Improvement Of Pulmonary Hypertension Research Articles

Intervention control of aerobic exercise in maintaining quality of life and pulmonary hypertension in hemodialysis patients.

Pulmonary hypertension is a serious complication in the treatment of maintenance hemodialysis patients, which seriously affects the quality of life of patients and threatens their life safety. Prevention, treatment and improvement of pulmonary hypertension are of great significance to improve the quality of life of patients. To investigate the intervention and control of pedal-powered bicycle in maintaining quality of life and pulmonary hypertension in hemodialysis patients. 73 patients with maintenance hemadialysis combined with pulmonary arterial hypertension at a hemodialysis center in a certain hospital from May 2021 to May 2022 are selected. Patients are divided into two groups, 37 cases in the control group (group C) and 36 cases in the intervention group (group I). Patients are divided into two groups, group C is treated with oral administration of betaglandin sodium combined with routine nursing care. Based on group C, group I conducts power cycling exercises. After treatment, group I patients had higher muscle strength, 36-Item Short Form Health Survey scores, and Kidney Disease Targets Areas scores; The 6-minute walk distance test index level was higher and the Borg score was lower; The group I had lower systolic blood pressure, greater vital capacity, higher positive emotion, lower systolic pulmonary artery pressure index level, higher arterial partial oxygen pressure level, lower pulmonary vascular resistance index level, and higher blood oxygen saturation level [158.91 ± 11.89 vs 152.56 ± 12.81, 1795.01 ± 603.18 vs 1907.20 ± 574.15, 24.00 (22.00, 29.00) vs 24.00 (22.00, 28.00), P < 0.001]. Aerobic exercise combined with Western medicine treatment can effectively improve patients' pulmonary hypertension, alleviate their negative emotions, and enable them to achieve a higher level of quality of life.

An Infant with High-Output Heart Failure and Pulmonary Hypertension Resulting from a Giant Cutaneous Hemangioma.

We present the clinical course of an 8-month-old infant with a giant cutaneous hemangioma resulting in high-output heart failure and pulmonary hypertension. The lesion was successfully embolized and excised, with rapid resolution of heart failure and improvement in pulmonary hypertension.

Improvement in Pulmonary Hypertension Following Metabolic and Bariatric Surgery: a Brief Review and Meta-analysis.

We reviewed the available evidence on the outcome of metabolic and bariatric surgery (MBS) in patients with pulmonary hypertension (PH). Five studies examining 174 patients were included; the mean age was 54.5 ± 9.27years; the mean BMI before surgery and at the end of follow-up were 47.2 ± 5.95kg/m2 and 37.4 ± 2.51kg/m2, respectively. Furthermore, the results showed a significant decrease in the right ventricle systolic pressure (RVSP) after MBS with a mean difference of 10.11% (CI 95%: 3.52, 16.70, I2 = 85.37%, p = < 0.001), at 16.5 3.8month follow-up with a morbidity rate of 26% and 0 mortality. Thirty-day postoperative complications included respiratory failure, pulmonary embolism, pulmonary edema, and anastomotic leak. There appears to be a significant improvement in PH with a decrease in medication requirements after MBS.

Clinical and Hemodynamic Improvement in Pulmonary Hypertension After Switching to Sacubitril/Valsartan in Patients With Heart Failure With Preserved Ejection Fraction.

Patients with heart failure with preserved ejection fraction (HFpEF) and pulmonary hypertension have poor survival, and established medical therapies for both conditions are not available. In this retrospective study of 69 patients with HFpEF and either isolated postcapillary pulmonary hypertension (IpcPH, n = 53) or combined postcapillary and precapillary pulmonary hypertension (CpcPH, n = 16), we investigated the effects of sacubitril/valsartan on pulmonary hypertension measured using right heart catheterization at baseline (ie, presacubitril/valsartan) and 99 (94-123) days after switching to sacubitril/valsartan. After switching to sacubitril/valsartan, right heart catheterization showed significantly lower pulmonary artery pressures (systolic/diastolic/mean) in both patient groups compared with presacubitril/valsartan [IpcPH: 44 (38-52)/15 (12-19)/28 (22-33) mm Hg vs. 47 (40-55)/18 (15-23)/31 (26-35) mm Hg, P < 0.01; CpcPH: 54 (43-57)/18 (12-23)/34 (30-36) mm Hg vs. 61 (50-79)/24 (19-30)/40 (31-53) mm Hg, P < 0.05]. The median sacubitril/valsartan dose at follow-up was 24/26 (24/26-49/51) mg twice daily in both patients with IpcPH and CpcPH. Clinically, the New York Heart Association functional class improved by at least 1 class in 32 of 69 patients ( P < 0.01). In conclusion, sacubitril/valsartan therapy improves pulmonary hypertension in patients with HFpEF and either IpcPH or CpcPH. Further prospective randomized trials are needed for confirmation of our results.

Congenital diaphragmatic hernia with fetal hydrops causing postoperative intestinal perforation: An unusual manifestation seen in a neonate

Congenital diaphragmatic hernia (CDH) is a common cause of neonatal mortality. If CDH is associated with non-immune fetal hydrops (FH), the mortality rate is even higher. However, the pathogenesis and management of FH associated with CDH are still under discussion. We present here a case of left-sided CDH accompanied by prolonged FH, where unexpected ileal perforation developed after CDH repair. The female patient was diagnosed with left-sided CDH with right-sided pleural effusion (PE) at 26 weeks' gestation. At 35 weeks' gestation, FH progressed with bilateral PE and the patient was delivered by cesarean section with a body weight of 2068 g and Apgar 3/7. Subcutaneous edema progressed, but PE attenuated with improvement of pulmonary hypertension, and CDH repair was performed on Day 4. Postoperatively, the patient's pulmonary hypertension worsened, and intestinal perforation was observed on Day 11. In the emergency laparotomy, there was a single small bowel perforation of 5 mm in diameter in the terminal ileum. A 5 cm of the ileum was resected and the bowel was anastomosed in a single stage. The patient was weaned from the ventilator on Day 28. Although bilateral periventricular leukomalacia was confirmed, the patient was discharged without significant neurological symptoms on Day 76.

Reduction of Pulmonary Hypertension After Transition to Sacubitril/Valsartan in Patients With Heart Failure With Preserved Ejection Fraction.

Objectives: Although the PARAGON-HF trial failed to reach its primary endpoint, subgroups of patients with heart failure with preserved ejection fraction (HFpEF) still appear to benefit from Sacubitril/Valsartan therapy. As HFpEF patients with pulmonary hypertension display a specifically high mortality and morbidity, we evaluated the effect of Sacubitril/Valsartan in this subgroup of HFpEF patients.Methods: In this retrospective case-series of 18 patients with HFpEF and pulmonary hypertension, right heart catheterisation (RHC) for determination of invasive pulmonary pressure were performed at baseline (pre-Sacubitril/Valsartan) and 99 (71–156) days after transition from angiotensin-converting enzyme inhibitors and angiotensin receptor blockers to Sacubitril/Valsartan (post-Sacubitril/Valsartan). Results are given as median and interquartile range.Results: After conversion to Sacubitril/Valsartan, RHC showed significantly reduced pulmonary artery pressure (PAP) and mean pulmonary capillary wedge pressure (PCWP) compared to pre-Sacubitril/Valsartan [PAP systolic/diastolic/mean 44 (38–55)/15 (11–20)/27 (23–33) mm Hg vs. 51 (41–82)/22 (13–29)/33 (28–52) mm Hg, p < 0.05 and p < 0.01, respectively; PCWP 16 (12–20) mm Hg vs. 22 (15–27) mm Hg, p < 0.05]. Median Sacubitril/Valsartan dosage was 24/26 mg BID (24/26 BID−49/51 mg BID). Clinically, New York Heart Association functional class improved in 12 of the 18 patients (p < 0.01) after conversion to Sacubitril/Valsartan. Echocardiographic parameters of left ventricular function and cardiovascular co-medication did not differ markedly between pre- and post-Sacubitril/Valsartan.Conclusion: Sacubitril/Valsartan therapy is associated with an improvement of pulmonary hypertension in HFpEF patients.

Exploratory Assessment of Levosimendan in Infants With Congenital Diaphragmatic Hernia.

Infants with congenital diaphragmatic hernia frequently suffer from cardiac dysfunction and pulmonary hypertension during the postnatal course. With the use of the inodilator levosimendan, a therapeutic approach is available in situations with catecholamine-refractory low-cardiac-output failure and severe pulmonary hypertension. Retrospective single-center cohort study. University-based, tertiary-care children's hospital neonatal ICU. Cohort of 24 infants with congenital diaphragmatic hernia and levosimendan therapy, without underlying major cardiac defect, treated at the University Children´s Hospital Bonn, Germany, between January 2017 and December 2018. None. Twenty-four infants with congenital diaphragmatic hernia were treated with levosimendan (41% of hospitalized congenital diaphragmatic hernia infants in the study period). In 88%, the congenital diaphragmatic hernia was left-sided. The median observed-to-expected lung-to-head ratio was 36%. About 60% of the infants were supported with venovenous extracorporeal membrane oxygenation and the mortality was 38% (9/24 infants). Levosimendan administration was associated with improvement of pulmonary hypertension severity (p = 0.013 and p = 0.000) and right ventricular dysfunction (p = 0.011 and p = 0.000) at 24 hours and 7 days after treatment. Similarly, the prevalence of left ventricular dysfunction decreased from 50% at baseline to 10% after 7 days (p = 0.026). A significant reduction in the peak inspiratory pressure was observed after drug application (p = 0.038) and a significant decrease of the Vasoactive-Inotropic Score was apparent (p = 0.022). A relevant arterial hypotension as a drug-related adverse event occurred in one patient. This is the first study exploring clinical and hemodynamic changes after levosimendan treatment in a cohort of infants with congenital diaphragmatic hernia. An association of levosimendan application and an improvement in pulmonary hypertension, right ventricular, and left ventricular dysfunction were observed within 7 days after drug infusion. However, due to the retrospective design of this study, the results should be interpreted carefully.

Thrombolysis in Acute Pulmonary Embolism: Are we overdoing it?

Aim and Methods:We aimed to study the clinical data and outcome of patients admitted in our center with acute pulmonary embolism (PE) over a 5-year period from May 2013 to April 2018. The main outcome data included were: in - hospital bleeding, in - hospital right ventricular (RV) function improvement, pulmonary arterial hypertension improvement, duration of hospital stay, and 30- and 90-day mortality.Results:A total of 114 (69 m, 55 f) patients with the mean age of 55 ± 15 years were included. Patients who had involvement of central pulmonary trunk called as “Central PE” group (n = 82) and others as “Peripheral PE” group (n = 32). There were more women in the peripheral PE group (53.1% vs. 34.1%, P = 0.05), while RBBB (22% vs. 3.1%, P = 0.02) and RV dysfunction (59.8% vs. 25%, P = 0.002) were noted more in the central PE group. Systemic thrombolysis was done in 53 patients (49 central, 4 peripheral), of which only 3 had hypotension and 28 patients were in the Intermediate-high risk group. The overall inhospital, 30-day, and 90-day mortalities were 3.6, 13.2, and 22.8%, respectively. Bleeding was significantly higher in the thrombolysis group compared to the nonthrombolysis group (18.9% vs. 0, P = 0.0003). However, improvement in pulmonary hypertension was noted more in thrombolysis group compared to nonthrombolytic group (49% vs. 21.2%, P = 0.01).Conclusion:This retrospective data from a tertiary center in South India showed that short- and mid-term mortality of patients with PE still remains high. The high nonguideline use of thrombolysis has been reflected in the increased bleeding noted in our study.

Large Granular Lymphocyte Leukemia and Precapillary Pulmonary Hypertension

Large Granular Lymphocyte Leukemia and Precapillary Pulmonary Hypertension

SAT0334 PERICARDIAL INVOLVEMENT IN SYSTEMIC SCLEROSIS

Background:Systemic sclerosis (SSc) is an autoimmune rheumatic disease characterized by visceral and skin fibrosis, vascular dysfunction, and immune dysregulation. Regarding cardiac manifestations, pericardial disorder is one of the most frequent but often asymptomatic.Objectives:To analyze clinical manifestations, diagnostic tools and treatments of a patient cohort with SSc and pericardial involvement associated.Methods:A descriptive, observational, cross-sectional study was carried out. We included all patients between 1975 and 2019 with diagnosis of SSc. Demographic, clinical and analytical data; imaging tests; treatments; and mortality rate were collected.Results:158 patients were included, 142 (89.9%) women and 16 (10.1%) men. 144 (91.1%) were Caucasians. Mean age at diagnosis was 57 years (range: 17-86). Type of scleroderma, clinical manifestations and autoimmunity profile were shown in table 1 and 2, respectively.Table 1.Clinical manifestationsClinical manifestationsn (%)Cutaneous- Limited104 (66.5%)- Diffuse40 (25.3%)- Sine13 (8.2%)Raynaud’s phenomenon156 (98.7%)Digital tip ulcers63 (39.9%)Telangiectasia111 (70.3%)Calcinosis29 (18.4%)Tendon friction rubs3 (1.9%)Joint involvement44 (27.6%)Musle involvement13 (8.2%)Interstitial Lung Disease46 (28.9%)Gastrointestinal100 (62.9%)Pulmonary hypertension22 (13.8%)Renal1 (0.6%)15 (9.4%) patients developed a pericardial disorder, 3 of them (1.8%) an acute pericarditis. In the majority of the patients (11, 73.3%) pericardial involvement appeared later than diagnosis of SSc with a mean time of 8 years (maximum period of time: 20 years), and the remaining 4 (2.5%) presented the pericardial involvement prior to SSc onset (up to 4 years before). Regarding to cutaneous involvement, limited SSc was the most frequent (13 patients, 86.6%), followed by diffuse SSc (3 patients, 13.4%). In relation to antibody profile, 11 (73.3%) patients were AC positive and 4 (26.6%) AT positive. Seven (46.6%) of the 15 patients with pericardial involvement had a prior pulmonary hypertension associated. Eleven (73.3%) were asymptomatic, 4 (26.6%) referred chest pain and 4 (26.6%) dyspnea. 100% of the pericardial effusions were detected through the performance of an echocardiogram, 3 of them (13.4%) with signs of diastolic dysfunction. Regarding to electrocardiographical findings, 2 (13.3%) showed low voltages. The size of the pericardial effusion was described as: mild in 9 (60%) patients, moderate in 5 (33.3%) and severe in 1 (6.7%). One Cardiac- Magnetic Resonance Imaging was performed which showed constriction data. None of the patients suffered cardiac tamponade or hemodynamic compromise, due to invasive therapies were not necessary. A spontaneous resolution of the pericardial effusion was registered in 6 (40%) patients, with a mean time of 8 months (range: 2-36 months); and 1 (6.7%) patient after an increment in immunosuppressive dose, without improvement of pulmonary hypertension. No renal crisis or deaths were recorded in these patients.Conclusion:In our cohort patient with SSc, 9.5% developed a pericardial involvement. This kind of disorder could be the first manifestation of the disease. Pulmonary hypertension is the most frequent underlying pathway, however serositis must also be taken into account. Most part of the pericardial effusions were mild. No hemodynamic compromise was registered, due to an invasive treatment was not required.Table 2.Antibody profileAntibodiesn (%)Anticentromere (AC) +92 (58.2%)Anti- topoisomerase I (AT) +36 (22.8%)Antinuclear (ANA) +155 (97.5 %)

Echocardiographic Changes with Positive Airway Pressure Therapy in Obesity Hypoventilation Syndrome. Long-Term Pickwick Randomized Controlled Clinical Trial.

Rationale: Obesity hypoventilation syndrome (OHS) has been associated with cardiac dysfunction. However, randomized trials assessing the impact of long-term noninvasive ventilation (NIV) or continuous positive airway pressure (CPAP) on cardiac structure and function assessed by echocardiography are lacking.Objectives: In a prespecified secondary analysis of the largest multicenter randomized controlled trial of OHS (Pickwick Project; N = 221 patients with OHS and coexistent severe obstructive sleep apnea), we compared the effectiveness of three years of NIV and CPAP on structural and functional echocardiographic changes.Methods: At baseline and annually during three sequential years, patients underwent transthoracic two-dimensional and Doppler echocardiography. Echocardiographers at each site were blinded to the treatment allocation. Statistical analysis was performed using a linear mixed-effects model with a treatment group and repeated measures interaction to determine the differential effect between CPAP and NIV.Measurements and Main Results: A total of 196 patients were analyzed: 102 were treated with CPAP and 94 were treated with NIV. Systolic pulmonary artery pressure decreased from 40.5 ± 1.47 mm Hg at baseline to 35.3 ± 1.33 mm Hg at three years with CPAP, and from 41.5 ± 1.56 mm Hg to 35.5 ± 1.42 with NIV (P < 0.0001 for longitudinal intragroup changes for both treatment arms). However, there were no significant differences between groups. NIV and CPAP therapies similarly improved left ventricular diastolic dysfunction and reduced left atrial diameter. Both NIV and CPAP improved respiratory function and dyspnea.Conclusions: In patients with OHS who have concomitant severe obstructive sleep apnea, long-term treatment with NIV and CPAP led to similar degrees of improvement in pulmonary hypertension and left ventricular diastolic dysfunction.Clinical trial registered with www.clinicaltrials.gov (NCT01405976).

PULMONARY HYPERTENSION IN ESSENTIAL THROMBOCYTOSIS: IS HYDROXYUREA A POTENTIAL TREATMENT? A CASE REPORT

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Essential thrombocytosis is a myeloproliferative disorder which is associated with group 5 pulmonary hypertension (PH). PH is uncommonly prevalent in patients with myeloproliferative disease. Here we present a case of essential thrombocytosis with elevated right ventricular systolic pressures that showed significant improvement with initiation of hydroxyurea. CASE PRESENTATION: 79 year old Caucasian female with past medical history of chronic obstructive pulmonary disease , paroxysmal atrial fibrillation, essential thrombocytosis who initially presented with shortness of breath. Physical examination revealed decreased bilateral air entry, with a loud pulmonary component of the second heart sound and a grade 2 systolic ejection murmur in the left lower sternal border. Laboratory studies revealed a platelet count of 1,368 x 103/cumm. Initial admission echocardiogram confirmed the physical findings revealing severe tricuspid regurgitation and severe pulmonary hypertension with right ventricular systolic pressure(RSVP) of 94 mm Hg. The interventricular septum was flattened , consistent with right heart pressure overload. A previous echocardiogram done 5 years ago demonstrated mild pulmonary hypertension with RVSP of 45 mmHg and mild tricuspid regurgitation. Computed tomography pulmonary angiogram performed during the admission did not show any significant pulmonary vascular pathology. Patient had discontinued hydroxyurea as an outpatient on her own due to a non-healing ankle ulcer. She was restarted on hydroxyurea and discharged on the same. Patient was readmitted two months later with cough and worsening expectoration, low-grade fever and was treated for an exacerbation of her underlying chronic obstructive pulmonary disease. A repeat echocardiogram performed during this admission for concerns of worsening pulmonary hypertension showed a significant improvement in pulmonary hypertension with right ventricular systolic pressure of 60 mmHg. Platelet count also improved to 389 x 103/cumm. DISCUSSION: We suspect pulmonary hypertension can have a significant impact on morbidity and mortality in patients with myeloproliferative disorder. One of the studies revealed an incidence of pulmonary hypertension in about 47.8%(1 )in patients with essential thrombocytosis. Pulmonary hypertension is often asymptomatic (1,2,3 )in these patients and treatment modalities have not been identified and established. The beneficial effect of hydroxyurea could be just due to its effect on underlying myeloproliferative disease by inactivating ribonucleotide reductase and inhibiting DNA synthesis causing cell death or via a alternate mechanism which needs to be studied at this point(4). CONCLUSIONS: The case report emphasizes the need for early evaluation of PH in myeloproliferative disorder. There is also a need for further studies regarding the use of hydroxyurea as a treatment modality for such cases. Reference #1: 1. Altintas A, Karahan Z, Pasa S, et al. Pulmonary hypertension in patients with essential thrombocythemia and reactive thrombocytosis. Leuk Lymphoma 2007;48(10):1981–1987. Reference #2: 2. Kadikoylu G, Onbasili A, Tekten T, Barutca S, Bolaman Z. Functional and morphological cardiac changes in myeloproliferative disorders (clinical study). Int J Cardiol 2004;97(2):213–220. Reference #3: 3. Dingli D, Utz JP, Krowka MJ, Oberg AL, Tefferi A. Unexplained Pulmonary Hypertension in Chronic Myeloproliferative Disorders. CHEST 2001;120(3):801–808. 4. Yarbro JW. Mechanism of action of hydroxyurea. Semin Oncol 1992 Jun;19(3 Suppl 9):1-10 DISCLOSURES: No relevant relationships by Shaylika Chauhan, source=Web Response No relevant relationships by Akhilesh Kumar Khuttan, source=Web Response No relevant relationships by Arjun Madhavan, source=Web Response No relevant relationships by Shalini Tripathi, source=Web Response

PB2145 POMALIDOMIDE AND DEXAMETHASONE IN RELAPSING POEMS SYNDROME: A FRENCH REPORT OF 5 CASES

Background:Significant improvements have been made in the management of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy and skin changes) syndrome, but patients (pts) still experience relapses or refractoriness. Immunomodulatory drugs are effective agents in the treatment of plasma cells dyscrasias. Lenalidomide has shown interesting activity in POEMS syndrome, but no data on pomalidomide have been published so far.Aims:We report the use of pomalidomide and dexamethasone in five patients with relapsing POEMS syndrome after lenalidomide regimen.MethodsDemographics: 5 patients (3 men), median age 74 years old (range, 67–77), median of 2,6 previous lines received (range, 2–4). All patients had received lenalidomide as previous therapy.Results:Hematological overall response rate (ORR) was 3/5 (60%): all 3‐responding patients experienced complete response (CR) according to local evaluation based on IMWG criteria, leading to clinical improvement of neuropathy in all of them, endocrinopathy and peripheral edema (2 of 2 pts). Other clinical responses concerned resolution of papilledema (2 of 2 pts) and improvement of pulmonary hypertension (1 of 1 pt). Noteworthy, serum Vascular Endothelial Growth Factor (sVEGF) level normalized after only one cycle of treatment in those 3‐responding patients (figure 1). No emergent toxicity was observed. As expected, adverse events were mainly hematological but of mild intensity and easily manageable. At this time, the 3‐responding patients are alive, including 2 without relapse after 11 months of follow‐up for both of them. The 2 other patients died, one of them from rapidly progressing disease, the other from heart disease not related to POEMS.Summary/Conclusion:In conclusion, pomalidomide and dexamethasone appears to be a safe and effective option in relapsing POEMS syndrome after lenalidomide regimen, demonstrating rapid and durable responses.image

Aspiration Thrombectomy for Treatment of Acute Massive and Submassive Pulmonary Embolism: Initial Single-Center Prospective Experience

PurposeTo evaluate the feasibility of aspiration thrombectomy in patients with acute massive or submassive pulmonary embolism (PE). Materials and MethodsThis prospective study analyzed patient demographic data, procedural details, and outcomes in 18 consecutive patients (8 men and 10 women; mean age, 60.1 y; range, 36–80 y), 10 with acute submassive PE and 8 with massive PE, treated with an Indigo Continuous Aspiration Mechanical Thrombectomy Catheter between January 2016 and February 2017. Three patients underwent concomitant systemic fibrinolytic treatment with 100 mg tissue plasminogen activator. Technical success was defined as successful placement of devices and initiation of aspiration thrombectomy. Clinical success was defined as stabilization of hemodynamic parameters; improvement in pulmonary hypertension, right heart strain, or both; and survival to hospital discharge. Complications were also analyzed. ResultsThe procedure was considered a technical success in 17 patients (94.4%) and a clinical success in 15 (83.3%). Echocardiography showed significant improvements in right ventricle size (46.36 mm ± 2.2 before treatment vs 41.79 mm ± 7.4 after; P = .041), tricuspid annular plane systolic excursion (16 ± 3 before treatment vs 18.57 ± 3.9 after; P = .011), and systolic wave (10 ± 2.1 before treatment vs 13.1 ± 3.8 after; P = .020). Two patients died of massive PE, and 1 died of submassive PE. Two patients who received systemic fibrinolytic agents experienced intracranial bleeding, and abdominal bleeding developed in 1. ConclusionsAspiration thrombectomy is a feasible option for the treatment of acute massive or submassive PE in patients with hemodynamic compromise or right ventricular dysfunction.

Two-dimensional knowledge-based volumetric reconstruction of the right ventricle documents short-term improvement in pulmonary hypertension.

Data are scarce about short-term right ventricular changes in pulmonary hypertension. Two-dimensional knowledge-based reconstruction of the right ventricle with 2D echocardiography (2DKBR) has been shown to be a valid alternative to Cardiac MRI. In this longitudinal study 25 pulmonary hypertension patients underwent 2DKBR of the right ventricle, assessment of NT-proBNP levels and functional class at baseline and after a mean follow-up of 6.1months. Patients were followed up clinically for a further mean of 8.2months. The majority of patients had connective tissue disease (CTD) associated pulmonary arterial hypertension (n=15) or chronic thromboembolic pulmonary hypertension (CTEPH; n=6). A total of 15 patients underwent an intervention, either new targeted therapy, escalation of targeted therapy or pulmonary endarterectomy. A total of 10 clinically stable patients were routinely followed up without any change in therapy. There were significant improvements in the right ventricular end-diastolic volume index (111±29mL/m² vs 100±36mL/m²; P=.038), end-systolic volume index (72±23mL/m² vs 61±25mL/m²; P=.001), and ejection fraction (35±10% vs 40±9%; P=.030). Changes in NT-proBNP levels correlated strongest with changes in end-systolic volume index (r=-.77; P=<.0001). Four patients experienced clinical worsening during extended follow-up, dilatation of the right ventricle was associated with clinical worsening. In a CTD and CTEPH dominated patient population significant reverse remodeling and improvement of ejection fraction occurred despite a short follow-up and was paralleled by significant changes in NT-proBNP levels. Further right ventricular dilatation was associated with worse clinical outcome. 2DKBR is a feasible substitute for Cardiac MRI to follow-up right ventricular indices in pulmonary hypertension.

Phosphodiesterase type 5 inhibitor to riociguat transition is associated with hemodynamic and symptomatic improvement in pulmonary hypertension.

Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. We studied the clinical and hemodynamics effects of transitioning 12 pulmonary hypertension patients from Phosphodiesterase type 5 inhibitor (PDE5i) to riociguat, and demonstrated a significant increase in cardiac index, fall in pulmonary vascular resistance, and improvement in functional class with this switch. Switch from PDE5i to riociguat appeared to be safe and fairly well tolerated in most patients.

ELECTROCARDIOGRAPHIC CHANGES OF RIGHT VENTRICULAR STRAIN DO NOT PREDICT HEMODYNAMIC OR CLINICAL IMPROVEMENT IN PATIENTS UNDERGOING PULMONARY THROMBOENDARTERECTOMY (PTE)

Electrocardiographic (ECG) changes track hemodynamic and clinical improvement in pulmonary hypertension. We evaluated those changes predictive of favorable outcomes in a cohort of chronic thromboembolic pulmonary hypertension patients undergoing pulmonary thromboendarterectomy (PTE) at our center

4:03 PMAbstract No. 214 - Improvement of pulmonary hypertension and right ventricular dysfunction after ultrasound-accelerated catheter-directed thrombolysis for submassive pulmonary embolism

4:03 PMAbstract No. 214 - Improvement of pulmonary hypertension and right ventricular dysfunction after ultrasound-accelerated catheter-directed thrombolysis for submassive pulmonary embolism

Positive Airway Pressure Therapy for Sleep Disordered Breathing in Congestive Heart Failure is Associated with Reduction in Pulmonary Artery Systolic Pressure

Background: Sleep disordered breathing and pulmonary hypertension are both highly prevalent in patients with congestive heart failure (CHF). Since (SDB) has been shown to increase pulmonary pressures, we hypothesized that initiation of positive airway pressure (PAP) may reduce pulmonary artery systolic pressure (PASP) in compliant patients. Methods: A total of 125 consecutive CHF patients admitted for decompensation were screened. Of these patients, 18 met inclusion criteria for the study which included a PASP ≥ 36 and polysomnogram-proven SDB. A repeat echocardiogram performed at mean 8 weeks post intervention was reviewed to compare PASP. Results: There was no statistically significant difference in mean age, BMI, AHI, baseline PASP, and ejection fraction between the PAP-compliant group and PAP non-compliant group. A statistically significant mean reduction in PASP of 13.8 ± 7.0 mmHg (range: -25, -1) was observed in CHF patients with SDB who were compliant with PAP therapy, versus a mean increase of 0.9 ± 8.7 mmHg (range: -8, 15) in heart failure patients with SDB who were noncompliant with PAP therapy. Conclusions: The findings suggest that PAP therapy is associated with improvement in pulmonary hypertension with congestive heart failure and sleep disordered breathing.

Pulmonary Embolism Response to Fragmentation, Embolectomy, and Catheter Thrombolysis (PERFECT): Initial Results From a Prospective Multicenter Registry

Systemic thrombolysis for acute pulmonary embolism (PE) carries up to a 20% risk of major bleeding, including a 2% to 5% risk of hemorrhagic stroke. We evaluated the safety and effectiveness of catheter-directed therapy (CDT) as an alternative treatment of acute PE. One hundred one consecutive patients receiving CDT for acute PE were prospectively enrolled in a multicenter registry. Massive PE (n = 28) and submassive PE (n = 73) were treated with immediate catheter-directed mechanical or pharmacomechanical thrombectomy and/or catheter-directed thrombolysis through low-dose hourly drug infusion with tissue plasminogen activator (tPA) or urokinase. Clinical success was defined as meeting all the following criteria: stabilization of hemodynamics; improvement in pulmonary hypertension, right-sided heart strain, or both; and survival to hospital discharge. Primary safety outcomes were major procedure-related complications and major bleeding events. Fifty-three men and 48 women (average age, 60 years [range, 22-86 years]; mean BMI, 31.03 ± 7.20 kg/m2) were included in the study. The average thrombolytic doses were 28.0 ± 11 mg tPA (n = 76) and 2,697,101 ± 936,287 International Units for urokinase (n = 23). Clinical success was achieved in 24 of 28 patients with massive PE (85.7%; 95% CI, 67.3%-96.0%) and 71 of 73 patients with submassive PE (97.3%; 95% CI, 90.5%-99.7%). The mean pulmonary artery pressure improved from 51.17 ± 14.06 to 37.23 ± 15.81 mm Hg (n = 92) (P < .0001). Among patients monitored with follow-up echocardiography, 57 of 64 (89.1%; 95% CI, 78.8%-95.5%; P < .0001) showed improvement in right-sided heart strain. There were no major procedure-related complications, major hemorrhages, or hemorrhagic strokes. CDT improves clinical outcomes in patients with acute PE while minimizing the risk of major bleeding. At experienced centers, CDT is a safe and effective treatment of both acute massive and submassive PE. ClinicalTrials.gov; No.: NCT01097928; URL: www.clinicaltrials.gov.