Functional health is becoming an important part of outcome assessment following congenital heart surgery.

The Child Health Questionnaire was used to evaluate self-reported functional health in a cohort of children operated on for congenital heart disease between 1996 and 2002, now aged 10–20 years. A total of 288 schoolchildren served as controls. The association between demographic and clinical factors such as the Risk Adjusted Classification for Congenital Heart Surgery, the Aristotle Basic Complexity Score, physical and psycho-social domains was explored by multivariate analysis.

In total 239 children who were operated on (response rate 68%, mean age at assessment 13.1 years, 50% male children) participated. There were no differences between children operated on for congenital heart disease and controls in nine out of thirteen domains. In multivariate analysis, male gender was positively associated with physical, mental and general health. Higher education of the parents was also associated with better scores for family activities, physical, emotional and general health. In contrast, living with a single parent was negatively associated with mental health. Category 4 in the Risk Adjusted Classification for Congenital Heart Surgery was associated with worse scores in all behaviour domains. The Aristotle Basic Complexity Score was not associated with any domain.

Functional health in children operated for congenital heart disease was overall similar to other children of the same age. Male gender of the child, education of the parents, living with a single parent, and category 4 in the Risk Adjusted Classification for Congenital Heart Surgery were important factors for functional health.

To conduct a prospective randomised study to show the efficacy of immune suppression with prednisolone, administered at the 3-month duration of acute myocarditis.

The diagnosis of acute viral myocarditis was made based on echocardiography and serum viral antibodies. The inclusion criterion was acute myocarditis of 3 months duration. In all, 68 of 173 children were available for randomisation into a prednisolone-treated group of 44 and a control group of 24 children. The follow-up period in the prednisolone-treated group was 15.1 plus or minus 9.2 months and 13.6 plus or minus 10.6 months for the control group.

Compared with controls, 1 month after randomisation significantly more children in the prednisolone-treated group increased their ejection fraction to more than 40% (p = 0.029). Discrete analysis of change in the ejection fraction from the one at randomisation to one after 1 month of randomisation of greater than 10% and less than 10% or no change between groups showed a significantly greater number with improvement in the prednisolone-treated group (p = 0.019). At the end of the follow-up visits, a significantly larger number of children in the prednisolone-treated group had an ejection fraction of more than 60% compared with the control group (p = 0.049).

It is concluded that immune suppression with prednisolone, administered at 3 months of the onset of acute myocarditis, is effective in significantly bringing about improvement and cure in persistent left ventricular failure.

The arterial switch operation is the corrective operation for transposition of the great arteries, defined as the combination of concordant atrioventricular and discordant ventriculo–arterial connections, but there have been concerns about silent subendocardial ischaemia on exercise and coronary artery growth. The arterial switch divides the majority of the sympathetic nerves entering the heart; we have studied the effects of coronary flow and sensitivity to catecholamine stimulation in an animal model.

A total of 10 piglets were operated on cardiopulmonary bypass with section and resuturing of aortic trunk, pulmonary artery and both coronary arteries, with 13 sham-operated controls. After 5–7 weeks of recovery, seven simulated switch survivors and 13 controls were studied.

Basal heart rate was significantly higher in switch piglets: in vivo mean (standard deviation) 112 (12) versus sham 100 (10) beats per minute, (p = 0.042); in vitro (Langendorff preparation): 89 (9) versus sham 73 (8) beats per minute (p = 0.0056). In vivo maximal heart rate in response to epinephrine was increased in switch piglets, 209 (13) versus 190 (17) beats per minute (p = 0.044). In vitro dose–response curves to norepinephrine were shifted leftward and upwards (p = 0.0014), with an 80% increase in heart rate induced by 0.095 (0.053) norepinephrine micromole per litre perfusate in switch hearts versus 0.180 (0.035) norepinephrine micromole per litre (p = 0.023). Increase in coronary flow on norepinephrine stimulation and maximal coronary flow were significantly reduced in switch hearts: 0.3 (0.2) versus 0.8 (0.4) millilitre per gram heart weight (p = 0.045) and 2.5 (0.4) versus 3.1 (0.4) millilitre per gram heart (p = 0.030), respectively.

A combination of increased intrinsic heart rate, increased sensitivity to chronotropic actions of norepinephrine, and a decreased maximal coronary flow creates potential for a mismatch between perfusion and energy demands.

To evaluate the safety and efficacy of transcatheter occlusion of the arterial duct without femoral arterial catheterization.

Patent arterial ducts have been closed percutaneously since the 1960s. It remains standard practice to use arterial access for aortography before, during, and after implantation of the device. Femoral arterial catheterisation has well recognised complications, and should be avoided unless absolutely necessary.

We reviewed prospectively collected data relating to 389 occlusions of the arterial duct performed consecutively between 1994 and 2004. We inserted Cook detachable coils in 288 instances using the Amplatzer duct occluder in the remaining 101. Information was obtained regarding procedural success, displacement of the device, and re-intervention. We have followed out patients for a median of 1.15 years in those closed with the Amplatzer device, and 1.09 years in those closed with a coil.

In the patients in whom we used coils, occlusion was possible in 75% using venous access alone. We reintervened in 25 patients, because of embolisation of the device in 6, haemolysis in 5, and residual shunting in 14. On follow-up, complete occlusion had been achieved in 98%. We found trivial stenosis of the left pulmonary artery in 3 patients. When using the Amplatzer device, closure using venous access alone was achieved in 82%, and 2 patients required reintervention because of embolisation of the device. Complete occlusion had been achieved in all patients as judged by follow-up at 1 year, and 2 patients had trivial stenosis of the left pulmonary artery.

Arterial catheterisation is unnecessary in the great majority of patients undergoing occlusion of the arterial duct. Use of venous catheterisation alone is safe, and does not appear to increase the risk of device-related complications.

Specimens of the normal and congenitally abnormal heart have been long preserved, collected, and studied. It is increasingly difficult to add to such pathological collections. These museum pieces are often inaccessible for teaching purposes. Magnetic resonance imaging of old pathological specimens could produce high-resolution unalterable datasets that could be processed to create three-dimensional reconstructions using inexpensive systems that could be used by untrained individuals. To our knowledge, the concept of “Virtual Autopsy” has not been applied to cardiac specimens of museum collections.

To determine optimal sequences and assure specimen safety, five different pulse sequences designed to create three-dimensional datasets were tried on a uterus specimen suspended in a fluid-filled glass container, using a 1.5 Tesla scanner with an eight-channel phased-array coil. Having found the best sequences and established specimen integrity, we scanned six historical heart specimens in their original fluid-filled glass containers. The datasets were processed on a laptop with a DICOM viewer available as freeware.

All specimens were successfully scanned. The best image quality was obtained by using a three-dimensional FSPGR and the BRAVO pulse sequences. High-resolution three-dimensional and multi-planar image processing was possible for all datasets. Detailed examination of the specimens could be easily performed.

Pathological specimens can successfully be scanned in minutes resulting in unalterable and portable high-resolution three-dimensional datasets that can be processed by using inexpensive readily available software. The final cardiac reconstructions can be widely shared for educational and scientific purposes and ensure a lasting access to pathological specimens.

A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve.

A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings.

A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement – 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively – than found at surgery – 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker.

Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.

Staged left ventricular rehabilitation is a novel surgical approach in patients undergoing single ventricle palliation for borderline hypoplastic left cardiac disease, in an attempt to salvage the left ventricle. The procedure includes resection of endocardial fibroelastosis from the left ventricular free wall and apex. We hypothesised that endocardial fibroelastosis removal may significantly affect ventricular conduction and myocardial electrical characteristics.

This study included 27 patients with borderline hypoplastic left cardiac syndrome who underwent staged left ventricle rehabilitation with endocardial fibroelastosis resection following single ventricle palliation. The effect on electrical synchrony was measured by ventricular depolarisation timing (QRS duration) on electrocardiogram. Patients were evaluated for a change in QRS duration before and after fibroelastosis removal and at most recent follow-up.

The QRS change in the immediate period after endocardial fibroelastosis resection ranged from −16 to 36 milliseconds with a median of 0 (p = 0.09). However, long-term conduction delay was common in 44% (12/27) of patients having a QRS duration greater than 98th percentile for the age at the most recent electrocardiogram. Only one patient had QRS duration greater than 98th percentile before any surgical procedure. Two patients developed left bundle branch block and one developed right bundle branch block with left, but anterior-fascicular block. Overall, the QRS duration correlated with left ventricular size (R = 0.54, p = 0.006) at the most recent electrocardiogram.

Electrical dyssynchrony is a common finding in patients undergoing staged left ventricular rehabilitation after single ventricle palliation; however, it is not acutely related to surgical endocardial resection. Left ventricular size is correlated with QRS duration. Diligent follow-up is required to evaluate the effects of left ventricular growth and consideration of resynchronisation in this population.

This study was undertaken to review the spectrum and surgical outcome of adolescents and adults with congenitally malformed hearts from January, 1993 to December, 2008. The lack of data on this emerging problem from the West African sub-region prompted this report.

This retrospective study is based on 135 adolescents and adults with congenitally malformed hearts. A review of their case notes and operative records was carried out and results analysed.

Selected patients made up 23% of all congenital cardiac surgeries performed at our institution in the same period. A total of 23 patients (17%) were non-Ghanaian West Africans. There was a female preponderance of 53.3%. The ages ranged from 16 to 70 years (mean 28.6 plus or minus 10.3 years). The mean follow-up was 7.5 plus or minus 4.4 years. Patients were functionally classified (New York Heart Association) as class I (23%), II (58%), and III (19%). In 14 (10.4%) patients, the defects were discovered incidentally. Ventricular septal defects, oval fossa type atrial septal defects, Fallot’s tetralogy, and patent arterial duct together accounted for 77.8% of the cases. Surgical correction was undertaken in 117 (86.7%) patients; the remainder had palliative procedures. There were six (4.3%) reoperations. The functional class improved to class I or II in 95% of patients within the first postoperative year. The overall hospital mortality was 3% with two late deaths (1.5%).

The study demonstrates the feasibility of surgery for adolescents and adults with congenitally malformed hearts in the sub-region with a good outcome. Majority (77.8%) of patients present with less complex lesions.

Correction of tetralogy of Fallot has excellent long-term results. The present retrospective study investigates the indications for reoperation late after corrective surgery.

Data from 914 consecutive cases who underwent correction of tetralogy of Fallot in our department between 1960 and 2002 were retrospectively reviewed and analysed. In 91 patients, a total of 102 reoperations were performed late after repair.

The mean time interval between corrective surgery and the first reoperation was 12.8 years. The main indication for reoperation was residual ventricular septal defect in nearly half of the cases, mostly isolated, but also in combination with a right ventricular outflow tract aneurysm or pulmonary stenosis. One-fourth of reoperated patients underwent a procedure on their pulmonary artery or pulmonary valve: replacement of pulmonary valve, replacement of primary implanted pulmonary artery conduits with or without concomitant surgery, and surgery for isolated peripheral pulmonary stenosis. The remaining indications were right ventricular outflow tract aneurysms and others. Aneurysms of the right ventricular outflow tract were seen mostly after the use of autologous – untreated – pericardial patch in 18 of 21 cases.

The number of reoperations for residual ventricular septal defect decreased during the study period. The primary use of conduits led to an increased number of reoperations for conduit exchange due to degeneration or failure. Use of an untreated autologous pericardial patch for enlargement of the right ventricular outflow tract should be avoided due to increased risk for aneurysm formation.

Sildenafil is increasingly being used in the management of pulmonary arterial hypertension in the newborn. Its role in patients with congenital cardiac disease is less well defined and as yet has only been reported sporadically.

Present our experience with sildenafil treatment in patients with a failing Fontan circulation.

Retrospective review of 13 symptomatic patients after Fontan palliation who received treatment with sildenafil between January, 2006 and July, 2008.

Three patients suffered from protein-losing enteropathy, four patients presented with bronchial casts, two had severe cyanosis after fenestrated Fontan procedure, two had prolonged chylous effusions, one had a previous failure of Fontan and take-down, and one patient had arrhythmias and end-stage cardiac failure requiring conversion to an extra-cardiac Fontan. Sildenafil was used in the dosage of 1–2 milligrams per kilogram 3–4 times per day. Protein-losing enteropathy and α-1-antitrypsin levels improved in all three patients on sildenafil treatment. One of these patients had a concomitant catheter creation of a fenestration, as did two patients presenting with bronchial casts and both patients with persistent chylous effusions. All four patients with bronchial casts and two patients with cyanosis improved significantly on sildenafil treatment. Chylous effusions decreased after sildenafil and stent enlargement of a fenestration. There were no significant side effects requiring sildenafil withdrawal over a treatment period ranging from 2 months to 2 years.

Sildenafil can be used safely and effectively in the treatment of patients with a failing Fontan circulation.