Permanent pacemaker implantation in post one-and-a-half ventricle repair with tricuspid valve replacement in Ebstein anomaly: An innovative technique.

INTRODUCTION: Transvenous pacemaker implantation in patients post bidirectional Glenn anastomosis in one-and-a-half ventricle repair is usually not feasible. However, with a modified surgical technique for Glenn anastomosis and a combined interventional and electrophysiologic approach, the transvenous pacemaker was successfully implanted. FINDINGS AND CONCLUSIONS: We reported a novel technique of pacemaker implantation in a 27-year-old woman, underlying Ebstein anomaly of the tricuspid valve, who developed intermittent complete atrioventricular block at 5 years after surgical repair. The patient had a tricuspid valve replacement and a novel modified bidirectional Glenn anastomosis for one-and-a-half ventricle repair. The Glenn circuit was conducted by opening a window between the posterior wall of the superior vena cava (SVC) and the anterior wall of the right pulmonary artery (RPA), combined with putting a Goretex membrane in the SVC below the SVC-RPA window without disconnecting the SVC from the right atrium. The transvenous pacemaker was implanted by perforating the Goretex membrane, then passing the leads from the axillary vein through the perforated membrane and placing them in the coronary sinus and right atrium.

Transcatheter Intervention for Coarctation of the Aorta: A Nordic Population-Based Registry With Long-Term Follow-Up.

BACKGROUND: Coarctation of the aorta (CoA), a congenital narrowing of the proximal descending thoracic aorta, is a relatively common form of congenital heart disease. Untreated significant CoA has a major impact on morbidity and mortality. In the past 3 decades, transcatheter intervention (TCI) for CoA has evolved as an alternative to surgery. OBJECTIVES: The authors report on all TCIs for CoA performed from 2000 to 2016 in 4 countries covering 25 million inhabitants, with a mean follow-up duration of 6.9 years. METHODS: During the study period, 683 interventions were performed on 542 patients. RESULTS: The procedural success rate was 88%, with 9% considered partly successful. Complications at the intervention site occurred in 3.5% of interventions and at the access site in 3.5%. There was no in-hospital mortality. During follow-up, TCI for CoA reduced the presence of hypertension significantly from 73% to 34%, but despite this, many patients remained hypertensive and in need of continuous antihypertensive treatment. Moreover, 8% to 9% of patients needed aortic and/or aortic valve surgery during follow-up. CONCLUSIONS: TCI for CoA can be performed with a low risk for complications. Lifetime follow-up after TCI for CoA seems warranted.

Recurrent Takotsubo syndrome in a boy with Duchenne muscular dystrophy: A case report.

Takotsubo syndrome is a rare cause of reversible ventricular dysfunction that imitate an acute coronary syndrome. The entity is unusual among pediatric populations and a recurrent episode is extremely rare. We report a case of recurrent takotsubo syndrome in an eight-year-old boy with Duchenne muscular dystrophy (DMD). His chest pain episodes were aggravated by a strong emotional stimuli. During episodes of chest pain, electrocardiograms (ECG) showed ST elevation while echocardiograms showed left ventricle apical ballooning; however, a coronary angiography was normal. Serial ECG and echocardiogram revealed a spontaneous resolution of ST elevation and normalized apical contraction which were compatible with the diagnosis of takotsubo syndrome. Interestingly, serial cardiac magnetic resonance imaging demonstrated increasing subepicardial enhancement which was compatible with progression of cardiac involvement in DMD. .

Clinical presentation and course of long QT syndrome in Thai children.

BACKGROUND: Congenital long QT syndrome (LQTS) is a genetically transmitted cardiac channelopathy that can lead to lethal arrhythmia and sudden cardiac death in healthy young people. The clinical characteristics of LQTS are variable and depend on the subtype of long QT syndrome, which differ among populations. This single hospital-based case review study examined the clinical presentation of long QT syndrome and the outcomes of its treatment in 20 Thai children at King Chulalongkorn Memorial Hospital in Bangkok, Thailand. METHODS: Inpatient and outpatient records of children (aged 0-14 years) diagnosed with long QT syndrome from January 1, 1998, to September 30, 2013, were retrospectively reviewed. Presentation at diagnosis, treatments, and clinical courses were collected and analyzed. In the 20 subjects, total Schwartz scores totaled 5.2±0.9 points, and mean age at diagnosis was 7.6±4.4 years (range, 1 day-13.8 years). The patients were assigned to one of 3 groups based on trigger events: 50% of patients had events at rest (sleep or at rest), 35% experienced adrenergic-mediated events (e.g., stress, exercise, startle), and 15% were asymptomatic. Excluding the 3 patients who died at first presentation, 100% of patients received a beta blocker, and 47.1% were treated with an automatic implantable cardioverter-defibrillator (AICD). RESULTS: At follow-up (median=959 days; range, 1-4170 days), 4 patients (20%) were known to have died, 3 of whom died shortly after the diagnosis. Among patients who survived the initial event, 52.9% (9 of 17) experienced cardiac events (appropriate AICD shock, death, and/or syncope) during the follow-up period. The mean duration from diagnosis to cardiac event was 1420±759 days (range, 497-2499 days). CONCLUSIONS: All 20 patients with LQTS were mostly symptomatic at presentation. Owing to the geographical region and ethnicity of the Thai population, we conclude that the ratio of patients who develop cardiac symptoms at rest or during sleep might be higher than in other Asian countries.

Balloon pulmonary valvuloplasty in tetralogy of Fallot: effects on growth of pulmonary annulus and transannular patch.

BACKGROUND: Balloon pulmonary valvuloplasty and its benefit on the growth of pulmonary annulus and pulmonary artery in tetralogy of Fallot (TOF) patients remains controversial. The purpose ofthe present study was to determine the growth of pulmonary valve annulus and pulmonary artery and to evaluate the need of transannular patch during total surgical correction in patients with tetralogy of Fallot (TOF) after balloon pulmonary valvuloplasty. MATERIAL AND METHOD: Fifty-one severely hypoxic TOF patients, who underwent balloon dilatation ofpulmonary valve, were included in the present study. The clinical outcomes, oxygen saturation, and echocardiographic parameters before and after balloon dilatation were analyzed. RESULTS: There were 33 males and 18females. The mean age was 3 years 5 months old (range, I month-15 years 8 months old). The mean oxygen saturation increasedfrom 73.9 +/- 9.1 to 84.8 +/- 6.7% immediately after the procedure (p-value < 0.05). There was no serious procedural-related complication. At the mean follow-up period of 2 years and 4 months, the mean Z-score of pulmonary annulus size increased from -2.56 SD to -1.87 SD (p-value < 0.05) and the right pulmonary artery size from -0.29 SD to + 0.46 SD (p-value < 0.05). Thirty-seven patients (66.1%) underwent corrective surgery. Pulmonary transannular patch was performed in 11 of37 patients (29.7%). CONCLUSION: Pulmonary valve dilatation in patients with TOF is safe. It promotes the growth ofthe pulmonary valve annulus and pulmonary artery and may decrease the need of transannular patch at the time of surgical correction.

Transcatheter closure of patent ductus arteriosus with a self-expanding platinum-coated nitinol device.

BACKGROUND: An occluding device for closure of patent ductus arteriosus (PDA) was developed from meshed nitinol wires coated with platinum for prevention of nickel release after implantation. OBJECTIVES: Our purpose was to assess the immediate and short-term results of transcatheter PDA closure with this device. METHODS: Sixty patients (13 males and 47 females) underwent catheter-based PDA closure. The age ranged from 9 months to 65 years, with a median age of 4 years. The weight ranged from 4.2-65 kg, with a median of 15.2 kg. The mean PDA diameter at the narrowest segment was 4.7 +/- 2.2 mm, with a range of 2.0-15.1 mm. Eighteen cases had serial blood samples for serum nickel analysis taken before and at 1, 3 and 30 days after device implantation. RESULTS: The devices were successfully deployed in all 60 patients. There were no serious procedural complications. Color Doppler demonstrated complete occlusion rate of 78.3%, 90.0% and 100% at 1 day, 1 month and 1 year after implantation, respectively. There was no statistical difference in serum nickel concentrations between pre- and post-implantation. CONCLUSION: Transcatheter PDA closure using a platinum-coated nitinol device can be performed safely and successfully. There was no evidence of nickel release or nickel reaction after device implantation. This device model may be an alternative for PDA closure, especially in patients with potential nickel allergy.