1. 순환기 내과 R3 임규성 Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

2. Definition Hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥ 25mmHg at rest as assessed by right heart catheterization.

3. Clinical classification of pulmonary hypertension Pulmonary arterial hypertension Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis Pulmonary hypertension due to left heart disease Pulmonary hypertension due to lung diseases and/or hypoxia Chronic thromboembolic pulmonary hypertension PH with unclear and/or multi-factorial mechanisms

4. Diagnosis  Symptom and sign (non-specific) Progressive onset of exertional dyspnea (m/c) Fatigue Chest pain or discomfort Dizziness and light-headedness, with possible history of near syncope or syncope Raynaud’s phenomenon Palpitation Ortner’s syndrome ; hoarseness (rare) Pulmonary component of S 2 accentuated (audible at apex) Early systolic ejection click Midsystolic ejection click RV S4 gallop Prominent jugular “a” wave

5. Signs of advanced disease Diastolic m (PR) &/or holosystolic m of TR Elevated JVP with accentuated “V” waves Marked distension of jugular veins, hepatojugular reflux, pulsatile hepatomegaly RV S3 gallop, pph edema, ascites Low BP, diminished pulse pressure, cool extremities

6.  Screening ; Echocardiography The most widely used screening test for pulmonary hypertension Estimates systolic pulmonary artery pressure Evaluates cardiac anatomy and function Bubble study should be considered if intracardiac shunt is suspected  Confirmatory test Right heart catheterization (with vasodilator challenge)

7. Echocardiography  Morphologic evaluation Enlarged RA and RV RV hypertrophy D-shaped LV cavity with flattening of the interventricular septum in systole Diminished/absent atrial wave of pulmonary valve Mid systolic closure or notching of pulmonary valve

8. Assessment of PA pressure PAPs (systolic PA pressure) = 4 X (TR peak velocity) 2 + RA pressure PAPm (mean PA pressure) = 4 X (peak PR velocity) 2 = 79 – 0.45 X (RVOT AT) PAPd (end-diastolic PA pressure) = 4 X (PR end-diastolic velocity) 2 + RAP

9. Continuous wave Doppler 최대유속 (Peak Velocity) m/sec TR PAPs = 4 X (TR peak velocity) 2 + RAP Tricuspid Regurgitation Velocity

10. PAPd (end-diastolic PA pressure) = 4 X (PR end-diastolic velocity) 2 + RAP Pulmonary Regurgitation Velocity Cardiol Clin 2004;22:383 PR end-diastolic velocity

11. PAPm (mean PA pressure) = 4 X (peak PR velocity) 2 = 79 – 0.45 X (RVOT AT) Pulmonary Regurgitation Velocity JASE 2007;20:773 Peak PR velocity

12. 폐동맥 고혈압 (Pulmonary Hypertension) 수축기 폐동맥압  35 mmHg 이완기 폐동맥압  15 mmHg 평균폐동맥압  25 mmHg RV 좌심실 (LV) ; D-shape, 축소 우심실 (RV) ; 확대 심실중격 ; 이상운동

13. Pulmonary vascular resistance 1. Important hemodynamic variable in the management of patients with severe heart failure or congenital heart disease. 2. Evaluation of candidates for cardiac transplantation PVR = (MPAP – PCWP) / Pulmonary blood flow = Δ P / Qp PVR ECHO = 10 X (TR Velocity/TVI RVOT ) + 0.16

14. TRV/TVI RVOT = 2.86/20.8 = 0.1375 PVR ECHO = 10 X (TRV/TVI RVOT ) + 0.16 = 10 X 0.1375 + 0.16 = 1.53 WU (TRV/TVI RVOT ) >0.2 = Elevated PVR (2 WU)

15. Assessment of RA pressure

16. Estimation of RA pressure Inferior Vena Cava Small (<1.5 cm) Normal (1.5-2.5 cm) Normal Dilated (>2.5 cm) Dilated with dilated hepatic vein Change with Respiration or Sniff Collapse Decrease by > 50% Decrease by < 50% No change RA pressure 0 - 5 5 - 10 10 - 15 15 - 20 > 20

17. E / E’ tricuspid ratio E / E’ > 6 → RAP greater than 10 mmHg JASE 2007;20p773

18. 우실면적변화율 （ %Fractional Area Change: %FAC) Apical 4 chamber view %FAC= 확장기면적 - 수축기면적 확장기면적 ×100 正常 : 30 - 60%

19. Tricuspid annular plane systolic excursion (TAPSE) Ghio. AJC 2000;85:8 37

20. Kaplan Meier estimates of event-free survival (end point: death or emergency transplantation) according to TAPSE. Tricuspid annular plane systolic excursion (TAPSE) TAPSE >14 TAPSE <14

21.  Right Heart Catheterization in PAH Confirm diagnosis Evaluate the severity and when PAH specific drug therapy is considered  Vasoactivity testing Idiopathic PAH, heritable PAH, and PAH associated with anorexigen use to detect patients who can be treated with high doses of CCB Postive result ( reduction of mean PAP ≥ 10mmHg or mean PAP ≤ 40mmHg with an increased or unchanged CO) Using NO, epoprostenol, adnosin

22. Evaluation of severity

24. Sign of RV failure and fluid retentsion PaO2 < 60mmHg Atrial tachyarrhythmia BAE : balloon atrial septostomy ERA : endothelin receptor antagonist PDE5 : phosphodiesterase type 5 inhibitor