Congenital heart defects with pulmonary hypertension

Czech version

Authors: T. Zatočil; A. Nečasová; T. Brychta
Authors‘ workplace: Ambulance vrozených srdečních vad v dospělosti, Interní kardiologická klinika, FN Brno-Bohunice
Published in: Kardiol Rev Int Med 2009, 11(4): 174-177
Category: Sympozium

Overview

Congenital heart defects with pulmonary hypertension represent small but heterogeneous group with the most pronounced symptoms and the worst long‑term prognosis. Owing to the advances in cardiology and cardiac surgery and, of course, pre‑natal diagnostics, the number of these patients is not expected to increase. Presence of the left to right shunt is prerequisite to pulmonary hypertension. Pulmonary hypertension frequently results from shunts found behind the tricuspid valve, owing to the high pressure gradient between systemic and pulmonary circulation in these locations. Compensation of the pressures in the systemic and pulmonary circulation leads to reversal of the shunt and to cyanosis – Eisenmenger’s syndrome develops. Complications are frequent, with hypoxia as the common denominator; advanced stages are considered to be a multi‑systemic disease. Treatment is symptomatic, impact of any drug on balanced haemodynamics must be taken into account, modern treatments of pulmonary hypertension are advantageous, combined transplantation of the heart and lungs might be considered in terminally ill patients. Considering the complexity of these issues, these patients should be managed by the specialised centres.

Keywords:
congenital heart defects – pulmonary hypertension – Eisenberger’s syndrome – left-to-right shunt

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Labels

Paediatric cardiology Internal medicine Cardiac surgery Cardiology

Pulmonary arterial hypertension

Pulmonary embolism

Pulmonary Hypertension in Patients with Chronic Obstructive Pulmonary Disease

Pleural effusion – aetiology and diagnostics

Treatment of diabetes with respect to cardiovascular risk

The future of dyslipidemia therapy

Who preaches water and drinks wine – Cardiovascular risk in a medical population in a project

Article was published in

Cardiology Review

2009 Issue 4