PubMed Health⌕ Search

Biomedical subjects

A R Castañeda

Publications and source records attributed to A R Castañeda.

At least 19 recordsLinked to original sources
PubMed1997

Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.

OBJECTIVES: The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. METHODS: The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. RESULTS: The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more (p < 0.001), younger age at operation (p = 0.001), heterotaxy syndrome (p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve (p = 0.001), pulmonary artery distortion (p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage (p = 0.001), the absence of a baffle fenestration (p = 0.002), and longer cardiopulmonary bypass time (p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation (p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure (p = 0.003). CONCLUSIONS: These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.

Child↗
PubMed1996

Tetralogy of Fallot with diminutive pulmonary arteries: preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries.

OBJECTIVES: This study sought to determine the results of a novel transcatheter management approach in tetralogy of Fallot with diminutive pulmonary arteries. BACKGROUND: Tetralogy of Fallot with diminutive pulmonary arteries and severe pulmonary stenosis is rare and resembles tetralogy of Fallot with pulmonary atresia: There is a high incidence of aortopulmonary collateral channels, arborization abnormalities, stenoses and need for multiple operations. Because a combined catheter-surgery approach facilitates repair in these patients, such an approach may benefit those with diminutive pulmonary arteries and pulmonary stenosis. METHODS: Clinical, catheterization and surgical data were studied retrospectively for 10 such patients undergoing preoperative pulmonary valve balloon dilation, among other transcatheter interventions, from January 1989 to January 1995. RESULTS: Initially, the Nakata index ranged from 20 to 98 mm2/m2 (mean 67 +/- 28 mm2/m2). The pulmonary valve was first balloon dilated (mean balloon/annulus 1.5 +/- 0.3), and the mean initial valve annulus Z score (-4.0 +/- 1) increased to -33 +/- 1.1 (p < 0.01) Other interventions included branch pulmonary artery balloon dilation (7 patients, 23 vessels) and coil embolization of aortopulmonary collateral channels (8 patients, 31 collateral channels). At preoperative follow-up catheterization, the mean pulmonary annulus Z score was -3.1 +/- 0.7, and the Nakata index increased to 143 +/- 84 mm2/m2 (p < 0.03). All patients underwent complete surgical repair successfully. At a mean follow-up period of 2.6 +/- 2 years, right ventricular pressure was < 70% systemic in all patients and < 50% systemic in seven. CONCLUSIONS: In patients with tetralogy of Fallot, severe pulmonary stenosis and diminutive pulmonary arteries, initial pulmonary valve balloon dilation increases the annulus Z score and anterograde pulmonary blood flow and facilities simultaneous coiling of aortopulmonary collateral channels and access for branch pulmonary artery dilation, all of which results in pulmonary artery growth, simplifying surgical management.

Abnormalities, Multiple↗
PubMed1996

Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis.

After modified Fontan procedures with atriopulmonary anastomoses or right atrium-right ventricle conduits, some patients have progressive exercise intolerance, effusions, arrhythmias, or protein-losing enteropathy. Theoretic advantages of a lateral atrial tunnel cavopulmonary anastomosis and published clinical results suggest that conversion of other Fontan procedures to the lateral atrial tunnel may afford clinical improvement for some patients. Eight patients (8 to 25 years old) with tricuspid atresia (n =4), double-inlet left ventricle (n = 3), and double-outlet right ventricle (n=1) underwent conversion to a lateral tunnel procedure between December 1990 and November 1994. An arbitrary clinical score was assigned before the lateral tunnel procedure and at follow-up. Before conversion, patients had decreased exercise tolerance (n = 8), arrhythmias (n = 6), effusions (n = 4), and protein-losing enteropathy (n = 8). At catheterization, all had a low cardiac index (1.9 +/- 0.7 L x min(-1) x M(-2), five had elevated pulmonary vascular resistance (>3 Wood units), and three had right pulmonary venous return obstruction by compression of an enlarged right atrium. Fenestrated lateral tunnel construction was undertaken 7.3 +/- 3.6 years after atriopulmonary anastomosis, with one early death related to low cardiac output. After the lateral tunnel procedure, two patients had no clinical improvement (no change in clinical score) but five patients had either marked or partial improvement. The right pulmonary vein compression present in three patients was resolved after conversion. The mean clinical scores improved from 4.5 +/- 1 to 3.0 +/- 2 (p < 0.04). In conclusion, conversion to a lateral tunnel procedure led to clinical improvement in five of eight patients at short-term follow-up and may be particularly indicated for patients with giant right atria or pulmonary vein compression who have symptoms. Pulmonary vein compression should be looked for in patients after modified Fontan procedures and can be relieved by conversion to the lateral tunnel procedure.

Adolescent↗
PubMed1995

Postoperative course and hemodynamic profile after the arterial switch operation in neonates and infants. A comparison of low-flow cardiopulmonary bypass and circulatory arrest.

BACKGROUND: The neurological morbidity associated with prolonged periods of circulatory arrest has led some cardiac surgical teams to promote continuous low-flow cardiopulmonary bypass as an alternative strategy. The nonneurological postoperative effects of both techniques have been previously studied only in a limited fashion. METHODS AND RESULTS: We compared the hemodynamic profile (cardiac index and systemic and pulmonary vascular resistances), intraoperative and postoperative fluid balance, and perioperative course after deep hypothermia and support consisting predominantly of total circulatory arrest or low-flow cardiopulmonary bypass in a randomized, single-center trial. Eligibility criteria included a diagnosis of transposition of the great arteries and a planned arterial switch operation before the age of 3 months. Of the 171 patients, 129 (66 assigned to circulatory arrest and 63 to low-flow bypass) had an intact ventricular septum and 42 (21 assigned to circulatory arrest and 21 to low-flow bypass) had an associated ventricular septal defect. There were 3 (1.8%) hospital deaths. Patients assigned to low-flow bypass had significantly greater weight gain and positive fluid balance compared with patients assigned to circulatory arrest. Despite the increased weight gain in the infants assigned to low-flow bypass, the duration of mechanical ventilation, stay in the intensive care unit, and hospital stay were similar in both groups. Hemodynamic measurements were made in 122 patients. During the first postoperative night, the cardiac index decreased (32.1 +/- 15.4%, mean +/- SD), while pulmonary and systemic vascular resistance increased. The measured cardiac index was < 2.0 L.min-1.m-2 in 23.8% of the patients, with the lowest measurement typically occurring 9 to 12 hours after surgery. Perfusion strategy assignment was not associated with postoperative hemodynamics or other nonneurological postoperative events. CONCLUSIONS: After heart surgery in neonates and infants, both low-flow bypass and circulatory arrest perfusion strategies have comparable effects on the nonneurological postoperative course and hemodynamic profile.

Cardiopulmonary Bypass↗
PubMed1995

Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries.

Between January 1983 and January 1992, 470 patients underwent an arterial switch operation at our institution. An intact (or virtually intact) ventricular septum was present in 278 of 470 (59%); a ventricular septal defect was closed in the remaining 192. Survivals at 1 month and 1, 5, and 8 years among the 470 patients were 93%, 92%, 91%, and 91%, respectively. The hazard function for death (at any time) had a rapidly declining single phase that approached zero by one year after the operation. Risk factors for death included coronary artery patterns with a retropulmonary course of the left coronary artery (two types) and a pattern in which the right coronary artery and left anterior descending arose from the anterior sinus with a posterior course of the circumflex coronary. The only procedural risk factor identified was augmentation of the aortic arch; longer duration of circulatory arrest was also a risk factor for death. Earlier date of operation was a risk factor for death, but only in the case of the senior surgeon. Reinterventions were performed to relieve right ventricular and/or pulmonary artery stenoses alone in 28 patients. The hazard function for reintervention for pulmonary artery or valve stenosis revealed an early phase that peaked at 9 months after the operation and a constant phase for the duration of follow-up. Incremental risk factors for the early phase included multiple ventricular septal defects, the rapid two-stage arterial switch, and a coronary pattern with a single ostium supplying the right coronary and left anterior descending, with a retropulmonary course of the circumflex. The need for reintervention has decreased with time. The arterial switch operation can currently be performed early in life with a low mortality risk (< 5%) and a low incidence of reintervention (< 10%) for supravalvular pulmonary stenosis. The analyses indicate that both the mortality and reintervention risks are lower in patients with less complex anatomy.

Child, Preschool↗
PubMed1995

Status of the left ventricle after arterial switch operation for transposition of the great arteries. Hemodynamic and echocardiographic evaluation.

BACKGROUND: The potential for improved preservation of systemic ventricular function represents an important reason for the increasing popularity of the arterial switch operation. In support of this expectation, prior studies in patients early after arterial switch operation have found normal ventricular contractility and function. This study was conducted to extend those observations to up to 10 years of follow-up and to directly examine the effects of a coexisting ventricular septal defect or short-term preparatory banding of the pulmonary artery before the arterial switch operation. METHODS: Patients operated on from 1983 through 1991 were included. Echocardiographic and catheterization data were collected as part of a prospective evaluation of outcome in all patients who undergo the arterial switch operation at Boston Children's Hospital, with inclusion of data from the most recent catheterization only. Echocardiograms performed at least 6 months after the operation were included, with assessment of both the most recent status as well as serial trends. Whenever possible, echocardiographic evaluation included data necessary to perform analysis of ventricular mechanics including indices of afterload, preload, and contractility. Comparison was made to normal values and between subgroups defined on the basis of an arterial switch operation with or without ventricular septal defect and those who had a rapid two-stage arterial switch operation. RESULTS: Invasive measures of left and right ventricular filling pressures, cardiac index, and pulmonary vascular resistance did not differ among the three groups. Overall, echocardiographic left ventricular end-diastolic dimension, wall thickness, mass, afterload (end-systolic wall stress), function (fractional shortening and rate-corrected velocity of fiber shortening), contractility (stress-velocity and stress-shortening relations), and preload were normal, and none of these variables was different between the groups with and without a ventricular septal defect. Serial evaluation indicated a slight but significant trend toward ventricular dilatation, perhaps related to a relatively high incidence of at least mild aortic regurgitation (30%). In contrast, in the rapid two-stage group the echocardiographic indices of left ventricular function (fractional shortening and velocity of fiber shortening) and contractility (stress-velocity and stress-shortening relations) were found to be mildly but significantly reduced compared with normal subjects and with the other arterial switch operation groups. Over the duration of follow-up encompassed by this study, no tendency toward progressive depression of function was seen. CONCLUSIONS: As the length of observation after the arterial switch operation continues to increase, left ventricular size, mass, functional status, and contractility continues to be normal, with no evidence of time-related deterioration of function. As previously reported, the rapid two-stage arterial switch operation does represent a higher risk for mild impairment of myocardial mechanics.

Aortic Valve Insufficiency↗
PubMed1994

Myocardial perfusion, function and exercise tolerance after the arterial switch operation.

OBJECTIVES: This study was conducted to determine the prevalence of myocardial perfusion abnormalities at rest and exercise and to assess exercise capacity in children after the arterial switch operation. BACKGROUND: There have been sporadic reports of myocardial ischemia or sudden death in children after the arterial switch operation for transposition of the great arteries, possibly related to inadequate coronary perfusion due to kinking or stenosis of the translocated coronary arteries. METHODS: Myocardial perfusion at rest and peak exercise was assessed using the scintigraphic agent technetium-99m methoxyisobutyl isonitrile (sestamibi). Exercise capacity was determined with a modified Bruce protocol. Ambulatory electrocardiographic (ECG) Holter monitoring was performed. Ventricular function, contractility and wall motion were assessed echocardiographically. RESULTS: Twenty-three children (aged 4.2 to 7.9 years) underwent evaluation. Abnormalities were found on the rest perfusion scans in 22 children (95.6%). The left ventricular myocardium was divided into 13 segments for analysis. Of 299 rest segments, 225 (75.3%) were normal, 11 (3.7%) showed mild defects, 45 (15%) moderate defects and 18 (6%) severe defects at rest. At peak exercise, 237 segments (79.3%) were normal, 24 (8%) showed mild defects, 33 (11%) moderate defects and 5 (1.7%) severe defects. Compared with rest studies, myocardial perfusion grade at exercise was unchanged in 246 segments (82.3%), improved in 42 (14%) and worsened in 11 (3.7%). All patients had normal exercise tolerance without symptoms or ischemic ECG changes. No ventricular tachycardia was seen on Holter monitoring. All patients had a shortening fraction > or = 27%. Left ventricular contractility was normal in 12 children in whom it was assessed. Regional wall motion was normal in 17 children with adequate echocardiographic images for this analysis. CONCLUSIONS: Myocardial perfusion scan abnormalities assessed by technetium-99m sestamibi are common after an arterial switch operation. These abnormalities are of uncertain clinical significance and generally lessen with exercise. The normal exercise tolerance without symptoms or ECG changes suggests that myocardial perfusion is adequate during the physiologic stress of exercise in children up to 8 years after an arterial switch operation.

Child, Preschool↗
PubMed1994

"Intramural" residual interventricular defects after repair of conotruncal malformations.

BACKGROUND: We report an unusual type of residual interventricular communication in patients with conotruncal malformations in which the aorta is completely or partly aligned with the right ventricle (RV). Interventricular communications after surgical repair usually result from additional defects, patch dehiscence, or incomplete closure and lie in the septal plane. However, after a right ventricular aorta is baffled to the left ventricle, the ventricular septal defect (VSD) patch and RV free wall form part of the systemic outflow tract. This "neo-left ventricular" outflow tract may provide a location for residual interventricular communications out of the septal plane. METHODS AND RESULTS: We reviewed echocardiographic, angiographic, and clinical records of patients who had one or more residual interventricular communications out of the plane of the ventricular septum after repair of a conotruncal anomaly. Between June 1990 and October 1992, we observed such defects in eight patients, 5 to 26 years old, after repair of double-outlet right ventricle (n = 6), tetralogy of Fallot (n = 1), or truncus arteriosus (n = 1). In each, the VSD patch was anchored to the RV free wall near the aortic root. Nonetheless, channels were observed around the edge of the patch, between the neo-systemic outflow tract and the right ventricle. All patients had right ventricular hypertension; in seven, the pulmonary-to-systemic flow ratio (Qp:Qs) was > or = 2. At multiple unsuccessful reoperations (two to four per patient), the patch edges appeared securely attached to myocardium. Angiographic views profiling the septum failed to localize these defects, since they are not in the native septum. Echocardiographic detection of such anterior defects can be difficult. Transcatheter umbrella closure was attempted in the seven patients with large shunts; success was limited by the multiplicity of flow channels. Umbrella closure eliminated the need for further reoperation in four of seven patients, one patient died suddenly awaiting reoperation, and two deaths followed reoperation. CONCLUSIONS: "Intramural" residual interventricular defects are difficult to diagnose by all modalities. Umbrella placement may reduce the left-to-right shunt. Successful surgical closure may require removal and reattachment of the anterior portion of the patch.

Angiocardiography↗
PubMed1994

Unique features and prognosis of primum ASD presenting in the first year of life.

BACKGROUND: Primum atrial septal defect (ASD) is a defect that is usually associated with few symptoms and can be electively repaired with good results. A review of our experience with primum ASD identified a subset of patients characterized by presentation with congestive heart failure (CHF) in the first months of life and a requirement for early operation. Echocardiograms, catheterizations, operative reports, and clinical data were reviewed and compared with that on all other patients with primum ASD. Patients with transitional or complete atrioventricular (AV) canal defects were excluded. METHODS AND RESULTS: From January 1984 to December 1992, significant CHF was present in the first year of life in 11 patients (10.5%) with primum ASD who were managed surgically among 105 total patients undergoing repair of primum ASD. This early CHF group had a higher incidence of hypoplastic left-sided cardiac structures (9 of 11 patients) compared with other primum ASD patients (5 of 94, P < .001), including patients with coarctation (CoA) (n = 9), abnormal mitral valve (n = 7), left ventricular hypoplasia (n = 5), and subaortic stenosis (subAS) (n = 7). Other differentiators from patients without early CHF included incidence of Downs syndrome (0% versus 19%), elevated pulmonary artery pressures (72% versus 33% systemic, P < .001), earlier mean age at operation (8 months versus 5.4 years), greater incidence of reoperation after initial ASD closure (5 of 8 patients, 12 operations [5 subAS, 3 mitral valve, 3 CoA, 1 pacemaker] versus 4 of 93 patients, P < .001), and higher mortality (36% versus 1%, P < .001). CONCLUSIONS: Left-sided obstructive lesions must be sought in children with primum ASD presenting with CHF in the first year of life. The presence of these lesions alters prognosis and surgical management and mandates close follow-up, with particular attention to late appearance or progression of subaortic stenosis or deterioration of mitral valve function.

Child, Preschool↗
PubMed1993

Enlarged bronchial arteries after early repair of transposition of the great arteries.

OBJECTIVES: This study was undertaken to define the incidence of enlarged bronchial arteries after early surgical repair of transposition of the great arteries by the arterial switch operation, and to report the results of catheter-directed therapy in five patients. BACKGROUND: Pathologic and angiographic studies have demonstrated enlarged bronchial arteries in patients with transposition of the great arteries. METHODS: A subjective 4-point scale was used to grade postoperative angiograms performed in 119 patients at our institution between January 1983 and December 1991. Grades 0 and 1 were designated if there was no opacification of the pulmonary arteries or veins, whereas grades 2 and 3 were assigned if there was such opacification. The median age at repair was 8 days (range 1 day to 13 months) and the median age at catheterization was 11.2 months (range 3.6 to 58.5). An intact ventricular septum was present in 84 (71%) of 119 patients. RESULTS: Significantly increased bronchial flow (grade 2 or 3) was present in 55 (46%) of 119 patients. Age at repair, age at catheterization and interval between repair and catheterization were not associated with significantly increased bronchial flow; however, an intact ventricular septum was weakly associated with increased flow (p = 0.04). Coil embolization was performed in five patients with complete occlusion of the vessels and no significant complications. CONCLUSIONS: Abnormally enlarged bronchial arteries are frequently identified at postoperative catheterization despite early repair and may explain continuous murmurs or persistent cardiomegaly in patients with otherwise normal noninvasive findings. When clinically indicated, catheter-directed therapy can be performed with good results.

Bronchial Arteries↗
PubMed1992

Age-dependent ventricular response to pressure overload. Considerations for the arterial switch operation.

Success with the arterial switch operation for D-transposition of the great arteries and the concept of left ventricular suitability for systemic work stimulated this literature review of the age-dependent mechanisms in normal cardiac growth and pressure-induced left ventricular hypertrophy. Normal postnatal myocardial growth is markedly influenced by hemodynamic factors. It consists of an early hyperplastic phase of both myocytes and capillaries that is followed by a myocyte hypertrophic phase. Similarly, imposition of a pressure overload induces both myocyte hyperplasia/hypertrophy and increased angiogenesis in neonates, but only myocyte hypertrophy at a later age. The functional consequences of ventricular hypertrophy are the result of adaptive and nonadaptive changes resulting from the overload stimulus, for example, induction of protooncogene expression, myosin isoenzyme shifts, degree of coronary perfusion, responsiveness to beta-adrenergic stimulation, and myocyte capacity to re-accumulate or sequester cytosolic calcium. Strikingly, both the capacity and the rapidity of left ventricular hypertrophy decrease with increasing age. This experimental information supports the current use of primary arterial switching for neonates with D-transposition of the great arteries and the use of "rapid" two-stage arterial switching in infants more than 3 to 4 weeks of age; it raises some concern about the practice of late retraining of the left ventricle in cases of failed atrial inversion operation.

Age Factors↗
PubMed1991

Function of the anatomic pulmonary valve in the systemic circulation.

The anatomic pulmonary valve, which has thin leaflets with little elastic tissue in the normal heart, must function as the neoaortic valve after arterial switch operation (ASO) for transposition of the great arteries, palliative surgery for hypoplastic left heart syndrome (HLHS), and pulmonary artery-to-aortic (P-A) anastomosis for complex heart disease with subaortic obstruction. The long-term function of this valve under these circumstances is not known. To investigate the function of this valve in the systemic circulation, the follow-up echocardiograms, catheterization data, and angiograms were reviewed for 189 patients at our institution after an ASO (n = 112), palliative surgery for HLHS (n = 45), or P-A anastomosis (n = 32). In addition, the effect on valve function of preoperative anatomy, prior placement of a pulmonary artery band (PAB), and length of follow-up was examined. Neoaortic regurgitation was present in 41% of patients after an ASO (mean +/- SD) follow-up (20 +/- 20; range, 2.2-80.5 months), 60% of patients after an HLHS repair (21 +/- 15; range, 3.7-62.4 months) and 50% after a P-A anastomosis (27 +/- 21; range, 2.6-89.4 months). Only eight patients had more than trivial/mild regurgitation. No neoaortic stenosis was observed. Minor preoperative valve abnormalities did not influence postoperative valve function. Prior PAB placement significantly increased the likelihood of postoperative neoaortic regurgitation after a two-stage ASO but not after a P-A anastomosis. In the ASO group, patients with an intact ventricular septum had a significantly higher prevalence of neoaortic regurgitation than those with a ventricular septal defect.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve↗
PubMed1991

Corrected transposition in situs inversus. Biventricular repair of associated cardiac anomalies.

Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.7 +/- 8.6 yr) underwent repair of associated cardiac anomalies, including ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) in all 13 patients and atrial septal defect (ASD) in 11. Systemic venous anomalies were present in four. Nine patients also had dextrocardia. Patches to close the VSD were placed on the right side of the ventricular septum (through a right [systemic] ventriculotomy) in two patients and on the left side of the ventricular septum in the other 11 via a left ventriculotomy in one or a morphological right (left-sided) atriotomy in 10. The POTO was relieved directly in five patients and bypassed by a left ventricle-pulmonary artery conduit in the other eight. There was one early death due to cerebral hemorrhage. Two patients required a pacemaker for permanent heart block. Other rhythm disturbances included transient heart block in three patients and late atrial fibrillation in one. All the survivors are asymptomatic as late as 15.5 years after the repair. One patient had conduit revision 10.5 years after the initial operation, two had residual POTO of greater than 40 mm Hg, and none had residual VSD. In conclusion, patients with [I,D,D] C-TGA present with a relatively uniform subset of anatomic lesions, including predominantly ASD, VSD, and POTO.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Vessel Prosthesis↗
PubMed1990

Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries.

A successful outcome of an arterial switch operation (ASO) for dextro-transposition of the great arteries (D-TGA) depends in large part on the transfer of the coronary arteries to the neoaorta without distortion or narrowing. However, the origins and distribution of the coronary arteries are quite variable in D-TGA; therefore, the entire experience with ASO at the Children's Hospital in Boston was reviewed. From 1983 through November 1989, 314 patients underwent surgery for D-TGA with the intent to perform an ASO. An ASO was actually performed in 290 patients, and 20 patients died, with 12 deaths (4.2%) clearly related to problems with the coronary arteries (CA deaths). The ASO was aborted to a Senning operation in 24 patients (7.6%), primarily due to coronary anatomy. Eight coronary patterns were identified and related to the outcome of the ASO. In 182 patients with the "usual" coronary pattern for D-TGA (i.e., anterior descending and circumflex arteries from the left sinus and right coronary artery from the right sinus), five CA deaths occurred, and two patients had Senning operations. Sixty-seven patients had right coronary and circumflex arteries from the right sinus and anterior descending arteries from the left sinus. Three CA deaths occurred, and one patient had a Senning operation. Single right coronary artery was found in 14; 12 had ASOs with three CA deaths (25%); and two had Senning operations. One late death was due to diffuse narrowing of the single right coronary artery. Single left coronary artery occurred in 11 patients, and seven had ASO with no early but one late "sudden" death, whereas four had Senning operations.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1989

Results of the arterial switch operation for transposition of the great arteries with ventricular septal defect. Surgical considerations and midterm follow-up data.

Between January 1983 and December 1987, 62 patients underwent an arterial switch operation for transposition of the great arteries with ventricular septal defect or double outlet right or left ventricle. There were three hospital deaths (4.8%), and no deaths occurred in neonates (less than 1 month of age, n = 18). There were three late deaths, one due to coronary obstruction and two due to pulmonary vascular obstructive disease. One child has been lost to follow-up. We have prospectively evaluated the remaining 55 survivors by clinical evaluation, echocardiography, cardiac catheterization, ambulatory electrocardiographic monitoring, and limited electrophysiologic studies. The mean length of follow-up has been 27 +/- 16 months since surgery. One child has required reoperation for a residual ventricular septal defect; no child has undergone reoperation for supravalvar pulmonary or aortic stenosis. Aortic regurgitation was identified in 12 children (22%), which was mild in 11 and moderate in one. One child has asymptomatic occlusion of the left main coronary artery, one child has a tiny right coronary artery-to-pulmonary artery fistula, and one child has abnormal left ventricular wall motion according to follow-up angiography. No other abnormalities of systemic (left) ventricular function have been identified at late follow-up. In addition to the two late deaths due to pulmonary vascular obstructive disease, three children, all of whom were repaired at more than 6 months of age, have elevated pulmonary vascular resistance. Notable postoperative arrhythmias include complete heart block in four patients and nonsustained supraventricular or ventricular tachycardia early after surgery in eight patients (all resolved without medication at later follow-up). Only two patients have evidence of sinus node dysfunction and have not required treatment. The low hospital mortality and encouraging early follow-up data represent a significant improvement over atrial level repairs, supporting the arterial switch operation as the procedure of choice for children who have transposition of the great arteries with ventricular septal defect or double outlet ventricle. Because of the potential for the development of early pulmonary vascular obstructive disease in these patients, repair is recommended within the first 2 months of life.

Coronary Vessels↗
PubMed1988

Isolated infundibuloarterial inversion (S,D,I): a newly recognized form of congenital heart disease.

A newly recognized form of congenital heart disease is presented that is characterized by viscero-atrial situs solitus (S), D-loop ventricles (D), and inverted normally related great arteries (I), the segmental combination being (S,D,I). This anomaly may be called isolated infundibuloarterial inversion because only the subsemilunar infundibulum and the great arteries are inverted, whereas the atrial and the ventricles are not. All three patients had atrioventricular concordance, ventriculoatrial concordance, dextrocardia, superoinferior ventricles, crisscross atrioventricular relations, underdevelopment of the right ventricle, a large ventricular septal defect, and an inverted tetralogy of Fallot type of malformation of the infundibulum and great arteries. The condition known as crisscross atrioventricular relations was found in these three patients to be a major ventricular malposition characterized by marked clockwise rotation of the ventricles, as seen from the front. Two of these three cases were diagnosed accurately and repaired successfully.

Angiocardiography↗
PubMed1983

Origin of pulmonary artery branch from ascending aorta. Primary surgical repair in infancy.

1. Surgical repair of origin of a pulmonary artery branch from the ascending aorta should be performed as early as possible in order to prevent death from congestive heart failure or the development of irreversible pulmonary vascular obstructive disease. 2. Deep hypothermic circulatory arrest greatly facilitates surgical repair of this lesion in infancy, as is illustrated by the three cases reported herein. 3. Direct anastomosis of the ectopic pulmonary artery branch to the main pulmonary artery is the surgical technique of choice. 4. Origin of the RPA or the LPA from the ascending aorta results from origin of the RPA or LPA from the aortic sac, instead of from the confluent sixth arches. Typically, the RPA has failed to migrate leftward because of abnormal development of the wall of the aortic sac, abnormal development of the sixth arches, or both. 5. Origin of the RPA or the LPA from the ascending aorta should be distinguished from origin of the "RPA" or of the "LPA" from the innominate artery or from the aortic arch via a PDA or a collateral artery.

Cardiac Catheterization↗