Incidence and Risk Factors for Right Ventricular Outflow Tract Obstruction after the Arterial Switch Operation.

BACKGROUND: The aim of this study was to evaluate the incidence and risk factors for the development of right ventricular outflow tract obstruction (RVOTO) after the arterial switch operation (ASO). METHODS: Between 1983 and 2014, a total of 688 patients underwent ASO. RVOTO was defined as any obstruction of the right ventricular outflow tract (RVOT) requiring reintervention. RESULTS: RVOTO developed in 79 patients (11%) at a median time of 3.8 years (range, 1 day-23.6 years) after ASO. Freedom from RVOT reintervention was 96 ± 1, 89 ± 1, and 83 ± 2% at 1, 10, and 25 years, respectively. Independent risk factors for the development of RVOTO in a Cox's regression model were side-by-side great arteries (p < 0.001), aortic arch anomalies (p < 0.001), use of a pericardial patch for augmentation of the coronary buttons (p < 0.001), and a peak gradient more than 20 mm Hg over the RVOT at discharge (p < 0.001). CONCLUSION: The incidence of RVOTO after ASO is not negligible. Complex morphology, such as side-by-side great arteries and aortic arch anomalies influences the development of RVOTO.

Risk Factors for Failure of Systemic-to-Pulmonary Artery Shunts in Biventricular Circulation.

Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease, but it is associated with high morbidity and mortality. Data of all patients with biventricular circulation who underwent systemic-to-pulmonary artery shunt implantation between 2000 and 2016 were reviewed. Endpoints of the study were shunt failure and shunt-related mortality. Shunt failure was defined as any shunt dysfunction requiring intervention or reoperation. Shunt-related mortality was defined as death due to shunt dysfunction. A total of 217 shunts (central shunt, n = 131, Blalock-Taussig shunt, n = 86) were implanted in 178 patients. The median age of the patients was 98 days [1 day to 1.2 years]. Corrective surgery was performed at a median time of 0.6 years [3 months to 7 years] after shunt placement. Shunt failure was diagnosed in 21 patients (9.6%) at a median time of 14.6 days [0 days to 2 years]. Causes of shunt failure were stenosis (n = 11; 5%) and thrombosis (n = 10; 4.6%). The rate of freedom from shunt failure was 89.9 ± 2.6% at 1 year, the rate of shunt-related mortality was 3% (n = 5), and the rate of freedom from shunt-related mortality at 1 year was 97.5 ± 1%. Platelet transfusion was required in 43 patients (20%), all for postoperative thrombocytopenia. Perioperative platelet transfusion (p = 0.03) and shunt size of 3 mm (p = 0.03) were identified as risk factors for shunt failure. Shunt size of 3 mm was also identified as a risk factor for shunt-related mortality. The ideal shunt size in patients with biventricular circulation requiring a systemic-to-pulmonary artery shunt is 3.5 mm or larger. Platelet transfusion increases the risk of shunt failure and should be avoided. Type of shunt and diagnosis have no influence on morbidity or mortality after shunt placement.

Neutrophil gelatinase-associated lipocalin reflects inflammation and is not a reliable renal biomarker in neonates and infants after cardiopulmonary bypass: a prospective case-control study.

Introduction Acute kidney injury is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Neutrophil gelatinase-associated lipocalin has been suggested to be a promising early biomarker of impending acute kidney injury. On the other hand, neutrophil gelatinase-associated lipocalin has been shown to be elevated in systemic inflammatory diseases without renal impairment. In this secondary analysis of data from our previous study on acute kidney injury after infant cardiac surgery, our hypothesis was that neutrophil gelatinase-associated lipocalin may be associated with surgery-related inflammation. METHODS: We prospectively enrolled 59 neonates and infants undergoing cardiopulmonary bypass surgery for CHD and measured neutrophil gelatinase-associated lipocalin in plasma and urine and interleukin-6 in the plasma. Values were correlated with postoperative acute kidney injury according to the paediatric Renal-Injury-Failure-Loss-Endstage classification. RESULTS: Overall, 48% (28/59) of patients developed acute kidney injury. Of these, 50% (14/28) were classified as injury and 11% (3/28) received renal replacement therapy. Both plasma and urinary neutrophil gelatinase-associated lipocalin values were not correlated with acute kidney injury occurrence. Plasma neutrophil gelatinase-associated lipocalin showed a strong correlation with interleukin-6. Urinary neutrophil gelatinase-associated lipocalin values correlated with cardiopulmonary bypass time. CONCLUSION: Our results suggest that plasma and urinary neutrophil gelatinase-associated lipocalin values are not reliable indicators of impending acute kidney injury in neonates and infants after cardiac surgery with cardiopulmonary bypass. Inflammation may have a major impact on plasma neutrophil gelatinase-associated lipocalin values in infant cardiac surgery. Urinary neutrophil gelatinase-associated lipocalin may add little prognostic value over cardiopulmonary bypass time.

Bridging patients in cardiogenic shock with a paracorporeal pulsatile biventricular assist device to heart transplantation-a single-centre experience.

OBJECTIVES: We evaluated the outcome of patients in cardiogenic shock receiving a paracorporeal pulsatile biventricular assist device as a bridge to transplantation. METHODS: We performed a retrospective single-centre analysis of all patients who received a Berlin Heart Excor® at our institution between 2004 and 2019. RESULTS: A total of 97 patients (90 adults, 7 paediatric) were analysed. Eighty-four patients were in Interagency Registry for Mechanically Assisted Circulatory Support level 1 (80 adults, 4 paediatric). Diagnoses were dilated cardiomyopathy (n = 41), ischaemic cardiomyopathy (n = 17) or myocardial infarction (n = 4), myocarditis (n = 15), restrictive cardiomyopathy (n = 2), graft failure after heart transplant (n = 7), postcardiotomy heart failure (n = 5), postpartum cardiomyopathy (n = 3), congenital heart disease (n = 1), valvular cardiomyopathy (n = 1) and toxic cardiomyopathy (n = 1). All patients were in biventricular heart failure and had secondary organ dysfunction. The mean duration of support was 63 days (0-487 days). There was a significant decrease in creatinine values after assist device implantation (from 1.83 ± 0.79 to 1.12 ± 0.67 mg/dl, P = 0.001) as well as a decrease in bilirubin values (from 3.94 ± 4.58 to 2.65 ± 3.61 mg/dl, P = 0.084). Cerebral stroke occurred in 16 patients, bleeding in 15 and infection in 13 patients. Forty-eight patients died on support, while 49 patients could be successfully bridged to transplantation. Thirty-day survival and 1-year survival were 70.1% and 41.2%, respectively. CONCLUSIONS: A pulsatile biventricular assist device is a reasonable therapeutic option in cardiogenic shock, when immediate high cardiac output is necessary to rescue the already impaired kidney and liver function of the patient.

Case report: Heart Mate III for systemic right ventricular support in a patient with hypoplastic left heart syndrome.

Ventricular assist device implantation presents a possible bridge to heart transplantation for patients with failing Fontan physiology. However, evidence regarding outcome and possible pitfalls associated with the Fontan circulation is still insufficient. We describe the course of a 13-year-old male, who was born with hypoplastic left heart syndrome and underwent HeartMate III implantation due to refractory failure of the systemic right ventricle.

Risk evaluation in adult congenital heart surgery: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database risk models on data from the European Congenital Heart Surgeons Association Congenital Database.

OBJECTIVES: We sought to evaluate the predictive power of the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score and the adult congenital heart surgery (ACHS) mortality score for the adults undergoing congenital heart operations entered into the European Congenital Heart Surgeons Association (ECHSA) database. METHODS: The data set comprised 17 662 major operations performed between 1997 and 2019, on patients 18 years of age or older, in European centres participating in the ECHSA database. Each operation was assigned a STAT mortality score and category and an ACHS mortality score. Operative mortality was based on the 30-day status and on the status at hospital discharge. The discriminatory power of the STAT and ACHS scores was assessed using the area under the receiver operating characteristic curve (c-index). RESULTS: A total of 17 214 (97.46%) operations were assigned ACHS scores. The 3 most frequent primary procedures were closure of the atrial septal defect (19.0%), aortic valve replacement (8.8%) and non-valve-sparing aortic root replacement (6.1%). Operative mortality for ACHS-coded operations was 2.07%. The procedures with the highest mortality were atrial septal defect creation/enlargement (19.0%), lung transplantation (18.8%) and heart transplantation (18.2%). A total of 17 638 (99.86%) operations were assigned a STAT score and category. The operative mortality for STAT-coded operations was 2.27%. The c-index for mortality was 0.720 for the STAT mortality score and 0.701 for the ACHS score. CONCLUSIONS: The ACHS mortality score and the STAT mortality score reached similar, moderate predictive power in adult patients undergoing congenital heart surgery in ECHSA database.

German Heart Centre Munich-45 years of surgery in adults with congenital heart defects: from primary corrections of septal defects and coarctation to complex reoperations.

BACKGROUND: Our historical overview in the form of a cohort study aimed to describe the changes in the cardiac operations in adults with congenital heart disease (ACHD), over 45 years in the German Heart Centre in Munich. METHODS: The study population comprised all consecutive patients aged 18 or more, who underwent surgery for congenital heart disease (CHD) at the German Heart Centre Munich, between 1974 and 2018. Operations were coded according to the Adult Congenital Heart Surgery (ACHS) score. Two-tailed chi-square test was used for testing on differences in the frequency of procedural groups between the decades. RESULTS: During the examined 45-year period, 2,882 operations were performed on ACHD. The number of operations per year increased with a correlation coefficient r=0.533, P<0.001. Overall operative mortality was 3.2%. There was no significant difference in mortality over the decades. The percentage of primary CHD diagnosis being the indication for the operation was 99% in the 1970s and decreased significantly to 56% in the 2010s, P<0.001. There was a significant decrease in the relative frequency of atrial septal defect closures from 45% in the 1970s and 1980s to 9% in 2010s (P<0.001). Coarctation repair made up 15% of all operations in the 1970s, then dropped significantly to 1% in the 2010s (P<0.001). The percentage of reoperations increased significantly from 7% in the 1970s to 50% in 2010s (P<0.001). CONCLUSIONS: The spectrum of cardiac surgery in ACHD changed significantly over the last 45 years, from primary repair of septal defects and coarctation of the aorta in the 1970s to complex reoperations in the 2010s.

Predicted clinical factors associated with the intensive care unit length of stay after total cavopulmonary connection.

OBJECTIVES: A longer length of stay (LOS) in the intensive care unit (ICU) after the total cavopulmonary connection (TCPC) is thought to be a predictive sign of late Fontan failure. This study was performed to determine the clinical risk factors for ICU LOS. METHODS: In total, 483 patients who underwent a TCPC between May 1994 and December 2016 were included the study. Patients' main diagnosis, morphologic characteristics, palliative procedures, hemodynamic parameters, and perioperative variables, were analyzed to identify risk factors influencing ICU stay based on Cox regression. Causes of longer ICU LOS and the impact of ICU LOS on late outcomes were evaluated. RESULTS: Age at TCPC, type of TCPC, and fenestration at TCPC did not affect the ICU LOS. With multivariable model, hypoplastic left heart syndrome (P = .001) and anomalous systemic venous drainage (P < .001) were identified as independent morphologic risk factors for prolonged ICU LOS. Of hemodynamic variables, preoperative high transpulmonary gradient (P = .037), and low aortic oxygen saturation (P = .031) were risks for longer ICU LOS. Of postoperative variables, pleural effusion (P < .001), chylothorax (P = .001), ascites (P < .001), and infection (P = .028) were risks for longer ICU LOS. The ICU LOS was found to be significantly associated with late mortality (P < .001) and late cardiac reoperation (P = .007). CONCLUSIONS: Patients with hypoplastic left heart syndrome and anomalous systemic venous drainage had longer ICU LOS. Extended cyanosis and elevated pulmonary artery pressure affect the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors.

Quality of life after surgical treatment of coarctation in long-term follow-up (CoAFU): Predictive value of clinical variables.

BACKGROUND: We sought to analyze the quality of life and the predictive value of clinical variables from previous follow-up study in patients late after surgical treatment of aortic coarctation on the quality of life. METHODS: All patients, who have participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for the health-related quality of life questionnaire SF-36 from January 2013 through December 2014. RESULTS: From 273 eligible patients, we received data from 135 patients, 9 of them died during the follow-up time at the median age of 46years (range 30-64years). Seventy-four patients did not participate in the study, other 64 patients moved to remote or unknown areas and could not be contacted. Quality of life was good in the fields of physical role and pain. However, patients reported a significant impairment in general health and in health transition, depending on the age. Arterial hypertension and variables from echocardiography or exercise testing from the COALA study were not predictive on functional health status. CONCLUSION: Quality of life in patients late after aortic coarctation repair is fairly good compared with healthy controls. Impairments in general health and health transition depend mainly on age, can be explained due to numerous comorbidities and reinterventions in long-term. The predictive value of the commonly assessed clinical variables on quality of life is limited.

Validation of the grown-ups with congenital heart disease score.

OBJECTIVES: Adults with congenital heart disease in need of heart surgery frequently present with significant comorbidity. Furthermore, additional technical difficulties often related to redo operations increase the risk for postoperative mortality and morbidity. Hence, next to the type of the procedure, additional procedure-dependent and procedure-independent factors have to be considered for risk evaluation. The recently proposed grown-ups with congenital heart disease (GUCH) mortality and morbidity scores account for these additional risk factors. We sought to validate their predictive power in a large population operated in a single centre. METHODS: Data of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease between 2005 and 2016, were collected. Mortality was defined as hospital mortality or mortality within 30 days following surgery. Morbidity was defined as occurrence of one or more of the following complications: renal failure requiring dialysis, neurologic deficit persisting at discharge, atrioventricular block requiring permanent pacemaker implantation, mechanical circulatory support, phrenic nerve injury and unplanned reoperation. The discriminatory power of the GUCH scores was assessed using the area under the receiver operating characteristics curve (c-index, including 95% CI). RESULTS: Eight hundred and twenty-four operations were evaluated. Additional procedure-dependent and procedure-independent factors, as defined in the GUCH scores, were present in 165 patients (20.0%) and 544 patients (66.0%), respectively. Hospital mortality and morbidity was 3.4% and 10.0%, respectively. C-index for GUCH mortality score was 0.809 (0.742-0.877). C-index for GUCH morbidity score was 0.676 (0.619-0.734). CONCLUSIONS: We could confirm the good predictive power of the GUCH mortality score for postoperative mortality in a large population of adults with congenital heart disease.

Long-term outcome after anomalous left coronary artery from the pulmonary artery repair: a 40-year single-centre experience.

OBJECTIVES: An anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, often associated with severely impaired left ventricular (LV) contractility and functional mitral valve (MV) regurgitation. Current data suggest that earlier correction of ALCAPA may result in a more complete recovery of LV function. By analysing the results of a large single-centre ALCAPA cohort, we sought to investigate whether these treatment paradigms remain valid. METHODS: A retrospective study was performed evaluating all patients undergoing repair of ALCAPA over a period of almost 40 years. All preoperative and postoperative echocardiographic reports were reviewed, focusing on the recovery of LV and MV function. RESULTS: The study cohort included 78 patients who underwent ALCAPA repair between 1977 and 2015, who were divided into 2 groups based on patient age at initial repair: Group A (n = 52, age <1 year) and Group B (n = 26, age >1 year). Following repair, systolic LV and MV function improved significantly (P < 0.01) in both groups. Patient age at the time of initial surgery had no significant influence on the improvement of LV function. Early mortality (within 30 days) was 10% (n = 8). No 30-day mortality was reported in the past 20 years. Survival at 20 years following ALCAPA repair was 86 ± 4%. CONCLUSIONS: Following ALCAPA repair, LV function significantly improved, regardless of age at the time of repair. In addition, preoperative functional MV regurgitation decreased over time. Concomitant mitral valve surgery at the time of ALCAPA repair is required in patients with structural abnormalities of the MV.

Evaluation of the Adult Congenital Heart Surgery Mortality Score at Two European Centers.

BACKGROUND: The adult congenital heart surgery (ACHS) score was derived from The Society of Thoracic Surgeons Congenital Heart Surgery Database. The score was validated with data for 1,603 operations and reached a good predictive power. We sought to evaluate its predictive power for 1,654 operations performed in two European centers. METHODS: Data of all consecutive patients aged 18 years or more who underwent surgery for congenital heart disease between 2004 and 2013 at center 1 (n = 830) and between 2005 and 2016 at center 2 (n = 824) were collected. Mortality was defined as hospital mortality or mortality within 30 days after surgery. The discriminatory power of the ACHS score was assessed using the area under the receiver-operating characteristics curve (c-index). RESULTS: During the examined 13-year period, 1,639 operations of 43 different procedural groups were eligible for scoring. The most frequent procedures were closure of atrial septal defect (n = 175, 10.7%), repair of partial anomalous pulmonary venous connection (n = 117, 7.1%), and aortic valve replacement (n = 112, 6.8%). Hospital mortality was 3.1%. The procedures with the highest mortality were heart transplantation (3 of 11, 27.3%), mitral valve replacement (9 of 39, 23.1%), and systemic venous stenosis repair (2 of 9, 22.2%). The c-index for the ACHS mortality score was 0.760 (0.750 in center 1 and 0.772 in center 2). CONCLUSIONS: The ACHS score reached similar, good predictive power in two different centers. The score is a useful tool to analyze surgical outcomes and to support individual decision making.

Atrioventricular valve regurgitation in patients undergoing total cavopulmonary connection: Impact of valve morphology and underlying mechanisms on survival and reintervention.

OBJECTIVE: The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection. METHODS: Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection. RESULTS: Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P < .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years (P < .01) were worse than in those who did not require atrioventricular valve surgery. CONCLUSIONS: Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory.

Outcomes of a total cavopulmonary connection in patients with impaired ventricular function.

OBJECTIVES: Our aim was to evaluate outcomes following a total cavopulmonary connection (TCPC) in patients with preoperatively impaired ventricular function (VF). METHODS: Of 483 consecutive TCPC patients, 44 (9.1%) had impaired VF (ejection fraction <50%, Group A), and 439 patients had normal VF (ejection fraction ≥50%, Group B). We compared the clinical outcomes between the groups. RESULTS: The median age at TCPC was 2.8 (interquartile range 1.9-8.3) years in Group A and 2.3 (1.8-3.5) years in Group B (P = 0.025). An atrioventricular valve (AVV) operation prior to (38.6 vs 27.1%, P < 0.001) and concomitant with (31.8 vs 12.1%, P < 0.001) the TCPC was performed more frequently in Group A. The median intensive care unit stay (7.0 vs 7.0 days, P = 0.737) and 30-day survival (97.7 vs 98.4%, P = 0.737) were not significantly different between groups. Freedom from death, transplantation (P = 0.115) and catheter intervention (P = 0.603) showed no difference between groups. However, freedom from cardiac reoperation was significantly lower in Group A (P < 0.001). VF was resolved in 22 of the 39 (56.4%) survivors in Group A. The recovered patients had a lower incidence of AVV reoperation (0 vs 6, P = 0.002) and pacemaker rhythm (0 vs 5, P = 0.006). CONCLUSIONS: In patients planned for TCPC, impaired VF is often associated with AVV regurgitation. TCPC can be performed with low risk and comparable clinical results except for cardiac reoperation in patients with impaired VF when compared to patients with normal VF. Following TCPC, VF recovers in half of the survivors. A competent AVV and sinus rhythm are prerequisites for recovery.

Long-term outcome of preadolescents, adolescents, and adult patients undergoing total cavopulmonary connection.

OBJECTIVES: Patients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes. METHODS: Between 1994 and 2015, 50 of 460 patients underwent total cavopulmonary connection as preadolescents, adolescents, or adults (group A). The patients' characteristics and operative results were compared with those of the remaining 410 patients aged less than 9 years who underwent total cavopulmonary connection (group B). Post-total cavopulmonary connection echocardiogram reports (n = 4862) were used to evaluate longitudinal ventricular function, and ejection fraction was characterized using nonlinear mixed-effects models and compared between groups. RESULTS: The median follow-up time was 10.3 (2.8-15.5) years. The differences between groups in 30-day mortality (P = .20), intensive care unit stay (P = .20), and incidence of prolonged effusion (P = .08) were not significant. The estimated survival at 15 years was lower in group A (86.5%) than in group B (94.0%, P = .04) patients. Long-term systemic ventricular ejection fraction, analyzed with linear mixed-effect models, was significantly reduced in group A than in group B patients (P < .001). At a median postoperative period of 8.4 (7.1-10.5) years, the peak oxygen uptake as measured by exercise capacity testing was lower in group A than in group B patients, respectively (22.3 ± 6.5 [n = 25] vs 30.6 ± 8.1 [n = 100] mL/kg/min, P < .001). CONCLUSIONS: The total cavopulmonary connection procedure was performed in preadolescent, adolescent, and adult patients with no significant difference in 30-day or hospital mortality compared with those in young children. However, long-term survival and ventricular performance were reduced in this older cohort.

Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants.

OBJECTIVES: Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. METHODS: Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. RESULTS: Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤ 1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on long-term follow-up magnetic resonance imaging. CONCLUSIONS: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life.

Recoarctation After Norwood I Procedure for Hypoplastic Left Heart Syndrome: Impact of Patch Material.

BACKGROUND: The development of recoarctation after the Norwood I procedure is a known complication in patients with hypoplastic left heart syndrome (HLHS). METHODS: We reviewed data on 194 consecutive patients with HLHS who underwent the Norwood I procedure between 2000 and 2015. All patients who survived until the second stage of the procedure were included. Recoarctation was defined by the need for intervention, either catheter based or surgical. The aim of our study was to determine if the patch material used for the initial arch reconstruction had an influence on the development of recoarctation. RESULTS: The study population consisted of 145 patients. The patch material used for aortic arch reconstruction was either a homograft (n = 87), autologous pericardium (n = 23), equine pericardium (n = 28), or other material (n = 7). Recoarctation was documented in 27 patients (18%) at a median time of 4.3 months (range, 1 to 28 months) after completion of Norwood I. Freedom from recoarctation was 85% ± 4%, 86% ± 7%, and 30% ± 22% at 2 years for homograft, autologous pericardium, and equine pericardium, respectively. Thirty-six percent of patients with equine pericardium exhibited recoarctation, significantly more than homograft patch (p < 0.01) or autologous pericardium (p = 0.002). Treatment for recoarctation was percutaneous balloon angioplasty in 19 patients, stent implantation in 3, and operative enlargement of the aortic arch in 5. On univariate analysis, arch reconstruction with equine pericardium was the only risk factor for recoarctation (hazard ratio, 6.0; 95% confidence interval, 2.6-14; p < 0.01). CONCLUSIONS: The patch material used for reconstruction of the aortic arch in HLHS influences the rate of recoarctation following the Norwood I procedure. Equine pericardium cannot be recommended.

Long-Term Outcome of Patients With Complete Atrioventricular Septal Defect Combined With the Tetralogy of Fallot: Staged Repair Is Not Inferior to Primary Repair.

BACKGROUND: Primary repair is the preferred strategy for surgical treatment of complete atrioventricular septal defect combined with the tetralogy of Fallot. However, a staged approach may be preferable for very small or cyanotic infants. The long-term outcomes of infants undergoing staged vs primary repair were compared. METHODS: Data from 47 patients with complete atrioventricular septal defect combined with the tetralogy of Fallot who were operated on at our institution between 1974 and 2013 were reviewed. The study end points were all-cause death and reoperation. The patients were classified into two groups: staged repair (SR) and primary repair (PR). The indications for staged repair were cyanosis and young age. RESULTS: There were 22 SR and 25 PR patients. The SR patients were younger at the time of the initial operation (p = 0.001), and were more frequently cyanotic (21 SR vs 5 PR patients, p = 0.003). The 10-year survival after repair of the SR and PR patients was 78% ± 11% and 83% ± 8%, respectively (p = 0.8). No risk factors for death were identified. The 10-year freedom from reoperation for atrioventricular valve regurgitation of SR and PR patients after repair was 73% ± 12% and 71% ± 10%, respectively (p = 0.5). At least moderate atrioventricular valve regurgitation before repair was the only risk factor for reoperation (p = 0.01). CONCLUSIONS: Cyanotic and very young children with complete atrioventricular septal defect combined with the tetralogy of Fallot who require urgent treatment have long-term outcomes after staged repair similar to those of patients who undergo primary repair. Preoperative atrioventricular valve regurgitation is associated with increased risk for reoperation over the long-term.

Impact of early Fontan completion on postoperative outcomes in patients with a functional single ventricle.

OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC ≤ 18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3-1.5) years in group A and 2.5 (1.9-4.5) years in group B. Duration of intensive care unit stay (6 vs 7 days), hospital stay (20 vs 20 days), and 30-day survival (100 vs 98%) was not significantly different between groups. Estimated survival (95.3 vs 92.1%), freedom from reoperation (93.7 vs 86.3%), freedom from catheter intervention (60.1 vs 77.0%), and freedom from protein losing enteropathy (97.6 vs 93.8%) at 10 years was not significantly different between groups. At last follow-up, no patient in group A but 13 patients in group B exhibited reduced ventricular function ( P = 0.035). Exercise-capacity testing showed that both peak oxygen uptake (36.4 vs 28.6 ml/kg/min; P = 0.026) and its percentage of predicted value (82.9 vs 70.0%; P = 0.004) were significantly higher in group A ( n = 6, mean postoperative period: 8.9 years) than in group B ( n = 119, mean postoperative period: 8.7 years). CONCLUSIONS: Fontan completion for TCPC can be performed at an early age with a low perioperative risk and good intermediate results. Earlier unloading of the systemic ventricle and earlier elimination of chronic cyanosis by staged cavopulmonary connection might be advantageous for preservation of long-term ventricular function.

Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation.

OBJECTIVES: This study sought to investigate the impact of tricuspid regurgitation (TR) on right ventricular function after percutaneous pulmonary valve implantation (PPVI). BACKGROUND: PPVI provides a less invasive alternative to surgery in patients with right ventricular-to-pulmonary artery (RV-PA) conduit dysfunction. Recovery of the right ventricle has been described after PPVI for patients with pulmonary stenosis and for those with pulmonary regurgitation. Additional TR enforces RV dysfunction by supplemental volume overload. Limited data are available on the potential of the right ventricle to recover in such a specific hemodynamic situation. METHODS: In a matched cohort study, we compared patients who underwent PPVI with additional TR with those without TR. RESULTS: The degree of TR improved in 83% of the patients. In our patients (n = 36) exercise capacity and right ventricular volume index improved similarly 6 months after PPVI in patients with and without important TR. None of them had significant TR in the long-term follow-up of median 78 months. CONCLUSIONS: PPVI improves not only RV-PA-conduit dysfunction, but also concomitant TR. In patients with a dysfunctional RV-PA conduit and TR, the decision whether to fix TR should be postponed after PPVI.