1. AN ADULT SINGLE VENTRICLE: CASE REPORT.
- Author
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Simion, M. F., Popară-Voica, Anca-Maria, Capşa, R., Platon, P., Ginghina, Carmen, and Coman, I. M.
- Subjects
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HEART ventricle abnormalities , *CONGENITAL heart disease - Abstract
Single ventricle represents less than 1% of all congenital heart diseases. We present the case of a 48 years patient, diagnosed after birth with double inlet single left ventricle, transposition of great arteries and pulmonary artery hypoplasia. Due to social and medical reasons, the malformation was not corrected during childhood. The patient was relatively paucisymptomatic until the age of 25 when he became intensely symptomatic, a central aortopulmonary surgical shunt being performed at that time. 20 years after the palliative intervention, the patient presents with impaired exercise tolerance and hemoptysis. Echocardiography revealed dilated single ventricle of left morphology with moderate systolic dysfunction, hypoplastic pulmonary artery at the origin, but with distal dilatation and turbulent flow in the pulmonary artery shunt. CT angiography examination described a central aortopulmonary shunt of 14 mm, dilated distal trunk and branches of the pulmonary artery, with multiple systemic-pulmonary artery anastomoses in periphery and interstitial pulmonary fibrosis. The suspicion of pulmonary hypertension was raised, but then it was infirmed by cardiac catheterization. The symptoms were interpreted as having a mixed cause, secondary to ventricular dysfunction and respiratory dysfunction. We considered that a standardized multistage palliative surgery as a preparation of the Fontane procedure would not improve prognosis, given the ventricular systolic dysfunction. The cardiac transplantation remains the ultimate option for this patient. The case is particularly interesting through the long favorable evolution in the absence of complete surgical correction and the absence of pulmonary hypertension despite the aortopulmonary shunt and the significant systemic-pulmonary arterial collateral network. [ABSTRACT FROM AUTHOR]
- Published
- 2015