Shunt Detection and Quantification

Presenter : Presenter : 蔣俊彥蔣俊彥Supervisor : Supervisor : 趙庭興 醫師趙庭興 醫師

Grossman’s cardiac catheterization, angiography, and intervention

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Outline

• Oximetry RunOximetry Run

• Flow Ratio Flow Ratio

• Early Recirculation of An indicator Early Recirculation of An indicator

• Angiography Angiography

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Shunt suspection

• Unexplained arterial desaturation Unexplained arterial desaturation

• Unexpectedly high Oxygen content in pulmonUnexpectedly high Oxygen content in pulmonary artery ary artery

• Consider an intracardiac shunt Consider an intracardiac shunt

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Oximetry Run• Obtain a 2-ml sample from each of the following locations:Obtain a 2-ml sample from each of the following locations: 1. Left and or right pulmonary artery 1. Left and or right pulmonary artery 2. Main pulmonary artery 2. Main pulmonary artery 3. Right Ventricle , outflow tract 3. Right Ventricle , outflow tract 4. Right ventricle , mid4. Right ventricle , mid 5. Right ventricle , tricuspid valve or apex 5. Right ventricle , tricuspid valve or apex 6. Right atrium , low or near tricuspid valve 6. Right atrium , low or near tricuspid valve 7. Right atrium , mild 7. Right atrium , mild 8. Right atrium , high 8. Right atrium , high 9. Superior vena cava , low (near junction with 9. Superior vena cava , low (near junction with right atrium)right atrium) 10. Superior vena cava , high (near junction with 10. Superior vena cava , high (near junction with innominate vein)innominate vein) 11. Inferior vena cava , high (just at or below diaphragm)11. Inferior vena cava , high (just at or below diaphragm) 12. Inferior vena cava , low L4-L5)12. Inferior vena cava , low L4-L5) 13. Left Ventricle 13. Left Ventricle

14. Aorta(distal to insertion of ductus )14. Aorta(distal to insertion of ductus )

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Oximetry Run

• A A significant step-upsignificant step-up is defined as an increas is defined as an increase in blood e in blood oxygen contentoxygen content or or saturationsaturation that e that exceeds the normal variability that might be obxceeds the normal variability that might be observed if multiple samples were drawn from tserved if multiple samples were drawn from that cardiac chamber. hat cardiac chamber.

• Dexter CriteriaDexter Criteria in oximtery run : in oximtery run :

a. Highest oxygen content in blood samples draa. Highest oxygen content in blood samples drawn from the wn from the right atriumright atrium exceeds the highest exceeds the highest content in the content in the venae cavaevenae cavae by by 2vol %2vol %

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b. a significant step-up at the b. a significant step-up at the ventricular levelventricular level is is present if the highest right ventricular sample present if the highest right ventricular sample is is 1 vol %1 vol % higher than the highest right atrial s higher than the highest right atrial sample.ample.

c. a significant step-up at the level of the c. a significant step-up at the level of the pulmopulmonary artery oxygennary artery oxygen content is more than content is more than 0.5% 0.5% vol%vol% greater than the highest right ventricular greater than the highest right ventricular sample. sample.

1 vol% = 1ml O2/100ml blood or 10mlO2/l1 vol% = 1ml O2/100ml blood or 10mlO2/l

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Example of Left-to-Right Shunt Detection:Atrial Septal Defect

• Vena Cava SPO2=Vena Cava SPO2=

(3x67.5+1x73)/4=69%(3x67.5+1x73)/4=69%

• Right Atrium SPO2=Right Atrium SPO2=

(74+84+79)/3=79%(74+84+79)/3=79%

• A significant step-up A significant step-up

79%-69%=10% >=7%79%-69%=10% >=7%

84%-68%=16%>=11%84%-68%=16%>=11% SPO2 from SVC to PASPO2 from SVC to PA

is 12%-13% >8% is 12%-13% >8%

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• PV O2 contentPV O2 content =0.96(14g Hgb /100ml blood) x (1.36 mlO2/g Hgb)=0.96(14g Hgb /100ml blood) x (1.36 mlO2/g Hgb) =18.3 mlO2/100ml blood =18.3 mlO2/100ml blood =183 mlO2/liter=183 mlO2/liter• PA O2 contentPA O2 content =0.80(14)1.36x10 =152ml O2/liter =0.80(14)1.36x10 =152ml O2/liter• QpQp = = O2 consumption (ml/min)O2 consumption (ml/min)/ PVO2 content – PAO2 / PVO2 content – PAO2 contentcontent = 240ml O2/min /(183-152) mlO2/L = 7.74 L/min= 240ml O2/min /(183-152) mlO2/L = 7.74 L/min QsQs = 240ml O2/min/ Systemic arterial O2 content – Mixed = 240ml O2/min/ Systemic arterial O2 content – Mixed venous O2 content venous O2 content = 240/(0.96-0.69)14(1.36)10=4.6 L/min= 240/(0.96-0.69)14(1.36)10=4.6 L/min• Qp/QsQp/Qs=7.74/4.6=1.68=7.74/4.6=1.68 Left-to-right ShuntLeft-to-right Shunt=7.7-4.7=3L/min =7.7-4.7=3L/min

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Ventricular Septal Defect

• Qp =Qp =

260/(0.97-0.885)15(1.36)10260/(0.97-0.885)15(1.36)10

= 15 L/min= 15 L/min

• Qs= Qs=

260/(0.97-0.66)15(1.36)10260/(0.97-0.66)15(1.36)10

= 4.1 L/min= 4.1 L/min

• Qp/Qs=15/4.1=3.7Qp/Qs=15/4.1=3.7

LLShunt=15-4.1=10.9 L/min Shunt=15-4.1=10.9 L/min

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Flow Ratio(Qp/Qs)

• The ratio Qp/Qs gives important physiologic information aboThe ratio Qp/Qs gives important physiologic information about the magnitude of a left-to-right shunt ut the magnitude of a left-to-right shunt

• A Qp/Qs<1.5 signifies a small left-to-right shunt and is often A Qp/Qs<1.5 signifies a small left-to-right shunt and is often felt to argue felt to argue against operative correctionagainst operative correction ,particularly if the p ,particularly if the patient has an uncomplicated atrial or ventricular septal defecatient has an uncomplicated atrial or ventricular septal defect t

• A A Qp/Qs between 1.5 and 2.0Qp/Qs between 1.5 and 2.0 are obviously intermediate in are obviously intermediate in magnitude ; magnitude ; surgical intervention is generally recommendedsurgical intervention is generally recommended if operation risk is low if operation risk is low

• A Qp/Qs <1.0 indicates a net right-to-left shunt and is often aA Qp/Qs <1.0 indicates a net right-to-left shunt and is often a sign of the presence of sign of the presence of irreversible pulmonary vascular diseirreversible pulmonary vascular dise

ase. ase. • A simplified formulaA simplified formula Qp (SAO2-MVO2)Qp (SAO2-MVO2) Qs (PVO2-PAO2)Qs (PVO2-PAO2)

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Calculation of Bidirectional Shunts

• Qeff = O2 Consumption (ml/min)Qeff = O2 Consumption (ml/min) PV O2 content -- MVO2 contentPV O2 content -- MVO2 content (ml/L) (ml/L)(ml/L) (ml/L)

• LL R = Qp(MVO2 content-PA O2 content ) R = Qp(MVO2 content-PA O2 content )

(MVO2 content-PV O2 content)(MVO2 content-PV O2 content)• R R L L = =Qp(PV O2 content-SAO2 content)(PAO2 content-PVO2 contentQp(PV O2 content-SAO2 content)(PAO2 content-PVO2 content

(SAO2 content –MV O2 content)x(MVO2 content-PVO2 content) (SAO2 content –MV O2 content)x(MVO2 content-PVO2 content)

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Limitations Of Oximetry Method

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Early Recirculation of An Indicator

• Standard indicator dilution cuStandard indicator dilution curves , performed by injection rves , performed by injection of indocyanine green into the of indocyanine green into the pulmonary artery with samplipulmonary artery with sampling in a systemic artery, are rang in a systemic artery, are rarely done today. rely done today.

• The technique can detect left-The technique can detect left-to-right shunts too small to dto-right shunts too small to detected by the oxygen step-uetected by the oxygen step-up method. p method.

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Angiography

• Selective angiography is effective in Selective angiography is effective in visualizing and lovisualizing and localizing the site of left-to-right shuntscalizing the site of left-to-right shunts and useful in and useful in lolocalizing the anatomic size of the shuntcalizing the anatomic size of the shunt . .

• Complicated lesionsComplicated lesions(e.g., endocardial cushion defects,(e.g., endocardial cushion defects, coronary artery/right heart fistula , ruptured aneurys coronary artery/right heart fistula , ruptured aneurysms at the sinus of valsalva) commonly require angiogrms at the sinus of valsalva) commonly require angiographic delineation before surgical intervention can be aphic delineation before surgical intervention can be undertaken and helps to assess the “routine” undertaken and helps to assess the “routine” more comore completelympletely. .

• However, Angiography , cannot replace the important However, Angiography , cannot replace the important physiologic measurements that allow quantitation of flphysiologic measurements that allow quantitation of flow and vascular resistance ow and vascular resistance

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Detection of Right-to-Left Intracardiac Shunts

• Injection of a dilute solution of saccharin Injection of a dilute solution of saccharin

• Angiography Angiography

• Oximetry Oximetry

• Echocardiographic methods –“bubble study ”Echocardiographic methods –“bubble study ”

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Chapter 34: Profiles in Congenital Heart Disease

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Outline

• Atrial septal DefectAtrial septal Defect• Ventricular Septal defect Ventricular Septal defect • Patent Ductus arteriosus Patent Ductus arteriosus • Valvar aortic stenosis Valvar aortic stenosis • Valvar Pulmonary stenosis Valvar Pulmonary stenosis • Coarctation of aorta Coarctation of aorta • Tetralogy of Fallot Tetralogy of Fallot • DTGADTGA• Single VentricleSingle Ventricle• Postop Fontan Postop Fontan

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Atrial Septal Defects

• Surgery can be done without catheterizationSurgery can be done without catheterization• Improving success with umbrella devices for transcatheter closurImproving success with umbrella devices for transcatheter closur

e of central located defects of small or moderate size of ASD e of central located defects of small or moderate size of ASD Anatomic TypesAnatomic Types three main types: three main types: ostium secundum (68%)ostium secundum (68%) , , stium primum stium primum (18%)(18%) , , sinus venosus defects (6%)sinus venosus defects (6%). . Secundum defectsSecundum defects are located in the are located in the fossa ovalis below the fossa ovalis below the limbs bandlimbs band and usually and usually single single and and central central and often and often amenable for device closureamenable for device closure. . Sinus venosus defectsSinus venosus defects occur in the occur in the posterior part of the interatrialposterior part of the interatrial septum , near the superior vena cavaseptum , near the superior vena cava , or rarely , the , or rarely , the inferior vena inferior vena cavacava and are and are not amenable to device closurenot amenable to device closure , largely because of , largely because of the proximity of nearby right pulmonary veins the proximity of nearby right pulmonary veins

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PhysiologyPhysiology right ventricular hypertrophyright ventricular hypertrophydecrease RV compliance ( pulmonary sdecrease RV compliance ( pulmonary s

tenosis , pulmonary hypertension ) tenosis , pulmonary hypertension ) a smaller left-to-right shunt or a la smaller left-to-right shunt or a larger right-to-left shunt arger right-to-left shunt reduce left ventricular compliance and prod reduce left ventricular compliance and produce a larger left-to-right shunt uce a larger left-to-right shunt

Suggest closing all ASDs at the time of diagnosis Suggest closing all ASDs at the time of diagnosis Catheterization TechniqueCatheterization Technique advance from femoral vein , and pass from right atrium to left atriumadvance from femoral vein , and pass from right atrium to left atrium An alternate approach is to withdraw the catheter from the SVC to thAn alternate approach is to withdraw the catheter from the SVC to th

e RA with the tip positioned posteromedially, so that it drops beneate RA with the tip positioned posteromedially, so that it drops beneath the limbic band into the h the limbic band into the fossa ovalisfossa ovalis (as in a (as in a Brockenbrough transsBrockenbrough transseptal punctureeptal puncture ) and then to the RA. ) and then to the RA.

Primum defectsPrimum defects are located more are located more inferiorlyinferiorly , and , and sinus venoussinus venous defect defects are located mores are located more superiorly superiorly . Diffiuclty in crossing a known ASD us . Diffiuclty in crossing a known ASD usually implies the presence of the latter type of defect. ually implies the presence of the latter type of defect.

Oximter Data Oximter Data

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Step-up in oxygen-contentStep-up in oxygen-content of blood in the of blood in the RA RA In children , In children , 10% 10% in in oxygen saturation between the high SVC and the RA indicates an oxygen saturation between the high SVC and the RA indicates an abnormal increase . abnormal increase .

ASDASD, , LV-RA VSD( a VSD and associated tricupid regurgitation) LV-RA VSD( a VSD and associated tricupid regurgitation) The absence of a measured shunt does not exclude an important The absence of a measured shunt does not exclude an important

ASD. ASD. Pressure DataPressure Data The absence of transatrial gradient does not make the diagnosis oThe absence of transatrial gradient does not make the diagnosis o

f an ASD ,pericardial tamponade , restrictive physiology f an ASD ,pericardial tamponade , restrictive physiology Angiography Angiography angiography for pressure equalization across the atria , catheter cangiography for pressure equalization across the atria , catheter c

ourse , and a step-up at the atrial level is important to make a diagourse , and a step-up at the atrial level is important to make a diagnosisnosis

Both angiography and balloon sizing of atrial defects are carried Both angiography and balloon sizing of atrial defects are carried out to assess whether transcatheter closure is feasible out to assess whether transcatheter closure is feasible

Suspect ostium primum ASD and perform LV angiogram in a so-caSuspect ostium primum ASD and perform LV angiogram in a so-called lled hepatoclavicular view (LAO cranial 40hepatoclavicular view (LAO cranial 40º )º )

Interventional Catheterization Interventional Catheterization About 50%-60% of patitents with About 50%-60% of patitents with secundum ASDssecundum ASDs and those lesio and those lesio

ns is ns is less than 24 mm diameterless than 24 mm diameter , balloon sizing of the defect with , balloon sizing of the defect with a soft , deformable balloon and angiography precede device placea soft , deformable balloon and angiography precede device placement .ment .

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Ventricular Septal Defects

• Loud murmur permit diagnosis of the patient with a VSD at an early aLoud murmur permit diagnosis of the patient with a VSD at an early age ; large defects produce symptoms early in life and demand early clge ; large defects produce symptoms early in life and demand early closure , and small defects tend to get smaller with advancing age. osure , and small defects tend to get smaller with advancing age.

• “ “ fish or cut bait”fish or cut bait” in patients with VSDs by 2 years of age , or certainly in patients with VSDs by 2 years of age , or certainly by age 5. by age 5.

• Most single, clinically significant VSDs are reparied surgically without Most single, clinically significant VSDs are reparied surgically without catheterization , particularly in infancycatheterization , particularly in infancy , there are patients with conge , there are patients with congenital , posoperative , posttraumatic or postinfarction VSD who require nital , posoperative , posttraumatic or postinfarction VSD who require diagnostic or interventional cardiac catheterization or both. diagnostic or interventional cardiac catheterization or both.

Anatomic TypesAnatomic Types Most congenital VSDs are defects in or about the membranous septuMost congenital VSDs are defects in or about the membranous septu

m ; m ; “ perimembranous “ type“ perimembranous “ type are located just are located just underneath the aortic vaunderneath the aortic valve. lve.

Atrioventricular canalAtrioventricular canal –type VSDs are less common, are development –type VSDs are less common, are developmentally related to ostium primum defects , and occur in the ally related to ostium primum defects , and occur in the posterior interposterior interventricuklar septum adjacent to the atrioventricular valvesventricuklar septum adjacent to the atrioventricular valves. EKG tend . EKG tend to be associated with a to be associated with a counterclock wisecounterclock wise superior QRS in the ECG fr superior QRS in the ECG frontal plane and are see more commonly in patients ontal plane and are see more commonly in patients

Down syndromeDown syndrome . .

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Subpulmonary VSDsSubpulmonary VSDs result from deficiency of the result from deficiency of the conal septumconal septum , occu , occur more r more commonly in Asian patientscommonly in Asian patients, and are frequently associated with , and are frequently associated with prolapse of the right coronary cusp and aortic regurgitationprolapse of the right coronary cusp and aortic regurgitation. In . In CaucasCaucasian patientsian patients, aortic regurgitation also occurs but it is more commonly , aortic regurgitation also occurs but it is more commonly associated with associated with membranous defectsmembranous defects and often with prolapse of nonc and often with prolapse of noncoronary cusp alone or in conjunction with right cusp. oronary cusp alone or in conjunction with right cusp.

Muscular VSDsMuscular VSDs can occur anywhere in the can occur anywhere in the interventricular septuminterventricular septum ; ; most are “mid muscular ” , located just most are “mid muscular ” , located just below the moderator band in tbelow the moderator band in the RVhe RV..

Swiss-cheese VSDsSwiss-cheese VSDs, have large openings on the LV side of septum bu, have large openings on the LV side of septum but then are divided into myriad of channels by muscle bundles on the Rt then are divided into myriad of channels by muscle bundles on the RV side. V side.

Physiology Physiology Shunt direction are determined by the afterload that each ventricule faShunt direction are determined by the afterload that each ventricule fa

ces. ces. Left-to-Right ShuntLeft-to-Right Shunt increase LV afterload (e.g., hypertension, coarctation) increase LV afterload (e.g., hypertension, coarctation) Decrease RV afterload ( Decrease RV afterload ( pulmonary resistance occur in early infant )pulmonary resistance occur in early infant ) Left-to-right ShuntLeft-to-right Shunt or a Right-to-Left Shunt or a Right-to-Left Shunt Decrease LV afterload (vasodilator therapy)Decrease LV afterload (vasodilator therapy) Increase RV afterload ( the development of pulmonary stenosis or Increase RV afterload ( the development of pulmonary stenosis or pulmonary vascular disease) pulmonary vascular disease)

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A strong natural tendency for VSDs to close with advancing age.A strong natural tendency for VSDs to close with advancing age. Medical and not surgical management is generally advised initally for aMedical and not surgical management is generally advised initally for a

ny ny restrictive VSD in any asymptomatic patientrestrictive VSD in any asymptomatic patient. . Catheterization TechniqueCatheterization Technique Catheter passage Catheter passage through a VSD was avoidedthrough a VSD was avoided for the most part, becaus for the most part, becaus

e it was unnecessary in making the diagnosis or estimating the size of e it was unnecessary in making the diagnosis or estimating the size of shunt. shunt.

Increase successfully efforts to close certain VSDs using a transcathetIncrease successfully efforts to close certain VSDs using a transcatheter umbrella approach. er umbrella approach.

For perimembranous defects near the tricuspid valve, the catheter is adFor perimembranous defects near the tricuspid valve, the catheter is advanced into the RV and turned posteriorly (clockwise) to cross VSD intvanced into the RV and turned posteriorly (clockwise) to cross VSD into the LV outflow tract. Midmuscular and apical VSDs, most easily croso the LV outflow tract. Midmuscular and apical VSDs, most easily crossed with balloon floating catheterization from LV. Anterior sed with balloon floating catheterization from LV. Anterior muscular VSmuscular VSDs are best crossed from RV to LVDs are best crossed from RV to LV with precurved stiff catheters and s with precurved stiff catheters and soft torque –control wires. oft torque –control wires.

A “reactive” pulmonary vascular bedA “reactive” pulmonary vascular bed Pressure DataPressure Data Small VSDs have a large pressure gradient acrosss the interventricular Small VSDs have a large pressure gradient acrosss the interventricular

septum. septum. A large VSD is not to be a large defectA large VSD is not to be a large defect. Small defects with asso. Small defects with associated pulmonary hypertension may mimic the pressure findings in a laciated pulmonary hypertension may mimic the pressure findings in a large defect. rge defect.

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AngiographyAngiography the membranous , conoventricular , middle and apical portions ofthe membranous , conoventricular , middle and apical portions of

interventricular septum are best interventricular septum are best seen in LAO veiw.seen in LAO veiw. anterior portion of the septum is best seen with AP veiw.anterior portion of the septum is best seen with AP veiw. Posterior portion of the septumPosterior portion of the septum (the location of atrioventricular ca (the location of atrioventricular ca

nal defects and posterior muscular defects) is best seen with a nal defects and posterior muscular defects) is best seen with a fofour-chamber view.ur-chamber view.

Injection of contrast material into the high-pressure chamber (LV) Injection of contrast material into the high-pressure chamber (LV) is required and inject nonionic contrast volumes(1to 1.5 ml/kg) at ris required and inject nonionic contrast volumes(1to 1.5 ml/kg) at rapid rates( <1 second) to outline the defectsapid rates( <1 second) to outline the defects

Interventional Cardiology Interventional Cardiology ApicalApical and and anterior muscular defectsanterior muscular defects and for most residual postop and for most residual postop

erative defects is treated by erative defects is treated by transcatheter closuretranscatheter closure Considering that the Considering that the aortic and tricuspid valves are close to the edaortic and tricuspid valves are close to the ed

ges of most perimembranous VSDsges of most perimembranous VSDs, , surgery remains the mainstay surgery remains the mainstay of management for these patientsof management for these patients. .

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Patent Ductus Arteriosus

• PDA is usually PDA is usually diagnosed in childrendiagnosed in children and and correct at the time of diagnoscorrect at the time of diagnosisis. PDA is maintained before birth by the production of PGE. . PDA is maintained before birth by the production of PGE. PrematurPremature babiese babies may have an may have an incidence of PDA as high as 40%.incidence of PDA as high as 40%. These are read These are readilyily closed closed by administration of the by administration of the cyclooxygenase inhibitor indomethacyclooxygenase inhibitor indomethacin. cin.

• The diagnosis of a PDA is made on the basis of The diagnosis of a PDA is made on the basis of physical examination aphysical examination and echocardiographynd echocardiography. PDA is closed surgically and . PDA is closed surgically and catheterization is rcatheterization is reserved for those patients with unusal findings or suspected pulmonareserved for those patients with unusal findings or suspected pulmonary hypertensiony hypertension. Alternatively, the PDA can be closed surgically by the . Alternatively, the PDA can be closed surgically by the video-assisted thoroscopy (VATS)video-assisted thoroscopy (VATS) technique. technique.

• PDAs are almost always located off the underside of the aortic arch PDAs are almost always located off the underside of the aortic arch jusjust distal to the origin of the left subclavicle arteryt distal to the origin of the left subclavicle artery, left of the trachea, and , left of the trachea, and proximal to the left main stem bronchus. proximal to the left main stem bronchus.

Physiology Physiology rarely large except in patients with rarely large except in patients with Down syndromeDown syndrome and and live at live at high altitudehigh altitude. . ‘‘Restrictive’ PDARestrictive’ PDA is characterized by a measurable step-up in PA blood is characterized by a measurable step-up in PA blood

oxygen saturation , perhaps some pulmonary hypertension , and no choxygen saturation , perhaps some pulmonary hypertension , and no change in aortic or RV blood oxygen saturation.ange in aortic or RV blood oxygen saturation.

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Catheterization TechniqueCatheterization Technique The techniques of a standard The techniques of a standard right-side heart catheterright-side heart catheter study estimate t study estimate t

he hemodynamic effects of a PDA. he hemodynamic effects of a PDA. Transcatheter closureTranscatheter closure is accomplished eitheter from a venous approac is accomplished eitheter from a venous approac

h for h for double-umbrelladouble-umbrella or or coil occlusioncoil occlusion or from or from a retrograde arterial roa retrograde arterial routine fro coil occlusionutine fro coil occlusion. .

Oximetry DataOximetry Data Calculation of the shunt in a patient with a PDA is technique difficult.Calculation of the shunt in a patient with a PDA is technique difficult. Left PA blood usually has a higher oxygen saturation than that fromLeft PA blood usually has a higher oxygen saturation than that from the right PA ; and a “mixed ” PA value cannot be defined accurately. the right PA ; and a “mixed ” PA value cannot be defined accurately. If it is necessary to determinate whether closing of the PDA will result If it is necessary to determinate whether closing of the PDA will result

in a fall in PA pressure, one must temporarily balloon-occlude the duct in a fall in PA pressure, one must temporarily balloon-occlude the duct and remeasure saturations and pressures. and remeasure saturations and pressures.

Pressure DataPressure Data Most PDAs do not alter right- or left-side heart pressure unless they arMost PDAs do not alter right- or left-side heart pressure unless they ar

e large.e large. In the face of any left-side heart abnormalities (e.g., poor LV function, In the face of any left-side heart abnormalities (e.g., poor LV function,

aortic stenosis ) , a PDA increases LV systolic pressure , LV diastolic aortic stenosis ) , a PDA increases LV systolic pressure , LV diastolic pressure or both . pressure or both .

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AngiographyAngiography

PDA is best done in a PDA is best done in a straight lateral viewstraight lateral view with contrast material in with contrast material injected distal to the PDA. jected distal to the PDA.

Interventional Catheterization Interventional Catheterization

Most workers use coils in small PDAs and a modification of the Most workers use coils in small PDAs and a modification of the dodouble-umbrellauble-umbrella technique of technique of Rashkind Rashkind or a or a Grifka coilGrifka coil and bag in la and bag in larger ones. rger ones.

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Aortic Stenosis

• Aortic stenosis, the great majority of which occurs at vavular level, remAortic stenosis, the great majority of which occurs at vavular level, remains a ains a common from of congenital heart disease in both children and acommon from of congenital heart disease in both children and adultsdults. .

• The advent of The advent of Doppler echocardiographyDoppler echocardiography has markedly improved the n has markedly improved the noninvasive assessment of oninvasive assessment of obstruction severityobstruction severity. .

• Catheterization indication Catheterization indication undertake for undertake for balloon vavotomyballoon vavotomy , which cardiac dysfunction appears in , which cardiac dysfunction appears in

the neonate or the peak-to-peak transvalvar gradient is the neonate or the peak-to-peak transvalvar gradient is higher than 50 higher than 50 mmhg , or associated with mild aortic regurgitation in older childrenmmhg , or associated with mild aortic regurgitation in older children, ,

Anatomic Types Anatomic Types More than 75% of children with valvar aortic stenosis have aMore than 75% of children with valvar aortic stenosis have a bicommissural valve with leaflet fusionbicommissural valve with leaflet fusion. . The absence of theThe absence of the intercoronary commissure is most commonintercoronary commissure is most common. . Progression of obstruction occurs in one third of those with valvarProgression of obstruction occurs in one third of those with valvar aortic stenosis, making careful follow-up mandatory.aortic stenosis, making careful follow-up mandatory. In a In a smlal proportionsmlal proportion, the obstruction is, the obstruction is subvalvar subvalvar owing to either a owing to either a thin fibrous ridgethin fibrous ridge or or fibromuscular dysplasia of the LV outflow tractfibromuscular dysplasia of the LV outflow tract..

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Surgery is generally indicated for significant stenosis or symptoms in ordSurgery is generally indicated for significant stenosis or symptoms in order to protect the aortic valve. It is probably er to protect the aortic valve. It is probably best delayed until after the first best delayed until after the first decade of lifedecade of life to reduce the risk of recurrence. to reduce the risk of recurrence.

An An hourglass deformity above the aortic valvehourglass deformity above the aortic valve , so-called , so-called supravalvar aortsupravalvar aortic stenosisic stenosis. Caused at least . Caused at least partly by thickening of the supracoronarypartly by thickening of the supracoronary ridg ridge , supravalvar aortic stenosis is often seen in association with e , supravalvar aortic stenosis is often seen in association with Williams sWilliams syndromeyndrome and with and with branch PA stenosisbranch PA stenosis. .

PhysiologyPhysiology The clinical findings in all three lesions are similar in these patients with uThe clinical findings in all three lesions are similar in these patients with u

ncompromized LV function. ncompromized LV function. Catheterization TechniqueCatheterization Technique Congenitally narrowed valves tend to have an opening in the Congenitally narrowed valves tend to have an opening in the posterior parposterior par

t of the valve, between the left and noncoronary cusps.t of the valve, between the left and noncoronary cusps. Because crossing congenitally stenotic aortic valves can be difficult, somBecause crossing congenitally stenotic aortic valves can be difficult, som

etimes use a side-arm arterial sheath . Beyond infancy, usually place a seetimes use a side-arm arterial sheath . Beyond infancy, usually place a second arterial catheter in ascending aorta via contralateral femoral artery. cond arterial catheter in ascending aorta via contralateral femoral artery.

Oximetry DataOximetry Data No oxygen saturation changes in the left or right heart blood No oxygen saturation changes in the left or right heart blood Pressure DataPressure Data Multiple pigtail catheters are used to enter the LV ; a pullback tracing with Multiple pigtail catheters are used to enter the LV ; a pullback tracing with

these catheters may not localize the presence of subvalvar or supravalvar these catheters may not localize the presence of subvalvar or supravalvar stenosis, making use of an end-hole catheter necessarystenosis, making use of an end-hole catheter necessary for the purposefor the purpose..

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AngiographyAngiography AortographyAortography and and left ventriculographyleft ventriculography should be obtained in patients wit should be obtained in patients wit

h aortic stenosis. h aortic stenosis. AortographyAortography usually in usually in anterioposterior anterioposterior and and lateral viewslateral views assesses the assesses the dd

egree of aortic regurgitationegree of aortic regurgitation as well as as well as anatomy anatomy and and mobility of the leaflemobility of the leafletsts and and coronary artery grosslycoronary artery grossly. .

VentriculographyVentriculography (LAO cranial view) is used to measure the annulus dia (LAO cranial view) is used to measure the annulus diameter to estimate ventricular function, to identify subvalvar pathology anmeter to estimate ventricular function, to identify subvalvar pathology and to further outline valvar and supravalvar anatomy. d to further outline valvar and supravalvar anatomy.

Multiple views may be required to best outline the Multiple views may be required to best outline the subvalvar regionsubvalvar region, incl, including a uding a right anterior oblique view with caudal angulationright anterior oblique view with caudal angulation. .

Interventional Catheterization Interventional Catheterization

Balloon valvotomyBalloon valvotomy has become the treatment of choice for valvar aortic has become the treatment of choice for valvar aortic stenosis at stenosis at Boston Children HospitalBoston Children Hospital. The results are roughly equivalent . The results are roughly equivalent to those of to those of surgical valvotomysurgical valvotomy. .

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Pulmonary Stenosis

• Obstruction to the RV outflow tractObstruction to the RV outflow tract usually are seen in association with o usually are seen in association with other congenital lesions, such as ther congenital lesions, such as TOFTOF, , transposition of the great arteriestransposition of the great arteries , , or or single ventriclesingle ventricle. . Isolated valvar pulmonary stenosisIsolated valvar pulmonary stenosis is common beyon is common beyond the neonatal period. d the neonatal period.

• Noninvasive diagnosis and severity assessment are now accurate in almNoninvasive diagnosis and severity assessment are now accurate in almost all circumstances and ost all circumstances and catheterization is reserved for those who requicatheterization is reserved for those who require balloon valvotomy , at any age. re balloon valvotomy , at any age.

Anatomic typesAnatomic types Valvar obstructionValvar obstruction accounts for more than accounts for more than 80% of isolated pulmonic sten80% of isolated pulmonic sten

osisosis. . In In typical valvar pulmonary stenosistypical valvar pulmonary stenosis the annulus is of normal sizethe annulus is of normal size, the , the lealea

flets are thinflets are thin , , the commissures are fusedthe commissures are fused and there is and there is marked poststenotmarked poststenotic dilation. ic dilation.

Dysplastic pulmonary valvesDysplastic pulmonary valves exhibit a different pathology; the exhibit a different pathology; the annulus is annulus is smallsmall , the , the leaflets are markedly thickenedleaflets are markedly thickened, they do not move during syst, they do not move during systole and they are fused. The ole and they are fused. The main PA is short and narrowmain PA is short and narrow. .

RV muscles bundles occur as anomalously thickened bundles of muscle RV muscles bundles occur as anomalously thickened bundles of muscle within the RV cavity and are usually associated with within the RV cavity and are usually associated with VSDVSD or or subvalvar aosubvalvar aortic stenosisrtic stenosis. .

The The rarest formrarest form of pulmonary stenosis is of pulmonary stenosis is branch PA stenosisbranch PA stenosis. It is often a. It is often associated with both ssociated with both supravalvar aortic stenosissupravalvar aortic stenosis and and Williams syndromeWilliams syndrome..

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Physiology Physiology Severe forms of stenosis in the neonate generally lead to markedly Severe forms of stenosis in the neonate generally lead to markedly reduredu

ced RV complianceced RV compliance with with increasing RA pressureincreasing RA pressure and then and then right-to-left shright-to-left shunting across a PFO. unting across a PFO.

Critical valvar pulmonary stenosisCritical valvar pulmonary stenosis commonly manifests with commonly manifests with cyanosiscyanosis a and nd sometimes with heart failuresometimes with heart failure. . Moderate degreeModerate degree of stenosis (e.g., gradi of stenosis (e.g., gradients of 40 to 80 mmhg) rarely cause symptomsents of 40 to 80 mmhg) rarely cause symptoms

Patients with moderate pulmonary stenosis have decreased exercise perPatients with moderate pulmonary stenosis have decreased exercise performance at cardiac catheterization. formance at cardiac catheterization. Balloon valvotomyBalloon valvotomy is both safe and is both safe and successful , successful , most cadiologist now recommend dilation in any patient witmost cadiologist now recommend dilation in any patient with more than mild stenosis before 5 years of ageh more than mild stenosis before 5 years of age. .

Catheterization Technique Catheterization Technique

The foramen ovale is usually open , allowing catheter access to the left The foramen ovale is usually open , allowing catheter access to the left side of the heart from a side of the heart from a femoral venous approachfemoral venous approach. Arterial catheters usu. Arterial catheters usually are required only in ill neonetes and those with severe or more ally are required only in ill neonetes and those with severe or more

obstruction. The opening in the pulmonary valve of an infant with severe obstruction. The opening in the pulmonary valve of an infant with severe pulmonary stenosis may be extremely small (less than 1 to 2 mm in diampulmonary stenosis may be extremely small (less than 1 to 2 mm in diameter)eter)

Do not routinely measure PA pressure directly in infants with critical valvDo not routinely measure PA pressure directly in infants with critical valvar pulmonary stenosis, rather perform 4 F catheters and a 0.018 inchar pulmonary stenosis, rather perform 4 F catheters and a 0.018 inch

torque wire to cross the valve , followed by rapid balloon dilation. torque wire to cross the valve , followed by rapid balloon dilation.

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Oximetry DataOximetry Data Most patient have neither a right-to-left nor a left-to-right shunt . In Most patient have neither a right-to-left nor a left-to-right shunt . In

critical severe valvar pulmonary stenosis, a right-to-left shunt occcritical severe valvar pulmonary stenosis, a right-to-left shunt occurs at the atrial level, produceing cyanosis.urs at the atrial level, produceing cyanosis.

In patients with both In patients with both mild pulmonary stenosis and an ASDmild pulmonary stenosis and an ASD , with a , with a large large left-to-right shunt at the atrial levelleft-to-right shunt at the atrial level, the gradient across the p, the gradient across the pulmonary valve is falsely elevated. ulmonary valve is falsely elevated. Closing the ASD reduces the RClosing the ASD reduces the RV pressure as well as the gradient across the pulmonic valveV pressure as well as the gradient across the pulmonic valve. .

Pressure DataPressure Data In mild pulmonary stenosis, the RV pressure is normal. As the deIn mild pulmonary stenosis, the RV pressure is normal. As the de

gree of stenosis increases, the RA a wave increases. With severe gree of stenosis increases, the RA a wave increases. With severe pulmonary stenosis , RV systolic pressure approaches or exceeds pulmonary stenosis , RV systolic pressure approaches or exceeds LV pressure, main PA pressure falls, and the PA pulse pressure dLV pressure, main PA pressure falls, and the PA pulse pressure dampens. Marked hypertrophy of the RV may cause the infundibulaampens. Marked hypertrophy of the RV may cause the infundibular os to close during late systole , resulting in further obstruction. r os to close during late systole , resulting in further obstruction.

AngiographyAngiography Right ventriculographyRight ventriculography in straight lateral view to outline in straight lateral view to outline pulmonarpulmonar

y valvey valve and and subvalvar regionsubvalvar region. . A straight AP view with crainal angulation may outline the pulmonA straight AP view with crainal angulation may outline the pulmon

ary valve, poststenotic dilation of the main PA often obscures the ary valve, poststenotic dilation of the main PA often obscures the PA branch origin. PA branch origin.

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Coarctation of the aorta

• CatheterizationCatheterization of the patient with isolated coarctation for diagnos of the patient with isolated coarctation for diagnostic purposes is tic purposes is rarely required before surgeryrarely required before surgery. Improved noninvasi. Improved noninvasive imaging , including ve imaging , including MRI allows precise anatomic definition of tMRI allows precise anatomic definition of the lesion. he lesion.

• The The continued incidence of recurrent coarctationcontinued incidence of recurrent coarctation after surgical rep after surgical repair , the common association of coarctation with air , the common association of coarctation with other forms of coother forms of congenital heart diseasengenital heart disease , and increasing use of balloon dilation in di , and increasing use of balloon dilation in discrete nonoperated coarctation screte nonoperated coarctation

Anatomic typesAnatomic types all forms of coarctation occur at or all forms of coarctation occur at or just distal to the left subclavian just distal to the left subclavian

arteryartery, , at or near the level of the old ductus arteriosusat or near the level of the old ductus arteriosus Coarctations have discrete “ Coarctations have discrete “ curtainscurtains ” of tissue indepting the pos ” of tissue indepting the pos

terior wall of the aorta associated with hypoplasia of the transversterior wall of the aorta associated with hypoplasia of the transverse aortic arch. e aortic arch.

PhysiologyPhysiology The gradient across a coarctation is influenced not only by the The gradient across a coarctation is influenced not only by the dede

gree of obstructiongree of obstruction but also the but also the degree of collateral flow around thdegree of collateral flow around the obstructione obstruction

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• Upper-extremity hypertension, the primary sequale of a coarctation , usuUpper-extremity hypertension, the primary sequale of a coarctation , usually resolves after surgical correction. In some children, hypertension pally resolves after surgical correction. In some children, hypertension persists despite adequate anatomic repair. The persist hypertension is mersists despite adequate anatomic repair. The persist hypertension is more common when repair occur s late in childhood. ore common when repair occur s late in childhood.

• The general recommendation that The general recommendation that coarctation should be diagnosed and coarctation should be diagnosed and corrected before the child reaches 3 years of age.corrected before the child reaches 3 years of age.

Oximetry DataOximetry Data no abnormalities in their intracardiac oxygen saturation no abnormalities in their intracardiac oxygen saturation Pressure DataPressure Data The gradient measured by pullback in the catheterization laboatory is freThe gradient measured by pullback in the catheterization laboatory is fre

quently smaller than the gradient measured by sphygmomanometer in clquently smaller than the gradient measured by sphygmomanometer in clinic. inic.

AngiographyAngiography a straight a straight lateral aortogramlateral aortogram usually provides excellent visualization of th usually provides excellent visualization of th

e coarctation. e coarctation. Interventional Catheterization Interventional Catheterization Although balloon dilation frequently reduces the gradient across an unoAlthough balloon dilation frequently reduces the gradient across an uno

perated coarctation site, the results particuly in babies are not generally perated coarctation site, the results particuly in babies are not generally as good as those seen surgical management. as good as those seen surgical management.

HoweverHowever,balloon dilation of recurrent coarctation,balloon dilation of recurrent coarctation has provided clinically has provided clinically invaluableinvaluable with or without stent placement. with or without stent placement.

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Tetralogy of Fallot

• The association of a The association of a malalignment VSDmalalignment VSD, , infundibular and valvar pinfundibular and valvar pulmonary stenosis with resultant aortic overridingulmonary stenosis with resultant aortic overriding , and , and cyanosis cyanosis is referred to as TOF complex. is referred to as TOF complex.

• It remains It remains a difficult and important surgical challengea difficult and important surgical challenge. . CatheterizatCatheterizationion of infants with the lesion, especially if they are cyanotic , may of infants with the lesion, especially if they are cyanotic , may bebe hazardous hazardous. Therefore, if echocardiography information is adeq. Therefore, if echocardiography information is adequate in this age group, uate in this age group, surgery is undertaken without catheterizatisurgery is undertaken without catheterization. on.

Anatomic TypesAnatomic Types (a) branch PA stenosis (5% to 10%) (a) branch PA stenosis (5% to 10%) (b) pulmonary atresia with PDA-dependent pulmpnary blood flow (b) pulmonary atresia with PDA-dependent pulmpnary blood flow (5% to 10%)(5% to 10%) (c) additional muscular VSDs (5% to 10%)(c) additional muscular VSDs (5% to 10%) (d) aortopulmonary collateral arteries supplying blood flow to the (d) aortopulmonary collateral arteries supplying blood flow to the lungs (5% to 10%)lungs (5% to 10%) (e) coronary arterial anomalies , especially the left anterior (e) coronary arterial anomalies , especially the left anterior descending arising from the right coronary artery (1% to 2%) descending arising from the right coronary artery (1% to 2%)

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Physiology Physiology TheThe combination combination of of pulmonary obstructionpulmonary obstruction and a and a VSD VSD produces a produces a right-to-left shuntright-to-left shunt at the at the ventricular defectventricular defect.The size of this shunt is .The size of this shunt is unrelated to the size of the VSD, which in TOF is almost unrelated to the size of the VSD, which in TOF is almost always largealways large and unrestrictiveand unrestrictive. The right-to-left shunt and degree of cyanosis are. The right-to-left shunt and degree of cyanosis are determined primarily by the degree of pulmonary obstruction and lessdetermined primarily by the degree of pulmonary obstruction and less by the level of systemic vascular resistance. by the level of systemic vascular resistance. ““tetrad spellstetrad spells” characterized by ” characterized by hyperventilationhyperventilation , , acidosisacidosis, , extremeextreme desaturationdesaturation , and , and unconsciousnessunconsciousness. Hypercyanotic episodes may be. Hypercyanotic episodes may be provoked by increased pulmonary obstruction or decreased systemicprovoked by increased pulmonary obstruction or decreased systemic resistance. They are best treated by resistance. They are best treated by sedationsedation, , intravenous volumeintravenous volume infusionsinfusions , and , and increasing the systemic vascular resistanceincreasing the systemic vascular resistance. . Previously , Previously , aortopulmonary shuntsaortopulmonary shunts such as the such as the Blalock-Tassig shuntBlalock-Tassig shunt were were created surgicallycreated surgically to increase pulmonary blood flow . Currently , so- to increase pulmonary blood flow . Currently , so- called complete cardiac correction , relieving the pulmonary stenosis, andcalled complete cardiac correction , relieving the pulmonary stenosis, and closing the VSD, is usually undertaken in infancy , or early childhood. closing the VSD, is usually undertaken in infancy , or early childhood. Catheterization Technique Catheterization Technique enter the left side of the heart from the RA without difficulty.enter the left side of the heart from the RA without difficulty. pass the catheter from the RV both to the aorta and into the PApass the catheter from the RV both to the aorta and into the PA Catheter passage Catheter passage into PAinto PA often provoke a often provoke a hypercyanotic spellhypercyanotic spell and cathete and cathete

r r from the RV to aortafrom the RV to aorta often produces often produces transient heart blocktransient heart block. .

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Oximetry DataOximetry Data No abnormalities in oxygen saturation at the atrial level or ventricular levNo abnormalities in oxygen saturation at the atrial level or ventricular lev

el. The right-to-left shunt is documented only in the aorta. el. The right-to-left shunt is documented only in the aorta. If the If the cyanosis is severecyanosis is severe , a , a right-to-left shuntright-to-left shunt may be present at may be present at the the atrial levelatrial level as well, across a PFO. The atrial shunts are signs of subs as well, across a PFO. The atrial shunts are signs of subs

tanially decreased RV compliance and suggest long-tanially decreased RV compliance and suggest long- standing RV hypertension. standing RV hypertension. Pressure DataPressure Data RV and LV pressures are equal in patients with TOF. RV and LV pressures are equal in patients with TOF. PA pressures are decreased in cyanotic patients without surgery. PA pressures are decreased in cyanotic patients without surgery. In the presence of surgically created Waterston or Potts In the presence of surgically created Waterston or Potts aortopulmonary shunts , the PA is often distorted and pressures are aortopulmonary shunts , the PA is often distorted and pressures are elevated but rarely after Blalock-Taussig shunts. elevated but rarely after Blalock-Taussig shunts. AngiographyAngiography Angiographic definitionAngiographic definition of anatomic detail is of anatomic detail is the key element in the the key element in the cardiac catheterizationcardiac catheterization of patients with TOF. of patients with TOF. A A biplane RV angiogram with cranial angulationbiplane RV angiogram with cranial angulation of the anteroposterior of the anteroposterior establishes the diagnosis , defines the anatomy of the pulmonary valve establishes the diagnosis , defines the anatomy of the pulmonary valve and subpulmonary region , and identifies the main PA and proximal PA and subpulmonary region , and identifies the main PA and proximal PA branches. branches.

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An An ascending aortogramascending aortogram may be necessary to further delineate may be necessary to further delineate cocoronary artery anatomyronary artery anatomy, and in particular to identify the origin of th, and in particular to identify the origin of the e left anterior anterior descending artery.left anterior anterior descending artery.

Aortography especially of the descending aortaAortography especially of the descending aorta is also often nece is also often necessary to see whether any ssary to see whether any aortopulmonary collateralsaortopulmonary collaterals are present. are present.

Interventional Catheterization Interventional Catheterization Interventional procedures are rarely required before definitive Interventional procedures are rarely required before definitive surgical correction in patients with uncomplicated TOF. surgical correction in patients with uncomplicated TOF. However, in infants with TOF, pulmonary atresia, and diminutive However, in infants with TOF, pulmonary atresia, and diminutive Pas, dilation of hypoplastic Pas and Pas, dilation of hypoplastic Pas and coil embolization of coil embolization of aortopulmonary arteriesaortopulmonary arteries make up an essential component of make up an essential component of management.management.

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Transposition of The Great Arteries

• In transposition of the great arteries (TGA), the great arteries arise In transposition of the great arteries (TGA), the great arteries arise from the from the wrong ventricleswrong ventricles (I.e., the aorta from the RV and the PA fr (I.e., the aorta from the RV and the PA from LV ) om LV )

• Two main types; the more common variety is Two main types; the more common variety is dextro-TGA(DTGA) adextro-TGA(DTGA) and levo-TGA(LTGA)nd levo-TGA(LTGA)

• DTGA,DTGA, the the ventricle position is normalventricle position is normal (I.e., the RV is right–sided a (I.e., the RV is right–sided and the LV is left-sided ,with thend the LV is left-sided ,with the RV RV giving rise to a right–sided giving rise to a right–sided antanterior aortaerior aorta and the and the LV LV to a to a left-sided posterior PAleft-sided posterior PA

• LTGA,LTGA, less common , the less common , the ventricles are invertedventricles are inverted (I.e., the LV is (I.e., the LV is

right-sided and RV is left-sided with theright-sided and RV is left-sided with the LV LV giving rise to a right- giving rise to a right-

sidedsided PA PA and the RV to a left-sided and the RV to a left-sided aorta.aorta. The type of The type of

transposition is almost always accompanied by a transposition is almost always accompanied by a VSDVSD and and

subpulmonary stenosissubpulmonary stenosis , is often by , is often by tricupid regurgitationtricupid regurgitation and and

atrioventricular conduction abnormalitiesatrioventricular conduction abnormalities, and is a very difficult, and is a very difficult

lesion to deal with lesion to deal with

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Physiology Physiology The pulmonary and systemic circulations in TGAThe pulmonary and systemic circulations in TGA run in parallelrun in parallel: : red blood coming back from the lungs returns to the lungs, and red blood coming back from the lungs returns to the lungs, and blue blood coming back the body return to the body. Without a blue blood coming back the body return to the body. Without a defect in circulation (e.g., ASD, PDA, VSD) to allow mixing defect in circulation (e.g., ASD, PDA, VSD) to allow mixing between the two circuits, the patient would die a few minutes after between the two circuits, the patient would die a few minutes after birth, birth, The early goal of therapy to The early goal of therapy to make a hole in the atrial septim (BAS)make a hole in the atrial septim (BAS), , or or between the great arteriesbetween the great arteries (using prostaglandins to open the (using prostaglandins to open the PDA) PDA) Catheterization Technique Catheterization Technique echocardiography definition is so precise and echocardiography definition is so precise and the only reasons to catheterthe only reasons to catheter

ize such neonates are to do a BAS. ize such neonates are to do a BAS. AngiographyAngiography RV angiography is standard anteroposterior and lateral views , RV angiography is standard anteroposterior and lateral views , establishes the diagnosis, assess RV function , determines the presence establishes the diagnosis, assess RV function , determines the presence of a VSD, or a PDA, and assesses tricupid regurgitation.of a VSD, or a PDA, and assesses tricupid regurgitation. LV angiogram demonstrates LV outflow tract lesionsLV angiogram demonstrates LV outflow tract lesions and and the location of a the location of a

VSD .VSD . ““laid-back” balloon occlusion aortogramlaid-back” balloon occlusion aortogram best outlines coronary arterial a best outlines coronary arterial a

natomy. natomy.

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• Intervention Catheterization Intervention Catheterization

an arterial switch operation is an arterial switch operation is performed electively in the first 10 performed electively in the first 10 days of life and perform a BAS in the early neonatal perioddays of life and perform a BAS in the early neonatal period. . Creation of an ASDCreation of an ASD remain the optimal method for remain the optimal method for stabilizationstabilization of of cyanotic infantscyanotic infants. .

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Single Ventricle

• The term “ single ventricle “ refers to a family of lesions and is The term “ single ventricle “ refers to a family of lesions and is onlonly one functional ventricular chambery one functional ventricular chamber. .

• Since introduction of the Since introduction of the Fontan procedureFontan procedure and other proceding p and other proceding palliative procedures such as the alliative procedures such as the Norwood stage 1Norwood stage 1 and and bidirectionabidirectional Glenn operationsl Glenn operations, this patient population with its evolving proble, this patient population with its evolving problem is rapidly increasing. m is rapidly increasing.

Anatomic TypesAnatomic Types The most common form of single ventricle is the The most common form of single ventricle is the highly lethal highly lethal hypoplastic left heart syndrome (HL-HS)hypoplastic left heart syndrome (HL-HS) , which is caused by , which is caused by aortic or mitral atresia . LV is diminutiveaortic or mitral atresia . LV is diminutive. HLHS . HLHS died in the past died in the past when PDA closewhen PDA close. The pioneering efforts of Norwood et.al, to creat . The pioneering efforts of Norwood et.al, to creat a new aorta surgically , a new aorta surgically , using the RV as a single ventricleusing the RV as a single ventricle , has , has improved the survival. improved the survival. A less commonA less common is is tricuspid atresiatricuspid atresia. Patients with. Patients with aplenia aplenia and and poly poly splenia syndormessplenia syndormes , also frequently have single ventricle . , also frequently have single ventricle . Most of the patient’ s catheterized Most of the patient’ s catheterized at least twice electivelyat least twice electively –betwee –betwee

n n the stage 1 and bidirectional Glenn proceduresthe stage 1 and bidirectional Glenn procedures and between and between the the latter and the Fontan operationlatter and the Fontan operation. .

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• Catheterization Techniques–Catheterization Techniques– Between Between the stage I and Bidirectional the stage I and Bidirectional Glenn Procedures. Glenn Procedures.

The patient have in common a pulmonary blood flow supplied by The patient have in common a pulmonary blood flow supplied by an an aortopulmonary shuntaortopulmonary shunt , usually , usually a Blalock-Taussig shunta Blalock-Taussig shunt , and , and the mandatory catheterization information consists of the mandatory catheterization information consists of measurement of PA pressuremeasurement of PA pressure, , resistanceresistance , , intracardiac and aortic intracardiac and aortic pressures, and OA and systemic venous anatomic detailspressures, and OA and systemic venous anatomic details. The . The catheter is advanced from the descending aorta into the mouth of catheter is advanced from the descending aorta into the mouth of the subclavian artery. Then the guidewire is passed from the tip of the subclavian artery. Then the guidewire is passed from the tip of the pigtail to the distal PA . the pigtail to the distal PA . An alternative method of An alternative method of PA pressure estimationPA pressure estimation is to measure the is to measure the

pulmonary venous wedge value , but this is a reliable approximatipulmonary venous wedge value , but this is a reliable approximation only when the on only when the PA mean is less than 20 mmhgPA mean is less than 20 mmhg. .

After the Directional Glenn Procedure and Just Before the ModifieAfter the Directional Glenn Procedure and Just Before the Modified Fontan Procedured Fontan Procedure

Because the SVC has been disconnected from the RA and anastoBecause the SVC has been disconnected from the RA and anastomosed to the PA, another venous line from the left subclavian vein mosed to the PA, another venous line from the left subclavian vein is required in the catheterization of these patients. is required in the catheterization of these patients.

, in addition to femoral venous and arterial lines. , in addition to femoral venous and arterial lines.

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The The development of pulmonary arteriovenous fistulasdevelopment of pulmonary arteriovenous fistulas in these in these

patients is increasingly recognized and is considered to be a patients is increasingly recognized and is considered to be a

consequence of the hepatic venous blood from the pulmonary consequence of the hepatic venous blood from the pulmonary

circulation by the bidirectional Glenn procedure. circulation by the bidirectional Glenn procedure. After the Fontan Procedure After the Fontan Procedure Many patients after a Fontan procedure have not had a Many patients after a Fontan procedure have not had a

fenestration placed in the baffle at the time of surgery and who fenestration placed in the baffle at the time of surgery and who

are doing well are not electively catherized. are doing well are not electively catherized. However, a significant number who had a fenestration placed who However, a significant number who had a fenestration placed who

later come to have this communication electively closed with alater come to have this communication electively closed with a

double-umbrella device. double-umbrella device. There are also others who have cyanosis for some other reason or There are also others who have cyanosis for some other reason or

evidence of congestive heart failure /venous hypertension who evidence of congestive heart failure /venous hypertension who

require catheterization for diagnostic and intervention purposes.require catheterization for diagnostic and intervention purposes. Excessive aortopulmonary collaterals may be present; they may Excessive aortopulmonary collaterals may be present; they may

lead to ventricular failure and require coil occlusion. lead to ventricular failure and require coil occlusion. Aortic arch obstructions similarly cause ventricular failure and Aortic arch obstructions similarly cause ventricular failure and

require dilation and stenting even gradients are small. require dilation and stenting even gradients are small.

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Thank Thank You !You !