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The use of interventional devices to complete or augment repair of congenital heart lesions capitalizes on the collaborative skills of the cardiovascular team: cardiology, cardiovascular surgery, and nursing. Placement of devices, eliminating the requirement for surgical intervention, places new demands on nursing staff regarding patient and family preparation. Supporting families, if the inability to place a device occurs, is a critical function of the nursing staff. The experimental nature of the devices and the complexity of the disease places additional stress on the family and increases the demands of postimplantation nursing care.

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Although the early mortality for repair of truncus arteriosus has decreased in the modern era, routine correction in the neonate has not been widely adopted. To assess the results of our protocol of early repair, we reviewed 46 neonates and infants undergoing repair of truncus arteriosus at the University of Michigan Medical Center from January 1986 to January 1992. Their ages ranged from 1 day to 7 months (median 13 days) and weights from 1.8 kg to 5.4 kg (mean 3.1 kg). Repair was performed beyond the first month of life in only 8 patients, because of late referral in 7 and severe noncardiac problems in 1. Associated cardiac anomalies were frequently encountered, the most common being interrupted aortic arch (n = 5), nonconfluent pulmonary arteries (n = 4), hypoplastic pulmonary arteries (n = 4), and major coronary artery anomalies (n = 3). Truncal valve replacement was performed in 5 patients with severe regurgitation, 3 of whom also had truncal valve systolic pressure gradients of 30 mm Hg or more. The truncal valve was replaced with a mechanical prosthesis in 2 patients and with a cryopreserved homograft in 3 patients. Right ventricle-pulmonary artery continuity was established with a homograft in 41 patients (range 8 mm to 15 mm), a valved heterograft conduit in 4 (range 12 mm to 14 mm), and a nonvalved polytetrafluoroethylene tube in the remaining patient (8 mm). There were 5 hospital deaths (11%, 70% confidence limits 7% to 17%). Multivariate and univariate analyses failed to demonstrate a relationship between hospital mortality and age, weight, or associated cardiac anomalies. Only 1 death occurred among 9 patients with interrupted aortic arch or nonconfluent pulmonary arteries. Hospital survivors were followed-up from 3 months to 6.3 years (mean 3 +/- 0.4 years). Late noncardiac deaths occurred in 3 patients, all within 4 months after the operation. Actuarial survival was 81% +/- 6% at 90 days and beyond. Despite the prevalence of major associated conditions, early repair has resulted in excellent survival. We continue to recommend repair promptly after presentation, optimally within the first month of life.

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BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is considered when respiratory failure (RF) persists despite increasing conventional mechanical ventilation (CMV). High-frequency jet ventilation (HFJV) can improve ventilation with comparable mean airway pressure (PAW) to that found on CMV. This study was undertaken to determine whether HFJV is an effective treatment and alternative to ECMO for RF after congenital heart surgery. METHODS AND RESULTS: HFJV was instituted in nine patients ranging in age from 6 days to 3.3 years with congenital heart disease meeting pulmonary criteria for ECMO. Indications for HFJV were pulmonary hypertension (six), adult-type respiratory distress syndrome (two), and pneumonitis (one). Seven patients (77%) were placed on HFJV within 24 hours of operation, and two patients required HFJV 2 weeks after operation. HFJV resulted in resolution of RF in eight of nine patients (89%). After 1 hour of HFJV, the arterial pH increased from 7.40 +/- 0.1 to 7.56 +/- 0.1 (p < 0.05) and the PaCO2 decreased from 44 +/- 15 to 29 +/- 12 mm Hg (p < 0.05). During HFJV there was no change in PaO2, although the FIO2 decreased from 0.99 +/- 0.0 to 0.73 +/- 0.2 (p < 0.05). There was no change in PAW, peak inspiratory pressures, positive end-expiratory pressures, heart rate, or mean arterial blood pressure during HFJV when compared with CMV. Mean duration of HFJV was 43 hours. Four patients were extubated and discharged from the hospital. Two patients were extubated but died from sepsis. Two patients had resolution of RF, but one died at reoperation and one from multisystem organ failure. The patient who failed HFJV therapy was placed on ECMO and died. CONCLUSIONS: This study suggests that HFJV improves ventilation and is an alternative to ECMO in patients with RF after surgery for congenital heart disease.

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Discrete subaortic stenosis typically appears as a well-defined membrane beneath the aortic valve. To assess the merits of alternative approaches to this problem, we have reviewed the results of operations for discrete subaortic stenosis from 1978 through 1990. Excision of the subaortic membrane alone was performed in 16 patients (group I). Excision of the membrane with resection of septal muscle was performed in 24 patients (group II). The groups were similar in age at operation, duration of follow-up, and preoperative and postoperative transvalvar gradients. There were no operative or late deaths. Reoperations for recurrent subaortic stenosis were performed in 4 group I patients (25%; 70% confidence limits, 16% to 38%) and 1 group II patient (4%; 70% confidence limits, 2% to 11%). Pacemakers were inserted for postoperative complete heart block in 1 group I patient (6%; 70% confidence limits, 2% to 16%) and 2 group II patients (8%; 70% confidence limits, 4% to 16%). We conclude that muscle resection combined with membrane excision in patients with discrete subaortic stenosis does not increase the risk of death or heart block, and does lower the risk of reoperation for recurrent subaortic stenosis.

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Hypoplastic left-heart syndrome is a common congenital heart defect that carries a mortality rate of 95% in the first month of life if left untreated. In the past, surgical options were not accepted. However, recent success with the Norwood operation as part of a staged reconstructive approach has renewed interest in operative intervention for these critically ill neonates. Assessment of the balance between systemic and pulmonary blood flow is critical to maintain cardiac output and systemic saturation.

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An assessment of late morbidity and mortality is essential before arterial repair can be considered truly corrective for patients with transposition of the great arteries. We describe the early and intermediate-term results in 126 patients who underwent arterial repair. Operation was performed at a median age of 6 days, with 76 patients operated on within the first 7 days of life. Coronary artery anatomy differed from the usual arrangement in 37 patients. Simultaneous procedures included ventricular septal defect closure (35) and repair of interrupted aortic arch (2) or coarctation (5). Hospital mortality was seven of 126 (5.5%), with three deaths among the most recent 100 patients (3%). There were one late, noncardiac death and one late death after reoperation. Reoperation for pulmonary artery stenosis was required in 10 of the first 63 patients (16%), all of whom underwent pulmonary artery reconstruction with separate patches for closure of the coronary excision sites. Of the last 63 patients, all of whom underwent pulmonary artery reconstruction with a single pantaloon-shaped pericardial patch, one (2%) required reoperation for pulmonary artery stenosis. Doppler flow studies and echocardiography performed in 115 of 119 surviving patients at a mean of 12 months after repair demonstrated normal left ventricular function, minimal left ventricular outflow gradients, and no more than trivial aortic regurgitation. Peak gradient across the right ventricular outflow tract was 19 +/- 3 mm Hg in patients with separate pulmonary artery patches and 5 +/- 2 mm Hg in those with a single pantaloon patch (p = 0.0001). Follow-up is 96% complete from 1 month to 8 years after operation (mean 2.5 years). The actuarial survival rate at 5 years, including operative mortality, was 92%. All patients are in sinus rhythm, and none requires antiarrhythmic medications. These data suggest that pulmonary artery reconstruction with a single pantaloon patch may be associated with a decreased requirement for reoperation. Intermediate-term survival and functional results are excellent after arterial repair for transposition of the great arteries.

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The nursing care of the infant with total anomalous pulmonary venous connection is complex and requires extensive nursing expertise. This article describes the presenting signs and symptoms of neonates and infants with total anomalous pulmonary venous connection, the surgical technique for repair, and the critical nursing assessment required postoperatively.

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Following cardiac surgery in children, accurate monitoring of right ventricular function, as reflected by central venous pressure (CVP) or right atrial pressure, is crucial. Positioning the child supine has been considered necessary to achieve an accurate CVP reading. In this study the effect of backrest elevation on CVP readings in children following cardiac surgery was explored. The sample consisted of 40 children, 1 day to 9 years of age, who had undergone cardiac surgery within the previous 72 hours. CVP measurements at 0- and 30-degree backrest elevations were obtained hourly for 8 hours for each child. CVP readings were not significantly altered by the bedrest elevation. These findings suggest children may remain elevated for CVP readings rather than being awakened and repositioned to a 0-degree backrest elevation. For children who have nasogastric feedings, respiratory insufficiency, or increased intracranial pressure, a 30-degree backrest elevation may be maintained for the CVP reading, as well as for the children's safety and comfort.

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Transposition of the great arteries is a common form of cyanotic congenital heart disease that without intervention has a 90% mortality rate within the first year of life. In the past, surgical options were limited to physiologic correction at the atrial level, but long-term follow-up has revealed problems with right ventricular dysfunction, tricuspid valve insufficiency, arrhythmias, and venous obstruction. This has renewed an interest in the arterial switch as an early, definitive form of anatomic correction for infants with transposition. As selection criteria have been refined, the mortality rate at experienced centers now equals that of physiologic correction. Nursing care centers around the critically ill neonate who may have hypotension and low cardiac output because of a poorly adapted left ventricle. Assessment for potential myocardial infarction resulting from movement and reimplantation of the coronary arteries is essential.

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Between 1976 and 1986, 19 children aged 1 month to 5 years underwent replacement of the mitral (systemic atrioventricular) valve. Indications for valve replacement included isolated congenital mitral stenosis (n = 2), valve dysfunction associated with a more complex procedure (n = 15), and failed valvuloplasty (n = 2). Seven different valve types were used; nine were mechanical valves and ten were bioprosthetic valves. There were 6 hospital deaths (32%; 70% confidence limits, 20% to 47%). Among the 13 survivors there were 3 late deaths at a mean of 14 months after operation. The late deaths were unrelated to valve malfunction. Thromboembolic events occurred in 2 patients, both with mechanical valves. One minor bleeding complication occurred among 10 patients on a regimen of Coumadin (crystalline warfarin sodium). Five patients, all with bioprostheses, required a second valve replacement. Indications for reoperation included prosthetic valve regurgitation (n = 1) and calcific stenosis (n = 4). No early or late deaths occurred after second valve replacement. Survival was 51% +/- 12% (standard error) at 112 months after valve replacement. Analysis failed to identify age, weight, sex, previous operation, underlying cardiac lesion, or prosthesis size and type as significant risk factors for mortality. Mechanical valves had a lower reoperation rate compared with bioprostheses. These data suggest that although mitral valve replacement within the first 5 years of life is associated with a high operative and late mortality, satisfactory long-term palliation for many patients can be achieved. Mechanical valves are superior to bioprosthetic valves, and offer the best long-term results.

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Infants with truncus arteriosus present a difficult management issue. Because of the high operative mortality, repair is often delayed beyond the first 3 to 6 months of age. We reviewed our experience with 11 neonates and young infants with truncus arteriosus undergoing repair (median age, 21 days). Five patients also had major truncal valve insufficiency, and 2 required valve replacement. Right ventricle-pulmonary artery continuity was established with a porcine valved conduit in 3 patients and an aortic or pulmonary homograft in 8. There was 1 operative death (9%; 70% confidence limits, 3%-22%) and 1 late death over a mean follow-up of 21 months (range, 4 to 32 months). Eight of the 9 late survivors are growing normally. Echocardiographic examination revealed normal ventricular function in all patients (mean shortening fraction, 39%). Doppler assessment demonstrated trivial prosthetic or homograft valve regurgitation in 7 patients and mild to moderate obstruction in 5 patients. This recent experience with repair of truncus arteriosus indicates that the operative risk is low even in the neonate. Repair in the first month of life should be recommended before the development of critical congestive heart failure or irreversible pulmonary vascular disease.

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