Medical therapy for the hydropic fetus with congenital complete atrioventricular block.

Early delivery, immediate ventricular pacing, and inotropic support have failed to improve the outcome of hydropic fetuses with congenital complete atrioventricular block. On detection of hydrops, two fetuses were treated with maternally administered digoxin and furosemide with prompt and sustained resolution of intrathoracic fluid accumulations. Clearance of serosal fluid collections may prevent pulmonary hypoplasia and improve cardiac function. Before proceeding with premature delivery in such patients, a brief trial of transplacental anti-congestive therapy is indicated.

Transesophageal echocardiography and the intraoperative management of pediatric congenital heart disease: initial experience with a pediatric esophageal 2D color flow echocardiographic probe.

Intraoperative color Doppler transesophageal echocardiography (TEE) was performed in 26 patients undergoing corrective or palliative surgery for congenital heart disease. Age ranged from 1 day to 15 years, and body weight ranged from 2.9 to 42 kg. Objectives of the study were to determine the smallest infant in whom the pediatric probe could be used safely, additional diagnostic value, and it role in the intraoperative assessment of the surgical repair. The insertion of the pediatric probe was possible in all 26 patients. The smallest infant in this series was a newborn weighing 2.9 kg. Excellent correlation was obtained with preoperative transthoracic echocardiographic findings and operative findings. Assessment of the surgical repair was obtained in the immediate postcardiopulmonary bypass period. No short-term complications occurred in this series. Intraoperative color Doppler TEE provided a detailed and accurate assessment of the morphology, the function of the heart, and altered the management of at least two patients.

Abnormalities in position of left ventricular papillary muscles in congenital aortic stenosis.

Subclinical structural abnormalities may accompany some congenital cardiovascular abnormalities. Echocardiographic observations led us to hypothesize that the positions of the left ventricular papillary muscles are abnormal in hearts with aortic valvar stenosis. To test this hypothesis, we examined 6 normal heart specimens and hearts with congenital cardiovascular malformations, including 5 with pulmonary atresia and an intact ventricular septum, 6 with tetralogy of Fallot and 5 with aortic valvar stenosis. We marked the papillary muscles and the mitral commissures, X-rayed the hearts, and measured the angular positions of the papillary muscles using the midpoint of a chord drawn between the mitral commissures as a reference point. The direction from the midpoint to the lateral commissure was designated as 0 degrees. The data (mean +/- SEM) were analyzed using a computer program (ANOVA). In normal hearts, the anterolateral and posteromedial papillary muscles were positioned, respectively, at 43 +/- 19 degrees and 126 +/- 26 degrees. The positions of the papillary muscles were similar to normal in the hearts with pulmonary atresia (62 +/- 38 degrees and 128 +/- 27 degrees) and tetralogy of Fallot (40 +/- 13 degrees and 130 +/- 37 degrees). In aortic stenosis, the locations of the papillary muscles (-76 +/- 42 degrees and 71 +/- 25 degrees) were significantly different from normal (P less than 0.05). The arc between the papillary muscles was 83 +/- 16 degrees in normals and 147 +/- 45 degrees in aortic stenosis (P less than 0.05). The length of the arc was similar to normal in other heart specimens. Thus, the papillary muscles were abnormally positioned in aortic stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Extended aortoplasty to relieve supravalvular aortic stenosis.

An extended aortoplasty was used to relieve severe supravalvular aortic stenosis in 5 patients whose preoperative left ventricular-aortic gradient ranged from 85 to 140 mm Hg (median, 120 mm Hg). The stenotic ring above the commissures was divided in two places by an inverted U incision extending into the right and noncoronary sinuses. A distal vertical incision in the ascending aorta converted this into an inverted Y. The ridge above the left coronary sinus was excised. The aortic incision was repaired with an inverted Y-shaped Dacron gusset. The postoperative gradient ranged from 0 to 30 mm Hg (median, 15 mm Hg). The extended aortoplasty provides excellent relief of supravalvular aortic stenosis and, in addition, restores the aortic root geometry to a much more anatomical configuration than is achieved with the simple patch technique.

Long-term palliation with the classic Blalock-Taussig shunt.

Eighty-five patients received a classic Blalock-Taussig shunt between 1973 and 1986. Their age range was 1 day to 9.3 years and their median age was 4 months. Forty-one percent (35/85) were less than 1 month of age. The basic operative technique was unchanged throughout the time period. The subclavian artery opposite the side of the arch was used in 89% (79/88) of the patients. All anastomoses were done with monofilament suture and there was a tendency toward smaller suture material (7-0) in the latter years. All anastomoses except one were done with an interrupted suture technique. The operative mortality rate was 4.7% (4/85) and was not statistically related to age, diagnosis, or year of operation. Palliation was considered to be satisfactory until either a second shunt or a premature corrective operation were necessary. Seven patients required a second shunt and three, a premature corrective operation. The mean time between the initial shunt and the second procedure, either a second shunt or a corrective operation, was 2.9 years and 2.4 years, respectively. Twenty-five patients have had an elective corrective operation and the mean interval to that procedure was 3.9 years. Two years after the operation, 97% of patients older than 1 month of age at operation remain in well-palliated condition, as do 87% of those less than 1 month of age. At 4 years, 87% of those older than 1 month and 54% of those less than 1 month of age continue to be in well-palliated condition. The classic Blalock-Taussig shunt provides excellent palliation at a low operative mortality for virtually all patients for a minimum of 2 years. It will provide adequate pulmonary blood flow for most patients for an extended period of time beyond 2 years.

Partial atrioventricular canal defect: the early and late results of operation.

Thirty-five patients underwent repair of a partial atrioventricular canal defect and have been followed for a mean of 8 years. Seventeen patients had either moderate or severe mitral incompetence prior to operation. The mitral valve was treated as a bileaflet structure. The cleft in its anterior leaflet was closed in each patient in whom the valve was incompetent. The septal defect was closed with pericardium, and the coronary sinus was left on the right atrium. Two patients required reoperation, 1 to close a recurrent septal defect and 1 to replace a mitral valve that had been competent for the first several years after the initial repair. All 33 survivors remain in New York Heart Association Functional Class I. Nineteen patients have no mitral incompetence, and the remaining 14 have only mild incompetence. These data demonstrate that excellent late results from repair of partial atrioventricular canal are possible when the mitral valve is managed as a bileaflet structure.

The long-term risk of warfarin sodium therapy and the incidence of thromboembolism in children after prosthetic cardiac valve replacement.

Thirty children less than 18 years of age underwent cardiac valve replacement with a prosthetic valve between 1967 and 1984 and have been followed up for a mean of 6 years (range 1 to 17 years). Their mean age at the time of operation was 13 years (range 6 to 17 years). All patients were begun on a regimen of warfarin before hospital discharge. One major and four minor bleeding episodes occurred in 211 patient-years of warfarin therapy, an incidence of 2.3 per 100 patient-years. Three of those five episodes occurred in patients who were receiving excessively anticoagulation or who were participating in physical activities inappropriate for a patient on warfarin therapy. Thus, the majority of the bleeding episodes were preventable. There were five thromboembolic events in 211 patient-years, an incidence of 2.3 per 100 patient-years. Three of those five patients had intentionally stopped their warfarin therapy. The majority of thromboembolic episodes, like the bleeding episodes, were preventable. Eight teenage patients were noncompliant with the warfarin therapy. More than one third of that group experienced a thromboembolic event, an incidence of 5.5 per 100 patient-years (55 patient-years). Twenty-two patients adhered to the warfarin regimen and only two (9%) of them had a thromboembolic event, an incidence of 1.3 per 100 patient-years (156 patient-years). Warfarin therapy presented no greater risk of serious bleeding to this pediatric age group than it does to an adult age group. The incidence of thromboembolism among these patients was less than that which is generally reported for adult patients. Discontinuation of or noncompliance with warfarin therapy substantially increased the risk of thromboembolism. Continuous warfarin therapy is recommended for every child after prosthetic valve replacement.

Late results of the Mustard procedure in transposition of the great arteries.

Thirty-five patients were discharged from the hospital after a Mustard procedure and have been followed a mean of 4.5 years. Thirty remain in a stable atrial or sinus rhythm, 3 have required hospitalization and medication to control atrial dysrhythmias, 1 is in a junctional rhythm, and 1 requires a pacemaker. In no patient has there been clinical evidence of systemic or pulmonary venous baffle obstruction. Twenty-two patients have had an elective postoperative cardiac catheterization, which has confirmed the absence of baffle obstruction. We attribute the lack of baffle obstruction and the low incidence of atrial dysrhythmias to several technical points in the operation including: direct high cannulation of the superior vena cava, aggressive resection of the atrial septum except in the region of the atrioventricular node, an extremely large pericardial baffle trimmed in the manner of Brom, sharp divergence of the upper and lower limbs of the inferior suture line away from one another and toward the caval orifices once they enter the right atrium, and very superficial endocardial bites of the baffle suture line in the region of the sinoatrial and atrioventricular nodes.

Bilateral phrenic nerve paralysis after the Mustard procedure. Experience with four cases and recommendations for management.

Unilateral phrenic nerve injury is a recognized complication of thoracic operations, but bilateral diaphragmatic paralysis after an intracardiac procedure in an infant has not previously been described. In the past 10 years, four infants have sustained a bilateral phrenic nerve injury during the performance of a Mustard procedure. They were managed with tracheostomy and prolonged mechanical ventilation. Their recovery period ranged between 30 and 103 days and each had a satisfactory outcome. This technique was preferred to bilateral diaphragmatic plication because the results of that procedure have been equivocal. Tracheostomy reduced the catastrophic risk of an obstructed endotracheal tube, allowed immediate oral intake, and simplified the weaning process from the ventilator.

Early and late results of repair of partial anomalous pulmonary venous connection to the superior vena cava with a pericardial baffle.

The early and late results of repair of partial anomalous pulmonary venous connection to the superior vena cava with a simple pericardial baffle without enlargement of the superior vena cava were examined. Fifteen consecutive patients received operations between 1973 and 1983, and all survived. They have been followed for a mean of 6 years and a minimum of 2 years. Nodal rhythm and atrial dysrhythmias were present in 6 patients (40%) early after operation. However, every patient resumed normal sinus rhythm prior to hospital discharge except 1 adult who remained in the preoperative rhythm of atrial flutter-fibrillation. No patient has clinical evidence of a residual atrial level shunt or superior vena cava obstruction. All have received an excellent clinical result, and none, except the patient in chronic atrial fibrillation, require cardiac medication. These results are comparable or superior to those obtained with more intricate procedures that use either complex atrial flaps or translocation of the superior vena cava to the atrial appendage.

The Hancock external valved conduit. A dichotomy between late clinical results and late cardiac catheterization findings.

The Hancock external valved conduit was inserted in 18 children between 1974 and 1977. Seventeen patients survived operation and 15 are long-term survivors of 6 to 9 years. All patients have been closely followed up by the same pediatric cardiologist and each has undergone serial cardiac catheterization studies, generally performed at 1 and 6 years after operation. All long-term surviving patients have been in either New York Heart Association Class I or II. A few continue to take digoxin or diuretics. Cardiac catheterization demonstrated a gradient of 50 mm Hg or greater across the conduit in two of 16 patients 1 year after operation and in seven of 15 patients 6 years after operation. Five patients (30% of all long-term survivors) have had this conduit replaced and each has survived reoperation. The finding of a severe conduit gradient in one third of all long-term surviving patients is particularly bothersome since these patients were essentially asymptomatic. This study emphasizes the need for serial follow-up cardiac catheterization studies in all patients who receive the Hancock conduit.

Echocardiographic definition of right pulmonary venous connection at catheterization.

Traditional methods of identifying partial anomalous pulmonary venous connection to the right atrium in the presence of an atrial defect are not always reliable. Twenty patients were studied with a new technique in which the catheter is introduced into the right superior pulmonary vein followed by echocardiographic assessment of the catheter position in relation to the left atrium and atrial septum. The insertion site of the right pulmonary veins was detected in every patient and in ten patients has been verified at operation. This approach can be performed rapidly and appears to be accurate as well as reliable.

Parenteral indomethacin for closure of the patent ductus arteriosus. Clinical experience with 67 preterm infants.

During a 19-month period, 23% of preterm infants had symptomatic patent ductus arteriosus (PDA). Intravenous indomethacin was administered to 67 infants with successful closure in 91% of the patients and in 83% of those with birth weights less than 1,000 g. No differences were found between the number of doses required and birth weight, gestational age, or age at initial therapy. Transient alterations in renal function were common after therapy, but mild renal failure occurred in only four infants. The incidence of intraventricular hemorrhage and necrotizing enterocolitis in treated infants was similar to that in the total preterm population. Cicatricial retrolental fibroplasia developed in one patient. Bronchopulmonary dysplasia occurred more frequently in infants with PDA, but a decreased incidence was found after early ductal closure. Intravenous indomethacin administration successfully effects ductal closure without major complications in preterm infants.

Respiratory syncytial viral infection in infants with congenital heart disease.

Occasional reports have suggested that infants with congenital heart disease may have an increased risk of severe illness from respiratory syncytial virus (RSV) infection. We prospectively studied 699 infants hospitalized during the winters of 1976 through 1980, when RSV was prevalent in the community; 229 of these infants had proved RSV infections acquired either before admission or during hospitalization; 27 had both congenital heart disease and RSV infection, and 46 had congenital heart disease without RSV infection. Infected infants with congenital heart disease had significantly more severe illness than those without congenital heart disease, as judged by the requirement for intensive care and assisted ventilation and by the mortality rate (37 per cent vs. 1.5 per cent, P less than 0.01). The infection was acquired nosocomially by 21 per cent of infected infants; the mortality rate from nosocomial infection was also higher in infants with congenital heart disease (44 per cent vs. 5 per cent, P less than 0.01). Pulmonary hypertension was the one condition particularly associated with severe RSV illness. Eight of the 11 infants (73 per cent) with congenital heart disease and pulmonary hypertension died during their RSV illness. The courses in infants with congenital heart disease with and without RSV infection were also compared. Their ages, types of cardiac lesions, and incidence of pulmonary hypertension were similar, but the infants with RSV infection had a higher mortality rate (37 per cent vs. 6.5 per cent, P less than 0.1).

Early closure of the patent ductus arteriosus in very low-birth-weight infants: a controlled trial.

A controlled clinical trial comparing early closure (mean = 48.8 hours) of the patent ductus arteriosus using indomethacin to conventional medical management, with intervention only after cardiopulmonary decompensation (mean = 167.4 hours), was undertaken in 24 preterm infants with severe respiratory distress syndrome and evidence of PDA. An interval analysis of one-half the projected sample revealed that infants undergoing early closure of the PDA had significantly reduced occurrence of BPD or mortality by 6 months of age. A comparison of birth weight, Apgar scores, gestational age, age of initial PDA diagnosis, and fluid therapy during the first seven days of life showed no significant differences between early intervention and control groups. At the time of the interval analysis, there were no differences between the groups in duration of intermittent mandatory ventilation or oxygen exposure. Studies will be required to determine whether these and other variables can be altered by early closure of the PDA.

Trial of bivalent influenza A vaccine in high-risk infants.

A vaccine trial involving 50 high-risk infants, aged six to 36 months, was performed for evaluation of the safety of a split-virus bivalent influenza A vaccine. After immunization, 18% of the infants developed a fever of greater than or equal to 100 F and 7% had fever of greater than or equal to 102 F. Other reactions to the vaccine were few. However, the reaction index of these high-risk infants to the bivalent vaccine was higher than those of older children receiving the same vaccine and normal infants receiving the monovalent vaccine. Forty-one percent of the infants responded with a titer of hemagglutination-inhibiting antibody of greater than or equal to 1:20 to the A/Victoria/75 component of the bivalent vaccine, and 35% responded to the A/New Jersey/8/76 component. This result suggested that this vaccine used in two doses would be an effective vaccine for infants. The infants generally tolerated the vaccine well. Nevertheless, the rapid development of fever would be of concern in the infant whose underlying disease was marginally compensated.