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Fontan fenestration decreases central venous pressure and preserves cardiac output while decreasing systemic oxygen saturation. Transcatheter fenestration closure increases oxygen saturation, though the persistence of this increase and the long-term incidence of adverse outcomes such as death and heart transplant remain unknown. We describe immediate and long-term clinical and adverse outcomes following fenestration closure. Catheterization, echocardiogram, and clinic reports were reviewed following transcatheter Fontan fenestration closure. Data were reported as n (%) and median (IQR). Continuous variables were compared using Wilcoxon ranked sum test. 51 patients had fenestration closure 0.9 (0.7-1.5) years following extracardiac Fontan operation. Most (84%) were closed with Amplatzer Septal Occluders. Systemic O 2 saturation immediately increased from 87 (83-89) to 95 (94-97)%, P < 0.05. Cardiac index decreased from 4 (3-5) to 2.9 (2.6-3.5) L/min/m 2 . Fontan pressure and pulmonary vascular resistance were not significantly changed. Clinical follow-up duration for all patients was 7.3 (range 1.3-16) years. Oxygen saturation at last follow-up was 94.5 (92-97)% and did not decrease over time (P < 0.05). One patient (2%) developed protein losing enteropathy, 1 (2%) had heart transplant, and 1 (2%) patient died 9.4 years following fenestration closure. No patient required fenestration re-creation following closure. Transcatheter Fontan fenestration closure leads to sustained increases in systemic oxygen saturation and a low incidence of adverse outcomes such as death and transplant. Further study comparing fenestration closure to non-closure and longer follow-up duration are required to determine if there is a survival benefit to fenestration closure., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS)., Background: Long-term data following BPV is limited to small, single center studies., Methods: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis., Results: Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention., Conclusion: Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV., (© 2019 Wiley Periodicals, Inc.)

Right ventricular outflow tract (RVOT) conduit stenosis remains a significant problem for patients with right ventricle-to-pulmonary artery (RV-to-PA) conduits placed as palliation for congenital heart disease. Previous reports on balloon dilation of RVOT conduits all describe small series with varying levels of success during limited follow-up evaluation. This study reviewed all patients with RV-to-PA conduits who underwent percutaneous balloon dilation for conduit stenosis at the authors' institution from 2000 to 2011. Patients with Carpentier-Edwards (CE) model 4300 porcine-valved conduits (Edwards Lifesciences Corp., Irvine, CA) (n = 19) were compared with patients who had all other types of conduits (n = 19). Successful balloon angioplasty was defined as a 20 % decrease in the RV-to-PA gradient, a 20 % decrease in the ratio of the RV systolic-to-aortic systolic pressure, or both. Balloon dilation was successful for 57.9 % of the patients with CE conduits and for 31.6 % of patients with other types of conduits (p = 0.10, Chi square test). Logistic regression analysis showed that balloon dilation was significantly more likely to be successful with CE valves than with other types (odds ratio [OR], 6.59; 95 % confidence interval [CI], 1.22-35.49). In a continuous series of patients with stenotic RV-to-PA conduits, the CE porcine-valved conduit was more amenable to percutaneous balloon dilation than other types of RV-to-PA conduits at the midterm follow-up evaluation. This has important ramifications in terms of valve selection for patients with congenital heart disease who will require surgical reintervention for RVOT stenosis.

Objectives: Certain congenital heart defects require the creation of an unrestrictive atrial septal defect (ASD) to relieve atrial hypertension, to maintain systemic cardiac output, or to achieve adequate atrial mixing to improve systemic oxygen saturation. We describe a series of patients, ranging in age from 5 weeks to 17 months, in whom we implanted a stent across the atrial septum to create a lasting, unrestrictive interatrial communication., Methods: Five patients with left heart obstructive defects presented with severe left atrial hypertension. The patients weighed 3.5-10.1 kg. Patients 1 and 2 presented at 5 and 9 weeks of age, respectively. Patient 1 had a restrictive patent foramen ovale, and patient 2 had an intact atrial septum. Patients 3, 4, and 5 had previous cardiac surgery including atrial septectomy. At presentation, all patients were poor surgical candidates., Results: Using standard percutaneous technique, a premounted Palmaz-Genesis stent (Cordis Corporation) was implanted across the atrial septum in all 5 patients, using balloons ranging from 7 to 10 mm diameter. Each patient had a dramatic decrease in left atrial pressure and increased ASD diameter. All stented ASDs remained widely patent, confirmed by Doppler echocardiography, until elective surgical stent explant., Conclusion: Patients presenting beyond the neonatal period with left heart obstructive defects and intact atrial septum or restrictive ASD present a challenging problem. We report a safe and effective technique for relief of left atrial hypertension in infants using premounted intravascular stents. All patients had immediate marked hemodynamic improvement.

Background: To date, cardiac catheterization and endomyocardial biopsy have been considered the "gold standard" for rejection surveillance after heart transplantation. Factors such as patient size (i.e., infant transplantation), loss of vascular access after repeated catheterizations, and anesthesia requirements all present unique problems and risks related to pediatric rejection surveillance. Therefore, additional methods to monitor for rejection in a non-invasive, reliable and frequent manner have been sought. We studied the utility of echocardiographic measurement of the left ventricular myocardial performance index (LVMPI), a reproducible measure of combined systolic and diastolic performance, in pediatric heart transplant recipients as a method of identifying acute rejection., Methods: Two-dimensional/Doppler echocardiographic studies (n = 36) were performed on 21 cardiac transplant patients (ages 6.2 to 21.9 years) at the time of endomyocardial biopsy. The LVMPI, the sum of the isovolumic contraction time and isovolumic relaxation time divided by aortic ejection time, was determined at each study, as well as other echocardiographic measures of systolic and diastolic function. Patients were grouped by concurrent histologic rejection grade and the results compared between groups., Results: Significant differences in LVMPI (p < 0.001) were noted between patients with no rejection (Grade 0; n = 23) and those with moderate to severe rejection (Grade 3; n = 5), as well as between those with no rejection and those with focal moderate (Grade 2; n = 8) rejection (p < 0.05). The LVMPI was 0.42 +/- 0.03 (mean +/- SEM) for the group without rejection, 0.57 +/- 0.06 for those with Grade 2 rejection and 0.73 +/- 0.05 for those with Grade 3 rejection. Although 9 of the 23 studies in the non-rejection group had LVMPI values exceeding 0.44 (upper threshold value), 12 of 13 patients in the rejection groups exceeded this threshold. LVMPI >or= 0.64 was associated with significant rejection in all cases in this study (n = 7). No significant differences were noted between groups for left ventricular ejection fraction or shortening fraction, percent septal or posterior wall thickening, left ventricular mass index or mitral valve deceleration time. In addition, for those individual patients with multiple studies, the LVMPI consistently increased with higher rejection grades and decreased after therapy., Conclusions: In this series, increased LVMPI correlated with biopsy-proven rejection, and frequent serial assessments using this technique may provide a relatively sensitive non-invasive means of rejection surveillance after pediatric cardiac transplantation. False positive results may be encountered, likely due to alterations in diastolic function that have been previously observed in transplant recipients.

Right aortic arch is a relatively rare congenital anomaly. The combination of a right aortic arch, a right patent ductus arteriosus, and an aberrant left subclavian artery in a child with an otherwise structurally normal heart is very uncommon. We report the successful transcatheter coil occlusion of a right-sided patent ductus arteriosus in a child with the above-mentioned anatomy. To our knowledge, such a case has not been reported in the English-language medical literature to date.

The objective of this study was to evaluate a new technique of sizing atrial septal defects (ASDs) for transcatheter device closure. ASD closure using the Amplatzer septal occluder (ASO) device is commonly performed. Complications, including arrhythmias, pericardial effusions, and perforations, may be related to oversizing ASDs and choosing larger devices. Two methods were used to size ASDs using a compliant balloon. In some patients, the balloon was inflated until a waist was visible [(+)waist]; in others, only until no shunting was demonstrable by echocardiogram [echo; (-)waist]. The device was selected and implanted using standard procedure and echo guidance. One hundred seventeen patients underwent secundum ASD closure with an ASO device. There were 43 patients in the (-)waist group and 74 in the (+)waist group. All devices were implanted successfully. The initial echo ASD diameter was larger in the (-)waist group compared to the (+)waist group (P = 0.01). There was a smaller difference between the initial echo and balloon-sized ASD diameters in the (-)waist group (P < 0.02). ASO device size implanted (in mm greater than echo ASD diameter) was smaller in the (-)waist group (P < 0.01). There were 0/43 complications in the (-)waist group and 5/74 in the (+)waist group. The complete closure rate was the same in both groups. Sizing an ASD by inflating a compliant balloon just until shunting is eliminated, and not until a waist is visible, results in less overstretching of the ASD and selection of a smaller ASO device, achieving similar closure rates and potentially fewer complications., (Copyright 2005 Wiley-Liss, Inc.)

In tetralogy of Fallot, severe cyanosis due to insufficient pulmonary blood flow necessitates early intervention. The Cutting Balloon, developed for percutaneous coronary angioplasty, was used successfully for transcatheter incision and dilation of infundibular and valvar pulmonary stenosis in four patients with tetralogy of Fallot., (Copyright 2005 Wiley-Liss, Inc.)

Background: Patients undergoing operative repair of aortic obstruction are at a lifelong risk of recurrent obstruction, and there is controversy regarding the optimal surgical technique. We have used an alternative strategy for recurrent aortic obstruction, typically involving anatomic reconstruction by means of a median sternotomy, and describe our techniques and results., Methods: Twenty-one patients presented with recurrent aortic arch obstruction. Mean age and weight were 7.8 +/- 5.4 years (range, 0.21 to 15.2 years) and 30.6 +/- 21.8 kg (range, 3.6 to 90 kg), respectively. Recurrence involved the aortic arch to some degree in each case, as the mean preoperative transverse aortic arch z score was -2.9 +/- 1.6 (range, -7.0 to 0.1). Thoracotomy was possible in 2 patients, using re-resection with end-to-end anastomosis (n = 1) and patch aortoplasty (n = 1). The remaining 19 patients required median sternotomy, cardiopulmonary bypass, and deep hypothermic circulatory arrest for complete relief of obstruction by aortic arch advancement (n = 10), patch aortoplasty (n = 8), or interposition grafting (n = 1)., Results: There was 1 hospital death. Invasive blood pressure monitoring revealed no residual arm-to-leg gradient in 19 patients and a 20-mm Hg gradient in 2 patients. There have been no late deaths. No patients have undergone subsequent aortic intervention, and all are asymptomatic up to 85 months postoperatively. Two patients are currently followed with a 10-mm Hg arm-to-leg blood pressure gradient., Conclusions: Anatomic reconstruction for recurrent aortic obstruction can be safely accomplished in the majority of patients. We favor median sternotomy because of the ability of establishing cardiopulmonary bypass, the facility of anatomic reconstruction techniques, and the ability to repair concomitant cardiovascular lesions.