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Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Hypoplastic left heart syndrome is characterised by underdevelopment of left heart structures. This is uniformly fatal in the first few weeks of life without intervention. Treatment includes prostaglandin therapy to maintain ductal patency, followed by resuscitation with inotropes, intubation and judicious ventilatory management to minimise pulmonary overcirculation, which is invariably present because of the need to maintain ductal patency in order to maintain systemic perfusion to the descending thoracic aorta via the ductus. Occasionally, subambient oxygen is needed in order to increase pulmonary vascular resistance and avoid pulmonary vascular congestion. Pulmonary overcirculation is manifested by tachypnoea, tachycardia, intercostal retractions and higher than usual oxygen saturations, typically >85%. Ideal oxygen saturations for single ventricle patients should be 75%–85%, which provides enough of a balance between systemic and pulmonary perfusion. Treatment is via the Norwood procedure, which involves aortic arch augmentation with a patch, a side-by-side anastomosis of the main pulmonary artery and the diminutive ascending aorta, to reconstruct a neoaortic outflow from the ventricular mass. Pulmonary blood flow is via a modified Blalock– Taussig shunt (from the innominate artery to the pulmonary artery) or via a Sano shunt (from the right ventricle to the pulmonary artery). Subsequent operations are the Glenn procedure (at 6 months of age) and the Fontan procedure (at approximately 2–3 years of age).
The Child With Suspected Congenital Heart Disease
Published in Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan, Diagnosing and Treating Common Problems in Paediatrics, 2017
Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan
In cases where an antenatal diagnosis has been made, to maintain patency of the ductus arteriosus, prostaglandin should be commenced immediately after birth. Where an antenatal diagnosis has not been made, neonates presenting with circulatory collapse should be resuscitated and commenced on prostaglandins. Surgical management of hypoplastic left heart syndrome involves three stages. The Norwood procedure is performed in the newborn period. The right ventricle is converted into the main ventricle for both the pulmonary and systemic circulation. A Blalock–Taussig shunt or Sano shunt is created to shunt blood to the pulmonary circulation. If coarctation is present, it is corrected. Post Norwood, children’s oxygen saturations are usually 70%–80%. Aspirin is given to prevent stent thrombosis. Children have a 75% survival rate at this time. At 3–5 months of age, a Glenn procedure is performed when circulation through the lungs no longer requires high ventricular pressure. The shunt is taken down and the superior vena cava is connected directly to the pulmonary circulation. The final stage takes place 18–36 months after the Glenn. The inferior vena cava is connected to the pulmonary circulation. At this stage, all deoxygenated blood flows passively into the lungs, so there is no mixing of oxygenated and deoxygenated blood.
Cardiac catheterization for the adult with complex congenital heart disease
Published in Debabrata Mukherjee, Eric R. Bates, Marco Roffi, Richard A. Lange, David J. Moliterno, Nadia M. Whitehead, Cardiovascular Catheterization and Intervention, 2017
A patient with a functional single ventricle typically proceeds with palliation, including the systemic shunt (Norwood opera- tion, Potts shunt, Waterston shunt, or other central shunts), followed by the SVC shunt or Glenn operation, and ultimately, the completion of the caval pulmonary shunt with the Fontan operation (Figure 20.10). Congenital defects that may neces- sitate such palliation include hypoplastic left heart, tricuspid atresia, pulmonary atresia with intact ventricular septum, or unbalanced complete AV canal. The ACHD patient with single-ventricle physiology is usually palliated with a Fontan, but few patients remain with a single lung supplied by a SVC to the pulmonary artery shunt (Glenn) or a systemic artery to pulmonary artery shunt. The Fontan procedure represents the final palliative procedure for single-ventricle physiologic status. This procedure completes the direction of the remain- ing systemic venous blood from the IVC and hepatic veins to the pulmonary arteries. This is accomplished in most cases by either an external conduit or an intraatrial lateral tunnel, which courses from the lateral and inferior aspect of the RA. [30]The atrial appendage or superior vena caval stump transected during the Glenn procedure is directed to the pulmonary artery, effectively “septating” the circulation. Pulmonary blood flow is achieved passively without the assistance of a ventricu- lar pumping chamber. For this reason, it is imperative to have low pulmonary pressures and vascular resistance.
Hypoplastic left heart syndrome (HLHS): molecular pathogenesis and emerging drug targets for cardiac repair and regeneration
Published in Expert Opinion on Therapeutic Targets, 2021
Anthony T Bejjani, Neil Wary, Mingxia Gu
The most common treatment for HLHS consists of three consecutive procedures done within the first 4 years of life: the Norwood, Glenn, and Fontan procedures. In brief, the Norwood procedure creates a new systemic circuit in the heart by redirecting blood from the left atrium into the right ventricle and connecting the aorta to the main pulmonary artery via a BT or Sano shunt [66,67]. This leads to the mixing of oxygenated and deoxygenated blood in the right ventricle. Despite this connection, pulmonary and systemic circulations are still in parallel. The Glenn procedure separates the superior vena cava from the RA and connects it directly to the pulmonary artery to be taken to the lungs, while the inferior vena cava remains connected to the RA. Finally, the Fontan procedure joins the superior and inferior venae cavae to the pulmonary trunk which is separated from the right ventricle, joining the left and right atria, and connecting the aorta to the right ventricle [68]. At the end of these procedures, the right heart would be solely responsible for the systemic circulation, with the venae cavae emptying directly into the pulmonary artery. A fenestration between the inferior vena cava and the RA remains to help reduce pressure to the lungs, while they adjust to the new circulation. At the end of the Fontan procedure, oxygen-rich and oxygen-poor continue to mix in the single-ventricle heart, while the pulmonary and systemic circulations are effectively in series.
Overview and surgical aspects of Uhls anomaly
Published in Expert Opinion on Orphan Drugs, 2020
Kotturathu Mammen Cherian, Lydia Jeris W, Mohamed Thayub
Glenn procedure is a good choice wherein right atrial reduction, right ventricular free wall plication and bidirectional cavopulmonary anastomosis are done [39,40]. Tricuspid valve insufficiency is usually noted secondary to enlarged right ventricle. Bidirectional Glenn is preferred as it will lead on to volume reduction leading on to increased tricuspid valve competence [41]. Delayed sternal closure can be attempted leaving the chest open for 24 hours to have a better hemodynamic control and for placement of epicardial implantable cardioverter-defibrillator (ICD). Oxygen supplementation should also be provided for a few days post-surgery till the body adjusts to single ventricle. After a few months, the oxygen saturation would pick up in the range of 80 to 90% and the patient becomes asymptomatic [42]. There is usually no recurrence of arrhythmias post-surgery because of the ICD placement.
Anesthetic management of a single ventricle pediatric patient with a major burn injury, case report
Published in Egyptian Journal of Anaesthesia, 2020
Ahmed Haroun M. Mahmoud, Ahmad Tareq Aboalfaraj, Turki Abdullah Almalki, Ahmed Mounir A. Metwally
The Glenn procedure is one of the steps of palliative surgeries performed to correct hypoplastic left heart syndrome and other single- ventricle heart defects. It is usually performed about six months after birth. In this operation, the superior vena cava is connected to the right pulmonary artery. Venous return from the head and upper limbs passively flow into the pulmonary artery and proceeds to the lungs to be oxygenated. However, oxygen-poor blood returning to the heart from the lower body through the inferior vena cava will mix with oxygen-rich blood in the left heart and circulate to the body so that the saturation remains in the low 80% range. This operation is preparation for the final step which is the Fontan procedure [1–3].