Extracorporeal membrane modality conversions.

We report the case of a patient with cardiovascular and respiratory failure due to severe anaphylaxis requiring multiple extracorporeal membrane oxygenation (ECMO) cannulation strategies to provide adequate oxygen delivery and ventilatory support during a period of rapid physiological change. ECMO provides partial or complete support of oxygenation-ventilation and circulation. The choice of which ECMO modality to use is governed by anatomical (vessel size, cardiovascular anatomy and previous surgeries) and physiological (respiratory and/or cardiac failure) factors. The urgency with which ECMO needs to be implemented (emergency cardiopulmonary resuscitation (eCPR), urgent, elective) and the institutional experience will also influence the type of ECMO provided. Here we describe a 12-year-old schoolgirl who, having been resuscitated with peripheral veno-venous (VV) ECMO for severe hypoxemia due to status asthmaticus in the setting of acute anaphylaxis, required escalation to peripheral veno-arterial (VA) ECMO for precipitous cardiovascular deterioration. Insufficient oxygen delivery for adequate cellular metabolic function and possible cerebral hypoxia due to significant differential hypoxia necessitated ECMO modification. After six days of central (transthoracic) VA ECMO support and 21 days of intensive care unit (ICU) care, she made a complete recovery with no neurological sequelae. The use of ECMO support warrants careful consideration of the interplay of a patient's pathophysiology and extracorporeal circuit dynamics. Particular emphasis should be placed on the potential for mismatch between cardiovascular and respiratory support as well as the need to meet metabolic demands through adequate cerebral, coronary and systemic oxygenation. Cannulation strategies occasionally require alteration to meet and anticipate the patient's evolving needs.

Paediatric heart transplantation in Australia comes of age: 21 years of experience in a national centre.

BACKGROUND: Heart transplantation (HT) is established therapy for end-stage heart failure in children with cardiomyopathy or congenital heart disease. AIMS: This review summarises experience at a national referral centre since the first local transplant. METHODS: Medical records of children referred for HT between 1 April 1988 and 1 January 2010 were retrospectively reviewed. All patients listed for HT were included. Survival analysis was used to summarise wait-list time to death/transplant, and separately, time to death in HT patients. RESULTS: One hundred and thirty-nine children were accepted on to the HT waiting list during the study (median age 7.7 (interquartile range (IQR) 2.5, 13.6) years), of whom 93 underwent HT (median age 10.9 (IQR 4.4, 14.6) years). Wait-list mortality was 32% (45 of 139 patients), lowest among children aged >10 years at listing (P < 0.001). Median time to HT was 69 days (range 29-146). Survival post-transplantation was 90% (95% confidence interval 82-95) at 1 year, 82% (72-89%) at 5 years and 68% (50-80%) at 10 years. Increasing case complexity over the study period included pre- and post-transplant circulatory support, management of pulmonary hypertension and introduction of ABO-incompatible HT for infants. Post-transplant survival did not vary according to age, pre-transplant diagnosis or use of pre-transplant circulatory support (all P > 0.05). CONCLUSIONS: Results of paediatric HT in Australia are comparable with international results, despite limitations of geographic isolation, small population and low organ donation rate. Increasing case complexity has not impacted on post-transplant survival.

Surgical Management of Ebstein Anomaly: The Australia and New Zealand Experience.

BACKGROUND: Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical difficulty of valve repair. The natural history of the anomaly differs between patients presenting in the neonatal period to those presenting in adulthood. METHODS: A retrospective review of all patients >15 years of age with Ebstein anomaly, undergoing surgery on the tricuspid valve at 6 centers across Australia and New Zealand was performed. Patients from 1985 to 2019 were included in the study. RESULTS: A total of 125 patients were included in the study, 76 patients (60%) undergoing tricuspid valve repair, of which 23 patients underwent a Cone repair and 49 (40%) had a tricuspid valve replacement. The mean follow-up was 7.9 ± 7.3 years. Postoperatively, early mortality was 3 patients (2%) and 10-year survival was 91.5%. A postoperative pacemaker was required in 24 patients (19%). Reoperation was required in 21 patients (17%). There was no statistically significant difference in survival or reoperation between patients who underwent repair or replacement of the tricuspid valve; however, with a small number of patients in long-term follow-up. CONCLUSION: Older children and adult patients undergoing surgery for Ebstein anomaly in Australia and New Zealand experience good medium-term postoperative survival. Repair of the valve is achieved in a significant proportion of patients with increasing use and success with the Cone repair technique.

Enteral feeding in duct-dependent congenital heart disease.

BACKGROUND: Infants with duct-dependent congenital heart lesions are treated with a prostaglandin E1 infusion. We aimed to describe the feeding strategies used at our institution in such infants, and to describe the incidence of necrotising enterocolitis (NEC) in this patient group, investigating whether enteral feeding is associated with a higher risk. METHODS: Patients diagnosed with hypoplastic left heart syndrome, coarctation of the aorta, pulmonary atresia, or transposition of the great arteries born over a defined period were identified. Premature infants, those with pre-existing gastrointestinal disease, and those who never received prostaglandin were excluded. Data were compared using univariable and multivariable logistic regression models. RESULTS: A total of 177 patients were identified, of them 18 received a diagnosis of suspected or confirmed NEC. There was no association between the diagnosis of NEC and enteral feeding (P = 0.9). CONCLUSIONS: Based on these data, there does not appear to be an association between enteral feeding and NEC in infants receiving prostaglandin.

Initial results of primary device closure of large muscular ventricular septal defects in early infancy using perventricular access.

OBJECTIVES: To report our experience with the use of the Amplatzer muscular ventricular septal defect (VSD) occluder, using direct right ventricle free wall puncture for primary closure of muscular VSDs in infants. BACKGROUND: Young infants with heart failure due to large or multiple muscular VSDs often require intervention at a stage when percutaneous device closure is impractical due to delivery system limitations. There are considerable benefits to avoiding bypass in these infants. METHODS: Patients with suitable muscular VSDs, considered too small for transvenous closure, underwent periventricular muscular VSD closure under transesophageal echo (TEE) guidance. RESULTS: Eight infants underwent the procedure. The median age was 14 weeks (2-41) with median weight of 4 kg (3-6.6). Four patients had additional VSDs and one patient had previous repair of coarctation. One patient had dextrocardia and severe left ventricular impairment due to an associated cardiomyopathy. The median VSD size was 8 mm (range, 4-10 mm). A single device (6-12 mm) was deployed in each patient, the size chosen being 1-2 mm larger than the TEE measurement. Two patients had subsequent pulmonary artery banding, with absorbable bands, because of moderate residual shunting through additional VSDs. The latest echo study at a median of 7.2 weeks (0.5-66) post device implantation revealed no patients with a hemodynamically significant shunt. CONCLUSION: Perventricular muscular VSD closure can be safely and effectively undertaken in small infants with suitable muscular defects, and this strategy can avoid the need for bypass.

Clinical features and management of isolated cleft mitral valve in childhood.

OBJECTIVES: We reviewed an institutional experience of isolated cleft mitral valve (ICMV), its clinical features, and management in a pediatric population. BACKGROUND: As ICMV is relatively uncommon, earlier reports highlighted its anatomical and echocardiographic features. Few studies have collated their clinical features with their outcome. METHODS: All patients with ICMV were retrospectively reviewed. Patients who were considered to have an atrioventricular septal defect or variant were excluded. RESULTS: Twenty patients (9 male, 11 female) were diagnosed with ICMV. Seven patients had associated cardiac lesions. The median age of diagnosis was 5.2 years (range 0.4 to 13.6 years). Echocardiography aided by color Doppler demonstrated the ICMV in all patients. However, an incomplete diagnosis was made in 4 of 20 patients before surgery. The severity of the mitral regurgitation (MR) at presentation was mild in 11, moderate in 8, and severe in 1 patient. In the 13 patients without associated cardiac lesions, 5 underwent mitral valve (MV) repair at median age of 5.2 years (range 1.2 to 7.7 years) for moderate to severe MR, 4 being symptomatic. The severity of the MR in seven of the eight unoperated patients has remained unchanged over the follow-up period (median 8.3 years, range 0.7 to 14.4 years). In total, 10 patients underwent MV repair (median 6.4, range 0.4 to 13.8 years). No patient required MV replacement. None of the 10 patients had more than mild MR over the follow-up period (median 0.6, range 0.2 to 11.0 years). CONCLUSIONS: Now readily diagnosable by echocardiography, ICMV is a correctable cause of MR with a good outcome. Surgery is indicated in those patients with moderate to severe MR and probably should be done early following diagnosis.

[Modified technique creating fenestration in the extracardiac Fontan completion; kissing anastomosis].

Acute or subacute occlusion of a fenestration between the Fontan chamber and the atrium causes low cardiac output status, sustained pleural effusion, and protein losing enteropathy in critical cases. To achieve reliable and long patency of the fenestration in extracardiac Fontan operations, we modified our technique for creating a fenestration, namely "kissing" anastomosis, in which the atrial incision is directly sutured to the surface of the extracardiac conduit leaving a few millimeters outside from the edge of the punched-out hole (fenestration). We applied this modification in 2 cases. The diameter of the hole is 4 mm (case 1) and 3 mm (case 2). The patients were anticoagulated by warfarin potassium after the surgery. The fenestrations were patent at 3- (case 1) and 1-month (case 2) after the surgery. Peripheral oxygen saturations at discharge were 86 (case 1) and 88% (case 2). There was no death or major complication. The described procedure is an effective alternative to maintain patency and function of the fenestration in extracardiac Fontan operations.

Hemoglobin Grange-Blanche [beta 27(B9) Ala----Val], a new variant with normal expression and increased affinity for oxygen.

Hemoglobin Grange-Blanche [beta 27(B9) Ala----Val] is a new variant found in a Portuguese family. The carriers present moderate erythrocytosis. Upon isoelectric focusing, Hb Grange-Blanche was slightly more cathodic than Hb A. beta Grange-Blanche chain migrated like the G gamma chain when submitted to electrophoresis in the presence of urea-Triton X-100. The precentage of Hb Grange-Blanche was about 50% in the heterozygous state. Oxygen affinity was increased (P50 = 22 mmHg), but heme-heme interaction was normal. An abnormal tryptic peptide (beta T3) was isolated using HPLC. Its composition allowed us to deduce unambiguously the amino acid change. The latter is the third mutation found in position 27 of the beta-chain. Because of its normal expression and its elevated affinity for oxygen, Hb Grange-Blanche contrasts with Hb Knossos [beta 27(B9) Ala----Ser], a beta-thalassemic variant with low affinity.

Transatrial-transpulmonary tetralogy of Fallot repair is effective in the presence of anomalous coronary arteries.

OBJECTIVES: The study's object was to analyze the outcomes of transatrial-transpulmonary repair in children with tetralogy of Fallot and anomalous coronary artery crossing the right ventricular outflow tract. METHODS: The transatrial-transpulmonary approach was used in 611 consecutive repairs, 36 (5.9%) of which were associated with a surgically relevant coronary artery anomaly. The median age and weight of the patients at repair were 23 months (2.8-170 months) and 9.9 kg (5.2-41 kg), respectively. Anomalies included left anterior descending coronary artery from right coronary artery or single right coronary artery (n = 22), right coronary artery from left coronary artery or left anterior descending coronary artery (n = 8), and large right coronary artery conal branch (n = 6). Diagnosis was established before the operation in 25 of 36 cases by angiography (n = 24) or echocardiography (n = 1). The approach was successful in 34 cases, in 25 of which placement of a limited transannular patch was necessary. Two patients had a right ventricle-pulmonary artery conduit as a result of proximity of the coronary branch to the pulmonary arterial anulus and inability to adequately relieve the right ventricular outflow tract obstruction. RESULTS: There have been no early or late deaths. Mean right ventricle-pulmonary artery gradient at last follow-up was 19 mm Hg (95% confidence interval 14.5-24 mm Hg), compared with 15 mm Hg (95% confidence interval 12.5-17.5 mm Hg) for patients with normal coronary arteries (P = .3). Actuarial freedom from reoperation at 120 months was 96.5% (95% confidence interval 79.8%-99.5%) and was also similar between patients with and without coronary artery abnormalities (P = .92). CONCLUSIONS: Surgically important coronary anomalies in tetralogy of Fallot can be dealt with through the transatrial-transpulmonary approach in most cases without major alterations in technique. Outcomes are similar to those of other patients with tetralogy of Fallot. The presence of anomalous coronary arteries does not impart incremental risk after this surgical strategy.

Angiographic and electron-beam computed tomography studies of retrograde cardioplegia via the coronary sinus.

Retroperfusion of the coronary sinus does not provide homogeneous distribution of cardioplegic solution. The goal of this study was to analyze the distribution of flow during retrograde cardioplegic infusion in cadaveric human hearts with two different techniques of coronary sinus cannulation: (1) internal occlusion of the coronary sinus by balloon inflation and (2) external occlusion by tightening the orifice of the coronary sinus around a simple catheter. To evaluate differences between the two techniques, angiographic and electron-beam computed tomographic studies were performed. Computed digital angiography was performed on 14 hearts. Angiographic patterns varied according to type of coronary sinus cannulation. With the balloon inflation technique, the marginal vein and the anterior descending vein were perfused first; the posterior descending vein was not perfused. This vein was opacified secondarily through a venovenous anastomosis located at the apex of the heart. Backward flow into the right atrium (steal phenomenon) was demonstrated. At completion of retroperfusion, the inferior part of the septum remained poorly opacified. Conversely, angiographic findings after external occlusion of the coronary sinus revealed simultaneous injection of all venous channels. The entire septum was well opacified at completion of retroperfusion. Electron-beam computed tomographic study was performed on eight hearts with the external occlusion technique and nine with the internal occlusion technique. The computed tomographic findings confirmed the results of digital angiography. The peak myocardial enhancement and the peak rising rate of myocardial enhancement within the interventricular septum were significantly more important (p < 0.0001) when the external coronary sinus occlusion mode was used than when the internal coronary sinus occlusion mode was used. In all hearts except one, the right ventricular wall was not opacified, regardless of the type of cannulation and the type of radiologic analysis. This study demonstrates the importance of coronary sinus cannulation technique in optimizing the protection of the interventricular septum with retrograde cardioplegic infusion.

Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery.

OBJECTIVE: The aim of this study was to review the results of operations for anomalous left coronary artery from the pulmonary artery and the late outcome for exercise capacity, left ventricular function, and mitral regurgitation. METHODS: Twenty-one patients underwent operations over an 18-year period (median age, 9 months; range, 6 weeks-26 years) with a median follow-up of 6.5 years (range, 2 months-18 years). In addition to clinical and echocardiographic follow-up, patients at our institution were also investigated with radionuclide scans (n = 10) and treadmill exercise testing (n = 8). RESULTS: There were no operative or late deaths (0%; 95% confidence interval [CI], 0% and 16%). Five patients required support with a left ventricular assist device. Eighteen patients are currently in New York Heart Association class I, and 3 patients are mildly symptomatic. On nuclear gated scan at a mean of 6 years after the operation, the left ventricular ejection fraction was 64% (SD, 3%) at rest and increased to 74% (SD = 3%) on exercise (95% CI for the difference, 6%, 14%; P =.001). Treadmill endurance was normal for age (9.8-14.5 minutes) in those old enough to exercise. On echocardiography (n = 18), the current fractional shortening was 34% (SD, 4%) in the 15 patients with normal or only mildly abnormal ventricular septal motion. Three patients have undergone mitral valve operations. The left ventricular end-diastolic dimension fell from 48 mm (SD, 5.8 mm) before surgery to 35.1 mm (SD, 5.2 mm) at 1 year after the operation, and the fractional shortening increased over the first year from 19.6% (SD, 9.3%) to 32.8% (SD, 5.9%; both P <.001). CONCLUSIONS: Long-term clinical outcome and left ventricular function are good, despite severe left ventricular dysfunction at presentation.

Model of complete separation of the hepatic veins from the systemic venous system.

BACKGROUND: In patients undergoing a Fontan operation, partial diversion of the hepatic veins to the pulmonary venous atrium has been tried with various techniques. They failed because of the development of intrahepatic collaterals leading to an unacceptable right-to-left shunting. We postulate that to avoid the formation of intrahepatic collaterals, the totality of the liver has to be drained into the same pressure compartment. We have designed a model of cavopulmonary anastomosis in which a prosthetic conduit reproduces an azygos continuation, associated with the diversion of the totality of the hepatic venous return. This article reports on the early hemodynamics and the fate of the separation of the two venous compartments in long-term survivors. METHODS: Eighteen goats were operated on; the pulmonary artery and hepatic vein pressures were recorded. During month 2, an opacification of the inferior vena cava and the cavopulmonary connection was performed. Between months 6 and 14, another opacification was performed, together with pressure recording at both ends of the conduit. RESULTS: Postoperatively the pulmonary artery pressure was pulsatile with a mean of 10 mm Hg and the hepatic vein pressure was 0 mm Hg. The first angiogram showed patent tubes with fast progression of the contrast. Throughout the inferior vena cava injection, there was no opacification of the portal or hepatic veins. The late study showed a narrowed conduit in all animals. During the injection, a collateral was injected, feeding into the inferior mesenteric vein. No collateral circulation could be seen draining directly into the liver. The median gradient between the two ends of the conduit was 11 mm Hg. CONCLUSIONS: The isolation of the entire hepatic venous drainage is feasible and efficient for the separation of two pressure compartments. No intrahepatic collaterals are observed with this model at short- or long-term follow-up. The separation of the hepatic venous drainage should persist without collateral circulation as long as the inferior vena cava pressure stays at the levels observed in Fontan circulation.

The surgical importance of coronary sinus orifice atresia.

Coronary sinus orifice atresia is rare. We describe two cases, one with an atrioventricular septal defect and another with supracardiac totally anomalous pulmonary venous drainage. The association with the latter has not been described previously. The importance of diagnosing the defect is emphasized and surgical treatment is discussed.

Downsizing of valve allografts for use as right heart conduits.

In recent years, there has been a worsening shortage of small and intermediate-sized aortic and pulmonary allografts for use as right ventricular-to-pulmonary artery conduits in infants and children. However, there is a surfeit of large pulmonary and aortic allografts from adult donors. The feasibility of reducing a large allograft to a more useful size was examined using human pulmonary and aortic allografts. Eleven pulmonary allografts (11 to 26 mm in diameter) and nine aortic allografts (5 to 27 mm in diameter) were studied. Valve competence before downsizing was tested with a column of saline to a static pressure equivalent to the normal pulmonary pressure (20 mm Hg). Regurgitant flow was measured for 15 minutes. One complete cusp of the valve was excised, together with a longitudinal strip of the arterial wall. A bicuspid valved conduit was created by suturing the allograft longitudinally. The diameter of the bicuspid valve was measured with a dilator. A nomogram was constructed that predicts the size of the bicuspid allograft based on the size of the original allograft. The competence of the bicuspid allograft was tested and the regurgitant flow was compared with that of the original tricuspid allograft. The transvalvular systolic pressure gradient was measured with the bicuspid allograft placed in a pulsatile extracorporeal perfusion circuit at a flow rate of 1 L/min and a mean pressure of 20.5 +/- 2.6 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Senning plus arterial switch operation for discordant (congenitally corrected) transposition.

BACKGROUND: Congenitally corrected transposition of the great arteries is a complex cardiac lesion, usually associated with ventricular septal defect, left ventricular outflow tract obstruction, and tricuspid valve abnormalities. A subset of patients without left ventricular outflow tract obstruction have undergone Senning plus arterial switch operation in an attempt to place the left ventricle in the systemic circuit and the right ventricle in the pulmonary circuit. METHODS: Fourteen patients have had the operation performed since July 1989. Age and weight medians were 12 months (range, 0.5 to 120 months) and 8.2 kg (range, 3.2 to 34 kg). All but 1 patient had a left ventricular to right ventricular pressure ratio greater than 0.7, due to a large ventricular septal defect (with or without a previous pulmonary artery band), severe congestive heart failure caused by right ventricular dysfunction and tricuspid insufficiency, or a pulmonary artery band for left ventricular retraining. At least 10 patients had strong contraindications to "classic" repair, including right ventricular hypoplasia (n = 2), moderate to severe right ventricular dysfunction (n = 5), or moderate to severe tricuspid insufficiency (n = 9). RESULTS: There was one hospital death, occurring in a neonate (7%; 95% confidence interval = 0% to 34%). Actuarial survival beyond 10 months is 81% (95% confidence interval = 42% to 95%), currently with 389 patient-months of total follow-up time. The median grade of tricuspid insufficiency fell from 3/4 preoperatively to 1/4 postoperatively (p = 0.003). Right ventricular function is normal in 11/12 current survivors, all but 1 of whom are in New York Heart Association class I or II. CONCLUSIONS: Senning plus arterial switch operation is a good option for selected patients with congenitally corrected transposition of the great arteries with a similar or lower early risk (as compared with classic repairs). Some of the long-term problems associated with congenitally corrected transposition of the great arteries may be avoided with this strategy.

Damus-Kaye-Stansel connections in children with previously transected pulmonary arteries.

BACKGROUND: In patients with a univentricular arteriovenous connection, transection of the main pulmonary artery may be performed as part of a bidirectional cavopulmonary shunt or Fontan procedure. The proximal stump of the pulmonary artery may remain in the systemic circulation. In cases with a discordant ventriculoarterial connection, subsequent restriction of the bulboventricular foramen may lead to subaortic stenosis. The subaortic stenosis can be corrected in some patients by directing the systemic flow through a combined nonobstructed aortopulmonary outlet, as in the Damus-Kaye-Stansel connection. Previous closure of the pulmonary artery has been considered by some investigators to be a relative contraindication to the Damus-Kaye-Stansel procedure, unless an allograft root can be added to the circuit after excision of the closed pulmonary stump. METHODS: Three patients with previously transected pulmonary arteries underwent a modified Damus-Kaye-Stansel connection using the native pulmonary valve and the proximal pulmonary artery stump. RESULTS: The native pulmonary valves have functioned well despite thrombus formation in the proximal stump in 2 patients before Damus conversion. All 3 patients are alive and well after 108, 19, and 3 months, with competent nonobstructed ventriculoarterial connections. CONCLUSIONS: If transection and closure of the pulmonary artery as part of a previous palliation has spared the pulmonary valve, then the native pulmonary outlet might be used for a safe Damus-Kaye-Stansel connection.

Does left ventricular outflow tract obstruction influence outcome of interrupted aortic arch repair?

BACKGROUND: In previous studies left ventricular outflow tract obstruction (LVOTO) has been recognized as an important factor affecting survival and reoperation probability in patients having repair of an interrupted aortic arch (IAA). METHODS: All 72 patients who underwent operation for IAA from January 1, 1985 to June 30, 1997 were reviewed. The presence or absence of LVOTO was noted and the immediate and long-term results were analyzed. RESULTS: Type A IAA was found in 23 patients and type B IAA in 49 patients. Anomalous right subclavian artery was noted in 15 patients, all of whom had type B IAA. LVOTO was identified in 36 patients before arch repair and was associated with the presence of type B IAA and anomalous right subclavian artery (p = 0.02 and 0.007, respectively). There were 2 hospital deaths (within 30 days) for a mortality of 2.8% (confidence limit [CL] = 0% to 6.6%). There were 7 late deaths over 3,737 patient-months of follow-up (9.7%, CL = 2.9% to 16.6%). Actuarial survival for the whole cohort was 84.8% (CL = 73.2% to 94.4%) at 12 years. There was 87% 10-year survival (CL = 60% to 93%) for patients with LVOTO at presentation compared with 83% (CL = 62% to 92%) for patients without LVOTO (p = 0.85, hazard function 0.87). Twenty-eight patients have required at least one reoperation. The use of conduits to restore right ventricular to pulmonary artery continuity predicted the need for reoperation (p = 0.0001). Patients with presence of a nonseptatable heart were also more likely to need reoperation (p = 0.027) when compared to the rest of the cohort. Freedom from reoperation was 47.3% (CL = 30% to 62%) at 12 years. In patients with LVOTO, freedom from reoperation (55% at 10 years, CL = 33% to 72%) was not significantly different from those patients without LVOTO at presentation (29% at 10 years, CL = 7% to 56%; p = 0.97, hazard function 0.7). Actuarial freedom from recurrent arch obstruction requiring reintervention was 82.7% (CL = 66.7% to 98.7%) at 12 years. CONCLUSIONS: Neonatal complete repair of patients with IAA is possible with low mortality. LVOTO was not a significant risk factor for hospital or late death in patients having complete repair of IAA during this period. The use of conduits for right ventricular to pulmonary artery continuity and the presence of a nonseptatable heart are important risk factors for further operation and will continue to provide added morbidity to these patients.

Cardiac hemangiomas.

Cardiac hemangiomas are rare benign vascular tumors of the heart. To date, 23 cases have been reported. Symptoms are usually due to compression of cardiac structures or obstruction of outflow tracts. Echocardiography usually directs the diagnosis toward a cardiac mass. Enhanced-contrast computed tomographic scan or magnetic resonance imaging establishes the diagnosis of hypervascularized cardiac tumor. Coronary arteriography establishes the diagnosis by showing the characteristic tumor blush. Surgical resection is done using cardiopulmonary bypass. Long-term outcome is favorable.

Arterial switch in hearts with left ventricular outflow and pulmonary valve abnormalities.

BACKGROUND: Pulmonary valve and left ventricular outflow tract abnormalities (LVOT) may not be absolute contraindications to arterial switch operation (ASO). METHODS: In this study we analyze long-term outcome for 26 such transposition patients (6.3% of our ASO cohort). Median age and weight were 69 days (7 to 3,631 days) and 4.5 kg (2.6 to 34 kg). Pulmonary valve abnormalities included bicuspid valve (n = 4) and dysplastic valve (n = 5). The LVOT abnormalities (n = 17) included accessory atrioventricular valve/endocardial cushion tissue, fibromuscular ring, anomalous muscle bands, and septal malalignment. Patients with dynamic LVOT obstruction were excluded. The median preoperative left ventricular to pulmonary artery peak systolic pressure gradient was 30 mm (0 to 93 mm), or 50 mm (16 to 93 mm) if patients with isolated valve abnormalities are excluded. The ASO was performed according to our standard technique with or without LVOT resection or pulmonary valvotomy as required. RESULTS: There were two perioperative deaths (7.7%; 95% confidence interval, 0.9% to 25%), and no late deaths during 1,934 patient-months of follow-up time. Actuarial freedom from reoperation for neoaortic valve or LVOT problems is 87% (+/- 7) at 130 months, representing two reoperations. One was performed for neoaortic insufficiency plus LVOT obstruction, and the other for isolated LVOT obstruction. One patient currently has significant neoaortic insufficiency, and median gradient at last follow-up is 0 mm Hg (range, 0 to 35 mm Hg). CONCLUSIONS: The ASO can be performed in selected patients with transposition of the great arteries and with LVOT abnormalities with early and late survival and functional status similar to that of matched patients with normal pulmonary valves and LVOT (p > 0.05), but with a greater hazard for reoperation (p < 0.05). Selection for ASO should be based on anatomic criteria rather than left ventricular to pulmonary artery gradient alone, to avoid assigning these patients with transposition of the great arteries to treatment strategies less satisfactory than ASO.

IL-6 and IL-8 levels after cardiopulmonary bypass are not affected by surface coating.

UNLABELLED: BACKGROUND.:Contact of blood with the surfaces of the cardiopulmonary bypass (CPB) circuit has been implicated as a cause of the inflammatory response. We undertook a prospective randomized trial of 200 pediatric patients, all with a calculated total bypass flow of less than 2.3 L/min (< 0.96 L/m2/min). METHODS: Patients were randomly assigned to 1 of 4 CPB groups: (1) Nonheparin-bonded circuit with no albumin preprime; (2) Nonheparin-bonded circuit with albumin preprime; (3) Heparin-bonded circuit with no albumin preprime; (4) Heparin-bonded circuit with albumin preprime. Measurements of cytokines, (interleukin [IL]-6, IL-8) and blood cell counts were made prebypass and 6 and 24 hours after institution of cardiopulmonary bypass. RESULTS: Analysis of variance showed no significant difference in any of the clinical or biochemical characteristics of the 4 groups. The interaction between heparin-bonded oxygenators and albumin preprime was not significant. No important differences in IL-6 or IL-8 concentrations were noted after CPB using either heparin or nonheparin-bonded oxygenators with albumin or albumin free preprime using two-way analysis of variance. CONCLUSIONS: Albumin preprime and heparin-bonding do not attenuate the inflammatory response component attributable to the concentration of these markers.