Long-term follow-up after recanalisation of aortic arch atresia.

AIMS: Aortic arch atresia (AAA) is one of the rarest obstructive defects. The presence of this anomaly in adult age is uncommon. The typical anatomic feature consists of a complete occlusion of the membranous obstruction resulting in an acquired atresia without flow continuity between the proximal and distal segments. This feature is important in determining the feasibility of percutaneous intervention. The aim of the present study was to share long-term follow-up data of adult patients with AAA requiring percutaneous interventions for the management of this rare anomaly involving five different centres. METHODS AND RESULTS: Retrospective data of 19 patients (12 males, 63.2%, mean age 32.2±18.9 years) diagnosed with AAA treated in five different centres between 1999 and 2017 were collected. All patients underwent percutaneous recanalisation by (1) radiofrequency (RF) system (five patients, 26.3%), (2) extra-stiff guidewire (12 patients, 63.2%), and (3) transseptal needle (two patients, 10.5%). All procedures were subsequently followed by covered stent implantation. Two patients developed complications during the procedure and one of them died. Over a median follow-up of 4.94 years, four (21%) patients were able to be weaned from medications for hypertension. All the patients underwent reassessment for recurrence or restenosis during the follow-up. Seven (36.8%) patients underwent successful stent dilatation with a balloon. After the intervention, one patient experienced a late complication; however, one patient died due to an unknown cause believed to be unrelated to the previous recanalisation procedure. CONCLUSIONS: Percutaneous treatment of AAA is feasible with good long-term survival. This study reports the largest case series so far available in the literature.

Stent dilatation of atretic aortic coarctation in an adult-case report and literature review.

BACKGROUND: Patients with functional aortic interruption of the descending thoracic aorta at the isthmus due to severe coarctation in association with atretic lumen are extremely rare in the adult population. The management is challenging and carries high morbidity and mortality. CASE PRESENTATION: We describe successful percutaneous reconstruction using a covered stent in a similar patient who is doing well two-years after intervention. A literature search was done to explore management strategies and their long-term outcomes for better understanding. CONCLUSIONS: This report is an attempt to highlight the role of minimal invasive approach in the management of rare, severe coarctation of the aorta in adult patients to avoid morbidity and mortality associated with more invasive procedures.

Three-dimensional printed prototypes refine the anatomy of post-modified Norwood-1 complex aortic arch obstruction and allow presurgical simulation of the repair.

Three-dimensional (3D) printed prototypes of malformed hearts have been used for education, communication, presurgical planning and simulation. We present a case of a 5-month old infant with complex obstruction at the neoaortic to transverse arch and descending aortic junction following the neonatal modified Norwood-1 procedure for hypoplastic left heart syndrome. Digital 3D models were created from a routine 64-slice CT dataset; then life-size solid and magnified hollow models were printed with a 3D printer. The solid model provided further insights into details of the anatomy, whereas the surgical approach and steps of the operation were simulated on the hollow model. Intraoperative assessment confirmed the anatomical accuracy of the 3D models. The operation was performed in accordance with preoperative simulation: sliding autologous flaps achieved relief of the obstruction without additional patching. Knowledge gained from the models fundamentally contributed to successful outcome and improved patient safety. This case study presents an effective use of 3D models in exploring complex spatial relationship at the aortic arch and in simulation-based planning of the operative procedure.

Late endocarditis of Amplatzer atrial septal occluder device in a child.

Bacterial endocarditis following atrial septal defect closure using Amplatzer device in a child is extremely rare. We report a 10-year-old girl who developed late bacterial endocarditis, 6 years after placement of an Amplatzer atrial septal occluder device. Successful explantation of the device and repair of the resultant septal defect was carried out using a homograft patch. The rare occurrence of this entity prompted us to highlight the importance of long-term follow up, review the management and explore preventive strategies for similar patients who have multiple co-morbidities and a cardiac device. A high index of suspicion is warranted particularly in pediatric patients.

Cribriform amplatzer device closure of fenestrated atrial septal defects: feasibility and technical aspects.

Fenestrated atrial septal defects (F-ASDs) may pose a challenge to device closure; recently, a cribriform device with a minimal connecting intrawaist diameter and large, equal left- and right-sided discs has been designed to cover more than one adjacent defect. This study demonstrates the feasibility and technical aspects of closing F-ASDs using this new device. Sixteen patients between August 2003 and January 2006 were included in this study. The inclusion criterion was the presence of a F-ASD diagnosed by transesophageal echocardiography. One of the three available cribriform ASD device sizes (18, 25, or 35 mm) was implanted. Patients were followed for at least 1 year after the procedure. Thirteen patients had successful cribriform ASD device implantation (median age and weight, 12.5 years and 36 kg, respectively). Ten patients (62%) had an associated atrial septal aneurysm. The mean procedure time was 75.6 +/- 28.5 min and the mean fluoroscopy time 14.8 +/- 6.3 min. The RVEDD was significantly reduced, from a mean of 24.2 mm to 21.0 (p < 0.05). One patient developed atrial tachycardia requiring cardioversion during the procedure. There were no embolic events, heart block, or mortality. Complete closure was 10 of 13 (77 %) the next day and 12 of 13 (92%) at 6 and 12 months. We conclude that the cribriform Amplatzer device can be successfully and safely used in patients with F-ASDs. Complete closure may take up to 6 months.

Management of superior vena cava obstruction syndrome due to thrombosis of a Contegra conduit used to re-establish the innominate vein-to-right atrium continuity.

Following intraoperative superior vena cava injury, venous drainage of the head, neck and upper extremities can be re-established with bovine jugular vein (Contegra) conduits. Inadequate anticoagulation, however, may lead to conduit thrombosis and superior vena cava obstruction syndrome. This can be successfully treated with percutaneous dilatation and stenting of the failed conduit.

A congenital fistula between the descending aorta and the right pulmonary vein in a neonate presenting with heart failure.

We report a large congenital fistula connecting the descending thoracic aorta to the right upper pulmonary vein in a newborn baby presenting on the seventh day of life with cardiac failure and a continuous murmur heard posteriorly. The fistula was detected echocardiographically, and shown at cardiac catheterisation not to be suitable for percutaneous occlusion. The anatomy of the fistula was confirmed at surgery, when it was ligated successfully.

Variations in captopril formulations used to treat children with heart failure: a survey in the United kingdom.

BACKGROUND AND OBJECTIVE: Different liquid formulations of a drug prepared for use in children cannot be assumed to have therapeutic equivalence. The objective of this study was to ascertain the interhospital constancy of unlicensed liquid captopril formulations used to treat children with heart failure in the UK. DESIGN: A questionnaire-based telephone survey. SETTING: 13 tertiary paediatric cardiac centres in the UK and 13 large hospitals referring patients to these centres. PARTICIPANTS: The study included pharmacists responsible for providing the pharmaceutical input to children with congenital heart disease or a pharmacist designated to cover paediatric services. Technical staff employed by "specials" manufacturers also participated. RESULTS: Four hospitals dispensed captopril tablets for crushing and dissolving in water before administration; the remaining 22 used nine different liquid formulations of captopril. Only three cardiac centres and their referring hospitals were found to be using the same liquid captopril formulations; 10 centres and their referring hospitals were using completely different captopril formulations. CONCLUSIONS: This survey shows that paediatric cardiac centres and their referring hospitals use a variety of unlicensed liquid captopril formulations interchangeably. This degree of inconsistency raises issues about optimal captopril dosing and potential toxicity, such that its use may influence paediatric cardiac surgical and interventional outcomes.

Catheter closure of patent ductus arteriosus in dogs: variation in ductal size requires different techniques.

BACKGROUND: : Catheter closure of patent ductus arteriosus Botalli (PDA) is increasingly replacing traditional surgical ligation via thoracotomy. A variety of techniques have been described in dogs, although the technique and implant chosen may depend on the minimum ductus diameter. OBJECTIVES: : To evaluate the feasibility and treatment of choice of catheter closure of large and small PDAs in dogs. METHODS: : In 16 dogs with a PDA, catheter closure was performed using transarterial embolisation using detachable or free coils, or transvenously using an Amplatzer, duct occluder (ADO). RESULTS: : In 8 dogs, closure of PDA with a minimum diameter of < 4 mm was achieved using detachable coils; 2 or more coils were required in 3 dogs. In 5 dogs with minimum ductus diameters of > 4 mm, detachable coils were not applicable. In one of these dogs, (incomplete) surgical ligation was performed and later a free coil placed for complete closure. In 2 dogs with moderately large PDA (5 mm), several free coils were implanted. Complete closure was not achieved in either dog and transient haemolysis occurred as a complication. In 2 dogs with a very large PDA (6 mm), implanted free coils embolised to pulmonary arteries and closure was then achieved using an ADO. In 3 dogs with an excessively large PDA (7.5-10 mm) closure was successfully achieved using an ADO with no complications. CONCLUSIONS: : Coil embolisation is readily feasible for closure of PDA < 4 mm, less feasible for PDA < 5 mm and unlikely to be feasible to close PDA > 5 mm. Detachable coils are safe for PDA < 4 mm, and the ADO is an excellent device for PDA > 5 mm.