Stephen E. Cyran

Neonatal pancytopenia and severe combined immunodeficiency associated with antenatal administration of azathioprine and prednisone

Immune thrombocytopenic purpura and pregnancy. N Engl J Med 306:826, 1982. 6. Chirico G, Duze M, Ugazio AG, Rondini G: High-dose intravenous gammaglobulin therapy for passive immune thromboeytopenia in the neonate. J PEDIATR 103:654, 1984. 7. Schulman l: Clinical disorders of the platelets. In Nathan DG, Osaki FA, editors: hematology of infancy and childhood. Philadelphia, 1974, WB Saunders, p 645. 8. Oski FA, Naiman JL: Hematologic problems in the newborn. Philadelphia, 1982, WB Saunders, p 188. 9. Jones RE, Asher MI , Rutherford C J, Monro HM: Autoimmune (idiopathic) thrombocytopenie purpura in pregnancy and the newborn. Br J Obstet Gynaecol 84:679, 1977. 10. Brecker G, Cronkite EP: Morphology and enumeration of blood platelets. Appl Physiol 3:365, 1950.

Efficacy of intraoperative transesophageal echocardiography in children with congenital heart disease

The feasibility and potential adverse effects of using intraoperative transesophageal echocardiography (TEE) in 19 children ages 7.5 to 16 years undergoing surgical repair of a variety of congenital heart defects were evaluated. The ability of TEE to assess the adequacy of surgical repair as well as left ventricular function and wall motion abnormalities in this setting was also examined. Intraoperative transesophageal 2-dimensional and Doppler evaluation, and, in selected patients, echo-contrast and color flow imaging, were performed with either a 3.5- or 5.0-MHz phased array probe mounted within the tip of a flexible gastroscope. Probe insertion was successful in 18 of 19 patients. Fiberoptic endoscopy (9 patients) and autopsy (1 patient--cardiac donor) performed within 24 hours of surgery demonstrated no significant esophageal abnormalities. Intraoperative wall motion abnormalities were identified in 8 patients but did not persist after the operation. An adequate surgical repair was demonstrated by contrast and color flow imaging in most patients. Microcavitation was detected in 6 patients for greater than 5 minutes after a standard debubbling procedure. No patient displayed any adverse neurologic effects. It is concluded that, with the currently available probes, intraoperative TEE can be performed safely and reliably in children as young as 7.5 years of age. The procedure provides valuable information regarding wall motion abnormalities, cardiac function and the adequacy of surgical repair.

The Norwood operation and subsequent Fontan operation in infants with complex congenital heart disease

From April 1987 to September 1993, 60 infants underwent a Norwood operation for complex congenital heart disease including hypoplastic left heart syndrome (n = 41), ventricular septal defect and subaortic stenosis with aortic arch interruption/severe coarctation (n = 7), complex single right ventricle with subaortic stenosis (n = 8), critical aortic stenosis with endocardial fibroelastosis (n = 2), and malaligned primum atrial septal defect with coarctation (n = 2). Age at operation ranged from 1 day to 3.9 months (mean 9 days, median 3.5 days). The operative mortality (< 30 days) was 33% (20 patients). Late mortality was 17% (10 patients). Nine of the 20 (45%) operative deaths occurred during the first 2 days after the operation as a result of sudden hemodynamic instability. All four infants with premature closure of the foramen ovale had pulmonary lymphangiectasia and died of pulmonary failure. Seven operative deaths have occurred in 36 patients since 1990 (19%); in the past 2 years, no operative deaths have occurred in 22 patients. Overall, there are 30 long-term survivors (50%). Twenty-one of these 30 infants have undergone a two-stage repair with a modified Fontan operation at 7.3 to 27.6 months of age (mean 18.1 months) with no mortality. Six patients have entered a three-stage repair strategy by undergoing a hemi-Fontan procedure at 6.8 to 23.0 months (mean 8.8 months) with no mortality, and two of these patients have now had their modified Fontan operation at 23.0 to 46.7 months of age with no mortality (four are still awaiting surgery). Two patients have undergone a two-ventricle repair with a Rastelli procedure, with no mortality at 7.4 and 14.1 months of age. Early in our experience, infants undergoing the Norwood operation had a high early mortality most often related to sudden hemodynamic instability. After we instituted a protocol that adds carbon dioxide to the inspired gas during postoperative mechanical ventilation, the postoperative course became more stable and there have been no operative deaths. In summary, the operative mortality for the Norwood operation continues to improve. A subsequent Fontan operation can be performed with excellent clinical results.

Evaluation of balloon aortic valvuloplasty with transesophageal echocardiography

Percutaneous balloon valvuloplasty for the relief of congenital aortic valve stenosis in pediatric patients is being performed with increasing frequency.‘a2 Balloon valvuloplasty, however, may be complicated by cusp tear or avulsion, resulting in a worsening of aortic valve incompetence.3s4 Recognition of these adverse effects during the procedure could caution against additional dilatations with larger balloon catheters and may prevent further deterioration in aortic valve competence. Transesophageal echocardiography, which has proven to be a valuable technique for monitoring intraoperative cardiac function and myocardial ischemia in adults, would appear to be ideally suited for such a task.5 We describe the first use of transesophageal echocardiography to monitor cardiovascular changes during percutaneous balloon valvuloplasty in an adolescent with congenital aortic stenosis. A 14-year-old obese boy with congenital valvar aortic stenosis was admitted for percutaneous balloon valvuloplasty. Prior cardiac catheterization had demonstrated a 58 mm Hg peak systolic aortic valve pressure gradient and mild aortic valve incompetence. Graded exercise testing had revealed 2 mm of left precordial ST segment depression. The patient remained asymptomatic until 3 months prior to admission, when he developed angina and decreased exercise tolerance. On physical examination, the patient was obese (115 kg), with a blood pressure of 110/80 mm Hg. A prominent left ventricular impulse, precordial thrill, and grade 4/6 systolic ejection murmur were present. An early diastolic murmur was not audible. Chest roentgenography showed left ventricular prominence, while electrocardiography revealed left ventricular hypertrophy with T wave inversion in the left precordial leads. Precordial two-dimensional echocardiography was of poor quality due to the patient’s obesity. Continuous wave Doppler examination demonstrated a 120 mm Hg peak instantaneous systolic gradient and mild aortic valve incompetence. Catheterization and valvuloplasty were performed under general anesthesia. Following endotracheal intubation, a 5 MHz transesophageal echocardiography probe (Hewlett-Packard Co., Andover, Mass.) capable

Transcarotid Balloon Valvuloplasty with Continuous Transesophageal Echocardiographic Guidance for Neonatal Critical Aortic Valve Stenosis: An Alternative to Surgical Palliation

Abstract. Neonatal critical aortic valve stenosis is a life-threatening malformation if untreated. Before the late 1980s, the preferred treatment was surgical valvotomy; however, operative mortality was high. Early reports of transcatheter balloon dilation were encouraging, although femoral artery damage and aortic valve insufficiency were procedural limitations. With new balloon catheter technology, transumbilical, transvenous, and transcarotid approaches have been advocated, although a comparison with recent surgical results has not been performed. We compared all neonates who presented to our institution since 1985 with the diagnosis of critical aortic stenosis. Ten patients underwent surgical transventricular valvotomy and 13 patients underwent balloon valvuloplasty via a right carotid cutdown with continuous transesophageal echocardiographic guidance. Prior to intervention, all patients had either left ventricular dysfunction, an aortic valve gradient >100 mmHg, significant mitral valve insufficiency, and/or ductal dependent systemic blood flow. All patients had successful relief of aortic valve obstruction with normalization of left ventricular function and successful discontinuation of prostaglandin E1. Use of continuous transesophageal echocardiographic guidance resulted in fluoroscopic exposure of only 12 ± 8 minutes. At the latest follow-up, a similar proportion of patients has required additional aortic valve procedures (38% vs 25%) and overall mortality (20% vs 15%) is similar. In the transcarotid group, 9 of 13 patients (69%) have a normal appearing right carotid artery by Duplex imaging, and no neurologic events have been reported. Balloon aortic valvuloplasty via a right transcarotid approach is safe, simplifies crossing the valve, and is effective for the initial palliation of neonatal critical aortic stenosis. The use of transesophageal echocardiographic guidance reduces fluoroscopy exposure, enables accurate assessment of hemodynamics without catheter manipulation or angiography, and avoids femoral artery injury.

A reconsideration of risk factors for the Fontan operation.

We reviewed our experience in 38 patients who underwent a Fontan operation. In the first five patients ages 7.5 to 23 years (mean, 15 years), a conduit was placed from the right atrium to the small right ventricle or the pulmonary artery (PA). The remaining 33 patients, ages 7 months to 14 years (mean, 4.8 years), had a modified Fontan operation with direct systemic venous or right atrial to PA anastomosis. The diagnoses were tricuspid atresia (n = 14), single ventricle (n = 10), hypoplastic right or left ventricle (n = 9), double-outlet right ventricle with inlet ventricular septal defect and pulmonary atresia or stenosis (n = 3), criss-cross ventricles and transposition of the great arteries (n = 1), and atrioventricular canal and anomalous pulmonary venous connection (n = 1). Thirty-two patients had previous surgery. Other procedures included PA banding (n = 7), systemic to PA shunts (n = 25), Norwood operation (n = 3), and a Damus-Kaye-Stansel anastomosis (n = 1), repair of total anomolous pulmonary venous connection (n = 1), a Blalock-Hanlon atrial septectomy (n = 1), and enlargement of a restrictive ventricular septal defect (n = 1). There were four operative deaths (10.5%), three from low cardiac output and one from subaortic obstruction. There were no deaths in patients younger than 3 years of age (n = 13). Subaortic obstruction developed in six of the seven patients who had pulmonary artery banding and resulted in three deaths. In our experience, diagnosis, previous surgery, type of previous operation, PA pressure, and younger age are not risk factors for early or late death. Subaortic obstruction is a major risk factor for late death. Accordingly we now perform a Damus-Kaye-Stansel anastomosis combined with a systemic to PA shunt in those children with excessive pulmonary blood flow who anatomically are likely to develop subaortic obstruction. A modified Fontan operation can be performed any time after 1 year of age and in some patients after 6 months of age, providing the anatomy and physiology of the patient are acceptable.

Role of balloon atrial septostomy before early arterial switch repair of transposition of the great arteries.

Preoperative balloon atrial septostomy is the standard therapy for babies with uncomplicated cyanotic dextrotransposition of the great arteries despite the effectiveness of prostaglandin E1 infusion in alleviating systemic hypoxemia and the reported success of arterial switch repair during the 1st weeks after birth. The clinical records and echocardiographic findings of 23 infants (mean birth weight +/- SD 3.3 +/- 0.5 kg) with uncomplicated transposition of the great arteries were analyzed. Fifteen infants (Group I) did not undergo septostomy, and 8 (Group II) underwent septostomy. Before prostaglandin infusion, mean arterial oxygen tension (Po2) in Group I (26 mm Hg) did not differ from that in Group II. After prostaglandin infusion, Po2 increased significantly in Group I (43 +/- 8 mm Hg, p less than 0.001) but not in Group II despite a widely patent ductus and predominant left to right ductal shunt in all. After septostomy, Po2 increased significantly (43 +/- 4 mm Hg, p less than 0.03), and did not differ from that in Group I. Echocardiographic features generally demonstrated a nonrestrictive foramen ovale in Group I and a restrictive foramen ovale in Group II. The latter was associated with persistent hypoxemia after prostaglandin. Thus, the diameter of the foramen ovale was the primary factor influencing arterial oxygenation during prostaglandin infusion. Babies underwent the arterial switch operation at a mean age of 70 +/- 65 h with an overall survival rate of 96%; there was only one postoperative death (Group II). Absence of septostomy had no negative influence on any postoperative variable, including duration of ventilatory and inotropic support, time to discharge, or mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

The Fontan operation in infants less than 2 years of age

Young age remains a reported risk factor for a successful Fontan operation despite improved survival rates. Since March 1978, the Fontan operation has been performed in 47 patients. To avoid a primary or secondary palliative shunt, an early Fontan procedure (Group 1: mean age 1.5 +/- 0.5 years, range 0.6 to 2) has been performed in 17 children with the outcome similar to that of the remaining 30 older patients (Group 2: mean age 7.5 +/- 5 years, range 2.4 to 23 years). Preoperatively both groups had acceptable hemodynamic status for a successful Fontan result. Operative variables including cardiopulmonary bypass time, aortic cross-clamp time and core temperature were similar between groups and did not affect mortality. The postoperative mortality rate including early surgical (0% vs. 13%, respectively), late (18% vs. 12%) and total (18% vs. 23%) was similar between Groups 1 and 2 (p greater than 0.05). Immediate postoperative arrhythmias were more frequent in Group 1 (71% vs. 25%, p less than 0.01) with no related mortality, while late arrhythmias occurred with equal frequency (29% vs. 39%, p greater than 0.05). Group 1 infants required a longer hospital stay (22 +/- 9 vs. 14 +/- 5 days, p less than 0.01). Thus, young age is not a risk factor for successful outcome of the Fontan operation in patients with acceptable preoperative hemodynamic status. An early Fontan operation may also avoid prolonged palliative procedures and their potential deleterious effects.

Comparison of the cardiac output and stroke volume response to upright exercise in children with valvular and subvalvular aortic stenosis

Cardiac output and stroke volume were evaluated in 17 children (mean age 11.5 +/- 3 years) with discrete, membranous subvalvular (Group I, n = 7) and valvular (Group II, n = 10) aortic stenosis during submaximal and maximal (greater than 75% predicted maximal oxygen consumption) upright cycle ergometry. Patients with valvular aortic stenosis were further subdivided on the basis of their aortic valve gradient at rest determined by cardiac catheterization (Group IIA, gradient less than 40 mm Hg; Group IIB, gradient greater than or equal to 40 mm Hg). These patients were matched with 17 control subjects on the basis of age, sex, height and intensity of exercise during maximal exertion. Cardiac and stroke indexes were determined by the acetylene rebreathing method at each exercise level. Stroke volume index in Group I was significantly greater at rest when compared with that in control subjects (69 +/- 13 versus 53 +/- 11 ml/m2, alpha = 0.01, p less than 0.05) and that in patients in Group II (69 +/- 13 versus 47 +/- 12 ml/m2, alpha = 0.01, p less than 0.05). Patients with subvalvular aortic stenosis were unable to increase their stroke volume index from rest to submaximal exercise and also decreased their stroke volume index at maximal exercise levels. In contrast, patients with mild valvular aortic stenosis (Group IIA) displayed a normal exercise response. Patients with severe valvular aortic stenosis (Group IIB) had a blunted stroke volume response at rest and at each level of exercise, as well as signs of myocardial ischemia (ST segment depression) during maximal exercise.(ABSTRACT TRUNCATED AT 250 WORDS)

Initial results and clinical follow-up after balloon angioplasty for native coarctation

This study describes the initial hemodynamic results and early to late clinical follow-up, including magnetic resonance imaging and exercise testing, following balloon angioplasty for native coarctation of the aorta. We advocate this approach as an alternative to surgical intervention in select patients based on age, aortic arch anatomy, or in those patients who have coexisting cardiac defects that are amenable to transcatheter intervention.