Download Dear Colleagues - Centre for Rare Cardiovascular Diseases

Article*
"Development of the European Network in Orphan Cardiovascular Diseases"
„Rozszerzenie Europejskiej Sieci Współpracy ds Sierocych Chorób Kardiologicznych”
Title:
Supportive therapy in pulmonary hypertension associated with congenital
heart disease.
RCD code: II.1.A.4.d
Author: Grzegorz Kopeć
Affiliation: Department of Cardiac and Vascular Diseases, Centre for Rare Cardiovascular
Diseases, John Paul II Hospital, Krakow, Poland
Date:2014.06.09
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
Supportive therapy is an important part of care of patients with pulmonary arterial
hypertension associated with congenital heart disease (PAH-CHD). It is important that each
patient is evaluated regularly by a physician experienced in PAH-CHD. Patients should be
advised prophylaxis of infective endocarditis, regular vaccinations against influenza and
pneumococcal infections. Women with PAH-CHD should avoid pregnancy and use effective
contraception.
Infective endocarditis prophylaxis comprises administration of 30-60
min prior to intervention amoxicillin or ampicillin in the dose of 2 g p.o. or i.v. or 50 mg / kg
p.o. or i.v. In case of allergy to penicillin, clindamycin 600 mg p.o. or i.v. or 20 mg / kg p.o.
or i.v is recommended. This recommendation is for cyanotic patients undergoing dental
procedures involving manipulations of the gums and periapical regions of the teeth or
perforation of the oral mucosa.
It was found that the risk of death of pregnant women with CHD-PAH reaches 28%,
which is significantly multiplied when using general anesthesia. Unfortunately, the
termination of pregnancy is also a risk. Patients using endothelin receptor antagonists should
use double contraception. This is especially true of patients treated with bosentan, which
interacts with progesterone pills. In view of the risk of thrombogenicity estrogen preparations
should be avoided. Intrauterine devices while effective carry the risk of infective endocarditis.
It is necessary to screen for iron deficiency especially in patients with cyanosis. In
patients with heart failure signs and symptoms diuretics are recommended. In patients with
Eisenmenger's syndrome it is important to avoid excessive dehydration. Patients should avoid
heavy physical exertion especially with static load, although regular physical activity limited
by symptoms is recommended, and some data suggest the beneficial effects of supervised
exercise and rehabilitation. Anticoagulation is recommended only in patients with additional
indications for their use, such as atrial fibrillation or the presence of thrombi in the pulmonary
arteries. The use of home oxygen therapy is not justified because it does not improve the
blood count, exercise capacity or quality of life. Patients with PAH-CHD have often very
poor response to cardiac arrhythmias, and therefore they should be treated according to
general recommendations.
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
References:
1. D'Alto M, Diller GP. Pulmonary hypertension in adults with congenital heart disease and
Eisenmenger syndrome: current advanced management strategies. Heart. 2014 May 14. pii:
heartjnl-2014-305574. doi: 10.1136/heartjnl-2014-305574. [Epub ahead of print]
2. Habib G, Hoen B, Tornos P, Thuny F, Prendergast B, Vilacosta I, Moreillon P, de Jesus
Antunes M, Thilen U, Lekakis J, Lengyel M, Müller L, Naber CK, Nihoyannopoulos P,
Moritz A, Zamorano JL; ESC Committee for Practice Guidelines.
Guidelines on the
prevention, diagnosis, and treatment of infective endocarditis (new version 2009): the Task
Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European
Society of Cardiology (ESC). Endorsed by
the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) and the
International Society of Chemotherapy (ISC) for Infection and Cancer. Eur Heart J. 2009
Oct;30(19):2369-413.
3. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis
MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E,
Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E; Task Force on the
Management of Grown-up Congenital Heart Disease of the European Society of Cardiology
(ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice
Guidelines (CPG). ESC Guidelines for the management of grown-up congenital heart disease
(new version 2010). Eur Heart J. 2010 Dec;31(23):2915-57.
4. Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society
of Cardiology (ESC); European Respiratory Society (ERS); International Society of Heart and
Lung Transplantation (ISHLT), Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL,
Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G,
Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G. Guidelines for the
diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009 Dec;34(6):1219-63
………………………………………..
Author’s signature**
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
[** Signing the article will mean an agreement for its publication]
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu