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Background and Aim of the Study: Ischemic mitral regurgitation (IMR), the incidence of which is increasing, results from annular and subvalvular remodeling after myocardial infarction (MI). Although a sheep model of IMR has been used extensively over the past two decades, the ventricular, coronary and leaflet anatomy in sheep is significantly different from that in humans. In contrast, pigs are more similar to humans with regard to these parameters, and therefore may serve as a better animal to test emerging new technologies designed to treat IMR., Methods: Twenty-nine pigs (body weight 30-35 kg) underwent left thoracotomy and ligation of the mid main circumflex and distal right posterior descending coronary arteries to create a posterolateral MI. Of these pigs, 18 were used for acute data acquisition, while 11 surviving animals in the chronic group were assessed at eight weeks after MI. Real-time three-dimensional echocardiography was performed at baseline, and at 30 min and eight weeks after MI, to assess geometric changes in the mitral annulus, mitral leaflets and left ventricle., Results: Compared to baseline, the MR grade was increased significantly at eight weeks (0.7 + 0.5 versus 2.0 +/- 1.2), together with a significant decrease in left ventricular ejection fraction (40.3 +/- 6.6% versus 25.8 +/- 7.7%). Significant increases were also noted at eight weeks in the commissural width (30.1 +/- 3.2 mm versus 35.1 +/- 2.9 mm) and septolateral diameter (25.0 +/- 2.0 mm versus 33.8 +/- 5.9 mm), with a resultant increase in mitral annular area (596 +/- 85 versus 931 +/- 181 mm3) and a decrease in the annular height to commissural width ratio (15.7 +/- 2.6% versus 13.7 +/- 1.9%). The mitral valve tenting volume was also increased significantly (1577 +/- 645 versus 2440 +/- 755 mm3). The distance between the papillary muscle tips at baseline and at eight weeks was increased significantly (23.9 +/- 2.5 versus 30.9 +/- 5.2 mm), as was the distance between the posterior papillary muscle tip and the posterior commissure (20.9 +/- 2.7 versus 24.1 +/- 2.8 mm)., Conclusion: The surgical model described here reliably replicates the changes seen in humans with IMR. Hence, this model can be used for further studies of the pathophysiology of IMR, and of any novel interventions in this challenging clinical area.

Background: Radiation exposure in the pediatric population may increase the risk of future malignancy. Children with congenital heart disease who often undergo repeated catheterizations are at risk. One possible strategy to reduce radiation is to use X-ray Magnetic Resonance Fusion (XMRF) to facilitate cardiac catheterization., Methods: Catheterization data of patients who underwent diagnostic XMRF procedures between January 1, 2009 and February 1, 2012 were reviewed. Cases were matched 1:1 to contemporary controls who did not undergo XMRF based on weight and diagnosis and were compared in radiation exposure, contrast dose, and procedural and anesthesia times., Results: Forty-four matched pairs were included. Baseline demographics were similar in both groups. Patients in the XMRF group had lower indices of radiation exposure measured by fluoroscopy time (14 vs. 16.4 vs. P = 0.047), dose-area product from fluoroscopy (513.2 vs. 589.1 µGy·m(2) , P = 0.042), total dose-area product (625.8 vs. 995.2 µGy·m(2) , P = 0.027), and total air kerma dose (94.5 vs. 153.8 mGy, P = 0.017). There was also a significant reduction in contrast dose (2 vs. 3.3 cc/kg, P <0.001). Procedural time tended to be shorter in the XMRF group but anesthesia time was significantly longer., Conclusion: Select diagnostic cardiac catheterization cases that utilized XMRF used less radiation and contrast than similar cases where XMRF was not used. Future work is needed to determine whether similar benefits can be extended to other types of diagnostic and complex interventional procedures., (© 2014 Wiley Periodicals, Inc.)

Deficient retro-aortic rim is of concern as a risk factor for aortic erosion after device closure of atrial septal defects (ASD). However, its prevalence and contribution to technical failure and adverse outcomes have not been delineated. A single-center retrospective cohort study of children and adults undergoing cardiac catheterization for device occlusion of ASD from 1 January 1999 to 1 April 2012 was performed. Risk factors for technical failure and early adverse outcome were assessed using multivariate logistic regression. During the study period, 445 consecutive subjects with a median age of 5.9 years (range, 0.8-80 years) underwent catheterization. Of the subjects with reviewable echocardiograms, 60 % had deficient retro-aortic rim. No attempt at device closure was made for 3.6 % of the subjects. Of the remaining 429 subjects, 96 % underwent successful device occlusion. Major early adverse events occurred in 1.2 % (95 % confidence interval 0.4-2.7 %) of the cases, all of them either device embolization or malposition. Deficient retro-aortic rim was not a risk factor for composite outcome of technical failure or early major adverse event. No deaths, late reinterventions, or erosion events occurred during 2,395 total person-years (median, 5.8 years) of follow-up evaluation. Deficient retro-aortic rim was associated with increased risk of device impingement on the aorta, but no association was seen between device impingement or deficient retro-aortic rim and the development of new/progressive aortic insufficiency. Deficient retro-aortic rim is highly prevalent but did not increase the risk of adverse outcomes. Its contribution to the risk of aortic erosion could not be addressed by this study.

Objective: To compare the interstage cardiac catheterization hemodynamic and angiographic findings between shunt types for the Pediatric Heart Network Single Ventricle Reconstruction trial. The trial, which randomized subjects to a modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS) for the Norwood procedure, demonstrated the RVPAS was associated with a smaller pulmonary artery diameter but superior 12-month transplant-free survival., Methods: We analyzed the pre-stage II catheterization data for the trial subjects. The hemodynamic variables and shunt and pulmonary angiographic data were compared between shunt types; their association with 12-month transplant-free survival was also evaluated., Results: Of 549 randomized subjects, 389 underwent pre-stage II catheterization. A smaller size, lower aortic and superior vena cava saturation, and higher ventricular end-diastolic pressure were associated with worse 12-month transplant-free survival. The MBTS group had a lower coronary perfusion pressure (27 vs 32 mm Hg; P<.001) and greater pulmonary blood flow/systemic blood flow ratio (1.1 vs 1.0, P=.009). A greater pulmonary blood flow/systemic blood flow ratio increased the risk of death or transplantation only in the RVPAS group (P=.01). The MBTS group had fewer shunt (14% vs 28%, P=.004) and severe left pulmonary artery (0.7% vs 9.2%, P=.003) stenoses, larger mid-main branch pulmonary artery diameters, and greater Nakata indexes (164 vs 134, P<.001)., Conclusions: Compared with the RVPAS subjects, the MBTS subjects had more hemodynamic abnormalities related to shunt physiology, and the RVPAS subjects had more shunt or pulmonary obstruction of a severe degree and inferior pulmonary artery growth at pre-stage II catheterization. A lower body surface area, greater ventricular end-diastolic pressure, and lower superior vena cava saturation were associated with worse 12-month transplant-free survival., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: The goal of this report is to describe a percutaneous approach to rerouting hepatic venous return in patients who developed progressive cyanosis due to unilateral pulmonary arteriovenous malformations (PAVM) after the total cavopulmonary connection (TCPC) operation., Background: Unilateral PAVM can develop in patients after TCPC operation when there is unequal distribution of hepatic venous return between the two lungs. This often results in progressive cyanosis and the need for surgical re-intervention. A percutaneous based approach for rerouting hepatic venous return has never been described., Methods: We retrospectively reviewed the clinical data on four patients who underwent percutaneous rerouting procedures. One patient with a misaligned TCPC underwent realignment of the circuit with a bare metal stent. In three patients a combination of bare metal and covered stents were needed to achieve the desired results., Results: The rerouting procedures were successful in all patients with significant improvement in oxygen saturation from a median of 75% (range 55-80%) to a median of 90% (range 84-92%) (P = 0.02). There were no recorded short term or intermediate term complications with maximum follow-up time of 43 months., Conclusions: Percutaneous rerouting of hepatic venous flow is feasible and should be considered when a surgical approach is not possible; this strategy may serve as a viable alternative to complex operative approaches in select cases. Furthermore studies are needed to determine the long-term efficacy of this procedure., (© 2013 Wiley Periodicals, Inc.)

Recoarctation of the aorta (RCoA) is a major cause of morbidity and mortality after the Norwood procedure. We sought to identify transthoracic echocardiographic (TTE) indexes associated with RCoA and to develop a highly sensitive and specific diagnostic score for accurate diagnosis. All subjects who underwent a Norwood procedure from December 2005 to December 2009 were identified. Subjects were excluded if they did not undergo a TTE within 1 month of an outcome-defining event (cardiac catheterization, autopsy, or surgery). RCoA was defined as arch intervention at catheterization or surgery or findings of RCoA at autopsy. Of 113 subjects included for analysis, RCoA occurred in 19 (17%). All TTE indexes were significantly associated with RCoA in univariate testing. In the final multivariate model, peak isthmus velocity >2.5 m/s (p <0.001), coarctation index, defined as the ratio of the narrowest region of the descending thoracic aorta to the distal descending thoracic aorta diameter <0.7 (p <0.01), and decrease in ventricular systolic performance (p = 0.03) were all significantly associated with RCoA. A composite score was developed using a peak velocity of >2.5 m/s (2 points), coarctation index <0.7 (1 point), and a decrease in ventricular systolic performance (1 point). A score of ≥2 diagnosed RCoA with 100% sensitivity and 85% specificity. The score performed equally well regardless of shunt type. In conclusion, a composite score of TTE indexes accurately discriminates RCoA in patients who have undergone the Norwood procedure., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: We previously reported that increased preoperative Beck Depression Inventory II (BDI-II) scores were associated with a 47% (95% CI 24%-64%) reduction in the rate of opioid cessation following surgery. We aimed to identify the underlying factors of the BDI-II (affective/cognitive vs somatic) associated with a decreased rate of opioid cessation after surgery., Methods: We conducted a secondary analysis of the data from a previously reported prospective, longitudinal, observational study of opioid use after five distinct surgical procedures (total hip replacement, total knee replacement, thoracotomy, mastectomy, and lumpectomy) in 107 patients. The primary endpoint was time to opioid cessation. After exploratory factor analysis of the BDI-II, mean summary scores were calculated for each identified factor. These scores were evaluated as predictors of time to opioid cessation using Cox proportional hazards regression., Results: The exploratory factor analysis produced three factors (self-loathing symptoms, motivational symptoms, emotional symptoms). All three factors were significant predictors in univariate analysis. Of the three identified factors of the BDI-II, only preoperative self-loathing symptoms (past failure, guilty feelings, self-dislike, self-criticalness, suicidal thoughts, worthlessness) independently predicted a significant decrease in opioid cessation rate after surgery in the multivariate analysis (HR 0.86, 95% CI 0.75-0.99, P value 0.037)., Conclusions: Our results identify a set of negative cognitions predicting prolonged time to postoperative opioid cessation. Somatic symptoms captured by the BDI-II were not primarily responsible for the association between preoperative BDI-II scores and postoperative prolonged opioid use., (Wiley Periodicals, Inc.)

Radiation exposure from pediatric cardiac catheterization may be substantial, although published estimates vary. We sought to report patient radiation dose across a range of diagnostic and interventional cases in a modern, high-volume pediatric catheterization laboratory. We retrospectively reviewed diagnostic and interventional cases performed in our pediatric catheterization laboratory from 1 April 2009 to 30 September 2011 for which radiation usage data were available as reported by the Artis Zee(®) (Siemens Medical Solutions) system. Electrophysiology cases were excluded. Radiation dose was quantified as air kerma dose (mGy) and dose-area product (DAP; μGy m(2)). The DAP was converted to an effective dose millisievert (mSv) using the Monte Carlo method. Radiation usage data were available from 2,265 diagnostic and interventional cases with an overall median air kerma dose of 135 mGy [interquartile range (IQR) 59-433], median DAP of 760 μGy m(2) (IQR 281-2,810), of which 75 % (IQR 59-90 %) was derived from fluoroscopy, and median effective dose of 6.2 mSv (IQR 2.7-14.1). Air kerma dose from a single camera >2,000 mGy occurred in 1.8 % of cases. Significant differences in all measures of radiation exposure existed based on procedural and interventional types (p = 0.0001), with interventional cases associated with the highest effective dose after adjusting for patient weight category (p < 0.001). Patient weight, age, fluoroscopy time, and proportional use of digital acquisition were independent predictors of exposure (p ≤ 0.001; R (2) = 0.59-0.64). In a modern, large-volume pediatric catheterization laboratory, the median effective dose is 6.2 mSv with a wide range of exposure based on patient- and procedure-specific factors. Radiation monitoring is an important component of a pediatric laboratory and further dose reduction strategies are warranted.

Background: Melody Transcatheter Pulmonary Valve (TPV) replacement therapy represents an important advance in congenital cardiovascular interventions. The off-label extension of the Melody TPV to patients with nonconduit outflow tracts (right ventricular outflow tract [RVOT]) has the potential to vastly expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. However, knowledge on the performance of the Melody TPV in this setting is limited., Methods and Results: This is a multicenter, retrospective review of the Melody TPV when placed in nonconduit RVOTs, in which at least a portion of the circumference was composed of native tissue. Five centers contributed data on 31 patients. The median age at implantation was 24 years (range, 7-66). At a median follow-up of 15 months, all patients were alive. No patient had greater than mild TPV insufficiency, and the median maximum instantaneous gradients across the RVOT was 23 mm Hg. Stent fracture occurred in 32%. Eight patients developed more than mild TPV obstruction, of whom 6 were associated with identified stent fracture. Three patients developed blood stream infections. There were 5 reinterventions in 3 patients, including 3 repeat TPV implantations and 2 TPV explantations., Conclusions: Melody TPV implantation is feasible in selected patients with RVOTs comprised solely or predominantly native tissue and has the potential to expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. In early follow-up, valve competency seems preserved. The dominant mechanism of valve dysfunction seems to be related to stent fracture with recurrent obstruction. Additional data are necessary to better understand how to safely expand TPV therapy to this population., (© 2014 American Heart Association, Inc.)

Objectives: Transcatheter patent ductus arteriosus (PDA) occlusion is a staple of pediatric catheterization laboratories. We present the phenomenon of significant PDA spasm to prevent failure to occlude a hemodynamically significant duct., Background: Transcatheter techniques have evolved, allowing safe and effective occlusion of PDAs in younger and smaller patients. Neonatal care is evolving with increasing survival at younger gestational ages. Premature infants often have PDAs, so the proportion of formerly premature children referred for transcatheter ductal occlusion will likely rise., Methods: We reviewed all transcatheter PDA occlusions performed at our institution since 2001 (N = 331). Retrospective data included: gestational age, age at catheterization, precatheterization echocardiographic parameters, PDA size (after spasm relief), device specifications, and most recent follow-up data., Results: Seven cases were identified. Median age was 12 months, median gestational age was 28 weeks. All were born prematurely. All PDAs were restrictive and six had left-heart volume overload. All patients were examined by the interventional cardiologist and had ductal murmurs. When reauscultated (three of seven), murmurs were absent during spasm. Once spasm relieved, PDA diameters ranged from 1.5 to 8 (median 2) mm. All patients accommodated a 6-mm-or larger-Amplatzer device. No significant complications occurred and all patients were well at follow-up., Conclusions: Ductal spasm occurs during transcatheter occlusion and may be an unrecognized cause of procedural failure. The phenomenon seems to occur in children born prematurely, and can occur after infancy. Loss of a continuous murmur confirms the diagnosis. Care should be taken to avoid device under-sizing when spasm occurs., (Copyright © 2013 Wiley Periodicals, Inc.)

Radiofrequency perforation and valvuloplasty (RFV) is an effective initial treatment in patients with pulmonary atresia and intact ventricular septum (PA-IVS) and mild to moderate right ventricle and tricuspid valve hypoplasia. Outcomes and risk factors for the need for additional interventions in these patients are poorly defined. All patients with PA-IVS who underwent RFV at our center between January 2000 and July 2011 were reviewed. Twenty-three patients met the inclusion criteria. All patients underwent successful valvuloplasty with no procedural deaths and one major complication. Excluding two patients with limited follow-up, 6 (29 %) patients underwent no subsequent interventions, whereas 9 (42 %) patients underwent surgical right-ventricular outflow tract augmentation. All except one patient with adequate follow-up have a biventricular circulation with saturation >92 %. Patients who did not undergo any right-ventricular outflow tract intervention after valvuloplasty had a significantly lower gradient across the pulmonary valve after valvuloplasty (9.9 mmHg ± 8.4 vs. 19.1 mmHg ± 10.4, p = 0.05). Significantly more patients who received a supplemental source of pulmonary blood flow had a tricuspid valve z-score <-0.7 compared with patients who did not receive supplemental blood flow [2 (15 %) vs. 7 (70 %), p = 0.008]. In our cohort of patients with PA-IVS, radiofrequency perforation with valvuloplasty was an effective and safe first step in establishing a biventricular circulation. Postvalvuloplasty pulmonary valve gradient may be predictive of subsequent outflow tract intervention, and tricuspid hypoplasia may be predictive of the need for a supplemental source of pulmonary blood flow.

Purpose of Review: Catheter-based valve technologies have evolved rapidly over the last decade. Transcatheter aortic valve replacement (TAVR) has become a routine procedure in high-risk adult patients with calcific aortic stenosis. In patients with congenital heart disease (CHD), transcatheter pulmonary valve replacement represents a transformative technology for right ventricular outflow tract dysfunction with the potential to expand to other indications. This review aims to summarize the current state-of-the-art for transcatheter valve replacement (TVR) in CHD; the expanding indications for TVR; and the technological obstacles to optimizing TVR., Recent Findings: Multiple case series have demonstrated that TVR with the Melody transcatheter pulmonary valve in properly selected patients is safe, effective, and durable in short-term follow-up. The Sapien transcatheter heart valve represents an alternative device with similar safety and efficacy in limited studies. Innovative use of current valves has demonstrated the flexibility of TVR, while highlighting the need for devices to address the broad range of postoperative anatomies either with a single device or with strategies to prepare the outflow tract for subsequent device deployment., Summary: The potential of TVR has not been fully realized, but holds promise in treatment of CHD.

Aim: Force due to leaning during cardiopulmonary resuscitation (CPR) negatively affects haemodynamics and intrathoracic airway pressures (ITP) in animal models and adults, but has not been studied in children. We sought to characterize the effects of sternal force (SF) comparable to leaning force on haemodynamics and ITP in anaesthetized children., Methods: Children (6 months to 8yrs) presenting for routine haemodynamic cardiac catheterization with anaesthesia and mechanical ventilation >6 months after cardiac transplant were studied. Haemodynamics and ITP were measured before and during incremental increases in SF of 10% and 20% body weight., Results: 20 subjects (5.4±1.7yrs of age and 18.3±3.3kg) were studied. Mean right atrial pressure (6.5±2.6 at baseline vs. 7.7±2.6 at 10% SF vs. 8.6±2.7mmHg at 20% SF), mean pulmonary capillary wedge pressure (10.2±2.9 at baseline vs. 11±3.3 at 10% SF vs. 11.8±3.4mmHg at 20% SF) and ITP (16.3±3.2 at baseline vs. 17.9±3.9 at 10% SF vs. 19.5±4cm H2O) all increased significantly with incremental SF (p<0.001 for all). Aortic systolic pressure (85±10mmHg at baseline vs. 83±10mmHg at 10% SF vs. 82±10mmHg at 20% SF, p=0.014) and coronary perfusion pressure (42±7mmHg at baseline vs. 39±7mmHg at 10% SF vs. 38±7mmHg at 20% SF, p<0.001) both decreased significantly with incremental SF., Conclusions: In asymptomatic, anaesthetized children after cardiac transplantation, sternal forces comparable to leaning previously reported to occur during CPR elevate ITP and right atrial pressure and decrease coronary perfusion pressure. These haemodynamic effects may be clinically important during CPR and warrant further study., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Background: Cardiac catheterization is routinely used as a diagnostic tool in single ventricle patients with superior cavopulmonary connection. This physiology presents inherent challenges in applying the Fick principle to estimate flow. We sought to quantitatively define the error in oximetry-derived flow parameters using phase-contrast cardiac MRI (CMR) as a reference., Methods and Results: Thirty patients with superior cavopulmonary connection who underwent combined CMR and catheterization between July 2008 and June 2012 were retrospectively analyzed. Estimates of flow and resistance calculated using the Fick equation were compared with CMR measurements. Oximetry underestimated CMR-measured pulmonary blood flow (Qp) by an average of 1.1 L/min per m(2) or 32% of the CMR value (P<0.0001). Oximetry overestimated systemic blood flow (Qs) by an average of 0.5 L/min per m(2) or 15% of the CMR value (P=0.009). There was no correlation between the Qp:Qs ratio derived by Fick and that measured by CMR (ρ(c)=0.01). The error in the Fick Qp correlated moderately with the measured systemic-to-pulmonary arterial collateral flow (r=0.39). The median total oxygen consumption calculated using combined CMR and oximetry data was 173 mL/min per m(2), higher than the assumed values used to calculate flows by the Fick equation. The upper body circulation received on average 51% of systemic blood flow while conducting only 39% of total body metabolism., Conclusions: Fick-derived estimates of flow are inherently unreliable in patients with superior cavopulmonary connections. Integrating flows measured by CMR and pressures measured by catheter will provide the best characterization of superior cavopulmonary connection physiology.

Objectives: To describe our experience with percutaneous closure of patent ductus arteriosus (PDA) in small infants and compare outcomes to matched surgical patients., Background: Ligation via thoracotomy has been used to close PDAs in small infants, but has been associated with respiratory and hemodynamic compromise. We hypothesized that percutaneous closure would offer faster recovery of respiratory function., Methods: Patients <4 kg requiring positive pressure ventilation who underwent percutaneous PDA closure between January 2000 and April 2012 were reviewed and matched to contemporary surgical patients on gestational age (GA), birth weight (BW), procedure weight (WT), and ventilation mode. Patients returned to baseline respiratory status when the product of mean airway pressure and FiO2 returned to pre-procedural levels., Results: Eight matched pairs were included. Median BW, GA, and WT were 1.43 kg (0.52-2.97), 29.8 weeks (24-39), and 2.8 kg (2.2-3.9) for catheter patients and 1.55 kg (0.48-3.04), 29 weeks (23-37), and 2.75 kg (2.3-4.2) for surgical patients. Complete PDA closure occurred in all. The median time to return to baseline respiratory status was significantly shorter in the percutaneous group (17 hr (range 0-113) vs. 53 hr (range 13-219), P < 0.05). In the percutaneous group, two patients developed mild aortic coarctation, one mild left pulmonary artery stenosis, and four femoral vascular thromboses which all resolved with medical therapy. Surgical complications included significant respiratory and cardiac compromise, rib fractures and urinary retention., Conclusions: Percutaneous closure of PDA in small infants on respiratory support is equivalent in safety and efficacy and may offer shorter recovery time than surgical ligation., (Copyright © 2013 Wiley Periodicals, Inc.)

Objectives: To describe the prevalence of and identify risk factors for acute occlusive arterial injury (AOAI) in a large volume pediatric cardiac catheterization laboratory., Background: AOAI is a known complication after pediatric cardiac catheterization. Prevalence and risk factors in the modern era are incompletely described., Methods: A retrospective cohort study including all cardiac catheterization procedures performed between January 1, 2005 and June 30, 2010 was performed. Case status was defined by ≥1 of the following: exam consistent with occlusive arterial injury, use of an anticoagulant within 48 hr of catheterization to restore or maintain patency of the artery, or documented occlusive arterial injury by radiologic study., Results: 3,254 patients had 5,715 catheterization procedures, which included 3,859 arterial access events. 167 cases of AOAI were identified for an overall prevalence of 4.3% among arterial access events. Multiple logistic regression identified independent risk factors: weight category [<4 kg: odds ratio (OR) 4.5 (95% CI: 2.6-7.7), P < 0.001; 4-6 kg: OR 2.1 (1.3-3.5), P = 0.002, compared to 6-8 kg referent group]; largest catheter outer diameter French size [OR 1.6 (1.3-1.9), P < 0.001]; final activated clotting time (ACT) <250 sec [OR 1.9 (1.4-2.7), P < 0.001]; and need for arterial catheter exchange [OR 1.8 (1.02-3.2), P = 0.04]., Conclusions: AOAI occurred in 4.3% of pediatric cardiac catheterizations, and was most likely in smaller children and those with larger arterial catheters. Risk was also independently increased by arterial catheter exchange and having a final ACT <250 sec. These data act as an important benchmark and identify areas for intervention for future studies., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial., Methods and Results: Recoarctation was defined by intervention, either catheter based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed with adjustment for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty, 39 surgical) for recoarctation at a median age of 4.9 months (range, 1.1-10.5 months). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, recoarctation was associated with the shunt type in place at the end of the Norwood procedure (hazard ratio, 2.0 for right ventricle-pulmonary artery shunt versus modified Blalock-Taussig shunt; P=0.02), and Norwood discharge peak echo-Doppler arch gradient (hazard ratio, 1.07 per 1 mm Hg; P<0.01). Subjects with recoarctation demonstrated comorbidities at pre-stage II evaluation, including higher pulmonary arterial pressures (15.4±3.0 versus 14.5±3.5 mm Hg; P=0.05), higher pulmonary vascular resistance (2.6±1.6 versus 2.0±1.0 Wood units·m(2); P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA(1.3), where BSA is body surface area; P=0.02). There was no difference in 12-month postrandomization transplantation-free survival between those with and without recoarctation (P=0.14)., Conclusions: Recoarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplantation/mortality, further evaluation is warranted to evaluate the effects of associated morbidities.

Purpose: Transcatheter mitral valve replacement would represent a major advance in heart valve therapy. Such a device requires a specialized anchoring and sealing technology. This study was designed to test the feasibility of a novel mitral valve replacement device (the sutureless mitral valve [SMV]) designed to anchor and seal in the mitral position without need for sutures., Description: The SMV is a self-expanding device consisting of a custom-designed nitinol framework and a pericardial leaflet valve mechanism., Evaluation: Ten sheep underwent successful surgical SMV device implantation. All animals underwent cardiac catheterization 6 hours postoperatively. Hemodynamic, angiographic, echocardiographic and necroscopic data were recorded. The mean aortic cross-clamp time was 9.5 ± 3.1 minutes. Echocardiography and angiography revealed excellent left ventricular systolic function, no significant perivalvular leak, no mitral valve stenosis, no left ventricular outflow tract obstruction, and no aortic valve insufficiency. Necropsy demonstrated that the SMV devices were anchored securely., Conclusions: This study demonstrates the feasibility and short-term success of sutureless mitral valve replacement using a novel SMV device., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The pigeon crop is specially adapted to produce milk that is fed to newly hatched young. The process of pigeon milk production begins when the germinal cell layer of the crop rapidly proliferates in response to prolactin, which results in a mass of epithelial cells that are sloughed from the crop and regurgitated to the young. We proposed that the evolution of pigeon milk built upon the ability of avian keratinocytes to accumulate intracellular neutral lipids during the cornification of the epidermis. However, this cornification process in the pigeon crop has not been characterised., Results: We identified the epidermal differentiation complex in the draft pigeon genome scaffold and found that, like the chicken, it contained beta-keratin genes. These beta-keratin genes can be classified, based on sequence similarity, into several clusters including feather, scale and claw keratins. The cornified cells of the pigeon crop express several cornification-associated genes including cornulin, S100-A9 and A16-like, transglutaminase 6-like and the pigeon 'lactating' crop-specific annexin cp35. Beta-keratins play an important role in 'lactating' crop, with several claw and scale keratins up-regulated. Additionally, transglutaminase 5 and differential splice variants of transglutaminase 4 are up-regulated along with S100-A10., Conclusions: This study of global gene expression in the crop has expanded our knowledge of pigeon milk production, in particular, the mechanism of cornification and lipid production. It is a highly specialised process that utilises the normal keratinocyte cellular processes to produce a targeted nutrient solution for the young at a very high turnover.

Background: The significance and optimal treatment of systemic-to-pulmonary arterial collateral (SPC) vessels in single ventricle patients are poorly understood. The acute efficacy of SPC embolization has not been demonstrated in a quantifiable fashion. We sought to assess the acute efficacy of SPC embolization on blood flow as quantified by phase contrast magnetic resonance imaging and hypothesized that embolization acutely decreases SPC flow and increases systemic blood flow (Q(S))., Methods and Results: Six superior cavopulmonary connection patients underwent SPC flow quantification by phase contrast magnetic resonance imaging, including quantification of superior and inferior caval, total pulmonary artery, total pulmonary vein, ascending and descending aortic flows (Q(SVC), Q(IVC), Q(PA), Q(PV), Q(Ao), and Q(Dao), respectively), both immediately before and after cardiac catheterization with coil and particle embolization of angiographically evident SPC vessels. All studies were performed under a single anesthetic. After embolization, we found a significant decrease in SPC flow of 0.9 (range, 0.6-1.3) L/(min·m(2)) (P=0.03); a median reduction of 47% (range, 32-60). There was a significant decrease in the median Q(P):Q(S) from 1.3 before to 0.8 after embolization (P=0.03), and an increase in Q(S) from a median of 3.4 to 4.4 L/(min·m(2)) (P<0.05), and Q(SVC) from a median of 1.7 to 2.3 L/(min·m(2)) (P=0.03)., Conclusions: We report on the acute efficacy of SPC embolization, demonstrating a significant decrease in SPC flow and Q(P):Q(S) and increase in Q(SVC) and Q(S). Further studies are needed to assess the durability of the procedure and the effect on Fontan and longer-term outcomes.

Background: Transcatheter pulmonary valve implantation using the Melody valve has emerged as an important therapy for the treatment of postoperative right ventricular outflow tract dysfunction. Melody-in-bioprosthetic valves (BPV) is currently considered an off-label indication. We review the combined experience with transcatheter pulmonary valve implantation within BPVs from 8 centers in the United States and discuss technical aspects of the Melody-in-BPV procedure., Methods and Results: A total of 104 patients underwent Melody-in-BPV in the pulmonary position at 8 US centers from April 2007 to January 2012. Ten different types of BPVs were intervened on, with Melody valve implantation at the intended site in all patients. Following Melody valve implant, the peak right ventricle-to-pulmonary artery gradient decreased from 38.7 ± 16.3 to 10.9 ± 6.7 mm Hg (P<0.001), and the right ventricular systolic pressure fell from 71.6 ± 21.7 to 46.7 ± 15.9 mm Hg (P<0.001). There was no serious procedural morbidity, and no deaths related to the catheterization or implant. At a median follow-up of 12 months (1-46 months), no patients had more than mild regurgitation, and 4 had a mean right ventricular outflow tract gradient ≥30 mm Hg. During follow-up, there were 2 stent fractures, 3 cases of endocarditis (2 managed with surgical explant), and 2 deaths that were unrelated to the Melody valve., Conclusions: Transcatheter pulmonary valve implantation using the Melody valve within BPVs can be accomplished with a high rate of success, low procedure-related morbidity and mortality, and excellent short-term results. The findings of this preliminary multicenter experience suggest that the Melody valve is an effective transcatheter treatment option for failed BPVs.

Background: Coronary ostial atresia is a rare anomaly, most frequently managed with coronary artery bypass grafting. This condition is often misdiagnosed as anomalous left coronary artery from the pulmonary artery until the time of surgical intervention, leaving the surgeon little time to contemplate the best operative technique., Methods: In this single-center experience, three cases of congenital atresia of the left main coronary artery presenting during childhood were surgically managed with homograft patch ostioplasty., Results: Antegrade left coronary artery blood flow was successfully established in all three cases without the use of a bypass graft. Two children survived and are clinically well; the third had visibly necrotic myocardium at the time of surgical ostioplasty and did not survive to hospital discharge., Conclusions: Surgical ostioplasty with a homograft patch should be considered as an alternative to bypass grafting for coronary ostial atresia in children., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Determinants of the duration of opioid use after surgery have not been reported. We hypothesized that both preoperative psychological distress and substance abuse would predict more prolonged opioid use after surgery., Methods: Between January 2007 and April 2009, a prospective, longitudinal inception cohort study enrolled 109 of 134 consecutively approached patients undergoing mastectomy, lumpectomy, thoracotomy, total knee replacement, or total hip replacement. We measured preoperative psychological distress and substance use, and then measured the daily use of opioids until patients reported the cessation of both opioid consumption and pain. The primary end point was time to opioid cessation. All analyses were controlled for the type of surgery done., Results: Overall, 6% of patients continued on new opioids 150 days after surgery. Preoperative prescribed opioid use, depressive symptoms, and increased self-perceived risk of addiction were each independently associated with more prolonged opioid use. Preoperative prescribed opioid use was associated with a 73% (95% confidence interval [CI] 0.51%-87%) reduction in the rate of opioid cessation after surgery (P = 0.0009). Additionally, each 1-point increase (on a 4-point scale) of self-perceived risk of addiction was associated with a 53% (95% CI 23%-71%) reduction in the rate of opioid cessation (P = 0.003). Independent of preoperative opioid use and self-perceived risk of addiction, each 10-point increase on a preoperative Beck Depression Inventory II was associated with a 42% (95% CI 18%-58%) reduction in the rate of opioid cessation (P = 0.002). The variance in the duration of postoperative opioid use was better predicted by preoperative prescribed opioid use, self-perceived risk of addiction, and depressive symptoms than postoperative pain duration or severity., Conclusions: Preoperative factors, including legitimate prescribed opioid use, self-perceived risk of addiction, and depressive symptoms each independently predicted more prolonged opioid use after surgery. Each of these factors was a better predictor of prolonged opioid use than postoperative pain duration or severity.

ATP7A is a copper-transporting ATPase critical for central and peripheral nervous system function. Mutations in ATP7A cause Menkes disease and occipital horn syndrome (OHS), allelic X-linked recessive conditions that feature vascular abnormalities ascribed to low activity of lysyl oxidase, a copper-dependent enzyme. From a recently created Menkes disease/OHS patient registry, we identified four of 95 patients with major congenital heart defects (4.2%), a proportion exceeding the general population prevalence (≈1%). In conjunction with mouse models of Menkes disease, OHS, and lysyl oxidase deficiency (which feature aortic aneurysms, irregular attachment between vascular endothelium and mesoderm, and other defects of embryological development) our observation suggests an important role of copper metabolism in cardiac development. Congenital heart disease may be an under-appreciated abnormality in Menkes disease, and should be considered in a broad differential diagnosis of cardiac defects found prenatally in male fetuses. Conversely, newborn infants with suspected or confirmed Menkes disease should be evaluated for heart disease by careful clinical examination and echocardiography, if indicated.

Background: The recurrence of regurgitation after surgical mitral valve (MV) repair remains a significant clinical problem. Mitral annuloplasty rings are commonly used in MV repair procedures. The purpose of this study was to demonstrate the feasibility of transvenous valve-in-ring (VIR) implantation using the Melody valve (Medtronic, Minneapolis, MN), which is a valved-stent designed for percutaneous pulmonary valve replacement, and 4 distinct types of annuloplasty ring (AR) in an ovine model., Methods: Ten sheep underwent surgical MV annuloplasty ring placement (n=10): CE-Physio, Edwards Lifesciences, Irvine, CA [n=5]; partial ring [n=3]; flexible ring [n=1]; and saddle ring [n=1]). All animals underwent cardiac catheterization, hemodynamic assessment, and Melody VIR implantation through a transfemoral venous, transatrial septal approach 1 week after surgery. Follow-up hemodynamic, angiographic, and echocardiographic data were recorded., Results: Melody VIR implantation was technically successful in all but 1 animal. In this animal a 26-mm partial AR proved too large for secure anchoring of the Melody valve. In the remaining 9 animals, fluoroscopy showed the Melody devices securely positioned within the annuloplasty rings. Echocardiography revealed no perivalvular leak, and angiography revealed no left ventricular outflow tract obstruction, vigorous left ventricular function, and no aortic valve insufficiency. The median procedure time was 55.5 (range, 45 to 78) minutes., Conclusions: This study demonstrates the feasibility of a purely percutaneous approach to MV replacement in patients with preexisting annuloplasty rings, regardless of ring type. This approach may be of particular benefit to patients with failed repair of ischemic mitral regurgitation., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Systemic-pulmonary collateral (SPC) flow occurs commonly in single ventricle patients after superior cavo-pulmonary connection, with unclear clinical significance. We sought to evaluate the association between SPC flow and acute post-Fontan clinical outcomes using a novel method of quantifying SPC flow by cardiac magnetic resonance (CMR) imaging., Methods and Results: All patients who had SPC flow quantified by CMR imaging before Fontan were retrospectively reviewed to assess for acute clinical outcomes after Fontan completion. Forty-four subjects were included who had Fontan completion between May 2008 and September 2010. SPC flow prior to Fontan measured 1.5±0.9 L/min/m(2), accounting for 31±11% of total aortic flow and 44±15% of total pulmonary venous flow. There was a significant linear association between natural log-transformed duration of hospitalization and SPC flow as a proportion of total aortic (rho=0.31, P=0.04) and total pulmonary venous flow (rho=0.29, P=0.05). After adjustment for Fontan type and presence of a fenestration, absolute SPC flow was significantly associated with hospital duration ≥7 days (odds ratio [OR]=9.2, P=0.02) and chest tube duration ≥10 days (OR=22.7, P=0.009). Similar associations exist for SPC flow as a percentage of total aortic (OR=1.09, P=0.048 for hospitalization ≥7 days; OR=1.24, P=0.007 for chest tube duration ≥10 days) and total pulmonary venous flow (OR=1.07, P=0.048 for hospitalization ≥7 days; OR=1.18, P=0.006 for chest tube duration ≥10 days)., Conclusions: Increasing SPC flow before Fontan, as measured by CMR imaging, is associated with increased duration of hospitalization and chest tube following Fontan completion.

Background and Aim of the Study: The treatment of pulmonary insufficiency (PI) following reconstructive surgery of the right ventricular outflow tract (RVOT) in repair of the tetralogy of Fallot remains a significant challenge. The study aim was to establish an ovine model of dilated RVOT and PI, and to quantify the degree of PI and right ventricular remodeling over an eight-week period, using magnetic resonance imaging (MRI)., Methods: Five sheep underwent baseline MRI scanning and catheterization. The weight-indexed right and left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF) and pulmonary regurgitant fraction (RF) were measured at baseline. The animals then underwent pulmonary valvectomy and transannular patch repair of the RVOT. Repeat MRI and hemodynamic measurements were obtained after an eight-week period., Results: The indexed RVEDV increased from 49 +/- 4.0 ml/m2 at baseline to 80 +/- 10.3 ml/m2 at eight weeks after valvectomy (p = 0.01), while the indexed RVESV increased from 13 +/- 3.4 ml/m2 to 33 +/- 8.8 ml/m2 (p = 0.01). The indexed RVSV increased from 36 +/- 3.7 ml/m2 to 47 +/- 1.7 ml/m2 (p = 0.01). The RVEF at baseline was 74 +/- 6%, and this decreased to 59 +/- 5% at follow up (p = 0.02). The RF at baseline was 0 +/- 0% and was increased to 37 +/- 3% at eight weeks after valvectomy (p < 0.001). The left ventricular (LV) function was also diminished: LVEF at baseline was 67 +/- 2%, versus 49 +/- 10% at follow up (p = 0.01). Both, the RV and LV end-diastolic pressures were significantly elevated at follow up., Conclusion: All five animals developed pulmonary regurgitation sufficient to cause significant RV dilatation and diminished RV and LV functions. This model may be used to investigate novel therapeutic approaches in the treatment of this difficult clinical problem.

Left ventricular (LV) diastolic dysfunction is associated with poor outcomes after tetralogy of Fallot (TOF) repair, although its cause is not known, and its relation to right ventricular (RV) performance has never been examined. The aim of this study was to test the hypothesis that RV dilation leads to LV diastolic dysfunction after TOF repair. Patients with repaired TOF who underwent cardiac catheterization and cardiac magnetic resonance imaging within 6 months from January 2003 and April 2011 were reviewed to assess the relation of LV end-diastolic pressure (LVEDP) and indexed RV end-diastolic volume (RVEDVi). Thirty-eight patients were included at a median age of 10.1 years (range 0.6 to 54.7). There was a significant linear association between RVEDVi and LVEDP (p = 0.05). RV end-diastolic pressure (p <0.001), right pulmonary artery systolic pressure (p = 0.009), left pulmonary artery systolic pressure (p = 0.02), and total cardiopulmonary support time (p = 0.04) during TOF repair were also significantly associated with LVEDP. Compared to patients with LVEDP <12 mm Hg, those with LVEDP ≥12 mm Hg had significantly higher mean RVEDVi (135.2 ± 47.8 vs 98.6 ± 28 ml/m(2), p = 0.007) and mean RV end-diastolic pressure (11.7 ± 1.6 vs 8.5 ± 2.8 mm Hg, p = 0.0003). In conclusion, after TOF repair, LVEDP is significantly associated with RVEDVi. Furthermore, mean RVEDVi is significantly higher in patients with LVEDP ≥12 mm Hg. These findings support the theory that RV dilation may impair LV diastolic function and that LV parameters may also be important to consider in determining timing of pulmonary valve replacement., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Pigeon 'milk' and mammalian milk have functional similarities in terms of nutritional benefit and delivery of immunoglobulins to the young. Mammalian milk has been clearly shown to aid in the development of the immune system and microbiota of the young, but similar effects have not yet been attributed to pigeon 'milk'. Therefore, using a chicken model, we investigated the effect of pigeon 'milk' on immune gene expression in the Gut Associated Lymphoid Tissue (GALT) and on the composition of the caecal microbiota. Chickens fed pigeon 'milk' had a faster rate of growth and a better feed conversion ratio than control chickens. There was significantly enhanced expression of immune-related gene pathways and interferon-stimulated genes in the GALT of pigeon 'milk'-fed chickens. These pathways include the innate immune response, regulation of cytokine production and regulation of B cell activation and proliferation. The caecal microbiota of pigeon 'milk'-fed chickens was significantly more diverse than control chickens, and appears to be affected by prebiotics in pigeon 'milk', as well as being directly seeded by bacteria present in pigeon 'milk'. Our results demonstrate that pigeon 'milk' has further modes of action which make it functionally similar to mammalian milk. We hypothesise that pigeon 'lactation' and mammalian lactation evolved independently but resulted in similarly functional products.

Objectives: The purpose of this study was to demonstrate the feasibility of percutaneous transvenous mitral valve-in-ring (VIR) implantation using the Melody valve in an ovine model., Background: The recurrence of mitral regurgitation following surgical mitral valve (MV) repair in both adult and pediatric patients remains a significant clinical problem. Mitral annuloplasty rings are commonly used in MV repair procedures and may serve as secure landing zones for percutaneous valves., Methods: Five sheep underwent surgical MV annuloplasty (24 mm, n = 2; 26 mm, n = 2; 28 mm, n = 1). Animals underwent cardiac catheterization with VIR implantation via a transfemoral venous, transatrial septal approach 1 week following surgery. Hemodynamic, angiographic, and echocardiographic data were recorded before and after VIR., Results: VIR was technically successful and required <1 h of procedure time in all animals. Fluoroscopy demonstrated securely positioned Melody valves within the annuloplasty ring in all animals. Angiography revealed no significant MV regurgitation in 4 and moderate central MV regurgitation in the animal with the 28-mm annuloplasty. All animals demonstrated vigorous left ventricular function, no outflow tract obstruction, and no aortic valve insufficiency., Conclusions: This study demonstrated the feasibility of a purely percutaneous approach to MV replacement in patients with preexisting annuloplasty rings. This novel approach may be of particular benefit to patients with failed repair of ischemic mitral regurgitation and in pediatric patients with complex structural heart disease., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Both male and female pigeons have the ability to produce a nutrient solution in their crop for the nourishment of their young. The production of the nutrient solution has been likened to lactation in mammals, and hence the product has been called pigeon 'milk'. It has been shown that pigeon 'milk' is essential for growth and development of the pigeon squab, and without it they fail to thrive. Studies have investigated the nutritional value of pigeon 'milk' but very little else is known about what it is or how it is produced. This study aimed to gain insight into the process by studying gene expression in the 'lactating' crop., Results: Macroscopic comparison of 'lactating' and non-'lactating' crop reveals that the 'lactating' crop is enlarged and thickened with two very obvious lateral lobes that contain discrete rice-shaped pellets of pigeon 'milk'. This was characterised histologically by an increase in the number and depth of rete pegs extending from the basal layer of the epithelium to the lamina propria, and extensive proliferation and folding of the germinal layer into the superficial epithelium. A global gene expression profile comparison between 'lactating' crop and non-'lactating' crop showed that 542 genes are up-regulated in the 'lactating' crop, and 639 genes are down-regulated. Pathway analysis revealed that genes up-regulated in 'lactating' crop were involved in the proliferation of melanocytes, extracellular matrix-receptor interaction, the adherens junction and the wingless (wnt) signalling pathway. Gene ontology analysis showed that antioxidant response and microtubule transport were enriched in 'lactating' crop., Conclusions: There is a hyperplastic response in the pigeon crop epithelium during 'lactation' that leads to localised cellular stress and expression of antioxidant protein-encoding genes. The differentiated, cornified cells that form the pigeon 'milk' are of keratinocyte lineage and contain triglycerides that are likely endocytosed as very low density lipoprotein (VLDL) and repackaged as triglyceride in vesicles that are transported intracellularly by microtubules. This mechanism is an interesting example of the evolution of a system with analogies to mammalian lactation, as pigeon 'milk' fulfils a similar function to mammalian milk, but is produced by a different mechanism.

Background: The primary goal of surgical mitral repair is the reestablishment of normal leaflet coaptation. Surgical techniques that maintain or restore leaflet geometry promote leaflet coaptation. Recent 3-dimensional (3D) echocardiographic studies have shown that saddle-shaped annuloplasty has a salutary influence on leaflet geometry. Therefore we hypothesized that saddle-shaped annuloplasty would improve leaflet coaptation in cases of repair for flail posterior leaflet segments., Methods: Sixteen patients with flail posterior segment and severe mitral regurgitation had valve repair using standard techniques. Eight patients received saddle-shaped annuloplasty and 8 patients received flat annuloplasty. Real-time 3D transesophageal echocardiography was performed before and after repair. Images were analyzed using custom software to calculate mitral annular area (MAA), septolateral dimension (SLD), intercommissural width (CW), total leaflet area (TLA), and leaflet coaptation area (LCA)., Results: Postrepair MAA (flat, 588.6±26.5 mm2; saddle, 628.0±35.3 mm2; p=0.12) and TLA (flat, 2198.5±151.6 mm2; saddle, 2303.9±183.8 mm2; p=0.67) were similar in both groups. Postrepair LCA was significantly greater in the saddle group than in the flat group (226.8±24.0 mm2 and 154.0±13.0 mm2, respectively; p=0.02)., Conclusions: Real-time 3D echocardiography and novel imaging software provide a powerful tool for analyzing mitral leaflet coaptation. When compared with flat annuloplasty, saddle-shaped annuloplasty improves LCA after mitral valve repair for severe mitral regurgitation secondary to flail posterior leaflet segment. Use of saddle-shaped annuloplasty devices may increase repair durability., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: X-ray magnetic resonance fusion (XMRF) allows for use of 3D data during cardiac catheterization. However, to date, technical requirements have limited the use of this modality in clinical practice. We report on a new internal-marker XMRF method that we have developed and describe how we used XMRF during cardiac catheterization in congenital heart disease., Methods and Results: XMRF was performed in a phantom and in 23 patients presenting for cardiac catheterization who also needed cardiac MRI for clinical reasons. The registration process was performed in < 5 minutes per patient, with minimal radiation (0.004 to 0.024 mSv) and without contrast. Registration error was calculated in a phantom and in 8 patients using the maximum distance between angiographic and 3D model boundaries. In the phantom, the measured error in the anteroposterior projection had a mean of 1.15 mm (standard deviation, 0.73). The measured error in patients had a median of 2.15 mm (interquartile range, 1.65 to 2.56 mm). Internal markers included bones, airway, image artifact, calcifications, and the heart and vessel borders. The MRI data were used for road mapping in 17 of 23 (74%) cases and camera angle selection in 11 of 23 (48%) cases., Conclusions: Internal marker-based registration can be performed quickly, with minimal radiation, without the need for contrast, and with clinically acceptable accuracy using commercially available software. We have also demonstrated several potential uses for XMRF in routine clinical practice. This modality has the potential to reduce radiation exposure and improve catheterization outcomes.

Objective: To determine the incidence, risk factors, and outcomes after early, unplanned intervention on systemic-to-pulmonary artery shunts in neonates., Methods: We retrospectively studied all neonates undergoing systemic-to-pulmonary artery shunt placement at The Children's Hospital of Philadelphia between September 1, 2002, and May 1, 2005. Patients requiring transcatheter or surgical systemic-to-pulmonary artery shunt intervention before discharge were compared with those not undergoing shunt intervention., Results: A total of 206 patients underwent shunt placement. Diagnoses included hypoplastic left heart syndrome (62.1%), pulmonary atresia (15%), tricuspid atresia (4.9%), tetralogy of Fallot (2.4%), and other lesions with obstruction to systemic (10.7%) or pulmonary blood flow (4.9%). Twenty-one interventions occurred in 20 patients (9.7%). Risk factors for intervention included heterotaxy syndrome (P = .04), congenital abnormality (P = .04), and a trend toward lower birthweight. In patients with a modified Blalock-Taussig shunt, similar risk factors were identified and the incidence of intervention decreased with increasing shunt size. In-hospital mortality was 30% (6/20) for the cases and 8.1% (15/186) for the nonintervention group (P = .02). Long-term survival was significantly lower in patients requiring intervention (P = .002). This group also had a higher incidence of infections (P < .001) and extracorporeal membrane oxygenation (P < .001), and longer hospital stay (P = .001)., Conclusions: In neonates undergoing systemic-to-pulmonary artery shunt placement, approximately 10% underwent shunt intervention before discharge. Some factors, such as low birthweight, shunt size, noncardiac congenital abnormalities, and heterotaxy syndrome, may help identify patients at risk. Patients undergoing intervention experienced increased morbidity and mortality., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: The practice of coiling aortopulmonary collaterals (APCs) before Fontan completion is controversial, and published data are limited. We sought to compare outcomes in subjects with and without pre-Fontan coil embolization of APCs using the Pediatric Heart Network Fontan Cross-Sectional Study database which enrolled survivors of prior Fontan palliation., Methods: We compared hospital length of stay after Fontan in 80 subjects who underwent APC coiling with 459 subjects who did not. Secondary outcomes included post-Fontan complications and assessment of health status and ventricular performance at cross-sectional evaluation (mean 8.6 ± 3.4 years after Fontan)., Results: Centers varied markedly in frequency of pre-Fontan APC coiling (range 0%-30% of subjects, P < .001). The coil group was older at Fontan (P = .004) and more likely to have single right ventricular morphology (P = .054) and pre-Fontan atrioventricular valve regurgitation (P = .03). The coil group underwent Fontan surgery more recently (P < .001), was more likely to have a prior superior cavopulmonary anastomosis (P < .001), and more likely to undergo extracardiac Fontan connection (P < .001) and surgical fenestration (P < .001). In multivariable analyses, APC coiling was not associated with length of stay (hazard ratio for remaining in-hospital 0.91, 95% CI 0.70-1.18, P = .48) or postoperative complications, except more post-Fontan catheter interventions (hazard ratio 1.74, 95% CI 1.04-2.91, P = .03), primarily additional APC coils. The groups had similar outcomes at cross-sectional evaluation., Conclusion: Management of APCs before Fontan shows marked practice variation. We did not find an association between pre-Fontan coiling of APCs and shorter postoperative hospital stay or with better late outcomes. Prospective studies of this practice are needed., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Objectives: We sought to investigate whether differential branch pulmonary artery (BPA) regurgitation correlates with differences in BPA anatomy and physiology., Background: Patients with repaired conotruncal anomalies such as Tetralogy of Fallot frequently have residual BPA stenosis or BPA size differences. Previous reports have demonstrated an increased left pulmonary artery (LPA) regurgitant fraction (RF) in these patients., Methods: We retrospectively reviewed 76 consecutive cardiac magnetic resonance (CMR) studies for BPA size and phase-contrast magnetic resonance data, including 13 consecutive patients who underwent both CMR and catheterization., Results: Thirty of the 76 patients had either BPA stenosis or significant size discrepancy. Whereas previous studies had shown an increased RF in the LPA, patients with BPA stenosis or size discrepancy showed no significant difference between right and left BPA RF (30% vs. 30%, p = 0.985). However, there was a significantly increased RF of the larger versus smaller BPA (39% vs. 21%, p < 0.001), resulting in an insignificant deviation from normal fractional flow distribution (RPA 63% vs. LPA 37%; normal net fractional flow distribution RPA 55% vs. LPA 45%). Retrospective review of patients who underwent both CMR and catheterization provides support for the preceding findings and validates differential BPA RF as strongly correlating with differential pulmonary vascular resistance (PVR) (r = 0.8364, p < 0.001)., Conclusions: BPA RF is a function of the relative PVR and the presence of BPA stenosis or size discrepancy. Contrary to prior reports, the LPA RF is only elevated in patients with relatively equal sized BPAs. In the setting of BPA stenosis or size discrepancy the larger BPA has a relatively increased RF and PVR. Therefore, the differential RF is an important tool for screening patients with unilateral stenosis for contralateral increases in PVR that cannot be identified with net flows alone. This can affect the indication and timing for BPA intervention., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

We present a case of simultaneous occurrence of 2 rare congenital anomalies. A 57-year-old woman undergoing evaluation of dyspnea was found to have a single coronary artery and persistent left superior vena cava. The incidence of single coronary artery is 0.024% to 0.066% in the general population. Persistent left superior vena cava occurs in 0.3% of those without other congenital anomalies and in up to 5% when other anomalies are present. The likelihood of both anomalies occurring as a random event in 1 patient is approximately 1 in 10 million. Patient characteristics and data are presented, with a discussion on the epidemiology, incidence, diagnosis and pathologic implications of each anomaly.

Background: Transannular patch (TAP) repair of tetralogy of Fallot often results in significant right ventricular outflow tract (RVOT) dilation and distortion. We hypothesized that insertion of Melody valves into the proximal right and left branch pulmonary arteries (PAs) would reduce pulmonary regurgitation fraction (PRF) in an ovine model of pulmonary insufficiency and dilated RVOT., Methods and Results: Ten sheep underwent baseline cardiac catheterization, surgical pulmonary valvectomy, and TAP placement. A subset (n=5) had Melody valves (2 devices per animal) inserted into the proximal right and left PAs during the surgical procedure. Melody valves were placed distal to the right-upper-lobe (RUL) artery branch, leaving the RUL "unprotected." Preoperative MRIs (n=5) were used to determine baseline RV ejection fraction (RVEF) and left ventricular (LV) EF. All surviving animals (n=9) underwent MRI and catheterization 6 weeks postsurgery. Mean PRF was lower in the Melody valve group (15±6% versus 37±3%; P=0.014). The unprotected RUL was responsible for 64% of the PRF measured in the Melody valve group. In the non-Melody group, the RVEF was lower than baseline (P=0.003) and than in the Melody group (P=0.05). The LVEF was also lower in the non-Melody group versus baseline (P=0.004) and versus Melody (P=0.01)., Conclusions: Bilateral branch PA Melody valve implantation significantly reduced PRF and altered RV and LV function favorably in a model of TAP for tetralogy of Fallot. This novel intervention may offer potential benefit in treating patients with anatomically heterogeneous disease of the RVOT.

Objectives: This study sought to retrospectively evaluate our initial experience using angiographic computed tomography (ACT) in a pediatric cardiac catheterization laboratory., Background: ACT provides cross-sectional CT images from a rotational angiography run using a C-arm mounted flat-panel detector in the interventional suite. A 3-dimensional (3D) angiographic image can be created from the CT volume set and used in real time during the procedure. To our knowledge, its use has never previously been described for congenital heart disease., Methods: 3D reconstructions were created and we retrospectively reviewed cases during our first year of ACT use. Images obtained were independently evaluated to determine their diagnostic utility. Radiation dose reduction protocols were defined using phantom testing and radiation dose calculation., Results: ACT was used during 41 cardiac catheterizations in patients at a median age of 5.1 years (range: 0.4 to 58.8 years) for evaluation of: right ventricular outflow tract (RVOT)/central pulmonary arteries (PAs) in 20; cavopulmonary connection (CPC) in 11; pulmonary veins in 5; distal PAs in 4; and other locations in 5. Four subjects had 2 anatomic areas studied by ACT. The mean contrast volume for ACT was 1.2 ± 0.4 ml/kg. Diagnostic-quality imaging was obtained in 71% of cases: 13/20 RVOT/central PAs; 9/11 CPC; 4/5 pulmonary veins; 2/4 distal PAs; and 4/5 others. In 12 cases, ACT contributed to clinical outcomes beyond standard angiography. Radiation dose reduction protocols allowed ACT to be comparable in exposure to a standard biplane cineangiogram., Conclusions: Diagnostic-quality imaging can be obtained using ACT in 71% of cases without a significant increase in contrast or radiation exposure. In certain cases, ACT provides additional anatomic detail and may aid complex catheter manipulations. Future work is needed to continue to define applications of this new technology., (Copyright © 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: To describe the use of the Amplatzer vascular plug (plug 1) and Amplatzer vascular plug II (plug 2) in patients with congenital cardiovascular disease (CCVD)., Background: Plugs 1 and 2 have recently been made available. We report our experience describing plugs 1 and 2 in patients with CCVD highlighting the versatility of these devices., Methods: All patients with CCVD who underwent a vascular occlusion procedure at the Children's Hospital of Philadelphia between August 1, 2004 and July 30, 2009 with plug 1 or 2 were included. A retrospective review was performed., Results: Fifty patients underwent vascular occlusion procedure with plug 1 or 2 at a median age of 2.0 years (range 1 day to 47 years) and median weight of 12.3 kg (range 3.1-98 kg). Fifty-eight plugs (43% plug 1, 57% plug 2) were placed in 52 vessels. Of these vessels, 20 (38%) were patent ductus arteriosus (PDA), 14 (27%) venous collaterals, 5 (10%) aorto-pulmonary collaterals, 4 (8%) modified Blalock Taussig shunts, 3 (5%) porto-systemic connections, and 6 (12%) miscellaneous structures. Excluding a patient who was lost to follow-up, complete occlusion was observed in 100% of vessels either at the time of the catheterization or at follow-up. There were two complications (3.8%)., Conclusions: Plugs 1 and 2 are safe and effective devices that can be used in a variety of blood vessels in patients with CCVD. Plug 2 is particularly useful in closure of high-flow, tubular structures, especially type C PDA's.

Background: The efficacy of annuloplasty for ischemic mitral regurgitation (IMR) has been difficult to establish. Using an established ovine model of IMR, we tested the ability of ring annuloplasty to durably relieve IMR and reverse or limit progression of left ventricular (LV) remodeling during a clinically relevant follow-up period., Methods: A posterolateral infarction known to result in chronic IMR was initiated in 33 sheep. Echocardiography was used to assess LV end diastolic and systolic volumes and IMR (0 to 4 scale) before and 8 weeks after infarction. Eight weeks after infarction, 20 surviving animals with > or = 2+ IMR were randomized (1:1) to no treatment or undersized, semi-rigid, complete ring annuloplasty placement. LV remodeling and IMR were assessed at 4 and 6 months after infarction., Results: All animals had similarly sized LV volumes at baseline (end systolic, 27.8 +/- 4.6 mL; end diastolic, 53.5 +/- 6.4 mL). The 20 randomized animals survived to complete the study. The degree of IMR before randomization was similar in treatment (2.6 +/- 0.4) and control (2.8 +/- 0.3) groups. At the 6-month follow-up, the degree of IMR was significantly less in the annuloplasty group (0.3 +/- 0.1 vs 3.4 +/- 0.6); however, LV volumes in the treatment group were not significantly different from the control group (end systolic, 82.1 +/- 15.6 vs 81.1 +/- 8.6 mL; end diastolic, 110.4 +/- 22.1 vs 111.1 +/- 16.5 mL)., Conclusions: In a clinically relevant ovine model of IMR, annuloplasty provides durable relief from IMR during an extended follow-up period but does not significantly influence LV remodeling., (2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Patients frequently undergo cardiac catheterization before the Fontan operation because of the limited echocardiographic windows in the region of the superior cavopulmonary connection and branch pulmonary arteries. Patients with obstruction to pulmonary blood flow are at increased risk for prolonged length of hospital stay after the Fontan operation. Cardiac magnetic resonance has unlimited imaging windows and can quantify both the branch pulmonary artery size and net flow distribution and thereby serve as a method for identifying patients at increased risk for prolonged length of stay., Methods: We retrospectively reviewed 24 cardiac magnetic resonance studies of patients (mean age, 3.1 +/- 1.0 years) referred before the Fontan operation. Cardiac magnetic resonance measured the cross-sectional area and flow to each branch pulmonary artery. Post-Fontan hospital course data were acquired from the medical record., Results: Prolonged length of stay after the Fontan operation is observed among patients with one branch that is less than 25% of the total cross-sectional area (18.0 +/- 5.5 vs 8.2 +/- 3.8 days, P = .01) or with less than 40% flow to one branch (12.5 +/- 6.9 vs 7.6 +/- 1.5 days, P = .04). There is moderate correlation between the total branch pulmonary area and length of stay (r = -0.75)., Conclusions: Cardiac magnetic resonance noninvasively assesses the branch pulmonary area size and flow before the Fontan operation. These data predict which patients are more likely to experience a prolonged hospital course.

Background: Systemic-to-pulmonary collateral flow (SPCF) is common in single-ventricle patients with superior cavopulmonary connections (SCPC). Because no validated method to quantify that SPCF exists, neither its hemodynamic burden nor its clinical impact can be systematically evaluated. We hypothesize that (1) the difference in total ascending aortic (Ao) and caval flow (superior vena cava [SVC]+inferior vena cava [IVC]) and (2) the difference between pulmonary vein and pulmonary artery flow (PV-PA) provide 2 independent estimators of SPCF., Methods and Results: We measured Ao, SVC, IVC, right (RPA) and left (LPA) PA, and left (LPV) and right (RPV) PV flows in 17 patients with SCPC during routine cardiac MRI studies using through-plane phase-contrast velocity mapping. Two independent measures of SPCF were obtained: model 1, Ao-(SVC+IVC); and model 2, (LPV-LPA)+(RPV-RPA). Values were normalized to body surface area, Ao, and PV, and comparisons were made using linear regression and Bland-Altman analysis. SPCF ranged from 0.2 to 1.4 L/min for model 1 and 0.2 to 1.6 L/min for model 2, for an average indexed SPCF of 0.5 to 2.8 L/min/m(2): 11% to 53% (mean, 37%) of Ao and 19% to 77% (mean, 54%) of PV. The mean difference between model 1 and model 2 was 0.01 L/min (P=0.40; 2-SD range, -0.45 to 0.47 L/min)., Conclusions: We present a noninvasive method for SPCF quantification in patients with SCPC. It should provide an important clinical tool in treating these patients. Furthermore, we show that SPCF is a significant hemodynamic burden in many patients with bidirectional Glenn shunt physiology. Future investigations will allow objective study of the impact of collateral flow on outcome.

Objective: We sought to determine the impact of relieving branch pulmonary artery stenosis on pulmonary valve insufficiency and right ventricular function. Long-standing pulmonary insufficiency causes progressive right ventricular dilatation, leading to decreased right ventricular function. Adults with pulmonary insufficiency are at risk of decreased exercise tolerance, arrhythmias, and sudden cardiac death. Branch pulmonary artery stenosis frequently occurs in these patients, and the presence of branch stenosis may exacerbate valve insufficiency., Methods: Neonatal piglets (n = 7) underwent surgery to create pulmonary insufficiency and left pulmonary artery stenosis. At 3 months of age, the animals underwent baseline cardiac magnetic resonance imaging followed by stenting of the left pulmonary artery. A repeat magnetic resonance imaging scan was performed 1 week after intervention. Magnetic resonance imaging evaluation included (1) velocity mapping to assess the forward and reverse flow at the main, left and right pulmonary arteries, and aorta; and (2) volumetric assessment of the right ventricle., Results: Left pulmonary artery flow increased from 14.5% to 36.3% of total net flow after stenting (P < .01). Pulmonary regurgitation decreased from 38.7% to 27.4% (P < .02). Right ventricular ejection fraction improved from a median of 53.5% to 58.2% after stenting (P < .01). Cardiac index improved from a median of 2.7 to 3.5 L/min/m(2) (P = .01)., Conclusion: Relief of branch pulmonary artery stenosis reduces insufficiency and improves right ventricular systolic function in this animal model. This supports the practice of aggressive intervention in patients with branch pulmonary artery stenosis and pulmonary insufficiency.

We describe a case of congenitally corrected transposition with a double aortic arch. This unique combination of lesions highlights the importance of a complete anatomic assessment prior to referral for surgery.

Background: There is no consensus on the utility of fluoroscopy in obtaining common femoral artery (CFA) access., Methods: Patients weighing < 136.4 kg (300 lbs) with palpable femoral pulses undergoing coronary angiography were randomized to arterial access with or without the use of fluoroscopy (using the center of the femoral head as the optimal site to enter the artery)., Results: 208 patients were enrolled with 110 randomized to the palpation group and 98 were randomized to the palpation +fluoroscopy group. Mean age (+/- SD) was 60 +/- 11 years, 61% were male, 35% had diabetes, and 40% had a body mass index (BMI) > 30 kg/m(2). Clinical characteristics and procedural factors were similar among the two groups with the exception that fewer needle passes were required and access was achieved faster in the palpation group. Arterial puncture over the femoral head occurred in 91% of the palpation group versus 95% of the palpation + fluoroscopy group (p = 0.27). Successful CFA puncture occurred in 85% of the palpation group versus 90% of the palpation + fluoroscopy group (p = 0.49). Cannulation of the external iliac artery occurred in 1 patient in each group, whereas arterial puncture distal to the CFA bifurcation occurred in 16 (15%) of the palpation group and in 9 (9%) of the palpation + fluoroscopy group (p = 0.33)., Conclusion: In this single-center, randomized trial, the use of fluoroscopy did not increase the probability of arterial puncture over the femoral head or the rate of successful CFA cannulation.

The effect of the contrast agent iohexol on reference vessel size in patients with proximal left anterior descending disease is unknown. Quantitative coronary angiography and intravascular ultrasound were performed in 15 patients with atherosclerotic disease of the proximal left anterior descending. Mean proximal reference vessel diameter was 2.95 +/- 0.59 mm with quantitative coronary angiography and 4.65 +/- 0.66 mm with intravascular ultrasound (P < .05). Intracoronary injection of iohexol resulted in a significant decrease in intravascular ultrasound-measured proximal reference vessel diameter from 4.65 +/- 0.66 mm to 4.47 +/- 0.68 mm (P = .002). Vasoconstrictive response to iohexol in the proximal reference vessel ranged from -0.04 mm to 0.5 mm with a mean of 0.18 +/- 0.16 mm. This study shows that iohexol can cause significant vasoconstriction of the proximal reference vessel in patients with severe disease involving the proximal left anterior descending.

We describe two cases in which a patent ductus arteriosus (PDA) was successfully occluded percutaneously, using the novel approach of prefilling an Amplatzer Vascular Plug with embolization coils to decrease the risk of residual shunting through the device. One patient was a small premature neonate, and the other was a child with an aneurismal, tubular PDA with no aortic ampulla. These are situations in which more popular PDA devices such as the Amplatzer Duct Occluder may be contraindicated., ((c) 2008 Wiley-Liss, Inc.)