Pulmonary atresia with Ventricular Pulmonary atresia with Ventricular septal defectseptal defect

““Pulmonary atresia-ventricular septal defect is Pulmonary atresia-ventricular septal defect is defined as a group of congenital cardiac defined as a group of congenital cardiac malformations in whom there is lack of malformations in whom there is lack of luminal continuity and absence of blood flow luminal continuity and absence of blood flow from either ventricle and the pulmonary from either ventricle and the pulmonary artery, in a biventricular heart that has an artery, in a biventricular heart that has an opening or a hole in the interventricular opening or a hole in the interventricular septum. ”septum. ”

Congenital Heart Surgery Nomenclature and Database Congenital Heart Surgery Nomenclature and Database Project: Pulmonary Atresia—Ventricular Septal DefectProject: Pulmonary Atresia—Ventricular Septal Defect

Christo I. Tchervenkov, MD, and Nathalie Roy, MDChristo I. Tchervenkov, MD, and Nathalie Roy, MD

Ann Thorac Surg 2000;69:97-105Ann Thorac Surg 2000;69:97-105

““Tetralogy of Fallot with Pulmonary atresia Tetralogy of Fallot with Pulmonary atresia is a congenital cardiac malformation, is a congenital cardiac malformation, characterized by the extreme characterized by the extreme underdevelopment of the right ventricular underdevelopment of the right ventricular infundibulum with marked anterior and infundibulum with marked anterior and leftward displacement of the infundibular leftward displacement of the infundibular septum often fused with the anterior wall of septum often fused with the anterior wall of the right ventricle resulting in complete the right ventricle resulting in complete obstruction of blood flow into the pulmonary obstruction of blood flow into the pulmonary artery and associated with a large outlet, artery and associated with a large outlet, subaortic ventricular septal defect”subaortic ventricular septal defect”

TOF, PA is a specific type of PA-VSD where TOF, PA is a specific type of PA-VSD where the intracardiac malformation is more the intracardiac malformation is more

accurately definedaccurately defined..

Other synonyms -Other synonyms -Type IV truncus and Type IV truncus and Pseudotruncus.Pseudotruncus.

PA-VSD has been proposed by the PA-VSD has been proposed by the International nomenclature committee of International nomenclature committee of Congenital Heart Surgery Nomenclature Congenital Heart Surgery Nomenclature and Database Project and Database Project as a unifying termas a unifying term..

Essence of tetralogy of Fallot with pulmonary Essence of tetralogy of Fallot with pulmonary atresia is atresia is cephalad malalignment of the cephalad malalignment of the infundibular septuminfundibular septum

Causes anatomic obstruction of the right Causes anatomic obstruction of the right ventricular outflow tract and a malalignment-ventricular outflow tract and a malalignment-type of ventricular septal defect.type of ventricular septal defect.

The aorta overrides the ventricular septal defect The aorta overrides the ventricular septal defect and is rotated in a counter-clockwise directionand is rotated in a counter-clockwise direction

Embryologic origin of the main, rightEmbryologic origin of the main, rightand left pulmonary arteries, andand left pulmonary arteries, and

the intrapulmonary arteriesthe intrapulmonary arteries..

Embryologic origin of the main, Embryologic origin of the main, rightright

and left pulmonary arteries, andand left pulmonary arteries, andthe intrapulmonary arteriesthe intrapulmonary arteries..

The main pulmonary artery (MPA) -septation of The main pulmonary artery (MPA) -septation of the truncus and aortic sac. the truncus and aortic sac.

Intrapericardial right (RPA) and left (LPA) Intrapericardial right (RPA) and left (LPA) pulmonary arteries -sixth aortic arches with pulmonary arteries -sixth aortic arches with contribution from the aortic sac.contribution from the aortic sac.

The intraparenchymal pulmonary arteries - The intraparenchymal pulmonary arteries - from the vascular plexuses of the lung buds.from the vascular plexuses of the lung buds.

Vascular plexuses are supplied by the Vascular plexuses are supplied by the intersegmental arteries (ISAs) in the early intersegmental arteries (ISAs) in the early embryonic period. embryonic period.

Survival of patients with tetralogy of Fallot and pulmonary Survival of patients with tetralogy of Fallot and pulmonary atresia depends on the adequacy of pulmonary blood flowatresia depends on the adequacy of pulmonary blood flow

Patients with a duct-mediated pulmonary circulation -early Patients with a duct-mediated pulmonary circulation -early mortality due ductal constriction and closure. mortality due ductal constriction and closure.

Half this group die by 6 months of age and 90% die by 1 Half this group die by 6 months of age and 90% die by 1 year of age year of age

Kirklin JW, Barratt-Boyes BG. Ventricula septal defect with pulmonary Kirklin JW, Barratt-Boyes BG. Ventricula septal defect with pulmonary stenosis or atresia. In: stenosis or atresia. In: Cardiac Surgery, 2nd edn. Cardiac Surgery, 2nd edn. New York: Churchill New York: Churchill Livingstone, 1993; 861–1012.Livingstone, 1993; 861–1012.

Adequate pulmonary blood flow - a greater Adequate pulmonary blood flow - a greater longevity is seen.longevity is seen.

Survival in to the sixth decade reported in Survival in to the sixth decade reported in unoperated patient with pulmonary atresia, unoperated patient with pulmonary atresia, ventricular septal defect, and multiple ventricular septal defect, and multiple aortopulmonary collaterals.aortopulmonary collaterals.

The median age at death was 11 months, ranging The median age at death was 11 months, ranging from 9 days to 30 years.from 9 days to 30 years.

66% of the patients are alive at age 6 months66% of the patients are alive at age 6 months

50% alive by 1 year50% alive by 1 year

8% alive at age 10 years.8% alive at age 10 years.

Various patterns of pulmonary Various patterns of pulmonary arterial anatomy and source of arterial anatomy and source of

blood supply blood supply

Environmental FactorsEnvironmental Factors

Maternal diabetesMaternal diabetes

Maternal phenylketonuria (PKU)Maternal phenylketonuria (PKU)

Maternal exposure to retinoic acids and to Maternal exposure to retinoic acids and to trimethadionetrimethadione

Infants of diabetic women.Infants of diabetic women.

EpidemiologyEpidemiology

Baltimore-Washington Infant StudyBaltimore-Washington Infant Study3 (BWIS) 3 (BWIS) recorded 4390 infants with cardiovascular recorded 4390 infants with cardiovascular malformations from 1981 – 1989.malformations from 1981 – 1989.

Tet-PA accounted for 1.4% of all forms of Tet-PA accounted for 1.4% of all forms of congenital heart disease and 0.07 per 100 live congenital heart disease and 0.07 per 100 live births.births.

26% of the patients with PA-VSD had 26% of the patients with PA-VSD had chromosomal abnormality, a recognizable chromosomal abnormality, a recognizable syndrome, or other single organ defectssyndrome, or other single organ defects

PA-VSD occurs more oftenPA-VSD occurs more often DiGeorge syndrome and associated with DiGeorge syndrome and associated with

Chromosome 22q11 microdeletion. Chromosome 22q11 microdeletion. VACTERVACTER CHARGE CHARGE AlagilleAlagille

22q11 deletion22q11 deletion

10% of patients with a 22q11 deletion have PA-10% of patients with a 22q11 deletion have PA-VSD . VSD .

Right aortic arch, or aberrant subclavian artery -Right aortic arch, or aberrant subclavian artery -more frequent more frequent

Branch pulmonary arteries are smaller in Branch pulmonary arteries are smaller in patients with a 22q11.2 deletion patients with a 22q11.2 deletion

Congenital Heart Surgeons SocietyCongenital Heart Surgeons Society ClassificationClassification

Type A: Native PAs present, pulmonary Type A: Native PAs present, pulmonary vascular supply through PDA and no APCs.vascular supply through PDA and no APCs.

Type B: Native PAs and APCs presentType B: Native PAs and APCs present

Type C: No native PAs, pulmonary blood Type C: No native PAs, pulmonary blood supply through APCs only.supply through APCs only.

Tchervenkov CI, Roy N. Congenital heart disease Tchervenkov CI, Roy N. Congenital heart disease nomenclature and database project: Pulmonary atresia - nomenclature and database project: Pulmonary atresia - ventricular septal defect. ventricular septal defect. Ann Thoracic Surg 2000; 69:S97-Ann Thoracic Surg 2000; 69:S97-S105S105

Type A pulmonary atresia with ventricular Type A pulmonary atresia with ventricular septal defectseptal defect

Type B pulmonary atresia with ventricular Type B pulmonary atresia with ventricular septal defectseptal defect

Type C pulmonary atresia with ventricular Type C pulmonary atresia with ventricular septal defectseptal defect

PDA in VSD,PAPDA in VSD,PA

In PA-VSD, PDA typically originates from either In PA-VSD, PDA typically originates from either the the undersurface of the arch (67%) undersurface of the arch (67%) or from the or from the undersurface of theundersurface of the

innominate artery (33%).innominate artery (33%).

PDA is S shaped, long and arises at an acute PDA is S shaped, long and arises at an acute angle from Aortaangle from Aorta

Unilateral PDA is usually associated with Unilateral PDA is usually associated with confluent PAsconfluent PAs

PDA can be bilateral with non-confluent PAs. PDA can be bilateral with non-confluent PAs.

When PDA is present, PAs are confluent in 80% When PDA is present, PAs are confluent in 80% of cases. of cases.

PDA is absent in 1/3 of casesPDA is absent in 1/3 of cases

Aortopulmonary collaterals (APCs)Aortopulmonary collaterals (APCs)

The term MAPCA(s) was first used by Macartney, The term MAPCA(s) was first used by Macartney, Deverall and Scott to differentiate them from the Deverall and Scott to differentiate them from the bronchial arteriesbronchial arteries

Aortopulmonary collaterals (APCs) are muscular Aortopulmonary collaterals (APCs) are muscular arteries until they enter the lung parenchyma, the arteries until they enter the lung parenchyma, the muscular layer is gradually replaced by elastic muscular layer is gradually replaced by elastic lamina that resembles true pulmonary arteries. lamina that resembles true pulmonary arteries.

APCs are seen in APCs are seen in 30 – 65% 30 – 65% of patients with PA - VSD of patients with PA - VSD and are usually and are usually 2 – 6 2 – 6 in number.in number.

Macartney F, Deverall P, Scott O. Haemodynamic Macartney F, Deverall P, Scott O. Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br characteristics of systemic arterial blood supply to the lungs. Br Heart J 1973;35:28–37.Heart J 1973;35:28–37.

Known sites of origin of APCs includeKnown sites of origin of APCs include descending thoracic aorta descending thoracic aorta subclavian arteriessubclavian arteries abdominal aortaabdominal aorta coronary arteries. coronary arteries.

Three types of SCAsThree types of SCAs

Type I: Bronchial artery branches- arising from Type I: Bronchial artery branches- arising from one of the normal bronchial arteries.one of the normal bronchial arteries.

Type Il: Direct aortic branches- arising directly Type Il: Direct aortic branches- arising directly from the descending thoracic aorta.from the descending thoracic aorta.

Type III. Indirect aortic branches-branches Type III. Indirect aortic branches-branches arising from branches of the aorta other than arising from branches of the aorta other than bronchial artery.bronchial artery.

e.g:from subclavian, internal mammary and e.g:from subclavian, internal mammary and intercostal arteries.intercostal arteries.

PDA is considered a less reliable source beyond PDA is considered a less reliable source beyond the first few days of life due to its tendency to the first few days of life due to its tendency to close. close.

APCs are also prone for stenosis over a period of APCs are also prone for stenosis over a period of weeks to months but are more reliable than weeks to months but are more reliable than PDA PDA

PDA VS MAPCASPDA VS MAPCAS

PDA MAPCA’s

ORGIN Opposite to LSCA DTA

COURSE STRAIGHT TORTOUS/RETROESOPHAGEAL

BRANCHING NO BRANCHING

STENOSIS PA end AORTIC END

DESTINY CENTRAL PA JOIN PA at HILUM / LOBAR/SEGMENTAL

FLOW IN CENTRAL PULMONARY ARTERIES

RETROGRADE ANTEGRADE

Clinical FeaturesClinical Features

PA-VSD presents as a cyanotic newbornPA-VSD presents as a cyanotic newborn

Infant becomes increasingly hypoxemic as the Infant becomes increasingly hypoxemic as the ductus constricts.ductus constricts.

If the ductus arteriosus remains patent or If the ductus arteriosus remains patent or because systemic collateral vessels are because systemic collateral vessels are sufficiently developed to provide adequate sufficiently developed to provide adequate pulmonary blood flow- not severely hypoxemicpulmonary blood flow- not severely hypoxemic

Hypoxemia and cyanosis increase as the patient Hypoxemia and cyanosis increase as the patient “outgrows” the relatively fixed sources of “outgrows” the relatively fixed sources of pulmonary blood flow.pulmonary blood flow.

If growth is delayed-suspect the presence of a If growth is delayed-suspect the presence of a 22q11.2 microdeletion. 22q11.2 microdeletion.

(growth failure due to heart failure caused by (growth failure due to heart failure caused by excessive pulmonary blood flow is uncommon)excessive pulmonary blood flow is uncommon)

Modes of presentationModes of presentation

Cyanosis - 50%Cyanosis - 50%

Heart failure – 25%Heart failure – 25%

Murmur with mild cyanosis – 25%Murmur with mild cyanosis – 25%

Peripheral pulsesPeripheral pulses

The peripheral pulses and blood pressure usually The peripheral pulses and blood pressure usually are normal in the neonatal period(even with a are normal in the neonatal period(even with a PDA)PDA)

Beyond the first 4 to 6 weeks of age - if Beyond the first 4 to 6 weeks of age - if pulmonary blood flow is through a PDA or pulmonary blood flow is through a PDA or collaterals ,the pulses are bounding, and only collaterals ,the pulses are bounding, and only minimal cyanosis is present. minimal cyanosis is present.

There is a normal first heart sound and a single There is a normal first heart sound and a single loud second heart sound. loud second heart sound.

A systolic murmur may be audible along the lower A systolic murmur may be audible along the lower left sternal border but usually is not more than left sternal border but usually is not more than grade 3/6 in intensity. grade 3/6 in intensity.

The right ventricular outflow tract is atretic-no The right ventricular outflow tract is atretic-no separate loud systolic ejection murmur at the separate loud systolic ejection murmur at the upper left sternal border -upper left sternal border -this is in contrast to this is in contrast to the finding in TOF with antegrade pulmonary the finding in TOF with antegrade pulmonary blood flow. blood flow.

If a PDA is present, a continuous murmur usually If a PDA is present, a continuous murmur usually is heard after the first 4 to 6 weeks of life. is heard after the first 4 to 6 weeks of life.

If systemic-to-pulmonary collateral vessels are If systemic-to-pulmonary collateral vessels are present, continuous murmurs can be heard-present, continuous murmurs can be heard-multiple and prominent over the back multiple and prominent over the back

(originate from the descending aorta)(originate from the descending aorta)

Electrocardiographic FeaturesElectrocardiographic Features

Right ventricular hypertrophy Right ventricular hypertrophy

Right-axis deviation Right-axis deviation

Increased pulmonary blood flow -combined Increased pulmonary blood flow -combined ventricular hypertrophy and left atrial ventricular hypertrophy and left atrial enlargement may occur.enlargement may occur.

Radiographic FeaturesRadiographic Features Characteristic appearance likened to the shape of a Characteristic appearance likened to the shape of a

boot (boot (coeur en sabotcoeur en sabot).). levorotation of the heart, a prominent upturned levorotation of the heart, a prominent upturned

cardiac apex, secondary to right ventricular cardiac apex, secondary to right ventricular hypertrophy. hypertrophy.

concavity in the region of the main pulmonary concavity in the region of the main pulmonary artery produced by underdevelopment of the artery produced by underdevelopment of the subpulmonary infundibulum. subpulmonary infundibulum.

The The frequency of a right-sided aortic arch is greater frequency of a right-sided aortic arch is greater in patients with PA-VSD in patients with PA-VSD (26% to 50% of these (26% to 50% of these patients) than in those with TOF (20% to 25%). patients) than in those with TOF (20% to 25%).

Pulmonary vascular markings have a typical Pulmonary vascular markings have a typical reticular pattern when there are multiple reticular pattern when there are multiple collaterals supplying the lungs. collaterals supplying the lungs.

Extent of pulmonary vascular markings will Extent of pulmonary vascular markings will depend on the extent of pulmonary blood flow.depend on the extent of pulmonary blood flow.

Echocardiographic FeaturesEchocardiographic Features

Parasternal long-axis show a large aortic valve that Parasternal long-axis show a large aortic valve that overrides a malaligned VSD . The infundibular portion overrides a malaligned VSD . The infundibular portion of the ventricular septum is anteriorly malpositioned. of the ventricular septum is anteriorly malpositioned.

Patient with TOF has a patent, although hypoplastic, Patient with TOF has a patent, although hypoplastic, right ventricular outflow tract anterior to the right ventricular outflow tract anterior to the infundibular septum. This outflow tract is in continuity infundibular septum. This outflow tract is in continuity with the main pulmonary artery.with the main pulmonary artery.

The infundibular septum is fused with the free wall The infundibular septum is fused with the free wall in patients with PA-VSD, and there is no separate in patients with PA-VSD, and there is no separate outflow from the right ventricle .outflow from the right ventricle .

Truncus arteriosus - resembles PA-VSD Truncus arteriosus - resembles PA-VSD

(in truncus arteriosus, the pulmonary arteries (in truncus arteriosus, the pulmonary arteries arise directly from the posterolateral aspect of arise directly from the posterolateral aspect of the truncal root prior to the arch.)the truncal root prior to the arch.)

Suprasternal notch and high parasternal Suprasternal notch and high parasternal windows - provide important information about windows - provide important information about the size and status of the proximal pulmonary the size and status of the proximal pulmonary arteries. arteries.

The position of the malalignment VSD, The position of the malalignment VSD, membranous or infundibular, can be determined. membranous or infundibular, can be determined.

ASDs and additional muscular VSDs can be ASDs and additional muscular VSDs can be detected.detected.

Short-axis parasternal and subcostal views-Short-axis parasternal and subcostal views-detecting coronary artery abnormalities .detecting coronary artery abnormalities .

Color flow imaging and continuous wave Doppler Color flow imaging and continuous wave Doppler techniques -assessment of surgically created techniques -assessment of surgically created right ventricular to pulmonary artery conduits right ventricular to pulmonary artery conduits

Cardiac Catheterization and Cardiac Catheterization and AngiographyAngiography

Delineate the size and distribution of the true Delineate the size and distribution of the true pulmonary arteries and to ascertain the extent of pulmonary arteries and to ascertain the extent of collateral blood supply to the lungscollateral blood supply to the lungs

Because of the large VSD, RV pressure is equal Because of the large VSD, RV pressure is equal to the left ventricle pressure. to the left ventricle pressure.

Right ventricular outflow tract is atretic -the Right ventricular outflow tract is atretic -the catheter will not enter the pulmonary arteries catheter will not enter the pulmonary arteries from the right ventricle (manipulated from the from the right ventricle (manipulated from the right ventricle through the VSD into the aorta.)right ventricle through the VSD into the aorta.)

Widened pulse pressure may be present if there Widened pulse pressure may be present if there

is a large runoff into the lungs through a PDA or is a large runoff into the lungs through a PDA or a previously constructed shunt. a previously constructed shunt.

Ventricular and aortic root angiography should be Ventricular and aortic root angiography should be donedone

Ventriculography should be performed with an Ventriculography should be performed with an injection into the left ventricular cavity while the injection into the left ventricular cavity while the cameras are positioned to record a 70-degree left cameras are positioned to record a 70-degree left anterior oblique view with 20 degrees of cranial anterior oblique view with 20 degrees of cranial angulation. This projection displays the middle angulation. This projection displays the middle portion and most of the upper interventricular portion and most of the upper interventricular septum tangentially.septum tangentially.

Coronary artery anatomy can be defined by an Coronary artery anatomy can be defined by an aortic root angiocardiogram and a 70-degree left aortic root angiocardiogram and a 70-degree left anterior oblique view (with 20 degrees of cranial anterior oblique view (with 20 degrees of cranial angulation). angulation).

An improved angiographic projection (frontal x-An improved angiographic projection (frontal x-ray tube is caudally angled) -ray tube is caudally angled) -"laid-back" "laid-back" position position of the image intensifier and cine camera results in of the image intensifier and cine camera results in superior visualization of the coronary arteries and superior visualization of the coronary arteries and their relation to the aorta and the pulmonary their relation to the aorta and the pulmonary artery. artery. 

Surgical importance - Surgical importance - origin of the left anterior origin of the left anterior descending coronary artery from the right descending coronary artery from the right coronary arterycoronary artery, which occurs in approximately , which occurs in approximately 5% 5% of patientsof patients

Angiographic delineation of the anatomy of Angiographic delineation of the anatomy of pulmonary blood supply - pulmonary blood supply - Venous approach by crossing the VSDVenous approach by crossing the VSD Retrograde arterial approach Retrograde arterial approach

The image should provide a large field of view, The image should provide a large field of view, ideally visualizing both lung fieldsideally visualizing both lung fields

Selective injections in the systemic-to-pulmonary Selective injections in the systemic-to-pulmonary collateral arteries -collateral arteries - to delineate the extent of the pulmonary to delineate the extent of the pulmonary

arterial tree supplied by each collateral vessel arterial tree supplied by each collateral vessel determine which type of pulmonary artery determine which type of pulmonary artery

connection is present connection is present Evanescent negative washout pattern -stream of Evanescent negative washout pattern -stream of

unopacified blood from a connecting pulmonary unopacified blood from a connecting pulmonary artery flowing into an area of opacified artery flowing into an area of opacified pulmonary arterial tree(may be the only pulmonary arterial tree(may be the only indication of an existing communication)indication of an existing communication)

Long-axis oblique view of left Long-axis oblique view of left ventriculogramventriculogram

Aortogram demonstrates large pulmonary Aortogram demonstrates large pulmonary confluenceconfluence

Selective injection into a collateral Selective injection into a collateral artery arising from middle portion of artery arising from middle portion of

descending thoracic aortadescending thoracic aorta

Pulmonary vein wedge angiogram Pulmonary vein wedge angiogram demonstrating a hypoplastic pulmonary demonstrating a hypoplastic pulmonary

artery confluenceartery confluence

CT / MR angiographyCT / MR angiography

Alternative modality to define RVOT, Alternative modality to define RVOT, MPA, branch PAs and APCsMPA, branch PAs and APCs

Needs lesser contrast.Needs lesser contrast.

Evaluation of adequacy ofEvaluation of adequacy ofpulmonary arteriespulmonary arteries

Complexity of pulmonary blood supply Complexity of pulmonary blood supply determines the extent of surgical exploration determines the extent of surgical exploration necessary to perform unifocalizationnecessary to perform unifocalization

Eligibility for complete repair is dependent as Eligibility for complete repair is dependent as RV-PA conduit needs to be placed to the vessel RV-PA conduit needs to be placed to the vessel which is connected to maximum possible which is connected to maximum possible pulmonary vascular bed. pulmonary vascular bed.

Closing the VSD at the time of placement of RV – Closing the VSD at the time of placement of RV – PA conduit needs to be determined.PA conduit needs to be determined.

McGoon's ratioMcGoon's ratio

McGoon's ratio is calculated by dividing the sum of McGoon's ratio is calculated by dividing the sum of the diameters of RPA (at the level of crossing the the diameters of RPA (at the level of crossing the lateral marginlateral margin

of vertebral column on angiogram) and LPA (just of vertebral column on angiogram) and LPA (just proximal to its upper lobe branch), divided by the proximal to its upper lobe branch), divided by the diameter of aorta at the level above the diaphragm diameter of aorta at the level above the diaphragm

[D RPA + D LPA] / D TAO [D RPA + D LPA] / D TAO An average value of 2.1 is normalAn average value of 2.1 is normal Ratio Ratio above 1.2 above 1.2 -acceptable postoperative RV -acceptable postoperative RV

systolic pressure in Tetralogy of Fallot.systolic pressure in Tetralogy of Fallot. Ratio Ratio below 0.8 - inadequate below 0.8 - inadequate for complete repair of for complete repair of

PA – VSDPA – VSD

Nakata indexNakata index

Nakata PA index is calculated from the diameter of PAs Nakata PA index is calculated from the diameter of PAs measured immediately proximal to the origin of upper measured immediately proximal to the origin of upper lobe branches of the respective branch PAslobe branches of the respective branch PAs..

The The sum of the cross sectional area (CSA) of right and sum of the cross sectional area (CSA) of right and left PAs is divided by the body surface area of the patientleft PAs is divided by the body surface area of the patient

Nakata index = CSA of RPA (mm2)+ CSA of LPA (mm2)/ Nakata index = CSA of RPA (mm2)+ CSA of LPA (mm2)/ BSA (m2BSA (m2))

A Nakata index of A Nakata index of >150 mm2/m2 >150 mm2/m2 is acceptable for is acceptable for completecomplete

repair without prior palliative shuntrepair without prior palliative shunt..

Nakata index is widely used in preoperative Nakata index is widely used in preoperative assessment of adequacy of pulmonary vascular assessment of adequacy of pulmonary vascular bedbed

Not useful in patients with multifocal pulmonary Not useful in patients with multifocal pulmonary blood supply, who are evaluated for single-stage blood supply, who are evaluated for single-stage repair of PA - VSD.repair of PA - VSD.

Total Neo-pulmonary artery index Total Neo-pulmonary artery index (TNPAI)(TNPAI)

APCs index was calculated by addition of CSA of APCs index was calculated by addition of CSA of all significant APCs divided by the BSA. all significant APCs divided by the BSA.

CSA of each APC is calculated from diameter of CSA of each APC is calculated from diameter of the respective vessels measured on preoperative the respective vessels measured on preoperative cineangiogramcineangiogram

The sum of total APC index and PA index is called TNPAI.The sum of total APC index and PA index is called TNPAI.

A A TNPAI index >200 mm2/m2 TNPAI index >200 mm2/m2 correlated well with low correlated well with low postoperative RV/LV pressure ratio and identified patients postoperative RV/LV pressure ratio and identified patients who were who were candidates for VSD closure at the time of candidates for VSD closure at the time of single-stage surgicalrepair.single-stage surgicalrepair.

Reddy MV, Petrossian E, McElhinney DB, Moore P, Teitel Reddy MV, Petrossian E, McElhinney DB, Moore P, Teitel DF, Hanley FL: One stage complete unifocalization in DF, Hanley FL: One stage complete unifocalization in infants: When should the ventricular septal defect be infants: When should the ventricular septal defect be closed? closed? J Thorac Cardiovasc Surg 1997;113:858-868.J Thorac Cardiovasc Surg 1997;113:858-868.

These indices are of limited value since they are These indices are of limited value since they are based on the size of the proximal vessels only. based on the size of the proximal vessels only.

The nature of the distal pulmonary vascular bed The nature of the distal pulmonary vascular bed and pulmonary vascular resistance are not and pulmonary vascular resistance are not expressed in these calculationsexpressed in these calculations

General principles of surgicalGeneral principles of surgicaltherapy of PA-VSDtherapy of PA-VSD

Connect as many lung segments as possible to Connect as many lung segments as possible to the blood flow from RV during early infancy - to the blood flow from RV during early infancy - to avoid significant histologic changes occurs in avoid significant histologic changes occurs in pulmonary vasculaturepulmonary vasculature

Complete repair should be attempted within Complete repair should be attempted within weeks to months during infancy.weeks to months during infancy.

Therapeutic catheterization procedures such as Therapeutic catheterization procedures such as balloon angioplasty help to rehabilitate balloon angioplasty help to rehabilitate pulmonary arteries with stenosis.pulmonary arteries with stenosis.

Components of surgical repairComponents of surgical repair

Placement of RV – PA conduitPlacement of RV – PA conduit

Unifocalization of APCs Unifocalization of APCs

VSD closure. VSD closure.

These components are performed in one-stage, These components are performed in one-stage, or at different operations depending on the or at different operations depending on the anatomy and institutional policy.anatomy and institutional policy.

RV – PA conduit placementRV – PA conduit placement

Cadaveric, cryopreserved homograft is used to Cadaveric, cryopreserved homograft is used to connect right ventricle to available central connect right ventricle to available central pulmonary arteries. pulmonary arteries.

In complex cases, where a central pulmonary In complex cases, where a central pulmonary artery is absent or the pulmonary blood flow is artery is absent or the pulmonary blood flow is multifocal, unifocalization of the diminutive multifocal, unifocalization of the diminutive native pulmonary arteries and APCs will be native pulmonary arteries and APCs will be performed before RV – PA conduit is placedperformed before RV – PA conduit is placed

Unifocalization of APCsUnifocalization of APCs

Unifocalize significant APCs during the first 3 Unifocalize significant APCs during the first 3 months of lifemonths of life

Median sternotomy is the preferred method Median sternotomy is the preferred method especially if single stage repair is planned.especially if single stage repair is planned.

In multi stage surgical approach, unifocalization In multi stage surgical approach, unifocalization is done through lateral thoracotomies.is done through lateral thoracotomies.

During unifocalization, APCs are ligated at the During unifocalization, APCs are ligated at the origin and mobilized to maximize their length . origin and mobilized to maximize their length .

Anastomosed in the mediastinum and connected Anastomosed in the mediastinum and connected to RV-PA conduit.to RV-PA conduit.

Aortic arch angiogram before (A) and main Aortic arch angiogram before (A) and main pulmonary arteriogram (B)after 1-stage pulmonary arteriogram (B)after 1-stage

complete unifocalization complete unifocalization

VSD closureVSD closure

Closure of VSD at the time of initial repair Closure of VSD at the time of initial repair avoids the need for further surgery. avoids the need for further surgery.

If any concerns about the adequacy of the If any concerns about the adequacy of the pulmonary vascular bed -defer VSD closure.pulmonary vascular bed -defer VSD closure.

Unrepaired VSD avoids supra-systemic RV Unrepaired VSD avoids supra-systemic RV pressure in the immediate postoperative period pressure in the immediate postoperative period by allowing RV to decompress through the VSD.by allowing RV to decompress through the VSD.

VSD closureVSD closure

Alternative strategy -Alternative strategy - closingclosing VSD with a fenestrated VSD with a fenestrated patchpatch and the fenestration can be closed later either by and the fenestration can be closed later either by surgery or transcatheter technique.surgery or transcatheter technique.

When VSD closure is deferred at initial repair, it is When VSD closure is deferred at initial repair, it is surgically closed after 6 – 12 months- when surgically closed after 6 – 12 months- when left to right left to right shuntshunt is established via the VSD with is established via the VSD with Qp/Qs exceeding Qp/Qs exceeding 2:1 2:1 by catheter evaluation .by catheter evaluation .

Reddy MV, Petrossian E, McElhinney DB, Moore P, Reddy MV, Petrossian E, McElhinney DB, Moore P, Teitel DF, Hanley FL: One stage complete unifocalization Teitel DF, Hanley FL: One stage complete unifocalization in infants: When should the ventricular septal defect be in infants: When should the ventricular septal defect be closed? closed? J Thorac Cardiovasc Surg 1997;113:858-868.J Thorac Cardiovasc Surg 1997;113:858-868.

Multi-stage versus single-stageMulti-stage versus single-stageapproachapproach

The choice between multi-stage and single-stage The choice between multi-stage and single-stage repair is dependent on various factors: repair is dependent on various factors:

Nature of PAs (small vs good size)Nature of PAs (small vs good size) Duct-dependent or collateral-dependent PBFDuct-dependent or collateral-dependent PBF Status of APCs Status of APCs Availability of surgical skills and results of the Availability of surgical skills and results of the

institution.institution.

Multi-stage approach:Multi-stage approach:

Traditional approach - palliative shunt in Traditional approach - palliative shunt in patients with good size, confluent central PA patients with good size, confluent central PA during neonatal period or early infancy to relieve during neonatal period or early infancy to relieve cyanosis and allow for growth of distal cyanosis and allow for growth of distal pulmonary arteries. pulmonary arteries.

With diminutive PAs, RV – PA continuity is With diminutive PAs, RV – PA continuity is established by placing a RV – PA conduitestablished by placing a RV – PA conduit

The VSD is typically left open at this first stage. The VSD is typically left open at this first stage.

Unifocalization of APCs Unifocalization of APCs

A subsequent surgery - A subsequent surgery - VSD closureVSD closure Relieve any residual right ventricular outflow Relieve any residual right ventricular outflow

tract obstructiontract obstruction Placement of a valved conduit. Placement of a valved conduit.

Right UnifocalizationRight Unifocalization

Left unifocalizationLeft unifocalization

Definitive repair inDefinitive repair ina patient with a previous bilateral a patient with a previous bilateral

unifocalizationunifocalization

Single-stage approachSingle-stage approach

Attempts to perform APCs unifocalization and Attempts to perform APCs unifocalization and cardiac repair at the same operation, through cardiac repair at the same operation, through median sternotomymedian sternotomy

Comparison of outcome betweenComparison of outcome betweenmulti and single-stage repairmulti and single-stage repair

The ultimate results are comparable but patients in the The ultimate results are comparable but patients in the single stage group undergo one or two operations less single stage group undergo one or two operations less than the patients in multi-stage group do.than the patients in multi-stage group do.

Tchervenkov CI, Salasidis G, Cecere R, Beland MJ, Jutras L, Paquet Tchervenkov CI, Salasidis G, Cecere R, Beland MJ, Jutras L, Paquet M, Dobell ARC: One-stage midline unifocalization and complete repair M, Dobell ARC: One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals. complex heart disease with major aortopulmonary collaterals. J J Thorac Cardiovasc Surg 1997;114:727-737.Thorac Cardiovasc Surg 1997;114:727-737.

Murthy KS, Rao SG, Krishnanaik S, Coelho R,Murthy KS, Rao SG, Krishnanaik S, Coelho R,Krishnan US, Cherian Krishnan US, Cherian KM: Evolving surgical management for ventricular septal defect, KM: Evolving surgical management for ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. pulmonary atresia, and major aortopulmonary collateral arteries. Ann Ann Thoracic Surg 1999;67:760-764.Thoracic Surg 1999;67:760-764.

Early 1-stage complete unifocalization can be Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia performed in >90% of patients with pulmonary atresia and MAPCAs, and yields good functional results.and MAPCAs, and yields good functional results.

Complete repair during the same operation is Complete repair during the same operation is achieved in two thirds of patients. achieved in two thirds of patients.

Actuarial survival 3 years after surgery is 80%, and Actuarial survival 3 years after surgery is 80%, and but there is a significant rate of reintervention.but there is a significant rate of reintervention.

Early and Intermediate Outcomes After Repair of Pulmonary Early and Intermediate Outcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries Collateral Arteries

Experience With 85 Patients :Experience With 85 Patients :V. Mohan Reddy, MD; Doff B. V. Mohan Reddy, MD; Doff B. McElhinney, MD; Zahid Amin, MD; Phillip Moore, MD; Andrew J. Parry, McElhinney, MD; Zahid Amin, MD; Phillip Moore, MD; Andrew J. Parry, MD; David F. Teitel, MD; Frank L. Hanley, MD MD; David F. Teitel, MD; Frank L. Hanley, MD

Circulation. 2000; 101: 1826-1832

Outcome of surgical Outcome of surgical repair repair

Early mortality - 4.5%Early mortality - 4.5%

Late mortality - 16% Late mortality - 16%

Ten- and 20-year survival - 86% and 75% Ten- and 20-year survival - 86% and 75%

Freedom from reoperation of 55%.Freedom from reoperation of 55%.

Early and long-term results of the surgical treatment of Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries:major aortopulmonary collateral arteries: John M. Cho et al John M. Cho et al

J Thorac Cardiovasc Surg 2002;124:70-81J Thorac Cardiovasc Surg 2002;124:70-81

Perioperative complicationsPerioperative complications

Phrenic nerve injury-0.3 %Phrenic nerve injury-0.3 % Reoperation for bleed-3%Reoperation for bleed-3% Sepsis-5%Sepsis-5% Heart block-0.5%Heart block-0.5% Pulmonary infarction-0.4%Pulmonary infarction-0.4%

Complementary role ofComplementary role ofinterventional catheterizationinterventional catheterization

Dilation of Distal stenosis within lung Dilation of Distal stenosis within lung parenchyma (inaccessible to the surgeon. )parenchyma (inaccessible to the surgeon. )

Coil occlusion of APCsCoil occlusion of APCs

Stent placement in RVOT Stent placement in RVOT

Palliative stenting of stenotic APC’SPalliative stenting of stenotic APC’S

Long term sequelaeLong term sequelae

Patients on palliative shunts, develop Patients on palliative shunts, develop progressive cyanosis progressive cyanosis and polycythemiaand polycythemia

Aortic regurgitationAortic regurgitation

Deterioration of conduit and valve function Deterioration of conduit and valve function by loss of by loss of luminal diameter, calcification, peel luminal diameter, calcification, peel formationformation

Pulmonary regurgitation worsens with RV Pulmonary regurgitation worsens with RV dilatation and dilatation and dysfunctiondysfunction

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