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Congenital Heart Disease Richard A. Krasuski, M.D. Director of Adult Congenital Heart Disease Services The Cleveland Clinic Current Landscape of CHD • 0.8% of live births in the U.S. excluding bicuspid AV, MVP –Improved diagnostic techniques –Improved medical, catheter-based and surgical techniques • Now >800,000 adults with congenital heart disease (adults>children) • Minimal exposure and training for medical residents Current Landscape of CHD • Can separate into 2 general groups –Patients recognized and rx as children –Adults diagnosed de novo • Due to complexity adults with complex CHD best followed by subspecialists • Nearly all ACHD patients followed by generalists +/- general cardiologists General Approach to Adults with CHD • Understand the anatomy and surgeries –Review the pediatric records • Be aware of warning signs and sxs –Syncope –Progressive exertional dyspnea –Increasing palpitations General Approach to Adults with CHD • Perform careful exam with auscultation –Look at JVP, feel pulses in all extremities –Listen for “hard to hear” murmurs • Utilize noninvasive diagnostic modalities –ECG –Echocardiogram –CT and MRI Case # 1 • 53 year old woman with no previous cardiac history who presents with progressive fatigue • ROS is notable for exertional dyspnea climbing 1-2 flights of stairs due to “aging” Case # 1 • Examination is notable for a split second heart sound unchanged with respiration and a soft SEM • Exercise stress test shows decreased exercise capacity but no ECG changes • Echocardiogram is perfomed Transthoracic Echocardiogram The most likely diagnosis in this patients is: A. B. C. D. E. Patent Foramen Ovale Atrial Septal Defect Tetralogy of Fallot Ventricular Septal Defect Ebstein anomaly The most likely diagnosis in this patients is: A. B. C. D. E. Patent Foramen Ovale Atrial Septal Defect Tetralogy of Fallot Ventricular Septal Defect Ebstein anomaly Atrial Septal Defects • Most common cardiac malformation in adults • More common in females 2-3:1 • 75% are secundum defects • Symptoms can be very subtle – Dyspnea and fatigue most common • Commonly mistaken for other disorders Types of Atrial Septal Defects Secundum ASD • Rule of 10% – 10% with multiple defects – 10% with anomalous veins – 10% unrepaired can develop Eisenmenger’s • Shunt determined by size of defect and compliance of ventricles • Decompensation can occur in older patients – LV diastolic dysfunction (HTN, CAD) – Atrial fibrillation – Development of pulmonary HTN Shunt Lesions • Most common form of ACHD • Frequently diagnosed in the adult population • Results in increased pulmonary blood flow –Pulmonary hypertension –Right heart enlargement –Arrhythmias → atrial fibrillation The Process of Atrial Septation Variations in Septal Anatomy Adapted from Krasuski RA, Bashore TS. Rev. in Cardiovasc. Med. 2005 Atrial Septal Defect vs PFO • ASD – Incidence ~1/1000 – Usually L to R shunt – Also has R to L shunt • PFO – Incidence ~1/4 – Usually only R to L shunt – “Stretched PFO” can result in L to R shunt – Association with stroke – Can be concurrent with ASA – Results in “flow” complications – Right heart enlargement – Pulmonary HTN – Atrial fibrillation – Association with stroke – Can be concurrent with ASA – No “flow” complications What is a significant ASD? • Qp/Qs > 1.5 • RA+RV Enlargement • ~ Normal PVR (<7-10 Wood units or PVR/SVR<0.3) • Anatomy conducive to percutaneous repair Therapeutic Approach to ASD • Medical – ??? • Surgical repair – 0-1.2% mortality – Significant morbidity, discomfort, scar • Percutaneous – New gold standard for simple, significant secundum defects “Medical Therapy” vs. Surgical Correction Konstantinides S. et al. NEJM 1995;333(8):469-73. Atrial Fibrillation as Source of Morbidity Following Surgical Repair Gatzoulis M.A. et al. NEJM 1999;340(11):839-46. ASD Occluders ASDOS Sideris Button Amplatzer CardioSeal Angel Wing Helex Guardian Angel StarFlex Atrial Septal Defect Closures I IIa IIb III I IIa IIb III I IIa IIb III 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. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease Case # 2 • 21 year old male college student referred after visit to the infirmary with upper respiratory symptoms • No prior medical problems or symptoms. Heart murmur since birth • On examination he has BP 124/70, normal S1 and normally splitting S2 and a IV/VI pansystolic murmur Case # 2 • He is referred for an echocardiogram which shows –Perimembranous VSD with 5 m/sec velocity (100 mm Hg gradient) across the lesion –Normal right and left ventricular function –No other associated lesions The most reasonable management in this patient is: A. B. C. D. E. Referral for surgical evaluation Referral for heart catheterization Referral for device closure Reassurance Reassurance and antibiotic prophylaxis The most reasonable management in this patient is: A. B. C. D. E. Referral for surgical evaluation Referral for heart catheterization Referral for device closure Reassurance Reassurance and antibiotic prophylaxis Ventricular Septal Defects (VSD) • Most common congenital lesion seen in children (~25%) • Less common in adults (2nd after ASD) – Smaller lesions often close spontaneously – Larger lesions present with heart failure and get repaired • Many different types – Membranous – Muscular − Inlet − Outlet Locations of VSDs 1. Membranous VSD - (80%) 2. Muscular VSD - (10% of VSD) often multiple defects 3. Supracristal VSD - (5%) involve LVOT/RVOT 4. AV canal defects - involve inflow portion of the septum General VSD Facts • Membranous often have associated “aneurysmal” tissue • Small (<0.5 cm) = restrictive – Loud murmurs – Clinically benign • Larger lesions – Softer Murmurs – LV volume overload and pulmonary hypertension – Most common cause of Eisenmenger syndrome Membranous VSD Muscular VSD Supracrystal VSD Case # 3 • 48 year old woman with a history of a “hole in my heart” • Was followed by cardiologists as a child but hasn’t seen one in 35 yrs • Tells you she’s been short of breath and very limited since childhood Case # 3 • Examination is notable for –BP 95/60 –cyanotic lips and clubbing of the fingers and toes –low pitched systolic murmur at the LLSB which increases on inspiration • Echocardiogram shows an enlarged right ventricle with estimated RV systolic pressure of 100 mm Hg Case # 3 • Blood work shows a hemoglobin of 21 with a HCT of 63 • BUN of 28 and Cr of 1.0 • Otherwise normal electrolytes • An echocardiogram was performed Case # 3 Echocardiogram The most reasonable next step would be: A. Referral to a cardiologist for right heart catheterization and possible initiation of vasodilator therapy B. Phlebotomize 2 units C. Phlebotomize 2 units with equal replacment of normal saline D. CT scanning to rule out pulmonary embolism E. Referral to hospice care The most reasonable next step would be: A. Referral to a cardiologist for right heart catheterization and possible initiation of vasodilator therapy B. Phlebotomize 2 units C. Phlebotomize 2 units with equal replacment of normal saline D. CT scanning to rule out pulmonary embolism E. Referral to hospice care Eisenmenger Syndrome • Pulmonary vascular disease progresses to systemic pressures and shunt reverses • Multiple systemic complications (hypoxia) – Erythrocytosis (Therapeutic phlebotomy only if symptomatic) – Proteinuria and ↓ GFR – Increased Uric Acid • Patients can rapidly deteriorate – ARF from contrast dye load – Arrhythmias – Anesthetic agents • Lifespan > PPH but still significantly reduced Survival in Eisenmenger Syndrome Vs. Other Forms of Pulmonary Artery Hypertension Percent Survival 1 0.9 0.8 Congenital 0.7 Portopulmonary 0.6 0.5 Idiopathic 0.4 Connective Tissue Disease 0.3 0.2 HIV 0.1 0 0 1 2 Years 3 McLaughlin V. et al. Chest 2004 4 5 The Current Landscape of Pulmonary Hypertension in the United States COPD CHD Scleroderma Cirrhosis Sickle Cell HIV Idiopathic 14% of the PH population = CHD Which of the following statements about coarctation of the aorta is correct: A. The most common presentation in adults is endocarditis B. Only surgical methods of correction are possible in the adult C. Endocarditis prophylaxis is indicated if unrepaired D. It should be considered as a possibility in young hypertensive patients E. Most commonly associated with VSD Which of the following statements about coarctation of the aorta is correct: A. The most common presentation in adults is endocarditis B. Only surgical methods of correction are possible in the adult C. Endocarditis prophylaxis is indicated if unrepaired D. It should be considered as a possibility in young hypertensive patients E. Most commonly associated with VSD Coarctation of the Aorta Coarctation of the Aorta • Common lesion (8% of all CHD) • Likely due to extraneous ductal tissue which contracts following birth • 50-85% have associated BAV • 10% with berry aneurysms • Most common presentation in adult is during w/u for secondary HTN • RAS activation – HTN seen after repair Aortic Coarctation Aortic Coarctation Aortic Coarctation CW Doppler Coarctation Angioplasty/Stenting • Native coarctation: –Reasonable success after 1 year of age –Long term concern regrading aneurysm formation • Post-op Re-coarctation: –Good short term results –Persistent long term success Coarctation Stenting Interventional and Surgical Treatment of Coarctation of the Aorta in Adults Intervention and Peak-to-Peak Coarctation Gradient I IIa IIb III I IIa IIb III Intervention for coarctation is recommended in the following circumstances: a) Peak-to-peak coarctation gradient greater than or equal to 20 mm Hg. b) Peak-to-peak coarctation gradient less than 20 mm Hg in the presence of anatomic imaging evidence of significant coarctation with radiological evidence of significant collateral flow. Choice of percutaneous catheter intervention versus surgical repair of native discrete coarctation should be determined by consultation with a team of ACHD cardiologists, interventionalists, and surgeons at an ACHD center. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease Which of the following statements about transposition of the great vessels is correct: A. The presence of a shunt in D-transposition is essential to survival during infancy B. The atrial switch (Mustard/Senning) procedure can eventually be complicated by heart failure C. The atrial switch (Mustard/Senning) procedure can eventually be complicated by valvular regurgitation D. Heart block can occur in “congenitally corrected” transposition E. All are correct Which of the following statements about transposition of the great vessels is correct: A. The presence of a shunt in D-transposition is essential to survival during infancy B. The atrial switch (Mustard/Senning) procedure can eventually be complicated by heart failure C. The atrial switch (Mustard/Senning) procedure can eventually be complicated by valvular regurgitation D. Heart block can occur in “congenitally corrected” transposition E. All are correct Transposition of the Great Arteries (D-TGA) Ventriculoarterial Discordance LA RA Ao PA Atrio-ventricular Concordance LV RV Echo of D-TGA Echo of D-TGA Mustard Operation for D-TGA SVC LA RA IVC Arterial Switch Operation for TGA LV RV Congenitally-corrected Transposition of the Great Arteries (L-TGA) Ventriculoarterial Discordance Ao PA LA RA Atrio-ventricular Discordance RV LV Echo of L-TGA Echo of L-TGA Problems with “Corrections” • Anatomic RV in LV position –Systemic AV valve regurgitation –Systemic ventricular dilatation • Complete heart block –32% lifetime prevalence –~2%/year incidence in adults Which of the following components does not comprise tetralogy of Fallot: A.Ventricular septal defect B.Atrial septal defect C.Overriding aorta D.Pulmonary outflow obstruction E.Right ventricular hypertrophy Which of the following components does not comprise tetralogy of Fallot: A.Ventricular septal defect B.Atrial septal defect C.Overriding aorta D.Pulmonary outflow obstruction E.Right ventricular hypertrophy Tetralogy of Fallot Ao LA RA LV RV Tetralogy of Fallot Tetralogy of Fallot Classic Repair of Tetralogy of Fallot AO PA TRANSANNULAR PATCH LV RV Tetralogy of Fallot • Surgically repaired adults usually do well for 2-3 decades, then have consequences due to PI – Right heart failure – Arrhythmias • When to consider pulmonic valve replacement – Progressive decline in exercise tolerance – Progressive increase in indexed RVESV, RVEDV – Severe decrement in RV function – Severe widening in QRS (>180 msec) Tetralogy Repair and It’s Residual Echo of the Repaired Tetralogy Patient Echo of the Repaired Tetralogy Patient RV Outflow Tract Tachycardia After Tetralogy of Fallot Repair Summary • Adult CHD is generally more common than realized • Due to a shortage of CHD specialists, general cardiologists/primary M.D.s will continue as major caretakers • Thorough history and review of pediatric records is essential in initial evaluation • Noninvasive imaging, particularly echo should be first-line in evaluation and is useful for serial follow-up