SUMMARY Twenty three patients with fragile X syndrome underwent cardiovascular assessment. Echocardiography showed dilatation ofthe aortic root in 12 (52%) and mitral valve prolapse in five (22%), four of whom had an apical mid-systolic click on auscultation.Patients with fragile X syndrome have cardiac defects similar to those seen in other disorders of connective tissue such as Marfan's syndrome and Ehlers-Danlos syndrome. These, and other somatic features, suggest an underlying connective tissue dysplasia.The fragile X syndrome is the commonest inherited cause of mental retardation and it affects approximately one in a 1000 males.'2 It is so called because there is a fragile site on the long arm of the X chromosome in affected patients.' Common somatic manifestations include a long face, large and prominent ears, prognathism, and macroorchidism. Other associated findings such as hyperextensible finger joints, pectus carinatum, a high-arched palate, and flat feet suggest an underlying connective tissue dysplasia.4 Previous reports of mitral valve prolapse and aortic root dilatation in patients with fragile X syndrome support this proposition.56This study was performed to establish the frequency of cardiac abnormalities in a population of patients with fragile X syndrome who underwent voluntary cardiac assessment. Patients and methods PATIENTSWe studied 23 men (aged 18-80, mean 51) with proven fragile X syndrome. Most were moderately or severely mentally handicapped. A few were living in the community while most were resident in a hospital for the mentally handicapped. They were drawn from a group of 28 patients identified by cytogenetic examination in a prospective survey of an inpatient population. Five

Primary cardiac tumours are rare. The majority are benign and 75% are atrial myxomas. One of the more unusual benign tumours affecting the heart is a cardiac paraganglioma. A 56-year male was presented with a 6-month history of vague, left-sided chest pain, intermittent parathesia of the left arm and dyspnoea on bending. Echocardiography documented a large, highly vascular mass, attached to the intra-atrial septum. All investigations, haematological, biochemical, neuroendocrine tumour markers and urinary cathecholamine levels, were within normal limits. Macroscopically the tumour involved the whole of the atrial septum, roof of the left atrium and extended to surround the superior vena cava, excluding total resection. The feeding vessels were ligated in the hope of infarcting the remainder of the tumour. In over 2-years of follow-up, the patient remains clinically stable. To our knowledge this is the first reported case where vascular ligation has been used to control a cardiac paraganglioma.

It is important to identify preoperatively coronary arteries that may interfere with right ventricular outflow tract reconstruction. An aortogram with steep caudal and left oblique angulation is useful in identifying anomalous coronary arteries and more importantly it defines the relation of these vessels to the right ventricular outflow tract.

Background: Lack of scientific data on the feasibility and safety of minimally invasive cardiac surgery (MICS) during the COVID-19 pandemic has made clinical decision making challenging. This survey aimed to appraise MICS activity in UK cardiac units and establish a consensus amongst front-line MICS surgeons regarding standard best MICS practise during the pandemic. Methods: An online questionnaire was designed through the ‘googleforms’ platform. Responses were received from 24 out of 28 surgeons approached (85.7%), across 17 cardiac units. Results: There was a strong consensus against a higher risk of conversion from minimally invasive to full sternotomy (92%; n = 22) nor there is increased infection (79%; n = 19) or bleeding (96%; n = 23) with MICS compared to full sternotomy during the pandemic. The majority of respondents (67%; n = 16) felt that it was safe to perform MICS during COVID-19, and that it should not be halted (71%; n = 17). London cardiac units experienced a decrease in MICS (60%; n = 6), whereas non-London units saw no reduction. All London MICS surgeons wore an FP3 mask compared to 62% ( n = 8) of non-London MICS surgeons, 23% ( n = 3) of which only wore a surgical mask. London MICS surgeons felt that routine double gloving should be done (60%; n = 6) whereas non-London MICS surgeons held a strong consensus that it should not (92%; n = 12). Conclusion: Whilst more robust evidence on the effect of COVID-19 on MICS is awaited, this survey provides interesting insights for clinical decision-making regarding MICS and aids to facilitate the development of standardised MICS guidelines for an effective response during future pandemics.

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