Pulmonary Arterial Hypertension from a Pediatric Perspective

Deutsch, Gail H.

doi:10.2350/07-12-0398.1

Cited by 9 publications

(2 citation statements)

References 72 publications

(72 reference statements)

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“…Pulmonary hypertension (PH) is a disease of both the pediatric and adult population, often with a severe prognosis. Compared to adults, PH in children is less likely to be primary or thromboembolic in origin, while it is more likely to be related to CHD or prematurity with lung disease [154]. While initial presentation may vary, eventual morbidity and mortality from this disease is typically related to right heart failure.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

Dorfman

Geva

Samyn

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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Cardiovascular magnetic resonance (CMR) is widely used for diagnostic imaging in the pediatric population. In addition to structural congenital heart disease (CHD), for which published guidelines are available, CMR is also performed for non-structural pediatric heart disease, for which guidelines are not available. This article provides guidelines for the performance and reporting of CMR in the pediatric population for non-structural (“non-congenital”) heart disease, including cardiomyopathies, myocarditis, Kawasaki disease and systemic vasculitides, cardiac tumors, pericardial disease, pulmonary hypertension, heart transplant, and aortopathies. Given important differences in disease pathophysiology and clinical manifestations as well as unique technical challenges related to body size, heart rate, and sedation needs, these guidelines focus on optimization of the CMR examination in infants and children compared to adults. Disease states are discussed, including the goals of CMR examination, disease-specific protocols, and limitations and pitfalls, as well as newer techniques that remain under development.

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Section: Pulmonary Hypertensionmentioning

confidence: 99%

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

Dorfman

Geva

Samyn

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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“…1 Affected newborns are typically term or late preterm and commonly develop symptoms of respiratory distress and severe hypoxemia almost always immediately after birth. [2][3][4][5] Clinically, neonates with PPHN have elevated pulmonary artery pressure with evidence of right-to-left shunt and invariably require supplementary oxygenation and mechanical ventilation. Without proper diagnosis and prompt treatment, the mortality rate of PPHN is high and survivors of PPHN carry with them various long-term functional impairments.…”

Section: Introductionmentioning

confidence: 99%

Abnormal Muscularization of Intra-acinar Pulmonary Arteries in Two Cases Presenting as Sudden Infant Death

et al. 2017

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Abnormal muscularization of acinar arteries is the hallmark of persistent pulmonary hypertension of newborn (PPHN), an uncommon disease with high rate of morbidity and mortality. PPHN presents with signs of respiratory distress immediately following birth. We herein report 2 cases presenting as a witnessed sudden unexpected death in the late neonatal period, preceded by respiratory deterioration and in whom the presence of abnormal muscularization of the acinar pulmonary arteries was reminiscent of PPHN. The significance of this report is twofold: to increase the awareness among pediatricians and pathologists of this feature that can present in some cases of Sudden Unexpected Death in Infancy/Sudden Infant Death Syndrome, and to highlight the importance of performing a thorough autopsy in order to identify the abnormality.

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Clinical practice

Healy

Hanna²,

Zinman

2009

Eur J Pediatr

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Pathologies in both the respiratory and cardiovascular systems frequently coexist and impact on each other. This manuscript introduces an approach to the interpretation of this complex relationship. Pulmonary hypertension can be a significant consequence of many respiratory diseases. This in turn can lead to right ventricular dysfunction and cor pulmonale. Many childhood illnesses can result in cor pulmonale and can be conveniently grouped into three categories: idiopathic pulmonary hypertension, neonatal lung diseases, and lung disease beyond the neonatal period. When considering the impact of cardiac disease on the lung, one must consider two main pathologies: compression of the pediatric airway and increased lung water. In conclusion, thorough attention must be given to the interpretation of the complex relationship between cardiac and respiratory diseases. Pulmonary hypertension is a complication to consider in respiratory illness at all ages. In addition, when dealing with the complexities of congenital heart disease, one must always be aware of the risks of pulmonary complications whether parenchymal or airway. Ongoing improvements in ventilation strategies, vasodilator therapy, and surgical interventions continue to improve the outlook for these complex cases.

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Pulmonary Arterial Hypertension from a Pediatric Perspective

Cited by 9 publications

References 72 publications

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

Abnormal Muscularization of Intra-acinar Pulmonary Arteries in Two Cases Presenting as Sudden Infant Death

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