The best thing for children with congenital heart disease would be to be brought to the care of a specialized team of medical professionals who can provide accurate diagnosis and timely corrective ...intervention and surgery as soon as possible after birth. The focus on body weight, nutritional status, optimal weight for surgery and the like should disappear from the language of doctors and other health care providers who are contributing to the delay in corrective interventions and surgeries that are indicated for these patients. The burden on such specialized teams then becomes to improve access of potential patients to the expert advise, timely diagnosis and optimal corrective intervention and surgery.
Laterality defects (LDs) or asymmetrically positioned organs are a group of rare developmental disorders caused by environmental and/or genetic factors. However, the exact molecular pathophysiology ...of LD is not yet fully characterised. In this context, studying Arab population presents an ideal opportunity to discover the novel molecular basis of diseases owing to the high rate of consanguinity and genetic disorders. Therefore, in the present study, we studied the molecular basis of LD in Arab patients, using next-generation sequencing method. We discovered an extremely rare novel missense variant in
MYO1D
gene (Pro765Ser) presenting with visceral heterotaxy and left isomerism with polysplenia syndrome. The proband in this index family has inherited this homozygous variant from her heterozygous parents following the autosomal recessive pattern. This is the first report to show
MYO1D
genetic variant causing left–right axis defects in humans, besides previous known evidence from zebrafish, frog and
Drosophila
models. Moreover, our multilevel bioinformatics-based structural (protein variant structural modelling, divergence, and stability) analysis has suggested that Ser765 causes minor structural drifts and stability changes, potentially affecting the biophysical and functional properties of
MYO1D
protein like calmodulin binding and microfilament motor activities. Functional bioinformatics analysis has shown that
MYO1D
is ubiquitously expressed across several human tissues and is reported to induce severe phenotypes in knockout mouse models. In conclusion, our findings show the expanded genetic spectrum of LD, which could potentially pave way for the novel drug target identification and development of personalised medicine for high-risk families.
Extracorporeal membrane oxygenation has been widely used after paediatric cardiac surgery due to increasing complex surgical repairs in neonates and infants having complex CHDs.
We reviewed ...retrospectively the medical records of all patients with CHD requiring corrective or palliative cardiac surgery at King Abdulaziz University Hospital that needed extracorporeal membrane oxygenation support between November 2015 and November 2018.
The extracorporeal membrane oxygenation population was 30 patients, which represented 4% of 746 children who had cardiac surgery during this period. The patients' age range was from 1 day to 20.33 years, with a median age of 6.5 months. Median weight was 5 kg (range from 2 to 53 kg). Twenty patients were successfully decannulated (66.67%), and 12 patients (40%) were survived to hospital discharge. Patients with biventricular repair tended to have better survival rate compared with those with single ventricle palliation (55.55 versus 16.66%, p-value 0.058). During the first 24 hours of extracorporeal membrane oxygenation support, the flow rate was significantly reduced after 4 hours of extracorporeal membrane oxygenation connection in successfully decannulated patients.
Survival to hospital discharge in patients requiring extracorporeal membrane oxygenation support after paediatric cardiac surgery was better in those who underwent biventricular repair than in those who had univentricular palliation. Capillary leak on extracorporeal membrane oxygenation could be a risk of mortality in patients after paediatric cardiac surgery.
Background
Surgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ...ventricle, and heterotaxy syndromes are thought to influence short- and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting.
Results
Between 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson’s test, Wilcoxson’s test, and generalized linear regression with Poisson distribution were used.
Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein stenosis occurred in three patients, and reoperation was done in one patient. Survival free from reoperation was 98%, 98%, and 90% at 1, 24, and 60 months in the absence of pulmonary vein stenosis. However, all three patients who developed pulmonary vein stenosis died, at 0.5, 2.7, and 6.3 months of follow-up, respectively. We were unable to detect a significant effect of anatomical type, preoperative obstruction, associated single ventricle, or heterodoxy syndrome on operative mortality or long-term freedom from death or reoperation. Patients who presented with obstruction and infracardiac or supracardiac TAPVD had longer hospital stay.
Conclusion
TAPVD repair outcomes are excellent except for patients who develop postoperative pulmonary venous stenosis. Anatomical type, obstructed presentation, associated single ventricle, or heterotaxy are not significant predictors of survival.
Persistent truncus arteriosus represents less than 3% of all congenital heart defects. We aim to analyze mid-term outcomes after primary Truncus arteriosus repair at different ages and to identify ...the risk factors contributing to mortality and the need for intervention after surgical repair.
This retrospective cohort study included 36 children, underwent repair of Truncus arteriosus in the period from January 2011 to December 2018 in two institutions. We recorded the clinical and echocardiographic data for the patients preoperatively, early postoperative, 6 months postoperative, then every year until their last documented follow-up appointment.
Thirty-six patients had truncus arteriosus repair during the study period. Thirty-one patients had open sternum post-repair, and two patients required extracorporeal membrane oxygenation. Bleeding occurred in 15 patients (41.67%), and operative mortality occurred in 5 patients (14.7%). Patients with truncus arteriosus type 2 (p = 0.008) and 3 (p = 0.001) and who were ventilated preoperatively (p < 0.001) had a longer hospital stay. Surgical re-intervention was required in 8 patients (22.86%), and 11 patients (30.56%) had catheter-based reintervention. Freedom from reintervention was 86% at 1 year, 75% at 2 years and 65% at 3 years. Survival at 1 year was 81% and at 3 years was 76%. High postoperative inotropic score predicted mortality (p = 0.013).
Repair of the truncus arteriosus can be performed safely with low morbidity and mortality, both in neonates, infants, and older children. Re-intervention is common, preferably through a transcatheter approach.
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 (β: 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) mm
2
/m
2
. Norwood palliation (β: − 0.61 (95% CI 62.63–20.18),
P
= 0.003) and preoperative saturation (β: − 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.
Our aim is to determine the prevalence of vitamin D deficiency in children with congenital heart disease (CHD). In addition, we demonstrated the effect of cardiopulmonary bypass (CPB) on vitamin D, ...parathyroid hormone (PTH), and calcium levels. The association between perioperative vitamin D levels and postoperative clinical outcomes has been explored. A prospective observational study was conducted from February 2018 to June 2019 on 69 children undergoing elective surgery for CHD under CPB. Blood samples were collected preoperatively, immediate postoperatively, and 24 h postoperatively. Vitamin D deficiency was present in 34 (49.3%) patients preoperatively and 63 (91.3%) patients immediately postoperative. We identified 42.03% decline of 25(OH)D immediately postoperative. Changes in ionized calcium (iCa) concentrations were accompanied by reciprocal alterations in PTH concentrations. Lower postoperative 25(OH)D was associated with higher maximum vasoactive inotropic score (VIS) in the first 24 h postoperative (
r
= − 0.259,
p
= 0.03).
Conclusion
: Vitamin D deficiency is common in children with CHD and the majority are vitamin D deficient following cardiac surgery with acute decline of serum 25(OH)D after CPB. Lower postoperative vitamin D levels in children undergoing cardiac surgery are associated with the need for increasing the inotropic support.
What is Known:
• Vitamin D is a pleiotropic hormone, important for calcium homeostasis.
• Vitamin D deficiency might affect the outcome in critically ill patients.
What is New:
• Cardiopulmonary bypass causes acute decline of vitamin D in children.
• Lower postoperative vitamin D is associated with higher inotropic support.
Abstract Morgagni hernia (MH) is a rare form of congenital diaphragmatic hernia, typically occurring predominantly on the right side and exhibiting a higher prevalence in females. Usually diagnosed ...incidentally, MH may coexist with congenital heart defects, chest wall abnormalities and certain genetic syndromes such as Down syndrome. A 4-year-old boy with Down syndrome underwent simultaneous repair of MH and closure of a ventricular septal defect (VSD). A vertical midline sternotomy was performed, and the VSD was repaired using the right atrium approach. Subsequently, MH repair was conducted. Three weeks after the surgery, this patient developed a complete heart block, which lead to the implantation of a VVI pacemaker.
Background
The appropriate age and weight for surgical repair of atrioventricular septal defect (AVSD) is an area of controversy. We aimed to study the effect of weight and age at the time of ...surgical repair for complete AVSD in children less than 2 years of age on postoperative outcomes. A retrospective data review was performed for patients who underwent the AVSD repair from 2012 to 2019 at our institutions. Our primary outcome was the postoperative in-hospital length of stay (LOS). Secondary outcomes included total positive pressure ventilation (PPV), ventilation time, maximum vasoactive–inotropic score (max VIS), and other postoperative complications.
Results
The study included fifty patients. The median age was 191 days, and the median weight was 4.38 kg at the time of surgery. Weight < 4 kg was associated with longer PPV time and postoperative in-hospital LOS (
p
value of 0.033 and 0.015, respectively). Additionally, they had higher max VIS at 24 h and 48 h than the other groups with bodyweight 4–5.9 kg or ≥ 6 kg (
p
value of 0.05 and 0.027, respectively). Patients with older age or lower weight at operation had a longer in-hospital LOS and total length of PPV after surgery. There were no postoperative in-hospital deaths.
Conclusions
Older age and lower weight at the time of surgical repair of atrioventricular septal defect could be independent predictors of prolonged postoperative in-hospital length of stay and total length of positive pressure ventilation.
Disorders of the inner parts of blood vessels have been significant triggers of cardiovascular diseases (CVDs). Different interventional methods have been employed, from complex surgeries to balloon ...angioplasty techniques to open the narrowed blood vessels. However, CVDs continue to be the lead cause of death in the world. Delivering a therapeutic agent directly to the inner wall of affected blood vessels can be a transformative step toward a better treatment option. To open the door for such an approach, a catheter delivery system is developed based on a conventional balloon catheter where a fluidic channel and microneedles (MNs) are integrated on top of it. This enables precise and localized delivery of therapeutics directly into vessel walls. Customizable MNs are fabricated using a high‐resolution 3D printing technique where MN's height ranges from 100 to 350 µm. The MNs penetration into a synthetic vascular model is investigated with a computerized tomography scan. Ex vivo tests on rabbit aorta confirm the MN‐upgraded balloon catheter's performance on real tissue. Delivery of fluorescent dye is accomplished by injecting it through the fluidic channel and MNs into the aortic tissue. The dye is observed at up to 180 µm of depth, confirming site‐specific endovascular delivery.
3D printed microneedles on a balloon catheter are developed to provide site‐specific delivery of therapeutics into the blood vessels. Successful penetration into phantom vascular tissue confirms sufficient microneedles stability. A fluorescent dye injection into a rabbit aorta at different depth levels of the tissue demonstrates the precise and targeted delivery.
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