Authors

• Darko Anić — University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia — ORCID: 0000-0002-7378-944X

Abstract

The number of patients with adult congenital heart disease (ACHD) is constantly increasing. In the University Hospital Centre Zagreb, a service was established in 2015 to treat such patients, including surgical treatment. We analyzed the database of patients with ACHD in the period between 2009 and 2015 to determine the number of patients as well as the most common diagnoses and outcomes, which we then compared to centers outside Croatia that are more experienced in treating ACHD. At our center, 111 surgical procedures were performed on 108 patients in that period, with an even ratio between women and men and a mean age of 35. Reoperations accounted for 44% of the procedures. Mortality and morbidity were low (0.9%). Since the establishment of the Working Group for ACHD of the Croatian Cardiac Society, the number of surgically treated patients has grown.

Keywords

congenital heart disease, adult patients, surgical treatment

DOI

Full Text

## Introduction Adult congenital heart disease (ACHD) affects a growing patient group, partly because of improved treatment of these diseases in childhood (1). Today, most newborns with congenital heart disease reach adulthood due to surgical treatment. A small group of patients is diagnosed only in adulthood, however. The most common diagnoses in this smaller group are bicuspid aortic valve disease, atrial septal defect, usually the sinus venosus type with or without partial anomalous pulmonary venous return to the right atrium, coarctation of the aorta, congenitally corrected transposition of the great vessels, and Ebstein's anomaly. However, most of these patients underwent some form of interventional treatment, either percutaneous or, more commonly, surgical. Surgical treatment of ACHD is a special problem due to the large number of heart defects, different pathoanatomical substrates, and complications caused by residual defects, as well as the fact that heart diseases also cause changes in other organ systems. ## Patients and methods We assessed the number of surgical procedures performed on patients with ACHD at the Cardiac Surgery Clinic of the University Hospital Centre Zagreb in the period between September 1, 2009 and October 8, 2015. Patients aged 15 and above were included in the analysis. The age and sex of the patients were analyzed, as well as diagnoses, previous surgical procedures, and current outcomes. The types of surgical procedures were classified by anatomical location and by complexity. ## Results Over the 5-year period, 108 patients with ACHD underwent surgical procedures in our institution, 54 men and an equal number of women. Three patients underwent two procedures each, bringing the total number of procedures to 111. One example is a female patient who underwent a surgical procedure in her childhood for tetralogy of Fallot with pulmonary atresia during which the ventricular septal defect was closed and a shunt with a biological prosthesis was implanted in place of the atretic valve. The patient also had a congenitally corrected transposition of the great vessels with dextrocardia. She presented at our institution 30 years after the shunt implantation with shunt stenosis and a dilated left ventricle performing the function of the right. Systemic ventricular function and the associated valve was competent. After shunt replacement, the patient was discharged from the hospital in good condition; however, dyspnea began to manifest some months later. Ultrasound revealed a significantly smaller left, i.e. pulmonary, ventricle and an increase in the size of the systemic ventricle, causing insufficiency of the systemic atrioventricular valve, which was not visible earlier due to the significant dilation of the pulmonary ventricle. The dysfunctional valve was replaced with a bioprosthetic valve. This is good example of the complexity of cases of ACHD and the difficulty in classifying such procedures. The patient was included in two groups of surgical procedures: among biological valve replacements in the shunt and as a replacement procedure for dysfunctional tricuspid valves in congenitally corrected transposition with a mitral, i.e. systemic atrioventricular valve. Among all patients, patient age was between 15 and 62 years of age (average ± standard deviation = 35.14±14.69). Age distribution is shown in Figure 1. The number of surgical procedures increased with time, and a significant increase is evident after the Working Group for ACHD of the Croatian Cardiac Society began its work in 2015 (Figure 2). Figure 1. Age distribution (in years) of patients operated from 2009 to 2015. Figure 2. Number of patients operated from 2009 to 2015. Out of all the procedures, 61 (56%) were the first surgical procedure performed on the patient and 47 (44%) were at least the second procedure performed on the patient. One female patient with tetralogy of Fallot had her sixth cardiac procedure performed at our institution. For simplicity’s sake, we divided the cardiac surgery procedures into large groups based on anatomical location (Figure 3). The most common location was the aortic valve (36 patients; 32.43%), all of which were bicuspid aortic valve cases. A simple prosthetic valve replacement procedure was performed in 8 patients. One of them also had an additional atrial septal defect. In addition to valve replacement, 9 patients also underwent a procedure for additional aortic annular expansion. One patient had a valvuloplasty, and 14 required replacement of the ascending aorta due to an additional aneurysm. One patient underwent a vavuloplasty procedure and had his ascending aorta replaced with a prosthetic, and another patient underwent pulmonary valve repair in addition to aortic valve replacement. A patient had Doty repair performed due to supravalvular aortic stenosis. A translocation of the left caval vein into the right was performed in a patient with a persistent left superior vena cava draining into the left atrium. Additionally, lead removal was performed on a patient with transposition of the great arteries after a Jatene procedure. Figure 3. The distribution of patients categorized by anatomic lesions. The second most numerous group were procedures on the pulmonary valve (21 patients, 18.92%). A biological implant was necessary in 4 patients after tetralogy of Fallot repair, and in another 5 as well due to other basic diagnoses. In 6 patients, valvuloplasty was performed on the right ventricular outflow tract, and the rest were patients after a Ross procedure. Three of them required pulmonary homograft replacement, and a further 3 also required replacement of a dilated ascending aorta. The third large group consisted of patients with an atrial septal defect (15 patients, 13.51%). All underwent valve repair with autologous pericardium. The rest of the patients, 39 of them (35.14%), were classified in a miscellaneous group due to very diverse procedures. A more detailed breakdown of surgical procedures are shown in Table 1, Table 2, and Table 3. The diagnosis, type, and number of procedures are shown, as well as the complexity of the procedures according to the Canadian model for ACHD patients (2, 3). Among the simple procedures (Table 1), most common were atrial septal defects (ASD) and aortic valve replacement. Of 14 patients with ASD, 3 cases were reoperations after a residual defect was found. ### Table 1: Number of operations with detailed diagnoses and surgical procedures classified as simple complexity category | **Diagnosis** | **Surgical procedure** | **Number of operations** | | --- | --- | --- | | ASD | Patch closure | 14 | | ASD+Aortic valve stenosis | Patch closure+AVR | 1 | | Bicuspid aortic valve | AVR AV repair | 7 1 | | BAV+AAA | Op. sec. Bentall AVR+aortic replacement AV repair+aortic replacement | 2 14 1 | | BAV+Pulmonary valve stenosis | AVR+PV repair | 1 | | CoA | Op. sec. Vossshulte | 1 | | Supravalvular aortic stenosis | Op. sec. Doty | 1 | | LVCS to LA connection | LVCS translocation | 1 | | S/p Jatene | PM electrode extraction | 1 | [†] ASD = atrial septal defect; AVR = aortic valve replacement; AAA = ascending aorta aneurysm; BAV = bicuspid aortic valve; CoA = coartation of aorta; LVCS = left vena cava superior; LA = left atrium; PM = pacemaker. ### Table 2: Number of operations with detailed diagnoses and surgical procedures classified as moderate complexity category | **Diagnosis** | **Surgical procedure** | **Number of operations** | | --- | --- | --- | | BAV+dysplastic valve | AVR+aortic root enlargement | 9 | | Tetralogy of Fallot | ToF correction ToF correction+MVP | 1 1 | | Pulmonary valve regurgitation | PVR | 4 | | RVOT stenosis | RVOT enlargement | 6 | | S/p Ross | PVR PVR+Op. sec. Bentall | 3 3 | | Pulmonary valve anomaly | PVR | 5 | | Mb. Ebstein | TVR | 5 | | Thoracic aorta aneurysm | Aneurysm resection | 1 | | MR+TR+ASD | ASD closure+MVR+TVP | 1 | | PAPVR | Intracardiac repair | 7 | | PAPVR+TI | Intracardiac repair+TVP | 1 | | Scimitar sy. | Intracardiac repair | 1 | | PAVC | PAVC correction | 2 | | PAVC+dysplastic mitral valve | PAVC correction+MVR | 4 | | VSD+RVOTO | VSD closure+RVOT enlargement | 3 | | PI+TI after cacinoid | PVR+TVR | 1 | | Double chambered right ventricle | Muscle bundle resection | 1 | [†] BAV = bicuspid aortic valve; AVR = aortic valve replacement; ToF = Tetralogy of Fallot; MVP = mitral valve repair; PVR = pulmonary valve replacement; RVOT = right ventricle outflow tract; TVR = tricuspid valve replacement; ASD = atrial septal defect; MVR = mitral valve replacement; TVP = tricuspid valve repair; PAPVR = partial anomalous pulmonary veins return; TI = tricupid valve insufficiency; PAVC = partial atrioventicular canal; VSD = ventricular septal defect; PI = pulmonary valve insufficiency. ### Table 3: Number of operations with detailed diagnoses and surgical procedures classified as complex category | **Diagnosis** | **Surgical procedure** | **Number of operations** | | --- | --- | --- | | S/p Senning | TVR+MVP | 2 | | ToF | HTx | 1 | | CAVC.TAPVR.S/p B-T shunt | HTx | 1 | | Cor triatriatum | Membrane resection | 1 | | ccTGA | Conduit replacement TVR | 1 1 | [†] TVR = tricuspid valve replacement; MVP = mitral valve repair; ToF = tetralogy of Fallot; Hex = heart transplantation; CAVC = complete atriventricular canal; TAPVR = total anomalous pulmonary veins return; B-T = Blalock-Taussig; ccTGA = congenitaly corrected transposition of great arteries. In the moderately complex procedure group (Table 2), 5 patients diagnosed with Ebstein's anomaly are shown in the late stage of the disease, and all five underwent tricuspid valve replacement with a bioprosthetic valve. One patient had a permanent pacemaker implanted due to conduction disorders. In the partial atrioventricular septal defect group, 2 patients underwent a primary procedure while 4 had a prosthesis implanted after a previous repair procedure due to severe changes on the mitral valve. Due to partial anomalous pulmonary venous return, 7 patients underwent an intra-atrial conduit procedure, and one patient also underwent plastic valve repair due to additional tricuspid insufficiency. A patient presenting with scimitar syndrome at an adult age was treated in the same way. Three patients had a ventricular septal defect and right ventricular outflow tract stenosis. After surviving a carcinoid, one patient was left with severe pulmonary and tricuspid valve regurgitation, which were then replaced with bioprosthetic valves. A muscle resection was performed on a patient with a double-chambered right ventricle. The complex procedure group (Table 3) included two patients with transposition of the great arteries that had undergone a Senning procedure in childhood. Both patients presented with severe atrioventricular regurgitation, so tricuspid valve replacement and mitral valve repair was performed. One female patient with congenitally corrected transposition of the great vessels, whom we have already mentioned, had two separate procedures performed: implantation of a bioprosthetic valve into the pulmonary shunt and tricuspid bioprosthetic valve replacement. Two female patients underwent heart transplant surgery due to different diagnoses. One was a young patient presenting with cardiomyopathy after tetralogy of Fallot correction; the heart transplant was successful. The other was a 38-year-old patient with a complex heart defect. It involved heterotaxy, completely anomalous inflow of pulmonary arteries into the right atrium, a complete atrioventricular canal defect, and pulmonary stenosis. She had received palliative treatment in childhood in the form of a Blalock-Taussig shunt and had not attended follow-up since then. She presented to the University Hospital Centre Zagreb with decompensated heart failure, and due to the complexity of the defect urgent heart transplantation was indicated. The surgical procedure was performed without complications, but there was a lethal outcome on the 33rd postoperative day, presenting with the multiorgan failure. This was the only lethal outcome in any patient with ACHD in the University Hospital Centre Zagreb (0.9%). All others were discharged to home care in a good overall state. ## Discussion In the Western world, patients with ACHD are a rapidly growing group. Advancements in pediatric cardiology and cardiac surgery have allowed these patients to reach adulthood, when they present with different problems. In Canada, the number of adult patients with congenital heart diseases has already overtaken the number of pediatric patients (2). The situation is similar in Great Britain, where that trend started as early as the nineties (4). In Croatia, the University Hospital Centre Zagreb is the only center systematically treating congenital heart defects, which includes surgical procedures. Several years ago, patients with ACHD started to appear in Croatia as well and if there was a need for surgical intervention, they were referred to pediatric surgeons in the University Hospital Centre Zagreb. Patients with ACHD or bicuspid aortic valve (BAV) disease that presented at the hospital in adulthood had been surgically treated in childhood at the University Hospital Centre Zagreb or in other cardiosurgical centers in Croatia, by general cardiac surgeons. Heart defects are very common and often do not cause any special problems for many years, so these patients would go to a cardiac clinic for adults (5, 6). Some patients are still treated at other centers in Croatia, receive surgeries there, and are not in the ACHD Registry at the University Hospital Centre Zagreb. Since ASD and BAV surgeries are comparatively simple and have a low mortality, general surgeons gladly accept them to their operation lists. Those two groups are the most common in Croatia, as in other centers abroad (7). Since 2010, such patients have been referred to pediatric surgeons in our center, which is when our patient registry was started. The Working Group for ACHD of the Croatian Cardiac Society was established in 2015, as well as a service for such patients at the University Hospital Centre Zagreb, so the number of such patients increased as can be seen in the number of surgeries performed in 2015. The third large group of surgical patients underwent procedures ranging from those on the pulmonary valve, although performed in the right ventricle, to those on the pulmonary artery branches. These were predominantly patients with previous procedures, most commonly tetralogy of Fallot correction, who developed pulmonary insufficiency later in life. According to a study from Toronto, 45.75% of patients who underwent total surgical correction for pulmonary insufficiency or shunt stenosis in childhood require a new procedure (8). This is another indicator of the complexity of congenital heart defects and the fact that these patients require life-long medical care. In the second subgroup, it was necessary to replace the shunt or the shunt valve due to severe stenosis and degeneration. The incidence of these procedures at our center matches the data from the literatue (1). We can say that these are typical complications for patients with ACHD that underwent surgical correction in childhood and later in adulthood presented with new or residual lesions, degenerated shunts, or artificial valves. In this group, almost all procedures were reoperations. If we add up the patients from the complex procedure group and those with previous aortic valve commissurotomies, reoperations made up 44% of the total number of procedures. The whole series of patients had only one lethal outcome, i.e. 2.13% in the reoperation subgroup with 47 patients. In the literature, post-reoperation mortality is between 3 and 7.6% but drops sharply in later patient series, which can be explained by increasing experience in such procedures (9, 10). Total mortality has been dropping over time as well, and the Toronto group shows a reduction from 4.7% to 1.9% over three decades (11). The most common cause of death was multi-organ failure and/or heart failure. The total mortality of 0.9% at the University Hospital Centre Zagreb does not differ significantly from international literature. Although surgical procedures on patients with ACHD have started only recently, the experience gained on pediatric and non-congenital cases is obviously bearing fruit. We expect an even greater influx of patients with ACHD in the future, and thus an increasing number of surgical procedures. It is not yet known in which direction the treatment of these patients will develop. Globally, the number of patients requiring surgical procedures with low complexity is going down. More and more patients with atrial septal defects or pulmonary insufficiency are being treated with percutaneous interventions. The same is true for patients with residual ventricular septal defects or pulmonary insufficiency after tetralogy of Fallot, belonging in the group of more complex procedures. It is estimated that 30 to 46% of patients that are treated surgically today will be treated with percutaneous interventions in the future (12-14). This may be our future, but it has become reality in many Western countries.

Literature

1. Dore A, Glancy DL, Stone S, Menashe VD, Somerville J. Cardiac surgery for grown-up congenital heart patients: survey of 307 consecutive operations from 1991 to 1994. Am J Cardiol. 1997;80(7):906–13. https://doi.org/10.1016/S0002-9149(97)00544-4
2. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI, et al. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol. 2001;37(5):1170–5. https://doi.org/10.1016/S0735-1097(01)01272-4
3. Srinathan SK, Bonser RS, Sethia B, Thorne SA, Brawn WJ, Barron DJ. Changing practice of cardiac surgery in adult patients with congenital heart disease. Heart. 2005;91(2):207–12. https://doi.org/10.1136/hrt.2003.032011
4. Report of the British Cardiac Society Working Party. Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. Heart. 2002;88 Suppl 1:i1–14. https://doi.org/10.1136/heart.88.suppl_1.i1
5. Hoffinann J. Incidence, Mortality and Natural History. In: Anderson RH, Macartney FJ, Shinebourne EA, Tynan M (eds): Paediatric Cardiology. Edinburgh, Churchill Livingstone, 1987, pp 4-14.
6. Kitchiner D, Jackson M, Walsh K, Peart I, Arnold R. The progression of mild congenital aortic valve stenosis from childhood into adult life. Int J Cardiol. 1993;42(3):217–23. https://doi.org/10.1016/0167-5273(93)90051-H
7. Berdat PA, Trost N, Pavlovic M, Pfammatter JP, Carrel T. Early Outcome in Guch Surgery. Cardiology. 2005;1(2):87–94.
8. Oechslin EN, Harrison DA, Harris L, Downar E, Webb GD, Siu SS, et al. Reoperation in adults with repair of tetralogy of fallot: Indications and outcomes. J Thorac Cardiovasc Surg. 1999;118(2):245–51. https://doi.org/10.1016/S0022-5223(99)70214-X
9. Berdat PA, Immer F, Pfammatter JP, Carrel T. Reoperations in adults with congenital heart disease: analysis of early outcome. Int J Cardiol. 2004;93(2-3):239–45. https://doi.org/10.1016/j.ijcard.2003.04.005
10. Geissler HJ, Südkamp M, Nowak J, de Vivie ER. [Congenital heart defects in adolescence and adulthood: fatalities and morbidity in primary and reoperation]. Z Kardiol. 1996;85(10):782-9. https://pubmed.ncbi.nlm.nih.gov/9036704/
11. Williams WG, Webb GD. The emerging adult population with congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2000;3:227–33. https://doi.org/10.1053/tc.2000.6509
12. Klcovansky J, Sřndergaard L, Helvind M, Andersen HŘ. Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase? Interact Cardiovasc Thorac Surg. 2008;7(1):84–9. https://doi.org/10.1510/icvts.2007.162941
13. Somerville J. Management of adults with congenital heart disease: an increasing problem. Annu Rev Med. 1997;48:283–93. https://doi.org/10.1146/annurev.med.48.1.283
14. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease); American Society of Echocardiography; Heart Rhythm Society; International Society for Adult Congenital Heart Disease; Society for Cardiovascular Angiography and Interventions; Society of Thoracic Surgeons. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;52(23):e143–263. https://doi.org/10.1016/j.jacc.2008.10.001