Surgical Procedures. Direct suture of small ASDs Patch repair Transcatheter closure with a prosthetic device called occluder

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1 PEDIATRIC Review

2 Surgical Procedures Atrial Septal Defect repair: Direct suture of small ASDs Patch repair Transcatheter closure with a prosthetic device called occluder Balloon atrial septostomy (Rashkind) Transcatheter procedure using an inflated balloon passed across the interatrial septum to create an ASD

3 Balloon valvotomy(balloon valvuloplasty) Transcatheter procedure using an inflated balloon to relieve obstruction in a stenosed valve. Blalock-Taussig shunt Palliative procedure used in cyanotic defects Direct anastomosis of the right subclavian artery to right pulmonary artery Directs blood flow from systemic to pulmonary circulation

4 Damus-Kaye-Stansel procedure Palliative procedure used to establish systemic arterial flow in the treatment of one functional ventricle associated with sub-aortic obstruction Main PA is divided, proximal end is anastomosed to ascending aorta, bypassing the AV. Distal end of PA is closed. Pulmonary flow is maintained with a B-T shunt from RSCA to RPA

5 Fontan procedure Staged surgical repair for defects characterized by one functional ventricle The goal is to separate pulmonary and systemic circulation Stage 1: if cyanosis is present, B-T shunt or PA banding if overcirculation Stage 2: Glenn: SVC to RPA Stage 3 Fontan completion: IVC to RPA, by placing an intracardiac tunnel within the RA( lateral tunnel Fontan) or creating a conduit for IVC outside the RA(extracardiac Fontan)

6 Glenn procedure or Glenn shunt Single ventricle defects, the goal is to separate pulmonary and systemic circulations Replaces B-T shunts with anastomosis of SVC to RPA Venous return from upper body flows directly into the RPA Also known as Hemi-Fontan procedure

7 Jatene procedure Arterial switch used to connect the PA to RV and AO to the LV when the great arteries are completely transposed. The aorta and pulmonary artery are detached from their roots and repositioned over their counterpart PA is anastomosed to aortic root becoming a neopulmonary artery Ao is anastomosed to PA root becoming a neo-aorta Coronary arteries are re-attached to neo-aorta

8 Konno procedure A procedure for aortic root replacement used in the treatment of left ventricular outflow tract obstruction in association with aortic valve stenosis Av and coronaries are removed, LVOT is opened and enlarged, an aortic or pulmonary homograph replaces the AV. Coronaries are transferred to new aorta

9 Mustard procedure Atrial switch procedure utilizing a surgically created intra-atrial tunnel to redirect flow in the atria The interatrial septum is excised and a patch is used to create a tunnel The tunnel redirects oxygenated pulmonary venous return into the RV where is ejected out of the transposed aorta Deoxygenated cavae flow is redirected into the left ventricle, where is ejected out of the transposed pulmonary artery

10 Norwood procedure Use in the management of hypoplastic left heart syndrome to convert the RV from a pulmonary function to a systemic and completely separate pulmonary and systemic circulation Stage 1 palliation: RV is converted into the main pumping chamber by dividing the main PA and attaching the pulmonary root and valve to the base of the aorta. An ASD is opened. A B-T shunt is created Stage 2 Glenn procedure- SVC to RPA Stage 3 Fontan completion-ivc to RPA

11 PDA repair PDA ligation: the PDA is tied off at both ends with sutures and cut in the middle Transcatheter coil occlusion: the PDA is closed with a spring-type device called a coil. Potts shunt Palliative procedure, establish a systemic to pulmonary connection and increase pulmonary circulation Anastomoses the descending Ao to LPA

12 Pulmonary artery band Palliative procedure used in patients with pulmonary over-circulation. An adjustable band is placed around the main PA and tightened, restricting flow to the branches and lungs

13 Rastelli procedure Used in a variety of cyanotic defects presenting in combination with VSD and severe RVOT obstruction Repair includes: Patch repair of VSD Excision of obstructive tissue in the RV Surgical closure of abnormal or damaged PV Creation of an extracardiac valved conduit from the RV to the bifurcation of the main PA

14 Ross procedure Procedure for the replacement of a stenotic aortic valve Aortic root is removed The patient s own pulmonary valve is excised and used to replace the aortic valve PV is replaced with a valved homograft

15 Waterston shunt Palliative procedure used to establish a systemicto-pulmonary connection and increase pulmonary circulation by directly anastomosing the ascending aorta and RPA

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18 Anomalous Coronary Arteries Left Main Coronary Artery Arising from the Pulmonary Artery Most common defect among anomalous coronary arteries and requires surgical intervention Systemic left main coronary SaO2 levels 70% Left Main Coronary Artery Arising from the RCA Less serious defect, there is fully saturated flow in the LMCA If LMCA is trapped between the 2 great arteries it might get trapped and cause symptoms during exercise. RCA Arising from the LMCA Also known as single coronary artery The vessel is fully saturated and no surgical repair is necessary

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22 Aortic Arch Abnormalities Right aortic Arch Is essentially a mirror image of the normal left aortic arch Is highly associated with TOF There is usually no surgical repair Interrupted Aortic Arch Is an absence of a segment of the descending thoracic aorta Type A-interruption occurs distal to LSA Type B-interruption occurs distal to LCCA and just proximal to LSA Type C-occurs distal to brachiocephalic artery

23 Persistent Right Aortic Arch Is a type of vascular ring characterized by the presence of an extra, right sided branch of the aorta that reconnects the aortic trunk distally. Abnormal origin of LCCA This is an abnormality where the LCCA arises from the brachiocephalic artery

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27 Persistent Left Superior Vena Cava Is the most common congenital abnormality occurring in association with other congenital defects. There are 2 types: PLSVC drains directly into coronary sinus(90%) PLSVC connects to LA PLSVC draining into RA via CS CS is enlarged RA and RV maybe dilated Coronary Sinus ASD PLSVC connects to LA CS is almost always enlarged Unroofed coronary sinus Associated cardiac abnormalities ASD-VSD TOF Endocardial cushion defect Cor triatriatum

28 Persistent Left SVC

29 Total Anomalous Pulmonary Venous Return Occurs when all 4 pulmonary veins drain oxygenated into the vena cavae Oxygenated and deoxygenated blood myxung takes place in the vena cavae and RA An interatrial communication with R-L shunting is required Types: Supracardiac Intracardiac Infracardiac Echocardiographic documentation of all 4 pulmonary veins is important.

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31 Tricuspid Atresia Is a cyanotic abnormality characterized by complete failure of the TV to form RA and RV are not connected, PA and AO maybe normally related or transposed An atrial septal defect with R-L shunting will be present RV is hypoplastic RA maybe enlarged and hypertrophic LV is usually enlarged with decreased ventricular function as a result of volume overload Pulmonary circulation maybe dependent on a PDA or VSD Patients with large VSD and no associated pulmonary obstructions will exhibit pulmonary circulation and have minimal cyanosis

32 Associated Cardiac Abnormalities may include: Transposition of the great arteries D-type Transposition of the great arteries L-type Persistent Left Superior Vena Cava Coartation of the aorta Right aortic arch Surgical Repair-Fontan Procedure B-T shunt at 4-6 weeks Glenn procedure at 4-6 months Fontan completion at 2-3 years

33 Tricuspid Atresia with D-Transposition Occurs when the TV fails to form and the AO and PA arise from the wrong ventricles. An atrial septal defect will be present A large VSD with left-to-right shunting will almost always be present Patients with no associated pulmonary obstructions ans a large VSD will usually have abnormally high pulmonary flow and severe pulmonary hypertension Cyanosis will usually be present. Subvalvular aortic stenosis is common Associated cardiac abnormalites: Coartation of the aorta Interrupted aortic arch

34 Tricuspid Atresia with D-transposition

35 Truncus Arteriosus Is a rare abnormality in which the embryologic truncus fails to divide There is a single great artery with a common trunk overriding a large ventricular septal defect. The aortic and pulmonic valves are fused into a large semilunar valve with multiple leaflets called truncal valve Truncus arteriosus is classified into 4 types: Type I: a single pulmonary trunk arises from the common arterial trunk with left and right pulmonary arteries arising from the pulmonary trunk Type II: the left and right pulmonary arteries arise separately from the posterior portion of the common arterial trunl Type III: the left and right pulmonary arteries arise as separate vessels from the lateral portion of the common trunk, widely separated from one another Type IV: this type is considered pulmonary atresia, neither of the pulmonary arteries arises from the common trunk

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37 Truncus Arteriosus

38 Cor Triatriatum Triatrial heart, is a rare abnormality characterized by the presence of a third atrial on the left side resulting from the incomplete absorption of the common pulmonary vein during fetal development There will almost always be an atrial septal defect with left-to-right shunting In most cases a VSD will also be present Echocardiography: Always document pulmonary inflow in multiple views Evaluate the direction of flow and the gradient of any shunts Do not confuse cor triatriatum with mitral stenosis. Mitral stenosis is rarely seen in neonates Associated cardiac abnormalities Coartation of the aorta AV Canal Pulmonary stenosis PAPVR

39 Cor Triatriatum

40 Branch Stenosis and Pulmonary Artery Band Is typically a vasoconstrictive response, is one of the most common pediatric heart conditions, especially in preemies and full term neonates. Usually occurs as a result of PHTN in order to reduce flow in response to elevated pressure in the lung Will usually resolve over time once the underlying cause of PHTN has been corrected Always measure the gradients across the RVOT, PV, MPA, RPA and LPA Any gradient above 16mmHg is considered abnormal

41 Pulmonary Banding

42 Hypoplastic Left Heart Syndrome Is a cyanotic abnormality characterized by severe underdevelopment of the left ventricle aortic valve and mitral valve and ascending aorta. HLHS is the most common cause of death among neonates with congenital heart defects The LV and ascending AO are severely hypoplastic Aortic atresia or aortic stenosis is common A coartation of the aortic isthmus opposite the PDA will usually be present Varying degree of hypoplasia of the left atrium RV is dilated and may be hypertrophic An atrial septal defect will usually be present Intracardiac mixing occurs in the RV. Systemic circulation is dependent on the PDA with right-to-left shunting

43 Surgical Repair is performed in 3 stages: Norwood-stage I (B-T shunt) Norwood-stage II (Glenn procedure) Norwood-stage III (Fontan completion)

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45 Hypoplastic Left heart syndrome Norwood Stage I

46 Syndromes and Conditions Associated with Congenital Heart Disease (CHD) Apert s syndrome: A genetic disorder resulting from premature closure of the cranial sutures between the bones of the skull. It is characterized by malformations of the skull and facial features and usually involves webbing and/or fusion of the bony structures of the hands and feet. Associated CHD: VSD ASD Cri du chat: A chromosomal disorder characterized by a distinctive cat-like cry and abnormalities of the skull and face. Associated CHD: PDA VSD ASD (secundum) Endocardial cushion defect (AV canal) Tetralogy of Fallot Truncus arteriosus

47 DiGeorge syndrome: A chromosomal disorder resulting in the absence of the thymus and parathyroid glands. Associated CHD: VSD PDA Interrupted aortic arch Tetralogy of Fallot Truncus arteriousus Down syndrome (Trisomy 21): A chromosomal disorder caused by the presence of extra genetic material from chromosome 21. It is characterized by distinctive facial features and mental deficiency ranging mild to severe. Associated CHD: VSD ASD (primum) Endocardial cushion defect (AV canal) Duchenne and Becker muscular dystrophies (MD): Duchenne MD is a type distinguished by progressive muscle degeneration, skeletal distortions (especially of the spine), paralysis, and eventual death. It almost exclusively affects boys. Becker MD is a milder variant of Duchenne. Associated CHD: Cardiomyopathy

48 Eisenmenger s syndrome: A progressive condition that develops in patients with an underlying heart defect with left-to-right shunting and pulmonary hypertension. Eventually, the pressure in the lungs becomes great enough to reverse the direction of the shunt resulting in cyanosis and organ damage. Associated CHD: VSD PDA Single ventricle Endocardial cushion defect (AV canal) Truncus arteriosus Glycogen storage disease: Any one of several genetic metabolic disorders involving defects in the enzymes responsible for the synthesis and degradation of glycogen. Associated CHD: Left ventricular hypertrophy Cardiomyopathy

49 Kawasaki disease: A disease of unknown etiology that may cause inflammation in the coronary arteries leading to coronary aneurysms. It is seen predominantly in children under the age of 5 (80%). Associated CHD: Enlarged coronary arteries, especially the left main coronary artery Marfan s syndrome: A genetic connective tissue disorder characterized by a tall lean body type with disproportionally long arms, legs, fingers, and toes; flat feet; stooped shoulders; and abnormal joint flexibility. Associated CHD: Aortic root dilation Mitral valve prolapse Aortic valve prolapse

50 Maternal risk factors: Both environmental factors and maternal medical conditions may increase the risk of CHD. The most common maternal risks include: Insulin dependent diabetes (VSD, AV canal, truncus arteriosus) Alcohol and drug abuse (VSD, ASD, tetralogy of Fallot) Phenylketonuria, or PKU (coarctation of the aorta, tetralogy of Fallot) Rubella (PDA, pulmonary stenosis, ASD) Lupus (heart block) Antiseizure medications (ASD, tetralogy of Fallot, VSD) Lithium (Ebstein s malformation, ASD, tricuspid atresia) Mitral atresia: The underdevelopment or absence of a normal mitral valve between the left atrium and left ventricle. Associated CHD: Hypoplastic left heart syndrome Single ventricle Noonan s syndrome: A genetic disorder characterized nu short stature, malformations of the breast bone ( pigeon breast ), webbed neck, and distinctive facial features. Associated CHD: Pulmonary stenosis ASD VSD Cardiomyopathy

51 Oral clefts: Cleft lip and cleft palate are the 2 main types of oral clefts. Infants with oral clefts are approximately 16 times more likely to have CHD than infants with normal nasal and palate development. Associated CHD: ASD PDA VSD Tetralogy of Fallot Scimitar syndrome: A variant of partial anomalous pulmonary venous return in which one or both of the right pulmonary veins drains into the inferior vena cava (instead of the left atrium) through a large anomalous pulmonary vein. Associated CHD: Dextrocardia ASD VSD Coarctation of the aorta Tetralogy of Fallot Pulmonary stenosis

52 Resources for this presentation: Congenital heart defects, simplified A Pediatric Registry Review By Ken Heiden RDCS (AE,PE) RVT