Comparison of heart failure in children with enterovirus 71 rhombencephalitis and cats with norepinephrine cardiotoxicity.

The mechanism of heart failure in patients with enterovirus 71 rhombencephalitis (brain stem encephalitis) remains unknown. Our previous reports hypothesized that a catecholamine storm induced by rhombencephalitis may account for the heart failure. The aim of this study was to develop a novel feline model of norepinephrine cardiotoxicity and compare the resulting heart failure to that in children with enterovirus 71 rhombencephalitis. Nine of 75 children (12%) with enterovirus 71 rhombencephalitis (5 boys and 4 girls; age, 4-28 months; median age, 16 months) were complicated with left ventricular hypokinesia (ejection fraction, 31 +/- 9%). Six cats (weight, 3.03 +/- 0.64 kg) were administered intravenous norepinephrine 30 microg/kg/min for 3 hours. Echocardiography assessed the left ventricular diameter and function before and after the administration of norepinephrine. Pathology studies included hematoxylin and eosin stain and in situ terminal deoxyribonucleotidyl transferase-mediated dUTP nick end-labeling assay. In the feline model, norepinephrine induced significant left ventricular dilatation (end diastolic diameter from 1.18 +/- 0.19 to 1.62 +/- 0.22 cm, p = 0.001; endsystolic diameter from 0.54 +/- 0.09 to 1.36 +/- 0.32 cm, p = < 0.001) and hypokinesia (ejection fraction from 87.5 +/- 4.1 to 35.2 +/- 16.3%, p = 0.001). Heart specimens from 4 patients and six cats showed similar pathology findings, including myocardial hemorrhage, cardiomyocyte apoptosis, and coagulative myocytolysis, which is characterized by sarcoplasmic coagulation, granulation, vacuolization, myofibrillar waving, and disruption. Both groups showed no significant inflammatory reaction. In conclusion, heart failure in patients with enterovirus 71 rhombencephalitis is similar to that in cats with norepinephrine cardiotoxicity. Norepinephrine cardiotoxicity may play a role in the pathogenesis of heart failure in enterovirus 71 rhombencephalitis.

Transcatheter closure of a large patent ductus arteriosus in a young child using the Amplatzer duct occluder.

The Amplatzer duct occluder (ADO) provides a safe and effective therapy for patients with moderate- to large-sized patent ductus arteriosus (PDA), but there have been few reports of transcatheter closure of very large PDAs in young children and infants. We report a successful transcatheter closure of a very large PDA, 10.5 mm in diameter at the narrowest point, with a 14/12-mm ADO. To our knowledge, this is the largest PDA ever closed by an interventional method in such a young child.

Fetal echocardiographic diagnosis of isolated ductus arteriosus aneurysm: a longitudinal study from 32 weeks of gestation to term.

OBJECTIVE: To investigate the echocardiographic characteristics of isolated fetal ductus arteriosus aneurysm (DAA) and the factors influencing its development. METHODS: Fetal echocardiograms for 509 low-risk singleton pregnancies were performed longitudinally from 32 weeks of gestation. The ventricular outflow tracts and great vessels were visualized, focusing on changes in the ductus arteriosus (DA), and fetuses with DAA or ductus arteriosus dilatation (DAD) were identified. Prenatal and perinatal findings were compared between those infants diagnosed postnatally with and those without neonatal DAA. RESULTS: Forty-one of the 509 fetuses (8.1%) studied had neonatal DAA; 2.2% (11/509) had DAA and 5.9% (30/509) DAD before delivery. The mean gestational age at the time of diagnosis of fetal DAA/DAD was 36.9 +/- 1.7 weeks. The mean maximal internal diameter of DAA/DAD was 8.0 +/- 0.8 mm at initial diagnosis, increasing to 10.8 +/- 1.6 mm before delivery. All cases originated from the aortic end of the DA. Compared with fetuses without neonatal DAA, the affected cases had a markedly more curved DA prior to diagnosis, and higher peak velocities at the aortic end of the DA (P < 0.05). Newborns with fetal DAA/DAD had a larger placental weight to birth body weight (BBW) ratio and were less likely to have a BBW appropriate for gestational age (P < 0.05). All cases of fetal DAA/DAD regressed spontaneously. CONCLUSION: Isolated neonatal DAA is a continuity of fetal DAA/DAD. The development of fetal DAA/DAD is likely to be related to the higher peak velocities in the more markedly curved DA occurring in the latter part of the third trimester. Fetal DAA/DAD usually initiates at the aortic end of the DA, followed by progression towards the pulmonary end.

Cardiac complications of enterovirus rhombencephalitis.

BACKGROUND: Epidemics of enterovirus 71 infection have caused the death of many children throughout the world. Rhombencephalitis, brain stem encephalitis, and heart failure were present in all of the fatal cases. However, no evidence of myocarditis was noted in the heart specimens, and the mechanism of heart failure remains unknown. AIMS: To characterise the presentation of cardiac complications in children with enterovirus rhombencephalitis and discuss its pathogenesis. METHODS: Ninety one consecutive patients with enterovirus rhombencephalitis underwent echocardiography. Of these, 17 patients (nine male, eight female; median age 14 months, range 4-57 months) with left ventricular dysfunction were studied. RESULTS: Tachycardia was noted in all patients and systemic hypertension in 12. Muscle-brain fraction of creatine kinase was >5% in 14 patients. Plasma norepinephrine and epinephrine levels were significantly raised in the three patients in whom these were analysed. Electrocardiographic abnormalities were noted in eight patients. Pulmonary oedema was complicated in 15 patients. The initial ejection fraction of the left ventricle was 22-58% (mean 37%, SD 11%). All patients deteriorated to hypotensive shock within 12 hours and 13 died. Heart specimens from seven patients showed no evidence of myocarditis, but significant coagulative myocytolysis, myofibrillar degeneration, and cardiomyocyte apoptosis were observed. CONCLUSIONS: Acute heart failure was noted in 19% of patients with enterovirus rhombencephalitis, which had a fatality rate of 77%. It was not caused by myocarditis but possibly by neurogenic cardiac damage.

Intracardiac ultrasound assessment of atrial septal defect: comparison with transthoracic echocardiographic, angiocardiographic, and balloon-sizing measurements.

PURPOSE: Accurate evaluation of the size, location and adjacent structure of an atrial septal defect (ASD) is very important in the selection of patients for further management. We directly compared the utility of transthoracic echocardiography, angiocardiography, balloon sizing, and intracardiac ultrasound (ICUS) in the detection of ASD. METHODS: Twenty-one children underwent an ICUS study of ASD after routine clinical and laboratory studies. All patients had received transthoracic echocardiography (TTE), cardiac catheterization, cineangiography, and balloon sizing before the ICUS to evaluate the ASD. RESULTS: There was a significant correlation between the ICUS-derived ASD diameter and the other methods (p < 0.001). The balloon-sizing diameter was estimated by the equation: TTE diameter x 1.09 + 3.9 mm. There was a good correlation between the predicted and measured balloon-sizing diameter (r = 0.963; p < 0.001). CONCLUSION: It is worthwhile spending a few minutes to perform ICUS during cardiac catheterization since it will provide more detailed information on and high resolution images of atrial septal morphology, especially for patients undergoing transcatheter closure by device.

Congenital complete heart block.

Congenital complete heart blocks (CCHB) are mostly related to the neonatal lupus syndrome. The purpose of this paper was to assess the clinical spectrum of CCHB in our hospital. Nine patients were retrospectively enrolled between 1994 and 1999. The birth history, electrocardiography, 24-hour Holter monitoring, pacemaker insertion and its complications, maternal disease, and maternal and infant autoantibody levels were studied. All nine cases were diagnosed prenatally. Hydrops fetalis was noted in five (55.6%). Six cases were live births and the other three were terminated. No anatomical heart defects were noted. Initial electrocardiography revealed the atrial rates ranged from 150 to 166 beats per minute. The minimal ventricular rates ranged from 46 to 80 beats per minute. VVI mode pacemakers were inserted through xyphoid approach in all live-birth infants. Complications were noted in three of them (50%). Antinuclear antibody and anti-SSA/Ro antibody were positive in all 8 mothers (100%). The anti-SSB/La antibody was positive in 6 of the eight mothers (75%). Five infants tested positive for anti-SSA/Ro antibody. None of the infants tested positive for anti-SSB/La antibody. In conclusion, all CCHBs in our series were associated with maternal autoantibodies. Because of high complication rate of permanent pacemaker insertion during the neonatal period, it should be restricted in certain conditions.

Transcatheter closure of atrial septal defect with a CardioSEAL device.

Transcatheter closure of an interatrial septal defect (ASD) with a CardioSEAL device was successfully performed on 12 Taiwanese children. There were five boys and seven girls, aged from 3.6-13.9 (8.3+/-3.2) years and with body weight of 15-57 (33.7+/-14.7) kgs. After one year of follow-up studies, which included physical examination, ECG, chest X-ray and echocardiography, complete closure of ASD was achieved in nine (75%) patients. Two children with a trivial residual shunt were asymptomatic and without audible cardiac murmur. A girl had a small residual left to right atrial shunt by color Doppler echocardiography, but without audible cardiac murmur. There were no immediate or intermediate complications. Transcatheter implantation of the CardioSEAL device is a safe and proper treatment for children with non-complicated small to medium secundum ASD.

Pseudoaneurysm formation after infected modified Blalock-Taussig shunt: echocardiographic findings.

The modified Blalock-Taussig (B-T) shunt is well recognized as a palliative procedure for cyanotic congenital heart disease in infants. Pseudoaneurysm formation after a modified B-T shunt is a rare complication. We present the case of a 9-month-old girl with tetralogy of Fallot who had undergone a modified left B-T shunt at 5 months of age and developed a pseudoaneurysm after an episode of infective endocarditis as detected by echocardiography. Compression of the left bronchus, displacement of the mediastinum to right chest, and paralysis of the left hemidiaphragm were found. Magnetic resonance images, computed tomography scans, aortograms, and selective angiograms demonstrated the presence of a large pseudoaneurysm. The compression syndrome gradually disappeared after aneurysmectomy.

Comparison of the one-and-a-half-year results of closure of patent ductus arteriosus by transcatheter coils placement with surgical ligation.

Patent ductus arteriosus (PDA) is a common type of congenital cardiovascular lesion. It usually needs surgical ligation in a full-term baby after 1 year of age. Transcatheter implantation of coils was introduced for the closure of small- to moderate-sized PDA in 1992. From November 1995 to November 1998, the authors closed the PDA in 153 patients by transcatheter implantation of coils and by surgical ligation in 10 patients. One hundred fourteen of them were studied for more than 1(1/2) years. The regular follow-up studies, including physical examination; electrocardiography; and pulsed, continuous-wave, and color Doppler flow mapping, were performed on day one and day 2, and 1 week, 1 month, 3 months, 6 months, and 1 year after the procedure. The results of the closure of PDA by surgical ligation or coil placement were compared and analyzed in all the patients.

Cardiac manifestations of fatal enterovirus infection during the 1998 outbreak in Taiwan.

BACKGROUND: An outbreak of hand-foot-and-mouth disease caused by enterovirus infection occurred in Taiwan in 1998 and more than 70 infants and children with fulminant courses died. We compared the cardiac manifestations of fatal cases with patients who survived the enterovirus infection. METHODS: A total 187 patients with enterovirus infection were treated at Taichung Veterans General Hospital between April and June 1998. Enterovirus infection was diagnosed by history, clinical features, polymerase chain reaction study and/or viral culture. Cardiac examinations including complete physical examinations, electrocardiography and echocardiography were performed on seven cases (group I) with or without central nervous system (CNS) involvement, 30 patients with CNS involvement (group II), and 150 patients without CNS involvement (group III). RESULTS: There were no significant differences in sex distribution, days of fever, heart rate, systemic blood pressure or time from the onset of symptoms to hospital admission among the three groups. All group I patients had features of acute congestive heart failure, pulmonary edema and neurologic signs except one who presented with right-sided heart failure and neurologic signs. The echocardiographic findings of group I were a lower fractional shortening, lower ejection fraction, and more severe and higher incidence of mitral regurgitation (p < 0.01) than in groups II and III, but there were no significant differences in end-systolic wall stress, left ventricular end-diastolic internal dimension and incidence of pericardial effusion among the three groups. CONCLUSIONS: We conclude that seven infants and children (group I) died due to either severe cardiomyopathy or encephalopathy. The possible pathogenesis of enterovirus infection leading to death is reviewed and analyzed.

Comparison of 201Tl SPET and treadmill exercise testing in patients with Kawasaki disease.

About 4% of children with Kawasaki disease ultimately develop ischaemic heart disease. Therefore, the early detection, non-invasive monitoring and long-term follow-up of myocardial ischaemia are essential. We compared the sensitivity and specificity of 201Tl single photon emission tomography (SPET) and treadmill exercise in the detection of myocardial ischaemia in 23 patients (19 boys, 4 girls) with Kawasaki disease. They were divided into two groups according to the results of coronary angiography. Group I consisted of 11 patients with coronary abnormalities; Group II consisted of 12 patients with no coronary abnormalities. The sensitivity, specificity, false-positive and false-negative rates for detecting coronary arterial lesions were 72.7% (8/11), 58.3% (7/12), 38.5% (5/13) and 30% (3/10) for 201Tl SPET, and 45.5% (5/11), 100% (12/12), 0% (0/5) and 33.3% (6/18) for treadmill exercise, respectively. We conclude that 201Tl SPET is more sensitive than treadmill exercise for the detection of coronary arterial abnormalities, but that the specificity of treadmill exercise is better than that of 201Tl scintigraphy. Coronary artery lesions detected by coronary angiography have good concordance of ischaemic areas with perfusion defects detected by 201Tl SPET. When ischaemic findings on 201Tl SPET and/or positive treadmill exercise testing are noted, coronary angiography is strongly indicated to detect possible stenotic lesions in the coronary arteries.

Intoxication after ingestion of toad soup: report of two cases.

Intoxication after ingestion of toad is rare, but it results in severe morbidity and high mortality. Recently, we encountered 2 children, one a 15-month-old boy and one a 20-month-old girl, who were fed cooked toad (Bufo melanostictus) soup. The boy expired of ventricular fibrillation. The girl presented with varying degrees of A-V block with congestive heart failure which, however, was completely resolved after intensive treatment. In our experience, early identification and intervention are necessary to decrease absorption of toxins; to correct hyperkalemia. Dysrhythmia should be treated with atropine, antiarrhythmic agents, and a temporary pacemaker, in order to avoid lethal results.