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In most instances, congenital arteriovenous fistula is only one manifestation of a more widespread abnormality; 60% of patients also have hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber syndrome). Among those with congenital pulmonary arteriovenous fistula, the diagnosis is made during infancy in only 15% of patients. We present a case of pulmonary arteriovenous fistula in a newborn and review the literature. This rare condition of newborns can be treated with different surgical procedures. Only 17 cases of newborn pulmonary arteriovenous fistula/have been reported, and only two of those had associated Rendu-Osler-Weber syndrome. The results of surgical procedures were good in most of these cases. We treated our case with lobectomy successfully.

Pulmonary artery anatomy is the key factor that determines the type of surgical treatment required in tetralogy of Fallot. Despite the fact that routine primary repair is now done on infants, inadequate pulmonary artery size can dictate the need for staged surgical repair in even the oldest age groups. From October 1986 to October 1998, 361 patients at our clinic underwent surgery to correct tetralogy of Fallot. A total of 292 cases were treated with primary repair, 69 surgeries were palliative, and 30 of these 69 underwent corrective surgery. The Nakata index was used as a pulmonary artery index (PAI), and PAI< 200 was the criterion for requirement of two-stage repair. Of the 30 patients that underwent staged repair, the Blalock-Taussig shunt (BTS) procedure was used in 24; the remaining six patients had right ventricular outflow tract reconstruction (RVOTR). The mean age of all the palliative surgery patients was 3.4 years (range 6 months to 11 years), and of those who received corrective surgery was 5.5 years (range 2-12 years). These patients' PAI values were 181 +/- 37.5 mm2/m2 and 359 +/- 130.7 mm2/m2, respectively. The period between the two operations ranged from two months to four years. Mortality rates were 2.8 percent for palliative surgery as a whole, 4.1 percent for primary repair, and 16.6 percent for staged repair. Our policy with regard to corrective surgery for tetralogy of Fallot is to do primary repair regardless of a patient's age and weight, except in cases where the pulmonary artery anatomy is appropriate for the patient's body size.

Vascular ring, in which the trachea and esophagus are completely surrounded by vascular structures, is one of the causes of respiratory distress in children. Right aortic arch with aberrant left subclavian artery is a common aortic arch anomaly; however, respiratory distress due to vascular ring is seldom associated with this anomaly. We report herein a newborn infant treated surgically because of severe respiratory distress caused by vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. As laryngomalacia was first thought to be the reason for respiratory distress, we suggest that patients with respiratory distress diagnosed with laryngomalacia be evaluated for possible vascular ring.