a Interní oddělení Nemocnice Přerov, AGEL Středomoravská nemocniční, a.s., a I. interní klinika – kardiologická, Fakultní nemocnice Olomouc b Kardiocentrum Lipsko, Německo c Kardiologické oddělení, Nemocnice Na Homolce, Praha d Centrum pro vrozené srdeční vady v dospělosti, Oddělení kardiochirurgie, Nemocnice Na Homolce, Praha e Centrum pro vrozené srdeční vady, Klinika kardiovaskulární chirurgie, 2. lékařská fakulta Univerzity Karlovy a Fakultní nemocnice v Motole, Praha f Interní kardiologická klinika, Fakultní nemocnice Brno a Centrum komplexní péče o vrozené srdeční vady v dospělosti, Brno

2020 ESC Guidelines for the management of adult congenital heart disease.

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.

Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society

Pediatric Pulmonary Hypertension

Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia.

Pulmonary vascular resistance was measured in air, oxygen, and after administration of vasodilators in 14 children with pulmonary hypertension and congenital heart disease. Lung morphology was examined by light microscopy and assessed quantitatively. In this selected group of patients (a) medial muscle thickness of greater than 20% in the intra-acinar arteries and Heath-Edwards changes of I or II were significantly associated with perioperative death from pulmonary complications after cardiac surgery; (b) children with lower percentage medial muscle thickness had a higher baseline resistance (r = -0.84) associated with Heath-Edwards grade III or higher changes (most of these patients were not offered corrective surgery); (c) when the lowest pulmonary vascular resistance was less than 3 units, Heath-Edwards grading was I or II (n = 4). When the pulmonary vascular resistance was greater than 6 units, however, there was no direct correlation with Heath-Edwards grading (n = 9). Four patients with a resistance of greater than 6 units had only grade I or II changes. Three had a medial muscle thickness above 20%, and were among those who died at or soon after operation. It is concluded that (a) patients with a lowest pulmonary vascular resistance of greater than 6 units have a bad prognosis whatever their lung morphology; and (b) some patients with Heath-Edwards grade I or II will have a high resistance (this group has a high medial muscle mass and a poor prognosis and would not be detected by Heath-Edwards grading alone).

https://heart.bmj.com/content/heartjnl/59/4/480.full.pdf

Correlations of lung morphology, pulmonary vascular resistance, and outcome in children with congenital heart disease.

Correlations oflungmorphology, pulmonary vascular resistance, andoutcome inchildren with congenital heart disease

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Correlations of lung morphology, pulmonary vascular resistance, and outcome in children with congenital heart disease.

Correlations oflungmorphology, pulmonary vascular resistance, andoutcome inchildren with congenital heart disease