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Grown-up Congenital Heart Disease (GUCH): Difference between revisions
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Development of complete atrioventricular block is the most significant of the transcatheter procedural complications. Approximately 6% of patients who underwent transcatheter closure of membranous defects, developed complete heart block necessitating pacemaker implantation. Real-time three-dimensional transesophageal echocardiography has been increasingly used to guide such procedures. Given the lack of data on long-term outcomes following catheter closure of VSD in adults, patients should be followed every one to two years at an adult congenital heart disease center. | Development of complete atrioventricular block is the most significant of the transcatheter procedural complications. Approximately 6% of patients who underwent transcatheter closure of membranous defects, developed complete heart block necessitating pacemaker implantation. Real-time three-dimensional transesophageal echocardiography has been increasingly used to guide such procedures. Given the lack of data on long-term outcomes following catheter closure of VSD in adults, patients should be followed every one to two years at an adult congenital heart disease center. | ||
=== Atrioventricular septal defect === | |||
==== Case report ==== | |||
==== Introduction ==== | |||
The atrioventricular septal defect (AVSD) consist of several different lesions with a common atrioventricular (AV) junction and abnormal AV valves, consisting of five leaflets (figure 5). The AVSD makes 3 procent of all congenital heart defects in children. | |||
When the AVSD is complete it consists of a defect on the atrial and on the ventricular side of the common AV-valve ring. (figure 5, middle). The complete AVSD is usually associated with Down Syndrome, but also with other cardiac defects like ASD type 2, persisting left inferior caval vein and tetralogy of Fallot. | |||
In an incomplete AVSD the superior and inferior bridging leaflets are connected with each other and with the interventricular septum in the centre. (figure 5, right) Due to this connection there are two divided AV inlets, leaving no open communication between the ventricles, thus no VSD exists. However there is a rather large defect in the interatrial septum. The incomplete AVSD is often referred to as ostium primum defect or ASD type 1. | |||
The left AV-valve consist of three leaflets (there is a cleft in the mitral valve) and is usually incompetent. Due to one common AV junction in both types of AVSD, the aortic valve is not in the usual wedged position between the two separate AV inlets, but located more anterior. Therefore the outflow tract of the left ventricle is elongated and slightly constricted. In angiography this abnormally shaped outflow tract is known as a gooseneck. | |||
==== Pathophysiology ==== | |||
The exact pathophysiology depends on the location and severity of the defect. In a complete AVSD there is combined right and left ventricular overload due to the left-to-right shunt at atrial and ventricular level combined with the regurgitation of the right and left AV valve. As a result the elevated pulmonary pressures due to the extremely high flow will rapidly convert to pulmonary hypertension. This will lead to bidirectional shunting across the defect with a preferably right-to-left shunt and cyanosis (Eisenmenger syndrome). | |||
In an incomplete AVSD the hemodynamic consequences are comparable to an ASD type 2. However the serious left AV-valve regurgitation can cause an increase in the atrial left-to right shunt. | |||
==== Evaluation ==== | |||
The complete AVSD leads to symptoms of heart failure early in childhood and most patients will have been surgically corrected in adulthood. During physical examination signs of a residual shunt or regurgitation of the AV-valves might be present. This defect is rarely diagnosed in adulthood and usually there is Eisenmenger syndrome present, with clinical signs previously described in isolated ASD or VSD. The clinical symptoms of an incomplete AVSD are comparable with those of an ASD type 2. During physical examination a murmur of the regurgitant AV-valve might be audible. | |||
The ECG shows a deviation of the heart axis to the left, in contrast to the right axis deviation in atrial septal defects. This is due to the abnormal position of the His bundle which causes a delay in conduction through the left anterior fascicular branch. | |||
==== Treatment and outcome ==== | |||
The survival for patients with a complete AVSD without corrective surgery is very limited, most patients will not reach adulthood. Therefore surgical correction at an early age (usually in the first year of life) is advised. Patients with a corrected complete AVSD need lifelong cardiologic follow up, due to frequent residual defects like residual VSD, progressive regurgitation of the left AV-valve, pulmonary hypertension and often rhythm- or conduction disorders. Patients with Down Syndrome and complete AVSD are more likely to develop progressive pulmonary hypertension, even after corrective surgery. | |||
The aberrant anatomy of the AV-valves, even after corrective surgery, is important when reviewing echocardiographic images. They can, for example, be easily mistaken for vegetations in endocarditis. | |||
The survival for patients with an incomplete AVSD is higher compared to the complete AVSD, but generally worse compared to other ASD types. This is due to the concomitant disorders of the left AV-valve and the conduction system. Left AV-valve regurgitation will lead to an increase in left-to-right shunt and earlier development of pulmonary hypertension compared to ASD type 2. In childhood there is usually already an indication for correction of the defect in which the anatomically abnormal AV-valve can be repaired. However a slight amount of regurgitation will remain present. In some cases the progressive failure of the AV-valve will require a second repair or replacement, but in most patients the insufficiency will remain mild. | |||
Besides the AV-valve problems there are frequent rhythm and conduction disorders; atrial fibrillation, supraventricular tachycardia, complete heartblock or sinus dysfunction. Depending on the kind of disorder patients can require medical treatment or a pacemaker | |||