How To Treat Hypoplastic Left Heart Syndrome

Transcription

1 April 2013 Hypoplastic Left Heart Syndrome Unnati Patel, ZJU, Year 6

2 Agenda Definition Imaging Treatment

3 What is it? DEFINITION Epidemiology Signs & Symptoms

4 What is Hypoplastic Left Heart Syndrome (HLHS)? Cyanotic congenital heart syndrome in which the left ventricle is severely underdeveloped. Associated with: PDA ASD Mitral Valve Stenosis/Atresia Aortic Valve Stenosis/Atresia Ascending Aorta Hypoplasia * * * * *

5 Comparison with Normal Anatomy Courtesy of Boston Children s Hospital

6 Epidemiology 1-2% of all congenital heart diseases 26% of all CHD deaths within the first month of life M>F

7 Signs & Symptoms Heart Failure within the first few hours or days of life if left untreated SOB Poor feeding Rapid HR Shock Acidosis Cyanosis

8 EKG Right axis deviation

9 IMAGING Antenatal Ultrasound Fetal Echo Plain Film Cardiac MRI Aortogram Discussed with pre and postop images

10 Patient 1: Hypoplastic Fetal Left Ventricle on Ultrasound Four Chamber View Transabdominal US At the level of the atria and ventricles Barboza J M et al. Radiographics 2002;22:

11 Patient 1: Hypoplastic Left Heart on Fetal Color Doppler Able to better visualize flow Through the valves and the outflow tracts Assymmetry in color perfusion Courtesy

12 Advantages of Pre-natal Diagnosis Possible fetal intervention in select situations Plan for an effective delivery Prepare for immediate stabilization of the infant at time of birth

13 Our Patient: Newborn CXR At Birth Cardiomegaly Pulmonary Venous Congestion Patient #1 * * Hypoplastic Ascending Aorta Courtesy of Boston Children s Hospital

14 Our Patient: CXR 3 Months At 3 months of age Patient #1 Less Pulmonary congestion, but still shows cardiomegaly Courtesy of Boston Children s Hospital

15 Patient #2: 6 months, Post Norwood Procedure Courtesy of Boston Children s Hospital

16 Patient #2:Cardiac MRI S/P Norwood Procedure AO PA RA LA RV LV Courtesy of Boston Children s Hospital

17 Patient #3 :Cardiac MRI Axial MRI of the Heart demonstrating hypoplastic left ventricle and compensatory right ventriclular hypertrophy Kellenberger C J et al. Radiographics 2007;27:5-18

18 Patient #2: Aorta Sagittal View MRI Courtesy of Boston Children s Hospital

19 Patient #2: RVOT MRI Sagittal Patient #2 Coronal Courtesy of Boston Children s Hospital

20 Patient #3: Cardiac MRI Kellenberger C J et al. Radiographics 2007;27:5-18

21 Ascending Aortogram AP Hypoplastic Ascending Aorta Lateral * * Courtesy of Boston Children s Hospital

22 Treatment Pre-surgical PGE to keep the DA patent until surgery Surgical (3 Surgeries within the first 3 years of life) 3 Stages

23 Surgical 3 Stages STAGE 1 (Individualized) (first few days of life) Norwood Operation Sano Modification Hybrid Procedure STAGE 2 (Bidirectional Gleen Shunt) at 3-6 mo STAGE 3 (Fontan) from 18mo to 4y

24 Stage 1: Norwood Procedure Objective: to get the Right Ventricle to pump blood into the systemic circulation Time: within the first few days of life Includes: Aortic Patch combines it with the Pulmonary Artery to form a single outlet vessel Blalock-Taussig shunt : Subclavian Artery is connected to the Pulmonary Artery to provide pulmonary circulation to the lungs OR Sano modification connect RV to Pulmonary Artery

25 Sano Modification no_shunt_procedure.html

26 Patient #2 at 6 months, Post Stages 1 & 2 Courtesy of Boston Children s Hospital

27 Post Norwood Procedure with 5mm Sano shunt Patient #1 Courtesy of Boston Children s Hospital

28 Hybrid Procedure Same results as the Norwood procedure, but without the need for sternotomy. Combination of catheter-based treatment and surgery. Stent is placed in the Ductus Arteriosos. Left and Right Pulmonary Artery Branches have bands placed over them to decrease pressure and decrease circulation to the lungs.

29 Stage 2: Glenn Shunt Objective: to decrease mixing of oxygenated and deoxygenated blood in the RV Time: at 3-6 months of age (mature lungs) Bidirectional Shunt Includes: SVC ligation from the RA, joined to the PA Blalock-Taussig or Sano shunt is taken out

30 Stage 3: Fontan Objective: Redirect venous return away from the RA to the PA Time: at 18 months to 4 years Includes: Lateral Tunnel Fontan Extracardiac Fontan: ligation of the IVC at the heart, conduit attached from the end of the IVC to the PA, totally bypassing the RA and RV

31 Prognosis If left untreated, HLHS is fatal. Early detection is key. The 3 Stage Surgery is palliative, not curative. Possible need of a heart transplant

32 Summary - Imaging Prenatal Ultrasound: 4 Chamber view Fetal Echo: diminished blood flow CXR: cardiomegaly, increased pulmonary vascular markings MRI: LV hypoplasia and compensatory RV hypertrophy, LVOT decreased in size, RVOT increased in size Aortogram

33 Acknowledgements Dr. Mark Masciocchi Dr. Ben Swan Dr. Gillian Lieberman Claire Odom Boston Children s Hospital

34 References Marcdante, Karen, Robert M. Kliegman, Hal B. Jenson. Nelson s Essentials of Pediatrics. 6e.USA Gil Wernovsky. Hypoplastic Left Heart Sydnrome. -left-heart-syndromehlhs.html. April 18, dex.cfm?cat=topic&topic_id=1083 September 2002 RadioGraphics, 22,