Background
Pediatric cardiomyopathies (CMP) can be familial or idiopathic with increasing detection of genetic mutations. The study is a retrospective single-center review of cardiomyopathy patients ...from January 2011 to May 2020. Results of the genetic study, as well as the outcome, were reported. Patients were divided according to the type of CMP, age of presentation, and EF at presentation. Univariate and multivariate analysis and ROC and survival curves were done.
Results
We reported 229 patients under 14 years of age with a diagnosis of cardiomyopathy, most commonly DCM (160 patients (70%)) followed by HCM (26.2%). 52% presented at 6 months of age or less and 119 (52%) required ICU admission at presentation. The genetic and or metabolic disorder was confirmed in 21.4% of patients, most commonly VLCAD defect (16, 7%) and ELAC2 gene defect (10, 4.4%). During the disease course, 88 patients (38.4%) died (48 with DCM, 39 with HCM, and 1 with RCM). An EF of 20% or less at presentation and presentation at 6 months of age or less carries a risk for mortality in patients with DCM and HCM, respectively (RR 3.88 and 2.06 and OR of 11.09 and 4.35, respectively). Death was more common among HCM patients especially patients with positive genetic abnormality compared with patients with DCM.
Conclusions
The mortality for CMP in children reaches up to 40%, (30% in DCM and 65% in HCM patients). Mortality was higher in those with HCM, DCM with EF of 20% or less, and HCM presented at 6 months of age or less. Whole-exome and/or whole-genome sequencing is advised for all patients of CMP and at-risk family members.
Background
Postcardiotomy extracorporeal membrane oxygenation (ECMO) in pediatric patients can be affected by the place of initiation, either in the operating room (OR) or the pediatric cardiac ...intensive care unit (PCICU). This study aimed to characterize and compare patients who had postcardiotomy ECMO initiation in the OR or PCICU and evaluate risk factors for hospital mortality.
Methods
This retrospective study included 103 patients who required postcardiotomy ECMO support after the repair of congenital cardiac lesions from 2010 to 2022. Patients were grouped according to the place of ECMO insertion into two groups. Group 1 (n = 69) had ECMO insertion in the OR, and Group 2 (n = 34) had ECMO insertion in the PCICU.
Results
Cardiac arrest occurred significantly more often in patients with ECMO insertion in the PCICU (21 (61.76%) vs. 13 (18.84%); p < 0.001). Pre-ECMO lactate levels, pH, VIS, base deficit, and PaO2 did not differ between the groups. Re-exploration for bleeding was significantly higher in Group 1 (32 (46.38%) vs. 8 (23.53%); p = 0.03). Cannula repositioning (4 (11.76%) v. 2 (2.90%); p = 0.09) and mechanical ventilation time were nonsignificantly higher in Group 2 (19.5 (10–31) vs. 11 (5–25) days; p = 0.07). No difference in mortality was found between groups (42 (60.87%) vs. 23 (67.65%), p = 0.50). By multivariable analysis, elevated lactate on ECMO and low pH before ECMO were associated with mortality.
Conclusions
ECMO insertion in the OR has a comparable mortality rate to PCICU insertion. Pre-ECMO low pH and high lactate during ECMO could predict mortality.
Intracardiac thrombosis and distant thromboembolic events are rare complications of patients diagnosed with any type of cardiomyopathies. The low prevalence of this entity makes it challenging and ...unfortunate for the patients and their families. This review aims to add to the current limited available data describing similar clinical entities.
Intracardiac thrombosis and distant thromboembolic events are rare complications of patients diagnosed with any type of cardiomyopathies. The low prevalence of this entity makes it challenging and unfortunate for the patients and their families. This review aims to add to the current limited available data describing similar clinical entities.
Background:
Improved survival with postoperative extracorporeal membrane oxygenation (ECMO) has expanded its application to children with single ventricle (SV) anomalies. We examine current-era ...outcomes of postoperative ECMO with special focus on patients with SV.
Methods:
Demographic, anatomic, surgical, and support details of 100 consecutive children requiring postoperative ECMO (2007-2012) were included into multivariable regression models to identify factors affecting survival.
Results:
Median age was 73 days (4 days-16.2 years), 31 patients had SV physiology. The ECMO indication was failure to wean cardiopulmonary bypass (34%) and postoperative low cardiac output (66%) including 37% having extracorporeal cardiopulmonary resuscitation (ECPR). Median ECMO duration was four days (1-21). The ECMO decannulation and survival to hospital discharge were 62% and 37%. In SV group, decannulation and survival rates were 55% and 32%. The SV-ECMO outcomes were best in ECPR subgroup (54%), following shunt (57%) or Norwood (46%) and worst following Glenn, Fontan, or total anomalous pulmonary venous connection repair (0% survival). On multivariable analysis, factors affecting odds of survival were performing angiogram (odds ratio OR: 15.28, confidence interval CI: 2.34-99.89, P = .004), prolonged ECMO duration (OR: 0.64, CI: 0.47-0.88 per day, P = .005), leaving cannulation snares (OR: 28.41, CI: 2.65-304.70, P = .006), higher HCO3 (OR: 1.19, CI: 1.04-1.36, P = .01), renal failure requiring hemodialysis (OR: 0.21, CI: 0.06-0.76, P = .02), bleeding requiring re-exploration (OR: 0.21, CI: 0.06-0.75, P = .02), ECPR in patients with SV (OR: 11.84, CI: 1.11-126.07, P = .04), delayed lactate normalization (OR: 0.95, CI: 0.90-0.99 per hour, P = .02), and elevated liver enzymes (OR: 0.97, CI: 0.95-1.00 per 10 unit/L, P = .05).
Conclusions:
The ECMO is valuable in patients with SV however results depend on anatomy, procedure, and support indication. Persistent markers of poor perfusion, end-organ injury, and prolonged ECMO duration are associated with mortality. Those factors could be modified by early ECMO application before organ damage, meticulous homeostasis to ensure adequate perfusion, early diagnosis, and reoperation on residual lesions to expedite weaning.
Coagulopathy is a well-recognized complication in patients with coronavirus disease 2019 (COVID-19). Pulmonary embolism (PE) has substantial morbidity and mortality if the diagnosis is missed or the ...management is delayed. Computed tomography pulmonary angiography (CT-PA) is the imaging modality of choice for PE. Therefore, this study aimed to investigate the positive rate of CT-PA for PE among patients with COVID-19.
We conducted a retrospective study examining the diagnostic yield of CT-PA in patients with confirmed COVID-19 and compared it with that in patients without COVID-19. The study included all adult patients with confirmed COVID-19 who presented from June 2020 to June 2021.
The study included 316 patients, including 158 patients with COVID-19, who underwent CT-PA for ruling out PE. Overall, 76 patients were found to have PE on the CT-PA scan, yielding a positive rate of 24.1%, with a significant difference between patients with COVID-19 (8.2%) and those without COVID-19 (39.9%). Further, 138 (87.3%) patients with COVID-19 had elevated D-dimer levels compared with 34 (21.5%) patients without COVID-19. A multivariable regression analysis model revealed that the smoking status (odds ratio OR = 1.94; 95% confidence interval CI: 1.4-3.8) and obesity (OR = 4.1; 95% CI: 1.5-8.9) were independent predictors of PE among patients with COVID-19. However, the elevated D-dimer level was not significantly associated with PE among patients with COVID-19 (OR = 0.7; 95% CI: 0.4-1.8).
The study found that the positive rate of CT-PA for PE was lower among patients with PE indicating probable overutilization of investigation in these patients. Additionally, patients with COVID-19 had a higher proportion of elevated D-dimer levels that may be a contributor to the increased investigation for PE. Lastly, patients with COVID-19 who were current smokers had a higher tendency of having PE.
Background
Chylothorax following cardiothoracic surgery is a rare condition in pediatric patients with significant morbidity and mortality. Pharmacological management with octreotide suggests ...possible efficacy; however, current evidence is inadequate. The objective of this study was to assess the safety and efficacy of octreotide as a therapeutic option in this clinical setting.
Methods
This was a 12-year single-center retrospective cohort study of all patients (birth to 18-years old) who received octreotide for management of post-cardiac surgery chylothorax between January 2003 to August 2015. The primary efficacy endpoint was resolution of chylothorax, categorized as complete (<2 mL·kg−1·day−1), partial (based on physician’s judgement), or failed. The primary safety endpoint was any significant adverse drug reaction leading to discontinuation of octreotide therapy. Of the 46 patients identified as receiving octreotide for post-cardiac surgery chylothorax, 29 were included in efficacy and safety analyses.
Results
Resolution of chylothorax was achieved in 62% (complete in 28%, partial in 34%) of the total sample. The 38% who did not respond to octreotide therapy required thoracic duct ligation. The mean initial dose and duration of octreotide was 4 ± 3 µg·kg−1·h−1 and 10 ± 5 days, respectively. Besides minor side-effects including transient hyperglycemia (45%), abdominal distension (3%), and tachycardia (>150 beats·min−1; 10%), no patient developed a significant side-effect that required discontinuation of therapy.
Conclusions
Pharmacological management of post-cardiac surgery induced chylothorax with octreotide shows promising benefits with an acceptable safety profile.
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.
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.
Ninety-eight patients were included in our study. Fifty-two (53%) were females and 46 (47%) were males, with a mean age of 26 ± 8.4 years and a mean weight of 62 ± 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 ± 2 days, with a mean ICU and hospital stay of 3.7 ± 3 days, and 10 ± 7 days, respectively. The overall mortality rate in our series was 4% with a 1–5-year survival of 96%.
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.
Surgical pulmonary artery reconstruction in patients with arterial tortuosity syndrome has excellent outcomes. In this study, we report our late outcomes after more than a decade of experience with ...such complex interventions.
We conducted a retrospective review of 33 arterial tortuosity syndrome patients who underwent pulmonary artery reconstruction. The mean preoperative right ventricular to left ventricular pressure ratio was 1.19 ± 0.2. Our surgical approach included either a single-stage complete repair through a median sternotomy (17 patients) or a two-stage repair through sternotomy/left thoracotomy (16 patients), depending on the degree of distal involvement in the left pulmonary artery.
Median age was 36 months. All patients had distal segmental peripheral pulmonary artery stenosis. Thirty patients (90.1%) were symptomatic before surgery. There was one hospital death due to viral pneumonia 78 days after the surgery (in-hospital mortality 3%). The mean right ventricular to left ventricular pressure ratio decreased to 0.31 ± 0.07 early postoperatively (P < 0.001), representing a 74% reduction compared with preoperative values. Follow-up was 100% complete for all hospital survivors (32 of 33) with a mean follow-up of 70.42 ± 43.32 months (range, 2 to 143). There was no late mortality or need for reintervention (surgical or catheter based) after hospital discharge. In late postoperative catheterization, the mean right ventricular to left ventricular pressure ratio was 0.27 ± 0.05 (P = .003 compared with early postoperative value). All patients were asymptomatic on their most recent follow-up.
A strategy of complete surgical reconstruction of all stenotic pulmonary artery segments in patients with arterial tortuosity syndrome is recommended for sustainable successful outcomes more than a decade later.
Pulmonary emphysemas are mainly of two types, congenital lobar emphysema (CLE) and pulmonary interstitial emphysema (PIE), which have a different pathophysiology, but both cause severe respiratory ...distress that may require intensive medical and most of the time surgical therapy. This article reports a case of CLE managed not by treating the aected side but by opening the persistently collapsed lung.
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