Pulse oximetry screening for clinically unrecognized critical congenital heart disease in the newborns.

AIM: To determine the incidence of clinically unrecognized critical congenital heart disease (CCHD) in the newborns by using pulse oximetric screening. METHODS: Pulse oximetry was performed on clinically normal newborns at 24-48 hours of age. If screening oxygen saturation (SpO(2)) was below 95%, echocardiography was then performed. Data regarding true and false positives as well as negatives were collected and analyzed. RESULTS: Pulse-oximetric screening was performed on 1847 clinically normal newborns. Low SpO2 (<95%) was found in three babies two of them had CCHD, including one with transposition of the great vessels, one with complete atrioventricular canal with moderate tricuspid regurgitation (sensitivity: 100%; specificity: 99.8%; positive predictive value: 100%; negative predictive value: 100%; accuracy: 99.8%). CONCLUSIONS: In addition to routine physical examination in the newborn infants pulse oximetry may improve the early diagnosis CCHD in the newborn. If oxygen saturation in clinically normal newborns is below 95% at 24-48 hours of age, referral to a cardiology unit is suggested.

Residual patent ductus arteriosus shunting after single Gianturco coil occluding.

Transcatheter occlusion with Gianturco coils has become the treatment of choice for small patent ductus arteriosus (PDA). Coil occlusion was attempted in 20 patients with ductus diameter less than 4 mm who did not require other cardiac surgery. Sixteen of 20 patients had successful implantation. The mean age was 4.2 years. Their mean weight was 14.1 +/- 5.9 kg. The mean ductus diameter was 2.21 +/- 0.91 mm (range 1-3.7 mm). Nine patients had complete occlusion but 7 had residual shunting immediately after the procedure. However, 4 patients had spontaneous resolution of residual shunts at 6 months after the procedure. The other 3 who had diameter of ductus greater than 3 mm still had significant residual shunt at 6 months and 1 year after the procedure. The second coil was successfully implanted in one of these 3 patients and the closure of PDA was accomplished. We concluded that the second coil should be implanted if the ductus diameter is greater than 3 mm and significant residual shunt is still demonstrated angiographically after the first coil implantation.

Chromosome 22q11 deletion syndrome: the first three cases reported in Thailand.

The DiGeorge, velocardiofacial, and conotruncal anomaly face syndromes were originally described as separate disorders due to different concerns regarding phenotypes. However, all these disorders have some common clinical manifestations, including congenital heart defect, facial anomaly, and developmental delay. It is now clear that most cases of these syndromes have a common cause resulting from microdeletion of chromosome 22q11. This study reports the first three cases of Thai children presented with developmental delays. All are females who were known cases of congenital heart diseases. Their minor facial anomalies were subtle and not previously recognized as of any syndromes. The chromosome study by fluorescent in situ hybridization technique yielded microdeletion of chromosome 22q11. Without known prevalence in Asian populations, except in Japanese children, further study for chromosome 22q11 deletion syndrome in Asian children with conotruncal heart defects, who also have minor facial anomalies or developmental delays, should be undertaken.

Balloon pulmonary valvuloplasty in children, one year follow-up experience at Ramathibodi Hospital.

UNLABELLED: Balloon valvuloplasty has become the treatment of choice in pulmonary valvular stenosis. The objective of this report is to review the experience of this procedure in children at the Department of Pediatrics, Ramathibodi Hospital in the past 4 years (1991-1994) with at least one year follow-up. During the study period, 19 children aged 2.1-14.3 years (mean 5.11 years) with the diagnosis of pulmonary valvular stenosis had successful pulmonary balloon valvuloplasty at this institution. The immediate peak systolic pressure gradient across the pulmonary valve by cardiac catheterization decreased from 92.05 +/- 46.92 to 34.26 +/- 25.30 mmHg, by Doppler from 78.58 +/- 26.55 to 34.83 +/- 15.60 mmHg. Peak pressure gradient across the pulmonary valve by Doppler of the last examinations, one to 4 years after the procedure (mean 1.9 years), was 23.05 +/- 9.40 mmHg in 17 patients. The mean ratio of balloon size and pulmonary valve ring was 1.06. One patient still has a residual gradient of 54 mmHg and two were lost to follow-up. No serious complication was encountered during the procedures. CONCLUSION: Balloon pulmonary valvuloplasty is effective in the management of children with valvular pulmonary stenosis. The intermediate term result is excellent.

Spontaneous closure of small ventricular septal defect first six months of life.

UNLABELLED: This study was designed to determine the incidence, types of the defects and the rate of spontaneous closure of the small ventricular septal defect (VSD) in infants who were born at Ramathibodi Hospital during the one year period April 1, 1991 to March 31, 1992. The diagnosis was confirmed by 2D-echocardiogram and Doppler and color flow imaging. RESULT: During the study period, the 15 infants diagnosed with small VSD, an incidence of 1.95 per 1,000 livebirths. The male to female ratio was 9 to 6. There were 11 muscular and 4 membranous VSD. Small VSD had a maximal diameter of 3 mm. of less. Spontaneous closure occurred in 9 to 15 (60%) during 6 months follow-up. The small muscular VSD spontaneously closed in 9 of 11 (82%) patients. CONCLUSION: This data supports the previous studies that the rate of spontaneous closure of small VSD is high and the muscular defect has a higher incidence of spontaneous closure.

Malignant pericardial tumor in childhood: a case report diagnosed by cross-sectional echocardiogram and magnetic resonance imaging.

Since primary malignant pericardial tumors are seldomly found in children, we reported a case with massive pericardial effusion. Pericardial tumor was diagnosed by cross-sectional echocardiogram. The extent of the tumor was well demonstrated by magnetic resonance imaging. The tumor was removed successfully and proved to be low grade fibrosarcoma. Modern technics can be very helpful in the diagnosis of pericardial tumor.

Cardiac beri-beri. Report of a case with an echocardiographic study.

An echocardiographic study in a patient with cardiac beri-beri has not been reported previously. We report serial studies in a 12-year-old boy with beri-beri. Tricuspid regurgitation was detected by Doppler in the early stage.

Acquired mitral stenosis in children under fifteen.

The clinical and hemodynamic studies of acquired MS in 30 children under the age of 15 are reviewed. There are 13 male and 17 female patients with an age range from 9 to 14 (mean 12.6) years. The majority of the patients (78%) are in New York Heart Association functional class 3 or 4. Only 55% of the patients had a history suggestive of previous rheumatic fever. All patients had pulmonary hypertension and 41% had severe pulmonary hypertension. A closed mitral commissurotomy was performed in all patients. There were 3 deaths and 2 patients required reoperation because of restenosis. The early onset of mitral stenosis in our population was suggestive of a very early onset of valvulitis with subsequent fibrosis and narrowing of the mitral valve. This is in contrast to the acute fulminating form of rheumatic fever with valvulitis manifested by severe mitral and or aortic insufficiency.

Juvenile rheumatoid arthritis: clinical characteristics in 100 Thai patients.

The clinical characteristic of 100 patients diagnosed for JRA at the Department of Pediatrics, Ramathibodi Hospital, Bangkok, Thailand during 1968-1984 are reported. There were 64 male and 36 female patients with a respective ratio of 1.7:1. In ninety-one percent the age of onset was over the age of five, the most common type being polyarticular onset (77%) followed by pauciarticular onset (13%) and systemic onset (10%). Twenty percent had a positive rheumatoid factor and 4% had cardiac complications. Over half of the patients (58%) responded to salicylate and other supportive treatment. The overall remission was 36 percent with a mean follow-up of 3.8 years. The highest remission rate was found in the polyarticular type of onset (43%) followed by systemic onset (33%) and pauciarticular onset (23%). Only 11 percent of the patients responded poorly to the treatment. As opposed to Western reports differences occurred in the type of onset, sex, and eye complications.

Hemostatic derangement in dengue haemorrhagic fever.

Forty children, aged 1/2-14 years, with serologically proven dengue haemorrhagic fever were daily studied for hemostatic tests. There were 4, 20 and 16 cases of grade I, II III respectively. Hemostatic derangements in DHF is a multifactorial mechanism. Vasculopathy, thrombocytopenia, platelet dysfunction were found in most cases. Mild to moderate degree of prothrombin complex deficiency was observed in 15% and 50% of grade II and grade III respectively while laboratory evidence of consumptive coagulopathy was noted in 30% of shock cases and 10% of non-shock cases. Hypofibrinogenemia and increased PTT are commonly seen in grade III reflect the presence of stimulation of intrinsic coagulation pathway probably from immunologic reaction. Frank DIC is very rarely observed. FDP is slightly increased but not as high as in classical DIC. Further study on the role of platelet-endothelial interaction should be elucidated including the efficient management to stop bleeding in severe shock cases.

Cardiac study in thalassemic children.

Cardiac function was studied in 54 thalassemic children, aged 3 to 18 years. Abnormalities were found by echocardiography in 58%, by electrocardiography in 15%, and by chest film cardiomegaly in 24%. There was a correlation between the severity of anemia and the degree of cardiomegaly.

Congestive cardiomyopathy in infants and children. Clinical features and natural history.

Fifty infants and children with a diagnosis of congestive cardiomyopathy were reviewed at the Department of Pediatrics, Ramathibodi Hospital. These patients were followed for an average of 2.9 years. Congestive heart failure was present in 82.6% of the cases. Of these patients, 43% died, 82% within the first year of their illness. Residual heart disease continues in 30%, while 27% made a complete recovery. Factors that were highly predictive of the clinical course are age, cardiothoracic ratio of initial and subsequent chest X rays and the initial response to treatment. Infants less than 1 year old had a poor prognosis. The higher the cardiothoracic ratio on the initial and subsequent examinations and the poorer the response to medical treatment, the poorer the prognosis.

Immunological studies on acute rheumatic fever and rheumatic heart disease.

The results of immunologic studies in 27 cases of acute rheumatic fever and 11 cases of chronic rheumatic heart disease are reported. In both groups, there are elevation of peripheral B lymphocytes and depression of T lymphocytes when compared to the normal control subjects. Circulating immune complexes and autoantibodies including anti-smooth muscle antibody, antimitochondria antibody and anti-parietal cell antibody are detectable in certain cases of both groups. Deposition of IgG, beta 1c and Coxsackie antigen are demonstrable in myocardial tissue in autopsy of two cases with acute rheumatic fever in association with the detection of immune complexes in their sera. Our results together with the previous reports suggested that the Coxsackie virus are one of the causative agent of the rheumatic fever. Moreover humoral and cellular immunological reaction are involved in the pathogenesis of the disease.

Immunopathology of acute rheumatic fever and rheumatic heart disease. The demonstration of Coxsackie group B viral antigen in the myocardium.

Immunopathologic studies were performed on heart tissue of patients with acute rheumatic fever with carditis and chronic rheumatic heart disease. Coxsackie group B viral antigen was demonstrated in 3 heart specimens of patients clinically compatible with active rheumatic fever. In two of these the pathologic findings were compatible with acute rheumatic carditis. Immunoglobulin was detected in 2 and complement in one of these heart specimens. All of the chronic rheumatic heart specimens and the controls were negative. These findings suggested that Coxsackie group B virus may be etiologically related to the pathogenesis of acute rheumatic fever.