A 13-year-old girl presented to our unit because of recurrent syncopal episodes. Echocardiography (A and B, Online Video 1) demonstrated left ventricular (LV) hypertrophy (*), poor aortic valve excursion, and intense echogenicity extending to the ascending aorta (arrows). Three-dimensional echocardiography showed dyskinesia of the LV with poor ejection fraction of 21%. The 32-slice computed tomography scan revealed the extension of the calcification up to the transverse arch (C to F, Online Video 2). The calcification was clearly delineated by fluoroscopy (arrow), with normal coronary angiogram (G to I).The patient underwent aortic root replacement with pulmonary autograft. One week postoperatively, 3-dimensional echocardiography demonstrated dramatically improved LV ejection fraction (50%) with no evidence of dyskinesia (Online Video 3).A diagnosis of Singleton-Merten syndrome, a rare genetic disorder characterized by calcium deposition, had been made recently. To our knowledge, cardiovascular imaging findings have not been reported in the literature.

A 14 year old boy presented to a Paediatric Assessment Unit with shortness of breath and chest pain. He had never been seen in primary or secondary care before. His first medical attendance was to A and E with these symptoms 8 days earlier, when he was started on amoxicillin for a suspected chest infection. He was pale, tachypnoeic and hypertensive. The initial impression was that he might be having a panic attack.He described 9 days of breathlessness, chest tightness, vomiting and abdominal pain. His chest was clear and heart sounds normal with a soft murmur. He appeared very anxious. There were two positive findings of significant concern; a 3 centimetre palpable liver and persistent hypertension of more than 160/120. A chest radiograph showed a large heart and fluid in the horizontal fissure. More focused history taking revealed the shortness of breath was worse on exertion and on lying down, and he had to prop himself up at night to sleep.The history and examination was consistent with heart failure. An ECG showed right ventricular hypertrophy and widespread T-wave inversion. An echocardiogram showed a structurally normal heart, mild mitral and aortic regurgitation, severely impaired left ventricular function (fractional shortening 13%) and bilateral pleural effusions. A provisional diagnosis of viral myocarditis was made. However, in addition to persistent hypertension, his urea was 30 and creatinine 523.He was admitted to a Paediatric Intensive Care Unit where acute management included oxygen, fluid restriction, furosemide and a milrinone infusion. In view of the deranged renal profile he was discussed with the renal team. Renal ultrasound revealed small, scarred kidneys and calyceal dilatation. The diagnosis was end stage renal failure due to undiagnosed grade 4 vesico-ureteric reflux, hypertension secondary to chronic kidney disease, and dilated cardiomyopathy secondary to hypertension.It is rare for a teenager to present in heart failure, having never been unwell before. Originally we thought this was a primary cardiac problem, but the learning point here is that despite antenatal screening and good health care, congenital renal disease can remain asymptomatic, and that end stage renal failure can present with heart failure.

5 week old male infant referred by the GP for poor feeding and increasing lethargy. On admission was poorly perfused, lethargic and further investigation showed metabolic acidosis and narrow complex tachycardia with a HR of 270. Chest xray showed enlarged cardiac shadow with congested lung fields and poor cardiac function on echocardiography. Furosemide at 1mg/kg and several doses of adenoside up to 350mcg/kg were given followed by propranolol and digoxin in order to achieve cardioversion. Only transiently sinus rhythm was achieved with the use of adenosine and even after the administration of propranolol and digoxin still HR remained high. At that point DC shock was attempted and referral to PICU. The patient on admission to PICU received a further dose of adenosine and magnesium which uncovered a ventricular rate of 90 and an atrial flutter. An echocardiogram was performed which showed a structurally normal heart with reduced cardiac function. Three further unsuccessful attempts of DC cardioversion were followed by esmolol infusion and oesophageal pacing without capture. The patient was started on amiodarone, receiving a loading dose and an infusion. The tachycardia resolved (HR 130), but atrial rate 2:1 persisted. The SVT continued while on digoxin and amiodarone. Flecainide was started and the patient was discharged from PICU on amiodarone and flecanide. Echocardiography showed a gradually improving cardiac function and after amiodarone and flecanide were gradually weaned off within a year of lifeConclusionThis was a case of complex atrial tachycardia which responded poorly to the conventional medical management in the district hospital. Due to the poor cardiac function the child had to be transferred to PICU for further management including amiodarone and esmolol infusions. Cases like this are challenging to be managed in district general hospitals where the presence of staff experienced and confident with the use of DC is limited.

A 15 year old girl was referred to the paediatric outpatient clinic by her GP with a 2 year history of chest pain at rest and during exercise. Pectus excavatum was noted and on examination there was tenderness of the costochondral joints.A chest x-ray revealed apparent cardiomegaly, possibly secondary to the pectus excavatum. While the results of the initial echocardiogram were unremarkable, ECG changes were noted in the ST-T segments for the inferior leads. Subsequently, exercise tolerance tests were carried out, consisting of 12 min on a treadmill. This elicited ST-T changes in the anterior leads. An heart MRI scan showed a dialted right ventriclu with impaired right ventricular systolic function. A repeat MRI scan with dobutamine confirmed this with the EF being 37%. Left ventricular systolic function was found to be at the lower end of normal, potentially consequent to the right ventricular dilatation.The patient continued to experience chest pain and breathlessness at rest, and repeat echocardiogram confirmed dilatation of the right ventricle with right ventricular dysfunction (left ventricle had normal contactility and no dilatation). There was also mild trucusipd regurguation at 2.6m/sec. Repair of the pectus excavatum took place 6 months later. During the surgery, it was noted that removal of the xiphisternum resulted in immediate decompression of the right ventricle. Thoracic surgeons were able to release tissues and increase the AP diameter of the chest with a modified Ravitch procedure using prolene mesh. The post-operative echocardiogram showed improved right ventricular contactility, no tricuspid or mitral regurgiation and no outflow obstruction. The patient has had a repeat stress test which was completely normal 6 months post surgery.ConclusionIn patients with pectus esxavatum, impaired cardiac function consequent to the distortive effects of chest wall deformity is an important differential diagnosis to consider. Surgical correction has the potential to very quickly and radically improve the patient’s symptoms and future quality of life.