Antithrombotic Therapy Duration after Patent Foramen Ovale Closure for Stroke Prevention: Impact on Long-Term Outcome.

Background: The optimal duration of antithrombotic therapy (ATT) after patent foramen ovale (PFO) closure remains under debate. This study sought to compare the clinical outcome of patients receiving antithrombotic agents for a short (6 months) versus extended (>6 months) period after the procedure. Methods: This was a retrospective cohort study using a propensity score matching analysis on 259 consecutive patients (131 males, 43 ± 10 years) undergoing PFO closure due to cryptogenic stroke, with complete follow-up (median duration of 10 [4-13] years). The outcome was compared between patients receiving short-term (Group short, N = 88) versus extended ATT (Group long, N = 171). Results: The PFO closure device was successfully implanted in all cases, with 3% of minor complications. After propensity score matching, there were no differences between Groups short and long in the rate of stroke (0.3 vs. 0.4% patient-year, p=1.00), bleeding (2 vs. 2% patient-year, p=0.17), and device thrombosis (0.3 vs. 0.1% patient-year; p=0.60). Univariate analysis showed that short-term ATT was not associated with an increased risk of recurrent stroke (HR: 1.271 [95% CI: 0.247-6.551], p=0.775) or prosthesis thrombus (HR: 0.50 [95% CI: 0.070-3.548], p=0.72). Kaplan-Meier analysis revealed similar overall survival in Group short and long (100 vs. 99 ± 1%, respectively; p=0.25). Conclusions: Short-term (6 months) ATT after PFO closure did not impair the clinical outcome, with a preserved low rate of recurrent stroke (0.3% patient-year) and device thrombosis (0.2% patient-year) at 10-year follow-up.

Treatment of a severe distal thoracic and abdominal coarctation with cutting balloon and stent implantation in an infant: From fetal diagnosis to adolescence.

Background and Aims: Abdominal coarctations are rare. Surgical treatment is difficult and requires re-interventions to adjust the graft material to patient growth. We report effective treatment by interventional catheterization in an infant with the concern to allow adjustment for growth and prevention of vessel damage. Methods and Results: After the diagnosis of abdominal coarctation at 27 weeks of gestation, an infant developed hypertension (170/70 mmHg) at 3 months of age despite medical therapy. Angio CT confirmed a 2 mm diameter, 2.3-cm-long coarctation of the descending aorta. At 4 months, a dilatation was performed using a 3 mm cutting balloon and a 5 mm Opta® balloon, Cordis®. Two noncovered Palmaz® Genesis™ XD PG1910P stents were required to keep the aortic lumen open. At 15 months, an Adventa™ V12 vascular 12 × 61 mm long covered stent was implanted to exclude an aneurysm which developed between the two stents. At 3 and 9.5 years, the stents were further dilated with a high-pressure balloon to reach 11 mm aortic diameter with no residual pressure gradient, and normal blood pressure. Conclusions: The use of cutting balloons and stent implantation is an effective way to relieve severe obstruction in middle aortic syndrome in neonates. The technical issues encountered were the need for a low profile sheath and material to avoid femoral artery damage, and the need to use stents that can be further expanded to adult size.

Results of Coarctation Repair by Thoracotomy in Pediatric Patients: A Single Institution Experience.

BACKGROUND: Aortic coarctation is among the most common cardiovascular congenital abnormalities requiring repair after birth. Besides mortality, morbidity remains an important aspect. Accordingly, we reviewed our 20-year experience of aortic coarctation repair by thoracotomy, with emphasis on both short- and long-term outcomes. METHODS: From 1995 through 2014, 214 patients underwent coarctation repair via left thoracotomy. Associated arch lesions were distal arch hypoplasia (n = 117) or type A interrupted aortic arch (n = 6). Eighty-four patients had isolated coarctation (group 1), 66 associated ventricular septal defect (group 2), and 64 associated complex cardiac lesions (group 3). Median follow-up was 8.4 years. RESULTS: There was one (0.5%) procedure-related death. Nine (4.2%) patients died during index admission. In-hospital mortality was 0.7% in group 1 and 2 and 12.5% in group 3 (P < .001). No patient had paraplegia. Actuarial five-year survival was 97.5% in group 1, 94% group 2 and 66% in group 3. Recurrent coarctation developed in 29 patients, all but four (1.8%) successfully treated by balloon dilatation. Freedom from reintervention (dilatation or surgery) at five years was 86%. At hospital discharge, 28 (13.5%) patients were hypertensive. At follow-up, hypertension was present in 11 (5.3%) patients. CONCLUSIONS: Long-term results of aortic coarctation repair by thoracotomy are excellent, with percutaneous angioplasty being the procedure of choice for recurrences. Patient prognosis is dependent on associated cardiac malformations. In this study, the prevalence of late arterial hypertension was lower than previously reported.

Autologous vascularised pericardial flap tunneling technique in Scimitar syndrome repair with dextrocardia: lessons from the Senning procedure.

Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. Surgery is required when pulmonary overcirculation is present. Following repair, Scimitar vein stenosis occurs in approximately 20%. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. This tunneling technique allowed tension-free anastomosis and minimal Scimitar vein rotation.

Quality of life assessment in children before and after a successful ablation for supraventricular tachycardia.

BACKGROUND AND OBJECTIVES: Young patients suffering from rhythm disorders have a negative impact in their quality of life. In recent years, ablation has become the first-line therapy for supraventricular arrhythmias in children. In the light of the current expertise and advancement in the field, we decided to evaluate the quality of life in young patients with supraventricular arrhythmias before and after a percutaneous ablation procedure. METHODS: The prospective cohort consisted of patients <18 years with structurally normal hearts and non-pre-excited supraventricular arrhythmias, who had an ablation in our centre from 2013 to 2018. The cohort was evaluated with the PedsQL™ 4.0 Generic Core Scales self-questionnaire prior to and post-ablation. RESULTS: The final cohort included 88 patients consisted of 52 males (59%), with a mean age at ablation of 12.5 ± 3.3 years. Forty-two patients (48%) had a retrograde-only accessory pathway mediating the tachycardia, 38 (43%) had atrio-ventricular nodal re-entrant tachycardia, 7 (8%) had ectopic atrial tachycardia, and 1 (1%) had atrial flutter. The main reason for an ablation was the patient's choice in 53%. There were no severe complications. Comparison between the baseline and post-ablation assessments showed that patients reported significant improvement in the scores for physical health, emotional and social functioning, as well as in the total scores. CONCLUSIONS: The present study demonstrates that the successful treatment of supraventricular arrhythmias by means of an ablation results in a significant improvement in the quality of self-reported life scores in young patients.

Liver and systemic hemodynamics in children with cirrhosis: Impact on the surgical management in pediatric living donor liver transplantation.

Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation (LT) was evaluated. Liver and systemic hemodynamics were studied prospectively in 52 children (median age, 1 year; 33 with biliary atresia [BA]). The hemodynamics of native liver were studied preoperatively by Doppler ultrasound and intraoperatively using invasive flowmetry. Portosystemic gradient was invasively measured. Systemic hemodynamics were studied preoperatively by Doppler transthoracic echocardiography and intraoperatively by using transpulmonary thermodilution. Hemodynamic parameters were correlated with Pediatric End-Stage Liver Disease (PELD) score and the histological degree of fibrosis (collagen proportionate area [CPA]). Cirrhosis was associated with a 60% reduction of pretransplant total liver flow (n = 46; median, 36 mL/minute/100 g of liver) compared with noncirrhotic livers (n = 6; median, 86 mL/minute/100 g; P = 0.002). Total blood flow into the native liver was negatively correlated with PELD (P < 0.001) and liver CPA (P = 0.005). Median portosystemic gradient was 14.5 mm Hg in children with cirrhosis and positively correlated with PELD (P < 0.001). Portal vein (PV) hypoplasia was observed mainly in children with BA (P = 0.02). Systemic hemodynamics were not altered in our children with cirrhosis. Twenty-one children met the intraoperative criteria for PV reconstruction using a portoplasty technique during the LT procedure and had a smaller PV diameter at pretransplant Doppler ultrasound (median = 3.4 mm; P < 0.001). Cirrhosis in children appears also as a hemodynamic disease of the liver, correlated with cirrhosis severity. Surgical technique for PV reconstruction during LT was adapted accordingly. Liver Transplantation 23 1440-1450 2017 AASLD.

Long-Term Results of Balloon Valvuloplasty as Primary Treatment for Congenital Aortic Valve Stenosis: a 20-Year Review.

In the presence of new surgical techniques, the treatment of congenital valvular aortic stenosis is under debate. We reviewed the results and late outcomes of all 93 patients aged 1 day to 18 years, treated with balloon valvuloplasty (BAV) as first-line therapy for congenital aortic valve stenosis in our center from January 1991 to May 2012. Mean age at procedure time was 2.4 years; 37 patients underwent BAV at age ≤30 days (neonates), 29 patients at age ≥1 month and <1 year (infants), and 27 patients were older than 1 year (children). The invasive BAV peak-to-peak aortic valve gradient (mean 59 ± 22 mmHg) was immediately reduced (mean 24 ± 12 mmHg). The observed diminution of gradient was similar for each age group. Four patients had significant post-BAV AI. Mean follow-up after BAV was 11.4 ± 7 years. The last echo peak aortic gradient was 37 ± 18 mmHg and mean gradient was 23 ± 10 mmHg, and two patients had significant AI. Actuarial survival for the whole cohort was 88.2 and 72.9 % for the neonates. All infants, except one, and all children survived. Sixty-six percent of patients were free from surgery, and 58 % were free from any reintervention, with no difference according to age. Freedom from surgery after BAV at 5, 10, and 20 years, respectively, was 82, 72, and 66 %. Our study confirms that BAV as primary treatment for congenital AS is an efficient and low-risk procedure in infants and children. In neonates, the prognosis is more severe and clearly related to "borderline LV."

Cardiovascular pathology in males and females with 45,X/46,XY mosaicism.

CONTEXT: The phenotype of 45,X/46,XY mosaicism is heterogeneous ranging from females with Turner syndrome (TS) to apparently normal males. Males with 45,X/46,XY frequently show stigmata typically associated with TS. We hypothesised that males with 45,X/46,XY have similar cardiovascular pathology as females with 45,X/46,XY. OBJECTIVE: To investigate cardiovascular abnormalities in 45,X/46,XY males and to compare them with 45,X/46,XY females. DESIGN: Patients with 45,X/46,XY mosaicism were selected from the Belgian Registry for Growth and Puberty problems and via the multidisciplinary clinic for disorders of sexual development. PATIENTS: EIGHTEEN PATIENTS WERE INCLUDED: 8 raised as females (F) and 10 as males (M). INTERVENTION: Complete cardiac examination with blood pressure measurement, ECG, echocardiography and MRI. MAIN OUTCOME MEASUREMENT: Cardiac parameters were registered for both groups. In a second phase, clinical features and external masculinisation score (EMS) were retrospectively collected from the medical files. RESULTS: A structural heart defect was diagnosed before inclusion in 1 F with coarctation and 1 M with spontaneously closed VSD. A bicuspid aortic valve was found in 8 (3 F, 5 M). Dilation of the ascending aorta was present in 4 M and was severe in 2 young boys. QTc was prolonged in 3 F and 2 M. CONCLUSION: Males with 45,X/46,XY mosaicism have similar cardiovascular pathology as 45,X/46,XY females. Dilation of the ascending aorta can be important, also in males. We advise cardiac screening and life-long monitoring in all males with 45,X/46,XY mosaicism according to the existing guidelines for Turner syndrome.

Catheter interventions in congenital heart disease without regular catheterization laboratory equipment: the chain of hope experience in Rwanda.

This report describes the feasibility and safety of cardiac catheterization in a developing country without access to a regular cardiac catheterization laboratory. The equipment used for imaging consisted of a monoplane conventional C-arm X-ray system and a portable ultrasound machine using the usual guidewires and catheters for cardiovascular access. In this study, 30 patients, including 17 children younger than 2 years and 2 adults, underwent catheterization of the following cardiac anomalies: patent ductus arteriosus (20 patients) and pulmonary valve stenosis (9 patients, including 2 patients with critical stenosis and 3 patients with a secundum atrial septal defect). Except for two cases requiring surgery, the patients were treated successfully without complications. They all were discharged from hospital, usually the day after cardiac catheterization, and showed significant clinical improvement in the follow-up evaluation. Cardiac catheterization can be performed safely and very effectively in a country with limited resources. If patients are well selected, this mode of treatment is possible without the support of a sophisticated catheterization laboratory.

Behavior of the electrocardiographic T peak to end interval in childhood.

BACKGROUND: The T-wave peak to T-wave end (TpTe) interval reflects spatial and transmural dispersion in repolarization and serves as an arrhythmogenic index. Normal TpTe interval data in children are lacking. We evaluated the effects of age, gender, heart rate, leads (II and V(5)) on TpTe and T-wave voltage. METHODS: Four hundred healthy children (age 4 days to 16.7 years) were enrolled. From a resting 12-lead digital ECG, TpTe, RR, QT, JT intervals, and T amplitude were measured (leads II and V(5)). Bazett and Fridericia formulas were applied to TpTe for heart rate correction and TpTe/QT and TpTe/JT were calculated. Descriptive and analytical statistics were applied, significance level set at P < or = 0.05. RESULTS: TpTe in leads II and V(5) correlate well. Contrary to adults, no gender differences in TpTe were observed in childhood. TpTe lengthens with increasing age, and is inversely related to heart rate. TpTe 98th percentile is 85 msec in first 5 years, increasing to 92 msec in adolescence. TpTe Fridericia is a good correction formula in childhood; TpTe Bazett overcorrects in the younger age. TpTe/QT and TpTe/JT are longer in younger subjects due to greater QT shortening than the TpTe interval at higher heart rates. CONCLUSIONS: In children, TpTe in lead II and V(5) correlate well. The TpTe interval lengthens with advancing age as heart rate diminishes. TpTe Fridericia is a good correction formula in children. Younger subjects have higher TpTe/QT and TpTe/JT indices compared to older children. T-wave voltage increases with age, tallest in the 5-10-year-age group particularly in V(5).