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Interact CardioVasc Thorac Surg 2008;7:84-89. doi:10.1510/icvts.2007.162941 © 2008 European Association of Cardio-Thoracic Surgery
Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase?
a Department of Cardiac Surgery, The Heart Centre, Rigshospitalet 2152, Blegdamsvej 9, 2100 Copenhagen, Denmark Received 7 July 2007; received in revised form 29 October 2007; accepted 30 October 2007
*Corresponding author. Tel.: +45 35 458741; fax: +45 35 452182.
The number of patients with grown-up congenital heart (GUCH) disease is steadily increasing. Although there is agreement that the medical service for GUCH patients should be expanded in coming years, it is still unknown whether this should also include the surgical service. In an attempt to elucidate this we reviewed our population of surgical GUCH patients (n=225) operated in our institution from 1998 to 2005. The patients' charts were reviewed. For details of the procedures, the hospital's internal database (PATS) was used. Patients were stratified according to diagnosis and complexity of the surgical procedures in a simple, moderate and complex category group. The yearly number of operations remained stable in the period. However, whereas the size of the simple complexity group was reduced in the study period, the size of both the moderate and the complex category groups increased progressively. Forty-four percent of the surgical procedures were reoperations. Morbidity and mortality (1.3%) were low. The present study suggests that the future surgical GUCH patients will be increasingly complex. However, it is speculative whether the total number of surgical GUCH patients will increase. This is especially explained by the continuous introduction of new percutaneous catheter techniques.
Key Words: Grown-up congenital heart disease; Heart; Adult surgery
Approximately 8 out of 1000 of live-born children have a congenital heart defect (CHD). Today up to 95% of these children are reported to survive to adulthood [1]. These patients form a group that is growing and becoming more important: grown-up congenital heart disease (GUCH) patients. Although there is broad agreement that the medical GUCH service should be expanded in coming years [2], it is still unknown whether such an expansion should also include the surgical GUCH service [3]. In an attempt to elucidate this further we reviewed our population of surgical GUCH patients operated in our institution in the period 1998–2005 according to diagnosis and complexity of the surgical procedures, and registered the development in these features during the observational period. It was further attempted to estimate how many of these surgical procedures that might have been performed using percutaneous techniques with the present and foreseeable technical availabilty.
Patients older than 15 years diagnosed with CHD who underwent surgery in the time period, from 1 January 1998 to 31 December 2005 in Copenhagen, were included in the study. The patients' charts were reviewed for various variables, including diagnoses, age, previous surgery, various surgeries performed, postoperative complication and admittance time. For parameters regarding details of the procedures, the hospital's internal database (PATS) was used. The anatomical diagnoses of the congenital heart defects were classified into simple, moderate, and complex categories according to the modified Canadian consensus conference criteria [4] as suggested by Srinathan et al. [1]. Further, to analyse the complexity of the operations themselves, the cardio-pulmonary bypass data were examined, acting as a surrogate marker of the technical demands of repeat procedures and complex reconstructions. Finally, patients were divided into groups according to their time of surgical procedure from 1st to 6th time operation and it was attempted to estimate how many of the surgical procedures that might be performed using percutaneous techniques in the near future given the present and foreseeing technical availability. Numbers are given as mean±S.D. when covering all patients and as median and range for sub-groups.
The number of GUCH patients operated for CHD in the years 1998–2005 at the Rigshospital is depicted in Fig. 1. Two hundred and twenty-five patients (113 men) had a total of 239 operations. The number of operations per year was rather stable over the years (Regression test: P=0.11 for slope different from 0). The patients' ages ranged from 16 to 77 years with the mean age at operation for the entire population being 37±16 years (Fig. 2). One hundred and thirty-three of the 239 surgical procedures were first time operations. One hundred and six (44%) patients had previously undergone from one to five previous surgical procedures (Fig. 3). Diagnosis, number of patients, age, cardio-pulmonary bypass (CPB) time, aortic cross-clamp time, mortality and length of ICU- and hospital stay are given in Table 1.
The simple complexity group consisted mainly of ASD patients. The majority of patients were in the moderate complexity category, where the largest groups were the TOF and CoA patients. The simple complexity group was reduced in the study period (P<0.05, Spearman), whereas the complex category group increased progressively in size (P<0.01) (Fig. 4). Furthermore, there was a trend towards an increase in the moderate lesion group (P<0.07).
Postoperative complications can be seen in Table 2. Early mortality was low, i.e. 3 out of 225 patients (1.3%) in 239 procedures. These three patients all belonged to the oldest age group aged 67, 72 and 77 years, respectively (Table 1).
It was estimated that from 79 to 111 (33–46%) of the 239 procedures might have been performed by percutaneous techniques (Table 3), depending on: 1) the use of percutaneously inserted pulmonary stent valves [5]; 2) whether an infundibular-reducer [5] is developed for clinical use in patients with dilated right ventricular outflow tract (RVOT) and pulmonary regurgitation (mainly Fallot-patients), making these patients suitable for percutaneous insertion of stent valves; and 3) whether perimembraneous VSDs are closed by catheter techniques [6], and whether coarctations will be treated with percutaneous techniques [7]. It was primarily first (38–46%) and second-time (38–57%) operations that might be converted to percutaneous procedures.
3.1. Atrial septal defect Forty-seven patients underwent one operation. The number of surgically closed ASDs declined in the study period, from nine to four ASD operations per year in 2005, whereas the number of device closed ASDs increased correspondingly (Fig. 5).
3.2. Tetralogy of Fallot Most patients had a pulmonary homograft inserted, three patients had closure of residual VSDs, two had reconstructive surgery of the pulmonary arteries with grafts and two other patients had their ascending aortas resected and reconstructed with tube grafts following aortic aneurysms. In 2006/2007, five patients have had percutaneous insertion of a pulmonary stent valve. Most patients had a tube graft inserted, whereas three had an end-to-end reconstruction. Stenting or balloon dilatation of coarctation was introduced in our GUCH unit in 2004 with four procedures in 2004, nine in 2005 and seven in 2006 (15 stents/5 balloon dilatations). The number of coarctation operations in 2004 and 2005 was 2 and 0, respectively. Thirteen patients had their cleft and the primum defect closed. Four patients had a left AV valve ring annuloplasty. The final two patients had a mechanical valve prosthesis inserted. 3.5. Sinus venosus ASD with partial anomalous pulmonary venous drainage Seventeen patients had one operation each. Sixteen patients had transposition of the venous drainage with an intra-atrial patch.3.6. Ventricular septal defect (VSD) In nine patients the VSD was closed. Three patients had an aortic prosthesis inserted. The final three patients had a pulmonary homograft, an annuloplastic of the tricuspid valve and mitral valve annuloplasty, respectively. None of the patients experienced heart block.3.7. Left ventricular outflow tract obstructions Twenty patients underwent 21 operations. Two patients with Williams syndrome were operated due to supravalvular aortic stenosis with either a patch reconstruction of the ascending aorta (n=1) or insertion of a tube graft (n=1).
3.7.1. Valvular aortic stenosis
3.7.2. Subvalvular aortic stenosis 3.8. Transposition of the great arteries (TGA) Eight patients that all previously had had a Mustard operation had eight operations. Due to baffle problems they all had insertion of a new baffle.3.9. Congenitally corrected TGA (ccTGA) Five patients were operated due to tricuspid regurgitation, one had a VSD closed, one had a pulmonary homograft inserted, one patient had a correction of a RVOT obstruction, while the last patient, who had a Fontan circulation, had an epicardial pacemaker implant.Six patients (3%) with one operation each made up the Single Ventricle-group. All patients had been through heart surgery before. The operations performed were: cavo-pulmonary anastomosis (n=4), insertion of a ring annuloplastic in the mitral valve (n=1) and, finally, a Glenn procedure (n=1).
The prevalence of GUCH patients in Copenhagen (covering East Denmark with a population of 2.5 million) is around 4000 patients with around 100 new patients per year. The surgical activity in these eight years was rather stable with an average of 30 operations per year, giving an incidence of cardiac surgical procedures in the entire GUCH population of 8/1000 patients per year. Our findings are in agreement with others [1, 3, 8, 9] with regard to mean age and range, frequency of different diagnoses, variety in diagnoses, surgical procedures, ICU and hospital stay. Although our early mortality was smaller than in these four studies, the early mortality in all studies was low, i.e. from 2% to 7.6% [1, 3, 8, 9]. As we separated our observational period into individual years, it was clear that the type of patients became increasingly complex during the study. Shrinidan et al. [1] also observed an increase in the size of their moderate complexity group in their three time periods, and our distribution of patients is most similar to their last period. The total number of operated GUCH patients, however, did not increase, most likely due to concomitant changes in the treatment of especially ASD and coarctation patients. Shrinidan et al. [1] and Dore et al. [3] likewise found that the number of surgical cases remained rather constant in their 12- and 4-year study periods. Earlier studies have demonstrated that the total number of GUCH patients will increase in the future [2]. However, the future need for surgical GUCH capacity seems unclear. Thus, treatment strategies are being continuously developed and adapted to present knowledge. In our group of surgically treated GUCH patients, from 33% to 46% of the patients might have been treated by percutaneous techniques at present or in the near future depending on the strategy in patients with pulmonary regurgitation, perimembraneous VSDs, and coarctations. In the more complex forms of CHD a possible change from surgical to percutaneous technique was less likely. The three largest diagnostic groups in the present study i.e. ASD, TOF and CoA represented approximately 50% of the patients. The change in treatment strategy in these three groups of patients during the observation period of eight years indicates that the surgical needs in GUCH patients with simple and/or moderate complex CHD might even be reduced. Thus, closure of secundum ASDs is now mainly a percutaneous procedure performed by cardiologists [10]. Fallot patients with significant pulmonary regurgitation and dilatation of the right ventricle (RV), may need insertion of a valved conduit to inhibit further deterioration of the RV-function and reduce the risk of sudden death [5]. The expected lifespan for a homograft is only between 10 and 20 years but an increased number of patients, who have earlier had a homograft insertion, may in the future have the subsequent valved conduit placed by percutaneous technique [5]. Furthermore, if clinically suitable infundibular-reducers will be introduced [5] this number will increase further, and surgical demands will decrease. Finally, GUCH patients with CoA will rather be treated with percutaneous placed stents [7]. Thus, it looks like the surgical needs in the three of the largest diagnostic groups in our and other studies [1, 8, 9], will decline dramatically in the coming years due to catheter-based interventions. For other large diagnostic groups, i.e. PAPVR, partiel AVSDs, VSD, TGA, and single ventricle, the future perspective is more unclear. However, the number of PAPVR-, primum defect- and VSD-patients may decline in the GUCH population caused by better detection by echocardiography and/or MR [11], and a more aggressive treatment strategy for these defects in childhood. For the VSD-group transcatheter closure may further reduce the number of surgical patients [6]. TGA patients are of two types in the surgical GUCH unit. Patients with atrial switch operations are mainly admitted due to baffle problems, which are taken care of by either surgical or transcatheter methods [12]. An increased number of arterial switch patients may be encountered in the future due to aortic valve regurgitation and coronary artery problems [13]. The single ventricle group may grow in size. However, the increased use of fetal echocardiography and possible pregnancy termination due to serious congenital heart disease [14] may lead to a decrease in the size of this group too. Held together with the above-mentioned considerations and reports, it seems that there may be an increase in the number of surgical GUCH patients with complex CHD [1], whereas the total number of surgical GUCH patients may remain stable or only increase a little. Thus, the predictions of the British Cardiac Society working group that the surgical workload will increase by 22–30% over the coming years [15] are challenged by these data and considerations.
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Abstract
1. Introduction
