3-D echocardiographic imaging of double aortic arch.

Delineation of arch abnormalities is difficult by conventional 2-D echocardiography and MRI has been the investigation of choice. 3-D echocardiography is increasingly used in congenital heart disease for both functional anatomy and morphology. This case report demonstrates that 3-D echocardiography can be used in delineating arch anomalies, which can avoid further imaging that needs a general anaesthetic.     

Safe retrieval of impacted central venous line.

Embolisation of impacted central venous lines is not uncommon in paediatric practice. We describe a method of preventing this complication and associated risks.     

Anesthetic management of an infant with Joubert syndrome for cardiac surgery

The anesthetic implications of Joubert syndrome in an infant who required cardiac surgery using cardiopulmonary bypass (CPB) is presented. Children with Joubert syndrome present with central apnea due to malformations in the midbrain and cerebellum. These patients have a marked sensitivity to opioids. The use of dexmedetomidine along with remifentanil was effective in this case.     

A multiplanar three dimensional echocardiographic study of mitral valvar annular function in children with normal and regurgitant valves

INTRODUCTION: The mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function. MATERIALS AND METHODS: Three-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review. RESULTS: The mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012). CONCLUSIONS: Mitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.     

Congenital mitral regurgitation due to papillary muscle infarction: a case report and approach to evaluation

Congenital mitral regurgitation is relatively rare. It can be due to congenital malformation of the valve apparatus, associated structural heart defects, or results from myocardial dysfunction leading to deformation of the valve annulus. Hence, careful delineation of the pathologic processes involved in the mechanism is critical when evaluating patients presenting with congenital mitral valve regurgitation. Congenital anomalies of the coronary system are one of the serious associations, which needs prompt recognition and treatment to prevent fatal complications. We report a case of congenital atresia of left main coronary artery in an asymptomatic 8-month-old presenting with an incidental finding of mitral valvar regurgitation. Appropriate investigations and management lead to restoration of normal coronary blood flow and valve function. This particular case illustrates that congenital coronary anomalies may present in an otherwise asymptomatic child. It should be considered as one of the possible differential diagnosis in unexplained mitral regurgitation. Early recognition and prompt surgical treatment can radically modify the prognosis.     

Pulmonary Arteriovenous Malformations in Dyskeratosis Congenita

Pulmonary arteriovenous malformations (PAVMs) are rare lesions known to cause cyanosis due to abnormal communication between the pulmonary arteries and veins. They are commonly seen in association with hereditary hemorrhagic telangiectasia, congenital heart disease, hepatopulmonary syndrome, and portopulmonary shunting, but rarely in patients with dyskeratosis congenita (DC). We describe a patient previously diagnosed with DC confirmed to have microscopic PAVMs after bone marrow transplantation and discuss possible pathogenic mechanisms.