1. Critical Aortic Stenosis in the Neonate
Christopher Calahan, HMS III
Gillian Lieberman, MD

2. Agenda Patient Presentation Normal Anatomy & Physiology
Christopher Calahan, HMS III
Gillian Lieberman, MD
Agenda
Patient Presentation
Normal Anatomy & Physiology
Background of Disease
Menu of Tests
Intervention
Follow-Up

3. Our Patient Birth History Newborn Exam
Christopher Calahan, HMS III
Gillian Lieberman, MD
Our Patient
Birth History
Full term, 1 day old baby boy, delivered via caesarean section at OSH
Pregnancy complicated by gestational diabetes & pre-eclampsia
Newborn Exam
APGARs 8 /9 (>7=Normal)
Harsh 2-3/6 systolic murmur best heard at LUSB

4. Fetal Circulation: Anatomy
Christopher Calahan, HMS III
Gillian Lieberman, MD
Fetal Circulation: Anatomy
Shunts
Foramen Ovale
Right Atrium  Left Atrium
Ductus Arteriosus
Pulmonary Artery  Descending Aorta
Ductus Venosus
Umbilical Vein  Inferior Vena Cava
UpToDate

5. Fetal Circulation: Physiology
Christopher Calahan, HMS III
Gillian Lieberman, MD
Fetal Circulation: Physiology
Flow
~15% of blood goes to lungs
~85% of blood is shunted
60% RV PDA Body
40% RA  PFO LA LV Aorta Body
UpToDate

6. Aortic Valve Morphology
Christopher Calahan, HMS III
Gillian Lieberman, MD
Aortic Valve Morphology
Normal
Tricuspid, 3 Leaflets
Right, Left & Posterior Cusps
Variation
Bicuspid ( %)
Unicuspid (0.02%)
Unicommissural
Acommissural
Quadricuspid & Pentacuspid (<.01%)

7. Critical Aortic Stenosis: Background
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Background
Prevalence
3.8 in 10,000 live births, 6% of all CHD
Pathophysiology
Biscupid or Unicuspid Aortic Valve
Presentation
Early Gestation
Hypoplastic Left Heart Syndrome
“No Flow, No Grow”
Late Gestation
Two Functional Ventricles, but PDA dependent
UptoDate

8. Critical Aortic Stenosis: Physical Exam
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Physical Exam
Vitals
Tachycardia, Tachypnea, Decreased O2 Sat%
General
Cyanosis
Cardiovascular
Hyperactive precordium, poor pulses
Murmur can be heard although often absent due to poor cardiac output
Pulmonary
Edema (Infantile AS)
Extremities
Distal pulses often poor or unappreciable
UptoDate

9. Critical Aortic Stenosis: Tests
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Tests
EKG
LV Hypertrophy
Inverted T Waves
Labs (Infantile AS)
CBC
Electrolytes
Liver Function Tests
UptoDate

10. Critical Aortic Stenosis: Imaging
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Imaging
Echocardiography
Abnormal Morphology
Aortic Valve, Ventricular Dysfunction
Increased Peak-to-Peak Gradient
Pressure difference between LV & Aorta, measured by catheterization
Reduced Aortic Valve Area
Reduced Transvalvular Flow
Reduced Ejection Fraction
CXR
Cardiomegaly
Aortic Root Dilation (Older Patients)
UptoDate

11. Critical Aortic Stenosis: Diagnosis
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Diagnosis
Classification of Aortic Stenosis
Mild (Valve >1.5cm2, Velocity m/s, Gradient <20mmHg)
Moderate (Valve cm2, Velocity m/s, Gradient 20-39mmHg)
Severe (Valve <1.0cm2, Velocity >4m/s, Gradient >40mmHg)
Critical AS Requiring Intervention
Peak-to-peak gradient < 25mmHg
Overall, 20% risk of requiring intervention over 25 year period
Peak-to-peak gradient > 50mmHg
Overall, 71% chance of needing intervention
UptoDate

12. Our Patient: Critical Aortic Stenosis on EKG
Christopher Calahan, HMS III
Gillian Lieberman, MD
Our Patient: Critical Aortic Stenosis on EKG
LVH
T Wave Inversion (LV Strain)
Image Courtesy of Michael Farias, MD at BCH

13. Our Patient: Critical Aortic Stenosis on Chest Radiograph
Christopher Calahan, HMS III
Gillian Lieberman, MD
Our Patient: Critical Aortic Stenosis on Chest Radiograph
 Tip of ET Tube
 Moderate Cardiomegaly
 NG Tube
Image Courtesy of Michael Farias, MD at BCH

14. Our Patient: Echo Findings
Christopher Calahan, HMS III
Gillian Lieberman, MD
Our Patient: Echo Findings
Small to moderately sized PDA
Patent Foramen Ovale
Critical aortic stenosis
Severe LV dysfunction
Moderate mitral regurgitation
Blausen Medical Communications on Wikipedia

15. Review of Echo Technique
Christopher Calahan, HMS III
Gillian Lieberman, MD
Review of Echo Technique
Our Patient (3 Views): -Apical Four Chamber
-Parasternal Short Axis
-Parasternal Long Axis
Other Views: -Subxyphoid
-Right Parasternal
-Suprasternal A B
Sagittal Plane (Right-Left)
Coronal Plane (Anterior-Posterior)
Axial Plane (Superior-Inferior)
American Society of Edchocardiography

16. Our Patient: Left Ventricular Dysfunction on Echo
Christopher Calahan, HMS III
Gillian Lieberman, MD
Apical Four Chamber View
Our Patient: Left Ventricular Dysfunction on Echo * * * * * * * *
* Right Atrium * Right Ventricle * Left Atrium * Left Ventricle * Aorta
Image Courtesy of Michael Farias, MD at BCH

17. LV Dysfunction Video Click to Play 
Christopher Calahan, HMS III
Gillian Lieberman, MD
LV Dysfunction Video Click to Play  * * * *
* Right Atrium
* Right Ventricle
* Left Atrium
* Left Ventricle
Image Courtesy of Michael Farias, MD at BCH

18. Our Patient: Mitral Valve Regurgitation on Echo
Christopher Calahan, HMS III
Gillian Lieberman, MD
Parasternal Short Axis
Our Patient: Mitral Valve Regurgitation on Echo * * * * * * * *
* Left Atrium * Mitral Valve * Left Ventricle * Aorta
Images Courtesy of Michael Farias, MD at BCH

19. Mitral Regurgitation Video Click to Play 
Christopher Calahan, HMS III
Gillian Lieberman, MD
Mitral Regurgitation Video Click to Play  * * *
* Left Atrium
* Mitral Valve
* Left Ventricle * Aorta *
Image Courtesy of Michael Farias, MD at BCH

20. Our Patient: Unicuspid Aortic Valve on Echo
Christopher Calahan, HMS III
Gillian Lieberman, MD
Parasternal Short Axis
Our Patient: Unicuspid Aortic Valve on Echo
Valve Closed
Valve Open
* Left Coronary Artery * Right Coronary Artery * Aortic Valve
Images Courtesy of Michael Farias, MD at BCH

21. Unicuspid Aortic Valve Video Click to Play 
Christopher Calahan, HMS III
Gillian Lieberman, MD
Unicuspid Aortic Valve Video Click to Play 
Left Coronary Artery
Right Coronary Artery
O Aortic Valve
Image Courtesy of Michael Farias, MD at BCH

22. Critical Aortic Stenosis: Management
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Management
Medical
Prostaglandin & Dopamine
Intubation & Transfer to ICU
Catheterization (First Line)
Angiography to Confirm Diagnosis
Intervention to Dilate Valve with Balloon
Surgery (Second Line)
Valvotomy

23. Our Patient: Angiography
Christopher Calahan, HMS III
Gillian Lieberman, MD
Our Patient: Angiography *
Diagnostic confirmation of critical aortic stenosis with left ventricular dysfunction
Click Play to Watch Video
Femoral Artery Pigtail Tip
Femoral Vein Catheter Tip
* Patent Ductus Arteriosus
* Left Ventricle
* Aorta
Image Courtesy of Michael Farias, MD at BCH

24. Our Patient: Intervention
Christopher Calahan, HMS III
Gillian Lieberman, MD
Our Patient: Intervention
Balloon valvuloplasty for dilation of the aortic valve
Flow Measurements (AoV Gradient)
Before- 41 mmHg
After- 19 mmHg
Click Play to Watch Video
 Balloon Waist
Femoral Artery Pigtail Tip
Femoral Vein Catheter Tip
Image Courtesy of Michael Farias, MD at BCH

25. Our Patient: 9mo Follow-Up on Echo
Christopher Calahan, HMS III
Gillian Lieberman, MD
Apical Four Chamber View
Our Patient: 9mo Follow-Up on Echo
Before (Ejection Fraction 25%)
After (Ejection Fraction 56%) * * * * * * * *
* Right Atrium * Right Ventricle * Left Atrium * Left Ventricle
Images Courtesy of Michael Farias, MD at BCH

26. Critical Aortic Stenosis: Outcomes
Christopher Calahan, HMS III
Gillian Lieberman, MD
Critical Aortic Stenosis: Outcomes
Journal American College of Cardiology published an article in 2013 comparing results between surgical valvotomy and interventional catheterization
N=123 (Surgical n=86, Catheter n=37)
Freedom of re-intervention at 5 years was 27% in catheterization and 65% after surgery, but that does not take into account the higher risks associated with cardiac surgery
Outcomes can vary widely by site based on the skill of surgery vs. interventional cardiology practices
At this point, there are no guidelines in place stating when these patients should undergo valve replacement, if at all
JACC 2013, UptoDate

27. Summary Reviewed Fetal Shunts, Circulation and Aortic Valve Anatomy
Christopher Calahan, HMS III
Gillian Lieberman, MD
Summary
Reviewed Fetal Shunts, Circulation and Aortic Valve Anatomy
Discussed Disease Prevalence & Work-Up for Critical Aortic Stenosis
Echocardiography
Treatment Options
Outcomes

28. Acknowledgments Gillian Lieberman, MD- Radiology, BIDMC
Christopher Calahan, HMS III
Gillian Lieberman, MD
Acknowledgments
Gillian Lieberman, MD- Radiology, BIDMC
Michael Farias, MD, MBA- Interventional Cardiology, BCH

29. References Christopher Calahan, HMS III Gillian Lieberman, MD
ACR Appropriateness Criteria: Known or Suspected Congenital Heart Disease in the Adult. Date of Origin: Last Review Date: Date Accessed: September 16, Aortic Stenosis, Stanford Children’s Health. Date Accessed: September 15, Aortic Valve Replacement, Cardiothoracic Surgery, USC. Date Accessed: September 18, ASE Guidelines for Pediatric Echocardiography. Adapted from Lai et al. “Guidelines and Standard for Performance of a Pediatric Echocardiogram: A Report from the Task Force of the Pediatric Council of the American Society of Echocardiography.” J Am Soc Echocardiography 2006, 19: Brown et al. “Valvar Aortic Stenosis in Children”. Up to Date. Date Accessed: September 13-19, Campbell et al. “2014 Appropriate Use Criteria for Initial Transthoracic Echocargiography in Outpatient Pediatric Cardiology”. Journal of the American College of Cardiology. 64(19): November Fetal Circulation, Up to Date Graphic 66765, Version 4.0. Date Accessed: September 14, Fonseca et al. “Chest Radiography and Evaluation of the Neonate for Congenital Heart Disease”. Pediatric Cardiology. 26(4): August Keane JF, Driscoll DJ, Gersony WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with aortic valvar stenosis. Circulation 1993; 87:I16. Lilly. “Pathophysiology of Heart Disease”, Fourth edition, Chapter 3, “Diagnostic Imaging and Cardiac Catheterizaxtion,” pp , Chapter 16, “Congenital Heart Disease,” pp Siddiqui et al. “Surgical Valvotomy and Repair for Neonatal and Infant Congenital Aortic Stenosis Achieves Better Results Than Interventional Catheterization.” Journal of the American College of Cardiology. 62(22): December Venkata Thota and Farouk Mookadam (2011). Unicuspid Aortic Valve, Aortic Valve, Prof. Chen Ying-Fu (Ed.), ISBN: , InTech, Available from: