This patient with complex congenital heart disease presented with progressive shortness of breath. She had previously undergone multiple open-heart surgeries, including a Rastelli procedure. Testing found elevated pulmonary vascular resistance due to a partially dehisced ventricular septal defect patch. Medical therapy improved her symptoms and reduced pulmonary pressures. A covered stent was then used to close the defect, further improving her condition. Her current treatment plan is to continue pulmonary hypertension medications.

1. Pulmonary Hypertension in an Adult with
Complex Congenital Heart Disease
Richard A. Krasuski, M.D.
Professor of Medicine and Pediatrics
Director of the Adult Congenital Heart Disease Program
Director of Hemodynamic Research
Duke University Health System

2. A Complicated Return to Care
• A 34-year-old woman with complex congenital heart disease
presented to the urgent care complaining of progressive
dyspnea on exertion
• Born with transposition of the great arteries, a ventricular septal
defect, a small atrial septal defect and pulmonic stenosis
• Followed in the pediatric cardiology clinic until the age of 26, but
has not seen a physician since then due to insurance reasons

3. D-transposition of the Great Arteries with Pulmonic
Stenosis and a Ventricular Septal Defect
•TGA accounts for ~5-7% of all
congenital heart disease
•Associated lesions
–40-50% ventricular septal defect
–25% LV outflow tract obstruction
–5% coarctation of the aorta

4. A Complicated Return to Care
• Atrial septostomy at birth and then a Rastelli procedure at age 3
• 3 subsequent operations described as “conduit revisions”
• For about 3 years she had been getting out of breath with activity –
first when working out and now with almost any activity
• Exam notable for O2 sat of 95%, JVP of 11 with large V-waves,
III/VI pansystolic murmur, clear lungs, 2+ edema

5. Rastelli Procedure for d-TGA with VSD and
RVOT Obstruction
• First performed in 1967 at the Mayo Clinic
• Aortic outflow routed through the VSD via a patch
• Pulmonic valve opening is oversewn
• Conduit placed from the RV to PA
• Restores the LV as systemic ventricle, so unlike
atrial switch procedures (Mustard or Senning), no
long-term risk for heart failure or systemic
atrioventricular valve regurgitation
• Results in frequent need for conduit interventions
Warnes CA. Circulation 2006;114:2699-2709.

6. Imaging Studies in Sequence
• Chest X-ray
–Enlarged cardiac silhouette, prominent PAs, post-
op changes
• V/Q – Low probability of pulmonary embolism
•Transthoracic/Transesophageal Echos
–Normal LV size and systolic function
–Mildly dilated right ventricle with mildly
reduced systolic function
–Mild tricuspid regurgitation with estimated
RVSP 65 mmHg

7. Sequential Imaging Studies
•Cardiac MRI
–Partially dehisced VSD patch
–Qp:Qs 1.3:1
–Moderately dilated right ventricle
•Cardiac Catheterization
–RA 8, RV 70/8, PA 70/30 m45, mPCW 8 mmHg
–Qp 7 l/min Qs 5 l/min Qp:Qs 1.4:1
–PVR 5.3 Wood units (9.0 Wu/m2)

8. What Would You Recommend for This Patient?
A. Refer the patient for reoperation to repair the shunt
B. Refer the patient for a percutaneous intervention to try to close
the shunt
C. Initiate advanced medical therapy for the increased pulmonary
vascular resistance
D. Make no changes and arrange for close observation

9. Stout KK et al. J Am Coll Cardiol 2019;73:1494-563; Condliffe R et al. Pulm Circ 2018;8:1-12;
Brida M and Gatzoulis MA. Heart 2018;104:1568-74
When Can Shunts Be Safely Closed?
• Close defect when PVRi <4 Wood units/m2, Qp:Qs is ≥1.5 and
patient is symptomatic
• Individual patient assessment recommended in tertiary centers
when PVRi 4-8 Wood units/m2
• Trial of balloon occlusion may be instructive
• Vasodilator testing - ↓ in PVRi ≥20% to <6 Wood units/m2;
PVRi/SVRi <0.3
• Treat-and-repair approach (advanced therapies and
reassessment followed by closure if indication) being studied
• Close follow-up with planned repeat invasive hemodynamics
recommended after closure
• Partial defect closure (fenestrated patch or device) may have
role in selected patients from “grey zone”

10. Medical Therapy Initiated
• Patient started on macitentan and tadalafil later added
• 6MWD improved from 310 to 350m
• NYHA function class improved from III to II
• Repeat Cardiac Catheterization 6 months later
–RA 6, RV 50/6, PA 50/24 m30, mPCW 6 mmHg
–Qp 9.5 l/min Qs 6 l/min Qp:Qs 1.6:1
–PVR 2.5 Wood units (4.3 Wu/m2)

11. What Would You Recommend for This Patient Now?
A. Refer the patient for reoperation to repair the shunt
B. Refer the patient for a percutaneous intervention to try to close
the shunt
C. Add a third drug for the persistently elevated pulmonary
vascular resistance
D. Make no further changes and arrange for close observation

12. Multiple Therapeutic Options Discussed
•With 5 prior sternotomies surgery was considered
prohibitive
•Device closure was attempted and was unsuccessful
•Transplant evaluation was pursued but patient was
prohibitively alloimmunized
•Decision to try to cover the defect with a covered stent
placed within the pulmonary artery

13. 3D Printing to Aid a Complex Intervention

14. The Complex Transcatheter Intervention

15. Follow-up 3 Months After the Procedure
• Patient had been continued on macitentan and tadalafil
• 6MWD further improved from 410m
• NYHA function class improved to I to II
• Repeat Echo showed near normal size RV and estimated
RVSP of 40 with 13/8 mmHg peak/mean gradients across
pulmonic valve

16. What Would You Recommend Now?
A. Continue both drugs
B. Stop both drugs
C. Stop the tadalafil
D. Stop the macitentan

17. Pulmonary Hypertension in d-transposition
•Usually occurs after late repair (> age 2) with atrial switches
•More common if shunt present before repair
•Need to exclude pulmonary venous pathway obstruction
•Associated with very high mortality
Baskaran J et al. Pulmonary Circulation 2017; 7(2) 547–550.