Chronic thromboembolic pulmonary hypertension: epidemiology, pathogenesis and natural history

Nadciśnienie płucne zakrzepowo-zatorowe (CTEPH) to postać nadciśnienia płucnego spowodowana częściowym zamknięciem tętnic płucnych przez zorganizowane skrzepliny z często towarzyszącą przebudową drożnych oporowych tętniczek płucnych. Stanowi ono tak zwaną grupę 4 w aktualnie obowiązującej klasyfikacji nadciśnienia płucnego. Z wielu względów jest to szczególne schorzenie. Wyjątkowość CTEPH wynika z wciąż istniejących, istotnych wątpliwości dotyczących przyczyn i mechanizmu jego rozwoju, a jednocześnie - z możliwości skutecznego leczenia znacznego odsetka pacjentów metodą chirurgiczną za pomocą endarterektomii tętnic płucnych. Ten złożony zabieg może przywrócić choremu wydolność wysiłkową, jakość życia i przewidywany okres przeżycia, bardzo ograniczone w przypadku braku możliwości zastosowania swoistej terapii CTEPH.Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension induced by the partial obliteration of pulmonary arteries by organized thromboemboli, usually with coexisting remodeling of still patent pulmonary arterioles. It is compatible with group 4, according to the current classification of pulmonary hypertension. CTEPH has several peculiarities. They include persistent incertainties regarding its causes and pathogenesis, but also the possibility of highly effective surgical treatment in a significant proportion of patients by means of pulmonary endarterectomy. This complex intervention may restore exercise capacity, quality of life and life expectancy, otherwise all significantly limited by CTEPH

2016 ESC Position Paper on cancer treatments and cardiovascular toxicity developed under the auspices of the ESC Committee for Practice Guidelines: The Task Force for cancer treatments and cardiovascular toxicity of the European Society of Cardiology (ESC)

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Acquired methemoglobinemia - case report

Methemoglobinemia jest rzadko rozpoznawaną, groźną dla życia patologią polegającą na obecności we krwi ponad 1% utlenionej hemoglobiny, która jest niezdolna do przenoszenia tlenu. Przypadek dotyczy 49-letniego mężczyzny z gwałtownie narastającymi objawami ostrej, hipoksemicznej niewydolności oddechowej, u którego wykluczono ostre i przewlekłe choroby układów krążenia i oddechowego. Istotna rozbieżność pomiędzy wartością saturacji tlenem, odczytanej z pulsoksymetru, a saturacją tlenem oraz ciśnieniem parcjalnym tlenu, obliczonymi w badaniu gazometrycznym krwi włośniczkowej, a także wyraźny brak reakcji na tlenoterapię były ważnymi wskazówkami do poszukiwania hemoglobinopatii i rozpoznania methemoglobinemii. Stężenie methemoglobiny wynosiło 16%. Objawy ustąpiły samoistnie. Czynnik wywołujący methemoglobinemię nie został zidentyfikowany. Pneumonol. Alergol. Pol. 2010; 78, 2: 153-158Methemoglobinemia; 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. Pneumonol. Alergol. Pol. 2010; 78, 2: 153-15

Centrilobular nodules in high resolution computed tomography of the lung in IPAH patients — preliminary data concerning clinico-radiological correlates

Introduction: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH. Material and methods: 52 IPAH patients (38 females, 14 males, mean age 41 years ± 15 years), entered the study. All available chest CT scans were reviewed retrospectively by the experienced radiologist, not aware about the clinical data of the patients. Results: CN were found in 10 patients (19%), FGGO — in 12 patients (23%). No lymphadenopathy or interlobular septal thickening suggestive of PVOD were found. The significant differences between CN and the remaining patients included: lower mean age — 31 and 43.5 years, (p = 0.02), lack of persistent foramen ovale (PFO) — 0% and 43% (p = 0.03), and higher mean right atrial pressure (mRAP) — 12.5 mm Hg and 7.94 mm Hg (p = 0.01). No significant survival differences were observed between the groups of CN, FGGO and the remaining patients. Conclusion: Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.  INTRODUCTION: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH. MATERIAL AND METHODS: 52 IPAH patients (38 females, 14 males, mean age 41 years ± 15 years), entered the study. All available chest CT scans were reviewed retrospectively by the experienced radiologist, not aware about the clinical data of the patients. RESULTS: CN were found in 10 patients (19%), FGGO — in 12 patients (23%). No lymphadenopathy or interlobular septal thickening suggestive of PVOD were found. The significant differences between CN and the remaining patients included: lower mean age — 31 and 43.5 years, (p = 0.02), lack of persistent foramen ovale (PFO) — 0% and 43% (p = 0.03), and higher mean right atrial pressure (mRAP) — 12.5 mm Hg and 7.94 mm Hg (p = 0.01). No significant survival differences were observed between the groups of CN, FGGO and the remaining patients. CONCLUSION: Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.