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.     

Subpulmonary obstruction in congenitally corrected transposition of the great arteries due to ventricular membranous septal aneurysms

The clinical, hemodynamic, and angiographic observations, as well as the surgical approach used for repair in three patients with congenitally corrected transposition of the great arteries and ventricular membranous septal aneurysms, are presented. In two of the three patients the membranous septal aneurysm caused subpulmonary obstruction, with 94 and 125 mm Hg systolic gradients. In each patient the aneurysm was demonstrated by angiocardiography, which also showed differences in size and shape with cardiac systole and diastole. Review of the previously described reports indicates that patients with congenitally corrected transposition often display various forms of pulmonary outflow obstruction and when a ventricular membranous septal aneurysm exists, a significant subpulmonary obstruction is present in most patients. The unique anatomic relationship between the pulmonary artery and a ventricular membranous septal aneurysm in patients with transposition of the great arteries with and without atrioventricular discordance explains why subpulmonary obstruction sometimes develops.     

Congenitally corrected transposition with atrioventricular septal defect

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement . All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ventriculography. In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the posterior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventricular valves.     

Congenitally corrected transposition of the great arteries: morphologic study of 32 cases

The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries.Tis condition is defined as the combination of atrioventricular (A-V) discordance and transposition of the great arteries. Examples of primitive (single) ventricle with "inverted" (that is, left-sided in situs solitus) outlet chamber are excluded. Six hearts with A-V discordance and pulmonary atresia are described in an appendix. In 29 cases of corrected transposition the heart was in situs solitus; in 3 it was in situs inversus totalis. Only 5 of these 32 hearts had no potential for intracardiac shunting. Anomalies of the tricuspid valve (91 percent of cases), ventricular septal defect (78 percent) and pulmonary outflow tract obstruction (44 percent) occurred with sufficient frequency to be considered part of the basic malformation and are described in detail. The precise anatomy and disposition of the A-V valve tension apparatus, the coronary arteries and the conducting tissues are described with special reference to possible surgical approaches for repair of the anomalies. In two hearts with situs solitus the aortic valve was right-sided with respect to the pulmonary valve. This finding is important for both diagnosis and nomenclature.     

Modulation of carotid baroreflex responsiveness in man: effects of posture and propranolol

Complete atrioventricular block proximal to the bundle of His in a patient with congenitally corrected transposition of the great vessels was documented using His bundle electrograms. The spontaneous rhythnm probably originated from the bundle of His and was responsive to carotid sinus massage, atropine and isometric and treadmill exercise. These electrophysiologic observations are consistent with recent anatomic studies of congenitally corrected transposition of the great vessels.     

Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure

BACKGROUND: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (réparation à l'étage ventriculaire) is possible without interposition of a conduit between the right ventricle and pulmonary artery. However, the anterior location of the pulmonary arteries after the Lecompte maneuver may be a potential cause for right ventricular outflow obstruction, which continues to be reported in 5% to 25% of cases. We have used a tubular segment of aortic autograft to connect the pulmonary artery, left in the orthotopic posterior position (without the Lecompte maneuver), to the right ventricle in 10 consecutive patients with transposition, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Ten consecutive patients aged 2 months to 11 years (mean 32 months) have undergone a modified Lecompte operation. Eight had severe pulmonary stenosis, two had pulmonary atresia, and four had a restrictive ventricular septal defect at the time of the operation. Two had multiple ventricular septal defects. Seven had undergone one (n = 5) or two (n = 2) previous modified Blalock-Taussig shunts. All patients underwent a total correction with left ventricular-aortic intraventricular connection (four needed a ventricular septal defect enlargement), connection between the right ventricle and pulmonary arteries with a tubular segment of autograft aorta, without the Lecompte maneuver (anterior location of the bifurcation of the pulmonary arteries) on the right (n = 6) or the left (n = 4) of the aorta. No valvular device was used for the right ventricular outflow repair. RESULTS: No early or late deaths occurred. One patient with multiple ventricular septal defects needed an early (2 weeks) reoperation for a residual muscular ventricular septal defect. All patients are currently in New York Heart. Association class I, without medications, in sinus rhythm, at a mean follow-up of 30 months. Late results up to 3.6 years show no calcification on the chest roentgenogram, and at the most recent echocardiogram, right ventricular pressures were low (25 to 40 mm Hg, mean 33 mm Hg) and no significant gradient (over 10 mm Hg) was found between the right ventricle and pulmonary arteries. Left and right ventricular function was satisfactory. CONCLUSION: This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year).     

Cancer: a response to prolonged tissue necrosis

We report the first case published in Spain of a palliative anatomic correction associated with aortic arch repair in a neonate with (S,D,L)-transposition of the great arteries, multiple ventricular septal defects, and severe hypoplasia of the right ventricle with subaortic obstruction and hypoplasic aortic arch with coarctation. A one stage palliative surgery on cardiopulmonary bypass was performed with reconstruction of the aortic arch and an arterial switch procedure which obtained a satisfactory result. The principle of this operation is to switch the subaortic obstruction into a subpulmonary obstruction and reconstruct a large natural aortic root from the principal ventricle. The right ventricle-pulmonary artery continuity may promote growth of the right ventricle with the possibility of a future biventricular repair. We conclude that this operation, when used by surgical teams experienced with arterial switch surgery, is the best treatment for the complex newborn group with single ventricles or severe ventricular disbalance, ventriculoarterial discordance and stablished subaortic stenosis.     

One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals

BACKGROUND: Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have traditionally required multiple unifocalization staging operations before undergoing complete repair. Recently, the feasibility of a single-stage unifocalization and repair was demonstrated by Hanley. In this report, we describe our experience with each approach. METHODS AND RESULTS: Since 1989, 11 of 12 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone complete surgical correction. The first seven patients were subjected to staged bilateral unifocalizations, with repair being achieved in six (group I). The last five patients have undergone a single-stage midline unifocalization and repair via a sternotomy (group II). Four of these were infants (2 weeks to 9 months) and one was 13 years old. All patients in group I had tetralogy of Fallot, whereas in group II three patients had tetralogy of Fallot, one patient had double-outlet right ventricle, and one patient had complete atrioventricular canal and transposition. In group I, the median age at the first operation was 43 weeks. Complete repair was performed at a median age of 3.5 years, with a mean number of 3.3 operations required. In group II, only one operation was required to achieve complete repair at a median age of 28 weeks. The postoperative right ventricular/left ventricular pressure ratio was 0.49 in group I and 0.45 in group II. One intraoperative death and one late death occurred in group I and no early or late deaths in group II. Currently, four patients in group I and all five patients in group II are alive and well. CONCLUSIONS: Early intervention with both surgical approaches can lead to complete biventricular repair in most patients. Because the single-stage midline unifocalization and repair can achieve a completely repaired heart in infancy with one operation, it is currently our approach of choice.     

Rastelli's procedure

In 1969, Rastelli conceived a new technique to repair of transposition of the great arteries in the presence of ventricular septal defect and severe pulmonary stenosis (TGA III), based on the redirection of ventricular outflows. An intracardiac tunnel connected the left ventricle to the aorta and an external valved conduit established continuity between the right ventricle and the pulmonary artery. TGA III and truncus arteriosus are underwent a Rastelli operation. The present report is an analysis of indication, operative technique, early and late results with this procedure. Early deaths were related to unfavourable anatomy, conduit compression and sepsis. Residual VSD and postoperative infection were the main factors contributing to the late deaths. A current Re-Rastelli operation for the problems of extracardiac valved conduit is a good result.     

Anatomic features and surgical strategies in double-outlet right ventricle

BACKGROUND: The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality. METHODS AND RESULTS: Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years. CONCLUSIONS: Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.     

Double switch operation in cardiac anomalies with atrioventricular and ventriculoarterial discordance

Since June 1987, 10 of 19 consecutive patients with atrioventricular and ventriculoarterial discordance (average age 4 +/- 2 years) had undergone a double switch operation with the morphologically left ventricle used as a systemic ventricle. There were two combinations of procedures. Atrial switch combined with arterial switch was used in two patients who had a normal pulmonary valve. Atrial switch combined with ventriculoarterial switch by Rastelli's procedure was used in eight patients with pulmonary stenosis or atresia and a large ventricular septal defect. One early death and two late deaths have occurred in a postoperative follow-up period of up to 4 years. Subsequent problems were mainly related to the results of atrial switch procedures in patients who had a small atrium because of low pulmonary flow, especially in patients with apicocaval juxtaposition. Our experience suggested that the double switch operation would open a new era of definitive surgical treatment in half of the patients with atrioventricular and ventriculoarterial discordance.     

The role of a bidirectional cavopulmonary anastomosis in the correction and palliation of complex congenital cardiopathies

Between May 1990 and January 1998, 68 patients underwent bidirectional cavo-pulmonary anastomosis. We evaluated all patients in whom the bidirectional cavo-pulmonary anastomosis was associated with additional pulmonary flow (group A) and those in whom it was associated with biventricular repair (group B). Group A included 23 patients (33.8%), 14 males and 9 females, mean age 25 years and 6 months (range 4 months-16 years). Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). Group B included 13 patients (19.1%), 6 males and 7 females, mean age 13 years and 7 months (range 1 year-37 years). Diagnoses were pulmonary atresia with intact ventricular septum (4), Ebstein's anomaly (3), tetralogy of Fallot (3), atrioventricular canal (1), hypoplastic right ventricle (1), and pulmonary and tricuspid insufficiency (1). Four patients (17.3%) in group A died in the postoperative period, whereas there was no postoperative mortality in group B. Follow-up data were available in 31 patients (19 from group A, 13 from group B). Mean follow-up was 1 year and 6 months (range 30 days to 6 years). Evaluation was done by NYHA class functional status. In group A, 14 patients are doing well (NYHA I or II), while five patients (26.3%) underwent Fontan operation with one death. All group B patients are currently doing well (NYHA class I or II). In group A, complications were pericardial effusion (7), transient superior vena cava syndrome (5), pleural effusion (4), chylothorax (1) and rhythm disturbance (1). Complications in group B involved neurological events (2), pleural effusion (1) and rhythm disturbance (1). Bidirectional cavo-pulmonary anastomosis can be associated with additional pulmonary flow with good short- and intermediate-term outcome. Concern remains for the ability to properly regulate the amount of effective pulmonary blood flow. Bidirectional cavo-pulmonary anastomosis can be associated with biventricular repair in patients with diminutive right ventricles, amenable to anatomic complete correction, with good clinical outcome.     

Cardiac surgery for grown-up congenital heart patients: survey of 307 consecutive operations from 1991 to 1994

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 +/- 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 +/- 17 vs 11 +/- 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.     

Outcome after operations for pulmonary atresia with intact ventricular septum

OBJECTIVE:Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries. METHODS: A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998. Patients were categorized on the basis of initial surgical strategy: strategy A, aortopulmonary shunt alone (n = 31); strategy B, right ventricular recruitment (n = 32); strategy C, heart transplantation (n = 4). Tricuspid valve size (Z-score) and coronary anatomy were determined. Right ventricular-coronary artery dependency was noted in 8 patients. RESULTS: Overall actuarial survivals at 1, 5, and 8 years were 82%, 76%, and 76%. Mortality was highest in infancy (10 of 16 deaths). Outcome was equivalent for all 3 strategies. There was no difference in tricuspid valve size between survivors and nonsurvivors (mean Z-score -2.0 (2.5) vs -2.0 (1.9), P =.83). There was no difference in survival based on severity of coronary abnormality. Only one third of patients had a successful biventricular repair, and the tricuspid valve was significantly larger in these than in patients who had Fontan operation (mean Z-score -0.53 [1.6], range -3.5 to 1, versus mean Z-score -3.03 [2.7], range -5.5 to 0, P =.002). CONCLUSIONS: Surgical outcome for patients born with pulmonary atresia with intact ventricular septum is satisfactory. The strategies of biventricular repair, single ventricle palliation, and heart transplantation allow for equal outcome among all anatomic subtypes.     

Repair of interrupted aortic arch: a ten-year experience

Eighty-two consecutive patients with interrupted aortic arch were referred to our institution between 1985 and 1995. Three died before any attempt at operation and 79 underwent surgical repair. Median age at operation was 9 days (range 1 day to 6 years) and median weight was 3.0 kg (range 1.8 to 20 kg). All but one were in severe congestive heart failure and 31.5% had oliguria or anuria. Preoperative pH varied between 6.8 and 7.4 (median 7.3). Sixty-nine received prostaglandin E1 infusion and 54 received mechanical ventilation. Aggressive preoperative ressucitation was necessary in 43 cases. Preoperative transfontanellar echography (performed routinely) since 1987 revealed intracerebral bleeding in six patients. Type A interrupted aortic arch was present in 37 cases, 41 patients had type B, and one had type C. Interrupted aortic arch was associated with single ventricular septal defect in 35 cases, 24 patients had associated complex heart defects, and 30 had significant subaortic stenosis (six had both subaortic stenosis and complex association). Aortopulmonary window was found in four patients, truncus arteriosus was found in eight, and transposition of the great arteries was found in five, double-outlet right ventricle was found in one, single ventricle was found in three, multiple ventricular septal defects were found in two and superior-inferior ventricles were found in one. Sixty-four patients underwent single-stage repair and 15 underwent multistage repair. Aortic arch repair consisted of direct anastomosis in 59 cases, patch augmentation in eight, and conduit interposition in 12. Ten patients underwent associated pulmonary artery banding and 19 underwent concomitant repair of complex associated lesions. The subaortic stenosis was addressed by four surgical techniques: myotomy or myectomy in five patients; creation of a double-outlet left ventricle, aortopulmonary anastomosis, and conduit insertion between the right ventricle and pulmonary artery bifurcation in four; no direct attempt to relieve the subaortic stenosis in six; and left-sided ventricular septal defect patch in 15. Mean duration of deep hypothermic circulatory arrest, crossclamp time, and cardiopulmonary bypass time were 38.8 +/- 15.6 min, 60.5 +/- 24.7 min, and 143 +/- 40.1 min, respectively. Postoperative mortality rate was 18.9% (70% confidence limits 14% to 24.6%), and overall mortality rate was 31% (70% confidence limits 20.9% to 42.2%). The results have improved with time, with an overall operative mortality rate of 12% since 1990. Univariate statistical analysis revealed that early survival was influenced by preoperative renal function, detection of cerebral bleeding by transfontanellar echography, the number of cardioplegic injections, and the date of operation. Multivariate analysis revealed that preoperative renal function and the number of cardioplegic injections were independent risk factors for early mortality. Echocardiographic measurements of the left heart-aorta complex with preoperative Z values as low as-4 demonstrated rapid growth after repair. In the presence of subaortic stenosis, better survival was obtained with a left-sided patch for ventricular septal defect closure (p < 0.05). Twenty-three patients underwent 26 reoperations for recoarctations (seven), left bronchial compression (two), second-stage repair (eight), right ventricle-pulmonary artery conduit replacement (three), and miscellaneous (four). One of the survivors was reoperated on for subaortic membrane. Survival at 5 years for the entire series was 70%. For isolated forms, it was 73.5% (90% for 1990 to 1995), for complex forms it was 70%, and in the presence of subaortic stenosis it was 60%. In conclusion, interrupted aortic arch remains a surgical challenge with continually improving results. Early diagnosis with preoperative resuscitation and adequate myocardial protection seem extremely important for further improvements. Associated subaortic stenosis or complex lesions     

The conversion of a modified Fontan procedure to a total extracardiac cavo-pulmonary conduit. The Medico-Surgical Cardiology Group

BACKGROUND: After a modified Fontan procedure with atriopulmonary or atrioventricular conduit, some patients present stress intolerance, supraventricular arrhythmia, recurrent pleuropericardial or ascitic effusions, and protein-losing enteropathy, all of which are signs that the previous procedure has failed. The aim of this study was to evaluate the midterm outcome after surgical therapy for this condition. MATERIAL AND METHODS: Between August 1994 and December 1997, nine patients (6 males and 3 females), age 10 to 39 (mean 21.5) years, underwent conversion of previous modified Fontan procedure to total extracardiac cavo-pulmonary connection. Time from the previous procedure was 6 to 18 years (mean 10). Diagnosis was tricuspid atresia with pulmonary stenosis (n = 2), double-inlet left ventricle and concordant ventriculoarterial connection (n = 3), double-inlet left ventricle and discordant ventriculoarterial connection (n = 3), Holmes heart (n = 1). Nine patients presented decreased stress tolerance, seven had arrhythmia, five had pleuropericardial effusions and two had protein-losing enteropathy. In all but one patient, right atrial pressure was higher than 15 mmHg, while in six patients the cardiac index was less than 2 l/min/m2. A polytetrafluoroethylene non-valved conduit was interposed between the inferior vena cava and the right pulmonary artery for conversion in all patients. A bidirectional cavo-pulmonary anastomosis (modified Glenn) was associated in all patients. Evaluation was done by NYHA Class and by an arbitrary score assigned to patients based on 7 parameters. RESULTS: There was no perioperative mortality. All patients were clinically improved at a mean follow-up of 24 months (range: 3 to 46). No patient had effusions, and the arrhythmias disappeared in 4 patients and were controlled by medical therapy in one. The two patients with protein-losing enteropathy improved markedly within 30 days and the score dropped below 10 points. CONCLUSIONS: The conversion of the modified Fontan procedure to total extracardiac cavo-pulmonary connection improves clinical condition by decreasing the right atrium-pulmonary gradient and right atrial preload, and by providing a laminar cavo-pulmonary flow without any need for intracardiac anastomoses. This procedure should be undertaken early in this subset of patients, before ventricular failure ensues.     

Small intestinal infusion studies in the calf

In this series of 13 patients undergoing repair of transposition of the great arteries with ventricular septal defect and pulmonary atresia, the operative risk and postoperative complications were greater than for repair of either transposition of the great arteries with ventricular septal defect and pulmonary stenosis or pulmonary arterial atresia with ventricular defect. Nevertheless, 6 of the 8 survivors improved clinically. Because operative and late mortality and morbidity rates are related to persistent right ventricular hypertension caused primarily by restricted pulmonary arterial outflow, results should be improved by performance of a preliminary systemic-pulmonary artery shunt for patients with hypoplastic pulmonary arteries and by use of the Hancock prosthesis, which has yielded lower gradients at both the proximal and distal anastomoses of the conduit. These improvements and the relief from cyanosis, exercise intolerance, and other symptoms seem to justify the continued application of surgical correction of transposition of the great arteries when associated with pulmonary atresia.     

A rapid, specific membrane filtration procedure for enumeration of enterococci in recreational water

BACKGROUND: Perioperative and long-term problems associated with the Fontan circulation are substantial. There has been an exploration of extending the limits of a biventricular ventricular repair by using a superior vena cava-to-pulmonary artery anastomosis. This type of repair is known as a 1 1/2 ventricle repair. METHODS: Patients having defects of the pulmonary ventricle in size or function have undergone 1 1/2 ventricle repairs with or without creation of an atrial septal defect. Repairs with tricuspid z values as small as -10 and predicted pulmonary ventricular volumes as low as 30% have been reported. The 1 1/2 ventricle repair technique has also been used in special situations associated with an arterial switch or double switch procedure. RESULTS: Mortality has ranged from 0% to 12%. Complications have included persistent elevation of superior vena cava pressure, intermittent periorbital edema, and 1 superior vena caval aneurysm requiring takedown. There appears to be an increased risk of perioperative pleural effusions and chylothorax. Protein-losing enteropathy and chronic atrial arrhythmias have not been present. CONCLUSIONS: Successful 1 1/2 ventricle repairs have been reported for morphologically small or poorly functioning pulmonary ventricles and special situations. Intermediate-term follow-up is favorable when compared with reported outcomes for the Fontan circulation.     

Right-sided maze procedure for right atrial arrhythmias in congenital heart disease

BACKGROUND: Atrial fibrillation and flutter, commonly associated with congenital heart anomalies that cause right atrial dilatation, may cause significant morbidity and reduction of quality of life, even after surgical repair of the anomalies. METHODS: In an effort to reduce the incidence of atrial tachyarrhythmias after repair of right-sided congenital heart disease, we performed a concomitant right-sided maze procedure. RESULTS: Eighteen patients with paroxysmal atrial fibrillation or flutter (n = 12) or chronic atrial fibrillation or flutter (n = 6) aged 10.9 to 68.4 years (mean 34.9 years) underwent a right-sided maze in association with repair of Ebstein's anomaly (n = 15), congenital tricuspid insufficiency (n = 2), and isolated atrial septal defect (n = 1). There were no early deaths, reoperations, or complete heart block. Discharge rhythm was sinus (n = 16) or junctional (n = 2). Follow-up was complete in all 18 patients and ranged from 3.1 to 17.2 months (mean 8.1 months); all are in New York Heart Association class I. Early postoperative arrhythmias developed in 3 patients (all were converted to sinus rhythm by antiarrhythmic drugs). There were no late deaths or reoperations. CONCLUSIONS: The inclusion of a right-sided maze procedure with cardiac repair in patients having congenital heart anomalies that cause right atrial dilatation and associated atrial tachyarrhythmias is effective in eliminating or reducing the incidence of those arrhythmias.     

A case report of one-stage repair for transposition of the great arteries with intact ventricular septum and coarctation of the aorta in neonate

We report a neonatal case of one-stage repair for transposition of the great arteries with intact ventricular septum (TGA with IVS) and coarctation of the aorta (CoA). The surgery was performed at 27 days of age when the patient, a male, weighed 3408 g. Preoperative cardiac catheterization and angiography confirmed the diagnosis of TGA with IVS, CoA, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava, left sided juxta-positioning of appendages and dextrocardia. The procedure was simultaneous subclavian flap aortoplasty through a left thoracotomy and an arterial switch operation through a median sternotomy. The patient's postoperative course was uneventful and he has grown well. Postoperative cardiac catheterization revealed mild pulmonary stenosis (pressure gradient of 19 mmHg between the right ventricle and the pulmonary artery) and mild aortic arch kinking from the arterial switch maneuver. One-stage repair, the combination of subclavian flap aortoplasty and arterial switch operation, provides a good clinical result in this complex malformation.