Decrease In Pulmonary Artery Pressure Research Articles

Safety and Efficacy of Dapagliflozin in Patients with Heart Failure with Reduced Ejection Fraction: Multicentre Retrospective Study on Echocardiographic Parameters and Biomarkers of Heart Congestion.

Objectives: Dapagliflozin has shown efficacy in clinical trials in patients with heart failure and reduced ejection fraction (HFrEF). However, real-world data on its use and outcomes in routine clinical practice are limited. We aimed to evaluate the utilisation and safety profile of dapagliflozin in a real-world population of HFrEF patients within the Marche region. Methods: Nine cardiology departments within the Marche region retrospectively included HFrEF patients who were initiated on dapagliflozin therapy in an outpatient setting. Data on medical history, comorbidities, echocardiographic parameters, and laboratory tests were collected at baseline and after 6 months. Telephone follow-up interviews were conducted at 1 and 3 months to assess adverse events. We defined the composite endpoint score as meeting at least 50% of four objective measures of improvement among: weight loss, NYHA decrease, ≥50% Natriuretic peptides (NP) decrease, and guideline/directed medical therapy (GDMT) up titration. Results: We included 95 HFrEF patients aged 66 ± 12 years, 82% were men, 48% had ischemic heart disease, and 20% had diabetes. At six months, glomerular filtration rate declined (p = 0.03) and natriuretic peptides levels decreased, on average, by 23% (p < 0.001). Echocardiographic measurements revealed a decrease in pulmonary artery pressure (p < 0.001) and E/e' (p < 0.001). In terms of drug therapy, furosemide dosage decreased (p = 0.001), and the percentage of the target dose achieved for angiotensin receptor-neprilysin inhibitors increased (p = 0.003). By multivariable Cox regression, after adjustment for age, sex, the presence of diabetes/prediabetes, and HF duration, higher baseline Hb concentrations (HR 1.347, 95% CI 1.038-1.746, p = 0.025), and eGFR levels (HR 1.016, 95% CI 1.000-1.033, p = 0.46). Conclusions: In a real-life HFrEF population, dapagliflozin therapy is safe and well-tolerated, improves echocardiographic parameters and biomarkers of congestion, and can also facilitate the titration of drugs with a prognostic impact.

A case of metastatic breast cancer complicated by pulmonary tumor thrombotic microangiopathy

Pulmonary tumor thrombotic microangiopathy is a malignancy-related complication with rapid progression and high mortality. To improve the understanding of the disease, early diagnosis and treatment are key to successful treatment. A 39-year-old patient with pulmonary hypertension transferred from another hospital was admitted to the First Affiliated Hospital of Guangzhou Medical University on September 26, 2021. The patient developed shortness of breath and progressive exacerbation over the past month. No pulmonary artery embolism was seen on computed tomography pulmonary angiography (CTPA) at the outside hospital where the breast cancer was diagnosed. Pulmonary tumor thrombotic microangiopathy was immediately considered on admission and oncological endocrine therapy was started. After treatment, the patient's dyspnoea improved, PET-CT showed significant tumor regression, and cardiac ultrasound showed a significant decrease in pulmonary artery pressure. The successful treatment experience of this case was summarized for reference.

Urokinase-loaded cyclic RGD-decorated liposome targeted therapy for in-situ thrombus of pulmonary arteriole of pulmonary hypertension.

Backgroud: In-situ thrombosis is a significant pathophysiological basis for the development of pulmonary hypertension (PH). However, thrombolytic therapy for in-situ thrombus in PH was often hampered by the apparent side effects and the low bioavailability of common thrombolytic medications. Nanoscale cyclic RGD (cRGD)-decorated liposomes have received much attention thanks to their thrombus-targeting and biodegradability properties. As a result, we synthesized urokinase-loaded cRGD-decorated liposome (UK-cRGD-Liposome) for therapy of in-situ thrombosis as an exploration of pulmonary hypertensive novel therapeutic approaches. Purpose: To evaluate the utilize of UK-cRGD-Liposome for targeted thrombolysis of in-situ thrombus in PH and to explore the potential mechanisms of in-situ thrombus involved in the development of PH. Methods: UK-cRGD-Liposome nanoscale drug delivery system was prepared using combined methods of thin-film hydration and sonication. Induced PH via subcutaneous injection of monocrotaline (MCT). Fibrin staining (modified MSB method) was applied to detect the number of vessels within-situ thrombi in PH. Echocardiography, hematoxylin-eosin (H & E) staining, and Masson's trichrome staining were used to analyze right ventricular (RV) function, pulmonary vascular remodeling, as well as RV remodeling. Results: The number of vessels with in-situ thrombi revealed that UK-cRGD-Liposome could actively target urokinase to in-situ thrombi and release its payload in a controlled manner in the in vivo environment, thereby enhancing the thrombolytic effect of urokinase. Pulmonary artery hemodynamics and echocardiography indicated a dramatical decrease in pulmonary artery pressure and a significant improvement in RV function post targeted thrombolytic therapy. Moreover, pulmonary vascular remodeling and RV remodeling were significantly restricted post targeted thrombolytic therapy. Conclusion: UK-cRGD-Liposome can restrict the progression of PH and improve RV function by targeting the dissolution of pulmonary hypertensive in-situ thrombi, which may provide promising therapeutic approaches for PH.

Contribution of Adenosine in the Physiological Changes and Injuries Secondary to Exposure to Extreme Oxygen Pressure in Healthy Subjects

Climbers and aviators are exposed to severe hypoxia at high altitudes, whereas divers are exposed to hyperoxia at depth. The aim of this study was to report changes in the adenosinergic system induced by exposure to extreme oxygen partial pressures. At high altitudes, the increased adenosine concentration contributes to brain protection against hypoxia through various mechanisms such as stimulation of glycogenolysis for ATP production, reduction in neuronal energy requirements, enhancement in 2,3-bisphosphoglycerate production, and increase in cerebral blood flow secondary to vasodilation of cerebral arteries. In the context of mountain illness, the increased level of A2AR expression leads to glial dysfunction through neuroinflammation and is involved in the pathogenesis of neurological disorders. Nonetheless, a high level of adenosine concentration can protect against high-altitude pulmonary edema via a decrease in pulmonary arterial pressure. The adenosinergic system is also involved in the acclimatization phenomenon induced by prolonged exposure to altitude hypoxia. During hyperoxic exposure, decreased extracellular adenosine and low A2A receptor expression contribute to vasoconstriction. The resulting decrease in cerebral blood flow is considered a preventive phenomenon against cerebral oxygen toxicity through the decrease in oxygen delivery to the brain. With regard to lung oxygen toxicity, hyperoxia leads to an increase in extracellular adenosine, which acts to preserve pulmonary barrier function. Changes in the adenosinergic system induced by exposure to extreme oxygen partial pressures frequently have a benefit in decreasing the risk of adverse effects.

Cinaciguat (BAY-582667) Modifies Cardiopulmonary and Systemic Circulation in Chronically Hypoxic and Pulmonary Hypertensive Neonatal Lambs in the Alto Andino.

Neonatal pulmonary hypertension (NPHT) is produced by sustained pulmonary vasoconstriction and increased vascular remodeling. Soluble guanylyl cyclase (sGC) participates in signaling pathways that induce vascular vasodilation and reduce vascular remodeling. However, when sGC is oxidized and/or loses its heme group, it does not respond to nitric oxide (NO), losing its vasodilating effects. sGC protein expression and function is reduced in hypertensive neonatal lambs. Currently, NPHT is treated with NO inhalation therapy; however, new treatments are needed for improved outcomes. We used Cinaciguat (BAY-582667), which activates oxidized and/or without heme group sGC in pulmonary hypertensive lambs studied at 3,600 m. Our study included 6 Cinaciguat-treated (35 ug kg−1 day−1 x 7 days) and 6 Control neonates. We measured acute and chronic basal cardiovascular variables in pulmonary and systemic circulation, cardiovascular variables during a superimposed episode of acute hypoxia, remodeling of pulmonary arteries and changes in the right ventricle weight, vasoactive functions in small pulmonary arteries, and expression of NO-sGC-cGMP signaling pathway proteins involved in vasodilation. We observed a decrease in pulmonary arterial pressure and vascular resistance during the acute treatment. In contrast, the pulmonary pressure did not change in the chronic study due to increased cardiac output, resulting in lower pulmonary vascular resistance in the last 2 days of chronic study. The latter may have had a role in decreasing right ventricular hypertrophy, although the direct effect of Cinaciguat on the heart should also be considered. During acute hypoxia, the pulmonary vascular resistance remained low compared to the Control lambs. We observed a higher lung artery density, accompanied by reduced smooth muscle and adventitia layers in the pulmonary arteries. Additionally, vasodilator function was increased, and vasoconstrictor function was decreased, with modifications in the expression of proteins linked to pulmonary vasodilation, consistent with low pulmonary vascular resistance. In summary, Cinaciguat, an activator of sGC, induces cardiopulmonary modifications in chronically hypoxic and pulmonary hypertensive newborn lambs. Therefore, Cinaciguat is a potential therapeutic tool for reducing pulmonary vascular remodeling and/or right ventricular hypertrophy in pulmonary arterial hypertension syndrome.

Impact of transcatheter edge-to-edge mitral valve repair on right ventricular function

Development of transcatheter techniques procedures have modified mitral regurgitation management. The technique of edge-to-edge mitral valve repair (TEEMVR) with MitraClip© induces a certain degree of mitral stenosis, that could impact right ventricular function. The aim of this study is to evaluate impact of TEEMVR on right ventricular morphology and function and pulmonary artery pressures up to 1 year after procedure. In this observational retrospective study, we collected echocardiographic parameters before, at 6 months and 1 year after procedure. We analyzed right ventricular dimensions and systolic function, pulmonary artery pressures, and tricuspid regurgitation. A comparison between patients with a transmitral gradient below or above 5 mmHg at day one after procedure was performed. Forty three patients aged 77,5 ± 11,8 years underwent TEEMVR between 2015 and 2020, 60% had primary mitral regurgitation. A significant reduction of tricuspid regurgitation severity was observed: grade 2,0 ± 0,8 before, 1,6 ± 0,7 at 6 months and 1,7 ± 0,8 at 1 year ( P = 0,03 and P = 0,02 respectively). No significant difference in right ventricular systolic function was observed during follow-up. In the population of secondary mitral regurgitation, a significant increase of right ventricular fractional area shortening between 6 months and 1 year was noted (25 ± 10% at 6 months and 31 ± 5% at 1 year; P = 0,04). A decrease in pulmonary artery pressures at 6 months was observed in the general population (50 ± 1 mmHg before clip vs 43 ± 14 mmHg at 6 months; P = 0,03). In patients presenting with a transmitral gradient below 5 mmHg at day one, a decrease in pulmonary pressures was observed at 6 months whereas those with a gradient above 5 mmHg did not. Left ventricular ejection fraction decreased (from 48 ± 17,2% before, 44,5 ± 15,4% at 6 months and 43,3 ± 16,1% at 1 year; P = 0,01, P = 0,02 respectively). End-diastolic left ventricular diameter and volume decreased at 6 months (59 ± 9 mm and 56 ± 13 mm at 6 months; P = 0,025 and 147 ± 58 mL vs 141 ± 75 mL at 6 months; P = 0,01). Few changes in right ventricular morphology and function after TEEMVR were observed in our study. However, patients with transmitral gradient below 5 mmHg demonstrated greater decrease in pulmonary artery pressures. Further studies with a longer follow-up could help in understanding impact of TEEMVR on right ventricular physiology.

Pulmonary artery denervation for pulmonary hypertension: a systematic review and meta-analysis of clinical studies

Objective. To analyze the results of clinical studies on pulmonary artery denervation (PADN) for pulmonary hypertension treatment.Design and methods. Study search was conducted in PubMed/Medline, Cochrane, Elibrary databases. The analysis included studies of surgical and transcatheter PADN. To assess the effectiveness of the PADN procedure, a mean pulmonary artery pressure (mPAP) and a 6-minute walk test distance (6-MWT) were selected. For each study, the mean change in mPAP and 6-MWT were calculated and included in the analysis.Results. A total of 8 studies were analyzed: 4 randomized and 4 observational, 320 patients were included in the study, of which 222 patients underwent PADN. The etiology of pulmonary hypertension (PH) was different in each study and included patients with idiopathic PH, PH associated with heart failure, chronic thromboembolic PH. According to the combined data analysis, mPAP decreased in all studies, and the overall change in mPAP was –8,59 [95 % confidence interval (CI) –10,96; –6,23]. The average increase in 6-MWT among studies was 60,00 [95 % CI 35,74; 84,27].Conclusions. Despite serious limitations (a small number of studies and subjects enrolled, non-randomized design of a half of the studies, high heterogeneity of data), this analysis demonstrated the association of the PADN procedure with a decrease in pulmonary artery pressure and prolongation of the 6-minute walking distance in patients with PH of various etiologies.

Clipping of an Aortopulmonary Window in a Small Neonate With Bronchial Bleeding: A Simple Solution for a Complex Condition

Although the conventional treatment of aortopulmonary (AP) window consists of reconstructive surgery with the use of cardiopulmonary bypass (CPB), some conditions like low birth weight or active respiratory tract bleeding may lead to diverting therapeutic options. We present a case of a premature 1.9 kg neonate with severe pulmonary arterial hypertension based on the association of an AP window and large patent ductus arteriosus. Because of intrabronchial hemorrhage, a conservative strategy was chosen excluding the need for heparinization and CPB. Through median sternotomy, the ductus arteriosus and AP window were clipped, effectively occluding both shunts. The postoperative course was uneventful with a rapid decrease of pulmonary artery pressure. Although classical surgical reconstruction is still advocated as primary therapy, this case illustrates the suitability of an alternative approach without the need for CPB and full heparinization in a patient with an increased risk of bleeding complications.

Study of the hemodynamic effect of inhaled milrinone versus inhaled nitroglycerin on mechanically ventilated patients with pulmonary hypertension after mitral valve replacement surgeries

IntroductionPulmonary arterial hypertension (PH) is commonly found in patients with mitral valve diseases. Most of the drugs used for PH treatment are nonselective pulmonary vasodilators that may cause a modest decrease in pulmonary arterial pressure, but they may cause a marked decrease in systemic vascular resistance as well, which leads to hypotension and a decrease in perfusion of vital organs. Inhaled drugs such as milrinone and nitroglycerine may be safer as a large dose of the drug can selectively target the pulmonary circulation, which leads to a reduction of their systemic adverse effects.AimThis double-blind, randomized study compared milrinone inhalation versus nitroglycerin inhalation on pulmonary artery pressure and other hemodynamics in postoperative patients immediately after mitral valve replacement surgery.Patients and methodsA total of 40 participants with isolated mitral stenosis or isolated mitral incompetence or their combination plus PH [mean pulmonary artery pressure (mPAP) >40 mmHg] scheduled for mitral valve replacement were interviewed. Immediately postoperatively in the ICU, we randomized the patients into two groups, with 20 patients each. Group A consisted of 20 patients who received milrinone inhalation, whereas group B consisted of 20 patients who received nitroglycerine inhalation. Hemodynamic parameters (heart rate, systolic blood pressure, diastolic blood pressure, mean blood pressure, central venous pressure, pulmonary artery systolic pressure, pulmonary diastolic pressure, mPAP, fractional shortening, ejection fraction, and pressure of oxygen/fraction of inspired oxygen ratio) were recorded in both groups at three time points: T1, before drug delivery; T2, just after drug delivery; and T3, just before extubation.ResultsThe study showed that both milrinone and nitroglycerin inhalation caused a marked reduction in systolic, diastolic, and mPAPs without causing any hemodynamic deterioration effects on systolic, diastolic, or mean arterial pressures.ConclusionAlthough both milrinone and nitroglycerin inhalation proved to be effective pulmonary artery vasodilators that avoided systemic adverse effects, nitroglycerin inhalation is a less expensive, easily obtained drug that can serve as an inhaled pulmonary vasodilator in patients with mitral valve diseases and PH following mitral valve replacement surgeries.

High-fat diet attenuates the improvement of hypoxia-induced pulmonary hypertension in mice during reoxygenation

BackgroundIt is widely recognized that metabolic disorder is associated with pulmonary hypertension (PH). It is known that hypoxia-induced elevated pulmonary artery pressure in mice returns to normal pressure during reoxygenation. However, it is still unclear how metabolic disorder affects the reverse remodeling of pulmonary arteries. In this study, we investigated the effects of high-fat diet (HFD) on the decrease in pulmonary artery pressure and reverse remodeling of pulmonary arteries in mice with hypoxia-induced PH.MethodsWe used female C57BL/6 mice aged 8 weeks. After being exposed to hypoxia (10% oxygen for four weeks) to induce PH, the mice were returned to normoxic conditions and randomized into a normal diet (ND) group and HFD group. Both groups were fed with their respective diets for 12 weeks.ResultsThe Fulton index and right ventricular systolic pressure measured by a micro-manometer catheter were significantly higher in the HFD group than in the ND group at 12 weeks after reoxygenation. The medial smooth muscle area was larger in the HFD group. Caspase-3 activity in the lung tissue of the HFD group was decreased, and the apoptosis of pulmonary smooth muscle cells was suppressed after reoxygenation. Moreover, the expression levels of peroxisome proliferator-activated receptor-γ and apelin were lower in the HFD group than in the ND group.ConclusionsThe results suggest that metabolic disorder may suppress pulmonary artery reverse remodeling in mice with hypoxia-induced PH during reoxygenation.

Evaluation of the Results of Balloon Pulmonary Angioplasty in Patients with Chronic Distal Pulmonary Thromboembolic Disease and Pulmonary Hypertension

Abstract Introduction The gold standard curative treatment for chronic pulmonary thromboembolic disease and pulmonary hypertension (CTEPH) is pulmonary thromboendarterectomy (PTE). Balloon pulmonary angioplasty (BPA) is emerging for distal CTEPH patients, where lesion is inaccessible for surgery. This is the first study conducted in Indian patients for evaluating the impact of BPA in patients with distal CTEPH. Aims and Objectives To evaluate the effect of BPA in patients with distal CTEPH with the help of New York Heart Association (NYHA) functional class and 6-minute walk distance (6MWD). To study decrease in pulmonary artery pressure and improvement in right atrial and right ventricular function with the help of echocardiography. Material and Methods Study population—CTEPH patients presenting to the hospital during the study interval of 16 months from January 2017 to April 2018. A. Method: This was an observational retrospective and prospective follow-up study. All distal CTEPH patients after meeting inclusion and exclusion criteria were taken for study. B. Detailed case records containing information on demographics, clinical features and necessary blood and imaging investigations, ventilation/perfusion (V/Q) scan, computed tomography pulmonary angiography (CTPA), right heart catheterization (RHC) study, pulmonary angiogram, and BPA procedure details were obtained for all participants. C. After 8 weeks of last BPA session, patients were assessed clinically and then 6-minute walk test, blood investigations and echocardiography were done. Data Analysis Statistical analysis was performed by using the software SPSS 22.0 version. We used paired t-test to test the significant difference in the mean pre- and post-BPA. p-value &lt; 0.05 will be considered as statistically significant. Results In our study, mean age of presentation was 39.81 ± 12 years. Out of 11 patients, 5 were females and 6 were males. Mean duration of symptoms was 40.5 months. The total number of BPA sessions performed were 30. The minimum number of BPA sessions undergone was 1 and maximum number of BPA sessions undergone by one patient was 5, with 45% of patients undergoing 2 BPA sessions. The total number of segmental arteries dilated was 104. Segmental vessels dilated per each session was 3.46. There was statistically significant improvement in NYHA class and 6MWD after BPA. 6MWD increased from 299 m to 421 m (p-value &lt; 0.001). This improvement in functional capacity is strongly associated with the improvement in right ventricular (RV) function (tricuspid annular plane systolic excursion [TAPSE] from 15.3 mm to 18.9 mm) and with the reduction in pulmonary artery systemic pressure (PASP) (from 92 mm Hg to 60 mm Hg). Conclusion In patients with distal CTEPH who undergo BPA, there was statistically significant improvement in 6MWD. These changes correspond to a treatment-induced reduction in pulmonary artery pressure and lend support to use of BPA in patients with distal CTEPH. ECHO and 6MWD can be used for evaluating BPA efficacy and monitoring disease progression.

MO746CARDIAC REMODELING AND PULMONARY HYPERTENSION IN HEMODIALYSIS PATIENTS

Abstract Background and Aims An important predictor of cardiovascular mortality and morbidity in hemodialysis patients is left ventricular hypertrophy. Also, pulmonary hypertension is a risk factor for mortality and cardiovascular events in hemodialysis patients. The aim of this study was to investigate cardiac remodeling and the dynamics of pulmonary arterial pressure during a year-long hemodialysis treatment and to evaluate relationship between pulmonary arterial pressure and blood flow in arteriovenous fistula. Method Hemodialysis patients (n=88; 42 males, 46 females, mean age was 51.7±13.0 years) were studied. Echocardiography and Doppler echocardiography were performed in the beginning of hemodialysis treatment and after a year. Echocardiographic evaluation was carried out on the day after dialysis. Left ventricular mass index (LVMI) was calculated. Left ventricular ejection fraction (LVEF) was measured by the echocardiographic Simpson method. Arteriovenous fistula flow was determined by Doppler echocardiography. Pulmonary hypertension was diagnosed according to criteria of Guidelines for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology. Results Pulmonary hypertension was diagnosed in 47 (53.4%) patients. Left ventricular hypertrophy was revealed in 71 (80.7%) patients. Only 2 (2.3%) patients had LVEF&amp;lt;50%. At the beginning of hemodialysis correlation was detected between systolic pulmonary arterial pressure and LVMI (r=0.52; P&amp;lt;0.001). Systolic pulmonary arterial pressure negatively correlated with left ventricular ejection fraction (r=-0.20; P=0.04). After a year of hemodialysis treatment LVMI decreased from 140.49±42.95 to 123.25±39.27 g/m2 (р=0.006) mainly due to a decrease in left ventricular end-diastolic dimension (from 50.23±6.48 to 45.13±5.24 mm, p=0.04) and systolic pulmonary arterial pressure decreased from 44.83±14.53 to 39.14±10.29 mmHg (р=0.002). Correlation wasn’t found between systolic pulmonary arterial pressure and arteriovenous fistula flow (r=0.17; p=0.4). Conclusion Pulmonary hypertension was diagnosed in half of patients at the beginning of hemodialysis treatment. Pulmonary hypertension in hemodialysis patients was associated with left ventricular hypertrophy, systolic left ventricular dysfunction. After a year-long hemodialysis treatment, a regress in left ventricular hypertrophy and a partial decrease in pulmonary arterial pressure were observed. There wasn’t correlation between arteriovenous fistula flow and systolic pulmonary arterial pressure.

Pulmonary Vasodilators in LVAD Patients:A Never-Ending Debate

Left ventricular assist device is a life-saving option in patients with end-stage left heart failure. It leads to a decrease of pulmonary arterial pressure, wedge pressure and pulmonary vascular resistences. However, a post capillary pulmonary hypertension persists in a significant proportion of patients despite adequate LV unloading. A possible explanation is the pulmonary venous congestion and subsequent decrease in the level of nitric oxide. This observation has led to an increased interest for pulmonary vasodilators, especially phosphodiesterase 5 inhibitors (PD-5 I). They enhance the nitric oxide (NO) signaling through the increase of cyclic guanosine monophosphate availability. Sildenafil allows to tritate down the iNO and succesfully extubated the patient; it has been shown to significantly reduce mPAP and PVR 2-4 weeks after LVAD implantation and to wean from inotropic support within the first 72 hours. However, whereas literature seems to support its use in the immediate postoperative period, there are not enough evidences about their long term use.

Clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension secondary to congenital portosystemic shunts.

The aim of this retrospective study was to investigate the clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension (PAH) secondary to congenital portosystemic shunts (CPSS). Thirty-three pediatric patients diagnosed in our institution with CPSS between 2012 and 2019 were enrolled in this study. The patients were divided into PAH and non-PAH groups. The PAH group included 15 patients who presented with unexplained PAH when CPSS was diagnosed. Two patients with microangiopathic hemolytic anemia died of right heart failure shortly after diagnosis. One patient received a liver transplant at the age of 4.3 years and showed a mild decrease in pulmonary artery pressure (PAP) 4 years after the operation. Seven patients underwent one-stage shunt closure at a median age of 2.8 years (1.4-13 years). Follow-up examinations, from 1.6 to 4.1 years after intervention, showed marked reduction of PAP in one patient and stabilization of PAH in six others. However, in one patient who underwent two-stage shunt closure, a marked increase in PAP was noted after partial ligation of the shunt. The remaining four patients received only pulmonary vasodilator therapy, and one of them died of right heart failure 12 years after the PAH diagnosis. The non-PAH group included 18 patients without evidence of PAH upon CPSS diagnosis. Shunt closure was carried out in eight of these patients, but one patient subsequently developed PAH after the resolution of hepatopulmonary syndrome.Conclusion: CPSS may be a more likely cause of unexplained PAH in pediatric patients than previously thought. Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.

Діагностичне значення катетеризації правих відділів серця та легеневої артерії у хворих із підозрою на легеневу гіпертензію. Частина 1. Методологія виконання процедури, нозологія захворювань та вазодилататорний тест

Мета роботи – здійснити критичний перегляд власного досвіду, його відповідності чинним рекомендаціям і даним міжнародних реєстрів, а також оцінити показники легеневої та системної гемодинаміки, отримані у хворих на легеневу артеріальну гіпертензію (ЛАГ) в Україні.Матеріали і методи. За 5 років 220 процедур катетеризації правих відділів серця і легеневої артерії виконано 195 пацієнтам із середньою або високою ймовірністю ЛАГ за даними ехокардіографії відповідно до сучасних рекомендацій. Усі пацієнти були госпіталізовані в ННЦ «Інститут кардіології імені акад. М.Д. Стражеска» НАМН України із підозрою на наявність ЛАГ (первинно), або із погіршанням перебігу захворювання (повторно), або для підтвердження статусу вазореактивності (повторно). Результати та обговорення. Проведено 220 успішних процедур катетеризації. Первинну (діагностичну) катетеризацію провели у 195 хворих, повторну – у 25. Діагноз ЛАГ підтверджено у 178 пацієнтів. У 17 пацієнтів за результатами катетеризації діагноз ЛАГ був знятий: величина середнього тиску в легеневій артерії була менше 20 мм рт. ст. Структура нозологій у хворих, яким було виконано катетеризацію була такою: ідіопатична ЛАГ спостерігалася у 68 (38 %) пацієнтів, з них 11 (6 %) – вазореактивних; ЛАГ, асоційована із захворюваннями сполучної тканини, – у 21 (12 %) пацієнта; ЛАГ, асоційована з ВІЛ-інфекцією, – у 4 (2 %) хворих; ЛАГ, асоційована з портальною гіпертензією, – у 4 (2 %); ЛАГ, асоційована з природженими вадами серця, – у 26 (15 %); хронічна тромбоемболічна легенева гіпертензія – у 50 (28 %). Тест на вазореактивність було проведено у 33 хворих на ідіопатичну ЛАГ, з них у 11 (33 %) він був позитивним. У хворих із позитивним вазидилататорним тестом середній тиск у легеневій артерії знизився у середньому на 21,8 мм рт. ст. – до 26,5 мм рт. ст. (р&lt;0,0001), при цьому серцевий індекс збільшився на 31 % – до 3,8 л · хв · м–2 (р&lt;0,1). Легеневий судинний опір знизився на 6,4 од. Вуда – до 2,7 од. Вуда (р&lt;0,0001). У хворих із негативним результатом тесту зниження середнього тиску в легеневій артерії і легеневого судинного опору було статистично не значущим (р&gt;0,05), а змін серцевого індексу не виявлено.Висновки. На підставі досвіду нашого центру оцінювання гемодинаміки за допомогою катетеризації є безпечним і залишається діагностичним стандартом для ЛАГ. Зокрема, катетеризація є необхідною для узгодженого аналізу 4 показників, що мають критичне значення для клінічного профілю пацієнтів з ЛАГ: тиск у правому передсерді, легеневий судинний опір, серцевий викид та тиск заклинювання в легеневій артерії. У пацієнтів з ідіопатичною ЛАГ також необхідно оцінювати вазореактивність для прогнозування чутливості до терапії антагоністами кальцію, наявність якої пов’язана з кращими результатами лікування та виживаності.

Endovascular Mechanical Thromboembolus Fragmentation in the Treatment of Critical Pulmonary Thromboembolism in Patients with Acute Hemorrhagic Stroke.

The aim of the study was to evaluate the effectiveness of endovascular mechanical thromboembolus fragmentation in the treatment of critical pulmonary thromboembolism in patients with acute hemorrhagic stroke.Materials and MethodsThe study included 47 patients with acute hemorrhagic stroke complicated by massive high-risk pulmonary thromboembolism with critical manifestations of the right ventricular failure. All patients were divided into two groups depending on the treatment method: the examined group (n=17) undergone endovascular mechanical thromboembolus fragmentation and the control group (n=30) received only basic intensive therapy.ResultsThromboembolus fragmentation was performed on patients of the examined group in order to transfer embolism of the trunk and main branches to the smaller branches of the pulmonary artery. The technical success of the procedure (destruction of the central thromboembolus) was achieved in 100% of cases. 14 patients (82.4%) showed positive clinical dynamics: improvement of general condition, reduction of pulmonary artery pressure, decreased volume of pulmonary bed damage according to CT angiography. Three patients (17.6%) died in the early postoperative period. Twenty five patients from the control group died, hospital mortality rate was 83.3%. There were no deaths in the examined group after 6–9 months of follow-up, signs of pulmonary hypertension persisted in 11 patients (64.7%).ConclusionEndovascular mechanical thromboembolus fragmentation in the treatment of critical pulmonary thromboembolism in patients with acute hemorrhagic stroke results in fast and safe decrease in pulmonary artery pressure. Fragmentation of central thromboembolus and its displacement into the peripheral vascular bed with a modified pigtail catheter is a minimally invasive surgical procedure which may be used as an alternative to surgical embolectomy in cases of an extremely high risk of surgery and absolute contraindications to thrombolytic therapy.

Long-term changes of right ventricular myocardial deformation and remodeling studied by cardiac magnetic resonance imaging in patients with chronic thromboembolic pulmonary hypertension following pulmonary thromboendarterectomy

BackgroundRight ventricular (RV) afterload in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is associated with reduced myocardial contractility and ventriculoarterial coupling. The impact of increased afterload on RV myocardial deformation was assessed by comparing the characteristics of CTEPH patients to healthy controls at baseline, and by comparing characteristics of CTEPH patients before and 12 months after pulmonary endarterectomy (PEA). MethodsCardiac deformation and function of CTEPH patients (n = 20) and healthy controls (n = 20) were assessed by cardiac magnetic resonance (CMR). CTEPH patients were also examined with right heart catheterization before and 12 months after PEA. ResultsPEA resulted in significant improvement of invasive hemodynamics and normalization of RV hypertrophy and right atrial, RV and left ventricular dimensions and volumes. RV ejection fraction improved from 30 ± 13% at baseline to 44 ± 10% at 12 months (p < 0.0001) but remained decreased compared with control subjects (54 ± 4%, p < 0.05). RV global circumferential strain (GCS) normalized 12 months after PEA, but RV global longitudinal strain (GLS) remained significantly lower in CTEPH patients than controls (baseline −12.9 ± 3.3% vs. −16.5 ± 3.6% at 12 months p < 0.01, vs. controls −19.3 ± 3.2%, p < 0.05). RV mass changes were significantly correlated with RV-ejection fraction, RV-GLS, and RV-GCS. RV-pulmonary arterial coupling with the volume method improved at 12 months (0.49 ± 0.30 vs. 0.84 ± 0.31, p < 0.0005), but remained significantly reduced compared with healthy controls (1.19 ± 0.20, p < 0.0005). ConclusionRV global longitudinal and circumferential myocardial three-dimensional strain by CMR improved significantly in CTEPH patients 12 months after PEA. Improvements in myocardial deformation were associated with regression of RV hypertrophy and decrease in pulmonary artery pressure.

Effect of inhaled nitric oxide with mechanical ventilation in persistent pulmonary hypertension of newborn

Objective To study the clinical effect of inhaled nitric oxide(iNO) with mechanical ventilation for the neonates with persistent pulmonary hypertension of newborn(PPHN). Methods A total of 40 neonates with PPHN poorly responding with routine treatment were treated with iNO therapy.Pulmonary artery pressure, blood gas ion analysis and respiratory function changes were observed at different time points before and after iNO. Results Treatment with iNO resulted in a rapid decrease in pulmonary artery pressure from(54.95±17.08) mmHg(1 mmHg=0.133 kPa) to(20.40±14.26) mmHg.Oxygenation of 40 cases improved in the first 0.5~1 hour after iNO therapy, the values of mean airway pressure(MAP)declined from(13.98±2.40)cmH2O(1 cmH2O=0.098 kPa) to(12.44±2.69)cmH2O, oxygenation index(OI)decreased from 26.89±18.62 to 13.84±5.52, PaO2/FiO2 increased from(60.34±23.49) mmHg to(144.46±23.49) mmHg, and the value of pH increased from 7.31±0.14 to 7.37±0.07.Furthermore, within 48 hours after treatment, PaO2/FiO2 and pH increased gradually, while MAP and OI decreased gradually, which all had statistical differences(P<0.01). Conclusion Mechanical ventilation combined with iNO therapy in the treatment of PPHN can rapidly improve the oxygenation status of neonates, reduce pulmonary vascular resistance, and then improve the survival rate and long-term prognosis. Key words: Mechanical ventilation; Inhaled nitric oxide; Persistent pulmonary hypertension of newborn

Acute Hemodynamic Effects of Negative Extrathoracic Pressure in Fontan Physiology.

We sought to assess acute hemodynamic changes after implementation of negative extrathoracic pressure (NEP) in spontaneously breathing ambulatory Fontan patients with symptomatic heart failure. We hypothesized that application of NEP would result in an acute decrease in pulmonary artery pressure. Ten patients with clinical evidence of Fontan failure underwent baseline hemodynamic catheterization while breathing spontaneously. Hemodynamic measurements were then repeated after 30min of continuous NEP. After 30min of continuous NEP, 4/10 patients had a decrease in their Fontan pressure by 2mmHg and one patient had a decrease by 1mmHg. There were three patients that had an increase in Fontan pressure by 2mmHg. In 7/10 patients, indexed pulmonary vascular resistance decreased by an average of 31%. In symptomatic Fontan patients with a favorable hemodynamic response to NEP during catheterization, potential benefit of longer-term NEP to improve clinical status should be explored.

PH Grand Rounds: A Case of Pulmonary Hypertension Associated With High Cardiac Output State from Arteriovenous Fistula–Or Is It?

PH Grand Rounds: A Case of Pulmonary Hypertension Associated With High Cardiac Output State from Arteriovenous Fistula–Or Is It?