scholarly journals Children with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension Treated with Pulmonary Vasodilators—The Pediatric Cardiologist Point of View

Children ◽  
2021 ◽  
Vol 8 (5) ◽  
pp. 326
Author(s):  
Anna Migdał ◽  
Anna Sądel-Wieczorek ◽  
Edyta Ryciak ◽  
Alicja Mirecka-Rola ◽  
Grażyna Brzezińska-Rajszys ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Prognostic Factors ◽  
Bronchopulmonary Dysplasia ◽  
Pressure Ratio ◽  
Point Of View ◽  
Term Outcome ◽  
Pulmonary Vasodilators ◽  
Long Term Effect ◽  
Postmenstrual Age ◽  
Pulmonary Arterial

Pulmonary hypertension in children with bronchopulmonary dysplasia (BPD-PH) significantly worsens the prognosis. Pulmonary vasodilators are often used in BPD-PH but the short-term outcome of treatment is not well described. The aim of this study was to evaluate BPD-PH children diagnosed beyond 36 weeks postmenstrual age treated with pulmonary vasodilators (sildenafil, bosentan, or both) and to assess the short and long-term effect of oral pulmonary vasodilators treatment. Twenty patients were included in the study. Cardiology evaluation (WHO-FC, NTproBNP, oxygen saturation, pulmonary to systemic pressure ratio PAP/SAP) was performed at diagnosis and after treatment initiation. In the majority of patients improvement in all evaluated factors was observed. No side effects of vasodilators were observed. PH resolved in 10 patients after a mean of 21.4 months of treatment. Six patients died. The number of poor prognostic factors commonly used to assess patients with pulmonary arterial hypertension (PAH) decreased significantly during BPD-PH treatment. The influence of BPD-PH perinatal risk factors on prognosis was considered but was not confirmed. In conclusion, the treatment of BPD-PH with pulmonary vasodilators was well tolerated and led to a clinical improvement with the possibility of discontinuation without recurrence of PH. Prognostic factors used in pediatric PAH risk stratification also seem to be useful in assessing treatment efficacy and prognosis in patients with BPD-PH.

scholarly journals Effect of Coronavirus Disease 2019 in Pulmonary Circulation. The Particular Scenario of Precapillary Pulmonary Hypertension

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 548 ◽  
Author(s):  
Jorge Nuche ◽  
Teresa Segura de la Cal ◽  
Carmen Jiménez López Guarch ◽  
Francisco López-Medrano ◽  
Carmen Pérez-Olivares Delgado ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Respiratory Infections ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The Elderly ◽  
Angiotensin Converting Enzyme 2 ◽  
Common Features ◽  
Pulmonary Vasodilators ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Low Incidence

The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease’s specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management.

Pathology and Pathobiology of Pulmonary Hypertension

2017 ◽  
Vol 38 (05) ◽  
pp. 571-584 ◽  
Author(s):  
Peter Dorfmüller ◽  
Christophe Guignabert
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Inflammation ◽  
Right Heart Catheterization ◽  
Point Of View ◽  
Heart Catheterization ◽  
Pulmonary Endothelium ◽  
Hemodynamic State ◽  
Pulmonary Arterial ◽  
Genetic And Environmental Factors ◽  
And Migration

Pulmonary hypertension (PH) is a hemodynamic state defined by a mean pulmonary artery pressure ≥ 25 mm Hg during resting right heart catheterization. PH can result from precapillary (arterial) or postcapillary (venous) pathophysiological mechanisms. Interestingly, recent PH pathology has shown that pulmonary arterial or pulmonary venous remodelling are rarely independent phenomena, but frequently occur in combined fashion in lungs from patients suffering from different forms of PH, including pulmonary arterial hypertension (PAH). In PAH, it is now becoming clear that aberrant signals present in vessel wall microenvironment, which is largely orchestrated by dysfunctional pulmonary endothelial cells, are key contributors of the pulmonary vascular remodeling process, fostering proliferation, and survival and migration of resident pulmonary vascular cells such as smooth muscle cells, myofibroblasts, and pericytes. In addition, both genetic and environmental factors are also critical in the development of pulmonary vascular inflammation and chronic impairment of the pulmonary endothelium. This article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

scholarly journals Targeting Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension (BPD-PH): Potential Role of the FGF Signaling Pathway in the Development of the Pulmonary Vascular System

Cells ◽  
2020 ◽  
Vol 9 (8) ◽  
pp. 1875
Author(s):  
Cho-Ming Chao ◽  
Lei Chong ◽  
Xuran Chu ◽  
Amit Shrestha ◽  
Judith Behnke ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Signaling Pathway ◽  
Preterm Infants ◽  
Bronchopulmonary Dysplasia ◽  
Potential Role ◽  
Vascular System ◽  
Point Of View ◽  
Pulmonary Vasculature ◽  
Curative Therapy

More than 50 years after the first description of Bronchopulmonary dysplasia (BPD) by Northway, this chronic lung disease affecting many preterm infants is still poorly understood. Additonally, approximately 40% of preterm infants suffering from severe BPD also suffer from Bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH), leading to a significant increase in total morbidity and mortality. Until today, there is no curative therapy for both BPD and BPD-PH available. It has become increasingly evident that growth factors are playing a central role in normal and pathologic development of the pulmonary vasculature. Thus, this review aims to summarize the recent evidence in our understanding of BPD-PH from a basic scientific point of view, focusing on the potential role of Fibroblast Growth Factor (FGF)/FGF10 signaling pathway contributing to disease development, progression and resolution.

Usefulness of Nitric Oxide Treatment for Pulmonary Hypertensive Infants during Cardiac Anesthesia

2002 ◽  
Vol 96 (4) ◽  
pp. 835-840 ◽  
Author(s):  
Mamoru Kadosaki ◽  
Takae Kawamura ◽  
Kotaro Oyama ◽  
Noriko Nara ◽  
Jicheng Wei ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Cardiopulmonary Bypass ◽  
Pressure Ratio ◽  
Systemic Blood Pressure ◽  
Inhalation Therapy ◽  
Biopsy Specimens ◽  
Pulmonary Arterial ◽  
Heart Lesions ◽  
Inhaled No

Background The beneficial effect of inhaled nitric oxide (NO) on pulmonary hypertension is well known. However, the indications for NO inhalation therapy for pulmonary hypertension associated with congenital heart lesions are still unclear. The aim of the current study was to seek a measure that would predict the effectiveness of inhaled NO in infants undergoing cardiac surgery. Methods Forty-six infants with pulmonary hypertension were studied. Pulmonary vascular resistance (PVR) measured at the time of cardiac catheterization was used as an indicator and compared with pulmonary arterial pressure/systemic blood pressure ratio (Pp/Ps) at the time of weaning from cardiopulmonary bypass. The effect of 40 ppm of inhaled NO for 15 min was evaluated in patients whose Pp exceeded systemic values. Results Preoperative PVR correlated positively with Pp/Ps at the time of weaning from cardiopulmonary bypass (r2 = 0.86; P < 0.05; n = 46). A Pp/Ps greater than or equal to 1 was not observed in any cases in which the preoperative PVR values were less than 7 Wood units m2; Pp/Ps ratio greater than or equal to 1 occurred in four patients. Each of these had PVR values greater than 7 Wood units m2. Three of these patients who had PVR values in the 7-12 Wood units m2 range were responsive to inhaled NO. The fourth patient, whose PVR value was greater than 15 Wood units m2, was unresponsive. Lung biopsy specimens were obtained in two patients whose preoperative PVR values were greater than 10 Wood units m2. Conclusion Preoperative PVR correlates reasonably well with postbypass Pp/Ps.

scholarly journals Fate of pulmonary hypertension associated with bronchopulmonary dysplasia beyond 36 weeks postmenstrual age

2020 ◽  
Vol 106 (1) ◽  
pp. 45-50 ◽  
Author(s):  
Sanne Arjaans ◽  
Meindina G Haarman ◽  
Marcus T R Roofthooft ◽  
Marian W F Fries ◽  
Elisabeth M W Kooi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Bronchopulmonary Dysplasia ◽  
Medical Center ◽  
Survival Rates ◽  
Patient Characteristics ◽  
University Hospital ◽  
Resolution Rate ◽  
Extremely Preterm ◽  
Postmenstrual Age ◽  
Preterm Born

ObjectiveTo determine the survival and evolution of pulmonary hypertension (PH) associated with bronchopulmonary dysplasia (BPD) in extremely premature born infants beyond 36 weeks postmenstrual age (PMA).DesignA single-centre retrospective cohort study from a university hospital.PatientsExtremely preterm (gestational age <30 weeks and/or birth weight <1000 g) infants, born between 2012 and 2017, in the University Medical Center Groningen with confirmed PH at/beyond 36 weeks PMA.Main outcome measuresSurvival, mortality rate and PH resolution. Patient characteristics, treatment, presence and evolution of PH were collected from patient charts.ResultsTwenty-eight infants were included. All had BPD, while 23 (82%) had severe BPD and 11 infants (39%) died. Survival rates at 1, 3 and 7 months from 36 weeks PMA were 89%, 70% and 58%, respectively. In 16 of the 17 surviving infants, PH resolved over time, with a resolution rate at 1 and 2 years corrected age of 47% and 79%, respectively. At 2.5 years corrected age, the resolution rate was 94%.ConclusionsThese extremely preterm born infants with PH-BPD had a survival rate of 58% at 6 months corrected age. Suprasystemic pulmonary artery pressure was associated with poor outcome. In the current study, infants surviving beyond the corrected age of 6 months showed excellent survival and resolution of PH in almost all cases. Prospective follow-up studies should investigate whether resolution of PH in these infants can be improved by multi-modal therapies, including respiratory, nutritional and cardiovascular treatments.

Pulmonary hypertension

2021 ◽  
pp. 839-848
Author(s):  
Massimiliano Palazzini ◽  
Nazzareno Galiè ◽  
Alessandra Manes
Keyword(s):  
Pulmonary Hypertension ◽  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Point Of View ◽  
Clinical Group ◽  
Mean Pulmonary Arterial Pressure ◽  
Assessment And Treatment ◽  
Haemodynamic Changes ◽  
Pulmonary Arterial ◽  
The Individual

Pulmonary hypertension is a haemodynamic and pathophysiological condition defined as an increase in the mean pulmonary arterial pressure of ?25 mmHg at rest, as assessed by right heart catheterization. Recently, a new definition has been proposed as mean pulmonary arterial pressure >20 mmHg combined with pulmonary vascular resistance ? 3 Wood units. While transthoracic echocardiography may provide clues on the presence of pulmonary hypertension, the haemodynamic evaluation offers a more precise and comprehensive assessment. Pulmonary hypertension is heterogeneous from a pathophysiological point of view, and the diversity is reflected in the haemodynamic definitions. The different haemodynamic and clinical forms of pulmonary hypertension can be found in multiple clinical conditions which have been classified into five main groups and at least twenty-six subgroups. Each main clinical group shows specific pathological changes in the lung distal arteries, capillaries, and small veins. If we combine the haemodynamic and clinical heterogeneity, we understand the complexity of an accurate diagnosis in the individual patient which is crucial for the prognostic assessment and treatment strategy. In addition, the concomitant presence of different haemodynamic and clinical mechanisms cannot be excluded in individual cases (e.g. in patients with congestive heart failure and associated lung diseases). The presence of pulmonary hypertension, as defined above, is always an ominous prognostic sign, even if the severity may differ according to the haemodynamic changes and underlying clinical condition. The therapeutic approach also is markedly different, according to the clinical group, and symptomatic and haemodynamic severity. For these reasons, the four more frequent clinical groups are discussed individually, while the classifications are described in the Introduction section.

scholarly journals The Efficacy and Safety of Pulmonary Vasodilators in Pediatric Pulmonary Hypertension (PH): A Systematic Review and Meta-analysis

2021 ◽  
Vol 12 ◽  
Author(s):  
Tingting Shu ◽  
Huaqiao Chen ◽  
Lu Wang ◽  
Wuwan Wang ◽  
Panpan Feng ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Pulmonary Hypertension ◽  
Meta Analysis ◽  
Control Group ◽  
Efficacy And Safety ◽  
Pulmonary Vasodilators ◽  
Ventilation Duration ◽  
Mechanical Ventilation Duration ◽  
Pulmonary Arterial

Background: We performed a meta-analysis to evaluate the efficacy and safety of pulmonary vasodilators in pediatric pulmonary hypertension (PH) patients.Methods: We searched electronic databases including PubMed, EMBASE, and the Cochrane Library up to May 2020, and conducted a subgroup analysis for pulmonary vasodilators or underlying disease.Results: Fifteen studies with 719 pediatric PH patients were included in the meta-analysis. Adverse events did not differ (p = 0.11, I2 = 15%) between the pulmonary vasodilators group and the control group, neither in the subgroups. In total, compared with the control group treatment, pulmonary vasodilators significantly decreased the mortality (p = 0.002), mean pulmonary artery pressure (mPAP, p = 0.02), and mechanical ventilation duration (p = 0.03), also improved the oxygenation index (OI, p = 0.01). In the persistent pulmonary hypertension of the newborn (PPHN) subgroup, phosphodiesterase type 5 inhibitors (PDE5i) significantly reduced mortality (p = 0.03), OI (p = 0.007) and mechanical ventilation duration (p = 0.004). Administration of endothelin receptor antagonists (ERAs) improved OI (p = 0.04) and mechanical ventilation duration (p &lt; 0.00001) in PPHN. We also found that in the pediatric pulmonary arterial hypertension (PPAH) subgroup, mPAP was pronouncedly declined with ERAs (p = 0.006). Systolic pulmonary artery pressure (sPAP, p &lt; 0.0001) and pulmonary arterial/aortic pressure (PA/AO, p &lt; 0.00001) were significantly relieved with PDE5i, partial pressure of arterial oxygen (PaO2) was improved with prostacyclin in postoperative PH (POPH) subgroup (p = 0.001). Compared with the control group, pulmonary vasodilators could significantly decrease PA/AO pressure (p &lt; 0.00001) and OI (p &lt; 0.00001) in the short-term (duration &lt;7 days) follow-up subgroup, improve mPAP (p = 0.03) and PaO2 (p = 0.01) in the mid-term (7–30 days) follow-up subgroup, also decrease mortality, mPAP (p = 0.0001), PA/AO pressure (p = 0.0007), duration of mechanical ventilation (p = 0.004), and ICU stay (p &lt; 0.00001) in the long-term follow subgroup (&gt;30 days).Conclusion: Pulmonary vasodilators decrease the mortality in pediatric PH patients, improve the respiratory and hemodynamic parameters, reduce the mechanical ventilation duration.

scholarly journals Overview of WHO Group 2 Pulmonary Hypertension Due to Left Heart Disease

2015 ◽  
Vol 14 (2) ◽  
pp. 70-78 ◽  
Author(s):  
Christopher F. Barnett ◽  
Van N. Selby
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Patient Outcomes ◽  
Left Heart ◽  
Pulmonary Vasodilators ◽  
Poor Patient ◽  
Left Heart Disease ◽  
Pulmonary Arterial ◽  
Transplant Evaluation ◽  
Group 2

Background: Left heart disease (LHD) is the most common cause of pulmonary hypertension (PH) and is associated with poor patient outcomes, especially among patients undergoing heart transplant evaluation. Implications for clinicians: Left heart disease should be considered in all patients undergoing an evaluation for PH. Correct management of PH from LHD is to optimize treatment of LHD. Pulmonary vasodilators used to treat pulmonary arterial hypertension should not be used in patients with PH from LHD. Conclusions: Additional research is needed to better understand how PH develops in patients with LHD and to investigate the role for treatment targeting PH in these patients.

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