IntelliStent for Pulmonary Flow Adjustment in Congenital Heart Disease and Dilated Cardiomyopathy

Inclusion Criteria:

1. Age: Adolescent (12-17 years) or Adult (age >18 years)

2. Uncorrected CHD (ASD, VSD, PDA, AVSD) with left to right + systemic pulmonary arterypressure or mixed shunting regardless if pulmonary vascular resistance is modifiableor fixed

3. Diagnosed with WHO Group 1 PH Classification pulmonary hypertension associated withcongenital heart disease, evidence by the following parameters measured at rest:

4. Mean pulmonary artery pressure (mPAP) ≥ 50 mmHg

5. Pulmonary capillary wedge pressure (PCWP) or left ventricular end diastolicpressure (LVEDP) ≤ 15 mmHg.

6. Pulmonary vascular resistance > 3 Wood Units

7. Patients with left ventricular dilated cardiomyopathy with symptoms despite optimalmedical therapy

8. Current WHO Functional Class III or IV.

9. Patients with the following anatomical dimensions (gated CT with angio or MRI withEKG) at target implantation site:

10. MPA diameter @ systolic ≥ 15 mm and ≤ 24 mm and length @ systolic ≥ 28 mm, or

11. Left and Right PA Branch diameter @ systolic ≥ 15 mm and ≤ 24 mm and length @systolic ≥ 28 mm

12. Main pulmonary artery (MPA) or left/right PA Branch anatomy suitable for placementof the device as defined in the Instructions For Use (IFU) and as assessed bycomputed tomography (CT), fluoroscopy or echocardiography.

13. Each patient, or his or her guardian or legal representative, is willing to giveinformed consent, subject to national law.

Exclusion Criteria:

1. Right ventricular dysfunction

2. Severe AV valve regurgitation of the pulmonary ventricle

3. Complex CHD

4. Ongoing infection

5. Patients where definitive correction of the CHD is indicated and available as apossible treatment option

6. Patients with pressure gradient across the systemic outflow tract/subaortic region >40 mmHg @ rest

7. PAH-CHD patients with small defects that may be incidental findings

8. PAH after corrective cardiac surgery

9. Anatomical limitation to IntelliStent® (e.g. pulmonary artery size)

10. Known or suspected thrombosis of the femoral or iliac veins on the proposed site ofvenous cannulation

11. Vasculature lesions or characteristics that prevent percutaneous transluminalcatheterization

12. Allergies or contraindications to prescribed procedural medications and contrastmedium Anomalous pulmonary venous return (total or partial)

13. Likely inability to comply with the protocol or cooperate fully with theinvestigator and site personnel

14. Mental incapacity, unwillingness or language barrier precluding adequateunderstanding of the trial procedure or cooperation with trial site personnel

15. Pregnant, lactating or planning pregnancy