Hypoplastic Left Heart Syndrome: Echocardiographic Assessment

Transcription

1 Hypoplastic Left Heart Syndrome: Echocardiographic Assessment Craig E Fleishman, MD, FACC, FASE Director, Non-invasive Cardiac Imaging The Hear Center at Arnold Palmer Hospital for Children, Orlando SCAI Fall Fellows Course 2015 Las Vegas

2 Disclosure Information HLHS: Echocardiographic Assessment Craig E Fleishman MD, FACC, FASE As a faculty member for this program, I disclose the following relationships with industry: (GRS): Grant/Research Support (C): Consultant (SB): Speaker s Bureau (MSH): Major Stock Holder (AB): Advisory Board (E): Employment (O):Other Financial or Material Support W.L. Gore Medical: C, Echo Core Lab

3 HLHS HLHS: Echo Assessment SCAI 2015

4 HLHS Spectrum of Malformations 7-9% CHD diagnosed in infancy Underdevelopment of left heart Left atrium Mitral Valve Left Ventricle Aortic Valve Aortic Arch

5 Left Atrium Hypoplastic Thick walled Variable size of ASD Some restriction helpful Intact atrial septum Look for levoatrial cardinal vein Malalignment of atrial septum Leftward and posterior attachment to LA free wall Small, superior, posterior PFO

6 Mitral Valve Stenotic, hypoplastic, or atretic MV annulus diameter 6 mm May not have well defined chordae (arcade) Papillary muscles Closely spaced Parachute

7 LV Variable hypoplasia Does not extend to cardiac apex LV area < 1.5 cm 2 (PLAX) Smallest with MA/AA Larger with VSD Hypertrophy Echo bright areas reflect endocardial changes Endocardial fibroelastosis

8 Aortic Valve and Aorta Aortic atresia (plate) or dysplastic Larger with VSD Hypoplastic ascending aorta <5 mm, usually smaller Main coronary artery Fills retrograde from ductus arteriosus Aorta becomes larger at 1 st arch vessel, but not normal until distal to PDA Aortic arch branching pattern Juxtaductal posterior shelf/coarctation

9 PDA PDA usually large, with aorta inserting onto ductal arch Right to left flow in systole Left to right in diastole Diastolic flow reversal in abdominal Ao L to R decreased with Pulm HTN PPHTN Intact atrial septum

10 Coronary Arteries Anomalous origins are rare Abnormalities more common with MS/AA Single coronary Tortuosity Sinusoids/fistulae between coronaries and LV? left ventricular dependent coronary circulation

11 Other areas of interest Pulmonary stenosis or regurgitation Right ventricular function Tricuspid regurgitation Left SVC Abnormal pulmonary venous connections (6%)

12 HLHS Intact Atrial Septum 18/316 infants over 6.5 yr period Atrial septal morphology evaluated Lung histopathology reviewed

13 Type A atrial morphology HLHS: Echo Assessment SCAI /18 infants Relatively large LA Thick septum secundum Thin septum primum 5/12 with leftward and posterior deviated atrial septum All had LA decompression pathway, 3 severely obstructed Rychik, J. et al. J Am Coll Cardiol 1999;34: Copyright 1999 American College of Cardiology Foundation. Restrictions may apply.

14 Type B atrial morphology HLHS: Echo Assessment SCAI /18 infants Small, muscular LA Circumferential thickening of atrial walls Thick atrial septum w/o distinction between septum primum and secundum 4/4 with absence or severe obstruction of LA decompression pathway Rychik, J. et al. J Am Coll Cardiol 1999;34: Copyright 1999 American College of Cardiology Foundation. Restrictions may apply.

15 Fetal echocardiographic image of type C atrial morphology HLHS: Echo Assessment SCAI /18 Giant LA Thin, rightward bulging atrial septum Severe MR No LA decompression pathway Rychik, J. et al. J Am Coll Cardiol 1999;34: Copyright 1999 American College of Cardiology Foundation. Restrictions may apply.

16 HLHS Intact Atrial Septum 17/18 stage I Norwood 6/17 Early survivors Type A atrial morphology Unobstructed decompression pathway 3 additional late deaths after CPB Lung pathology Severely dilated lymphatics Arterialization of the pulmonary veins Rychik, J. et al. J Am Coll Cardiol 1999;34:

17 HLHS Intact Atrial Septum Example

18 HLHS Restrictive Atrial Septum Example

19 Time Passes Stage I with atrial septectomy #1 Aortic arch stent via carotid cutdown ASD stent #1 for increased restriction Bidirectional Glenn with atrial septectomy #2 PA stent ASD stent #2 for increased restriction Presents at 20 months of age with worse cyanosis

20 HLHS Restrictive Atrial Septum Example

21 HLHS Restrictive Atrial Septum Example

22 Pre-Hybrid Screening HLHS: Echo Assessment SCAI 2015

23 PFO/ASD HLHS: Echo Assessment SCAI 2015

24 HLHS Pre-Hybrid Screening Follow path from PDA to ascending aorta Lack of alignment can signal twisting Retrograde flow obstruction despite unobstructed PDA can be worsened with PDA stent Potential areas for stenoses

25 HLHS Pre-Hybrid Screening HLHS: Echo Assessment SCAI 2015

26 Pre-Hybrid Screening Stenosis at isthmus and at ascending aorta/transverse arch transition Treated with traditional Stage I Anatomy confirmed at surgery

27 Pre-Hybrid Screening Transverse arch and ascending aorta adequate Obstruction at distal arch despite PGE and unobstructed PDA Confirmed by color flow and CW Doppler

28 Thank You HLHS: Echo Assessment SCAI 2015

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