9 Long-term outcomes of adults with alcapa repair. (28th April 2017)
- Record Type:
- Journal Article
- Title:
- 9 Long-term outcomes of adults with alcapa repair. (28th April 2017)
- Main Title:
- 9 Long-term outcomes of adults with alcapa repair
- Authors:
- Ooues, G
Thorne, S
Bowater, S
Clift, P
Hudsmith, L - Abstract:
- Abstract : Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital abnormality, which, if untreated, can cause complications such as myocardial infarction, heart failure and death; only few untreated patients survive to adulthood. We sought to determine long-term outcomes of these patients in our Adult Congenital Heart Disease service. Methods: A retrospective review of case notes of patients with ALCAPA was performed, reviewing clinic letters, operation details, cardiac imaging and exercise tests. Results: We identified 8 patients (6 female) with mean age 26.3±6.2 years. Mean follow-up was 4.1 years (range, 18 months to 12 years). All patients have had surgical repair; one patient required concomitant mitral valve repair. Mean age at operation was 9.7 years (range, 3 months to 34 years old); 3 patients (38%) were operated as adults. At last clinic review, one patient had NYHA III symptoms, the rest were well (NYHA I). Mean peak oxygen consumption on cardiopulmonary exercise testing was 36±2.4 ml/kg/min (range 33.4 to 38.8 ml/kg/min, mean 93%±15.9% predicted, range 78% to 112%). All patients were in sinus rhythm and no ischaemia or arrhythmias were identified. 7 patients (88%) had good left ventricular function (mean EF 61%); 1 patient had mildly impaired function (EF 50%) due to an apical transmural infarction. Moderate mitral regurgitation was seen in 3 patients (38%) and all had normal aortic root size. Conclusion: WeAbstract : Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital abnormality, which, if untreated, can cause complications such as myocardial infarction, heart failure and death; only few untreated patients survive to adulthood. We sought to determine long-term outcomes of these patients in our Adult Congenital Heart Disease service. Methods: A retrospective review of case notes of patients with ALCAPA was performed, reviewing clinic letters, operation details, cardiac imaging and exercise tests. Results: We identified 8 patients (6 female) with mean age 26.3±6.2 years. Mean follow-up was 4.1 years (range, 18 months to 12 years). All patients have had surgical repair; one patient required concomitant mitral valve repair. Mean age at operation was 9.7 years (range, 3 months to 34 years old); 3 patients (38%) were operated as adults. At last clinic review, one patient had NYHA III symptoms, the rest were well (NYHA I). Mean peak oxygen consumption on cardiopulmonary exercise testing was 36±2.4 ml/kg/min (range 33.4 to 38.8 ml/kg/min, mean 93%±15.9% predicted, range 78% to 112%). All patients were in sinus rhythm and no ischaemia or arrhythmias were identified. 7 patients (88%) had good left ventricular function (mean EF 61%); 1 patient had mildly impaired function (EF 50%) due to an apical transmural infarction. Moderate mitral regurgitation was seen in 3 patients (38%) and all had normal aortic root size. Conclusion: We describe long-term outcomes of patients with ALCAPA syndrome. Postoperatively, the majority remain asymptomatic with good exercise capacity. Their left ventricular systolic function is good. Life-long follow up is warranted. … (more)
- Is Part Of:
- Heart. Volume 103(2017)Supplement 3
- Journal:
- Heart
- Issue:
- Volume 103(2017)Supplement 3
- Issue Display:
- Volume 103, Issue 3 (2017)
- Year:
- 2017
- Volume:
- 103
- Issue:
- 3
- Issue Sort Value:
- 2017-0103-0003-0000
- Page Start:
- A4
- Page End:
- A5
- Publication Date:
- 2017-04-28
- Subjects:
- Heart -- Diseases -- Treatment -- Periodicals
Cardiology -- Periodicals
616.12 - Journal URLs:
- http://www.bmj.com/archive ↗
http://heart.bmj.com ↗
http://www.heartjnl.com ↗ - DOI:
- 10.1136/heartjnl-2017-311499.9 ↗
- Languages:
- English
- ISSNs:
- 1355-6037
- Deposit Type:
- Legaldeposit
- View Content:
- Available online (eLD content is only available in our Reading Rooms) ↗
- Physical Locations:
- British Library DSC - BLDSS-3PM
British Library HMNTS - ELD Digital store - Ingest File:
- 19677.xml