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Journal of cardiothoracic and vascular anesthesia 07/2012; · 1.06 Impact Factor
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Journal of cardiothoracic and vascular anesthesia 03/2011; 26(2):286-90. · 1.06 Impact Factor
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ABSTRACT: Pompe disease (acid α-glucosidase deficiency) is one of several lysosomal storage diseases amenable to treatment with enzyme replacement therapy (ERT). While echocardiography (echo) has been the standard method to evaluate the cardiac response to ERT, cardiac magnetic resonance imaging (CMR) has the advantage of a better tissue definition and characterization of myocardial fibrosis. However, CMR for Pompe disease is not frequently performed due to a high risk of sedation. We report the first use of CMR in a feasible protocol to quantify left ventricular (LV) mass, function, and the presence of myocardial fibrosis in the Pompe population.
Children with Pompe disease on ERT were assessed with transthoracic echo and CMR over a 3 year period at a single institution. Echocardiography was performed using standard techniques without sedation. CMR was performed using retrospectively gated and real-time imaging, with and without sedation. LV mass indexed to body surface area (LVMI) and ejection fraction (EF) were measured by both echo and CMR, and evaluated for change over time. Myocardial fibrosis was assessed by CMR with delayed enhancement imaging 5-10 min after gadolinium contrast using single shot inversion recovery sequences with inversion time set to null the signal from normal myocardium.
Seventeen CMR scans were successfully performed in 10 subjects with Pompe disease (median age at first CMR is 9 months, range 1-38 months, 80% male), with sedation only performed in 4 studies. There was a median interval of 5 months (range 0-34 months) from the start of ERT to first CMR (baseline). At baseline, the median indexed LVMI by CMR (140.0 g/m(2), range 43.8-334.0) tended to be lower than that assessed by echo (median 204.0 g/m(2), range 52.0-385.0), but did not reach statistical significance. At baseline, CMR EF was similar to that assessed by echo (55% vs. 55%). Overall, there was no significant decrease in CMR measured LVMI over time (CMR median LVMI at baseline 94 g/m(2) (range 43.8-334) vs. CMR median at most recent study 44.5 g/m(2) (range 34-303), p=0.44). In 5 patients with serial CMR scans over time, LVMI decreased in 2, was similar in 2, and increased in 1 patient with high sustained antibodies to exogenous enzyme. Delayed enhancement was noted in only l separate patient who also had high sustained antibodies to exogenous enzyme.
CMR is an imaging tool that is feasible to use to serially follow LVMI and EF in children with Pompe disease on ERT. Real-time imaging is adequate for quantification purposes in these patients and minimizes the need for sedation. Quantitative CMR LVMI is generally lower than echo derived LVMI. Delayed enhancement appears to be a rare finding by CMR in Pompe disease. A further follow-up is necessary to better understand the long term effects of ERT in infantile Pompe survivors, especially those with high sustained antibody titers or advanced cardiac disease at treatment outset.
Molecular Genetics and Metabolism 07/2010; 101(4):332-7. · 3.19 Impact Factor
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ABSTRACT: Intraoperative near-infrared spectroscopy cerebral oxygenation monitoring assists intraoperative decision-making in environments without extracorporeal membrane oxygenation (ECMO), left ventricular assist device (LVAD) or access to cardiac transplantation. We report a case of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), undergoing cardiac surgery. A 4-month-old infant presented in extremis with cardiac failure. We discuss the pathophysiology and challenging intraoperative management of ALCAPA with extensive ischaemia and myocardial infarction.
South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 01/2007; 96(12):1266-8. · 2.04 Impact Factor
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ABSTRACT: Cytokine dysregulation contributes to the systemic inflammatory response after cardiopulmonary bypass. Clearance of cytokine binding proteins may be important in the resolution of inflammation. Our aim was to determine whether the cytokine binding protein alpha 2 -macroglobulin and its soluble receptor were upregulated in endotracheal aspirates from infants and children undergoing cardiopulmonary bypass.
Seventy tracheal aspirates were collected before and after cardiopulmonary bypass from 35 infants and children undergoing surgical correction of congenital heart defects. alpha 2 -Macroglobulin and the soluble alpha 2 -macroglobulin receptor were identified by Western blot. With the use of multi-analyte cytokine profiling, pro-inflammatory and anti-inflammatory cytokines were quantified, normalized to total protein, and expressed as ratios. Paired t tests and Wilcoxon signed-rank tests were performed between prebypass and postbypass samples. Correlations were examined among alpha 2 -macroglobulin, soluble alpha 2 -macroglobulin receptor, cytokine ratios, and the clinical variables of cardiopulmonary bypass, aortic crossclamp, and circulatory arrest times.
alpha 2 -Macroglobulin increased by 50% (mean densitometry increase 82,683 +/- 184,594, P = .012), and soluble alpha 2 -macroglobulin receptor increased by 17% (mean densitometry increase 506,148 +/- 687,037, P = .0001) after cardiopulmonary bypass. The ratio of interleukin-8/interleukin-4 increased by 136% ( P = .0001), and interleukin-8/interleukin-10 increased by 102% ( P = .001). The increase in soluble alpha 2 -macroglobulin receptor was positively correlated with the ratios of interleukin-8/interleukin-4 and interleukin-8/interleukin-10. There were no statistically significant positive correlations between the increase in alpha 2 -macroglobulin or soluble alpha 2 -macroglobulin receptor and measured clinical variables.
We report for the first time the upregulation of alpha 2 -macroglobulin and soluble alpha 2 -macroglobulin receptor in tracheal aspirates after cardiopulmonary bypass in infants and children. Soluble alpha 2 -macroglobulin receptor correlates with increased alpha 2 -macroglobulin and a disproportionate increase in pro-inflammatory to anti-inflammatory cytokine ratios.
Journal of Thoracic and Cardiovascular Surgery 06/2005; 129(5):1098-103. · 3.41 Impact Factor
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ABSTRACT: Complete DiGeorge syndrome results in the absence of functional T cells. Our program supports the transplantation of allogeneic thymic tissue in infants with DiGeorge syndrome to reconstitute immune function. This study reviews the multidisciplinary care of these complex infants.
From 1991 to 2001, the authors evaluated 16 infants with complete DiGeorge syndrome. All infants received multidisciplinary medical and surgical support. Clinical records for the group were reviewed.
Four infants died without receiving a thymic transplantation, and 12 children survived to transplantation. The mean age at time of transplantation was 2.7 months (range, 1.1 to 4.4 months). All 16 infants had significant comorbidity including congenital heart disease (16 of 16), hypocalcemia (14 of 16), gastroesophageal reflux disease or aspiration (13 of 16), CHARGE complex (4 of 16), and other organ involvement (14 of 16). Nontransplant surgical procedures included central line placement (15 of 16), fundoplication or gastrostomy (10 of 16), cardiac repair (10 of 16), bronchoscopy or tracheostomy (6 of 16), and other procedures (12 of 16). Complications were substantial, and 5 of the 12 transplanted infants died of nontransplant-related conditions. All surviving infants have immune reconstitution, with follow-up from 2 to 10 years.
Although the transplantation of thymic tissue can restore immune function in infants with complete DiGeorge syndrome, these children have substantial comorbidity. Care of these children requires coordinated multidisciplinary support.
Journal of Pediatric Surgery 12/2004; 39(11):1607-15. · 1.45 Impact Factor
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Journal of Cardiothoracic and Vascular Anesthesia 09/2004; 18(4):472-4. · 1.64 Impact Factor
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ABSTRACT: Pompe or Glycogen Storage Disease type II (GSD-II) is a genetic disorder affecting both cardiac and skeletal muscle. Historically, patients with the infantile form usually die within the first year of life due to cardiac and respiratory failure. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anaesthesia for these patients. Understanding the unique cardiac physiology in patients with GSD-II is essential to providing safe general anaesthesia.
Pediatric Anesthesia 07/2004; 14(6):514-9. · 2.10 Impact Factor
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ABSTRACT: To determine how much patients are willing to pay to avoid intraoperative awareness?
Observational study
University-affiliated metropolitan hospital.
60 patients who completed a questionnaire (39 F, 21 M). The mean age was 43 years and the median household income range of 45,000-60,000 US dollars.
Patients completed an interactive computer-generated questionnaire on the value of preventing intraoperative awareness and their willingness to pay for a "depth of anesthesia" monitor. Their willingness to pay for the prevention of postoperative pain, nausea and vomiting, postoperative grogginess, and sleepiness was also determined as a means of comparison.
Patients were willing to pay (WTP) 34 US dollars, 10 US dollars to 42 US dollars (median, interquartile range) for a monitor that would assist an anesthesia care provider assess the depth of anesthesia in an effort to avoid awareness. This increased to 43 US dollars, 20 US dollars to 77 US dollars (p < 0.0,001) (median, interquartile range), if the insurance company was making the payment and the WTP value only decreased minimally to 33 US dollars if the incidence of awareness was reduced 10-fold.
The incidence of intraoperative awareness and WTP value for monitoring awareness have a nonlinear relationship (a risk averse utility function), which suggests that patients assign an intrinsic base value for a rare or very rare possibility of an event. Other healthcare economic analyses (such as cost effectiveness) do not take this factor into account and assume a linear value relationship (i.e., if something occurs ten times less frequently, it has ten times less value).
The median value for patients' WTP for a monitor that might prevent awareness under anesthesia was 34 US dollars given an incidence of 5/1,000 cases. The incidence of awareness and WTP value have a nonlinear relationship suggesting that patients assign an intrinsic base value for the possibility of awareness.
Journal of Clinical Anesthesia 03/2003; 15(2):108-12. · 1.21 Impact Factor
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ABSTRACT: In children, radiofrequency catheter ablation (RFCA) is typically performed under general anesthesia. With the use of volatile anesthetics, postoperative nausea and vomiting (PONV) are common, with an incidence of emesis as frequent as 60%. We tested the hypothesis that a propofol (PRO)-based anesthetic would have a less frequent incidence of PONV than an isoflurane (ISO)-based anesthetic. Patients were randomly assigned to receive either an ISO- or PRO-based anesthetic. Prophylactic ondansetron was given to all patients and droperidol was used as a rescue antiemetic postoperatively while PONV was monitored postoperatively for 18 h. The incidence of nausea, vomiting, use of rescue antiemetic drugs, and sedation scores were recorded. The cost for the anesthetic was also calculated. Fifty-six subjects were included in this study. The cumulative incidence of PONV was significantly more frequent in group ISO (63% nausea/55% emesis) compared with group PRO (21% nausea/6% emesis). After the administration of droperidol, further vomiting occurred in 70% of the patients in group ISO versus 0% of the patients in group PRO. We conclude that RFCA using ISO has a high PONV risk and the prophylactic use of ondansetron as well as antiemetic therapy with droperidol are ineffective. In contrast, a PRO-based anesthetic is highly effective in preventing PONV in children undergoing RFCA. IMPLICATIONS: In children undergoing radiofrequency catheter ablation and receiving prophylactic ondansetron, a frequent incidence (60%) of postoperative vomiting was observed under an isoflurane-based anesthetic, whereas the incidence was significantly reduced to a very low level (5%) under a propofol-based anesthetic.
Anesthesia & Analgesia 01/2003; 95(6):1577-81, table of contents. · 3.29 Impact Factor
