Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006.

Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI) >6 W.U. x m(2) have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 +/- 5.6 years and a mean time from listing to HTX of 43 +/- 60 days. Preoperative and postoperative (114 +/- 40 days) PVRI were 5.9 +/- 4.4 and 2.9 +/- 1.5 W.U. x m(2), respectively. At time of most recent follow-up (mean = 5.7 +/- 4.6 years), the mean PVRI was 2.0 +/- 1.0 W.U. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. x m(2) (chi(2)= 7.4, p < 0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI > 6 W.U. x m(2) survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.

Comparative roles of intraoperative epicardial and early postoperative transthoracic echocardiography in the assessment of surgical repair of congenital heart defects.

In 94 consecutive patients undergoing surgical repair of congenital heart defects the results of intraoperative (after cardiopulmonary bypass) epicardial two-dimensional and Doppler color flow imaging were compared with those of sequential transthoracic echocardiography performed within 24 h of surgery and again before hospital discharge to define the precise role of intraoperative imaging. In 6 of 7 patients with a residual defect requiring immediate surgical revision, intraoperative imaging correctly identified the defect; spectral Doppler imaging underestimated or did not identify a residual outflow tract gradient in 17 patients. Left atrioventricular (AV) valve regurgitation after repair of complete AV septal defect was underestimated in three patients. Although intraoperative documentation of good ventricular function was usually associated with a good outcome, in three patients poor systemic ventricular function after cardiopulmonary bypass was not associated with early mortality. A minor degree of shunting around the patch was a common finding on epicardial and early postoperative imaging and persisted at the time of hospital discharge in 17 of 46 patients who had undergone patch closure of a ventricular septal defect as part of the surgical procedure. Additional trabecular septal defects were missed on color flow imaging after cardiopulmonary bypass in three patients, one of whom required subsequent reoperation. Although intraoperative two-dimensional and color flow imaging permitted the recognition of the majority of residual defects requiring immediate revision, residual outflow obstruction or AV valve regurgitation was usually underestimated.

Heart transplantation in children with congenital heart disease.

OBJECTIVES: The aim of this study was to describe heart transplantation in children with congenital heart disease and to compare the results with those in children undergoing transplantation for other cardiac diseases. BACKGROUND: Reports describe decreased survival after heart transplantation in children with congenital heart disease compared with those with cardiomyopathy. However, transplantation is increasingly being considered in the surgical management of children with complex congenital heart disease. Present-day results from this group require reassessment. METHODS: The diagnoses, previous operations and indications for transplantation were characterized in children with congenital heart disease. Pretransplant course, graft ischemia time, post-transplant survival and outcome (rejection frequency, infection rate, length of hospital stay) were compared with those in children undergoing transplantation for other reasons (n = 47). RESULTS: Thirty-seven children (mean [+/- SD] age 9 +/- 6 years) with congenital heart disease underwent transplantation; 86% had undergone one or more previous operations. Repair of extracardiac defects at transplantation was necessary in 23 patients. Causes of death after transplantation were donor failure in two patients, surgical bleeding in two, pulmonary hemorrhage in one, infection in four, rejection in three and graft atherosclerosis in one. No difference in 1- and 5-year survival rates (70% vs. 77% and 64% vs. 65%, respectively), rejection frequency or length of hospital stay was seen between children with and without congenital heart disease. Cardiopulmonary bypass and donor ischemia time were significantly longer in patients with congenital heart disease. Serious infections were more common in children with than without congenital heart disease (13 of 37 vs. 6 of 47, respectively, p = 0.01). CONCLUSIONS: Despite the more complex cardiac surgery required at implantation and longer donor ischemic time, heart transplantation can be performed in children with complex congenital heart disease with success similar to that in patients with other cardiac diseases.

Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique.

Ten consecutive patients (age range 4 to 44 years, mean 22) underwent surgical repair of Ebstein's anomaly by vertical plication of the right ventricle and reimplantation of the tricuspid valve leaflets. No patient died during or after operation. Intraoperative postbypass echocardiography documented a good result in nine patients but severe tricuspid regurgitation in one patient, who then underwent prosthetic valve replacement during a second period of cardiopulmonary bypass. Two of four patients who had had right ventricular papillary muscle dysfunction in the early postoperative period showed improved papillary muscle function with concomitant reduction of tricuspid regurgitation 6 months later. All patients were evaluated clinically and by echocardiography 2 to 23 months later. All patients showed clinical improvement, seven by one functional class and three by two classes. All were in sinus rhythm. The mean cardiothoracic ratio decreased by 6% (p less than 0.05). On bicycle ergometry performed in six patients, peak oxygen consumption exceeded 20 ml/kg per min in five. Tricuspid regurgitation diminished in eight patients (by three grades in two patients, by two grades in five and by one grade in one patient); it remained unchanged in two. Comparison of preoperative and postoperative pulsed Doppler flow velocities across the pulmonary valve showed an increase in the peak velocity of flow across the valve (mean 83 +/- 14 versus 97 +/- 11 cm/s, p less than 0.005) and a decrease in the time to peak velocity (mean 130 +/- 16 versus 91 +/- 23 ms, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Assessment of ventricular septal defect closure by intraoperative epicardial ultrasound.

Intraoperative epicardial two-dimensional echocardiographic imaging, color flow mapping and contrast echocardiography were used in 31 patients after patch closure of a ventricular septal defect to determine their respective values in the assessment of residual shunting after cardiopulmonary bypass and for the prediction of long-term results. Epicardial imaging showed no incidence of patch dehiscence. Residual shunting detected by color flow mapping or contrast echocardiography was graded into one of four categories (0 to III). Real time analysis of color flow mapping studies suggested no shunting (grade 0) in 2 patients, grade I shunting in 20, grade II in 8 and grade III in 1; contrast studies suggested grade 0 in 15, grade I in 6, grade II in 8 and grade III in 2. Interobserver variation in real time encoding of grade I or II shunting was 25% by color flow mapping and 6% by contrast echocardiography. Subsequent frame by frame analysis revealed that both diastolic and early systolic right ventricular turbulence gave rise to false positive results during real time analysis of color flow mapping studies. Color flow mapping allowed exact localization of residual shunting, whereas contrast echocardiography allowed better semiquantification. Postbypass results were correlated in 30 patients with late postoperative precordial studies (mean interval 7.5 months). Persistent shunts were found in 6 (20%) of 30 patients. No patient required reoperation for residual shunting. The predictive value of immediate grade I or II shunting as a marker for persistent long-term shunting was poor, whereas both patients with immediate grade III shunting had shunt persistence, indicating that immediate revision should be considered in such patients. Intraoperative epicardial ultrasound is valuable for the immediate exclusion of important residual shunting after ventricular septal defect closure. Maximal information is obtained when color flow mapping and contrast echocardiography are used in combination.

Influence of electrogenic Na/Ca exchange on the action potential in human heart muscle.

STUDY OBJECTIVE: The plateau of the action potential in heart muscle is largely due to the inward Ca2+ current, ica; however, Ca2+ extrusion via Na+/Ca2+ exchange may also generate a significant current, ina/ca. The aim was to assess the influence of ina/ca on the action potential in isolated human heart muscle. DESIGN: Action potentials and force of isometric contractions were recorded in ventricular trabeculae. The muscle was subjected to various stimulus patterns, Ca2+ antagonists, and variations in the ionic composition of the extracellular medium. PATIENTS: From 49 patients, aged 0.5 to 14 years, small right ventricular trabeculae were obtained during open heart surgery. The operations concerned corrections of ventricular septal defects. Data presented in this paper were from nine preparations in which action potentials were recorded during several hours. MEASUREMENTS AND MAIN RESULTS: The results confirmed that: (1) the amplitude of the early part of the plateau was depressed by low [Ca2+] and by Ca2+ antagonists, showing that ica dominates this early part; and (2) that low [Na2+] and post-extrasystolic potentiation also depressed the early component of the plateau of the action potential, which can be explained by inactivation of ica due to increased levels of intracellular Ca2+. A novel observation was that post-extrasystolic potentiation led to an increase in action potential duration (APD). An explanation is that the potentiated contraction follows from an increased amount of intracellular Ca2+ which also activates an inward current, possibly ina/ca. This assumption is strengthened by the finding that lengthening of APD after extrasystoles was abolished: (a) at low [Ca2+], ie, when force was small and there was little Ca2+ to be extruded; and (b) at low [Na+], ie, when force was large but the driving force of the Na+ gradient for extrusion of Ca2+ was small. CONCLUSIONS: The early part of the plateau is dominated by ica, whereas ina/ca is relatively more important during the later part, and tends to lengthen the action potential.

Usefulness of preoperative echocardiography in predicting left ventricular outflow obstruction after primary repair of interrupted aortic arch with ventricular septal defect.

Residual left ventricular outflow tract (LVOT) obstruction is a significant problem after repair of interrupted aortic arch (IAA) and ventricular septal defect. Resection of subaortic tissue at the time of primary repair, however, is associated with increased morbidity and mortality. We reviewed the preoperative echocardiograms and the postoperative clinical course and echocardiograms of 23 consecutive patients who underwent primary repair of IAA without widening of the subaortic region. Nine patients (39%) developed significant LVOT obstruction (pressure gradient >40 mm Hg). LVOT obstruction was noted postoperatively in 7 of 9 patients by 1 month, 8 of 9 by 2 months, and 9 of 9 by 1 year. On retrospective analysis of the preoperative echocardiograms, the indexed cross-sectional area of the LVOT, the subaortic diameter index, and the subaortic diameter Z score were all significantly smaller in those requiring reintervention (p <0.04, p <0.05, p <0.05, respectively). Of these, indexed cross-sectional area had the least reproducibility and subaortic diameter index the most (coefficient of variation of 26.3% vs 11.2%). In conclusion, most patients who develop significant LVOT obstruction after repair of IAA do so within 1 month of operation. Although subaortic indexed cross-sectional area is the most sensitive predictor of LVOT obstruction after primary repair of IAA, other more simple standardized measurements of the subaortic diameter were comparably predictive and had better reproducibility.

Survival and risk factor analysis for the Norwood procedure for hypoplastic left heart syndrome.

The optimal approach to hypoplastic left heart syndrome (HLHS) is controversial. The palliative Norwood operation, cardiac transplantation, and no surgical intervention have all been advocated. Centers that perform the Norwood operation have met with varied results, and conflicting reports exist regarding factors predictive of stage I outcome. From January 1990 to January 1996, 67 patients with HLHS were admitted with intent to perform the staged Norwood procedure. Fourteen patients did not undergo surgery. In the 53 patients treated surgically, outcome was reviewed, and 10 potential risk factors for first stage mortality were analyzed. Forty-one infants survived the Norwood I operation to hospital discharge (77% of the surgically treated patients and 61% of the entire group, including those who did not undergo operation) with 6 additional deaths 3 to 5 months after operation. Univariate analysis showed cardiopulmonary bypass time and circulatory arrest time to be significant risk factors for hospital mortality. Multivariate analysis revealed only cardiopulmonary bypass time as significant (p <0.01). Of the 15 prenatally diagnosed newborns who underwent surgery, 11 survived (p = 0.72). Ten of 11 patients with preoperative organ damage survived (p = 0.42). Among the 35 bidirectional Glenn (Norwood II) and Fontan (Norwood III) procedures performed, there were 2 deaths. The 5-year actuarial survival for patients who underwent operations was 61%. The Norwood procedure is a favorable option for the infant with HLHS. Surgical survival may be affected by a prolonged cardiopulmonary bypass time, but is not affected by other factors analyzed, including prenatal diagnosis and preoperative organ damage.

Serial echocardiographic measurements of the pulmonary autograft in the aortic valve position after the Ross operation in a pediatric population using normal pulmonary artery dimensions as the reference standard.

Serial echocardiographic measurements of the annulus and sinus were obtained in children before the Ross operation, and early and late postoperatively. Values were compared with normal standards for the aorta and pulmonary artery (PA). There was no significant difference between PA annulus measurements before surgery and the corresponding autograft immediately afterward (1.73 +/- 0.60 cm preoperatively; 1. 63 +/- 0.58 cm postoperatively, p = NS). Late after surgery the mean annulus diameter was enlarged compared with the normal aorta (DeltaZ 1.9 +/- 2.4), but remained relatively unchanged compared with the normal PA (DeltaZ 0.7 +/- 1.1, p <0.01). In contrast, the autograft sinus was dilated early after surgery (1.83 +/- 0.58 cm preoperatively; 2.18 +/- 0.73 cm postoperatively, p <0.01). Mean sinus Z score further increased compared with both the aorta (DeltaZ 1.3 +/- 1.7) and PA (DeltaZ 1.3 +/- 1.6). Use of standard PA measurements may be important in the assessment of autograft enlargement. Minimal change in autograft Z scores over time suggests that annulus enlargement is mainly due to somatic growth. In contrast, the autograft sinus showed an immediate and continued disproportionate increase in size over time, suggesting that sinus enlargement is largely due to passive dilation.

Cerebral blood flow velocity in pediatric patients is reduced after cardiopulmonary bypass with profound hypothermia.

Transcranial Doppler sonography of the middle cerebral artery was used to determine whether cerebral perfusion was detectable in low flow states during operations with cardiopulmonary bypass in pediatric patients. Quantitative and qualitative differences in cerebral blood flow velocity after rewarming in patients treated with continuous low-flow bypass or deep hypothermic circulatory arrest were assessed. To determine whether the alterations in cerebrovascular resistance pattern observed in our patients undergoing profound hypothermia was more a function of perfusion technique than of minimum temperature during operation, a third group of patients treated with moderate hypothermia was studied. The three patient groups were the arrest group (N = 16), comprised of patients undergoing circulatory arrest at 18 degrees to 20 degrees C; the low-flow group (N = 16), patients treated with continuous low-flow (cardiac index 0.5 L/min per square meter) bypass at 18 degrees to 20 degrees C; and the moderate group (N = 5), patients treated with moderate hypothermia at 24 degrees to 28 degrees C. Flow velocity was detectable in all patients in the low-flow group, with mean arterial pressures as low as 15 mm Hg. Mean flow velocity was reduced after bypass as compared with prebypass values in both the arrest and low-flow groups (p = 0.0001). Mean flow velocity increased after bypass in the moderate group (p = 0.0001). A Doppler waveform pattern consistent with high cerebrovascular resistance was found in 67% of patients in the arrest group and 44% of those in the low-flow group. None of the patients in the moderate group exhibited such a pattern. Patients treated with profound hypothermia who underwent a period of cold full-flow reperfusion before rewarming did not exhibit this high resistance pattern after rewarming. The present findings indicate that profound hypothermia may evoke changes in the cerebral vasculature that result in decreased mean cerebral blood flow velocity after cardiopulmonary bypass rewarming. A period of cold full-flow reperfusion before rewarming may prevent these alterations and improve cerebral perfusion during rewarming.

Plasma neuropeptide Y and catecholamines in pediatric patients undergoing cardiac operations.

OBJECTIVE: Our objective was to assess the sympathoadrenal response in pediatric patients undergoing repair of congenital cardiac defects. METHODS: Plasma catecholamine (norepinephrine and epinephrine) and neuropeptide Y concentrations were quantified before and after cardiopulmonary bypass to assess the response to cardiopulmonary bypass. To determine the response to aortic occlusion, levels of plasma catecholamines and neuropeptide Y were measured at the time of and immediately after release of the aortic crossclamp. RESULTS: During cardiopulmonary bypass, no significant change in levels of plasma norepinephrine (n = 43), epinephrine (n = 37), or neuropeptide Y (n = 46) was observed. Aortic occlusion induced a significant increase in plasma neuropeptide Y, but not in catecholamines. There was a greater increase in plasma neuropeptide Y in children older than age 1 year than in those younger than 1 year. CONCLUSIONS: Plasma neuropeptide Y may be a useful marker of sympathetic nervous system activity. Children younger than age 1 year showed a lesser sympathetic response compared with the response in older children.

Outcomes in patients with interrupted aortic arch and ventricular septal defect. A multiinstitutional study. Congenital Heart Surgeons Society.

Among 183 neonates with interrupted aortic arch and ventricular septal defect entering a multiinstitutional study between 1987 and 1992, nine died before repair was accomplished. Among the remaining 174, survival at 1 month and 1, 3, and 4 years after repair was 73%, 65%, 63%, and 63%, respectively. The risk factors for death were low birth weight, younger age at repair, interrupted arch type B, outlet and trabecular ventricular septal defects, smaller size of the ventricular septal defect, and subaortic narrowing. Echocardiographically measured dimensions (expressed as Z-values) at all levels of the left heart-aorta complex were small. Two among thirty institutions were risk factors, and two others possibly were. Procedural risk factors for death after repair were (1) repair without concomitant procedures in patients with other important levels of obstruction in the left heart-aorta complex, (2) a Damus-Kaye-Stansel anastomosis, and (3) subaortic myotomy/myectomy in the face of subaortic narrowing. One-stage repair plus ascending aorta/arch augmentation had the highest predicted time-related survival in the 20% of patients with interrupted aortic arch and one or more coexisting levels of obstruction in the left heart-aorta complex, as did initial repair without or with aorta/arch augmentation in the 80% without these.

Outflow obstruction after the arterial switch operation: a multiinstitutional study. Congenital Heart Surgeons Society.

OBJECTIVE: Our objectives were to discover whether outflow obstruction immutably accompanies the arterial switch operation and to identify factors that may decrease its prevalence. METHODS: Percutaneous or surgical reintervention for obstruction after an arterial switch was selected as an end point for obstruction. Its risk factors were identified by time-related multivariable analyses of yearly follow-up data from 514 neonates with simple transposition or transposition with ventricular septal defect entering 23 institutions before 15 days of age between January 1, 1985, and March 1, 1989. RESULTS: Sixty-two patients underwent 86 reinterventions for right-sided obstruction (83% free at 10 years) and six for left-sided obstruction (98% free at 10 years). After 2 years, right-sided obstruction occurred at a rate of about 1% per year and left-sided at a rate of about 0.1% per year. Right ventricular infundibular or valvular obstruction was associated with the aorta and pulmonary trunk positioned side-by-side, coexisting coarctation, use of prosthetic material in sinus reconstruction, one institution, and earlier institutional experience. Pulmonary trunk or pulmonary artery obstruction was associated with lower birth weight, left coronary artery arising from sinus 2, coronary explantation away from the transection site, three institutions, and earlier institutional experience. CONCLUSIONS: A risk-adjusted base incidence (0.5% per year) of reintervention for right-sided obstruction continues late after operation. It is due in part to congenital variability or abnormality of right ventricular outflow structures and to experience and surgeon variability resulting in suboptimal pulmonary trunk reconstruction. The same sources of variability probably affect the aortic root, but its native characteristics plus higher distending pressure make the base incidence considerably less (0.1% per year).

Outcomes in seriously ill neonates with coarctation of the aorta. A multiinstitutional study.

Among 326 severely symptomatic neonates with coarctation with or without ventricular septal defect, four died before an initial procedure was performed. Among the 322 undergoing an initial procedure, survival for at least 24 months was 84%; the hazard function for death was lower initially but more prolonged in patients without than in those with ventricular septal defect. Important mitral valve anomalies coexisted in 5% of patients, left ventricular hypoplasia in 5% (more commonly in patients without ventricular septal defect), narrowing of the left ventricular outflow tract in 9% (more common in patients without ventricular septal defect), and narrowing of the proximal arch in 1%; one or more of these anomalies was present in most patients without ventricular septal defect who died. Five percent of the 322 patients had more than one of these coexisting anomalies, and 8% had just one. The most commonly used technique of repair of the coarctation was resection and end-to-end anastomosis, but no technique was a risk factor for death by multivariable analysis. Extension of the area of resection so that the end-to-end anastomosis was proximal to the left subclavian artery but distal to the left common carotid artery did not increase risk; extensions beyond this, and in the case of patch graft repair, extensions proximal to the left subclavian artery, did increase risk. Patch graft repair was associated with the highest prevalence (21%) of reintervention to the coarctation repair. Among patients with coexisting moderate-sized or large ventricular septal defects, repair of the coarctation, pulmonary trunk banding, and subsequent repair of the defect were associated with the highest 2-year survival, 97% in those with single ventricular septal defect. The risk-adjusted outcomes in two institutions were less good than in all others.

A modification of staged septation for univentricular atrioventricular connection.

A case is reported in which is described a modification of staged septation for univentricular atrioventricular connection with double-inlet left ventricle and left-sided rudimentary outlet chamber beneath the aorta. This modification diminishes the risk of atrioventricular conduction disturbances by gluing the perforated septation patch on the myocardium in the area overlying the central conduction system. Fixation of the patch was facilitated by exact fitting of the patch in this area, by ample contact of the patch and the myocardium in this area, and by immobilization of the patch because of equal pressures on both sides because of the perforation of the patch. In addition, this modification facilitates evenly drawing in of the ventricular free wall because already, in the first stage, the complete circumference of the patch is fixed, defining the maximal contribution of prosthetic material to the newly created septum.

Growth of an aorta-coronary anastomosis. An experimental study in pigs.

The influence of growth of an aorta-coronary anastomosis, comparable to the coronary translocation anastomosis during the arterial switch operation, was studied in pigs. The anastomosis between the right coronary artery and the aorta did not grow, and this lack of growth may result in stenosis. With another technique, by which the coronary artery was excised with a cuff of aortic wall, the effects caused by absence of growth were circumvented and a normal-sized coronary ostium was present after growth. However, when no cuff was used, stenosis occurred at the suture line and caused growth retardation of the animal as well as histologic damage to the right ventricle.

Double-inlet ventricle: morphologic analysis and surgical implications in 32 cases.

We analyzed, using a sequential segmental approach, 32 cases of double-inlet ventricle to assess the feasibility of surgical "correction" by either ventricular septation or a modified Fontan procedure. Twenty-two hearts had two atrioventricular valves, connected to a left ventricle in 19, a right ventricle in two, and a solitary indeterminate ventricle in one. Septation was possible in only 13. In contrast, the Fontan procedure seemed feasible in 20. The remaining 10 specimens had double inlet via a common valve to the left ventricle in two, the right ventricle in six, and an indeterminate ventricle in two. Seven of these had right atrial isomerism. Ventricular septation was not considered a possibility in these hearts. The Fontan procedure combined with atrial septation was a possibility in seven cases. From the morphologic stance, although the modified Fontan procedure seemed suitable in most cases, a significant number of hearts with two atrioventricular valves were suitable for ventricular septation.

Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications.

An anatomic study of 28 heart specimens with tricuspid atresia showed 15 hearts with ventriculoarterial concordance and a right-sided anterior outlet chamber. Of these 15 hearts, 12 showed evidence of restricted pulmonary blood flow. In eight of these 12 specimens, an anterior deviation of the outlet septum, relative to the primary septum, was present. This deviation caused a narrowing inside the outlet chamber and functioned as infundibular pulmonary stenosis. The outlet chambers of the 15 hearts with ventriculoarterial concordance were compared with those of 13 hearts with tricuspid atresia and ventriculoarterial discordance and also a right-sided anterior outlet chamber. In the latter group of 13 hearts, five showed evidence of restricted pulmonary blood flow, at least partially caused by posterior deviation of the outlet septum relative to the primary septum. A clear difference in outlet chamber morphology was found without exception between specimens with and without ventriculoarterial concordance. Clinical data in three long-term survivors of the Fontan procedure demonstrate that the special morphology of the outlet chamber can pose surgical problems. Especially in patients with ventriculoarterial concordance, when the outlet chamber is incorporated into the pulmonary circuit, the surgeon must give special attention to the specific morphology to prevent undesirable sequelae.

Juxtaductal pulmonary artery coarctation. An underestimated cause of branch pulmonary artery stenosis in patients with pulmonary atresia or stenosis and a ventricular septal defect.

An angiographic and clinical study was performed to establish the prevalence of juxtaductal pulmonary artery coarctations in patients with pulmonary atresia or stenosis and a ventricular septal defect or a complex intracardiac defect. The present study is an adjunct to a previously reported portmortem study, in which the incidence of these pulmonary artery coarctations was found to be unexpectedly high. Pulmonary artery coarctations were identified angiographically in 10 of the 15 patients with pulmonary atresia. One additional patient had a bilateral ductus arteriosus and confluent pulmonary arteries, but did not have a pulmonary artery coarctation. Pulmonary artery coarctations were much less prevalent in the cases with pulmonary stenosis (5/50). However, these pulmonary artery coarctations appeared identical to those of the cases with pulmonary atresia. Fourteen pulmonary artery coarctations were located in the pulmonary artery at the side of the ductus arteriosus; this was left sided in 12 and right sided in two patients. In one patient the side of the ductus could not be established. The types and the locations of the pulmonary artery coarctations in the present study were identical to those in the previous postmortem study. Ductal tissue was found in many of the pulmonary artery coarctations of the postmortem study and is likely to be present in the clinical cases as well. The majority of the angiographically identified pulmonary artery coarctations were subsequently confirmed at operation or at autopsy. The clinical outcome and follow-up of the patients is discussed, and it is concluded that juxtaductal pulmonary artery coarctations should be specifically looked for before and during any type of surgical intervention in these patients.

Long-term results of atrial correction for transposition of the great arteries. Comparison of Mustard and Senning operations.

Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition (p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation (p < 0.001). In multivariate analysis, significant independent risk factors for late death turned out to be complex transposition (versus simple) and active arrhythmias. The only significant risk factor for the occurrence of sinus node dysfunction was the Mustard operation. We conclude that apart from the difference in the loss of sinus rhythm, no differences were found in the long-term clinical results of the two types of operations.