Welcome to the Multidisciplinary Critical Care Review Course for Pediatrics. This presentation is on Congenital Cardiac Malformations. My name is Ravi Theogarajan. I'm the Chief of the Cardiac Intensive Care Unit at Boston Children's Hospital. These are my disclosures. I want to acknowledge that illustrations used in this presentation were the kind courtesy of Boston Children's Hospital Department of Cardiology Image Library. In this presentation, I hope to give you an overview of congenital cardiac disease. The talk does not contain all details of all forms of congenital heart disease, but perhaps the most important ones. I want to provide you a little bit of epidemiology and a general approach to the diagnosis of children with congenital heart disease and describe circulation and management of common forms of congenital heart disease. To start off with some epidemiology of congenital cardiac malformations, congenital cardiac malformations occur in 4 to 10 cases per 1,000 live births, usually reported as 8 per 1,000 live births. The estimated prevalence of congenital heart disease in the US in 2002 was 650,000 to 1.3 million people living with congenital heart disease. The mortality for congenital heart disease is declining, and therefore the prevalence of congenital heart disease, including those of adults with congenital heart disease, is growing. Children with congenital heart disease are often admitted to intensive care units, either in the course of their medical management or following cardiac surgery. And about a third of children with congenital heart disease require at least one hospitalization in infancy, often in intensive care units. It is important to recognize that congenital heart disease is often a component of a number of genetic syndromes. For example, Down syndrome or trisomy 21, 40% of children born with Down syndrome have some form of congenital heart disease, with ventricular septal defects or VSTs being the most common. Similarly, in patients with Dijard syndrome or 22q deletion, tautology of fallot, interrupted arch, or truncus arteriosus is seen in 40% of patients with Dijard syndrome. So genetic syndromes are commonly associated with congenital heart disease, and they're often associated with specific forms of congenital heart disease. And that gives you a clue to what to look for in patients with specific genetic syndromes. Extracardiac anomalies are often commonly present in children with congenital heart disease. For example, gastrointestinal or genitourinary tract abnormalities, CNS abnormalities, and lung abnormalities are common in children with congenital heart disease. Congenital heart disease can also occur in children with nonsyndromic associations, such as vactral or CHARGE associations. In vactral syndrome, which is vertebral anomalies, anal atresia, tracheoesophageal fistula, renal defects in limb, and skeletal abnormalities, 50% of these children have congenital heart disease, and they're typically associated with ventricular septal defect, double-loaded right ventricle, or tautology of fallot. So if you have a child with a constellation of extracardiac anomalies, it's important to look for cardiac defects in these patients. Now that we've heard a little bit about the incidence prevalence of congenital heart disease and the genetic syndromes and other extracardiac anomalies associated with congenital heart disease, let's turn to congenital heart disease itself. Congenital heart disease commonly is classified into asynodic congenital heart disease or cyanotic congenital heart disease. In cyanotic congenital heart disease, the predominant presenting feature of congenital heart disease is cyanosis. Asynodic heart disease consists of children with left-to-right shunt abnormalities, such as ASDs and VSDs, or out-protective obstructions, such as aortic stenosis. Cyanotic congenital heart disease include patients with altered pulmonary blood flow, such as tetralogy of fallot, or truncus arteriosus, or total anomalous pulmonary weakness return, and single ventricle congenital heart disease. If you look at the prevalence of common forms of congenital heart disease, ventricular septal defects are the most common forms of congenital heart disease, followed by pulmonary stenosis, endocardial cushion defect, atrial septal defect, tetralogy of fallot. Complex forms of congenital heart disease, such as single ventricle congenital heart disease, tend to be relatively rare compared to these defects. Let's turn to evaluation of children and making a diagnosis of congenital heart disease. Like any other disease, taking a history and physical exam are really the cornerstones of evaluation and diagnosis of congenital heart disease. Specific to examination of children with heart disease is four-limb blood pressure measurement, which measures blood pressures in all four limbs. And if you have a gradient with the upper limb blood pressure being higher than the lower limb blood pressure, then that's often a sign of coarctation. Pulse oximetry is commonly used as a way of screening tool for children with critical forms of congenital heart disease, and it's now part of newborn evaluation in the newborn nursery. EKG, chest x-ray, echocardiography, cardiac catheterization, and specialized testing, such as CT scan and MRI, are used to specifically define the type of congenital heart disease, the intracardiac anatomy, both for diagnostic as well as planning surgical or interventional procedures for management of congenital heart disease. Pulse oximetry has become an important tool in the screening of normal newborn for the presence of critical congenital heart disease and is adopted in many newborn nurseries. A room mass saturation of less than 95% in the leg 24 hours post-birth suggests critical congenital heart disease, and these children require evaluation by a specialist to rule out critical forms of congenital heart disease. The hyperoxia test can be used in the evaluation of newborn with congenital heart disease when differentiating a pulmonary cause compared to a cardiac cause for cyanosis desaturation or hypoxia. Here, a pre-ductal arterial blood gas is obtained in rumen and 100% oxygen. Newborn with cyanosis or desaturation related to lung disease will show a progressive increase in PaO2 and saturation, whereas children with cyanotic congenital heart disease will not increase their oxygen saturation. Echocardiography and cardiac catheterization are the cornerstones of the diagnosis and management of children with congenital heart disease. Echocardiography is perhaps the most commonly used tool. It is noninvasive, can be done at point of care, can help define anatomy for surgical planning as well as patient management. It also provides physiological information such as measurement or estimation of pressure gradients across areas of narrowing. For example, in a patient with pulmonary stenosis, it can help measure a gradient across the pulmonary valve that helps define the severity of pulmonary stenosis. It can estimate chamber pressures. For example, in patients with pulmonary hypertension, one could estimate the right ventricular pressure using tricuspid valve regurgitation. One could estimate function of the ventricle using ejection fraction, such as in a patient with dilated cardiomyopathy. And therefore, it is one of the most commonly used tools in children for evaluation of children with congenital heart disease. Cardiac catheterization, on the other hand, is less frequently used, but it's invaluable in the assessment of the severity of congenital heart disease, perhaps assessing physiological aspects of congenital heart disease, and providing intervention for many forms of congenital heart disease. Cardiac catheterization helps measure pressures across chambers, can help measure gradients across areas of obstruction, and measure a number of physiological aspects of cardiovascular functions, such as cardiac output, estimate intracardiac shunts, left or right shunts, and measure resistances, such as systemic and pulmonary vascular resistances. The formulas for the physiological aspects of cardiac catheterization are shown on this slide, and these are really important to know for managing children with congenital heart disease, and for estimating various aspects of cardiovascular function and physiology. Now that we've heard a little bit of an overview of the incidence, prevalence of congenital heart disease, its association with genetic syndromes, diagnostic tools, and evaluation of children with congenital heart disease, let's turn our attention to specific congenital heart disease. Perhaps the most common forms of congenital heart disease are septal defects, and these include atrial septal defects, ventricular septal defects, and children with AV canal defects. Atrial septal defects, or ASDs, ASDs are defects in the atrial septum and result in a left to right shunt at the atrial level, so left atrium to right atrial shunt, that results in right ventricular volume overload and dilation. Osteum secundum ASDs that occur in the septum secundum are perhaps common forms of ASDs. Osteum premum ASDs that occur in septum premum may be associated with the AV canal type defects as well as cluffs of the mitral valve. Ventricular septal defects are defects that occur in the intraventricular septum and result in a left to right shunt at the ventricular level, so blood flows from the left ventricle to the right ventricle, resulting in volume load to the left heart structures and dilation of the left atrium and the left ventricle. A VSD that's larger than the size of the aortic valve often results in a large left to right shunt. Children with ventricular septal defects that are large often present in infancy with left ventricular volume overload and congestive heart failure. The type of VSD depends on the location of the VSD in the intraventricular septum. VSDs that occur in the inlet portion of the septum are often associated with AV canal type defects. VSDs that occur in the outflow portion of the septum, such as conoventricular VSDs, are often the VSDs seen in patients with tetralogy of fallot or truncus arteriosus. Membranous and muscular VSDs are also common. VSDs occur in the muscular portion of the intraventricular septum and may close spontaneously. Cardiac catheterization can help pinpoint the location and estimate the magnitude of left to right shunts in septal defects. And this may be important for planning either catheter-based closure of septal defects or surgical intervention. For atrial septal defects, the fully saturated blood from the left atrium mixes with desaturated blood from systemic venous return at the level of the atrium. So step up in oxygen saturations occur between the systemic venous saturation and the right atrium. Ventricular septal defects, on the other hand, fully saturated blood from the left ventricle mixes with systemic venous return at the level of the right ventricle. And therefore, step up in saturations occur at the level of the right ventricle. Therefore, cardiac catheterization and measuring saturations in various chambers of the heart can help pinpoint the location of a septal defect. Using a formula and using saturations data from cardiac catheterization, one could estimate the magnitude of left to right shunts using QPQS or the amount of pulmonary and systemic blood flow ratio. And this helps determine the magnitude of the left to right shunt and to decide whether a surgical or catheter-based intervention for closure of the septal defect is warranted. Children with ASDs often have their ASDs either closed interventionally in the cath lab with their older children or their younger children have it surgically closed, usually at about after five years of age. For children with ventricular septal defects, closure of VSDs depend on the size of the VSD and symptomatology, such as congestive heart failure. Some ventricular septal defects, depending on the size of the ventricular septal defects, often require closure in infancy. Another common form of congenital heart disease is coarctation of the aorta, which is obstruction to the left heart at the level of the aorta. In coarctation of the aorta, narrowing of the aorta occurs at the level of the PDA or patent ductus arteriosus. The coarctation can be post-ductal, can be juxtaductal, or pre-ductal. Regardless, the physiology is really severe narrowing of the aortic arch. Because there's narrowing of the aortic arch, blood pressure distal to the coarctation is usually lower than those proximal to the coarctation. Typically, the right arm blood pressure is much higher than the lower limb blood pressures, resulting in a gradient. And depending on the severity of the coarctation, the coarctation can present in the newborn period with cardiogenic shock. And these children often need their ductus opened with prostaglandins. Because of the increased afterload of the left ventricle, left ventricular dysfunction and congestive heart failure are common features. Coarctation of the aorta is seen in association with Turner syndrome. And patients with coarctation of the aorta may also have cerebral aneurysms. Management of coarctation depends on severity of coarctation. Patients who have severe coarctation presenting in the newborn period have to be stabilized with prostaglandins. The left ventricular function needs to be supported and improved. And then once stabilization has occurred, then the coarctation is repaired with an end-to-end anastomosis. In patients who have less severe coarctation or coarctation that is diagnosed at a late may have elective repair of coarctation. Post-operative issues after coarctation repair include systemic arterial hypertension. This is more common in patients who have less severe forms of coarctation that are diagnosed later and often require antihypertensive agents, both in the post-operative period as well as in the post-follow-up period. Symptomatic laryngeal nerve injury can occur in children with coarctation. And this is something that we as intensivists should watch for in the post-operative period. Moving on to other forms of congenital heart disease, Tetralogy of Fallot is a common form of cyanotic congenital heart disease. The features of Tetralogy of Fallot include a large conoventricular VSD, right ventricular outflow tract obstruction, either at the level of the valve or below the valve or sometimes even above the valve, aortic override, the aorta overriding the crest of the ventricular septum, and RV hypertrophy. Hypertrophy of the right ventricle results in the classic diagnostic feature of Tetralogy of Fallot in chest X-rays, which is called the boot-shaped heart, where the apex of the heart is lifted because of right ventricular hypertrophy. Cyanosis in children with Tetralogy of Fallot results from right ventricular outflow tract obstruction, which, when severe enough, results in a right-to-left shunt at the ventricular level. So blue blood from the right ventricle mixes with pink blood from the left ventricle, resulting in desaturation or cyanosis. Thus, the severity of the right ventricular outflow tract obstruction determines the systemic oxygen saturations by influencing the magnitude of right-to-left shunt across the ventricular septal defect. Older children with Tetralogy of Fallot who lived with chronic cyanosis can be polycythemic. Tet spell or acute hypocyanotic episodes in children with Tetralogy of Fallot results from an acute increase in right ventricular outflow tract obstruction, resulting in a larger right-to-left shunt and, therefore, desaturation. And this can happen at times of infundibular vasospasm that could be associated with agitation, crying often during procedures. They can also result from loss of systemic vascular resistance, such as during a febrile illness or administration of anesthesia, where a reduction in systemic vascular resistance increases the magnitude of right-to-left shunt in children with Tetralogy of Fallot. Management of an infant with Tetralogy of Fallot and an acute hypocyanotic episode, especially in the context of agitation, requires providing comfort and supplemental oxygen. If an IV is available, a dose of morphine or volume expansion may be useful. Raising the infant in knee-chest position, where the knees are drawn into their chest, can increase systemic vascular resistance and reduce the magnitude of right-to-left shunt. However, care should be taken not to agitate an already upset child and prolong an acute hypocyanotic spell. Bolus-dosed phenylephrine administered intravenously can raise systemic vascular resistance and reduce the magnitude of right to left shunt. This is especially useful when systemic vascular resistance may be reduced during administration of an anesthetic. Intubation ECMO and emergency surgery may be necessary, rarely, in patients who have an acute hypersynodic spell that's refracted to medical management. In patients who are progressively becoming synodic, use of a beta blocker, such as azomole or propranolol, can reduce right ventricular outflow tract spasm and improve pulmonary blood flow. Children with tautology or fallot require surgical correction of right ventricular outflow tract obstruction and closure of VSD. Indications for surgery include cyanosis or a TET spell. And in patients who are asymptomatic, correction at six months of age is generally what's followed in most centers. The surgical approaches include complete repair, where the VSD is closed and the right ventricular outflow tract is relieved through a patch, or a systemic to pulmonary artery shunt, such as a BTT shunt or a Baloc-Tarsic-Thomas shunt, followed by complete repair at an older age is also practiced in some centers. Other forms of tautology or fallot include tautology or fallot with pulmonary atresia, where there's atresia of the pulmonary valve or the main pulmonary artery. And depending on the size of the branch pulmonary artery and the presence of AP collaterals, these patients require establishment of continuity of the right ventricular outflow tract and the branch pulmonary arteries. Another form of tautology or fallot is tautology or fallot with the absent pulmonary valve or dysplastic pulmonary valve. Here the absent pulmonary valve or dysplastic pulmonary valve results in free pulmonary regurgitation and dilation of the main pulmonary artery and branch pulmonary arteries that can compress the bronchi, right and the left bronchi, resulting in airway obstruction. These are also repaired using an RV to PA conduit. Another form of cyanotic congenital heart disease is transposition of the great arteries. In detransposition of the great arteries, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. So this is desaturated blood from systemic venous return, enters the right ventricle and is pumped into the systemic circulation. Highly saturated blood from pulmonary venous return enters the left ventricle and then is pumped into the pulmonary arteries. So the two circulations are in parallel. And saturation and cardiac output in patients with detransposition depends on mixing. Mixing at the ductus level or mixing at a patent foramen ovale level or at atrial level communication. The best and most efficient areas of mixing is at the atrial level followed by the ductal level and if a VSD is present at the VSD level. Detransposition is associated with ventricle septal defects in 40% of cases. Detransposition with VSD and may be associated with varying coronary artery configurations. In patients with detransposition of the great arteries, hypoxemia and acidosis can occur because of inadequate mixing. Management of patients with inadequate mixing resulting in hypoxemia and acidosis requires maintaining a patent ductus arteriosus patency with prostaglandins. An emergent echocardiogram is necessary to assess the status of the atrial communication. And in patients who have an inadequate atrial communication, an emergent pulmonary septostomy is necessary to improve mixing. These patients may also require intubation, 100% FiO2, volume expansion, correction of acidosis, inotropic support, inhaled nitric oxide. And rarely, in some patients where there is inadequate mixing, ECMO is necessary. Balloon atrial septostomy can be done at the bedside and in patients who require balloon atrial septostomy, the atrial septum is accessed through access at the IVC or sometimes the umbilical vessels. A balloon catheter is passed across the atrial septum through an existing foramen ovale. The balloon is blown up in the left atrium and then pulled across the septum to create an atrial communication. Patients with detransposition of the great vessels need surgical correction. The preferred operation is arterial switch operation where the great arteries are switched such that the aorta that arose from the right ventricle is switched across to the left ventricle and the pulmonary artery that arose from the left side of the heart is connected to the right ventricle. The coronaries have to be re-implanted into the new aorta. Patients whose diagnosis is dismissed at greater than eight weeks of age, the left ventricle which is hooked up to the pulmonary circulation which is a low resistance circulation may result in involution of the left ventricle and the left ventricle may need to be retained with a PA band prior to the arterial switch operation. The atrial switch operation, the mustard ascending operation where the atrial return is directed from the right side to the left side of the heart and therefore the pulmonary arteries and the left-sided atrial return is directed to the right side of the heart and therefore into the aorta is rarely undertaken as a primary operation these days. Total anomalous pulmonary venous return is another form of cyanotic congenital heart disease. In total anomalous pulmonary venous return, the connection between the pulmonary veins and the left atrium is erratic. Therefore pulmonary venous return coming back from the lungs is decompressed via a vertical vein to a systemic venous circulation. Therefore there's common mixing of the pulmonary vein and systemic vein circulation that then enters the right atrium and is distributed via the foramen ovale to the left side of the heart for systemic output and via the tricuspid valve for pulmonary blood flow. If the veins are, the vertical vein is unobstructed, this results in right atrium and right ventricular volume overload. If the vertical vein is obstructed, then pulmonary venous return coming back to the confluence cannot empty and therefore these patients present with severe hypoxemia and severe pulmonary edema and chest x-ray. This constitutes an emergency and this needs the total anomalous pulmonary venous return that is obstructed needs emergency operation to relieve the obstruction. The types of total anomalous pulmonary venous return include supradiaphragmatic or supracardiac TAPVR where the vertical vein enters the venous circulation in the thorax often into the vertical vein or the SVC or infradiaphragmatic TAPVR where the vertical vein enters the systemic venous circulation below the diaphragm or infradiaphragmatic TAPVR often in the pulmonary vein. The infradiaphragmatic TAPVR is more likely to be obstructed and requires emergency care and emergency operations. Patients with obstructed total anomalous pulmonary venous return are critically ill with refractory hypoxemia and respiratory distress and a typical chest x-ray looks like the one showed here with severe pulmonary edema, sometimes even whiteout with lungs. Surgical management of obstructed pulmonary venous return is reconnection of the confluence of the pulmonary artery to the left atrium and ligation of the vertical vein. In patients who do not have obstruction, the TAPVR repair can be done more electively. Postoperative issues in patients with total anomalous pulmonary venous return include pulmonary hypertension that may require management with inhaled nitric oxide in the postoperative period. Other forms of congenital heart disease include single ventricle congenital heart disease such as hyperplastic left heart syndrome. Hyperplastic left heart syndrome is a constellation of left heart hyperplasia which may include atresia or hyperplasia of the mitral valve, hyperplasia of the left ventricle, atresia or hyperplasia of the aortic valve and the ascending aorta. In patients with hyperplastic left heart syndrome, fully saturated blood that enters the left atrium cannot traverse into the left side of the heart and therefore crosses the patent for amino valley and mixes with systemic venous return coming back into the right atrium. The commonly mixed blood then traverses via the tricuspid valve into the right ventricle and is pumped into the pulmonary arteries where it's distributed to the systemic circulation via a patent ductus arteriosus and the pulmonary circulation via the branch PAs. The distribution of blood to the systemic circulation and pulmonary circulation is determined by a balance of pulmonary vascular resistance and systemic vascular resistance. If the pulmonary vascular resistance is low or the systemic vascular resistance is high then blood flows preferentially into the pulmonary circulation and results in low cardiac output or cardiogenic shock. If the patent ductus arteriosus is crucial for maintaining systemic circulation and loss of the patent ductus arteriosus, such as in a patient with undiagnosed hyperplastic left heart syndrome, the presentation is usually cardiogenic shock. Similarly, an unobstructed communication between the left atrium and the right atrium is necessary to maintain circulation in children with hyperplastic left heart syndrome. If the patent foramen ovale is restrictive, then left atrial hypertension and pulmonary edema can occur. In hyperplastic left heart syndrome, peripheral oxygen saturation is the same as pulmonary artery oxygen saturation because it's common mixing of blood in the right atrium. Therefore, QPQS is calculated as systemic saturation minus SVC saturation divided by pulmonary vein saturation minus pulmonary artery saturations. Systemic saturations and pulmonary saturations are equal. So if your peripheral oxygen saturation is 80%, then QPQS would be 80 minus SVC saturation, which is 60. If your pulmonary veins are fully saturated or 100 minus 80, which is a one-to-one distribution of QP and QS. If your systemic oxygen saturations are 90%, and if the SVC saturation is 60 and the left atrial saturation is 100, then QPQS is 90 minus 60 divided by 100 minus 90, which is three-to-one. So if your peripheral oxygen saturation is high, then the amount of pulmonary blood flow is high and the amount of systemic blood flow is low. So peripheral oxygen saturations often give you an idea of how pulmonary and systemic oxygen saturations are distributed in children with hypoplastic left arch syndrome with higher saturations indicating higher QP and lower QS. And ideally, one would like to have these saturations, peripheral oxygen saturations in the 80% to 85% range to balance QP and QS. Preoperative management of hypoplastic left arch syndrome includes maintaining the patency of the PDA using a prostaglandin infusion and allowing spontaneous ventilation in room air. Where possible, intubation and administration of oxygen should be avoided as these interventions may result in a reduction in pulmonary vascular resistance and steal from the systemic circulation into the pulmonary circulation, resulting in low cardiac output. In patients who present with cardiogenic shock because of loss of the PDA, require immediate institution of a prostaglandin infusion to open the PDA. Many of these patients may need mechanical ventilation. And if mechanical ventilation is initiated, then care must be taken to ventilate these patients as much as possible in room air, not overventilating the patient, maintaining carbon dioxide in the 45 to 50 range to maintain pulmonary vascular resistance and to avoid dropping pulmonary vascular resistance and stealing from the systemic circulation. Inhaled carbon dioxide and hypoxic gas mixture with the use of nitrogen to raise pulmonary vascular resistance are rarely used these days. Many of these patients may need inotropic support to support the function of the right ventricle and rarely inodilators to reduce systemic vascular resistance to allow increase in QS with infusions such as milrinone may be needed. Many of these patients also need diuresis because a large amount of QP results in pulmonary overcirculation and wet lungs. Patients with hyperplastic leptarotid syndrome require surgical intervention in the newborn period. The first of the operations is stage one palliation, which consists of disconnecting the pulmonary outflow tract and creating a systemic outflow tract using the pulmonary artery, anastomosing the aorta to the pulmonary artery called the Damus case stencil anastomosis, reconstruction of the narrowed aortic arch, atal septectomy, and then creating a source of pulmonary blood flow either using a belag-tostig-thomas shunt or a sauna conduit or an RV to PA conduit. Following the stage one palliation operation in the newborn period, the systemic venous and systemic arterial circulation are separated through a series of operations, the first of which is a Glenn operation, where the systemic venous return from the head and neck vessels are brought into the pulmonary arteries. This is usually undertaken at three to six months of age. The Fontan operation then brings the inferior vena cava blood also into the pulmonary arteries, thus separating the pulmonary and systemic arterial circulation. And this is usually undertaken at two years of age. So children with hyperplastic leptarotid syndrome undergo a staged operations to separate the systemic and pulmonary venous circulations. Anomalous origin of the left coronary artery from the pulmonary artery or al kappa. This lesion is in the differential diagnosis of cardiogenic shock in early infancy, usually presenting at around 10 weeks of age with cardiogenic shock. In children with anomalous origin of the left coronary artery from the pulmonary artery, the left coronary artery arises from the pulmonary artery instead of the aorta. The physiology is myocardial ischemia brought on by left ventricle that's supplied by desaturated blood from the pulmonary artery and communications between the right and the left coronary artery eventually leads to coronary steel as the pulmonary vascular resistance decreases, resulting in myocardial ischemia and infarction. The clinical presentation of anomalous origin of the left coronary artery from pulmonary artery includes cardiogenic shock approximately at 10 weeks of age as the pulmonary vascular resistance decreases. Chest x-ray usually shows cardiomegaly and the EKG has typical findings of Q waves in lead I, ABL, and V4 to V6, which is the distribution of the left coronary artery. This is a typical chest x-ray in a patient with anomalous origin of the left coronary artery from the pulmonary artery showing cardiomegaly and pulmonary edema. The EKG shows deep Q waves in this EKG in lead I and AVF and in V4 to V6, which is the distribution of the left coronary artery. Preoperative management of children with anomalous left coronary artery from the pulmonary artery includes management of cardiogenic shock and ventricular arrhythmias in the preoperative period, which may include intubation, inotropic support, and rarely extracorporeal membrane oxygenation. The surgical aspects of correction of this lesion include re-implantation of the left coronary artery into the aorta or creation of a tunnel that connects the aorta to the left origin of the left coronary artery in the pulmonary artery called the Takiyuchi's operation. Postoperative issues in children who've had repair of an anomalous left coronary artery from the pulmonary artery include continuation of cardiogenic shock, increased predisposition to ventricular arrhythmias that may need to be managed, and some patients may need extracorporeal life support to support the function of the left ventricle. Conditions of truncus arteriosus, tricuspid atresia, other forms of cyanotic congenital heart disease, and vascular rings are provided in the slides at the end of this lecture. Thank you very much for listening to this lecture on congenital cardiac malformations.

congenital cardiac malformations

children

genetic syndromes

echocardiography

surgical interventions

preoperative care

postoperative care