Pulmonary hypertension

Question 1

• Increased pressure in the pulmonary circulation
• suspect in patients with increasing dyspnea and a knwon cause
• defined as mean pulmonary arterial pressure mmHg or greater at rest; systolic pressure > 25 mmHg(nml 18-20mmHg)

Answer 1

Pulmonary hypertension

Question 2

epidemiology, complications and prognosis of pulmonary hypertension?

Answer 2

Epidemiology: females > males, 20-40 years of age
complications: cor pulmonale and right heart failure
prognosis: if untreated, median survival after diagnosis is 2.8 years

Question 3

Patient presentation in pulmonary hypertension

Answer 3

• exertional dyspnea and fatigue, exertional chest pain, weakness, cyanosis and edema; exertional syncope, anorexia
• less common: cough, hemopysis and hoarseness
• Accentuated S2 (narrowed 2nd heart sound)
• signs of right sided heart failure: Increased JVP, peripheral edema, ascities

Question 4

what would you see on CXR of pulmonary edema?

Answer 4

• enlargment of central pulmonary arteries
• attenuation of peripheral vessels
• right atrial dilatation
• right ventricular enlargment

Question 5

what EKG findings can be seen in pulmonary hypertension?

Answer 5

• RVH- right ventricular strain pattern: ST segment and T wave inversion in V1-V3 and occasionally in inferior leads (II, III, AVF)
• RAE- P wave amplitude > 2.5mm in inferior leads
• RBBB

Question 6

other possible test findings in pulmonary hypertension?

Answer 6

• PFT: obstructive or restrictive pattern
• oximetry: nocturnal desaturations common
• polysomnography: if OSA possible cause
• exercise testing- detects exerccise induced PH and guids therapy for WHO classes
• High resolution chest CT

Question 7

Diagnosis of pulmonary hypertension?

Answer 7

• 1st in the diagnsotic workup (screen) echocardiography
• Gold standard: Right hearth catheterization (definitive diagnostic study)

Question 8

• Pulmonary arterial hypertension that is idiopathic, genetic, inf.
• PH w/o physicial limitation

Answer 8

group 1

Question 9

• pulmonary HTN secondary to left heart disease
• PH w/ slight limitations (exertions sxs, causing mild fatigue, DOE and chest pain

Answer 9

group 2

Question 10

• pulmonary HTN secondary to lung disease (COPD, interstitial)
• PH with markedly limited physical active due to their DZ (mild resting sxs and undue symptoms with any exertion)

Answer 10

group 3

Question 11

• chronic thromboembolic pulmonary HTN
• PH and unable to perform any physical activity w/o symptoms (RH and failure and increased dyspnea at rest)

Answer 11

Group 4

Question 12

unclear or multifactorial etiologies of pulmonary hypertension

Answer 12

group 5

Question 13

Primary therapies of pulmonary HTN?

Answer 13

• diuretics: treat fluid overload, careful not to limit cardiac output
• O2: Cornerstone of therapy in group 3, improves hypoxia in PH
• Anticoagulation: increased risk for thrombosis due to RAE and sluggish blood flow
• exercise: improves overall function
• vaccinations: influenza and pneumo

Question 14

when can advanced therapy for pulmonary HTN be completed?

Answer 14

• advanced therapis only after R heart catherterization and diagnosed according to functional classification

Question 15

advanced pharmacologic therapies in pulmonary hypertension?

Answer 15

• CCBs: works well in vasoreactive patients, causeing PA vasodilation (diltiazem, amlodapine), do not use short acting nifedipine
• postanoids: 1st line agents in severe disease
• phosphodiesterase 5 inhibitors
• transcatheter potts shunt: is possible palliative measure to shunt left PA blood to the descending aorta

Question 16

• Inhibits platelet activation and is also an effective vasodilator
• epoprostenol and treprostinil
• improved survivial & exercise capacity

Answer 16

prostanoids

Question 17

• MOA: smooth muscle relaxation and vasodilation of PA
• sildenafil: improved pulmonary hemodynamics and exercise capacity
• combination of PDEF and prostanoid showed improved clinical outcomes

Answer 17

Phosphodiesterase 5 inhibitors