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Description : Check out this medical presentation on Aorta, which is titled "Congenital Heart Disease in Adolescents and Adults", to know about the natural history of common cardiac defects in both operated and unoperated adults, the likely complications and how to manage, and understand the nature and palliative procedures available for previously lethal heart defects which now allow survival to adulthood.

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Published on : Jun 02, 2021 | Views : 2183 | Downloads : 11

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Slide 1-

CONGENITAL HEART DISEASE IN ADOLESCENTS AND ADULTS Michael Giuliano MD,MHPE Jacqueline A. Noonan, MD Associate Professor Pediatrics Professor Emeritus SUNY Downstate Kentucky Children's Hospital NJMS, Rutgers Department of Pediatrics Division of Cardiology
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Objectives To understand natural history of common cardiac defects in both operated and unoperated adults To understand likely complications and how to manage To understand nature and palliative procedures available for previously lethal heart defects which now allow survival to adulthood 2
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Overview US: 1,000,000 adults with congenital heart dz 20,000 more patients reach adolescents yearly Cardiologists/Internists NNP’s and PA’s must Have detailed knowledge of congenital dz, both repaired and unrepaired Clearly define each patients surgical and corrective procedures (read surgical notes) *All figures from ACCSAP V unless otherwise noted
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Population trends in CHD 1985-2000 Circulation 2007;115:163.
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Overview: CHD 1:100 Births LESION Ventricular Septal Defect Pulmonic Stenosis ASD, secundum AV Septal defects Tetralogy of Fallot D-transposition Coarctation of aorta PERCENT 32 % 9 % 7.7 % 7.4 % 6.8 % 4.7 % 4.6 %
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Overview LESSION Hypoplastic left heart Aortic Stenosis Patent ductus arteriosus Heterotaxia Double outlet R ventricle Bicuspid aortic valve Pulmonary atresia PERCENT 3.8 % 2.9 % 2.4 % 2.3 % 2.0 % 1.9 % 1.7 %
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Overview LESSION Total anomalous pulmonary venous return Common arterial trunk L-Transposition Ebstein anomaly Tricuspid atresia Interrupted aortic arch PERCENT 1.4 % 1.2 % 1.1 % 1.0 % 0.7 % 0.7 %
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Increased Risk of CHD Chromosomal abnormalities Congenital anomalies, especially GI Congenital Infections, esp. Influenza, Rubella Medications like benzo’s, ibuprofen, macrodantin, corticosteroids, dilantin Drugs of abuse -alcohol, marijuana, cocaine Chemicals esp. hair dyes and pesticides Maternal conditions like diabetes, PKU and SLE
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Recurrence risk if parents have CHD Sibling has CHD, next child's risk doubles to 2% Father has CHD risk increases to 3-4% Mother with Coarctation, PDA, or Tetralogy of Fallot risk is 4 to 6X higher Mother with AS, ASD, AV canal, PS or VSD risk rises to 14%
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10 ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease Developed in Collaboration With: The American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons ACHD Guidelines Slide Set
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11 Applying Classification of Recommendations and Level of Evidence Alternative Phrasing:
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12 Applying Classification of Recommendations and Level of Evidence Level of Evidence:
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Common CHD Seen in Adult Patients Repaired conditions: ASD, VSD with no residual, PDA long term complications unlikely Unrepaired conditions: ASD, small VSD, PDA, mild PS, AV disease 13
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Ventricular Septal Defect Operated (5%)– surgery under age 2 yrs, good prognosis, even for large VSD’s Later surgery risk for continued pulmonary hypertension if present preop. Conduction defects, arrhythmia and occasional sudden unexpected death 14
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Isolated Small VSD Long term prognosis good with late spontaneous closure possible in 50% Look for aortic valve insufficiency resulting from location of VSD. This may require surgery. VSD associated with cardiac enlargement should be further evaluated for surgical closure. 15
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16 Surgical Ventricular Septal Defect Closure Surgeons with training and expertise in CHD should perform VSD closure operations. Closure of a VSD is indicated when there is a Qp/Qs (pulmonary-to-systemic blood flow ratio) of 2.0 or more and clinical evidence of LV volume overload. Closure of a VSD is indicated when the patient has a history of IE. VSD Closure Operations
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17 Surgical Ventricular Septal Defect Closure Closure of a VSD is reasonable when net left-to-right shunting is present at a Qp/Qs greater than 1.5 with pulmonary artery pressure less than two thirds of systemic pressure and pulse volume recording (PVR) less than two thirds of systemic vascular resistance. Closure of a VSD is reasonable when net left-to-right shunting is present at a Qp/Qs greater than 1.5 in the presence of LV systolic or diastolic failure. VSD closure is not recommended in patients with severe irreversible PAH. VSD Closure Operations
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18 Device closure of a muscular VSD may be considered, especially if the VSD is remote from the tricuspid valve and the aorta, if the VSD is associated with severe left-sided heart chamber enlargement, or if there is PAH. Interventional Catheterization for VSD
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Atrial Septal Defects(ASD)- Clinical Majority repaired in childhood, but may present in adolescence/adulthood Asymptomatic murmur, abnl ECG/CXR Symptomatic dyspnea/CHF CVA/emboli Atrial Fibrillation
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ASD- Anatomy/Prevalence Secundum 75% Primum 15% Sinus Venosus 10% Cor Sinus (rare) Braunwauld’s Heart Disease, 6th ed
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Auscultation in ASD Increased flow across the pulmonary valve produces a systolic ejection murmur and fixed splitting of the second heart sound. Fixed splitting of S2 may in part be due to delayed right bundle conduction. Increased flow across the TV produces a diastolic rumble at the mid to lower right sternal border. Older pt loses pulm ejection murmur as shunt becomes bidirectional signs of pulm HTN/ CHF may predominate
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ASD: Therapy Percutaneous Closure only for secundum (contra in others) adequate superior/inferior rim around ASD no R-L shunting Surgical Closure Good prognosis: closure age < 25, PA pressure <40 If >25 or PA>40, decreased survival due to CHF, stroke, and afib
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Atrial Septal Defect Operated Surgery in childhood – excellent prognosis Surgery 25-40 – good, but arrhythmia frequent Surgery >40 – not as good, but better than medical management 23
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Atrial Septal Defect Unoperated Survival to adulthood likely After 40-50 few remain asymptomatic because Increased BP and ischemic heart disease increases shunt Atrial tachyarrhythmia's, fib or flutter Mild to moderate pulmonary hypertension 24
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Atrial Septal Defect If right sided dilatation present should be closed unless pulmonary vascular disease present Need follow up post repair to follow for arrhythmias Today many closed at cath with a device 25
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26 Atrial Septal Defect Closures Closure of an ASD either percutaneously or surgically is indicated for right atrial and RV enlargement with or without symptoms. A sinus venosus, coronary sinus, or primum ASD should be repaired surgically rather than by percutaneous closure. Surgeons with training and expertise in CHD should perform operations for various ASD closures.
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27 Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not undergo ASD closure. Atrial Septal Defect Closures
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Patent Ductus Arteriosus PDA diagnosed only by ECHO with no cardiac mumur or cardiac enlargement needs no treatment. Significant PDA in an adult carries a higher risk at surgery compared to children. PDA can be closed with a device at cardiac cath. 28
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Eisenmenger’s Syndrome Final common pathway for all significant LR shunting in which unrestricted pulmonary blood flow leads to pulmonary vaso-occlusive disease (PVOD); RL shunting/cyanosis devleops Generally need Qp:Qs >2:1
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Eisenmenger Complications Coagulopathy/platelet consumption Brain abscesses Cerebral micro emboli Airway hemorrhage especially moving from lowerhigher altitudes (air travel, mountains)
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Eisenmenger: Treatment Sxs +polycythemia  phlebotomy Careful if microcytosis, strongest predictor of cerebrovascular events RULE OUT CORRECTABLE DISEASE Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures Diuretics prn, oxygen Definitive: Heart Lung transplant Prostacyclin therapy may delay, expensive
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Pulmonary Valve Stenosis Mild PS with normal EKG is a benign condition If significant pulmonary stenosis was not treated in childhood pulmonary balloon valvuloplasty should be considered. 34
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Common Congenital Heart Defects “Repaired” in childhood requiring life long follow-up. Coarctation of the aorta Aortic stenosis Tetralogy of Fallot D-Transposition 35
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Coarctation of the Aorta All patients require lifelong follow-up Systemic hypertension common May occur with no residual coarctation May be due to re-coarctation Need to get records to learn type of repair End to end anastomosis Subclavian flap with arch augmentation using left SCA. Left arm pressure may be unreliable Coarctation repaired using a graft. Stenting of coarc at cath Increased incidence of coronary heart disease, stroke, aneurysm formation and associated Bi AV. 36
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Coarctation of Aorta Narrowing in proximal descending aorta May be long/tubular but most commonly discrete ridge Natural hx: poor prognosis if unrepaired Aortic Aneurysm/dissection CHF Premature CADz
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Coarctation of Aorta: Clinical Most repaired, but adult presentation may be: HTN murmur (continuous or systolic murmur heard in back or SEM/ejection click of bicuspid AV) weak/delayed LE pulses Rib notching on CXR pathognomonic
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Rib notching
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Coarctation Repair Edmunds’ Cardiac Surgery in the Adult, Ch 47 Surgical correction 1) Patch aortoplasty with removal of segment and end to end anastomosis or subclavian flap repair 2) bypass tube grafting around segment
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Coarcation: Treatment Despite surgery, patients still have significant morbidity/mortality with average age 38 Up to 70% of repaired patients still go on to develop HTN, pathology not well understood Recurrence in 8-54% of repairs, can undergo repeat surgery or balloon angioplasty Aortic Aneurysm/rupture may occur despite successful repair and correction of HTN (freq around anastomosis site on patch repair – 30% in one study)
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Coarctation: Follow-up Every 1-2 years Document arm/leg BP Screen/treat CAD risk factors HTN: rest, provoked by exercise or seen on ambulatory monitoring ECHO/Doppler to eval recurrent MRI for aneurysm
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Aortic Valve Disease Bicuspid AV often unrecognized in childhood ejection click helpful sign All patients with Bi AV require periodic follow-up Patients frequently develop AS and/or AI overtime Aortic aneurysm a known risk If mild AS diagnosed in childhood lifelong follow-up needed Mild subaortic stenosis – look for aortic valve insufficiency. 44
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Aortic Valve Stenosis, SAS, SVAS Unoperated – Tendency for gradient to increase and for AI to develop, endocarditis a risk. Sudden death a risk if severe Operated – Surgery must be considered palliative. Obstruction may recur, regurgitation may progress and if valve replaced risk of thromboemboli, valve failure, complication of anticoagulant therapy. Sudden death a risk. 45
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Aortic Stenosis – “Repaired” AV valvotomy often requires further surgery – restenosis and/or aortic valve insufficiency. Aortic Valve replacement – mechanical valve – lifelong anticoagulation. Homograft – will usually need to be replaced Ross procedure – pulmonary homograft may need replacement, aortic valve insufficiency may develop. 46
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Tetralogy of Fallot 4 features Malalignment VSD Overriding Aorta Pulmonic Stenosis RVH Variability correlates with degree of RVOT obstruction and size/anatomy of PA
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Tetralogy: Surgical Treatment Systemic – Pulmonary Shunt Blalock-Taussig-Thomas Shunt- Left Subclavian to Pulmonary Artery Waterston –Ascending aorta to Right PA Potts-Descending Aorta to Left PA Complete Repair takedown of prior shunt patch VSD resection of subpulmonic obstruction transannular patch around pulm valve annulus (usually leads to severe PI)
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Modified Blalock Taussig Thomas Shunt
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Other Shunts to the Lung-Glenn
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Tetralogy: Treatment/complications Systemic-Pulm shunt leads to high flow through PA, elevated PVR and branch PA distortion survival after repair worse in pt with prior Waterston or Potts shunt (?higher flow); some pt with Blalock-Taussig shunts may survive unrepaired into adulthood these pt should be evaluated for pulm artery stenosis and Pulm HTN
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Tetralogy: Treatment/complications Prior pulmonary valve atresia or anomalous LAD may have had prosthetic or homograft conduit ± valve placed between RV and PA Conduits can undergo endothelial overgrowth and obstruction of “pseudo RVOT” – can Rx with balloon angioplasty or operative conduit replacement
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Tetralogy: Risk/follow-up SCD ↑ 25-100 fold risk can occur 2 decades after correction related to QRS duration> 180msec ? Due to PI/RV conduction defect atrial arrhythmias also common Hemodynamic effects of PI Chronic RV volume overload, RV dysfunction and exercise intolerance Pulmonic Valve Replacement can decrease QRS duration and stabilize RV fxn; timing unclear but earlier better than later RV fxn: ECHO or MRI
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Less Expected Problems after Tet Residual VSD AI Aortic root dilatation TR RV or LV dysfunction PA HTN Dysrhythmia 55
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Residua and Sequelae After Surgery Electrophysiologic Conduction defects – CRBBB Sinoatrial dysfunction – sick sinus Tachyarrhythmias Complete heart block Ventricular ectopy 56
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Transposition D-type PA arises from LV, Aorta from RV and anterior/right of PA cyanosis corrected initially with prostaglandin to keep ductus open and balloon atrial septostomy to improve systemic saturation Old repair via “atrial switch” Mustard procedure SVC/IVC baffled to LA/LV/PA Pulm Veins baffled to RA/RV/Ao Symptom free survival until 2nd-3rd decade of life new repair via “arterial switch” long term data ? pulmonic valve (neo-aortic valve) competence?, reimplanted coronaries may develop ostial stenoses
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Uncommon Cardiac Defects Often Undiagnosed in Childhood Ebstein’s malformation “Corrected transposition of Great Arteries Coronary artery abnormalities 58
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Epstein's Anomaly Atrialization of RV, sail-like TV, TR 50% ASD/PFO 50% ECG evidence of WPW Age at presentation varies from childhoodadulthood and depends on factors such as severity of TR, Pulm Vascular resistance in newborn, and associated abnormalities such as ASD www.ucch.org
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Massive cardiomegaly, mainly due to RAE
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Epstein's: Clinical Presentation Pediatric murmur Adult (unrepaired with ASD) atrial arrhythmias murmur cyanosis RL shunt NOT due to PulmHTN but TR jet directed across ASD exercise intolerance Surgery in pts with significant TR/sxs
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Ebstein Malformation Risk of sudden death from arrhythmia Tricuspid insufficiency often progressive. 62
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L-type Transposition Atrial-ventricular AND ventricular-arterial discordance Physiologically correct, anatomically incorrect “congenitally corrected” transposition RV is systemic ventricle, TV is systemic AV valve Asymptomatic for many years, often into adulthood
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L-type transposition: complications Although seemingly benign, survival is reduced with one study showing 25% of patients died by mean age 38 Progressive Heart Failure Arrhythmias SCD AV block Atrial arrhythmias Severe AV (tricuspid) regurgitation – TVR difficult to image using conventional ECHO MRI becoming test of choice for RV function
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Coronary Artery Problems 1. Variations of normal anatomy 2. Anomalous origin of coronary artery LCA from pulmonary trunk 3. Stenosis or atresia of a coronary ostium 4. Aneurysms and fistulas of coronaries 5. Diseases of the coronary arteries A. Kawasaki’s disease B. Premature atherosclerosis C. Fibromuscular dysplasia, inflammation or infiltration 65
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Cyanotic Adolescents and Adults Palliated “shunt” e.g. pulmonary artery atresia with inadequate pulmonary arteries for “repair” Eisenmenger Physiology Unoperated large VSD, truncus, AV canal 66
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Cyanotic Heart Disease in Adults Polycythemia Compensated erythrocytosis hematocrit in iron-replete state results in appropriate physiologic adaptations to tissue hypoxia. In this stage hyperviscosity symptoms rare with hematocrit up to 65% and are mild-to-moderate up to 70% 67
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Iron—Deficient Erthropoiesis If myalgia and muscle weakness suggesting hyperviscosity symptoms occur and hematocrit below 65% -- iron deficiency likely cause of symptoms 68
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Phlebotomy Phlebotomy should not be based on hematocrit per se. Need to rule out iron deficiency as cause of hyperviscosity symptoms. Phlebotomy must be done with volume repletion. 69
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Hemostasis Bleeding tendency frequent. May develop von Willebrand abnormality. Platelet counts usually mildly reduced, but occasionally may be severely reduced to 50,000 / mm3 or less Bleeding gums, nose bleeds, easy bruising and hemoptysis frequent Avoid aspirin and other anticoagulants 70
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Complex Cardiac Defects Truncus Arteriosus Tricuspid Atresia HLHS 71
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Truncus Arteriosus Reoperation for pulmonary outflow likely 72
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Tricuspid Atresia Some adults will have had a “shunt” to increase pulmonary blood flow and will still be cyanotic. Today most will undergo a Fontan operation. 73
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The Fontan Patient Any congenital anomaly with an effective “single” or “common” ventricle may lead to a Fontan procedure Tricuspid Atresia – also any other form of right sided hypoplasia or atresia. Double Inlet LV. Hypoplastic Left Heart. Some variations of Double Outlet RV Staged Procedure Basic concept is to provide systemic venous return directly to PA and bypass ventricle systemic-pulm shunt to stabilize pulm blood flow bi-directional Glenn or hemi-Fontan procedure SVC flow directed to PA and sys-pulm shunt ligated Finally, Fontan procedure with IVC directed to PA Older Fontan: includes RA in circuit; newer methods bypass RA
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www.heartcenteronline.com
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Fontan: complications Arrhythmias most pt develop SSS/tachy-brady Heart Failure RA may become enlarged and source for thrombus ( with older Fontan), can undergo Fontan revision with bypass of RA/extracardiac graft Uncorrected patients develop polycythemia and treatment becomes palliative at this point Protein losing enteropathy
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Hypoplastic Left Heart (HLHS) Need a triple jump to fix this problem *Norwood procedure *Bidirectional Glenn-after the PA pressure falls *Fontan Procedure 78
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Norwood Procedure This procedure is performed shortly after birth. It converts the right ventricle into the main ventricle pumping blood to both the lungs and the body. The main pulmonary artery and the aorta are connected and the main pulmonary artery is cut off from the two branching pulmonary arteries that direct blood to each side of the lungs. Instead, a modified BTT shunt is placed between the pulmonary arteries and the aorta to supply blood to the lungs
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Norwood Procedure for HLHS
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Bi-directional Glenn Operation This operation usually is performed about six months after the Norwood to divert half of the blood to the lungs when circulation through the lungs no longer needs as much pressure from the ventricle. The shunt to the pulmonary arteries is disconnected and the right pulmonary artery is connected directly to the superior vena cava, the vein that brings deoxygenated blood from the upper part of the body to the heart. This sends half of the deoxygenated blood directly to the lungs without going through the ventricle.
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Fontan Operation This is the third stage, usually performed about 18 to 36 months after the Glenn. It connects the inferior vena cava, the blood vessel that drains deoxygenated blood from the lower part of the body into the heart, to the pulmonary artery by creating a channel through or just outside the heart to direct blood to the pulmonary artery. At this stage, all deoxygenated blood flows passively through the lungs
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83 Infective Endocarditis ACHD patients must be informed of their potential risk for IE and should be provided with the AHA information card with instructions for prophylaxis. When patients with ACHD present with an unexplained febrile illness and potential IE, blood cultures should be drawn before antibiotic treatment is initiated to avoid delay in diagnosis due to “culture-negative” IE. Transthoracic echocardiography (TTE) should be performed when the diagnosis of native-valve IE is suspected. Notification of Potential Risk for IE
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84 Transesophageal echocardiography (TEE) is indicated if TTE windows are inadequate or equivocal, in the presence of a prosthetic valve or material or surgically constructed shunt, in the presence of complex congenital cardiovascular anatomy, or to define possible complications of endocarditis (e.g., sepsis, abscess, valvular destruction or dehiscence, embolism, or hemodynamic instability). ACHD patients with evidence of IE should have early consultation with a surgeon with experience in ACHD because of the potential for rapid deterioration and concern about possible infection of prosthetic material. Patients with Evidence of IE Infective Endocarditis
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85 Antibiotic prophylaxis before dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa is reasonable in patients with CHD with the highest risk for adverse outcome from IE, including those with the following indications: a) Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. b) Previous IE. c) Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. Dental Procedures and Patients with CHD Infective Endocarditis
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86 d) Completely repaired CHD with prosthetic materials, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure. e) Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device that inhibit endothelialization. Dental Procedures and Patients with CHD Infective Endocarditis
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87 It is reasonable to consider antibiotic prophylaxis against IE before vaginal delivery at the time of membrane rupture in select patients with the highest risk of adverse outcomes. This includes patients with the following indications: a) Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. b) Unrepaired and palliated cyanotic CHD, and including surgically constructed palliative shunts conduits. Antibiotic Prophylaxis and Vaginal Delivery Infective Endocarditis
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88 Prophylaxis against IE is not recommended for non dental procedures (such as esophagogastroduodenoscopy or colonoscopy) in the absence of active infection. Antibiotic Prophylaxis and Vaginal Delivery Infective Endocarditis
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Cardiac Conditions Associated with the Highest Risk of Adverse Outcome from Endocarditis for Which prophylaxis with Dental Procedures is Recommended Prosthetic cardiac valve Previous IE Congenital heart disease (CHD)* Unrepaired cyanotic CHD, including palliative shunts and conduits Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization) Cardiac transplantation recipients who develop cardiac valvulopathy 89 * Except for the conditions listed above, antibiotic prophylaxis is no longer recommended by any other form of CHD. +Prophylaxis is recommended because endothelialization of prosthetic material occurs within 6 months after the procedure.
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90 General Health Issues for Cyanotic Patients Cyanotic patients should drink nonalcoholic and noncaffeinated fluids frequently on long-distance flights to avoid dehydration. Supplemental oxygenation may be considered for cyanotic patients during long-distance flights. Cyanotic Patients and Long Distance Flights
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91 Pregnancy and Contraception Patients with CHD should have consultation with an ACHD expert before they plan to become pregnant to develop a plan for management of labor and the postpartum period that includes consideration of the appropriate response to potential complications. This care plan should be made available to all providers. Patients with intracardiac right-to-left shunting should have fastidious care taken of intravenous lines to avoid paradoxical air embolus. Consultation and Assessment of Risk
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92 Pregnancy and Contraception Prepregnancy counseling is recommended for women receiving chronic anticoagulation with warfarin to enable them to make an informed decision about maternal and fetal risks. Consultation and Assessment of Risk
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93 Meticulous prophylaxis for deep venous thrombosis, including early ambulation and compression stockings, can be useful for all patients with intracardiac right-to-left shunt. Subcutaneous heparin or low-molecular-weight heparin is reasonable for prolonged bed rest. Full anticoagulation can be useful for the high-risk patient. The estrogen-containing oral contraceptive pill is not recommended for ACHD patients at risk of thromboembolism, such as those with cyanosis related to an intracardiac shunt, severe PAH, or Fontan repair. Full Anticoagulation Estrogen and Oral Contraceptives Pregnancy and Contraception
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94 Top 10 Points to Remember from ACHD Guidelines 1. The care of ACHD patients should be coordinated by regional ACHD centers of excellence, providing education, patient care and follow-up by specialists for affected individuals. 2. Individual and family psychosocial screening and information should be included in the care of ACHD patients. 3. Thorough clinical evaluation should be undertaken before anticipated non-cardiac surgery, including ECG, chest x-ray, TTE, and blood tests. 4. Counseling before pregnancy is important to ensure that patients understand the risks to both mother and baby, including the risks of transmitting coronary heart disease to their offspring. Any non-cardiac surgery in cyanotic patients, however minor, is potentially life-threatening and should be coordinated with regional centers of excellence. 5. Pacemaker implantation can be beneficial in ACHD patients, but access may be challenging and implantation should be performed at centers where staff is familiar with unusual anatomy of congenital heart defects.
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95 Top 10 Points to Remember on ACHD Guidelines 6. Closure of a secundum atrial septal defect, either percutaneously or surgically, is indicated for right atrial and right ventricular enlargement with or without symptoms. 7. Primary imaging and hemodynamic assessment of aortic stenosis and aortic valve disease are recommended by echocardiography-Doppler to evaluate aortic stenosis or aortic regurgitation, left ventricular size, function and mass, and dimensions and anatomy of the ascending aorta and associated lesions. 8. Lifelong cardiology follow-up is recommended for all patients with aortic valve disease. 9. All patients with tetralogy of Fallot should have regular follow-up with a cardiologist with expertise in ACHD, the frequency of which may be determined by the extent and degree of residual abnormalities. 10. There is a lack of scientific evidence demonstrating proven benefit for IE prophylaxis, although prophylaxis is reasonable in patients with CHD with the highest risk for adverse outcome from infective endocarditis.
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References ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease. Published online Nov 7, 2008; J Am Coll Cardiol at Onlinejacc.org. Prevention of Infective Endocarditis. Guidelines from the American Heart Association. Published online April 19, 2007; Circulation at Circ.ahajournals.org. 96