scholarly journals Hemodynamic and functional effectiveness of the patient undergoing pulmonary thromboendarterectomy

2019 ◽  
pp. 29-34
Author(s):  
Gabriela Valdez ◽  
Mayra Lucia Antonio ◽  
Adriana Alejandra Pineda
Keyword(s):  
Circulatory Arrest ◽  
Functional Class ◽  
Selective Cerebral Perfusion ◽  
Pulmonary Thromboendarterectomy ◽  
Mean Pulmonary Arterial Pressure ◽  
Kaplan Meier ◽  
Dichotomous Responses ◽  
Pulmonary Arterial ◽  
Total Circulatory Arrest

Objective: to evaluate the hemodynamic and functional effectiveness of the patient undergoing thromboendarterectomy. Methods: a descriptive study of series of cases in postoperative patients of thromboendarterectomy from 2002 to 2016 with complete records was carried out. The data collection was done with an "ex profeso" sheet of 10 demographic items, 2 of somatometry, 6 hemodynamics, 1 functional, 6 gasometric and 9 of perfusion conduction, with open and dichotomous responses. For the data analysis, descriptive statistics, U Mann-Whitney tests and Kaplan-Meier survival analysis were applied; the significance was set at p <0.05. Results: 25 patients were studied, male prevalence (68%), age 43 ± 18 years. The cardiopulmonary bypass time was 222 ± 73 min. and the aortic clamp of 121 ± 71 min; in 80%total circulatory arrest was performed for periods of 20 min and reperfusion times of 10 min between each one. 20% of the patients were managed with selective cerebral perfusion. Thromboendarterectomy decreased pulmonary vascular resistance (p <0.0001) and mean pulmonary arterial pressure (p = 0.001) and increased cardiac output (p = 0.009), PaO2 (p = 0.035) and SaO2 (p = 0.015). 72% improved the functional class from III-IV to I-II. There was mortality of 20% associated to persistence of pulmonary arterial hypertension and the three-year survival was 80%. Two patients required ECMO A-V both successful. Conclusion: Thromboendarterectomy is the treatment of choice for patients with chronic pulmonary thromboembolism, significantly improving hemodynamic and functional parameters, reflected in their quality of life and survival.

scholarly journals Oral treprostinil in transition or as add-on therapy in pediatric pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401985647 ◽  
Author(s):  
D. Dunbar Ivy ◽  
Jeffrey A. Feinstein ◽  
Delphine Yung ◽  
Mary P. Mullen ◽  
Edward C. Kirkpatrick ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Cardiopulmonary Exercise Testing ◽  
Functional Class ◽  
World Health ◽  
Walk Distance ◽  
Pulmonary Arterial ◽  
Health Organization

Treprostinil, a prostacyclin analogue, is approved for the treatment of pulmonary arterial hypertension (PAH) in adults. Transition from parenteral to oral treprostinil has been successfully accomplished in adults with PAH but not in children. In this multicenter study, pediatric patients treated with parenteral (Cohort 1) or inhaled (Cohort 2) treprostinil were transitioned to oral treprostinil. Prostacyclin-naïve individuals on background oral PAH therapy received oral treprostinil as add-on therapy (Cohort 3). Successful transition was oral treprostinil dose maintenance through week 24. Patients were monitored for adverse events (AEs), 6-min walk distance (6MWD), PAH symptoms, World Health Organization (WHO) Functional Class (FC), cardiac magnetic resonance imaging (cMRI), cardiopulmonary exercise testing (CPET), and quality of life through 24 weeks. A total of 32 patients were enrolled in the study; 23 (72%) were girls (mean age = 12.2 years). All patients were on background oral PAH therapy. Overall, patients (96.9%) maintained transition to oral treprostinil; one patient (Cohort 1) transitioned to oral treprostinil, then back to parenteral after experiencing syncope and WHO FC change from II to III. Cohorts 1, 2, and 3 received a final mean oral treprostinil dose of 5.6, 3.3, and 4.5 mg t.i.d., respectively. All cohorts had variable changes in 6MWD, cMRI, and CPET. Overall, 12 serious AEs were reported. All patients had drug-related AEs including headache (81%), diarrhea (69%), nausea (66%), vomiting (66%), and flushing (56%). Pediatric patients maintained transition to oral treprostinil with preservation of exercise capacity and WHO FC. Prostanoid-related AEs were most common and similar to those reported in adults.

P3676Hemodynamic phenotypes in systemic sclerosis patients screened for pulmonary hypertension (PH): impact of the new definition of PH

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Xanthouli ◽  
N Milde ◽  
A M Marra ◽  
N Benjamin ◽  
C Nagel ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Functional Class ◽  
Walking Distance ◽  
Pulmonary Vascular Disease ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial ◽  
Definition Of ◽  
The Impact

Abstract Background Patients with systemic sclerosis (SSc) are at high risk of developing concomitant pulmonary hypertension (PH) which has a crucial impact on the patients' symptoms, quality of life and prognosis. Purpose The aim of this study was to analyse the impact of the new hemodynamic definition of precapillary PH as proposed at the 2018 World Symposium on PH in Nice in patients with SSc. Although recent data suggest that PVR >2 WU could be considered as abnormal in the new definition a conservative cut-off value of PVR ≥3 WU has been used. Methods SSc-patients were screened for PH using clinical assessments as WHO-functional class, 6 minute walking distance and right heart catheterisation. Patients were divided into hemodynamic subgroups according to their mean pulmonary arterial pressure (mPAP) values with mPAP ≤20 mmHg, 21–24 mmHg and ≥25 mmHg. These subgroups were further divided according to their pulmonary vascular resistance (PVR) with PVR <3 WU or ≥3 WU. Results One-hundred-twenty-two patients (79% female, age 57.6±12.7 years, 6MWD 439.5±98.1 meters, 70% diffuse cutaneous SSc, 30% limited cutaneous SSc, 53% WHO-FC II, 25% WHO-FC III) who were prospectively screened for PH were included in the analysis (Figure 1). Among them 26 had a symptomatic manifest PH using the cut-off value of mPAP ≥25 mmHg. Only half of this group presented with PVR ≥3 WU, the others had PVR <3 WU. Eight of these 26 PH-patients presented with PH due to left heart disease. Out of 21 patients with mildly elevated mPAP 21–24 mmHg, two (10%) met the new definition criteria of PH (pulmonary arterial wedge pressure <15 mmHg, mPAP 23 and 24 mmHg, PVR 3.0 and 3.2 WU, CI 2.2 L/min/m2 both, WHO-FC II both, respectively). Out of 75 patients with mPAP <21 mmHg, three presented with PVR ≥3 WU. Overview of systemic sclerosis patients Conclusions The new definition of precapillary pulmonary hypertension may on the one hand allow detecting an additional 10% of PH patients with mild elevated mPAP. On the other hand, eight of 13 patients (62%), who met the former definition of pulmonary arterial hypertension, would be classified as “normal” due to a lack of increase in PVR according to the new definition. The data of this study suggest that for SSc-patients the cut-off value of mPAP >20 mmHg is useful, but the criteria of PVR ≥3 WU may be too strict. Further studies with larger sample sizes will be needed to better characterise these hemodynamic subgroups and to define the extent of pulmonary vascular disease and treatability.

scholarly journals Pulmonary Hypertension

2015 ◽  
Vol 10 (1) ◽  
pp. 9
Author(s):  
Brendan P Madden ◽  
Keyword(s):  
Pulmonary Hypertension ◽  
Positive Impact ◽  
Vascular Dysfunction ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Pulmonary Thromboendarterectomy ◽  
Mean Pulmonary Arterial Pressure ◽  
Number Of Patients ◽  
Impact On Survival ◽  
Pulmonary Arterial

Pulmonary hypertension is said to occur when the mean pulmonary arterial pressure exceeds 25 mmHg at rest or 30 mmHg during exercise. There are many causes but the term Pulmonary arterial hypertension (PAH) is used to describe a rare group of illnesses that share histopathological similarities in the small muscularised pulmonary arterioles leading to vascular remodelling (plexogenic pulmonary arteriopathy) and progressive elevation in the pulmonary vascular resistance. Left untreated, patients die as a consequence of right heart failure and the mortality approaches that of commonly encountered malignancies. There is no effective cure. Most treatment for PAH patients has focused on the endothelial cell vascular dysfunction known to occur in these disorders and indeed agents such as endothelin receptor antagonists, phosphodiesterase pathway V inhibitors and prostacyclin analogues have been shown to improve morbidity and delay rate of deterioration. More recently evidence has emerged that they may have a positive impact on survival. These agents have also been applied to treat patients with chronic thromboembolic pulmonary hypertension (CTEPH) and selected patients with CTEPH may also benefit from pulmonary thromboendarterectomy. For a small number of patients with PAH lung transplantation may be considered

scholarly journals Factors related to the quality of life in patients with pulmonary arterial hypertension

2021 ◽  
Vol 20 (Supplement_1) ◽  
Author(s):  
K Sarzynska ◽  
J Polanski ◽  
G Kopec ◽  
E Mroczek ◽  
B Jankowska-Polanska
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Sociodemographic Factors ◽  
Functional Class ◽  
Life Questionnaire ◽  
Elderly Age ◽  
Comprehensive Therapy ◽  
Pulmonary Arterial

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Pulmonary arterial hypertension (PAH) is a rare disease, with an incidence of 15–50 cases per million annually. The disease is associated with further progress and worsening of symptoms despite pharmacotherapy. The severity of PAH symptoms and their effects reduce the patients" quality of life (QoL). Numerous studies show that the quality of life is related to the therapeutic effect of the treatment. Available studies demonstrate that patients QoL deteriorates as the condition progresses. Consequently, the goals of PAH therapy have expanded from increasing survival to improving health-related quality of life. The aim of the study was to determine the clinical and sociodemographic factors influencing the quality of life in PAH patients. Methods The study involved 55 patients with PAH who were examined using the Polish version of the PAH-SYMPACT quality of life questionnaire. The PAH-SYMPACT questionnaire results were correlated with several  variables. Results It was shown that the quality of life correlates significantly (p˂0,05) with WHO Functional Class, NT-pro BNP (N-terminal prohormone of brain natriuretic peptide) value, elderly age (the higher the value, the worse the QoL). On the other hand, the lower the 6MWT (6 minuts walk test) result, the worse the QoL. Moreover, the intensity of the accompanying PAH symptoms, such as chest pain, dyspnoea, arrhythmias, fatigue and weakness were associated with a lower quality of life in patients (p &lt; 0,05). Conclusion Patients" quality of life correlates with many variables, both clinical and sociodemographic. In addition, there is a correlation between the quality of life of patients with PAH and the severity of several symptoms associated with this condition. Determination of factors affecting the quality of life of patients with PAH enables the selection of a more comprehensive therapy.

scholarly journals Dasatinib-Induced Pulmonary Arterial Hypertension Treated with Upfront Combination Therapy

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Makoto Nishimori ◽  
Tomoyuki Honjo ◽  
Kenji Kaihotsu ◽  
Naohiko Sone ◽  
Sachiko Yoshikawa ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Rare Complication ◽  
Functional Class ◽  
World Health ◽  
First Line Therapy ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary arterial hypertension (PAH) is a rare complication of dasatinib that was approved as a first-line therapy for chronic myelocytic leukemia (CML). A 24-year-old man presenting dyspnea at rest and leg edema was admitted to our hospital. He had been diagnosed with CML and prescribed dasatinib for 4 years. Chest X-ray showed significant bilateral pleural effusion and heart enlargement. Echocardiography revealed interventricular septal compression and elevated peak tricuspid regurgitation pressure gradient of 66.7 mmHg indicating severe pulmonary hypertension. After the other specific diseases to provoke PAH were excluded, he was diagnosed with dasatinib-induced PAH. Despite discontinuation of dasatinib and intravenous administration of diuretic for two weeks, World Health Organization (WHO) functional class was still II and mean pulmonary arterial pressure (PAP) was high at 37 mmHg. Therefore, we administered sildenafil and bosentan together as an upfront combination therapy three weeks after dasatinib discontinuation. Six months later, his symptoms improved to WHO functional class I and mean PAP was decreased to 31 mmHg. Although PAH is a rare complication of dasatinib, symptomatic patients prescribed with dasatinib should have an echocardiogram for PAH screening. Moreover, the upfront combination therapy would be a useful option for symptomatic patients after discontinuation of dasatinib.

Mechanisms of injury and methods of protection of the brain during cardiac surgery in neonates and infants

1993 ◽  
Vol 3 (3) ◽  
pp. 317-330 ◽  
Author(s):  
William J. Greeley ◽  
Frank H. Kern ◽  
James R. Mault ◽  
Lynn A. Skaryak ◽  
Ross M. Ungerleider
Keyword(s):  
Congenital Heart ◽  
Heart Defects ◽  
Circulatory Arrest ◽  
Surgical Techniques ◽  
Developmental Abnormalities ◽  
Cardiac Morbidity ◽  
Neonates And Infants ◽  
Total Circulatory Arrest

With substantial effort and research devoted to improving surgical techniques and myocardial protection, superb results have been achieved for repair of complex congenital heart defects in children. As a result, investigative efforts now have begun to examine the quality of life for patients surviving these operations. Because these neonates and infants are exposed to severe physiologic extremes of temperature (15–18 °C) and severe alterations from normal perfusion (total circulatory arrest), the nature of long-term neuropsychological outcome has been a prominent concern. Recent preliminary reports suggest that transient and permanent neuropsychologic injury occur in as many as 25% of all infants undergoing hypothermic cardiopulmonary bypass with circulatory arrest.'Since improved surgical techniques have significantly reduced rates of operative mortality and cardiac morbidity, one of the greatest risks remaining for the patient with congenital heart disease may be long term neuropsychologic and developmental abnormalities.

scholarly journals Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study

2016 ◽  
Vol 26 (7) ◽  
pp. 1250-1259 ◽  
Author(s):  
Pascal Amedro ◽  
Adeline Basquin ◽  
Virginie Gressin ◽  
Pierre Clerson ◽  
Xavier Jais ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Health Related Quality ◽  
Nyha Functional Class ◽  
Related Quality ◽  
Health Related ◽  
Pulmonary Arterial

BackgroundThe aim of this study was to assess health-related quality of life in patients with pulmonary arterial hypertension associated with CHD and correlations with clinical status.MethodsThis prospective cross-sectional observational study included CHD patients with pulmonary arterial hypertension in 14 tertiary-care centres in France. We used two health-related quality of life questionnaires – SF-36 and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) – and one anxiety/depression Hospital Anxiety and Depression Scale (HADS) questionnaire.ResultsClinical data were collected for the 208 included patients (mean age: 42.6 years, range from 15.1 to 85.8 years, 69.7% female). Most patients were in NYHA functional class II (48.1%) and III (37.5%). Patients’ phenotype was classified as Eisenmenger syndrome (70.7%), pulmonary arterial hypertension associated with systemic-to-pulmonary shunts (12.0%), with small defects (3.4%), or after corrective cardiac surgery (13.9%). In total, 76.4% of the patients were receiving pulmonary arterial hypertension-specific treatments. SF-36 scores showed impairment compared with normalised data. Health-related quality of life scores were significantly lower in females than in males for most dimensions of both questionnaires and were independent of the patients’ phenotype, even after gender adjustment – except for CAMPHOR functioning – but significantly depended on NYHA functional class. The Hospital Anxiety and Depression Scale (HADS) scores suggested anxiety and depression associated with increasing NYHA functional class but independent of patients’ phenotype. NYHA functional class, 6-minute walk distance, HADS, gender, and recent stressful event significantly affected quality of life in the multivariate analysis.ConclusionsThis study showed impairment of quality of life in a large cohort of patients with pulmonary arterial hypertension associated with CHD with both generic and specific questionnaires. NYHA functional class and HADS scores were predictive of most quality of life scores.

Depression: Beyond Description As an Important Comorbidity in Pulmonary Arterial Hypertension—The Next Steps

2012 ◽  
Vol 10 (4) ◽  
pp. 227-232 ◽  
Author(s):  
Deborah H. McCollister ◽  
Philippe Weintraub ◽  
David B. Badesch
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Important Determinant ◽  
Provider Education ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Broad Array ◽  
Pulmonary Arterial

The recent identification of depression as an important comorbidity in pulmonary arterial hypertension (PAH)12 is leading to a broad array of efforts to further refine our understanding of this disorder, enhance patient and provider education about it, and encourage prompt recognition, appropriate diagnosis, and treatment of affected individuals. We will provide an update on the nature and extent of the problem, and describe ongoing and future efforts to address this very important determinant of quality of life and possible long-term outcome in patients with PAH.

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