Case Presentation : Pulmonary Hypertension: Diagnosis and Imaging

Transcription

1 Case Presentation : Pulmonary Hypertension: Diagnosis and Imaging Eftychia Demerouti MD, MSc, PhD Cardiologist Onassis Cardiac Surgery Center

2 Conflicts of interest Consulting fees and fees for educational presentations from Actelion Pharmaceuticals, as a board member. Travel and educational support from Actelion Pharmaceuticals, Bayer Healthcare, Galenica, Glaxosmithkline and Pfizer.

3 Woman, 53 y.o (34 y.o.) ASD closure & CABG (LIMA to LAD) Atrial Fibrillation Dyspnoea the last 3 years, classification III the last 6months Clinical signs BP 120/70 mmhg SaO2: 98% (po2: 77.4 mmhg, pco2: 31.2mmHg) 6MWT: 330m 90%, Borg Dyspnea Scale (0 5) NT-proBNP: 950 pg/ml ECG: Afib 85bpm, RVH

4 Drugs: Acenocumarol, Salospir 100 mg, Bisoprolol 2,5mg Echo (2010) : PASP: 40 mmhg, RV mild dilatation, MR 1+ Cardiac MRI (2012): RV moderate dilated, RVEF: 40%, No ASD Spirometry: FEV1: 90% pred FVC: 98% pred DLCO: 65%

5 Echo

6 Echocardiography

7 Echocardiography RVFAC 35%

8 Q1. Which is the following diagnostic step? 1. Chest Computed Tomography & Angiography 2. Myocardial Perfusion Scintigraphy 3. Right & Left Heart Catheterization 4. V/Q lung scan 5. Cardiac MRI

9 Q1. Which is the following diagnostic step? 1. Chest Computed Tomography & Angiography 2. Myocardial Perfusion Scintigraphy 3. Right & Left Heart Catheterization 4. V/Q lung scan 5. Cardiac MRI

10 JACC ; 25: D42-50

11 CTEPH diagnosis VQ scan: screening test in PH CTPA may lead to misdiagnosis of PAH and underdiagnosis of CTEPH Pulmonary Angiography: the gold standard for confirmation of CTEPH and evaluation of operability. High-quality multidetector CTEPH: in centers with experience in CTEPH J Am Coll Cardiol 2013; 62:D92-9

12 V/Q lung scan

13 RHC RA (mmhg) 11 RV (mmhg) 64/29 PA (mmhg) 64/47/55 (67,4%) PCWP (mmhg) 11 LV (mmhg) 110/80/75 (98%) C.I. (l/min/m2) 2.2 PVR (WU) 8.2 Qp/Qs 1.0

14 LHC

15 LHC

16 LHC Ostial narrowing of the LMCA

17 DSCT

18 CT

19 Q2. Which is the appropriate diagnosis? 1. Idiopathic Pulmonary Artery Dilatation 2. Chronic Thromboembolic PH 3. PAH associated with CHD 4. Idiopathic PAH

20 Q2. Which is the appropriate diagnosis? 1. Idiopathic Pulmonary Artery Dilatation 2. Chronic Thromboembolic PH 3. PAH associated with CHD 4. Idiopathic PAH

21 Classification of PAH-CHD JACC 2013: D34-41

22 LEFT MAIN COMPRESSION SYNDROME (LMCS) Extrinsic compression of left main coronary artery (LM) due to an enlarged pulmonary artery trunk Unusual cause of angina, left ventricular dysfunction and sudden cardiac death in patients with Severe Pulmonary Hypertension (PH) Lee MS et al. Catheter Cardiovasc Interv Oct 1;76(4):

23 Sudden Death in Pulmonary Hypertension Sudden Death is an increasingly recognized entity in PH patients. Pulmonary Artery (PA) dilatation seems to represent the precipitating factor for this complication. In the presence of significant dilatation of the main PA, further evaluation should be performed to exclude LMCS 1 Routine assessment of the coronary tree with Cardiac Dual-Source CT should be performed in PH patients with angina. 1. Catheter Cardiovasc Interv Jan;52(1): Am J Med Mar 15;116(6):369-74

24 Q3. Which is the following therapeutic step? 1. Pulmonary Artery Surgical Reconstruction 2. Left Main Coronary Artery Angioplasty 3. Specific PAH including Prostanoids 4. Specific PAH per os drug therapy 5. Lung Transplantation

25 Q3. Which is the following therapeutic step? 1. Pulmonary Artery Surgical Reconstruction 2. Left Main Coronary Artery Angioplasty 3. Specific PAH including Prostanoids 4. Specific PAH per os drug therapy 5. Lung Transplantation

26 Catheter Cardiovasc Interv Oct 1;76(4): Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: a contemporary review and argument for percutaneous revascularization. Lee MS 1, Oyama J, Bhatia R, Kim YH, Park SJ. Optimal treatment: debatable PCI: FEASIBLE, SAFE, EFFECTIVE TREATMENT OPTION

27 Q3. Which is the following therapeutic step? 1. Pulmonary Artery Surgical Reconstruction 2. Left Main Coronary Artery Angioplasty 3. Specific PAH including Prostanoids 4. Specific PAH per os drug therapy 5. Lung Transplantation

28 Evidence-Based Treatment Algorithm Combination therapy and interventional procedures Galiè N, et al. J Am Coll Cardiol 2013; 62:D60-72.

29 Initial therapy with PAH approved drugs JACC 2013; 62: 25 D60-72

30 6 months later Drugs: ERA, PDE5, Acenocumarol, Bisoprolol, Furosemide RHC: PAP mean 50 mmhg PVR: 7.2 WU C.I. 2.4 l/min/m 2 6MWT: 330m 360m BNP: 950 pg/ml 220 pg/ml

31 CONCLUSION CHD can be repaired but PAH recurs months or years later, with an aggressive phenotype. LMCS seems to be a rare complication in PH patients with severely dilated PA, but exists. DSCT represents a useful tool for screening LMCS Suspicion is crucial when LM coronary disease appears in Left Heart Catheterization

32 THANK YOU FOR YOUR ATTENTION