Disease targeting therapies in patients with Eisenmenger syndrome: Response to treatment and long-term efficiency

• Published in: International journal of cardiology 2013-08, Vol.167 (3), p.840-847
• Main Authors: Diller, Gerhard-Paul, Alonso-Gonzalez, Rafael, Dimopoulos, Konstantinos, Alvarez-Barredo, Maria, Koo, Chiehyang, Kempny, Aleksander, Harries, Carl, Parfitt, Lisa, Uebing, Anselm S, Swan, Lorna, Marino, Philip S, Wort, Stephen J, Gatzoulis, Michael A
• Format: Article
• Language: English
• Subjects: 6-minute walk test distance; Adolescent; Adult; Aged; Angiotensin-Converting Enzyme Inhibitors - administration & dosage; Antihypertensive Agents - administration & dosage; Biological and medical sciences; Cardiology. Vascular system; Cardiovascular; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Double-Blind Method; Drug Delivery Systems - methods; Eisenmenger Complex - drug therapy; Eisenmenger Complex - physiopathology; Eisenmenger syndrome; Female; Follow-Up Studies; Functional class; Heart; Humans; Male; Medical sciences; Middle Aged; Pneumology; Pulmonary arterial hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Retrospective Studies; Therapy; Time Factors; Treatment Outcome; Young Adult
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2012.02.007

Abstract Objectives To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome. Methods All adult patients with Eisenmenger syndrome treated with DTT at our institution were included. Functional class (FC), oxygen saturation and 6-minute walk test distance (6MWTd) were analysed retrospectively. Results Between 2002 and 2010, 79 Eisenmenger patients (21 males, 16 with Down syndrome) aged 34 ± 10 years (range 17–68 years) were included. Median follow-up was 3.3 years (range 0.2 to 8.9 years). 6MWTd increased early after initiation of DTT, with a plateau after approximately 3 years and no obvious trend towards a deterioration on average during longer-term follow-up. Two patients died during follow-up and escalation of treatment was required in 18 patients after a median period of 2.5 years. Escalation of therapy was also associated with an increase in 6MWTd. In addition, FC improved on DTT and oxygen saturation, increased, both at rest and peak exercise. This effect was more pronounced in the patients with the lowest baseline oxygen saturation at rest. Conclusions Long-term DTT is safe and improves objective exercise capacity and subjective symptoms. Response to DTT was typically observed early after initiation of DTT and was, on average, maintained longer-term. However, 1 in 5 patients required escalation of DTT, with time, due to symptomatic deterioration and this was associated with an afresh improvement in 6MWTd.