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Atrial septal defect is one of the most common lesions in adult congenital cardiac anomalies. It is characterized by the defects occurring in structures that separate the two atria, and thus permit shunting of blood from the high pressure atrium to the low pressure atrium. Patients may remain asymptomatic during the first few decades of life. Most patients are accidentally diagnosed in cardiology clinics after presenting with a heart murmur incidently, and fixed split second heart sound, right bundle branch block on ECG or signs of right heart dilatation on chest X-ray or transthoracic echocardiography. Later an exertional and dyspnea, atrial arrhythmia may become the common presentation that guides diagnosis.

Cor triatriatum is a rare congenital cardiac anomaly in which a membranous structure divides the left atrium into two chambers. The distal chamber consists of pulmonary veins and the proximal chamber includes true left atrium and left atrial appendage. The communication between the pulmonary venous chamber and the rest of the atrium is usually restrictive; therefore, it commonly results in pulmonary venous congestion. The most common associated lesion with cor triatriatum is atrial septal defect. The atrial septal defect can either communicate with the pulmonary venous chamber or with the proximal chamber. A large secundum atrial septal defect or even two separate defects can communicate with the two chambers.

Normal aortic valve is trileaflet. Congenital forms of valve disease are either unicuspid, bicuspid, or quadricuspid. Bicuspid valve is the most common congenital aortic valve disease, where the aortic valve is anatomically made of two leaflets or more but often functioning as bileaflet valve due to leaflet fusion. In this case, the overall valve function may be normal for years until late in life when calcification develops and the valve becomes stenotic or regurgitant. Although development of aortic stenosis or regurgitation of a bicuspid aortic valve is slow, fast deterioration can be triggered by en docarditis. This may destroy the valve and cause either significant stenosis or severe regurgitation. More than half of the patients with bicuspid aortic valve may present with aortic root dilatation partially due to intrinsic abnormality of aortic wall and partially due to poststenotic dilatation caused by eccentric jet.

Patent ductus arteriosus is a blood vessel connecting the proximal left pulmonary artery to the descending aorta just distal to the left subclavian artery. During fetal life, the ductus arteriosus is a vital structure that bypasses the pulmonary circulation. A persistent shunt that remains patent well after birth is usually managed either surgically or interventionally by device (mostly coils) implantation early in life. Most adult cases with patent ductus arteriosus are small or silent without clinical hemodynamic significance. Moderate-sized ductus with significant left-to-right shunt causes left heart enlargement, and some degree of pulmonary hypertension are rarely seen. Large ducts in adults usually result in Eisenmenger physiology with eventual right-to-left shunt that may not be easy to diagnose using echocardiography. These patients have distal (lower body) cyanosis and toe clubbing.

Ebstein malformation is the most common form of congenital tricuspid valve disease. It is defined as apical displacement of parts of the hinge point of the tricuspid valve leaflets, within the right ventricular cavity and away from the atrioventricular junction. It is almost always accompanied by dysplasia of the leaflets. Leftward and inferior displacement of the proximal attachment is the most common form of Ebstein anomaly that may involve the septal and posteroinferior (mural) leaflets, whereas the anterosuperior leaflet is usually normally attached, but enlarged with a sail-like appearance.

Valvar pulmonary stenosis can present as isolated congenital anomaly or as part of other complex congenital heart abnormalities, such as tetralogy of Fallot, transposition of great arteries, or syndromes such as Noonan syndrome.

Tetralogy of Fallot is the most common form of cyanotic heart anomalies, accounting for approximately 10% of all congenital heart diseases. In anatomic terms, this malformation consists of four components: subpulmonary infundibular stenosis, ventricular septal defect, overriding of the aorta, and right ventricular hypertrophy.

The arrangement in which both atria connect to one ventricle is described as double inlet ventricle. Univentricular atrioventricular connection by means of an absent left or right connection is frequently referred to in the literature as mitral and tricuspid atresia. In most “univentricular hearts”, there are two ventricular chambers with one major (dominant) ventricle and a second rudimentary chamber. The rudimentary ventricle is usually situated anteriorly either rightward or leftward and is more often of right ventricular morphology. The pulmonary artery usually arises posteriorly from the large main chamber, which frequently is of left ventricular morphology, whereas the aorta usually rises anteriorly from the rudimentary outlet chamber. Where the associated ventricular septal defect is small in this setting, it leads effectively to subaortic stenosis.

There is a considerable anatomical variety with these entities. The two most common patterns associated with actual or potential compromise of coronary artery flow and myocardial perfusion are:

Nearly all adult patients with congenital heart disease (repaired, palliated, or unoperated) are at risk of infective endocarditis. Echocardiography plays an important role in the diagnosis and follow up of patients with endocarditis, and its unique high frame rate makes it ideal for detecting even small vegetations that could easily be missed by other noninvasive imaging techniques. Echocardiography also assists in clinical decision making, for example, surgical intervention during the treatment period. The most common lesions susceptible to superimposed infection, are those of the left ventricular outflow tract, which include subaortic stenosis, aortic valve disease, and post aortic valve replacement. The second high risk group of lesions are small unoperated ventricular septal defects and tetrology of Fallot.