PULMONARY HTN

2. (PH) is characterized by elevated pulmonary
arterial pressure≥ 25 mmHg and 2ry RVF(ie,
cor pulmonale). It is a progressive, fatal
disease if untreated, although the rate of
progression is highly variable.

4.  a normal pulmonary artery pressure is defined as a mean pressure at rest
≤20 mmHg, while PHTN is defined as a mean pulmonary artery pressure ≥25
mmHg at rest.
 Ohm's Law:
Change in pressure = flow x resistance
Ppa - Ppv = Q x PVR
Ppa = (Q x PVR) + Ppv
Ppa = (Q x PVR) + PCWP

5. 1-Genetic mutations:
 ) Bone morphogenetic protein receptor type II (BMPR2)
 Serine/threonine-protein kinase receptor R3, also called Activin-like kinase type 1 receptor
(ALK1; ACVRL1)
 5-hydroxytryptamine (serotonin) transporter (5HTT)
 Potassium channel subfamily K member 3 (KCNK3
2-PAH second hits :
 Congenital heart disease : left-to-right intracardiac shunting increases flow through the
pulmonary vasculature, which causes shear forces that disrupt the vascular endothelium
and activate cellular mechanisms critical to the pathogenesis and progression of PAH .
 HIV: due to Plexogenic arteriopathy
 K-channel dysfunction :could lead sequentially to a change in resting membrane
potential, elevation of the intracytoplasmic free calcium concentration, and an
increase in pulmonary vascular tonehypoxia

6. 3-PH second hits :
 COPD : increasing pulmonary vascular resistance through two
mechanisms: hypoxia-induced vasoconstriction and obliteration of the
vascular bed .
 Heart failure — Pulmonary venous pressure increases when the left
ventricle fails, causing increased pulmonary vascular smooth muscle
tone and, to a lesser extent, structural remodeling. The net effect is PH.
The same is true with other causes of left atrial hypertension, including
mitral and aortic valve disease, left atrial myxoma, pulmonary venous
obstruction, restrictive cardiomyopathy, and constrictive pericarditis.

7.  Spmtoms:
1-Exertional dyspnea , chest pain , syncope & LL edema
2-Couph,Hemoptysis & Hoarsness (Ortner’s Ѕ)
 OE:
1-RVH:prominent A wave,Rt-sided 4th HS and left parasternal heave
2-RVF: widens the splitting of the 2nd HS. systolic ejection murmur,
elevated jugular venous pressure, a right ventricular third heart
sound, and a prominent V wave in the jugular venous pulse.
Hepatomegaly, a pulsatile liver, peripheral edema, ascites, and
pleural effusion may also exist.

8.  CXR
 ECG
 Echo: PASP = (4 x [TRV]2) + RAP
PH is likely if the PASP is >50 and the TRV is >3.4
PH is unlikely if the PASP is ≤36, the TRV is ≤2.8, and there are no other suggestive findings
PH is possible with other combinations of findings
 PFTs
 Overnight oximetry
 PSG
 VQ scan & CTPA
 Lab: HIV,LFT, Rheumatological markers
 Excerccise test
 RT. HEART CARHETRIZATION


14.  PH: PH is confirmed when the mean pulmonary
artery pressure is ≥25 mmHg at rest
 PAH:
●MPAP is ≥25 mmHg at rest
●MPCWP<15 mmHg (to exclude PH owing to left
heart disease
●Chronic lung diseases and other causes of
hypoxemia are mild or absent
●Venous thromboembolic disease is absent
●Certain miscellaneous disorders are absent,
including systemic disorders (eg, sarcoidosis),
hematologic disorders (eg, myeloproliferative
diseases), and metabolic disorders (eg, glycogen
storage disease)..

16.  BASELINE ASSESSMENT
 PRIMARY THERAPY
Group 1 PAH: no effective primary ttt
Group2-5 PH: ttt the cause
All groups : Diuretics ,O2, Anticoagulation ,
Digoxin , Exercise
 ADVANCED THERAPY
Patient selection :
Group 1 PAH
Group 2 PH : contraindicated

17. Group 3 PH : occasionally considered for
patients who remain WHO functional class III
or IV despite correction of hypoxemia and
optimization of the underlying disease
Group 4 PH :for patients who remain WHO
functional class IIl or IV even after
anticoagulation or thromboendarterectomy
Group 5 PH: few studies

18.  Vasoreactivity test : involves the administration
of a short-acting vasodilator and then
measurement of the hemodynamic response
using a right heart catheter. Agents
commonly used for vasoreactivity testing
include epoprostenol, adenosine, and
inhaled NO
is considered positive if mean pulmonary artery
pressure decreases at least 10 mmHg and to
a value less than 40 mmHg

22.  RIGHT TO LEFTSHU NT:
atrial septostomy
Transcatheter Potts Shunt
 Bilateral lung or heart-lung transplantation :
WHO functional class III or IV
Mean right atrial pressure >10 mmHg
Mean pulmonary arterial pressure >50 mmHg
Cardiac index <2.5 L/min per m2
Failure to improve functionally despite medical
therapy
Rapidly progressive disease