertiary adult congenital heart disease referral center were retrospectively analyzed. In 58 adults (30 ± 8 years, 75% men, 14% New York Heart Association (NYHA) functional class ≥2) who underwent 79 catheterizations, PTI was attempted in 50 (baffle leak (n = 10, 20%), stenosis (n = 27, 54%), or both (n = 13, 26%)). PTI was successful in 45 and 5 were referred for surgery due to complex anatomy. A total of 40 bare metal stents, 18 covered stents, 16 occlusion devices, 2 angioplasties, and 1 endovascular graft were deployed. In isolated stenosis, there was improvement in NYHA functional class after PTI (8 vs. 0 patients were NYHA FC > 2, p = 0.004), which was matched by improvement in maximal oxygen consumption on exercise testing (VO2) (25.1 ± 5.4 mL/kg/min vs. 27.9 ± 9 mL/kg/min, p = 0.03). There were no procedure-related deaths or emergent surgeries in this cohort. This single-center cohort is the largest reported series of adult atrial switch operation patients who have undergone PTI for baffle stenosis and/or leak. We demonstrate that PTI with an expert multi-disciplinary team is a safe and effective alternative to surgery in adult patients with an atrial switch operation.

Early results of neurodevelopment following hybrid stage I for hypoplastic left heart syndrome.

Motor skills and neurodevelopment in infants with hypoplastic left heart syndrome (HLHS) who have undergone Hybrid Stage I palliation is unknown. The purpose of this study is to assess early neurodevelopment in infants with HLHS after Hybrid Stage I palliation. Developmental assessment was performed in HLHS infants who underwent Hybrid Stage I palliation at 2 and 4 months of age using the Test of Infant Motor Performance, and at 6 months of age, prior to undergoing the second staged surgery, using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III). Results were compared to healthy control subjects and norm-referenced data. The HLHS group scored between -1 and -2 standard deviations (SD) below the mean at 2 months of age (p = 0.002), and within -1 SD of the mean, at 4 months of age (p = 0.0019), on the TIMP. Compared to the control group, composite motor skills were significantly lower at 6 months of age on the Bayley-III in the HLHS group (p = 0.0489), however, not significant for cognitive (p = 0.29) or language (p = 0.68). Percentile rank motor scores were 17 ± 20 % in the HLHS group compared to 85 ± 12 % for the healthy age-matched control group. Infants with HLHS who undergo Hybrid Stage I palliation score lower on standardized motor skill tests compared to healthy age-matched controls and the norm-referenced population. This suggests that infants with HLHS have poorer motor skill performance than typically developing infants at 6 months of age.

Device closure of secundum atrial septal defects in infants weighing less than 8 kg.

This study aimed to assess the technical aspects of atrial septal defect (ASD) closure using the Amplatzer septal occluder (ASO) and the Gore Helex septal occluder (GHSO) for infants weighing less than 8 kg and to determine the safety, effectiveness, and near-to-intermediate-term outcome of the closure. The Mid-Atlantic Group of Interventional Cardiology Registry of percutaneous, transcatheter ASD closure procedures was reviewed for this analysis. Patients from 10 hospitals in the United States were included. The cohort for this report consisted of 68 patients weighing less than 8 kg (range, 2.3-7.8 kg; mean, 5.5 ± 1.6 kg) and ranging in age from 1 to 24 months (mean, 8.6 ± 4.7 months). The indications for ASD closure were failure to thrive, significant right heart enlargement, shunts otherwise thought to be hemodynamically significant, and poor overall clinical status. Devices were successfully implanted in 66 of the 68 infants (97.1 % procedural success rate). Five minor procedure-related complications occurred. At follow-up assessment, clinical status had improved significantly as measured by improved weight gain and decreased ventilator or oxygen dependence. All residual shunts spontaneously closed during the follow-up period. Six late deaths occurred, none of which were clearly device related. The ASO and GHSO can be safely and effectively implanted for ASD closure in infants weighing less than 8 kg. These procedures usually are successful and seldom complicated, resulting in significant clinical improvement.

The Medtronic Melody® transcatheter pulmonary valve implanted at 24-mm diameter--it works.

OBJECTIVES: We report the Melody valve implanted and/or expanded to 24-mm diameter. BACKGROUND: The Medtronic Melody valve has been implanted up to 22 mm in the pulmonary position for over a decade. METHODS: A retrospective chart review was performed on 82 patients who underwent Melody valve implant. Technical implant method, pre- and postimplant echocardiographic findings, and initial follow-up were reviewed. RESULTS: Between 04/2008 and 12/2011, 13 Melody valves were successfully implanted in 11 patients, median age 35 years (range 16-61 years), in the pulmonary (bioprosthetic valve, right ventricle to pulmonary artery conduit, native valve) position (n = 9), tricuspid position (bioprosthetic valve n = 3), and aortic position (bioprosthetic valve n = 1). Ten valves were delivered on a 24-mm balloon in balloon catheter and three were implanted using a 22-mm Ensemble balloon delivery system, followed by postdilation using a 24-mm × 2-cm Atlas balloon catheter. Postimplant, the median peak systolic gradient across the pulmonary valve was 7 mm Hg and median gradient across the tricuspid valve was 3 mm Hg. There was no change in gradient across the Melody valve in the aortic position where valve prosthesis-patient mismatch was present. Postimplant intracardiac echocardiography demonstrated none or mild valve regurgitation. No more than mild regurgitation was noted at a median follow-up of 9.5 months. CONCLUSIONS: The Melody valve can be implanted at 24 mm in the stenotic/regurgitant bioprosthetic pulmonary, tricuspid, and aortic valve, dysfunctional right ventricle to pulmonary artery conduit, and the native right ventricular outflow tract, whereas the valve remains competent with only mild regurgitation.

Strain echocardiographic assessment of ventricular function after percutaneous pulmonary valve implantation.

INTRODUCTION: Percutaneous pulmonary valve implantation (PPVI) is an emerging therapy for pulmonary valve dysfunction. Minimal data on the midterm effects of PPVI on ventricular function exist. We describe the effects of PPVI on right and left ventricular (RV, LV) function with speckle tracking echocardiography. METHODS: Patients who met the inclusion criteria of the Food and Drug Administration Phase 1 Feasibility Clinical Trial PPVI were identified. Patients were studied with echocardiograms at baseline, post-PPVI (day of discharge), 3 months, and at 6 months. Patients were studied by cardiac magnetic resonance at baseline and at 6 months. Longitudinal strain was measured at the basal, mid, and apical portions of the RV, interventricular septum (IVS), and LV. Global RV and LV strain and strain rates were recorded. Paired t-tests were used for analysis. RESULTS: Ten patients were analyzed: nine patients were a variant of tetralogy of Fallot and one patient had complex LV outflow obstruction requiring a Ross and RV-pulmonary atresia conduit. Mean age was 24.4 ± 7.6 years. Indication for PPVI was pulmonary regurgitation in six patients, stenosis in two patients, and stenosis/regurgitation in two patients. After PPVI, both RV systolic pressure and RV to pulmonary artery pressure gradient significantly decreased. Cardiac magnetic resonance RV end-diastolic volume significantly decreased. IVS-mid, IVS-apical, and LV-global strain significantly increased and RV-basal decreased immediately after PPVI. Global RV a' strain rate significantly increased immediately after PPVI. However, RV, IVS, and LV strain/strain rate values between baseline and the 6 month echocardiographic study were either similar or significantly decreased. CONCLUSION: Despite improvement in RV hemodynamics, there was a decrease or no improvement in RV and LV function as measured by strain echocardiographic values at midterm follow-up. Larger studies with longer follow-up are needed to determine if these results remain consistent.

Pulmonary atresia with ventricular septal defect and multifocal pulmonary blood supply: does an intensive interventional approach improve the outcome?

INTRODUCTION: Pulmonary atresia with ventricular septal defect (VSD) continues to be associated with significant morbidity and mortality, with significant institutional variation in therapeutic strategies. This study reports a single center experience utilizing an intensive transcatheter approach to promote pulmonary vascular growth. METHODS: A retrospective analysis of 20 patients undergoing surgical and transcatheter treatment for pulmonary atresia with VSD between 2002 and 2010. RESULTS: The median age at initial surgical palliation was 6.3 months (8 days to 2.5 years). Eleven patients (group 1) underwent initial surgical palliation without VSD closure and nine patients (group 2) underwent an initial complete repair with fenestrated or complete VSD closure. Group 1 had a smaller Nakata index (54 mm2/m2 vs. 134 mm2/m2 , P = .04) and a smaller absolute native pulmonary artery diameter (2.7 mm vs. 4.5 mm, P = .01) than group 2. Intraoperative angiography was performed in 10 cases to evaluate if early transcatheter intervention was warranted. The median follow-up during the study period was 2.3 years (1.6 months to 8.3 years). Of the 16 patients who survived the initial early postoperative period, 15 patients (94%) went on to receive surgical (n = 11) and/or interventional (n = 25) catheterization procedures. There was improvement in the mean Nakata index from the initial presurgical evaluation to the most recent catheterization data (38.4 mm2/m2 vs. 169.7 mm2/m2, P ≤ .05). To date, two of 11 (18%) patients in group 1 ultimately underwent surgical VSD closure. Overall mortality was six of 20 (30%) with four deaths in group 1 and two deaths in group 2. There were no procedural deaths. CONCLUSIONS: Combining surgical unifocalization procedures with subsequent early and intensive catheter-based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions.

Use of a dose-dependent follow-up protocol and mechanisms to reduce patients and staff radiation exposure in congenital and structural interventions.

BACKGROUND: Increasingly complex structural/congenital cardiac interventions require efforts at reducing patient/staff radiation exposure. Standard follow-up protocols are often inadequate in detecting all patients that may have sustained radiation burns. METHODS: Single-center retrospective chart review divided into four intervals. Phase 1 (07/07-06/08, 413 procedures (proc)): follow-up based on fluoroscopy time only; frame rate for digital acquisition (DA) 30 fps, and fluoroscopy (FL) 30 fps. Dose-based follow-up was used for phase 2-4. Phase 2 (07/08-08/09, 458 proc): DA: 30 fps, FL: 15 fps. Phase 3 (09/09-06/10, 350 proc): DA: 15-30 fps, FL: 15 fps, use of added radiation protection drape. Phase 4 (07/10-10/10, 89 proc): DA: 15-30 fps, FL: 15 fps, superior noise reduction filter (SNRF) with high-quality fluoro-record capabilities. RESULTS: There was a significant reduction in the median cumulative air kerma between the four study periods (710 mGy vs. 566 mGy vs. 498 mGy vs. 241 mGy, P < 0.001), even though the overall fluoroscopy times remained very similar (25 min vs. 26 min vs. 26 min vs. 23 min, P = 0.957). There was a trend towards lower physician radiation exposure over the four study periods (137 mrem vs. 126 mrem vs. 108 mrem vs. 59 mrem, P = 0.15). Fifteen patients with radiation burns were identified during the study period. When changing to a dose-based follow-up protocol (phase 1 vs. phase 2), there was a significant increase in the incidence of detected radiation burns (0.5% vs. 2%, P = 0.04). CONCLUSIONS: Dose-based follow-up protocols are superior in detecting radiation burns when compared to fluoroscopy time-based protocols. Frame rate reduction of fluoroscopy and cine acquisition and use of modified imaging equipment can achieve a significant reduction to patient/staff exposure.

An institutional approach to interventional strategies for complete vascular occlusions.

Complete vascular occlusions are rare but potentially lethal. Reports on transcatheter therapy are limited to solitary case reports. The study was conducted as a single-center retrospective chart review. Between January 2003 and December 2009, 26 patients underwent cardiac catheterizations for either a known complete thoracic vascular occlusion or for incidental complete vascular occlusion that was noted during cardiac catheterization. Procedural technique, risk factors, and outcomes are reviewed. Median age at the time of diagnosis was 13.3 years (range 2 months to 54 years). In 6 of 26 (23%) patients, the vascular occlusion was of acute onset. Ninety-two percent of patients had at least 1 known risk factor for vascular occlusion, whereas 54% of patients had ≥ 2 risk factors. Successful rehabilitation was achieved in 19 patients, with direct catheter and/or wire manipulation being used to cross the occluded vessel in 15 (75%) patients, radiofrequency (RF) perforation in 3 patients, and perforation with Brockenbrough needle in 1 patient. Subsequent techniques included AngioJet (n = 2), balloon angioplasty and/or stent implantation (n = 18), and adjuvant local administration of recombinant tissue-plasminogen activator (n = 3). Reinterventions were required in 5 patients, and 8 patients died during the study period from causes unrelated to the catheterization procedure(s). Median follow-up of patients after successful recanalization was 12.6 months (range 1 day to 64 months), and the median duration of survival free from reintervention was 11.8 months (range 1 day to 64 months). In most patients, risk factors can be identified that are associated with the occurrence of a complete vascular occlusion. Different treatment strategies are used in patients having chronic compared with acute occlusions. Residual vascular lesions (hypoplasia or stenosis) are often associated with occurrence of reobstructions and should therefore be treated to prevent such an occurrence. Postprocedural anticoagulation is important in maintaining vascular patency.

Hybrid procedures: adverse events and procedural characteristics--results of a multi-institutional registry.

INTRODUCTION: Procedural cooperation between cardiac surgeon and interventional cardiologist to facilitate interventions such as device delivery or angioplasty (hybrid procedure) has become increasingly common in the management of patients with congenital heart disease. DESIGN: Data were prospectively collected using a multicenter registry (C3PO). Between February 2007 and December 2008, seven institutions submitted data regarding 7019 cardiac catheterization procedures. Procedural data and adverse events (AEs) of 128 hybrid procedures were evaluated. RESULTS: There was significant variability in the number of hybrid procedures per center, ranging from one to 89 with a median of eight. A total of 60% of interventional (vs. strictly diagnostic) hybrid procedures were performed by one center. The median weight was 3.7 kg (0.7-86 kg). Single-ventricle circulation was present in 60% of the procedures. Hybrid procedures included: patent ductus arteriosus (PDA) stent placement (n = 55), vascular rehabilitation (n = 25), ventricular septal defect (VSD) device closure (n = 7), valvotomy (n = 3), and diagnostic hybrid procedures (n = 38). Sixteen AEs occurred in 15/128 (12%) procedures. These included minor or trivial AEs (n = 9), moderate AEs (n = 5), major AEs (n = 1), and catastrophic AEs (n = 1). The type of AE documented included arrhythmias (n = 6), hypoxia or hypotension (n = 3), vessel or cardiac trauma (n = 2), and other events (n = 5). Of documented AEs, 9/16 (56%) were classified as not preventable, 6/16 (38%) as possibly preventable, and 1/16 (6%) as preventable. The incidence of AE related to PDA stent placement with surgical exposure (5/50, 10%) was significantly lower when compared with PDA stent placement performed percutaneously (4/5, 80%, P= .002). CONCLUSION: Hybrid procedures appear to have a low incidence of associated major AEs. PDA stent placement performed as a palliation of hypoplastic left heart syndrome (HLHS) or complex single/two ventricle patients may have a lower incidence of AEs if performed using a direct approach with surgical exposure rather than a percutaneous approach. Accurate definitions of these innovative procedures are required to facilitate prospective data collection.