Objectives : The main objective of the study is to characterize the effects of genetic abnormalities/syndromes (GA/S) on perioperative outcomes of cardiac surgeries involving repair of conotruncal ...heart defects (CTHD).
Design : The study involves a single-center retrospective analysis of patients who underwent complete repair of CTHDs (tetralogy of Fallot TOF, truncus arteriosus, interrupted aortic arch, and ventricular septal defect with coarctation) between January 2000 and December 2015. The primary outcome was the post operative length of stay (PLOS). The secondary outcomes were mortality, cardiac complications, hematologic complications, infections, and number of medications-at-discharge.
Setting : Cardiac intensive care unit in a tertiary pediatric hospital in South Florida that performs around 300 open-heart surgeries a year.
Subjects : A total of 177 patients with CTHDs who underwent cardiac surgeries in the stated time period were included in the final study cohort.
Measurements and Main Results :Majority of patients had TOF (72.5%) and 46 (26%) had GA/S. The most common GA/S was 22q11 deletion (37%). PLOS was significantly increased in patients with GA/S (P < 0.05). Patients with GA/S were 4.5 times more likely to have a postoperative cardiac complication, 4.2 times more likely to have a postoperative infection, and received 1.6 times more medications at discharge than those without GA/S. However, GA/S was not associated with increased perioperative mortality. Black patients were three times more likely to have a longer PLOS than White patients.
Conclusions : Perioperative outcomes in patients with GA/S suggested an increased residual cardiovascular disease and increased resource usage. Notably, this is the first study demonstrating the effect of race and ethnicity on PLOS in CTHD patients.
We describe the use of a novel interventional approach to the histopathologic diagnosis of a ventricular septal tumor using intraoperative ultrasound-guided trans-epicardial biopsy without the need ...for cardiopulmonary bypass in a 2-year-old child. This novel approach has not been previously reported. Multidisciplinary collaboration between cardiothoracic surgery, cardiology, cardiac imaging, and interventional radiology provided the ability to perform cardiac biopsy. This technique may be used in specific cases of cardiac tumors where tissue diagnosis is important, but surgical resection is deemed excessively risky or impossible.
Salbutamol and levosalbutamol are the two most frequently used medications for asthma in pediatrics. Levosalbutamol is more expensive than salbutamol and is usually considered safer option for ...patients with existing cardiac condition and, to reduce tachycardia in otherwise healthy patients. In this article, we reviewed the literature which included randomized controlled trials, retrospective studies and case reports and compared the cardiac side effects of these two medications with primary emphasis on tachycardia
Pediatric cardiac interventions via percutaneous common carotid artery (CCA) access have been shown to be safe and effective. However, the impact of placement of a sheath in the carotid artery for ...interventions on cerebral perfusion is unknown. In this study we used cerebral near-infrared spectroscopy (NIRS) to analyze the effects of percutaneous CCA access for cardiac interventions on cerebral perfusion. This study is a retrospective chart review carried out at a tertiary care center on all pediatric patients who underwent percutaneous cardiac catheterization via carotid artery access from January 2010 to January 2020. All patients who had ipsilateral NIRS recorded on the side of carotid artery access were included. Patients with only partial or no ipsilateral NIRS data were excluded. The primary outcome measure was the change in NIRS upon CCA access; the mean NIRS for 15 min before obtaining access was compared to the mean NIRS during the procedure and to the mean NIRS 15 min after removal of the CCA sheath. We hypothesized that there would be a significant drop in NIRS values on the side of CCA access. There were 48 catheterizations in the study period where percutaneous CCA was accessed. Of those, 21 catheterizations had complete data and were included in the study. 13 (62%) were of males. The median age was 23 days (IQR 7–79). The indications for CCA access were patent ductus arteriosus stent implantation (
n
= 13; 62%), aortic valvuloplasty (
n
= 5; 24%), balloon angioplasty of coarctation of aorta (
n
= 2; 10%), and renal artery angioplasty (
n
= 1; 4%). In 16 patients (72%), the left common carotid artery was accessed. The median weight of the patients was 3.3 kg (IQR 2.8–2.9). The most common sheath size used was 4F, in 16 patients (72%). The mean NIRS prior to the procedure was 67 ± 15%, during the procedure was 68 ± 20%, and after removal of sheath was 68 ± 21%. Paired t test of cerebral NIRS before, during, and after the procedure showed no significant change with CCA access (
P
= 0.08). No patient in the series had a documented neurologic deficit following the procedure. Percutaneous CCA access was not associated with a decrease in NIRS on the side of the access during the procedure, suggesting there was no significant acute change in cerebral perfusion with CCA access.
Aortic aneurysm in children is rare, but has been described in the tuberous sclerosis complex (TSC) population. While surgical repair has been utilized as the primary means of intervention, we ...present the first known case reporting exclusion of a descending thoracic aortic aneurysm with percutaneous covered stent implantation in a pediatric patient with TSC. A review of the literature is also included herein.
A Case of Male Goltz Syndrome Ghoshal, Bhaswati; Lahiri, Subhrajit; Nandi, Debabrata
Case reports in pediatrics,
01/2012, Letnik:
2012
Journal Article
Recenzirano
Odprti dostop
We present the case of a boy with a clinical diagnosis of Goltz syndrome (focal dermal hypoplasia), a rare genodermatosis characterized by widespread dysplasia of mesodermal and ectodermal tissues. A ...9-year-old male patient with Goltz syndrome presented with typical skin lesions along with progressive dimness of vision and mental retardation since birth. It is inherited in an X-linked dominant fashion and is normally lethal in male patients, and so very few male patients, like the index case, have been reported.
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