Dave R. Koolbergen

More Than 30 Years' Experience With Surgical Correction of Atrioventricular Septal Defects

BACKGROUND Outcome of surgical correction of atrioventricular septal defects (AVSD) still varies despite enhanced results. We reviewed our 30-year experience with AVSD repair and identified risk factors for mortality and reoperation. METHODS Between 1975 and 2006, 312 patients underwent surgery for complete AVSD (n = 209; 67.0%), partial AVSD (n = 76; 24.4%), or intermediate AVSD (n = 27; 8.6%). Mean age was 2.4 ± 3.9 years; 142 patients (45.5%) were younger than 6 months. Follow-up was 99.0% complete. RESULTS There were 26 in-hospital deaths (8.3%) and 6 late deaths (2.1% of 283). Estimated overall survival for the total study population was 91.3%, 90.6%, and 88.6% at 1, 5, and 15 years, respectively. In the multivariable logistic regression analysis, surgical era 1975 to 1995 (p < 0.001) and younger age (p = 0.004) were found to be independent risk factors for early mortality, whereas preoperative AV valve insufficiency showed a tendency toward statistical significance (p = 0.052). Of the hospital survivors, 43 patients required a late reoperation. Estimated freedom from late reoperation was 96.4%, 89.3%, and 81.8% at 1, 5, and 15 years, respectively. Multivariable Cox regression analysis showed associated cardiovascular anomalies (p < 0.001), left AV valve dysplasia (p < 0.001), and absence of cleft closure (p = 0.003) to be independent risk factors for late reoperation. CONCLUSIONS AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation.

Surgical versus percutaneous treatment of aortic coarctation: new standards in an era of transcatheter repair

Aortic coarctation is a common congenital cardiovascular defect, which can be diagnosed over a wide range of ages and with varying degrees of severity. Surgery has proven to be an effective treatment for the management of native aortic coarctation, and remains the treatment of choice in neonates. Balloon angioplasty with or without stenting has evolved rapidly over the past decade. Balloon angioplasty is the treatment of choice in children with re-coarctation, and currently available immediate results in native coarctation are similar with regards to gradient reduction as compared with surgery. However, both treatment options carry the risk of restenosis and aortic wall complications, especially after balloon angioplasty without stenting in native coarctation. On the other hand, stent implantation has shown excellent short-term results in both children beyond infancy and in adults with native coarctation. In patients with recurrent coarctation who are at high surgical risk, balloon angioplasty and stent repair offer a less invasive and equally effective method. Stent repair is preferred over balloon angioplasty in adults and outgrown children with a recurrent coarctation, as the risk for re-coarctation and aneurysm formation seems to be lower. Data with regard to long-term outcome after percutaneous treatment strategies are scarce. This review summarizes the current insights in the efficacy and safety of both surgical and transcatheter treatment options for aortic coarctation.

Long-Term Results of Reoperation for Left Atrioventricular Valve Regurgitation After Correction of Atrioventricular Septal Defects

BACKGROUND Long-term results of reoperation for left atrioventricular valve regurgitation (LAVVR) after previous correction of atrioventricular septal defect (AVSD) are scarce. We evaluated long-term outcome of reoperation for LAVVR and identified risk factors for reoperation. METHODS Between December 1976 and July 2006, 45 of 312 patients with correction of different AVSDs underwent reoperation for LAVVR. The cohort of 267 patients who did not need reoperation for LAVVR allowed for the identification of risk factors for reoperation and evaluation of overall survival after primary AVSD repair in a competing risk scenario. Clinical data were obtained by retrospective review. RESULTS The left atrioventricular valve (LAVV) was repaired in 31 patients (68.9%) and replaced in 14 (31.1%). There were 3 in-hospital deaths (6.7%) and 2 late deaths (4.4%). Estimated overall survival was 88.1% at 15 years after the reoperation, and estimated incidence of death after reoperation in the total patient cohort was 2% at 15 years after the primary AVSD repair. Overall survival was significantly higher after LAVV repair than after replacement (p=0.010). Ten patients with LAVV repair required a second reoperation for LAVVR. At follow-up, survivors were in New York Heart Association functional class I (n=36) or II (n=4). Independent risk factors for first reoperation for LAVVR were associated cardiovascular anomalies (p<0.001), LAVV dysplasia (p<0.001), and nonclosure of the cleft (p=0.027). CONCLUSIONS After previous correction of AVSD, LAVVR can usually be corrected by valve repair. A very dysplastic valve may necessitate replacement. Overall survival is higher after repair than after replacement. In general, overall survival of patients reoperated on for LAVVR is favorable. The overall mortality rate after primary repair of AVSD is explained only for a small part by mortality after reoperation for LAVVR.

Severe tricuspid regurgitation is predictive for adverse events in tetralogy of Fallot

Objective Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR. Methods In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included. Preoperative and postoperative imaging data and a composite of adverse clinical events (death, sustained ventricular tachycardia, heart failure, or supraventricular tachycardia) were collected. Multivariate Cox hazards regression analysis was used to determine which factors were predictive for adverse events after PVR. Results A total of 129 patients (61% men, age at PVR 32.9±10.4 years) were included. The composite endpoint occurred in 39 patients during 8.4±4.2 years of follow-up. In multivariate analysis, severe preoperative TR (HR 2.49, 95% CI 1.11 to 5.52), right ventricular end-systolic volume (HR 1.02/mL/m2, 95% CI 1.01 to 1.03) and age at PVR (HR 1.07/year, 95% CI 1.04 to 1.09) were predictive for adverse events. Early postoperative TR was not predictive for adverse events (p=0.96). In patients without any risk factor (age >40 years, right ventricular end-systolic volume >90 mL/m2 or severe TR), 5-year event-free survival was 100% as compared with 61% in patients with two or three risk factors. Conclusions In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR.

Determinants of Clinical Right Ventricular Failure After Congenital Heart Surgery in Adults

OBJECTIVES Right ventricular (RV) failure after cardiac surgery is a clinical entity with high morbidity and mortality. Patients with congenital heart disease (CHD) often undergo right-sided cardiac surgery. The authors aimed to identify determinants of RV failure after cardiac surgery to differentiate patients with increased risk. DESIGN A retrospective chart review. SETTING University hospital. PARTICIPANTS Adults with CHD operated on between January 2001 and January 2011. INTERVENTIONS Clinical characteristics, laboratory tests, surgical data, and intensive care unit outcome were obtained from medical records. MEASUREMENTS AND MAIN RESULTS The diagnosis of clinical RV failure was made by careful review of the medical records by 2 independent physicians. Patients only were identified as having RV failure if (1) they had elevated jugular venous pressure, (2) they had impaired postoperative RV function on transthoracic echocardiography, and (3) a diagnosis of RV failure was documented clearly in the medical charts by the treating physician. Data of 412 consecutive patients (median age 36 [range 18-74] years, 56% male) were studied. Eighteen patients had clinical RV failure (4.4%) postoperatively, of whom 6 patients died. Patients undergoing left- and both-sided surgery had an equal risk of developing clinical RV failure as compared with patients undergoing right-sided surgery. In multivariate logistic regression analysis, preoperative impaired RV function, supraventricular tachycardia, and cardiopulmonary bypass time >150 minutes were the strongest determinants of clinical RV failure (p<0.05, for all). CONCLUSIONS RV failure after cardiac surgery is a serious complication, and occurs regardless of the side of surgery. A tailored approach in patients with CHD at highest risk of RV failure should be considered.

Structural degeneration of pulmonary homografts used as aortic valve substitute underlines early graft failure.

OBJECTIVES The limited availability of donor valves and experimental evidence that pulmonary valves can withstand systemic pressure made us use cryopreserved pulmonary homografts as aortic valve substitutes. We observed a high incidence of early reoperation because of severe graft insufficiency due to cuspal tears. The mid-term results are evaluated in this study and histological analysis of explanted homografts is performed to investigate the cause of graft failure. METHODS From December 1991 to April 1994, 16 patients (13 male; mean age 37.3 years, range 21-59 years) underwent aortic valve replacement with a cryopreserved pulmonary homograft. The indication was endocarditis (n = 4), bioprosthesis degeneration (n = 3) or congenital aortic valve disease (n = 9). All homografts were implanted freehand in the subcoronary position. All patients were contacted for follow-up and recent echo-Doppler studies were reviewed. Six explanted homografts were examined microscopically using routine histological techniques to analyze changes in cell population, collagen and elastic fiber structure. RESULTS Follow-up was complete in all patients. Reoperation was required in ten patients because of severe graft incompetence (mean implantation time 5.9 years, range 2.8-8.0 years). In two patients, recurrent endocarditis was the cause of graft failure. In the other eight patients the leaflets looked pliable and thin with gross tearing in one or more cusps. The histopathologic changes observed were remarkably similar in all examined grafts: the cusp tissue was almost non-cellular and the collagen fiber structure had mostly disappeared. At the site of rupture, the tissue had become thin with strongly degenerated collagen and elastic fiber structure. In the six patients with a homograft remaining in situ, echo-Doppler showed trivial to mild insufficiency in five cases and moderate to severe in one case, whereas no significant gradients were observed. CONCLUSIONS We concluded that structural reduction of cell number and degenerative alterations in the molecular composition of the extracellular matrix in valve tissue is the main cause of early graft failure in this series. The use of cryopreserved pulmonary homografts in the systemic circulation is therefore not advised.

Results of surgical repair of atrioventricular septal defect with double-orifice left atrioventricular valve

OBJECTIVE The outcome of surgical correction of atrioventricular septal defect with double-orifice left atrioventricular valve has improved in recent years but is still reported to be associated with high mortality and reoperation rates. Controversy exists about the management of the accessory orifice. We evaluated our results with correction of atrioventricular septal defect with double-orifice left atrioventricular valve. METHODS Between 1975 and 2006, 21 patients underwent correction of atrioventricular septal defect with double-orifice left atrioventricular valve. Clinical data were obtained by means of retrospectively reviewing inpatient and outpatient medical records. To evaluate the influence of double-orifice left atrioventricular valve on mortality and the need for reoperation, a comparison was made with 291 consecutive patients who, during the same period, underwent correction of atrioventricular septal defect without double-orifice left atrioventricular valve. RESULTS None of the 21 patients with double-orifice left atrioventricular valve had undergone a previous operation. The accessory orifice was managed with different techniques depending on the severity of the regurgitation. There was no in-hospital mortality, and there were 3 late deaths. Seven patients required 12 reoperations, 7 for left atrioventricular valve insufficiency. Double-orifice left atrioventricular valve had no influence on mortality but was a significant predictor for reoperation compared with repair of atrioventricular septal defect without double-orifice left atrioventricular valve. At the latest follow-up, all 18 survivors were in New York Heart Association functional class capital I, Ukrainian without medication. Only 1 patient showed residual mild left atrioventricular valve insufficiency. CONCLUSION Atrioventricular septal defect with double-orifice left atrioventricular valve can be repaired with low mortality. However, double-orifice left atrioventricular valve is a predictor for reoperation. The accessory orifice is often competent and should then be left untouched. If regurgitation of the accessory orifice is present, this is best managed with suture or patch closure.

A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect

OBJECTIVES To evaluate our experience with patients reoperated after primary repair of atrioventricular septal defect (AVSD) and identify predictors of poor outcome. METHODS Between 1976 and 2014, 69 patients were reoperated after primary repair of partial (n = 28), intermediate (n = 15) or complete (n = 26) AVSD. RESULTS Median age at first reoperation was 62.4 (range, 1.6-845) months, median interval to first reoperation was 22.3 (range, 0.2-598) months. Main indications for first reoperation included left atrioventricular valve (LAVV) pathology (66%), residual septal defect (19%) and left ventricle outflow tract obstruction (LVOTO; 4%). Procedures to address LAVV pathology included various valvuloplasties in 47 (77%) patients and valve replacement in 14 (23%) patients. A second, third, fourth and fifth reoperation was required in 27, 12, 4 and 1 patient, respectively. Most common procedures were LAVV replacement (LAVVR), LVOTO relief, pacemaker implantation and right atrioventricular valve procedure. Freedom from reoperation after LAVV valvuloplasty (LAVVP) was 84 and 62% at 1 and 10 years, respectively. There were 10 early and 4 late deaths. Estimated overall survival at 1, 5 and 10 years was 87, 83 and 83%, respectively. Double orifice LAVV (DOLAVV) was a risk factor for in-hospital and overall mortality [odds ratio (OR) = 14.5; 95% confidence interval (CI) = 1.2-178.7; P = 0.037 and hazard ratio (HR) = 6.8; 95% CI = 1.5-31.7; P = 0.015, respectively]. Patients with LAVVP and LAVVR differed significantly in overall survival (P = 0.014). At the last follow-up (median, 9.8; range, 0-34 years), 84% survivors were in New York Heart Association Class I or II. CONCLUSIONS Many patients reoperated after primary AVSD repair needed surgical reintervention. LAVV pathology was the most common indication for reoperation. DOLAVV was a risk factor for mortality. Particular AVSD type did not appear to be a risk factor for mortality or LAVVP failure. There is some evidence for the close relationship between LAVV pathology and LVOTO in subjects undergoing reoperation after primary AVSD repair as some patients with initial LAVV problems needed LVOTO repair later on and vice versa.

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

OBJECTIVES 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. METHODS 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. RESULTS 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. CONCLUSIONS 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.

Individualised prediction of pulmonary homograft durability in tetralogy of Fallot

Background In patients with repaired tetralogy of Fallot (rTOF), multiple reoperations or percutaneous interventions after pulmonary valve replacement (PVR) may be necessary due to limited homograft durability. However, data to guide individualised prediction of homograft durability remain scarce. The aim of this study was to provide risk models for RV to pulmonary artery homograft durability. Methods This retrospective multicentre study included consecutive patients with rTOF who had undergone PVR at an age of >12 years. Homograft dysfunction was defined as at least moderate pulmonary regurgitation (PR) or pulmonary stenosis (PS) (pressure gradient ≥36 mm Hg) as assessed by echocardiography. Reintervention was defined as percutaneous intervention or redo-PVR. Results A total of 153 patients with rTOF were included (62% male, mean age at PVR 31±11 years, pulmonary homograft 96%, follow-up 9.6 years (IQR 5.9, 13.3)). Average freedom from homograft dysfunction and reintervention after 10 years was 74% and 89%, respectively. In multivariable Cox proportional hazards analysis, postoperative PS ≥20 mm Hg (HR 6.52, 95% CI 3.09 to 13.7), postoperative PR ≥ grade 1 (HR 3.13, 95% CI 1.45 to 6.74) and age at PVR <18 years (HR 3.52, 95% CI 1.64 to 7.53) were independently predictive for homograft dysfunction. In patients without any risk factor, 10-year freedom from homograft dysfunction and reintervention was excellent (91% and 96%, respectively) in contrast to patients with ≥2 risk factors (25% and 73%, respectively). Conclusions Individualised prediction of homograft durability in patients with rTOF can be guided by early postoperative echocardiography. In adult patients without early postoperative PS or PR, homograft dysfunction and reintervention are unlikely to occur within 10 years, and follow-up may be less stringent.