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Background:A 10% prevalence of intracranial aneurysms in patients with coarctation of the aorta has been described in a few studies. Our objective is to describe the rate of intracranial aneurysm detection in patients with coarctation of the aorta in the current era. We hypothesise that, with earlier detection and coarctation of the aorta intervention, the rate of intracranial aneurysm is lower than previously reported and screening imaging may only be warranted in older patients or patients with certain risk factors.Methods:This is a retrospective study of 102 patients aged 13 years and older with coarctation who underwent brain computed tomography angiography, magnetic resonance imaging (MRI), or magnetic resonance angiography between January, 2000 and February, 2018.Results:The median age of coarctation repair was 4.4 months (2 days–47 years) and the initial repair was primarily surgical (90.2%). There were 11 former smokers, 4 current smokers, and 13 patients with ongoing hypertension. Imaging modalities included computed tomography angiography (13.7%), MRI (41.2%), and magnetic resonance angiography (46.1%), performed at a median age of 33.3 years, 22.4 years, and 25 years, respectively. There were 42 studies performed for screening, 48 studies performed for neurologic symptoms, and 12 studies performed for both screening and symptoms. There were no intracranial aneurysms detected in this study.Conclusions:These results suggest that the rate of intracranial aneurysms may be lower than previously reported and larger studies should explore the risk of intracranial aneurysms in coarctation of the aorta in the current era.

Background There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve. Objectives This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve. Methods Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy. Results Twenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40 mm Hg. During limited follow-up (n = 349; median: 12 months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR. Conclusions Acute outcomes after TPVR with balloon-expandable valves were generally excellent in all types of RVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

Objectives To describe a multicenter experience of percutaneous transcatheter pulmonary valve replacement (TPVR) using the Edwards SAPIEN S3 valve without the use of a prior stent ("pre-stenting"). Background The SAPIEN S3 and XT valves have durable cobalt-chromium stent frames which may allow for TPVR in large diameter dysfunctional right ventricular outflow tracts (RVOTs) without pre-stenting the landing zone. Methods A retrospective review was performed of all patients with Congenital Heart Disease and dysfunctional RVOT who underwent TPVR using the SAPIEN valve without the use of a pre-stent. Imaging data, procedural elements, and clinical follow-up data were collected to evaluate short and intermediate-term results. Results Fifty-seven patients underwent percutaneous placement of the SAPIEN valve in the pulmonary position without the use of pre-stenting. The anatomical substrate varied: native RVOTs (n = 41), conduits (n = 10), and bioprosthetic valves (n = 6). There were no cases in which the valve could not be implanted and no cases of valve embolization or misplacement. On follow-up (range 1 month to 2.2 years, median 5.3 months), no patients had significant obstruction or regurgitation around the valve. There were no frame fractures. There were no procedural deaths. Major complications included severe aortic compression (n = 1) requiring surgical explantation and tricuspid valve injury requiring surgical intervention (n = 2). Conclusions This limited multi-institutional experience demonstrates that the SAPIEN valve can be used for TPVR without the use of a pre-stent without medium-term risk of frame fracture, paravalvar leak, or embolization. Longer term follow-up is required to fully assess this method.

Background Transcatheter pulmonary valve replacement (TPVR) is increasingly utilized to address residual or recurrent right ventricular outflow tract pathology in congenital heart disease patients. We aimed to evaluate clinical outcomes and resource utilization comparing TPVR to surgical pulmonary valve replacement (SPVR) for this patient population. Methods From January 2010 until December 2015, 224 patients underwent pulmonary valve replacement (SPVR = 100 and TPVR = 124). These groups were assessed and compared for survival, reoperation, reintervention (surgical or catheter based), hospital costs, and hemodynamic performance. Results There were no mortalities in either group. Despite SPVR patients being significantly younger (12 ± 7 years of age versus 19 ± 13 years of age; p Conclusions Hemodynamic performance is similar between SPVR and TPVR with similar rates of reintervention. While SPVR patients have a longer hospital length of stay, TPVR was associated with higher rates of infective endocarditis, hospital costs, and failure to implant.

Objectives: This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular out...

BACKGROUND Since FDA approval of the Melody valve, transcatheter pulmonary valve replacement (TPVR) has been offered to an expanding population. Limited data exist regarding the safety and feasibility of TPVR in smaller patients. METHODS All patients weighing

Hemoptysis may occur in patients with pulmonary venous obstruction and prominent decompressing vessels in the airways adjacent to the affected pulmonary veins. The options for treatment of hemoptysis are limited, particularly when efforts to alleviate pulmonary venous obstruction have failed. Here we describe a patient with hemoptysis associated with stenosis of the central left upper pulmonary vein and occlusion of the central left lower pulmonary vein. The left upper pulmonary vein was dilated with balloon catheters and a vascular plug was placed in the left lower pulmonary artery. Vascular engorgement regressed in the left bronchus and hemoptysis has not recurred for 4 years despite recurrence of left upper pulmonary vein stenosis. Selective occlusion of branch pulmonary arteries may be an effective option for the treatment of hemoptysis from bleeding in lung segments with inoperable pulmonary venous obstruction.

In adults with congenital heart disease, multiple studies have found high rates of anxiety and depression but there is sparse evidence regarding the effect of pregnancy on mental health. We aim to describe the rate of, and risk factors for, postpartum depression (PPD) in women with heart disease.

This study sought to evaluate the safety, feasibility, and outcomes of transcatheter pulmonary valve replacement (TPVR) in conduits ≤16 mm in diameter.The Melody valve (Medtronic, Minneapolis, Minnesota) is approved for the treatment of dysfunctional right ventricular outflow tract (RVOT) conduits ≥16 mm in diameter at the time of implant. Limited data are available regarding the use of this device in smaller conduits.The study retrospectively evaluated patients from 9 centers who underwent percutaneous TPVR into a conduit that was ≤16 mm in diameter at the time of implant, and reported procedural characteristics and outcomes.A total of 140 patients were included and 117 patients (78%; median age and weight 11 years of age and 35 kg, respectively) underwent successful TPVR. The median original conduit diameter was 15 (range: 9 to 16) mm, and the median narrowest conduit diameter was 11 (range: 4 to 23) mm. Conduits were enlarged to a median diameter of 19 mm (29% larger than the implanted diameter), with no difference between conduits. There was significant hemodynamic improvement post-implant, with a residual peak RVOT pressure gradient of 7 mm Hg (p 0.001) and no significant pulmonary regurgitation. During a median follow-up of 2.0 years, freedom from RVOT reintervention was 97% and 89% at 2 and 4 years, respectively, and there were no deaths and 5 cases of endocarditis (incidence rate 2.0% per patient-year).In this preliminary experience, TPVR with the Melody valve into expandable small diameter conduits was feasible and safe, with favorable early and long-term procedural and hemodynamic outcomes.

Background— Transcatheter pulmonary valve replacement (TPVR) is an established therapy for dysfunctional right ventricular (RV) outflow tract conduits. TPVR in patients with congenitally corrected transposition of the great arteries, subpulmonary left ventricle, and left ventricular outflow tract (LVOT) conduit dysfunction has not been studied. Unique anatomic and physiological aspects of this population may contribute to distinct risks and outcomes. Methods and Results— Across 10 US centers, 27 patients with a dysfunctional LVOT conduit were evaluated in the catheterization laboratory between December 2008 and August 2015 with the intent to perform TPVR. TPVR was successful in 23 patients (85%). Five serious adverse events occurred in 4 cases (15%), including pulmonary hemorrhage, hypotension requiring vasoactive support, conduit disruption requiring covered stent (n=2), and acute RV dysfunction with flash pulmonary edema. After TPVR, the LVOT peak systolic ejection gradient decreased from median of 35 to 17 mm Hg ( P P =0.02) and higher post-TPVR RV sphericity index (median 0.88 versus 0.52; P =0.004). Post-TPVR, there were 2 late deaths because of RV failure and 1 cardiac transplantation because of progressive RV dysfunction and tricuspid regurgitation. Conclusions— TPVR in dysfunctional LVOT conduits is feasible but associated with an important rate of TPV nonimplantation and procedural serious adverse events. Worsening systemic RV function and tricuspid regurgitation may develop after LVOT TPVR.

Percutaneous transcatheter pulmonary valve replacement with the Melody Valve is fast becoming an important adjunct in the treatment of older children and adults with failing right ventricular outflow tract conduits. Recently, the Melody Valve has also been successfully implanted in the tricuspid, mitral, and aortic positions, typically within a failing bioprosthetic valve. We present a patient who underwent Fontan palliation for hypoplastic left heart syndrome variant and subsequently developed severe neoaortic regurgitation, which was successfully treated with a transcatheter neoaortic valve replacement. To our knowledge, this is the first successful use of the Melody Valve in the neoaortic position in a patient with single-ventricle physiology. Successful relief of neoaortic valve regurgitation using replacement with a transcatheter valve may allow avoidance of additional surgery, increase functional longevity of single-ventricle palliation, and postpone the need for orthotopic heart transplantation.

Objectives Describe outcomes following unplanned cardiac catheterization after congenital heart surgery. Background Utility of cardiac catheterization following congenital heart surgery is relatively understudied. Methods Retrospective study examining demographics, indications, and outcomes of unplanned cardiac catheterization after congenital heart surgery at a single institution. Results Between October 2004 and April 2011, 120 patients underwent 150 unplanned postoperative cardiac catheterizations. Median day of catheterization was postoperative day 20 (range 1–269 days). Survival 30 days postcatheterization was 85%; overall survival to hospital discharge was 72%. Indications for catheterization: 63 for hemodynamic evaluation, 46 for likely intervention, and 41 for assessment of surgical repair. Of the 150 hemodynamic/interventional catheterizations, 103 (69%) were associated with a change in clinical management: 59 trans-catheter interventions, 22 re-operations, 11 changes in medication, six changes in surgical plan, and five withdrawals of support. Complications included hemorrhage in two patients, supraventricular tachycardia in two patients, and transient complete heart block requiring cardiopulmonary resuscitation in one patient. Conclusions Cardiac catheterization following congenital heart surgery may enable important diagnostic and therapeutic changes in clinical and surgical management. Complications were rare. © 2014 Wiley Periodicals, Inc.

While the Fontan operation is a reliable treatment option for many complex congenital heart defects, the development of pulmonary arteriovenous malformations (PAVMs) remains a problematic outcome for some Fontan patients. Pulmonary arteriovenous malformations stem from an imbalance of hepatic blood flow in the pulmonary system. Balancing this hepatic flow has shown promising results in the treatment of PAVMs. We report the clinical course of a young patient with heterotaxy syndrome and an unbalanced right dominant atrioventricular septal defect. This patient developed PAVMs following a Fontan procedure, however, the PAVMs were resolved following the revision of the original Fontan conduit to a bifurcated conduit.

Background— Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit. Methods and Results— This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mm Hg; P P Conclusions— Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis.

Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk factors for abnormalities, and impact on right ventricular function in patients with single right ventricle lesions after arch reconstruction.Prestage II aortic angiograms from the Pediatric Heart Network Single Ventricle Reconstruction trial were analyzed to define arch geometry (Romanesque [normal], crenel [elongated], or gothic [angular]), indexed neoaortic dimensions, and distensibility. Comparisons were made with 50 single-ventricle controls without prior arch reconstruction. Factors associated with ascending neoaortic dilation, reduced distensibility, and decreased ventricular function on the 14-month echocardiogram were evaluated using univariate and multivariable logistic regression.Interpretable angiograms were available for 326 of 389 subjects (84%). Compared with controls, study subjects more often demonstrated abnormal arch geometry (67% vs 22%, P .01) and had increased ascending neoaortic dilation (Z score 3.8 ± 2.2 vs 2.6 ± 2.0, P .01) and reduced distensibility index (2.2 ± 1.9 vs 8.0 ± 3.8, P .01). Adjusted odds of neoaortic dilation were increased in subjects with gothic arch geometry (odds ratio [OR], 3.2 vs crenel geometry, P .01) and a right ventricle-pulmonary artery shunt (OR, 3.4 vs Blalock-Taussig shunt, P .01) but were decreased in subjects with aortic atresia (OR, 0.7 vs stenosis, P .01) and those with recoarctation (OR, 0.3 vs no recoarctation, P = .04). No demographic, anatomic, or surgical factors predicted reduced distensibility. Neither dilation nor distensibility predicted reduced right ventricular function.After Norwood surgery, the reconstructed neoaorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these features.

Objectives: Aortic arch reconstruction in patients with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe potentially maladaptive aortic anatomy and physiology, risk factors, and impact on right ventricle (RV) function in patients with single RV lesions after Norwood. Methods: Pre-stage II angiograms for subjects from the Single Ventricle Reconstruction (SVR) Trial were compared to 50 single left ventricle controls with no prior arch reconstruction. Arch geometry was defined as normal, crenel (elongated), or gothic (angular). Aortic index (ascending /descending aorta diameter) and distensibility index (systolic aortic area - diastolic area) / (diastolic area x pulse pressure) were calculated. Univariable tests were used for comparisons, and Spearman’s rho was used for correlation. Results: Interpretable angiograms were available for 326/389 (84%) SVR subjects. Table 1 summarizes findings. Age at catheterization was similar for SVR subjects and controls. SVR subjects more often demonstrated abnormally elongated (crenel) arch geometry with dilated ascending and transverse aortae tapering to a small isthmus. Distensibility was reduced in both the ascending and descending aorta. Risk factors for ascending aortic dilation (aortic index ≥ 2.5) included native aortic stenosis (OR = 2.2 [95% CI: 1.2, 3.8] vs. aortic atresia) and receipt of a RV-pulmonary artery shunt (OR = 2.6 [1.47, 4.5] vs. Blalock-Taussig shunt). There was no association between reduced distensibility and any demographic, anatomic, or reported surgical factors. Aortic dilation and reduced distensibility did not correlate with RV function at 14-month echocardiogram (p=0.5). Conclusions: After Norwood single RV surgery, the reconstructed aorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these findings.

Objectives To demonstrate the technical feasibility and describe intermediate outcomes for the initial patients undergoing handcrafted, area-preserving, Y-graft modification of the Fontan procedure. Methods A retrospective review of a pilot study was undertaken to describe preoperative, intraoperative, and postoperative results. Results Six patients underwent successful procedures and remain alive 3 to 4 years later. The median age at operation was 3.3 years, and median weight was 13.2 kg. Five operations were done without cardiopulmonary bypass and no intraoperative pressure gradients were found. Five patients were extubated by postoperative day 1, Fontan pressures were 12 to 14 mm Hg, transpulmonary gradients were 6 to 8 mm Hg, and no renal or hepatic function abnormalities were found. Length of stay was 10 to 64 days. One patient required venovenous extracorporeal membrane oxygenation for previously undiagnosed plastic bronchitis (64-day stay); another required reoperation for an incidentally diagnosed aortic thrombus (44-day stay). One patient had occlusion of a Y-graft limb noted on magnetic resonance imaging follow-up at 3 months. Catheterization showed excellent hemodynamic parameters and no Fontan obstruction. Occlusion was believed to be due to right-sided pulmonary arteriovenous malformations and widely discrepant flow (80%) to the right lung leading to low flow in the left limb. Conclusions The area-preserving, bifurcated Y-graft Fontan modification is technically feasible and shows excellent intermediate outcomes. Additional study is required to determine whether the advantages seen in the computational models will be realized in patients over the long-term, and to optimize patient selection for each of the various Fontan options now available.

We describe a case of a patient admitted with apparent life threatening events characterized by hypotension and bradycardia. The patient was ultimately found to have intermittent severe aortic insufficiency. Upon surgical exploration, abnormalities were discovered in the aortic valve, which had a small left coronary cusp with absence of the nodulus of Arantius. Following surgical repair of the valve, aimed at preventing the small cusp from becoming stuck in the open position, the patient has remained episode free for over one year.