Complications of pulmonary hypertension: a pictorial review

Mak, Sze Mun; Strickland, Nicola H.; Gopalan, Deepa

doi:10.1259/bjr.20160745

Cited by 12 publications

(15 citation statements)

References 39 publications

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“…PAH is a group of heterogeneous rare diseases (prevalence estimated around 30-50/1000,000) that is characterized by elevated pulmonary arterial resistance, leading to end-stage heart failure, and has a significant impact on survival [17][18][19]. PAH is mainly diagnosed at its advanced stage and is ultimately a fatal disorder.…”

Section: Discussionmentioning

confidence: 99%

Changes in heart morphometric parameters over the course of a monocrotaline-induced pulmonary arterial hypertension rat model

et al. 2020

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Background: Aim of this study was to assess changes in cardiac morphometric parameters at different stages of pulmonary arterial hypertension (PAH) using a monocrotaline-induced rat model. Methods: Four groups were distinguished: I-control, non-PAH (n = 18); II-early PAH (n = 12); III-end-stage PAH (n = 23); and IV-end-stage PAH with myocarditis (n = 7).Results: Performed over the course of PAH in vivo echocardiography showed significant thickening of the right ventricle free wall (end-diastolic dimension), tricuspid annular plane systolic excursion reduction and decrease in pulmonary artery acceleration time normalized to cycle length. No differences in end-diastolic left ventricle free wall thickness measured in echocardiography was observed between groups. Significant increase of right ventricle and decrease of left ventricle systolic pressure was observed over the development of PAH. Thickening and weight increase (241.2% increase) of the right ventricle free wall and significant dilatation of the right ventricle was observed over the course of PAH (p < 0.001). Reduction in the left ventricle free wall thickness was also observed in end-stage PAH (p < 0.001). Significant trend in the left ventricle free wall weight decrease was observed over the course of PAH (p < 0.001, 24.3% reduction). Calculated right/left ventricle free wall weight ratio gradually increased over PAH stages (p < 0.001). The reduction of left ventricle diameter was observed in rats with end-stage PAH both with and without myocarditis (p < 0.001).Conclusions: PAH leads to multidimensional changes in morphometric cardiac parameters. Right ventricle morphological and functional failure develop gradually from early stage of PAH, while left ventricle changes develop at the end stages of PAH.

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Section: Discussionmentioning

confidence: 99%

Changes in heart morphometric parameters over the course of a monocrotaline-induced pulmonary arterial hypertension rat model

et al. 2020

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“…Children with T21 are also at risk for vascular abnormalities, such as left-to-right shunts and persistent double capillary networks [ 17 ]. PAH may lead to DAH through increased hydrostatic pressure, vasodilator adverse effects, or aneurysmal rupture [ 18 , 19 ]. At least 25% of children with T21 have PAH due to issues such as abnormal vascular walls, OSA, and heart disease [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

“…Re-introduction and removal repeated pattern. Aceti, 2012 [ 16 ] F Italian History of VSD, ASD, PDA, necrotizing enterocolitis 4 Recurrent anemia and respiratory distress, hemoptysis Negative ANA, ANCA, anti-GBM, TTG-IgA Idiopathic Pulmonary Hemosiderosis glucocorticoids Recurred within 7 months, added hydroxychloroquine, recurred within 1 year Schwab, 1996 [ 17 ] M German – 6 Recurrent pneumonia, hemoptysis, anemia, renal failure +pANCA AAV glucocorticoids, cyclophosphamide No recurrence in 2 years Niimi, 1992 [ 18 ] F VSD 17 Dyspnea +ANA Negative RF, Anti-GBM glucocorticoids 3 recurrences in 4 years Sato, 1986 [ 19 ] F Japanese – 3 Cough, wheeze, anemia – – azathioprine, disodium cromoglycate inhalation, milk avoidance 7 recurrences in 1 year Koyama, 1995 [ 20 ] F Japanese – 9 Fever, dyspnea, anemia, pallor – IPH glucocorticoids No recurrence in 20 months (−) = unknown Abbreviations : PAH Pulmonary Arterial Hypertension, ANCA Anti-neutrophil cytoplasmic antibody, ANA Anti-nuclear antigen, CCP Anti-Cyclic Citrullinated Peptide Antibody, PR3 Serine Protease 3 antibody, MPO Anti-Myeloperoxidase Antibody, IgE-CMP Immunoglobulin E to Cow’s Milk Protein, IPH Idiopathic Pulmonary Hemosiderosis, AAV ANCA-Associated Vasculitis, AVSD Atrioventricular Septal Defect, dsDNA Anti-double stranded DNA antibody, IgD Immunoglobulin D, VSD Ventricular Septal Defect, ASD Atrial Septal Defect, PDA Patent Ductus Arteriosus, GBM Glomerular Basement Membrane, TTG-IgA Tissue Transglutaminase Immunoglobulin A Antibody, pANCA Perinuclear ANCA, RF Rheumatoid Factor ...…”

Section: Case Presentationsmentioning

confidence: 99%

Diffuse alveolar hemorrhage in children with trisomy 21

et al. 2021

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Background Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case presentation Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for 10 years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. Very few patients had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation. Conclusion These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, particularly given the low frequency of hemoptysis at presentation, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.

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“…2. PAH may lead to DAH through increased hydrostatic pressure, vasodilator adverse effects, or aneurysmal rupture 20,21 . At least 25% of children with T21 have PAH due to issues such as abnormal vascular walls, OSA, and heart disease 1 .…”

Section: Discussionmentioning

confidence: 99%

Diffuse Alveolar Hemorrhage in Children with Trisomy 21

Bloom

Frank

Weinman

et al. 2021

Preprint

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Background: Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case Presentation: Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for ten years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. A minority had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation.Conclusion: These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.

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Complications of pulmonary hypertension: a pictorial review

Cited by 12 publications

References 39 publications

Changes in heart morphometric parameters over the course of a monocrotaline-induced pulmonary arterial hypertension rat model

Changes in heart morphometric parameters over the course of a monocrotaline-induced pulmonary arterial hypertension rat model

Diffuse alveolar hemorrhage in children with trisomy 21

Diffuse Alveolar Hemorrhage in Children with Trisomy 21

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