Cover Page. The handle holds various files of this Leiden University dissertation.

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1 Cover Page The handle holds various files of this Leiden University dissertation. Author: Hoohenkerk, Gerard Joannes Franciscus Title: Surgical correction of atrioventricular septal defect Date:

2 28 Years Experience With Transatrial- Transpulmonary Repair of Atrioventricular Septal Defect With Tetralogy of Fallot Gerard J. F. Hoohenkerk¹, MD, Paul H. Schoof¹, MD, PhD, Eline F. Bruggemans¹, MS, Marry Rijlaarsdam², MD, PhD, and Mark G. Hazekamp¹, MD, PhD. Departments of Cardiothoracic Surgery¹, and Pediatric Cardiology², Leiden University Medical Center, Leiden, the Netherlands. Annals of Thoracic Surgery2008;85: Pediatric Cardiac Surgery 81

3 Abstract Background. The outcome of surgical correction of atrioventricular septal defect and tetralogy of Fallot has improved in recent years but is still reported to be associated with high mortality. Controversy exists about the need of a right ventriculotomy or a right ventricular to pulmonary artery conduit. The purpose of this study was to evaluate our results of atrioventricular septal defect and tetralogy of Fallot repair by transatrialtranspulmonary approaches. Methods. Between 1979 and 2007, 20 consecutive patients underwent correction of atrioventricular septal defect and tetralogy of Fallot. Five patients had undergone prior palliative shunts. In all patients, a transatrialtranspulmonary approach was used and repair was accomplished without a conduit. The two-patch technique was used to correct the atrioventricular septal defect. Clinical data were obtained by retrospective review of inpatient and outpatient clinical charts. Results. There was no in-hospital mortality and one late, noncardiac death. Six patients required eight reoperations, six for left atrioventricular valve insufficiency (repair: n = 4; replacement: n = 2), one for residual ventricular septal defect, and one for pulmonary artery branch obstruction. Follow-up was complete for all patients (median, 17 years; range, 1.5 to 28 years). All 19 survivors were in good clinical condition at last control, without medication, and in New York Heart Association class I (n = 18) or II (n = 1). Transesophageal echocardiography revealed good right ventricular function, low right ventricular outflow tract gradients (mean, 9 ± 7.4 mm Hg), and trace pulmonary valve insufficiency (n =11). Conclusions. Atrioventricular septal defect and tetralogy of Fallot can be repaired with low mortality by the transatrialtranspulmonary approach without the use of a conduit. 82

4 Introduction The combination of complete atrioventricular septal defect and tetralogy of Fallot (cavsd- TOF) is a well-recognized congenital heart malformation [1 4]. Although surgical mortality has decreased in the last decade, postoperative complications such as residual ventricular septal defect, incompetence of the left atrioventricular (AV) valve, residual right ventricular outflow tract obstruction, and pulmonary regurgitation do occur and sometimes require reoperation [5, 6]. Concerning these complications, controversy exists about the best surgical approach [7 13]. This discussion is related to the use of a right ventriculotomy, cleft closure of the left AV valve, and the best way to reconstruct the right ventricular outflow tract [14]. Other controversies concern the use of palliative procedures and the timing of complete repair [15 18]. We evaluated our 28 years experience with total correction of cavsd-tof, in which a transatrial-transpulmonary approach without a valved conduit was used in all patients. Patients and Methods Patients The study was approved by the ethics committee of our institution, and because of its retrospective nature, patient consent was waived. Between January 1979 and January 2007, 20 consecutive patients with cavsd-tof could be identified for inclusion in this study. Patients with cavsd pulmonary atresia were excluded. Patient characteristics are summarized in Table 1. 83

5 Twelve patients had Down syndrome. Major extracardiac anomalies were not present. Palliative procedures consisted of a systemic-to-pulmonary artery shunt and were performed in 5 patients. All other patients underwent total correction at the initial procedure. Diagnosis was established by echocardiography or angiography dependent on the era of operation. Right ventricular outflow tract obstruction with infundibulum stenosis was present in 19 patients. Eighteen patients had pulmonary valve stenosis. Operations were performed by 4 surgeons. Operative Technique Repair was performed with the aid of cardiopulmonary bypass with moderate systemic hypothermia and cold crystalloid cardioplegia (St. Thomas s solution). Atrioventricular septal defect repair. A two-patch technique and a transatrial approach were used for AVSD repair in all patients. Different patches were used, including autologous pericardium (n = 5), xenograft pericardium (n = 3), or Dacron patch (n = 12). The left AV valve cleft was closed in 15 patients using interrupted sutures. Except for 2 patients, the coronary sinus was left draining into the right atrium. Right ventricular outflow tract. Right ventricular outflow tract enlargement was performed by a transatrialtranspulmonary approach in all patients. Infundibular stenosis was relieved through the pulmonary valve in the absence of a valvular stenosis. In the presence of a valvular obstruction, the valve was incised by extending the longitudinal incision in the pulmonary trunk. In all patients, the incision did not extend for more than 3 to 4 mm into the right ventricular myocardium. Whenever possible, the valve leaflets were spared by opening the pulmonary annulus through a commissure. Infundibulum obstruction was resected thereafter. In all patients, a transannular patch was used. Transannular patches were intentionally kept small to avoid pulmonary insufficiency. Homografts or other valved conduits were not used. Data Acquisition and Statistical Analysis Patient data were collected by reviewing both inpatient and outpatient medical records, including operative lists, patient databases, hospital records, and cardiac catheterization and echocardiographic reports. Recent echocardiographic Doppler studies were present for all surviving patients. All data were compiled in a computerized data bank and analyzed with the statistical computing package SPSS (SPSS Inc, Chicago, IL). 84

6 Results Follow-up data were complete for all 20 patients (median follow-up, 17 years; range, 1.5 to 28 years). Total follow- up was 300 patient-years. Timing of Repair The timing of correction of cavsd-tof has moved to earlier repair (Fig 1). An unpaired Student s t test revealed that mean age at repair before 1990 was significantly higher (34.6 ± 18.5 months) (p = ) compared with mean age at repair after 1990 (15.1 ± 11.9 months). Mortality There was no early mortality. There was one late, noncardiac, death, 4 years after repair of cavsd-tof, as a result of a traffic accident. 85

7 Reoperation Six patients required eight reoperations. Four patients received one reoperation, and 2 patients had two reoperations. Median interval for the first reoperation was 1.8 years after initial repair (range, 4.5 months to 26 years). Only 2 patients underwent a reoperation within 1 year after initial repair. Left AV valve incompetence was the most frequent indication for reoperation (six of eight reoperations). In 3 patients, the cleft had not been closed at initial repair, and in 1 patient the valves had been noted to be dysplastic at first surgery. At first reoperation, the cleft of the left AV valve was closed in 3 patients, and an annuloplasty by plication of a lateral commissure was performed in 1 patient. In 1 patient in whom the left AV valve was observed to be dysplastic, replacement was necessary at first reoperation. In 1 patient who had two reoperations for left AV valve incompetence, the valve had to be replaced at the second reoperation. Other indications for reoperation were residual ventricular septal defect (n = 2), residual or recurring right ventricular outflow tract obstruction (n = 2), branch pulmonary artery obstruction (n = 1), and pulmonary incompetence (n = 1). One patient required a pacemaker implantation after the second reoperation. There was no mortality related to reoperation. A summary of reoperation data per patient is given in Table 2. The Kaplan Meier curve for freedom of reoperation is shown in Figure 2. Freedom from reoperation at 25 years was 71%. The sharp decline in the curve at 26 years is caused by the patient with the longest follow-up (28 years), who had to be reoperated on 26 years after initial repair. At last follow-up, transesophageal echocardiography revealed residual or remaining left AV valve insufficiency to be present in 1 patient. Right ventricular outflow tract gradients were low (mean, 9 ± 7.4 mm Hg). Trivial pulmonary valve insufficiency was present in 11 patients, and pulmonary insufficiency was absent in all others. Right ventricular function was good. All 19 survivors were in good clinical condition at last control, without 86

8 medication and in New York Heart Association class I (n = 18) or class II (n = 1). All patients were in sinus rhythm (1 had a pacemaker). Comment In the last decade, different opinions have developed regarding the appropriate timing for complete repair of cavsd-tof. As in many other centers, timing of correction for congenital heart disease in our institution has moved to earlier primary repair with good results. Total correction of cavsd-tof remains a surgical challenge. Several factors are thought to contribute to morbidity and mortality after repair of cavsd-tof. These include AV valve incompetence, pulmonary valve incompetence, residual shunts, and right ventricular outflow tract obstruction. Since 1969, more than 38 studies of complete cavsd-tof repair have been published. The majority of these studies consist of small patient groups, usually operated on with different surgical strategies and with short follow- up. In reports of earlier years, operative mortality was high, ranging from 29% to 40% [19 21]. During the last decade, reports have shown a reduction in mortality. The mortality rate of our series corresponds with the low mortality rate found in other more recent studies, in which the mortality ranged from 0% to 11% [22 25]. This improvement can be attributed to several factors, including perioperative transesophageal 87

9 echocardiography and improved perioperative care. As in early reports, left AV valve incompetence remains a postoperative problem, probably owing to multifactor causes. This study encompasses a single-institution experience in a period of 28 years. Our two-patch technique to repair cavsd has not been changed throughout these years and is not different for cavsd-tof. The main complication has been residual or recurrent incompetence of the left AV valve for which reason 6 of 20 patients (30%) required reoperation. Residual left AV valve insufficiency occurred significantly more in the earlier years of our experience. Nonclosure of the cleft was the reason for reoperation in 3 patients, and important AV valve dysplasia had been noted in another patient. Dehiscence of a previously closed cleft occurred in 1 patient. It has been reported by others that initial closure of the cleft of the left AV valve prevents later reoperations [26, 27]. When reoperation is required, repair of the AV valve is possible in the majority of patients. Our current policy is to close the left AV valvular cleft in all cases. Discussion remains concerning the management of the right ventricular outflow tract in cavsd-tof. A right ventriculotomy allows a good exposure of the ventricular septal defect, and the infundibulum can be resected more easily than by a transatrial-transpulmonary approach. The potential drawbacks of a right ventriculotomy include postoperative ventricular dysfunction and late dysrhythmias. Retrospective reviews of dysrhythmia rates on transatrialtranspulmonary and RV approaches in cavsd-tof report an increased incidence of dysrhythmias associated with ventriculotomy [24, 28]. In our series, all patients were treated with a transatrial- transpulmonary approach. A transannular patch was used in all patients. Right ventricular failure and dysrhythmias were not observed during follow-up. Only 1 patient required a pacemaker. One of 20 patients required reoperation for pulmonary valve incompetence. The extent of incision in the right ventricular myocardium in the transatrial-transpulmonary approach is limited to no more than 3 to 4 mm proximal to the valve annulus. The ventricular septal defect can normally not be closed through this approach, and in our view the damage to the right ventricular myocardium is less than in a conventional right ventriculotomy. In conclusion, our method of repair of cavsd-tof has so far been associated with no cardiac-related mortality and good functional outcome in all survivors. Left AV valve incompetence is a factor affecting the reoperation rate. The transatrial-transpulmonary approach can be used to manage the cavsd-tof with a limited reoperation rate. Our current policy is to correct cavsd-tof electively at an age between 6 and 12 months, the main reason being sufficiently sized pulmonary arteries. A modified Blalock shunt would be placed (by sternotomy) if important cyanosis or cyanotic spells occur neonatally or in early infancy. 88

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