Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies

AbstractPulmonary arterial thrombosis is an extremely rare occurrence in the neonatal population. We describe a 2-week-old female neonate who presented in critical condition with severe cyanosis and dehydration and was found to have a large thrombus in the main branches of the pulmonary arteries. She was successfully treated with surgical embolectomy. Pulmonary arterial thrombosis should always be considered in the differential diagnosis of a dehydrated neonate presenting with severe cyanosis and evidence of pulmonary hypertension.

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The first Russian experience of sildenafil application in patients with idiopathic pulmonary hypertension

Systemic Hypertension ◽

10.26442/sg29107 ◽

2015 ◽

Vol 12 (3) ◽

pp. 50-55

Author(s):

Z Kh Dadacheva ◽

A A Belevskaya ◽

M A Saidova ◽

N M Danilov ◽

T V Martyniuk ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Clinical Status ◽

Systolic Pressure ◽

Functional Class ◽

Pulmonary Artery Systolic Pressure ◽

Heart Catheterization ◽

Phosphodiesterase Type 5 Inhibitors ◽

6Mwt Distance ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension

Over the last decade, the interest in the treatment of idiopathic pulmonary hypertension (IPAH) has greatly increased because of the development and implementation of innovative therapeutic approaches. One of the modern drug groups for the treatment of pulmonary arterial hypertension are phosphodiesterase type 5 inhibitors (PDE5), which can cause pulmonary vasodilation via a NO-dependent mechanism.The aim of the research was to study the efficacy and safety of sildenafil treatment in patients with IPAH received 16-week course and to study sildenafil impact on clinical, hemodynamic and functional status.Material and methods: 20 patients with IPAH (mean age 40.9±11.5 years) functional class (FC) II-IV (WHO), who were receiving standard therapy (anticoagulants, diuretics, glycosides, calcium antagonists), were prescribed sildenafil 20 mg three times a day for 16 weeks. Initially, and after 16 weeks of treatment we studied FC, and the results of the six-minute walk test (6MWT), transthoracic echocardiography (ECHO), right heart catheterization (RHC) and laboratory tests to determine the safety of the therapy were carried out, as well.Results: the group of patients had 6MWT distance of 344±116 m at the beginning of the study. For about 90% of the patients suffered from dizziness, 75% - from the pain in the heart, 65% of the patients had edema and 35% - hepatomegaly. The patients suffered from severe pulmonary hypertension according to the functional and hemodynamic status. The estimated pulmonary artery systolic pressure (PASP) according to ECHO results was 89.6±22 mm Hg. According to the data from RHC we noted the elevation of mean pulmonary arterial pressure (mPAP) to 56.3±19 mm Hg, of pulmonary vascular resistance (PVR) to 1218±709 dyne/sec x per x cm-5 and the decrease in cardiac output (CO) - 3.4±0.8 l/min/m2.There were slight increasing in 6MWT distance (+23 m, p>0.05) and a statistically significant improvement in clinical status in comparison with the initial data: dizziness - 71%, heart pain - 38%, edema and hepatomegaly were observed in 15% and 14% (p

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A clinical case of long-term macitentan therapy in a female patient with idiopathic pulmonary hypertension

Terapevticheskii arkhiv ◽

10.17116/terarkh2016881288-93 ◽

2016 ◽

Vol 88 (12) ◽

pp. 88-93 ◽

Cited By ~ 1

Author(s):

O A Arkhipova ◽

T V Martynyuk ◽

I E Chazova

Keyword(s):

Pulmonary Hypertension ◽

Female Patient ◽

Clinical Case ◽

Endothelin Receptor ◽

Pathogenetic Therapy ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension ◽

The Russian Federation ◽

The Given

In late 2015, the Russian Federation registered the new non-selective endothelin receptor antagonist macitentan for the pathogenetic therapy of pulmonary arterial hypertension. The given clinical case demonstrates the possibility of using macitentan in a female patient with idiopathic pulmonary hypertension and its ability to affect the clinical, hemodynamic, and functional status of patients and to slow down the progression of the disease.

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IL-6 receptor overexpression in pulmonary arterial smooth muscle cells in idiopathic pulmonary hypertension

10.1183/13993003.congress-2015.pa2436 ◽

2015 ◽

Author(s):

Yuichi Tamura ◽

Carole Phan ◽

Ly Tu ◽

Raphael Thuillet ◽

Morane Le Hiress ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Smooth Muscle ◽

Smooth Muscle Cells ◽

Muscle Cells ◽

Arterial Smooth Muscle ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension ◽

Arterial Smooth Muscle Cells ◽

Pulmonary Arterial Smooth Muscle

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Bosentan Inducing Autoimmune Hepatitis in a Patient with Idiopathic Pulmonary Arterial Hypertension

Journal of Gastrointestinal and Liver Diseases ◽

10.15403/jgld.2014.1121.271.ajo ◽

2018 ◽

Vol 27 (1) ◽

pp. 89-92 ◽

Cited By ~ 1

Author(s):

Alexandre De Araujo ◽

Augusto Mantovani ◽

Carlos Thadeu Schmidt Cerski ◽

Antonio Barros Lopes ◽

Luiza Cristina Bortoncello ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Liver Function ◽

Autoimmune Hepatitis ◽

Normal Liver ◽

Idiopathic Pulmonary Arterial Hypertension ◽

Rapid Progression ◽

Normal Liver Function ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension

A 41-year-old woman diagnosed with idiopathic pulmonary hypertension presented symptoms despite the use of vasodilators, requiring treatment with bosentan. Previously, the patient had no signs of autoimmunity and had normal liver function. After three years of bosentan use, aminotransferase levels increased, without improvement after bosentan suspension, leading to complementary investigation. The diagnosis of autoimmune hepatitis was confirmed by biopsy, already in the stage of cirrhosis. In conclusion, in case of aminotransferase levels that remain persistently elevated, despite the reduction in doses and/or suspension of bosentan, autoimmune hepatitis must be investigated and treated urgently due to possibly rapid progression to cirrhosis.

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In search of markers of treatment failure and poor prognosis in IPAH – the value of mosaic lung attenuation pattern on thin-section CT scans

Multidisciplinary Respiratory Medicine ◽

10.4081/mrm.2010.562 ◽

2010 ◽

Vol 5 ◽

Author(s):

Monika Szturmowicz ◽

Aneta Kacprzak ◽

Barbara Burakowska ◽

Marcin Kurzyna ◽

Anna Fijałkowska ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Early Recognition ◽

Pulmonary Veins ◽

Connective Tissue Diseases ◽

Non Invasive ◽

Radiological Features ◽

Lung Sample ◽

Major Interest ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension

Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease (PVOD). Underdiagnosed PVOD occurs probably in 5-10% of idiopathic pulmonary hypertension (IPAH) and in a substantial proportion of PAH related to connective tissue diseases (mainly in scleroderma). A definite diagnosis of PVOD requires histologi- cal examination of lung sample, but surgical lung biopsy in pulmonary hypertension is combined with high risk of bleeding. Thus major interest is focused on a non-invasive diagnostic approach enabling early recognition of PVOD and referral for lung transplantation. The present review is focused on the radiological features suggestive of PVOD-like vasculopathy in PAH.

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Pulmonary thrombectomy in a patient with hemoglobin Nottingham

Perfusion ◽

10.1177/0267659107084143 ◽

2007 ◽

Vol 22 (4) ◽

pp. 299-301 ◽

Cited By ~ 1

Author(s):

Stavros G. Memtsoudis ◽

Robert W. Lekowski ◽

Peter Rosenberger ◽

Zain Khalpey ◽

Daniel J. FitzGerald ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Cardiopulmonary Bypass ◽

Hemolytic Anemia ◽

Arterial Thrombosis ◽

Multidisciplinary Approach ◽

Circulatory Arrest ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Deep Hypothermic Circulatory Arrest ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Arterial

A 36-year-old female with hemoglobin Nottingham (betaFG 5(98) Val → Gly) causing severe hemolytic anemia and chronic thromboembolic pulmonary hypertension presented with symptomatic subacute right lower lobar pulmonary arterial thrombosis requiring surgical pulmonary thrombectomy. We describe a successful, multidisciplinary approach to the problems associated with this disease, particularly with the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. Perfusion (2007) 22, 299—301.

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Idiopathic pulmonary arterial hypertension with coexisting CHD

Cardiology in the Young ◽

10.1017/s1047951117002876 ◽

2018 ◽

Vol 28 (5) ◽

pp. 743-746

Author(s):

Thomas G. Day ◽

Marina Hughes ◽

Bejal Pandya

Keyword(s):

Blood Flow ◽

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Pulmonary Blood Flow ◽

Management Strategy ◽

Septal Defect ◽

Idiopathic Pulmonary Arterial Hypertension ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension

AbstractSinus venosus atrial septal defect can result in an increase in pulmonary blood flow and vascular resistance, leading to pulmonary hypertension. Rarely, the degree of pulmonary hypertension is out of proportion to the degree of intra-cardiac shunting. This case outlines the differences between pulmonary hypertension secondary to CHD and idiopathic pulmonary hypertension, and illustrates the investigation and management strategy used in a patient with features of both.

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Type of QRS complex as an indicator of the severity of heart damage in patients with pulmonary hypertension

Kardiologicheskii vestnik ◽

10.36396/ms.2020.82.98.006 ◽

2020 ◽

pp. 37-45

Author(s):

Т.А. Сахнова ◽

Е.В. Блинова ◽

Г.В. Рябыкина ◽

А.А. Белевская ◽

Е.С. Юрасова ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Systolic Function ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Systolic Pulmonary Artery Pressure ◽

Heart Damage ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Arterial ◽

Idiopathic Pulmonary Hypertension ◽

Systolic And Diastolic Function ◽

The Right

Резюме Изменения электрокардиограммы, в частности, конфигурация комплекса QRS типа qR в отведении V1, являются фактором риска летального исхода у больных легочной артериальной гипертензией. Цель исследования. Сопоставить конфигурацию комплекса QRS в отведении V1 с выраженностью поражения сердца по данным эхокардиографии (ЭхоКГ) у больных идиопатической легочной гипертензией (ИЛГ) и хронической тромбоэмболической легочной гипертензией (ХТЭЛГ). Материалы и методы. У 40 больных ИЛГ и 40 больных ХТЭЛГ в возрасте 45±12 лет при ЭхоКГ оценивали систолическое давление в легочной артерии (СДЛА), размеры камер сердца, показатели систолической и диастолической функции правого желудочка (ПЖ), систолической функции левого желудочка, сердечно-сосудистое сопряжение ПЖ. Конфигурацию комплекса QRS в отведении V1 определяли на 10-ти секундной цифровой электрокардиограмме. Результаты. Было выявлено 18 вариантов конфигурации QRS в отведении V1. У 30 больных (37,5% случаев) в отведении V1 имелась конфигурация rS ил RS; у 16 пациентов (20% случаев) - различные варианты конфигурации rsR’; у 21 пациентов (26% случаев) - конфигурация qR. У пациентов конфигурацией qR в отведении V1 по сравнению с остальными группами были больше СДЛА и размеры правых камер сердца, отмечалось более выраженное ухудшение систолической функции правого и левого желудочков, а также более тяжелые нарушения межжелудочкового взаимодействия и сердечно-сосудистого сопряжения. Конфигурация qR в отведении V1 позволяла с чувствительностью от 46% до 89% и специфичностью от 89% до 95% выявлять больных с наличием прогностически неблагоприятных изменений ЭхоКГ: наличием перикардиального выпота, площадью правого предсердия больше 26 см2, TAPSE меньше 1,5 см. Заключение. Конфигурация комплекса QRS в отведении V1 у больных ИЛГ и ХТЭЛГ отличается большим полиморфизмом. Наиболее тяжелое поражение сердца наблюдается у пациентов с «qR-типом» комплекса QRSв отведении V1. Summary Changes in the electrocardiogram, in particular, the qR pattern in lead V1, are a risk factor for death in patients with pulmonary arterial hypertension. The aim of the work was to compare the QRS pattern in lead V1 with the severity of heart damage according to echocardiography (EchoCG) in patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods. In 40 patients with IPH and 40 patients with CTEPH aged 45 ± 12 years systolic pulmonary artery pressure (SPAP), sizes of heart chambers, systolic and diastolic function of the right ventricle (RV), systolic function of the left ventricle, RV ventricular-arterial coupling were evaluated with EchoCG. The QRS pattern in lead V1 was determined on a 10second digital electrocardiogram. Results. 18 QRS patterns in lead V1 were identified. In 30 patients (37.5% of cases) in lead V1, there was an rS or RS pattern; 16 patients (20% of cases) had different patterns of rsR ’ type; 21 patients (26% of cases) had a qR pattern. Patients with a qR pattern in lead V1 compared with other groups had greater SPAP and sizes of the right chambers of the heart, a more pronounced worsening of systolic function of the right and left ventricles, as well as more severe disturbances of interventricular interaction and ventricular-arterial coupling. The qR pattern in lead V1 made it possible with sensitivity from 46% to 89% and specificity from 89% to 95% to identify patients with prognostically unfavorable changes in EchoCG: presence of pericardial effusion, area of the right atrium more than 26 cm2, TAPSE less than 1.5 cm. Conclusions. The QRS pattern in lead V1 in patients with IPH and CTEPH is characterized by a large polymorphism. The most severe heart damage is observed in patients with the qR pattern in lead V1.

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