Clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital.

摘要: Background: Children with single ventricle physiology comprise 10% of all children congenital heart disease (CHD) and one-third cyanotic CHD seen at King Chulalongkorn Memorial Hospital. The prognosis these is generally thought to be poor, but no study the outcome has previously been done in this hospital Thailand. Objectives: To clinical course Hospital during current era. Material Method: One hundred seventeen patients were initially year 1999-2001. Retrospective chart reviews carried out 90 available medical records. status was determined 2003 clinic visits, by phone calls mail. Results: main diagnoses tricuspid atresia (TA, n = 10), pulmonary intact ventricular septum (PA/IVS, 16), associated cardiac malposition or heterotaxy syndrome (malposition, 35), hypoplastic left (HLHS, 11) other complex (others, 18). Most did not have major anomalies. Survival TA PA/IVS approximately 92 87% 1 4 years, respectively. For three groups, survival 69 42%, Patients HLHS had worse survival, partly because decisions pursue further treatments parents. Among who underwent Norwood procedure, 83% Approximately 40 90% surgery years follow-up, Conclusion: Despite limited resource, short-term Thai morphologic (TA PA/IVS) reasonably good. Timely evaluation treatment toward Fontan procedure should ensured. After proper discussion parents, palliative care still acceptable for right due poor As access health improves, re-evaluation outcomes necessary find best strategy management patients.