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Clinical Presentation and Diagnosis

Clinical Presentation and Diagnosis in Pulmonary Hypertension Due To Recurrent Pulmonary Thromboembolism Numan EKİM MD. Gazi University School of Medicine Chest Diseases Department. Recurrent pulmonary thromboembolism. Epidemiology and risk factors -1

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Clinical Presentation and Diagnosis

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  1. Clinical Presentation and Diagnosis in Pulmonary Hypertension Due To Recurrent Pulmonary Thromboembolism Numan EKİM MD. Gazi University School of Medicine Chest Diseases Department

  2. Recurrent pulmonary thromboembolism Epidemiology and risk factors -1 • Venous thromboembolism (VTE) is a frequent, multicausal,and potentially fatal disease • Despite adequate treatment, up to one quarter ofpatients with symptomatic deep vein thrombosis(DVT) and/or pulmonary embolism (PE) willexperience recurrent venous thromboembolism (VTE)within the subsequent 5 years • Even after adequate anticoagulant therapy some 3-5 % of patients have reccurence of their VTE • Patients with thromboembolic events ofunknown origin (idiopathic) have a more than two-fold higher rateof recurrent VTE in comparison to patients whosethrombosis is associated with acquired, transient riskfactors Eichhinger S. et al.Circulation 2007 Prandoni P. et al. Ann Intern Med 2002 Lobo Jl et al.British J Haemat 2007 Trow TK, Mc Ardle JR. Clin Chest Med2007

  3. Recurrent pulmonary thromboembolism Epidemiology and risk factors - 2 • Patients withcontinuous risk factors, such as cancer or the antiphospholipidantibody syndrome, and those with idiopathic thrombosishave a two- to threefold increased risk for recurrencecompared with patients who developed a thrombotic eventin association with a transient risk factor • Risk of recurrence may be higher in the presence of residual venous thrombosis or elevation of D-dimer • The risk of recurrencedepends on the number and severity of risk factors in anindividual patient.

  4. The risk of recurrent venous thromboembolism afterdiscontinuing anticoagulation in patients with acute proximaldeep vein thrombosis or pulmonary embolism. A prospectivecohort study in 1,626 patientsPaolo Prandoni, Franco Noventa, Angelo Ghirarduzzi, Vittorio Pengo, Enrico Bernardi,Raffaele Pesavento, Matteo Iotti, Daniela Tormene, Paolo Simioni, Antonio PagnanHaematologica 2007; 92:199-205 • 1626 consecutive patients who had discontinued anticoagulation after a first episode of clinically symptomatic proximal DVT and/or PE were followed up to a maximum of 10 years • All patients with clinically suspected reccurent VTE underwent objective tests to confirm or rule out the clinical suspicion

  5. The risk of recurrent venous thromboembolism afterdiscontinuing anticoagulation in patients with acute proximaldeep vein thrombosis or pulmonary embolism. A prospectivecohort study in 1,626 patientsPaolo Prandoni, Franco Noventa, Angelo Ghirarduzzi, Vittorio Pengo, Enrico Bernardi,Raffaele Pesavento, Matteo Iotti, Daniela Tormene, Paolo Simioni, Antonio PagnanHaematologica 2007; 92:199-205

  6. The risk of recurrent venous thromboembolism afterdiscontinuing anticoagulation in patients with acute proximaldeep vein thrombosis or pulmonary embolism. A prospectivecohort study in 1,626 patientsPaolo Prandoni, Franco Noventa, Angelo Ghirarduzzi, Vittorio Pengo, Enrico Bernardi,Raffaele Pesavento, Matteo Iotti, Daniela Tormene, Paolo Simioni, Antonio PagnanHaematologica 2007; 92:199-205

  7. The risk of recurrent venous thromboembolism afterdiscontinuing anticoagulation in patients with acute proximaldeep vein thrombosis or pulmonary embolism. A prospectivecohort study in 1,626 patientsPaolo Prandoni, Franco Noventa, Angelo Ghirarduzzi, Vittorio Pengo, Enrico Bernardi,Raffaele Pesavento, Matteo Iotti, Daniela Tormene, Paolo Simioni, Antonio PagnanHaematologica 2007; 92:199-205 • The results of this study clearly show that after discontinuinganticoagulation the rate of recurrent VTE increasessteadily over time, approaching 40% among allpatients after 10 years • More than 10% of allrecurrences were either documented fatal PE or suddenandotherwise inexplicable deaths, in which PE could notbe ruled out • The results of this study fully confirm that patients who presentwith thrombotic episodes of unknown origin have a morethan two-fold higher risk of recurrences than thatobserved in patients with temporary risk factors. Ofinterest, in the latter category of patients, those with associatedmedical diseases had the highest risk, while thosewith VTE triggered by recent trauma or surgery the lowest,and this is consistent with previous reports • Improving thelong-term prognosis of patients with acute VTE stillremains a challenging task.

  8. Association of Persistent Right Ventricular Dysfunctionat Hospital Discharge After Acute PulmonaryEmbolism With Recurrent Thromboembolic EventsStefano Grifoni, MD; Simone Vanni, MD; Simone Magazzini, MD; Iacopo Olivotto, MD; Alberto Conti, MD;Maurizio Zanobetti, MD; Gianluca Polidori, MD; Filippo Pieralli, MD; Nazerian Peiman, MD;Cecilia Becattini, MD; Giancarlo Agnelli, MDArch Intern Med2006;166:2151-2156 • In 301 consecutive patients with the first episode of acut PE, echocardiography was used to assess right ventricular dysfunction (RVD) on admission and before hospital discharge • Patients were followed up at 2,6, and 12 months and yearly thereafter. The primary endpoint was symptomatic,reccurent fatal or nonfatal VTE • According to the ECHO features on hospital admission and at discharge, patients were classified into 3 groups ; 1st. group- patients without RVD on admission, 2nd. group- patients with RVD regression at discharge, and 3rd. group - patients with RVD persistance at discharge

  9. Association of Persistent Right Ventricular Dysfunctionat Hospital Discharge After Acute PulmonaryEmbolism With Recurrent Thromboembolic EventsStefano Grifoni, MD; Simone Vanni, MD; Simone Magazzini, MD; Iacopo Olivotto, MD; Alberto Conti, MD;Maurizio Zanobetti, MD; Gianluca Polidori, MD; Filippo Pieralli, MD; Nazerian Peiman, MD;Cecilia Becattini, MD; Giancarlo Agnelli, MDArch Intern Med2006;166:2151-2156

  10. Association of Persistent Right Ventricular Dysfunctionat Hospital Discharge After Acute PulmonaryEmbolism With Recurrent Thromboembolic EventsStefano Grifoni, MD; Simone Vanni, MD; Simone Magazzini, MD; Iacopo Olivotto, MD; Alberto Conti, MD;Maurizio Zanobetti, MD; Gianluca Polidori, MD; Filippo Pieralli, MD; Nazerian Peiman, MD;Cecilia Becattini, MD; Giancarlo Agnelli, MDArch Intern Med2006;166:2151-2156 • The results of the present study show thatpersistenceofRVD at hospital discharge is a frequent finding,occurringin approximately 20% of patients who present witha first episode of PE • RVD persistence is common at hospitaldischarge after the first episode of PE. Following discharge,RVD persistence is associated with an increasedrisk of recurrent VTE and death related to PE. Patientswith RVD persistence should receive a strict surveillancefor recurrences. • Extending anticoagulanttherapy may be particularly useful for patients withRVD persistence and idiopathic PEpresentation, both representingindependent risk factors for VTE recurrence inthe present study. I Indeed, in thesepatients the estimatedrisk of recurrence was at least 5-fold higher than that ofpatients without the 2 combined risk factors.

  11. Recurrent pulmonary thromboembolism Important risk factors of recurrence include ; • antithrombin deficiency, • the lupus anticoagulant, • Highfactor VIII, • factor V Leiden and prothrombinG20210A mutation • hyperhomocysteinemia, • previous venous thrombosis, • cancer, • male sex. • aging ( importance for VTE ? ) • clinical presentation with primary PE • the duration of anticoagulation following theinitial thrombotic episode Mortality • Approximately 5% of patientswith recurrence die of pulmonary embolism In many patientswithrecurrent VTE, Eichhinger S. et al.Circulation 2007 Prandoni P. et al. Ann Intern Med 2002

  12. Chronic thromboembolic pulmonary hypertension(CTEPH) Definition • Chronic thromboembolic pulmonary hypertension(CTEPH) is an important cause of pulmonary hypertensionthat is commonly considered to be the consequenceof acute pulmonary embolic disease. • Following an acute event,unresolved residual thrombus becomes organised and fibrosed,leading to ongoing obstruction to pulmonary blood flow. • Untreated, this leads to progressive pulmonary hypertension,right ventricular dysfunction and death Suntharalingam J. et al. Thorax 2007 Trow TK, Mc Ardle JR. Clin Chest Med2007

  13. Progression of CTEPH Acute or recurrent PTE in pulmonary arteries Organisation these thrombi Occurence in situ thrombus due to slow blood flow in obstructed pulmonary arteries Occurence of arteritis in not obstructed small distal pulmonary arteries(remodelling) Increased PVR, pulmonary hypertension CTEPH

  14. Chronic thromboembolic pulmonary hypertension(CTEPH) Clinical presentation - 1 • The diagnosis of CTEPH is usually not made until the degree of pulmonary hypertension is advanced • A patient may carry on relatively normal activities following a pulmonary embolic event, whether clinically apparent or occult, even when extensive pulmonary vascular occlusion hasoccurred (asymptomatic –honeymoon – period) Fedullo PF et al.N Engl J Med 2001

  15. Chronic thromboembolic pulmonary hypertension(CTEPH) Clinical presentation - 2 • Patients who have CTEPH typically complain of exertional dyspnea and a gradual decrease in exercise tolerance over months to years • Diagnostic delay : • Nonspesific nature of symptoms • Absence a history of prior acute symptomatic venous thromboembolism (DVT / PE) • The average delay from the onset of cardiopulmonary symptoms to establisment of the correct diagnosis can range from 2 to 3 years Fedullo PF et al.Semin Resp Crit Care Med 2003 Auger WR et al. Clin Chest Med 2007

  16. Chronic thromboembolic pulmonary hypertension(CTEPH) Clinical presentation - 3 Progressive dyspnea and exercise intolerance due to CTEPH are often erroneously attributed to ; • coronary artery disease • cardiomyopathy • congestive heart failure • interstitial lung disease • COPD (mild) • asthma • physical deconditioning • psychogenic dyspnea Prior to consideration of a pulmonary vascular problem as a basis for their complaints, many patients with CTEPH have undergone ; • left-sided cardiac catheterizations (one or more ) • coronary angiograms • lung biopsy. • enrolling in an exercise program • seeking psychiatric help. Fedullo PF et al.N Engl J Med 2001 Auger WR et al. Clin Chest Med 2007

  17. Chronic thromboembolic pulmonary hypertension(CTEPH) Clinical presentation Symptoms • Progressive dyspnea • Nonproductive cough (especially with exertion) • Hemoptysis • Palpitations • A change voice quality or hoarseness • Exertional chest pain • Near-syncope or syncope • Lower extremity edema Fedullo PF et al.Semin Resp Crit Care Med 2003 Auger WR et al. Clin Chest Med 2007

  18. Chronic thromboembolic pulmonary hypertension(CTEPH) Clinical presentation Physical examination - 1 May be subtle early in the course of the illness. In time obvious findings develop, which may include : • Right ventricular lift • Jugular venous distension • Prominent A and V wave venous pulsations • Fixed splitting of S2 with an accentuated pulmonic component • A right ventricular S4 gallop • A tricuspid regurgitation murmur • Hepatomegaly • Ascites • Peripheral edema, which may be a result of either chronic lower extremity venous outflow obstruction or right ventricular failure. Fedullo PF et al.N Engl J Med 2001 Auger WR et al. Clin Chest Med 2007

  19. Chronic thromboembolic pulmonary hypertension(CTEPH) Clinical presentation Physical examination - 2 The presence of flow murmurs over the lung fields(30 percent of patients). • turbulent flow through partially obstructed or recanalized pulmonary arteries • high pitched and blowing in quality • heard over the lung fields rather than the precordium, accentuated during inspiration • frequently heard only during periods of breath-holding • they have not been described in primary pulmonary hypertension, which represents the most common competing diagnostic possibility Fedullo PF et al.N Engl J Med 2001 Auger WR et al. Clin Chest Med 2007

  20. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Pulmonary function tests • Useful for excluding coexisting parenchymal lung disease or airflow obstruction • Often within normal limits • The majority of patients with CTEPH have a reduction in the single breath diffusing capacity for carbon monoxide (DLCO); a normal value, however, does not exclude the diagnosis • Approximately 20 percent of patients demonstrate a mild to moderate restrictive defect • A mild obstructive defect may be present as a result of mucosal hyperemia, which is related to development of a large bronchial arterial collateral circulation Steenhuis KS. Et al. Eur Respir J 2000 Auger WR et al. Clin Chest Med 2007

  21. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Blood gas analysis • Resting arterial PO2 may be within normal limits • Hypoxemia at rest implies very severe right ventricular disfunction or the presence of a right -to- left shunt, as through a patent foramen ovale • Majority of patients have a decline in the arterial PO2 with exercise • The alveolar-arterial oxygen gradient is typically widened • Dead space ventilation (VD/VT) is often increased at rest and worsens with exercise • Minute ventilation is typically elevated as a result of the increased dead space ventilation. Fedullo PF et al. N Engl J Med 2001 Auger WR et al. Clin Chest Med 2007

  22. Diagnosis Chest radiography Often normal Enlargement of both main pulmonary arteries or asymmetry in the size of the central pulmonary arteries Areas of hypoperfusion or hyperperfusion Evidence of old pleural disease, unilaterally or bilaterally Right atrial or right ventricular enlargement, based on the outline of the right cardiac border ( especially on the lateral film by encroachment on the normally empty retrosternal space) Cardiomegaly Chronic thromboembolic pulmonary hypertension(CTEPH) Fedullo PF et al.N Engl J Med 2001 Auger WR et al. Clin Chest Med 2007

  23. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Electrocardiography (ECG) • Right axis deviation • Right ventricular hypertrophy • Right atrial enlargement • Right bundle – branch block • ST segment displacement • T- wave inversions in anterior precordial and inferior limb leads Auger WR et al. Clin Chest Med 2007

  24. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Echocardiography • Enlargement and reduced systolic function of the right ventricle are usually apparent, • Leftward septal displacement can impair left ventricular filling and performance • ECHO is useful for the excluding; • Left ventricular dysfunction • Valvular disease • Cardiac malformations Menzel T et al. Chest 2000 Auger WR et al. Clin Chest Med 2007

  25. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Radioisotopic V / Q scanning – 1 • In chronic thromboembolic disease, at least one (and more commonly, several) segmental or larger mismatched ventilation-perfusion defects are present but not spesific for this condition • In idiopathic pulmonary arterial hypertension (IPAH) , perfusion scans are either normal or exhibit a "mottled" appearance characterized by subsegmental defects • V- scannig of the lungs is almost always normal Hasegawa I et al. AJR 2004 Fedullo PF et al. N Engl J Med 2001

  26. IN a case with CTEPH, 2008 Perfusion scan;Right lung ; 5 segmentary,3 subsegmentary, Left lung; 4 segmentary,2 subsegmentary perfusion defects

  27. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Radioisotopic V / Q scanning – 2 • Conditions indistinguishable from CTEPH in V/Q appearance : • Extrinsic vascular compression from mediastinal adenopathy or fibrosis • Primary pulmonary vascular tumors ( ie. Angiosarcoma ) • Pulmonary veno-occlusive disease • Large-vessel pulmonary arteritis • Additional imaging studies are needed to define the vascular abnormality and establish the diagnosis Hasegawa I et al. AJR 2004 Fedullo PF et al. N Engl J Med 2001

  28. Diagnosis Computed tomogaphy (CT) CT findings in CTEPH : Right atrial and ventricular enlargement Chronic thromboembolic material within dilated central pulmonary arteries Central pulmonary artery enlargement Variations in the size of lobar and segmental- level vessels Mosaic perfusion of the lung parenchyma Peripheral, scar- like densities in hypo-attenued lung regions Presence of mediastinal collateral vessels arising from the systemic arterial circulation Chronic thromboembolic pulmonary hypertension(CTEPH)

  29. Kronik tromboembolik pulmoner hipertansiyon (KTEPH) Diagnosis Computed tomogaphy (CT) - 2 CT imaging is also valuable in : • Assesment of the lung parenchyma in patients who have coexisting emphysematous or restrictive lung disease • Detection mediastinal pathology that might account for occlusion of the central pulmonary arteries

  30. Diagnosis Pulmonary angiography Pouch defects Pulmonary artery webs or bands Intimal irregularities Abrupt narrowing of the major pulmonary arteries Obstruction of lobar or segmental vessels at their point of origin, with complete absence of blood flow to pulmonary segments normally perfused by those vessels Chronic thromboembolic pulmonary hypertension(CTEPH) Fedullo PF et al. N Engl J Med 2001

  31. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Cardiac catheterization • Defines the severity of the pulmonary hypertension and degree of cardiac dysfunction • Biplane imaging provides optimal anatomical detail • When dilated and overlapping vessels are present, the lateral view provides more detailed images of lobar and segmental anatomy than those obtained with an anterior–posterior view alone Fedullo PF et al. N Engl J Med 2001 Auger WR et al. Clin Chest Med 2007

  32. Chronic thromboembolic pulmonary hypertension(CTEPH) Diagnosis Pulmonary angioscopy A diagnostic fiberoptic device, was developed specifically for preoperative evaluation. The angioscopic features of organized, chronic emboli : • Roughening or pitting of the intimal surface, • Bands and webs traversing the vascular lumen, • Pitted masses of chronic embolic material within the lumen, • Partial recanalization. • Intimal plaques are a nonspecific finding in pulmonary hypertension of any cause. • Angioscopy is performed in approximately 30 percent of patients undergoing evaluation for thromboendarterectomy, Fedullo PF et al. N Engl J Med 2001

  33. Conclusions • Up to one quarter ofpatients with acut VTE willexperience recurrent venous thromboembolism (VTE)within the subsequent 5 years • Recurrent VTE is an important problem in patients with thromboembolic events ofunknown origin (idiopathic)rather than in patients whosethrombosis is associated with acquired, transient riskfactors • The patients, those with associatedmedical diseases had the highest risk, while thosewith VTE triggered by recent trauma or surgery the lowest • RVD persistence is associated with an increasedrisk of recurrent VTE and death related to PE. Patientswith RVD persistence should receive a strict surveillancefor recurrences.

  34. Conclusions • Chronic thromboembolic pulmonary hypertension(CTEPH) is an important complication of acute VTE • The average delay from the onset of symptoms to establisment of the correct diagnosis can range from 2 to 3 years • ECO is an important laboratory method to determine the severity of the disease • V/Q sscannig gives considerable clues in the diagnosing of CTEPH • Right- heart catheterization should be considered in any patient with unexplained dyspnea and segmental or larger defects on V/Q perfusion scanning, especially if there is echocardiographic evidence of right atrial or right ventricular dysfunction

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