Original Article--Risk Factors for Neurological Complications and Short-term Outcomes After Pediatric Heart Surgery: A Retrospective Analysis.

Objective: To assess the risk factors associated with neurological complications and poor short-term outcomes following pediatric heart surgery. Methodology: A cross-sectional study was conducted in a cardiac intensive care unit between June 2019 and June 2022. The data of all children less than 15 years old who underwent open-heart surgery and had CT brain were extracted from hospital records. The primary outcome was the incidence of CNS insult, and secondary outcomes included death after surgery, length of stay in ICU and hospital. Data analysis was performed using SPSS version 23, and a p-value less than or equal to 0.05 was considered statistically significant. Results: Total 1850 surgeries were performed in the specified period of time. The study included 208 children who had CT Brain, with a median age of 5 months. 2.81 % children had neurological complications, with 25 % of patients who had CT brain. The most common neurological complication was seizure (7.2 %). There were no significant differences observed between CNS insult and age, gender, syndrome, or prematurity (p > 0.05), except for a significant association between previous CNS insult and CNS insult after surgery (p = 0.001). Children with CNS insult had significantly higher ICU and hospital length of stay, mortality after surgery, and mortality within 2 weeks of surgery (p ≤ 0.05). Conclusion: Seizure was most common neurological manifestation after cardiac surgeries in children. CNS insult after surgery was associated with worse outcomes, including longer hospital stays and increased mortality.

Central pontine myelinolysis and locked-IN syndrome associated with tacrolimus after pediatric heart transplantation.

BACKGROUND: Locked-in syndrome represents the most severe form of central pontine myelinolysis and varies in presentation from asymptomatic to fully developed locked-in-syndrome characterized by the combination of quadriplegia, loss of the ability to communicate except through the use of the eyes, and an inability to follow commands. METHODS: We report a 10-year-old boy who developed a severe case of locked-in syndrome after heart transplantation. RESULTS: Patient had a spontaneous recovery, treated with supportive treatment and the improvement was detected with cessation of calcineurin inhibitor therapy by substituting with an mTOR inhibitor (sirolimus). No cases of locked-in syndrome post-heart transplant in pediatrics cases have been documented in the literature. CONCLUSION: Physicians should recognize a rapid progression of central pontine myelinolysis and locked-in syndrome in the context of heart transplant and although several factors likely contributed to this outcome, adjustment of immunosuppression including by substituting tacrolimus with sirolimus could be effective.

Loss of engagement in controlling chronic anticoagulation therapy during Covid-19 stringency measures. A single center experience of disproportioned increase of stuck mechanical valves.

Stuck valve is a very rare and severe complication that occurs in mechanical valve replacement patients with ineffective anticoagulation. However, with COVID-19 restriction measures, it became challenging to regularly assess INR to make sure it falls within the target therapeutic range to prevent this complication. We present a series of 10 patients who either underwent transthoracic echocardiography for a suspected stuck valve or were seen at the outpatient valve clinic with the residual consequences of a stuck valve during the COVID-19 restriction measures in our institute. Stuck prosthetic valves incident has increased significantly during this period, particularly those in the mitral position for which urgent replacement and prolonged hospitalization were necessary. Particularly with the COVID-19 restrictions in place, these cases highlight the need for physicians to be aware of the dramatic increase in the incidence of stuck prosthetic valves in patients on chronic warfarin therapy.

Postcardiosurgical Pseudoaneurysm.

Takayasu's arteritis is an autoimmune inflammatory disease of large arteries. We report a case of postcardiac surgery pseudoaneurysm. Anesthetic concerns, high risk related to surgery, necessary anesthetic preparations, and considerations will be mentioned here.

Fetal aortic valvuloplasty: first report of two cases from Saudi Arabia.

BACKGROUND: Fetal aortic stenosis may progress to hypoplastic left heart syndrome (HLHS), which carries a poor prognosis. We report two infants with fetal aortic stenosis successfully treated with fetal aortic valvuloplasty (FAV) using balloon dilatation. CASE PRESENTATION: Of five fetuses with aortic stenosis fulfilling the FAV criteria of severe aortic stenosis with a left ventricular length Z-score of ≥ - 2, retrograde flow in the transverse aortic arch, left-to-right flow across the foramen ovale, monophasic mitral inflow, and significant left ventricular dysfunction, we obtained permission for FAV in two fetuses. FAV was performed successfully under echocardiographic guidance using balloon dilatation. Both fetuses survived to birth. During FAV, mild pericardial effusion developed when introducing the stylet needle in the second fetus, and this resolved within 48 h. No intraprocedural complications occurred in the first patient, and no maternal complications occurred. The first infant underwent the Ross procedure after birth and is currently 7 years old and doing well. The second patient underwent aortic and mitral valve repair with endocardial fibroelastosis resection approximately 2 weeks after birth, which temporarily addressed the mitral valve stenosis; high doses of inotropes were subsequently required. The infant died of sepsis at 2 months of age. CONCLUSION: FAV using balloon dilatation to treat fetal aortic stenosis was successful in our two patients, with subsequent neonatal biventricular repair resulting in long-term survival in one patient and death secondary to sepsis in the second patient.

Outcome of cardiac surgery in adults with congenital heart disease: A single center experience.

OBJECTIVES: Adult survivors with congenital heart diseases represent a large and growing population, yet the published data does not represent the magnitude of their needs specifically in the Middle East. We aimed to review our experience at King Faisal Heart Center, Riyadh, Saudi Arabia for the outcome of adult patients with congenital heart disease who underwent either primary or redo surgery. METHODS: A retrospective study at a tertiary care hospital. All patients who underwent surgery either as the first surgery or as a reoperation for congenital heart disease aged >16 years old at the time of cardiac surgery in the period between January 1, 2008 and January 1, 2013. We looked for incidence of postoperative bleeding, arrhythmias, acute kidney injury, neurological complications, duration of mechanical ventilation, hospital and intensive care unit (ICU) stay. Additionally, we assessed the mortality and 1- and 5-year survival. RESULTS: Ninety-eight patients were included in our study. Fifty-two (53%) were females and 46 (47%) were males, with a mean age of 26 ±â€¯8.4 years and a mean weight of 62 ±â€¯22.8 kg. Forty-nine patients (50%) required redo surgery. Ten patients (10%) suffered from postoperative bleeding. Eight patients (8%) had postoperative arrhythmias, of which two patients required permanent pacemaker insertion. Three patients (3%) had postoperative acute kidney injury and seven patients (7%) suffered from neurological complications. The mean duration of ventilation was 1.3 ±â€¯2 days, with a mean ICU and hospital stay of 3.7 ±â€¯3 days, and 10 ±â€¯7 days, respectively. The overall mortality rate in our series was 4% with a 1-5-year survival of 96%. CONCLUSION: Adult patients with congenital heart disease are prone to immediate postoperative multisystem complications, yet the majority of them are reversible. Their 1- and 5-year survival rate is excellent. Further follow up studies are required.

Assessment of Late Thromboembolic Complications Post-Fontan Procedure in Relation to Different Antithrombotic Regimens: 30-Years' Follow-up Experience.

Background: The current CHEST guidelines recommend the use of antithrombotic therapy, either aspirin or warfarin, as a primary thromboembolic complications (TECs) prophylaxis in patients who undergo Fontan procedure, without specification on drug selection or duration of therapy. Objective: To investigate the incidence rate of late TECs, occurring after 1-year post-Fontan procedure and to assess the difference in rate of late TECs between warfarin and aspirin. Methods: A retrospective cohort study included patients who had Fontan procedures between 1985-2010 at our institution. Patients were stratified according to the antithrombotic regimen-warfarin, aspirin, or no therapy-at the time of TECs. Results: We screened 499 patients who underwent Fontan procedures; 431 procedures met the inclusion criteria. Over a median follow-up of 13.6 years (IQR= 8.7), freedom from late TECs at 5, 10, 15, and 20 years was 97.54%, 96.90%, 90.78%, and 88.07%, respectively. There was no difference in late TEC incidence rates per 1000 patient-years between warfarin and aspirin: 7.82 and 5.83 events, respectively; rate ratio= 1.34 (95% CI= 0.68-2.60). Warfarin was associated with a higher major bleeding incidence rate per 1000 patient-years: 3.70 versus 2.91 events with aspirin; rate ratio= 1.27 (95% CI= 0.49 to 3.29). Conclusion and Relevance: The incidence rate of late clinical TECs post-Fontan procedure in our population is low. Warfarin was not superior to aspirin for prevention of late TECs. Yet warfarin was associated with a higher rate of bleeding. This finding suggests a simpler antithrombotic regimen for prevention of TEC after 1-year post-Fontan procedure.

Left circumflex artery injury postmitral valve surgery, single center experience.

The left circumflex (LCX) artery is located close to the mitral valve (MV), making it susceptible to injury during MV surgery. We are reporting our experience in the diagnosis and management of this complication. We retrospectively reviewed our surgical and coronary angiography databases for patients with documented LCX artery injury during MV surgery between January 2000 and December 2016. The complication was associated with MV replacement (9/1313, 0.7%) but not MV repair (0/393, 0.0%). Eight patients (88.9%) were female and the mean age was 40.4 ±â€¯14.2 years. There was roughly similar distribution of left and right dominant coronary circulations (5 and 4 patients, respectively). Eight patients (88.9%) had ischemic changes on electrocardiogram and ventricular arrhythmias were documented on six patients (66.7%). Three patients (33.3%) were treated with percutaneous coronary intervention while six patients (66.7%) required redo surgery to graft the LCX artery. The 30-day mortality was high (33.3%). A high index of suspicion is required to diagnose this injury. At the moment, no consensus is available on the optimal treatment strategy. We propose percutaneous approach as the first option to spare the patients from undergoing open-heart surgery for the second time.

Transcatheter versus surgical valve replacement for a failed pulmonary homograft in the Ross population.

BACKGROUND: Patients who undergo the Ross procedure are at increased risk of pulmonary valve (PV) homograft dysfunction. For those who require reintervention on the homograft, transcatheter PV replacement (tPVR) provides a less invasive therapeutic option than surgical PVR (sPVR). We examined the outcomes following tPVR versus sPVR in a cohort of patients who underwent the Ross procedure. METHODS: We performed a retrospective analysis of Ross patients age ≥14 years who underwent tPVR (n = 47) or sPVR (n = 41) at our institution. The patients' clinical and echocardiographic data were reviewed. RESULTS: Baseline parameters, including demographic data and left ventricular and right ventricular (RV) systolic function, were similar in the 2 groups. The mean follow-up was 56 ± 24 months for the tPVR group and 89 ± 46 months for the sPVR group (P < .001). No procedure-related mortality was noted in either group. At 6-year follow-up, there was no significant between-group difference in event-free survival (tPVR, 79% ± 7% vs sPVR, 91% ± 4%; P = .15) or PV reintervention (tPVR, 26% ± 9% vs sPVR, 8% ± 5%; P = .31). PV-associated infective endocarditis (IE) was significantly more common with tPVR (tPVR, 13% vs sPVR, 0%; P = .04), with an annualized rate of 2.98% per patient-year. In addition, there was a trend toward more valve dysfunction following sPVR (sPVR, 67% ± 8% vs tPVR, 35% ± 8%; P = .08). CONCLUSIONS: In Ross patients who require reintervention on the PV homograft, both tPVR and sPVR provide low procedural mortality and comparable midterm outcome with no significant difference in mortality or PV reintervention. However, IE is more common following tPVR. A larger randomized study is needed to determine the role of each procedure in patient management.

Grave aortic aneurysmal dilatation in DOCK8 deficiency.

Hyperimmunoglobulin E syndrome (HIES) is a primary immunodeficiency disorder with multisystem abnormalities including the vascular system. We report a child with autosomal recessive (AR)-HIES secondary to dedicator of cytokinesis 8 (DOCK8) deficiency who developed critical aortic aneurysm involving the ascending aorta and aortic arch with narrowing of descending aorta that was successfully managed surgically. This report highlights the underrecognized and serious complication of DOCK8 deficiency that could contribute to significant morbidity and mortality in such patients.