A 20-month-old girl presented with severe dilated cardiomyopathy and decompensated congestive cardiac failure. Despite escalating inotropic and mechanical ventilation support, she required placement on extracorporeal membrane oxygenation and transfer to the transplant centre in Newcastle, England. She was placed on biventricular assist device and then Berlin Heart but failed to show any recovery of ventricular function. She underwent orthotopic heart transplantation at 2 years of age. She developed bacterial endocarditis with Enterococcus faecalis resulting in severe aortic valve regurgitation requiring aortic valve replacement with a 19 mm On-X valve (Airtivion) 11 days after her transplant. Given the size of the donor heart, it was possible to implant a 19-mm valve in this 12 kg child with minimal risk of patient prosthesis mismatch. She was anticoagulated with warfarin (On-X valve INR 2-3 for first 3 months; INR 1.5-2.0 thereafter). Although she suffered several other post-operative complications, including malabsorption, nasojejunal feeding, liver dysfunction, vertebral fractures, renal impairment and renal calcification, and need for repeat opening of her tracheostomy site following her initial decannulation, her aortic valve function has remained stable.

Acute coronary syndrome is a cause of cardiac-induced chest pain and rarely seen among childhood. It should be kept in mind in patients presenting with typical chest pain with a history of CHD and/or surgery, as this will lead to life-threatening complications and death. We present an adolescent with a history of Bentall operation who had acute coronary syndrome owing to coronary thrombosis as a result of inappropriate drug use.

A 51-year-old man presented with a 5-day history of progressive facial swelling, sensation of head fullness, increasing shortness of breath and paroxysmal nocturnal dyspnea. He denied chest pain, syncope or presyncope. Pastmedical history included mechanical aortic valve replacement 7 years prior and atrial fibrillation treated with warfarin. A clinical diagnosis of acute superior vena cava (SVC) syndrome was made. Portable chest radiograph showed a widened superior mediastinum. Computed tomography scan of the thorax demonstrated a large type A aortic dissection almost completely effacing the SVC. Acute type A aortic dissection (AD) is an emergency requiring prompt diagnosis and treatment. Patients typically present with acute onset of chest and/or back pain, classically described as “ripping” or “tearing.” SVC syndrome is rarely, if ever, mentioned as a presentation, as it is usually due to more chronic conditions. This case illustrates a rare incidence of type A AD actually presenting as SVC syndrome.

Degenerative-calcific aortic stenosis is mainly a disease of old age. Patients with mild to moderate stenosis without symptoms and those with aortic valve sclerosis do not require mechanical intervention. There is no firm evidence that the rate of progression can be modified by medical therapies, though statins might have some effect. Patients who develop severe stenosis with symptoms have a very poor prognosis if managed medically. Surgical aortic valve replacement greatly improves symptoms and mortality rates and remains the treatment of choice for those fit for major surgery, even above the age of 80 years. For those not suitable for surgery, or who are unwilling to have an operation, outcomes can be significantly improved by trans-catheter aortic valve replacement, which has become an important option for frail elderly patients. Balloon aortic valvuloplasty improves symptoms and short-term survival, but has a less clear impact on mortality beyond 1 year. It is useful as a palliative treatment and as a bridging procedure for patients who are not ready for valve replacement. There is less agreement on the best approach to patients with asymptomatic severe aortic stenosis. Trials indicate that early valve replacement results in outcomes that are comparable to those seen in symptomatic patients, though guidelines advocate delaying surgery until symptoms occur or left ventricular function begins to decline rapidly. All elderly patients with severe aortic stenosis should be considered for a mechanical intervention unless there is a properly considered reason for not doing so, or they are not willing to receive such treatment.

We describe a case of severe aortic stenosis in a 16-year-old male with Hurler's syndrome who had prior bone marrow transplantation. The excised aortic valve leaflets showed characteristic pathologic findings of Hurler's syndrome. This is the first case report of aortic valve replacement in a patient with Hurler's syndrome treated with bone marrow transplantation that demonstrates progression of the aortic valve disease despite treatment.

A 10-year-old girl required replacement of the aortic valve because of massive regurgitation caused by Kawasaki disease at the age of 3 months. Postoperatively, left ventricular function and mass improved remarkably, with reduction of both hypertrophy and interstitial fibrosis. During a follow-up period of 10 years, she has been free from any cardiac event.

Replacement of the aortic valve exposes the left ventricle to myocardial ischaemia and imposes on it abrupt changes in loading conditions and geometry. We studied the immediate changes in the left ventricular function of patients undergoing aortic valve replacement by transoesophageal echocardiography. Patients with aortic regurgitation responded to surgery with a decrease in global systolic function associated with a fall in preload. Patients with aortic stenosis showed an impairment in myocardial contractility and ventricular filling, and their global systolic function did not improve despite the marked reduction in afterload. This can be a consequence of inadequate myocardial protection and intra-operative ischaemic injury of the hypertrophic myocardium. Information provided by transoesophageal echocardiography contributes to optimal and individualized management of the period immediately after aortic valve replacement.