Heart failure biomarker levels correlate with invasive haemodynamics in pulmonary valve replacement.

BACKGROUND: Although widely used in cardiology, relation of heart failure biomarkers to cardiac haemodynamics in patients with CHD (and in particular with pulmonary insufficiency undergoing pulmonary valve replacement) remains unclear. We hypothesised that the cardiac function biomarkers N-terminal pro-brain natriuretic peptide (NT-proBNP), soluble suppressor of tumorigenicity 2, and galectin-3 would have significant associations to right ventricular haemodynamic derangements. METHODS: Consecutive patients ( n = 16) undergoing cardiac catheterisation for transcatheter pulmonary valve replacement were studied. NT-proBNP, soluble suppressor of tumorigenicity 2, and galectin-3 levels were measured using a multiplex enzyme-linked immunosorbent assay from a pre-intervention blood sample obtained after sheath placement. Spearman correlation was used to identify significant correlations (p ≤ 0.05) of biomarkers with baseline cardiac haemodynamics. Cardiac MRI data (indexed right ventricular and left ventricular end-diastolic volumes and ejection fraction) prior to device placement were also compared to biomarker levels. RESULTS: NT-proBNP and soluble suppressor of tumorigenicity 2 were significantly correlated (p < 0.01) with baseline mean right atrial pressure and right ventricular end-diastolic pressure. Only NT-proBNP was significantly correlated with age. Galectin-3 did not have significant associations in this cohort. Cardiac MRI measures of right ventricular function and volume were not correlated to biomarker levels or right heart haemodynamics. CONCLUSIONS: NT-proBNP and soluble suppressor of tumorigenicity 2, biomarkers of myocardial strain, significantly correlated to invasive pressure haemodynamics in transcatheter pulmonary valve replacement patients. Serial determination of soluble suppressor of tumorigenicity 2, as it was not associated with age, may be superior to serial measurement of NT-proBNP as an indicator for timing of pulmonary valve replacement.

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

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

Anesthetic Management of Pheochromocytoma Resection in Adults with Single Ventricle Physiology.

Survival rates for patients with palliated congenital heart disease are increasing, and an increasing number of adults with cyanotic congenital heart disease (CCHD) might require surgical resection of pheochromocytoma-paraganglioma (PHEO-PGL). A recent study supports the idea that patients with a history of CCHD and current or historical cyanosis might be at increased risk for developing PHEO-PGL. We review the anesthetic management of two adults with single-ventricle physiology following Fontan palliation presenting for PHEO-PGL resection and review prior published case reports. We found the use of epidural analgesia to be safe and effective in the operative and postoperative management of our patients.

Immediate Outcomes of Covered Stent Placement for Treatment or Prevention of Aortic Wall Injury Associated With Coarctation of the Aorta (COAST II).

OBJECTIVES: This study aimed to describe the safety and short-term efficacy of the Covered Cheatham-Platinum stent (CCPS) in treating or preventing aortic wall injury (AWI) in patients with coarctation of the aorta (CoA). BACKGROUND: The COAST II trial (Covered Cheatham-Platinum Stents for Prevention or Treatment of Aortic Wall Injury Associated with Coarctation of the Aorta Trial) is a multicenter, single-arm trial using the CCPS for the treatment and/or prevention of AWI in patients with CoA and pre-existing AWI or increased risk of AWI. METHODS: Patients were enrolled if they had a history of CoA with pre-existing AWI (Treatment group) or with increased risk of AWI (Prevention group). Pre/post-implant hemodynamics and angiography were reported. A core laboratory performed standardized review of all angiograms. One-month follow-up was reported. RESULTS: A total of 158 patients (male = 65%; median age 19 years) underwent placement of CCPS. Eighty-three patients had pre-existing AWI. The average ascending-to-descending aorta systolic gradient improved from 27 ± 20 mm Hg to 4 ± 6 mm Hg. Complete coverage of pre-existing AWI was achieved in 66 of 71 patients (93%) with AWI who received a single CCPS. Ultimately, complete coverage of AWI was achieved in 76 of 83 patients (92%); 7 patients had minor endoleaks that did not require repeat intervention. Four patients experienced important access site vascular injury. There were no acute AWI, repeat interventions, or deaths. CONCLUSIONS: The CCPS can effectively treat and potentially prevent AWI associated with CoA. Access site arterial injury is the most common important complication. Longer-term follow-up is necessary to define mid- and late-term outcomes.

Coarctation of the aorta in adolescents and adults: A review of clinical features and CT imaging.

Coarctation of the aorta (CoA), while usually identified and treated in the neonatal/infant period, is increasingly seen in adults, either primarily or (more often) following repair. Imaging plays a crucial role in the diagnosis, therapeutic planning, and follow-up of patients with CoA. Clinical management of CoA in adults optimally involves a multidisciplinary team; accordingly, imagers should be familiar with the underlying pathology, associations, and management of CoA in addition to imaging protocoling and interpretation. We will review the relevant clinical and imaging features of CoA, with an emphasis on patients beyond childhood.

NuMED Covered Cheatham-Platinum Stent™ for the treatment or prevention of right ventricular outflow tract conduit disruption during transcatheter pulmonary valve replacement.

OBJECTIVE: Retrospectively assess the frequency of right ventricular outflow tract (RVOT) conduit disruption during transcatheter pulmonary valve replacement (TPVR) and the effectiveness and safety of NuMED Covered Cheatham-Platinum Stents™ (CCPS) for its prevention or treatment. BACKGROUND: There have been no published reports to date describing the safety and effectiveness of covered stents to prevent or treat RVOT conduit disruption during TPVR. METHODS: Data regarding 251 TPVR procedures performed at multiple US investigational sites were retrospectively reviewed to explore the incidence and potential predictors of conduit disruption. In addition, data on the use of 69 CCPS implanted in 50 patients during TPVR was reviewed. RESULTS: The overall incidence of conduit disruption requiring intervention was 6%. The only predictor identified was a very elevated RVOT conduit systolic pressure gradient. A pre-existing conduit tear was present in nine, while 31 developed tears after conduit dilation and three, after TPVR. The CCPS was used prophylactically in seven. Conduit tears were prevented or repaired in 49/50 patients. No CCPS-related acute complications were reported. At 6-month follow-up, no patient had more than mild pulmonary regurgitation and the mean Doppler RVOT gradient (12.7 ± 5.8 mm Hg) comparing favorably with that reported in the Melody TPV® IDE trial (20.0 ± 8.6 mm Hg). CONCLUSION: CCPS implantation can successfully treat RVOT conduit disruption without negative impact on the TPV function. This retrospective analysis suggests high RVOT conduit systolic pressure gradient is a risk factor for conduit tears during TPVR.

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

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

Acute outcome of stent therapy for coarctation of the aorta: results of the coarctation of the aorta stent trial.

OBJECTIVES: The coarctation of the aorta stent trial (COAST) is a pivotal trial of the NuMED Cheatham Platinum Stent(®) for treatment of coarctation of the aorta (CoA). BACKGROUND: CoA is a congenital obstruction to flow through the aorta. Stents have been used since the 1990s to treat CoA; none have been approved by the Food and Drug Administration for this indication. METHODS: In a prospective, multicenter study 105 patients received stents for treatment of CoA. Data was collected in the catheterization laboratory, on discharge and at 1-month postprocedure. RESULTS: Of 105 patients, 69% were male; 57% had native CoA. Noninvasive, baseline systolic blood pressure (SBP) showed upper extremity (UE) SBP to be 140 ± 16 mm Hg and UE to lower extremity (LE) SBP difference 29 ± 17 mm Hg. At catheterization, baseline ratio of minimum CoA diameter to diameter of descending aorta (CoA:DAo) was 0.46 ± 0.16. Implants (104/105) were successful with one stent migration. There were no deaths or serious complications. Paradoxical hypertension occurred in 6%. Four percent of patients experienced somewhat serious adverse events related to the procedure. All patients achieved relief of ascending aorta to Dao pressure gradient: mean = 2.0 ± 4.0 mm Hg (P < 0.001). The CoA:DAo increased to 0.84 ± 0.18. At 1-month, UE SBP was 120 ± 12 mm Hg, UE to LE SBP difference = -1 ± 12 mm Hg. Ninety-nine percent of patients had UE to LE SBP difference <20 mm Hg. CONCLUSIONS: Stenting of CoA, using the NuMED Cheatham Platinum Stent, is acutely safe and effective for treatment of CoA.

Percutaneous balloon-expandable covered stent implantation for treatment of traumatic aortic injury in children and adolescents.

Surgical treatment of pediatric acute traumatic aortic injury (TAI) after blunt chest trauma is standard of care. Use of endovascular stent grafts for treatment of TAI in adults is common but has important limitations in children. We sought to describe the use of balloon-expandable covered endovascular stents for treatment of TAI in children and adolescents. Participants of the multicenter Coarctation of the Aorta Stent Trial (COAST) had access to investigational large-diameter, balloon-expandable, covered stents (covered Cheatham-platinum stents; NuMed, Inc., Hopkinton, New York) on an emergency-use basis. From 2008 through 2011, 6 covered stents were implanted in 4 patients at 3 COAST centers for treatment of TAI. Median patient age was 13.5 years (range 11 to 14) and weight was 58 kg (40 to 130). All patients sustained severe extracardiac injuries that were judged to preclude safe open surgical repair of TAI. Median aortic isthmus and stent implantation balloon diameters were 16.4 mm (13.2 to 19) and 19 mm (16 to 20), respectively. Stent implantation was technically successful in all attempts. Complete exclusion of aortic wall injury was achieved in all cases. There were no access site complications. At a median follow-up of 24 months, there was 1 early death (related to underlying head trauma) and 1 patient with recurrent aortic aneurysm who required additional stent implantation. In conclusion, balloon-expandable covered-stent implantation for treatment of pediatric TAI after blunt trauma is generally safe and effective. Availability of this equipment may alter the standard approach to treatment of pediatric TAI.

Coarctation of the Aorta Stent Trial (COAST): study design and rationale.

BACKGROUND: Coarctation of the aorta (CoA) accounts for 4% to 5% of congenital cardiac abnormalities. Stent therapy has become an accepted alternative to surgery for older children and adults, although there are no balloon-expandable stents approved by the Food and Drug Administration for use in the aorta. The Cheatham-Platinum (CP) stent was designed for CoA therapy and is widely used outside the United States. We have designed the first prospective trial of stent therapy for CoA to serve as the pivotal trial for Food and Drug Administration approval of the CP stent. METHODS: The COAST study is a prospective, multicenter, single-arm clinical study. The population includes patients with native or recurrent CoA. Four primary outcome variables were defined. For each variable, the stent will be compared to performance guidelines derived from surgical experience. The first efficacy outcome is reduction in arm-leg systolic blood pressure gradient, and the second is reduction in hospital length of stay. Safety outcomes include the following: the occurrence of any serious or somewhat serious adverse event attributed to the stent or implantation procedure and the occurrence of postprocedure paradoxical hypertension. A total of 105 patients treated with the CP stent will be enrolled. DISCUSSION: To ascertain the effectiveness and safety of an interventional device, randomized controlled trials have been offered as the criterion standard. However, these trials are not well suited to study rare conditions such as CoA, especially once the therapy in question has received acceptance within the medical community. New clinical trial and statistical approaches are needed to evaluate such therapies. The COAST study is an example of this kind of innovative trial design.

The IMPACT registry: IMproving Pediatric and Adult Congenital Treatments.

Tremendous advances have occurred in catheter-based interventions for congenital heart disease. Multicenter trials of these advances are either out of date or have been limited in scope. As such little is known on the application of these techniques in the current era. The IMPACT Registry (IMproving Pediatric and Adult Congenital Treatments) will allow us to measure variability in the performance and outcomes of both diagnostic and interventional cardiac catheterization procedures in all children and adults with congenital heart disease. The IMPACT Registry will be harmonized with the Society of Thoracic Surgeons Congenital Heart Disease Database, thereby allowing us to compare catheter-based interventions with surgical interventions when appropriate. The initial release of the registry will only include hospital-based outcomes, but ultimately it will transition to a longitudinal registry. The IMPACT Registry will provide the necessary benchmark tools for quality improvement activities for cardiac catheterization procedures in congenital heart disease.

Laser recanalization of the subclavian vein.

We report the use of a long wire and introducer as a rail for the laser recanalization of a chronically occluded subclavian vein following extraction of a fractured permanent pacing lead. This allowed new pacing leads to be placed through the previously occluded vessel.