Evaluation of the GORE PV1 Device in Patients With Pulmonary Valve Dysfunction

• GORE PV1 (Device): Implantation of GORE PV1 Device for procedure to replace the pulmonary valve and reconstruct the right ventricular outflow tract (RVOT).

Primary outcomes

• Valve-related mortality and device-related re-intervention [6 months]: The composite of valve-related mortality and device-related re-intervention measured at 6 months post-procedure

Secondary outcomes

• MRI change in right ventricular end diastolic volume index [6 months]: Six month change from baseline in right ventricular end diastolic volume index as measured from Magnetic Resonance Imaging
• MRI change in right ventricular end systolic volume index at 6 months [6 months]: Six month change from baseline in right ventricular end systolic volume index as measured from Magnetic Resonance Imaging
• MRI change in left ventricular diastolic volume index at 6 months [6 months]: Six month change from baseline in left ventricular diastolic volume index as measured from Magnetic Resonance Imaging
• MRI change in right ventricular ejection fraction at 6 months [6 months]: Six month change from baseline in right ventricular ejection fraction index as measured from Magnetic Resonance Imaging
• Echo change in right ventricular diastolic area at 12 months [12 months]: Twelve month change from baseline in right ventricular diastolic area as measured from echocardiography
• Echo change in right ventricular systolic area at 12 months [12 months]: Twelve month change from baseline in right ventricular systolic area as measured from echocardiography
• Echo change in left ventricular end diastolic volume index at 12 months [12 months]: Twelve month change from baseline in left ventricular end diastolic volume index as measured from echocardiography
• Echo change in right ventricular fractional area change at 12 months [12 months]: Twelve month change from baseline in right ventricular fractional area change as measured from echocardiography
• Six month mean pressure gradient <= 40 mmHG [6 months]: Number of subjects with mean pressure gradient across conduit (RV to PA) less than or equal to 40 mmHg at 6 months
• Twelve month mean pressure gradient <= 40 mmHG [12 months]: Number of subjects with mean pressure gradient across conduit (RV to PA) less than or equal to 40 mmHg at 12 months
• Six month pulmonary regurgitation < moderate [6 months]: Number of subjects with pulmonary regurgitation less than moderate at 6 months
• Twelve month pulmonary regurgitation < moderate [12 months]: Number of subjects with pulmonary regurgitation less than moderate at 12 months
• Device-related re-intervention at 1, 3, 6, 12, 24, 36, 48, and 60 months [1, 3, 6, 12, 24, 36, 48, and 60 months]: Kaplan-Meier estimates of device-related re-intervention summarized at 1, 3, 6, 12 months, and annually through 5 years
• All-cause mortality at 1, 3, 6, 12, 24, 36, 48, and 60 months [1, 3, 6, 12, 24, 36, 48, and 60 months]: Kaplan-Meier estimates of all-cause mortality summarized at 1, 3, 6, 12 months, and annually through 5 years

Sex: All, ages: 5 Years to any

Inclusion Criteria:

1. A symptomatic or appropriate asymptomatic subject with a native or repaired right ventricular outflow tract requiring reconstruction with a valved conduit
2. Age ≥5 years at the time of informed consent signature.

Note: Additional Inclusion Criteria may apply

Exclusion Criteria:

1. An artificial valve at another position or will need an artificial valve at another position (i.e. replacement at the time of index procedure or anticipated within 3 years).
2. Subjects with previously implanted pacemaker (including defibrillators).
3. Major or progressive non-cardiac disease (liver failure, renal failure, cancer) that has a life expectancy of less than one year.

Note: Additional Exclusion Criteria may apply

• Pedro del Nido, MD, Principal investigator, Boston Children's Hospital