Presentation Outline ICHP Annual Meeting September 13-15

Etiology (Dana Point, 2008)
1’. Pulmonary veno-occlusive disease
(PVOD) and/or pulmonary capillary
hemangiomatosis (PCH)
2. PH due to left heart disease 2.1 Systolic dysfunction
2.2 Diastolic dysfunction
2.3 3 Valvular a u a disease d sease
3. PH due to lung diseases and/or 3.1 COPD
hypoxia
3.2 Interstitial lung disease
3.3 Other pulmonary diseases with
mixed restrictive and obstructive pattern
3.4 Sleep-disordered breathing
3.5 Alveolar hypoventilation disorders
3.6 Chronic exposure to high altitude
3.7 Developmental abnormalities
Simonneau G, et al. J Am Coll Cardiol. 2009;54(1s1):S43-54.
Etiology (Dana Point, 2008)
4. CTEPH
5. PH with unclear 5.1 Hematologic Myeloproliferative disorders
multifactorial
mechanisms
disorders
5.2 Systemic
Splenectomy
Sarcoidosis
disorders
Pulmonary Langerhans cell histiocytosis
LLymphangioleiomyomatosis h i l i t i
Neurofibromatosis type 1
Vasculitis
5.3 Metabolic Glycogen storage disease
disorders
Gaucher disease
Thyroid disorders
5.4 Others Tumoral obstruction
Fibrosing mediastinitis
CKD on dialysis
Simonneau G, et al. J Am Coll Cardiol. 2009;54(1s1):S43-54.
Epidemiology
• PAH prevalence1 – Estimated 50,000–100,000 Americans
– 15,000–20,000 diagnosed and receiving
treatment
• Diagnosed typically 3rd –4th decade of life2 • Female:male ratio 1.7:1
• 2.8-year median survival without treatment3 1 DeMarco T. Cardiol Rev. 2006;14:312–8.
2 Rich S, et al. Ann Intern Med. 1987;107:216-23.
3 D’Alonzo GE, et al. Ann Intern Med. 1991;115:343-9.
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