The role of pulmonary artery anatomy in repair of tetralogy of Fallot.

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.

Pulmonary arteriovenous fistula in the newborn: a case report of Rendu-Osler-Weber syndrome and a review of the literature.

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.

Vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum: a rare cause of respiratory distress in newborn infants.

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.

Anatomic correction in transposition of the great arteries with unusual coronary pattern and side-by-side location.

Switch operation is the treatment of choice in infants with transposition of the great arteries. The anatomic correction restores the left ventricle to its normal systemic function. In complete transposition, however, the diverse spectrum of coronary pattern influences the surgical outcome. We describe a successful arterial switch operation without LeCompte manoeuvre in a neonate with unusual coronary artery anatomy and side-by-side great arteries.

Critical tricuspid regurgitation secondary to ruptured chordae tendineae mimicking a mass on the tricuspid valve in a newborn.

Tricuspid regurgitation (TR) due to chordal rupture is a rare condition in newborns. We present a newborn with critical TR. Cross-sectional echocardiography demonstrated a dense, mobile mass localized at the tricuspid valve and resembling an atrial thrombus or myxoma. At operation, ruptured chordae tendineae of the tricuspid valve were found, forming a tangled mass. Histopathologic examination revealed collagen degeneration and calcification in the ruptured chordae, producing the hyperechoic image. It should be emphasized that ruptured chordae of the tricuspid valve might mimic a tricuspid valve myxoma or a thrombus on cross-sectional echocardiography.

Mitral valve and left ventricular thrombi in an infant with acquired protein C deficiency. A case report.

The authors present an infant with left ventricular and mitral valve thrombi diagnosed by cross-sectional echocardiography. Thrombosis was due to acquired transient protein C deficiency, which was caused by impaired liver function due to sepsis. Because the thombi were very large and mobile, urgent surgery was performed. Eight weeks later, the patient's protein C level returned to normal ranges. The authors suggest that in all cases with intracardiac thrombosis, protein C deficiency should be investigated.

An unusual late complication following surgical repair of tetralogy of Fallot.

A case is presented of extrusion of right ventricular outflow tract patch material through a mediastino-cutaneous fistula occurring five years after repair of tetralogy of Fallot. The focus of the mediastinal infection was an infected epicardial pacemaker electrode placed at the time of initial surgery. Subsequent echocardiographic studies showed minimal right ventricular outflow tract or pulmonary arterial dilatation, and, with no further surgery, the child is well after two and a half years follow-up.

Thyroid hormones mediated effect of insulin on alloxan diabetic rat atria.

1. The influence of alloxan-induced diabetes was studied on spontaneously beating rat atria. Diabetic atria were found to have decreased rates, increased contractility and decreased responsiveness to both inotropic and chronotropic effects of isoprenaline. 2. Thyroid hormone levels were significantly reduced in diabetic animals. This revealed that the decrease in atrial beta-adrenergic responses was associated with a reduction in serum levels of thyroid hormones. 3. Insulin treatment of diabetic rats for 10 days corrected the changes observed in diabetic atria. Serum levels of thyroid hormones returned to normal following insulin treatment as well. 4. Administration of insulin to thyroidectomized-diabetic rats did not reverse the diabetes-induced changes suggesting that thyroid hormones are needed for insulin to normalize the alterations observed in diabetic atria.

Aorto-left ventricular communication arising from the left aortic sinus of Valsalva: echocardiographic diagnosis and surgical repair.

A rare case of aorto-left ventricular communication arising from the left aortic sinus is described. This is the first reported case, known to us, in which the diagnosis was established by two-dimensional echocardiography and angiocardiography and a successful surgical repair was accomplished.

Open mitral commissurotomies. Analysis of 100 cases.

Routine use of open mitral commissurotomy in all cases of rheumatic mistral stenosis is the recommended treatment of choice in recent years. Effective division of the subvalvular apparatus (chordae tendineae and papillary muscles) can be accomplished easily during open commissurotomy and therefore recurrences after the open approach is less than the recurrences after closed commissurotomy. These subvalvular elements can not be freed easily during closed mitral commissurotomy. Low mortality rates after open approach suggests that this operation can be performed safely in cases of MS. Open approach has several advantages versus closed commissurotomy. We believe that closed commissurotomy should be replaced by open commissurotomy in all cases of MS.

Inhalation of foreign bodies in children. Report of 500 cases.

Inhalation of foreign bodies is a major cause of accidental death during childhood. Aspiration of foreign bodies is common in children aged 1 to 3 years, especially in boys. A past history of foreign body aspiration is itself an indication for bronchoscopic examination of the airways, because some children with aspirated foreign bodies are without symptoms and chest x-ray films may not show abnormalities. Bronchoscopic removal of the foreign bodies requires close communication between the anesthesiologist and the endoscopist. Forgotten foreign bodies in the airways cause chronic pulmonary infections, allergic asthma, bronchiectatic changes, and lung abscess. Foreign bodies that cannot be grasped by bronchoscopic forceps should be removed by thoracotomy and bronchotomy. This report describes our experience in 500 children with suspected foreign body inhalation. We routinely use prednisolone, 1 to 2 mg. per kilogram, and nebulization just after bronchoscopic examination of the airways. This medication greatly diminishes the rate of postbronchoscopic complications such as laryngeal edema, which require tracheostomy. In our series of 500 case, the incidence of postbronchoscopic tracheostomy is 1.4 per cent and the total mortality rate is 1.8 per cent.