Outcome of truncus arteriosus repair: 20 years of single-center experience comparing early versus late surgical repair.

BACKGROUND: Truncus arteriosus is a rare CHD. Neonatal and early infancy repair is recommended though some cases may present late. The aim of our study is to investigate the current results of truncus arteriosus repair and to analyse the differences in outcome and reintervention need between early versus late truncus arteriosus surgical repair. MATERIAL AND METHODS: In this cohort study, we reviewed all children who underwent truncus arteriosus repair from 2001 till 2021. We divided patients into two groups; early repair group including patients repaired at age less than 3 months and late repair group including patients who had repair at 3 months of age and later. We compared both groups for outcome variables. RESULTS: Sixty-four children had truncus arteriosus repair including 48(75%) patients in early repair and 16(25%) patients in late repair groups. Peri-operative course was comparable between both groups. Post-surgery, we observed pulmonary hypertension in 6(12%) patients in early repair group comparing with 11(69%) patients in late repair group (p = 0.0001). In the last follow-up visit, pulmonary hypertension resolved in all early repair group patients while 6(37.5%) patients in late repair group continued to have pulmonary hypertension (p = 0.0001). Twenty-three(36%) patients required reintervention including 22(48%) in early repair group versus 1(6%) in late repair group (p = 0.007). CONCLUSION: In general, the outcome of early truncus arteriosus repair is excellent with resolution of pulmonary hypertension following early repair. Late repair caries higher risk of persistent pulmonary hypertension (37.5%). About one-third of the patients who had truncus arteriosus repair will require re-intervention within 38±38.4 months after initial surgery.

Original Article--Surgical site infections following pediatric cardiac surgery in a tertiary care hospital: Rate and risk factors.

BACKGROUND: Surgical site infections [SSIs] are the second most common type of healthcare-associated infections and leading cause of postoperative morbidity and mortality in pediatric cardiac surgery. This study aims to determine the rate of, risk factors for, and most common pathogen associated with the development of SSIs after pediatric cardiac surgery. METHODS: Patients aged ≤14 years who underwent cardiac surgery at our tertiary care hospital between January 2010 and December 2015 were retrospectively reviewed. RESULTS: The SSI rate was 7.8% among the 1510 pediatric patients reviewed. Catheter-associated urinary tract infection [CAUTI] [odds ratio [OR] 5.7; 95% confidence interval [CI] 2.3-13.8; P < 0.001], ventilator-associated pneumonia [VAP] [OR 3.2; 95% CI 1.4-7.2; P = 0.005], longer postoperative stay [≥25 days] [OR 4.1; 95% CI 2.1-8.1; P < 0.001], and a risk adjustment in congenital heart surgery [RACHS-1] score of ≥2 [OR 2.4; 95% CI 1.2-5.6; P = 0.034] were identified as risk factors for SSIs. Staphylococcus aureus was the most common pathogen [32.2%]. CONCLUSIONS: SSI risk factors were longer postoperative stay, CAUTI, VAP, and RACHS-1 score of ≥2. Identification and confirmation of risk factors in this study is important in order to reduce the rate of SSIs following cardiac surgery.

Palivizumab prophylaxis against respiratory syncytial virus infection in patients younger than 2 years of age with congenital heart disease.

BACKGROUND: Respiratory syncytial virus (RSV) is a viral pathogen that causes annual epidemics of lower respiratory tract infection with substantial morbidity and mortality in young children, especially those with congenital heart disease (CHD). Palivizumab is the only immunoprophylaxis therapy approved for RSV infection in infants with hemodynamically significant acyanotic or cyanotic CHD. OBJECTIVE: Identify the compliance rate with vaccination and study the effect of RSV vaccination on hospital admissions. DESIGN: Retrospective descriptive study. SETTING: Cardiac center. PATIENTS AND METHODS: Patient data was obtained from outpatient clinic records, inpatient records, and a surgical database for the period from October 2010 to March 2016. Infants with hemodynamically significant CHD, cyanotic CHD and moderate-to-severe pulmonary hypertension were included in the study. Palivizumab 15/mg/kg was given monthly starting from October, the usual beginning of the epidemic season, with five doses in the first season and six doses in the remaining season scheduled for administration. Patients were interviewed at every clinic visit for any side effects during the previous month, occurrence of any RSV infection and any hospital admission. Selection criteria included RSV vaccination and absence of RSV disease. Patients were excluded if they had RSV infection or a repaired cardiac lesion. MAIN OUTCOME MEASURES: Compliance rate, hospital admission frequency and period of stay. SAMPLE SIZE: 530 during six seasons of RSV epidemics. RESULTS: Fourteen patients (2.6%) developed RSV infection and 13 (2.5%) required hospital admission, but only one patient (0.1%) needed intensive care admission. There were no deaths related to RSV infection; however 11 patients died due to causes unrelated to RSV infection. The average compliance rate was 97%. CONCLUSION: Palivizumab was well tolerated and effective in the prophylaxis of severe RSV infection in children with CHD. As in other studies of palivizumab prophylaxis, we observed a reduction in hospital admissions. LIMITATIONS: Retrospective design. CONFLICT OF INTEREST: None.

Predictors of Reopening the Sternum in Children After Cardiac Surgery.

OBJECTIVES: Capillary leak syndrome can be severe in children after open-heart surgery which may hinder sternum closure and described as mediastinal tamponade. Reopening the sternum postoperatively may help maintaining hemodynamics and respiratory function. We looked for predictors that indicate the need for reopening the sternum. DESIGN: A retrospective cohort study. SETTING: A single cardiac center experience from 2009 to end of 2015. PATIENTS: All children who required emergent reopening the sternum in the pediatric cardiac ICU after cardiac surgery were grouped as index cases and matched to a control group for age, body weight, cardiac diagnosis, and type of repair (single vs biventricular). INTERVENTIONS: Emergent reopening the sternum. MEASUREMENTS AND MAIN RESULTS: With a ratio of two control cases for each index case, variables related to cardiac output (predictors) were collected in a time line of 12, 6 hours, and just before reopening the sternum. Morbidities and mortality were also reviewed. Thirty-three index cases were compared with 63 control cases. Hospital stay and hospital-acquired infections were the same between the groups. Ventilation hours were longer in the index cases. Temperature gap more than 3°C, inotropic score more than 14, and acute kidney injury indicated by doubled blood urea nitrogen and creatinine were higher in the index group 6 hours before reopening the sternum. Mortality was more in the reopening sternum group with higher risk when extracorporeal membrane oxygenation was needed. CONCLUSIONS: Low cardiac output after cardiac surgery in children in form of temperature gap more than 3°C, inotropic score more than 14, and acute kidney injury may predict the need of reopening the sternum. Rate of mortality was higher in the reopening sternum group when extracorporeal membrane oxygenation was needed.

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.

Role of intraoperative transesophageal echocardiography in pediatric cardiac surgery.

BACKGROUND: Intraoperative transesophageal echocardiography (TEE) has a major role in detecting residual lesions during and/or after pediatric cardiac surgery. METHODS: All pediatric patients who underwent cardiac surgery between July 2001 and December 2008 were reviewed. The records of surgical procedure, intraoperative TEE, and predischarge transthoracic echocardiograms were reviewed to determine minor and major residual cardiac lesions after surgical repair. RESULTS: During the study period, a total of 2268 pediatric cardiac patients were operated in our center. Mean age was 21 months (from 1 day to 14 years). Of these patients, 1016 (48%) had preoperative TEE and 1036 (46%) were evaluated by intraoperative echocardiography (TEE or epicardial study). We identified variations between TEE and preoperative transthoracic echocardiography in 14 patients (1.3%). Only one surgical procedure was cancelled after atrial septal defect exclusion. The other 13 patients had minor variation from their surgical plan. Major residual lesions requiring surgical revision were detected in 41 patients (3.9%), with the following primary diagnoses: tetralogy of Fallot in 12 patients (29%), atrioventricular septal defect in seven patients (17%), ventricular septal defect in seven patients (17%), double outlet right ventricle in two patients (5%), Shone complex in two patients (5%), subaortic stenosis in two patients (5%), mitral regurgitation in two patients (5%), pulmonary atresia in two patients (5%), and five patients (12%) with other diagnoses. CONCLUSION: Intraoperative TEE has a major impact in pediatric cardiac surgery to detect significant residual lesions. Preoperative TEE has a limited role in case of a high quality preoperative transthoracic echocardiography. We recommend routine use of intraoperative TEE during and/or after intracardiac repair in children.

Noncompaction cardiomyopathy: A new mechanism for mitral regurgitation with distinct clinical, echocardiographic features and pathological correlations.

UNLABELLED: Noncompaction cardiomyopathy (NCCM) is a primary, genetic cardiomyopathy with variable clinical manifestations that include mitral regurgitation (MR). METHODS: This study comprised patients diagnosed with NCCM and MR in two cardiac centers (King Abdul-Aziz Cardiac Center, Riyadh, Kingdom of Saudi Arabia and Sudan Heart Institute, Khartoum, Sudan), and seen in the period between 2002 and 2013. The study describes follow up, clinical, echocardiographic, and histopathological findings. RESULTS: Nineteen cases (85% females) were identified. Ten percent of the cases had relapses and remissions of heart failure. Echocardiographic features included leaflet retraction in all patients, characteristic malcoaptation, and a zigzag deformity of anterior leaflet in 57% of patients. Ruptured chordae were found in 15% of the patients. One patient had pathological examination of the mitral valve which showed myxomatous degeneration, and sclerotic and calcific changes. CONCLUSION: We describe and discuss a new mechanism for MR caused by NCCM with identifiable clinical and echocardiographic features, and pathological correlations.

Outcome of low body weight (<2.2 kg) infants undergoing cardiac surgery.

INTRODUCTION: Infants with low body weight (LBW) following cardiac surgery are a major challenge for the post cardiac surgery care unit. It has been observed that post surgery outcome for LBW infants is worse compared to the outcome of normal body weight infants. A study was conducted to compare post operative course and outcome of infants with body weight of 2.2 kg or less against infants with normal body weight who underwent similar cardiac surgeries. METHODS: A retrospective review was performed for all infants below 2.2 kg who underwent cardiac operations at King Abdulaziz Cardiac Center from January 2001 to October 2011. Cases with LBW (Group A) were compared with matching group (Group B) of normal body weight infants who had similar cardiac surgeries and matching surgical risk category. The demographic, ICU parameters, complications, and short-term outcome of both groups were analyzed. RESULTS: Two groups were formed, with 37 patients in Group A, and 39 patients in Group B. Except for weight (2.13 ± 0.08 kg in Group A vs 3.17 ± 0.2 kg in Group B), there was no statistical difference in demographic data between both groups. Cardiac procedures included coarctation repair, arterial switch, ventricular septal defect (VSD) repair, tetralogy of Fallot repair, systemic to pulmonary shunt and Norwood procedures. Patients in Group A had statistically significant difference from Group B in terms of bypass time (p = 0.01), duration of inotropes (p = 0.01), duration of mechanical ventilation (p = 0.004), number of re-intubations (p = 0.015), PCICU length of stay (p = 0.007), and hospital mortality: 13.5% in Group A vs 0% in Group B (p value 0.02). CONCLUSION: Patients with LBW (<2.2 kg) underwent cardiac surgery with overall satisfactory results, but with increased risk of ICU morbidity and mortality.

Short- and mid-term outcomes of total correction of Taussig-Bing anomaly.

Double-outlet right ventricle (DORV)/Taussig-Bing (TB) anomaly is the second most common type of DORV. This study evaluates our experience and outcomes of total correction of DORV-TB anomaly at King Abdulaziz Cardiac Center. We conducted a retrospective study for all cases of TB anomaly repaired between June 2001 and April 2009. Patients were divided into two groups: Group A included patients repaired with arterial switch operation, and group (B) included patients repaired with Rastelli procedure. Thirteen patients with TB anomaly underwent total correction. There were 5 male (38%) and 8 female (62%) patients. Mean age and weight at surgery were 6.8 ± 6 weeks and 3.6 ± 0.7 kg, respectively. Of the 13 patients, 9 (69%) were in group A, and 4 (31%) were in group B. Aortic arch abnormalities were present in 9 patients (69%); abnormal coronary artery patterns were present in 7 patients (54%); side-by-side great arteries were present in 5 patients (38%); dextrotransposition of the great arteries was present in 7 patients (54%); and levo-malposition of the great arteries was present in 1 patient (8%). At postoperative follow-up, 4 patients (31%) had developed either left- or right-ventricular outflow tract (VOT) obstruction requiring surgical and/or catheter intervention. There was no early mortality, but there was 1 late mortality caused by left-ventricle dysfunction. DORV-TB is often associated with other congenital cardiac anomalies. In general, total repair is feasible in the majority of patients with satisfactory results and improved outcome. Residual lesion and development of VOT obstruction can occur, requiring close follow-up and intervention for residual lesion.

Outcome of tracheostomy after pediatric cardiac surgery.

OBJECTIVE: To investigate the incidence, timing indications and outcome of tracheotomy in children who underwent cardiac surgeries. METHODS: All pediatric cardiac patients (under 14 years of age) who underwent cardiac surgeries and required tracheotomy from November 2000 to November 2010 were reviewed. The data were collected and reviewed retrospectively. RESULTS: Sixteen children underwent tracheotomy after cardiac surgery. Fifteen of these cases had surgery for congenital heart disease, and one had surgery for an acquired rheumatic mitral valve disease. The mean ± SEMs of the durations of ventilation before and after tracheotomy were 60.4 ± 9.8 and 14.5 ± 4.79 days respectively (P value 0.0002). The means ± SEM of the lengths of ICU stay before and after tracheotomy were 63.31 ± 10.15 and 22 ± 5.4 days respectively (P value 0.0012). After the tracheotomy 12/16 patients (75%) were weaned from their ventilators and 10/16 were discharged from the PCICU. Six patients were discharged from the hospital and 3 were successfully decannulated. The overall survival rate was 9/16 (56%). CONCLUSION: Tracheostomy shortens the duration of mechanical ventilation and facilitates discharge from the ICU. The mortality of tracheotomy patients is still significant but is mainly related to the primary cardiac disease.

Dextrocardia, aortopulmonary window with transposition of the great arteries, case report.

Transposition of the great arteries (D-TGA) in combination with aortopulmonary window (APW) is a very rare entity and carries high morbidity and mortality. Only few cases have been reported with this association. We report the first case of D-TGA and APW with mirror image dextrocardia which was repaired successfully.

Outcome of Norwood and Damus-Kaye-Stansel procedures for univentricular congenital heart anomalies.

OBJECTIVES: To review the experience with Norwood and Damus-Kaye-Stansel (DKS) staged repair in the management of hypoplastic left heart syndrome (HLHS), or functional single ventricle (FSV) with systemic outflow tract obstruction (SOTO). METHODS: A retrospective study was conducted from a single center from January 2001 to September 2007 at the Cardiac Sciences Department, King Abdulaziz Cardiac Center, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia. The cardiac departmental database was reviewed, together with the echocardiographic findings. Demographic data representing age and weight at operation, gender, cardiac anatomy, non-cardiac abnormalities, and operative details were collected. RESULTS: Twenty-eight patients with a diagnosis of HLHS were included in the study. The mean +/- SD for weight was 3.4 +/- 0.85kg and 32 +/- 37 days for age. All infants at our institution who underwent a Norwood or DKS surgery for HLHS, or other forms of FSV with SOTO were included. All included patients underwent first stage palliation consisting of either a classical Norwood procedure in 6/23 (21%) patients, or a modified Norwood with right ventricle to pulmonary artery (RV-PA) conduit in 22/28 (79%) patients. After first stage palliation, 23 patients (82%) survived, and all but one underwent second stage palliation with bidirectional cavopulmonary anastomosis (BCPA). The survival rate after second stage repair was 91%. Subsequently, 4 patients completed a third stage Fontan with 100% survival. All deceased patients had HLHS. CONCLUSION: The Norwood procedure is applied to a heterogeneous group of patients with variable outcomes in certain subgroups. Patients with HLHS palliated with the Norwood procedure are at a greater risk for morbidity and mortality compared to those with other forms of FSV with SOTO.

Early outcome of children with complex atrial isomerism undergoing uni-ventricular cardiac palliation.

OBJECTIVE: To review the postoperative intensive care unit (ICU) course and early outcome of children with complex atrial isomeric hearts undergoing cardiac surgery for uni-ventricular heart repair. METHODS: A retrospective review and analysis of ICU course of pediatric patients with atrial isomerism admitted from January 2000 to December 2004 in King Abdul-Aziz Medical City, Riyadh, Kingdom of Saudi Arabia, who underwent uni-ventricular repair. RESULTS: During the study period, 18 (n=18) patients were identified to have complex congenital heart disease (CHD) associated with atrial isomerism. They were in the form of right atrial isomerism (n=12), and left atrial isomerism (n=6). Eight patients did not meet the inclusion criteria of the study and were excluded. Ten of the 18 patients fulfilled the study criteria and underwent first stage uni-ventricular heart repair with 8 survivals (80%). Three of the 8 operated survival cases underwent second stage repair with 2 survivals (66%) and one patient completed Fontan surgery with a good outcome. An uneventful ICU course was noted in 3 of 10 operated patients (30%). Complications were noted in (40%) of cases including sepsis (n=4), tracheostomy (n=1), prolonged mechanical ventilation >7days (n=2). Over 50% of the operated patients required prolonged ICU stay (>2 weeks). CONCLUSION: Atrial isomerism is frequently associated with complex cardiac defects that often present in the neonatal stage and requires multiple staged cardiac surgeries. The surgical repair and peri-operative management of this group of patients can be difficult, challenging, and with potential risk of significant morbidity or mortality. In our setting, we found the outcome of children with atrial isomerism undergoing uni-ventricular palliation comparable to the literature.

A familial variant of the Scimitar syndrome with a meandering pulmonary vein.

We report a rare variant of the Scimitar syndrome, in which the right lower pulmonary vein takes a meandering course before finally connecting in normal fashion to the left atrium. The pulmonary parenchymal segment drained by the tortuous vein is supplied by a systemic collateral artery, which was coiled via a catheter. We also closed the intracardiac defects by surgery. The elder brother of the patient had classical Scimitar syndrome.

Bilateral scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly that affects classically the right lung and the heart. We present a rare variant that involves both lungs in association with totally anomalous pulmonary venous connection, horseshoe lung, and right pulmonary cyst.