Document Detail

Pulmonary arterial hypertension associated with congenital heart disease.
MedLine Citation:
PMID:  23204121     Owner:  NLM     Status:  MEDLINE    
Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.
Michele D'Alto; Vaikom S Mahadevan
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  European respiratory review : an official journal of the European Respiratory Society     Volume:  21     ISSN:  1600-0617     ISO Abbreviation:  Eur Respir Rev     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-12-03     Completed Date:  2013-05-24     Revised Date:  2014-02-20    
Medline Journal Info:
Nlm Unique ID:  9111391     Medline TA:  Eur Respir Rev     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  328-37     Citation Subset:  IM    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Antihypertensive Agents / therapeutic use
Clinical Trials as Topic
Down Syndrome / complications
Eisenmenger Complex / complications*,  physiopathology
Endothelium, Vascular / physiopathology
Epoprostenol / therapeutic use
Health Behavior
Heart Defects, Congenital / complications*,  epidemiology,  physiopathology
Heart Transplantation
Hypertension, Pulmonary / complications*,  epidemiology,  physiopathology,  therapy*
Lung Transplantation
Oxygen Inhalation Therapy
Phosphodiesterase 5 Inhibitors / therapeutic use
Piperazines / therapeutic use
Practice Guidelines as Topic
Pulmonary Circulation / physiology
Purines / therapeutic use
Sulfonamides / therapeutic use
Sulfones / therapeutic use
Thrombosis / etiology,  prevention & control
Grant Support
FS/12/13/29307//British Heart Foundation
Reg. No./Substance:
0/Antihypertensive Agents; 0/Phosphodiesterase 5 Inhibitors; 0/Piperazines; 0/Purines; 0/Sulfonamides; 0/Sulfones; 3M7OB98Y7H/sildenafil; DCR9Z582X0/Epoprostenol; Q326023R30/bosentan

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Pathways in pulmonary arterial hypertension: the future is here.
Next Document:  Pulmonary hypertension in left heart disease.