CT of transposition of the great arteries in adults.

Transposition of the great arteries is one of the most common cyanotic congenital heart diseases. It is characterised by an abnormal connection of the ventricles and great arteries, so that the aorta arises from the morphological right ventricle and the pulmonary artery arises from the morphological left ventricle. Historically, as with many congenital heart diseases, patients with transposition of the great arteries had poor life expectancy. Advances in surgical and medical management have resulted in patients surviving into adulthood. As these patients are living longer, they will be encountered more frequently in practice. The purpose of this article is to familiarise the general radiologist with the expected postoperative anatomy, and the appearance on cross-sectional imaging as well as the long-term complications in this group of patients.

Pregnancies in women with Turner syndrome: a retrospective multicentre UK study.

OBJECTIVE: To determine the characteristics and outcomes of pregnancy in women with Turner syndrome. DESIGN: Retrospective 20-year cohort study (2000-20). SETTING: Sixteen tertiary referral maternity units in the UK. POPULATION OR SAMPLE: A total of 81 women with Turner syndrome who became pregnant. METHODS: Retrospective chart analysis. MAIN OUTCOME MEASURES: Mode of conception, pregnancy outcomes. RESULTS: We obtained data on 127 pregnancies in 81 women with a Turner phenotype. All non-spontaneous pregnancies (54/127; 42.5%) were by egg donation. Only 9/31 (29%) pregnancies in women with karyotype 45,X were spontaneous, compared with 53/66 (80.3%) pregnancies in women with mosaic karyotype 45,X/46,XX (P < 0.0001). Women with mosaic karyotype 45,X/46,XX were younger at first pregnancy by 5.5-8.5 years compared with other Turner syndrome karyotype groups (P < 0.001), and more likely to have a spontaneous menarche (75.8% versus 50% or less, P = 0.008). There were 17 miscarriages, three terminations of pregnancy, two stillbirths and 105 live births. Two women had aortic dissection (2.5%); both were 45,X karyotype with bicuspid aortic valves and ovum donation pregnancies, one died. Another woman had an aortic root replacement within 6 months of delivery. Ten of 106 (9.4%) births with gestational age data were preterm and 22/96 (22.9%) singleton infants with birthweight/gestational age data weighed less than the tenth centile. The caesarean section rate was 72/107 (67.3%). In only 73/127 (57.4%) pregnancies was there documentation of cardiovascular imaging within the 24 months before conceiving. CONCLUSIONS: Pregnancy in women with Turner syndrome is associated with major maternal cardiovascular risks; these women deserve thorough cardiovascular assessment and counselling before assisted or spontaneous pregnancy managed by a specialist team. TWEETABLE ABSTRACT: Pregnancy in women with Turner syndrome is associated with an increased risk of aortic dissection.

Employment characteristics of a complex adult congenital heart disease cohort.

BACKGROUND: Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. AIMS: To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK. METHODS: Records of all patients with a univentricular (Fontan) circulation at the Queen Elizabeth Hospital were reviewed. Employment status was categorized according to the Standard Occupational Classification criteria (2010). RESULTS: A total of 210 patient records were reviewed. There was the same proportion of professionals in our cohort compared to the rest of the UK (20% versus 20%). There were greater proportions working in the caring, leisure and other service occupations (15% versus 9%), the elementary occupations (17% versus 11%), sales and customer service occupations (14% versus 8%) and administrative and secretarial occupations (12% versus 11%). The reverse trend was observed for associate professions and technical occupations (7% versus 14%), skilled trades (10% versus 11%), process, plant and machine operatives (3% versus 6%) and managers, directors and senior officials (2% versus 10%). CONCLUSIONS: The data show that ACHD patients with a single ventricle are engaged in a diverse range of occupations. It is essential that early education and employment advice are given to this cohort to maximize future employment potential.

Suitability of cardiac resynchronisation therapy in patients with Fontan circulation and congenitally corrected transposition of the great arteries.

BACKGROUND: Cardiac resynchronisation therapy (CRT) is a well-recognised treatment in systolic heart failure. There is limited evidence in congenital patients with univentricular hearts or systemic right ventricles. In 2014 PACES/HRS published a consensus statement recommending CRT if ventricular ejection fraction (EF)≤35%, QRS duration≥150ms (with RBBB in systemic RV), NYHA II-IV and ventricular dilatation. The incidence of patients meeting these criteria in whom CRT is possible is not known. METHODS: Retrospective analysis of 203 patients with a univentricular Fontan circulation and 55 patients with ccTGA under specialist ACHD care. RESULTS: Univentricular functional data was available for 194 (96%), 10 (5%) having EF≤35%. QRS duration was available for 190 (94%) and was ≥150ms in five (3%). EF data was available for 54 (98%) ccTGA patients, and was ≤35% in 6 (11%). QRS duration was ≥150ms in 13 (26%). Only four patients fulfilled recommendations and two received CRT. CONCLUSIONS: Only a small proportion of patients with single ventricles or ccTGA meet the criteria for CRT. In many of these patients there are significant anatomical barriers to CRT which limit its use in this population. The decision to implant CRT in complex ACHD requires discussion in a combined ACHD electrophysiology surgical multidisciplinary meeting and close collaboration with patients.

Reproducibility of 31P cardiac magnetic resonance spectroscopy at 3 T.

The purpose of this work was to take advantage of the new clinical field strength of 3 T to implement and optimize a chemical shift imaging (CSI) acquisition protocol to produce spectra of high quality with high specificity to the myocardium within a clinically feasible scan time. Further, an analysis method was implemented dependent purely on anatomical location of spectra, and as such free from any potential user bias caused by inference from spectral information. Twenty healthy male subjects were scanned on two separate occasions using the optimized CSI protocol at 3 T. Data were analyzed for intra- and inter-subject variability, as well as intra- and inter-observer variability. The average phosphocreatine (PCr)/adenosine triphosphate (ATP) value for scan 1 was 2.07 +/- 0.38 and for scan 2 was 2.14 +/- 0.46, showing no significant difference between scans. Intra-subject variability was 0.43 +/- 0.35 (percentage difference 20%) and the inter-subject coefficient of variation was 18%. The intra-observer variability, assessed as the absolute difference between analyses of the data by a single observer, was 0.14 +/- 0.24 with no significant difference between analyses. The inter-observer variability showed no significant differences between the PCr/ATP value measured by four different observers as demonstrated by an intra-class correlation coefficient of 0.763. The increased signal available at 3 T has improved spatial resolution and thereby increased myocardial specificity without any significant decrease in reproducibility over previous studies at 1.5 T. We present an acquisition protocol that routinely provides high quality spectra and a robust analysis method that is free from potential user bias.

Assessment of left atrial volumes at 1.5 Tesla and 3 Tesla using FLASH and SSFP cine imaging.

PURPOSE: To investigate left atrial volumes and function and their variability in healthy volunteers using steady state free precession (SSFP) and fast low angle shot (FLASH) sequences at both 1.5 and 3 T using both the short-axis and biplane area-length methods. MATERIALS AND METHODS: Ten healthy volunteers underwent CMR at both 1.5 and 3 Tesla. The biplane area-length method utilized volumes from the horizontal and vertical long axis images. RESULTS: There were no significant differences between left atrial short-axis volumes or function between 1.5 and 3 T assessed using either FLASH or SSFP sequences. The biplane area-length method underestimated maximal left atrial volume using FLASH by 12 mL at 3 T (18%) and by 10 mL (14%) at 1.5 T (p = 0.003 and p = 0.05 respectively). Variability was larger for left atrial measurements using the biplane area-length method. CONCLUSION: Field strength had no effect on left atrial volume and function assessment using either FLASH or SSFP. The use of the short-axis method for the acquisition of left atrial parameters is more reproducible than the biplane area-length for serial measurements.

Coronary artery stents and non-cardiac surgery.

The utility of interventional cardiology has developed significantly over the last two decades with the introduction of coronary angioplasty and stenting, with the associated antiplatelet medications. Acute coronary stent occlusion carries a high morbidity and mortality, and the adoption of therapeutic strategies for prophylaxis against stent thrombosis has major implications for surgeons and anaesthetists involved in the management of these patients in the perioperative period. Currently, there is limited published information to guide the clinician in the optimal care of patients who have had coronary stents inserted when they present for non-cardiac surgery. This review examines the available literature on the perioperative management of these patients. A number of key issues are identified: the role of surgery vs percutaneous coronary intervention for coronary revascularization in the preoperative period; the different types of coronary stents currently available; the emerging issues related to drug-eluting stents; the pathophysiology of coronary stent occlusion; and the recommended antiplatelet regimes that the patient with a coronary stent will be receiving. The role of preoperative platelet function testing is also discussed, and the various available tests are listed. Appropriate management by all the clinicians involved with patients with coronary stents undergoing a variety of non-cardiac surgical procedures is essential to avoid a high incidence of postoperative cardiac mortality and morbidity. The review examines the evidence available for the perioperative strategies aimed at reducing adverse outcomes in a number of different clinical scenarios.

Back pain in pre-registration house officers.

Back pain is a major burden on the working population. It is a particular problem amongst hospital staff, especially nurses. It has been poorly studied amongst doctors. Pre-registration house officers (PRHOs) starting their careers are exposed to a number of risk factors for back problems, both physical and psychological. This questionnaire-based study investigated the prevalence of back pain and its impact on the work of new graduates from two UK medical schools. Around half of the newly qualified PRHOs had significant back pain, one-quarter at least once a week. The frequency of back pain doubled once they started work, although the overall prevalence remained static. One in 10 of them had been unable to perform their normal work activities at some stage because of back pain. One in eight had sought professional help for back problems in the previous 5 years. Fewer than 50% of newly qualified doctors could recall any formal training in lifting and handling.

Occupational asthma: a community based study.

The incidence and prevalence of occupational asthma has been extensively studied in industry settings and specialist clinics. However, it has been much less studied in the community. This study looked at the general practice notes of asthmatics in an attempt to assess the overall load of occupational asthma in the community. Eighty-six per cent of the patients with adult onset asthma in the practice population studied had at least one occupation recorded in their notes. Nearly a third of these (32%) were in jobs known to be significant causes of occupational asthma, yet a potential link between their occupation and symptoms had only been recorded in 18% of patients in these jobs. Overall 4% of the patients with adult onset asthma had been given a diagnosis of occupational asthma although in nearly half these cases the diagnosis had been made by a general practitioner and not a specialist.