Adult congenital heart disease: a review of the simple lesions.

There has been a dramatic improvement in mortality rates among children with congenital heart disease with advances in neonatal screening and surgical techniques, resulting in a significant increase in the prevalence of adults living with congenital heart disease. The most common simple lesions of congenital heart disease include atrial and ventricular septal defects, patent ductus arteriosus and coarctation of the aorta, which are typically detected and treated in childhood. However, they may also present in adulthood with non-specific symptoms or incidental findings, such as refractory hypertension. As the adult population of those living with congenital heart disease grows, it is imperative that all clinicians remain abreast of these common cardiac conditions, irrespective of their specialty, as patients may present with sequelae of their congenital heart disease or other non-cardiac conditions.

Twenty-three year-old with pleuritic chest pain.

CLINICAL INTRODUCTION: A 23-year-old woman followed at another medical centre for congenital heart disease (CHD) presented to our emergency clinic with 3 weeks of bilateral pleuritic chest pain. She returned from holiday in Greece 6 weeks earlier where a tattoo and nasal piercing had been performed. There was no history of night sweats or fever.Her temperature was 37.5°C, heart rate 120 beats/min, oxygen saturations 94% on room air and blood pressure 110/74. Her chest was clear and there was systolic murmur on auscultation. The chest radiograph showed peripheral bilateral lower zone atelectasis. The ECG demonstrated sinus tachycardia. The haemoglobin was 11.2 g/dL, white cell count 10.18×109/L, C-reactive protein 67 mg/L (normal <5 mg/L) and D dimer=430 ng/mL (normal <230 ng/mL).A pulmonary embolus was suspected and a CT pulmonary angiogram was performed (figure 1). QUESTION: Based on the CT findings, what is the most likely underlying congenital heart lesion in this patient?Bicuspid aortic valveCoarctation of the aortaFontan circulationParachute mitral valveVentricular septal defect heartjnl;105/6/464/F1F1F1Figure 1CT pulmonary angiogram (coronal views).

Current guidance on the management of acute coronary syndrome.

Acute coronary syndrome (ACS) describes a spectrum of symptoms arising from the development of atherosclerosis. The degree of myocardial ischaemia depends on plaque stability and the extent of vessel occlusion. This article examines underlying pathophysiological processes and reviews current guidance and principles of managing ACS through symptom control, reducing mortality and maximizing secondary prevention. Nurses have a vital role in all aspects of delivering this care and meeting National Service Framework Standards for Coronary Heart Diseases (CHD) (2000). A clear understanding of the pathophysiological basis for ACS will reinforce clinical work, particularly in the recognition, monitoring and early management.

Sialic acid: a novel marker of cardiovascular disease?

The global burden posed by cardiovascular disease (CVD), due to a rising incidence of known risk factors, underlines an urgent need to identify other potential risk factors. Sialic acid (SA), an abundant terminal monosaccharide of glycoconjugates, is a possible risk factor for CVD. Although large-scale epidemiological surveys have shown that serum total sialic acid (TSA) is positively associated with mortality from coronary artery disease (CAD) and stroke, studies investigating the correlation between serum TSA and the severity of atherosclerosis are conflicting. Clinical and epidemiological studies indicate that serum TSA is a marker of a sustained inflammatory response in CVD, rather than causal in nature. Data also indicates ethnic variation in baseline TSA. This article reviews current methods for determining serum TSA and evidence supporting serum TSA as a risk factor for CVD. Potential mechanisms for this role are examined. The use of serum TSA as a marker of atherosclerotic disease is evaluated.

The inborn errors of sialic acid metabolism and their laboratory investigation.

Sialic acid (SA), a terminal monosaccharide of glycoconjugates, has a central role in human biological function. Various point mutations result in the malmetabolism of SA and inherited disorders: Defective SA synthesis causes sialuria and defective SA catabolism causes sialidosis and sialic acid storage disease (SASD). These inborn errors of metabolism are characterised by increased urinary free SA. This article reviews biochemical and clinical features that are distinct to each disorder. In view of recent evidence indicating a wide underestimation in the prevalence of sialic acid disorders, laboratory methods for determining urinary free SA and its implications for screening and prenatal diagnosis are evaluated.