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CONGENITAL HEART DISEASE PDF

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4/18/NursePub/UCSF & Mt Zion Nursing Services/Unit Documents/6picu/ cardiac defects pixia-club.info 2. Congenital Heart Defects. CHD with Increased. result in congenital heart defects. ♥ Discuss 3 important components of a cardiovascular assessment. ♥ List the 3 cardinal signs of congenital heart disease. Congenital Heart Disease. • Abnormalities of the heart/ great vessels since birth. • Incidence higher in premature infants. • Faulty embryogenesis during


Congenital Heart Disease Pdf

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Heart Disease & Stroke Statistical Update Fact Sheet The most common complex congenital cardiovascular defects* and percent. ABSTRACT. Congenital heart defects (CHD) are cardiovascular malformations that generally occur due to aberrant development of a normal structure in the. Congenital heart defects may be classified into acyanotic and cyanotic depending upon whether the patients clinically exhibit cyanosis.

Spin Echo — uses a radiofrequency pulse that tilts the hydrogen protons by 90 degrees followed by a second degree pulse, which are used to generate images.

This provides static anatomic information, with excellent blood myocardium contrast. Used for assessing cardiac tumours, pericardial disease and thoracic masses. Takes a relatively longer time. Used to assess ventricular function and flow of blood in the cardiac pathways and for identifying stenotic or regurgitant jets.

This produces stripes across the image, tagging the myocardium. As the heart moves the tags are followed and this allows calculation of myocardial strain. Velocity encoded Cine MRI- can be used to measure blood flow velocity and quantify blood flow rate. Contrast enhanced MRI — Uses gadolinium chelate which produces bright blood signal used for clear delineation of spatial relationship and for imaging of baffles and outflow tracts.

ESC Clinical Practice Guidelines

Hyper enhancement of myocardial regions observed minutes after administration of gadolinium contrast is indicative of scar tissue and irreversible myocardial injury. MRI in patients younger than years would require sedation, Surgical clips, sternotomy wires, coils stents and occluding devices are MRI safe once the surrounding fibrous tissue grows over these implants and makes them immobile.

Cardiac pacemakers, presence of intracranial, intra ocular or intracochlear implant are considered contraindication to MRI. Cardiovascular MRI is fast becoming a tool which can provide us with anatomic and functional information not provided by echo or cardiac catheterization.

Catheterisation used to be the main diagnostic modality available when it was first introduced in The era of angiographic anatomic delineation is fading. Echocardiography is now preferred for evaluation of valvar and congenital cardiac defects with 3D echo promising real time surgical images of the valves for repair. MRI and CT angiography is fast replacing angiogram for delineation of complex relationship, volume estimation of chamber, extra cardiac vessels, aortic arches and venous anomalies.

The field of MR imaging, has the potential to completely replace diagnostic angiography. The routine use of catheterization before single ventricle surgeries is being questioned, as the same information can be made available through non-invasive means. Visualising branch PA anatomy beyond hilum- in case of tetralogy of Fallot with aortopulmonary collaterals. Measurement of flow, pressure, reactivity and resistance of pulmonary vasculature to various drugs in patients with elevated pulmonary artery pressure.

O 2 is used as the indicator and all the systemic organs or lungs are considered as one organ to estimate the systemic or pulmonary output respectively. The consumption is made available through estimates based on age, sex and heart rate. Systemic output would be the difference between the arterial oxygen content and mixed venous oxygen content ideally measured in the middle of right atrium to average for the superior vena caval, inferior vena caval or coronary sinus venous blood whose oxygen content may be different.

Pulmonary output would be the difference between the pulmonary venous and pulmonary artery oxygen content. Pressure measurements are based on fluid filled catheters, or large bore catheters with multiple side holes which would yield accurate description of intracardiac waveforms.

PVR Pulmonary vascular resistance would be transpulmonary gradient difference between the mean PA and LA pressures divided by the pulmonary flow. With the availability of modified catheters and catheter delivery devices, the role of intervention in pediatric cardiology is increasingly becoming important. Open things that are closed: Atrial septostomy — done in infants who need mixing of blood for maintaining saturation and cardiac output.

Most common indication is transposition of great arteries with intact ventricular septum, other rarer indications are TAPVC with restriction at the interatrial septum, tricuspid atresia, pulmonary atresia, mitral atresia.

Widen things that are narrow — involving the blood vessels and heart valves, done using balloon catheters and stents to prevent recoil in case of vessels. The stent gets incorporated into the vessel wall. There can be neointimal proliferation causing restenosis, usually in the first 2 years The procedure uses special plastic polymer balloons which will not inflate beyond predetermined size even under high pressure.

This needs a guide wire to be placed across the narrow area with balloon placed across so as to place the waist middle of the sausage in the narrow region which is dilated with dilute contrast. Coarctation of aorta — preferred for re-coarctation or for coarctation in grown up children where it can be combined with stenting. Not for coarctation in infancy which has high rate of recurrence and surgery is better option. Mitral valves — works well for rheumatic heart disease, not very successful with congenital MS.

Heart block, tearing of leaflet and occurrence of MR are possible complications. Close things that are open — this uses devices mounted on catheters that are passed through long and large sheaths after crossing the area to be closed. Amplatzer devices are FDA approved and have the longest track record. Closure of VSD requires careful assessment to ensure that the device would not interfere with tricuspid or aortic valvar mechanisms.

Arrhythmias, stroke, perforation, device embolization, incomplete closures are the risks involved. The thrombus formation around the coil plugs the vessel. Embolization, incomplete closure and hemolysis are possible risks. Other defects which have been plugged are ruptured sinus of Valsalva especially the ones arising from the non-coronary sinus, aorta LV tunnels, veno-venous collaterals plugging following cavopulmonary connection.

The success of any pediatric cardiac surgical programme depends on a team effort and anesthetists and intensive care personnel are critical members of the team. The importance of effective communication between the various team members cannot be overemphasized.

Care of pediatric patients and care of the neonates in particular needs appreciation of the differences in physiology of the immature organ systems. The neonate responds rapidly to any stressful situations which reflect in the sudden changes of various metabolic and hemodynamic parameters. The metabolic rate of neonates is fold have increased compared to adults.

The Basis of Management of Congenital Heart Disease

The myocardium has a reduced ability to respond to afterload stress and the compliance is reduced. Acute pressure afterload is poorly tolerated, and can lead to rapid ventricular dysfunction; chronic pressure load is longer tolerated than chronic volume load.

Symptoms of CHF are rare unless the obstruction is severe and prolonged. The stroke volume is relatively fixed and cardiac output is more heart rate dependent. They are more dependent on the trans-sarcolemma movement of calcium to initiate and sustain contraction as the sarcoplasmic reticulum and T-tubules are relatively underdeveloped. Cardiorespiratory interactions and ventricular interdependence are particularly marked in infants.

Positive pressure ventilation reduces the preload of both ventricles, increases the afterload of right ventricle and reduces the afterload of left ventricle. Implying that in situations where right ventricle is dysfunctional or in situations of single ventricle, early extubation with reduced afterload would be useful and in situations of left ventricular dysfunction, positive pressure ventilation would reduce the afterload of the heart and act as positive inotrope. This principle should be tempered against the background of ventricular interdependence where dysfunction of one ventricle can rapidly affect the other due to septal interactions.

Neonates rely on diaphragm as the main muscle of respiration. Raised intra-abdominal pressure due to any cause like gastric distension, hepatic congestion and ascites can compromise its function. In neonates a larger portion of energy expenditure is used for ventilation and therefore they fatigue easily and have failure to thrive in the presence of increased work of breathing.

They have increased closing capacity with airway closure occurring during normal tidal ventilation putting them at risk of developing hypoxemia and atelectasis. In addition dilated pulmonary arteries and left atrium can compress bronchi causing lobar collapse. Pulmonary hypertension both pre and postoperatively plays an important role in the planning of surgery, anesthesia and postoperative care. Preoperatively it can be due to large left to right shunt lesions, or due to pulmonary venous obstruction, rarely due to pulmonary vascular obstructive disease.

Intraoperatively- light anesthesia, hypoxemia, hypoventilation, lung hyperinflation or hypoinflation, hypothermia, respiratory and metabolic acidosis, protamine, blood products, prolonged bypass with inflammatory response and capillary leak, compression and atelectasis of lung, pulmonary edema from inadequate venting of left atrium can all contribute to increased pulmonary vascular resistance PVR.

Intravenous drugs can be used to reduce the PVR, but they lack selectivity and can cause systemic hypotension. Nitroprusside, glyceroltrinitrate, milrinone, Prostaglandin E1 and I2, tolazoline and isoproterenol have been used.

Inhaled NO is most selective pulmonary vasodilator currently available; it is rapidly taken up and inactivated by haemoglobin as it diffuses from the alveoli. Oral drugs in the form of PDE type V inhibitor, sildenafil and endothelin I blocking drugs bosentan have shown encouraging results. Preoperative evaluation should keep in mind the physiology of defect, and the changes that the preoperative treatment could have caused diuretics causing hypokalaemia and the presence and severity of cyanosis and pulmonary hypertension.

Avoiding dehydration is very important to avoid tissue hypoxia and to maintain renal function postoperatively. Non-invasive monitoring using electrocardiography, pulse oximetry, capnography, non-invasive blood pressure is placed before induction, invasive arterial and central venous line should be planned according to procedure. Neurologic monitoring and cerebral protection is of concern during congenital heart surgery. Nasopharyngeal temperature, continuous EEG, transcranial Doppler, frontal lobe infrared spectroscopy and cerebral oximetry can be used to evaluate cerebral blood flow velocity and perfusion.

Intraoperative echocardiography has achieved a significant role in repair of CHD. It helps in re-evaluation of anatomy before intervention, adequacy of surgical repair and de-airing after weaning from cardiopulmonary bypass and has become a integral part of monitoring in many units including ours.

Maintenance of diastolic pressure and coronary perfusion is important particularly in the setting of duct dependent lesions and in situations of altered coronary perfusion. There is a choice of induction techniques, inhalational, IV or IM. Midazolam 0. Isoflurane and midazolam can be used during bypass for maintenance and blunt awareness.

Reducing stress response using high dose opioid anesthesia and extending this to immediate postoperative period was considered important to reduce morbidity and mortality. With changes in surgical practice and particularly the timing of surgery, a strategy of using high dose opiod may be a less critical determinant of outcome. The main aim however is to maintain hemodynamic stability so that the team can focus on surgery without the distraction of side effects of anesthetic drugs.

A co-ordinated approach must be used during weaning so that a smooth transition can be ensured - there should be close communication between surgeon, anesthesiologist and perfusionist. The need for vasopressors and inotropes is decided by close observation of the heart during re-warming period. Once adequate temperature is reached, ventilation is restarted after a good inflation of the lungs to prevent atelectasis and for deairing.

Acid base status is normalized, adequate heart rate is ensured and the drainage from venous cannula is slowly reduced and CPB is stopped. Sternal closure is frequently delayed in neonates due to this reason. Avoiding hypothermia and hypoglycaemia, maintenance of optimal filling pressures, and preventing abdominal distension due to ascites by using catheter which can be used for peritoneal dialysis are important in the early postoperative period.

An LA line and PA line may be inserted by the surgeons in the theatre in situations when LV dysfunction is anticipated or when pulmonary artery pressures are labile.

This would help to fine tune management and help in decision making with regards to inotropes and ventilation management. Postoperative management requires a precise knowledge about the anatomy, pathophysiology and details of the surgical and CPB technique.

For most patients the recovery is uncomplicated and whenever the clinical progress does not follow the expected course possible residual or additional defects should be investigated. Inadequate analgesia while the patient is on ventilator may be manifested by tachycardia, hypertension, pupillary dilation, diaphoresis etc, Changes in the respiratory pattern like tachypnea, grunting and splinting of the chest wall may also be seen.

Fever, hypoxemia, hypercapnia, seizures, vasoactive infusions, early low cardiac output may also present in similar fashion and should be ruled out. Opioid analgesics are the mainstay of pain management as they blunt hemodynamic response to procedures such as endotracheal suctioning.

Disadvantage is that it can cause histamine release with systemic vasodilation and elevation of PVR. There is wide variation in the metabolism of fentanyl, tolerance and dependence develops rapidly and chest wall rigidity can develop as a rare idiosyncratic reaction.

It blocks the stress response and maintains systemic and pulmonary hemodynamic stability. Dexmedetomidine commonly called the dexmed is alpha-2 agonist which is increasingly being used due to its sedative, anxiolytic, and its non-respiratory depressant property. This can cause hypotension and bradycardia and should be used with caution in children with CHD. Recognition and early intervention for the management of low cardiac output is one of the pivotal roles of the intensivists.

The following are some of the clues and the entire clinical picture should be considered rather than a isolated finding. Physical examination: Core hyperthermia, tachycardia, cool peripheries with impalpable peripheral pulses, hypotension with narrow pulse pressure, ascites, hepatomegaly, oliguria, obtundation of sensorium.

Monitoring — Dampened arterial upstroke, narrow pulse pressure, elevated venous pressures systemic or pulmonary — loss of sinus rhythm, residual outflow obstruction, tamponade, AV valve regurgitation should be ruled out.

Strategies for management of Low CO should focus on optimizing the balance between oxygen supply and demand. Demand — Maintaining adequate analgesia, sedation and paralysis when necessary, strict avoidance of hyperthermia and occasionally using mild hypothermia to reduce metabolic rate. For optimizing delivery — Oxygen content can be optimized by managing Hemoglobin and fiO2, and the factors which determine output.

Requirement greater than 0. Calcium infusion may also be necessary especially in patients with diGeorge syndrome and 22q11 deletion. Afterload — Milrinone 0. Nitroprusside and GTN can be used in the setting of normal ventricular function. Norerpinephrine upto Heart rate and sinus rhythm —temporary atrial and ventricular pacing wires are kept after any major cardiac procedure and can be used to optimize the heart rate, though a little trial and error would be required to find the best settings for the individual patient.

No consistent correlation has been found between the serum cortisol level and low cardiac output state. Due to risks of infection and poor wound healing its use should be restricted to 3- 5 days. T3 at dose of 0. Furosemide 0. Fenoldopam is a new selective dopamine DA1 receptor agonist with renal and mesenteric vasodilating properties 0.

Peritoneal dialysis, hemodialysis and continuous veno-venous hemofiltration provide renal replacement therapy for patients with persistent oliguria and renal failure. Besides helping in solute and water clearance, they help in nutritional support by allowing additional volume, and may also remove the inflammatory mediators.

Weaning from ventilation and extubation should be possible once patients are hemodynamically stable and normothermic with good cardiac output. Extubation of an infant after a major cardiac procedure is both an art and science. Residual volume and pressure load, ventricular dysfunction.

Phrenic nerve injury, bronchomalacia, retained secretions, vocal cord injury, Diuretic therapy with contraction alkalosis, Inadequate nutrition and an evolving sepsis are all factors to be considered for failed extubation. Early tracheostomy can sometimes help if there is no correctable lesions, and if predominantly retained secretions and nutrition are the major causes of failed extubation. Cardiopulmonary bypass machine essentially takes over the function of pumping and oxygenating the blood allowing surgery to be performed on arrested still heart.

Even now the morbidity associated with the use of CPB is held to be the major limitation for completely successful outcomes. Due to immature organ function, fetal contractile protein isoforms, immature calcium cycling, pulmonary dysfunction and exaggerated stress and inflammatory response the morbidity associated with CPB is more profound in infants and neonates. Oxygenators - microporous membrane 0. Pumps — Roller pumps are used and the flow rate is governed by the revolutions per minute, occlusion produced by the rollers and the internal diameter of the tubing.

Venous reservoirs — Open rigid reservoirs are usually used to which the blood flows by gravity, the level of blood in the reservoir is an important safety mechanism — source of volume for arterial inflow and to judge adequacy of venous return. Malposition of venous cannulas and lost blood in the surgical field can reduce the venous volume which may need urgent attention. Collapsible venous reservoir with reduced air blood contact is increasingly being used.

Vacuum can improve drainage through small venous cannulas and tubing which can theoretically reduce organ edema and improve organ function; venoarterial air embolism is a potential complication.

Arterial cannula — aorta is the usual site of cannulation, the location needs to be tailored according to surgery. Venous cannula — SVC and IVC cannulas are used to maximize venous return and to minimize interference with the operative field, and optimally sited to prevent obstruction and kinking. Filters — 0. Arterial filter 40um may reduce microemboli, though some consider its use optional. Prime — The fluid used to fill the oxygenator, minimum level required in the reservoir and the tubing is the priming volume.

The prime consists of physiologic crystalloid solutions, packed red cells, colloids like albumin and FFP, and mannitol used for its membrane stabilization, antioxidant and osmotic diuretic properties. At low temperature and low flows, low hematocrit may improve microvascular flow and oxygen delivery.

ACT should be maintained between seconds to prevent activation of blood coagulation and clot formation. Inadequate concentration of heparin is believed to contribute to excess coagulation and fibrinolytic system activation. Once the arterial and venous cannulae are in place, after confirming adequate anticoagulation and absence of air especially at the arterial cannula and tubing is confirmed, CPB is slowly initiated by beginning arterial inflow and unclamping the venous line.

Any systemic to pulmonary shunts should be closed prior to, or immediately after CPB initiation. These can contribute to systemic runoff, contributing to organ malperfusion, increased left heart return, heart distension, and inadvertent rewarming. It is absolutely essential to prevent myocardial distension at all times after initiation of CPB.

Arterial line pressures typically in the range of mm Hg to drive blood through the infant arterial cannula and tubing to maintain mean pressures around mm Hg. Pump flow rate - which is a calculated value in roller pumps based on rotation, internal tubing diameter and occlusion pressure. Temperature- thermistors measure the temperature of the water bath, arterial and venous blood.

The gradient between the patient and the perfusate should not exceed 10 degree C, especially during rewarming to prevent formation of gaseous bubbles. Hypothermia delays loss of ionic hemostasis, slows consumption of ATP, decreases free radical generation, inflammatory cytokine production, white cell activation and leucocyte adhesion molecule synthesis, suppresses the release of excitatory amino acid neurotransmitters. It continues to be the mainstay for cerebral and other organ protection during CPB.

Patient mean arterial and venous pressures — femoral arterial line preferred especially in small infants and in deep hypothermia. Nasopharyngeal reflecting brain temperature , rectal or bladder core and esophageal aortic temperatures are measured using appropriate thermistors, slow cooling over a period of minutes- is necessary whenever deep hypothermic circulatory arrest DHCA is planned.

Arterial and venous blood gases are measured every minutes. Oxygen saturation of venous blood SvO2 and blood lactates are an important index of tissue perfusion and SvO2 can also be measured continuously using calibrated inline monitor.

Blood glucose should be monitored frequently particularly in neonates and infants who are prone to hypoglycemia due to low glycogen reserves. Alpha stat - maintains electrical neutrality at lower temperatures, pH measured is 7. Advantages are better preservation of metabolic functions and buffering capacity, useful during mild and moderate hypothermia. The coagulation factors reach the adult level at 6 — 12 months of age. The effects of CPB on blood activation and coagulation is far greater in neonates and infants because of hemodilution, hypothermia, greater shear stress, and more blood- air contact activation.

Platelets are the initial therapy for bleeding after adequate heparin reversal. Cryoprecipitate which is a good source of fibrinogen is the next blood component usually used. Antifibrinolytic agents like aprotinin, e-aminocaproic acid, tranexamic acid have become popular to reduce bleeding after complex surgeries and in infants.

Lung, kidney and brain are at risk of injury during CPB. The stress and inflammatory response is times greater in neonates and infants. High dose steroids have been used to mask the response and have been shown to be more beneficial in neonates and infants. Ultrafiltration, both conventional and modified are being used for hemoconcentration, removing inflammatory mediators, and decreasing total body water, with beneficial effects being shown for hematocrit, oxygenation, pulmonary vascular resistance and decreasing duration of mechanical ventilation.

Wide application in pediatric age group is limited by the need for miniaturization and ECMO is still the most common form of mechanical circulatory support for pediatric patients. ECMO is now accepted modality of treatment in neonates with a variety of parenchymal and vascular lung disease meconium aspiration, diaphragmatic hernia, persistent hypertension of newborn.

The outcome irrespective of the indication depends on early diagnosis, prompt institution and reversible nature of dysfunction. The use of ECMO for respiratory indications has progressively decreased due to increased availability of High frequency oscillatory ventilation, nitric oxide, and surfactant therapy and there is a steady increase in its use after congenital cardiac surgery or as a bridge to transplantation to support failing circulation.

Venovenous ECMO is used in patients who need ventilatory support alone. Arteriovenous cannulation is used for cardiac ECMO. In the postoperative period single right atrial and ascending aortic cannula is preferred, internal jugular vein and carotid artery can be used in other settings.

The daily management of ECMO needs assessment of cardiorespiratory function, end organ perfusion and evolving complications such as bleeding or sepsis. Assessing adequacy of flow and systemic perfusion is of paramount importance. When instituted in post cardiac surgery setting, myocardial recovery should be anticipated in days, failing which listing for transplantation or withdrawal of support must be considered. Patients with cardiomyopathies or with severe bronchiolitis due to viral infection may require longer period of support weeks.

Weaning depends on the underlying indication. Inotropes are recommenced or increased, intravascular status is optimized and ventilator settings are adjusted, flows gradually decreased over a period of time and the circuit is clamped. ABG, serum lactated levels and mixed venous oxygen saturation levels are closely monitored and decannulation is done once patient has maintained stable circulation and acceptable gas exchange for upto 4hrs.

IABP Intra-aortic Balloon Pump has not been very successful in paediatric population because of a number of reasons, the usual right heart nature of pathology, rapid heart rate in children making timing difficult, distensible nature of aorta and collaterals making coronary flow augmentation and afterload reduction less.

The smallest size available is 2. Technical improvements including smaller sized consoles, catheters and M-mode echocardiography for timing may enhance its applicability in the future. Ventricular assist devices require direct cannulation of heart. Reported indications are decompensated cardiomyopathy, ALCAPA ischemic myocardium as a result of anomalous origin of coronary artery , and retraining of poorly prepared left ventricle after arterial switch procedure.

Grown-Up Congenital Heart Disease (Management of)

They are simple in design and require less technical assistance once established. VAD are more suited as bridge to transplantation. Berlin Heart Excor VAD is the first extracorporeal pneumatically driven pulsatile VAD designed specifically for paediatric use and is available in 6 different sizes depending on stroke volume.

Significant advances in the circulatory support technology for children are expected in the near future with various devices being in preclinical stages of development. Allografts — tissues obtained from human body which are cryopreserved are used in repair of variety of congenital cardiac defects.

Allografts are currently available standards against which any conduit is compared. No particular method is proven to enhance the longevity of allograft.

Decellularisation process using anionic detergents and nucleases have been used to provide acellular matrix for the native tissue to repopulate and grow, but the long term structural impact of the decellularisation process is yet to be seen. Treating the decellularised tissue in a bioreactor using the recipient cells so as to repopulate the matrix is now in experimental stages.

Contegra which is made from Bovine jugular vein is one of the popular valved conduits in pediatric cardiac surgery; This conduit shows predictable function with survival approaching homografts over mid-term. The advantages are easy availability 22mm sizes and predictable quality. Alternative conduits have been used made of Polytetrafluoroethylene PTFE wall and leaflets, which has minimal tissue reactivity.

Long term results are still awaited. Conduits fashioned out of autologous pericardium with PTFE 0. While a variety of choices are available for older children and adults, an ideal conduit for neonates and small children is still elusive. A simplified approach to such a variety of congenital cardiac defects is to broadly classify them as.

Left to right shunts are the most gratifying to treat as they are potentially life-saving and promote the growth of the child. The common variations of ASD [ 29 ] figure 5 are ostium secundum, ostium primum, sinus venosus, coronary sinus and various combinations of these.

Ostium primum ASD is usually associated with abnormal mitral and sometimes tricuspid valves. Repair involves repair of the valves with closure of the ASD with a patch. Failure to close the cleft in the mitral valve increases reoperation rate for mitral regurgitation [ 30 ]. These patients require lifelong follow-up for mitral regurgitation and left ventricular outflow tract obstruction [ 31 ] Complete heart block is a potential complication due to the proximity of the bundle of His.

Sinus venosus ASD is usually superior vena cava SVC ASD with partial anomalous pulmonary venous connection usually right upper lobe pulmonary veins draining anomalously to the right atrium instead of left atrium. Complications range from SVC obstruction, pulmonary venous obstruction and sino-atrial nodal dysfunction. Coronary sinus ASD is rare and involves patch repair. Common atrium is complete absence of interatrial septum and surgery is partitioning of the atria. Autologous pericardial patch is the material of choice.

Ventricular septal defect VSD is deficiency of the interventricular septum. There are various classifications based on anatomy and embryology. Indications for surgery are large VSD with congestive cardiac failure refractory to medical management, left atrial and left ventricular dilatation, aortic valve prolapse and aortic regurgitation and prevention of infective endocarditis [ 34 ] Open heart surgery entails closure directly is small or patch Gore-tex, Dacron, bovine or autologous pericardium.

Patent ductus arteriousus PDA is the persistence of the fetal ductus arterious beyond two months after birth. This results in increased left heart return and failure to thrive with congestive cardiac failure. Surgical approach is via left posterolateral thoracotomy.

Aortopulmonary window APW is a communication between the aorta and pulmonary artery resulting in a large left-to-right shunt. This condition requires early surgical closure.

This condition is rare and requires early surgery as there is steal from the systemic circulation.

Surgical approaches vary but basically consist of division of the APW with suturing of a patch [ 36 ]. Double outlet right ventricle is a separate and complex entity wherein both great vessels arise predominantly from the right ventricle. When there is a VSD, this results in unrestricted pulmonary blood flow and physiologically is a left-to-right shunt. The classification of DORV by Lev is self-explanatory and the varying anatomy decides the clinical presentation which varies from simple left-to-right shunt, tetralogy of Fallot physiology, transposition of great arteries physiology to single ventricle physiology.

They occur with VSD which then presents as increased pulmonary blood flow. Truncus arteriosus figure 8 is a condition wherein the pulmonary arteries arise from the aorta directly.

This results in torrential pulmonary blood flow and warrants early surgery in the neonatal period. The PA is detached from the aorta and reconnected to the right ventricle utilizing a conduit and closing of the VSD.

Alternatives include direct connection of RV-PA utilizing neighbouring tissue. Mid-term and long-term results are determined by the fate of the RVOT- the presence of pulmonary regurgitation and the deterioration of the conduit. Coarctation of aorta is narrowing of the aorta near the insertion of the ductus arteriousus to the descending aorta This presents at various stages. Presentation in the neonatal period is usually as an emergency with closure of the ductus and sudden cessation of blood flow to the lower body.

Treatment includes commencing prostaglandin to reopen the ductus, commencement of inotropes and correction of acidiosis. Emergency surgical repair of coarctation is warranted. Surgical techniques are varied and each has merits and demerits. The preferred technique is resection of the coarctation and end-to-end anastomosis [ 39 ] Alternatives include subclavian flap plasty and patch plasty.

Interruption is total disconnection of the aorta and is usually associated with VSD. The classification is based on the location of the interruption type A is distal to the left subclavian artery, type B is distal arch and type C proximal arch.

Surgery is complex and involves disconnection of the PDA which supplies blood to the lower body and reconnection of the two ends of the aorta. The VSD is closed [ 40 ]. Pulmonary vein anomalies are rare and can present as stenosis of individual veins or as they enter the LA as a confluence.

Prognosis is poor when all four pulmonary veins are involved [ 41 ] Surgery or balloon dilatation are both associated with high rates of restenosis. Tetralogy of Fallot figure 8 is the commonest cyanotic congenital cardiac condition. The condition described originally consists of four components- VSD, overriding of aorta, right ventricular outflow tract obstruction RVOTO and right ventricular hypertrophy.

Babies present early with cyanosis or cyanotic spells due to infundibular spasm. Surgical strategies vary in different parts of the world. In the neonatal period, whenever the baby presents with symptoms, the tendency is surgical correction with closure of VSD and judicious relief of RVOTO.

However, most centers follow the policy of performing Blalock-Taussig shunt if the baby presents with symptoms at less than 6 months of age or less than 5 kgs. If older, surgical correction is attempted [ 42 ]. B-T shunt is still performed if the branch pulmonary arteries are hypoplastic or there are significant co-morbidities that preclude placing the baby on cardiopulmonary bypass. The postoperative course varies depending on the anatomically variations. Since pulmonary arterial pressure plays an important role in pulmonary blood flow of several conditions, the pathophysiology of pulmonary hypertension is discussed.

Pulmonary arterial pressure may be elevated by either increased pulmonary vascular resistance or pulmonary blood flow. These two states must be differentiated as the prognosis is very different. Clinical and laboratory findings, particularly echocardiographic, are described which allow diagnosis and estimates of severity. Finally, the chapter emphasizes that in the evaluation of each patient, a diagnosis must be made, the severity of the condition estimated and an etiology identified if possible.

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Chapter 3.

Walter H. Johnson, Jr. James H.

The incidence of congenital heart disease.

Twenty four percent of these patients had EM. Chromosomal anomalies were identified in 9. In addition, 4. The genitourinary system was the most frequently affected In , Calzolari et al 30 conducted a retrospective study of 1, livebirths and stillbirths with CHD, also in Italy.

Interventricular communication, ostium secundum atrial septal defects and complex heart defects were the cardiac malformations more frequently associated with EM.

The most common EM were found in the musculoskeletal Karyotypical analysis was performed in In , Eskedal et al 31 studied the prevalence of major EM anomalies in children with CHD and its impact on the survival. The registers of children with CHD who were born from to in Norway were analyzed. All the patients had the diagnosis of the heart defect confirmed by echocardiography, surgery, cardiac catheterization or necropsy.

The authors observed an improvement in the survival of children born from to in comparison with those born from to , except for those with EM, whose survival did not differ between the periods. However, patients with Down's syndrome with CHD and no major EM had a better survival in the second period of the study. Also, children with right atrial isomerism were observed to have a higher risk for asplenia.

Still in , Stephensen et al 32 retrospectively evaluated livebirths with CHD from to in Iceland. The diagnosis of the heart defect was confirmed by echocardiography, cardiac catheterization or autopsy.

Chromosomopathies were observed in 4. Among the patients with chromosomopathies and syndromes, genitourinary and gastrointestinal malformations were the most frequently observed EM.

Among those with major heart defects, The prevalence of CHD was 9. In addition, one or more EM were observed in The gastrointestinal and spleen malformations comprised Still in , Wojtalik et al 34 retrospectively studied 1, children admitted for heart surgery in the period from to , in a pediatric tertiary hospital in Poland.

The diagnosis of CHD was confirmed by echocardiography. The presence of other malformations was evaluated by physical examination, cranial and abdominal ultrasound, X-Ray or bronchoscopy, and was detected in 84 children 4. Anomalies of the digestive In children with multiple organs malformations, the multivariate logistic regression analysis detected a significant association of age, the primary heart surgery and the type of CHD with mortality.

The mortality rate of the total sample was 8. EM were more frequently observed among children with ventricular septal defects 7. In the prospective observational study by Meberg et al 19 conducted in Norway in , which evaluated 57, births from to , CHD confirmed by echocardiography or autopsy were observed.

Forty six percent of the EM malformations were not related to any chromosomal anomaly, genetic syndrome, microdeletion or teratogenic syndromes. Atrioventricular septal defects, intra-atrial communication, tetralogy of Fallot and single ventricle were the heart defects most frequently associated with EM.

In the case control study by Amorim et al 2 published in , newborns and 75 stillbirths with CHD diagnosed from to in a University Hospital in Minas Gerais, Brazil, were evaluated. The livebirths had a control group, composed by the next newborn of the same gender, with no malformations, born in the same hospital.

The CHD was confirmed by postnatal echocardiography or necropsy. Noncardiac anomalies were detected in EM constituted part of some syndrome in The most common EM among the newborns were those of the genitourinary system Among the stillbirths with no diagnosed syndromes, EM were more frequently observed in the genitourinary In , Gonzalez et al 20 conducted a retrospective analysis of the medical charts of neonates with a prenatal diagnosis of structural CHD from to , in a general and a cardiologic hospital in the US.

The CHD was confirmed by postnatal echocardiography. The EM detected by cranial and abdomen ultra-sonogram were classified as significant, moderately significant and nonsignificant variants of normal. Abdominal ultrasound performed in Patients with septal defects were 3. The authors concluded, thus, that a universal screening program using the three tests represent a reasonable strategy for the neonates who need a cardiac surgery. The diagnosis of CHD was established by physical examination, electrocardiogram, X-Ray, echocardiography, cardiac catheterization or autopsy.

Congenital rhythm disturbances and cardiomyopathies were also included. During the five years period, 57 3. Among the individuals who did not have any chromosomal disorder or syndrome, the most common EM were the gastrointestinal defects 8. The CHD most frequently associated with extra cardiac malformations were the major heart defects In the retrospective study by Miller et al 36 in , 7, patients with CHD born from to were evaluated in the US.

The CHD was diagnosed by physical examination, autopsy or echocardiography. Newborns, stillbirths and elective abortions from 20 gestational weeks were included. Among the 7, patients, The frequency of multiple congenital abnormalities was greater in individuals with intra-atrial communication Therefore, The clinical information was reviewed by a clinical geneticist, who classified the patients as having isolated CHD, CHD associated with EM or with syndromes.

The authors observed a higher frequency of specific combinations, such as hydronephrosis or urethral atresia with cardiac malrotation, right ventricular outlet obstruction, intra-atrial and interventricular communication. When the analysis was limited to the cases with EM who underwent necropsy, the skeleton Nevertheless, we must emphasize that none of the studies described herein used the same method to evaluate all the patients.

In the present review, we did not find any study that used the dysmorphological physical examination performed by a clinical geneticist, karyotype tests, fluorescent in situ hybridization FISH test for detection of the 22q11 deletion syndrome and abdominal ultrasound in all children with CHD.

Table 1 summarizes the main features of these studies. Conclusions In summary, EM, including abdominal abnormalities, are frequent in patients with CHD, and those who have both conditions may present higher risk of morbidity and mortality. Consequently some authors have been discussing the importance and cost-effectiveness of the screening of children with CHD for EM using ancillary tests such as abdominal ultra-sonogram.This will be replaced by tougher parameters- complication rates and success rates with the more complex congenital cardiac conditions.

The most frequent abnormalities of the upper renal system were the urethral anomalies, hydronephrosis and unilateral renal agenesis. Your password has been changed. Initially the blood coming from the lung buds drain into the splanchnic plexus which connects to the paired common cardinal and umbilicovitelline veins. Moderate PAH- , the central vessels dilate further, Severe PAH 50 mmHg - there is central dilation with reduction in the calibre of peripheral vessels peripheral pruning.

Pitx-2, a homeodomain containing protein plays a role in development of laterality. Related Information. Patients with chronic atrial fibrillation may benefit from the Maze procedure antiarrhythmic surgery.

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