Anomalous origin of the left coronary artery from pulmonary artery constitutes 0.5% of all CHD (Boutsikou M, Shore D, Li W, et al, Int J Cardiol 261: 49–53, 2018). Fifteen percent survive into adulthood undiagnosed and 90% present with sudden death (Yau JM, Singh R, Halpern EJ, Fischman D, Clin Cardiol 34: 204–210, 2011; Alexi-Meskishvili V, Berger F, Weng Y, Lange PE, Hetzer R, J Card Surg 10: 309–315, 1995). We describe an enigmatic case of a 29-year-old female who presented after an aborted cardiac arrest and was diagnosed with anomalous origin of the left coronary artery from pulmonary artery.

Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

Takayasu arteritis is an idiopathic chronic granulomatous panarteritis predominantly affecting the aorta and its main branches. Although idiopathic, genetic contribution to disease susceptibility is being increasingly recognised. Rare in children, Takayasu arteritis is a worldwide disease with significant morbidity and mortality. Its diagnosis is a challenge and requires awareness of the condition as clinical features at presentation are non-specific and assessing disease activity is difficult. In the inflammatory stage, treatment is essential to prevent the insidious course and vascular damage: stenotic, occlusive lesions, aneurysms, and aortic regurgitation. New imaging modalities, such as CT scan, MRI, and 18F-fluorodeoxyglucose positron emission tomography, have expanded the possibilities for non-invasive diagnosis and monitoring; however, digital subtraction arteriography remains the gold standard for the diagnosis of Takayasu arteritis. Steroids are the first-line medical treatment. The combined use of methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, and biological agents is common. Revascularisation therapy should be considered in uncontrolled hypertension secondary to renal artery stenosis, symptomatic coronary ischaemia, cerebrovascular disease, severe aortic regurgitation, limb ischaemia, and aneurysms at risk of rupture, using surgical or endovascular procedures and taking into consideration that complications, especially restenosis, are frequent. Disease activity increases the likelihood of complications after revascularisation. Surgical intervention has shown better long-term outcomes, although the endovascular approach is evolving. The aim of this review was to describe key points of the diagnosis, treatment, and follow-up of Takayasu arteritis in childhood.

Congenital atresia of the left coronary artery is a very rare condition. We report a case of a 13-year-old boy who was followed-up at our outpatient clinic from 6 months of age because of heart murmur. He had no symptoms for more than 10 years, and his left ventricular contraction was normal. Exercise electrocardiogram was performed at 7 and 10 years of age, and ST depression was not observed. He presented with chest pain and syncope on exertion at the ages of 11 and 12 years, respectively, and ST depression on exercise electrocardiogram was detected for the first time at 13 years of age. Cardiac echocardiography showed a high-choic antero-lateral papillary muscle of the mitral valve, prolapse of the mitral anterior leaflet, and mild mitral valve regurgitation. Coronary angiography did not demonstrate antegrade left coronary flow or dilation of the right coronary artery. We performed coronary artery bypass grafting using the left internal thoracic artery. Postoperatively, the ST depression resolved, and his exercise tolerability improved.

The function of the heart was studied in 20 asphyxiated term neonates by measuring the longitudinal peak systolic strain and peak systolic strain rate by tissue Doppler in 18 segments of the heart on days 1, 2, and 3 of life. The fractional shortening was assessed at each examination as well. Measurements were compared against measurements in 48 healthy term neonates examined by the same protocol. The function of the heart was lower in the asphyxiated neonates – peak systolic strain (mean (95% confidence interval) −19.4% (−20.4, −18.5), peak systolic strain rate −1.65 (−1.74, −1.56) per second) than in the healthy term neonates (peak systolic strain −21.7% (−22.3, −21.0), peak systolic strain rate −1.78 (−1.84, −1.74) per second; p < 0.001). Fractional shortening was similar in the asphyxiated (29.2% (26.8, 31.5)) and healthy term neonates (29.0% (27.9, 30.1); p = 0.874). The peak systolic strain differed significantly between the asphyxiated and healthy term neonates for the left basal and right basal groups of segments (p < 0.05) but not for the left apical, right apical, septum apical, or septum basal groups of segments. The peak systolic strain rate differed significantly only for the septum apical group of segments. The differences were largest on the second day of life. Measurements were similar in asphyxiated neonates with elevated and normal cardiac troponin T levels. The peak systolic strain and strain rate were in this study more sensitive indices than fractional shortening for assessing the reduced myocardial function in asphyxiated term neonates.

We have previously shown that dietary red palm oil (RPO) supplementation improves functional recovery in hearts subjected to ischaemia–reperfusion. However, little knowledge exists concerning the effects of RPO supplementation of a high-cholesterol diet on ischaemia–reperfusion injury. The signalling mechanisms responsible for RPO's effects in the presence of cholesterol also remain to be elucidated. Therefore, the aim of the present study was to examine the effects of RPO, given with a high-cholesterol diet, on mitogen-activated protein kinase (MAPK) phosphorylation and apoptosis. Long–Evans rats were fed a control diet, a control diet containing 2 % cholesterol, or a control diet containing 2 % cholesterol and 7 g RPO per kg (CRPO) for 5 weeks. Hearts were excised and mounted on an isolated working heart perfusion apparatus. Cardiac function was measured after which hearts were freeze-clamped and used to assess MAPK phosphorylation and to evaluate apoptosis. Cholesterol supplementation caused a poor aortic output (AO) recovery compared with the control group (35·5 (sem 6·2) v. 55·4 (sem 2·5) %), but when RPO was added, the percentage AO increased significantly. The cholesterol group's poor AO was associated with a significant increase in p38-MAPK phosphorylation, whereas the CRPO-supplemented group showed as significant reduction in p38-MAPK phosphorylation when compared with the cholesterol-supplemented group. This significant reduction in p38-MAPK was also associated with reduced apoptosis as indicated by significant reductions in caspase-3 and poly(ADP-ribose) polymerase cleavage.

Patients with anomalous origin of the left coronary artery from the pulmonary trunk may present with paroxysmal angina on exertion, congestive heart failure, dyspnoea, syncope or sudden death. The association of such anomalous origin of the left coronary artery from the pulmonary trunk with a hypertrophic left ventricle is extremely rare. In our cohort of patients with anomalous origin of the left coronary artery from the pulmonary trunk, two presented with a hypertrophic left ventricle. We discuss these cases, accompanied by a review of the English literature describing different morphological anomalies of the coronary arteries associated with left ventricular hypertrophy. Whether the hypertrophy is a result of the evolvement of the collateral coronary system, or due to an additional pathological gene for hypertrophic obstructive cardiomyopathy, remains an enigma. The hypertrophy may have served as a compensatory mechanism accounting for the atypical clinical presentation. We further discuss the possible factors associating the occurrence of anomalous origin of the left coronary artery from the pulmonary trunk and myohypertrophy, supported by the documentation of the regression of hypertrophy following surgical correction of the anomalous arterial origin as seen in our patients.

Cortical spreading depression (CSD) produces propagating waves of transient neuronal hyperexcitability followed by depression. CSD is initiated by K+ release following neuronal firing or electrical, mechanical or chemical stimuli. A triphasic (30–50 s) cortical potential transient accompanies localized transmembrane redistributions of K+, glutamate, Ca2+, Na+, Cl− and H+. Accumulated K+ in the restricted interstitial space can cause both further neuronal depolarisation and inward movement of K+ into astrocytes that buffers this increased extracellular K+ concentration ([K+])o. However, astrocyte interconnections may then propagate the CSD wave by K+ liberation through an opening of remote K+ channels by volume, Ca2+ or N-methyl-D-aspartate receptor activation. Changes in cerebral blood volume and in apparent water diffusion co-efficient (ADC) accompanying CSD were first studied using magnetic resonance imaging (MRI) in whole lissencephalic brains. Diffusion-weighted echoplanar imaging in gyrencephalic brains went on to demonstrate CSD features that paralleled classical migraine aura. The ADC activity persisted minutes/hours post KCl stimulus. Pixelwise analyses distinguished single primary events and multiple, spatially restricted, slower propagating, secondary events whose detailed features varied with the nature of the originating stimulus. These ADC changes varied reciprocally with T2*-weighted (i.e. referring to spin-spin relaxation times) waveforms reflecting local blood flow. There followed prolonged decreases in cerebral blood flow culminating in late cerebrovascular changes blocked by the antimigraine agent sumatriptan. CSD phenomena have possible translational significance for human migraine aura and other cerebral pathologies such as the peri-infarct depolarisation events that follow ischaemia and brain injury.

Brain injury following cerebral ischaemia (stroke) involves a complex combination of pathological processes, including excitotoxicity and inflammation leading to necrotic and apoptotic forms of cell death. At the cellular level, excitotoxicity is mediated by glutamate and its cognate receptors, resulting in increased intracellular calcium and free radical production, and eventual cell death. Recent evidence suggests that scaffolding molecules that associate with glutamate receptors at the postsynaptic density allow coupling of receptor activity to specific second messengers capable of mediating excitotoxicity. These findings have important implications in the search for effective neuroprotective therapies in treating stroke.

We report the case of a 75-year-old man who presented with an ischaemic tongue. He was known to have external carotid artery stenosis and a history of a transient ischaemic attack. He was treated with a heparin infusion and the tongue healed well.

Ischaemia reperfusion injury is a common and important phenomenon that occurs predictably in patients undergoing such procedures as cardiopulmonary bypass, thrombolysis, surgery under tourniquet, organ transplantation or embolectomy. Oxidative stress and the resulting lipid peroxidation play a major role in reperfusion injury. Membrane and cellular dysfunction result and, subsequently, organ injury or failure may ensue. Traditional methods of quantifying ischaemia reperfusion injury, including measurement of malondialdehyde, lack specificity and sensitivity. It was reported in 1990 that isoprostanes, a series of prostaglandin-like compounds, are produced by the free radical-catalyzed peroxidation of arachidonic acid. Measurement of the isoprostane concentration in urine or plasma provides the most reliable, non-invasive method currently available to assess oxidative stress in vivo. Serial measurement of isoprostanes in biological fluids has enhanced our understanding of the mechanisms underlying ischaemia reperfusion injury itself and its role in certain diseases. Furthermore, measurement of the isoprostane concentration provides a means to assess the effects of prophylactic and therapeutic interventions. In the future, the development of rapid, simple assays for isoprostanes offers the potential to assess prognosis during and after ischaemia reperfusion events.

Background and objective Ischaemic colitis can be a serious complication after aortic surgery. The paucity of clinical symptoms makes its diagnosis particularly difficult and often delayed. Automated on-line tonometry is now proposed to monitor intestinal perfusion. This study was designed to assess the use of semi-continuous sigmoid-to-arterial [P(r-a)CO2] PCO2 gap monitoring in aortic surgery to detect colonic ischaemia.

Methods This prospective clinical study was realized at the University Hospital of Lille, France, including eight males scheduled for abdominal aortic aneurysm surgery. Intraoperative and postoperative P(r-a)CO2 values were compared with conventional monitoring and colonic mucosa aspect performed by sigmoidoscopy 48 h after surgery. Haemodynamic variables, O2 delivery (DO2), O2 consumption (VO2), O2 extraction (ERO2), lactate, P(v-a)CO2, P(r-a)CO2 were measured peroperatively and every 4 h during a 48-h postoperative period.

Results Intraoperative P(r-a)CO2 values increased significantly with the highest value (4.36 ± 3.42 kPa) observed during aortic clamping when DO2 was the most altered. P(r-a)CO2 continued to deteriorate after surgery with the maximal values between 8 (4.79 ± 3.85 kPa) and 12 (4.68 ± 3.26 kPa) h after surgery. This peak was associated with a significant ERO2 increase counterbalancing an increase of VO2 whereas DO2 tended to decrease. P(r-a)CO2 values began to decrease only at the end of the study. The highest values of P(r-a)CO2 were registered in patients with the most altered haemodynamic variables, severe ischaemic colitis along with higher hospital lengths of stay.

Conclusion Taken together, these data suggest that regional and automated capnometry may be easily used non-invasively to detect peroperative intestinal ischaemia in aortic surgery.

Ischaemia and reperfusion phases represent critical events during liver transplantation. The purpose of this study was to describe morphological alterations of both vascular and parenchymal compartments after ischaemia and reperfusion and to evaluate the possible relationship between morphometric parameters and biochemical/clinical data. Three needle biopsies were drawn from 20 patients who underwent orthotopic liver transplantation. The first biopsy was taken before flushing with preservation solution, and the second and the third to evaluate respectively the effects of cold ischaemia and of warm ischaemia/reperfusion. Biopsies were examined by an image analyser and morphometric parameters related to the liver parenchyma were evaluated. At the second biopsy we observed a decrease of the endothelium volume fraction while the same parameter referred to the sinusoidal lumen achieved a peak value. The hepatocytes showed a lower surface parenchymal/vascular sides ratio. This parameter was reversed at the end of the reperfusion phase; furthermore the third biopsy revealed endothelial swelling and a decreased volume fraction of the sinusoidal lumen. The results quantify the damage to the sinusoidal bed which, as already known, is one of the main targets of cold ischaemia; warm ischaemia and reperfusion accentuate endothelial damage. The end of transplantation is characterised by damage chiefly to parenchymal cells. Hepatocytes show a rearrangement of their surface sides, probably related to the alterations of the sinusoidal bed. In addition, the fluctuations of morphometric parameters during ischaemia/reperfusion correlate positively with biochemical data and clinical course of the patients.

Background and objective Increased glutamate concentration in the cerebrospinal fluid has been reported in severely head–injured patients, suggesting that an excessive release of glutamate may be involved in the process of neuronal damage. Ischaemic damage after subdural haematoma has been reported to be reduced by glutamate (N-methyl-D-aspartate: NMDA) receptor antagonists such as dizocilpine and CGS 19755; even though these drugs were given 20–30 min after insult. Excessive release of excitatory amino acids may produce the neural damage after subdural haematoma and NMDA receptor antagonists may become valuable therapeutic drugs. This study compared the effects of ketamine and dizocilpine, on intracranial pressure and histopathological changes after acute subdural haematoma produced by an injection of autologous blood (150 μL) in rats.

Methods The control (n =9), ketamine (n =9) and dizocilpine (n =9) groups, respectively, received saline, ketamine (total dose: 210 mgkg−1) or dizocilpine (total dose: 1.0 mgkg−1) from 0.5 to 8 h after acute subdural haematoma. A silicone group (n =9) had the same volume of silicone injected subdurally.

Results The volume of ischaemic damage in the silicone group (1.3±1.2mm3) was significantly smaller than in the control group (11.9±3.8mm3). Ketamine and dizocilpine did not increase intracranial pressure. Dizocilpine significantly decreased the volume of ischaemic damage (6.1±3.8mm3). Ketamine failed to significantly decrease damage (7.8±5.0mm3).

Conclusions These results suggest that the factors elicited by the clotted blood contribute to the ischaemic damage after subdural haematoma, and that the glutamate receptor antagonist dizocilpine reduces the damage, while ketamine shows only a trend reduction of the damage.

Sevoflurane has been reported to generate oxygen free radicals. We have investigated whether sevoflurane or isoflurane enhances oxygen free radical formation in the post-ischaemic reperfused heart. An isolated rat heart–lung preparation was used. Thirty male Wistar rats were allocated to four groups: control, no drug, 2.5% sevoflurane and 1.4% isoflurane. The heart was perfused initially at a cardiac output of 30 mL min−1 and a mean arterial pressure of 70 mmHg. Ten minutes after the start of perfusion, the heart was rendered globally ischaemic for 10 min by reducing the preload and afterload to zero. Then, the heart was reperfused for 10 min. At the end of reperfusion, the heart was freeze dried for 6 days. The perfusate blood was collected just before and just after ischaemia and at the end of reperfusion. The formation of hydroxyl radicals in perfusate blood and heart was measured with high-performance liquid chromatography using salicylic acid. Hydroxyl radicals react with salicylic acid, yielding 2,3-, 2,4-, 2,5- and 3,4-dihydroxybenzoic acid (DHBA). Before and after ischaemia, there were no significant differences in cardiac output, systolic pressure, heart rate and right arterial pressure among the groups. The concentrations of 2,3-, 2,4-, 2,5- and 3,4-DHBA in the perfusate blood after ischaemia and reperfusion were significantly higher than those before ischaemia in all groups. However, there were no differences in the DHBA levels among groups. This study indicates that sevoflurane and isoflurane do not enhance hydroxyl radical formation in the post-ischaemic reperfused heart.

This study was designed to determine the intra-operative incidence of right-sided ventricular ischaemia and any association with left ventricular ischaemia. In 60 patients, undergoing coronary artery bypass grafting surgery, a right-sided precordial lead V5R was used. ST segment deviation of more than 1 mm in V5R was considered significant for myocardial ischaemia. Right ventricular ischaemia occurred in 14 patients(23.3%) but was not associated with left ventricular inferior wall ischaemia. In 4 patients (6.6%) presenting with right ventricular ischaemia, ischaemia of the left inferior wall also developed but in all cases was transient and disappeared by the end of surgery. No myocardial infarction was detected in the post-operative period. The present study showed that the use of a right-sided lead may improve intra-operative electrocardiographic monitoring, by revealing ischaemia in those patients in whom ECG abnormalities were not detected by conventional leads. The transient right ventricular ischaemia recorded in this study was probably related to a reduced hypothermic protection of the right ventricle during aortic cross clamping.

Subarachnoid haemorrhage (SAH) following cerebral aneurysm rupture or trauma can result in the induction of secondary ischaemic brain damage via a decrease in microvascular perfusion, a disruption of the blood-brain barrier and consequent vasogenic oedema, and the delayed spasm of the major cerebral arteries (i.e. vasospasm). It is increasingly apparent that oxygen radical-induced, iron-catalyzed lipid peroxidation (LP) within the subarachnoid blood and vascular wall plays a key role in the occurrence of these secondary events. Tirilazad mesylate is a potent cytoprotective inhibitor of LP that works by a combination of radical scavenging and membrane stabilizing properties. It has been demonstrated to attenuate the acute and delayed vascular consequences of SAH and to protect the brain against ischaemic insults. Much of its action is mediated by an effect on the vascular endothelium, although it also appears to exert some direct neuroprotection and to inhibit LP in the subarachnoid blood. These actions of tirilazad in experimental SAH are reviewed.