Cecille O. Sunderland

Late complications after femoral artery catheterization in children less than five years of age

Fifty-eight children who underwent diagnostic femoral artery catheterization before 5 years of age, from 5 to 14 years before the study, were randomly selected from approximately 300 surviving patients undergoing diagnostic femoral artery catheterization at our institution during the interval. Each patient underwent vascular laboratory segmental pressure and waveform examination and arterial duplex scanning, as well as lower extremity bone length radiographs, which were considered positive if the catheterized leg was greater than or equal to 1.5 cm shorter than the opposite leg. Thirteen children who had only venous catheterization served as controls. No arterial abnormalities were present in the control patients (mean ankle/brachial index, 1.01). Arterial occlusion was present in both limbs of five patients who had bilateral diagnostic femoral artery catheterization and in 14 limbs of 51 patients who had unilateral diagnostic femoral artery catheterization. Thus arterial occlusion was present in 33% of patients (19 of 58) and in 37% of limbs (24 of 65). The mean ankle/brachial index in the catheterized limbs was 0.79. Leg growth retardation was present in four limbs (8%) of 51 children undergoing unilateral diagnostic femoral artery catheterization and in one (8%) control patient. The inverse relationship between ankle/brachial index and leg growth retardation was significant (R = 0.47, p less than 0.0005). Only one patient had symptoms of arterial occlusion (claudication), and one patient had symptoms of leg growth retardation (gait disturbance). We conclude that arterial occlusion is common after diagnostic femoral artery catheterization in children less than 5 years of age, but that excellent collateral supply prevents leg growth retardation and/or symptomatic arterial insufficiency in most children.(ABSTRACT TRUNCATED AT 250 WORDS)

Ehlers-Danlos syndrome associated with multiple pulmonary artery stenoses and tortuous systemic arteries

A young girl with Ehlers-Danlos syndrome had evidence of multiple severe peripheral pulmonary artery stenoses and of tortuosity of the aorta and its major thoracic divisions, including the coronary arteries. It appears likely that the basic defect of Ehlers-Danlos syndrome may involve many arteries and may cause important vasular obstruction.

Total correction of tetralogy of Fallot in infancy. Postoperative hemodynamic evaluation.

Total intracardiac repair of symptomatic tetralogy of Fallot was accomplished in twenty-nine infants under two years of age with a mortality of six per cent. All twenty-seven survivors are asymptomatic. Postoperative hemodynamic evaluation of 17 randomly selected children was performed at least 12 months following surgery. Pulmonary regurgitation was present in 12 of the 17 children but was well tolerated, with only minimal cardiac enlargement and in no case were there symptoms or important hemodynamic consequences. Growth of the pulmonary artery with respect to the aorta occurred. Normal left ventricular performance was indicated by normal ejection fraction and normal systemic arterial pressure. Intellectual and social development were indistinguishable from a “normal” group of randomly selected children as assessed by detailed psychological testing.

Natural History of Tetralogy of Fallot in Infancy Clinical Classification and Therapeutic Implications

We employ a classification system for tetralogy (TF) that correlates clinical findings with the nature and severity of right ventricular (RV) outflow obstruction. Group 0 infants are acyanotic, Group 1 and 2 infants have intermittent or mild cyanosis with hypoxic spells, Group 3 infants have severe cyanosis without spells, and Group 4 infants have severe cyanosis and pulmonary artery (PA) hypoplasia without spells. Since 1964, 59 infants were identified as having TF, documented by catheterization. Contrary to previous reports, the majority (40 of 59) were acyanotic (Group 0) at birth. Cyanosis and hypoxic spells subsequently developed at mean ages of 6.1 and 13.4 mo respectively, due to increasing muscular infundibular hypertrophy, as well as fibrosis and growth failure of the RV outflow tract, due to diminished pulmonary blood flow. At catheterization (mean age 12.7 mo) only two were still in Group 0. An understanding of the progressive nature of TF suggests that infants in groups 1-3 can have total correction before significant undergrowth of the RV outflow tract occurs. Shunts are performed only in Group 4 infants. Twenty-eight infants from ten weeks to two years old had total correction. Five were under six months. There were two operative deaths (7%) and no late deaths. Heart block did not occur. Late hemodynamic studies in 17 patients reveal good relief of RV obstruction (mean RV-PA gradient 19 mm Hg) and no significant shunts. The natural history of TF in infancy can be successfully interrupted in most cases by early correction.

Postoperative hemodynamic and electrophysiologic evaluation of the interatrial baffle procedure

Hemodynamic and electrophysiologic studies were performed in 11 children with dextrotransposition of the great arteries an average of 26 months after the interatrial baffle procedure and, in 2 patients, additional closure of a ventricular septal defect. All children are clinically well. Right to left shunts ranging from 28 to 63 percent of systemic blood flow were found at the superior vena caval-baffle junction in four children. The superior vena caval-baffle gradient averaged 7 mm Hg (range 0 to 22). Right ventricular stroke work index averaged 39 g-m/beat per m2 and right ventricular end-diastolic pressure 9 mm Hg. These values were not significantly different from the values for the systemic left ventricle in a comparable group of normal children (average left ventricular stroke work index 45 g-m/beat per m2 and average left ventricular end-diastolic pressure 8 mm Hg). Cardiac index, heart rate and arteriovenous oxygen difference were also normal. No child has complete heart block. His bundle recording demonstrated normal H-V intervals (range 27 to 40 msec); 4 of the 11 had a prolonged A-H interval. Left ventricular systolic pressure was less than 40 mm Hg in all but two children who had significant subpulmonary stenosis. Pulmonary vascular resistance averaged 1.9 units and was decreased in all children. We conclude that up to 37 months postoperatively, despite some residual abnormalities, the clinical and hemodynamic condition of these children is excellent.

Percutaneous cardiac catheterization and atrial balloon septostomy in pediatrics

Between November, 1971, and July, 1975, 688 patients ranging in age from six hours to 21 years have undergone percutaneous transfemoral venous and/or arterial catheterization at the University of Oregon Health Sciences Center. This represents 93% of all venous catheterizations of the right side of the heart and 97% of all arterial catheterizations of the left side of the heart in the pediatric age range. One hundred and ninety-five patients (29% of the group) weighed 5 kg or less and 133 (20% of the group) were neonates. There were virtually no complications following the venous studies. The complication rate following percutaneous arterial studies is lower than is the reported experience with cut-down arteriotomies. Long-term survival following percutaneous atrial balloon septostomy in D-transposition of the great arteries was comparable to that following cut-down balloon septostomy. The ease of accomplishment, significantly lower complication rate, and successful accomplishment of atrial balloon septostomy prompt us to advocate that the percutaneous technique of cardiac catheterization be extended to include the neonate and young infant who weighsless than 5 kg.

Complete heart block in a fetus associated with maternal Sjögren's syndrome

This report illustrates the case of a fetus who was diagnosed by M-mode echocardiography, at the twentieth week of gestation, as having a complete heart block. The mother had a positive antinuclear antibody test result with antibodies directed against the Ro (SSA) and La (SSB) antigen system consistent with the diagnosis of Sjögrens syndrome. Management and outcome of the pregnancy are discussed.

Cerebral Arteriovenous Fistula Producing Tem porary Heart Failure in a Newborn Infant Report of Prolonged Survival Without Surgical Therapy

Cerebral arteriovenous malformations producing heart failure in in fancy with or without neurologic manifestations are usually fatal. This infant with a large cerebral arteriovenous fistula exhibited severe heart failure at 12 hours of age, yet never had any neurologic abnormality. His heart failure regressed with medical management. At 20 months of age the only abnormality on physical examination was a cranial bruit. An arteriovenous fistula anywhere in the body can cause conges tive heart failure in infancy, even in the absence of localizing symptoms.Cerebral arteriovenous malformations producing heart failure in in fancy with or without neurologic manifestations are usually fatal. This infant with a large cerebral arteriovenous fistula exhibited severe heart failure at 12 hours of age, yet never had any neurologic abnormality. His heart failure regressed with medical management. At 20 months of age the only abnormality on physical examination was a cranial bruit. An arteriovenous fistula anywhere in the body can cause conges tive heart failure in infancy, even in the absence of localizing symptoms.

Congenital pulmonary artery-subclavian steal†

A 3-month-old infant with bounding pulses and cardiac enlargement was proved to have a “steal” whereby blood was diverted from the vertebral-basilar system to the subclavian artery and to the lungs. Although relatively common as an acquired phenomenon in adults, the congenital form of subclavian steal is quite rare.

Respiratory Characteristics of Blood following the Interatrial Baffle Procedure for Dextro Transposition of the Great Arteries

Five children with D-transposition of the great arteries continued to have an elevated blood 2,3-DPG concentration and a lowered blood oxygen affinity for at least 10 days following corrective surgery. Five of ten children in whom arterial oxygen saturation was elevated at least 6 months following surgery, continued to have saturations of less than 90%. Thus, some postoperative patients appear to have a continuing need for facilitated tissue oxygen availability following the Mustard operation (interatrial baffle procedure).