Truncus arteriosus from prenatal diagnosis to clinical outcome: a single-centre experience.

BACKGROUND: The aim of this study was to review our institution's experience with truncus arteriosus from prenatal diagnosis to clinical outcome. METHODS: and results: We conducted a single-centre retrospective cohort study for the years 2005-2020. Truncus arteriosus antenatal echocardiographic diagnostic accuracy within our institution was 92.3%. After antenatal diagnosis, five parents (31%) decided to terminate the pregnancy. After inclusion from referring hospitals, 16 patients were offered surgery and were available for follow-up. Right ventricle-to-pulmonary artery continuity was preferably established without the use of a valve (direct connection), which was possible in 14 patients (88%). There was no early or late mortality. Reinterventions were performed in half of the patients at latest follow-up (median follow-up of 5.4 years). At a median age of 5.5 years, 13 out of 14 patients were still without right ventricle-to-pulmonary artery valve, which was well tolerated without signs of right heart failure. The right ventricle demonstrated preserved systolic function as expressed by tricuspid annular plane systolic excursion z-score (-1.4 ± 1.7) and fractional area change (44 ± 12%). The dimensions and function of the left ventricle were normal at latest follow-up (ejection fraction 64.4 ± 6.2%, fractional shortening 34.3 ± 4.3%). CONCLUSIONS: This study demonstrates good prenatal diagnostic accuracy of truncus arteriosus. There was no mortality and favourable clinical outcomes at mid-term follow-up, with little interventions on the right ventricle-to-pulmonary artery connection and no right ventricle deterioration. This supports the notion that current perspectives of patients with truncus arteriosus are good, in contrast to the poor historic outcome series. This insight can be used in counselling and surgical decision-making.

Surgical Repair of a Sinus of Valsalva Aneurysm.

A surgically challenging case of an unruptured Sinus of Valsalva aneurysm (SoVA) with severe aortic regurgitation (AR) due to cusp prolapse is presented. Sinus reconstruction with a patch cut out from the sinus portion of a Gelweave Valsalva graft (Terumo Vascutek) was performed. Intraoperative measurements showed insufficient effective height of the right coronary cusp; therefore, cusp plication and pericardial patch augmentation of the right coronary cusp were performed with satisfying result.

Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study.

OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

Long-Term Follow-Up of Pericardium for the Ventricular Component in Atrioventricular Septal Defect Repair.

BACKGROUND: Despite the improved outcome in complete atrioventricular septal defect (AVSD) repair, reoperations for left atrioventricular valve (LAVV) dysfunction are common. The aim of this study was to evaluate the effect of fresh untreated autologous pericardium for ventricular septal defect (VSD) closure on atrioventricular valve function and compare the results with the use of treated bovine pericardial patch material. METHODS: Clinical and echocardiographic data were collected of patients with complete AVSD with their VSD closed with either untreated autologous pericardial or treated bovine pericardial patch material between January 1, 1996, and December 31, 2003. Evaluation closed in September 2019. RESULTS: A total of 77 patients were analyzed (untreated autologous pericardial VSD patch: 59 [77%], treated bovine pericardial VSD patch: 18 [23%]). Median age at surgery was 3.6 (interquartile range [IQR]: 2.7-4.5) months, and median weight was 4.5 (IQR: 3.9-5.1) kg. Trisomy 21 was present in 70 (91%) patients. Median follow-up time was 17.5 (IQR: 12.6-19.8) years. Death <30 days occurred in two (3%) patients. Reinterventions occurred in eight patients (early [within 30 days] in two, early and late in one, and late in five), all in the autologous pericardium group. Log-rank tests showed no significant difference in mortality (P = .892), LAVV reinterventions (P = .228), or LAVV regurgitation (P = .770). CONCLUSIONS: In AVSD, the VSD can safely be closed with either untreated autologous pericardium or xeno-pericardium. We found no difference in LAVV regurgitation or the need for reoperation between the two patches.

Mechanical Mitral Valve Replacement: A Multicenter Study of Outcomes With Use of 15- to 17-mm Prostheses.

BACKGROUND: The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses. METHODS: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated. RESULTS: Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]). CONCLUSIONS: Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk.

Mitral Valve Replacement With the 15-mm Mechanical Valve: A 20-Year Multicenter Experience.

BACKGROUND: The aim of this study was to evaluate early and long-term outcomes (mortality and prosthetic valve replacement) after mitral valve replacement with the 15-mm St Jude Medical prosthesis (St Jude Medical, St Paul, MN). METHODS: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15-mm St Jude Medical Masters prosthesis at 4 congenital cardiac centers in The Netherlands. Operative results were evaluated and echocardiographic data studied at 0.5, 1, 2, 3, 5, and 10 years after surgery. RESULTS: Surgery was performed in 17 infants. Ten patients (59%) were treated in the intensive care unit before surgery; 8 (47%) were on ventilator support. Median age at surgery was 3.2 months (interquartile range [IQR], 1.2-5.6), and median weight was 5.2 kg (IQR 3.9-5.7). There was 1 early cardiac death and 1 late noncardiac death. Median follow-up time was 9.6 years (IQR, 2.4-13.2), including 8 patients with a follow-up more than 10 years. The first prosthetic valve explantation (n = 11) occurred at a median of 2.9 years (IQR, 2.0-5.4). Other reinterventions were permanent pacemaker implantation (n = 3), subaortic stenosis resection (n = 2), and paravalvular leak repair (n = 1). Prosthetic valve gradients increased from a mean of 5.0 mm Hg (at discharge) to a mean of 14.3 mm Hg (at 5-year follow-up). CONCLUSIONS: Mitral valve replacement with the 15-mm prosthesis can safely be performed in infants and even in neonates. Median freedom from prosthesis replacement for outgrowth is 3.5 years. Thromboembolic complications were rare.

Follow-up after biventricular repair of the hypoplastic left heart complex.

OBJECTIVES: In hypoplastic left heart complex patients, biventricular repair is preferred over staged-single ventricle palliation; however, there are too few studies to support either strategy. Therefore, we retrospectively characterized our patient cohort with hypoplastic left heart complex after biventricular repair to measure left-sided heart structures and assess our treatment strategy. METHODS: Patients with hypoplastic left heart complex who had biventricular repair between 2004 and 2018 were retrospectively reviewed. Operative results were evaluated and echocardiographic mitral valve (MV) and aortic valve (AoV) dimensions, left ventricular length and left ventricular internal diastolic diameter (LVIDd) were measured preoperatively and during follow-up after 0.5, 1, 3, 5 and 10 years. RESULTS: In 32 patients, the median age at surgery was 10 (interquartile range 5.0) days. The median follow-up was 6.19 (interquartile range 6.04) years. During the 10-year follow-up, the mean Z-scores increased from -2.82 to -1.49 and from -2.29 to 0.62 for MV and AoV, respectively. Analysis of variance results with post hoc paired t-tests showed that growth of left-sided heart structures was accelerated in the first year after repair, but was not equal, with the MV lagging behind the AoV (P = 0.033), resulting in significantly smaller MV Z-scores compared with AoV Z-scores at 10-year follow-up (P < 0.001). There were 2 (6%) early deaths. The major adverse events occurred in 4 (13%) patients. The surgical or catheter-based reintervention was required in 14 (44%) patients. CONCLUSIONS: The growth rate of heart structures was most prominent during the first year after biventricular repair with lower growth rate of the MV compared with the AoV.

Left ventricular function and exercise capacity after arterial switch operation for transposition of the great arteries: a systematic review and meta-analysis.

BACKGROUND: The arterial switch operation for transposition of the great arteries was initially believed to be an anatomical correction. Recent evidence shows reduced exercise capacity and left ventricular function in varying degrees in the long term after an arterial switch operation. OBJECTIVE: To perform a meta-analysis on long-term exercise capacity and left ventricular ejection fraction after an arterial switch operation. METHODS: A literature search was performed to cover all studies on patients who had undergone a minimum of 6 years of follow-up that reported either left ventricular ejection fraction, peak oxygen uptake, peak workload, and/or peak heart rate. A meta-analysis was performed if more than three studies reported the outcome of interest. RESULTS: A total of 21 studies reported on the outcomes of interest. Oxygen uptake was consistently lower in patients who had undergone an arterial switch operation compared with healthy controls, with a pooled average peak oxygen uptake of 87.5±2.9% of predicted. The peak heart rate was also lower compared with that of controls, at 92±2% of predicted. Peak workload was significantly reduced in two studies. Pooled left ventricular ejection fraction was normal at 60.7±7.2%. CONCLUSION: Exercise capacity is reduced and left ventricular ejection fraction is preserved in the long term after an arterial switch operation for transposition of the great arteries.

Anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS), fractional flow reserve- and intravascular ultrasound-guided management in adult patients.

OBJECTIVES: To describe the use of fractional flow reserve (FFR) and intravascular ultrasound (IVUS) in the evaluation of patients with anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). BACKGROUND: ACAOS of the right and left coronary are rare, but may lead to symptoms and impose a risk for sudden cardiac death, depending on several anatomical features. Assessment and risk estimation is challenging in (nonathlete) adults, especially if they present without symptoms or with atypical complaints. METHODS: The team retrospectively studied 30 consecutive patients with ACAOS with interarterial course, who received IVUS- and FFR-guided treatment at our institution between October 2010 and September 2017. RESULTS: FFR was abnormal in only seven patients. IVUS showed the typical slit-like anatomy of the orifice in 23 patients. Based on FFR and/or IVUS results, in conjunction with the clinical presentation, clinical decision was made. A decision for intervention was made if at least two out of three entities were abnormal. Intervention implied unroofing of the coronary artery (n = 10) or coronary artery bypass grafting (n = 1). In all other patients a conservative strategy was followed. No adverse events occurred in the total population after a median of 37 (0-62) months of follow-up. CONCLUSIONS: Conservative treatment may be justifiable in adult patients with ACAOS in the presence of normal FFR and nonsuspicious symptoms, despite the presence of an interarterial course and/or slitlike orifice on IVUS. We recommend the use of FFR and IVUS in the standard work-up for adult patients with ACAOS and propose the use of a flowchart to aid in decision-making.

Long-term results of balloon angioplasty for native coarctation of the aorta in childhood in comparison with surgery.

OBJECTIVES: Coarctation of the aorta (CoA) can be treated either surgically or with balloon angioplasty (BA). Long-term follow-up for either treatment has been limited. Our objective was to compare long-term results of BA and surgery for treatment of native CoA in childhood. METHODS: Retrospective cohort study of patients with native CoA treated with BA or surgery between 3 months and 16 years of age. Forty-eight patients filled out questionnaires and approved review of their medical records. Twenty-four patients underwent additional testing, including 24-h ambulatory blood pressure measurement, cardiopulmonary exercise testing and cardiac magnetic resonance imaging. Results were analysed cross-sectionally and longitudinally. RESULTS: Nineteen and 29 patients received BA and surgery, respectively. Prevalence of hypertension and aneurysms was similar in both groups. Fifty percent of patients were hypertensive. Two-thirds of patients demonstrating hypertension were not receiving antihypertensive medication. Aneurysm formation occurred in 1 BA (5%) and 1 surgery (3%) patient. The BA group had a significantly higher risk of recoarctation (47% vs 24%) and reintervention (hazard ratio 2.95, 95% confidence interval 1.04-8.32). Exercise capacity and global left ventricular function were preserved in both groups and not significantly different after correction for age. Quality of life was good to excellent in the majority of the patients. CONCLUSIONS: After CoA repair in childhood, most patients perform well in daily life. However, on the long term, more than half of the patients develop hypertension and many develop re-CoA, especially in those who underwent BA. Therefore, we do not recommend BA for the treatment of native CoA in children.

Sudden Death Due to Coronary Artery Lesions Long-term After the Arterial Switch Operation: A Systematic Review.

BACKGROUND: The arterial switch operation (ASO) is the preferred procedure for children with dextrotransposition of the great arteries or Taussig-Bing anomaly. Short- as well as long-term outcome of ASO are excellent, but coronary artery stenoses are reported as a common long-term complication. It has been hypothesized that these might result in sudden cardiac death late after ASO. METHODS: A systematic search of PubMed and EMBASE was conducted to evaluate sudden cardiac death because of coronary complications late after ASO. Data on patients surviving ≥ 5 years post-ASO were collected from selected studies, corrected for duplicate data, and analyzed. RESULTS: After duplicate data correction 52 studies remained for data analysis. Among the 8798 survivors with follow-up, 27 patients died ≥ 5 years post-ASO (0.3%). Of these patients, 10 were known with relevant residual lesions. Five late deaths were sudden, possibly from a cardiac cause. None of the late sudden deaths were confirmed to be coronary-related. CONCLUSIONS: Sudden cardiac death in asymptomatic patients as a result of coronary artery stenosis or occlusion is extremely rare, with 5 possible cases and no proven cases of coronary artery-related sudden cardiac death in 8798 patients with 66,450 patient follow-up years. Therefore, routine coronary imaging of asymptomatic, single-stage ASO patients is not justified.

Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study.

BACKGROUND: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. METHODS: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%]). RESULTS: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). CONCLUSIONS: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.

Biomechanics of Failed Pulmonary Autografts Compared to Native Aortic Roots.

BACKGROUND: Progressive autograft dilatation after a Ross operation suggests that remodeling does not effectively reproduce native aortic root biomechanics. In the first of this two-part series, we compared mechanical properties of explanted autografts to pulmonary roots at pulmonary pressures. The goal of this study was to compare mechanical properties of explanted autografts to native aortic roots at systemic pressures. METHODS: Autograft specimens were obtained from patients undergoing reoperation after Ross operation. For comparison, native aortic roots were obtained from unused donor hearts. Biaxial stretch testing was performed to determine tissue mechanical properties. Tissue stiffness was determined at patient-specific physiologic stresses corresponding to systemic pressures (80 and 120 mm Hg) and hypertensive state (200 mm Hg). RESULTS: Nonlinear stress-strain curves were present for both failed autografts and native aortic roots. Explanted autografts were significantly more compliant than native aortic roots at 80 mm Hg (1.53 ± 0.68 versus 2.99 ± 1.34 MPa; p = 0.011), 120 mm Hg (2.54 ± 1.18 versus 4.93 ± 2.21 MPa; p = 0.013), and 200 mm Hg (4.79 ± 2.30 versus 9.21 ± 4.16 MPa; p = 0.015). Autograft tissue stiffness at 80, 120, and 200 mm Hg was not correlated with age at the time of Ross operation (p = 0.666, p = 0.639, and p = 0.616, respectively) or time in the systemic circulation (p = 0.635, p = 0.637, and p = 0.647, respectively). CONCLUSIONS: Failed pulmonary autografts retained a nonlinear response to mechanical loading typical of healthy arterial tissue. Despite similar wall thickness between autografts and aorta, autograft stiffness in this patient population was significantly reduced compared with native aortic roots. We demonstrated that biomechanical remodeling was inadequate in these specimens to achieve native aortic mechanical properties, which may have resulted in progressive autograft root dilatation.

Surgical Repair of Ventricular Septal Defect; Contemporary Results and Risk Factors for a Complicated Course.

Surgical closure of the ventricular septal defect is the most commonly performed procedure in pediatric cardiac surgery. There are conflicting data on weight at operation as risk factor for a complicated course. We performed a retrospective evaluation of mortality and morbidity in all patients undergoing surgical ventricular septal defect closure at our institution between 2004 and 2012 to identify risk factor for a complicated course. Multivariate logistic regression modeling was performed to identify risk factors for a complicated course. 243 patients who underwent surgical ventricular septal defect closure were included. Median age at operation was 168.0 days (range 17-6898), the median weight 6.0 kg (range 2.1-102.0). No deaths occurred. Two patients (0.8%) required a pacemaker for permanent heart block. Five patients (2.1%) underwent reoperation for a hemodynamically important residual ventricular septal defect. No other major adverse events occurred. No risk factors for major adverse events could be established. Multivariate analysis identified a genetic syndrome, long bypass time and low weight at operation as independent risk factors for a prolonged intensive care stay (>1 day) and prolonged ventilation time (>6 h). Contemporary results of surgical VSD closure are excellent with no mortality and low morbidity in this series. Although it is associated with increased ventilation time and a longer hospital stay, low bodyweight at operation is not associated with an increased risk of complications or major adverse events in our series.

Biomechanics of Failed Pulmonary Autografts Compared With Normal Pulmonary Roots.

BACKGROUND: Progressive dilatation of pulmonary autografts after the Ross operation may reflect inadequate remodeling of the native pulmonary root to adapt to systemic circulation. Understanding the biomechanics of autograft root dilatation may aid designing strategies to prevent dilatation. We have previously characterized normal human pulmonary root material properties; however, the mechanical properties of failed autografts are unknown. In this study, failed autograft roots explanted during reoperation were acquired, and their material properties were determined. METHODS: Failed pulmonary autograft specimens were obtained from patients undergoing reoperation after the Ross operation. Fresh human native pulmonary roots were obtained from the transplant donor network as controls. Biaxial stretch testing was performed to determine tissue mechanical properties. Tissue stiffness was determined at patient-specific physiologic stresses at pulmonary pressures. RESULTS: Nonlinear stress-strain response was present in both failed autografts and normal pulmonary roots. Explanted pulmonary autografts were less stiff than were their native pulmonary root counterparts at 8 mm Hg (134 ± 42 vs 175 ± 49 kPa, respectively) (p = 0.086) and 25 mm Hg (369 ± 105 vs 919 ± 353 kPa, respectively) (p = 0.006). Autograft wall stiffness at both 8 and 25 mm Hg was not correlated with age at the Ross procedure (p = 0.898 and p = 0.813, respectively) or with time in the systemic circulation (p = 0.609 and p = 0.702, respectively). CONCLUSIONS: Failed pulmonary autografts retained nonlinear response to mechanical loading typical of healthy human arterial tissue. Remodeling increased wall thickness but decreased wall stiffness in failed autografts. Increased compliance may explain progressive autograft root dilatation in autograft failures.

Ross Procedure in Neonates and Infants: A European Multicenter Experience.

BACKGROUND: Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability. METHODS: A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013. RESULTS: Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts. CONCLUSIONS: Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding.

Main pulmonary artery area limits exercise capacity in patients long-term after arterial switch operation.

OBJECTIVES: Despite excellent survival in patients after the arterial switch operation, reintervention is frequently required and exercise capacity is decreased in a substantial number of patients. This study relates right-sided imaging features in patients long-term after the arterial switch operation to exercise capacity and ventilatory efficiency to investigate which lesions are functionally important. METHODS: Patients operated in the UMC Utrecht, the Netherlands (1976-2001) and healthy controls underwent cardiac magnetic resonance imaging and cardiopulmonary exercise testing within 1 week. We measured main, left, and right pulmonary artery cross-sectional areas, pulmonary blood flow distribution, peak oxygen uptake, and minute ventilation relative to carbon dioxide elimination. RESULTS: A total of 71 patients (median age, 20 [12-35] years, 73% were male) and 21 healthy controls (median age, 26 [21-35] years, 48% were male) were included. Main, left, and right pulmonary artery areas were decreased compared with controls (190 vs 269 mm(2)/m(2), 59 vs 157 mm(2)/m(2), 98 vs 139 mm(2)/m(2), respectively, all P < .001); however, pulmonary blood flow distribution was comparable (P = .722). Peak oxygen uptake and minute ventilation relative to carbon dioxide elimination were 88% ± 20% and 23.7 ± 3.8, respectively, with 42% and 1% of patients demonstrating abnormal results (≤ 84% and ≥ 34, respectively). The main pulmonary artery area significantly correlated with peak oxygen uptake (r = 0.401, P = .001) and pulmonary blood flow distribution with minute ventilation relative to carbon dioxide elimination (r = -0.329, P = .008). Subanalysis (<18, 18-25, >25 years) showed that the main pulmonary artery area was smaller in older age groups. In multivariable analysis, the main pulmonary artery area was independently associated with peak oxygen uptake (P = .032). CONCLUSIONS: In adult patients after the arterial switch operation, narrowing of the main pulmonary artery is a common finding and is the main determinant of limitation in functional capacity, rather than pulmonary branch stenosis.

High incidence of Dacron conduit stenosis for extracardiac Fontan procedure.

OBJECTIVES: Extracardiac conduits are widely used to complete a Fontan circulation in patients with univentricular hearts. Although polytetrafluoroethylene conduits have proven good long-term patency, Dacron (polyethylene terephthalate) prostheses are still infrequently applied, with, as yet, no information on the long-term patency. METHODS: All patients who received an extracardiac Dacron conduit (n = 12) were retrospectively studied. The initial conduit size was 16 mm in all recipients. The mean age at Fontan completion was 3.1 ± 0.7 years. Patients with clinical symptoms and/or significant conduit stenosis (>50% of diameter) underwent reoperation. RESULTS: Of the 12 patients, 8 underwent reoperation (75%) at a mean interval of 6.5 ± 1.8 years after the Fontan operation. All conduits were replaced by an 18-mm polytetrafluoroethylene graft. The explants showed ubiquitous tissue deposits on the inner surface, with a residual internal diameter from 8 to 11 mm. All patients survived the extracardiac conduit replacement. Recovery was uneventful, except that 1 patient experienced long-lasting pleural fluid drainage. The mean hospital stay was 10.6 ± 12.0 days. CONCLUSIONS: The incidence of extracardiac Dacron conduit stenosis in total cavopulmonary connection patients is high. These data indicate that the use of this type of conduit should be avoided. Vigilant follow-up is advised for those patients who have undergone Fontan completion with a Dacron extracardiac conduit.

Neurological injury after neonatal cardiac surgery: a randomized, controlled trial of 2 perfusion techniques.

BACKGROUND: Complex neonatal cardiac surgery is associated with cerebral injury. In particular, aortic arch repair, requiring either deep hypothermic circulatory arrest (DHCA) or antegrade cerebral perfusion (ACP), entails a high risk of perioperative injury. It is unknown whether ACP results in less cerebral injury than DHCA. METHODS AND RESULTS: Thirty-seven neonates with an aortic arch obstruction presenting for univentricular or biventricular repair were randomized to either DHCA or ACP. Preoperatively and 1 week after surgery, magnetic resonance imaging was performed in 36 patients (1 patient died during the hospital stay). The presence of new postoperative cerebral injury was scored, and results were entered into a sequential analysis, which allows for immediate data analysis. After the 36th patient, it was clear that there was no difference between DHCA and ACP in terms of new cerebral injury. Preoperatively, 50% of patients had evidence of cerebral injury. Postoperatively, 14 of 18 DHCA patients (78%) had new injury versus 13 of 18 ACP patients (72%) (P=0.66). White matter injury was the most common type of injury in both groups, but central infarctions occurred exclusively after ACP (0 vs. 6/18 [33%]; P=0.02). Early motor and cognitive outcomes at 24 months were assessed and were similar between groups (P=0.28 and P=0.25, respectively). Additional analysis revealed lower postoperative arterial Pco2 as a risk factor for new white matter injury (P=0.04). CONCLUSIONS: In this group of neonates undergoing complex cardiac surgery, we were unable to demonstrate a difference in the incidence of perioperative cerebral injury after ACP compared with DHCA. Both techniques resulted in a high incidence of new white matter injury, with central infarctions occurring exclusively after ACP. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01032876.

Low-flow antegrade cerebral perfusion attenuates early renal and intestinal injury during neonatal aortic arch reconstruction.

OBJECTIVE: Deep hypothermic circulatory arrest (DHCA) and antegrade cerebral perfusion (ACP) are 2 cardiopulmonary bypass strategies mainly used in aortic arch reconstructions. It has been suggested that during ACP, abdominal organs are better protected than during DHCA owing to partial perfusion via collaterals. We tested this hypothesis using intraoperative near-infrared spectroscopy (NIRS), lactate measurements, and biomarkers for early abdominal injury in neonates undergoing complex aortic arch repair. METHODS: Neonates scheduled for aortic arch reconstruction via median sternotomy between 2009 and 2011 were randomized to either DHCA or ACP. During surgery, regional oxygen saturations of the abdomen were monitored using NIRS. Immediately aafter DHCA or ACP, lactate concentrations from the inferior vena cava were compared with those from the arterial cannula. Postoperatively, biomarkers for early abdominal organ injury were measured in urine. RESULTS: Twenty-five neonates were analyzed (DHCA, n = 12; ACP, n = 13). Procedures were performed at 18°C, and ACP flow was set at 35 to 50 mL · kg(-1) · min(-1). Median abdominal NIRS value during DHCA was 31% (IQR, 28%-41%) whereas during ACP it was 56% (IQR, 34%-64%; P < .01 between groups). Immediately after DHCA, median lactate from the inferior vena cava was 4.2 mmol/L (IQR, 3.3-5.3 mmol/L) compared with 3.1 mmol/L (IQR, 2.9-4.4 mmol/L) after ACP (P = .03). Postoperatively, biomarkers for renal and intestinal damage (gluthatione s-transferase and intestinal fatty acid binding protein, respectively) were higher in the DHCA group than for the ACP group (P = .03, P = .04, respectively). CONCLUSIONS: These results substantiate earlier suggestions that ACP provides more abdominal organ protection than DHCA in neonates undergoing aortic arch reconstruction.