Delayed diagnosis of Kawasaki disease: risk factors and outcome of treatment.

BACKGROUND: Kawasaki disease (KD) is associated with a high incidence of coronary artery aneurysms in untreated children. Treatment with intravenous immunoglobulin (IVIG) within the 1st 10 days of illness reduces by approximately fivefold the prevalence of coronary artery abnormalities (CAA). Data regarding delayed diagnosis of KD in Thailand have not been reported in the literature. AIMS: To determine the prevalence, risk factors and outcome of delayed diagnosis of KD in Thai patients. METHODS: We retrospectively reviewed the medical records of patients at Chiang Mai University Hospital diagnosed as KD during 2000-2008. Patients were classified into two groups: Group I were diagnosed ≤10 days of fever and Group II were diagnosed >10 days of fever. RESULTS: Of 170 patients, 150 were in Group I [mean (SD) fever 7 (1·45) d] and 20 (11·7%) in Group II [mean (SD) fever 15 (4) d]. There were no statistical differences between the two groups in age, gender, number of KD clinical manifestations or laboratory results, except that Group II were of lower weight (p = 0·01). Group II were younger (p = 0·09) and had more incomplete criteria (p = 0·09) but the differences were not statistically significant. Group II had a higher incidence of CAA (75% vs 19%) (p<0·001), more severe CAA and more resistant cases (31·2% vs 9·5%) (p = 0·04). CONCLUSION: Patients with delayed diagnosis of KD have a higher risk of developing CAA and of a more severe outcome for coronary artery disease. Education is needed to make healthcare providers and physicians more aware of KD, especially in small children or those with incomplete KD.

Management and outcome of intravenous gammaglobulin-resistant Kawasaki disease.

INTRODUCTION: This study aimed to determine the prevalence and risk of intravenous gammaglobulin (IVIG)-resistant Kawasaki disease (KD) and report the outcome of treatment in patients with persistent or recurrent fever. METHODS: 70 KD patients, who received IVIG treatment (2 g/kg) at a tertiary care hospital from January 1995 to June 2004, were retrospectively reviewed. RESULTS: Nine (13 percent) of the 70 patients failed to respond to initial treatment with IVIG. The patients who did not respond to IVIG had higher erythrocyte sedimentation rate (ESR) (104 versus 74 mm/h; p-value is 0.003), longer total days of fever (14.4 +/- 3.8 versus 9.2 +/- 2.3 days; p-value is 0.003) and higher initial coronary artery lesions (CAL) (7 of 9 [77.7 percent] versus 10 of 61 [16.3 percent]; p-value is 0.001) than those who responded to initial treatment. Seven of the nine patients who were retreated with IVIG (2 g/kg) responded to the second dose. The remaining two patients (two of nine, 22 percent) had persistent fever, which subsided after two to three doses of pulse intravenous methylprednisolone. At two months follow-up, IVIG-resistant patients had higher CAL by echocardiogram than IVIG-responsive patients (33 percent versus 3.2 percent, p-value is less than 0.05). Two IVIG-resistant KD patients had delayed diagnosis and developed giant aneurysms. CONCLUSION: Patients with high ESR had increased risk of IVIG-resistant KD. IVIG-resistant Kawasaki patients had a higher prevalence of CAL at the acute phase and two months after onset.

Are outcomes of surgical versus transcatheter balloon valvotomy equivalent in neonatal critical aortic stenosis?

BACKGROUND: For neonates with critical aortic valve stenosis who are selected for biventricular repair, valvotomy can be achieved surgically (SAV) or by transcatheter balloon dilation (BAV). METHODS AND RESULTS: Data regarding 110 neonates with critical aortic valve stenosis were evaluated in a study by the Congenital Heart Surgeons Society from 1994 to 1999. Reduced left ventricular function was present in 46% of neonates. The initial procedure was SAV in 28 patients and BAV in 82 patients. Mean percent reduction in systolic gradient was significantly greater with BAV (65+/-17%) than SAV (41+/-32%; P<0.001). Higher residual median gradients were present in the SAV versus BAV group (36 mm Hg [range, 10 to 85 mm Hg] versus 20 mm Hg [0 to 85 mm Hg], P<0.001). Important aortic regurgitation was more often present after BAV (18%) than SAV (3%; P=0.07). Time-related survival after valvotomy was 82% at 1 month and 72% at 5 years, with no significant difference for SAV versus BAV, even after adjustment for differences in patient and disease characteristics. Independent risk factors for mortality were mechanical ventilation before valvotomy, smaller aortic valve annulus (z score), smaller aortic diameter at the sinotubular junction (z score), and a smaller subaortic region. A second procedure was performed in 46 survivors. Estimates for freedom from reintervention were 91% at 1 month and 48% at 5 years after the initial valvotomy and did not differ significantly between groups. CONCLUSIONS: SAV and BAV for neonatal critical aortic stenosis have similar outcomes. There is a greater likelihood of important aortic regurgitation with BAV and of residual stenosis with SAV.

Echocardiographic assessment of obstructive lesions in atrioventricular septal defects.

OBJECTIVES: We sought to determine the accuracy of transthoracic echocardiography (TTE) in identifying risk factors in patients with an atrioventricular septal defect (AVSD). BACKGROUND: Atrioventricular septal defect is a common lesion, and many decisions about it are based on echocardiography alone. The identification of associated left-sided inflow and outflow obstructive lesions is important, as they are responsible for mortality and morbidity. METHODS: Between 1983 to 1998, 549 patients with AVSD underwent repair. The TTE findings were correlated with surgery, angiocardiography, autopsy or postoperative TTE. Papillary muscle measurements were made in those with either a left ventricular outflow tract (LVOT) or left ventricular inflow abnormality and compared with those measurements from control subjects. Measurements of the LVOT were made in patients with an identified LVOT abnormality. RESULTS: There were 63 missed lesions, decreasing over time. Double-orifice left atrioventricular valve (DOLAVV) and nonobstructive chordae in the LVOT were more often missed. Reoperation was performed to address a missed lesion in 2 of 68 patients. Two of 55 patients died of reasons related to a missed lesion. In 67% of patients, DOLAVV was missed. Abnormal papillary muscle angles were seen with either a LVOT abnormality or DOLAVV. High insertion of the anterolateral papillary muscle was a risk factor for death or residual LVOT obstruction. Abnormal LVOT measurements were found in patients with tunnel obstruction and those with an acquired subaortic ridge. CONCLUSIONS: Transthoracic echocardiography provides accurate preoperative information on AVSD.

Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society.

OBJECTIVES: We sought to determine factors that would predict whether a biventricular repair or Norwood procedure pathway would give the best survival in neonates with critical aortic stenosis. METHODS: Survival and risk factors were determined with parametric time-dependent event analysis for patients undergoing either the Norwood procedure or biventricular repair, and predicted optimal pathway and survival benefit were derived from multivariable linear regression. RESULTS: From 1994 to 2000, 320 neonates with critical left ventricular outflow obstruction were entered into a prospective multi-institutional study. Patients who died without intervention (n = 19) and those with primary cardiac transplantation (n = 6) were excluded. An initial intended biventricular repair pathway was indicated in 116 patients, with survival of 70% at 5 years. An initial Norwood procedure was performed in 179 patients, with survival of 60% at 5 years. Using predictions from separate multivariable hazard models for survival with each of the 2 pathways, we determined predicted optimal pathway and survival benefit for each patient. Significant independent factors associated with greater survival benefit for the Norwood procedure versus biventricular repair included younger age at entry, lower z-score of the aortic valve and left ventricular length, higher grade of endocardial fibroelastosis, absence of important tricuspid regurgitation, and larger ascending aorta. Predicted survival benefit favored the Norwood procedure in 50% of patients who had biventricular repair, and it favored biventricular repair in 20% of patients who had the Norwood procedure. CONCLUSIONS: Morphologic and functional factors can be used to predict optimal pathway and survival benefit in neonates with critical left ventricular outflow obstruction.

A case report: Alagille syndrome.

Alagille syndrome consists of 5 major features comprising paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. The female patient in this report met 4 of the 5 major features except ocular abnormality. The first clinical presentations were prolonged jaundice and generalized ecchymoses. She was treated by plasma replacement and vitamin supplement, particularly vitamin K1, which produced clinical improvement. This report also reviews the literature of Alagille syndrome.

Anti Ro/SSA positive undifferentiated connective tissue disease in a mother with a newborn with complete congenital heart block: a case report.

An association between complete congenital heart block (CCHB) and anti-Ro/SSA antibody is well recognized but has never been reported in Thailand. We report here a 37-year-old female who was admitted because of massive epistaxis secondary to immune thrombocytopenia. She had given birth to a child with CCHB 2 years previously, when she was healthy. Antinuclear antibody and anti-Ro/SSA were positive in her sera, but were negative in her son. The relationship between anti-Ro/SSA antibody and outcome of mothers with infants with CCHB is reviewed.