Correlations Between Echocardiographic Systolic and Diastolic Function with Cardiac Catheterization in Biventricular Congenital Heart Patients.

Newer echocardiographic techniques may allow for more accurate assessment of left ventricular (LV) function. Adult studies have correlated these echocardiographic measurements with invasive data, but minimal data exist in the pediatric congenital heart population. Purpose of this study was to evaluate which echocardiographic measurements correlated best with LV systolic and diastolic catheterization parameters. Patients with two-ventricle physiology who underwent simultaneous echocardiogram and cardiac catheterization were included. Images were obtained in the four-chamber view. LV systolic echocardiographic data included ejection fraction, displacement, tissue Doppler imaging (TDI) s' wave, global longitudinal strain, and strain rate (SR) s' wave. Diastolic echocardiographic data included mitral E and A waves, TDI e' and a' waves, and SRe' and SRa' waves. E/TDI e', TDI e'/TDI a', E/SRe', and SRe'/SRa' ratios were also calculated. Catheterization dP/dt was used as a marker for systolic function, and LV end-diastolic pressure (EDP) was used as a marker for diastolic function. Correlations of the echocardiographic and catheterization values were performed using Pearson correlation. Twenty-nine patients were included (14 females, 15 males). Median age at catheterization was 3.4 years (0.04-17.4 years). dP/dt was 1258 ± 353 mmHg/s, and LVEDP was 10.8 ± 2.4 mmHg. There were no significant correlations between catheterization dP/dt and systolic echocardiographic parameters. LVEDP correlated significantly with SRe' (r = -0.4, p = 0.03), SRa' (r = -0.4, p = 0.03), and E/SRe' (r = 0.5, p = 0.004). In pediatric congenital heart patients, catheterization dP/dt did not correlate with echocardiographic measurements of LV systolic function. Further studies are needed to determine which echocardiographic parameter best describes LV systolic function in this population. Strain rate analysis significantly correlated with LVEDP. Strain rate analysis should be considered as an alternative method to estimate LVEDP in this patient population.

Teaching the "hybrid approach": a novel swine model of muscular ventricular septal defect.

This report describes a reproducible swine model for creating muscular ventricular septal defects (VSDs). The model not only facilitates the development and modification of hybrid techniques for closing muscular VSDs, but also serves as a teaching tool that allows operators to become accustomed to the specific technical requirements necessary when using the hybrid approach to perform periventricular VSD device closure. The authors' institutional experience using this novel animal model is presented.

Evaluation of the AMPLATZER vascular plug for embolization of peripheral vascular malformations associated with congenital heart disease.

OBJECTIVE: The purpose of this study is to evaluate the recently FDA-approved AMPLATZER Vascular Plug in the embolization of vascular lesions associated with congenital heart disease (CHD). BACKGROUND: Fistulas and arteriovenous malformations have been occluded using various devices. The AMPLATZER Vascular Plug is a self-expandable, cylindrical device, used for embolization in the peripheral vasculature. METHODS: A total of 84 vessels in 52 patients with CHD from 11 centers were occluded with 89 AMPLATZER Vascular Plugs, delivered through a coronary guide catheter in various vascular sites, including collaterals, pulmonary arterio-venous and coronary artery fistulas, transhepatic tracts, central shunts, patent ductus arteriosus (PDA), and excluded hepatic vein. Complete vessel occlusion was demonstrated within 10 min in 94% of patients. RESULTS: There was no device embolization, vascular disruption, or procedure-related complication. One vascular plug implanted in a large type C PDA required surgical removal followed by PDA ligation, after 5 weeks from successful implant because of significant residual flow through the device. CONCLUSIONS: The AMPLATZER Vascular Plug is an effective transcatheter occlusion device in the embolization of a wide variety of vascular lesions associated with CHD. Based on our early experience, caution should be used when considering the Vascular Plug as a closure device for large PDA.

Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome.

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.

Multicenter experience with perventricular device closure of muscular ventricular septal defects.

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure. Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.

Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome.

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.

Stenting of coarctation of the aorta.

The purpose of the present study was to assess the usefulness of balloon expandable Palmaz intravascular stents in the transcatheter treatment of children and young adults with native and recurrent coarctation of the aorta, and to develop an improved intravascular stent and balloon delivery catheter specifically developed for vascular obstructions associated with congenital heart disease, including coarctation of the aorta. Twenty-one patients, 8 native and 13 recurrent coarctations, were successfully treated with the Palmaz stent. However, limitations and disadvantages in stent design and the single balloon delivery system were uncovered. Therefore, the NuMED CP stent and BIB delivery catheter were developed and used to treat 25 patients with native (17) and recurrent (8) coarctation successfully. Improvements in stent design and long-term follow-up using three-dimensional spiral CT scan will be helpful in determining the role of transcatheter stent therapy for native and recurrent coarctation of the aorta. Cathet Cardiovasc Intervent 2001;54:112-125.

Outcomes of transcatheter balloon angioplasty of obstruction in the neo-aortic arch after the Norwood operation.

Obstruction of the reconstructed aortic arch, or the neoaortic arch, is now known to be an important factor increasing mortality after the Norwood operation for hypoplastic left heart syndrome. Transcatheter balloon angioplasty has been shown to provide effective relief of both native aortic coarctation and obstructions of the aortic arch occurring subsequent to therapeutic intervention. We sought to determine the outcomes of balloon angioplasty used as an initial treatment for obstruction of the neoaortic arch occurring after the Norwood operation. We gathered the characteristics of 58 patients with such obstruction from 8 institutions, noting procedural factors and outcomes of initial balloon dilation. Obstruction occurred at a median interval of 4 months, with a range from 1.5 months to 6.3 years, after a Norwood operation. Ventricular dysfunction was present before dilation in 13 patients. Mean peak to peak systolic pressure gradients were acutely reduced from 31+/-20 mm Hg to 6+/-9 mmHg (p<0.001), with outcome subjectively judged to be successful in 89%. Three patients with pre-existing ventricular dysfunction died within 48 hours of dilation. There were 10 additional deaths during the period of follow-up, with Kaplan Meier estimates of survival after intervention of 87% at 1 month, 77% at 12 months, and 72% after 15 months. In addition, 9 patients required re-intervention during the period of follow-up, with Kaplan Meier estimates of freedom from re-intervention after dilation of 87% at 6 months, 78% at 12 months and 74% after 18 months. Although transcatheter dilation of neoaortic arch obstructions after Norwood operation is successful, there is a high risk of re-intervention and ongoing mortality in this subgroup of patients. Close follow-up is recommended.

Results of the phase I food and drug administration clinical trial of duct-occlud device occlusion of patent ductus arteriosus.

The purpose of this study was to evaluate the safety and the efficacy of transcatheter patent ductus arteriosus (PDA) occlusion with the Duct-Occlud device. Six centers participated in this phase I U.S. Food and Drug Administration-approved clinical trial with investigational device exemption. Patients with restrictive PDA (less than 4-mm minimum diameter) were eligible for the study. Sixty-two patients were enrolled. Forty-eight patients had successful implantation of Duct-Occlud devices. Patient follow-up evaluations were conducted at hospital discharge and after 2 and 12 months. At discharge and after 1 year, all patients had clinical PDA closure. Closure assessed by color flow Doppler was 55% at discharge, 88% at 2 months, and 94% at 1 year. There were no complications related to implantation or noted in follow-up evaluations. The Duct-Occlud device is safe and efficacious for the closure of small- to moderate-size patent ductus arteriosus.

Intervention in the critically ill neonate and infant with hypoplastic left heart syndrome and intact atrial septum.

Neonates that present with hypoplastic left heart syndrome (HLHS) and intact atrial septum (IAS) pose a major management problem for the pediatric cardiac team. They are critically ill newborns with profound hypoxemia and acidosis that require immediate attention. Controversy exists as to the most appropriate management strategy. In one series where a primary and emergent surgical-staged reconstructive procedure was performed, the in-house hospital mortality was 65% and the overall survival was 17%. With equal abysmal results, transcatheter creation of an atrial septal defect (ASD) using conventional balloon atrial septostomy (BAS) with or without the combination of blade atrial septotomy had an unacceptable high risk of cardiac perforation leading to tamponade and death. However, using more modern transcatheter techniques of transseptal perforation of the atrial septum followed by progressive and serial balloon septoplasty, creating an ASD, significantly reduced the risk of the procedure. In one series, 16 consecutive neonates underwent this type of interventional procedure without procedural mortality. The management strategy of creating an ASD in the catheterization lab followed by Stage I reconstructive surgical repair 3-5 days after the initial catheterization procedure improved the in-house survival to 57%. Unfortunately, there continues to be significant attrition of these patients undergoing Stage II and III reconstructive repair, which supports cardiac transplantation as an alternative strategy. There have been echocardiographic and histopathologic studies of these neonates, and an important echo classification of left atrial morphology has been described with perhaps some prognostic implication. In addition, autopsy specimens have demonstrated significant "arterialization" of the pulmonary venous architecture that likely dooms the patient with single ventricle physiology to a poor outcome. Future improvement in transcatheter techniques and materials offer promise in palliating these critically ill neonates. The concept of radiofrequency energy perforating catheters has great merit and may reduce the risk of cardiac perforation as compared with the rigid and long transseptal needle. Echocardiographic imaging at the time of entry through the IAS may improve the safety as well. The novel concepts of "butterfly" or "dog-bone" stents placed across the atrial septum creates a precisely sized ASD that may be more conducive to effectively lower left atrial hypertension, yet avoids excessive pulmonary blood flow associated with large atrial communications. In addition, new materials, such as the Cutting Balloon Catheter, may offer promise in creating ASDs in these patients. A more aggressive approach would be to consider intrauterine fetal transcatheter opening of the IAS using modified techniques that have been attempted for left ventricular outflow tract obstruction. Unfortunately to date, the results of attempted relief of aortic valve stenosis have been extremely poor. Finally, we as interventionalists need to continue to improve our skills to help in the complex management of these critically ill neonates and infants. Only through continued efforts of the entire cardiac team of intensivists, cardiologists, cardiothoracic surgeons, and interventionalists will our management strategy be defined to maximize the future outcome in this group of patients.

Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions.

OBJECTIVE: To compare neurodevelopmental outcome (NDO) in patients with hypoplastic left heart syndrome (HLHS), other functional single ventricle lesions, and the standard population and to investigate predictors of NDO in the population of children with functional single ventricle (FSV). STUDY DESIGN: A time- and age-defined cohort of patients with the Fontan circulation was recruited to participate in neurodevelopmental testing, behavioral evaluation, and imaging of the central nervous system. The Wechsler Intelligence test was the primary measure of NDO. Analysis included comparison of patients with HLHS with other patients with functional single ventricles. Other potential clinical predictors of NDO were investigated. RESULTS: The mean Full Scale Wechsler Intelligence score was 101.4+/-5.4. For the HLHS subgroup the mean Full Scale Wechsler score was 93.8+/-7.3, and for the non-HLHS subgroup it was 107.0+/-7.0. Although the HLHS group had significantly lower scores than the non-HLHS subgroup, neither subgroup scored significantly different from the standard population on the Wechsler Scales. Socioeconomic status, circulatory arrest, and perioperative seizures also were predictive of neurodevelopmental outcome. CONCLUSION: Neurodevelopmental and behavioral outcome in patients who have undergone the Fontan procedure including patients with HLHS is good in the preschool and early school years, with Wechsler Intelligence scores generally in the normal range.

Primary transcatheter treatment of congenital pulmonary arteriovenous malformation causing cyanosis of the newborn.

We report on successful transcatheter therapy of congenital pulmonary arteriovenous malformation resolving persistent cyanosis and obviating the need for surgical pneumonectomy or lobectomy.

CT angiography of complications in pediatric patients treated with intravascular stents.

OBJECTIVE: Our goal was to determine whether CT angiography can reveal complications in pediatric patients and young adults treated with intravascular stents for obstructive vascular lesions. CONCLUSION: CT angiography can reveal complications in pediatric patients treated with intravascular stents for obstructive lesions. Potentially, CT angiography could replace the more invasive conventional angiography currently used for intravascular stent placement and follow-up examinations.

Percutaneous translumbar cardiac catheterization and central venous line insertion: an alternative approach in children with congenital heart disease.

Children with congenital heart disease present major problems with venous access, eliminating conventional routes for cardiac catheterization. Although the transhepatic approach has recently gained popularity, we describe here an alternative approach using percutaneous translumbar approach for cardiac catheterization and/or in-dwelling central line insertion in three children with congenital heart disease. Diagnostic hemodynamic studies, transcatheter delivery of an intravascular stent for left pulmonary artery (LPA) stenosis, and chronic central venous line insertion were performed using this technique. Disadvantages include interventionalist's unfamiliarity with technique, awkward patient positioning, technically more difficult than transhepatic, and potential injury to kidney and bowel. Advantages include avoidance of vascular-rich hepatic parenchyma, thus reducing risk of hemorrhage; providing an alternative where transhepatic entry may be contraindicated; avoidance of bile duct, portal vein, and hepatic artery injury; and providing another alternative for not only transvenous, but also transarterial access that may be required for intravascular aortic stent delivery. The interventional radiologist should be utilized as a valuable resource to the cardiologist to help teach and supervise this technique in selected infants and children with limited vascular access.

Successful transcatheter perforation of the atretic pulmonary valve membrane in a newborn using the new Coe radiofrequency end hole catheter.

The new 2 French Coe radiofrequency (RF) end hole catheter was first used to successfully perforate the atretic pulmonary valve membrane using an antegrade approach in a newborn with intact ventricular septum (IVS). Nine watts of energy for 8 sec was required with simultaneous delivery of a 0.014 in. coronary guidewire coaxially through the end hole RF catheter for balloon valvuloplasty. This new ringed-tip end hole RF catheter offers considerable advantages to the pediatric interventionalist in the transcatheter therapy in neonates with pulmonary atresia (PA) and IVS.