Pulmonary arterial hypertension associated with congenital heart disease. Personal perspectives

International Heart Journal
Toshio Nakanishi

Abstract

The management of patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) has changed dramatically with the development of targeted therapy with selective pulmonary vasodilators. The number of adult Japanese patients with PAH associated with CHD is increasing. It is important to develop evidence-based guidelines for the management of these patients, and to achieve this, a register of adult Japanese patients with PAH associated with CHD should be established. At the World Symposium in Nice, France, in 2013, the consensus was reached that patients with a pulmonary resistance of < 4 Wood Units (WU)·m(2) have operable disease, and patients with a pulmonary resistance of > 8 WU·m(2) have inoperable disease. However, these criteria are conservative. Some patients with a pulmonary resistance of > 8 WU·m(2) and a good response to a pulmonary vasodilator test have operable disease and a favorable clinical course long after repair of CHD. The criteria determining operability in patients with PAH associated with CHD in the era of pulmonary vasodilators should be established using data obtained from patient registers and/or multicenter studies. The optimal management of Eisenmenger syndrome should also be est...Continue Reading

References

Dec 22, 2006·International Journal of Cardiology·M G J DuffelsB J M Mulder
May 29, 2007·Circulation Journal : Official Journal of the Japanese Circulation Society·Hidemi KajimotoToshio Nakanishi
Sep 27, 2012·Progress in Cardiovascular Diseases·Erika B Rosenzweig, Robyn J Barst
Dec 4, 2012·European Respiratory Review : an Official Journal of the European Respiratory Society·Michele D'Alto, Vaikom S Mahadevan
Dec 21, 2013·Journal of the American College of Cardiology·Gerald SimonneauRogiero Souza

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