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Ann Thorac Surg 1998;66:1383-1388
© 1998 The Society of Thoracic Surgeons

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Original articles: cardiovascular

Perioperative conduction and rhythm disturbances after the Ross procedure in young patients

^a Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^b Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^c Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
^d Department of Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA

Accepted for publication April 25, 1998.

Address reprint requests to Dr Rhodes, Division of Cardiology, The Children’s Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104
e-mail: (rhodes{at}Email.chop.edu)

	Abstract

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
Background. The Ross procedure is performed for a variety of left ventricular outflow tract diseases in children. The preoperative hemodynamic burden of pressure or volume overload and associated ventricular hypertrophy can predispose to ventricular arrhythmias. Additional procedures performed with the Ross procedure (eg, Konno) may damage the conduction system.

Methods. Between January 1995 and February 1997, the Ross procedure was performed in 42 patients, 31 (74%) of whom had 71 prior interventions. Concomitant procedures (n = 42 in 23 patients) included 17 annular-enlarging procedures. Screening was performed for perioperative conduction and rhythm abnormalities.

Results. There was one postoperative death. Perioperative ventricular tachycardia occurred in 12 patients (29%), with 2 receiving antiarrhythmic medication for ventricular tachycardia at discharge. Transient complete heart block occurred in 3 patients, all of whom had concomitant procedures performed in the subaortic area; all patients were discharged in sinus rhythm and no patient received a permanent pacemaker.

Conclusions. The Ross procedure can be performed successfully in children with complex cardiac disease with low mortality and perioperative morbidity. The incidence of perioperative ventricular tachycardia is high (29%), suggesting the need for vigilant perioperative monitoring and long-term surveillance.

	Introduction

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
In patients with significant aortic valve disease, ventricular arrhythmias are a common finding both before and after intervention [1]. Ventricular arrhythmias have been implicated as a cause of sudden death in patients with unrepaired aortic stenosis (AS) or aortic insufficiency, [2–4] after operation for critical AS in infants [5] and prosthetic aortic valve replacement [4, 6, 7], and late after aortic valvotomy [8]. The operative approaches to aortic valve lesions that cause ventricular pressure or volume load may not completely alleviate the potential for serious cardiac arrhythmias, even after relief of the stenosis or regurgitation.

The Ross procedure has become the operative management of choice for a variety of left ventricular outflow tract diseases in children and young adults [9–14]. In addition to the underlying potential for ventricular arrhythmias in these patients, the Ross procedure itself may introduce additional factors that are proarrhythmic. Excision of the pulmonary root may damage the septal perforating branches of the left anterior descending coronary artery, which may lead to areas of myocardial ischemia and ventricular ectopy [15]. Explantation and reimplantation of the coronary arteries at the time of the Ross procedure may potentially lead to areas of myocardial ischemia and rhythm abnormalities. Finally, annulus-enlarging procedures (eg, Konno) or muscle resection in the subaortic area performed with the Ross procedure may damage the conduction system [16]. The purpose of this review is to report our experience in children and young adults who have undergone the Ross procedure, with specific attention to perioperative conduction and rhythm disturbances.

	Material and methods

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
Patient demographics
Patient records and operative notes were reviewed on all patients undergoing the Ross procedure at The Children’s Hospital of Philadelphia between January 1995 and February 1997 (n = 42). Patients were defined as having "complex" left ventricular outflow tract disease if there were multiple abnormalities including two or more of the following: valvar AS or regurgitation, subvalvar AS, supravalvar AS, aortic arch hypoplasia, coarctation of the aorta, or interrupted aortic arch. The mean age at theRoss procedure was 11.7 ± 7.1 years (median, 10.4 years; range, 5 months to 26.6 years); there were 31 male and 11 female patients. The primary anatomic abnormality before any intervention was as follows:

Valvar disease (n = 28) Valvar AS 18 Valvar aortic insufficiency 5 Valvar AS and insufficiency 5 Complex left ventricular outflow tract disease (n = 11) Valvar AS and coarctation 4 Valvar AS and aortic arch hypoplasia 1 Valvar AS and supravalvar AS 1 Valvar AS and sub-AS and supravalvar AS 1 Subaortic stenosis and coarctation 1 Interrupted aortic arch 3 Other (n = 3) Hypertrophic cardiomyopathy 1 Ventricular septal defect 2

Seventy-six prior interventions were performed in 31 patients at multiple institutions:

Aortic valve (n = 33) Aortic valvotomy 16 Aortic valve balloon dilation 11 Aortic valvuloplasty 4 Aortic valve replacement 2 Aortic arch (n = 21) Native coarctation repair 3 Native coarctation balloon dilation 1 Recoarctation repair 2 Recoarctation balloon dilation 8 Interrupted aortic arch repair 2 Ascending to descending aorta jump graft/pulmonary artery band 1 Ascending to descending aorta jump graft (redo) 1 Aortic arch augmentation 3 Subaortic (n = 8) Subaortic membrane resection 6 Subaortic membrane balloon dilation 1 Konno procedure 1 Other (n = 14) Apical aortic conduit 2 Mitral valvuloplasty 1 Ventricular septal defect closure (n = 5) Primary 3 Residual 2 Patent ductus arteriosus ligation 1 Patent ductus arteriosus device closure 1 Pulmonary artery band 1 Pulmonary artery band removal 1 Pulmonary artery band removal/PA-plasty 1 Ascending aortic aneurysm resection 1 Total 76

The hemodynamic indications for the Ross procedure were as follows:

Valvar AS 2 Aortic insufficiency 16 Mixed AS and aortic insufficiency 13 Complex left ventricular outflow tract disease (n = 11); 5/11 had concomitant aortic insufficiency Valvar AS and Sub-AS 3 Valvar AS and supravalvar AS 1 Valvar AS and coarctation 1 Valvar AS and sub-AS and supravalvar AS 2 Valvar AS and sub-AS and arch hypoplasia 1 Sub-AS and arch hypoplasia 2 Sub-AS and coarctation 1

Although only 5 patients had aortic insufficiency at their initial presentation, a total of 34 (81%) had aortic insufficiency as a significant component of their disease at the time of the Ross procedure. Additional procedures were performed in conjunction with the Ross procedure in 23 patients, including annular enlargement in 17:

Annulus-enlarging procedure—Konno 9 Annulus-enlarging procedure—other 8 Subaortic membrane resection 7 Aortic arch augmentation 4 Mitral valvuloplasty 3 Residual ventricular septal defect closure 3 Apical aortic conduit closure 2 Pulmonary autograft plication 2 Coronary arterioplasty 2 Aortic annuloplasty 1 Septal myectomy 1 Total 42

The mean total cardiopulmonary bypass time was 112 ± 25 minutes (median, 110; range, 78 to 183 minutes), and the total aortic cross-clamp time was 83 ± 18 minutes (median, 80; range, 53 to 128 minutes). Deep hypothermic circulatory arrest was used in 4 patients during arch reconstruction for a mean of 25 ± 6 minutes (median, 24; range, 19 to 32 minutes).

Rhythm surveillance
The perioperative course was initially monitored for conduction and rhythm abnormalities by surface electrocardiography (ECG) (Table 1) and continuous bedside monitoring. All available ECGs were evaluated for rate, rhythm, conduction intervals, voltages, ST segment, and T wave morphology. Standard age-corrected ECG criteria for conduction intervals, durations, and chamber hypertrophy were used for interpretation. Preoperative ECGs were performed at a mean of 1.1 ± 0.5 days (range, 1 to 3 days) before the Ross procedure. Postoperative ECGs were performed within 24 hours after the Ross procedure. Discharge ECGs were performed at hospital discharge at a mean of 5.5 ± 4.9 days (range, 2 to 20 days) after the Ross procedure. Twenty-four-hour continuous bedside and ambulatory monitoring (telemetry) were used to assess the presence of tachyarrhythmias and ectopy in the postoperative period.

	Results

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
Mortality
The only death was the first patient in this series, a 6-year-old boy with AS, aortic insufficiency, and arch hypoplasia with severe left ventricular hypertrophy. He died suddenly on postoperative day 3 from presumed arrhythmia after a previously unremarkable course after a Ross/Konno procedure with arch reconstruction.

Sinus node function
Preoperative findings
Electrocardiograms revealed sinus rhythm in 91% of patients (see Table 1) and sinus bradycardia for age in 12%, with heart rates ranging from 48 to 59 beats/min. Ectopic atrial rhythm was present in 3 patients (range, 55 to 84 beats/min). Holter monitoring revealed sinus rhythm in 100% of patients (see Table 2) and sinus bradycardia for age in 52% with low heart rates ranging from 38 to 57 beats/min.

Postoperative findings
In the immediate postoperative period, ECGs revealed normal sinus rhythm in 90% of patients, 1 had junctional escape rhythm (78 beats/min), and 3 were paced for complete heart block (CHB).

Discharge findings
All patients had sinus rhythm at the time of hospital discharge on ECG and Holter monitoring. Sinus bradycardia for age was detected in 5% of ECGs.

Atrioventricular node function
Preoperative findings
First-degree AV block was detected on ECGs in 5% of the patients. No higher grade AV block was seen. No AV block was seen on Holter monitoring.

Postoperative findings
In the immediate postoperative period, ECGs revealed 15% of patients had first-degree AV block and 3% had CHB. Two additional patients (6%) were noted to have transient CHB by bedside monitoring. All 3 patients with CHB had procedures performed on the subaortic area in addition to the Ross procedure. The first patient, a 19-year-old with first-degree AV block on preoperative ECG, underwent a fibrous subaortic membrane resection at the time of the Ross procedure. In the immediate postoperative period this patient had transient CHB lasting 5 hours that required temporary ventricular pacing. At time of discharge the patient had first-degree AV block. The second patient, a 6-year-old with right bundle-branch block (RBBB) on preoperative ECG, experienced CHB with a rate of 40 beats/min on postoperative day 2 after a Ross-Konno procedure, requiring ventricular pacing. The CHB resolved during the subsequent 12 hours, and the patient was discharged with RBBB. The third patient, a 10-year-old who had preoperative RBBB on his ECG, underwent a Ross/Konno procedure and was noted to have bifascicular block immediately postoperatively; CHB with a ventricular rate of 84 beats/min was noted 2 days postoperatively. Temporary pacing was not performed secondary to adequate escape rate and AV conduction returned to normal before discharge. The patient was discharged on postoperative day 5 with bifascicular block. All 3 patients were discharged in sinus rhythm, and remain in sinus rhythm from 7 to 17 months after the operation.

Discharge findings
Electrocardiograms revealed 14% of patients with first-degree AV block, with a similar incidence on Holter monitoring (15%).

His/Purkinje function
Preoperative findings
Electrocardiograms revealed RBBB in 7% of patients, all of whom had previous surgical procedures. Left bundle-branch block was not seen.

Postoperative findings
Electrocardiograms revealed RBBB in 8% of patients. All patients with postoperative RBBB had documented RBBB preoperatively. One of the patients with RBBB who underwent a Ross/Konno, ventricular septal defect closure, aortic arch augmentation, and subaortic membrane resection experienced bifascicular block. Left bundle-branch block occurred in 5% of patients, 1 after a Ross/Konno procedure, and 1 after a Ross procedure with concomitant septal myotomy/myomectomy. Thus 3 of 24 (13%) patients with an annulus-enlarging procedure or subaortic resection incurred damage to their left bundle branch. None of the patients with isolated subaortic membrane resection with their Ross procedure had left bundle-branch block. Nonspecific intraventricular conduction delay was seen in 23% of patients.

Discharge findings
No significant changes were noted on discharge ECGs as compared with postoperative ECGs.

Supraventricular ectopy
Preoperative findings
Isolated premature atrial contractions were not seen on ECGs; however, 1 patient had nonsustained supraventricular tachycardia (SVT) with a rate of 204 beats/min. Premature atrial contractions were seen on Holter monitoring, ranging from occasional to frequent in 44% of patients, and nonsustained SVT was seen in 7% of patients with rates ranging from 100 to 160 beats/min.

Postoperative findings
Electrocardiogram revealed premature atrial contractions in 5% of patients. Supraventricular tachycardia was documented by bedside monitoring in 2 patients.

Discharge findings
No atrial ectopy or SVT was noted on discharge ECGs. Holter monitoring revealed premature atrial contractions ranging from occasional to frequent in 62% of patients and nonsustained SVT in 8%, with rates ranging from 140 to 192 beats/min. Both patients with postoperative SVT had documented SVT preoperatively.

Ventricular ectopy
Preoperative findings
Premature ventricular contractions were not seen in any patient on preoperative ECGs (Table 5). However, Holter monitoring revealed occasional to frequent uniform premature ventricular contractions in 59%, multiform premature ventricular contractions in 30%, and ventricular couplets in 11% of patients. Idioventricular rhythm and nonsustained ventricular tachycardia (VT) were detected in 1 patient (4%) each.

Postoperative findings
No ventricular ectopy was noted on postoperative ECGs.

Discharge findings
Similarly, no ventricular ectopy was noted on discharge ECGs. However, Holter monitoring revealed occasional premature ventricular contractions in 65%, multiform premature ventricular contractions in 35%, and ventricular couplets in 15% of the patients, similar to the preoperative findings. Idioventricular rhythm was noted in 12% and nonsustained VT in 4%.

Although nonsustained VT was rarely seen in patients on preoperative (4%) or discharge (4%) Holter monitoring, postoperative bedside continuous monitoring detected nonsustained VT in 29% of patients. Most (10 of 12, 83%) patients had VT only in the first 24 hours after the operation, but 2 patients had 8 to 12 episodes of recurring nonsustained VT throughout their postoperative course. The first patient was a 10-year-old with multiple 5- to 6-beat runs of VT who was treated with mexiletine with a reduction in the frequency of ventricular ectopy and tachycardia. The other patient was a 20-year-old with persistent 3- to 6-beat runs of VT who was discharged on mexiletine. The patient’s regimen was subsequently changed to amiodarone because of continued episodes of nonsustained VT.

Patients with postoperative VT were significantly older at the Ross procedure (n = 12; mean age, 15.0 ± 4.5; median, 16.2; range, 3.6 to 20 years) compared with patients without VT (n = 30; mean age, 10.3 ± 7.5; median, 7.8; range, 0.35 to 26.6 years; p = 0.017). No other risk factors for postoperative VT were identified, including the number or type of prior interventions, total cardiopulmonary bypass time (VT, 105.1 ± 39.8; non-VT, 111.2 ± 24.8 minutes), length of aortic cross-clamp time (VT, 83.6 ± 23.0; non-VT, 82.7 ± 16.9 minutes), or number or type of concomitant procedures at the time of the Ross procedure.

	Comment

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
We found that ventricular arrhythmias are common after the Ross procedure with short episodes of nonsustained VT seen in 29% of the study group. This nonsustained VT was limited to the first 24 hours after the operation in all but 2 patients. The only variable identified that predicted postoperative nonsustained VT was older age at operation. As postoperative ventricular ectopy has been previously reported to occur after other types of aortic valve operations [17, 18], our findings suggest that the underlying hemodynamic burden, and possibly a longer period of pressure or volume load on the left ventricle, may predispose patients to postoperative ventricular arrhythmias rather than specific aspects of the Ross procedure itself.

In Ross’ group’s early experience [15], 2 of 18 (11%) patients had SVT and 5 of 18 (27%) patients had "ventricular dysrhythmias." In 3 (17%) of these patients death was attributed to ventricular dysrhythmias. In a more recent report of the 20-year follow-up of Ross’s group’s surgical experience involving 241 patients [12], there had only been one additional early death and one late death attributed to arrhythmias since the early experience reported in 1971 [15]. This dramatic change in arrhythmia-related mortality was most likely caused by the realization of the importance of preserving the first septal branch of the left anterior descending coronary artery when harvesting the pulmonary root [15]. Other more recent studies have not reported a high incidence of perioperative arrhythmias. Elkins and colleagues [10] reported one death secondary to a "fatal arrhythmia." Other groups have reported isolated cases of "transient atrial" [19] and ventricular [19, 20] arrhythmias. The higher incidence of perioperative ventricular tachycardia in our study is possibly related to the methodology of daily screening for ventricular ectopy on bedside monitoring. However, this high frequency of ventricular ectopy could also be attributed to the complexity and heterogeneity of the patient population, with most patients having multiple prior procedures, and many having concomitant procedures at the time of the Ross procedure.

The incidence of CHB after isolated aortic valve replacement, either with human tissue valves or mechanical prostheses, has ranged from 0% to 6% [12–14, 21–23]. Cartier and coworkers [22] reported 3 of 64 (5%) patients who underwent the Ross procedure experienced CHB, Randolph and associates [23] reported 2 of 57 (4%) patients required a pacemaker for CHB, and Kouchoukos and colleagues [14] reported 1 of 23 (4%) patients received a pacemaker for CHB after the Ross procedure. Several causes for CHB after the Ross procedure have been suggested, including damage to the septal perforating coronary artery branches resulting in septal infarction [24], removal of subaortic fibrous tissue [25], and a concommitant Konno modification for annular enlargement [16, 26, 27]. Although the incidence of CHB after subaortic stenosis resection or an isolated Konno procedure has been reported to range between 0% and 8% [28–33], the previously reported incidence of CHB with a combined Ross-Konno procedure has been reported to be as high as 9% to 14% [16, 34].

In our series, permanent CHB did not occur, despite 17 of 42 (41%) patients having annulus-enlarging procedures. Although 3 of these 17 patients had transient CHB, the timing of presentation of the heart block was variable, occurring from 5 hours to 2 days after the procedure, as previously described, and was transient in all cases. Although all of our patients were discharged in normal sinus rhythm, the variability in timing and potential risks of the various rhythm and conduction abnormalities stresses the need for continuous ECG monitoring after the Ross procedure.

This study is a descriptive account of ECG abnormalities noted in a very heterogeneous patient population. The analysis of risk factors is therefore limited by the complexity of the congenital heart diseases and the variety of approaches to medical and surgical management before the Ross procedure. The relatively small sample size limits the ability to perform reliable multivariate analysis on many of the variables that may contribute to postoperative arrhythmias. An additional limitation in this report is that preoperative and postoperative Holter monitoring was not performed in the first 15 patients, limiting our ability to establish "baseline" characteristics of the conduction system in all patients.

In conclusion, the Ross procedure can be performed successfully in children and young adults with complex cardiac disease with low mortality and perioperative morbidity. The incidence of perioperative ventricular arrhythmias is high (29%), possibly related to older age at operation, with a small subset of patients receiving treatment for ventricular arrhythmias at hospital discharge (n = 2; 5%). This suggests the need for vigilant perioperative monitoring and long-term surveillance of this group of patients. Because older age at the Ross procedure may be a risk factor for perioperative ventricular arrhythmias, and the Ross procedure can be performed safely in younger patients, it may be prudent to consider earlier timing of surgical intervention to potentially decrease the incidence of ventricular arrhythmias. Although CHB may occur with annulus-enlarging procedures or subaortic resection, this was a rare finding and transient in all patients, with no patient receiving pacemaker placement. Long-term prospective ECG monitoring is needed to determine the late sequelae of these perioperative rhythm and conduction abnormalities.

	Footnotes

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
¹ Doctor Bockoven’s current address is Children’s Medical Center, Wright State University School of Medicine, Dayton, OH 45404-1958.

	References

Top Footnotes Abstract Introduction Material and methods Results Comment References

 

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