Survival analysis of Down syndrome with congenital heart disease: a 5-years registry at QSNICH.

BACKGROUND: Down syndrome (DS) is the most common chromosomal abnormality in children. Atrio-ventricular septal defect (AVSD) is the most common congenital heart disease (CHD) reported in DS. The mortality rate of DS with congenital heart disease (CHD) is 5-7 times higher than normal population. The survival rate in DS has improved with time and has reported up to 91% and 85% at one and ten years of age, respectively. OBJECTIVE: To study the prevalence of CHD, clinical course, treatment, the overall survival in patients with DS compare with those who are associated with CHD. STUDY DESIGNS: DS registry, multidisciplinary approach, single centre. MATERIAL AND METHOD: All DS patients at QSNICH with parental signed consent were enrolled in the registry. The study was conducted for 5 years starting from May 2007 to April 2012. All patients were followed-up according to schedule modified from American Academic of Pediatrics (AAP) health supervision guideline. Standard treatment was given to all those children with diagnosis of CHD and/or other associated diseases. RESULTS: Four hundred and two cases of DS were enrolled. Two cases were excluded due to the parental inconvenience. The mode and mean age of the patients at registration were 1 and 7 months (1-62). Two hundred and seventy-one cases had an initial echocardiographic diagnosis of CHD, which included 91 of Patent ductus arteriosus (PDA), 49 of Ventricular septal defect (VSD), 34 of AVSD, 34 of secondum Atrial septal defect (ASD), 6 of Tetralogy of Fallot (TOF), 2 of Coractation of Aorta (CoA), 11 of other CHD and 44 of combined lesions. During the follow-up period, spontaneous closure of PDA, VSD and ASD occurred in 46, 12 and 15 cases, respectively. After the exclusion of those who had spontaneous closure, the prevalence rate of CHD in DS was 49.8%. VSD was the most common lesion, slightly more than PDA. Ninety-eight cases underwent cardiac surgery; including 39 of associated VSD, 24 of AVSD, 26 of PDA, 3 of TOF, 4 of CoA/AA repaired, one each of Cor triatriatum and primum ASD. Twelve cases had catheter interventions, including PDA occlusion (10 cases), and 1 case of balloon dilatation of re-CoA and coils embolization of isolated major aorto-pulmonary collateral artery. Seven cases were lost at follow-up. By using the hospital information and National Health Security Office (NHSO) database, 47 infants died during the follow-up period. The causes of death were immediate post cardiac surgery in 10 cases (AVSD in 9, VSD in 1) and not associated with surgery in 37 cases including CHF in 8, pneumonia in 7, leukemia in 4, airway disease in 3, others 7 and unknown 8 cases. The overall survival at 1 and 5 years of age were 96% and 86%, respectively. DS with CHD had a significantly lower survival rate than those without CHD (p < 0.001). CONCLUSION: According to this study, the prevalence rate of CHD in DS was high. VSD was the most common lesion and has better prognosis than AVSD. The main cause of death was a cardiac problem. Follow-up patients by using modified AAP guideline and standard treatment can improve their overall survival.

A comparison of oral chloral hydrate and sublingual midazolam sedation for echocardiogram in children.

OBJECTIVE: To compare the efficacy and safety of oral chloral hydrate and sublingual midazolam to sedate the children undergoing echocardiography. MATERIAL AND METHOD: A double-blind, randomized trial study in the children judged to require sedation prior echocardiogram were performed. Two hundred sixty-four patients between 6 months and 5 years of age were randomized to chloral hydrate or midazolam groups. Either 50 mg/kg of chloral hydrate orally or 0.3 mg/kg of midazolam sublingually was given in each groups. If the child was not responded within 30 minutes after the first dose, another half dose of each drug for the second dose will be required. The action duration time, sedation score level and the ability to complete echocardiogram were collected. RESULTS: Both groups were comparable with respect to age, sex, body weight, underlying heart disease, baseline O2 saturation and functional heart classification. The children in chloral hydrate group needed the second dose for sedation more than midazolam group (10.6%, 5.3% p = 0.111). The onset, action duration and total study time were significantly shorter in midazolam than in chloral hydrate group (p < 0.001). The number of the patients who had the action duration within the optimal time (< 45 min) were significantly more cases in midazolam than in chloral hydrate group (93.1%, 43.5% p < 0.001). Success rate of echocardiogram was 99.2% in each group. There was no difference in echocardiographic time performed in both groups. The children in chloral hydrate group had deeper in level of sedation (p < 0.001). Both groups showed no significant difference in term of the ability to complete echocardiographic examination. The reaction of the children to take the medication and the number of the patients who had systemic O2 saturation change more than 5%from the baseline were higher in chloral hydrate group significantly (14.4%, 4.5% p = 0.006 and 9.9%, 3.1% p = 0.025). CONCLUSION: Sublingual midazolam at the dose of 0.3 mg/kg can be used to sedate the children at age group between 6 months to 5 years who undergoing echocardiogram with comparable rate of success and safety as 50 mg/kg of chloral hydrate orally. The less depth in the level of consciousness after sedation with midazolam compare to chloral hydrate may be advantage in a high risk patient to avoid deep sedation but may be disadvantage in case who need more comprehensive echocardiographic evaluation.

Natural aortic valve complications of ventricular septal defect: a prospective cohort study.

OBJECTIVE: To study the incidence and onset of aortic valve prolapse (AVP) and aortic regurgitation (AR) in the ventricular septal defect (VSD). STUDY DESIGN: A prospective cohort study POPULATION: The less than one-year-old children with diagnosis of isolated VSD were studied from October 2000 to September 2006 at Queen Sirikit National Institute of Child Health. Clinical follow-up and echocardiographic studies were scheduled every 2-3 months in the first year of age and then every 6 months to evaluate the size, location, flow across VSD, aortic valve morphology and aortic regurgitation. RESULTS: Three hundred and twenty-one cases of VSD were followed up. One was excluded due to associated hypoplastic RV An overall of 2,644 echocardiograms were performed. The percentage of perimembranous, subpulmonic, muscular inlet and multiple types were 70.3%, 19.4%, 5.6%, 3.1% and 1.6%, respectively. Size of the VSD was diagnosed to be small, moderate, and large VSD in 62.5%, 15.9% and 21.6% respectively. At the end of the study, the incidence of AVP in subpulmonic VSD was 87.1% compared to 16.4% in perimembranous VSD, with a relative risk of 5.30 and the incidence of AR in subpulmonic VSD was 37.1% compared to 5.3% in perimembranous VSD, with a relative risk of 6.95. From the survival analysis, the patient with subpulmonic VSD developed AVP at 46%, 77%, 90% and 94% compare to 8%, 13%, 20% and 23% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). The patient with subpulmonic VSD developed AR at 8%, 17%, 35% and 38% compare to 2%, 4%, 5% and 7% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). At the end of the study, ninety-six cases (30%) underwent cardiac operation with the indication of heart failure or the occurrence of AR. Sixty one cases (19.1%), including two cases of subpulmonic type had spontaneous closure of VSD. Seven cases (2.2%) had lost to follow up and five cases (1.6%) died during the follow up period. CONCLUSION: The incidence of AVP and AR are high in subpulmonic VSD being much higher than perimembranous VSD with a relative risk of 5.30 and 6.95 respectively. These complications are significantly from infancy period and are an indication for early cardiac surgery.