Key words coronary artery fistura, coronary ishchemia, coronary steal, myocardial ischemia, neonate.Coronary artery fistula (CAF) is a rare heart anomaly defined as abnormal communication of coronary arteries with cardiac chambers or other vessels. Although symptoms such as exercise intolerance with dyspnea, angina, and arrhythmia may occur, more than half of patients are clinically asymptomatic even when they have moderate volume overload. 1 The symptoms have been known to increase with age, and the majority of pediatric CAF are asymptomatic. 2 Rarely, infants with a large CAF have symptoms such as heart murmur or congestive heart failure. 1,2 Myocardial ischemia, however, is extremely rare in infancy, and only three cases have been found in the literature. [3][4][5] In addition, CAF in all three cases originated from the left coronary artery (LCA), and not from the right coronary artery (RCA).Herein, we describe the case of a neonate with a CAF originating from the RCA who was asymptomatic at birth. The Fig. 1 (a) Echocardiography on the first day of age showing a markedly enlarged right coronary artery (RCA). (b) Color Doppler echocardiography showing the coronary artery fistula (CAF; open arrows) draining into the right ventricle (RV) just below the tricuspid valve. (c) Electrocardiography at 7 days of age showing significant ST depression in V1-V4. (d) Coronary angiography of the RCA showing a significantly dilated RCA draining into the RV through the CAF. In addition, the distal RCA is not visible. (e) Collateral arteries (white arrows) from the left coronary artery feed the distal RCA.Neonatal right CAF with coronary ischemia 417 patient rapidly developed heart failure in 1 week because of left-to-right shunt and coronary ischemia. The study was approved by the local ethics committee and informed consent for publication was obtained from the parents.A male neonate born at 39 weeks of gestation was transferred to Asahikawa Kosei General Hospital at 5 h of age because of a heart murmur. On admission, he had a Levine 3/6 to-and-fro murmur at the lower left sternal border. His vitals were stable with normal respiratory status.Chest X-ray showed a normal-sized heart with a cardiothoracic ratio (CTR) of 51% and no pulmonary congestion. Electrocardiogram (ECG) on the first day of age showed no ST-segment or T-wave abnormalities, suggesting no coronary ischemia.Echocardiography indicated an enlarged RCA and a large CAF draining from the RCA into the right ventricle (RV; Fig. 1a,b), with the RV to RCA flow in systole and the reverse flow in diastole. On Doppler echocardiography an apparent diastolic reverse flow was seen in the ascending aorta, suggesting a significant diastolic steal through the CAF to the RV, whereas there was neither chamber enlargement nor valvular regurgitation.His general condition and echocardiography findings were stable at 3 days of age; therefore, he was transferred back to the initial clinic and subsequently discharged 2 days later.Upon his visit to hospital at 7 days of age, he presente...

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Background: A heterozygous mutation of STAT3 causes autosomal dominant hyper immunoglobulin E (IgE) syndrome; however, there are still many unclear points regarding the clinical spectrum of this syndrome. Methods: In addition to a clinical description of patients in terms of pedigree, a genetic analysis, quantitation of peripheral blood Th17 and ex vivo IL-17 production were carried out. Results: The proband, a 2-year-old boy (Patient 1) with early onset atopic dermatitis-like eczema and recurrent bacterial infections, was suspected of autosomal dominant hyper immunoglobulin E syndrome on the basis of his symptoms and family history. His mother (Patient 2) also had skin eczema and recurrent bacterial infections, and his sister (Patient 3) had skin eczema. A novel STAT3 mutation (p.S476F) was detected in all three patients, but not in the father, who had no such symptoms. A significant decrease in peripheral blood Th17 subsets and IL-17 production was found in all the patients. Curiously, all three patients carrying the p.S476F mutation in STAT3 lacked connective tissue signs such as distinctive facial features, retention of primary teeth, and joint hyperextensibility. Conclusions: Autosomal dominant hyper IgE syndrome should, perhaps, be considered even if patients lack connective tissue signs, as long as hypersensitivity to infection and skin manifestations with hyper IgE are present.

Background: Assessing the hepatic status of children with CHD is very important in the post-operative period. This study aimed to assess the usefulness of paediatric liver T1/T2 values and to evaluate the impact of respiration on liver T1/T2 values. Methods: Liver T1/T2 values were evaluated in 69 individuals who underwent cardiac MRI. The mean age of the participants was 16.2 ± 9.8 years. Two types of imaging with different breathing methods were possible in 34 participants for liver T1 values and 10 participants for liver T2 values. Results: The normal range was set at 620–830 msec for liver T1 and 25–40 ms for liver T2 based on the data obtained from 17 healthy individuals. The liver T1/T2 values were not significantly different between breath-hold and free-breath imaging (T1: 769.4 ± 102.8 ms versus 763.2 ± 93.9 ms; p = 0.148, T2: 34.9 ± 4.0 ms versus 33.6 ± 2.4 ms; p = 0.169). Higher liver T1 values were observed in patients who had undergone Fontan operation, tetralogy of Fallot operation, or those with chronic viral hepatitis. There was a trend toward correlation between liver T1 values and liver stiffness (R = 0.65, p = 0.0004); and the liver T1 values showed a positive correlation with the shear wave velocity (R = 0.62, p = 0.0006). Conclusions: Liver T1/T2 values were not affected by breathing patterns. Because liver T1 values tend to increase with right heart overload, evaluation of liver T1 values during routine cardiac MRI may enable early detection of future complications.

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