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Grown-up Congenital Heart Disease (GUCH): Difference between revisions
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Grown-up Congenital Heart Disease (GUCH) (view source)
Revision as of 14:56, 25 January 2012
, 25 January 2012→Pathophysiology
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=== Pathophysiology === | === Pathophysiology === | ||
[[File:15. HLHS.svg|thumb|left|Figure 15. Schematic drawing representing the hypoplastic left heart syndrome.]] | [[File:15. HLHS.svg|thumb|left|Figure 15. Schematic drawing representing the hypoplastic left heart syndrome.]] | ||
The hypoplastic left heart syndrome (HLHS) is the most common type of univentricular heart. (Figure 15) Not only the left ventricle, but often the aortic valve, ascending aorta and aortic arch are hypoplastic as well. This will redirect blood from the left atrium into the right atrium, where is will be mixed with venous blood and pumped into the right ventricle and pulmonary artery. The whole systemic circulation depends on the shunt from pulmonary artery through the ductus arteriosus into the aorta. When the ductus starts closing the consequences are dramatic, with severe cyanosis and acidosis. | The hypoplastic left heart syndrome (HLHS) is the most common type of univentricular heart. (Figure 15) Not only the left ventricle, but often the aortic valve, ascending aorta and aortic arch are hypoplastic as well. This will redirect blood from the left atrium into the right atrium, where is will be mixed with venous blood and pumped into the right ventricle and pulmonary artery. The whole systemic circulation depends on the shunt from pulmonary artery through the ductus arteriosus into the aorta. When the ductus starts closing the consequences are dramatic, with severe cyanosis and acidosis. | ||
[[File:24. univentricular heart.PNG|thumb|right|Figure 24. Echocardiographic image of a male patient with a univentricular heart.]] | |||
When a hypoplastic right ventricle is present with associated atresia of the pulmonary artery, the pulmonary circulation after birth will solely depend on the left-to-right shunt through the ductus arteriosus.When the ductus starts closing, progressive cyanosis is the main presenting symptom. | When a hypoplastic right ventricle is present with associated atresia of the pulmonary artery, the pulmonary circulation after birth will solely depend on the left-to-right shunt through the ductus arteriosus.When the ductus starts closing, progressive cyanosis is the main presenting symptom. | ||
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All patients with one functioning ventricle have complete mixing of saturated and desaturated blood leading to chronic hypoxemia ( | All patients with one functioning ventricle have complete mixing of saturated and desaturated blood leading to chronic hypoxemia (Figure 24). Furthermore there is a chronic volume overload to the ventricle, serving as both pulmonary and systemic ventricle, leading to an early development of heart failure. | ||
Due to the obligatory intracardiac shunting the pulmonary ‘filter’ is bypassed, which will increase the chance of cardiovascular accidents and brain abscesses. | Due to the obligatory intracardiac shunting the pulmonary ‘filter’ is bypassed, which will increase the chance of cardiovascular accidents and brain abscesses. | ||