Anthony P. Salmon

Implementing transition: Ready Steady Go.

There is good evidence that morbidity and mortality increase for young persons (YP) following the move from paediatric to adult services. Studies show that effective transition between paediatric and adult care improves long-term outcomes. Many of the issues faced by young people across subspecialties with a long-term condition are generic. This article sets out some of the obstacles that have delayed the implementation of effective transition. It reports on a successful generic transition programme ‘Ready Steady Go’ that has been implemented within a large National Health Service teaching hospital in the UK, with secondary and tertiary paediatric services, where it is now established as part of routine care.

Long-term follow-up after primary complete repair of common arterial trunk with homograft: A 40-year experience

BACKGROUNDnWe sought to determine the long-term performance of homograft and truncal valve after complete repair of common arterial trunk.nnnMETHODSnFrom January 1964 to June 2008, 32 patients (median age, 14 days; range, 5 days to 2.5 years) underwent primary homograft repair of common arterial trunk. Twenty-four (75%) were neonates. The homograft used in the right ventricular outflow tract was aortic in 24 patients and pulmonary in 8 patients (mean diameter, 15.8 +/- 3.5 mm; median diameter, 16 mm [range, 8-24 mm]). The median follow-up was 24.5 years (range, 5.6 months to 43.5 years).nnnRESULTSnThere were 3 hospital deaths and 1 late death. The actuarial survival at 30 years was 83.1% +/- 6.6%. Of the 28 survivors, 25 reoperations were performed in 19 (76%) patients. The mean and median times to homograft reoperation were 11.5 +/- 7.4 and 12.1 years (range, 1.0-26.1 years), respectively. Overall freedom from homograft reoperation after 10, 20, and 30 years was 68.4% +/- 8.7%, 37.4% +/- 9.5%, and 26.7% +/- 9.3%, respectively. Twelve patients retained the original homografts at a median follow-up of 16.4 years (range, 0-30.2 years). Six underwent a truncal valve replacement with a mechanical prosthesis at a median of 10.5 years (range, 3.4-22 years) after truncus repair. Freedom from truncal valve replacement at 10 and 30 years was 93.1% +/- 4.7% and 81.8% +/- 8.9%, respectively. In the 22 surviving patients who did not undergo truncal valve replacement, the peak truncal valve gradient was 8.9 +/- 8.3 mm Hg at a median follow-up of 24.5 years (range, 5.6 months to 32.9 years). At the last follow-up, 27 (96.4%) patients had good left ventricular function, and 24 patients (85.7%) were New York Heart Association class I.nnnCONCLUSIONSnOversizing the homograft at the time of the initial repair can lead to a homograft lasting more than 12 years. During long-term follow-up, 20% of patients require truncal valve replacement.

Primary Biventricular Repair of Atrioventricular Septal Defects: An Analysis of Reoperations

BACKGROUNDnThe purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair.nnnMETHODSnBetween April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle.nnnRESULTSnThere was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients.nnnCONCLUSIONSnDown syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

The outcome of antibiotic sterilised aortic homografts used in the Fontan procedure

Between 1977 and 1988, 27 patients between the ages of 4 and 22 years (mean 8.9) underwent a Fontan procedure with the use of an antibiotic sterilised aortic homograft. There were 15 patients with tricuspid atresia, 9 with univentricular heart and 3 others. The homograft was anastomosed to a right-sided pulmonary artery in ten, to a left-sided main pulmonary artery in eight and in nine patients the homograft connected the right atrium to the right ventricle. There were five early deaths (18.5%) unrelated to the homograft and two late deaths at 7 and 10 years postoperatively. Five patients have required removal of the calcified obstructed homograft with no death. In three patients patches were inserted, but in two patients with good right ventricles a second homograft was inserted. In three of the re-operated patients the homograft lay directly behind the sternum and the femoral artery was exposed and in two of them the femoral artery was cannulated before the chest was opened to control haemorrhage. Of the patients 74% are alive up to 15 years later, 15 with their original homograft. Eight (57%) of those still have their original homograft more than 10 years post-operatively.

Midterm Evaluation of Biological Prosthetic Valves in the Pulmonary Position of Grown-Up Patients

OBJECTIVESnTo examine the midterm clinical outcome of pulmonary valve replacement (PVR) with prosthetic valves.nnnMETHODSnWe reviewed 37 consecutive patients who underwent PVR with biological prosthetic valves between September 1999 and June 2010. The median age was 22.6 years (range: 6 to 70 years; three children). The primary diagnosis was Tetralogy of Fallot in 20 patients (54%). Valve pathology was regurgitation in 27 patients (72.9%). Cardiac surgery had been previously performed in 35 patients (94.5%). The median size of the prosthesis was 25 mm (range: 21 to 31 mm). The median follow-up was 42 months (range: 1.2 to 129 months).nnnRESULTSnThere were no early valve-related deaths. Hospital mortality was 2.7% (n = 1) and no patient required early rereplacement of prosthesis. Two patients required permanent pacemaker insertion. During follow-up, there was no late death, reoperation for structural valve degeneration, or valve thrombosis. Only one patient required repeated operation for endocarditis at 37 months follow-up. The actuarial survival at 5 years was 95.1 ± 3.8%. Overall freedom from reoperation after PVR at 5 years was 93.0 ± 8.6%. At last follow-up, 34 patients (91.8%) were NYHA class I versus 20 patients (54%) preoperatively (p < 0.05). In the 35 surviving patients who did not undergo redo-PVR, there was no-mild regurgitation and the peak PV gradient was 20.4 ± 10.2 mm Hg (16.2 ± 9.3 mm Hg preoperatively). Thirty-two patients (91.4%) had good right ventricular function compared with 26 patients (74.2%) preoperatively.nnnCONCLUSIONSnPVR with biological prosthetic valves can be performed with good midterm survival, functional status, and haemodynamics.

Esmolol-Assisted Balloon and Stent Angioplasty for Aortic Coarctation

The objective of this study was to evaluate the effectiveness and safety of esmolol-induced negative ino- and chronotropism during stent/balloon angioplasty for aortic coarctation. Balloon angioplasty and stent placement have become widely accepted therapies for native and recurrent coarctation of the aorta (CoA). Trauma to the vessel wall and stent migration related to forward displacement of the balloon and/or stent by cardiac output, are the most common complications. Controlling stroke volume and heart rate may assist in balloon stability and accurate deployment of stents. All methods currently used to achieve this have significant limitations. We describe our experience using esmolol to control stroke volume and heart rate during balloon/stent angioplasty of CoA. We performed a retrospective review of all patients who had intravenous esmolol during percutaneous treatment of CoA. Six interventions were performed in six patients: coarctation stent angioplasty in five patients (two native coarctation) and balloon angioplasty alone in one patient. The median systolic blood pressure achieved during the procedure was 65 mmHg (range, 57–75) representing a median reduction of 40 mmHg (range, 20–80; p = 0.008) from baseline. The median heart achieved was 50 beats/min (range, 20–80), representing a median reduction of 20 beats/min (range, 15–90, p = 0.048) from baseline. Optimal stent position was obtained in all patients. Intravenous esmolol controls periprocedural hemodynamics effectively and safely during percutaneous therapy for aortic coarctation, thereby aiding accurate stent placement. Further evaluation of its use during other percutaneous left heart interventions is required.

Intrapericardial giant left atrial appendage

Contrary to what the name implies, the left atrial appendage is increasingly becoming an organ of clinical importance. Its role in the pathogenesis of thromboembolism is now well established. Enlargement of the appendage due to haemodynamic disturbances or anatomic weakness increases this risk, along with the potential for arrhythmia. We report a rare case of gross enlargement of the left atrial appendage within the pericardial cavity.

Hypothermia and Respiratory Distress Syndrome

Moderate hypothermia is widely used to reduce the risk of cellular damage intraoperatively during cardiopulmonary bypass, and deep hypothermia is used for cerebral protection during periods of circulatory arrest during pediatric cardiac surgery. These techniques are based on the principle that there is a progressive fall in oxygen consumption with reduction in temperature. In 1980 Reasbeck et al. [7] described a case of intractable right ventricular failure following cardiac surgery in a 7-month-girl that responded dramatically to whole-body hypothermia. In the Wessex Cardiothoracic Center, moderate hypothermia has been used postoperatively to treat refractory low cardiac output states. In 1992 Moat et al. [6] reviewed the results of the use of this technique for management of 20 infants and children following cardiac surgery between the period July 1986 and June 1990. Hypothermia was induced in each case by surface cooling with a thermostatically controlled water blanket. They concluded that it was a useful adjunct to other therapeutic interventions in the management of these sick children. The following report describes the case of an infant who suffered respiratory distress syndrome (RDS) in our unit. Moderate induced hypothermia was used for 48 hours in addition to standard treatment for this infant. The use of hypothermia in the management of RDS in a neonate has not been previously reported.

A hole in the heart: a hole in the head?

A physiological left-to-right shunt through a patent foramen ovale (PFO) can be demonstrated in 32–55% of young adults with unexplained stroke compared with 10–35% of the general population.1 Transoesophageal or transthoracic echocardiographic evidence of a PFO consists of demonstration of a defect in the wall between the atria and ideally the passage of bubble contrast from right to left at this level. Provocative measures may be used to raise right atrial pressure, such as coughing, Valsalva manoeuvre or pressure on the liver, and increase the detection rate of PFO by at least 25%, but are not always performed rigorously or even routinely in children. The characteristics of the PFO, such as the size of the defect and of the shunt, are also important considerations; associated atrial septal aneurysm, larger defects and those with a greater right-to-left shunt may carry a higher risk of recurrence, particularly in patients with cryptogenic stroke. However, prospective population-based studies have failed to confirm the association (even with atrial septal aneurysm), a recent study failed to find any link with recurrent stroke2 and there are very few data in children. Despite this, studies of the effect of closure of PFO detected echocardiographically with devices placed at catheterisation are in progress,3 and such procedures have been advocated in children who have had a stroke.4nnTranscranial Doppler (TCD) with bubble contrast and Valsalva appears to be a more sensitive technique than echocardiography for detecting PFO in adults.5 Benidik et al s careful bubble contrast TCD study published in this issue6 (in …