Congenital Heart Disease Cases
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1 Congenital Heart Disease Cases Sabrina Phillips, MD FACC FASE Mayo Clinic Congenital Heart Disease Center 2013 MFMER slide-1 No Disclosures 2013 MFMER slide-2 1
2 CASE MFMER slide-3 63 year old Woman Healthy throughout life except: Rheumatic fever at age 7 Palpitations for several years 2 months ago noticed increasing shortness of breath 2013 MFMER slide-4 2
3 Recent Hospitalization Anasarca - responded to diuresis Atrial fibrillation treated with rate control and anti-coagulation 2013 MFMER slide-5 Physical Examination Neck: JVP is moderately elevated with prominent V wave Heart: 2+ RV; Normal LV impulse Irreg RR; soft S 1 ; single S 2 ; S 3 present III/VI systolic murmur I/IV diastolic murmur at L sternal border Abdomen: Liver is pulsatile 4cm below costal margin Ext: Trace edema 2013 MFMER slide-6 3
4 Medications Aspirin 81 mg QD Furosemide 40 mg QD Lanoxin 250 mcg tablet QD Metoclopramide 10 mg TID Metoprolol Tartrate 50 mg BID Potassium Chloride 20 meq QD Warfarin 3 mg QD 2013 MFMER slide-7 Exercise Stress Test Exercise Time: 3.8 minutes FAC: 48% Peak VO 2 = 13.4 ml/kg/min (59%) 2013 MFMER slide-8 4
5 ECG 2013 MFMER slide-9 Chest X-Ray 2013 MFMER slide-10 5
6 2013 MFMER slide MFMER slide-12 6
7 2013 MFMER slide MFMER slide-14 7
8 2013 MFMER slide-15 RVSP = 29 mmhg 2013 MFMER slide-16 8
9 2013 MFMER slide MFMER slide-18 9
10 2013 MFMER slide MFMER slide-20 10
11 2013 MFMER slide MFMER slide-22 11
12 2013 MFMER slide-23 Diagnosis? A. Ebstein anomaly B. Membranous VSD C. Gerbode defect D. Coronary artery fistula 2013 MFMER slide-24 12
13 Diagnosis? A. Ebstein anomaly B. Membranous VSD C. Gerbode defect D. Coronary artery fistula 2013 MFMER slide-25 Echo Results Enlarged left coronary system Circumflex to RV fistula Flail Tricuspid valve leaflet with severe TR Severe RV enlargement with mildly decreased function; RVSP = 29 mmhg Severe RA enlargement LV EF = 58% 2013 MFMER slide-26 13
14 Catheterization 2013 MFMER slide MFMER slide-28 14
15 2013 MFMER slide-29 What Next? A. Catheter intervention B. Surgical consultation C. More testing 2013 MFMER slide-30 15
16 Surgical Intervention 2013 MFMER slide-31 Surgery Repair of coronary artery fistula with 2 layer primary closure in the RV 31-mm Epic porcine tricuspid valve replacement PFO closure Radiofrequency maze procedure Uncomplicated hospital course 2013 MFMER slide-32 16
17 Case MFMER slide Year old Woman Diagnosed with tetralogy of Fallot in the neonatal period Operative repair at age 2 Sporadic follow up after age 12 Presented with progressive decline in stamina Elsewhere underwent mitral valve replacement with a tissue prosthesis for mitral valve prolapse and regurgitation Presents 4 weeks after surgery with intractable pleural effusions, fatigue and high grade AV block 2013 MFMER slide-34 17
18 Echocardiogram 2013 MFMER slide MFMER slide-36 18
19 2013 MFMER slide MFMER slide-38 19
20 2013 MFMER slide MFMER slide-40 20
21 2013 MFMER slide MFMER slide-42 21
22 2013 MFMER slide MFMER slide-44 22
23 2013 MFMER slide MFMER slide-46 23
24 2013 MFMER slide MFMER slide-48 24
25 2013 MFMER slide-49 Diagnosis? A. Constriction B. Severe tricuspid valve regurgitation C. Severe pulmonary valve regurgitation D. Pulmonary hypertension 2013 MFMER slide-50 25
26 Diagnosis? A. Constriction B. Severe tricuspid valve regurgitation C. Severe pulmonary valve regurgitation D. Pulmonary hypertension 2013 MFMER slide-51 ECHO Report Severe RV enlargement, moderate-severe decrease in function, RVSP 39 mmhg. Severe (free) pulmonary regurgitation LV EF 30% - 35% Abnormal hepatic vein Doppler related to junction rhythm Normal mitral tissue prosthesis Patient medically optimized then referred for PVR 2013 MFMER slide-52 26
27 Post-Operative Hemodynamic Concerns Pulmonary valve regurgitation Residual/recurrent RVOT obstruction Residual VSD Tricuspid valve regurgitation Aortic root enlargement +/- aortic valve regurgitation 2013 MFMER slide-53 Consequences of Pulmonary Valve Regurgitation Exercise intolerance Right ventricular dilatation Right ventricular dysfunction Increased risk of ventricular tachycardia Increased risk of atrial arrhythmia Left ventricular dysfunction 2013 MFMER slide-54 27
28 Evolution of PVR Timing Pulmonary Valve Not Important Pulmonary valve should be replaced for right heart failure symptoms Pulmonary valve should be replaced to prevent right ventricular dysfunction 2013 MFMER slide MFMER slide-56 28
29 Case MFMER slide year old Denies complaints Echo obtained after murmur was heard on exam for assessment to be kidney donor 2013 MFMER slide-58 29
30 24 year Old : Wants to be kidney donor 2013 MFMER slide MFMER slide-60 30
31 2013 MFMER slide-61 Diagnosis? A. Parachute mitral valve B. Cleft mitral valve C. Supravalvular mitral ring D. Rheumatic mitral stenosis 2013 MFMER slide-62 31
32 Diagnosis? A. Parachute mitral valve B. Cleft mitral valve C. Supravalvular mitral ring D. Rheumatic mitral stenosis 2013 MFMER slide-63 Parachute Mitral Valve Abnormal compaction of ventricular trabecular myocardium and abnormal delamination of the trabecular ridge Unifocal attachment of the mitral valve cordae to a single/fused papillary muscle Papillary muscle usually centrally placed Often associated with other left heart abnormalities Mitral stenosis most common hemodynamic consequence 2013 MFMER slide-64 32
33 Mitral Ring Supramitral ring: fibrous membrane just above the mitral annulus. Does not adhere to the leaflets. Subvalvular apparatus normal Intramitral ring: thin membrane withni the funnel created by the valve leaflets. Always combined with an abnormal subvalvular apparatus Results in stenosis 2013 MFMER slide-65 Supramitral Ring 2013 MFMER slide-66 33
34 2013 MFMER slide MFMER slide-68 34
35 2013 MFMER slide MFMER slide-70 35
36 2013 MFMER slide-71 Cor Triatriatum Failure of proper embryologic development of the common pulmonary vein Mitral valve structure usually normal Fibromuscular membrane proximal to the left atrial appendage that divides the atrium into two parts 2013 MFMER slide-72 36
37 2013 MFMER slide MFMER slide-74 37
38 2013 MFMER slide MFMER slide-76 38
39 2013 MFMER slide MFMER slide-78 39
40 4/12/ MFMER slide-79 Supramitral Ring Cor Triatriatum 2013 MFMER slide-80 40
41 Mitral Arcade Caused by an arrest in the developmental stage of the mitral valve before attenuation and lengthening of the chordae Chords are thickened, very short, or absent Fibrous bridge may join the two papillary muscles Results in both stenosis and regurgitation 2013 MFMER slide MFMER slide-82 41
42 2013 MFMER slide MFMER slide-84 42
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