Outcome of Medically Versus Surgically Treated Patients With Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is an ominous disease leading to progressive right heart failure. Selected patients can be treated by pulmonary endarterectomy (PEA). We assessed long-term clinical outcome of patients with CTEPH who underwent PEA and patients who remained on medical treatment alone. A total of 112 consecutive patients with CTEPH referred between 1998 and 2008 to one center were followed for a mean of 35 (range 0-128) months after diagnosis. All the patients had advanced pulmonary hypertension at baseline. The operated group had higher World Health Organization functional class compared to the nonoperated group. No other differences in hemodynamic, echocardiographic, or biochemical parameters were observed at baseline. Despite the perioperative mortality rate of 9.1%, patients who underwent PEA had significantly lower long-term mortality compared to nonoperated patients (12.7% vs 34.8%; P = .003), and PEA survivors showed sustained clinical improvement. All efforts should be undertaken to perform PEA in all patients with operable CTEPH.

[Acute lupus pneumonitis--case report and literature review]. / Ostre toczniowe zapalenie pluc--opis przypadku i przeglad pismiennictwa.

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease that is characterized by its chronic course and the involvement of many organs and systems. The most common abnormality in the respiratory system of SLE patients is lupus pleuritis. Less common is parenchymal involvement, which may present as acute lupus pneumonitis (ALP) or chronic interstitial lung disease. Other possible pulmonary manifestations of SLE include pulmonary embolism, diffuse alveolar haemorrhage, acute reversible hypoxaemia, and shrinking lung syndrome. We present the case report of a young woman with previously diagnosed membranous glomerulonephritis with nephrotic syndrome and antiphospholipid syndrome, who was admitted with marked of shortness of breath. The diagnostic process, including imaging studies and laboratory tests, enabled us to confirm a diagnosis of ALP. After initiation of treatment with high doses of methyloprednisolone, nearly complete remission of pulmonary changes was observed. We also perform a literature review regarding acute lupus pneumonitis.

Pulmonary artery dilatation correlates with the risk of unexpected death in chronic arterial or thromboembolic pulmonary hypertension.

BACKGROUND: Right ventricular failure does not explain all cases of death in patients with chronic pulmonary hypertension. Searching for alternative explanations, we evaluated the prognostic significance of main pulmonary artery (PA) dilatation in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: A retrospective outcome analysis was made of 264 patients (aged 46 ± 17 years; women, 69%; PAH, 82%) who underwent both CT scan measurement of the PA and right-sided heart catheterization (mean PA pressure, 57.6 ± 16.5 mm Hg) at initial evaluation. RESULTS: The diameter of the PA ranged from 28 to 120 mm (mean, 39 ± 8.6 mm; median, 38 mm) and was largest in patients with unrepaired congenital defects (42.6 ± 7.6 mm). Pulmonary pulse pressure (P = .04), lower age (P = .03), and duration of symptoms (P < .001) were independently but weakly related to PA diameter. During follow-up (median, 38 months), 99 patients (37%) died. Of these 99 deaths, 73 (74%) were due to heart failure or comorbidities, and 26 (26%) were unexpected deaths (UE-Ds). PA diameter (hazard ratio [HR], 1.06 per 1 mm; 95% CI, 1.03-1.08), heart rate (HR, 1.30 per 10 beats/min; 95% CI, 1.01-1.66), and systolic pulmonary arterial pressure (HR, 1.02 per 1 mm Hg; 95% CI, 1.01-1.04) were the only independent predictors of UE-D and differed from the usual predictors found in the study group for all-cause mortality. PA diameter ≥ 48 mm had 95% specificity and 39% sensitivity and carried 7.5 times higher risk of UE-D (95% CI, 3.4-16.5; P < .0001) during follow-up. CONCLUSIONS: PA dilatation emerges as an independent risk factor for death unexplained by right ventricular failure or comorbidities in patients with PAH and CTEPH. The possible mechanisms include, but are not limited to, PA compression of the left main coronary artery, PA rupture, or dissection with cardiac tamponade.

[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.

[Eosinophilic pneumonia in response to cutaneous larva migrans syndrome--a case report]. / Eozynofilowe zapalenie pluc w przebiegu zespolu larwy skórnej wedrujacej--opis przypadku.

Cutaneous larva migrans is a parasitic dermatosis imported by travelers returning from tropical and subtropical regions. In cutaneous larva migrans syndrome humans are incidental hosts and the larvae are unable to complete their natural cycle. Adult hookworms live in the intestines of dogs and cats, shedding eggs in feces that hatch and mature into larvae that can remain infective for months in the soil. Larvae penetrate the skin after contact with infected soil and cause an itchy creeping eruption. Cutaneous larva migrans is not usually associated with systemic symptoms and is rarely accompanied by peripheral blood eosinophilia. We report a patient who had both cutaneous larva migrans syndrome caused by Ancylostoma brasiliense and eosinophilic pneumonia after returning from Sri Lanka. The patient has been applied intravenous corticosteroids and local treatment with albendazole ointment with a very good clinical response.

[Recurrent effusive pericarditis in the course of adult-onset Still's disease--case reports of two patients]. / Nawracajace wysiekowe zapalenie osierdzia w przebiegu choroby Stilla u doroslych--opis dwóch przypadków.

Pericardial effusion is caused by various pathological agents. In differential diagnosis infectious as well as non-infectious factors have to be considered. Adult-onset Still disease (AOSD)--relatively uncommon systemic inflammatory disorder of unknown etiology--is among possible diagnosis. The disease typically affects patients in the age between 16-35 years and is characterized by spiking fever, arthralgia, evanescent salmon rash with other abnormalities including pharingitis, serositis (especially pleuritis and pericarditis) and leucocytosis as well as increased serum levels of inflammatory indicators. We present two patients with recurrent pericardial effusion in the course of AOSD.

Primary Sjögren's Syndrome with two extraglandular sites involvement - case report.

Primary Sjögren's Syndrome (pSS) is a chronic, slowly progressive inflammatory autoimmune disorder, characterised by lymphocytic infiltration of the exocrine glands, leading to decrease of glandular secretion. In 40-60% of pSS patients, extraglandular disease develops. We present the case of a patient with two extraglandular sites involvement in the course of pSS manifesting with progressive respiratory and gastrointestinal symptoms.

[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.

Characteristics and prognosis of patients with decompensated right ventricular failure during the course of pulmonary hypertension.

BACKGROUND: New therapies for pulmonary arterial hypertension have prolonged survival but simultaneously increased the number of hospital admissions because of decompensated right heart failure (DRHF). The optimal approach in DRHF has not been established yet. AIM: Analysis of clinical course of DRHF in a group of patients with pulmonary hypertension treated in a single referral centre. METHODS: We retrospectively analysed 60 episodes of DRHF in 37 patients (29 females, mean age 44+/-17 years) with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension admitted to our hospital between 2005 and 2007. We assessed the cause of decompensation, vital signs at admission, functional class and laboratory values. We classified all episodes into four haemodynamic profiles using the value of systolic blood pressure together with presence of peripheral perfusion abnormalities (profile cold vs. warm) and symptoms of venous congestion (profile wet vs. dry). Primary end-point was in-hospital mortality. RESULTS: The most common causes of DRHF were infection (27%), drug noncompliance (20%), and pulmonary embolism (3%). In 48% no causative factor was indentified. There were 19 (32%) in-hospital deaths. The highest mortality was observed among patients with connective tissue disease (61%). The haemodynamic profile 'warm-wet' was the most common (48%) and the profile 'cold-dry' was the rarest but was associated with a 100% mortality. Patients who died had higher value of functional class (3.84+/-0.38 vs. 3.51+/-0.55, p=0.01) and higher activity of aspartate transaminase (61+/-61 vs. 42+/-78 U/l, p=0.02) compared with those who survived. In multivariate analysis higher dopamine dose (RR 2.0/1 microg/kg/min, 95% CI 1.00-5.00, p <0.001) was an independent factor of in-hospital death. In contrast 'rescue therapy' with iloprost or treprostinil decreased mortality (RR 0.09, 95% CI 0.01-0.99, p=0.04). Mortality in patients receiving dopamine was higher (60 vs. 18%, p=0.001) than in patients treated without dopamine. CONCLUSION: Mortality in patients with pulmonary hypertension and DRHF remains very high and seems to be related to haemodynamic profile on admission. The newly introduced therapy with parenteral prostanoids may be more beneficial than dopamine infusion.

[Wegener's granulomatosis presenting with ischaemic stroke]. / Udar niedokrwienny mózgu w przebiegu ziarniniakowatosci Wegenera.

Wegener's granulomatosis (WG) is a systemic disease of unknown origin characterized by necrotizing granulomatous vasculitis that involves primarily the upper and the lower respiratory tracts and the kidneys. A 44-year-old woman presenting with unusual neurological manifestation is reported. The patient with multiple pulmonary round shadows and hilar lymphadenopathy on the chest X-ray pictures, suffered from sudden hemiparesis episode. Computed tomography revealed ischemic stroke focus. A diagnosis of WG was confirmed by histological examination of specimen obtained during open lung biopsy. During the combination chemotherapy with prednisolone and cyclophosphamide, neurological symptoms decreased and clinical condition significantly improved.

[Unusual cause of right heart failure decompensation in 21-years old patient with idiopathic pulmonary arterial hypertension -- a case report]. / Nietypowa przyczyna zaostrzenia prawokomorowej niewydolnosci serca u 21-letniej pacjentki z idiopatycznym nadcisnieniem plucnym - opis przypadku.

The authors describe a case of 21-years old woman with idiopathic pulmonary arterial hypertension with atypical clinical consequences of massive internal bleeding. Despite significant hypovolemia clinical and laboratory presentation was one of RV failure with dilatation of right heart ventricle and increased plasma level of markers of myocardial stretch and injury (NT-proBNP and troponin, respectively). This is attributed to impaired right ventricular coronary perfusion and hypoxia. Intensive treatment restored baseline RV conditions and at 15 months follow-up no persistent right heart impairment was observed. This case demonstrates that bleeding should be also considered in differential diagnosis of exacerbation of right ventricular failure in patients with pulmonary arterial hypertension.

Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension.

BACKGROUND: Right ventricular (RV) failure is the main cause of death in patients with pulmonary hypertension (PH). Balloon atrial septostomy (BAS) is believed to relieve symptoms of PH by increasing systemic flow and reducing RV preload. METHODS: Fourteen BAS procedures were performed in 11 patients (5 men and 6 women; mean [+/- SD] age, 33 +/- 12 years) with RV failure in the course of PH that was refractory to conventional treatment. BAS consisted of a puncture of the interatrial septum and subsequent dilatations with balloons of increasing diameter in a step-by-step manner. RESULTS: After BAS, the mean oxygen saturation of aortic blood decreased (before, 93 +/- 4%; after, 84 +/- 4%; p = 0.001), while mean cardiac index increased (before, 1.54 +/- 0.34 L/min/m(2); after, 1.78 +/- 0.35 L/min/m(2); p = 0.001), resulting in a positive trend for mean systemic oxygen transport (before, 270 +/- 64 mL/min; after, 286 +/- 81 mL/min; p = 0.08). Pulmonary vascular resistance (PVR) slightly increased immediately after the procedure, and this rise inversely correlated with mixed venous blood partial oxygen pressure both before BAS (r = -0.69; p = 0.009) and after BAS (r = -0.64; p = 0.018). Mean functional class improved from 3.2 +/- 0.4 to 2.6 +/- 0.7 (p = 0.03) after 1 month. At follow-up (mean time to follow-up, 8.1 +/- 6.2 months; range, 0.8 to 20.2 months), seven patients died and two underwent lung transplantation. There was no difference in the survival rate compared to that obtained from National Institutes of Health equation. A significant size reduction in the created defect was observed in six patients, requiring repeat BAS procedures in three cases. CONCLUSIONS: The current BAS technique improves cardiac index and functional class without significant periprocedural complications, except for a transient increase in PVR related to acute desaturation of mixed venous blood. At long-term follow-up, a high incidence of spontaneous decrease in orifice size has been observed.

[A case of a patient with idiopathic pulmonary arterial hypertension treated with lung transplantation: a "bumpy road" to success]. / Przypadek idiopatycznego nadcisnienia plucnego leczony transplantacja pluc. Trudnosci okresu przed transplantacja i po transplantacji.

We describe a case of 29 year old man, a first Polish patient with idiopathic arterial hypertension (IPAH) listed from Poland and successfully treated with lung transplantation in Vienna. Time from diagnosis to lung transplant was merely 11 months. Rapid clinical deterioration required treatment with most of currently approved or emerging methods, including oral and parenteral prostacyclin analogues administration by inhalation and chronic subcutaneous infusion. Atrial balloon septostomy was used to bridge the patient to transplant. We describe multiple problems in providing pharmacotherapy and in arranging logistics for lung transplantation. Peri- and multiple post-transplantation complications including dehiscence of right main bronchial anastomosis and its successful therapy are also presented. We consider good long term outcome as assessed 26 months post transplantation as an encouragement for other attempts at lung transplantation in patients with IPAH and for development of this method of therapy in Poland.

[Infective endocarditis in a patient with multiple myeloma. A case report]. / Infekcyjne zapalenie wsierdzia w przebiegu szpiczaka mnogiego -- trudnosci diagnostyczno-terapeutyczne. Opis przypadku.

A case of a 59 year old male with infective endocarditis is presented. Antibiotic therapy seemed effective, however, inflammation laboratory parameters increased two weeks after clinical improvement and body temperature normalisation. Subsequent extensive laboratory investigations revealed multiple myeloma. The patient underwent successful aortic valve replacement and received pharmacological therapy for multiple myeloma. Difficulties in diagnosing and treatment of patients with infective endocarditis who have other concomitant diseases, are discussed.

[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.

[Scimitar syndrome--case report]. / Zespól szabli tureckiej--opis przypadku.

Scimitar syndrome is a rare congenital anomaly. This syndrome is characterized by partial or complete anomalous pulmonary venous drainage of the right lung to the inferior vena cava. There is a characteristic abnormal radiographic shadow which descends along the right cardiac border (scimitar sign). We examined 71-year old woman with severe pulmonary hypertension due to a large shunt between pulmonary veins and right atrium. Other cause of pulmonary hypertension is atrial septum defect. Our patient required permanent pacemaker implantation for tachy-brady syndrome.

[Diagnostic difficulties in primary mesothelioma]. / Trudnosci diagnostyczne w rozpoznawaniu pierwotnego zlosliwego miedzybloniaka osierdzia.

A 54-year-old woman with a history of fatigue and shortness of breath was found to have a pericardial effusion and mild mediastinal lymphadenopathy. Video-assisted pericardioscopy revealed thickened pericardium studded with multiple nodules. Histologically the tumor was diagnosed as papillary adenocarcinoma. The site of the primary tumor could not be identified. As lung cancer is one of the most frequent causes of pericardial metastases the patient was treated with cisplatin and vinblastin. Following 5 courses of chemotherapy--given over a 4 month period--the amount of pericardial effusion and pericardial thickness did not change. The material from pericardial biopsy was reexamined and positive immunostaining for calretinine was found. The final diagnosis was primary pericardial mesothelioma of epithelioid type. Palliative radiotherapy of mediastinum was planned but the patient deteriorated and died due to disease progression with venous thrombosis and superior vena cava syndrome. The case illustrates the difficulties in establishing diagnosis of primary pericardial mesothelioma which is a rare tumor with poor prognosis.

[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.