From 1991 to 2002, we managed 72 patients with mucopolysaccharidoses. We retrospectively reviewed the records of 37 for whom detailed physical findings and cardiovascular evaluation were available. Twenty patients had serial electrocardiographic (ECG) examinations, and none had arrhythmias. All 20 patients had low R wave voltage in V6. One type VI patient had ischaemic changes secondary to severe cardiomyopathy at the terminal stage of her disease. Among the 37 patients in whom echocardiography was performed, cardiovascular abnormalities progressed with age, although most had mild clinical signs and symptoms. The most common changes were abnormalities of the mitral and aortic valves hypertrophy of the interventricular septum, especially in types I, II, and VI. During the follow-up period of up to 5 years, 3 type II patients and I type VI patient expired. In at least 3 of these patients, death was related to cardiopulmonary failure. As MPS patients are surviving longer, complete cardiac evaluation at regular intervals is mandatory and should be part of their routine assessment.

Systemic-pulmonary collateral arteries are known to develop in children with congenital heart disease, chronic pulmonary infection, and prematurity. At present, these abnormal connections between the systemic and the pulmonary systems are thought to develop from the vascular plexus, which normally gives rise to the pulmonary and bronchial vasculature. The objective of this study was to review our patients with systemic-pulmonary collateral arteries and evaluate possible risk factors. The records of patients with systemic-pulmonary collateral arteries seen at our hospital over a 4-year period were retrospectively reviewed. They were grouped into one of the following five categories: premature, heart disease, pulmonary disease, healthy, and others. Age, gender, weight, and the results of echocardiography were recorded, as was the condition on follow-up. We reviewed the records of 284 patients: 130 premature, 13 heart disease, 30 pulmonary disease, 92 healthy, and 19 others. Over the same period, 3314 healthy 1-month-old infants had undergone echocardiography for health screening. Among the 92 healthy children with systemic-pulmonary collateral arteries, 52 were diagnosed at the health-screening exam. Therefore, we estimate the incidence of unsuspected systemic-pulmonary collateral arteries in healthy 1-month-old infants to be 1.57% (52/3314). We conclude that systemic-pulmonary collateral arteries may be present normally after birth and then gradually disappear. However, if there are certain predisposing factors, they may persist in order to augment pulmonary flow.

Intervention: A 2-level heart disease screening program was undertaken. Level 1 screening included a history questionnaire and phonocardiography and electrocardiography. Level 2 screening consisted of examination by a pediatric cardiologist of all children who had abnormal findings on level 1 screening. A control group comprised children whose level 1 screening results were normal. Children with abnormalities on level 2 screening were referred for a detailed cardiac evaluation. Main Outcome Measures: Previously unsuspected heart diseases. Results: Of 25 816 children undergoing the level 1 screening program, 5330 had positive findings, of whom 5235 underwent level 2 screening, along with 1104 children in the control group. The pediatric cardiologist referred 780 children to the hospital for full evaluation, including 18 controls and 114 patients with a previous diagnosis of heart disease. A total of 292 children, including 2 controls, had evidence of heart disease, which was previously unsuspected in 178 (61.0%). Excluding children with a previous diagnosis of heart disease, the sensitivity of the screening program was 82.6%. The specificity and the positive predictive value were 98.3% and 28.6%, respectively. Conclusion: The 2-level heart disease screening program has good sensitivity and detects asymptomatic but potentially life-threatening heart disease in some children.

SUMMARYIsolated left subclavian artery has been documented to be associated with several congenital cardiac defects, including tetralogy of Fallot and transposition of the great arteries. In some cases subclavian or pulmonary artery steal phenomenon may occur when the isolated left subclavian artery connects to the main pulmonary artery via a ductus arteriosus. The isolated left subclavian artery may be part of the cardiac manifestations in multiple congenital anomalies, such as VACTERL association. We describe subclavian and pulmonary steal phenomenon in a neonate with complex congenital heart disease, including an isolated left subclavian artery, and left lung agenesis. An isolated left subclavian artery is rare and its association with agenesis of the left lung has not been reported previously. CASE REPORTA male Taiwanese infant weighing 2760 grams was born at term to a G2P2 mother by vaginal delivery. The Apgar scores were 3 and 7 at one and five minutes, with a delay of initial crying for one minute. There was no family history of cardiac or skeletal anomalies. Physical examination at birth showed a slightly hypoplastic left hemithorax. The heart rate was 136 beats per minute, the respiratory rate 50 per minute, and blood pressure 45/25 mmHg and 62/34 mmHg in the left and right upper limbs, respectively. Systemic oxygen saturation was 80% on room air. The left brachial pulse was weak with a normal right brachial and femoral pulse. There was no cranial bruit. A grade 2/6 systolic ejection murmur was heard at the left midsternal border. Breath sounds were absent over the left chest. A chest x-ray showed almost complete opacification of the left hemithorax with a shift of the heart to the left, hemivertebrae of T4 to T7, scoliosis (Figure 1), and absence of the left radius. The baby's karyotype was 46XY. An electrocardiogram showed right axis deviation, right atrial enlargement, and right ventricular hyperFrom

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