J Coll Physicians Surg Pak. 2018 Aug;28(8):594-596. doi: 10.29271/jcpsp.2018.08.594.

Pulmonary Hypertension: An Emerging Problem in Patients Undergoing Regular Hemodialysis.

Afzal A¹, Bhatti MA², Manzoor S³.

Author information:

1. Department of Nephrology, Lahore General Hospital, Lahore.
2. Department of Medicine, Lahore General Hospital, Lahore.
3. Department of Pulmonology, Lahore General Hospital, Lahore.

Abstract

OBJECTIVE:
To find out the frequency of pulmonary hypertension (PH) and its association with various contributing factors in patients undergoing regular hemodialysis (HD) due to end-stage renal disease (ESRD).

STUDY DESIGN:
Cross-sectional analytical study.

PLACE AND DURATION OF STUDY:
Nephrology Department, Lahore General Hospital, Pakistan, from July to December 2016.

METHODOLOGY:
Fifty patients with end-stage renal disease. Various parameters, including the pulmonary arterial systolic pressure (PASP), were recorded. Pulmonary arterial pressure greater than 25 mmHg at rest was defined as pulmonary hypertension. It was further divided into three sub categories as mild (25-40 mmHg), moderate (40-55 mmHg), and severe (greater than 55 mmHg). Data were correlated with age, gender, body mass index, systemic hypertension, diabetes, duration of dialysis, and Hb (hemoglobin) concentration. Data was analysed using SPSS version 23.0.

RESULTS:
The median (IQR) duration of dialysis was 12 (11.25) months. Eighteen (36%) patients were found to have PH. It is greater in patients who had been on dialysis for more than 5 months. A positive association was seen between the duration of dialysis and the prevalence of PH (p=0.024).

CONCLUSION:
A considerable number of patients on hemodialysis have pulmonary hypertension which is associated with the longer duration of maintenance hemodialysis.

PMID: 30060786

Cardiovasc J Afr. 2018 Jul 7;29:1-5. doi: 10.5830/CVJA-2018-007. [Epub ahead of print]

Pulmonary hypertension as seen in a rural area in sub-Saharan Africa: high prevalence, late clinical presentation and a high short-term mortality rate during follow up.

Dzudie A¹, Dzekem BS², Tchoumi CT³, Aminde LN⁴, Mocumbi AO⁵, Abanda M², Thienemann F⁶, Kengne AP⁷, Sliwa K⁸.

Author information:

1. Departments of Internal Medicine and Physiology, Faculty of Medicine, University of Yaoundé, Yaoundé, Cameroon; Douala General Hospital and Clinical Research Education, Networking and Consultancy, Douala, Cameroon; Soweto Cardiovascular Research Group, Department of Medicine, University of the Witwatersrand, Johannesburg, South Africa; NIH Millennium Fogarty Chronic Disease Leadership Programme.
2. Departments of Internal Medicine and Physiology, Faculty of Medicine, University of Yaoundé, Yaoundé, Cameroon; Douala General Hospital and Clinical Research Education, Networking and Consultancy, Douala, Cameroon.
3. Shisong Cardiac Centre, Kumbo, Cameroon.
4. Douala General Hospital and Clinical Research Education, Networking and Consultancy, Douala, Cameroon; School of Public Health, Faculty of Medicine and Biomedical Sciences, University of Queensland, Brisbane, Australia.
5. Instituto Nacional de Saúde, and Faculty of Medicine, Eduardo Mondlane University, Maputo, Mozambique.
6. Clinical Infectious Diseases Research Initiative, Institute of Infectious Diseases and Molecular Medicine, Faculty of Health Science, University of Cape Town, Cape Town, South Africa.
7. Non-communicable Diseases Unit, South African Medical Research Council, Cape Town, South Africa.
8. NIH Millennium Fogarty Chronic Disease Leadership Programme; Hatter Institute for Cardiovascular Research in Africa, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.

Abstract

INTRODUCTION:
The epidemiology of pulmonary hypertension (PH) in low- to middle-income countries is poorly characterised. We assessed the prevalence, baseline characteristics and mortality rate in patients with echocardiographically diagnosed PH at a rural cardiac centre in Cameroon.

METHODS:
We conducted a prospective cohort study in a subsample of 150 participants, aged 18 years and older, diagnosed with PH [defined as right ventricular systolic pressure (RVSP) ≥ 35 mmHg in the absence of pulmonary stenosis and right heart failure]. PH was classified as mild (RVSP: 35-50 mmHg), moderate (RVSP: 51-60 mmHg) and severe (RVSP: > 60 mmHg).

RESULTS:
Of 2 194 patients screened via echocardiograms, 343 (crude prevalence 15.6%) had PH. The sub-sample of 150 patients followed up (54.7% women, mean age of 62.7 ± 18.7 years) had a mean RVSP of 68.6 mmHg. They included 7.3% mild, 29.3% moderate and 63.4% severe PH cases. Co-morbidities included log smoke (80.7%), hypertension (52.0%), family history of cardiovascular disease (50.0%), diabetes (31.3%), alcohol abuse (21.3%) and HIV infection (8.7%). Main clinical features were dyspnoea (78.7%), fatigue (76.7%), palpitations (57.3%), cough (56.7%), jugular venous distension (68%) and peripheral oedema (66.7%). Overall, 70% presented in World Health Organisation functional class III/IV. PH due to left heart disease (PHLHD) was the commonest (64.7%), and rheumatic valvular disease accounted for 36.1%. The six-month mortality rate was 28%.

CONCLUSION:
PH, dominated by PHLHD, was common among adults attending this rural centre and was associated with a high mortality rate. Related co-morbidities and late clinical presentation reflect the poor socio-economic context. Improved awareness of PH among physicians could promote early diagnosis and management.

PMID: 30059129

Eur Rev Med Pharmacol Sci. 2018 Jul;22(14):4683-4690. doi: 10.26355/eurrev_201807_15529.

Study on sildenafil combined with inhalational nitric oxide therapy on the curative effects and serum levels of HIF-1α, ET-1, and calcium in neonatal pulmonary hypertension.

Liu G¹, Wu HW, Li ZG.

Author information:

1. Department of Neonatology, Xuzhou Children’s Hospital, Xuzhou, Jiangsu, China. zhenguangli29@163.com.

Abstract

OBJECTIVE:
To explore the effect of sildenafil combined with inhalational nitric oxide (NO) therapy on the curative effects and serum levels of hypoxia-inducible factor (HIF)-1α, endothelin-1 (ET-1), and calcium in persistent pulmonary hypertension of the newborn (PPHN).

PATIENTS AND METHODS:
Eighty-six patients with neonatal pulmonary hypertension treated in Xuzhou Children’s Hospital from March 2015 to February 2016 were randomly divided into the observation group and control group, treated with sildenafil and sildenafil combined with inhalational NO, respectively. The clinical efficacy of newborns in the two groups was compared. Fraction of inspiration O2 (FiO2), Oxygen Index (OI), blood oxygen partial pressure (PaO2), blood oxygen saturation (SpO2), and pulmonary arterial pressure of newborns in the two groups were compared before treatment and 2 h, 12 h, and 24 h after treatment. The serum levels of HIF-1α, ET-1, and calcium of patients in the two groups were compared before treatment and 3, 5, 7 days after treatment.

RESULTS:
The total effective rate of the observation group (95.34%) was significantly higher than that of the control group (74.41%) (p<0.05). After treatment, FiO2, OI, and pulmonary arterial pressure of patients in the two groups decreased, and the decrease in the observation group was significantly lower than in the control group (p<0.05). After treatment, PaO2 and SpO2 of patients in the observation group were higher than those of the control group. The levels of HIF-1α and ET-1 of patients in the two groups decreased and were significantly lower in the observation group compared with the control group. The levels of calcium of patients in the two groups increased and were significantly higher in the observation group than the control group (p<0.05).

CONCLUSIONS:
Sildenafil combined with inhalational NO therapy for neonatal pulmonary hypertension can quickly improve oxygenation, effectively reduce pulmonary arterial hypertension, and is worthy of clinical application.

PMID: 30058708

Clin Transplant. 2018 Jul 29:e13364. doi: 10.1111/ctr.13364. [Epub ahead of print]

Initial experience with bosentan for the management of pulmonary hypertension after heart transplantation.

Santiago-Vacas E¹, Farrero M¹, Ivey-Miranda JB¹, Castel MÁ¹, García-Álvarez A¹, Rios J², Perez-Villa F¹.

Author information:

1. Heart Failure and Heart Transplantation Unit, Cardiovascular Institute, Hospital Clinic, Barcelona, Spain.
2. Medical Statistics Core Facility, August Pi and Sunyer Biomedical Research Institute (IDIBAPS), Biostatistics Unit, Faculty of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain.

Abstract

BACKGROUND:
Pulmonary hypertension (PH) after heart transplantation (HT) is associated to right ventricular (RV) dysfunction and increased morbidity and mortality. We present our experience with bosentan for the treatment of PH after HT.

METHODS:
A retrospective evaluation of patients with PH receiving bosentan post-transplant was performed. Pulmonary hemodynamics before and after bosentan (BG) and clinical outcomes were assessed and compared to a historical control group (CG) not receiving bosentan.

RESULTS:
Between 2013 and 2016, 21 patients were treated post-transplant with bosentan. Twenty-four hours after bosentan initiation, there were significant decreases in systolic (42.5 ± 8 to 38.1 ± 8 mm Hg, P = 0.015), diastolic (21.4 ± 4 to 17.8 ± 6 mm Hg, P = 0.008) and mean (29.6 ± 5 to 25 ± 6 mm Hg, P = 0.001) pulmonary artery pressures (PAP), transpulmonary gradient (13.1 ± 3 to 9.7 ± 4 mm Hg, P < 0.001), diastolic gradient (5.2 ± 4 to 2.3 ± 3 mm Hg, P = 0.001) and pulmonary vascular resistance (PVR) (2.2 ± 1 to 1.6 ± 1WU, P = 0.015). This effect was maintained at day 3. Compared with CG, BG showed significantly more decrease in PVR (0.7 ± 0.9 vs 0.3 ± 1.7WU, P = 0.025) and mean PAP (4.6 ± 5.2 vs 1.5 ± 4.4 mm Hg, P = 0.040). RV function 7 days post-transplant was significantly better in BG compared to CG, P = 0.004. There were not clinically significant interactions between bosentan and immunosuppressive treatment.

CONCLUSIONS:
Bosentan, initiated early post-transplant, was associated with a significant decrease in PVR. Bosentan was well tolerated and did not interact with immunosuppressive treatment.

© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

PMID: 30058129

Cardiovasc Diagn Ther. 2018 Jun;8(3):279-296. doi: 10.21037/cdt.2018.01.10.

Imaging of pulmonary hypertension: an update.

Goerne H^1,2, Batra K³, Rajiah P³.

Author information:

1. IMSS Centro Medico Nacional De Occidente, Guadalajara, Jalisco, Mexico.
2. CID Imaging and Diagnostic Center, Guadalajara, Jalisco, Mexico.
3. Radiology Department, UT Southwestern Medical Center, Dallas, Texas, USA.

Abstract

Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure caused by a broad spectrum of congenital and acquired disease processes, which are currently divided into five groups based on the 2013 WHO classification. Imaging plays an important role in the evaluation and management of PH, including diagnosis, establishing etiology, quantification, prognostication and assessment of response to therapy. Multiple imaging modalities are available, including radiographs, computed tomography (CT), magnetic resonance imaging (MRI), nuclear medicine, echocardiography and invasive catheter angiography (ICA), each with their own advantages and disadvantages. In this article, we review the comprehensive role of imaging in the evaluation of PH.

Free PMC Article
PMCID: PMC6039805
PMID: 30057876

Cardiovasc Diagn Ther. 2018 Jun;8(3):272-278. doi: 10.21037/cdt.2018.06.07.

Chronic thromboembolic pulmonary hypertension: emerging endovascular therapy.

Menon K¹, Sutphin PD¹, Bartolome S², Kalva SP¹, Ogo T³.

Author information:

1. Department of Interventional Radiology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
2. Department of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.
3. Department of Advanced Medicine for Pulmonary Hypertension, National Cerebral and Cardiovascular Centre, Osaka, Japan.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a debilitating but potentially reversible complication of chronic pulmonary thromboembolic disease characterized by progressive right heart dysfunction secondary to pulmonary arterial stenosis or occlusion. Balloon pulmonary angioplasty (BPA) has recently emerged as an alternative intervention for non-surgical candidates with CTEPH. Modern reperfusion angioplasty techniques relieve sequela of chronic pulmonary hypertension, ameliorate right ventricular failure, and improve functional status. This article will discuss the diagnosis and treatment of patients with CTEPH and the current state of endovascular management with BPA.

Free PMC Article
PMCID: PMC6039800
PMID: 30057875

Can J Cardiol. 2018 Aug;34(8):1069-1078. doi: 10.1016/j.cjca.2018.04.016. Epub 2018 Apr 25.

Prognostic Value of Right Ventricular Strain Using Speckle-Tracking Echocardiography in Pulmonary Hypertension: A Systematic Review and Meta-analysis.

Shukla M¹, Park JH², Thomas JD³, Delgado V⁴, Bax JJ⁴, Kane GC⁵, Howlett JG¹, White JA¹, Fine NM⁶.

Author information:

1. Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular Institute of Alberta, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
2. Cardiology Division of Internal Medicine, Chungnam National University, Chungnam National University Hospital, Daejeon, Korea.
3. Centre for Heart Valve Disease, Bluhm Cardiovascular Institute, Northwestern Memorial Hospital, Chicago, Illinois, USA.
4. Department of Cardiology, Leiden University Medical Centre, Leiden, the Netherlands.
5. Division of Cardiovascular Ultrasound, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.
6. Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular Institute of Alberta, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. Electronic address: nmfine@ucalgary.ca.

Abstract

BACKGROUND:
Right ventricular (RV) strain imaging using speckle-tracking echocardiography (STE) is a quantitative method of assessing RV systolic function that has shown prognostic utility in patients with pulmonary hypertension (PH). However, its prognostic value for a large and mixed PH population remains poorly defined.

METHODS:
A systematic review and meta-analysis was performed using the MedLine, Embase, and Cochrane Library databases for publications reporting the prognostic value of RV strain calculated using 2-dimensional STE in PH patients for the clinical end point of all-cause mortality.

RESULTS:
Screening of 687 publications yielded 10 that were included in the meta-analysis, representing data for 1001 PH patients, among whom 76% had pulmonary arterial hypertension with the remainder having a range of PH etiologies. The pooled free wall RV strain was -16.2% (95% confidence interval [CI], -14.3 to -18.1; I2 = 94.1%; Q = 102.8; P < 0.001), and the global (free wall and septum) RV strain was -14.5% (95% CI, -12.9 to -16; I2 = 84.9%; Q = 20; P < 0.001). There were 193 (18%) deaths (follow-up period range, 7.4 months to 4.2 years). From 6 publications, the pooled unadjusted hazard ratio for a binary cut off RV strain value for the primary outcome was 3.67 (95% CI, 2.82-4.77; P < 0.001; I2 = 0; Q = 1.8; P = 0.87), whereas the pooled unadjusted hazard ratio of RV strain as a continuous variable (per 1% change) was 1.14 (95% CI, 1.11-1.8; P < 0.001; I2 = 0; Q = 2.0; P = 0.85), and were superior to corresponding values for tricuspid annular systolic plane excursion (1.45; P = 0.071, hazard ratio = 1.00, and P = 0.82, respectively).

CONCLUSIONS:
RV strain performed using 2-dimensional STE provides important prognostic value within a large and mixed population of PH patients.

Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

PMID: 30056845

Ann Card Anaesth. 2018 Jul-Sep;21(3):328-332. doi: 10.4103/aca.ACA_19_18.

Inhaled levosimendan versus intravenous levosimendan in patients with pulmonary hypertension undergoing mitral valve replacement.

Kundra TS¹, Nagaraja PS¹, Bharathi KS¹, Kaur P², Manjunatha N¹.

Author information:

1. Department of Cardiac Anaesthesia, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India.
2. Department of Critical Care, Sir Ganga Ram Hospital, New Delhi, India.

Abstract

Context:
Inhaled levosimendan may act as selective pulmonary vasodilator and avoid systemic side effects of intravenous levosimendan, which include decrease in systemic vascular resistance (SVR) and systemic hypotension, but with same beneficial effect on pulmonary artery pressure (PAP) and right ventricular (RV) function.

Aim:
The aim of this study was to compare the effect of inhaled levosimendan with intravenous levosimendan in patients with pulmonary hypertension undergoing mitral valve replacement.

Settings and Design:
The present prospective randomized comparative study was conducted in a tertiary care hospital.

Subjects and Methods:
Fifty patients were randomized into two groups (n = 25). Group A: Levosimendan infusion was started immediately after coming-off of cardiopulmonary bypass and continued for 24 h at 0.1 mcg/kg/min. Group B: Total dose of levosimendan which would be given through intravenous route over 24 h was calculated and then divided into four equal parts and administered through inhalational route 6th hourly over 24 h. Hemodynamic profile (pulse rate, mean arterial pressure, pulmonary artery systolic pressure [PASP], SVR) and RV function were assessed immediately after shifting, at 1, 8, 24, and 36 h after shifting to recovery.

Statistical Analysis Used:
Intragroup analysis was done using paired student t-test, and unpaired student t-test was used for analysis between two groups.

Results:
PASP and RV-fractional area change (RV-FAC) were comparable in the two groups at different time intervals. There was a significant reduction in PASP and significant improvement in RV-FAC with both intravenous and inhalational levosimendan. SVR was significantly decreased with intravenous levosimendan, but no significant decrease in SVR was observed with inhalational levosimendan.

Conclusions:
Inhaled levosimendan is a selective pulmonary vasodilator. It causes decrease in PAP and improvement in RV function, without having a significant effect on SVR.

Free Article
PMID: 30052230

Conflict of interest statement
There are no conflicts of interest

Adv Exp Med Biol. 2018;1065:511-528. doi: 10.1007/978-3-319-77932-4_31.

The Role of Sex in the Pathophysiology of Pulmonary Hypertension.

Docherty CK¹, Harvey KY¹, Mair KM¹, Griffin S¹, Denver N¹, MacLean MR².

Author information:

1. Research Institute of Cardiovascular and Medical Sciences, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK.
2. Research Institute of Cardiovascular and Medical Sciences, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK. mandy.maclean@glasgow.ac.uk.

Abstract

Pulmonary arterial hypertension (PAH) is a progressive disease characterised by increased pulmonary vascular resistance and pulmonary artery remodelling as result of increased vascular tone and vascular cell proliferation, respectively. Eventually, this leads to right heart failure. Heritable PAH is caused by a mutation in the bone morphogenetic protein receptor-II (BMPR-II). Female susceptibility to PAH has been known for some time, and most recent figures show a female-to-male ratio of 4:1. Variations in the female sex hormone estrogen and estrogen metabolism modify FPAH risk, and penetrance of the disease in BMPR-II mutation carriers is increased in females. Several lines of evidence point towards estrogen being pathogenic in the pulmonary circulation, and thus increasing the risk of females developing PAH. Recent studies have also suggested that estrogen metabolism may be crucial in the development and progression of PAH with studies indicating that downstream metabolites such as 16α-hydroxyestrone are upregulated in several forms of experimental pulmonary hypertension (PH) and can cause pulmonary artery smooth muscle cell proliferation and subsequent vascular remodelling. Conversely, other estrogen metabolites such as 2-methoxyestradiol have been shown to be protective in the context of PAH. Estrogen may also upregulate the signalling pathways of other key mediators of PAH such as serotonin.

PMID: 30051404

Pediatr Cardiol. 2018 Apr;39(4):829-836. doi: 10.1007/s00246-018-1834-7. Epub 2018 Feb 23.

Cardiac Function Assessments in Left Bochdalek’s Hernia: Clinical Relevance.

Sehgal A^1,2, Tan K^3,4, Ferguson P⁵.

Author information:

1. Monash Newborn, Monash Children’s Hospital, 246, Clayton Road, Clayton, Melbourne, VIC, 3168, Australia. Arvind.Sehgal@monash.edu.
2. Department of Pediatrics, Monash University, Melbourne, Australia. Arvind.Sehgal@monash.edu.
3. Monash Newborn, Monash Children’s Hospital, 246, Clayton Road, Clayton, Melbourne, VIC, 3168, Australia.
4. Department of Pediatrics, Monash University, Melbourne, Australia.
5. Pediatric Surgery, Monash Health, Melbourne, Australia.

Abstract

The objectives of this study were to characterize peri-operative echocardiographic (ECHO) assessments of right ventricular (RV) function and pulmonary hypertension (PH) and ascertain correlation with clinical outcomes in infants with left Bochdalek’s hernia (LBH). This retrospective study in a quaternary neonatal intensive care unit involved electronic database search for infants with LBH during January 2009 to July 2017. Demographics, outcomes, and ECHO parameters were accessed from archived databases. Thirty-one infants with mean gestational age and birthweight (BW) 38.4 ± 1.4 weeks and 3079 ± 450 g were included. Postnatal age at surgery was 4.3 ± 2 days. Two infants died before surgery (none after surgery). The survival rate in this cohort of late preterm and term infants with LBH not accompanied by congenital heart disease was 29/31 (93.5%). PH was noted in 28 (90%). Significant changes in biventricular function and pulmonary vascular haemodynamics were noted when reassessed after surgery [RV area change (%), 28 ± 3 vs. 34 ± 4, p = 0.007 and pulmonary artery capacitance (PAC) (ml3 × mmHg-1), 0.05 ± 0.03 vs. 0.09 ± 0.05, p = 0.02]. The median duration of all respiratory support in those who were operated was 14.7 days (8.5, 25). Significant correlations were noted between pre-operative ECHO assessments and the duration of respiratory support (tricuspid regurgitation, r = 0.63, p = 0.0001 and PAC, r = - 0.52, p = 0.004). Peri-operative functional ECHO provided information about the evolution of cardiac function in infants with LBH.

PMID: 29473101 [Indexed for MEDLINE]

Endocr J. 2017 Dec 28;64(12):1173-1180. doi: 10.1507/endocrj.EJ17-0155. Epub 2017 Sep 9.

Effect of treatment with epoprostenol and endothelin receptor antagonists on the development of thyrotoxicosis in patients with pulmonary arterial hypertension.

Satoh M¹, Aso K¹, Nakayama T¹, Saji T².

Author information:

1. Department of Pediatrics, Toho University Omori Medical Center, Tokyo 143-8541, Japan.
2. Advanced and Integrated Cardiovascular Research Course in the Young and Adolescence, Toho University, Tokyo 143-8540, Japan.

Abstract

Thyroid disease is known to be associated with pulmonary arterial hypertension (PAH). We investigated the prevalence of thyroid disease in patients with idiopathic PAH (IPAH) or heritable PAH (HPAH), and the factors affecting the pathogenesis of thyroid disease. We retrospectively evaluated 59 patients with IPAH or HPAH who had been diagnosed with PAH before the age of 20 years. Thyrotoxicosis was detected in 12 of the 59 patients (6 patients with Graves’ disease, 3 with hashitoxicosis, and 3 with silent thyroiditis) after the start of PAH treatment. The proportion of patients who received epoprostenol in the thyrotoxicosis group was significantly higher than that in the euthyroid group (12/12 vs. 27/47, p=0.015). In the 39 patients treated with epoprostenol, the proportion of patients who received combination therapy with epoprostenol and an endothelin receptor antagonist (ERA) in the thyrotoxicosis group was significantly lower than that in the euthyroid group (5/12 vs. 23/27, p=0.016). Logistic regression analysis revealed that thyrotoxicosis development was significantly associated with administration of epoprostenol (odds ratio [OR] 8.22, 95% confidence interval [CI] 1.26-53.74, p=0.028) and non-administration of ERA (OR 5.33, 95% CI 1.29-22.06, p=0.021). The prevalence of thyrotoxicosis was high in patients with IPAH or HPAH. The onset of thyrotoxicosis might be promoted by epoprostenol and inhibited by ERA.

Free Article
PMID: 28890480 [Indexed for MEDLINE]

Clin Exp Rheumatol. 2018 Mar-Apr;36 Suppl 111(2):46-50. Epub 2017 Jun 12.

Cardiopulmonary involvement in Takayasu’s arteritis.

Brennan DN¹, Warrington KJ², Crowson CS³, Schmidt J⁴, Koster MJ².

Author information:

1. Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA. brennan.david1@mayo.edu.
2. Division of Rheumatology, Mayo Clinic, Rochester, MN, USA.
3. Health Sciences Research, Mayo Clinic, Rochester, MN, USA.
4. Amiens University Hospital and INSERM U-1088, Department of Internal Medicine and RECIF, Jules Verne University of Picardy, Amiens, France.

Abstract

OBJECTIVES:
To evaluate cardiopulmonary (CP) involvement in patients with Takayasu’s arteritis (TAK) and assess the impact on disease outcomes.

METHODS:
A retrospective cohort of patients with newly diagnosed TAK from 1984 to 2009 was assembled. Demographics, baseline disease characteristics, relapse events, surgeries and mortality were abstracted from direct medical record review. Angiograms, advanced imaging and cardiac studies were reviewed for evidence of CP involvement. Cox models with time-dependent covariates were used to assess the association between CP involvement and outcomes.

RESULTS:
A total of 124 patients with TAK were identified. Forty-five (36%) patients had at least one objective CP abnormality observed within 6 months of TAK diagnosis. Age at diagnosis was higher in those with CP involvement than those without (34.6 vs 30.1 yrs; p=0.04). Baseline characteristics and symptoms were similar, except shortness of breath, which was more frequently observed at TAK diagnosis in patients with CP involvement compared to those without (53% vs 21%; p=0.001). Composite CP involvement was not associated with risk of first surgery [Hazard ratio (95% CI): 1.21 (0.64-2.30); p=0.56]. However, pulmonary hypertension (PH) on echocardiogram was significantly associated with risk of first surgery [HR (95% CI): 12.9 (1.86- 89.14); p=0.01]. CP involvement was not significantly associated with mortality [HR (95% CI): 2.51 (0.45- 14.02); p=0.29].

CONCLUSIONS:
Cardiopulmonary abnormalities in TAK are common at the time of initial presentation. In this population, the presence of PH predicted a 13-fold increased risk for vascular or valvular surgery. In this cohort, the presence of CP involvement did not increase mortality.

PMID: 28628465 [Indexed for MEDLINE]

Eur J Cardiothorac Surg. 2017 Oct 1;52(4):775-780. doi: 10.1093/ejcts/ezx180.

Prognostic value of mean pulmonary artery pressure in the stable phase after heart transplantation.

Molkentin JP¹, Nägele MP¹, Frank M¹, Sudano I¹, Enseleit F¹, Wilhelm MJ², Lüscher TF¹, Maisano F², Ruschitzka F¹, Flammer AJ¹.

Author information:

1. Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.
2. Cardiac Surgery, University Heart Center, University Hospital Zurich, Zurich, Switzerland.

Abstract

OBJECTIVES:
In heart transplant recipients, elevated mean pulmonary artery pressure (mPAP) shortly before or after transplantation represents a powerful predictor for an adverse short-term outcome. Less is known on cardiac and pulmonary pressures measured in the stable phase after heart transplantation. The aim of this study was to assess the predictive value of mPAP, mean pulmonary capillary wedge pressure and mean central venous pressure in the stable phase after transplantation.

METHODS:
All patients (n = 260, mean age 47.4 ± 12.7 years, 224 males) who received a cardiac allograft at the University Hospital Zurich between September 1985 and August 2014 and who had undergone at least 1 right heart catheterization after transplantation (median 358 days after transplantation) were included and survival analysis was performed (median follow-up 11.9 years).

RESULTS:
The median mPAP, mean pulmonary capillary wedge pressure and mean central venous pressure were 15 mmHg (interquartile range 12-19 mmHg), 8 mmHg (interquartile range 6-11 mmHg) and 3 mmHg (interquartile range 1-5 mmHg), respectively. In mPAP median split survival analysis, patients with an mPAP above the median had a significantly lower long-term survival than patients with or below median mPAP (P = 0.012). mPAP but not mean central venous pressure or mean pulmonary capillary wedge pressure was independently associated with long-term mortality in multivariable Cox-hazard survival analysis (hazard ratio 1.10, confidence interval 1.04-1.16, P = 0.001). Other factors independently associated with mortality were age at transplantation (hazard ratio 1.03 per year, confidence interval 1.01-1.04, P = 0.002) and serum creatinine (μmol/l) (hazard ratio 1.003, confidence interval 1.001-1.010, P = 0.021).

CONCLUSIONS:
Our results demonstrate that mPAP measured in the stable phase after heart transplantation is an independent prognostic factor for long-term mortality.

© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

PMID: 28605420 [Indexed for MEDLINE]

PLoS One. 2018 Jul 30;13(7):e0201468. doi: 10.1371/journal.pone.0201468. eCollection 2018.

Evaluation of the CT imaging findings in patients newly diagnosed with chronic thromboembolic pulmonary hypertension.

Grosse A¹, Grosse C¹, Lang I².

Author information:

1. Department of Radiology, Medical University of Vienna, Vienna, Austria.
2. Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria.

Abstract

PURPOSE:
The aim of this study was to evaluate the vascular and parenchymal CT imaging findings, including vessel and cardiac chamber diameter measurements, in patients newly diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). The CT imaging findings were correlated with hemodynamic measurements and patient outcome.

METHODS:
Vascular and parenchymal CT findings were assessed on retrospectively ECG-gated MDCT angiography scans in 76 patients newly diagnosed with CTEPH. The diameters of the right and left ventricle (dRV, dLV), the right and left atrium (dRA, dLA), the ascending aorta (dAA), the right and left pulmonary arteries (drPA, dlPA), and the main pulmonary artery (dPA) were measured on axial CT scans. The CT imaging findings were correlated with demographic and hemodynamic data and adverse patient outcome due to right heart failure (RHF).

RESULTS:
The majority of patients showed chronic PE, mosaic perfusion, disparity in segmental vessel size, parenchymal densities, bronchial dilatation, and bronchial collaterals in CT. Mean pulmonary artery pressure (mPAP) was not significantly different in patients with and those without chronic PE, mosaic perfusion, disparity in segmental vessel size, parenchymal densities, bronchial dilatation, and bronchial collaterals. Mean PAP showed significant correlations with the CT metrics of dRV/dLV ratio, dRA, dRV, dPA and dPA/dAA ratio, but no correlation with the central thrombi score. By backward linear regression, the dPA/dAA ratio independently correlated with mPAP. Patients who died of RHF tended to have a higher frequency of exclusively chronic peripheral PE and greater dRV/dLV ratios on presenting CT scans compared with survivors.

CONCLUSION:
The majority of patients newly diagnosed with CTEPH show vascular signs of chronic PE, mosaic perfusion, parenchymal densities, disparity in segmental vessel size, bronchial dilatation, and bronchial collaterals on presenting CT scans. Particularly CTEPH patients with exclusively chronic peripheral PE and increased dRV/dLV ratios seem to be at risk of adverse outcome due to RHF.

Free PMC Article
PMCID: PMC6066236
PMID: 30059549

Conflict of interest statement
The authors have declared that no competing interests exist.

PLoS One. 2018 Apr 17;13(4):e0195092. doi: 10.1371/journal.pone.0195092. eCollection 2018.

Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts.

Vijarnsorn C¹, Durongpisitkul K¹, Chungsomprasong P¹, Bositthipichet D¹, Ketsara S¹, Titaram Y¹, Chanthong P¹, Kanjanauthai S¹, Soongswang J¹.

Author information:

1. Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

Abstract

OBJECTIVE:
To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort.

METHODS:
All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis.

RESULTS:
A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2.

CONCLUSION:
Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.

Free PMC Article
PMCID: PMC5903600
PMID: 29664959 [Indexed for MEDLINE]

J Am Soc Echocardiogr. 2018 Aug 6. pii: S0894-7317(18)30283-9. doi: 10.1016/j.echo.2018.05.015. [Epub ahead of print]

Right Atrial Function Predicts Clinical Outcome in Patients with Precapillary Pulmonary Hypertension.

Mouratoglou SA¹, Dimopoulos K², Kamperidis V¹, Feloukidis C¹, Kallifatidis A³, Pitsiou G⁴, Stanopoulos I⁴, Grosomanidis V⁵, Hadjimiltiades S¹, Karvounis H¹, Giannakoulas G⁶.

Author information:

1. First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
2. Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.
3. Radiology Department, Agios Loukas Clinic, Panorama, Thessaloniki, Greece.
4. Respiratory Failure Unit, “G. Papanikolaou” Hospital, Exohi, Thessaloniki, Greece.
5. Department of Anesthesiology and Intensive Care Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
6. First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. Electronic address: ggiannakoulas@auth.gr.

Abstract

BACKGROUND:
Although the primary role of right atrial (RA) size in the diagnosis and risk stratification of precapillary pulmonary hypertension (PH) has been studied, little is known about the clinical significance of RA function. In line with studies assessing left atrial function in heart failure, the aim of this study was to introduce the RA function index (RAFi) and to explore its prognostic power in precapillary PH.

METHODS:
RA emptying fraction was calculated as (RA end-systolic volume – RA end-diastolic volume) × 100/(RA end-systolic volume). RAFi was calculated as (RA emptying fraction × right ventricular outflow tract velocity-time integral)/(RA end-systolic volume index). Patients were followed for the end point of clinical failure, which was defined as death, hospitalization because of PH, or disease progression.

RESULTS:
In total, 47 patients with precapillary PH were included. Mean RAFi was 16.1 ± 22.3%. Over a median follow-up period of 25 months (interquartile range, 9.5-41.1 months), 29 patients experienced clinical failure. Univariate Cox proportional-hazard analysis showed that RAFi was a predictor of clinical failure (hazard ratio, 0.935; 95% CI, 0.890-0.981; P = .007). Addition of RAFi to established predictors of outcomes, including 6-minute walk distance, N-terminal pro-B-type natriuretic peptide, and RA area, improved their prognostic power.

CONCLUSIONS:
RAFi is an easily assessed echocardiographic parameter, which is strongly predictive of clinical outcomes in patients with precapillary PH. Further studies are needed to validate RAFi and define its role in clinical practice.

Copyright © 2018 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

PMID: 30093146

J Thromb Thrombolysis. 2018 Aug 7. doi: 10.1007/s11239-018-1720-4. [Epub ahead of print]

Balloon pulmonary angioplasty is a promising option in thalassemic patients with inoperable chronic thromboembolic pulmonary hypertension.

Karyofyllis P¹, Tsiapras D², Papadopoulou V³, Diamantidis MD⁴, Fotiou P⁴, Demerouti E², Voudris V³.

Author information:

1. Division of Interventional Cardiology, Onassis Cardiac Surgery Centre, Sygrou Ave 356, 17674, Athens, Greece. pakar768@yahoo.gr.
2. Noninvasive Cardiology Diagnostic Unit, Onassis Cardiac Surgery Centre, Athens, Greece.
3. Division of Interventional Cardiology, Onassis Cardiac Surgery Centre, Sygrou Ave 356, 17674, Athens, Greece.
4. Thalassemia Unit, Department of Hematology, General Hospital of Larissa, Larissa, Greece.

Abstract

Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. β-Thalassaemia, among other haematological disorders, develop PH which is not an infrequent finding and worsens the prognosis. Haemolysis, iron overload and hypercoagulable state are among the main pathogenetic mechanisms. Haemoglobinopathies and congenital haemolytic anaemia constitute a unique patients population more predisposed to developing chronic thromboembolic pulmonary hypertension (CTEPH). Although pulmonary endarterectomy is accepted as the best treatment for CTEPH, surgery in these patients poses significant practical challenges given the distinct nature of the disease. As drug therapy is not expected to offer relief from the mechanical obstructions of pulmonary arteries and the use of specific pulmonary arterial hypertension drugs is not established in thalassaemia patients, the novel technique of balloon pulmonary angioplasty (BPA) may emerge as a new therapeutic option for patients with inoperable CTEPH and thalassaemia. We are reporting the case of a thalassaemic patient with a history of splenectomy suffering from progressive severe pulmonary hypertension related to chronic thromboembolic disease, who was successfully treated by BPA with substantial improvement.

PMID: 30088118

Echocardiography. 2018 Aug 5. doi: 10.1111/echo.14117. [Epub ahead of print]

Pulmonary hypertension due to a stiff left atrium: Speckle tracking equivalents of large V-waves.

Caravita S¹, Mariani D^1,2, Blengino S¹, Branzi G¹, Crotti L^1,2, Parati G^1,2.

Author information:

1. Department of Cardiovascular, Neural and Metabolic Sciences, Ospedale San Luca IRCCS Istituto Auxologico Italiano, Milano, Italy.
2. Department of Medicine and Surgery, University of Milano-Bicocca, Milano, Italy.

Abstract

Heart failure with preserved ejection fraction (HFpEF) is a widely heterogeneous clinical condition. Left ventricular diastolic dysfunction is the leading etiology of HFpEF, but there might be patients presenting with a predominant disease of the left atrium (LA). We report a case of HFpEF secondary to a stiff LA, in which we corroborated invasive hemodynamic assessment with LA strain analysis. Pathognomonic, tall V-waves were observed in the wedge position in the absence of mitral regurgitation and with a near-normal QRS-gated, pre-V-wave pressure, indicating that left ventricular diastolic dysfunction was not a major issue in this case. These hemodynamic findings were mirrored by very low LA strain values, compatible with a stiff and noncompliant chamber.

PMID: 30079533

JACC Cardiovasc Interv. 2018 Jul 27. pii: S1936-8798(18)31218-4. doi: 10.1016/j.jcin.2018.05.045. [Epub ahead of print]

An Unusual Cause of Suspected Pulmonary Hypertension: Iatrogenic Left Internal Mammary Artery Pseudoaneurysm.

Toscano O¹, Bergonti M¹, Teruzzi G², Trabattoni D¹.

Author information:

1. Centro Cardiologico Monzino, IRCCS, Department of Cardiovascular Sciences, Milan, Italy.
2. Centro Cardiologico Monzino, IRCCS, Department of Cardiovascular Sciences, Milan, Italy. Electronic address: giovanni.teruzzi@ccfm.it.

PMID: 30077682

Pediatr Res. 2018 Jul;84(Suppl 1):68-77. doi: 10.1038/s41390-018-0082-0.

Application of Neonatologist Performed Echocardiography in the assessment and management of persistent pulmonary hypertension of the newborn.

de Boode WP¹, Singh Y², Molnar Z³, Schubert U⁴, Savoia M⁵, Sehgal A⁶, Levy P^7,8, McNamara P⁹, El-Khuffash A^10,11; European Special Interest Group “Neonatologist Performed Echocardiography” (NPE).

Author information:

1. Department of Neonatology, Radboud University Medical Center, Radboud Institute for Health Sciences, Amalia Children’s Hospital, Nijmegen, The Netherlands. willem.deboode@radboudumc.nl.
2. Department of Paediatrics, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
3. Department of Paediatrics, John Radcliffe Hospital, Oxford, UK.
4. Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden.
5. Department of Paediatrics, Azienda Ospedaliero-Universitaria S Maria della Misericordia, Udine, Italy.
6. Department of Paediatrics, Monash University, Melbourne, Australia.
7. Department of Pediatrics, Washington University School of Medicine, Saint Louis, MO, USA.
8. Department of Pediatrics, Goryeb Children’s Hospital, Morristown, NJ, USA.
9. Departments of Paediatrics and Physiology, University of Toronto, Toronto, Canada.
10. Department of Neonatology, The Rotunda Hospital, Dublin, Ireland.
11. Department of Paediatrics, The Royal College of Surgeons in Ireland, Dublin, Ireland.

Abstract

Pulmonary hypertension contributes to morbidity and mortality in both the term newborn infant, referred to as persistent pulmonary hypertension of the newborn (PPHN), and the premature infant, in the setting of abnormal pulmonary vasculature development and arrested growth. In the term infant, PPHN is characterized by the failure of the physiological postnatal decrease in pulmonary vascular resistance that results in impaired oxygenation, right ventricular failure, and pulmonary-to-systemic shunting. The pulmonary vasculature is either maladapted, maldeveloped, or underdeveloped. In the premature infant, the mechanisms are similar in that the early onset pulmonary hypertension (PH) is due to pulmonary vascular immaturity and its underdevelopment, while late onset PH is due to the maladaptation of the pulmonary circulation that is seen with severe bronchopulmonary dysplasia. This may lead to cor-pulmonale if left undiagnosed and untreated. Neonatologist performed echocardiography (NPE) should be considered in any preterm or term neonate that presents with risk factors suggesting PPHN. In this review, we discuss the risk factors for PPHN in term and preterm infants, the etiologies, and the pathophysiological mechanisms as they relate to growth and development of the pulmonary vasculature. We explore the applications of NPE techniques that aid in the correct diagnostic and pathophysiological assessment of the most common neonatal etiologies of PPHN and provide guidelines for using these techniques to optimize the management of the neonate with PPHN.

PMID: 30072805

Lancet Respir Med. 2018 Aug;6(8):577-579. doi: 10.1016/S2213-2600(18)30268-6. Epub 2018 Jun 29.

Pulmonary hypertension: the unaddressed global health burden.

Rich S¹, Haworth SG², Hassoun PM³, Yacoub MH⁴.

Author information:

1. The Bluhm Cardiovascular Institute, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA. Electronic address: srich@nm.org.
2. Department of Cardiology, Great Ormond Street Hospital for Children, London, UK.
3. Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
4. National Heart and Lung Institute, Imperial College School of Medicine, London, UK.

PMID: 30072105

Int J Dermatol. 2018 Jan;57(1):40-45. doi: 10.1111/ijd.13835. Epub 2017 Dec 2.

Cardiopulmonary anomalies in incontinentia pigmenti patients.

Onnis G¹, Diociaiuti A², Zangari P³, D’Argenio P³, Cancrini C³, Iughetti L⁴, El Hachem M².

Author information:

1. Dermatology Unit, Department of Surgical, Microsurgical and Medical Sciences, University of Sassari, Sassari, Italy.
2. Dermatology Unit, Academic Department of Pediatrics, Children’s Hospital Bambino Gesù -IRCCS, Rome, Italy.
3. Unit of Immune and Infectious Diseases, Academic Department of Pediatrics, Children’s Hospital Bambino Gesù, Rome, Italy.
4. Department of Pediatrics, University of Modena and Reggio Emilia, Modena, Italy.

Abstract

BACKGROUND:
Incontinentia pigmenti (IP) is a rare inherited genodermatosis that usually involves the skin, and also teeth, oral cavity, central nervous system, eyes, blood with eosinophilia, and rarely skeletal system, breast, heart, and lungs. Skin lesions usually appear early, at birth or within the first 2 weeks of life, with four different phases tending to follow Blaschko lines that may overlap.

CASE REPORT:
We report a rare case of a neonate with transient reversible pulmonary hypertension that presented at day 9 of life. She manifested increasing dyspnea and deterioration of respiratory dynamics with a serious pulmonary hypertension without a primary pulmonary disease. Hence, oxygen therapy at high flows and nitric oxide have been administered with an initial response, but, subsequently, because of the worsening of the respiratory activity, she underwent sildenafil and bosentan treatment with respiratory dynamics improvement and progressive decrease of the pulmonary pressures.

CONCLUSION:
In literature only a few cases of cardiopulmonary anomalies in IP have been described with different outcomes, and these rare complications are probably underestimated by physicians. We could suppose that microangiopathic damages may have a critical role in endothelial alterations, and these processes are probably shared by multiple organs involved in IP and rarely by lungs and heart.

© 2017 The International Society of Dermatology.

PMID: 29197078 [Indexed for MEDLINE]

Eur Radiol. 2018 Apr;28(4):1438-1448. doi: 10.1007/s00330-017-5143-y. Epub 2017 Nov 16.

Non-invasive methods for estimating mPAP in COPD using cardiovascular magnetic resonance imaging.

Johns CS¹, Rajaram S², Capener DA³, Oram C³, Elliot C², Condliffe R², Kiely DG², Wild JM³, Swift AJ^3,4.

Author information:

1. Academic Unit of Radiology, C floor, Royal Hallamshire Hospital, Glossop Rd, Sheffield, S10 2JF, UK. c.johns@sheffield.ac.uk.
2. Sheffield Pulmonary Vascular Disease Institute, Sheffield, UK.
3. Academic Unit of Radiology, C floor, Royal Hallamshire Hospital, Glossop Rd, Sheffield, S10 2JF, UK.
4. Insigneo, Institute of In-Silico Medicine, Sheffield, UK.

Abstract

PURPOSE:
Pulmonary hypertension (PH) is associated with a poor outcome in chronic obstructive pulmonary disease (COPD) and is diagnosed invasively. We aimed to assess the diagnostic accuracy and prognostic value of non-invasive cardiovascular magnetic resonance (CMR) models.

METHODS:
Patients with COPD and suspected PH, who underwent CMR and right heart catheter (RHC) were identified. Three candidate models were assessed: 1, CMR-RV model, based on right ventricular (RV) mass and interventricular septal angle; 2, CMR PA/RV includes RV mass, septal angle and pulmonary artery (PA) measurements; 3, the Alpha index, based on RV ejection fraction and PA size.

RESULTS:
Of 102 COPD patients, 87 had PH. The CMR-PA/RV model had the strongest diagnostic accuracy (sensitivity 92%, specificity 80%, positive predictive value 96% and negative predictive value 63%, AUC 0.93, p<0.0001). Splitting RHC-mPAP, CMR-RV and CMR-PA/RV models by 35mmHg gave a significant difference in survival, with log-rank chi-squared 5.03, 5.47 and 7.10. RV mass and PA relative area change were the independent predictors of mortality at multivariate Cox regression (p=0.002 and 0.030).

CONCLUSION:
CMR provides diagnostic and prognostic information in PH-COPD. The CMR-PA/RV model is useful for diagnosis, the RV mass index and PA relative area change are useful to assess prognosis.

KEY POINTS:

• Pulmonary hypertension is a marker of poor outcome in COPD.
• MRI can predict invasively measured mean pulmonary artery pressure.
• Cardiac MRI allows for estimation of survival in COPD.
• Cardiac MRI may be useful for follow up or future trials.
• MRI is potentially useful to assess pulmonary hypertension in patients with COPD.

Free PMC Article
PMCID: PMC5834560
PMID: 29147768 [Indexed for MEDLINE]

J Am Soc Hypertens. 2017 Dec;11(12):842-852. doi: 10.1016/j.jash.2017.10.009. Epub 2017 Oct 28.

Angiotensin-converting enzyme 2 activation ameliorates pulmonary endothelial dysfunction in rats with pulmonary arterial hypertension through mediating phosphorylation of endothelial nitric oxide synthase.

Li G¹, Zhang H¹, Zhao L², Zhang Y¹, Yan D¹, Liu Y³.

Author information:

1. Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing, China.
2. Department of Molecular Physiology and Biophysics, Holden Comprehensive Cancer Center, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
3. Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing, China. Electronic address: lylanzhen@126.com.

Abstract

This study aims to investigate the effect of angiotensin-converting enzyme 2 (ACE2) activation on pulmonary endothelial function in the process of preventing pulmonary arterial hypertension (PAH) in rat models and to explore the underlying mechanisms. Specific pathogen free rats were randomly divided into five groups including control group, PAH group, PAH + Resorcinolnaphthalein (Res) group (ACE2 activation), PAH + Res + MLN4760 group (ACE2 inhibition), and PAH + Res + L-NAME group (endothelial nitric oxide synthase [eNOS] inhibition). Rat PAH model was constructed using combined left pneumonectomy with a single dose of monocrotaline injection 1 week after the surgery, and the rats were then given corresponding reagents. Hemodynamics, endothelial function, and pathologic changes were evaluated 3 weeks after monocrotaline injection. The concentration of nitric oxide (NO), expression of eNOS, and phosphorylation of eNOS at Ser1177 and Thr495 in the lung tissues from rats were also investigated.The Res-induced activation of ACE2 led to decreased mean pulmonary arterial pressure (mPAP) and pulmonary artery remodeling in the PAH + Res group comparing with the PAH rats (P < .05). In addition, the reduction in mPAP induced by acetylcholine (Ach) was augmented in PAH + Res group (P < .05), but this was not observed under the treatment with sodium nitroprusside (SNP) (P > .05). The ratio of decrease in mPAP caused by Ach to that caused by SNP (Ach/SNP) was also increased (P < .05) in ACE2-activated rats. However, the protective effects of ACE2 activation on PAH were counteracted by co-administration of MLN4760, an ACE2 antagonist (all P > .05). The mechanistic study showed that the concentration of NO in the lung tissues was downregulated in the PAH group but upregulated in the PAH + Res group (P < .05), whereas the NO concentration in the PAH + Res + MLN4760 group was not obviously different from that in the PAH group (P > .05). Regarding the factors regulating NO release, we found that the eNOS was upregulated in the PAH group, and Res did not affect the expression of eNOS. The phosphorylation of eNOS at Ser1177 was increased but at Thr495 was reduced after Res injection, when compared with the PAH group (P < .05). As expected, co-injection of MLN4760 eliminated these differences (P > .05). The reduction in mPAP induced by Ach was attenuated in the PAH + Res + L-NAME group compared with the PAH + Res group (P < .05), but this was not observed in rats treated with SNP (P > .05). The Ach/SNP ratio of decline in mPAP was also decreased in the PAH + Res + L-NAME group (P < .05). Activation of ACE2 had a protective role in the development of PAH via improving the function of pulmonary arterial endothelium. This effect was potentially mediated by promoted NO release as a consequence of increased phosphorylation of eNOS at Ser1177 and dephosphorylation of eNOS at Thr495.

PMID: 29146157 [Indexed for MEDLINE]

Pediatr Cardiol. 2018 Feb;39(2):365-374. doi: 10.1007/s00246-017-1764-9. Epub 2017 Nov 1.

Changes in Bi-ventricular Function After Hematopoietic Stem Cell Transplant as Assessed by Speckle Tracking Echocardiography.

Covi S^1,2, Ravindranath Y³, Farooqi A^4,3, Savasan S³, Chu R³, Aggarwal S⁴.

Author information:

1. Division of Pediatric Cardiology, Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, 48201, USA. scovi2@gmail.com.
2. Division of Pediatric Cardiology, St. John Hospital and Medical Center, Detroit, MI, 48236, USA. scovi2@gmail.com.
3. Division of Pediatric Hematology/Oncology and Pediatric Blood and Marrow Transplantation Program, Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, 48201, USA.
4. Division of Pediatric Cardiology, Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, 48201, USA.

Abstract

Hematopoietic stem cell transplant (HSCT) is a therapeutic option for patients with sickle cell disease (SCD) and severe acquired aplastic anemia (SAA). HSCT may have beneficial effects on ventricular function in damaged myocardium. We hypothesized improvement in ventricular performance and pulmonary hypertension following HSCT with strain echocardiography in SCD and SAA. Echocardiographic strain and other standard functional data were obtained via retrospective cohort analysis of patients (n = 23) with SCD and SAA who underwent HSCT and were followed at a single center between 2000 and 2014. Left ventricular global longitudinal strain was below normal at baseline, and decreased significantly (from – 16.6 to – 11.1, P = 0.05) from pre-HSCT to the initial post-HSCT echocardiogram at 109 (SD ± 83) days. At 351 (SD ± 115) days, longitudinal strain improved significantly from initial decline (from – 11.1 to – 17.5, P = 0.009) but was comparable to baseline (P = 0.43). Other measurements of bi-ventricular function did not change significantly. Tricuspid regurgitation velocities as surrogates for pulmonary hypertension improved in the subset of patients with baseline elevated values although data points were limited. Abnormal myocardial systolic function was detected at baseline with strain imaging. HSCT was associated with initial worsening longitudinal strain values, followed by improvement to baseline levels by 1 year. Insufficient data exist on whether pulmonary hypertension improves after HSCT.

PMID: 29094192 [Indexed for MEDLINE]

Am J Cardiol. 2018 Jun 15. pii: S0002-9149(18)31191-3. doi: 10.1016/j.amjcard.2018.05.019. [Epub ahead of print]

Clinical and Echocardiographic Predictors of Outcomes in Patients With Pulmonary Hypertension.

Siddiqui I¹, Rajagopal S², Brucker A³, Chiswell K³, Christopher B², Alenezi F⁴, Mandawat A⁵, Rivera D⁴, Arges K⁴, Tapson V⁶, Kisslo J⁷, Velazquez E⁷, Douglas PS⁷, Samad Z⁸.

Author information:

1. Department of Medicine, East Carolina University, Greenville, North Carolina.
2. Division of Cardiology, Department of Medicine, Duke University, Durham, North Carolina.
3. Duke Clinical Research Institute, Duke University, Durham, North Carolina.
4. Duke Cardiac Diagnostic Unit, Durham, North Carolina.
5. Division of Cardiology, Department of Medicine, Duke University, Durham, North Carolina; Duke Cardiac Diagnostic Unit, Durham, North Carolina.
6. Center for Pulmonary Vascular Medicine, Cedar Sinai Medical Center, University of California at Los Angeles, Los Angeles, California.
7. Division of Cardiology, Department of Medicine, Duke University, Durham, North Carolina; Duke Clinical Research Institute, Duke University, Durham, North Carolina.
8. Division of Cardiology, Department of Medicine, Duke University, Durham, North Carolina. Electronic address: zainab.samad@duke.edu.

Abstract

In pulmonary hypertension (PH), measurement of various echocardiographic parameters that assess right heart function is recommended by current clinical guidelines. Limited data exists on the combined value of clinical and echocardiographic parameters in precapillary PH in the modern era of therapy. We examined the association of clinical and echocardiographic parameters with surrogate outcomes (6-minute walk distance) and hard outcomes (hospitalization or death) in patients with precapillary PH. A cohort of patients with an established diagnosis of precapillary PH who underwent transthoracic echocardiography at the Duke Echo Lab were prospectively enrolled from 2010 to 2014. Univariable and multivariable models were constructed to examine the relation of clinical and echocardiographic parameters with surrogate and hard outcomes. Of the 98 patients with analyzable echocardiograms with good image quality, 85 were woman, mean age was 59.4 years, and 47% had ≥World Health Organization functional class III symptoms. The mean 6-minute walk distance was 354(±132) m, and 83% were on pulmonary arterial hypertension medications. At 24 months, the cumulative incidence rate for hospitalization or death was 47%. In univariable analyses, the REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) risk score (HR 1.72 per 1 SD (2.81) increment, 95% CI 1.34, 2.22; p=<0.001), RV global longitudinal strain (RVGLS) (HR 1.54 per 1 SD (5.31) worsening, 95% CI , 2.12; p=0.008) and log-2 NT proBNP (HR 1.43 per 1-fold increase, 95% CI 1.25, 1.63; p=<0.001) were significantly associated with hospitalization or death.

PMID: 30093068

Crit Care Med. 2018 Aug 7. doi: 10.1097/CCM.0000000000003358. [Epub ahead of print]

Early Right Ventricular Systolic Dysfunction and Pulmonary Hypertension Are Associated With Worse Outcomes in Pediatric Acute Respiratory Distress Syndrome.

Himebauch AS¹, Yehya N¹, Wang Y², Conlon T¹, Kilbaugh TJ¹, McGowan FX¹, Mercer-Rosa L².

Author information:

1. Department of Anesthesiology and Critical Care Medicine, Perelman School of Medicine at the University of Pennsylvania, The Children’s Hospital of Philadelphia, Philadelphia, PA.
2. Division of Cardiology, Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, The Children’s Hospital of Philadelphia, Philadelphia, PA.

Abstract

OBJECTIVES:
The prevalence and importance of early right ventricular dysfunction and pulmonary hypertension in pediatric acute respiratory distress syndrome are unknown. We aimed to describe the prevalence of right ventricular dysfunction and pulmonary hypertension within 24 hours of pediatric acute respiratory distress syndrome diagnosis and their associations with outcomes.

DESIGN:
Retrospective, single-center cohort study.

SETTING:
Tertiary care, university-affiliated PICU.

PATIENTS:
Children who had echocardiograms performed within 24 hours of pediatric acute respiratory distress syndrome diagnosis.

INTERVENTIONS:
None.

MEASUREMENTS AND MAIN RESULTS:
Between July 1, 2012, and June 30, 2016, 103 children met inclusion criteria. Echocardiograms were analyzed using established indices of right ventricular and left ventricular systolic function and for evidence of pulmonary hypertension. Echocardiographic abnormalities were common: 26% had low right ventricular fractional area change, 65% had low tricuspid annular plane systolic excursion, 30% had low left ventricular fractional shortening, and 21% had evidence of pulmonary hypertension. Abnormal right ventricular global longitudinal strain and abnormal right ventricular free wall strain were present in 35% and 40% of patients, respectively. No echocardiographic variables differed between or across pediatric acute respiratory distress syndrome severity. In multivariable analyses, right ventricular global longitudinal strain was independently associated with PICU mortality (odds ratio, 3.57 [1.33-9.60]; p = 0.01), whereas right ventricular global longitudinal strain, right ventricular free wall strain, and the presence of pulmonary hypertension were independently associated with lower probability of extubation (subdistribution hazard ratio, 0.46 [0.26-0.83], p = 0.01; subdistribution hazard ratio, 0.58 [0.35-0.98], p = 0.04; and subdistribution hazard ratio, 0.49 [0.26-0.92], p = 0.03, respectively).

CONCLUSIONS:
Early ventricular dysfunction and pulmonary hypertension were detectable, prevalent, and independent of lung injury severity in children with pediatric acute respiratory distress syndrome. Right ventricular dysfunction was associated with PICU mortality, whereas right ventricular dysfunction and pulmonary hypertension were associated with lower probability of extubation.

PMID: 30095502

Eur J Cardiothorac Surg. 2018 Jan 1;53(1):178-185. doi: 10.1093/ejcts/ezx212.

Lung transplantation for idiopathic pulmonary arterial hypertension on intraoperative and postoperatively prolonged extracorporeal membrane oxygenation provides optimally controlled reperfusion and excellent outcome.

Moser B¹, Jaksch P¹, Taghavi S¹, Muraközy G¹, Lang G^1,2, Hager H³, Krenn C⁴, Roth G⁴, Faybik P⁴, Bacher A⁴, Aigner C¹, Matilla JR¹, Hoetzenecker K¹, Hacker P¹, Lang I⁵, Klepetko W¹.

Author information:

1. Department of Thoracic Surgery, Medical University of Vienna, Vienna, Austria.
2. Department of Thoracic Surgery, National Institute of Oncology, Semmelweis University, Budapest, Hungary.
3. Department of Cardiothoracic and Vascular Anesthesiology and Intensive Care Medicine, Medical University of Vienna, Vienna, Austria.
4. Department of Anesthesiology, General Intensive Care and Pain Medicine, Medical University of Vienna, Vienna, Austria.
5. Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria.

Abstract

OBJECTIVES:
Lung transplantation for idiopathic pulmonary arterial hypertension has the highest reported postoperative mortality of all indications. Reasons lie in the complexity of treatment of these patients and the frequent occurrence of postoperative left ventricular failure. Transplantation on intraoperative extracorporeal membrane oxygenation support instead of cardiopulmonary bypass and even more the prolongation of extracorporeal membrane oxygenation into the postoperative period helps to overcome these problems. We reviewed our experience with this concept.

METHODS:
All patients undergoing bilateral lung transplantation for idiopathic pulmonary arterial hypertension on intraoperative extracorporeal membrane oxygenation with or without prophylactic extracorporeal membrane oxygenation prolongation into the postoperative period between January 2000 and December 2014 were retrospectively analysed.

RESULTS:
Forty-one patients entered the study. Venoarterial extracorporeal membrane oxygenation support was prolonged into the postoperative period for a median of 2.5 days (range 1-40). Ninety-day, 1-, 3- and 5-year survival rates for the patient collective were 92.7%, 90.2%, 87.4% and 87.4%, respectively. When compared with 31 patients with idiopathic pulmonary arterial hypertension transplanted in the same period of time without prolongation of extracorporeal membrane oxygenation into the postoperative period, the results compared favourably (83.9%, 77.4%, 77.4%, and 77.4%; P = 0.189). Furthermore, these results are among the best results ever reported for this particularly difficult patient population.

CONCLUSIONS:
Bilateral lung transplantation for idiopathic pulmonary arterial hypertension with intraoperative venoarterial extracorporeal membrane oxygenation support seems to provide superior outcome compared with the results reported about the use of cardiopulmonary bypass. Prophylactic prolongation of venoarterial extracorporeal membrane oxygenation into the early postoperative period provides stable postoperative conditions and seems to further improve the results.

© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.

Free PMC Article
PMCID: PMC5848802
PMID: 28950326 [Indexed for MEDLINE]