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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries

Yueh-Tze Lan, MD^*, Ruey-Kang Chang, MD, MPH^*,* and Hillel Laks, MD

^* Division of Cardiology, Department of Pediatrics, Los Angeles, California, USA
Division of Cardiothoracic Surgery, Department of Surgery, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, California, USA

Manuscript received April 29, 2003; revised manuscript received July 14, 2003, accepted July 22, 2003.

^* Reprint requests and correspondence: Dr. Ruey-Kang Chang, Division of Cardiology, David Geffen School of Medicine at UCLA, B2-427 MDCC, 10833 Le Conte Avenue, Los Angeles, California 90095-1743, USA.
rkchang{at}ucla.edu

OBJECTIVES: We sought to determine the long-term outcomes and risk factors for mortality in patients with double-inlet left ventricle (DILV) or tricuspid atresia with transposed great arteries (TA-TGA).

BACKGROUND: Patients with DILV or TA-TGA are at risk of systemic outflow obstruction and a poor outcome. The impact of various management strategies on the long-term outcomes of these patients remains unknown.

METHODS: We reviewed the outcomes of 164 consecutive pediatric patients with DILV or TA-TGA who underwent surgical palliation between 1983 and 2002. Patients with a Holmes heart or heterotaxy syndrome or who were lost to follow-up (n = 24) were excluded. Risk factors for mortality or the need for orthotopic heart transplantation (OHT) were assessed by multivariate analysis.

RESULTS: There were 105 patients with DILV and 35 patients with TA-TGA. The overall mortality rate, including OHT, was 29%. Patients with DILV had a lower mortality rate than patients with TA-TGA (23% vs. 49%, p = 0.007). Multivariate analysis showed the presence of arrhythmia and pacemaker requirement as independent risk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality. Gender, era of birth, aortic arch anomaly, and systemic outflow obstruction were not risk factors. The perioperative and overall mortality were similar between patients who underwent the Damus-Kaye-Stansel procedure beyond the neonatal period and those had subaortic resection.

CONCLUSIONS: The mortality of patients with DILV or TA-TGA remains high. The outcomes of these patients are influenced by restriction of pulmonary blood flow, arrhythmia, and pacemaker requirement. Surgical palliation to relieve systemic outflow obstruction is not associated with a poor outcome.

Abbreviations and Acronyms   BVF = bulboventricular foramen   DILV = double-inlet left ventricle   DKS = Damus-Kaye-Stansel   LV = left ventricle/ventricular   OHT = orthotopic heart transplantation   OR = odds ratio   PAB = pulmonary artery band/banding   Qp/Qs = pulmonary blood flow to systemic blood flow ratio   TA-TGA = tricuspid atresia with transposed great arteries

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