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Open in new tab Download slide Open in new tab Download slide OBJECTIVES The right ventricular outflow tract reconstruction is a common necessity in congenital cardiac surgery. As homograft availability is limited, alternatives need to be evaluated. The Labcor® conduit consists of a porcine tricomposite valve assembled inside a bovine pericardium tube. This study presents intermediate-term results for its utilization for right ventricular outflow tract reconstruction. METHODS Labcor conduits were implanted in 53 patients (February 2009–July 2016). We analysed perioperative data, freedom from conduit failure and risk factors for conduit dysfunction. RESULTS The most common diagnosis was Tetralogy of Fallot (n  = 20, 37.7%). The median age at surgery was 10.0 [interquartile range (IQR) 4.9–14.3] years. Pulmonary artery plasty (n  = 37, 69.8%) and augmentation of the right ventricular outflow tract (n  = 16, 30.2%) were often part of the procedure. The median conduit size was 21 (range 11–25) mm. There was no in-hospital death. The median follow-up after surgery was 4.6 (IQR 3.4–5.6) years. Fourteen patients (27.5%) developed conduit failure with stenosis being the main cause. Freedom from conduit failure was 98.0% at 2 and 80.5% at 5 years. The median longevity of the conduit was 7.4 years (95% confidence interval 5.1–9.8 years). Younger age and smaller conduit size were related to conduit failure. CONCLUSIONS Utilization of the Labcor conduit revealed acceptable intermediate-term results. The conduit appeared to be functioning sufficiently well within the first 5 years in the majority of patients. The higher rate of failure concerning smaller conduits might be associated with somatic outgrowth; however, conduit degeneration as common and long-term outcome still needs to be evaluated. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Since 1996, our centre performs restrictive enlargement of the pulmonary annulus at surgical repair of tetralogy of Fallot. A transannular patch is only used if the z-score of the pulmonary annulus is smaller than -2. We sought to determine whether this strategy reduces pulmonary insufficiency (PI) and reoperation rate compared to a nationwide contemporary cohort that has not been operated using a uniform strategy. METHODS: Eighty-seven tetralogy of Fallot patients were included in the study (Group 1). One hundred sixty-seven tetralogy of Fallot patients from the Competence Network for Congenital Heart Disease served as controls (Group 2). Clinical, echocardiographic, electrocardiogram, cardiovascular magnetic resonance and outcome data were analysed. RESULTS: Follow-up time since repair was not different between groups [12.9 (7.8-18.8) vs 13.1 (5.2-16.9) years, P = 0.96] while transannular patch rate was significantly lower in Group 1 (32.2% vs 64.7%, P < 0.001). Ten-year freedom from reoperation for PI was significantly higher in our cohort (98% vs 92%, P = 0.01). Multivariable analysis identified restrictive enlargement as the only predictor for no need of reoperation [hazard ratio 0.4 (95% confidence interval 0.24-0.84), P < 0.01]. In Group 1, cardiovascular magnetic resonance-derived volumes were smaller (indexed end-diastolic volume: 103 ± 24 vs 123 ± 31 ml/m², P < 0.001; indexed end-systolic volume: 53 ± 19 vs 59 ± 20 ml/m², P = 0.04) and regurgitation fraction was lower (21 ± 14 vs 31 ± 17%, P < 0.001). CONCLUSIONS: Restrictive enlargement of the pulmonary annulus reduces PI and limits the amount of right ventricular dilatation at intermediate-term follow-up. The need for reoperation to alleviate PI is significantly lower compared to a contemporary cohort. Restrictive enlargement strategy has a stronger influence on freedom from reoperation than the use of a transannular patch. Clinical Trial Registration: WHO Main ID: DRKS00010087. URL: http://apps.who.int/trialsearch/Trial2.aspx?TrialID=DRKS00010087. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass. METHODS: Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence. Cognitive functions were assessed with the German 'Kognitiver Entwicklungstest fu¨r das Kindergartenalter' (KET-KID). Risk factors for impaired neuro-development were evaluated. RESULTS: Neuro-developmental assessment was completed in 95 patients (Norwood: n = 69; non-Norwood: n = 26). Median (interquartile range) IQ and KET-KID scores were in the normal range. Except for verbal KET-KID, scores did not differ between Norwood and non- Norwood patients (verbal IQ: 98 (86-105) vs 93 (85-102), P = 0.312; performance IQ: 91 (86-100) vs 96 (86-100), P = 0.932; full-scale IQ: 93 (86-101) vs 89 (84-98), P = 0.314; KET-KID verbal: 48 (17-72) vs 25 (2-54), P = 0.020; KET-KID non-verbal: 33 (18-62) vs 45 (15-54), P = 0.771; KET-KID global: 42 (14-65) vs 28 (6-63), P = 0.208). Full-scale IQ was below average (<85 points) in 14 (20%) Norwood and 9 (35%) non-Norwood cases (P = 0.181). Global KET-KID was below average (<16th percentile) in 19 (28%) and 10 (38%) patients (P = 0.326). Smaller head circumference z-score and complications before neonatal surgery were independently associated with lower scores. CONCLUSIONS: Neuro-developmental outcome of preschool-aged Fontan patients was in the normal range. The Norwood procedure was not a risk factor for neuro-developmental impairment. Preoperative condition and patient-related factors were more important determinants than variables related to surgical palliation. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Despite advances in perioperative management during surgical treatment of hypoplastic left heart syndrome (HLHS), patients are still at risk for adverse neurodevelopmental sequelae including cognitive dysfunction. This study aimed to assess a neuropsychological profile of HLHS patients at school age who underwent the Norwood operation between 1996 and 2003 with deep hypothermic circulatory arrest (DHCA) or antegrade selective cerebral perfusion (ASCP), respectively. METHODS: Forty HLHS patients (DHCA: n = 11 and ASCP: n = 29) were compared with healthy controls (DHCA controls: n = 10 and ASCP controls: n = 24), recruited according to age, sex and socioeconomic status. Neuropsychological assessment included non-verbal intelligence (IQ norms) and raw score measures of visual and verbal short- and long-term memory as well as executive functions, processing speed and concentration. Neuropsychological data were correlated with bypass and circulatory arrest times. RESULTS: Compared with control subjects, both patient groups had average non-verbal intelligence scores [DHCA: 102 (72-112) and ASCP: 92 (70-127)], but showed reduced long-term memory capacities and decreased executive performance as well as reduced processing speed. DHCA patients, furthermore, had a reduced visual attention span, and ASCP patients scored less on the verbal learning task than controls. The duration of DHCA and cardiopulmonary bypass (CPB) was associated with visual executive functions and short-term memory. In the DHCA group, the duration of DHCA was correlated with the time to complete the Trail Making Test A measuring visual executive functions (Spearman rank correlation, rS = 0.867, P = 0.018). In patients provided with ASCP, the cumulated CPB duration was negatively correlated with the score of the block span test measuring visual short memory (rS = -0.476, P = 0.020) as well as with the copy score of the Rey Figure assessing visual executive functions (rS = -0.399, P = 0.032). CONCLUSIONS: School-age children with HLHS who underwent the Norwood procedure either with DHCA or ASCP show cognitive impairments compared with healthy controls. Our data indicate deficits in specific cognitive domains such as memory, executive functions and processing speed rather than basic intellectual dysfunction. [ABSTRACT FROM AUTHOR]

OBJECTIVES Low cerebral tissue oxygenation saturations have been observed by near-infrared spectroscopy (NIRS) after the Norwood procedure. Altered cerebral vascular resistance and pharmacological afterload reduction redirecting blood flow away from the cerebral circulation are possible mechanisms. METHODS Two different afterload reduction strategies were evaluated in patients with hypoplastic left heart syndrome or variants after the Norwood procedure. In patients of Group 1 (n = 34), afterload reduction was controlled with sodium nitroprusside or with the α-blocker phentolamine. In addition, a phosphodiesterase-III inhibitor was administered. Patients of Group 2 (n = 34) received a phosphodiesterase-III inhibitor only. Cerebral and somatic tissue oxygenation saturations and routine intensive care monitoring data were recorded for 24 h before and 48 h after the Norwood procedure. Mean values of the last 4 preoperative (baseline) and of the first and last 4 postoperative hours (early and late course) were calculated. RESULTS Baseline, early and late cerebral saturations were 58 ± 7, 52 ± 9 and 60 ± 6% for Group 1 and 58 ± 7, 52 ± 12 and 61 ± 7% for Group 2 and somatic saturations were 59 ± 8, 76 ± 10 and 67 ± 9% and 58 ± 9, 78 ± 8 and 69 ± 10%, respectively. Regional saturations were not different between groups. The postoperative cerebral tissue oxygen saturation was below 40% for 50 (0–1040) min in Group 1 and for 45 (0–720) min in Group 2 (P = 1.00). Preoperative cerebral NIRS values (OR 0.85 [0.76–0.96], P = 0.007), age at operation (OR 1.39 [1.02–1.88], P = 0.034) and early postoperative diastolic blood pressure (OR 0.88 [0.78–0.99], P = 0.038) were associated with cerebral tissue oxygen saturations below 40% for more than 60 min. Patients with a prolonged period of low cerebral tissue oxygen saturation had longer duration of mechanical ventilation (69 (37–192) vs 60 (33–238) h, P = 0.039) and afterload reduction therapy was terminated later (95 (47–696) vs 74 (39–650) h, P = 0.006). Early mortality was 9.4% (3 of 32) compared with 2.8% (1 of 36) in the remainder (P = 0.336). CONCLUSIONS The postoperative decline of cerebral tissue oxygen saturation was observed with both afterload reduction strategies. The difference between cerebral and somatic NIRS values may indicate a mismatch between cerebral and splanchnic oxygenation. Other strategies to improve cerebral tissue oxygenation are warranted. [ABSTRACT FROM PUBLISHER]

OBJECTIVES Near-infrared spectroscopy (NIRS) offers continuous non-invasive monitoring of regional tissue oxygenation. We evaluated NIRS monitoring during the postoperative course after superior cavopulmonary anastomosis in patients with hypoplastic left heart syndrome and anatomically related malformations. METHODS Cerebral (cSO2) and somatic (sSO2) tissue oxygenations were recorded for 48 h and compared with routine measures of intensive care monitoring. Changes in parameters in the case of postoperative complications were evaluated. RESULTS Data were obtained from 32 patients. Median age at operation was 2.9 (1.5–10.0) months and weight was 5.3 ± 1.0 kg. Postoperative complications occurred in 7 patients (pulmonary artery thrombus n = 4, pneumothorax n = 1, cardiopulmonary resuscitation n = 1 and low-cardiac output n = 1). cSO2 was 44 ± 14% at the end of the operation and reached its minimum of 40 ± 11% 2 h later (P = 0.018). Overall, cSO2 was depressed early after surgery and increased from a mean of 42 ± 11% during the first 4 postoperative hours to 57 ± 8% in the last 4 h of the study period (P < 0.001). The sSO2 decreased from 77 ± 11% during the early postoperative course to 68 ± 9% within the later course (P < 0.001). The cSO2 correlated with the arterial partial pressure of oxygen (pO2, r = 0.364, P < 0.001), with the arterial oxygen saturation (SaO2, r = 0.547, P < 0.001) and with the central venous oxygen saturation providing the strongest correlation (SvO2, r = 0.686, P < 0.001). Analysis of agreement between cSO2 and SvO2 measurements revealed a mean bias of 0.97 with limits of agreement between 19.8 and −17.9%. Inclusion of both cSO2 and sSO2 into a linear regression model slightly improved the prediction of SvO2 from NIRS values (r = 0.706, P < 0.001). The mean values of cSO2, sSO2, SaO2 and SvO2 during the early postoperative period were lower in patients with complications (cSO2: 45 ± 9 vs 29 ± 5%, P < 0.001; sSO2: 80 ± 11 vs 70 ± 6%, P = 0.004; SaO2: 76 ± 8 vs 66 ± 6%, P = 0.004; SvO2: 48 ± 14 vs 32 ± 6%, P < 0.001). CONCLUSIONS NIRS technology allows inferring the global oxygenation from continuous non-invasive measurements of regional tissue oxygenation. The cSO2 is lowered in the early postoperative course. Lower cSO2 values in the early postoperative course may be predictive of postoperative complications. [ABSTRACT FROM AUTHOR]

To evaluate the ability of non-contrast enhanced magnetic resonance imaging (MRI) techniques to characterize Fontan associated liver disease (FALD) in adolescent and adult Fontan patients. Fontan patients (n = 29) and healthy controls (n = 13) underwent an MRI protocol with T 1 , T 2 and Apparent Diffusion Coefficient (ADC) mapping. Routine FALD screening included abdominal ultrasound and laboratory testing. Median follow-up after Fontan operation was 15.1 (IQR 12.0–16.8) years. Distinct differences in tissue characteristics were visualized. T 1 and T 2 relaxation times were prolonged in Fontan patients, particularly of the right lobe (T 1 : 745 (IQR 715–784) ms vs. 586 (IQR 555–602) ms, p < 0.001; T 2 : 63 (IQR 59–64) ms vs. 58 (IQR 56–60) ms, p = 0.002). Left lobe ADC was lower in Fontan patients (1.10 (IQR 1.06–1.18) x 10−3 mm2/s vs. 1.23 (IQR 1.19–1.29) x 10−3 mm2/s, p < 0.001). T 2 mapping was able to differentiate between controls and Fontan patients with different FALD severity. Right lobe T 2 was higher in patients with moderate or severe in comparison to those with no or mild changes and healthy controls (64 (IQR 61–67) ms vs. 60 (IQR 59–63) ms vs. 58 (IQR 56–60) ms, p = 0.001). Non-contrast enhanced MRI methods are able to visualize regional differences in liver tissue characteristics. T 1 and T 2 relaxation times were prolonged in Fontan patients suggestive of fibrosis or congestive hepatopathy, while reduced ADC might reflect impaired microperfusion. These methods have promising clinical potential for detection of liver abnormalities in Fontan patients. The usefulness of T 2 mapping to grade FALD severity merits further investigation. Non-contrast enhanced liver MRI in Fontan circulation Liver fibrosis is a serious morbidity in patients with Fontan circulation (Fontan associated liver disease, FALD). Fontan patients and healthy controls underwent non-contrast enhanced liver MRI with Diffusion weighted imaging and T1- and T2-mapping. Distinct differences in tissue characteristics were visualized. T1- and T2-relaxation times were prolonged in Fontan patients, particularly of the right lobe. Right lobe T2-relaxation times were higher in Fontan patients with moderate or severe FALD in comparison to healthy controls and those with no or mild FALD. T2-mapping may be a promising non-invasive screening marker to detect and grade FALD. [Display omitted] • Reliable non-invasive methods to monitor Fontan associated liver disease have not been established. • Liver tissue characterization with contrast-free magnetic resonance imaging techniques in Fontan patients and healthy controls • Relaxometry and diffusion weighted imaging maps showed distinct differences between Fontan patients and healthy controls. • Especially T 2 -mapping has promising potential for non-invasive screening of Fontan associated liver disease. [ABSTRACT FROM AUTHOR]