Pulmonary Hypertension: Group. Patient case

Transcript

1 3 Pulmonary Hypertension: Group Patient case Αναστασία Ανθη Β Κλινική Εντατικής Θεραπείας & Ιατρείο Πνευμονικής Υπέρτασης Π.Γ.Ν. «ΑΤΤΙΚΟΝ»

2 Nice classification of PH Pulmonary hypertension WHO GROUP 1 PAH WHO GROUP 2 Left-heart related WHO GROUP 3 Lung/hypoxia related WHO GROUP 4 CTEPH WHO GROUP 5 Other Idiopathic (IPAH) Heritable Drug- and toxininduced Associated with other conditions (APAH) WHO Group 1 Pulmonary venoocclusive disease Pulmonary capillary hemangiomatosis WHO Group 1 Persistent pulmonary hypertension of the newborn (PPHN) Systolic dysfunction Diastolic dysfunction Valvular disease Congenital/acquired left heart inflow/ outflow tract obstruction Chronic obstructive pulmonary disease (COPD) Interstitial lung disease (ILD) Other pulmonary diseases with mixed restrictive and obstructive pattern Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure Chronic thromboembolic pulmonary hypertension PH with unclear multifactorial mechanisms WHO, World Health Organization. Simonneau G et al. J Am Coll Cardiol 2013;62(25 Suppl):D

3 Nice classification of PH Pulmonary hypertension WHO GROUP 1 PAH WHO GROUP 2 Left-heart related WHO GROUP 3 Lung/hypoxia related WHO GROUP 4 CTEPH WHO GROUP 5 Other Idiopathic (IPAH) Heritable Drug- and toxininduced Associated with other conditions (APAH) WHO Group 1 Pulmonary venoocclusive disease Pulmonary capillary hemangiomatosis WHO Group 1 Persistent pulmonary hypertension of the newborn (PPHN) Systolic dysfunction Diastolic dysfunction Valvular disease Congenital/acquired left heart inflow/ outflow tract obstruction Chronic obstructive pulmonary disease (COPD) Interstitial lung disease (ILD) Other pulmonary diseases with mixed restrictive and obstructive pattern Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure Chronic thromboembolic pulmonary hypertension PH with unclear multifactorial mechanisms WHO, World Health Organization. Simonneau G et al. J Am Coll Cardiol 2013;62(25 Suppl):D

4 Nobel Prize Winner 1956 Am J Physiol, 1947

5 Nobel Prize Winner 1956 Am J Physiol, 1947

6 vasoconstriction shear stress Alveolar Hypoxia vascular nonmuscularised proliferative smooth induces pulmonary associated with high fluid pulmonary alterations medial wall thickening and muscularisation of arterioles with muscle-like cells (SMLCs)

7 Parenchymal destruction may induce neointimal lesions vasoconstriction shear stress Alveolar Hypoxia vascular nonmuscularised proliferative smooth induces pulmonary associated with high fluid pulmonary alterations medial wall thickening and muscularisation of arterioles with muscle-like cells (SMLCs)

8 Mechanisms underlying the pulmonary vascular remodelling in Eur Respir Rev 2014; 23:

9 Pulmonary Hypertension in Chronic Lung Disease In proportion PH in lung disease: the underlying parenchymal remodeling causes some natural loss of overall vascular cross-sectional area Out of proportion PH in lung disease: the severity of PH is high in relation to the degree of lung parenchymal abnormalities

10 Pulmonary Hypertension in Chronic Lung Disease In proportion PH in lung disease: the underlying parenchymal remodeling causes some natural loss of overall vascular cross-sectional area Out of proportion PH in lung disease: the severity of PH is high in relation to the degree of lung parenchymal abnormalities severe PH in lung disease: mpap > 35 mm Hg or mpap > 25 mm Hg with low CI (<2 l/min/m 2 )

11 Pulmonary Hypertension in Chronic Lung Disease In proportion PH in lung disease: the underlying parenchymal remodeling causes some natural loss of overall vascular cross-sectional area Out of proportion PH in lung disease: the severity of PH is high in relation to the degree of lung parenchymal abnormalities limitation of exercise capacity due to exhausted circulatory reserve rather than an ventilatory reserve low carbon monoxide diffusion capacity

12 Pulmonary Hypertension in Chronic Lung Disease In proportion PH in lung disease: the underlying parenchymal remodeling causes some natural loss of overall vascular cross-sectional area Out of proportion PH in lung disease: the severity of PH is high in relation to the degree of lung parenchymal abnormalities The severe PH group : minority of chronic lung disease patients (for COPD is 1 % of the entire population)

13 Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease Over/underestimation:>10mmHg discrepancy between Dopper Echo and RHC Arcasoy, AJRCCM, 2003

14 Estimating pulmonary artery pressures by echocardiography in patients with emphysema Eur Respir J 2007; 30:

15 RHC indications in chronic lung disease Evaluation for lung transplantation Clinical worsening & progressive exercise limitation disproportionate to ventilatory impairment Progressive gas exchange abnormalities (low DlCO and arterial hypoxemia upon exercise) disproportionate to ventilatory impairment PH is suspected by non-invasive measures & further therapy is considered Adapted from Nathan & Cotin, Eur Resp Mon 2012;57:143

16 Γυναίκα, 70 ετών Ατομικό αναμνηστικό: Αρτηριακή Υπέρταση Ιδιοπαθής πνευμονική ίνωση (Διάγνωση με βιοψία πνεύμονα πρίν 1,5 έτος) Σε οξυγονοθεραπεία από διμήνου Παραπέμπεται στο ιατρείο πνευμονικής υπέρτασης από πνευμονολογική κλινική με το ερώτημα εναρξης ειδικής αγωγή για ΡΑΗ

17 Γυναίκα, 70 ετών Ατομικό αναμνηστικό: Αρτηριακή Υπέρταση Ιδιοπαθής πνευμονική ίνωση (Διάγνωση με βιοψία πνεύμονα πρίν 1,5 έτος) Σε οξυγονοθεραπεία από διμήνου Παραπέμπεται στο ιατρείο πνευμονικής υπέρτασης από πνευμονολογική κλινική με το ερώτημα εναρξης ειδικής αγωγή για ΡΑΗ Αιτία: Μεγάλη δύσπνοια δυσανάλογη (;) με τον λειτουργικό ελεγχο της αναπνοής U/S καρδιάς, PASP: 85 mmhg

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19 3/ 2014 WHO class 6MWT: δύσπνοιας) NT-proBNP ΙΙΙ 240m (μη ολοκλήρωση λόγω 1426 pg/ml Echocardiography TRV 3,9 m/s PASP 66 mmhg TAPSE 19 mm Διάταση δεξιάς κοιλίας με διάχυτη υποκινησία και μέτρια έκπτωση της λειτουργικότητας

20 3/ 2014 WHO class 6MWT: δύσπνοιας) NT-proBNP ΙΙΙ 240m (μη ολοκλήρωση λόγω 1426 pg/ml Pulmonary function tests: FEV 1 : 1.48 L (99%) FVC : 1.67 L (90.4%) FEV 1 /FVC : 88.7% TLC : 3.73 (86%) DLCOc SB : 25%

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24 3/ 2014 Right Heart Catheterization RAP 1 mmhg PAP 61/20/34 mmhg (S/D/M) PAWP 3 mmhg CO 3.6 l/min CI 2.5 l/min/m 2 PVR 8.6 Wood Units SvO2 77 %

25 Pulmonary Hypertension in Chronic Lung Diseases 2013:D JACC Vol. 62, No. 25,

26 Pulmonary Hypertension in Chronic Lung Diseases 2013:D JACC Vol. 62, No. 25,

27 Nathan and King. Drug Design, Development and Therapy 2014:8 Wednesday, March , 15

28 3/ /2014 Right Heart Catheterization RAP 1 mmhg 3 mmhg PAP 61/20/34 mmhg (S/D/M) 43/20/28 mmhg (S/D/M) PAWP 3 mmhg 7 mmhg CO 3.6 l/min 4,2 l/min CI 2.5 l/min/m 2 2,9 l/min/m 2 PVR 8.6 Wood Units 5 Wood Units SvO2 77 % 79 %

29 PH & IPF mpap<17mmhg mpap>17mmhg Hamada K et al. CHEST 2007; 131:

30 Survival of patients with COPD and no other detectable cause of pulmonary hypertension 2005,172: Chaouat A et al. AJRCCM

31 Cumulative survival from date of diagnosis for common diagnostic subgroups of PH Hurdman J et al. Eur Respir J 2012; 39:

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