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

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.

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

Low DLCO in idiopathic pulmonary arterial hypertension — clinical correlates and prognostic significance

INTRODUCTION: Decreased diffusing capacity of the lung for carbon monoxide (DLCO) is observed in some idiopathic pulmonary arterial hypertension (IPAH) patients, but its clinical significance is uncertain. We aimed to assess clinical correlates and prognostic significance of low DLCO in IPAH patients. MATERIAL AND METHODS: In the group of 65 IPAH patients the cut off value for low DLCO was set up based on histogram as < 55% of predicted value. Demographic data, exercise capacity, lung function tests, hemodynamic parameters and survival of the patients were compared depending on DLCO value. RESULTS: Low DLCO was found in 18% of the patients, and it was associated with male sex, older age, worse functional status and exercise capacity, and higher prevalence of coronary artery disease. Low DLCO carried a 4-fold increase of death risk in 5-year perspective. CONCLUSIONS: Low DLCO was a marker of worse functional capacity and increased risk of death in studied IPAH patients.INTRODUCTION: Decreased diffusing capacity of the lung for carbon monoxide (DLCO) is observed in some idiopathic pulmonary arterial hypertension (IPAH) patients, but its clinical significance is uncertain. We aimed to assess clinical correlates and prognostic significance of low DLCO in IPAH patients. MATERIAL AND METHODS: In the group of 65 IPAH patients the cut off value for low DLCO was set up based on histogram as < 55% of predicted value. Demographic data, exercise capacity, lung function tests, hemodynamic parameters and survival of the patients were compared depending on DLCO value. RESULTS: Low DLCO was found in 18% of the patients, and it was associated with male sex, older age, worse functional status and exercise capacity, and higher prevalence of coronary artery disease. Low DLCO carried a 4-fold increase of death risk in 5-year perspective. CONCLUSIONS: Low DLCO was a marker of worse functional capacity and increased risk of death in studied IPAH patients

Non-tuberculous mycobacterial lung disease (NT MLD ) in patients with chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension

Wstęp: Mikobakteriozy są chorobami rzadkimi, rozpoznawanymi głównie u osób z grup ryzyka. Wśród płucnych czynników ryzyka nie wymieniono dotychczas nadciśnienia płucnego. Celem pracy była analiza obrazu klinicznego i przebiegu mikobakteriozy płuc, którą rozpoznano w ośrodku autorów pracy w latach 2002–2012 u chorych na nadciśnienie płucne zakrzepowo-zatorowe (CTEPH) i idiopatyczne (IPAH), oraz próba określenia czynników sprzyjających zachorowaniu.Materiał i metody: Badaną grupę stanowiło 13 chorych — 10 z CTEPH i 3 z IPAH. Nadciśnienie płucne potwierdzono metodą inwazyjną. Mediana średniego ciśnienia w tętnicy płucnej w okresie rozpoznania mikobakteriozy wynosiła 49 mm Hg (39–65 mm Hg). Mikobakteriozę płuc rozpoznano zgodnie z kryteriami Amerykańskiego Towarzystwa Chorób Płuc z 2007 roku.Wyniki: Czynnikiem etiologicznym był u większości chorych gatunek M. kansasii. Najczęstszymi objawami mikobakteriozy płuc były nasilenie duszności i produktywny kaszel. W tomografii komputerowej klatki piersiowej z opcją naczyniową w 7 przypadkach stwierdzono zagęszczenia miąższowe z rozpadem, w 6 — jamy otoczone drobnymi guzkami. U wszystkich chorych na CTEPH zmiany związane z mikobakteriozą pojawiły się na obszarach o upośledzonej perfuzji, ale bez widocznych zmian pozawałowych. Wszyscy chorzy otrzymali leczenie przeciwprątkowe, uzyskano poprawę w 12/13 przypadków. Do kwietnia 2014 roku 7 chorych zmarło z powodu ciężkiej prawokomorowej niewydolności serca, nie obserwowano zgonów w przebiegu mikobakteriozy.Wnioski: Nowe płucne zmiany miąższowe z rozpadem u chorych na CTEPH i na IPAH z towarzyszącym produktywnym kaszlem powinny skłonić do diagnostyki w kierunku mikobakteriozy płuc. W CTEPH niepokój budzą szczególnie ogniska rozpadu, którym nie towarzyszą nowe skrzepliny w naczyniach doprowadzających. Czynnikami sprzyjającymi rozwojowi mikobakteriozy płuc są: wysokie nadciśnienie płucne (CTEPH i IPAH) oraz obniżona perfuzja (CTEPH).Introduction: Non-tuberculous mycobacterial lung diseases (NTMLD) occur rarely and are diagnosed mainly in patients belonging to risk groups. Pulmonary hypertension (PH) has not been recognised as a risk factor for NTMLD yet. The aim of the study was to analyse the clinical course and predisposing factors of NTMLD recognised in our centre between 2002 and 2012 in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (IPAH).Material and methods: Thirteen patients (10 — CTEPH, 3 — IPAH) entered the study. PH was recognised during right heart catheterisation. Median value of mean pulmonary artery pressure (mPAP) was 49 mm Hg (39–65 mm Hg). NTMLD was diagnosed according to ATS guidelines (2007).Results: M. kansasii was the most frequent pathogen. Most patients complained of the exaggeration of dyspnoea and productive cough. Computed tomography of the chest with angiography revealed infiltrations with cavitation in seven patients and cavities surrounded by micronodules in six patients. In all CTEPH patients, NTMLD developed in the hypoperfused lung areas. No parenchymal abnormalities preceded the development of NTMLD. After diagnosis all of the patients received antituberculous treatment; in 12/13 improvement was achieved. By the end of March 2014 seven patients died due to right heart insufficiency, no deaths due to NTMLD were noted.Conclusions: NTMLD should be suspected in patients with CTEPH or IPAH, presenting with productive cough and a new pulmonary infiltrate with cavitation. In patients with CTEPH, special attention should be paid to a new cavitary lesions without accompanying thrombus in the artery supplying the area. High mPAP (CTEPH/IPAH) and hypoperfusion (CTEPH) are predisposing to NTMLD