Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

BACKGROUND: Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. METHODS AND RESULTS: A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49-11.62; P=0.0065 and HR, 4.76; 95% CI, 1.76-12.88; P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. CONCLUSIONS: The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients.

[C-reactive protein in circulatory diseases]. / Bialko C reaktywne w chorobach ukladu krazenia.

C-reactive protein is the widely used marker in a clinical practice but also a mediator of the inflammatory process. In the introduction the authors describe the structure and function of the C-reactive protein in relation with inflammatory response to different morbidity stimulations. After this, there have been seen the oscillations of C-reactive protein plasma level of healthy people and in inflammatory, neoplastic and immunological diseases with an explanation of the clinical utilities. The current knowledge about presence of C-reactive protein in a different type of circulatory diseases, in heart failure and arrhythmia has been discussed. The importance of the role of C-reactive protein, especially high-sensivity C-reactive protein, in the prediction of circulatory diseases of the healthy members of the population and the prognostic value of C-reactive protein has been underlined.

[The role of spiral computer tomography in diagnostic algorithms in patients with suspected pulmonary embolism]. / Rola spiralnej tomografii komputerowej w algorytmach diagnostycznych u chorych z podejrzeniem zatorowosci plucnej.

The recognizing of the EP is still not satisfied because specifity and sensitivity of clinical symptoms is relatively low. SCT is an important examination in the diagnostic of pulmonary embolism and is the alternative to often nonconclusive scintigraphy and conclusive but invasive pulmonary angiography. The authors describe the role of SCT in the diagnostic algorithm of the suspected pulmonary embolism of the high and not high risk, discuss the interpretation of result of SCT due to the sort of SCT (SDCT or MDCT) and to clinical probability of PE and they discuss the problem of subsegmental PE.

Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. METHODS AND RESULTS: The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%- 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension-targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. CONCLUSIONS: Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension-targeted treatments.

[Pulmonary heart disease--cardiologist's point of view]. / Przewlekle serce plucne--punkt widzenia kardiologa.

This paper presents cardiologist's point of view on definition, diagnosis and treatment of pulmonary heart disease. Difficulties in interpretation of definition in clinical praxis and the role of medical history, physical examination, lung function tests, gasometry, chest X-ray and ECG in initial diagnosis of pulmonary heart disease are described. Author underlines the importance of echocardiographic study and pulmonary hemodynamics in confirmation of diagnosis and in differential diagnosis of "out of proportion" pulmonary hypertension. The current results of specific for pulmonary artery hypertension vasodilatory treatment in patients with pulmonary heart disease are discussed.

[Non-invasive electrophysiologic study and radiofrequency current ablation of tachycardia responsible for numerous inappropriate ICD interventions in patient with post-infarction dilated cardiomyopathy]. / Nieinwazyjne badanie elektrofizjologiczne i ablacja czestoskurczu bedacego przyczyna bardzo licznych interwencji ICD-DDD u chorego z pozawalowa kardiomiopatia rozstrzeniowa.

We present a case of 75-year-old man with numerous inappropriate interventions of an implanted cardioverter-defibrillator (ICD). In this patient with ischaemic cardiomyopathy, ICD was implanted for primary prevention of sudden cardiac death following recurrent syncope. ICD interrogation and non-invasive electrophysiologic study (NIPS) confirmed a risk of reentry-mediated tachycardia and excluded the presence of a manifest or concealed accessory pathway. Invasive electrophysiologic study revealed a wide zone that triggered atrioventricular nodal reentrant tachycardia, and ablation of the slow pathway resulted in complete remission of the arrhythmia. NIPS performed using atrial and ventricular ICD leads and subsequent ICD interrogation confirmed long-term effectiveness of the procedure. At the same time, clinical improvement of heart failure was seen, leading to a suspicion of tachycardia-induced cardiomyopathy coexisting with ischaemic cardiomyopathy.

[Dysfunction of coagulation processes in patients after acute coronary syndrome--therapeutic implications]. / Zaburzenia procesów krzepniecia u chorych po ostrym zespole wiencowym--implikacje terapeutyczne.

In the guidelines for patients with acute coronary syndrome (ACS), reperfusion, antiplatelet treatment, completed with parenteral anticoagulant are the standard therapy. It is because ACS is the result of occlusion of related artery by thrombus compound mostly of platelets, with processes of aggregation and adhesion in its pathogenesis. However, many patients after ACS experience major adverse cardiovascular events (MACE) despite optimal long term antiplatelet therapy. The possible reasons seem to be not only the resistance to this drugs but also underestimated coagulation processes. This review describes the dysfunction of particular coagulation parameters in patients with coronary artery disease and their relationship with MACE after ACS.

[Combined pulmonary fibrosis and emphysema - case report and literature review]. / Wspólistnienie wlóknienia i rozedmy pluc - opis przypadku i przeglad literatury.

We describe the case of a 61-year-old male patient, in which the search for the cause of chronic respiratory failure, severe pulmonary hypertension and secondary erythrocytosis resulted in a diagnosis of combined pulmonary fibrosis and emphysema (CPFE). This is a unique, recently characterised syndrome with upper-lobe emphysema and pulmonary fibrosis of the lower lungs. The cause is unknown, but one of the main risk factor remains smoking. The patient was a heavy smoker (over 40 pack-years). He complained of dyspnoea on exertion and cough. Physical examination revealed basal crackles and cyanosis. The patient had severe reduction in diffusing capacity, out of proportion to his lung volumes (DLCO 27% of predicted value, FEV1 2.95 l (100%), FVC 4.41 l (118%), FEV1/FVC (66%). The blood gas showed hypoxemia (pO2 37 mm Hg), hypocapnia and respiratory alkalosis. Diagnosis was based on chest computer tomography, which revealed upper lobe emphysema and lower lobe ground glass changes and honeycombing. Severe pulmonary hypertension (SPAP 80 mm Hg) was confirmed by echocardiography and right cardiac catherisation. The patient received long-term oxygen therapy, inhaled corticosteroid and Ca-blocker.

[Treprostinil therapy for distal chronic thromboembolic pulmonary hypertension]. / Obwodowa postac przewleklego zakrzepowo-zatorowego nadcisnienia plucnego. Trudnosci diagnostyczne, nowe mozliwosci terapeutyczne.

We present a case of a 77-year-old female with distal chronic thromboembolic pulmonary hypertension. Diagnostic and therapeutic difficulties are discussed. Clinical and haemodynamic benefits resulting from treprostinil therapy added to continuous anticoagulation are shown.

[Hypoxemia in pulmonary embolism--the occurrence, patomechanism and significance]. / Hipoksemia w zatorowosci plucnej--wystepowanie, patomechanizm, znaczenie.

Blood gas analysis is often performed in the initial diagnosis of acute pulmonary embolism (APE), and it is recognized that hypoxemia (H) strengthen its suspicion. However, the diagnostic power of hypoxemia is very week. Hypoxemia, usually deep, occurs in almost all patients with massive APE whereas moderate hypoxemia occurs in about 75% of unselected normotensive APE population without co-morbitides. H occurs also in most patients with chronic thromboembolic pulmonary hypertension (CTEPH). The patomechanism of H in pulmonary embolism is not completely known and is associated mainly with obstruction of pulmonary vasculature, pulmonary hypertension, and with co-morbitides. However, the secondary failure of ventilation followed by alveolar hypoxia can not be excluded in many cases of chronic pulmonary embolism. Hypoxemia seems to have moderate value in prognosis in APE and in CTEPH. H in massive APE requires oxygen therapy, and it can be speculated, whether long term oxygenation should be added to the anticoagulation in nonoperated, hypoxemic patients with CTEPH.

[Congestive heart failure and continuous murmur in patient after lumbar disc surgery--a case report]. / Zastoinowa niewydolnosc krazenia i szmer maszynowy u chorej po operacji przepukliny dysków ledzwiowych--opis przypadku.

We present a case of a 61-year-old female who was admitted to the hospital with symptoms of congestive heart failure. Diagnosis of arteriovenous fistula was suggested by the echocardiographic signs of high-output state and a continuous murmur heard especially close to the surgical scar from an intervention on the L4-L5 disc that the patient had undergone eight months before. Aortography confirmed arteriovenous fistula between the right common iliac artery and inferior vena cava. After surgical closure of the fistula, normal cardiac function was restored.

Pulmonary thromboembolism in 102 consecutive patients with chronic atrial fibrillation. Diagnostic value of echocardiography.

BACKGROUND: Little is known about the incidence and diagnosis of pulmonary thromboembolism (PE) in patients with chronic permanent atrial fibrillation (CAF). Also it has not been established if echocardiography, a diagnostic tool useful in clinical evaluation of both diseases, is of value in diagnosis of PE in CAF patients. AIM: To establish the prevalence of PE among patients suffering from CAF without or with poorly controlled anticoagulation as well as to evaluate the possibility to detect PE and to assess the diagnostic role of echocardiography. METHODS: Prevalence of PE in a population of 102 patients (52 males and 50 females at the mean age of 68 years, range 32-88 years) admitted to hospital between January and December 2004 with diagnosis of CAF was studied retrospectively. Echocardiography-based original algorithm of PE diagnosis in such patients was analysed. RESULTS: Among 102 patients with CAF, 20 (19%) cases of PE were diagnosed, including 12 with acute PE (APE) and 8 suffering from chronic thromboembolic pulmonary hypertension (CTEPH). Patients with CAF and APE as well as with CAF and CTEPH had increased right ventricular dimension (p=0.0002 and p=0.001, respectively), higher tricuspid pressure gradient (p=0.005 and p=0.001, respectively) and shorter pulmonary artery acceleration time (p=0.00006 and p=0.0004, respectively) estimated in echocardiography as compared to patients with CAF but without PE. Subjects with CAF and PE had also significantly decreased left ventricular dimension and better left ventricular performance. CONCLUSIONS: A relatively high incidence of PE among patients with CAF not treated with anticoagulants or with poorly controlled anticoagulation therapy was noted. The important value of a diagnostic algorithm employing echocardiography in a diagnosis of clinically significant APE and CTEPH in this group of patients was also shown.

[The current view on pathophysiology of pulmonary arterial hypertension]. / Aktualne spojrzenie na patofizjologie tetniczego nadcisnienia plucnego.

The article refers to actual data on pathophysiology of pulmonary arterial hypertension (PAH). PAH is a group of diseases characterized by a progressive increase of resistance and pressure in pulmonary vascular bed, leading to systolic overload and failure of the right heart ventricle, and in consequence - to premature death. The latest classification of PAH, basing on pathophysiology of the disease, was proposed in 2003 in Venice. The genetic background, including genetic mutations disturbing intracellular TGF-beta-like ligands signaling pathway, is essential for the development of PAH. In some cases secondary risk factors (e.g.: portal hypertension, congenital heart defects, HIV infection, connective tissue disorders, intoxications, myeloproliferative and storage diseases) are also important. In all types of PAH the same four pathological processes are reported: vasoconstriction, inflammation, thrombosis and remodelling. The growing knowledge of pathophysiology of PAH has enabled its better diagnosing and treatment, which has improved both symptoms and survival in the patients suffering from the disease.

[Nocturnal and diurnal oxygen saturation in patients with chronic pulmonary embolism]. / Dzienne i nocne wysycenie krwi tetniczej tlenem u chorych na przewlekla, zatorowosc plucna.

UNLABELLED: Little is known on oxygen saturation in patients with chronic pulmonary embolism. AIM OF THE STUDY WAS: Tto assess the occurrence and importance of oxygen desaturations (D) in these patients. MATERIAL AND METHODS: The study involved 58 normotensive patients with chronic pulmonary embolism (18 males, 40 females, mean age 60 years, range 22-87 years) and was carried out 6 weeks to 2 years after an acute pulmonary embolic event. During 24-hour pulse oximetry mean oxygen saturation (SpO2), and number and duration of desaturations (D), defined as at least a 6% drop of pSO2, below 88%, lasting a minimum of 8 s, were recorded. Simultaneously echocardiographic study and blood gases analysis was performed. RESULTS: Desaturations were found in 39 (67.2%) patients, whereas 79% patients had pSO2 <95% in gasometry. 27 patients had both diurnal (06(00)-22(00)) and nocturnal (22(00)-06(00)) D, 9 patients only nocturnal D, and 3 patients only diurnal D. The number (14.7 vs 36.1) and duration (733.9 vs 1528 s) of D episodes were approximately 2 times greater at night than day. There were 18 (75%) desaturators among patients with pulmonary hypertension (defined as an echocardiographic tricuspid gradient >30 mmHg), and 21 (61.8%) desaturators among patients with chronic pulmonary embolism and without pulmonary hypertension. The patients with pulmonary hypertension had a lower mean SpO2 (p=0.005) and a lower number and duration of nocturnal (p=0.008, 0.03) and diurnal (p=0.008, 0.035) D. CONCLUSION: A large number of D episodes were found in the patients with chronic pulmonary embolism, mostly at night and in the patients with pulmonary hypertension. It is unclear whether D merely reflects pulmonary vasculature embolization or whether they are capable of impacting development of chronic thromboembolic pulmonary hypertension on the basis of vicious circle. In the latter case the need for a long term oxygen therapy, or at least nighttime oxygenation, should be taken into account to slow down progression of the disease.

[Results of one year pulse oxymetry in patients with chronic thromboembolic pulmonary hypertension]. / Roczna obserwacja przezskórnego wysycenia krwi tetniczej tlenem u chorych na przewlekle zakrzepowo-zatorowe nadcisnienie plucne.

UNLABELLED: It is little known on oxygen saturation in patients with chronic thromboembolic pulmonary hypertension (CTEPH). AIM OF THE STUDY: To assess the occurrence, evolution and clinical significance of oxygen desaturations (D) during 1-year observation in CTEPH patients. MATERIAL AND METHOD: The study involved 24 consecutive patients with CTEPH (6 males, 18 females, mean age 63, range 22-75 years). During 24-hour pulse oxymetry mean oxygen saturation (SpO2), number and duration of desaturations, defined as at least a 6% drop of SO2, below 88%, lasting a minimum of 8 s, were recorded at baseline and after 6 and 12 months of follow-up. Simultaneously echocardiographic study and blood gases analysis was performed. RESULTS: At baseline 18 of 24 (75%) CTEPH patients had desaturations. During follow-up none of nondesaturators had desaturations episodes and all baseline desaturators had desaturations episodes after 6 and 12 months. In desaturators there was trend to aggravate the number and duration of diurnal but not nocturnal desaturations episodes after 6 and 12 months. It was accompanied by trend (p = 0.05) to increase of pulmonary pressure as assessed by echocardiographic study, whereas the opposite trend to decrease of pulmonary pressure was seen in nondesaturators. CONCLUSIONS: Results of the study show that desaturations occur in most patients with CTEPH and desaturators may have worse clinical course than nondesaturators. It may suggest the need for long oxygen therapy in desaturating CTEPH patients to slow down progression of the disease.

[Pulmonary hypertension in course of interstitial lung diseases]. / Nadcisnienie plucne w sródmiazszowych chorobach pluc.

Pulmonary hypertension is often and serious illness which develops in course of interstitial lung diseases. In this paper clinical classification, the basic statistical data concerning developing of pulmonary hypertension as well as contemporary opinion referred to pathogenesis of this disorder were shortly presented. Impact of pulmonary hypertension on prognosis in people with interstitial lung diseases, significance of particular markers and spirometric parameters and methods of measurement of blood pressure in pulmonary artery were also include in this article.

[Normal coronary angiogram in patient with diagnosis of acute coronary syndrome with ST segment elevation. Was it possible to recognise acute pulmonary embolism earlier?]. / Prawidlowy wynik koronarografii u pacjenta z rozpoznaniem ostrego zespolu wiencowego z uniesieniem ST zakwalifikowanego do przezskórnej interwencji wiencowej. Czy mozna bylo wczesniej rozpoznac ostra zatorowosc plucna?

A case of a 48 year old male scheduled for early invasive treatment of acute coronary syndrome with ST segment elevation is presented. Clinical suspicion of acute pulmonary embolism was undertaken because of normal coronary angiogram and was confirmed by pulmonary angiography. Differential diagnosis based on medical history, electrocardiogram and laboratory findings was discussed.

Results of one-year anticoagulation in patients with newly detected chronic thromboembolic pulmonary hypertension not treated with pulmonary endarterectomy.

INTRODUCTION: Currently pulmonary endarterectomy is considered the method of choice in patients with chronic thromboembolic pulmonary hypertension (CTEPH). It is not known if this option should be recommended in all suitable patients as it is highly variable with respect to prognosis. There is also doubt about selection of adequate time to refer patients with CTEPH for surgery. AIM: To establish whether some patients with CTEPH may clinically benefit from isolated anticoagulation with drugs and if the use of anticoagulation may have any impact on the time of patient referral for pulmonary endarterectomy. METHODS: The prospective analysis involved 29 patients (9 male, 20 female) aged 37 to 82 years, with pulmonary arterial systolic pressure ranging from 39 to 133 mmHg and newly diagnosed CTEPH who had not been treated with pulmonary endarterectomy and were not receiving anticoagulation. Survival, functional status according to NYHA classification, duration of thromboembolism, exercise tolerance and echocardiographic parameters of right ventricular overload before and at one year after initiation of therapy with anticoagulants were evaluated. RESULTS: During follow-up, 3 patients with PASP ranging from 120 to 133 mmHg died. In 26 patients with PASP 39-115 mmHg, who survived, improvement in echocardiographic parameters of right ventricular overload, better exercise tolerance as well as functional status according to NYHA classification was observed. In 12 survivors, pulmonary pressure returned to normal. CONCLUSIONS: The results of this study suggest that favourable effects of isolated anticoagulation are likely in patients with newly detected CTEPH, mild and moderate baseline pulmonary hypertension and acceptable exercise tolerance. They also indicate the necessity of anticoagulation in these patients prior to possible referral for pulmonary endarterectomy.

[Cardiac tamponade due to angiosarcoma--was surgical treatment necessary? A case report]. / Tamponada serca wywolana miesakiem naczyniowym--czy nalezalo wykonac zabieg kardiochirurgiczny?

We present a case of a 49-year-old patient with cardiac tamponade due to haemopericardium. The decision to perform surgery, although controversial, allowed to diagnose cardiac angiosarcoma with metastases to pericardium, vena cava superior and pulmonary trunk. Consequently, chemotherapy was instituted and was initially effective, however, the patient died 12 months later due to the progression of the disease.

[One-year controlled therapy with doxazosin in patients with pulmonary hypertension due to chronic obstructive pulmonary disease]. / Roczna, kontrolowana ocena stosowania doksazosyny u chorych na nadcisnienie plucne na tie przewlekle j obturacyjnej choroby pluc.

UNLABELLED: It was shown recently that a thirty day doxazosin therapy, that was undertaken to prove alpha1-adrenergic hypothesis for pulmonary hypertension, resulted in a significant improvement of pulmonary hemodynamics in patients with hypoxic pulmonary hypertension (HPH) due to COPD. AIM OF THE STUDY: The objective of this study was to learn if 12 months long therapy with 2 mg of doxazosin daily (dosage effective in recent research) may result in persistent hemodynamical and clinical benefits. MATERIAL AND METHODS: To this end, baseline and after 1 year results of echocardiography, symptom-limited maximal exercise test, and arterial blood gaseous analysis were evaluated at baseline and after 12-month study in 32 patients with HPH randomized either to doxazosin treated group (16 patients, aged 60.2 +/- 12 years, or to control group (16 patients, aging 60.5 +/- 4 years). At baseline doxazosin treated COPD patients had less advanced disease than COPD control patients (FVC 2.4 +/- 0.7 vs 1.3 +/- 0.31, p<0,001 and FEV1 1.6 +/- 0.6 vs 0.7 +/- 0.31, p<0,001). RESULTS: This study showed that results of tricuspid gradient, acceleration time, right ventricular enddiastolic diameter, left ventricular enddiastolic diameter and left ventricular ejection fraction did not differ significantly in both groups between baseline and 1-year assessment. The number of METs achieved during exercise test and results of PaO2 and PaCO2 did not change significantly in both groups either. CONCLUSION: The study indicates lack of hemodynamical and clinical improvement during a 1-year doxazosin therapy in patients with HPH due to COPD.