Assessmet of Patients With PAH Right Ventricular Volume

The objective of this study is: The comparison of the VMS and MRI values for EDV, ESV, and
EF using 75 subjects.

Secondary objectives are:

The determination of VMS inter-observer and intra-observer variability of these quantities
using 30 subjects.

Inclusion Criteria:

- Patients with Group 1 Pulmonary Arterial Hypertension

- IPAH

- HPAH

- APAH-CTD

- APAH-HIV

- APAH-PoPH

- APAH-Drugs/Toxins

- APAH-CHD repaired simple systemic to pulmonary shunts, i.e. ASD, VSD and/or PDA

- APAH-CHD unrepaired simple systemic to pulmonary shunts, i.e. ASD, VSD and/or PDA
Patients who can be expected to lie motionless during imagine Males and females 12
years of age and older

Exclusion Criteria:

- Lack of informed consent (and assent as appropriate)

- Other forms of PH not included in inclusion criteria

- Left heart disease including clinically significant valvular disease, ,i.e. moderate
or greater mitral regurgitation or stenosis or mild or greater aortic insufficiency
or stenosis, pericardial disease, LV systolic dysfunction, i.e. LVEF <40% or LVSF
<22%, and/or clinically significant LVDD

- Known/detected arrhythmia that interferes with image acquisition

- Implanted cardiac defibrillator, pacemaker, or other devices containing ferromagnetic
materials

- Pregnant or breast-feeding females

- Contraindications for MRI (for those patient that undergo MRI)

- Clinically significant obstructive or restrictive lung disease

- Subjects with known HIV infection who have any clinical or laboratory evidence of any
opportunistic pulmonary disease (e.g., tuberculosis, Pneumocystis carinii pneumonia,
or other pneumonias)

- PAH associated with thyroid disorders, glycogen storage disease, Gaucher's disease,
hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative
disorders or splenectomy

- Any subjects with congenital heart disease other than the simple congenital to
systemic shunts specified in the inclusion criteria

- PAH associated with significant venous or capillary involvement (PCWP ˃ 15 mmHg),
known pulmonary veno-occlusive disease, or pulmonary capillary hemangiomatosis

- Clinically significant cardiac ischemic disease

- Systemic hypertension defined as SBP ˃ 160 mmHg and/or DBP ˃ 95 mmHg (treated or
untreated)

- Moderate or severe hepatic impairment, i.e., Child-Pugh Class B or C

- Any subject with obstructive sleep apnea or who requires the use of CPAP or BiPAP
device