Critical Congenital Heart Disease

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Information about Critical Congenital Heart Disease
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Published on February 5, 2009

Author: loweryp

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Critical Congenital Heart Disease : Critical Congenital Heart Disease Patricia V. Lowery, M.D. Neonatal-Perinatal Medicine Fellow BSOM/PCMH Objective for Today’s Discussion: Got a Blue Baby? : Objective for Today’s Discussion: Got a Blue Baby? Although the routine tools of cardiac evaluation (physical examination, ECG, and chest x-ray films) are not very helpful in diagnosing a specific cyanotic heart defect, these tools, your eyes, hands, and ears, are often useful in reducing the diagnostic possibilities. Slide 5: Ductal dependent CHF Cyanotic and acyanotic Moving Target Slide 8: Source: Silberbach and Hannon. 2007. PIR Fetal Circulation : Fetal Circulation Ductus Venosus Ductus Arteriosus Foramen Ovale Three Shunts of Fetal Circulation : Three Shunts of Fetal Circulation Ductus Arteriosus Protects lungs against circulatory overload Allows RV to strengthen High pulmonary vascular resistance, low pulmonary blood flow Carries moderately saturated blood Ductus Venosus Connects umbilical vein to IVC Flow regulated via sphincter (Eustachian valve) Conducts highly oxygenated blood Foramen Ovale Shunts highly oxygenated blood from RA to LA Fetal Circulation : Fetal Circulation Placenta oxygenates blood and returns to right atrium (RA) via IVC. Preferentially shunts across FO to LA. LV ejects most oxygenated blood to carotids and coronaries. Fetal Circulation : Fetal Circulation Superior vena cava (SVC) returns deoxygenated blood to RA where it mixes with oxygenated blood from the placenta. Preferentially enters RV. RV ejects into PA. Low pulmonary artery flow, so PA is shunted into the descending aorta via the ductus arteriosus. Slide 13: There are two mechanisms used for preferentially directing blood flow: Anatomic mechanism:The mechanism is characterized by a parallel movement of the flap of the foramen ovale and of the Eustachian valve.At the onset of ventricular systole, the flap valve of the foramen ovale moves towards the left atrial cavity whereas the Eustachian valve moves towards the atrial septum.The Eustachian valve appears to form a conduit within the right atrium directing the inferior vena cava blood into the left atrium. It also prevents the flow coming from the superior vena cava from crossing into the left atrium. The flow coming from the superior vena cava is now directed toward the right ventricle through the tricuspid valve. Hemodynamic mechanism:Beside this “anatomic” explanation, another hemodynamic element seems to favor the passage of the ductus venosus blood through the foramen ovale.The ductus venosus (with a high velocity flow) joins the left side of the inferior vena cava (with low velocity flow). This high velocity permits the ductus venosus stream to remain separated from the low velocity inferior vena cava stream until the foramen ovale. These 2 mechanisms (mechanically and hemodynamically) appear to work simultaneously. Slide 14: Bartolomeo Eustachi (born between 1500 and 1513, died 1574 ). Fate of the shunts… : Fate of the shunts… Infant Age as a Clue for You : Source: Silberbach and Hannon, PIR, 2007. Infant Age as a Clue for You Slide 18: Parallel Slide 19: Where are these found? Central Cyanosis vs. Acrocyanosis : Central Cyanosis vs. Acrocyanosis General Causes of Central Cyanosis : Source: Park: Pediatric Cardiology for Practitioners, 5th ed. General Causes of Central Cyanosis Slide 23: Congenital Heart Disease Cyanosis-Distribution : Cyanosis-Distribution Differential- preductal saturation higher than postductal- great vessels normally aligned and deoxygenated blood from PA crosses into descending Ao through DA CoAo, PDA, VSD AoA interruption, PDA, VSD Mitral stenosis, PDA Persistence of transitional circulation with ductal right-to-left shunting and minimal atrial right-to-left shunting Cyanosis-Distribution : Cyanosis-Distribution Reverse differential-preductal saturation lower than postductal occurs exclusively with physiology of transposition of the great arteries when oxygenated blood from PA enters the descending Ao through the DA Seen in TGA with Ao arch obstruction or pulmonary hypertension Physical Exam-Inspection & Palpation : Physical Exam-Inspection & Palpation Dysmorphic features- Holler out some examples! Chest wall configuration, hyperactive precordium Tachypnea: High Qp:Qs or diminished LV function, note degree of respiratory distress Cardiac impulse: Where is it? Is it ?? Is there a heave or a thrill? (What are these two entities?) Genetic Evaluation : Genetic Evaluation Genetic Trisomies 13, 18, 21 Monosomy X (Turner’s syndrome): Coarctation 22q11 Deletion (DiGeorge syndrome): Conotruncal abnormalities 7q11 Deletion (Williams syndrome) Single gene defects (Noonan’s, Holt-Oram, Ellis-van Crevald, Alagille) Unknown cause VACTERL CHARGE Physical Exam-Inspection & Palpation : Physical Exam-Inspection & Palpation Hepatomegaly? Or a Midline liver? Pulses: diminished or delayed in lower ext or right to left ext suggests CoAo, ?4 ext pulses in left-sided obstructive lesions; bounding pulses in PDA and severe aortic valve regurgitation Four extremity blood pressures?- ? > 10mmHg UE/LE; specific but often not sensitive (hypotension, PDA, feisty infant, equipment variability and inaccuracy) Physical Exam-Listen! : Physical Exam-Listen! Breath Sounds? Murmur: Timing (S or D), intensity (GI-VI), quality (harsh, musical, high or low pitched) Systolic murmur shortly or right after birth: consider valvular stenosis (pulmonic or aortic) or tricuspid or mitral regurgitation S2: Is it single? Is it loud or of increased intensity? Abnormal Second Heart Sound : Abnormal Second Heart Sound Single (no splitting) Only one semilunar valve Truncus arteriosus Pulmonary Atresia HLHS Abnormal semilunar valve positions TGA Abnormal physiology: pulmonary valve closes early or aortic valve closes late Pulmonary HTN Aortic stenosis Stabilization : Stabilization Delivery at term preferred Delivery at pediatric tertiary cardiac care center Guidelines for neonatal resuscitation Avoid hypoxemia, acidosis, bradycardia Intubation: RSI, sedation and NM blockade Oxygen with caution: sats 80-85% Vascular access (UAC, UVC) Prostaglandin-if no improvement or worsening consider HLHS/IAS, TGA/IAS or restrictive atrial communication, TAPVR with obstruction IAS-intact atrial septum Slide 34: SUGGESTED STEPS IN THE MANAGEMENT OF CYANOTIC NEWBORNS   1.    Chest x-ray films. Chest x-ray films may reveal pulmonary causes of cyanosis and the urgency of the problem. They can also hint at the presence or absence of cardiac defects and the type of defect.    2.    EKG if cardiac origin of cyanosis is suspected.    3.    Arterial blood gases in room air. Arterial blood gases in room air confirm or reject central cyanosis. An elevated Pco2 suggests pulmonary or CNS problems. A low pH may be seen in sepsis, circulatory shock, or severe hypoxemia.    4.    Hyperoxitest. Repeating arterial blood gases while the patient breathes 100% oxygen helps separate cardiac causes of cyanosis from pulmonary or central nervous system causes.  Pre and post ductal saturations.  5.    Umbilical artery line. A Po2 value in a preductal artery (such as the right radial artery) that is 10 to 15 mm Hg higher than that in a postductal artery (an umbilical artery line) suggests a right-to-left ductal shunt.    6.    Prostaglandin E1. If a cyanotic defect is suspected that depends on the patency of the ductus for survival, prostaglandin E1 (Prostin VR Pediatric) should be started or made available. (consult with cardiologist/neonatologist) What Are You Looking For On A Chest X-Ray? : What Are You Looking For On A Chest X-Ray? Heart: size, shape, situs Cardiothoracic index? <0.5-0.65, severe cardiomegaly consider Ebstein’s or cardiomyopathy Situs-where are heart, stomach? (levocardia, mesocardia, dextrocardia) Aortic arch-right or left? “The Shape of Things” on CXR : “The Shape of Things” on CXR Airway and Aortic Arch : Airway and Aortic Arch The normal airway is midline in the neck and at the thoracic inlet but is slightly to the right in patients with a left aortic arch. The intrathoracic airway does not reside in the midline. A truly "midline" intrathoracic trachea is likely actually deviated leftward, as is of course a frankly leftward deviated trachea: mediastinal pathology such as aortic or pulmonary artery anomalies should be considered. Right aortic arch in the face of situs solitus (right dominant liver, left sided stomach, normally "sided" right and left branch bronchi) with left sided cardiac apex is an important plain film tip-off to cyanotic lesions. The same would apply to situs inversus, dextrocardia and left aortic arch. Incidence of Right Arch in Cyanotic Congenital Heart Disease : Incidence of Right Arch in Cyanotic Congenital Heart Disease 35-40% of truncus arteriosus 30-35% of tetralogy of Fallot with pulmonary atresia 25% of tetralogy of Fallot 5-10% of D-TGA 5-10% of tricuspid atresia 5% of VSD Why don’t all D-TGA’s have right arches? Slide 40: “PMI in our eye” when we discover dextrocardia by CXR Slide 41: The Republican Heart? What Else Are You Looking For On A Chest X-Ray? : What Else Are You Looking For On A Chest X-Ray? Pulmonary vasculature pattern? (?, ?, n’l) Pulmonary pathology? Or hypoxemia with normal lung fields? Coexisting abnormalities/anomalies? Bone anomalies such as sternal deformities, rib and vertebral anomalies, scoliosis Pulmonary Vascularity : Pulmonary vascularity, when distinctive, is frequently a more valuable guide to differential diagnostic sets. Frankly oligemic lungs are a tipoff to a pulmonary vascular obstructive lesion. Admixture lesions are typified by cyanosis plus increased pulmonary blood flow. Signs of pathologically increased pulmonary blood flow include: Prominent intermediate pulmonary artery (larger diameter than the trachea) Second-, or third-order branch artery larger diameter than its companion bronchus Increased general vascular conspicuity, especially as seen through the hemidiaphragms Pulmonary Vascularity Pulmonary Vascularity : Pulmonary Vascularity With increased pulmonary blood flow of at least twice the systemic flow, (Qp=2Qs), the pulmonary vessels appear too large, and visible branches extend more peripherally than usual. When the vessels are "prominent," one must decide if the pattern is of increased pulmonary blood flow or pulmonary venous engorgement, a determination that can be difficult in the neonate. To further complicate things, the two conditions may coexist. With isolated pulmonary venous hypertension, the veins enlarge, but not the arteries Pulmonary Vascularity : Pulmonary Vascularity Once pulmonary overcirculation is determined, 3 major states to consider: High flow states: anemia, thyrotoxicosis, peripheral arteriovenous fistula Cyanotic congenital heart disease, with mixture of right and left sided blood: truncus arteriosus, transposition of the great vessels, and total anomalous pulmonary venous return or connection (TAPVR or TAPVC) Intracardiac or great vessel left to right shunt lesions Slide 46: Frontal chest radiograph of a normal infant shows normal range vascularity Slide 47: Frontal chest radiograph of an infant with pulmonary atresia demonstrates decreased vascularity Slide 48: Frontal chest radiograph of an infant with truncus arteriosus shows shunt vascularity (there are too many vessels, and these are enlarged but well-defined). Slide 49: Frontal chest radiograph of an infant with obstructed total anomalous pulmonary venous connection shows venous congestion (the vessels are less well-defined and there is pulmonary interstitial edema). Pulmonary Vascularity-Pearls : Pulmonary Vascularity-Pearls Normal newborns have relatively diminutive appearing vascularity, but are normal. Why? Truly undercirculated lung fields may look a little "too clear", "too black". Patients with left to-right shunts, including admixture lesions, may not have a significantly large enough shunt to recognize "shunt vascularity"; it takes at least a 2:1 shunt to recognize such on a plain radiograph. If one can make a clear judgment about vascularity, do so, but don't force the findings if they are not really there. Pulmonary Vascularity-Pearls : Pulmonary Vascularity-Pearls Venous congestion is a less frequent in children as compared to adults, as "pump" failure is less prevalent, but the pattern certainly occurs in structural left-sided obstructions. Subtle pulmonary vascular redistribution is uncommon. However, anatomic left heart obstructions including inflow obstructions, can cause congestive changes. In newborns, mild venous congestion looks like the "wet lung" pattern seen in transient tachypnea of the newborn; more severe venous congestion may appear similar to that seen in congenital lymphangiectasia. Severe congestive failure in newborn infants may appear as a diffusely increased pulmonary opacification, a "white-out" lung Not Mutually Exclusive : Not Mutually Exclusive One must remember also that primary cardiovascular disease and its treatment can be greatly complicated by concomitant pulmonary disease Hyperoxitest : Hyperoxitest Confirm central cyanosis by ABG PaO2 10 minutes 100% O2 (oxyhood) With pulmonary disease, arterial PaO2 usually rises to more than 150 mm Hg With significant intracardiac right-to-left shunt, the arterial PaO2 does not exceed 150 mm Hg, and the rise is usually not more than 10 to 30 mm Hg Hyperoxitest Limitations : Hyperoxitest Limitations In cyanotic defects with large pulmonary blood flow or no obstruction to PBF, such as TAPVR, may have a rise in arterial PO2 to 100 mm Hg or higher. Large left-to-right shunts Critical Ao stenosis, CoAo, IAA O2 is a pulmonary vasodilator, increases PBF Conversely, massive intrapulmonary shunt from lung disease (but with a normal heart) may not have a rise in arterial PO2 to 100 mm Hg. Hyperoxitest-A Final Word : Hyperoxitest-A Final Word The response of PaO2 to 100% oxygen inhalation should be interpreted in light of clinical picture, especially the degree of pulmonary pathology seen on chest x-ray films. EKG : EKG Check for sinus rhythm Superior axis: consider atrioventricular canal or tricuspid atresia (single ventricle) Left axis deviation: ECD, tricuspid atresia, Noonan syndrome, LVH RBBB(QRS terminal slurring)- Ebstein anomaly Normal EKG does not rule out CHD Congenital Heart Disease-Classification : Congenital Heart Disease-Classification Cyanotic: Poor/inadequate mixing (D-TGA), restricted pulmonary blood flow (TOF, TA, PA with IVS, critical PS, Ebstein anomaly), complete mixing (TAPVR, TA), variable physiology (single ventricle, DORV) Acyanotic: valvar PS, VSD, ASD, ECD, PDA, aortopulmonary window, L-TGA Systemic hypoperfusion with eventual CHF (AS, CoAo, AoAI, HLHS) Shone’s syndrome: four cardiac defects: a supravalvular mitral membrane (SVMM), parachute mitral valve, VSD (membranous or muscular) and coarctation of the aorta Cyanotic Heart Lesions : Cyanotic Heart Lesions The 5(?6) Ts Tetralogy of Fallot Transposition of the Great Arteries Truncus Arteriosus Total Anomalous Pulmonary Venous Return Tricuspid Atresia ? Tingle (single) Ventricle Physiology Critical Congenital Heart Disease : Critical Congenital Heart Disease Right heart obstructive lesions Left heart obstructive lesions Inadequate mixing lesions Inadequate gas exchange Can categorize infant without benefit of ECHO Focus on shock in DR (HLHS/IAS,TGA/restrictive or IAS), symptoms on first day (severe Ebstein’s, TOF/absent PV, obstructed TAPVR, D-TGA/restrictive or IAS), symptoms in first week Right Heart Obstructive Lesions : Right Heart Obstructive Lesions Blue but comfortable (cyanosis, tachypnea, no respiratory distress) Normal pulses and perfusion Single second heart sound (all four groups, doesn’t distinguish btwn groups but does diff btwn those with sepsis or lung dz IF you can appreciate it; is it sharp-normal or muffled-abnormal) Murmur-may or may not have ABG: moderate to marked hypoxemia p02 30s-40s, (pO2 in utero=25), no acidosis, pCO2 n’l CXR: decreased PBF so decreased pulmonary vascular markings (reason for cyanosis), normal or large heart Tetralogy of Fallot-Most Common Example of Right Heart Obstruction : Tetralogy of Fallot-Most Common Example of Right Heart Obstruction 4 lesions Overriding aorta/right ventricular outflow tract obstruction-develops over gestation, the “tighter” this is, the more ductal dependent for pulmonary blood flow Right ventricular hypertrophy Malalignment VSD-anteriorly Pulmonic stenosis Cyanosis varies with the degree of outflow tract obstruction and size of VSD Characterized by hypercyanotic episodes- Tet Spells Cyanosis is caused by Right Left shunting through the VSD TOF – Other Anomalies : TOF – Other Anomalies ~20-25% have right aortic arch, the majority with mirror image branching ~5-10% have a major anomaly of the atrioventricular valves: AV canal defect Absence of the left pulmonary artery or ductal origin of the left pulmonary artery Pentalogy of Fallot: has an associated ASD DiGeorge association (thymic aplasia/hypoplasia, hypocalcemia, spine anomalies, and facial anomalies) Velocardiofacial syndrome (cleft palate, heart disease, facial anomalies) 15; deletions in 22q11 FISH for all infants with conotruncal defects TOF Plain Films Findings : TOF Plain Films Findings Overall cardiac size is typically normal Apex is uplifted by the right ventricular hypertrophy Mediastinal vascular waist can be narrowed as the pulmonary outflow tract and main pulmonary artery are small Pulmonary vascularity is diminished or within normal range, depending on the severity of pulmonary obstruction No major anomalies of visceral situs or cardiac position Right aortic arch: 25% Slide 66: Frontal radiograph in a patient with tetralogy of Fallot shows the "boot-shaped heart" an upturned apex, and the narrowed mediastinal waist. The pulmonary vascularity is normal-to-reduced. The patient was clinically cyanotic Slide 67: Frontal radiograph in a patient with tetralogy of Fallot where the uplifted apex is more subtle, but a right sided arch and right descending aorta are present Slide 68: Frontal and lateral radiographs in a patient with tetralogy of Fallot shows "coeur en sabot" cardiac shape, narrowed mediastinum, diminished vascularity, and right aortic arch on the frontal view. Lateral view shows the limited vascularity especially the very small central right pulmonary arteries. TOF Variant : TOF Variant Tetralogy of Fallot with absent pulmonary valve shows the enlarged pulmonary artery segments and the compression of the central airways. Hyperinflation may result, or distorted aeration of the lungs can result in regional hyperinflation and regional collapse Single S2 Distal pulmonary artery stenosis Decreased PBF Slide 71: Mod B-T shunts are not done very often anymore unless complex anatomy Slide 72: Source:http://www.adhb.co.nz/newborn/TeachingResources/Radiology/Cardiology.htm Pulmonary atresia with restrictive foramen ovale and resultant RAE Slide 73: “Carpet Heart” Slide 74: Displacement of the tricuspid valve results in insufficiency of the valve, which causes the right atrium, or collecting chamber, to be larger than normal. In addition, the "leaflets" or flaps of the tricuspid valve are usually abnormal in form. This enlargement of the right atrium can predispose children to dysrythmias. Atrialization of the right ventricle. Frequently associated ASD Slide 75: Frontal radiograph in Ebstein's anomaly shows a characteristic severe cardiomegaly, marked right atrial contour enlargement and diminished pulmonary vascularity Airway compression EKG: peaked T waves in lead II=RAE, widened QRS=RBBB, WPW in 20-30%, other atrial arrhythmia Critical Right Heart Obstructive Lesions-Management : Critical Right Heart Obstructive Lesions-Management PGE1-”fixes” the problem Supplemental O2-won’t hurt, may not help Surgical intervention Left Heart Obstructive Lesions : Left Heart Obstructive Lesions Grey or ashen (not really blue) Tachypnea-increased blood flow to lungs, may be first sign Decreased 4 ext pulses (HLHS, Crit Ao Stenosis), differential pulses (CoAo) Poor perfusion Left Heart Obstructive Lesions : Left Heart Obstructive Lesions Usually single second heart sound Murmur often not always Gallop sometimes Right heart heave-RV working hard!! Hepatomegaly-may take time to develop CXR: large heart, increased PBF, venous congestion ABG: key thing is metabolic acidosis (if worsens with O2 must think left heart obstructive lesions), paO2s 40-50s Left Heart Obstructive Lesions : Left Heart Obstructive Lesions HLHS Coarctation of Aorta: discrete vs. long segment hypoplasia AS Slide 86: Decreased pulses, cool extremities Slide 88: PGE1 allows ductal patency and “swirl” past the coarctation Slide 90: Patch For Recurrent <1yo, no arm growth discrepancy Recurr 10% same as others Most often used Slide 91: Infant with Coarctation. Frontal chest radiograph shows an enlarged heart with perihilar vascular congestion. Slide 92: Coarctation and VSD. Frontal chest radiograph shows cardiomegaly and increased pulmonary blood flow with superimposed vascular congestion Slide 95: Anatomy HLHS PDA Slide 96: External View-HLHS http://pediatrics.aappublications.org/cgi/content/full/119/1/109#T1 HLHS - Pathophysiology : HLHS - Pathophysiology The right ventricle maintains both pulmonary and systemic circulations. Pulmonary venous blood passes through an atrial defect or dilated foramen ovale from the left to the right side of the heart, where it mixes with systemic venous blood (TOTAL MIXING LESION). The ventricular septum is usually intact. HLHS - Pathophysiology : HLHS - Pathophysiology The major hemodynamic abnormalities are: Inadequate maintenance of the systemic circulation. Pulmonary venous hypertension (if restrictive foramen ovale). Pulmonary overcirculation (if moderate or large ASD). HLHS – Clinical manifestations : HLHS – Clinical manifestations Signs of CHF usually appear within the first few days or weeks of life (dyspnea, hepatomegaly, low cardiac output). Peripheral pulses may be weak or absent. Cardiac enlargement is usual, with a right ventricular parasternal lift. A nondescript systolic murmur is usually present. HLHS – Diagnostic tools : HLHS – Diagnostic tools CXR: Heart variable in size (1st day of life). Cardiomegaly develops rapidly, associated with increased pulmonary vascularity. “The Shape of Things” on CXR : “The Shape of Things” on CXR HLHS – Diagnostic tools : HLHS – Diagnostic tools Electrocardiogram: Initially, may show only the normal right ventricular predominance. Later, P waves become prominent and right ventricular hypertrophy is usual. HLHS – Diagnostic tools : HLHS – Diagnostic tools Echocardiogram: There is absence or hypoplasia of the mitral valve and aortic root, a variable small left atrium and left ventricle, and a large right atrium and right ventricle. The size of the atrial communication can be assessed directly and by pulsed and color flow Doppler studies. HLHS – Diagnostic tools : HLHS – Diagnostic tools Echocardiogram (cont.): Suprasternal notch views identify the small ascending aorta and transverse aortic arch (may also demonstrate a discrete CoAo). Doppler demonstrates the absence of anterograde flow in the ascending aorta and retrograde flow via the ductus arteriosum. HLHS - Treatment : HLHS - Treatment Norwood procedure: Preoperative management -- avoid excessive 02 and hyperventilation correction of acidosis and hypoglycemia maintenance of the patency of the PDA (with PGE1) to support systemic blood flow prevention of hypothermia 21% FiO2, with addition of 2% to 4% CO2 (PCO2 aimed at 50, pH 7.30), also inspired nitrogen to raise PVR and limit PBF Selective use of small amount of inotropic agents (sepsis or RV failure) HLHS - Treatment : HLHS - Treatment Norwood Procedure (Classic): First Stage Atrial septectomy. Transection and ligation of the main pulmonary artery. Connection of the proximal pulmonary artery to the transversely opened hypoplastic aortic arch (“neoaorta”). The coarcted segment of the aorta is repaired. HLHS - Treatment : HLHS - Treatment Norwood Procedure: First Stage (cont.) Modified Blalock-Taussig shunt. 90% early survival (if in a timely fashion at selected institutions). **Sano Modification First-Stage Reconstructive Surgery – RV-PA shunt **PAB/DS-pulmonary artery banding/ductal stenting Slide 111: AFTER NORWOOD STAGE I Hypoplastic Left Heart Syndrome: S/P Stage One Norwood : Hypoplastic Left Heart Syndrome: S/P Stage One Norwood Surgical issues: Unobstructed aortic arch Adequate atrial septectomy Balanced pulmonary and systemic blood flow (Qp:Qs 1:1) Survival at major centers: 90% Hypoplastic Left Heart Syndrome: HemiFontan Procedure : Hypoplastic Left Heart Syndrome: HemiFontan Procedure Shunt ligated, superior vena cava anastomosis to pulmonary artery, pulmonary arteries augmented, flap of tissue closes SVC-RA junction Performed around 6 months of age following Norwood Volume load on right ventricle removed Excellent survival statistics Slide 114: AFTER NORWOOD STAGE II Hypoplastic Left Heart Syndrome: Fontan Procedure : Hypoplastic Left Heart Syndrome: Fontan Procedure Performed around 18-24 months Venous and systemic circulations are separated; cavopul-monary isolation Survival: excellent Long term issues: RV function, arrhythmias Slide 116: After Stage III of Norwood Procedure Hypoplastic Left Heart Syndrome: Fenestrated Fontan Procedure : Hypoplastic Left Heart Syndrome: Fenestrated Fontan Procedure Transposition of Great Vessels : Transposition of Great Vessels Ventriculoarterial discordance in D-TGA What is the difference between D-TGA and L-TGA? L-TGA : L-TGA Ventriculoarterial and ventriculoatrial discordance Slide 123: The heart is enlarged with a narrow "pedicle" giving the so called "egg on a string" appearance. The superior mediastinum appears narrow due to the antero-posterior relationship of the transposed great vessels and "radiologic-absence of the thymus". Slide 124: The stress of hypoxia in the newborn period is believed responsible for thymic regression. Right posterior oblique view demonstrates widening of structures in the superior mediastinum due to the anteroposterior relationship of the aorta and pulmonary artery. Slide 125: Posteroanterior view of the chest roentgenogram from a 2-month-old infant with complete transposition of the great arteries. Note cardiomegaly (cardiothoracic ratio of 0.7), “egg-shaped” heart with narrow waist, and increased pulmonary vascular markings, which are characteristic of this condition. Slide 129: intramural or single coronary artery, have a significantly increased mortality risk Intramural or single coronary artery, have a significantly increased mortality risk. Slide 131: Respiratory distress!! TAPVR : Embryology Failure of the pulmonary venous plexus to unite with the atrial portion of the heart results in TAPVC Type I-supracardiac type (most common form, representing 50% of cases) Type II-intracardiac type (next most common type, 25 % of cases) Type III-infracardiac type (20-25% of cases)-most common to present as critical in neonatal period-obstructive Type IV-mixed type (less than 5% of cases) TAPVR Slide 133: In the most common form of this defect (shown in the diagram), the pulmonary veins (PV) are connected by a vessel to the superior vena cava (SVC), which carries blue (oxygen-depleted) blood back to the heart. This is known as the supracardiac type. There is usually no obstruction with this type of anomalous veins. Less commonly, the pulmonary veins may be connected to the inferior (IVC) vena cava (infracardiac) or to the right atrium itself (intracardiac). It is also possible for the veins to be of mixed type. For example, the left sided veins may return via a supracardiac route and the right sided veins via an infracardiac route. Slide 135: Most commonly obstructed Slide 136: Total anomalous pulmonary venous connection (infradiaphragmatic-obstructed). PA chest radiograph demonstrates increased pulmonary venous pattern with a normal sized heart. There is a right sided pleural effusion. The endotracheal tube is just above the level of the carina. Critical CHD: Initial Evaluation and Management : Critical CHD: Initial Evaluation and Management ABC’s Oxygen (judicial) to saturations of 80-85% Place umbilical lines PGE (0.025-0.1 micrograms/Kg/min) History Complete physical with four extremity BP’s Pre and post-ductal oxygen saturations Hyperoxia test CXR EKG Echocardiogram 4 Your Management Information : 4 Your Management Information Thermoneutrality-avoid acidosis and inc O2 consumption pH- 7.4, BD- <4.0 pO2 >/= 40 (O2 is a pulmonary vasodilator) pCO2- 40 (CO2 is a pulmonary vasoconstrictor) iCa- >4.0 MAP >/40 O2 potent pulmonary vasodilator (dec PVR) Avoid hyperventilation and alkalosis (dec PVR) CO=SV X HR SV determinants: preload, afterload, myocardial contractility Side Effects of PGE1 : Side Effects of PGE1 More common in premature infants Clinical deterioration if pulmonary venous obstruction present HLHS with restrictive/intact atrial septum TGA with intact ventricular septum and a restrictive/intact atrial septum TAPVR with obstruction Apnea Hypotension References : References Fanaroff and Martin’s Neonatal-Perinatal Medicine, 2006,Vol. 2, pp. 1195-1283. https://www.cchs.net/pediatricradiology/ APLS: Congenital Heart Disease Park. Pediatric Cardiology for the Practitioner via MD Consult Silberbach,M and Hannon,D. PIR. 2007; 28:123-131 http://www.aapalaska.org/Dr%20Brekke%20Newborn%20and%20infant%20murmurs.pdf http://www.nicuniversity.org/cmeEngine_content.asp?=RLS0008 http://www.adhb.co.nz/newborn/TeachingResources/Radiology/Cardiology.htm http://www.pted.org/?id=cyanotic1 Vetter, VL. Pediatric Cardiology: The Requisites in Pediatrics. 2006

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