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Pulmonary hypertension in congenital shunts

Tissot, Cecile
Published in Revista Española de Cardiología. 2010, vol. 63, no. 10, p. 1179-1193
Abstract Pulmonary arterial hypertension frequently arises in patients with congenital heart disease. The vast majority present with congenital cardiac shunts. Initially these may manifest as left-to-right (i.e. systemic-to-pulmonary) shunts. The natural history of disease progression involves vascular remodeling and dysfunction that lead to increased pulmonary vascular resistance and, finally, to the development of Eisenmenger's syndrome, which is the most advanced form. The anatomical, pathological and structural abnormalities occurring in the pulmonary circulation of these patients are, to some extent, similar to those observed in other forms of pulmonary arterial hypertension. This understanding has recently led to significant changes in the management of Eisenmenger's syndrome, with the introduction of treatment specifically targeting pulmonary vascular disease. Early closure of the cardiac shunt remains the best way of preventing pulmonary vascular lesions. However, it is still not clear which preoperative parameters predict safe and successful repair, though hemodynamic evaluation is still routinely used for assessment. Postoperative pulmonary hypertension, both in the immediate period after surgical repair and during long-term follow-up, remains a real therapeutic challenge. The clinical situation of a single ventricle with Fontan circulation also presents difficulties when pulmonary vascular lesions are present. This article reviews pulmonary hypertension associated with congenital shunts and discusses a number of the specific problems encountered.
Keywords Eisenmenger Complex/drug therapy/physiopathology/surgeryHeart Defects, Congenital/*complications/surgeryHumansHypertension, Pulmonary/classification/epidemiology/*etiology/surgeryInfantInfant, NewbornTreatment Outcome
PMID: 20875358
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Article (Published version) (225 Kb) - public document Free access
Research group L'hypertension pulmonaire (pédiatrie) (228)
(ISO format)
BEGHETTI, Maurice, TISSOT, Cecile. Pulmonary hypertension in congenital shunts. In: Revista Española de Cardiología, 2010, vol. 63, n° 10, p. 1179-1193. https://archive-ouverte.unige.ch/unige:20433

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Deposited on : 2012-05-22

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