IntelliStent Pulmonary Flow Adjustment in Congenital Heart Disease and Dilated Cardiomyopathy (HEARTFUL-CHD)

Inclusion Criteria: Age: Adolescent (12-17 years) or Adult (age >18 years) Uncorrected CHD (ASD, VSD, PDA, AVSD) with left to right + systemic pulmonary artery pressure or mixed shunting regardless if pulmonary vascular resistance is modifiable or fixed Eisenmenger or Pre-Eisenmenger syndrome, regardless if pulmonary vascular resistance is modifiable or fixed Diagnosed with WHO Group 1 PH Classification pulmonary hypertension associated with congenital heart disease, evidence by the following parameters measured at rest: Mean pulmonary artery pressure (mPAP) ≥ 50 mmHg Pulmonary capillary wedge pressure (PCWP) or left ventricular end diastolic pressure (LVEDP) ≤ 15 mmHg. Pulmonary vascular resistance > 3 Wood Units Reactive pulmonary vascular bed, defined as a reduction in mean PAP by 10 mmHg to < 40 mmHg (Sitbon Criteria) or as a decrease of mPAP or the mPAP/mean systemic arterial pressure (mSAP) ratio by ≥ 20% (Borst Criteria) using inhaled nitric oxide. (Seemingly) fixed pulmonary vascular resistance, not modifiable with oxygen, NO or other medications. Patients with the following anatomical dimensions (gated CT with angio or MRI with EKG) at target implantation site: MPA diameter @ systolic ≥ 15 mm and ≤ 24 mm and length @ systolic ≥ 28 mm, or Left and Right PA Branch diameter @ systolic ≥ 15 mm and ≤ 24 mm and length @ systolic ≥ 28 mm Main pulmonary artery (MPA) or left/right PA Branch anatomy suitable for placement of the device as defined in the Instructions For Use (IFU) and as assessed by computed tomography (CT), fluoroscopy or echocardiography. Current WHO Functional Class III or IV. Each patient, or his or her guardian or legal representative, is willing to give informed consent, subject to national law. Exclusion Criteria: Complex CHD Anomalous pulmonary venous return (total or partial) Aortic atresia Heterotaxy syndrome Ongoing infection Anatomical limitation to IntelliStent® (e.g., femoral vein impeded access, pulmonary artery size...) Patients where definitive correction of the CHD is indicated and available as a possible treatment option and who have an acceptable risk of surgery Patients with single ventricle defects Patients with truncus arteriosus Patients with pressure gradient across the systemic outflow tract/subaortic region >40 mmHg @ rest PAH-CHD patients with small defects (ASD <2 cm) PAH after corrective cardiac surgery Severe AV valve regurgitation of the pulmonary ventricle (or of the systemic AV valve in single ventricle patients) Known or suspected thrombosis of the femoral or iliac veins on the proposed site of venous cannulation Allergies or contraindications to prescribed procedural medications and contrast medium Vasculature lesions or characteristics that prevent percutaneous transluminal catheterization Likely inability to comply with the protocol or cooperate fully with the investigator and site personnel Mental incapacity, unwillingness or language barrier precluding adequate understanding of the trial procedure or cooperation with trial site personnel Pregnant, lactating or planning pregnancy