1 / 67

Pulmonary Hypertension Heart Disease (Braunward)

Pulmonary Hypertension Heart Disease (Braunward). Presenter: Cheng-Han Lee Supervisor: Ting-Hsing Chao. Normal Pulmonary circulation. Dual vessels supply and drainage : pulmonary a. and bronchial a. pulmonary v. and azygos v. Pul. a. arteriolescapillariespul. v.

makaio
Download Presentation

Pulmonary Hypertension Heart Disease (Braunward)

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Pulmonary HypertensionHeart Disease (Braunward) Presenter: Cheng-Han Lee Supervisor: Ting-Hsing Chao

  2. Normal Pulmonary circulation • Dual vessels supply and drainage : pulmonary a. and bronchial a. pulmonary v. and azygos v. • Pul. a.arteriolescapillariespul. v. • Bronchial a.capillariespul.v. azygos vein • Bronchial circulationphysiological right to left shunt

  3. Normal Pulmonary circulation • Normal pul. artery pressure: systolic: 18 to 25mmHg diastolic: 6 to 10 mmHg mean: 12 to16 mmHg • PG of pul. circulation: 2-10 mmHg • PG of systemic circulation: 90 mmHg • Pul. vascular resistence: 67±23 dyn sec.cm (around 1/10 of systemic vascular resistence).

  4. Normal Pulmonary circulation • Exercise PVR decreased, pul.flow and pul.a pressure increased • Aging PA pressure and PVR increased mild pul. HTN in 45 y/o  13% 75 y/o  28%

  5. Normal Pulmonary circulationResponse to hypoxia, Drugs, and neural and environmental factors • Hypoxia (PaO2≤ 55 mmHg) pul. vasoconstriction (K, Ca channel) • Neural regulation less certain (α1 receptors & norepinephrine)

  6. Normal Pulmonary HypertensionResponse to Hypoxia, Drugs, and Neural and Environmental Factors • Drugs Prostaglandins : PGI2 and PGE2(vasodilator) PGA2 and PGF2(vasoconstrictor) NO: vasodilator&inhibit platelet aggregation Endothelin: vasoconstrictor Serotonin: enhance release NO, medial hypertrophy and vascular remodeling Angiotensin II: vasoconstrictor, cell proliferation, cell migration and extracellular matrix synthesis

  7. Normal Pulmonary HypertensionResponse to Hypoxia, Drugs, and Neural and Environmental Factors

  8. PGI2 and NO Inhibit Platelet Relax smooth muscle

  9. Endothelin

  10. Primary Pulmonary Hypertension (PPH) • Definite Pul. HTN: systolic: larger than 30 mmHg mean: larger than 20 mmHg NIH definition: mean PA >25mmHg at rest mean PA >30mmHg at exercise • Require careful exclusion of secondary causes • Endothelial dysfunction • Marked vasoconstrictive tendency • Reduced expression of NO synthase • Increased level of endothelin • Intimal proliferation

  11. Possible pathogenesis of PPH

  12. Possible pathogenesis of PPH

  13. Possible pathogenesis of PPH

  14. Genetics • Variability about response of pulmonary vessel to stimuli like acidosis or hypoxia

  15. Familial PPH • 6% of PPH • Similar F/M ratio, age of onset, natural history • Trinucleotide repeat expansion • 2q31,33

  16. Risk Factors • Portal hypertension • Anorexigens • HIV infection • Systemic HTN • Increased pulmonary blood flow

  17. Portal Hypertension • Portosystemic shunt increased vasoactive mediators like serotonin& IL1&endothelial growth factor vascular remodeling

  18. Anorexigens • In 1981, Appetite suppressants (fenfluramine or amphetamine-like drugs) inhibit voltage-gated K channel vasoconstriction

  19. HIV infection • Possibility mediated by release of inflammatory cytokines or by autoimmune mechanism • Antiviral drugs seemed to retard the progression of pulmonary HTN

  20. Systemic HTN • Neurohumoral factors • Pulmonary vascular seems to be overactive to sympathetic stimulation • In some patients, the mechanism which increases SVR also increases PVR

  21. Increased pulmonary blood flow • Increase blood flow will induce disturbance of pulmonary endothelium • Hyperthyroidism and beriberi  unexplained pulmonary HTN

  22. PPHPathologic findings

  23. PPHPathologic findings

  24. PPHClinical features • Natural history NIH Registry on PPH (1981~1987)—194 patients a. 63% female b. mean age: 36±15 y/o at the time of diagnosis c. Time from onset to diagnosis: 2 years d. initial presentation: dyspnea(80%), fatugue(19%), syncope or near-syncope(13%), Raynaud phenomenon(10%)

  25. PPHClinical features • NIH Registry on PPH • PE findings P2 accentuation(93%) TR-related murmur(40%) peripheral edema(32%) • Lab findings: ECG: RVH pattern(87%) chest x-ray: enlarged main PA

  26. PPHClinical features • NIH Registry on PPH Lung function: restrictive DLCO: reduced Mild to moderate hypoxemia(mean PaO2=72±16 mmHg) Hemodynamic fingings: rising RA pressure, reduced cardiac output reflect RV function mean PA pressure rises early in the course

  27. PPHClinical features • NIH Registry on PPH Univariate analysis Mean RA, PA pressure, cardiac index and DLCO significantly related to mortality NYHA class  strongly related to survival The most common cause of death RV failure Sudden cardiac death: 26%  limited to NYHA class IV

  28. Mechanisms of RV failure • 1. Pressure and volume overload • 2. Animal study: pressure overload reduced RCA perfusion RV ischemia • 3. Shared IVS can affect both ventricle • 4. Left main coronary A. was compressed by PA

  29. Physical Examination • Large a wave in the jugular venous pulse • RV heave • Systolic pulsation in left 2nd ICS (dilated ,tense PA) • P2 accentuation and S4 gallop • RV failure signs hepatomegaly, ascites and peropheral edema) • Cyanosis in the late course • Uncommonly, laryngeal nerve was compressed by PA (Ortner syndrome)

  30. Laboratory findings • CBC and BCS: hypoxemia with polycythemia, abnormal liver function some reports: abnormal platelet function, hypercoagulopathy, defect in fibrinolysis • ECG: RA and RV enlargement pattern • Chest x-ray: RA, RV and main PA, its major branch enlargement with tapering of peripheral vessels • Cardiac echo: RA and RV enlargement with TR and pulmonary HTN, thick IVS, systolic prolapse of MV

  31. Lung Scintigraphy • Perfusion scan helps diffentiate PPH and pulmonary HTN secondary to chronic pulmonary thromboembolism

  32. Pulmonary angiography • Typically, large central PA with marked peripheral tapering • The procedure increased the risk adequate O2 avoid vasovagal reactions non-ionic contrast

  33. Others • Chest CT: central pulmonary embolism, caliber of PA • Exercise testing: objective assessment of severity and predictive of survival and drug efficacy

  34. Cardiac catheterization

  35. WHO 1998 diagnostic classification of pulmonary hypertension

  36. Diagnosis

  37. Diagnosis

  38. Treatment • Life style changes graded exercise activities are better than isometric ones • Pregnancy increase blood volume and O2 consumption, procoagulant factors, the risk of pulmonary embolism from DVT and amniotic fluid

  39. Treatment • Digoxin increase 10% of cardiac output of RV and significantly reduction in norepinephrine • Diurectics relieve symptoms main factor limiting cardiac output is PAR, not pulmonary blood volume • O2  hypoxemia causes pulmonary vasoconstriction

  40. TreatmentVasodilator

  41. TreatmentVasodilator

  42. Acute testing with IV vasodilators • Adenosine A. potent vasodilator and predictive of the subsequent effects of IV prostacyclin and oral CCB B. IV infusion with 50ng/kg/min and titrated up every 2 minutes until discomforts

  43. Acute testing with IV vasodilators • Prostacyclin (epoprostenol or PGI2) A. vasodilator effect mediated by adenylate cyclase and inhibition of platelet B. short half-life as adenosine, given IV infusion with increase in 2ng/kg/min every 15 to 30 minutes until side effect C. Adenosine and Prostacyclin both had inotropic effect

  44. Acute testing with IV vasodilators • Nitric oxide A. vasodilator via activation c-GMP B. no inotropic effect C. also effective in patients with secondary pulmonary HTN due to congenital heart disease and ARDS

  45. Chronic treatmentCCB • CCB may worsen pulmonary HTN and have negative inotropic effect on RV ( under conventional dose)

  46. Chronic treatmentCCB • High dose of CCB may reduce PA pressure and PVR which were maintained for over 5 years.

  47. Chronic treatmentCCB • Patients who do not exhibit dramatic hemodynamic response to CCB do not benefit from their long-term usage • Prescribing conventional dose of CCB is a malpractice because it may deteriorate pulmonary HTN should be strongly discouraged

  48. Chronic treatmentChronic Prostacyclin Infusion Therapy • The long term effect: A. Vasodilator B. Antithrombosis C. Restore the integrity of endothelium • Prostacyclin is given via central venous catheter that is surgically implanted. The complications: infection, thrombosis and interruption of delivery (rebound pulmonary HTN) • Side effect: flushing, headache, nausea, diarrhea and jaw discomfort with eating

  49. Chronic treatmentChronic Prostacyclin Infusion Therapy • Easy tachyphylaxis • A bridge to transplantation • Chronic use will result in high-cardiac output (inotropic effect or neurohormone effect)

  50. Chronic treatmentChronic Prostacyclin Infusion Therapy

More Related