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Biomedical subjects

B L Perry

Publications and source records attributed to B L Perry.

At least 19 recordsLinked to original sources
PubMed1990

Down's syndrome, complete atrioventricular canal, and pulmonary vascular obstructive disease.

We reviewed our experience over a 10-year period to determine whether children with Down's syndrome and complete atrioventricular canal develop pulmonary vascular obstructive disease earlier than children with normal chromosomes and this defect. Comparisons were made between Down's syndrome and normal chromosome children regarding (1) pulmonary blood flow and pulmonary vascular resistance at initial catheterization, (2) operability as related to elevation in pulmonary vascular resistance, and (3) age at diagnosis of fixed pulmonary vascular obstructive disease. The 45 patients with Down's syndrome catheterized under 1 year of age had a lower mean pulmonary blood flow (3.2 versus 5.7; p = 0.0001) and higher mean pulmonary vascular resistance (8.3 versus 4.6 Wood units.m2; p = 0.0003) than their 34 normal chromosome counterparts. When all ages were included, 38 of 81 (47%) of the children with Down's syndrome and 32 of 40 (80%) of the normal children were considered operable. Non-Down's syndrome patients who had operations had a higher pulmonary blood flow (5.8 versus 3.3; p = 0.004) and lower pulmonary vascular resistance (3.6 versus 6.0 Wood units.m2; p = 0.005) than Down's syndrome patients. Of the 34 patients who did not have operations because of pulmonary vascular obstructive disease, 31 had Down's syndrome. In 10 of 81 children with Down's syndrome, fixed pulmonary vascular obstructive disease was diagnosed before the age of 1 year, while this was found in none of 40 normal children. Our data demonstrate that Down's syndrome patients with complete atrioventricular canal have a greater degree of elevation of pulmonary vascular resistance in the first year of life and more rapid progression to fixed pulmonary vascular obstructive disease than children with normal chromosomes.

Adolescent↗
PubMed1987

Surgical and medical results of complete atrioventricular canal: a ten year review.

The files of 121 patients who presented to Children's Hospital of Michigan over the last 10 years with complete atrioventricular (AV) canal were reviewed to evaluate long-term management and overall outcome. Of 121 patients, 70 underwent corrective surgery, 21 (30%) of whom died perioperatively. The surgical mortality rate was 13% when patients with hypoplastic left or right ventricle (n = 6), double-orifice mitral valve or extreme deficiency of mitral tissue (n = 5), and pulmonary vascular obstructive disease (n = 5) were excluded. Of the 49 patients who survived operation, 36 are in New York Heart Association class I, 1 patient requires a pacemaker and 3 died late. In 34 of the 51 patients (28%) who did not undergo operation, pulmonary vascular obstructive disease developed; it occurred within 12 months in 10 patients (8%). Eight other patients who did not undergo operation died before planned surgery (age 1 to 9 months). Although surgical prognosis in good candidates is acceptable, the overall prognosis for children with complete AV canal is guarded because of the risk of early death or early pulmonary vascular obstructive disease and frequently unfavorable anatomy.

Cardiac Catheterization↗
PubMed1987

Pacing in children and young adults with nonsurgical atrioventricular block: comparison of single-rate ventricular and dual-chamber modes.

A prospective comparison of physiologic response to single-rate ventricular and dual-chamber atrioventricular pacing was conducted in 14 pediatric patients (age 1 to 24 years, median 14) with symptomatic nonsurgical second- or third-degree atrioventricular block. All patients were studied acutely during cardiac catheterization before and after 1 hour of both pacing modes. Following pacemaker implant, eight patients were reevaluated after 1 month of each mode with symptom questionnaire, resting ECG, resting echocardiogram, and Doppler cardiac output measurement at rest and at peak treadmill exercise. Cardiac outputs (mean +/- standard error) increased acutely (n = 14) with both ventricular (32 +/- 12%) and dual-chamber (39 +/- 10%) pacing over intrinsic rhythm values (p less than 0.01 in both). During chronic pacing (n = 8), symptoms were reported only with the ventricular mode. Dual-chamber synchronous pacing was associated with improved mean resting shortening fraction and cardiac output, slower mean resting sinus rate (89 +/- 5 compared to 73 +/- 4 bpm (p less than 0.02), and a 23% increase in mean excerise cardiac output (4.2 +/- 0.4 compared to 3.4 +/- 0.3 L/min/m2) compared to single-rate ventricular pacing. Exercise-induced dysrhythmias occurred only with ventricular pacing. This study demonstrates that pediatric patients with nonsurgical atrioventricular block can compensate for loss of atrioventricular synchrony at rest but exhibit improved cardiac function with chronic dual-chamber atrioventricular compared to single-rate ventricular pacing.

Adolescent↗
PubMed1983

Myocardial dysfunction in hypothyroid children.

Eleven children with primary hypothyroidism were studied by echocardiography. Eight patients had pericardial effusion. Systolic time intervals (preejection period-ejection time ratio and left ventricular isovolumic contraction time) were suggestive of abnormal myocardial function. Asymmetric septal hypertrophy without left ventricular out-flow obstruction (ventricular septal-left ventricular posterior wall diastolic thickness ratio, greater than 1.3) was present in two hypothyroid children. After full replacement therapy, these cardiac abnormalities reversed to normal. Our data suggest that the myopericardial response to hypothyroidism in children is similar to that seen in adults.

Adolescent↗
PubMed1982

Classic shunting operations for congenital cyanotic heart defects.

A total of 297 consecutive classic systemic artery-pulmonary artery shunts were performed in 281 patients over the past 9 years. This experience includes 200 Blalock-Taussig shunts (67.3%), 84 Waterston shunts (28.2%), and 13 Potts shunts (4.3%). The overall early mortality after the Blalock-Taussig shunt (5.5%) was significantly lower (p less than 0.02) than for the Waterston shunt (13%). The early mortality after either the Blalock-Taussig or Waterston shunt was not statistically related to the age of the patient at operation. Both the Blalock-Taussig and Waterston shunts demonstrated a higher mortality (p less than 0.02) among patients in Group II (complex defects) than in patients in Group I (tetralogy of Fallot and pulmonary atresia with ventricular septal defect [VSD]). However, the Blalock-Taussig shunt had a significantly lower (p less than 0.04) probability of early postoperative death than the Waterston shunt among patients with complex lesions. The Blalock-Taussig shunt also revealed a lower incidence of important late postoperative complications and did not affect adversely the hemodynamic result after intracardiac correction, as evidenced by late postoperative cardiac catheterization. This study also confirms the feasibility of the Blalock-Taussig shunt during the neonatal period without significantly increased age-related risk of postoperative death (p = 0.13) and with good early and late long-term patency rates.

Cardiac Output, Low↗
PubMed1981

Surgical treatment of cor triatriatum.

Six patients with cor triatriatum underwent surgical correction. They ranged for 1.5 to 93 months old (mean, 22 months). Congestive heart failure was present in 3 patients. Cardiomegaly and increased pulmonary vascularity were evident roentgenographically in all patients. Cardiac cineangiography demonstrated the subdividing left atrial membrane in 5 patients and suggested the correct diagnosis by revealing an abnormal configuration of the left atrium in the other patient. The opening in the anomalous left atrial membrane was stenotic in every instance. The proximal left atrial chamber communicated with the right atrium through an atrial septal defect in 5 patients and with the systemic venous circuit through a persistent left superior vena cava in the other patient, in whom the atrial septum was intact. A right atrial-transseptal approach provided ample exposure for complete excision of the obstructing membrane and repair of the atrial septum in all patients. One patient died of low cardiac output during the early postoperative period. The other 5 are alive and well at an average of 48 months after operation.

Blood Pressure↗
PubMed1981

Results of the Mustard operation for dextro-transposition of the great arteries.

One hundred twenty patients with dextro-transposition of the great arteries (TGA) underwent intra-arterial baffle repair using thin Dacron fabric from 1971 to 1979. The ages of the patients ranging from 29 days to 17 years (mean age 28 months). Thirty-five patients had undergone 49 preliminary palliative operations. Early postoperative mortality was 4.8% for patients with simple TGA but was higher among patients with associated ventricular septal defect (26%), VSD and left ventricular outflow tract obstruction (28.5%) and intact ventricular septum with left ventricular outflow tract obstruction (12.5%). Early nonfatal postoperative complications included low cardiac output (23.3%), respiratory insufficiency (35.8%), junctional rhythm (34.1%), superior vena caval (SVC) obstruction (9.1%), and chylothorax (7.5%). Late postoperative mortality for all groups was 7.5% (nine patients). Late cardiac dysrhythmias occurred in 33 patients (40.7%). Normal sinus rhythm has been preserved in all patients since direct, high SVC cannulation was instituted. Late postoperative hemodynamic and angiographic evaluation in 61 patients revealed severe to total SVC obstruction in 12 patients (20.2%) and pulmonary venous obstruction in four patients (6.5%). Among the latter, two patients died following reoperation and another patient died without operation. SVC obstruction was clinically important in only three patients, two of whom have undergone successful reoperation. All other late survivors are acyanotic and clinically well. The Mustard operation has dramatically improved the survival rate and quality of life for patients with TGA. However, postoperative caval and pulmonary venous obstruction are problems which require additional technical modifications and stimulate the search for alternative corrective operations.

Adolescent↗
PubMed1980

Early and late results of total correction of tetralogy of Fallot.

Two hundred and nine patients underwent total repair of tetralogy of Fallot without congenital pulmonary atresia from 1971 to 1979. The age of the patients ranged from 22 months to 27 years (mean 6.8 years). Previous palliative shunts were present in 130 patients (62.2%). The right ventricular outflow tract obstruction was relieved by infundibulectomy in 31 patients (14.8%), by transannular patching in 142 patients (67.9%), and by insertion of a right ventricular--to--pulmonary artery prosthetic conduit in 10 patients (4.7%) with associated coronary artery anomalies. A porcine valve was inserted after transannular patching only in patients with undersized pulmonary arteries, unilateral pulmonary artery, or congenitally absent pulmonary valve. There were 10 early postoperative deaths (4.7%). Previous palliative shunts did not adversely affect early postoperative mortality rate. Surgically induced permanent complete heart block occurred in one patient (0.4%). Six patients (3%) died in the late postoperative period, three of them of unrelated causes. Late results were good in 170 patients (87%), fair in 10 patients (5.1%), and poor in 15 patients (7.6%). Poor results were due to residual stenosis at the origin of the pulmonary arteries in 13 patients (6.5%) and to residual ventricular septal defect in two patients (1%). The mean cardiothoracic ratio was 0.61 for patients with poor results and 0.54 for the entire group. Pulmonary valvular insufficiency was well tolerated postoperatively in the absence of distal pulmonary artery obstruction. This experience supports a policy of aggressive relief of the right ventricular outflow tract obstruction including liberal use of transannular patching and, when indicated, extensive reconstruction of the pulmonary artery branches. We also recommend a two-stage treatment program for symptomatic infants with unfavorable anatomy consisting of initial Blalock-Taussig shunt followed by total repair at about 3 years of age.

Adolescent↗
PubMed1978

Estimation of pulmonary/systemic resistance ratios from echocardiographic systolic time intervals in young patients with congenital or acquired heard disease.

Previous work has shown the positive correlation of echocardiographic right ventricular preejection period/right ventricular ejection time ratio (RPEP/RVET) with pulmonary vascular resistance and pulmonary arterial diastolic pressure obtained at cardiac catheterization. However, the correlation was insufficient to predict pulmonary arterial diastolic pressure or vascular resistance from a given RPEP/RVET ratio. In this study the RPEP/RVET ratio was compared with left ventricular preejection period/ejection time ratio (LVEP/LVET) in 25 patients undergoing cardiac catheterization, and a strong correlation was found between the ratio (RPEP/RVET)/(LPEP/LVET) = R/L and the ratio of pulmonary arteriolar resistance/systemic arteriolar resistance (PAR/RS), especially when R/L was correlated with log10 PAR/RS (r = 0.902). A very high correlation (r = 0.960) was found between R/L and log10 PAR/RS when the group was restricted to patients with a ventricular septal defect or a complete endocardial cushion defect. Regression equations for prediction of PAR/RS have been derived for the various groups.

Adolescent↗
PubMed1977

Ventricular septal defect: results after repair in infancy.

During the 19 years from 1957 through 1975, there have been 106 patients under age 2 years who have undergone surgery for repair of a large ventricular septal defect at the University of Michigan Medical Center. The majority of the patients had either severe pulmonary hypertension or intractable congestive heart failure. Eighty-three infants survived operation; there has been one late death. The greatest mortality occurred in patients under age 6 months and in those with severe pulmonary hypertension. Surviving infants showed marked symptomatic improvement and change in growth patterns. Complications included the development of complete right bundle branch blodk or left anterior hemiblock in approximately 50 percent of patients and, in one instance, complete atrioventricular block. Forty-five patients have undergone cardiac catheterization 1 to 8 years postoperatively. Although 17 were found to have residual septal defects only 3 of these had a pulmonary to systemic flow ratio of 1.5:1 or more, and reoperation was accomplished without incident in these 3 patients and in 3 others with smaller shunts. With one exception, postoperative pulmonary arterial pressures and pulmonary to systemic vascular resistance ratios were normal or near normal, thus representing a significant contrast with findings in patients operated on after age 2 years. Whereas the complications of surgery appear no greater in the infant than in the older patient, many of the benefits can be realized only with operation at the earlier age.

Cardiac Catheterization↗