CRISP: Catheterization RISk score for Pediatrics: A Report from the Congenital Cardiac Interventional Study Consortium (CCISC).

OBJECTIVES: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

Mid-Term Outcomes after Percutaneous Closure of the Secundum Atrial Septal Defect with the Figulla-Occlutech Device.

OBJECTIVES: To evaluate the mid-term outcomes after percutaneous closure of the secundum atrial septal defects (ASD) using the Figulla-Occlutech device (FOD). BACKGROUND: Transcatheter closure has become the method of choice for most patients with ASD. Although the FOD may have some advantageous characteristics there is a paucity of data on later outcomes after the use of this relatively new device. METHODS: Observational, single arm study including 200 non-consecutive patients who underwent ASD closure between 04/09 and 07/15 in 2 centers. Device performance, deployment technique, and immediate and mid-term outcomes were assessed. RESULTS: Median age and weight were 24 years (4-72) and 58 kg (15-92), respectively. Single defects were observed in 171 patients (median size of 19 mm). The remainder had multiple or multifenestrated defects. Implantation of FOD (median size of 24 mm) was successful in all (99%), but 2 patients (1 with deficient postero-inferior rim; 1 with a large ASD for the size of the child). Embolization with device retrieval occurred in 2 (1%). Median follow-up of 36 months was obtained in 172 patients. Serial echocardiographic assessment showed complete closure in all but 2 patients, in whom an additional small non-significant posterior defect was purposely left untouched. There have been no episodes of late arrhythmias, device embolization, cardiac erosion, endocarditis, thromboembolism, wire fracture, or death. CONCLUSIONS: Transcatheter closure of ASDs in older children, adolescents, and adults using the FOD was highly successful in a wide range of anatomical scenarios with high closure rates and no complications in mid-term follow-up.

Immediate and short-term outcomes after percutaneous atrial septal defect closure using the new nit-occlud ASD-R device.

OBJECTIVES: To evaluate the feasibility, safety, and efficacy of implantation of the new Nit Occlud ASD-R® (NOASD-R) device for percutaneous closure of ostium secundum atrial septal defects (ASD-OS). BACKGROUND: Device catheter implantation has become the method of choice for most patients with ASD-OS. No single device has proven to be ideal for this type of procedure. The NOASD-R has a distinct design that may help to overcome limitations of other devices. METHODS: A prospective, single arm, observational study including all consecutive patients receiving the NOASD-R device for ASD-OS closure between October 2011 and September 2013 was performed. Patient selection, device design, deployment technique, complications, and procedural outcomes were evaluated. RESULTS: Seventy-four patients underwent attempted transcatheter ASD-OS closure using the NOASD-R device. Implantation of the occluder was successful in 73 patients (98.6%). The majority of patients were female (79.5%) with a median age of 17.2 years (range: 2-74). A 2-D transthoracic color-Doppler echocardiogram (TTE) obtained at the 3 or 6 month follow-up visit showed complete occlusion of the ASD-OS in 72/73 patients (98.6%). At a mean follow-up interval of 11.4 ± 6.8 months there have been no episodes of late device embolization, cardiac perforation or erosion, endocarditis, thromboembolism, wire fracture, embolic neurologic events, or death. CONCLUSIONS: We report the first worldwide clinical experience using the NOASD-R device for ASD-OS closure. The procedure was feasible, with a high rate of successful implantations, and safe. High ASD-OS closure rates and no complications were encountered during short-term follow-up. © 2014 Wiley Periodicals, Inc.

New imaging modalities in the catheterization laboratory.

PURPOSE OF REVIEW: To provide an overview of the various modalities that have emerged in the last years as adjunctive tools to provide detailed imaging of the cardiovascular system in the catheterization laboratory. RECENT FINDINGS: Completion angiography after cardiac surgery for congenital heart disease using a dedicated hybrid operating cardiac suite may aid in early diagnosis and therapy of postsurgical abnormalities. The application of three-dimensional rotational digital angiography results in a more precise delineation of the three-dimensional anatomy of vascular structures outside the heart with rapid image processing and reduction of the amount of contrast used and ionizing radiation dose. Combined catheterization/magnetic resonance (MR) hybrid laboratories have the potential to accurately assess changes in the anatomy and physiology pretranscatheter and posttranscatheter interventions. Fully MR-guided transcatheter procedures have become possible in animal models. Real-time three-dimensional transesophageal echocardiography (TEE) has helped to guide transcatheter procedures in septal and valvar structures. Miniaturization of probes has expanded the applications of TEE for smaller neonates. The use of intravascular ultrasound has helped to better understand the mechanisms of vascular diseases and transcatheter treatments. SUMMARY: Knowledge and proper application of new imaging modalities during interventions in the catheterization laboratory may improve the outcomes of patients with congenital and acquired structural heart disease.

Coarctation of the aorta treated with the Advanta V12 large diameter stent: acute results.

OBJECTIVES: To report on the early results of treatment of coarctation of the aorta by dilation with a new polytetrafluoroethylene covered stent. BACKGROUND: Transcatheter dilation of aortic coarctation carries the risk of aneurysm or rupture. Covered stent implantation reduces this risk but requires a large delivery system. The Advanta V12 LD covered stent is premounted and requires a 9-11 Fr delivery system. METHODS: Covered stents on balloons of a diameter sufficient to anchor the stent in the coarctation were implanted using the smallest available delivery system. Secondary dilation with larger diameter balloons was performed until the pressure gradient was <20 mm Hg and the stent was opposed to the aortic wall. RESULTS: Twenty-five patients with aortic coarctation underwent stent implantation. Coarctation diameter increased from (6.3 + or - 3.5) mm to (14.4 + or - 2.3) mm (P < 0.0001). Peak pressure gradient decreased from (25.3 + or - 11.6) mm Hg to (2.5 + or - 3.0) mm Hg (P < 0.0001). The stent achieved the desired diameter in all cases. There were no complications. At short-term median follow-up of 4.9 months, all patients are alive and well with no evidence of recoarctation or aneurysm. CONCLUSIONS: These initial results show that the covered Advanta V12LD stent is safe and effective in the immediate treatment of coarctation of the aorta through a low profile delivery system of 8-11 Fr. Long term follow up is required.

Homocysteine concentrations and molecular analysis in patients with congenital heart defects.

BACKGROUND: Congenital heart defects are the result of incomplete heart development and, like many diseases, have been associated with high homocysteine concentration. METHODS: We evaluated homocysteine, folic acid and vitamin B(12) concentrations, and the mutations 677C>T and 1298A>C in MTHFR, 844ins68 in CBS and 2756A>G in MTR genes in 58 patients with congenital heart defects, 38 control subjects, and mothers of 49 patients and 26 controls. RESULTS: Control and patients presented normal range concentrations for homocysteine (7.66 +/- 3.16 microM and 6.95 +/- 3.12 microM, respectively), folic acid (8.31 +/- 3.00 ng/mL and 11.84 +/- 10.74 ng/mL) and vitamin B(12,) (613.56 +/- 307.57 pg/mL and 623.37 +/- 303.12 pg/mL), which did not differ among groups. For the mothers studied, homocysteine and vitamin B(12) concentrations also did not differ between groups. However, folic acid concentrations of mothers showed significant difference, the highest values being in the group of patients. No difference was found in allele frequencies among all groups studied. CONCLUSIONS: In the studied groups, high homocysteine seems not to be correlated with congenital heart defects, as well as folic acid and vitamin B(12). The mutations studied, in isolation, were not related to congenital heart defects, but high concentration of maternal homocysteine is associated with the presence of three or four mutated alleles.

Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries.

OBJECTIVES: This research was undertaken to assess the status of the coronary wall morphology late after the arterial switch operation (ASO) for transposition of the great arteries employing intravascular ultrasound (IVUS). BACKGROUND: Long-term patency of the reimplanted coronary arteries is a key issue after ASO. Follow-up studies have demonstrated coronary obstruction in up to 8% of patients that may be related to progressive fibrocellular intimal thickening. METHODS: Twenty-two asymptomatic children were enrolled at a median age of 9.5 years (range 5 to 22 years); IVUS images were obtained in 20 children at cardiac catheterization 5.0 to 21.6 years after the operation (in two cases IVUS study was not feasible due to technical constraints). Quantitative analysis was performed in 37 coronary arteries involving segments with a mean length of 28.4 +/- 1.8 mm. RESULTS: Thirty-three arteries (89%) displayed variable degrees of proximal eccentric intimal proliferation, with the maximal intimal thickening being 0.26 +/- 0.14 mm (range 0.06 to 0.71 mm) at the most thickened site. According to the Stanford classification, all children had coronary artery involvement with 50% having moderate-to-severe lesions (>0.3 mm). No risk factors for such abnormalities were encountered, including age, origin of the coronary arteries, hemodynamics, and follow-up duration after surgery. CONCLUSIONS: Intravascular ultrasound assessment late after the ASO revealed proximal eccentric intimal thickening in most of the studied vessels. This observation suggests the development of early atherosclerosis in the reimplanted coronary arteries, which may have a role in the genesis of late coronary events.

Serial assessment of arterial structure and function in patients with coarctation of the aorta undergoing stenting.

Stenting for CoA has become an acceptable treatment modality in the last 20 years. However little is known about arterial changes after this procedure. To assess arterial structure and function including peripheral reactivity and stiffness and intima-media thickness (IMT) pre and post stenting for coarctation of the aorta (CoA). Twenty-one patients [median age: 15 years (8-39)] were studied at baseline, 1 day, 6 months and 1 year after stenting. Twenty-one healthy subjects (1:1 matched) were used as controls. Left ventricular (LV) mass, ejection fraction, flow-mediated dilation (FMD) and nitrate-mediated dilation (NMD) of left brachial artery, common carotid (CC) and right subclavian artery (RSCA) IMT and pulse wave velocity (PWV) were assessed by echocardiography and vascular ultrasound. CoA patients had higher LV indexed mass (p < 0.0001), impaired FMD (p < 0.0001) and NMD (p < 0.0001), increased PWV (p < 0.0001), carotid and RSCA IMT (both p < 0.0001). All procedures were successful and resulted in significant gradient reduction (p < 0.001). One year after stenting there was improvement in LV function (p = 0.034) and although there was significant reduction of LV mass (103.29 ± 24.77 vs. 74.39 ± 22.07 g/m(2), p < 0.0001) values did not normalize. There was no significant change in FMD, NMD, PWV and CC or RSCA IMT. In patients with CoA, arterial reactivity is impaired and LV mass, arterial stiffness and thickness are increased. Although stenting is successful to relieve the obstruction resulting in better LV function and mass reduction, arterial structure and function remains abnormal after 1 year of follow-up.

Percutaneous stent implantation to stenotic bioprosthetic valves in the pulmonary position.

BACKGROUND: We evaluated stent implantation across stenotic bioprosthetic pulmonary valves in 9 patients. METHODS: Nine patients (6 male patients) underwent stent implantation across stenotic bioprosthetic pulmonary valves between July 1996 and July 1999 at the Hospital for Sick Children, Toronto. Catheter intervention was indicated if echocardiography revealed Doppler estimates of right ventricular pressure of more than two thirds of systemic arterial pressure (or systolic septal flattening with an estimated gradient of >60 mm Hg across the valve prosthesis). Catheterization was performed during general anesthesia at an age (mean +/- SD) of 9.3 +/- 3.5 years and a weight of 32.0 +/- 17.1 kg 5.9 +/- 1.8 years after surgical insertion of a bioprosthetic valve in the pulmonary position: 7 patients with tetralogy of Fallot, 1 patient with congenital pulmonary stenosis-insufficiency, and 1 patient after a Rastelli operation. All had systolic septal flattening and right ventricular dilatation with moderate-to-severe pulmonary insufficiency before intervention. Fluoroscopy times were 33.1 +/- 9.5 minutes. Seven patients received a single P4014 stent, and 2 received single P308 stents (Palmaz; Johnson & Johnson Interventional Systems, Warren, NJ) without significant complications. RESULTS: The right ventricular systemic pressure decreased acutely from 83% +/- 16% to 41% +/- 10% (P <.001, n = 9), and the transvalvular gradient decreased from 49.7 +/- 8.5 to 11.0 +/- 5.9 mm Hg (P <.001, n = 8). During the follow-up period (10.9 +/- 8.1 months, n = 8), 1 patient had an unsuccessful attempt at redilation of the stent (right ventricular pressure, 60% systemic) and underwent uneventful surgical pulmonary valve replacement. None of the remaining patients had echocardiographic evidence of systolic septal flattening, and right ventricular dimensions did not change significantly. CONCLUSION: Stent implantation is a safe and effective means of providing palliative relief of obstructed bioprosthetic valves in the pulmonary position and can safely delay the requirement for pulmonary valve replacement.

Transcatheter closure of perimembranous ventricular septal defects.

The isolated perimembranous ventricular septal defect is one of the most common congenital cardiac malformations. Although surgery has been performed and has a low associated risk, it still involves morbidity due to factors such as residual leaks, atrioventricular block, postpericardiotomy syndrome and arrhythmias. It has been speculated that percutaneous closure of these defects could minimize such complications. Recently, a device designed specifically for perimembranous ventricular septal defect closure, the Amplatzer membranous ventricular septal defect occluder, has been developed. Initial clinical experience with this device has been very encouraging with results showing a high rate of complete closure and a low incidence of complications at mid-term follow-up. In this article, the authors review their own and others' experience with this device.

Obliteration of a competitive forward flow from the ventricle after a bidirectional cavopulmonary shunt with an Amplatzer duct occluder.

A 7-month-old boy who was status post-bidirectional Glenn shunt implantation with residual ventricular flow to the pulmonary arteries (PA) presented with massive edema of the head and systemic desaturation soon after surgery. After test occlusion, an Amplatzer duct occluder was implanted at the PA banding site with subsequent decrease in the PA pressure, resolution of the edema and extubation.

Percutaneous occlusion of the patent ductus arteriosus with the amplatzer device for atrial septal defects.

We report two cases in which adult patients with large and short patent ductus arteriosus with shallow/no aortic ampulla and associated pulmonary hypertension had the defects successfully closed percutaneously using Amplatzer devices designed for atrial septal defect occlusion. The defects were stretched with a sizing balloon for optimal ductal size determination and device selection. Surgical treatment for associated discrete membranous subaortic stenosis was deferred in one patient because of significant gradient reduction in the left ventricular outflow tract after ductal occlusion. The technique of this novel approach and its possible advantages are discussed.

Transcatheter closure of secundum atrial septal defects with complex anatomy.

The aim of this study was to evaluate the feasibility, safety and efficacy of transcatheter closure of secundum atrial septal defects (ASD) in patients with complex anatomy. From September 1997 to July 2003, a total of 40 patients (median age, 34 years; 65% female) with complex ASDs, defined as the presence of a large defect (stretched diameter >26 mm) associated with a deficient rim (n=23); multiple defects (n=8); a multi-fenestrated septum (n=5); and defects associated with an aneurysmal septum irrespective of their size (n=4) underwent closure. The Helex device was used in 4 patients and the Amplatzer in the remaining. Two devices were implanted in 2 patients each. Implantation was unsuccessful in 5 patients, with 4 having large defects associated with a deficient anterior rim and a floppy posterior septum. Occlusion was observed in 22 of 35 patients (63%) immediately after implantation and in 31 (89%) at a mean follow-up of 18+/-9 months. No major complications occurred. Right ventricular end-diastolic dimensions (indexed for body surface area) decreased from 135+/-25% before closure to 124+/-15% 24 hours after closure, and to 92+/-12% after 12 months. Two patients with 2 distant defects and 2 patients with large defects remained with shunts (<4 mm) at the latest visit. Transcatheter closure of complex secundum ASDs was feasible, safe and effective; however, large defects associated with a deficient anterior rim and a floppy posterior septum may not be suitable for this approach.

A new use for the amplatzer duct occluder device.

We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel.

Initial experience in Brazil with the Helex septal occluder for percutaneous occlusion of atrial septal defects.

OBJECTIVE: To evaluate the initial clinical experience with the Helex septal occluder for percutaneous closure of atrial septal defects. METHODS: Ten patients underwent the procedure, 7 patients with ostium secundum atrial septal defects (ASD) with hemodynamic repercussions and 3 patients with pervious foramen ovale (PFO) and a history of stroke. Mean age was 33.8 years and mean weight was 55.4 kg. Mean diameter by transesophageal echocardiography and mean stretched ASD diameter were 11.33 3.3mm, and 15.2 3.8mm, respectively. The Qp/Qs ratio was 1.9 0.3 in patients with ASD. RESULTS: Eleven occluders were placed because a patient with 2 holes needed 2 devices. It was necessary to retrieve and replace 4 devices in 3 patients. We observed immediate residual shunt (< 2mm) in 4 patients with ASD, and in those with patent foramen ovale total occlusion of the defect occurred. No complications were noted, and all patients were discharged on the following day. After 1 month, 2 patients with ASD experienced trivial residual shunts (1mm). In 1 patient, we observed mild prolapse in the proximal disk in the right atrium, without consequences. CONCLUSION: The Helex septal occluder was safe and effective for occluding small to moderate atrial septal defects. Because the implantation technique is demanding, it requires specific training of the operator. Even so, small technical failures may occur in the beginning of the learning curve, but they do not involve patient safety.

Short- and midterm follow-up results of valvuloplasty with balloon catheter for congenital aortic stenosis.

OBJECTIVE: To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis. METHODS: Seventy-five patients (median age: 8 years) underwent the procedure through the retrograde femoral or carotid route. RESULTS: The procedure was completed in 74 patients (98.6%). The peak-to-peak systolic gradient dropped from 79.6 27.7 to 22.3 17.8 mmHg (P<0.001), the left ventricular systolic pressure dropped from 164 39.1 to 110 24.8 mmHg (P<0.001), and the left ventricular end diastolic pressure dropped from 13.3 5.5 to 8.5 8.3 mmHg (P< 0.01). Four patients (5.3%) died due to the procedure. Aortic regurgitation (AoR) appeared or worsened in 27/71 (38%) patients, and no immediate surgical intervention was required. A mean follow-up of 50 38 months was obtained in 37 patients. Restenosis and significant AoR were observed in 16.6% of the patients. The estimates for being restenosis-free and for having significant AoR in 90 months were 60% and 50%, respectively. CONCLUSION: Aortic valvuloplasty was considered the initial palliative method of choice in managing congenital aortic stenosis, with satisfactory short- and midterm results.

Incremental cost-effectiveness of percutaneous versus surgical closure of atrial septal defects in children under a public health system perspective in Brazil.

INTRODUCTION: Cost-effectiveness (CE) studies of percutaneous (PC) versus surgical (SC) atrial septal defect closure are lacking. METHODS: A systematic literature review in children and a CE analysis based on a model of long-term outcomes were performed. Direct costs of PC and SC were US$8700 (defined arbitrarily) and US$5700 (actually paid), respectively. Three-times the Brazilian GDI (US$28,700) per year of life saved (with a discount rate of 5%) was used as a limit for willingness-to-pay. DISCUSSION: PC had a high (US$104,500) incremental CE ratio despite lower complication rates, shorter hospital stay and better (nonsignificant) adjusted life expectancy. PC would be cost-effective if it cost US$6400 or SC had an 8% loss of utility or its indirect costs were US$2250. Costs of PC should be reduced to be cost-effective in the Brazilian public health system. Indirect costs and impact on quality of life should be further assessed.

Estado atual do tratamento dos defeitos do septo atrial / Current status of the treatment of atrial septal defects

A comunicação interatrial tipo ostium secundum (CIA OS) é uma cardiopatia congênita relativamente frequente, que causa repercussão hemodinâmica para o ventrículo direito, com sobrecarga volumétrica e dilatação da câmara. A maioria dos pacientes é assintomática, porém sintomas podem aparecer depois da segunda década de vida. O tratamento eletivo é usualmente realizado próximo ao quinto ano de vida, podendo ser indicado mais precocemente quando houver sintomatologia exuberante. O fechamento transcateter usando uma prótese de duplo disco é bem estabelecido como a primeira escolha de tratamento para a maioria dos pacientes de anatomia favorável. Foi comprovado que o forame oval patente (FOP) está associado à acidente vascular cerebral isquêmico (AVCI) em pacientes com poucos ou nenhum fator de risco de doença cardiovascular. Recentemente, foi demonstrado que a oclusão transcateter do FOP é superior ao tratamento clínico para prevenir recorrências de AVCI nessa população, em um estudo randomizado com acompanhamento clínico de longo prazo. Este manuscrito revisa as atuais indicações, critérios de seleção do paciente e o seguimento clínico dos pacientes com CIA OS e FOP submetidos ao tratamento transcateter

Ostium secundum atrial septal defect (OS-ASD) is a relatively frequent congenital heart defect that causes hemodynamic burden on the right ventricle with volume overload and chamber dilatation. Most patients are asymptomatic, however symptoms can appear after the second decade of life. Elective treatment is usually performed around the fifth year of life, and may be occasionally indicated earlier if there are exuberant symptoms. Transcatheter closure using a double disc device is well established as the first choice of treatment for most patients with suitable anatomy. Patent foramen ovale (PFO) has been shown to be associated with ischemic stroke in patients with no or limited risk factors for cardiovascular disease. It was recently demonstrated in a randomized trial with long-term follow-up that transcatheter closure of PFO is superior to medical treatment for preventing recurrences of stroke in this patient population. This manuscript reviews the current indications, patient selection criteria, and long-term follow-up in patients with OS-ASD and PFO submitted to transcatheter closure

Perventricular device closure of congenital muscular ventricular septal defects.

Muscular ventricular septal defects (MVSDs) account for approximately 20% of all congenital ventricular septal defects. Large defects in infants result in early heart failure, failure to thrive and pulmonary hypertension. Although percutaneous closure of MVSDs has been employed safely and effectively in children, adolescents and adults, its application in the small infant (weight <6 kg) carries a higher risk for complications including arrhythmias, hemodynamic compromise, cardiac perforation, tamponade and death. Perventricular closure of such defects, introduced by Amin and coworkers in the late 1990s, has become an attractive treatment modality for these small and high-risk patients. Experience worldwide has shown that the procedure is feasible, reproducible, safe and effective. In this article, the authors review the indications, the step-by-step technique and the results of perventricular closure of MVSDs using the AMPLATZER mVSD device (AGA Medical, MN, USA).