Transapical Delivery of a Sapien Valve for Transcatheter Aortic Valve Replacement in an 11-Year-Old Patient with Truncus Arteriosus.

Complex congenital heart defects may necessitate repeated surgical interventions throughout a patient's lifetime. Each subsequent procedure exposes patients to a greater cumulative risk, thus adding to the potential morbidity and mortality of the surgery. Transcatheter interventions can help mitigate the surgical risk for many defects and can delay or mitigate the need for surgery. This case report describes the rare use of a transapically delivered transcatheter aortic valve replacement (TAVR) therapy in a high-risk pediatric patient to postpone the need for surgery and potentially reduce the number of lifelong surgical interventions. The case highlights how transcatheter aortic valve therapies can be considered for non-standard, higher risk pediatric patients to postpone the need for surgical valve replacement and may serve as a paradigm shift in the care of complex patients with aortic valve pathology.

Standardizing radiation dose reporting in the pediatric cardiac catheterization laboratory-a multicenter study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database. BACKGROUND: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions. METHODS: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight. RESULTS: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 µGy m(2) /kg), followed by diagnostic (59, 105, and 175 µGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 µGy m(2) /kg). CONCLUSION: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.

Safety and utility of CardioMEMS device for remote pulmonary artery monitoring in paediatric Fontan patients: a case series.

Background: Patients with single ventricle congenital heart disease who undergo total cavo-pulmonary anastomosis (Fontan surgery) suffer from elevated pulmonary artery pressure (PAP), which leads to multiple adverse sequelae. Traditionally, the Fontan pressures are assessed via invasive haemodynamic catheterization that exposes these medically fragile patients to the risks of vascular injury and anaesthesia. While the CardioMEM remote PAP monitor has been extensively used in adults with heart failure, the safety of this device has not been established in children. Case summary: We report safety and utility of this device in eight paediatric Fontan patients. Our patients ranged from 9 to 18 years of age. There were no reported complications related to the implantation of the CardioMEMS device in our population. Discussion: This is the first case series of safety of CardioMEMS device in paediatric Fontan Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation patients. Our experience indicates that the device may be safely utilized for the management of Fontan-related complications in this vulnerable population.

Decreased incidence of right-ventricular outflow tract complications using a retrograde snare technique for radiofrequency pulmonary valve perforation.

Perforation of the atretic pulmonary valve with balloon dilation in infants with pulmonary atresia with intact ventricular septum (PA-IVS) is standard initial therapy for right-ventricular (RV) decompression. This procedure often results in adequate pulmonary blood flow, thus eliminating the need for neonatal surgery. Nonetheless, the incidence of RV outflow-tract complications and mortality for this intervention is significant. We report our experience with retrograde snare-guided radiofrequency (RF) perforation in an attempt to improve accuracy and decrease procedural complications. Medical records were reviewed for the period between March 2007 and May 2010 for all patients with PA-IVS who presented to the catheterization laboratory for attempted RF perforation in infancy. Specific details reviewed included demographics, preprocedural echocardiographic (echo) data, procedural technique and complications, pre- and post-RV pressures and pulmonary valve gradients, need for surgical intervention in the neonatal period, and short- to medium-term follow-up. Eleven neonates with PA-IVS underwent RF perforation using a retrograde snare-guided technique during the study period. The pulmonary valve was successfully perforated and the wire snared in all 11 patients. Six of 11 atretic valves were crossed on the first attempt with low energy (5 W × 2 s). No episodes of tamponade or RV/PA perforation occurred as confirmed by echocardiogram performed immediately after the procedure. There was no ductal spasm with retrograde catheter manipulations. Sequential dilation of the perforated valve was not necessary. RV pressures decreased from 169 % systemic before dilation to 93 % after dilation (p < 0.001) with a residual pulmonary valve gradient of 16 ± 6 mm Hg. Eight of 11 patients (73 %) were discharged without surgery at an average 16 days after the intervention. Three patients required Blalock-Taussig shunts to augment pulmonary blood flow. Femoral artery thrombus occurred in 4 of 11 patients treated with anticoagulation, and 2 patients had atrial arrhythmias during the procedure. No other complications occurred. No preprocedural echo parameters predicted requirement for surgical shunt before hospital discharge. Retrograde snare-guided pulmonary valve perforation in infants with PA-IVS is safe and may decrease the incidence of significant procedural complications.

Late catheter interventions in hypoplastic left heart syndrome.

Interventional cardiology plays a key role in the diagnosis and management of patients with functionally univentricular physiology after the various stages of surgical palliation. The interventions performed are widely variable in type, including angioplasty of stenotic vessels and implantation of stents in stenotic vessels; closure of defects such as collaterals, leaks in baffles, and fenestrations; creation of fenestration; and more. In the setting of venous hypertension associated with stenosis at the Fontan baffle, conduit, or pulmonary arteries, stent implantation is often preferred, as the aim is to eliminate completely the narrowing, given that relatively mild stenosis can have a significant detrimental hemodynamic effect in patients with functionally univentricular circulation. The procedure is highly successful. In patients who fail after Fontan procedure, creation of a fenestration is often performed, with variable technique depending on the underlying anatomic substrate. To increase chances of patency of the fenestration, implantation of a stent is often required, particularly in the setting of an extracardiac conduit. For those patients with cyanosis and favorable Fontan hemodynamics, closure of the fenestration is performed using atrial septal occluder devices with high success rate. Coils compatible with magnetic resonance imaging are used widely to treat collateral vessels, although on occasion other specific embolization tools are required, such as particles or vascular plugs. Postoperative arch obstruction is successfully managed with angioplasty at a younger age, while implantation of a stent in the aorta is reserved for older patients. Specifics of these interventional procedures as applied to the population of patients with functionally univentricular hearts are described in this manuscript.

Extracorporeal cardiopulmonary resuscitation for post-operative cardiac arrest: indications, techniques, controversies, and early results--what is known (and unknown).

Extracorporeal cardiopulmonary resuscitation may be defined as the use of extracorporeal membrane oxygenation for the support of patients who do not respond to conventional cardiopulmonary resuscitation. Data from national and international paediatric databases indicate that the use of extracorporeal cardiopulmonary resuscitation is increasing. Guidelines from the American Heart Association suggest that any patient with refractory cardiopulmonary resuscitation and potentially reversible causes of cardiac arrest is a candidate for extracorporeal cardiopulmonary resuscitation. One possible framework for selection of patients for extracorporeal cardiopulmonary resuscitation includes dividing patients on the basis of favourable or unfavourable characteristics. Favourable characteristics include cardiac disease, witnessed event in the intensive care unit, ability to deliver effective cardiopulmonary resuscitation, active patient monitoring present, favourable arterial blood gases, and early institution of extracorporeal membrane oxygenation. Unfavourable characteristics potentially include non-cardiac disease, an unwitnessed cardiac arrest, ineffective cardiopulmonary resuscitation, and severely acidotic arterial blood gases. Considering the significant resources and cost involved in the use of extracorporeal cardiopulmonary resuscitation, its use needs to be critically examined to improve outcomes, assess neurological recovery and quality of life, and help identify populations and other factors that may help guide in the selection of patients for successful extracorporeal cardiopulmonary resuscitation.

Intermediate-term outcomes after paediatric cardiac extracorporeal membrane oxygenation--what is known (and unknown).

The use of extracorporeal membrane oxygenation in infants and children with cardiac disease who develop refractory cardiogenic shock, cyanosis, or cardiac arrest is increasing. Early mortality in children with cardiac disease who require extracorporeal membrane oxygenation remains an important issue, as only 40% of cannulated patients survive to discharge from the hospital. However, it is encouraging that 90% children who are discharged alive from the hospital after extracorporeal membrane oxygenation are still alive at intermediate-term follow-up. Surviving patients are at risk for long-term dysfunction of multiple organ systems related to their underlying cardiac disease, non-cardiac comorbidities, treatment-related complications, and exposure to extracorporeal membrane oxygenation. Among the most important acute complications related to support with extracorporeal membrane oxygenation is injury to the central nervous system, which may contribute to adverse neurodevelopmental outcomes. All of these factors, in turn, influence quality of life. Many survivors remain medically complex related to their underlying cardiac disease, comorbidities, and sequelae of complications acquired over their lifetime. Neurological morbidity clearly plays an important role in approximately one-third of survivors, with significant deficits in approximately 10%. The limited data about quality of life data that are available for survivors of cardiac extracorporeal membrane oxygenation suggests that approximately 15-30% of survivors have at least moderately decreased quality of life. Overall, published data support the ongoing use of support with extracorporeal membrane oxygenation in children with acute cardiac failure, most of whom would die without it. However, programmatic efforts to improve the selection of patients and the preservation of the function of end organs during extracorporeal membrane oxygenation are clearly needed in order to improve long-term outcomes.

Adrenocortical response in infants undergoing cardiac surgery with cardiopulmonary bypass and circulatory arrest.

OBJECTIVE: To detail changes in adrenocorticotropic hormone (ACTH), cortisol, and aldosterone levels following cardiac surgery and to test the hypothesis that postcardiotomy infants requiring excessively high-dose vasopressor support will demonstrate adrenal insufficiency which will be proportional to cardiopulmonary bypass (CPB)/circulatory arrest times and vasopressor requirements. DESIGN: Prospective observational pilot study. SETTING: A tertiary care pediatric cardiac intensive care unit. PATIENTS: Prospectively enrolled infants were divided into three subgroups: CPB, CPB with deep hypothermic circulatory arrest (DHCA), and control subjects. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A representative patient sample from each surgical group underwent preoperative synthetic ACTH testing. Postoperative serum samples for cortisol, ACTH, and inotrope score (IS) were collected at discrete intervals over 48 hrs along with patient demographics, surgical procedure, and CPB/DHCA times. Fifty-eight patients were classified by subgroup: 31 CPB, 22 DHCA, and 5 controls. Ten patients with DHCA, analyzed separately, received intraoperative steroids. Tested patients demonstrated preoperative adrenal competence. Cortisol peaked within 2 hrs of surgery without differences among groups. ACTH inversely correlated with bypass time in patients with DHCA (p = .03) but not with circulatory arrest time. Peak cortisol level did not correlate with simultaneous IS. Although not noted in any DHCA-steroid patients, nine patients had increased ACTH/cortisol ratios in association with elevated ISs suggesting inadequate adrenal responsiveness to endogenous ACTH. CONCLUSIONS: The majority of infants with congenital heart disease and intact hypothalamic-pituitary-adrenal axes demonstrated an appropriate adrenocortical stress response to cardiac surgery. Peak serum cortisol was unrelated to CPB/DHCA time and did not predict the level of inotrope support. However, a subset of patients with elevated ACTH/cortisol ratios seemed to have a clinical status consistent with adrenal insufficiency and may be a target group for early postoperative steroid therapy.

Challenging Case of Percutaneous Closure of Right Coronary Artery to Right Atrial Fistula.

We demonstrate the percutaneous closure of a coronary fistula and subsequent utilization of a vascular plug in a patient with challenging anatomy.

Percutaneous Closure of a "Doughnut Leak" Around an Atrial Septal Occluder Device in a Patient With Recurrent Migraines.

A 10-year-old girl with pulmonary atresia status post transannular patch repair and secundum atrial septal defect (ASD) underwent percutaneous ASD closure 5 years earlier. There was improvement in arterial saturation from 86% to 98% with minimal residual right-to-left shunt. Despite subsequent medical therapy, she developed recurrent migraines, which led us to pursue percutaneous closure of the shunt.

Melody Transcatheter Pulmonary Valve Placement in a HeartMate II Patient.

Transcatheter pulmonary valve placement is emerging as a standard therapy for dysfunctional right ventricular outflow tract conduits. The Melody transcatheter pulmonary valve is indicated for use in the management of pediatric and adult patients with right ventricular outflow tract conduits measuring at least 16 mm in diameter. This is the first reported case of placement in a patient with a left ventricular assist device. We outline the preprocedural evaluation process, the procedural methods, and the outcomes of a successful implantation in a complex patient. With a team-based approach including thoughtful preprocedural evaluation, and close monitoring, successful deployment of a transcatheter pulmonary valve is possible in complex patients in the setting of mechanical circulatory support.

Management of adults with coarctation of aorta.

Coarctation of the aorta (CoA) is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose. The hallmark finding on physical examination is upper extremity hypertension, and for this reason, CoA should be considered in any young hypertensive patient, justifying measurement of lower extremity blood pressure at least once in these individuals. The presence of a significant pressure gradient between the arms and legs is highly suggestive of the diagnosis. Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications. Surgical repair has traditionally been the mainstay of therapy for correction, although advances in endovascular technology with covered stents or stent grafts permit nonsurgical approaches for the management of older children and adults with native CoA and complications. Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease, which, remains the greatest cause of long-term mortality. Thus, blood pressure control and periodic reassessment with transthoracic echocardiography and three-dimensional imaging (computed tomography or cardiac magnetic resonance) for should be performed regularly as cardiovascular complications may occur decades after the intervention.

Relation of left ventricular diastolic function as measured by echocardiography and pulmonary capillary wedge pressure to rejection in young patients (< or = 31 years) after heart transplantation.

To investigate the association of catheter-derived and newer echocardiographically derived measures of diastolic function with rejection in heart transplant recipients, 48 transplant recipients had Doppler and Doppler tissue imaging assessment along with catheter-measured pulmonary capillary wedge pressure (PCWP) at the time of endomyocardial biopsy. Of echocardiographic measures, propagation velocity (Vp), mitral E-wave velocity (E)/Vp, and E/annular mitral E-wave velocity (Em) were significantly associated with rejection (p <0.02), and an elevated PCWP was associated with rejection (p = 0.023) but with poor sensitivity. Weak but insignificant correlations with PCWP were found for E/Vp (r = 0.28) and E/Em (r = 0.37).

Late migration of a Sideris buttoned device for occlusion of atrial septal defect.

We describe a 17-year-old patient with an atrial septal defect who underwent device closure with a second generation Sideris buttoned device at 4 years of age. She presented 13 years after the procedure with the acute onset of chest discomfort, at which time a chest radiograph showed migration of the wire of the right atrial counter-occluder to the lateral aspect of the right atrial wall, with an associated right atrial perforation. The patient underwent uneventful surgical device retrieval and atrial septal defect closure. Late migration of a portion of the Sideris buttoned device can occur, suggesting the need for continued follow up, and a high index of suspicion for device failure should a patient become symptomatic.

Decompression of the left atrium during extracorporeal membrane oxygenation using a transseptal cannula incorporated into the circuit.

OBJECTIVES: When extracorporeal membrane oxygenation (ECMO) is used in the setting of severe myocardial dysfunction, left ventricular end-diastolic and left atrial pressure can rise to extremely high levels. Decompression of the left atrium in this setting is essential for resolution of pulmonary edema and recovery of left ventricular function. We sought to evaluate whether adequate left atrial decompression can be achieved via percutaneous placement of a transseptal left atrial drain incorporated in the ECMO venous circuit. DESIGN: Retrospective case series. SETTING: Tertiary care center pediatric intensive care unit and cardiac catheterization laboratory. PATIENTS: Seven patients (age 8 months to 28 yrs) with cardiac failure on venoarterial ECMO with left atrial hypertension. INTERVENTIONS: All patients underwent left atrial decompression with transseptal puncture and placement of a drain (8- to 15-Fr) incorporated into the ECMO venous circuit. Catheterization and ECMO records and echocardiograms were reviewed, as were the clinical course and outcome for each patient. MEASUREMENTS AND MAIN RESULTS: The median time from ECMO cannulation to left atrial decompression was 11 hrs. Average initial left atrial pressure was 31 mm Hg. Successful drain placement was achieved in seven patients with no major procedural complications. Echocardiographic improvement in left atrial dilation was achieved in five patients (71%). Inability to decompress the left atrium was fatal in two patients. Four patients were decannulated (57%), and three survived to hospital discharge (43%). Larger sheath size and higher flow rate correlated with a greater likelihood of success. CONCLUSIONS: Adequate decompression of the left atrium can be achieved by transseptal placement of a left atrial drain incorporated into the ECMO circuit. This technique represents a reasonable alternative to blade or balloon atrial septostomy for patients requiring left atrial decompression.

Catheter-based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective.

Infants with hypoplastic left heart syndrome (HLHS) and restrictive or intact atrial septum (rAS) present with cyanosis, pulmonary edema, and are critically ill. A previous report from our institution on emergent Norwood for HLHS with rAS showed 10% survival. We hypothesized that transcatheter left atrial (LA) decompression in HLHS with rAS would safely and effectively relieve LA hypertension, improve oxygenation, and improve Norwood survival. Between 1996 and 2004, 30 patients with HLHS and rAS underwent cardiac catheterization for pre-Norwood intervention. Twenty-eight atrial septostomies were performed: 23 static balloon dilations, 4 Rashkind septostomies, and 1 intra-atrial stent. Two procedures were aborted due to perforation (n = 1) or inability to enter the LA (n = 1). Eight total patients required surgical septectomy, for a failure rate of 27%. There were no catheter-related mortalities, although two patients died within 36 hr of the procedure after surgical septectomy. Major complications occurred in three patients (10%)--atrial perforations requiring intervention. Mean atrial septal defect gradient fell from 16.7 +/- 4.9 to 6.3 +/- 3.4 mm Hg (P < 0.001; n = 18). Mean LA pressure dropped from 21.8 +/- 5.5 to 13.1 +/- 6.5 mm Hg (P < 0.001; n = 16). Mean PaO(2) rose from 29.5 +/- 9.1 to 36.5 +/- 5.1 torr (P < 0.001; n = 23). Seventeen of 30 patients (57%) survived to discharge from Norwood. Thirteen have undergone hemi-Fontan and nine Fontan. Sixteen of 22 successful decompressions (73%) survived to discharge. Transcatheter decompression of the LA for patients with HLHS and rAS can be performed safely, reduces the transatrial gradient, and improves oxygenation. Catheter intervention improves survival compared to historical controls undergoing emergent Norwood.

Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group.

BACKGROUND/PURPOSE: Patients with congenital diaphragmatic hernia (CDH) are known to have associated cardiac anomalies. Data from the Congenital Diaphragmatic Hernia Study Group has allowed better definition of the types of heart defects (HDs) and survival for these patients. METHODS: Since 1995, 2636 patients were enrolled in the Congenital Diaphragmatic Hernia Study Group from 82 centers. Patients with hemodynamically significant HD, excluding patent ductus arteriosus (PDA); patent foramen ovale (PFO); and atrial septal defect (ASD), were selected. Cardiac anatomy and survival data for all patients were reviewed. RESULTS: Two hundred eighty (10.6%) patients had significant HDs: ventricular septal defect (VSD); (42.2%), aortic arch obstruction (15%), univentricular anatomy (13.9%), tetralogy of Fallot variants (11.1%), total anomalous pulmonary venous return (3.9%), double outlet right ventricle (RV) (3.2%), pulmonary stenosis (2.5%), transposition of the great arteries (2.5%), and various other defects in 5.7%. Survival for the entire group was 67.1%; survival for patients without HD was 70.2% and for patients with HD was 41.1% (P < .001). Patients with biventricular cardiac anatomy had a 47% survival, whereas those with univentricular anatomy had a 5% survival (P < .001). CONCLUSION: Significant HD is associated with 10.6% of CDH. Survival for patients with HD is significantly lower than for patients with normal cardiac anatomy. Patients with CDH and univentricular cardiac anatomy have a poor prognosis.

Transcatheter occlusion of aortopulmonary shunts during single-ventricle surgical palliation.

Development of aortopulmonary collaterals during the course of surgical palliation for single-ventricular anatomy has been linked to adverse outcomes following Fontan palliation. We investigated the hemodynamic significance of aortopulmonary collaterals during presurgical cardiac catheterization of patients with single-ventricle surgically palliated anatomy. Thermal indicator dilution studies were performed to determine degree of shunt. A total of 52 patients were studied and the data were analyzed. Measurements by thermal indicator dilution correlated significantly with qualitative angiographic grading of aortopulmonary collaterals. However, the hemodynamic significance of these aortopulmonary collaterals, as measured by thermal indicator dilution, did not correlate with postoperative outcome variables. This study demonstrated that thermal indicator dilution measurement holds promise for hemodynamically quantifying the significance of aortopulmonary shunts in surgically palliated single-ventricular patients. However, the degree of recirculation from aortopulmonary collaterals does not correlate with outcome after Fontan surgery in this patient cohort.