He Gets to Ten More Years From Acleistocardia

Brook was a 24-year-old man. He had acleistocardia which means a failure of the foramen ovale of the heart to close. Transthoracic echocardiography and multiple-detector computed tomography showed that the aortic valve was pentacuspid, which consisted of three relatively equal cusps and two larger or smaller cusps. Color Doppler showed severe aortic regurgitation and mild regurgitation due to left ventricular enlargement. Based on Brook's case which is rare, the doctor may cautiously conclude that the sizes of cusps vary among deformed pentacuspid aortic valves. One cusp may or may not coaptate with the other four cusps, and if they can not coaptate well then aortic regurgitation will occur and progress, which finally requires aortic valve replacement. The pentacuspid artic valve is an extremely rare form of congenital heart malformation. It may be accompanied with other malformations, such as acleistocardia, coronary–pulmonary artery fistula, and prolapse of mitral valve. In some cases, transthoracic echocardiography may not be able to provide adequate information on the pentacuspid artic valve due to poor acoustic window or angle; instead, transesophageal echocardiography and multiple-detector computed tomography may provide a good choice for diagnosis. The preoperative imaging results of these two techniques are very close to the real intraoperative findings; therefore, their clinical values are promising. He had to go through a Pulmonary valve replacement which is rarely performed and is usually done with biological prostheses or grafts. The use of mechanical prostheses is still a debated issue. The doctor analyzed the outcome after pulmonary valve replacement with a mechanical prosthesis. During follow-up, Brook remained asymptomatic, with no arrhythmias and good anticoagulation. An echocardiogram showed preserved ventricular contraction and normal function of the prostheses, with low pressure gradients. No infective endocarditis occurred. Mechanical prostheses appear to be a good choice for pulmonary valve replacement. Surgical and clinical results were satisfactory with no complications, acceptable pressure gradients, and good ventricular function after more than 10 years. Lifelong anticoagulation is mandatory, but this is usually well tolerated. Brook was miraculously granted more than 10 years of lifespan. However, after the 10 years, he would have to go through the surgery again and the risk would increase by 60 due to his age.