Group Subgroup 1. Pulmonary arterial hypertension (PAH) 1.1. Idiopathic PAH 1.2. Heritable 1.2.1. BMPR2 1.2.2. ALK1, endoglin, SMAD9, CAV1, KCNK3 1.2.3. Unknown 1.3. Drug- and toxin-induced 1.4. Associated with 1.4.1….
Fig. 18.1 Right ventricular pressure–volume curve from normal subject (a) and from patient with increased right ventricular afterload (b). Adapted from Redington et al. [7] Coronary perfusion also differs between…
© Springer Science+Business Media New York 2015James R. Klinger and Robert P. Frantz (eds.)Diagnosis and Management of Pulmonary HypertensionRespiratory Medicine1210.1007/978-1-4939-2636-7_17 17. Investigative Therapies in Pulmonary Arterial Hypertension Karen A. Fagan1 (1) Pulmonary Diseases and Critical Care Medicine,…
Abnormalities Subjects Reference Platelet aggregation related Increased urinary thromboxane metabolite (11 dehydroxy TxB2) IPAH [41] Decreased urinary PGI2 metabolite (PGI-M) IPAH [41] Decreased nitric oxide (exhaled or urinary excretion) IPAH,…
Definition Hemodynamic characteristics Pulmonary hypertension mPAP ≥ 25 mmHg Pre-capillary PH mPAP ≥ 25 mmHg PAOP ≤ 15 mmHg CO normal or reduced Post-capillary PH mPAP ≥ 25 mmHg PAOP > 15 mmHg Combined post-capillary and pre-capillary PH mPAP ≥ 25 mmHg…
Fig. 3.1 Signaling pathways downstream of BMPR2 Interestingly, it is clear that not all persons with BMPR2 mutation develop PAH and within the same family there is variation in age…
