ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
The evaluation of cases with double-inlet left ventricle-ventriculoarterial discordance
Pelin Ayyıldız1, Taner Kasar 1, Öykü İsal Tosun 1, Yakup Ergül 1, Ender Ödemiş 1, Alper Güzeltaş 1, İhsan Bakır2
1Departments of Pediatric Cardiology, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey
2Departments of Pediatric Cardiovascular Surgery, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey
DOI : 10.5606/tgkdc.dergisi.2016.11900
Background: This study aims to evaluate the cases of functional univentricle with the diagnosis of double-inlet left ventricleventriculoarterial discordance.

Methods: Between January 2010 and October 2014, a total of 34 patients (17 males, 17 females; median age 2.64 months; range 1 day to 24 years) with double-inlet left ventricleventriculoarterial discordance were analyzed retrospectively. The demographic characteristics of the patients, echocardiographic and hemodynamic measurements, invasive, surgical procedures performed and their outcomes were reviewed.

Results: Of the patients, 24 had pulmonary hypertension with arch obstruction in eight of them. Eight had pulmonary stenosis, and two had pulmonary atresia. None of the patients had outflow obstruction as assessed by echocardiography. Surgery was performed in 25 patients. Of these, 14 patients underwent initial pulmonary artery banding procedure and four of these also underwent arch reconstruction. Bidirectional cavopulmonary connection was performed in six patients, aortopulmonary shunt in four patients, and the Norwood-type operation in one patient. The median follow-up was 9.96 months (range 0.24 to 53.88 months). During follow-up, 12 patients underwent a bidirectional cavopulmonary connection. Systemic outflow restriction did not develop in any of the patients who were initially palliated with pulmonary artery banding with or without arch reconstruction and proceeded to the bidirectional cavopulmonary shunt stage.

Conclusion: Based on our study findings, although the primary treatment strategy in patients with double-inlet left ventricleventriculoarterial discordance displays a great variability, aortopulmonary shunting or ductal stenting can be performed in patients with restricted pulmonary blood flow, pulmonary artery banding with or without arch reconstruction in patients with unrestricted pulmonary blood flow. The Norwood type operation can be performed initially in patients with bulboventricular foramen restriction.

Keywords : Childhood; double-inlet ventricle; ventriculoarterial discordance
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