Impact of chylothorax on the post operative outcome after pediatric cardiovascular surgery

BackgroundChylothorax is the accumulation of chyle in the pleural cavity, usually develops after disruption of the thoracic duct along its intra-thoracic route. In the majority of cases this rupture is secondary to trauma (including cardio thoracic surgeries). Chylothorax is a potentially serious complication after cardiovascular surgeries that require early diagnosis and adequate management.MethodsA retrospective study of all cases complicated with chylothorax after pediatric cardiac surgery in King Abdulaziz Cardiac Center between January 2007 and December 2009 was conducted. The study aim to determine the risk factors, and the impact on the post operative course.ResultsWe have 1135 cases operated during the study period, 57 cases (5%) were complicated with chylothorax in the post operative period. The most common surgeries complicated with chylothorax were the single ventricle repair surgeries (Glenn-Fontan) 15 cases (27%), followed by the arch repair cases 10 cases (18%), the ventricular septal defect cases 10 cases (18%), the Atrioventricular septal defect cases 7 cases (12%), the arterial switch cases 6 cases (11%), and others 8 cases (14%). The ICU stay, the length of hospital stay and the bypass time were significantly longer in the chylothorax group, also the ventilation time, the inotropes duration and number were higher in the chylothorax group.ConclusionChylothorax after pediatric open heart surgery is not an uncommon complication, it occurs more commonly with single ventricle repair and aortic arch repair surgeries, it has a significant impact on the post operative course and post operative morbidity

ADAMTS19-associated heart valve defects: Novel genetic variants consolidating a recognizable cardiac phenotype

Recently, ADAMTS19 was identified as a novel causative gene for autosomal recessive heart valve disease (HVD), affecting mainly the aortic and pulmonary valves. Exome sequencing and data repository (CentoMD) analyses were performed to identify patients with ADAMTS19 variants (two families). A third family was recognized based on cardiac phenotypic similarities and SNP array homozygosity. Three novel loss of function (LoF) variants were identified in six patients from three families. Clinically, all patients presented anomalies of the aortic/pulmonary valves, which included thickening of valve leaflets, stenosis and insufficiency. Three patients had (recurrent) subaortic membrane, suggesting that ADAMTS19 is the first gene identified related to discrete subaortic stenosis. One case presented a bi-commissural pulmonary valve. All patients displayed some degree of atrioventricular valve insufficiency. Other cardiac anomalies included atrial/ventricular septal defects, persistent ductus arteriosus, and mild dilated ascending aorta. Our findings confirm that biallelic LoF variants in ADAMTS19 are causative of a specific and recognizable cardiac phenotype. We recommend considering ADAMTS19 genetic testing in all patients with multiple semilunar valve abnormalities, particularly in the presence of subaortic membrane. ADAMTS19 screening in patients with semilunar valve abnormalities is needed to estimate the frequency of the HVD related phenotype, which might be not so rare

The Changing Landscape for Stroke\ua0Prevention in AF: Findings From the GLORIA-AF Registry Phase 2

Background GLORIA-AF (Global Registry on Long-Term Oral Antithrombotic Treatment in Patients with Atrial Fibrillation) is a prospective, global registry program describing antithrombotic treatment patterns in patients with newly diagnosed nonvalvular atrial fibrillation at risk of stroke. Phase 2 began when dabigatran, the first non\u2013vitamin K antagonist oral anticoagulant (NOAC), became available. Objectives This study sought to describe phase 2 baseline data and compare these with the pre-NOAC era collected during phase 1. Methods During phase 2, 15,641 consenting patients were enrolled (November 2011 to December 2014); 15,092 were eligible. This pre-specified cross-sectional analysis describes eligible patients\u2019 baseline characteristics. Atrial fibrillation disease characteristics, medical outcomes, and concomitant diseases and medications were collected. Data were analyzed using descriptive statistics. Results Of the total patients, 45.5% were female; median age was 71 (interquartile range: 64, 78) years. Patients were from Europe (47.1%), North America (22.5%), Asia (20.3%), Latin America (6.0%), and the Middle East/Africa (4.0%). Most had high stroke risk (CHA2DS2-VASc [Congestive heart failure, Hypertension, Age  6575 years, Diabetes mellitus, previous Stroke, Vascular disease, Age 65 to 74 years, Sex category] score  652; 86.1%); 13.9% had moderate risk (CHA2DS2-VASc = 1). Overall, 79.9% received oral anticoagulants, of whom 47.6% received NOAC and 32.3% vitamin K antagonists (VKA); 12.1% received antiplatelet agents; 7.8% received no antithrombotic treatment. For comparison, the proportion of phase 1 patients (of N = 1,063 all eligible) prescribed VKA was 32.8%, acetylsalicylic acid 41.7%, and no therapy 20.2%. In Europe in phase 2, treatment with NOAC was more common than VKA (52.3% and 37.8%, respectively); 6.0% of patients received antiplatelet treatment; and 3.8% received no antithrombotic treatment. In North America, 52.1%, 26.2%, and 14.0% of patients received NOAC, VKA, and antiplatelet drugs, respectively; 7.5% received no antithrombotic treatment. NOAC use was less common in Asia (27.7%), where 27.5% of patients received VKA, 25.0% antiplatelet drugs, and 19.8% no antithrombotic treatment. Conclusions The baseline data from GLORIA-AF phase 2 demonstrate that in newly diagnosed nonvalvular atrial fibrillation patients, NOAC have been highly adopted into practice, becoming more frequently prescribed than VKA in Europe and North America. Worldwide, however, a large proportion of patients remain undertreated, particularly in Asia and North America. (Global Registry on Long-Term Oral Antithrombotic Treatment in Patients With Atrial Fibrillation [GLORIA-AF]; NCT01468701

Cardiac emergencies in neonates and young infants

Cardiac emergencies in children are not infrequent. Early recognition and management are essential to save life and prevent any comorbidity. The presentation of cardiac emergencies and etiologies is variable depending on the age of child at the time of presentation and type of cardiac lesion. Cyanotic and noncyanotic congenital heart diseases are the main causes in neonates and infants. Acquired heart diseases and dysrhythmia are more common causes for cardiac emergencies in toddler and childhood. In this review, we discuss the most common causes for cardiac emergencies in neonates and young infants highlighting important points in the presentation and management that are essential for early recognition and timely management of infants presenting with these conditions

Successful use of recombinant tissue plasminogen activator (r-TPA) for management of chylothorax associated with central venous thrombosis after neonatal cardiac surgery

Postoperative chylothorax is a frequently encountered pathology occurring in up to 5% of patients undergoing surgery for repair of congenital heart disease. Neck vein thrombosis can be associated with chylothorax and may contribute to its severity and duration. Furthermore, neck vessel thrombosis resulting in permanent vessel occlusion may hinder future management, diagnostic studies and cardio-surgical interventions. In this report we are describing a neonate who developed chylothorax on the 7th post-operative day following open-heart surgery. The chylothorax was linked to venous thrombosis in the cannulated right internal jugular vein with thrombus extending to the right atrium. After using low dose tissue plasminogen activator (r-TPA) infusion, the thrombi disappeared and the chylothorax resolved with no complications

Scimitar syndrome with tetralogy of fallot and pulmonary atresia

Scimitar syndrome is a rare variant of partial anomalous pulmonary venous connection. The association of Scimitar syndrome with another cardiac congenital anomaly such as tetralogy of Fallot with pulmonary atresia is extremely rare; we are reporting a successful combined treatment using transcatheter closure of major aorto-pulmonary collateral and a single-stage surgical correction in eighteen month old boy diagnosed as Scimitar syndrome with tetralogy of Fallot and pulmonary atresia

Bedside ultrasonography screening for congenital renal anomalies in children with congenital heart diseases undergoing cardiac repair

Introduction: Ultrasound (US) assessment of renal anomalies in children requiring pediatric cardiac surgery is not a standard practice. This study is highlighting the role of bedside US performed by intensivist to detect occult renal anomalies associated with congenital heart disease (CHD). Methods: A cross sectional study for 100 consecutive children with CHD admitted to Pediatric Cardiac Intensive Care Unit (PCICU) in 2015. US of kidneys screening was performed by trained pediatric cardiac intensivists to ascertain the presence of both kidneys in renal fossae without gross anomalies and to investigate if early detection of occult kidney anomaly would have any impact on outcome. Results: After screening of 100 consecutive children with CHD with renal US, we identified in 94 cases (94%) normal right and left kidney in the standard sonographer shape within the renal fossae. In 6 cases further investigation revealed ectopic kidney in 3 patients (50%), solitary functional kidney in 2 patients (33%) and bilateral grade IV hydronephrosis in one patient (17%). Urinary tract infection developed peri-operatively in 66% of the cases with kidney anomalies with statistical significance compared to patients with normal renal US (P: 0.0011). No significant renal impairment was noted in these patients post-surgery. We observed no specific association between the type of renal anomaly and specific CHD. Conclusion: Routine renal US in children with CHD demonstrated prevalence of associated congenital renal anomalies in 6% of children undergoing cardiac surgery. The presence of occult renal anomalies was associated with higher UTI risk. Performing routine renal US as a standard practice in children with CHD is justifiable. Keywords: Renal ultrasound, Congenital renal anomalies, Congenital heart diseas

Incidence and causes of prolonged mechanical ventilation in children with Down syndrome undergoing cardiac surgery

Introduction: Trisomy 21 is the most common syndrome in children with a 30–50% association with congenital heart disease (CHD). Cardiac surgeries are required in the majority of Down syndrome (DS) with CHD cases. Because of the distinctive abnormalities in their respiratory system, children with DS may require longer positive pressure ventilation after cardiac surgery. The aim of this study is to investigate the incidence and possible risk factors for prolonged mechanical ventilation (PMV) need in DS patients undergoing cardiac surgery. Methods: We conducted a prospective study on all DS children who underwent cardiac surgery from 2013 to 2016. Demographic and perioperative data were collected including the duration of mechanical ventilation, respiratory risk factors such as previous infection, evidence of pulmonary hypertension during the intensive care unit (ICU) stay, the presence of lung collapse, secretion and wheezy chest, inotropes score, sedation score, arrhythmias, and low cardiac output syndrome. Based on the duration of mechanical ventilation, cases were divided into two groups: the control group, comprising of children who required mechanical ventilation for less than 72 hours, and the PMV group, which consisted of children who required mechanical ventilation for 72 hours or more. Risk factors were compared and analyzed between both groups. Results: A total of 102 participants fulfilled the inclusion criteria, 90 of whom were assigned to the control group and 12 to the PMV group (11.7%). Compared with the control group, the PMV group had a higher incidence of pulmonary hypertension at a younger age (83% vs. 23%, p = 0.012) and 50% of them required chronic treatment for pulmonary hypertension upon home discharge. Pneumonia during ICU stay was encountered more frequently in the PMV group (33.3% vs. 2.2%, p = 0.0042). In addition, the PMV group had more frequent signs of low cardiac output syndrome after surgery (25% vs. 2.2%, p = 0.019), longer ICU stays (7 ± 0.3 days vs. 15.6 ± 2.1 days, p = 0.0001), needed more days of inotropes infusion (7.5 ± 0.4 days vs. 11.1 ± 1.6 days, p = 0.0045), and required more sedative and paralytic agents postoperatively (6 ± 0.6 days vs. 8.7 ± 1 days, p = 0.022). Conclusion: Overall, 11.7% of DS patients required prolonged ventilation after cardiac surgery. Pulmonary hypertension was seen more frequently in cases requiring PMV, and half of PMV cases required antipulmonary hypertension medication upon discharge. Early recognition of pulmonary hypertension and proper perioperative management are recommended to avoid serious complication and comorbidity after cardiac surgery. Keywords: Down syndrome, Pediatric cardiac surgery, Prolonged mechanical ventilation, Pulmonary hypertensio

Late presenting complete heart block after surgical repair of ventricular septal defect

Late onset of complete heart block is a life-threatening uncommon complication after surgical repair of congenital heart diseases. In this report, we discuss two cases of Perimembranous ventricular septal defect (VSD) that had late presentation of complete heart block after surgical repair. We are aiming to highlight this unusual complication for more awareness, better understanding and management of this unusual complication after pediatric cardiac surgery. Keywords: Complete heart block, Cardiac surgery, Ventricular septal defect (VSD

Predictors for the outcome of aortic regurgitation after cardiac surgery in patients with ventricular septal defect and aortic cusp prolapse in Saudi patients

Background and Aim: Aortic valve (AV) prolapse and subsequent aortic regurgitation (AR) are two complications of ventricular septal defects (VSD) that are located close to or in direct contact with the AV. This finding is one of the indications for surgical VSD closure even in the absence of symptoms to protect the AV integrity. The goal of our study was to assess the outcome and to identify the predictors for improvement or progression of AR after surgical repair. Materials and Methods: A retrospective study of all children with VSD and AV prolapse who underwent cardiac surgery at King Abdulaziz Cardiac Centre in Riyadh between July 1999 and August 2013. Results: A total of 41 consecutive patients, operated for VSD with prolapsed AV, with or without AR, were reviewed. The incidence of AV prolapse in the study population was 6.8% out of 655 patients with VSD. Thirty-six (88%) patients had a perimembranous VSD, and four had doubly committed VSD. Only one patient had an outlet muscular VSD. Right coronary cusp prolapse was found in 38 (92.7%) patients. Preoperative AR was absent in five patients, mild or less in 25 patients, moderate in seven, and severe in four patients. Twenty-six patients showed improvement in the degree of AR after surgery (Group A), 14 patients showed no change in the degree of AR (Group B) while only one patient showed the progression of his AR after surgery. Those with absent AR before surgery remained with no AR after surgery. Improvement was found more in those with mild degree of AR preoperatively compared to those with moderate and severe AR. Female gender also showed a tendency to improve more as compared to male. Conclusion: Early surgical closure is advisable for patients with VSD and associated AV prolapse to achieve a better outcome after repair and to prevent progression of AR in future