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Pulmonary Hypertension for general physicians
This patient, a 30-year-old woman, presented with progressive shortness of breath on exertion for two years. Tests revealed her pulmonary artery pressure was elevated at 55 mm Hg. Right heart catheterization confirmed a diagnosis of pulmonary arterial hypertension, showing a mean pulmonary artery pressure of 66 mm Hg and pulmonary vascular resistance of 15 wood units. As treatment for this non-vasoreactive pulmonary arterial hypertension, she will begin oral combination therapy based on her intermediate risk status.
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Pulmonary Hypertension for general physicians
- 1. Pulmonary Hypertension A casebased presentation for general physicians Sarfraz Saleemi MD PH program of Excellence King Faisal Specialist Hospital & Research Center Riyadh, Saudi Arabia
- 2. A 30-year-old pediatricnurse who is non-smoker, married with one healthy child, presents to the family health clinic with progressive shortness of breath on exertion for two years. She has been diagnosed to have bronchial asthma in her local hospital for which she takes inhaled steroids. She denies cough, chest pain, fever and weight loss. There is no family history of heart and lung disease. Her only medication in addition to inhaler steroid is oral contraceptives. Her vital signs shows normal temperature, regular pulse, blood pressure 105/50 and oxygen saturation 95% breathing room air. Her BMI is 23 . Her clinical examination is unremarkable. Routine laboratory investigations including CBC, renal and hepatic panels are within normal range. You order a chest X-ray and pulmonary function test which are reported as normal. You request a Ventilation/perfusion lung scan as you are worried she may have pulmonary embolism. The V/Q scan is reported as negative.
- 3. How will youmanage this patient? A. Prescribe a course of oral steroids and add an inhaled beta-agonist B. Order CT pulmonary angiogram as you are not satisfied with result of V/Q scan. C. Refer the patient to pulmonologist as you feel you have done all the investigations with no clear diagnosis. D. Prescribe anxiolytic medications as you feel her symptoms are anxiety related. E. Arrange an urgent transthoracic echocardiogram
- 4. When to suspectPH Clinical & Lab finding suggestive of PH Raised JVP ECG RBBB RVH tall P wave Exertional dyspnea Elevated pro-BNP Enlarged PA on X-ray Para-sternal heave Reduced DLCO
- 5. Symptoms (%) Dyspnea 98 Fatigue73 Chest pain 47 Near Syncope 41 Syncope 36 Leg Edema 37 Palpitations 33 SYMPTOMS OF PULMONARY HYPERTENSION IJ Clin Pract Suppl. 2007 Sep;(156):5-14. Review.
- 6. Physical exam findings •Loud pulmonary second heart sound • Tricuspid regurgitant murmur • Parasternal heave indicating right ventricular enlargement • Jugular venous distension • Peripheral edema • Hepatomegaly • Ascites – in advance right heart failure
- 7. ECG- abnormal in~ 80% of patients at diagnosis .
- 8. - abnormal in~ 90% of patients at diagnosisChest X-ray
- 9. Chest CT -PA/Ao Diameter >1 to identify PH Ao/PA Ng CS et al. J Thorac Imaging.1999 Oct;14(4):270-8. • Sensitivity 0.70 • Specificity 0.92
- 10. Badesch D etal. J Am Coll Cardiol. 2009;54:S55-S66. McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619. Hemodynamic Definition of PH/PAH PH PAH Mean PAP ≥25 mmHg PCWP/LVEDP ≤15 mmHg PVR >3 Wood Units (>240 dynes) Mean PAP ≥25 mm Hg
- 11. Pulmonary Circulations –Low pressure, Low resistance, High capacitance Pulmonary Circulation Systemic Circulation Arteries Arteries Veins Veins 120/80, mean 93 25/8, mean 14 Right Ventricle 25/2-5 Left Ventricle 120/5-10 LungBody SVR= 17.6 PVR= 1.8 High capacitance 3.8 billion capillaries
- 12. Ibn al-Nafis • DiscoveredPulmonary circulation nearly 350 years before Sir William Harvey(1616) of England • Described the purification of blood in the lungs where it was refined in contact with the air inhaled from the outer atmosphere Historical Perspective
- 13. PH is notone disease! The spectrum of diseases with high pulmonary pressure
- 14. LV LA RA RV PT Pre-capillary vs. Post-capillarypulmonary hypertension (PAH) (PVH) Pre-capillary PH PCWP<15 mmHg Post -capillary PH PCWP >15 mmHg
- 15. PH Venous Group 2 Hypoxemic Group 3 Miscellaneous /unclear mechanism Group5 Thrombo- embolic Group 4 Arterial Group I Classification of Pulmonary Hypertension J Am Coll Cardiol. 2013 Dec 24
- 16. Epidemiology PAH -10 to 52 cases per million (70–80% Females) Average age of PAH patients at diagnosis - 53 ± 14 years Idiopathic PAH - 1-2 cases per million HIV- infected patients - 0.5% Systemic sclerosis - 7-12% Sickle cell disease - 2-3.7.5% CTEPH -1-4% of patients following acute PE. British Journal of General Practice, November 2012
- 17. REVEAL Registry –PAH (group 1) 2967 patients. Adapted from Badesch DB et al. Chest. 2010;137:376-387. Overall PAH Associated PH Associated (50.7%) Idiopathic (46.2%) Heritable (2.7%) Pulmonary veno-occlusive (0.4%) Connective tissue/ collagen vascular (49.9%) Congenital heart disease (19.5%) HIV (4.0%) Other (5.5%) Portopulmonary (10.6%)
- 18. Vasoconstrictors Vasodilators Imbalance of vasoconstrictorsand vasodilators Endothelin-1 Thromboxane A2 Leukotrienes Serotonin Angiotensin II Platelet activating factor NO PGI2 Vasodilators Antiproliferatives Anticoagulants Vasoconstrictors Proliferative Procoagulants Pathophysiology of PH Normal PAH
- 19. Right Ventricular Changesin PH Increased pulmonary arterial pressure RV pressure overload Myocardial remodeling with RV hypertrophy RV dilatation, increased wall stress and dysfunction RV failure and Cor Pulmonale Peña, Elena, et al. Radiographics 32.1 (2011): 9‐32.
- 20. 2015 ESC/ERS Guidelinesfor the diagnosis and treatment of pulmonary hypertension DIAGNOSIS
- 21. Echocardiographic probability of pulmonaryhypertension in symptomatic patients with a suspicion of pulmonary hypertension
- 22. Back to thecase …… Echocardiogram was done and reported as: Normal LVEF Normal aortic and mitral valves Normal Left atrium Tricuspid valve – mild regurgitation TRV 2.9 m/s Right atrium and right ventricle mildly dilated RVSP = 55 mm Hg No pericardial effusion Bubble study did not show evidence of shunt
- 23. Back to thecase …… Echo – High probability for PH What should we do next? Left heart disease and intra-cardiac shunt ruled out Lung disease unlikely – Chest X-ray and PFT – normal What is the next step in the management?
- 24. 2015 ESC/ERS Guidelinesfor the diagnosis and treatment of pulmonary hypertension DIAGNOSIS
- 25. Natural History ofPAH: NIH Registry Predicted survival according to the NIH equation. Predicted survival rates were 69%, 56%, 46%, and 38% at 1, 2, 3, and 4 years, respectively. The numbers of patients at risk were 231, 149, 82, and 10 at 1, 2, 3, and 4 years, respectively. *Patients with primary pulmonary hypertension, now referred to as idiopathic pulmonary hypertension. 1. Rich et al. Ann Intern Med. 1987;107:216-223. 2. D’Alonzo et al. Ann Intern Med. 1991;115:343-349. Predicted survival 69% 56% 46% 38% Percentsurvival Years Median survival = 2.6 years
- 26. PAH is aFatal Disease Comparable to Cancer D’Alonzo et al; Ann Internal Med (1991), Kato et al; Cancer (2001), Soerjomataram et al; Breast Cancer Res. Treatment (2008) Advanced lung cancer Survival (years) WHO class IV PAH Advanced colon cancer Advanced breast cancer Advanced prostate cancer WHO class III PAH WHO class I-II PAH Early breast cancer 2.6 years 4.9 years 6 months 0 1 2 3 4 5 6 >10years
- 27. Sitbon et al.Slides presented at European Respiratory Society; September 16-18, 2007; Stockholm, Sweden. Survival With Modern Therapies
- 28. McLaughlin VV, etal. Circulation. 2002;106:1477-1482. 0 20 40 60 80 100 Survival(%) 0 12 24 36 48 60 72 84 No. at risk 162 33 95 70 48 30 20 10 Months FC=3 FC=4 p=0.0001 by log-rank test 847260483624120 100 80 60 40 20 0 FC=1 No. at risk: FC=2 FC=3 FC=4 Survival(%) Months Impact of Functional Class on Survival Functional Class at Baseline Functional Class at 17 ± 15 months 102030466386112115
- 29. WHO functional classfor PH patients Class I: Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope Class II: Slight limitation of physical activity. Comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope Class III: Marked limitation of physical activity. Comfortable at rest. Less than ordinary physical activity causes symptoms Class IV: Inability to carry out physical activity without symptoms. Manifest signs of right heart failure. Symptoms may be present at rest.
- 30. 1% 24% n=674 12% (%) 63% 100 80 60 40 20 0 FCI FC II FC III FC IV WHO FC FC at the time of diagnosis of PAH Humbert et al; Am J Respir Crit Care Med (2006) French National Registry (2002-2003)
- 31. Pulmonary Circulation |January-March 2013 | Vol 3 | No 1 D E L A Y S T U D Y
- 32. Pulmonary Circulation |January-March 2013 | Vol 3 | No 1 95% 5% 96% 5% 0% 0%
- 33. WHO SHOULD BESCREENED Annual screening recommended in patients with… • Systemic sclerosis • Family history of a heritable form of PAH • Portal hypertension considered for organ transplant
- 34. Back to thecase …… Right heart catheterization result • Mean PAP PCWP • PVR • CO • RAP • Vaso-reactivity (using NO) (A decrease in mean PAP >10 mmHg to ≤ 40 mmHg Normal or ↑ Cardiac output) 66 mmHg 11 mmHg 15 WU 4.2/2.1 Negative 11 mmHg
- 35. Back to thecase …… What is the diagnosis? Pulmonary arteria hypertension What is the next step in managing this patient?
- 36. 2015 ESC/ERS Guidelinesfor the diagnosis and treatment of pulmonary hypertension
- 37. Back to thecase …… CTD antibody profile normal Liver US with Doppler – negative HIV serology – Negative Schistosoma antibodies – Negative No significant drug history No family history of pulmonary hypertension Echo with bubble study has ruled out CHD
- 38. Back to thecase …… What is the final diagnosis? Pulmonary arteria hypertension What is the next step in managing this patient?
- 39. Treatment Treatment naive patient PAH confirmedby expert center General measures Supportive therapy Acute vasoreactivity test (I-PAH, H-PAH, D-PAH only) Vasoreactive CCB Therapy Non-vasoreactive (or not IPAH/HPAH/DPAH) Low/int Risk High Risk Oral Monotherapy Oral Combo Therapy Parenteral Therapy Inadequate response Double/triple sequential therapy OR lung transplant . Galie N, ESC/ERS 2015 Guidelines
- 40. Risk Assessment . Galie N,ESC/ERS 2015 Guidelines Estimated 1-year mortality Low Risk (<5%) Intermediate (5-10%) High Risk (>10%) Right heart failure No No Yes Progression No Slow Rapid Syncope No Occasional Repeated WHO FC I or II III IV 6MWD >440 m 165–440 m <165 CPET VO2 max >15 Ve/VCO2 <36 VO2 max 11-15 Ve/VCO2 36-45 VO2 max <11 Ve/VCO2 >45 NT-pro BNP <50 50-300 >300 Hemodynamics RA <8 mmHg CI >2.5 SvO2 >65% RA 8-14 mmHg CI 2.0-2.4 SvO2 60-65% RA >14 mmHg CI <2 SvO2 <60%
- 41. Back to thecase …… Intermediate Right heart failure No Progression Slow Syncope No WHO FC III 6MWD 350 m NT-pro BNP 290 Hemodynamics RA 11 mmHg CI 2.1
- 42. <1995 1995 20012002 2004 2005 2007 2009 2013 2015 CCB Anticoagulation Digitalis Diuretics IV Epoprostenol Bosentan SC Treprostenol IV Treprostenol Inhaled Iloprost Sildenafil Ambrisartan Tadalafil Inhaled Treprostinil Macitentan Riociguat Pulmonary Hypertension Treatment Timeline IV Sildenafil Oral Treprostinil Selexipag BREATHE SUPER ARIES PHIRST AIR SERAPHIN PATENT CHEST GRIPHON FREEDOM
- 43. Route Indication (WHO) Drawback Cost /year Bosentan, Ambrisentan Macitentan PO II-IV Hepatotoxicity (mainly with Bosentan) $35,000 Sildenafil PO I-IV Headache $10,000 Iloprost INH III-IV Fluctuating levels $75,000 Treprostinil SQ IV II-IV Site pain Catheter-related $50,000- 150,000 Epoprostenol IV III-IV Catheter-related $50,000- 100,000 SimplicityEfficacy
- 44. Back to thecase …… Patient was started on double vasodilator therapy including Sildenafil 20 mg TID and Macitentan 10 mg daily She was seen 3 months later …. FC II (previous III) 6 MWD = 410 ( previous 350) NT-pro BNP = 200 ( previous 290) Clinical examination did not show evidence of right heart failure No history of syncope during this period
- 45. Back to thecase …… What should we advise her at this stage? Continue same medication. Discontinue Macitentan and continue Sildenafil Add inhaled prostacyclin to maximize the therapy Patient profile has improved to low risk group – continue the same treatment
- 46. Back to thecase …… Patient comes to family health for a routine follow up after her visit to PH clinic. What will you advise her?
- 47. Role of primarycare physician in management of PH patient Advise on healthy lifestyle Sodium restriction Abstinence from smoking Avoid high altitude <4,000 feet above sea level Physical activity improve exercise capacity and quality of life Avoid heavy physical exertion in the setting syncopal symptoms Avoid pregnancy – advise contraceptive measures Pregnancy is absolute contraindication – peripartum mortality upto 30% Update vaccination status – Influenza and Pneumococcal vaccine Check the oxygen status and provide oxygen if needed. Advise on compliance of PH patients – interruption of scheduled doses can have devastating effect because of rebound increase in PAP
- 48. Treatment for non-PAHgroup pulmonary hypertension Group 2 (Left heart disease) – treat the under lying heart condition Group 3 ( Hypoxemic) – Oxygen therapy and treat underlying lung disease Group 4 ( Thromboembolic – CTEPH)) – Surgery – pulmonary endarterectomy non-operable cases should receive vasodilator treatment CTEPH is the only type of PH which is curable Group 5 (Miscellaneous group) – no clear guidelines how to treat these patient. Assess individual cases for treatment decisions. Patients with no response to maximum medical treatment should be referred for lung transplant
- 49. Conclusion PAH isa rare but fatal disease if not recognized and treated early. Think of pulmonary hypertension in any patient with unexplained dyspnea. Screen by Transthoracic Doppler Echocardiogram. Echo request should mention PH as and indication. Patient with intermediate to high probability of PH on echocardiogram should be referred to PH physician after doing initial screening for heart and lung conditions as a possible cause for PH Definitive diagnosis of PAH is made by right heart catheterization. PAH treatment depends on risk profile and individual patient assessment. Calcium channel blockers is treatment of choice in vaso-reactive PH on cardiac cath. Pregnancy may cause significant challenges and is contraindicated Patient with PH need a comprehensive general health and life style evaluation and advice.
- 50. Thank you Saudi guidelineson the diagnosis and treatment of pulmonary hypertension: 2014 updates. Ann Thorac Med 2014;9, Suppl S1:1-15