Rastelli Group Research Articles

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Long-Term Influence of Atrial Switch Operation on Hemodynamics After the Rastelli Procedure.

The study aimed to investigate the long-term influence of atrial switch on post-Rastelli hemodynamic condition. Of 112 patients with transposition of the great arteries (TGA) or TGA-type double outlet right ventricle, ventricular septal defect (VSD), and pulmonary stenosis (PS) who underwent intra-cardiac repair between 1979 and 2018, 50 patients with levo-TGA underwent atrial switch and Rastelli as an anatomic repair and 62 patients with dextro-TGA underwent Rastelli. Postoperative outcomes were retrospectively compared. The median follow-up durations were 20.1years (interquartile range: 4.3, 32.4) in the Rastelli group and 15.3years (7.1, 23.0) in the atrial switch plus Rastelli group (p = 0.19). Sex, age, and weight at operation were similar in both groups. Overall survival rates at 30years were 69.8% in the Rastelli group and 80.1% in the atrial switch plus Rastelli group (p = 0.18). The atrial switch plus Rastelli group required more frequent catheter interventions (p < 0.001), mainly for caval obstruction (n = 8) and atrial arrhythmia (n = 6). Medication was more frequent in the atrial switch plus Rastelli group (p = 0.009). Exercise capacity was similarly reduced in two groups. Protein-losing enteropathy (PLE) occurred in three long-term survivors in the atrial switch plus Rastelli group (p = 0.07). Concomitantly performed atrial switch operation did not affect long-term survival and exercise capacity after Rastelli procedure. However, the occurrence of PLE, a frequent need for medication, and catheter interventions after atrial switch plus Rastelli may result from atrial switch under the post-Rastelli condition.

Long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction

ObjectiveThe objective of this study was to evaluate the long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction.MethodsWe conducted a retrospective study of patients with transposition of the great arteries or double outlet right ventricle with left ventricular outflow tract obstruction undergoing biventricular repair between 1980 and 2017.ResultsOne hundred and eleven patients were enrolled and classified into five groups: atrial switch (n = 20), arterial switch (n = 12), Nikaidoh (n = 7), Rastelli (n = 48), and REV operation groups (n = 24). Early mortality was highest in Nikaidoh group (29%). Median follow-up was 18.2 years. Long-term survival was by far lowest in Nikaidoh group and comparable among the other 4 groups. Freedom from reoperation at 20 years was lowest in Rastelli group (32.1%) due to right ventricular outflow tract-related reoperations. While having no recurrence of left ventricular outflow tract obstruction, the arterial switch operation group had a high proportion of substantial neo-aortic regurgitation (29%).ConclusionsThe long-term survival was satisfactory regardless of the surgical technique except Nikaidoh group. The surgical option for transposition of the great arteries with left ventricular outflow tract obstruction should be selected based on the features of the respective procedures.

Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis: Modified REV versus Rastelli

The objective of this study was to evaluate and compare the results of the modified réparation à l'ètage ventriculaire (REV) and the Rastelli operation for the treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS). Records of 38 patients who underwent the modified REV (n = 16) or the Rastelli operation (n = 22) for the treatment of TGA, VSD, and PS between 2010 and 2019 were reviewed. The median age was 2.2 years (range 0.6-8.0 years) and the median weight was 11.3 kg (range 6.4-22.0 kg). No in-hospital death occurred and there were 4 early reoperations (two in each group). Overall survival at 10 years was 97.4% (100% in Modified REV group and 95.5% in Rastelli group, P = 0.39). Freedom from left ventricular outflow tract (LVOT) reoperation was 100% in both groups. Freedom from right ventricular outflow tract (RVOT) reoperation was 100% in Modified REV group and 75.4% in Rastelli group (P = 0.073). Event-free survival was 100% in Modified REV group and 72.0% in Rastelli group (P = 0.048). The most recent echocardiography showed that LVOT peak gradient was less than 10 mmHg in all patients. In Modified REV group, 30.8% of patients (4/13) had either RVOT obstruction (RVOT peak gradient more than 40 mmHg) or moderate or severe pulmonary insufficiency, while conduit stenosis (peak gradient more than 40 mmHg) was found in 25.0% of patients (3/12) in Rastelli group. The modified REV and the Rastelli operation provide satisfactory early results, as well as long-term survival and LVOT performance. However, the modified REV has better RVOT performance.

Outcomes of the anatomical repair in patients with congenitally corrected transposition of the great arteries: lessons learned in a high-volume centre.

The physiological repair of the congenitally corrected transposition of the great arteries (ccTGA) has been associated with a long-term risk of the right ventricular dysfunction and tricuspid valve regurgitation. On the other side, the anatomical repair with the restoration of the left ventricle in a systemic position has been hypothesized to improve long-term outcomes. The aim of this study was to determine the results of the anatomical repair. Between 1990 and 2015, 39 patients underwent an anatomical repair of a ccTGA. The median age was 7 years (range from 8 days to 42.8 years). Eighteen (46%) patients had a double switch. Of these, 21 (54%) patients had an atrial switch with a Rastelli technique (n = 16, 41%), with a réparation à l'étage ventriculaire (REV) procedure (n = 4, 10%) or a truncal rotation (n = 1, 3%). The follow-up was 100% complete at 2 years with a median follow-up of 3.17 years (range 0-26 years). The early mortality was 10% (n = 4) and the late mortality was 20% (n = 8). The long-term survival was 77 ± 10% at 10 years for the double switch group and 62 ± 11% at 10 years for the Rastelli group (P = 0.25). Eight (20%) patients required reintervention. Freedom from reoperation at 10 years was 82 ± 11% in the double switch group and 88 ± 11% in the Rastelli group. At the last follow-up, 23 (82%) patients were in New York Heart Association (NYHA) functional class I and 2 (7%) patients in NYHA class II. The anatomical repair of the ccTGA is associated with significant early mortality and morbidity. However, the long-term functional status is satisfactory. The anatomical repair of the ccTGA should be reserved for a carefully selected group of patients.

Pulmonary annular motion velocity reflects right ventricular outflow tract function in children with surgically repaired congenital heart disease.

Right ventricular (RV) dysfunction is generally evaluated using analyses of tricuspid annular motion. However, it represents only one aspect of RV performance. Whether measuring pulmonary annular motion velocity could serve as a novel way to evaluate global RV and/or RV outflow tract (RVOT) performance in pediatric congenital heart disease (CHD) patients with surgically repaired RVOT was evaluated. In this prospective study, tissue Doppler-derived pulmonary annular motion velocity was measured in children (aged 2-5years) with RVOT reconstruction (RVOTR group, n=48) and age-matched healthy children (Control, n=60). The types of RVOTR procedures were as follows: pulmonary valve-sparing procedure (PVS, n=7); transannular patch with monocusp valve reconstruction (TAP, n=29); and RV-to-PA conduit reconstruction using a pericardial valve with expanded polytetrafluoroethylene conduit (Rastelli, n=12). Pulmonary annular motion velocity waveforms comprised systolic bimodal (s1' and s2') and diastolic e' and a' waves in all participants. The peak velocities of s1', s2', e', and a' were significantly lower in the RVOTR group than in the control group (all p<0.0001). Furthermore, these parameters depended significantly on the type of surgical procedure. The peak velocities of s1', s2', and e' had significant correlations with RVOT ejection fraction (RVOT-EF) (r=0.56, 0.49, and 0.34, respectively), and RVOT fractional shortening (RVOT-FS) (r=0.72, 0.55, and 0.41, respectively), although there were no significant correlations between pulmonary annular motion and global RV function, including RV ejection fraction (RVEF) and RV fractional area change (RVFAC) in the assessment of all RVOTR group patients. The pulmonary annular motion parameters in the PVS group had significant correlations with both global RV and RVOT performance. The TAP group showed significant correlations between RVOT function and pulmonary annular motion. The Rastelli group showed almost no significant correlations between RV/RVOT function and tissue Doppler parameters. Pulmonary annular motion velocity is a simple, rapid, reproducible, and useful method of assessing RVOT function in children with surgically repaired CHD.

Clinical Outcomes after Anatomic Repair Including Hemi-Mustard Operation in Patients with Congenitally Corrected Transposition of the Great Arteries.

Background and ObjectivesThe aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure.Subjects and MethodsWe conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4).ResultsAtrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation.ConclusionAnatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.

Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries

The Senning or Mustard procedure combined with the arterial switch operation (ASO) (± VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients. Between 2004 and 2011, eight patients underwent hemi-Mustard/BCPA with the Rastelli operation (n = 6) or ASO (n = 2). The median age was 2.9 (range: 1.2-9.1) years. Positional anomalies were present in 75% of the patients. Both patients with ASO had dysplastic and insufficient tricuspid valves. In the Rastelli group, four patients had previously received shunts followed by BCPA in one patient. In the ASO group, both patients underwent pulmonary artery banding initially. There was one in-hospital death and no late mortality. Two patients received a pacemaker. One patient from the Rastelli group required conduit change 6 years later. At the mean follow-up of 4.5 years, six and one patients are in NYHA classes I and II, respectively; six patients showed good biventricular function, while one had LV dysfunction. Systemic venous obstruction and sinus node dysfunction were not observed, and BCPA was functioning well in all patients. Hemi-Mustard/BCPA is useful in anatomical repair of CCTGA in selected patients. When compared with the classic atrial switch operation, it is technically easier which makes it especially helpful in atrio-apical discordance; it unloads an RV with limited size or function, and avoids complications related to the upper limb of the classic atrial switch procedure. Mid-term results of this approach are favourable. Further follow-up is needed to prove long-term benefits.

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

Aim of the studyTo investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.MethodsA total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.ResultsExcluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were 62% (CI 48-76%) and for the Rastelli group 67% (CI 51-83%). Freedom of reoperation at 20 years was 32% (CI 19-45%) in the overall group. In the non-Rastelli group the data at 20 years were 47% (CI 11-83%) and for the Rastelli group 21% (CI 0-54%) after almost 19 years.ConclusionsLong term follow up confirms that surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. Death occurred mostly as a result of cardiac failure.

Surgical Treatment of Transposition of Great Arteries With Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction

Our purpose was to evaluate our single-center experience with the treatment of transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO). Between 1992 and 2009, 42 patients were operated on. Twenty-three patients underwent the Rastelli operation, 8 patients underwent arterial switch operation (ASO) with associated LVOTO procedures, 4 patients underwent the réparation à l'étage ventriculaire (REV) procedure, 3 patients underwent the Bex/Nikaidoh (BN) procedure, and the Fontan operation was performed in 4 patients. The median age at final operation was 20.7 months (range, 0.3-234). The overall survival rate was 97% (1 early death), with a median follow-up of 8.2 years. There were no differences in survival among the surgical groups. Event-free survival was 100%, 84%, 59%, and 24% at 1, 5, 10, and 15 years of follow-up, respectively, with it being worse in the Rastelli group (P < .0348). The last echocardiography showed good function of the systemic ventricle in all patients; LVOTO pressure gradient greater than 30 mm Hg was observed in 2 patients (5%), and right ventricular outflow tract obstruction (RVOTO) pressure gradient >30 mm Hg was observed in 12 patients (31%). All patients are in sinus rhythm, and 74% of them are without medication. All surgical approaches are safe and show excellent midterm functional outcome. ASO is the best option if the LVOTO is resectable. Intraventricular rerouting (Rastelli or REV) is the method of choice in the majority of patients, but Rastelli has a significant reintervention rate. The BN operation has the potential to minimize utilization of the Fontan operation, which was used in the past if the intracardiac anatomy was unfavorable.

Anatomic repair for congenitally corrected transposition of the great arteries

Anatomic repair is being actively evaluated as the preferred option for congenitally corrected transposition of the great arteries. We present our 13-year experience with this approach. Between May 1994 and September 2007, 68 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Thirty-one patients (group 1, mean age of 94.8 +/- 42.3 months) underwent a combined Rastelli and atrial switch operation. Thirty-seven patients (group 2, mean age of 36.1 +/- 46.9 months) underwent an arterial switch operation and atrial rerouting. Eight patients in group 2 had an intact ventricular septum. Group 1 had 5 early deaths (17%) but no late deaths. Three patients underwent conduit revision at a mean follow-up of 62 months. Group 2 had 5 early deaths (13.5%). There were 4 late reoperations (2 pulmonary baffle revisions, 1 mitral valve replacement, and 1 permanent pacemaker implantation) and 4 late deaths (1 secondary to progressive left ventricular dysfunction, 2 secondary to uncontrolled atrial tachyarrhythmia, and 1 secondary to pulmonary hypertension and right ventricular failure). In group 2, 4 patients have a left ventricular ejection fraction less than 40%, 5 patients have moderate aortic incompetence, 5 patients have symptomatic tricuspid incompetence, 1 patient has tricuspid stenosis, 1 patient has superior cava obstruction, and 3 patients are receiving antiarrhythmic therapy. The occurrence of left ventricular dysfunction indicate that anatomic repair in the arterial switch group is still fraught with imperfections. The Rastelli group required conduit revisions but has otherwise performed well.

Strategy for biventricular outflow tract reconstruction: Rastelli, REV, or Nikaidoh procedure?

Three techniques have been developed as the surgical management for patients with anomalies of ventriculoarterial connection, ventricular septal defect, and pulmonary outflow tract obstruction (stenosis): the Rastelli, Lecompte, (REV), and Nikaidoh procedures. This study was designed to compare these procedures in terms of hemodynamics of the reconstructed biventricular outflow tract, early clinical consequences, and follow-up. Between March 2004 and September 2006, a total of 30 consecutive patients underwent double root translocation procedures (modified Nikaidoh n = 11, REV n = 7, Rastelli n = 12). In the Nikaidoh procedure, both aortic and pulmonary roots were translocated. A single-valved bovine jugular vein patch was used to repair the stenotic pulmonary artery in both Nikaidoh and REV procedures. The Senning procedure was added for those with atrioventricular discordance. The Nikaidoh procedure was the most time-consuming in terms of mean cardiopulmonary bypass and aortic crossclamp times. The average mechanical ventilation time was significantly shorter in the Rastelli group (63.3 +/- 89 hours) than that in the Nikaidoh group (188.7 +/- 159 hours, P = .016), but not different from that in the REV group (76.4 +/- 112.5 hours, P = .395). Two patients in the REV group and 1 in the Rastelli group died. There were no in-hospital or late deaths in the Nikaidoh group. Postoperative echocardiography demonstrated physiologic hemodynamics in the left ventricular outflow tract and normal heart function in the Nikaidoh group. Abnormal flow pattern in the left ventricular outflow tract was noted in both REV and Rastelli groups. There were no late deaths or reoperations in any group during follow-up. The modified Nikaidoh procedure is a better surgical option for transposition of the great arteries, ventricular septal defect, and pulmonary stenosis in terms of physiologic cardiac hemodynamics. Its long-term benefits need to be evaluated with a larger number of patients and longer follow-up.

Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction

During a 10-year period (1980 to 1990), 62 patients underwent complete repair for transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Twenty-two patients (35%) (mean age 8.1 +/- 7.2 years) underwent the Rastelli operation: The ventricular septal defect was enlarged anteriorly in eight patients, and right ventricular-pulmonary artery continuity was established with an extracardiac valved (9/22) or nonvalved (13/22) conduit. Forty patients (65%) (mean age 3.3 +/- 3.2 years) underwent the Lecompte modifications: The conal septum was extensively excised when present (30/40), anterior translocation of the pulmonary bifurcation was performed in 32 patients, and right ventricular-pulmonary artery continuity was established by direct anastomosis without a prosthetic conduit. There were seven early deaths (11%; 70% confidence limits, 7% to 17%): two after the Rastelli procedure (9%; 70% confidence limits, 3% to 20%) and five after the Lecompte operation (12.5%; 70% confidence limits, 7% to 20%). Four patients were lost to follow-up, yielding a 93% complete follow-up (mean follow-up 55 months). There were two late deaths (one in each group). Actuarial probability of survival (+/- standard error) at 5 years was 83% +/- 9% after the Rastelli operation and 84% +/- 6% after the Lecompte procedure. All long-term survivors (except one in the Rastelli group) were in functional class I. Five patients in the Rastelli group underwent late reoperation for obstruction of the extracardiac conduit (28%; 70% confidence limits, 16% to 42%). Three late reoperations (10%; 70% confidence limits, 4% to 19%) were required after the Lecompte operation (one for residual ventricular septal defect and two for residual pulmonary outflow tract obstruction). At most recent examination, residual pulmonary outflow tract obstruction was present in seven patients of the Rastelli group (39%; 70% confidence limits, 26% to 53%) and in six patients of the Lecompte group (19%; 70% confidence limits, 12% to 29%). The combined likelihood of reoperation for pulmonary outflow tract obstruction and residual pulmonary outflow tract obstruction was significantly higher in the Rastelli group (67% versus 26%; p = 0.005). Both procedures provide satisfactory early and late results. The Lecompte operation allows complete repair in infancy, is feasible in patients with anatomic contraindications to the Rastelli operation, and may reduce the need for reoperation and the prevalence of residual pulmonary outflow tract obstruction.

A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow

A new angiographic method for quantitative standardization of cross-sectional area of bilateral pulmonary arteries, the PA-index, and retrospective analysis of the PA-index in different types of operative procedures are presented. This study included 40 subjects in the normal control group, 46 patients in the tetralogy group, 26 patients in the Rastelli group, and 15 patients in the Fontan group. The normal value of the PA-index was 330 +/- 30 mm2/BSA and was consistent in a wide range of body surface areas from infancy to adolescence. The PA-index in the tetralogy and Rastelli groups ranged from 100 to 400 mm2/BSA. There were no early deaths in the tetralogy group, but the incidence of low cardiac output was higher in patients with a smaller PA-index, especially when the PA-index was less than 150 mm2/BSA. Low cardiac output was more severe in the Rastelli group. The operative mortality was significantly affected by the PA-index. In the Rastelli group, all of the patients with a PA-index of less than 200 mm2/BSA died, whereas the mortality rate in patients with a PA-index of more than 200 was only 6% (p less than 0.01). The mortality rate was not influenced by any other factors, such as aortic cross-clamp time or age at operation. In the Fontan group, two patients with a PA-index of less than 250 mm2/BSA died of severe heart failure, and 12 of 13 patients with a PA-index of more than 250 survived (p less than 0.01). Our results indicated the validity of the PA-index in predicting the postoperative prognosis of the various entities. In tetralogy, all patients with a PA-index over 100 mm2/BSA can undergo correction safely; in Rastelli operation, those with a PA-index under 200 should have a palliative procedure first, whereas those with a PA-index over 250 can be considered good candidates for the Fontan procedure. The PA-index may also serve a useful guide in comparing surgical results from different institutions with patients having anomalies of varying severity.