The Use and Outcomes of Small,Medium and Large Premounted Stents in Pediatric and Congenital Heart Disease

We sought to describe the use and outcomes of small, medium and large premounted stents in patients with congenital heart disease, including incidence of and risk factors for re-intervention and development of in-stent stenosis. Premounted stents offer several advantages over traditional manually crimped bare-metal stents, especially in small patients. There are no data describing the medium-term effectiveness and outcomes of premounted stents in congenital heart disease. We performed a single-center retrospective review of all small, medium and large premounted stents implanted over an 8-year period. Premounted stents were implanted in 71 vessels within 59 patients with a 97 % success rate. Regardless of implantation site, premounted stent implantation resulted in significant improvements in vessel size and pressure gradient (p < 0.0001). Over a median follow-up duration of 3.1 years [interquartile range (IQR) 1.3–5.2], 25 patients (43 %) required re-intervention (18 catheter based, 3 surgical and 4 hybrid) at a median time of 1.4 years (IQR 0.9–3.0) from implant. Factors associated with re-intervention included age ≤1.9 years [hazard ratio (HR) 2.4, p = 0.03], weight ≤11.5 kg (HR 2.5, p = 0.03) and bare-metal premounted stents compared to covered premounted stents (HR 4.2, p = 0.001). In-stent stenosis was diagnosed in 42 % of premounted stents which underwent follow-up angiography. No identified characteristics were associated with in-stent stenosis, including “oversizing” the premounted stent. Small, medium and large premounted stents are effective in treating vascular stenosis in congenital heart disease. Frequent follow-up is required, especially in smaller patients with expected somatic growth, and to evaluate for in-stent stenosis which occurs in nearly half of premounted stents.     

Implanted Defibrillators in Young Hypertrophic Cardiomyopathy Patients: A Multicenter Study

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, with an annual risk of sudden cardiac death (SCD) estimated at 1 %. Limited data are available regarding both the risk of SCD in the young HCM population and the use of implantable cardioverter-defibrillators (ICDs). This retrospective study included all patients with HCM who underwent ICD implantation for primary or secondary prevention of SCD before the age of 30 years at five institutions between 1995 and 2009. There were 99 devices implanted in 73 patients. Appropriate shocks occurred for 11 % of all the patients. None of the previously identified conventional risk factors for SCD in HCM patients were associated with increased risk of appropriate shocks in the young study cohort. During a median follow-up period of 2.4 years, inappropriate shocks occurred for 22 % of the patients. Older age at implant was associated with a decreased risk of inappropriate shock. Those who underwent implantation in the earlier decade had a higher incidence of inappropriate shocks. Late complications including lead fracture or dislodgement, generator malfunction, and infection occurred for 32 % of the patients. Three patients died (4 %), one of whom had an arrhythmic sudden death. A greater proportion of primary prevention implantations was performed for patients from the latter decade. Over time, ICD use in young HCM patients has become increasingly primary prevention oriented. Shock rates mirror those reported in adult series, and there is a substantial incidence of device complications.     

经导管应用弹簧圈封堵术治疗儿童心血管畸形

目的　探讨应用弹簧圈治疗儿童心血管畸形的指征、方法学和并发症的预防。方法　133例心血 管畸形患儿接受了经导管应用弹簧圈封堵术。术后随访,定期行心脏超声。结果　101例动脉导管未闭(PDA)中 应用Gianturco弹簧圈14例,Duct Occlud和Nit Occlud弹簧圈87例,PDA最小直径为1.6±0.6mm(0.5～ 3.8mm)。有4例植入2只弹簧圈。即刻封堵率为90.1%(91/101);1月后封堵率98.0%(99/101),1年后封堵率 99.0%(100/101)。应用Gianturco弹簧圈封堵主动脉至肺动脉侧枝血管14例,直径3.5±0.8mm(2.1～5.0mm), 5例植入1只弹簧圈,9例植入2～4只弹簧圈。14例封堵后10～15min完全封堵。封堵冠状动脉瘘共14例,直径 3.8±1.1mm(2.0～5.1mm),其中右冠状动脉右室和右房瘘8例,左冠状动脉前降支或回旋支右室瘘和右房瘘6 例,10例植入1只Gianturco弹簧圈,2例植入2～4只Gianturco弹簧圈,应用Duct Occlud弹簧圈1例,即刻封堵率 为38.5%(5/13),1月后封堵率84.6%(11/13);1例右冠状动脉右室瘘,在封堵后即刻发生弹簧圈漂移至左肺小 动脉,用异物钳取出后再外科手术。肺动静脉瘘2例,全部应用Gianturco弹簧圈,分别植入6和16只弹簧圈,封堵 后外周动脉血氧饱和度从76%上升至91%和96%。应用Nit Occlud弹簧圈各1只封堵有假性室隔     

Transcatheter Occlusion of Residual Patent Ductus Arteriosus After Surgical Ligation

The reported frequency of residual leaks after surgical ligation of patent ductus arteriosus (PDA) varies from 6% to 23%. Reports on percutaneous closure of PDA also involve patients with residual PDA after ligation, but specific data regarding this type of PDA are rare. Our objective was to assess retrospectively the characteristics of residual PDA relevant to transcatheter closure and occlusion results using three types of occluders. Twelve consecutive patients underwent transcatheter occlusion of residual PDA after surgical ligation at a median age of 4.6 years (range 3.2–44.6 years) and median weight 16.5 kg (range 13–62 kg). Three types of occluder were used: Gianturco coils, detachable Cook PDA coils, and the new Amplatzer duct occluder. The median diameter of residual PDA after ligation was 1.5 mm (range 0.9–4.2 mm). All PDAs were of type A morphology. Thirteen devices were successfully placed in the 12 patients, without embolization. There were no complications. At 1 month and 1 year follow-up all residual shunts were completely closed. Coils are particularly suitable for complete closure of residual leaks after surgical ligation of PDA. A 100% closure rate was achieved with a low number of implanted coils.     

Prevalence of Deficient Retro-Aortic Rim and Its Effects on Outcomes in Device Closure of Atrial Septal Defects

Deficient retro-aortic rim is of concern as a risk factor for aortic erosion after device closure of atrial septal defects (ASD). However, its prevalence and contribution to technical failure and adverse outcomes have not been delineated. A single-center retrospective cohort study of children and adults undergoing cardiac catheterization for device occlusion of ASD from 1 January 1999 to 1 April 2012 was performed. Risk factors for technical failure and early adverse outcome were assessed using multivariate logistic regression. During the study period, 445 consecutive subjects with a median age of 5.9 years (range, 0.8–80 years) underwent catheterization. Of the subjects with reviewable echocardiograms, 60 % had deficient retro-aortic rim. No attempt at device closure was made for 3.6 % of the subjects. Of the remaining 429 subjects, 96 % underwent successful device occlusion. Major early adverse events occurred in 1.2 % (95 % confidence interval 0.4–2.7 %) of the cases, all of them either device embolization or malposition. Deficient retro-aortic rim was not a risk factor for composite outcome of technical failure or early major adverse event. No deaths, late reinterventions, or erosion events occurred during 2,395 total person-years (median, 5.8 years) of follow-up evaluation. Deficient retro-aortic rim was associated with increased risk of device impingement on the aorta, but no association was seen between device impingement or deficient retro-aortic rim and the development of new/progressive aortic insufficiency. Deficient retro-aortic rim is highly prevalent but did not increase the risk of adverse outcomes. Its contribution to the risk of aortic erosion could not be addressed by this study.     

Antegrade continence enema (ACE): predictors of outcome in 111 patients

Purpose

The antegrade continence enema (ACE) is used as a means of managing faecal incontinence and constipation with varying outcomes. We aim to evaluate our outcomes of ACEs and identify predictors of outcome.

Methods

A retrospective case-note review of patients ≤16 years of age undergoing an ACE (March 2000–September 2013) was carried out. Data collected included: patient demographics, functional outcomes and complications. Data are quoted as median (range) and compared using Mann–Whitney and Fisher’s exact test. Univariate analysis was performed to identify predictors of successful outcomes. P < 0.05 is significant. Successful outcome = total continence/occasional leakage and failed outcome = regular soiling and/or constipation.

Results

111 patients with complete data sets underwent an ACE [59 % male, median age = 9.5 years (3.4–16 years)] and median follow-up = 48 months (4 months–11 years 4 months). Underlying diagnoses were idiopathic constipation (n = 68), anorectal malformation (n = 27), neuropathic bowel (n = 7), Hirschsprung disease (n = 5) and gastrointestinal dysmotility (n = 4). Social continence was achieved in 87/111 (78 %). Fifteen percent of patients underwent reversal of ACE due to resolution of symptoms. There was no difference in outcomes related to diagnosis, gender, age or follow-up duration. Complication rate was 20.7 % (23/111).

Conclusions

The ACE is safe and effective in the management of intractable constipation and soiling. No predictors of outcome were identified.     

Initial Experience with the Nit-Occlud ASD-R: Short-Term Results

We aim to assess the safety, feasibility and efficacy of the new Nit-Occlud ASD-R (NOASD-R) device. From 2014 to 2015, transcatheter closure of atrial septal defect (ASD) using the NOASD-R was performed in 30 consecutive patients. The standard deployment technique as the left upper pulmonary vein approach was used in 25 patients. Right upper pulmonary vein approach was required in five. The median age was 6 years (range 3.5–60 years), and median weight was 21.5 kg (14–79 kg). Implantation was successful in all patients. The median size of devices was 16 mm (12.0–26.0 mm). The mean device size/2D defect diameter ratio was 1.26 ± 0.09 (1.12–1.40). The mean device size/color flow diameter ratio was 1.07 ± 0.06 (range 1.0–1.22). Releasing problem was encountered in three patients. A device-related erosion on the day after the closure was observed in one patient. No further device-related complication (erosion, embolization or dislodgement of the device) was encountered in a median follow-up period of 10 months (range 2–14 months). Complete occlusion has occurred in all at follow-up. NOASD-R is a feasible and effective device for use in the transcatheter occlusion of moderate to large secundum ASDs in selected patients. The occurrence of the erosion on the right atrial roof may be due to the high localization of the device and the larger size of the right disk.     

Safety and efficacy of mitoxantrone in pediatric patients with aggressive multiple sclerosis

ObjectiveThe purpose of this study was to assess the safety and efficacy of mitoxantrone (MX) in pediatric patients with aggressive multiple sclerosis (MS).MethodsA retrospective analysis on pediatric MS patients treated with MX was performed with regards to demographic/clinical parameters and magnetic resonance imaging (MRI) findings.Results19 definite pediatric MS cases with mean ± SD age of 15.4 ± 2.8 years underwent 20 mg MX for control of their severe/frequent relapses, high EDSS score or new and active brain MRI lesions. After a median [IQR] follow-up period of 30[12–60] months, 14 cases (73%) were relapse free; the EDSS score decreased by at least 0.5 in 16 cases (84.2%); and gadolinium-enhancing lesion volume fell by 84.2% in 16 cases. Adverse events included nausea and vomiting, fatigue, alopecia, palpitation, cardiomyopathy and mild leukopenia. All adverse events were mild and transient.ConclusionOur results suggest MX is a good candidate for treatment of children with worsening RRMS and SPMS. Recommendations regarding patient selection, treatment administration, and close follow-up should be considered. Continuing research is needed to establish its efficacy and safety profile in a multinational collaboration with careful follow-up of adverse events.     

Great Vessel Root and Artery Dimensions in Transposition of the Great Arteries Repaired With Atrial Switch Operation

To describe great-vessel dimensions in patients with D-loop transposition of the great arteries (TGA) who have undergone atrial switch operation (ATSO). Patients who have undergone arterial switch operation for TGA have a high incidence of dilation of the neoaortic root. The incidence and degree of great artery dilation in patients who have undergone ATSO for TGA has not previously been described. A retrospective database review identified patients with TGA and intact ventricular septum who underwent ATSO at <1 year of age with cardiac magnetic resonance (CMR) within the previous 5 years (n = 39). A control group of patients referred for CMR with normal findings was identified for comparison (n = 40). Measurements of the annulus, root, sinotubular junction, and great vessels were performed, and interobserver/intraobserver variability was assessed. Median age of subjects at ATSO was 3 months (range 1–12) with median age at CMR of 29 years (range 18–40). For aortic measurements, mean z scores (± SDs) for patients relative to body surface area (BSA)–adjusted normal controls were as follows: annulus 1.41 (0.80), root 2.04 (1.48), sinotubular junction 2.16 (1.26), and great vessel 1.86 (1.53). For pulmonary measurements, similar values were as follows: annulus 1.82 (1.42), root 3.25 (2.01), sinotubular junction 2.47 (1.79), and great vessel 3.96 (3.08). In all cases, the p value was <0.001, and no confidence interval included the value 0. Adult patients with TGA repaired with ATSO in infancy have a greater incidence of dilation of both great vessels, particularly the pulmonary artery. These results may indicate abnormalities in the vascular structure of both great arteries in TGA that may predispose to progressive arterial dilation.     

Intermediate-Term Results After Extracardiac Conduit Fontan Palliation in Children and Young Adults with Single Ventricle Physiology—A Single-center Experience

The Fontan operation and its modifications are currently the standard palliation in patients with single ventricle physiology. The advantages of extracardiac conduit Fontan operation (ECF) have previously been described. We evaluated the outcome of patients who underwent ECF operation at our center since the year 2000 to assess morbidity, mortality and New York Heart Association (NYHA) functional class in the current era. Medical records of 97 patients who underwent ECF operation from November 2000 till October 2013 were reviewed to determine the diagnosis, age at surgery, surgical technique, postoperative complications, NYHA class at last follow-up, echocardiographic parameters, and most recent Holter monitor results. The median age at surgery was 3.4 years with 4 total deaths until last follow-up (hospital mortality was 1 %, and mortality after hospital discharge was 3 %). Seventy-eight of the 97 patients (80 %) underwent fenestrated Fontan surgery. Median duration of follow-up was 3.9 years (IQR 0.2–12.2 years). At the latest follow-up, 62 patients (67 %) were determined to be in NYHA class I, 25 patients (27 %) in NYHA class II, and 6 (6 %) patients in NYHA class III, and the median oxygen saturation was 92 % (IQR 86–96). There were 15 patients with history of preoperative arrhythmias and 6 of them required pacemaker placement at the time of surgery. One patient developed ectopic atrial tachycardia, and 1 patient had clinical thromboembolism on follow-up. Systolic function of the single ventricle by transthoracic echocardiogram at the latest follow-up was described as normal in 77 patients (83 %), fair in 15 patients (16 %), and poor in 1 patient (1 %). The results of our study show that patients who undergo extracardiac conduit Fontan operation in the modern era may have significantly less morbidity and mortality when compared to earlier years.     

Use of Pulmonary Hypertension Medications in Patients with Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collaterals

Tetralogy of Fallot (TOF) with pulmonary atresia (PA) and multiple aortopulmonary collaterals (MAPCAs) is a rare and severe form of congenital heart disease with poor prognosis. Aortopulmonary collaterals expose pulmonary arterioles to systemic pressure resulting in pulmonary hypertension (PH). To date, reports regarding the role of PH medications in this population are sparse. The objective of this study was to assess the effect of PH medications in patients with TOF, PA and MAPCAs or similar anatomy, with emphasis on symptoms, echocardiography and invasive hemodynamics. A retrospective review was performed for patients at a single tertiary care pediatric center. Twelve of 66 patients were treated with PH medications (18 %), and eight of these patients had adequate follow-up for further analysis. Median age at last follow-up was 6 years (range 1.4–21 years). Median length of therapy with PH medication was 4 years (range 0.3–17 years). PH medications included sildenafil, bosentan, ambrisentan, inhaled treprostinil and prostacyclin infusion. PH therapy was associated with improvement in symptoms in all patients and improvement in PH by hemodynamic measures in the majority of patients. All patients underwent at least one cardiac intervention by catheterization or surgery while taking PH medication. Two patients died from non-PH-related causes. The remaining six patients are alive and remain on PH medication. This review indicates that PH medications are well tolerated by this patient group and provide symptomatic improvement. Further studies are required to determine whether PH medications provide long-term survival benefit for patients with complex congenital heart disease.     

Late Outcome and Predictors of Adverse Events Related to the Implantation of a Permanent Pacemaker in Patients with Isolated Congenital Atrioventricular Block

Isolated congenital atrioventricular block (ICAVB) is a rare, and pacemaker implantation is the only effective treatment. We sought to identify the predictive factors of adverse events related to pacemaker implantation in ICAVB. This is a cohort study of patients diagnosed with ICAVB who underwent pacemaker implantation from 1980 to 2014 in a single center. During the studied period, a total of 647 patients underwent implantation of their first permanent cardiac pacemaker before 30 years of age. Of these, only 62 (9.5 %) were diagnosed with ICAVB. This condition was diagnosed in utero in 15 (24.2 %) cases, 5 (8.1 %) in the neonatal period, 32 (51.6 %) during childhood, and 10 (16.1 %) during adolescence and young adulthood. The presence of autoantibodies (anti-Ro/SSA) was observed in 41 % of mothers who underwent serological evaluation. Age at the time of the initial pacemaker implant was 9.8 ± 9 years. During a mean follow-up time of 15 years, 1 (1.7 %) death occurred due to infectious endocarditis. Complications related to pacemaker implant were reported in 24 patients (38.7 %). The number of complications was significantly higher in the group with an epimyocardial implantation site (HR 6; CI 2.45–14.95). Ventricular dysfunction occurred in 6 (11.7 %) patients; however, we were not able to identify any predictors of it. Our results showed a low mortality rate after permanent therapy. However, these patients exhibited high morbidity related to the pacemaker system, and the epimyocardial implant site was an independent predictor of complications. Predictors of left ventricular dysfunction were not found in the present study.     

Porcine Bioprosthetic Valve in the Pulmonary Position: Mid-Term Results in the Right Ventricular Outflow Tract Reconstruction

Pulmonary valve replacement (PVR) for pulmonary valve insufficiency (PVI) currently represents the most frequent reoperation performed for adults with congenital heart disease. A variety of pulmonary valve substitutes have been used, but none has proved to be ideal. This report reviews the authors’ experience using a porcine prosthetic valve in the pulmonary position. Between January 2001 and December 2011, 76 patients (mean age, 36 years; range, 18–64 years) underwent PVR for chronic PVI using a porcine bioprosthesis. All the patients had previously undergone surgery: 65 for repair of tetralogy of Fallot and 11 for pulmonary surgical valvotomy. Magnetic resonance imaging (MRI) evaluations before surgery and at the 1-year postoperative follow-up evaluation were compared. Aside from the PVR, 59 patients (59/65, 78 %) received 94 associated cardiac surgical procedures. Two hospital deaths occurred. The mean hospital stay was 13 days (range, 7–48 days). At the 1-year control MRI, pulmonary regurgitation fraction, right ventricular end diastolic volume (RVEDV), and RV/LV EDV had improved significantly. During a mean follow-up period of 52 months (range, 6–132 months), one patient died. All the patients were categorized as New York heart association (NYHA) functional class 1. No episodes of structural valve deterioration, endocarditis, or thromboembolic event were noted. Echocardiography showed trivial or no PVI in all the patients. The porcine bioprosthetic valves demonstrated excellent midterm results in the RV outflow tract reconstruction. The hemodynamic characteristics of this valve are comparable with those of homografts or valved conduits. It is easy to implant and allows for avoiding extensive dissection, especially of the pulmonary arteries.     

Retrograde Transcatheter Coil Embolization of Congenital Coronary Artery Fistulas in Infants and Young Children

We report the success of transcatheter coil embolization of congenital coronary artery fistulas in infants and young children, primarily using a retrograde coaxial delivery system. Transcatheter coil embolization of coronary artery fistulas has been used successfully in the adult population with limited reports in the pediatric population, especially in infants. Ten patients underwent transcatheter coil embolization of congenital coronary artery fistulas between January 1993 and August 1999. The median age was 4.5 years (ranging from 5 weeks to 9.8 years). Coils were delivered exclusively using a retrograde arterial approach in 8 of 10 patients. A coaxial system was used in 8 patients. Angiography immediately after placement of coils revealed no residual shunt in any patient. Recent follow-up evaluation is available for 9 patients (median duration of follow-up, 7.9 months). Echocardiographic and clinical evaluations show complete occlusion in 8 of 9 patients. All patients are asymptomatic with normalization of chamber dimensions and coronary artery caliber. Transcatheter coil embolization of congenital coronary artery fistulas can be performed safely and successfully in infants and young children. The use of a retrograde approach and coaxial system is safe and effective and may provide better accuracy, stability and precision for placement of coils. Current address: Texas Childrens Hospital, Baylor College of Medicine, 6621 Fannin MC 19345-C, Houston, TX 77030, USA     

An assessment of iron overload in children treated for cancer and nonmalignant hematologic disorders

Our goal was to assess the natural fate of iron overload (IO) following transfusions of packed red blood cells (PRBCs) in children treated for cancer and nonmalignant disorders according to the intensity level of their treatment. Sixty-six children were followed up from February 2010 to March 2013. The transfusion burden was compared between three treatment intensity groups assigned according to the Intensity of Treatment Rating Scale 3.0 (ITR-3). IO was assessed by serial measurements of serum ferritin (SF) (n?=?66) and quantification of tissue iron by magnetic resonance imaging (MRI) (n?=?12). Of the children studied, 36 % (24/66) received moderately intensive treatment (level 2), 21 % (14/66) received very intensive treatment (level 3), and 42 % (28/66) received the most intensive treatment (level 4). The number of PRBC (p?=?0.016), the total transfused volume (p?=?0.026), and transfused volume adjusted to body weight (p?=?0.004) were significantly higher in the level 4 group. By the median follow-up time of 35.5 months (range 8–133), 21–29 % of patients (including level 2 and level 3 children) had SF >1,000 μg/l 1 year after cessation of transfusions. The slowest decrease of SF was observed in the level 4 group. Initial MRI examination demonstrated either mild or moderate IO in the liver and spleen. Repetitive MRI showed significant improvement in relaxation time between the initial and follow-up MRI performances in the liver (5.9 vs. 8.6 ms, p?=?0.03) and the spleen (4.3 vs. 8.8 ms, p?=?0.03). Conclusion: IO diminished over time, but in the level 4 patients, it was detectable for years after cessation of transfusions.     

Radiofrequency Ablation in the Sinus of Valsalva for Ventricular Arrhythmia in Pediatric Patients

The need to perform catheter ablation of ventricular arrhythmia from within the sinuses of Valsalva in a pediatric patient is uncommon. This has been reported in adults, but there are little data about the feasibility, safety or efficacy of catheter ablation in the sinuses of Valsalva in the pediatric patients. This is a retrospective review of all patients aged 18 years or less, at two separate institutions with no structural heart disease that underwent an ablation procedure for ventricular arrhythmia mapped to the sinus of Valsalva from 2010 to 2015. We identified 8 total patients meeting inclusion criteria. Median age was 16 years and the median weight was 61 kg. All patients were symptomatic or had developed arrhythmia-induced ventricular dysfunction. Ablation was performed in the left sinus in 4 patients and the right sinus in 4 patients. No ablations were required in the non-coronary sinus. All 8 patients had an acutely successful ablation using radiofrequency energy. There were no complications. At a mean follow-up of 7 months (4–15 months), all patients were known to be living. Follow-up data regarding arrhythmia were available in 6 of the 8 patients, and none had recurrence of their ventricular arrhythmia off of all antiarrhythmic medications. Radiofrequency catheter ablation of ventricular arrhythmia in the sinus of Valsalva can be done safely and effectively in pediatric patients.     

Mechanical Occlusion of the Patent Ductus Arteriosus with Jackson Coils

The effectiveness and safety of a protocol for transcatheter patent ductus arteriosus (PDA) closure was assessed. Our goal is complete mechanical occlusion of the PDA in the catheterization laboratory by adding coils until it is no longer possible to cross the PDA with a guidewire. Detachable coil closure of a PDA with a narrowest diameter of 2.4 ± 0.1 mm was attempted in 83 patients with a median age of 2.8 years (0.7 to 27.8 years) and whose median weight was 14.5 kg (6 to 61.6 kg). Coils were successfully implanted in 82 of 83 patients, and in 1 patient a large Rashkind double umbrella was used instead. Complete closure was obtained in 80 (97.6%) patients, 48 of those (59%) received more than one coil. Reintervention for residual shunting was required in only 1 patient and another patient has a trivial residual shunt. Device embolization occurred in three cases. Despite the use of multiple coils there was no evidence of significant left pulmonary artery stenosis. The fluoroscopy time increased from 14.0 ± 2.0 minutes for a single coil to 25.3 ± 2.9 minutes for multiple coils (p < 0.01). Attempting to obtain complete mechanical occlusion of the PDA during the implant procedure by adding extra coils reduces the need for reintervention for residual or recurrent shunting.     

Aortic Aneurysms Remain a Significant Source of Morbidity and Mortality After Use of Dacron® Patch Aortoplasty to Repair Coarctation of the Aorta: Results from a Single Center

Aortic aneurysm formation after coarctation repair is a serious and life-threatening complication. Repairs using synthetic materials such as Dacron^® may carry the highest risk of aneurysm formation and rupture. The authors sought to determine the prevalence of aneurysm formation in patients who previously underwent coarctation repair using Dacron^® patch aortoplasty at their institution. Between 1977 and 1994, 63 patients underwent isolated coarctation repair using Dacron^® patch aortoplasty. Aneurysms were defined as an aortic dimension 1.5 times that of the aorta at the level of the diaphragm as shown by angiography, computed tomography (CT) scan, or magnetic resonance imaging (MRI). Of 61 early survivors, 29 (47 %) experienced an aneurysm in the area of previous repair. Nine patients (31 %) had spontaneous rupture of the aneurysm, which caused death in seven cases. Elective or emergent aneurysm repair was performed for 20 patients without complication, and 2 patients are being monitored at this writing. The mean interval from patch placement to aneurysm repair was 15 years (range, 4–27 years). Overall freedom from the development of an aortic aneurysm was 97 % at 5 years, 90 % at 10 years, 69 % at 20 years, and 42 % at 25 years. After repair of coarctation using Dacron^® patch aortoplasty, the risk for aneurysm formation in the area of repair and death from rupture is extremely high. Therefore, in accordance with the 2008 American Heart Association/American College of Cardiology (AHA/ACC) guidelines, all patients with repaired aortic coarctation should undergo either CT or MRI imaging at least every 5 years to assess for aortic aneurysm formation. More frequent imaging should be obtained for patients previously repaired with Dacron^® patch aortoplasty.     

Valve-Sparing Tetralogy of Fallot Repair With Intraoperative Dilation of the Pulmonary Valve

Deleterious long-term effects of pulmonary regurgitation after tetralogy of Fallot (TOF) repair have become evident during the last two decades. Subsequently, different groups have developed strategies aimed at preserving the pulmonary valve function. However, the results of these approaches are not well known. From July 2009 through March 2012, 38 patients underwent primary repair of TOF at the authors’ institution. Of these, 12 children (7 boys) underwent attempted pulmonary valve-sparing surgery with intraoperative dilation of the pulmonary valve. The technical details as well as the echocardiographic preoperative and follow-up data for this repair were recorded, with a special focus on the feasibility of the technique and the effects on pulmonary valve function. No patient in the series died. At repair, the median age was 6 months (range 3.4–126 months), and the median weight was 7.6 kg (range 4.7–47 kg). Intraoperative dilation of the pulmonary valve was technically feasible for all the patients. Two patients had unsuccessful dilation and underwent a transannular patch procedure. During a median follow-up period of 22 months (range 6–30 months), the pulmonary valve diameter and z-score improved significantly. Moreover, the annular size normalized, whereas the mean right ventricular outflow tract (RVOT) gradient remained at the mild level (median, 24 mmHg; range 12–36 mmHg). At the most recent follow-up evaluation, three patients showed moderate pulmonary regurgitation. Intraoperative dilation of the pulmonary valve in patients undergoing TOF repair is feasible and provides good relief of obstruction. Moreover, the pulmonary valve annulus grows through the follow-up period. Longer follow-up studies are needed to evaluate the exact role of this strategy in this population.