Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?

INTRODUCTION: Pulmonary hypertension (PH) is a well-recognised complication of interstitial lung diseases (ILD), which worsens prognosis and impairs exercise capacity. Echocardiography is the most widely used, non-invasive method for PH assessment. The aim of our study was to identify the factors predictive for echocardiographic signs of PH in newly recognised ILD patients. METHODS: Ninety-three consecutive patients (28F/65M) with different ILD were prospectively evaluated from January 2009 to March 2014. Pulmonary function testing, 6-min walk distance (6MWD), initial and sixth minute room air oxygen saturation, NT-proBNP and echocardiography were assessed in each patient. Echocardiographic PH probability was determined according to the 2009 ESC/ERS guidelines. RESULTS: In 41 patients (Group B) increased PH possibility has been diagnosed on echocardiography, in 52 patients (Group A)-low PH probability. Most pronounced differences (p ≤ 0.0005) between groups B and A concerned: age, 6MWD, room air oxygen saturation at 6 min, DLCO and TLC/DLCO index (57.6 vs 43.8 years; 478 vs 583 m; 89.1% vs 93.4%; 54.8% predicted vs 70.5% predicted and 1.86 vs 1.44; respectively). Univariate analysis showed four-fold increased probability of PH when TLC/DLCO exceeded 1.67. A scoring system incorporating age, TLC/DLCO index, 6MWD and room air oxygen saturation at 6 min provided high diagnostic utility, AUC 0.867 (95% CI 0.792-0.867). CONCLUSION: ILD patients with TLC/DLCO index > 1.67 have a high likelihood of PH and should undergo further evaluation. The composite model of PH prediction, including age, 6-min walk test and TLC/DLCO was highly specific for recognition of PH on echocardiography.

Sarcoidosis-associated pulmonary hypertension treated with sildenafil - a case report.

Development of sarcoidosis-associated pulmonary hypertension (SAPH) significantly worsens prognosis in sarcoidosis patients. Unfortunately, there is no treatment of proven benefit for this condition. Medications used for treatment of pulmonary arterial hypertension are of great interest in this respect. Here, we report a case of a patient with severe SAPH treated with sildenafil. A significant, but only temporary improvement in functional status was observed, and the patient died of gradually progressing heart and respiratory failure while awaiting for lung transplantation.

"The significance of cardiac magnetic resonance imaging in detection and monitoring of the treatment efficacy of heart involvement in eosinophilic granulomatosis with polyangiitis patients".

BACKGROUND: Cardiac magnetic resonance imaging (CMRI) has emerged as a sensitive and non-invasive technique in the evaluation of cardiac lesions in eosinophilic granulomatosis with polyangiitis (EGPA) patients. OBJECTIVES: To evaluate the ability of CMRI to detection and monitoring of the treatment efficacy in EGPA patients with cardiac involvement. METHODS: To the retrospective-prospective study were enrolled 33 cardiac involvement EGPA patients. In 19 of them CMRI at the moment of diagnosis was performed, in 14 - CMRI after treatment was made, when this method was available - in this group the cardiac involvement was based on the clinical findings. All patients were treated with corticosteroids (CSs) and/or cyclophosphamide (CY). In the first group the control CMRI after one year of treatment was performed, but in the second group the time from the end of the treatment to execution of CMRI was 2-5 years. RESULTS: All patients had heart injury in CMRI. Myocardial edema was present in 87.8% cases, 54.5% of patients had perfusion defects and in all - late gadolinium enhancement was observed. Control CMRI was performed in 32 cases. Improvement was observed in 81% of patients - in 11% of them all lesions undergone completely remission and in 35% of them evolution to global fibrosis was found. In 7% of patients stabilization was achieved and in 12% - progression was observed. CONCLUSIONS: CMRI is a sensitive method detecting cardiac lesions in EGPA patients. It helps to detect patients, who need combined therapy and helps evaluate the therapeutic effect.

Non-high risk PE in the patients with acute or exacerbated respiratory disease: the value of the algorithm based on D-dimer evaluation and Revised Geneva Score.

INTRODUCTION: The diagnostic algorithm of non-high risk pulmonary embolism (PE) is based on probability scoring systems and plasma D-dimer (DD) assessment. The aim of the present study was to investigate the efficacy of Revised Geneva Scoring (RGS) and DD testing for the excluding of non-high risk PE, in the patients admitted to the hospital due to acute respiratory diseases. MATERIAL AND METHODS: The consecutive patients, above 18 years of age, referred to the department of lung diseases, entered the study. The exclusion criteria were: the pregnancy and the suspicion of high risk PE. Plasma DD was measured with quick ELISA test, VIDAS D-dimer New, bioMerieux, France. Multislice computed tomography angiography was performed in all of the patients. RESULTS: 153 patients, median age 65 (19-88) years entered the study. The probability of PE was: low - in 58 patients (38%), intermediate - in 90 (59%), high - in 5 (3%). DD < 500 ng/ml was found in 12% of patients with low and intermediate probability of PE. PE was recognized in 10 out of 153 patients (7%). None of the patients with DD < 500 ng/ml was diagnosed with PE (NPV 100%). Median DD value was significantly higher in PE patients comparing to non-PE (4500 ng/ml and 1356 ng/ml respectively, p = 0.006). CONCLUSION: In the group of the patients with acute respiratory symptoms, low or intermediate clinical probability scoring combined with normal DD had a high NPV in excluding PE. Nevertheless, such approach was not very effective, as the increased DD was noted in 88% of the examined population.

Severe congestive heart failure as the main symptom of eosinophilic granulomatosis and polyangiitis (Churg-Strauss syndrome).

Patients with cardiovascular symptoms are mainly diagnosed in cardiological wards. However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3. High eosinophilia is connected with production of cytotoxic eosinophilic proteins which can cause eosinophilic vasculitis or eosinophilic myocarditis. One of the better known hypereosinophilic syndromes is EGPA described by the pathomorphologists Churg and Strauss. The further research works allowed for the clinical characteristics of patients with EGPA. In the course of this disease the following three phases were recognized : prodromal-allergic, eosinophilic, vasculitic. The definitive diagnosis can be established only in the third phase, when vasculitis causes organ involvement. Besides symptoms of the respiratory tract (asthma, nasal polyps, eosinophilic lung infiltrations) also cardiovascular symptoms, gastrointestinal tract symptoms, as well as skin lesions and kidneys involvement can appear. The most dangerous for patients is involvement of the nervous and cardiovascular systems. We present a patient with asthma and eosinophilia in whom EGPA was diagnosed in the course of acute recurrent substernal chest pain, with subsequent signs of cardiac insufficiency.

[Severe hypoxemic respiratory insufficiency in a patient with hepato-pulmonary syndrome coexisting with interstitial lung disease of unknown etiology]. / Ciezka hipoksemiczna niewydolnosc oddechowa u chorego z zespolem watrobowo-plucnym i choroba sródmiazszowa pluc o nieznanej etiologii.

The coexistence of the interstitial lung disease and respiratory failure is rarely associated with extrapulmonary pathology. In patients with liver cirrhosis, hypoxemia may develop in the course of hepato-pulmonary syndrome (HPS), but radiological pathology seen in the course of HPS is of vascular origin, and thus typically not classified as interstitial lung disease. We present a patient with severe hypoxemic respiratory insufficiency in whom hepato-pulmonary syndrome coexisted with interstitial lung disease of unknown etiology. The mechanisms of hypoxemia in the course of hepatic diseases and reasons of possible coincidence of lung and hepatic pathology are discussed.

[Constrictive pericarditis as complication of viral respiratory infection]. / Zaciskajace zapalenie osierdzia jako powiklanie wirusowej infekcji dróg oddechowych.

A 24 year-old man with 3-months medical history of recurrent respiratory infections and pericardial effusion, despite treatment with nonsteroid anti-inflammatory drugs, was admitted to the hospital with dyspnea on exertion. On admission he presented the symptoms of right heart insufficiency. Computed tomography of the chest demonstrated a thickened pericardium. Echocardiographic examination and right heart catheterisation established the diagnosis of constrictive pericarditis. Serologic tests suggested viral aetiology. The patient was referred to cardiothoracic surgery, partial pericardiectomy was performed with marked haemodynamic improvement.

[Normal D-dimer concentration in hospitalized patients with lung diseases]. / Czestosc prawidlowego stezenia D-dimerów u pacjentówhospitalizowanych z chorobami pluc.

INTRODUCTION: The use of D-dimer testing is an established part of the diagnosis of suspected pulmonary embolism (PE). However, in hospitalized patients many various factors might be responsible for increased D-dimer concentration and they could lower utility of D-dimer in exclusion of PE in such population. According to some published data, calculating the index D-dimer/fibrinogen could increase the specificity of D-dimer in the recognition of venous thromboembolism (VTE). The aim of the present study was to determine the frequency of normal D-dimer concentration in hospitalized patients with lung diseases in whom the differential diagnosis of PE is particularly difficult and to evaluate the utility of the index D-dimer/fibrinogen in subgroups of patients: with acute VTE and with lung cancer. MATERIAL AND METHODS: 619 consecutive patients aged 54.9 (± 15.4) hospitalized in reference pulmonary center were enrolled into observation. Among them, there were 96 (15%) patients with acute VTE, 65 (10%) with exacerbation of COPD and 172 (27%) with lung cancer. RESULTS: Mean D-dimer concentration (Vidas D-dimer New) was 1956 ± 3691 ng/ml and median value 842 (45-35 678) ng/ml. Normal D-dimer concentration (〈 500 ng/ml) was found in 225/523 (43%) without acute VTE. In 49% (32/65) patients with COPD and in 25% (43/172) patients with lung cancer D-dimer concentration was below 500 ng/ml as well. The index D-dimer/fibrinogen was significantly higher in acute VTE patients compared to lung cancer patients - 808 ± 688 and 289 ± 260 respectively, p 〈 0.001. CONCLUSIONS: Normal D-dimer concentration was found in more than 40% of patients with lung diseases hospitalized in reference pulmonary center. This observation could suggest higher than described in the literature utility of D-dimer measurement in exclusion of PE in such a population. The value of the index D-dimer/fibrinogen, which is significantly higher in acute VTE than in lung cancer requires further evaluation to establish its clinical utility.

[The basis of Churg-Strauss syndrome diagnosis in own material]. / Podstawy rozpoznania zespolu Churga-Strauss w materiale wlasnym.

INTRODUCTION: The diagnosis of Churg-Strauss syndrome (CSS) is difficult because pathological criteria are present in minority of patients and in advanced stages. Several centers elaborated criteria which allowed to suspect CSS in patients with asthma, hypereosinophilia and clinical manifestations consistent with systemic vasculitis with or without histologic evidence. The aim of the study is the presentation of the basis of CSS diagnosis in our material. MATERIAL AND METHODS: The analysis included 38 patients. Blood and biochemical analysis, radiological examinations of the chest and sinuses, echocardiography were performed in all patients. In 22/23 patients with cardiac symptoms magnetic resonance of the heart was performed. In two patients mediastinoscopy was performed, in 4--laparotomy. RESULTS: Only in 13/38 patients vasculitis was histologically proven, in the rest the diagnosis was established mainly on clinical ground. In 23 patients the diagnosis was established on the clinical cardiac symptoms--in all of them the cardiac involvement was confirmed by magnetic resonance. In 9 cases skin leasions were cause of further diagnostic procedures, 6 patients presented gastrointestinal symptoms, in 15--paralysis of peroneal nerve was observed, and 1 patient had polyneuropathy and symptoms of central nervous system. CONCLUSIONS: The diagnosis of CSS in our material was established mainly on clinical ground, because histologic material was difficult to obtain (it needs invasive procedures). Only in 13 from 38 patients vasculitis was histologically proven. The presence of asthma, sinusitis associated with peripheral eosinophilia and organ symptoms suggesting a vasculitis could support the diagnosis, without histologic evidence.

Manageability of acute severe heart failure complicated with left ventricular thrombosis during therapy for breast cancer.

Trastuzumab-related cardiac dysfunction may be manageable and completely reversible with suitable cardiac medication, allowing optimal breast cancer treatment to continue. We present the case of a 42-year-old woman who developed severe systolic left ventricular failure with impaired contractility of the right ventricle, pulmonary hypertension, and clots in the left ventricular cavity during adjuvant treatment for breast cancer. The patient was initially diagnosed with early breast cancer and underwent surgery on her left breast. She received 6 cycles of anthracycline chemotherapy followed by radiation therapy in the left breast area, then 5 cycles of trastuzumab. After the fifth cycle of trastuzumab, she experienced dyspnoea and leg edema. Fluid was detected in the pleural cavities but no lung metastases were identified. Echocardiography was performed, revealing a severely reduced left ventricular ejection fraction (10%) with impaired contractility of the right ventricle and pulmonary hypertension. Standard medication for heart failure resulted in complete recovery of normal systolic and diastolic function of the left and right ventricles. The combination of low molecular weight heparin and acetylsalicylic acid completely resolved the thrombotic complications. The patient regained her full range of social, occupational, and family activities. This case study is the first to demonstrate the manageability and reversibility of trastuzumab-related cardiac complications in a patient who had developed severe heart failure complicated with left ventricular thrombosis during sequential anthracycline and trastuzumab therapy for breast cancer. The findings contradict other opinions that trastuzumab-related acute heart failure is analogous to stunning or hibernation and recovers without specific cardiac treatment.

[Acquired methemoglobinemia--case report]. / Nabyta methemoglobinemia--opis przypadku.

Methemoglobinemia; an increased concentration of methemoglobin in the blood, is an altered state of hemoglobin whereby the ferrous form of iron is oxidized to the ferric state, rendering the heme moiety incapable of carrying oxygen. The authors present a case of 49-year-old man who was admitted to the department of chest medicine with dyspnea, weakness and cyanosis in whom differential diagnosis excluded acute and chronic pulmonary and cardiovascular disease. Clinical cyanosis and low measured oxygen saturation in the presence of normal arterial oxygen tension was highly suggestive of methemoglobinemia ("saturation gap"). Methemoglobin level, measured at the acute phase of disease was elevated at 16%. Episode resolved spontaneously. Causes of methemoglobinemia was not established.

[Chest ultrasound in the diagnosis of pulmonary embolism in a pregnant patient - a case report]. / Ultrasonografia klatki piersiowej w diagnosytce zatorowosci plucnej u ciezarnej - opis pryzpadku.

Pregnancy is a risk factor for both pulmonary embolism (PE), and an incorrect diagnostic assessment in cases of suspected PE with potentially dangerous consequences for the mother and foetus. The major concern is ionising radiation utilized by diagnostic tests and its potential negative effect on foetal safety. This paper presents diagnostic difficulties encountered in a 31-year-old patient at 20 weeks of gestation who was admitted to hospital with non-specific chest pain and suspected PE as a complication of right lower limb venous thrombosis. The case study reminds of chest ultrasound as a useful tool in the diagnosis of PE. The official clinical practice guidelines do not recommend the use of chest ultrasound for diagnosing of PE due to lack of a sufficient number of published studies. This case report may encourage further, prospective studies in the hope to define whether and when chest ultrasound might find its place in the diagnostic strategy of PE, especially in pregnant women.

Stress Doppler echocardiography in relatives of patients with idiopathic and familial pulmonary arterial hypertension: results of a multicenter European analysis of pulmonary artery pressure response to exercise and hypoxia.

BACKGROUND: This large, prospective, multicentric study was performed to analyze the distribution of tricuspid regurgitation velocity (TRV) values during exercise and hypoxia in relatives of patients with idiopathic and familial pulmonary arterial hypertension (PAH) and in healthy control subjects. We tested the hypothesis that relatives of idiopathic/familial PAH patients display an enhanced frequency of hypertensive TRV response to stress and that this response is associated with mutations in the bone morphogenetic protein receptor II (BMPR2) gene. METHODS AND RESULTS: TRV was estimated by Doppler echocardiography during supine bicycle exercise in normoxia and during 120 minutes of normobaric hypoxia (FIO(2)=12%; approximately 4500 m) in 291 relatives of 109 PAH patients and in 191 age-matched control subjects. Mean maximal TRVs were significantly higher in PAH relatives during both exercise and hypoxia. During exercise, 10% of control subjects but 31.6% of relatives (P<0.0001) exceeded the 90% quantile of mean maximal TRV seen in control subjects. Hypoxia revealed hypertensive TRV in 26% of relatives (P=0.0029). Among control subjects, TRV at rest was not related to age, sex, body mass index, systemic blood pressure, smoking status, or heart rate. Within kindreds identified as harboring deleterious mutations of the BMPR2 gene, a hypertensive TRV response occurred significantly more often compared with those without detected mutations. CONCLUSIONS: Pulmonary hypertensive response to exercise and hypoxia in idiopathic/familial PAH relatives appears as a genetic trait with familial clustering, being correlated to but not caused by a BMPR2 mutation. The suitability of this trait to predict manifest PAH development should be addressed in long-term follow-up studies.

Accuracy of compression ultrasound in screening for deep venous thrombosis in acutely ill medical patients.

The value of vein ultrasonography for diagnosis of symptomatic deep vein thrombosis (DVT) is widely accepted. We are unaware of published data comparing ultrasonography with the "gold standard" of venography for DVT diagnosis in asymptomatic persons in the patient group of acutely ill medical patients. It was the objective of this study to evaluate sensitivity and specificity of compression ultrasound (CUS) examinations in the diagnosis of proximal and distal DVT in acutely ill medical patients [with congestive heart failure (NYHA class III and IV), exacerbations of respiratory disease, infectious disease, and inflammatory diseases] considered to be at moderate risk of venous thromboembolism (VTE). CUS examination was performed prior to ascending venography on day 6-15 of the hospital stay. Both investigations were done on the same day, each interpreted without knowledge of the other's result. Proximal and calf veins were separately evaluated. Technically satisfactory venography was obtained in 160 patients. In 12 of 160 patients (7.5%, 95% CI=[4.0%-12.7%]), venography confirmed the presence of DVT, all of which was asymptomatic. Proximal DVT was detected in five patients (3.1%, 95% CI=[1.0%-7.1%]) and distal DVT in seven patients (4.4%, 95% CI=[1.8%-8.8%]). CUS of proximal veins was technically satisfactory in all 160 patients and CUS of distal veins in 150 patients. In three of five patients with venographically proven proximal DVT, the diagnosis was confirmed by CUS (sensitivity 60%, 95%CI=[23%-88%]). In one patient, the CUS was false positive (specificity 99.4%, 95%CI=[96%-99%]). Positive and negative predictive values (PPV and NPV) of CUS in the diagnosis of proximal DVT were 75% (95%CI=[30%-95%]) and 98% (95% CI=[95%-99%]), respectively. In two of seven patients with venographically proven calf DVT, the diagnosis was confirmed by CUS (sensitivity 28.6%, 95%CI=[8%-64%]) and in two patients, CUS was false positive (specificity 98.6, 95%CI=[95%-99%]). PPV and NPV of CUS in diagnosis of distal DVT were 50% (95%CI=[15-85%]) and 96% (95% CI=[92%-98%]), respectively. In conclusion, CUS underestimates the incidence of proximal and distal DVT compared to contrast venography in acutely ill medical patients without thrombosis symptoms.

[Value of spiral computed tomography in pulmonary hypertension]. / Ocena wartosci spiralnej tomografii komputerowej w nadcisnieniu plucnym.

UNLABELLED: Pulmonary hypertension (PH) represents an important diagnostic problem, because of its non-specific clinical presentation. AIMS OF THE STUDY: Estimation of sensitivity and specificity of spiral computed tomography (SCT) in diagnosis of PH. Estimation of the influence of the cause and character of PH on morphology of pulmonary arteries and right ventricle-pulmonary artery coupling. MATERIAL AND METHODS: 102 patients with clinical suspicion of acute pulmonary embolism (APE) or chronic PH (54F, av. age 51.3 +/- 17.3). Diameters of pulmonary arteries obtained in SCT, systolic pulmonary pressure calculated from the tricuspid gradient pressure, and indices of the right ventricle-pulmonary artery dynamical coupling (AcT) obtained from the echo-Doppler method were compared. RESULTS: Increased diameter (> 18 mm) of interlobar arteries was the most specific sign of PH (specificity 92%, sensitivity 45%). Diameter of proximal pulmonary arteries and pulmonary pressure was correlated in patients with APE (r = 0.487, p < 0.003), but not in those with chronic PH (r = 0.223). In a group of patients with pulmonary arterial hypertension (PAH), enlargement of proximal pulmonary arteries seemed to result in less disturbed right ventricle-pulmonary artery dynamic coupling. CONCLUSIONS: 1. SCT measurements of diameters of pulmonary arteries have relatively low sensitivity for PH, precluding its reliable exclusion. 2. The relationship between the pulmonary pressure and the diameters of pulmonary arteries was found in acute but not in chronic PH, in which right ventricle-pulmonary artery dynamical coupling seemed less disturbed when pulmonary arteries were markedly dilated.

[Right ventricle structure and function in patients with obstructive sleep apnea (OSA)]. / Budowa i funkcja prawej komory u chorych na obturacyjny bezdech senny (OBS).

UNLABELLED: The structure and function of right ventricle was echocardiographically assessed in patients (pts) with OSA and analyzed taking into account the presence of obesity and/or systemic hypertension (SH). Therefore, 118 pts (98 M, 20 F, aged 48,5 8,4 yr) were divided into OH = obese with z OSA and SH (n = 22), ON = obese with OSA, and no SH (n = 20), OC = OSA without either obesity and SH (n = 21), GH = obese without OSA but with SH (n = 18), GN = obese without either OSA or SH (n =17). Control group (ZZ) comprised 20 healthy subjects (14M,6F). Right ventricular diameter (RVD), diastolic (DRVW), and systolic right ventricle free wall thickness (SRVW) were measured and its systolic thickening (ST-RVW) was calculated. Acceleration time of ejection into pulmonary artery (AcT) was measured with Doppler echocardiography. RESULTS: St. sign. p < 0.05 between: 1: all groups except OH and ON, GH and GN, GH and OC, GN and OC; 2--like 1 except ZZ and OC; 3--OH and all subgroup except ON, ON and GH, ON and ZZ, OC and GN; 4-- ON and all subgroups except OH, OH and OC, OH and GH, OH and GN. CONCLUSIONS: Enlargement and functionally compromised RV is found in OBS mostly with concomitant obesity. Pulmonary hypertension at rest in patients with isolated OSA is rare and also requires additional contributing factors.

[Effect of localization of pulmonary arterial changes on echocardiographic indices of right heart function and their correlation with exercise capacity: comparison of arterial and thromboembolic pulmonary hypertension]. / Wplyw umiejscowienia zmian w lozysku plucnym na echokardiograficzne wskazniki czynnosci prawego serca i ich zwiazek z tolerancja wysilku: porównanie tetniczego i zakrzepowo-zatorowego nadcisnienia plucnego.

AIM: To assess correlation between exercise tolerance and Doppler-derived indices of right ventricular (RV)--pulmonary arterial coupling in patients with different location of vascular lesions causing pulmonary hypertension (PH). MATERIAL: 45 patients: 21 with thromboembolic (TE-PH) and 24 with arterial PH defined as systolic pulmonary arterial pressure (SPAP) > 40 mm Hg. The two groups were similar as far as SPAP (74,8 +/- 15,2 vs 76.5 +/- 14,1 mm Hg, p = ns) and distance covered during 6 minute walk test (339 +/- 116 vs 320 +/- 127, p = ns) are concerned. RESULTS: Flow velocity curve in the RV outflow tract were different in the two groups. Time from onset to peak velocity of ejection (acceleration time, AcT) as well as to midsystolic deceleration were shorter in TE-PH then in arterial PH (59.0 +/- 6.3 vs 68.1 +/- 15.4 msec, p = 0.015 and 123.8 +/- 17.9 vs 137.9 +/- 29.7 msec, p = 0.071, respectively). In the whole studied group as well as in arterial PH significant correlation between exercise tolerance and Doppler-derived indices of RV--pulmonary arterial coupling were found (r = 0.51, p < 0.001 and r = 0.72, p = < 0.001, respectively). Interestingly, no such relationship was found in TE-PH, (r = 0.16, p = ns). CONCLUSION: In contrast to arterial PH, pressure wave prematurely reflected from the intraluminal thrombi seem to disturb the RV ejection pattern and its correlation with exercise tolerance in TE-PH. Whether progression or effects of treatment on arterial PH can be assessed by monitoring changes in Doppler-derived indices of RV--pulmonary arterial coupling requires further studies.

[Effect of long-term therapy with oral Beraprost on survival of patients with arterial and inoperable thromboembolic pulmonary hypertension]. / Wplyw przewleklego leczenia Beraprostem sodu na rokowanie pacjentów z tevtniczym i zakrzepowo-zatorowym nadcisnieniem plucnym.

Beraprost sodium (BPS)--an orally active prostacyclin analogue--improves haemodynamic parameters and quality of life in group of patients with pulmonary arterial hypertension. Effect of long-term therapy with BPS is not well defined. This study assesses influence of long-term therapy with BPS on the survival of patients with precapillary pulmonary hypertension. Studied group consisted of 25 patients with precapillary PH (18 F, 7M, aged 34 +/- 13,9 years). Sixteen patients were diagnosed with primary PH, 3 pts had PH associated with connective tissue disease, 5 pts developed PH in course of congenital systemic to pulmonary shunt, and 1 patient suffered from inoperable chronic thromboembolic PH. At time of diagnosis 15 pts presented exercise impairment of WHO class II and 10 pts were in functional class III. All studied subjects had complete hemodynamic assessment of right heart and obtained values were used for estimation of hypothetic survival using prognostic equation proposed by D'Alonzo et al. On follow-up period patients received BPS in the highest tolerated dose (80-480 mg daily). During a follow-up period (mean: 22 months) 7 patients died. Cumulative survival rate BPS group was significantly higher in BPS group comparing to hypothetical survival at 6 months (96% (95% CI: 88-104%) vs 73% (95% CI: 67-78%), p = 0.02) and 12 months (94% (95% CI: 84-104%) vs 65% (58-71%), p = 0.01), respectively. At 18 and 24 months differences between BPS virtual and hypothetical survival were not statistically significant. There was no correlation between survival and maximal achieved dose of BPS. These results suggest, that BPS improves prognosis of patients with precapillary PH during 12 months after initiation of therapy. Later effect of BPS seems to decrease, requiring changing or intensification of therapy.

[Autoimmune thyroid diseases in patients with primary pulmonary hypertension]. / Autoimmunologiczne choroby tarczycy u chorych na pierwotne nadcisnienie plucne.

UNLABELLED: Abnormal metabolism due to thyroid disorders could cause the additional significant right ventricular (RV) overload in patients with primary pulmonary hypertension (PPH). The purpose of this study was the prospective determination of autoimmune thyroid status in PPH patients. MATERIAL: 45 patients (33 women and 12 men, mean age 38 years+/-13) with moderate and severe PPH (mean pulmonary artery pressure 61 mmHg+/-18 mmHg). PPH was diagnosed when other reason of pulmonary hypertension had been excluded especially thromboembolic, venous and hypoxemic. METHODS: Titer of antibodies to thyroglobulin and thyroid peroxidase were measured. Thyroid gland dysfunction was determined by biochemical measurements of thyrotropin and free thyroxine; thyroid ultrasonographic examination was also performed. The prevalence of thyroid autoantibodies is estimated at 13% in general population. RESULTS. In 14 patients (31%) increased titer of thyroid antibodies was found in our group. Hyperthyroidism was recognized in 2 patients and in 3 cases we diagnosed hypothyroidism with high antibodies level requiring treatment. Ultrasonographic abnormalities were revealed in 21 patients (nodular changes--11 patients, fibrosis--3 patients, chronic inflammation symptoms--7). Ultrasonographic abnormalities were present in all patients with detectable antithyroid antibodies. We haven't confirmed more frequent prevalence of hormonal disorders in patient treated with prostacyclin's analogues. CONCLUSION: The prevalence of autoimmune thyroid diseases is about 3 times higher in PPH patients than in general population. The treatment of patients with clinically evident hyper- or hypothyroidism is necessary. The monitoring of clinically silent disorders seems to be recommended especially prior to lung transplantation qualification.

Evaluation of intrapericardial cisplatin administration in cases with recurrent malignant pericardial effusion and cardiac tamponade.

GOALS: To evaluate the effectiveness and side effects of intrapericardial administration of cisplatin in prevention of recurrent malignant pericardial effusion. PATIENTS AND METHODS: Forty-six patients (33 men, 13 women; mean age 55.6+/-10.5 years) entered this study. The diagnosis of malignancy was based upon histological examination of samples from primary tumor. The majority of patients suffered from a neoplasm localized in the thorax (41 out of 46 patients; 89%). In 35 cases, pericardiocentesis, and in 11 cases, video-assisted thoracoscopic surgery (VATS) of pericardium was performed. Malignant etiology of pericardial fluid was confirmed by cytological examination, histology being obtained by VATS pericardial biopsy or by echocardiography (ECG). If daily drainage of pericardial fluid observed during 5-7 days exceeded 50 ml, cisplatin was instilled according to one of three regimens: (1) 10 mg of cisplatin dissolved in 20 ml of normal saline administered over 5 min during 5 consecutive days directly into the pericardial space (39 patients); (2) 50 mg of cisplatin dissolved in 100 ml of normal saline administered during 30 min (six patients); and (3) 20 mg of cisplatin dissolved in 40 ml of normal saline administered over 10 min during 5 consecutive days (one patient). Treatment was considered as successful when recurrence of symptoms of large pericardial effusion was not observed in ECG and other interventions directed to the pericardium were not required. Efficacy of investigated treatment was assessed also in the group of patients with survival longer than 30 days. Safety of treatment was assessed in the whole group of patients. RESULTS: Because of advanced malignancy eight out of 46 patients (17.4%) survived less than 30 days. Thirty-eight out of 46 cases (82.6%) survived more than 30 days. Positive effect of intrapericardial treatment with cisplatin was achieved in 43 out of 46 patients (93.5%) in the entire investigated group and in 35 out of 38 patients (92%) who survived more than 30 days. In the subgroup of patients with non-small cell lung cancer (NSCLC) and survival longer than 30 days, high efficacy was documented (29 out of 31 cases; 93.5%). Median survival time in the group of 38 patients who survived more than 30 days was 102.5 days. Atrial fibrillation due to cisplatin administration was observed in seven out of 46 patients (15.2%). Sclerosis of the pericardial space without symptoms of constriction occurred in five out of 46 cases (10.9%). CONCLUSIONS: Cisplatin administered directly into the pericardial space is a very effective and relatively safe method of treatment of recurrent malignant pericardial effusion, especially in the course of NSCLC.