Experience with 169 patients less than 25 years old and operated on at the Mayo Clinic for congenital aortic stenosis is reviewed. Aortic valvotomy is primarily a palliative procedure, allowing adequate relief of the transaortic valve gradient in only 56 percent of patients and leaving a severe gradient in 20 percent. A low operative mortality justifies its use in patients having valid cardiac ...

The bicuspid aortic valve is a common congenital cardiac anomaly, having an incidence in the general population of 0.9% to 2.0% and a frequency of 54% in all patients aged >15 years with valvular aortic stenosis. In most cases it remains undetected until infective endocarditis or calcification supervenes. The bicuspid aortic valve may function normally throughout life, may develop progressive c...

Congenital nonfamilial supravalvular aortic stenosis (SVAS) is relatively rare, its diffuse type being the least common. We present a 30-year-old woman with diffuse SVAS complicated with left ventricular apical aneurysm. We believe that subtle left ventricular myocardial ischemia or infarction and long-lasting severe pressure overload to the apical chamber caused LV apical aneurysm in our case....

We report the case of a 35-year-old man admitted due to heart failure, who had had moderate cognitive deficit, craniofacial dysmorphism, epilepsy, panic attacks and congenital heart disease (subvalvular aortic stenosis) associated with chronic atrial fibrillation since childhood. In view of his facial dysmorphism and clinical presentation, karyotype analysis was performed and revealed a de novo...

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...

Aortic valve dysfunction may be the consequence of congenital malformation of the aortic valve. Unicuspid,'2 bicuspid,'-6 tricuspid,23 and quadricuspid68 aortic valves have all been described. We describe a patient who presented with cardiac enlargement caused by aortic valve incompetence and stenosis. Echocardiograms repeatedly showed multiple closing lines in the aortic valve orifice. This pa...

ease (RHD). Certain forms of congenital heart disease such as tetralogy of Fallot, small ventricular septal defect, and aortic stenosis are at particular risk for BE. Following BE, patients with ventricular septal defect and tetralogy of Fallot have less morbidity and higher survival rates than children with aortic outflow lesions. Over the entire time period, alpha Streptococcus is the most co...

For more than 30 years, the development of concentric left ventricular (LV) hypertrophy in pressure overload was considered adaptive because the parallel deposition of new sarcomeres and the corresponding LV wall thickening succeeded in normalizing LV systolic wall stress despite the high intracavitary systolic pressure. In aortic stenosis, the validity of this paradigm was demonstrated by haem...

Care of pregnant patients with congenital heart disease requires understanding of the specific congenital defect, the nature of previous surgical correction, and the residua and sequelae. General risks and principles can be adduced in management decisions. In addition, lesion- and patient-specific details are important. There are only a few conditions that place patients at a high enough risk t...