Hypoplastic Left Heart Syndrome

The hypoplastic left heart syndrome consists of a hypoplasia of the left ventricle and the ascending aorta, a malformation and hypoplasia of the aortic and mitral valve (often is an aortic atresia before), an atrial septal defect and an open ductus arteriosus. If the normal closure of the ductus arteriosus is not prevented by a Prostaglandininfusion enters a cardiogenic shock resulting in death. A loud, single second heart sound (S2) and a non-specific systolic murmur are common. The diagnosis is made with the emergency echocardiography. The treatment of choice is a multi-stage surgical correction.

The hypoplastic left heart syndrome accounts for 2% of congenital heart defects. Since the mitral valve, the left ventricle and the aortic valve are hypoplastic (often with aortic atresia), is oxygenated blood flowing in the left atrium is diverted from the lungs through the atrial wall connection in the right heart, where it is systemic to the entsättigtem mixed venous return (Hypoplastic left heart.). This deoxygenated blood leaves the right ventricle through the pulmonary artery toward the lungs and through the ductus arteriosus in the systemic circulation. The circulation is maintained only through the shunt between the right ventricle and the ductus arteriosus. So the immediate survival depends on a patent ductus arteriosus.

The hypoplastic left heart syndrome consists of a hypoplasia of the left ventricle and the ascending aorta, a malformation and hypoplasia of the aortic and mitral valve (often is an aortic atresia before), an atrial septal defect and an open ductus arteriosus. If the normal closure of the ductus arteriosus is not prevented by a Prostaglandininfusion enters a cardiogenic shock resulting in death. A loud, single second heart sound (S2) and a non-specific systolic murmur are common. The diagnosis is made with the emergency echocardiography. The treatment of choice is a multi-stage surgical correction. The hypoplastic left heart syndrome accounts for 2% of congenital heart defects. Since the mitral valve, the left ventricle and the aortic valve are hypoplastic (often with aortic atresia), is oxygenated blood flowing in the left atrium is diverted from the lungs through the atrial wall connection in the right heart, where it is systemic to the entsättigtem mixed venous return (Hypoplastic left heart.). This deoxygenated blood leaves the right ventricle through the pulmonary artery toward the lungs and through the ductus arteriosus in the systemic circulation. The circulation is maintained only through the shunt between the right ventricle and the ductus arteriosus. So the immediate survival depends on a patent ductus arteriosus. Hypoplastic left heart. The left ventricle, the ascending aorta and the aortic and mitral valves are hypoplastic. Similarly, an atrial septal defect and patent ductus arteriosus much is present. AO = aorta; IVC = inferior vena cava; LA = left atrium; LV = Left ventricle; PA = pulmonary artery, PDA = ductus arteriosus; PV = pulmonary veins, RA = right atrium; RV = right ventricle; SVC = superior vena cava. Symptoms and signs Symptoms begin when the ductus arteriosus closes within the first 24-48 hours of life. Then rapidly, the clinical picture of cardiogenic shock (eg. As tachypnea, dyspnea, weak pulses, paleness, cyanosis, hypothermia, metabolic acidosis, lethargy, oliguria, anuria) developed. In case of deterioration of the circuit situation, the coronary and cerebral blood flow is reduced, resulting in myocardial and cerebral infarctions. If the ductus arteriosus does not open immediately, the baby dies. The study shows a very active precordium associated with a very poor peripheral circulation, cold extremities, bluish-gray skin color and missing or barely perceptible pulses with a significant parasternalen lifting. The second heart sound (S2) is noisy and individually. Sometimes a low non-specific systolic murmur is heard. A severe metabolic acidosis is typical. Diagnostic chest X-ray and ECG echocardiogram Diagnosis is clinically suspected especially in newborns with metabolic acidosis that worsens after receiving O2; O2 lowers the pulmonary vascular resistance, thus increasing the relative proportion of the right ventricular output, which flows into the lungs rather than through the patent ductus arteriosus in the body. The diagnosis is confirmed with the emergency echocardiography. A cardiac catheterization is necessary often to diagnose. The radiograph shows cardiomegaly, increased pulmonary vascular pattern or pulmonary edema. The ECG can usually recognize a right ventricular hypertrophy. Treatment infusion of prostaglandin E1 (PGE1) Multi-stage surgical correction Sometimes heart transplant all affected newborns should be immediately transferred to a neonatal intensive care unit or a cardiac intensive care unit for children and stabilized. A vascular access should be established quickly through a umbilical catheter and / or peripheral intravenous, whichever is faster. PGE1 (initial 0.01-0.1 ug / kg / min i.v.) infused to stop the closure of the ductus arteriosus or to open it again. The newborn must normally be intubated and ventilated. The metabolic acidosis is corrected with intravenous sodium bicarbonate (NaHCO3). Seriously ill newborns with cardiogenic shock may need inotropic drugs (eg. As milrinone) and diuretics to improve heart function and control the volume status. It is important to keep the pulmonary vascular resistance is relatively high and systemic vascular resistance to a minimum to prevent a significant pulmonary circulatory stress at the expense of systemic perfusion. This resistance areas are maintained by avoiding hyperoxia, alkalosis and Hypocarbie which can all lead to pulmonary vasodilation. Because O2 is one of the strongest pulmonary vasodilators, infants are ventilated with room air or hypoxic gas mixtures to achieve systemic saturation of 70-80%. If the child needs mechanical ventilation, Pco2 can be kept in the high normal or slightly elevated area. The systemic vascular resistance is controlled by the avoidance or minimization of vasoconstrictor drugs (eg. As epinephrine or high-dose dopamine). Milrinone can be advantageous because it can cause systemic vasodilation. Tips and risks The pulmonary vascular resistance is relatively high and the systemic vascular resistance kept low to prevent an increasing pulmonary circulatory stress at the expense of systemic perfusion. Therefore, hyperoxia, alkalosis and hypocapnia (the pulmonary vasodilation caused) avoids and minimizes the use of vasoconstrictors. The survival depends on a multi-stage surgical operation method that allows the right ventricle to assume the function of a systemic ventricle and to control pulmonary blood flow. The first stage, the Norwood procedure is performed during the first week of life. The Hauptpulmonalarterie is severed, closed distal end with a patch and the hypoplastic aorta and pulmonary artery proximal be combined into a Neoaorta. The ductus arteriosus is ligated. The pulmonary blood flow is obtained by substituting a right-side modified Blalock-Taussig shunt (tetralogy of Fallot: Final Operation) restored or a right ventricular-pulmonary artery conduit (Sano modification). Finally, the Vorhofseptumkommunikation is increased. An alternative hybrid process, often a joint effort of cardiac surgeons and interventional cardiologists, inserting a stent into the ductus arteriosus comprises (to the systemic blood flow to maintain), and the onset of bilateral branch pulmonary arteries bands (around the pulmonary blood flow limit). In some clinics, the hybrid method patients with a higher risk (eg. As premature babies, those with multiple organ dysfunction) reserved. Procedimiento de Norwood var model = {thumbnailUrl: ‘/-/media/manual/professional/images/norwood_surgical_correction_high_blausen_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / norwood_surgical_correction_high_blausen_de. ? jpg lang = en & thn = 0 ‘, title:’ Procedimiento de Norwood ‘description:’ u003Ca id = “v37897571 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eEn el procedimiento de Norwood (etapa 1) se establece una fuente de flujo sanguíneo pulmonary mediante la inserción de una de derivación Blalock-Taussig modificada del lado derecho (parte superior). Se secciona el tronco pulmonar

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