B. Anderson

Catheter interventions in the staged management of hypoplastic left heart syndrome

AIM To analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome. METHODS This study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.5 years. RESULTS A staged Norwood/Fontan palliation for hypoplastic left heart syndrome was performed in 527 patients. The 30-day survival rate after individual stages was 76.5% at Stage I, 96.3% at Stage II, and 99.4% at Stage III. A total of 348 interventions were performed in 189 out of 527 patients. Freedom from catheter intervention in survivors was 58.2% before Stage II and 46.7% before Stage III. Kaplan-Meier freedom from intervention post Fontan completion was 55% at 10.8 years of follow-up. Post-stage I interventions were mostly directed to relieve aortic arch obstruction--84 balloon angioplasties--and augment pulmonary blood flow--15 right ventricle-to-pulmonary conduit interventions; post-Stage II interventions centred on augmenting size of the left pulmonary artery--73 procedures and abolishing systemic venous collaterals--32 procedures. After Stage III, the focus was on manipulating the size of the fenestration--42 interventions--and the left pulmonary artery -31 procedures. CONCLUSION Interventional cardiac catheterisation constitutes an integral part in the staged palliative management of patients with hypoplastic left heart syndrome. Over one-third (37%) of patients undergoing staged palliation required catheter intervention over the follow-up period.

Transcatheter Closure of the Patent Ductus Arteriosus: An Intention to Treat Analysis

BACKGROUND In patients with patent ductus arteriosus (PDA), transcatheter closure is the current procedure of choice. There are multiple devices available with limited current comparative data and varied recommendations for device selection. OBJECTIVE To assess the efficacy and safety of the Flipper coil (FC) and the Amplatzer Duct Occluder (ADO). METHODS An intention to treat analysis of all children admitted to the catheter laboratory at a single institution for occlusion of PDA from 2003 to 2011 was performed. Patient and device selection were determined by the treating physician. Standard techniques for FC and ADO implantation were used. RESULTS Two hundred and twenty eight children with median weight of 14.2 kg (range; 5.5-68 kg) underwent cardiac catheterisation, with successful occlusion in 96.2% of patients when attempted. In 16 patients, median angiographic PDA diameter of 0.8 mm (range; 0.4-1.2 mm), was considered too small to warrant closure. Eight patients with large PDAs underwent surgical ligation. FC was successfully used in 70 (34.3%) and ADO in 134 (66.7%) patients. ADO patients were smaller (P=0.004) with larger PDAs (P<0.0001) than the FC group. Median fluoroscopy time was longer for ADO patients (10.1 min vs 8.0 min; P<0.0001). ADO had a lower embolisation rate (0% vs 6.6%; P=0.005) and a higher complete occlusion rate at follow-up (100% vs 73.4%; P<0.0001). Length of hospital admission decreased with time in both groups. CONCLUSION Transcatheter closure of the PDA has a high degree of safety and efficacy. This study suggests that the ADO may be the device of first choice in the current era.

Cardiac catheter procedures during extracorporeal life support: a risk-benefit analysis.

Background: Extracorporeal life support (ECLS) is a valuable tool for situations in which cardiac disease acutely threatens the life of a child. Residual anatomic lesions have a strong negative influence on survival when ECLS is used after cardiac operations. Accurate diagnostic information is essential, and although noninvasive studies are preferred (eg, echocardiography and thoracic computed tomographic angiography), they are not always logistically practical nor adequate in complex situations under the loading and nonpulsatile flow conditions of ECLS. Methods: We analyzed our experience (February 2009 to August 2012) with cardiac ECLS for 59 children. Of the 59 children, 22 (median age and weight 19.5 days and 4 kg) with advanced cardiac dysfunction had catheter studies performed during support. Results: The 22 patients had 28 studies, without major adverse events relating to the procedure or transport. Problems leading to further therapeutic procedures (catheter based seven, hybrid two, or surgical eight) were discovered in 17 of the 22 patients. For 22 catheterized patients, total time on ECLS, weaning probability, and survival to discharge were 151.6 ± 122.6 hours, 81%, and 68%, respectively, similar to that for the 37 cardiac support patients not requiring catheter studies during support (P = 0.94, 0.37, and 0.59, respectively). Conclusion: Assuming that undiscovered anatomic and/or hemodynamic issues would have had a strong negative influence on survival, we may conclude that the risk–benefit ratio was positive and favorable. Catheter studies during ECLS are safe and should be performed expeditiously when diagnostic questions cannot be resolved by noninvasive means.

Extracorporeal life support in the acute management of tumour lysis syndrome

A 16-month old boy presented with a severe tumour lysis syndrome (TLS) complicating induction therapy for acute myeloid leukaemia. This was further complicated by a respiratory syncytial virus infection. The failure of response to escalating treatment necessitated the use of extracorporeal life support (ECLS) during continuation of his induction chemotherapy. He was weaned from support after the resolution of the TLS and completed chemotherapy successfully. ECLS may have a role to play in the acute support of children with cardiorespiratory failure as a result of malignancy or the required treatment.

Ventricular septal defect closure in a child with osteogenesis imperfecta: risk factors and management.

Cardiac surgery in patients with osteogenesis imperfecta is challenging as the friability of the tissues can be hazardous before, during, and after the operation. A multidisciplinary approach with a planned strategy is essential for the successful management of these patients. We present a 6-year old child with osteogenesis imperfecta, who underwent ventricular septal defect closure without any complication.