Preoperative Venovenous ECMO in an Infant With Late Diagnosis of Hypoplastic Left Heart Syndrome.

Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.

Mortality Following Congenital Heart Surgery in Adults Can Be Predicted Accurately by Combining Expert-Based and Evidence-Based Pediatric Risk Scores.

OBJECTIVES: Currently, there are few specific risk stratification models available to predict mortality following congenital heart surgery in adults. We sought to evaluate whether the predictive power of the common pediatric scores is applicable for adults. In addition, we evaluated a new grown-ups with congenital heart disease (GUCH) score specifically designed for adults undergoing congenital heart surgery. METHODS AND RESULTS: Data of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease (CHD) between 2004 and 2013 at our institution, were collected. We evaluated the Aristotle Basic Complexity (ABC), the Aristotle Comprehensive Complexity (ACC), the Risk Adjustment in Congenital Heart Surgery (RACHS-1), and the Society of Thoracic Surgeons (STS)-European Association for Cardiothoracic Surgery (EACTS) scores. The proposed GUCH score consists of the STS-EACTS score, the procedure-dependent and -independent factors of the ACC score, and age. The discriminatory power of the scores was assessed using the area under the receiver-operating characteristics curve (c-index). A total of 830 operations were evaluated. Hospital mortality was 2.9%. C-indexes were 0.67, 0.80, 0.62, 0.78, and 0.84 for the ABC, ACC, RACHS-1, STS-EACTS, and GUCH mortality scores, respectively. CONCLUSION: The evidence-based EACTS-STS score outperforms the expert-based ABC score. The expert-based ACC score is superior to the evidence-based EACTS-STS score since comorbidities are considered. Our proposed GUCH score outperforms all other scores since it integrates the advantages of the evidence-based EACTS-STS score for procedures and the expert-based ACC score for comorbidities. Evidence-based scores for adults with CHD should include comorbidities and patient ages.

Durability of bioprostheses for the tricuspid valve in patients with congenital heart disease.

OBJECTIVES: Only little data exist on the durability of bioprostheses in the tricuspid position in patients with congenital heart disease (CHD). The aim of the study was to determine the reoperation rate and the valve function after primary implantation. METHODS: Between 1990 and 2013, 51 patients with CHD underwent tricuspid valve (TV) replacement with a bioprosthesis. The median age at operation was 32 years (range: 8-69). The underlying morphology was Ebstein's anomaly in 62% of the patients. Implanted valves included 38 pericardial and 13 porcine valves. All available echocardiographic examinations (n = 714) and clinical data were retrospectively reviewed. Dysfunction was defined as an at least moderate regurgitation or a mean diastolic gradient ≥9 mmHg. Freedom from death, reoperation and prosthetic valve dysfunction was estimated using the Kaplan-Meier method. RESULTS: The 30-day mortality rate was 9%. The estimated survival rate was 86% at one and 80% at ten years. The freedom from reoperation at 1, 5 and 10 years was 100, 86 and 81%, and that from prosthesis dysfunction detected by echocardiography at 1, 5 and 10 years was 89, 66 and 58%, respectively. The main reason for dysfunction was insufficiency (89%). Valve implantation at an age below 16 years was associated with earlier reoperation and dysfunction (the 5-year freedom rate from reoperation/dysfunction was 70%/30% compared with 89%/78% in the rest of the patients, P = 0.016/0.0009). CONCLUSIONS: Serial echocardiography shows a high rate of dysfunction of TV bioprosthesis in patients with CHD, which already occurred a few years after implantation. In patients below 16 years of age, most prostheses are dysfunctional within 5 years.

Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience.

OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis. CONCLUSIONS: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern.

Improved Exercise Performance in Patients With Tricuspid Atresia After the Fontan-Björk Modification With Pulsatile Systolic Pulmonary Flow.

BACKGROUND: After the Fontan-Björk modification for tricuspid atresia, some patients show pulsatile systolic pulmonary flow. We compared the hemodynamic findings and the clinical presentation of patients with and without pulsatile systolic flow after atrioventricular connection. METHODS: According to the pulmonary flow pattern by pulsed-wave Doppler assessment of transthoracic echocardiography, 41 patients after atrioventricular connection were divided into two groups: patients who showed dominant pulsatile systolic pulmonary flow (group P, n = 11), and patients who did not (group N, n = 30). RESULTS: Mean follow-up time was 27.8 ± 4.7 years in group P and 25.3 ± 3.8 years in group N (p = 0.1). Patients in group P had significantly less frequently catheter ablation procedures for tachyarrhythmia (9% versus 50%, p = 0.03). No patient in group P had had cardiac decompensation, whereas 7 patients (23%) in group N had had an episode of cardiac decompensation (p = 0.08). Cardiopulmonary exercise testing revealed that patients in group P showed higher oxygen uptake compared with patients in group N (25.0 ± 7.3 versus 19.6 ± 6.0 mL · kg(-1) · min(-1), p = 0.03). Patients in group P showed higher systolic pulmonary artery pressure (21.3 ± 8.4 versus 16.8 ± 4.5 mm Hg, p = 0.05), higher right ventricular end-diastolic volume index (88.6 ± 30.2 versus 50.3 ± 28.5 mL · L(-1) · m(-2), p = 0.03), and higher right ventricle to left ventricle ratio of end-diastolic volume index (1.4 ± 0.6 to 0.7 ± 0.3, p = 0.01). CONCLUSIONS: Patients with pulsatile systolic flow in the pulmonary artery had better hemodynamic and better exercise performance compared with patients without pulsatile systolic flow after atrioventricular connection. A sufficient volume and function of the right ventricle is a prerequisite to create pulsatile systolic flow.

Durability of down-sized homografts for the reconstruction of the right ventricular outflow tract.

OBJECTIVES: Small-sized homografts are rare but may be required for the reconstruction of the right ventricular outflow tract (RVOT). Down-sizing adult-sized homografts can be an option to overcome the shortage of availability. METHODS: Since 1994, we have been down-sizing adult-sized homografts by excising one cusp. The aim of the study was to analyse the durability of down-sized homografts and compare it with small-sized homografts in a paediatric population. All patients below a body weight of 14 kg were included in the study. The end-point of the study was homograft failure. RESULTS: A total of 152 patients met the inclusion criteria of the study, of which 82 patients (54%) received a down-sized homograft. The median age was 17.1 (0.3-64.8) months and the mean weight 8.4 ± 3.4 kg. Fifty-eight patients (38%) were under 1 year and 10 (6.5%) under 1 month of age at the time of homograft implantation. The mean homograft size of the whole study population was 14.7 ± 2.5 mm and the mean z-score was 1.6 ± 0.9. The median follow-up time was 10 (0.03-19.7) years. Early mortality after homograft implantation was 5% (n = 8), 4 of these patients had received a down-sized homograft. The study population comprised early survivors, that is, 144 patients. During follow-up, a total of 46 homografts failed, 23 in each group, after a mean time of 5.7 ± 4.2 years. Freedom from homograft failure was 94.6 ± 2.6, 87.2 ± 4 and 68.6 ± 6.6% for down-sized homografts and 95.2 ± 2.7, 78.7 ± 5.5 and 61 ± 7% for small-sized homografts at 1, 5 and 10 years, respectively (P = 0.3). Risk factors for homograft failure in the multivariable analysis were a homograft z-score of <1 and age below 1 year at the time of implantation (P = 0.02). CONCLUSION: Down-sized homografts demonstrated a durability similar to that of small-sized homografts. Therefore, down-sizing adult-sized homografts by creating a bicuspid valve to fit into the corresponding RVOT in children with congenital heart defects is an excellent method to overcome the shortage of small-sized homografts.

Left pulmonary artery originating from the right coronary artery in a patient with pulmonary atresia and ventricular septal defect.

We describe a patient with pulmonary atresia and ventricular septal defect in whom the left pulmonary artery was originating from the dilated right coronary artery. Successful division of the left pulmonary artery from the right coronary artery and the connection with the right ventricular outflow tract was carried out with closure of the end of the origin. The preoperative accurate diagnosis of this anomaly with ascending aortography is the most important in the surgical management of patients with pulmonary atresia and ventricular septal defect.

Conversion to Total Cavopulmonary Connection Improves Functional Status Even in Older Patients with Failing Fontan Circulation.

BACKGROUND: Facing longer follow-up of patients after Fontan operation, Fontan conversion was proposed to treat failing Fontan circulation. We reviewed our patients who reached up to 42 years of age. METHODS: Fifteen consecutive patients underwent Fontan conversion to extracardiac conduit combined with biatrial maze procedure between October 2006 and January 2014. Mean age at conversion was 30.9 ± 5.6 years and mean interval from primary Fontan palliation to conversion was 23.2 ± 3.3 years. RESULTS: There was one early death. Ten of 14 survivors were extubated within 24 hours, and 11 were discharged from intensive care unit (ICU) within 7 days. The impaired left ventricular function, presented in four patients prior to surgery, improved from EF 43.8 to 54.8% (p = 0.02). During follow-up time of 3.6 ± 2.3 years after conversion, there was no late death and no reoperation. Two patients developed recurrent atrial arrhythmia. Older age at Fontan procedure, lower left ventricular function, higher New York Heart Association (NYHA) class, and anatomy other than tricuspid atresia emerged as risk factors for longer ICU stay. CONCLUSION: Fontan conversion improved the functional status in almost all patients. A concomitant maze procedure effectively eliminated atrial arrhythmia. This procedure provides a benefit even for older patients if all additional cardiac pathologies are addressed.

Creating an arc-shaped aorta: use of the subclavian artery for interrupted aortic arch repair.

BACKGROUND: With interrupted aortic arch (IAA), a direct anastomosis may produce an angular-shaped aortic arch instead of the normal arc-shaped aorta, when the discontinuity is considerably long. That may lead to aortic stenosis and to compression of the pulmonary artery or the main bronchus. If a tube graft is used, reoperation for graft exchange is inevitable. We demonstrate the results of using the subclavian artery for creating an arc-shaped aorta in IAA repair. METHODS: Between February 2006 and October 2012, 23 patients underwent IAA repair using the subclavian artery. The subclavian artery was closed distally, divided, and longitudinally incised from the transected end to the aorta. This flap was used to bridge the gap of the IAA, by forming the posterior wall of a new aortic segment. The arch was completed using glutaraldehyde-fixed autologous pericardium (52%) or homograft (48%). RESULTS: Median follow-up time was 4.8 years (range, 1.3 to 6.9). There were no early deaths and 1 late death. On postoperative angiographic imaging, the aorta takes an arc-shaped course in all cases. Aortic arch stenosis developed in 7 patients (30%). Four patients were treated interventionally, and 3 surgically. During follow-up, there was no compression of the pulmonary artery or the main bronchus. CONCLUSIONS: By using the subclavian artery, an arc-shaped aorta can be accomplished without the use of tube grafts. With this technique, compression of the pulmonary artery or the main bronchus can be avoided. This technique is recommended if a direct anastomosis might be not applicable to bridge a long interruption.

A Comparative Histopathological Study of Heparin Coated and Uncoated Polytetrafluoroethylene Shunts in Children With Congenital Heart Defect.

OBJECTIVE: Recently, heparin coated polytetrafluoroethylene (PTFE) shunts are available and are believed to improve inherent shunt problems such as thrombosis and excessive and incomplete neointima formation or occlusion. We aimed at comparing the potential histopathological differences in the neointima (in) between uncoated (UCS) PTFE shunts and heparin coated (HCS) PTFE shunts. MATERIALS AND METHODS: Thirteen shunts (six UCS and seven HCS) were analyzed. The specimens were fixed in formalin, embedded in paraffin or in methylmethacrylate, and characterized by standard and immunohistochemical staining. The thickness of pseudointima proliferation was graded as follows: 0 = no cell layers, 1 = few layers <100 µm, 2 = partial layers >100 µm, 3 = complete layers <300 µm, 4 = complete layers >300 µm, and 5 = occlusion. RESULTS: Mean shunt size was 3.4 ± 0.2 mm in UCS and 3.1 ± 0.2 mm in HCS (P = .053). Mean time of implantation was 163 ± 75 days in UCS and 97 ± 52 days in HCS (P = .091). There were no significant differences in the proportion of patients with functionally single ventricle, body surface area, age at implantation, or implantation type, between both groups. Shunt occlusion did not occur. Unplanned shunt explantation due to cyanosis was performed in one patient in each group. Partial thrombus formation was observed in one UCS (P = .462). There was complete endothelialization in 50% of UCS and 86% of HCS (P = .266). The grade of pseudointima proliferation was 1.8 ± 0.4 in UCS and 1.7 ± 0.5 in HCS (P = .646). CONCLUSIONS: The histopathological workup of PTFE shunts revealed equally partial endothelialization and discrete pseudointima proliferation in both the groups. The process of endothelialization may be faster in HCS.

Which type of conduit to choose for right ventricular outflow tract reconstruction in patients below 1 year of age?†.

OBJECTIVES: Reconstruction of the right ventricular outflow tract (RVOT) with a conduit in patients below 1 year of age remains a matter of concern due to limited availability and durability of conduits. We sought to analyse the freedom from conduit exchange in this subgroup of patients by comparing different conduits. METHODS: Data of 145 consecutive patients below 1 year of age, requiring a conduit for RVOT reconstruction between 1994 and 2011 were reviewed. The endpoints of the study were defined as: 'conduit exchange for any reason', 'at least moderate conduit stenosis' and 'at least moderate insufficiency'. RESULTS: Homografts, bovine jugular vein conduits (Contegra) and porcine-valved Dacron conduits (Hancock) were implanted in 62 (43%), 35 (24%) and 48 (33%) patients, respectively. The mean conduit diameter was 12.9 ± 1.3 mm. A conduit exchange was necessary in 72 patients (55%) at a median time of 5.9 years [1.1-10.8]. The rate of freedom from conduit exchange at 5 years was 69.4 ± 6.6, 59.4 ± 8.7 and 53.8 ± 7.4%, respectively (P = 0.4). The rate of freedom from at least moderate stenosis was 85.4 ± 5.6, 75.1 ± 9.1 69.1 ± 7.9% at 5 years and 59.2 ± 11.1, 35.8 ± 12.0, 49.7 ± 10.1% at 10 years, for homografts, Contegra and Hancock conduits, respectively. The rate of freedom from at least moderate conduit insufficiency was 91.7 ± 4, 74.6 ± 9.1, 86.9 ± 7.4% at 5 years and 64.8 ± 14.1, 44.2 ± 13.7, 52.1 ± 14.2% at 10 years, for homografts, Contegra and Hancock conduits, respectively. Patients with a Contegra conduit developed moderate conduit stenosis or insufficiency faster than patients with a homograft (P = 0.01). Age below 1 month and heterotopic implantation of the conduit emerged as risk factors for conduit exchange in the univariate analysis (P = 0.05, P = 0.02, respectively). There was no significant influence of the conduit type, conduit size, z-score or the body surface area. In the multivariate analysis, heterotopic implantation emerged as the only risk factor for conduit exchange (P = 0.02, hazard ratio = 1.6, 95% confidence interval = 1.0-2.7). CONCLUSIONS: Homografts, bovine jugular vein conduits and porcine-valved Dacron conduits exhibit equal durability after implantation in patients below 1 year of age independent of their size. Nonetheless, moderate conduit stenosis or insufficiency develops earlier in patients with a Contegra conduit. Conduit placement in the neonatal period and implantation in a heterotopic position shortens the durability.

[Congenital coronary artery fistulas: clinical and therapeutic consideration]. / Kongenitalne koronarne arterijske fistule: klinicko i terapijsko razmatranje.

A coronary artery fistula is a link between one or more coronary arteries with another heart cavity or a segment of systemic or pulmonary circulation. Arterial blood from a coronary vessel enters another segment via myocardial capillary bed. These are very rare anomalies which constitute approximately 0.2 - 0.4% of all congenital heart defects. Still, they are clinically significant if they are of medium or large size and are manifested with a series of clinical symptoms such as angina pectoris, arrhythmias, myocardial infarction, endocarditis, progressive dilatation, heart failure and cardiomyopathy, pulmonary hypertension, thrombosis of the fistula and formation of aneurysms with possible ruptures. We present six patients with a coronary arterial fistula, their history, diagnostic procedures and outcomes. Therapeutic closure of coronary artery fistulas is recommended in all symptomatic, but also in asymptomatic patients, if there are significant roentgenographic, electrocardiographic and other abnormalities. In recent times transcatheter closure of coronary fistulas has become a possible alternative to surgery and is becoming increasingly used thanks to improved diagnostic possibilities and technology. If possible, interventional closure of fistulas is precisely the method preferred in pediatric patients. The choice of method depends on the anatomy of the fistula, presence or absence of additional defects, and on the experience of an interventional cardiologist or a heart surgeon. If performed well, the effects of both methods are good. This paper presents two children with a fistula between the right coronary artery and the right ventricle (RV), one child with a fistula between LAD and RV, one child with a fistula between the main tree of the left coronary artery (LCA) and RV, one child with a fistula between LCA and the right ventricular outflow tract (RVOT), and one child with a fistula between LCA and the right atrium (RA). The last one (LCA-RA) is not described in the latest classification of anomalies of coronary blood vessels in children based on MSCT coronarography, so we consider our presentation to be a contribution to the new classification. Along with the descriptions of fistulas and presentations of interventional and cardiosurgical interventions, we are also presenting a rare case of spontaneous closing of the fistula within the first six months and of a reopening of the fistula between the right coronary artery and the right ventricle after six years.

Patient age at the Ross operation in children influences aortic root dimensions and aortic regurgitation.

BACKGROUND: The Ross operation provides the advantage of growth potential of the pulmonary autograft in the aortic position. However, development of autograft dilatation and regurgitation may occur. We sought to assess the progression of autograft diameters and aortic regurgitation (AR) with regard to patient age at the time of the Ross operation. METHODS: Autograft echo dimensions from 48 children <16 years of age at the time of the Ross operation, who had follow-up echocardiograms at <20 years of age, were analyzed using hierarchical multilevel modeling. The z values of autograft dimensions were calculated according to the normal aortic dimensions. Mean follow-up was 5.1 ± 3.3 years. The mean age at the time of the Ross operation was 10.0 ± 4.3 years. RESULTS: The mean z values of all patients showed a significant increase with follow-up time at the sinus (0.5 ± 0.1/year, P < .001) and the sinotubular junction (0.7 ± 0.2/year, P < .001) but not at the annulus (0.1 ± 0.1/year, P = .59). There was no significant difference in the z values of sinus and the sinotubular junction between younger and older children at implantation and with time. The initial annulus z value was significantly larger in younger children (P < .0001), whereas the annual increase was significantly higher in older children (P = .021). Age at operation has no impact on the initial AR grade (P = .60). The AR tends to increase more quickly in older patients (P = .040). Sinus and sinotubular junction dilate with time, regardless of patient age. CONCLUSIONS: Young children show larger initial annulus sizes than older children. However, annulus diameters tend to normalize in young children, whereas they increase in older children. Autograft regurgitation develops slowly, but significantly, and predominantly in older children. Stabilizing measures to prevent autograft root dilatation are warranted in adolescents, but they are not required in young children.

Treatment of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome.

OBJECTIVES: To determine the incidence of right ventricle-to-pulmonary artery (RV-PA) conduit stenosis after the Norwood I operation in patients with hypoplastic left heart syndrome (HLHS), and to determine whether the treatment strategy of RV-PA conduit stenosis has an influence on interstage and overall survival. METHODS: Ninety-six patients had a Norwood operation with RV-PA conduit between 2002 and 2011. Details of reoperations/interventions due to conduit obstruction prior to bidirectional superior cavopulmonary anastomosis (BSCPA) were collected. RESULTS: Overall pre-BSCPA mortality was 17%, early mortality after Norwood, 6%. Early angiography was performed in 34 patients due to desaturation at a median of 8 days after the Norwood operation. Fifteen patients (16%) were diagnosed with RV-PA conduit stenosis that required treatment. The location of the conduit stenosis was significantly different in the patients with non-ringed (proximal) and the patients with ring-enforced conduit (distal), P = 0.004. In 6 patients, a surgical revision of the conduit was performed; 3 of them died prior to BSCPA. Another 6 patients had a stent implantation and 3 were treated with balloon dilatation followed by a BSCPA in the subsequent 2 weeks. All patients who were treated interventionally for RV-PA conduit obstruction had a successful BSCPA. Patients who received a surgical RV-PA conduit revision had a significantly higher interstage (P = 0.044) and overall mortality (P = 0.011) than those who received a stent or balloon dilatation of the stenosis followed by an early BSCPA. CONCLUSIONS: RV-PA conduit obstruction after Norwood I procedure in patients with HLHS can be safely and effectively treated by stent implantation, balloon dilatation and early BSCPA. Surgical revision of the RV-PA conduit can be reserved for patients in whom an interventional approach fails, and an early BSCPA is not an option.

What to expect after repair of total anomalous pulmonary venous connection: data from 193 patients and 2902 patient years.

OBJECTIVES: Total anomalous pulmonary venous connection (TAPVC) occurs as isolated cases, in combination with single ventricle physiology, and may be complicated by pulmonary venous obstruction. We sought to identify potential risk factors for long-term mortality and reoperations. METHODS: Data from 193 consecutive patients who had undergone repair of TAPVC between 1974 and 2011 were analysed using multivariate Cox regression. Mean follow-up time was 15.0 ± 11.0 years, 95% complete. RESULTS: Survival was 82.7 ± 2.9% at 20 years. Single ventricle physiology (5.9% of the patients, P < 0.001) emerged as the only significant risk factor for mortality in multivariate analyses. Freedom from cardiac reoperation was 82.2 ± 3.3% at 20 years. Single ventricle physiology (P < 0.001) was the only risk factor for cardiac reoperations in multivariate analyses. Freedom from reoperations for pulmonary venous obstruction was 90.4 ± 2.5% at 20 years. An age at operation of ≤30 days (52.8% of the patients, P = 0.007) was the only risk factor for reoperations for pulmonary venous obstruction in univariate analyses. In patients with isolated TAPVC (n = 177), preoperative pulmonary venous obstruction (53.7% of the patients, P = 0.030) and deep hypothermic circulatory arrest (78.5% of the patients, P = 0.017) emerged as risk factors for mortality in univariate analyses. An age at operation of ≤30 days (53.7% of the patients, P = 0.022) was the only risk factor for reoperations for pulmonary venous obstruction in univariate analyses. CONCLUSIONS: Survival into the third decade without reoperations is excellent in patients with isolated TAPVC without preoperative pulmonary venous obstruction, irrespective of the type of anomalous connection. In contrast, survival of patients with TAPVC and single ventricle physiology is among the poorest of all congenital heart defects. Reoperations for pulmonary venous obstruction are rare and are predominantly required in patients who were operated on as neonates. Survival may be improved by using a strategy of low-flow cardiopulmonary bypass.

Recoarctation after the norwood I procedure for hypoplastic left heart syndrome: incidence, risk factors, and treatment options.

BACKGROUND: Early survival after the Norwood I procedure has improved over the years, but subsequent morbidity is not yet well assessed. The aim of this study was to review the incidence of recoarctation, evaluate risk factors, and analyze treatment options. METHODS: We reviewed the medical records of 124 consecutive patients with hypoplastic left heart syndrome (HLHS) who underwent the Norwood I procedure. Reconstruction of the aortic arch was performed in a standardized manner, removing all visible ductal tissue and enlarging the distal anastomosis with a Y incision into the descending aorta. Angiographic assessment with measurement of the peak gradient across the aortic arch was performed before the second stage was performed. RESULTS: Recoarctation of the aorta was documented in 13 patients (13.4%) at a mean time of 6.4 ± 5 months after the Norwood procedure. One patient died before the recoarctation could be treated. Right ventricular function was good in all except 1 patient at the time of diagnosis. Ten patients underwent 16 percutaneous balloon angioplasties, and 2 patients underwent operative enlargement of the neoaorta. The pretreatment peak gradient of 24.1 ± 16 mm Hg (10-64 mm Hg) across the aortic arch was significantly reduced to 6.3 ± 4 mm Hg (0-14 mm Hg) after angioplasty or operation (p = 0.003). There were no procedure-related deaths. No risk factor for recoarctation could be identified. CONCLUSIONS: A standardized surgical technique for reconstruction of the aorta leads to a low recoarctation rate. Balloon angioplasty can be performed in the majority of patients before the second-stage procedure.

Evaluation of the Aristotle complexity models in adult patients with congenital heart disease.

OBJECTIVES: The adult congenital heart disease (CHD) population has surpassed the paediatric CHD population. Half of all mortality caused by CHD occurs in adulthood; in some patients, it occurs during surgery. We sought to assess the potential risk factors for adverse outcome after cardiac operations in adults with CHD, and to evaluate the predictive power of the Aristotle score models for hospital mortality. METHODS: Procedure-dependent and independent factors, as well as the outcome factors of all consecutive patients aged 16 or more who underwent surgery for CHD between 2005 and 2008 at our institution were evaluated according to the European Association for Cardio-Thoracic Surgery Congenital Database nomenclature. An Aristotle basic complexity (ABC) and an Aristotle comprehensive complexity (ACC) score were assigned to each operation. The discriminatory power of the scores was assessed using the area under the receiver operating characteristics (AuROC) curve. RESULTS: During 542 operations, 773 procedures were performed. The early mortality rate was 2.4%, and the early complication rate was 53.7%. Tricuspid valve replacement (P = 0.009), mitral valve replacement (P < 0.001), elevated lung resistances (P = 0.002), hypothyroidism (P = 0.002) and redosternotomy (P = 0.003) emerged as risk factors for 30-day mortality. Tricuspid valve replacement (P < 0.001), tricuspid valvuloplasty (P = 0.006), mitral valve replacement (P = 0.003), shunt implantation (P = 0.009), surgical ablation (P = 0.024), myocardial dysfunction (P = 0.014), elevated lung resistances (P = 0.004), hypothyroidism (P = 0.002) and redosternotomy (P < 0.001) emerged as risk factors for complications. Mean ABC and ACC scores were 6.6 ± 2.3, and 9.0 ± 3.7, respectively. The AuROCs of the ABC and the ACC scores for 30-day mortality were 0.663 (P = 0.044), and 0.755 (P = 0.002), respectively. The AuROCs of the ABC and the ACC scores for complications were 0.634 (P < 0.001), and 0.670 (P < 0.001), respectively. CONCLUSIONS: Surgery for adults with CHD can be performed with low early mortality. However, complications are frequent, especially in patients who require repeat operations for atrioventricular valve incompetence. The ACC score may be helpful to estimate the risk of early mortality.

Unloading of right ventricle by bidirectional superior cavopulmonary anastomosis in hypoplastic left heart syndrome patients promotes remodeling of systemic right ventricle but does not improve tricuspid regurgitation.

OBJECTIVE: To evaluate the influence of volume unloading by bidirectional superior cavopulmonary anastomosis on the systemic right ventricle in patients with hypoplastic left heart syndrome. METHODS: A total of 90 consecutive patients with hypoplastic left heart syndrome, who had survived the early postoperative period after bidirectional superior cavopulmonary anastomosis, were studied. Seven patients were excluded because of tricuspid valve surgery before or in association with bidirectional superior cavopulmonary anastomosis. The echocardiograms of the remaining 83 patients were reevaluated for tricuspid valve regurgitation and the size of the tricuspid annulus before bidirectional superior cavopulmonary anastomosis and at the last available follow-up examination before total cavopulmonary connection. RESULTS: Echocardiograms were performed a median of 5 days before bidirectional superior cavopulmonary anastomosis. Tricuspid valve regurgitation was graded as 0 in 11 patients, I in 37 patients, II in 24 patients, and III in 11 patients. Follow-up echocardiograms were performed a median of 17 months after bidirectional superior cavopulmonary anastomosis. Postoperatively, tricuspid valve regurgitation was graded as 0 in 14 patients, I in 37 patients, II in 21 patients, III in 6 patients, and IV in 5 patients. Postoperatively, the mean Z value of the tricuspid annulus stayed the same in patients with significant tricuspid valve regurgitation (grade III or IV) after bidirectional superior cavopulmonary anastomosis but had decreased in the remaining patients. No significant change was seen in the level of tricuspid valve regurgitation after bidirectional superior cavopulmonary anastomosis compared with the preoperative data. CONCLUSIONS: The relative size of the tricuspid annulus in patients with hypoplastic left heart syndrome decreases after bidirectional superior cavopulmonary anastomosis, most likely owing to volume unloading and promotion of the remodeling of the systemic right ventricle. However, this remodeling of the right ventricle does not improve the grade of tricuspid regurgitation.

Pulmonary stenosis is a predictor of unfavorable outcome after surgery for supravalvular aortic stenosis.

We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100 %) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65 %) were diagnosed with Williams-Beuren-Syndrome, six (17 %) had a diffuse form of SVAS and 10 (39 %) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p = 0.30) or diffuse form of SVAS (p = 0.13). Patients with PS were operated at younger age (p = 0.028). Median follow-up time was 14.6 years. Overall mortality was 11.5 %. One patient with preoperatively severely decreased LV-function died 27 days postoperatively. Two late deaths occurred 7 and 10 years after the initial operation. Reoperations were required in 4 patients (15 %), 4-19 years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p = 0.005) and for the combined reoperation/death end-point (p = 0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.