Eisenmenger syndrome and complications in children – case report
Authors:
T. Doušová 1; J. David 1; K. Špičáková 1; K. Koubský 2; V. Koukolská 3; P. Jansa 4
Authors‘ workplace:
Pediatrická klinika 2. lékařské fakulty Univerzity Karlovy a FN Motol, Praha
1; Dětské kardiocentrum 2. lékařské fakulty Univerzity Karlovy a FN Motol, Praha
2; Klinika zobrazovacích metod 2. lékařské fakulty Univerzity Karlovy a FN Motol, Praha
3; Centrum pro plicní hypertenzi, 1. lékařská fakulta Univerzity Karlovy, Všeobecná fakultní nemocnice, Praha
4
Published in:
Čes-slov Pediat 2020; 75 (3): 178-182.
Category:
Case Report
Overview
3–10% patients with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH). The natural course of untreated CHD with significant left-to-right shunt is gradual increase in pulmonary vascular resistance, eventually resulting in reversal of the shunt (Eisenmenger syndrome, ES). PAH-CHD is associated with significant morbidity and mortality. Due to early diagnosis and surgical possibilities of CHD repair, there has been a significant decrease in number of patients with ES.
Authors present a case report of a fifteen-year-old boy with ES and a life-threatening complication – multiplex septic intracranial embolisation.
Keywords:
congenital heart disease – pulmonary arterial hypertension – Eisenmenger syndrome – cerebral abscess
Sources
1. Hansmann G, Koestenberger M, Alastalo TP, et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant 2019; 38 (9): 879–901.
2. Mezinárodní databáze vzácných onemocnění. http://www.orpha.net.
3. Ivy D, Abman S, Barst R, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol 2013; 62 (Suppl 25): D117–126.
4. Jansa P. Plicní arteriální hypertenze u vrozených srdečních vad. Interní Med 2006; 6: 266–269.
5. Hansmann G. Pulmonary hypertension in infants, children, and young adults. J Am Coll Cardiol 2017; 69 (20): 2551–2569.
6. Aschermann M, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Summary document prepared by the Czech Society of Cardiology. Cor et Vasa 2016; 58: e129–e152.
7. Pascall E, Tulloh RM. Pulmonary hypertension in congenital heart disease. Future Cardiol 2018; 14 (4): 343–353.
8. Kozlik-Feldmann R, Hansmann G, Bonnet D, et al. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart 2016; 102 (Suppl 2): ii24–48.
9. Hjortshoj CS, Jensen AS, Sorensen K, et al. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977–2012. Heart 2017; 103 (17): 1353–1358.
10. Hlavinová E, Zatočil T, Nečasová A, et al. Mozkový absces u atrezie plicnice s defektem komorového septa. Cor et Vasa 2018; 24–29.
11. Dogan F, Sen Dokumaci D, Yildirim A, et al. Brain diffusion changes in Eisenmenger syndrome. Br J Radiol 2016; 89 (1068): 20151007.
12. Frank DB, Hanna BD. Pulmonary arterial hypertension associated with congenital heart disease and Eisenmenger syndrome: current practice in pediatrics. Minerva Pediatr 2015; 67 (2): 169–185.
13. Skattum L, van Deuren M, van der Poll T, et al. Complement deficiency states and associated infections. Mol Immunol 2011; 48 (14): 1643–1655.
14. Kingman M, Archer-Chicko Ch, Bartlett M, et al. Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension. Pulm Circ 2017; 7 (3): 598–608.
15. Le Pavec J, Hascoët S, Fadel W. Heart-lung transplantation: current indications, prognosis and specific considerations. J Thorac Dis 2018; 10 (10): 5946–5952.
16. Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014 - An update from the Pulmonary Transplantation Council of the International Society for heart and lung transplantation. J Heart Lung Transplant 2015; 34: 1–15.
17. Galié N, McLaughlin VV, Rubin LJ, et al. An overview of the 6th World Symposium on Pulmonary Hypertension. Classification of pulmonary hypertension. Eur Respir J 2019; 53 (1): 1802148.
Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2020 Issue 3
Most read in this issue
- Genital bleeding in childhood view by pediatric gynecologist
- Abdominal pain at girls in childhood and adolescent age
- Female genital tract congenital anomalies – development defects of Müllerian ducts and their derivatives – ultrasound diagnosis in pediatric and adolescent gynecology
- Eisenmenger syndrome and complications in children – case report