HDL Acute Lipid Optimization in Homozygous Familial Hypercholesterolemia

The PDS-2™ System is intended to reduce coronary atheroma in patients with Homozygous
Familial Hypercholesterolemia (HoFH). Subjects will receive serial infusions of autologous
selectively delipidated HDL/preβ enriched plasma following use of HDL Therapeutics' PDS-2
System.

Key Inclusion Criteria:

- Clinical diagnosis of Homozygous Familial Hypercholesterolemia (HoFH) due to a defect
in the LDL receptor, or the identification of a defect in apoB-100, or a gain of
function of PCSK9, or a genetic defect resulting in the HoFH clinical phenotype

- No other condition that would preclude the subject from successfully completing the
series of plasmapheresis visits in the investigator's opinion

- At least one (1) coronary artery study segment will be identified for each subject and
all will remain constant throughout the study. The qualifying study segment(s) will
have 20% to 40% stenosis.

Key Exclusion Criteria:

- Planned change in current lipid lowering therapy

- Use of oral anticoagulants, unless the dose has been stable for 4 weeks

- LDL or plasma apheresis within 1 week prior to enrollment and through 8-week primary
endpoint

- New York Heart Association (NYHA) class III or IV or last known left ventricular
ejection fraction < 30%

- Myocardial infarction, unstable angina, percutaneous coronary intervention (PCI),
coronary artery bypass graft (CABG) or stroke within 3 months of enrollment