Elhadi H. Aburawi

Surgical and transcatheter (Amplatzer) closure of atrial septal defects: a prospective comparison of results and cost

Objective: To compare effectiveness, complications, and cost of Amplatzer with surgical atrial septal defect (ASD) closure. Design: Prospective study. Setting: Tertiary cardiac referral centre. Patients: 43 consecutive patients (excluding non-UK residents) aged between 2.1 and 56.8 years (median 7) undergoing ASD closure. Main outcome measures: Procedural success, complications, regression of right ventricular dilatation (up to one year postprocedure), cost, inpatient stay, and home convalescent time. Results: Amplatzer ASD closure was successful in 24 of 27 (89%) patients. Surgical closure was successful in all 19 cases. Cardiac complications affecting management occurred in three (11%) of the Amplatzer group (two procedural failures, one device embolisation) and 4 of 19 (21%) surgical patients (one pericardial pain, one global pericardial effusion requiring drainage, and one patient with anaemia requiring haematinics in addition to an incidental pericardial effusion and one further incidental pericardial effusion) (p = NS). There were complications that did not affect management in a further 5 of 19 surgical patients. There was no significant difference in regression of right ventricular dilatation by six months postprocedure (median right ventricular end diastolic diameter decrease: Amplatzer group 17.5%, surgical group 15.1%; median cardiothoracic ratio decrease: Amplatzer 7.9%, surgical 7.5%). Both hospital stay and home convalescent times were significantly shorter after Amplatzer closure (median hospital stay: Amplatzer one day, surgery six days; median convalescent time: Amplatzer two weeks, surgery 5.5 weeks). Median cost was similar for both groups (Amplatzer £5375, surgical £5412). Conclusions: Amplatzer ASD closure has a lower chance of success with a single procedure than surgery. Overall, there were more complications in the surgical group but the majority of these were minor and did not require any change in management. Resolution of right ventricular dilatation over the study period was similar for both techniques. Time spent in hospital and away from work or school was shorter for the Amplatzer group. The cost of both techniques was similar.

Persistent wandering atrial pacemaker after epinephrine overdosing – a case report

BackgroundLong-term complications of sympathomimetic drug overdosing have not been adequately investigated in infants and young children. Despite reports discouraging their use in children, these formulations are frequently administered for “cold-like symptoms”. Their frequent adverse events are different forms of arrhythmias, including multifocal atrial tachycardia.Case presentationA 3-year-old toddler developed multifocal atrial tachycardia following an iatrogenic overdose of epinephrine accidentally administered intravenously. His ECG showed wandering atrial pacemaker (p-waves with different origins and configurations) that persisted for at least one year. This event demonstrated the sensitivity of young children to the sympathomimetic drugs, especially overdosing.ConclusionsHealth care providers and parents should be warned of toxicities associated with sympathomimetic drug overdosing. Future studies are needed to determine whether wandering atrial pacemaker is a potential long-term complication of high-dose sympathomimetics.

Residual adverse changes in arterial endothelial function and LDL oxidation after a mild systemic inflammation induced by influenza vaccination

Background. Several clinical studies have suggested possible increase in cardiovascular risk during and in the first weeks after an acute inflammatory disease. Using influenza vaccine as inflammatory stimulus, we investigated whether arterial endothelial dysfunction could persist beyond the inflammatory state, and whether amplified oxidative modification of low‐density lipoprotein (LDL) accompanies this vascular disturbance. Methods and subjects. The brachial artery responses to hyperemia (flow‐mediated dilatation (FMD), and to sublingual glyceryl trinitrate (GTN), and the carotid intima‐media thickness were assessed by external ultrasound in eight healthy male volunteers (age 17–30 y) before, and 2 and 14 days after intramuscular administration of influenza vaccine. Plasma levels of high‐sensitivity C‐reactive protein (CRP), fibrinogen, cyclic guanosine monophosphate (cGMP), and antibodies against oxidized LDL (oxLDL) were measured at each time point. Data are means±standard errors of the mean (SEM). Results. Influenza vaccination caused a slight elevation in CRP (from 0.5±0.1 at baseline, to 2±0.6 mg/L, P = 0.01) and fibrinogen (from 2.3±0.1 to 2.7±0.1 g/L, P = 0.01) at 2 days, which completely resolved at 14 days (CRP: 0.6±0.2 mg/L, P = 0.9, and fibrinogen: 2.3±0.1 g/L, P = 0.8 versus baseline). OxLDL antibody levels rose significantly at 2 days (from 1±0.1 at baseline to 2±0.4, P = 0.04), and remained elevated at 14 days (1.7±0.3, P = 0.1 versus baseline). FMD of the brachial artery decreased at 2 days (from 8.3±1.2% at baseline, to 5.4±1%, P = 0.05) with a further decrease at 14 days (4.9±0.8%, P = 0.03 versus baseline). The dilatory responses to GTN and the carotid IMT remained unchanged throughout the study period (P>0.5). Conclusion. Abnormalities in arterial function and LDL oxidation may persist for at least 2 weeks after a slight inflammatory reaction induced by influenza vaccination. These could explain in part the earlier reported increase in cardiovascular risk during the first weeks after an acute inflammatory disorder.

TECRL, a new life‐threatening inherited arrhythmia gene associated with overlapping clinical features of both LQTS and CPVT

Genetic causes of many familial arrhythmia syndromes remain elusive. In this study, whole‐exome sequencing (WES) was carried out on patients from three different families that presented with life‐threatening arrhythmias and high risk of sudden cardiac death (SCD). Two French Canadian probands carried identical homozygous rare variant in TECRL gene (p.Arg196Gln), which encodes the trans‐2,3‐enoyl‐CoA reductase‐like protein. Both patients had cardiac arrest, stress‐induced atrial and ventricular tachycardia, and QT prolongation on adrenergic stimulation. A third patient from a consanguineous Sudanese family diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT) had a homozygous splice site mutation (c.331+1G>A) in TECRL. Analysis of intracellular calcium ([Ca2+]i) dynamics in human induced pluripotent stem cell‐derived cardiomyocytes (hiPSC‐CMs) generated from this individual (TECRLHom‐hiPSCs), his heterozygous but clinically asymptomatic father (TECRLHet‐hiPSCs), and a healthy individual (CTRL‐hiPSCs) from the same Sudanese family, revealed smaller [Ca2+]i transient amplitudes as well as elevated diastolic [Ca2+]i in TECRLHom‐hiPSC‐CMs compared with CTRL‐hiPSC‐CMs. The [Ca2+]i transient also rose markedly slower and contained lower sarcoplasmic reticulum (SR) calcium stores, evidenced by the decreased magnitude of caffeine‐induced [Ca2+]i transients. In addition, the decay phase of the [Ca2+]i transient was slower in TECRLHom‐hiPSC‐CMs due to decreased SERCA and NCX activities. Furthermore, TECRLHom‐hiPSC‐CMs showed prolonged action potentials (APs) compared with CTRL‐hiPSC‐CMs. TECRL knockdown in control human embryonic stem cell‐derived CMs (hESC‐CMs) also resulted in significantly longer APs. Moreover, stimulation by noradrenaline (NA) significantly increased the propensity for triggered activity based on delayed afterdepolarizations (DADs) in TECRLHom‐hiPSC‐CMs and treatment with flecainide, a class Ic antiarrhythmic drug, significantly reduced the triggered activity in these cells. In summary, we report that mutations in TECRL are associated with inherited arrhythmias characterized by clinical features of both LQTS and CPVT. Patient‐specific hiPSC‐CMs recapitulated salient features of the clinical phenotype and provide a platform for drug screening evidenced by initial identification of flecainide as a potential therapeutic. These findings have implications for diagnosis and treatment of inherited cardiac arrhythmias.

Down Syndrome and congenital heart disease : why the regional difference as observed in the Libyan experience?

Background Children with Down syndrome (DS) have about a 40 to 50% incidence of congenital heart disease (CHD). The objectives of this study were to evaluate the distribution and frequency of CHD patterns in Libyan children with DS. Methods All patients with DS who were referred to the cardiology clinic between January 1995 and December 2008 were reviewed. Results Of the 1 193 patients reviewed, 537 (45%) had an associated CHD. Overall there were 349 (65%) patients who had a single cardiac lesion, and 188 (35%) had multiple cardiac lesions. The most common isolated cardiac lesion was atrial septal defect (ASD), found in 125 (23%) patients, followed by atrioventricular septal defect (AVSD) in 103 (19%), and ventricular septal defect (VSD) in 76 (14%). Conclusion Atrial septal defect was the most common cardiac lesion. The distribution of CHDs in Libyan children with DS was similar to what has been reported internationally, but the frequency was not compared with international rates.

Consultation on the Libyan health systems: towards patient-centred services

The extra demand imposed upon the Libyan health services during and after the Libyan revolution in 2011 led the ailing health systems to collapse. To start the planning process to re-engineer the health sector, the Libyan Ministry of Health in collaboration with the World Health Organisation (WHO) and other international experts in the field sponsored the National Health Systems Conference in Tripoli, Libya, between the 26th and the 30th of August 2012. The aim of this conference was to study how health systems function at the international arena and to facilitate a consultative process between 500 Libyan health experts in order to identify the problems within the Libyan health system and propose potential solutions. The scientific programme adopted the WHO health care system framework and used its six system building blocks: i) Health Governance; ii) Health Care Finance; iii) Health Service Delivery; iv) Human Resources for Health; v) Pharmaceuticals and Health Technology; and vi) Health Information System. The experts used a structured approach starting with clarifying the concepts, evaluating the current status of that health system block in Libya, thereby identifying the strengths, weaknesses, and major deficiencies. This article summarises the 500 health expert recommendations that seized the opportunity to map a modern health systems to take the Libyan health sector into the 21st century.

Effects of Balloon Valvuloplasty on Coronary Blood Flow in Neonates With Critical Pulmonary Valve Stenosis Assessed With Transthoracic Doppler Echocardiography.

BACKGROUND Treating pulmonary valve stenosis with balloon valvuloplasty (BV) is a good model to study the effect of right ventricular (RV) pressures on coronary flow. METHODS Transthoracic Doppler echocardiography was used to register coronary flow in 10 age-matched healthy controls and 7 neonates before and 1 day after BV. RESULTS Left ventricular fractional shortening and cardiac output increased significantly after BV. Right coronary artery diameter decreased from 1.2 +/- 0.2 to 1.1 +/- 0.1 mm (P < .02). Posterior descending coronary artery flow parameters decreased significantly, with blood flow decreasing from 8.4 to 5.7 +/- 1.9 mL/s (P < .003). RV end-diastolic pressure and RV systolic pressure explained almost totally the variation in coronary flow (r(2) = 0.87). CONCLUSIONS RV end-diastolic pressure and RV systolic pressure determined coronary flow in neonates with critical pulmonary valve stenosis. Cardiac output and left ventricular fractional shortening increased after pulmonary valve BV.

Coronary flow before and after surgical versus device closure of atrial septal defect.

BACKGROUND Cardiopulmonary bypass (CPB) affects coronary flow after the operation. Surgical as compared to device closure of atrial septal defect (ASD) serves as a good model to clarify the effects of surgery with CPB on coronary flow. METHODS Coronary flow parameters were determined by transthoracic Doppler echocardiography before and after ASD closure. Thirteen children underwent surgery on CPB and fourteen children had device closure of their ASD under interventional cardiac catheterisation. Fourteen age-matched healthy controls were studied. RESULTS Left ventricular fractional shortening increased and cardiac output increased after the device closure but there were no significant changes after the surgery. After the surgery the mean diameter of left anterior descending coronary artery increased from 1.7 +/- 0.6 to 2.1 +/- 0.4 mm (p = 0.03), the peak flow velocity in diastole (PFVd) from 48 +/- 10 to 70 +/- 12 cm/s (p = 0.0001) and basal blood flow (BF) from 62 +/- 18 to 105 +/- 35 ml/min (p = 0.0001). Flow parameters in the right coronary artery increased similarly. In contrast, all coronary flow parameters decreased substantially after catheter interventions, but still remained significantly elevated as compared with controls. CONCLUSIONS Surgery with cardiopulmonary bypass but not the device closure affects coronary flow beyond the pure effects of anatomical correction. Cardiac output increases after the device closure. The reported decrease of coronary flow reserve is obviously due to increased basal coronary flow.

Pathophysiology of coronary blood flow in congenital heart disease

OBJECTIVES The aim was to investigate the effects of volume and pressure overload and increased coronary perfusion pressure on coronary flow (CF) in congenital heart disease (CHD) patients. BACKGROUND The effects of CHD on CF are poorly mapped. METHODS A total of 65 patients with acyanotic CHD and 49 age-matched healthy controls were examined by transthoracic Doppler echocardiography. Posterior descending artery flow was measured in patients with pulmonary valve stenosis (PS) and atrial septal defects (ASDs) i.e. in lesions with right ventricular pressure or volume overload, and left anterior descending artery flow in patients with coarctation of the aorta (CoA) and ventricular septal defect (VSD), in lesions with left ventricular pressure or volume overload. The CF data in each patient group were expressed as the percent of the median for healthy controls from the same age group. RESULTS The CF values were in VSD 172%, ASD 185%, PS 233%, and CoA 773% patients. In CoA patients body surface area (r=0.90, p<0.0001), systolic blood pressure (r=0.72, p<0.0001), diastolic blood pressure (r=0.77, p<0.0001), systolic wall tension (r=-0.77, p=0.004), and signs of inflammation (log CRP, r=-0.75, p=0.007) correlated with CF. CONCLUSIONS The increase in CF and velocity was most significant in patients with CoA. In newborns, increased coronary perfusion pressure seems to be the most important factor for increased CF, even if the pressure is not assumed to cause a significant increase in flow over the auto-regulatory range of 70-130mmHg. We also showed that inflammation decreases CF.

Lowered levels of serum albumin and HDL-cholesterol in children with a recent mild infection.

BACKGROUND. Infections are associated with atherogenic changes in serum. AIM. To elucidate effects of recent infections on risk factors for coronary heart disease in children. SUBJECTS AND METHODS. In 1983 and again 3 years later, 2458 individuals aged 9, 12, 15, 18 and 21 years were investigated. In 1986, 106 subjects had symptoms of infection during the past 2 weeks before their follow‐up visit. Their serum albumin and lipid concentrations were compared to those in 1983 when these individuals probably were healthy. An age‐ and sex‐matched healthy control group from the cohort 1986 was chosen for comparison. For cholesterol age, sex and body mass index specific Z‐scores in addition to actual values were used in statistical comparisons. RESULTS. Serum albumin was 42 g/L in subjects with positive history of infection and 46 g/L in healthy controls (P<0.0001). HDL‐cholesterol and the ratio of HDL‐ to total cholesterol were lower with increasing evidence of infection. Elevated serum C‐reactive protein (CRP) or orosomucoid grouped the subjects with high and low serum HDL‐cholesterol concentrations better than history of infection alone. CONCLUSION. A mild infection lowers serum HDL‐cholesterol and serum albumin concentrations, which both favour atherogenesis.