Total pulmonary arterial reconstruction in a patient with arterial tortuosity syndrome affecting the pulmonary artery: a case report and review of the literature.

BACKGROUND: Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events. CASE PRESENTATION: A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg. CONCLUSION: ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

Custodiol versus blood cardioplegia in pediatric cardiac surgery: a randomized controlled trial.

BACKGROUND: Blood-based cardioplegia is the standard myocardial protection strategy in pediatric cardiac surgery. Custadiol (histidine-tryptophan-ketoglutarate), an alternative, may have some advantages but is potentially less effective at myocardial protection. This study aimed to test whether custadiol is not inferior to blood-based cardioplegia in pediatric cardiac surgery. METHODS: The study was designed as a randomized controlled trial with a blinded outcome assessment. All pediatric patients undergoing cardiac surgery with cardiopulmonary bypass and cardioplegia, including neonates, were eligible. Emergency surgery was excluded. The primary outcome was a composite of death within 30 days, an ICU stay longer than 5 days, or arrhythmia requiring intervention. Secondary endpoints included total hospital stay, inotropic score, cardiac troponin levels, ventricular function, and extended survival postdischarge. The sample size was determined a priori for a noninferiority design with an expected primary outcome of 40% and a clinical significance difference of 20%. RESULTS: Between January 2018 and January 2021, 226 patients, divided into the Custodiol cardioplegia (CC) group (n = 107) and the blood cardioplegia (BC) group (n = 119), completed the study protocol. There was no difference in the composite endpoint between the CC and BC groups, 65 (60.75%) vs. 71 (59.66%), respectively (P = 0.87). The total length of stay in the hospital was 14 (Q2-Q3: 10-19) days in the CC group vs. 13 (10-21) days in the BC group (P = 0.85). The inotropic score was not significantly different between the CC and BC groups, 5 (2.6-7.45) vs. 5 (2.6-7.5), respectively (P = 0.82). The cardiac troponin level and ventricular function did not differ significantly between the two groups (P = 0.34 and P = 0.85, respectively). The median duration of follow-up was 32.75 (Q2-Q3: 18.73-41.53) months, and there was no difference in survival between the two groups (log-rank P = 0.55). CONCLUSIONS: Custodial cardioplegia is not inferior to blood cardioplegia for myocardial protection in pediatric patients. Trial registration The trial was registered in Clinicaltrials.gov, and the ClinicalTrials.gov Identifier number is NCT03082716 Date: 17/03/2017.

Factors affecting the outcomes after bidirectional Glenn shunt: two decades of experience from a tertiary referral center.

BACKGROUND: Despite the improved management of patients with a single ventricle, the long-term outcomes are not optimal. We reported the outcomes of the bidirectional Glenn procedure (BDG) and factors affecting the length of hospital stay, operative mortality, and Nakata index before Fontan completion. RESULTS: This retrospective study included 259 patients who underwent BDG shunt from 2002 to 2020. The primary study outcomes were operative mortality, duration of hospital stay, and Nakata index before Fontan. Mortality occurred in 10 patients after BDG shunt (3.86%). By univariable logistic regression analysis, postoperative mortality after BDG shunt was associated with high preoperative mean pulmonary artery pressure (OR: 1.06 (95% CI 1.01-1.23); P = 0.02). The median duration of hospital stay after BDG shunt was 12 (9-19) days. Multivariable analysis indicated that Norwood palliation before BDG shunt was significantly associated with prolonged hospital stay (ß: 0.53 (95% CI 0.12-0.95), P = 0.01). Fontan completion was performed in 144 patients (50.03%), and the pre-Fontan Nataka index was 173 (130.92-225.34) mm2/m2. Norwood palliation (ß: - 0.61 (95% CI 62.63-20.18), P = 0.003) and preoperative saturation (ß: - 2.38 (95% CI - 4.49-0.26), P = 0.03) were inversely associated with pre-Fontan Nakata index in patients who had Fontan completion. CONCLUSIONS: BDG had a low mortality rate. Pulmonary artery pressure, Norwood palliation, cardiopulmonary bypass time, and pre-BDG shunt saturation were key factors associated with post-BDG outcomes in our series.

A robotic-assisted hybrid coronary revascularization program: Establishment and early experience in the Middle East.

BACKGROUND: Hybrid coronary revascularization (HCR) is a technique that merges coronary artery bypass grafting surgery and percutaneous coronary intervention (PCI) approaches for the treatment of multivessel coronary artery disease. The surgical component of the procedure is minimally invasive and can be done using robotic technology that avoids the need for sternotomy. Our objective is to study all patients who underwent robotic-assisted HCR (RHCR) to evaluate the feasibility and safety of the procedure during the establishment phase. METHODS: This study is a retrospective chart review conducted at King Faisal Specialist Hospital and Research Centre in Jeddah (KFSRC-J). The study focuses on patients who underwent RHCR between July 2018 to December 2020. The study was approved by the institutional review board #2020-103. RESULTS: Robotic-assisted HCR was performed on 78 patients (mean age, 56 years (range, 43-72 years); 89.75% males) during the study phase. Left internal mammary artery grafting was used in all patients. There was no hospital mortality, and the mean hospital and intensive care unit (ICU) stay were 5.8 and 1.4 days, respectively. We found that 93.6% of the patients had no blood transfusion. There were no major adverse cardiac events (MACE) and perioperative MI recorded. There was a 3.8% rate of postoperative complications. The percentage of surgeries converted to conventional and re-exploration for bleeding were 1.2% and 2.6%, respectively. The average operation time was 164 min. CONCLUSION: This study emphasizes on the safety and effectiveness of RHCR in treating patients with multivessel coronary artery disease. Moreover, robotic-assisted hybrid coronary revascularization offers an alternative, functionally complete revascularization option to a selected group of patients with minimal surgical trauma, short hospital and ICU length of stay, quick recovery, and little to no blood transfusion requirement.

Cardiac Catheterization During Extracorporeal Membrane Oxygenation After Congenital Cardiac Surgery: A Multi-Center Retrospective Study.

Cardiac catheterization can affect clinical outcomes in patients on extracorporeal membrane oxygenation (ECMO) after congenital heart surgery; however, its effect in this group of patients remains unclear. This study aimed to evaluate the safety and outcome of cardiac catheterization in patients undergoing ECMO after congenital cardiac surgery and determine predictors that influence successful weaning. This retrospective cohort study included pediatric patients who underwent cardiac catheterization while on ECMO after congenital heart surgery in two cardiac centers between November 2012 and February 2020. Predictors of successful weaning from ECMO were studied using univariate and multivariate logistic regression analyses. Of 123 patients on ECMO support after congenital cardiac surgery, 60 patients underwent 60 cardiac catheterizations (31 diagnostic and 29 interventional). Thirty-four (56.7%) and 22 patients (36.7%) underwent successful decannulation from ECMO support and survived after hospital discharge, respectively. Patients who underwent earlier catheterization (within 24 h of ECMO initiation) had more successful weaning from ECMO and survival compared to others. Patients who underwent an interventional procedure (interventional catheterization or redo cardiac surgery after cardiac catheterization) had better survival than those who underwent only diagnostic catheterization (P = 0.038). Shorter durations of ECMO was the most important predictor of successful weaning from ECMO. Early cardiac catheterization greatly impacts successful weaning from ECMO and survival. Patients with correctable lesions amenable either by catheterization or redo surgery are more likely to survive. Shorter durations of ECMO could have a significant influence on successful weaning from ECMO and survival.

The Role of Cardiovascular Surgery in the Management of a Patient Diagnosed With Congenital Cutis Laxa Syndrome Complicated by Multivalvular Heart Disease.

Cutis laxa syndrome is an uncommon connective tissue disorder affecting the major ultrastructure of the skin by progressive loss of elasticity. The results of this syndrome lead to the appearance of premature aging, which might also affect the internal organs. The disorder can be either congenital or acquired. The congenital form consists of autosomal dominant, autosomal recessive, and X-linked recessive patterns. The autosomal recessive pattern is the most common and severe one. Different systemic complications have been described in congenital cutis laxa syndrome, but the most serious and lethal one is cardiopulmonary abnormalities. In this report, we discuss the presentation of congenital cutis laxa syndrome with successful cardiovascular surgical management of multiple valvular heart diseases.

Mitral valve replacement in infants and younger children.

Data on mitral valve replacement (MVR) in young children is still limited. Our objective was to evaluate MVR in children below 5 years and identify factors affecting the outcomes. This retrospective study included 29 patients who had MVR from 2002 to 2020. We grouped the patients into two groups according to their age: age ≤ 24 months (n = 18) and > 24 months (n = 11). Primary cardiac diagnoses were Shone complex (n = 7; 24%), isolated congenital mitral valve abnormality (n = 11; 38%), and complete atrioventricular septal defect (n = 3; 10%). The median age was 19 month (25th-75th percentile: 11-32) and 59% were females (n = 17). The hemodynamic lesions were mitral regurgitation in 66%, mitral stenosis in 10%, and combined mitral stenosis and regurgitation in 24% of the patients. St. Jude mitral valve was the most common valve implanted (n = 19, 66%), followed by CarboMedics in 21% of the patients (n = 6). The mitral valve was implanted in the supra-annular position in 6 cases (21%). Preoperative and operative data were comparable between both groups. There was no association between valve size and position with postoperative heart block (P > 0.99, for both). The median follow-up duration was 19.4 months (8.6-102.5). Nine patients had mitral valve reoperation, six had MVR, and three had clot removal from the mitral valve. There was no effect for age group on reoperation (SHR 0.89 (95% CI 0.27-2.87), P = 0.84). Valve size significantly affected reoperation (SHR 0.39 (95% CI 0.18-0.87), P = 0.02). The supra-annular position was associated with an increased risk of reoperation (SHR 3.1 (95% CI 1.003-9.4), P = 0.049). There was no difference in survival according to the age (Log-rank P = 0.57) or valve size (Log-rank P = 0.66). Mitral valve replacement in children is associated with low morbidity and mortality. The risk of reoperation could be affected by the valve size and position rather than the age.

Bovine jugular vein valved xenograft for extracardiac total cavo-pulmonary connection: The risk of thrombosis and the potential liver protection effect.

BACKGROUND: Currently, non-valved conduits are preferred for extracardiac total cavo-pulmonary connection (TCPC). However, previous work has failed to provide objective data comparing the postoperative outcome between non-valved TCPCs and bovine jugular vein valved xenograft (BJV) TCPCs. Hence, the objective of this study is to compare the postoperative outcomes in extracardiac TCPC patients who received BJV vs synthetic non-valved conduits and evaluate the effect of BJV on liver fibrosis. METHODS: Of 206 patients who had extracardiac TCPC from 2002 to 2017 were divided into three groups. Group A (n = 66) received BJV, group B (n = 37) received PET conduits and group C (n = 103) received polytetrafluoroethylene (PTFE) tube. Study endpoints were hospital outcomes, conduits thrombosis, reinterventions, and survival. Liver stiffness and fibrosis were assessed in eight patients with BJV. RESULTS: Preoperative parameters were comparable among groups. Thrombosis was significantly lower in group C (P < .0003) but no difference between groups A and B (P = .951). Reinterventions did not differ significantly among groups (Log-rank P = .598). Hospital deaths occurred in seven patients (3.4%). There was no difference in survival between groups (Log-rank P = .221). The median liver stiffness score was 18.65 kPa and the eight patients had advanced liver fibrosis (grade F3-4) in group A. CONCLUSION: PTFE is the recommended conduit for TCPC with a lower risk of thrombosis compared to BJV and PET. BJV conduits in TCPC circuits may not protect against liver fibrosis. BJV should not be considered as an option for TCPC.

Evolution of the Norwood operation outcomes in patients with late presentation.

OBJECTIVES: We present the evolution of Norwood operation outcomes and practice pattern changes over 15 years from a single institution in Saudi Arabia. We intended to identify time trends in patient selection, procedural details, and outcome predictors over time. METHODS: Patients who underwent a Norwood operation (n = 145) between 2003 and 2018 with the use of a Blalock-Taussig shunt (BT group; n = 72), right ventricle to pulmonary artery shunt (Sano group; n = 66), or a primary cavopulmonary shunt (CPS group; n = 7) were included. The study outcomes were operative mortality, long-term survival, and multistate transition to CPS, Fontan, and death. RESULTS: Median age was 29 days. Predictors of operative mortality were lower weight (P = .026), and longer bypass time (P = .014), whereas age, and type of shunt were not. Predictors of improved long-term survival were greater weight at operation (P = .0016), later era (P = .006), and shorter bypass time (P = .001). The multistate model revealed that patients with lower weight were more likely to undergo Sano versus BT (P < .001), and if BT was chosen in such patients, they were more likely to die (P = .027). The likelihood of receiving Sano shunt was 3-fold greater in the recent era (P = .003). CONCLUSIONS: Improved outcomes of the Norwood operation are evident in the recent era and with Sano shunt, especially in patients of smaller weight. Late presentation or older age is not a contraindication to Norwood operation. The incorporation of a primary CPS at stage one operation is feasible in selected patients.

Extracorporeal Membrane Oxygenation in Postcardiotomy Pediatric Patients-15 Years of Experience Outside Europe and North America.

BACKGROUND: The increasing complexity of congenital cardiac surgery has resulted in the increased use of extracorporeal membrane oxygenation (ECMO) support for children who cannot be weaned from cardiopulmonary bypass. The purpose of this research was to assess the mortality and morbidity in children requiring ECMO support after the repair of congenital heart defects (CHDs). METHODS: The hospital records of all patients with CHD who required ECMO after a cardiac surgical procedure between January 2001 and December 2016 were retrospectively reviewed. Various outcomes were reported and tested for any association with hospital death. RESULTS: A total of 113 children required ECMO for cardiopulmonary support after congenital cardiac surgery; 88 (77.9%) were placed on ECMO in the operating room. Median age of the patients was 3 months (range, 4 days-15 years) and median weight was 3.5 kg (range, 2.2-42.5). Forty-two (37.2%) survived to hospital discharge. In children with single-ventricle physiology, survival to discharge was 37.3% (19/51 patients) and for biventricular physiology, it was 37.1% (23/62 patients). Univariate analysis revealed number of days on ECMO support, renal failure, and stroke as risk factors for hospital mortality, while age and cross-clamp time were found to be statistically nonsignificant. CONCLUSION: Satisfactory results can be achieved in pediatric patients by using ECMO support for postoperative cardiac and pulmonary failure refractory to medical management. Prolonged ECMO support, renal failure, and stroke are risk of mortality.

Staged biventricular repair for neonates with left ventricular outflow tract obstruction, ventricular septal defect, and aortic arch obstruction.

BACKGROUND: The purpose of this study is to evaluate clinical outcomes of neonates who underwent a Norwood operation as a first step of a planned biventricular repair and the impact of associated risk factors. METHODS: A retrospective cohort study was performed on all neonates (n = 44) undergoing the Norwood operation as the first stage of a biventricular (Norwood-Rastelli) repair from January 2000 to December 2012 at a single center. Multivariable analysis was performed to identify predictors of survival. RESULTS: Stage one mortality was 9%. The interstage survival for nonsyndromic and syndromic patients was 100% versus 46%, respectively (p < 0.001). Twenty-four patients (55%) underwent biventricular completion repair with no mortality. Freedom from reintervention after biventricular completion was 53% at 6 years. The overall survival for nonsyndromic patients versus syndromic patients was 86% versus 43%, respectively (p = 0.01). Genetic syndromes and prematurity were significant predictors of interstage mortality on multivariable analysis. CONCLUSIONS: Staged biventricular repair for patients with complex left ventricular outflow tract obstruction, ventricular septal defect, and aortic arch obstruction can be achieved with excellent outcomes for neonates without genetic syndromes. The staged approach is associated with longer time to reintervention after the biventricular completion.

Selective antegrade cerebral perfusion during aortic arch surgery confers survival and neuroprotective advantages.

OBJECTIVE: To assess the impact of using antegrade cerebral perfusion during aortic arch surgery on postoperative survival and neurologic outcomes. METHODS: All operations were performed at the same hospital between January 2001 and January 2009. Patients undergoing aortic arch surgery using antegrade cerebral perfusion during deep hypothermia were compared with patients undergoing aortic arch surgery without antegrade cerebral perfusion during the same study period. Multivariable logistic regression and Cox proportional hazards model were used to identify predictors of postoperative cerebrovascular accidents and midterm survival, respectively. There were 46 patients in the antegrade cerebral perfusion group and 78 patients in the non-antegrade cerebral perfusion group. RESULTS: There were no statistically significant differences in age, proportion of emergency operations, or proportion of type A aortic dissection between the 2 groups. There was a statistically significant and clinically important difference in the rates of postoperative cerebrovascular complications (2% antegrade cerebral perfusion vs 13% non-antegrade cerebral perfusion, P = .03), postoperative duration of mechanical ventilation (1.15 ± 0.19 days antegrade cerebral perfusion vs 2.13 ± 0.38 days non-antegrade cerebral perfusion, P = .02), and 3-year survival (93% antegrade cerebral perfusion vs 78% non-antegrade cerebral perfusion, P = .03). Antegrade cerebral perfusion was shown to be a significant predictor of reduced postoperative stroke rates and better survival at 3 years. CONCLUSIONS: Antegrade cerebral perfusion was associated with improved survival and neurologic outcomes in patients undergoing aortic arch surgery, especially for cases requiring prolonged aortic arch repair periods.

Repair of an acute type A aortic dissection combined with an emergency cesarean section in a pregnant woman.

This case report describes a 35-week pregnant woman presenting with an acute type A aortic dissection. She underwent a successful emergency surgical repair and a concomitant cesarean section with a favorable outcome for the mother and the child.

Ex vivo bilateral pulmonary embolectomy for donor lungs prior to transplantation.

Pulmonary arterial embolic disease of donor lungs is a known entity. Its implications on the technicality, the perioperative management, and the outcome of lung transplantation depend on the extent of the embolic disease and the indicators of lung function at the time of procurement. We report a case of lung transplantation from a donor who was known to have significant acute pulmonary embolic disease and the perioperative management used to optimize the outcome.

Bilateral diaphragmatic plication in the setting of bilateral sequential lung transplantation.

Diaphragmatic paralysis can lead to significant ventilatory impairment, especially if associated with underlying lung disease. Adequate ventilatory mechanics are essential for good outcomes after lung transplantation. We report a case of bilateral diaphragmatic plication at the time of double lung transplantation as an attempt to improve posttransplant ventilation, with good outcome.

Spontaneous closure of atrial septal defects within the oval fossa.

OBJECTIVES: To estimate the incidence and timing of spontaneous closure of atrial septal defects within the oval fossa, to study the modalities for diagnosis, and predict the need for therapeutic intervention. METHODS: We reviewed retrospectively the medical records of patients with isolated atrial septal defects within the oval fossa, so-called "secundum defects", diagnosed between January 1990 and February 2003. Based on the initial echocardiographic evaluation, we divided defects into small ones measuring from 3 to 5 mm, medium ones from 5 to 8 mm, and large ones greater than 8 mm. RESULTS: We identified 121 patients, 50 (41.3%) of whom had failed to thrive, and 14 (11.6%) had congestive heart failure. At a mean of 44.9 +/- 22.1 months following diagnosis, with a range from 12 to 102 months, the defects had closed spontaneously in 31 patients (25.6%). Of 22 patients having small defects, spontaneous closure occurred in 18 (82%) at a mean age of 18.9 +/- 10.2 months. Of 27 patients with defects of medium size, 12 (44%) either experienced spontaneous closure, or else the defect effectively became a patent oval foramen, at a mean age of 51.2 +/- 32.2 months. Only 1 (1.4%) of the 72 patients with a defect larger than 8 mm in size underwent spontaneous closure. The defects increased in size in 8 patients (6.6%). Intervention was necessary in 76 patients (63%) at a mean age of 75.5 +/- 15.2 months. CONCLUSION: The initial size of a defect within the oval fossa at diagnosis is the best predictor of its natural history. Some defects increase in size with growth, irrespective of their initial size.