Clinical course of unoperated mild chronic thromboembolic pulmonary hypertension.

BACKGROUND: Pulmonary endarterectomy is the treatment of choice for a majority of patients with chronic thromboembolic pulmonary hypertension (CTEPH), however, haemodynamic criteria for the surgery have not been established. It is still unknown whether patients with mild CTEPH should be operated on and what is the clinical course of unoperated, anticoagulated mild CTEPH. AIM: To determine the clinical course in chronically anticoagulated, unoperated patients with mild CTEPH. METHODS: A single-centre, non-randomised, follow-up study involved 10 anticoagulated, unoperated patients (3 males and 7 females aged 46 to 77 years) with mild CTEPH (mean pulmonary artery pressure [MPAP] £ 30 mm Hg and pulmonary vascular resistance £ 300 dynes x s x cm(-5), ≥ 2 METs achieved during symptom limited treadmill exercise test) selected from 73 consecutive patients with CTEPH. The 3-year follow up included yearly echocardiographic evaluation of pulmonary artery systolic pressure (PASP), right ventricular end-diastolic diameter (RVEDD) and acceleration time (AcT) as well as NYHA functional class and symptom-limited treadmill exercise test. RESULTS: All the patients survived the 3-year follow-up. The PASP, RVEDD and AcT (mean ± SD) at baseline and at the end of follow-up were 43.9 ± 6.1 mm Hg and 25.6 ± 8.0 mm Hg, p = 0.0017, 25.4 ± 4.9 mm and 17.8 ± 3.82 mm, p = 0.00006, 68.3 ± 10.0 ms and 104.4 ± 16.48 ms, p = 0.0004, respectively. The NYHA functional class improved from 2.1 ± 0.32 to 1.3 ± 0.48, p = 0.002 and was accompanied by the trend to improve results of exercise test. CONCLUSIONS: The results of the study suggest that patients with mild CTEPH can be treated successfully by anticoagulation alone with excellent 3-year survival rate, improved functional status and with gradual decrease of pulmonary pressure and right ventricular overload.

[Chronic thromboembolic pulmonary embolism not preceded by clinical thromboembolic event - clinical, echocardiographic and haemodynamic course]. / Przebieg przewleklego zakrzepowo−zatorowego nadcisnienia plucnego niepoprzedzonego incydentem zatorowym ­ ocena kliniczna, echokardiograficzna i hemodynamiczna.

Chronic thromboembolic pulmonary embolism not preceded by clinical thromboembolic event(s) in a 50 year-old male with a history of three-year dyspnea on exertion was detected. Proximal chronic pulmonary embolism, mean pulmonary artery pressure 32 mm Hg, and pulmonary vascular resistance of 444 dyn x s(-1) x cm(-5) were found. We describe a 11-year clinical course of anticoagulated patient with monitoring of functional status and echocardiography and with control of pulmonary haemodynamics at the end of follow-up.

[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.

[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.

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.

[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.

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.

[Incidence, pathogenesis and importance of nocturnal hypoxaemia in patients with chronic obstructive pulmonary disease]. / Wystepowanie, patogeneza i znaczenie nocnych hipoksemii u chorych na przewlekla obturacyjna chorobe pluc.

Noctural hypoxaemia (NH) in patients with chronic obstructive pulmonary disease (COPD) was first described over 40 years ago, however, its importance is not fully established. NH is defined most frequently as a drop in oxygen saturation below 90% and it occurs in almost all COPD patients PaO2 < 55 mmHg and in 30-40% of those with PaO2 < 60 mmHg. The main reason for NH is alveolar hypoventilation which is most pronounced during ohase REM. It is known that even short term episodes of NH increase pulmonary artery pressure, yet, the impact of chronic, isolated NH on pulmonary hypertension development was not found. Also, the relationship of induced by NH cardiac arrythmias with sudden death in COPD was not documented and association of NH with prognosis in COPD was not proved. Actually, there is no need to perform NH examination routinely. According to the ATS/ERS latest standards, the determination of NH can be helpful to qualify COPD patients with PaO2 55-65 mmHg to longterm oxygen therapy.

[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.

[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.

[Characteristics of acute, hemodynamically stable pulmonary embolism presenting as a syncope]. / Charakterystyka ostrej, hemodynamicznie stabilnej zatorowosci plucnej ujawniajacej sie omdleniem.

Syncope (S) occurs in approximately 10% patients with acute pulmonary embolism (APE) and is commonly ascribed to the massive, hemodynamically instable APE. The aim of the study was to assess the occurrence and significance of S revealing hemodynamically stable APE. We found syncope in 6 of 21 (29%) consecutive patients (16 females, 5 males; age from 46-87 years, mean age of 71 years) who were diagnosed with APE and in whom other reasons for S were excluded. All patients were treated with anticoagulation. They all survived hospitalization and no APE recurrences were found during in-hospital period. Patients with APE-S compared to patients with APE and without S had smaller baseline RVED (21.2 +/- 2 vs. 27.3 +/- 5.6, p = 0.01), however both groups did not differ statistically in baseline vital signs, angiographic, hemodynamic, other echocardiographic parameters as well as in the results of laboratory findings. It is concluded, that S signals hemodynamically stable APE more frequently than is quoted. APE-S patients could not be clearly discriminate from APE patients without S on the basis of the parameters studied and S did not impact the course of APE during in- hospital period.

[Bronchial adenocarcinoma mimicking acute pulmonary embolism -- a case report]. / Gruczolowy rak oskrzelikowy nasladujacy ostra zatorowosc plucna.

A case of a 31-year-old male with medical history, clinical symptoms and additional diagnostic tests strongly suggesting diagnosis of acute pulmonary embolism is presented. However, pulmonary emboli were not seen during pulmonary angiography. At autopsy bronchial carcinoma was found and this definitive diagnosis was confirmed by the results of histopathological study.

[Impact of 30 day therapy with doxazosin--alpha1 adrenergic blocker on pulmonary hemodynamics in patients with secondary pulmonary hypertension due to chronic obstructive pulmonary disease]. / Wplyw 30-dniowego leczenia alpha1 adrenolitykiem--doksazosyna--na hemodynamike plucna u chorych z nadcisnieniem plucnym w przebiegu przewleklej obturacyjnej choroby pluc.

To prove alpha1-adrenergic hypothesis for pulmonary hypertension we studied 6 patients aged 63.3 +/- 8 (range 50-75) years with stable, advanced chronic obstructive pulmonary disease (COPD), FEV=1.51 +/- 0.3 I and with pulmonary hypertension, mean pulmonary artery pressure 28.3 +/- 8 (range 21-40) mm Hg. a1 blocker, doxazosin was administered per os in a daily dose of 2-3 mg. Pulmonary hemodynamics was performed before and after 30 day of treatment. After therapy, compared to baseline results, pulmonary artery systolic pressure decreased from 48.6 +/- 14 to 42.5 +/- 13 mm Hg, p=0.003, pulmonary artery diastolic pressure from 14.5 +/- 6 to 7 +/- 4 mm Hg, p=0.02), mean pulmonary artery pressure from 28.3 +/- 8 to 21.1 +/- 5 mm Hg, p=0.01 and pulmonary vascular resistance from 389.6 +/- 14 to 234.6 +/- 73 dyn x s x cm5. No significant changes in pulmonary artery wedge pressure, cardiac output, arterial oxygen content, percent saturation of hemoglobin with oxygen and oxygen delivery were found. In summary, the results of 30 day doxazosin therapy support alpha1 adrenergic hypothesis for pulmonary hypertension and suggest potential clinical benefits during long term treatment in patients with pulmonary hypertension due to COPD.

Electrocardiographic signs of right ventricular overload in patients who underwent pulmonary embolism event(s). Are they useful in diagnosis of chronic thromboembolic pulmonary hypertension?

Diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) remains a major clinical problem. An attempt was made to learn whether electrocardiography has the potential to alleviate that problem. Sensitivity, specificity, negative and positive predictive value as well as a likelihood ratio of electrocardiogram (ECG) signs of right ventricular overload (RVO) were determined in 56 patients with chronic pulmonary embolism and a mean pulmonary artery pressure (mPAP) ranging from 15 to 80 mm Hg. CPE was recognized as the only disease in 44 patients (Group A) whereas the remaining 12 CPE patients suffered from concomitant cardiac and pulmonary diseases (Group B). Thirty three (59%) of the patients were diagnosed with CTEPH (mPAP exceeding 30 mm Hg). Twelve ECG signs of RVO were identified in the entire population of patients. At least a single ECG sign of RVO was found in 72% of the patients and 7 ECG signs were found exclusively in CTEPH patients. Negative T wave in precordial V1-V5 leads, negative T wave in II, III, aVF, pulmonary P wave and right axis deviation >90% occurred with the highest incidence that was determined to be 43%, 32%, 30%, and 30% respectively. These ECG signs of RVO had positive predictive value of those signs ranged from 80 to 100% in Group A and 25 to 75% in Group B, whereas their negative predictive value ranged from 44 to 76% and from 66.5 to 87.5% respectively. It is concluded that ECG signs of RVO appear to have the potential to aid in diagnosing CTEPH in the patients who underwent acute embolic event in the past.

[Infective endocarditis--still a great challenge. Two case reports]. / Infekcyjne zapalenie wsierdzia - wciaz wielkie wyzwanie. Opis dwóch przypadków.

We present two patients with aortic valve disease who developed acute infective endocarditis. In both patients the disease started with infection of the upper respiratory tract. The patients were treated with antibiotics due to pneumonia. The diagnosis of infective endocarditis was established 4 months and 9 weeks after the onset of infection. The first patient died whereas the second underwent successful aortic valve replacement.

[Effect of a 2-year pulmonary rehabilitation on the 7-year prognosis in patients with advanced chronic obstructive pulmonary disease]. / Wplyw 2-letniej rehabilitacji oddechowej na 7-letnie rokowanie u chorych z zaawansowana przewlekla obturacyjna choroba pluc.

Pulmonary rehabilitation (PR) improve the quality of life in COPD patients, however, little has been known whether it may improve the survival. We assessed 7-year prognosis in 46 patients with advanced COPD, 27 of which completed successfully 2-year course of PR whereas 19 COPD patients made a control (C) group. At the end of 7-year observation 41.3% of total number of patients survived, among them 12 (42.8%) PR patients and 7 (38%) C patients. COPD progression was the main (80%) reason of mortality in both groups. The survivors had better results of baseline FVC, FEV1 and treadmill exercise test. They did not differ from non-survivors with age, baseline results of the weight, PaO2, PaCO2 and the number of PR patients. The Cox proportional hazard analysis showed that neither of baseline parameters influenced significantly the 7-year prognosis in this group of COPD patients. In conclusion, PR failed to improve 7-year prognosis in the studied group of patients with advanced COPD.

[Oxygen saturation at rest, on exercise and during sleep in COPD patients undergoing pulmonary rehabilitation program. Two years prospective controlled study]. / Kontrolowane badanie przezskórne wysycenia tlenem krwi tetniczej w czasie dnia, w nocy oraz w czasie wysilku u rehabilitowanych chorych na POChP.

The role of pulmonary rehabilitation (PR) in COPD patients is well established. However, the PR programs includes physical exercise that may deteriorate chronic hypoxemia. The objective of the study was to evaluate impact of a long term PR program on oxygen saturation at rest, on exercise and during sleep in COPD patients no receiving supplemental oxygenation. A two--year controlled study involved 27 rehabilitated (PR-COPD patients (6 females) aged 32-76 years, mean 58.1 years on average and 19 control (C-COPD patients (3 females) aged 58-83 years, mean 68. COPD patients participating in PR program patients were younger, had higher value of FVC and FEV1 and better results of METs during symptom-limited exercise test (ET, p = 0.007). They also had better results of transcutaneus oxygen saturation (SaO2) at rest (p = 0.02), a lower maximal decrease in SaO2 during ET(p = 0.003) and a higher value of medan SaO2 during (p = 0.02). Following PR program the number of METs achieved during ET increased in PR-COPD patients (p = 0.007) and did not change in C-COPD, p = NS. The SaO2 at rest did not change significantly in both groups. Maximal drop in SaO2 during ET decreased in PR-COPD patients (p = 0.02) and did not change in C-COPD(p = NS) group. Median SaO2 at sleep did not change in PR-COPD patients (p = NS) but it decreased in C-COPD patients (p = 0.02). The study indicates that long term PR does not deteriorate the resting diurnal hypoxaemia, it prevent COPD patients from time depending exacerbation of nocturnal hypoxaemia and can improve oxygen saturation on exertion. The results could be influenced by baseline differences between PR-COPD and C-COPD patients.

[Electrocardiographic signs of right ventricular overload useful in improving diagnosis of chronic thromboembolic pulmonary hypertension (CTE-PH) in patients with chronic exertional dyspnea]. / Elektrokardiograficzne cechy przeciazenia prawej komory serca przydatne w rozpoznawaniu nadcisnienia plucnego na tle przewleklej choroby zakrzepowo-zatorowej u chorych z przewlekla dusznoscia wysilkowa.

The early recognition of chronic thromboembolic pulmonary hypertension (CTE-PH) is troublesome because of "honey moon" period with chronic dyspnea on exertion as the only one symptom. To find if routine electrocardiography may be useful in improving the diagnosis, value of right ventricular overload (RVO) signs in recognition echocardiographically determined chronic pulmonary hypertension (CPH) and angiographically confirmed CTE-PH in 90 consecutive patients hospitalized due to chronic exertional dyspnea was studied. CPE was found in 76.5% and CTE-PH in 33% of those patients, at least one of twelve founded electrocardiographic signs of RVO was present in 50% patients. The most frequently occurred signs: negative T wave in lead V1-V6, right axis deviation and pulmonale P wave as well as 9 other occurred at frequency below 20% signs of RVO had low to medium sensitivity and negative predictive value and 80-90% positive predictive value for diagnosing CPE and 90-100% positive predictive value for diagnosing CTE-PH. It was concluded that in patients with chronic exertional dyspnea electrocardiographic signs of RVO may be useful in initial diagnosing of CPH and CTE-PH in particular.