Characteristics and outcomes of double outlet left ventricle

Carolin Imai-Compton, Maryam Elmi, Cedric Manlhiot, Alejandro A. Floh, Fraser Golding, William G. Williams, Brian W. Mccrindle

Research output: Contribution to journalArticle

Abstract

Background. Double outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. There are limited data regarding the anatomical spectrum, characteristics and outcomes of DOLV.Methods. All patients with DOLV between 1960 and 2008 were identified. Patients' presentation, anatomy, surgical history, and outcomes were recorded. Kaplan-Meier estimates were used to describe time-related survival and freedom from surgical intervention.Results. There were 19 patients with DOLV during the study period, 14 of whom were male (74%). Median age at presentation was 2 days (birth-5.5 years). The most prominent associated cardiac morphologies (in addition to DOLV) were atrial septal defects (14, 74%), ventricular septal defects (13, 68%), right ventricular hypoplasia (12, 63%), and patent ductus arteriosus (15, 80%). At presentation, nine patients (47%) were cyanosed, nine (42%) had cardiomegaly, and all had some degree of right ventricular dysfunction. Of the 19 patients, seven underwent biventricular repairs, nine underwent univentricular palliations, one had only palliative care, and two were seen for consultation only. There were six deaths in this series, five were cardiac in nature (one due to genetic syndrome). Five-year survival after presentation for both biventricular and univentricular repairs was 70-75% (P= 0.80). Most surviving patients had residual cardiac abnormalities at last follow-up despite surgical repair.Discussion. DOLV is a rare cardiac malformation associated with high mortality and guarded outcomes from residual lesions.

Original languageEnglish (US)
Pages (from-to)532-536
Number of pages5
JournalCongenital Heart Disease
Volume5
Issue number6
DOIs
StatePublished - Nov 1 2010
Externally publishedYes

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Heart Ventricles
Right Ventricular Dysfunction
Patent Ductus Arteriosus
Survival
Atrial Heart Septal Defects
Ventricular Heart Septal Defects
Kaplan-Meier Estimate
Cardiomegaly
Palliative Care
Anatomy
Referral and Consultation
Arteries
History
Parturition
Mortality

Keywords

  • Congenital Heart Disease
  • Double Outlet Left Ventricle

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging

Cite this

Imai-Compton, C., Elmi, M., Manlhiot, C., Floh, A. A., Golding, F., Williams, W. G., & Mccrindle, B. W. (2010). Characteristics and outcomes of double outlet left ventricle. Congenital Heart Disease, 5(6), 532-536. https://doi.org/10.1111/j.1747-0803.2010.00456.x

Characteristics and outcomes of double outlet left ventricle. / Imai-Compton, Carolin; Elmi, Maryam; Manlhiot, Cedric; Floh, Alejandro A.; Golding, Fraser; Williams, William G.; Mccrindle, Brian W.

In: Congenital Heart Disease, Vol. 5, No. 6, 01.11.2010, p. 532-536.

Research output: Contribution to journalArticle

Imai-Compton, C, Elmi, M, Manlhiot, C, Floh, AA, Golding, F, Williams, WG & Mccrindle, BW 2010, 'Characteristics and outcomes of double outlet left ventricle', Congenital Heart Disease, vol. 5, no. 6, pp. 532-536. https://doi.org/10.1111/j.1747-0803.2010.00456.x
Imai-Compton, Carolin ; Elmi, Maryam ; Manlhiot, Cedric ; Floh, Alejandro A. ; Golding, Fraser ; Williams, William G. ; Mccrindle, Brian W. / Characteristics and outcomes of double outlet left ventricle. In: Congenital Heart Disease. 2010 ; Vol. 5, No. 6. pp. 532-536.
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