Because the dawning of the era in which interventions for congenital heart disease (CHD) became possible, the primary intellectual framework undergirding pediatric cardiology and cardiothoracic surgery has been based on morphology and hemodynamics (henceforth, the M/H model). This model has been wildly successful, enabling practitioners to devise creative interventions for even the most complex...

OBJECTIVES To determine whether offering cardiac screening to relatives of patients with left ventricular outflow tract obstructions (LVOTOs) would be justified. BACKGROUND LVOTOs have been recognised as a group of congenital heart diseases with 'high heritability'. One of the LVOTOs, the bicuspid aortic valve, is often asymptomatic, but has become known to be associated with sudden, unexpect...

https://e-kcj.org A bicuspid aortic valve (BAV) is the most common congenital heart valve disease and is more common in males.1) BAV patients demonstrate various clinical courses, including a normally functioning BAV, asymptomatic aortic valve dysfunction and severe aortic valve dysfunction that requires surgery.1) 2) Occasionally, infective endocarditis might be the first clinical manifestatio...

Thoracic aortic aneurysm is often an asymptomatic but potentially lethal disease if its most catastrophic complication - aortic dissection - occurs. Thoracic aortic dissection is associated with a high mortality rate despite ongoing improvement in its management. We report a fatal outcome of thoracic aortic aneurysm in a male patient with bicuspid aortic valve. The patient was qualified for ele...

Patients with bicuspid aortic valve (BAV) are more likely to develop a calcific aortic stenosis (CAS), as well as a number of other ailments, as compared to their cohorts with normal tricuspid aortic valves (TAV). It is currently unknown whether the increase in risk of CAS is caused by the geometric differences between the tricuspid and bicuspid valves or whether the increase in risk is caused ...

We describe a case of Gemella bergeriae endocarditis in a patient with a bicuspid aortic valve. Diagnosis was confirmed by sequencing of 16S rRNA genes in heart valve tissue. This is the first report of Gemella endocarditis confirmed by molecular detection of bacterial genes in heart valve tissue.

Valve-sparing aortic root repair (V-SARR) using the David reimplantation method is an increasingly popular alternative to composite valve graft aortic root replacement in patients with aortic root aneurysms or dissections who wish to avoid anticoagulation. Computed tomography (CT) with retrospective electrocardiograph (ECG)-gating has become routine before and following V-SARR at Stanford. CT a...

A 64-year-old male with acute-onset dyspnea and diastolic murmur was referred to our hospital. Eight months earlier, he had developed atrial fibrillation. At that time, echocardiography showed a reduced ejection fraction of 41% and a bicuspid aortic valve (BAV) with mild aortic stenosis (Fig. 1a, b, Video 1). On admission, echocardiography showed prolapse of the conjoined cusp and severe aortic...

Calcific Aortic Valve Disease (CAVD) occurs in >2% of the population over 65 years of age and often leads to valvular stenosis that necessitates valve replacement [1]. CAVD is a pro‐ gressive disease, often manifesting first as aortic valve sclerosis and later developing into stenosis and valve dysfunction [2]. The specific molecular and cellular mechanisms of CAVD initiation and advancement ar...

The use of antiepileptic drugs increases the risk of major congenital malformations during pregnancy. Here, we report an infant who had a history of in-utero carbamazepine exposure and who was born with a cardiac malformation. The infant was born at 39 weeks of gestation vaginally to an epileptic mother who had been treated with carbamazepine throughout her pregnancy. He was referred due to car...