Next Article in Journal
Natural Killer T Cells in Liver Ischemia–Reperfusion Injury
Next Article in Special Issue
Emerging Metabolic Therapies in Pulmonary Arterial Hypertension
Previous Article in Journal
Outcome of Clinical Trials with New Extended Half-Life FVIII/IX Concentrates
Previous Article in Special Issue
A Review of Targeted Pulmonary Arterial Hypertension-Specific Pharmacotherapy
Article Menu
Issue 4 (April) cover image

Export Article

Open AccessFeature PaperReview
J. Clin. Med. 2017, 6(4), 40; doi:10.3390/jcm6040040

The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease

1
Department of Cardiology, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
2
Netherlands Heart Institute, Moreelsepark 1, 3511 EP Utrecht, The Netherlands
*
Author to whom correspondence should be addressed.
Academic Editor: Bernhard Rauch
Received: 31 January 2017 / Revised: 9 March 2017 / Accepted: 23 March 2017 / Published: 30 March 2017
(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Arterial Hypertension)
View Full-Text   |   Download PDF [1320 KB, uploaded 13 April 2017]   |  

Abstract

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH) and a frequent complication of congenital heart disease (CHD). PAH-CHD represents a heterogeneous patient population and it is important to distinguish between the underlying cardiac defects considering the prognostic and therapeutic implications. Improved interventional techniques have enabled repair or palliation of most cardiac defects, though a substantial number of patients remain at high risk for PAH after closure. Traditionally, the treatment and management of PAH-CHD patients has been limited to palliative and supportive care, and based on expert opinion rather than clinical trials. Recently, however, the availability of advanced PAH-specific treatment has opened up a new field for the clinical management of this condition. Nevertheless, there is limited evidence on the optimal therapeutic approach for PAH-CHD. Herein, we discuss the current and novel therapeutic options for PAH-CHD as well as highlight several challenges in the clinical management at present. View Full-Text
Keywords: pulmonary arterial hypertension; pulmonary hypertension; congenital heart disease; Eisenmenger syndrome; therapy pulmonary arterial hypertension; pulmonary hypertension; congenital heart disease; Eisenmenger syndrome; therapy
Figures

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

Scifeed alert for new publications

Never miss any articles matching your research from any publisher
  • Get alerts for new papers matching your research
  • Find out the new papers from selected authors
  • Updated daily for 49'000+ journals and 6000+ publishers
  • Define your Scifeed now

SciFeed Share & Cite This Article

MDPI and ACS Style

van Dissel, A.C.; Mulder, B.J.M.; Bouma, B.J. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease. J. Clin. Med. 2017, 6, 40.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
J. Clin. Med. EISSN 2077-0383 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top