Against all odds-late repair of multiple shunt lesions in a patient with Down syndrome: a case report.

BACKGROUND: Children with congenital heart defects (CHD) usually undergo elective surgical repair of haemodynamically relevant shunt lesions within the first year of life. Due to susceptibility for pulmonary arterial hypertension (PAH) in patients with Down syndrome, repair is usually aimed for no later than 6 months of life. However, with rising immigration from developing countries to Europe, more patients with unrepaired CHD are diagnosed at a later age. Anatomical repair may be precluded, when advanced pulmonary vascular disease has been established. CASE SUMMARY: We report a 39-month-old male patient with Down syndrome with a large non-restrictive perimembranous ventricular septal defect, a large patent ductus arteriosus, and a secundum-type atrial septal defect with a prominent left-to-right shunting. Haemodynamic assessment revealed only a mild increase of pulmonary artery pressures (mPAP) with low pulmonary vascular resistance index (PVRi). Vasodilator testing led to a further increase of the left-to-right shunt and decrease of PVRi, suggesting operability. After careful consideration, the patient underwent complete surgical repair with a good post-operative clinical outcome. Cardiac catheterization 6 months after corrective repair showed a normal mPAP. No signs of PAH have been detected in the medium-term follow-up. DISCUSSION: Expertise, increased physician awareness, and a thorough pre-operative multidisciplinary evaluation are paramount to determine the best treatment approach for patients, who may present late with multiple shunts, and-in our case-underlying Down syndrome. Long-term close post-surgical follow-up in an expert centre is warranted to promptly diagnose and treat a possible late presentation of PAH appropriately.

Cobra-Head Cuffed Vascular Graft as Right Ventricle-to-Pulmonary Artery Shunt in Norwood Procedure.

BACKGROUND: Right ventricle-to-pulmonary artery (RV-PA) shunt as a part of the Norwood procedure underwent many modifications. We present our experience with a commercially available polytetrafluoroethylene vascular graft with cobra-head cuff as an RV-PA shunt. METHODS: A consecutive series of 52 children with hypoplastic left heart syndrome (median age 8 [range, 2-68] days, median weight 3200 [range, 2060-4400] g) underwent the Norwood procedure with a cobra-head cuffed RV-PA shunt (6 mm). The cuffed end was used for the central PA reconstruction. A retrospective analysis of clinical results, PAs development, and shunt-related complications, interventions, and technique of Glenn operation was performed. The study endpoint was Glenn operation with shunt removal or interstage death. RESULTS: The hospital and late interstage mortality was 3.8% (n = 2 of 52) and 4% (n = 2 of 50), respectively, and was not shunt-related. During mean follow of 3.7 ± 2.5 years, 48 (92.3%) children underwent Glenn operation at a median age of 6 (range, 2.6-9.1) months. Angiography before the second stage revealed satisfactory branch PAs development (maximum and minimum McGoon ratio of 1.95 ± 0.36 and 1.38 ± 0.38, respectively). The mean maximal diameter of the left PA was smaller than that of the right PA (7.13 ± 2.1 mm vs 8.42 ± 2.2 mm; P = .017), without differences in mean minimal diameter. Two infants required stent implantation in proximal shunt end and 1 required urgent Glenn operation because distal shunt thrombosis. During Glenn operation, 11 (22.9%) children required patch reconstruction of central PAs. CONCLUSIONS: The cobra-head cuffed graft allowed easy and reproducible reconstruction of the central PA during the Norwood procedure. Using this technique, the development of PAs is satisfactory, the rate of shunt-related complications and interventions is low, and the second stage can be performed without patch material.

Right atrial cardiac myxoma and multiple pulmonary artery aneurysms: Is there a causal relationship?

Cardiac myxomas are rare occurrences in pediatric populations, as are pulmonary artery aneurysms. We report a 17-year-old adolescent with right atrial cardiac myxoma and concomitant multiple peripheral pulmonary artery aneurysms. Histological examination indicated infiltration of the pulmonary artery wall through the embolic cardiac myxoma cells, thereby weakening it. This report highlights the probable causal relationship between these two entities, proposes the possible pathomechanism and presents the diagnostic and therapeutic dilemmas in the management of this condition.

Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

OBJECTIVES: The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS: A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3-9.8) years and median weight 12.7 (range 7.6-26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS: Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS: Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

Temporary Tracheal Stenting After Pulmonary Artery Sling Repair in a Newborn.

Pulmonary artery sling (PAS) is a rare disease frequently associated with severe malacia and stenosis of the trachea. We present a two-day-old newborn that underwent urgent surgery for PAS and needed prolonged respiratory support afterward. Temporary airway stenting above the level of the tracheal bifurcation was performed five days after surgery to overcome severe airway obstruction caused by tracheomalacia and laceration of the tracheal mucosa after diagnostic bronchoscopy. Two days after the procedure, the child could be extubated and after two weeks the stent was removed without complications. Temporary tracheal stenting after PAS repair can be an effective therapeutic strategy in newborns with tracheal obstruction and allows timely weaning from ventilator support.

Extubation on the operating table in patients with right ventricular pressure overload undergoing biventricular repair†.

OBJECTIVES: Right ventricular pressure overload, which can result in restrictive right ventricular physiology, predicts slow recovery after biventricular repair of congenital heart defects. The goal of the study was to assess how extubation in the operating room influences the postoperative course in these patients. METHODS: Between January 2013 and June 2017, a total of 65 children [median age 0.96 (0.13-9.47) years; median weight 8 (3.05-25.8) kg] with right ventricular pressure overload underwent an intracardiac correction. The most common malformations were tetralogy of Fallot (n = 34) and double outlet right ventricle with pulmonary stenosis (n = 11). The patients were divided into 2 groups: the first (n = 36) comprised late extubated (LE) and the second (n = 29), early extubated (EE) children, immediately after chest closure in the operating room. Preoperative, perioperative and postoperative records were analysed retrospectively. RESULTS: Children who had EE had a lower heart rate (EE 124.2 vs LE 133.6 bpm; P = 0.03), higher arterial blood pressure (systolic: EE 87.9 ± 9.35 vs LE 81.4 ± 12.0 mmHg; P = 0.029; diastolic: EE 51.1 ± 6.5 vs LE 45.9 ± 6.64 mmHg; P = 0.003), lower central venous pressure (EE 8.6 ± 1.89 mmHg vs LE 9.9 ± 2.42 mmHg; P = 0.03), fewer pleural effusions in the first 6 postoperative days (EE 1.38 ml/kg/day vs LE 5.98 ml/kg/day; P = 0.009), shorter time of dopamine support ≥3 µg/kg (EE 7.29 ± 12.26 h vs LE 34.78 ± 38.05 h, P < 0.001), shorter stays in the intensive care unit (EE 2.7 ± 2.67 vs LE 5.0 ± 4.77 days, P = 0.001) and hospital (EE 11.8 ± 4.79 vs LE 15.5 ± 7.8 days; P = 0.022). CONCLUSIONS: Extubation in the operating room of children with right ventricular pressure overload undergoing biventricular correction is feasible and safe and has a beneficial effect on the postoperative course.

Elastica degeneration and intimal hyperplasia lead to Contegra® conduit failure.

OBJECTIVES: Currently, Contegra® grafts (processed bovine jugular vein conduits) are widely used for reconstructive surgery of the right ventricular outflow tract in patients with congenital heart disease (CHD). We analysed explanted Contegra conduits from 2 institutions histologically to get a possible hint at the underlying pathomechanisms of degenerative alterations and to find histological correlations of graft failure. Additionally, we compared the explants with a non-implanted processed graft and a native jugular vein obtained from a young bull. METHODS: The explanted Contegra grafts were gathered during reoperations of 13 patients (male: n = 9, 69.2%; female: n = 4, 30.8%). After standardized histological preparation, samples were stained with dyes haematoxylin and eosin and Elastica van Gieson. Additionally, X-ray pictures revealed the extent of calcification and chelaplex (III)-descaling agent was used to decalcify selected explants. RESULTS: Processing of the native jugular vein leads to tissue loosening and a loss of elastic fibres. For graft failure after implantation, 2 pathomechanisms were identified: original graft alteration as well as intimal hyperplasia. Elastica degeneration and rearrangement with interfibrillary matrix structures were the main developments observed within the graft itself. Intimal hyperplasia was characterized by fibrous tissue apposition, calcification and heterotopic ossification. CONCLUSIONS: Regression of the elastic fibre network leads to rigidification of the conduit. In Contegra grafts, atherosclerosis-like changes can be considered the leading cause of graft stenosis and insufficiency. We conclude that both observed mechanisms lead to early reoperation in CHD patients.

Use of dexmedetomidine in pediatric cardiac anesthesia.

PURPOSE OF REVIEW: In recent years, ultrafast-track anesthesia with on-table extubation and concepts of accelerated postoperative care have gained increasing support in pediatric congenital cardiac surgery. It is believed that such approaches might ideally combine economic benefits with a striving for continuous improvement of patient outcomes. The present review summarizes the role of dexmedetomidine (DEX) in this setting. RECENT FINDINGS: DEX is a clinical multipurpose drug that mediates its diverse responses through the activation of α2-adrenoreceptors. In pediatric cardiac surgery it has various applications. Used as a premedication, DEX provides arousable sedation and anxiolysis. As an intraoperative adjunctive agent of balanced general anesthesia the primary objectives for its administration are attenuation of the neuro-humoral stress response and facilitation of early extubation. During ICU treatment DEX spares opioids, prevents the risk of postoperative delirium or emergence agitation and impacts on important patient-centered outcomes, such as duration of mechanical ventilation, restart of enteral nutrition or length of ICU stay. SUMMARY: Due to a favorable mix of beneficial physiologic actions and a limited adverse effect profile, DEX is established in the perioperative pediatric cardiac surgery setting. However, evidence from high-quality randomized controlled trials on the effects of supplemental DEX on meaningful patient outcomes is scarce, and research on the role of DEX in providing cardioprotection, neuroprotection, or renoprotection is still at its beginning. DEX has developed to one of the main agents in the armamentarium of cardiac anesthesiologists and pediatric intensivists, but it should not be regarded as the new 'magic bullet'.

Aortic atresia with the interrupted aortic arch and an pulmonary-coronary fistula: the unique constellation for survival.

Aortic atresia with interrupted aortic arch is a rare anatomical and physiological combination. Survival is possible if there is blood flow in the ascending aorta supplying the coronary circulation from coexisting malformations. We present a newborn with aortic atresia, interrupted aortic arch type B, ventricular septal defect and a pulmonary artery to left coronary artery fistula as a source of blood flow to the coronary circulation, ascending aorta and proximal aortic arch, successfully treated with neonatal biventricular repair. To our knowledge, this is the first patient described in the literature with coronary blood flow dependent on a pulmonary-coronary arterial fistula.

Valve-Sparing Aortic Root Replacement in an 8-Month-Old Infant With Loeys-Dietz Syndrome.

The clinical experience with Loeys-Dietz syndrome (LDS) reveals fateful natural history with intracerebral incidents and aortic dissections. A newborn child was referred to our hospital with significantly dilated aortic root and clinical signs of LDS phenotype later genetically confirmed as LDS type I. A therapy with antihypertensive medicines was initiated to postpone the surgery. Despite that, aortic root dilatation progressed to 33 mm (z-score +9.3). To avoid aortic rupture, a valve sparing aortic root replacement was performed at patient age of 8 months. The purpose of this report is to share our dilemmas and experience in the treatment of this child.

Extubation in the Operating Room After Fontan Procedure: Does It Make a Difference?

Early extubation appears to have beneficial effects on the Fontan circulation. The goal of this study was to assess the impact of extubation on the operating table in comparison with extubation during the first hours after Fontan operation (FO) on the early postoperative course. Between 2013 and 2016, 114 children with a single ventricle heart malformations (mean age, 3.8 ± 2.3 years) underwent FO: 60 patients were extubated in the operating room (ORE) and 54 in the intensive care unit (ICUE) in the median time of 195 min (range 30-515 min) after procedure. Pre-, peri-, and postoperative records were retrospectively analyzed. The hospital survival rate was 100%. One patient from the ORE group needed an immediate reintubation because of laryngospasm. The ORE group showed lower heart rate (106.5 vs. 120.3 bpm; p < 0.001) and lower central venous pressure (10.4 vs. 11.4 mmHg; p = 0.001) than patients in the ICUE group within the first 24 h after FO, as well as higher systolic blood pressure within 7 h after operation (88.6 ± 2.5 vs. 85.6 ± 2.6 mmHg; p = 0.036). The ORE children manifested significantly less pleural effusions during 48 h after FO (38.0 vs. 49.5 ml/kg; p = 0.004), received less intravenous fluid administration within 24 h after FO (54.1 vs. 73.8 ml/kg; p = 0.019), less inotropic support (9.8 vs. 12.8 h of dopamine; p = 0.033), and less antibiotics (4.7 vs. 5.8 days; p = 0.037). ICUE children manifested metabolic acidosis more frequently than the ORE group 3-4 h after FO (p < 0.05). Immediate extubation after FO in comparison with extubation in the ICU appears to be associated with improved hemodynamics and reduced application of therapeutic interventions in the postoperative course.

Single Ostium of the Right and Left Coronary Artery From the Right Pulmonary Artery.

We describe a newborn with single-ventricle malformation and anomalous origin of both coronary arteries from single ostium in the middle portion of the right pulmonary artery whose coronary anatomy was discovered during the operation and who underwent successful staged operative management. This report presents a unique anatomic association, proposes a means of management, and highlights the importance of intraoperative analysis.

Lateral Atrial Tunnel Fontan Operation Predisposes to the Junctional Rhythm.

The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis. Medical records were retrospectively reviewed. The hospital survival was 100%. In EC group, cardiopulmonary bypass time (CPB) was shorter (p = 0.004) and less patients needed aortic cross-clamping (p < 0.001). Children after EC stayed longer in the hospital (p = 0.016) and manifested more often prolonged effusions (p = 0.038). The incidence of all forms of junctional rhythm was higher in the LT group, early postoperatively (p < 0.001), during hospitalization (p = 0.004) and at discharge (p < 0.001). Children after LT required more often temporary pacemaker stimulation (p < 0.001). Patients without postoperative normofrequent sinus rhythm had longer CPB time (p = 0.008) and were more often operated on with aortic cross-clamping (p = 0.028). Lateral atrial tunnel Fontan operation with fenestration facilitates early adaptation to the total passive pulmonary flow, but predisposes the patients to the loss of sinus rhythm. The crucial role in the preservation of sinus rhythm plays the last step of the multistage surgery of the single ventricle malformations, probably not only the surgical technique but also factors associated with the cardiopulmonary bypass.

Results of the Fontan operation with no early mortality in 248 consecutive patients.

BACKGROUND: The Fontan operation has undergone several modifications and today is the primary way to treat a broad spectrum of congenital heart defects. AIM: The purpose of this study is to present the results of treatment of children with a single ventricle operated by the same surgical team and managed according to a uniform strategy. METHODS: In the years 2007-2015, in 248 children aged 3.7 ± 2.6 years and weighing 14.6 ± 6.1 kg with a single ventricle, Fontan surgery was performed. In 56 (22.6%) children surgery was based on the creation of an intra-atrial lateral tunnel, and in 192 (77.4%) patients extracardiac modification was performed. In most patients, the operation was carried out with the normothermic cardiopulmonary bypass, on a "beating heart" without aortic cross-clamp. The average cardiopulmonary bypass time was 53.9 ± 23.9 min. The most common indication for surgery was hypoplastic left heart syndrome (53.6%). All patients with a single ventricle referred to our hospital for the Fontan procedure were enrolled into the surgery programme. RESULTS: All patients survived the operation and were discharged home. Thirty-six (14.5%) patients were extubated in the operating room, in other patients the mean duration of the mechanical ventilation was 9.7 ± 16.1 h (median 7 h). The average time of hospitalisation in the whole study group was 17.5 ± 18.5 days (median 15 days). After surgery, in four children transient seizures occurred, and three patients had an ischaemic stroke. CONCLUSIONS: Developing and obeying a fixed perioperative protocol is crucial for low mortality and small number of complications after Fontan operation.

Scimitar Syndrome Associated With Abnormal Hepatic Venous Drainage.

Scimitar syndrome is a rare congenital cardiopulmonary anomaly. We describe a 1.5-year-old boy with this uncommon malformation and an unusual hepatic venous drainage, which allows atypical surgical repair.

Validation of cell-cycle arrest biomarkers for acute kidney injury after pediatric cardiac surgery.

BACKGROUND: The lack of early biomarkers for acute kidney injury (AKI) seriously inhibits the initiation of preventive and therapeutic measures for this syndrome in a timely manner. We tested the hypothesis that insulin-like growth factor-binding protein 7 (IGFBP7) and tissue inhibitor of metalloproteinases-2 (TIMP-2), both inducers of G1 cell cycle arrest, function as early biomarkers for AKI after congenital heart surgery with cardiopulmonary bypass (CPB). METHODS: We prospectively studied 51 children undergoing cardiac surgery with CPB. Serial urine samples were analyzed for [TIMP-2]•[IGFBP7]. The primary outcome measure was AKI defined by the pRIFLE criteria within 72 hours after surgery. RESULTS: 12 children (24%) developed AKI within 1.67 (SE 0.3) days after surgery. Children who developed AKI after cardiac surgery had a significant higher urinary [TIMP-2]•[IGFBP7] as early as 4 h after the procedure, compared to children who did not develop AKI (mean of 1.93 ((ng/ml)²/1000) (SE 0.4) vs 0.47 ((ng/ml)²/1000) (SE 0.1), respectively; p<0.05). Urinary [TIMP-2]•[IGFBP7] 4 hours following surgery demonstrated an area under the receiver-operating characteristic curve of 0.85. Sensitivity was 0.83, and specificity was 0.77 for a cutoff value of 0.70 ((ng/ml)²/1000). CONCLUSIONS: Urinary [TIMP-2]•[IGFBP7] represent sensitive, specific, and highly predictive early biomarkers for AKI after surgery for congenital heart disease. TRIAL REGISTRATION: www.germanctr.de/, DRKS00005062.

Hypoplastic left heart syndrome with dextrocardia and situs solitus.

Hypoplastic left heart syndrome with dextrocardia and situs solitus is a very rare condition. We describe a 3.5-year-old male child with this constellation who underwent multistage repair and a successful extracardiac Fontan operation in our hospital.

Influence of pulmonary artery size on early outcome after the Fontan operation.

BACKGROUND: Small pulmonary arteries (PAs) are often considered as a contraindication for the Fontan operation (FO). The aim of this study was to evaluate if the PA size is still one of the major impact factors on the postoperative early outcome. METHODS: Data of 146 patients, with a median age of 2.0 years (range, 1.5 to 18 years) and a median weight of 12.45 kg (range, 7.7 to 64.7 kg) who underwent a modified FO in the same center between 2007 and 2012 were retrospectively analyzed with respect to the traditional McGoon ratio, Nakata index, and modified indices (measuring the narrowest diameters). RESULTS: Patients with a McGoon ratio of 1.6 or less (modified≤1.2) or a Nakata index of 150 mm2/m2 or less (modified≤100 mm2/m2) were not at a higher risk of longer mechanical ventilation (p=0.87 [0.1] and p=0.68 [0.52], respectively), longer stay (p=0.52 [0.18] and p=0.54 [0.38], respectively) in the intensive care unit, prolonged hospital stay (p=0.08 [0.26] and p=0.22 [0.29], respectively) or effusions (p=0.25 [0.37] and p=0.13 [0.06]), respectively). Younger and smaller children tended to have smaller PAs, but younger age (<24 months) and lower weight (<12 kg) were not predictive for poor early postoperative outcome. CONCLUSIONS: Small PAs do not significantly affect the early postoperative period after FO. In our opinion, there is no need to postpone the FO due to "smaller" PAs. The palliative procedures performed before FO to increase the size of the PA at the expense of volume overload of the single ventricle and the possible complications of prolonged cyanosis must be carefully weighed.