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1.
Cleft Palate Craniofac J ; : 10556656231154814, 2023 Mar 27.
Article in English | MEDLINE | ID: mdl-36972482

ABSTRACT

Osteosarcomas arising within the pterygomaxillary/infratemporal fossa region are rare among the pediatric population. Survival rates are most influenced by tumor resection with negative margins, which can be dependent on surgical accessibility of the tumor site. The pterygomaxillary/infratemporal fossa location poses several challenges to safe and adequate tumor resection, including proximity of the facial nerve and great vessels and scarring associated with traditional transfacial approaches. In this article, we present the case of a 6-year-old boy with an osteosarcoma of the left pterygomaxillary/infratemporal fossa region successfully managed with an "oncoplastic" approach, incorporating the use of CAD/CAM and mixed reality technologies.

2.
Pediatr Cardiol ; 43(7): 1444-1454, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35305111

ABSTRACT

The objective of this study was to determine whether treatment with human fibrinogen concentrate decreases the need for component blood therapy and blood loss in neonate and infant patients undergoing cardiopulmonary bypass. Pediatric patients (N = 30) undergoing elective cardiac surgery were randomized to receive human fibrinogen concentrate or placebo following cardiopulmonary bypass termination. The primary endpoint was the amount of cryoprecipitate administered. Secondary endpoints included estimated blood loss during the 24 h post-surgery; perioperative blood product transfusion; effects of fibrinogen infusion on global hemostasis, measured by laboratory testing and rotational thromboelastometry; and adverse events. No clinically significant differences were identified in baseline characteristics between groups. A significantly lower volume of cryoprecipitate was administered to the treatment group during the perioperative period [median (interquartile range) 0.0 (0.0-0.0) cc/kg vs 12.0 (8.2-14.3) cc/kg; P < 0.0001] versus placebo. No difference was observed between treatment groups in blood loss, laboratory coagulation tests, use of other blood components, or incidence of adverse events. FIBTEM amplitude of maximum clot firmness values was significantly higher among patients treated with human fibrinogen concentrate versus placebo (P ≤ 0.0001). No significant differences were observed in post-drug HEPTEM, INTEM, and EXTEM results. Human fibrinogen concentrate (70 mg/kg) administered after the termination of cardiopulmonary bypass reduced the need for transfusion with cryoprecipitate in a neonate and infant patient population.ClinicalTrials.gov identifier: NCT02822599.


Subject(s)
Cardiopulmonary Bypass , Fibrinogen , Blood Loss, Surgical/prevention & control , Cardiopulmonary Bypass/adverse effects , Cardiopulmonary Bypass/methods , Child , Fibrinogen/therapeutic use , Humans , Infant , Infant, Newborn , Pilot Projects , Prospective Studies , Thrombelastography/methods
3.
J Cardiothorac Surg ; 14(1): 139, 2019 Jul 22.
Article in English | MEDLINE | ID: mdl-31331371

ABSTRACT

INTRODUCTION: Rotational thromboelastometry (ROTEM) has been shown to reduce the need for transfused blood products in adult and pediatric cardiac surgery patients. However, similar evidence in newborns, neonates, and young infants is lacking. We quantified ROTEM value changes in pediatric patients on cardiopulmonary bypass (CPB) before, during and after blood product transfusion. METHODS: Each surgery had at least four interventions: initiating CPB; platelet administration during rewarming phase; post-CPB and following protamine and human fibrinogen concentrate (HFC) administration; and further component therapy if bleeding persisted and ROTEM indicated a deficiency. ROTEM assays were performed prior to surgery commencement, on CPB prior to platelet administration and following 38 mL/kg platelets, and post-CPB after protamine and HFC administration. ROTEM assays were also performed in the post-CPB period after further blood component therapy administration. RESULTS: Data from 161 patients were analyzed. Regression models suggested significant changes in HEPTEM clotting time after all interventions. PLT administration during CPB improved HEPTEM α by 22.1° (p < 0.001) and FIBTEM maximum clot firmness (MCF) by 2.9 mm (p < 0.001). HFC administration after CPB termination significantly improved FIBTEM MCF by 2.6 mm (p < 0.001). HEPTEM MCF significantly increased after 3/4 interventions. HEPTEM α significantly decreased after two interventions and significantly increased after two interventions. Greatest perturbances in coagulation parameters occurred in patients ≤90 days of age. CONCLUSION: CPB induced profound perturbations in ROTEM values in pediatric cardiac surgery patients. ROTEM values improved following PLT and HFC administration. This study provides important clinical insights into ROTEM changes in pediatric patients after distinct interventions.


Subject(s)
Blood Coagulation Disorders/diagnosis , Blood Component Transfusion , Cardiopulmonary Bypass/adverse effects , Thrombelastography , Blood Coagulation Disorders/etiology , Blood Coagulation Disorders/therapy , Blood Component Transfusion/methods , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , Thrombelastography/methods , Treatment Outcome
4.
Clin Appl Thromb Hemost ; 25: 1076029618816382, 2019.
Article in English | MEDLINE | ID: mdl-30518238

ABSTRACT

This study evaluated whether rotational thromboelastometry (ROTEM; Tem International GmbH, Munich, Germany) FIBTEM maximum clot firmness (MCF) can be used to predict plasma fibrinogen level in pediatric patients undergoing cardiac surgery. Linear regression was conducted to predict plasma fibrinogen level using FIBTEM MCF (0.05 level of significance). Scatter plot with the regression line for the model fit was created. Fifty charts were retrospectively reviewed, and 87 independent measurements of FIBTEM MCF paired with plasma fibrinogen levels were identified for analysis. Linear regression analysis suggested a significant positive linear relationship ( P < .0001) between plasma fibrinogen levels and MCF. Both MCF intercept and slope were significantly correlated with fibrinogen level ( P < .0001). The estimated regression equation (predicted fibrinogen = 78.6 + 12.4 × MCF) indicates that a 1-mm increase in MCF raises plasma fibrinogen level by an average of 12.4 mg/dL. The statistically significant positive linear relationship observed between MCF and fibrinogen levels ( P < .001) suggests that MCF can be used as a surrogate for fibrinogen level. This relationship is of clinical relevance in the calculation of patient-specific dosing of fibrinogen supplementation in this setting.


Subject(s)
Blood Coagulation Tests/methods , Cardiac Surgical Procedures/methods , Fibrin/metabolism , Fibrinogen/metabolism , Thrombelastography/methods , Thrombosis/metabolism , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Retrospective Studies
5.
Perfusion ; 33(2): 130-135, 2018 03.
Article in English | MEDLINE | ID: mdl-28925857

ABSTRACT

BACKGROUND: The aim of this study was to compare three different blood washing techniques and describe the differences for the composition of the washed red blood cells (RBC). METHODS: Stored RBCs less than 5 days old were washed using three different techniques. 1) Washing with normal saline with the COBE Model 2991 blood processor in the blood bank (BB-S). 2) Washing with normal saline with the Continuous AutoTransfusion System (C.A.T.S) in the operating room (OR-S). 3) Washing with Plasma-Lyte with the C.A.T.S in the operating room (OR-PL). Then, we compared the values for hemoglobin (Hb), hematocrit (Hct), blood volume, RBC volume, lactate, glucose, sodium and potassium of the three different groups. RESULTS: Forty-five units of RBCs were washed and analyzed (15 for each technique). The OR-S RBCs, when compared to the BB-S RBCs, had lower hemoglobin (g/dL) (22.8 vs 24.1, p=0.006), lower hematocrit (%) (67 vs 71, p=0.006), higher RBC volume (ml) (161 vs 130, p<0.001), higher glucose (mg/dL) (185 vs 46, p<0.001) and lower sodium (mmol/L) (153 vs 158, p<0.001). When compared to the OR-S RBCs, the OR-PL RBCs showed higher potassium (mmol/L) (5.3 vs 2, p<0.001) and lower sodium (mmol/L) (129 vs 153, p<0.001). CONCLUSION: RBCs washed with an autotransfusion device had a higher RBC volume and more physiological levels of glucose and sodium when compared with the blood processor in the blood bank. It can be an alternative option to use RBCs washed with an autotransfusion device for priming the extracorporeal circuits utilized in patients undergoing cardiac surgery.


Subject(s)
Blood Preservation/methods , Erythrocyte Transfusion/methods , Erythrocytes/metabolism , Extracorporeal Circulation/methods , Humans
6.
J Clin Anesth ; 38: 129-132, 2017 May.
Article in English | MEDLINE | ID: mdl-28372651

ABSTRACT

STUDY OBJECTIVE: To validate a non-invasive cardiac output monitor in pediatric cardiac surgery patients. DESIGN: Prospective trial. SETTING: Operating room. PATIENTS: 20 pediatric cardiac surgery patients ASA physical status 3 and 4. INTERVENTIONS: Aesculon noninvasive cardiac output monitor was used and compared to the cardiac output derived from the Fick equation. Oxygen consumption was measured and blood samples were taken from the arterial line and from the superior and inferior vena cava. MEASUREMENTS: Noninvasive cardiac output and cardiac index and Fick cardiac output and cardiac index. Oxygen consumption was measured by the TreyMed metabolic monitor. Blood samples were simultaneously drawn from the arterial line and from the superior and inferior vena cava purse string sites by the surgeon, prior to commencing cardiopulmonary bypass. Another data set was obtained right after termination of cardiopulmonary bypass. RESULTS: There was a direct, significant relationship between Fick CO/CI and NICOM CO/CI measurements. More dispersion is detected when the magnitude of the measure increases, i.e., for older and larger patients. CONCLUSIONS: There is a strong correlation between the cardiac output values derived from the Fick equation and the Aesculon non-invasive cardiac output monitor.


Subject(s)
Cardiac Output , Cardiac Surgical Procedures , Monitoring, Intraoperative/instrumentation , Oxygen Consumption , Adolescent , Child , Child, Preschool , Electric Impedance , Humans , Infant , Infant, Newborn , Monitoring, Intraoperative/methods , Prospective Studies
7.
Pediatr Cardiol ; 38(1): 115-122, 2017 Jan.
Article in English | MEDLINE | ID: mdl-27833994

ABSTRACT

In infants with a single right ventricle (RV), stage I palliation involves aortic reconstruction, systemic-to-pulmonary shunt placement, and atrial septectomy. Many require re-intervention for residual coarctation of the aorta (CoA). Doppler echocardiography can detect residual CoA in most infants, but its ability to predict severity has not been studied. This study compares gradients from Doppler interrogation to those from cardiac catheterization in infants with residual CoA. We performed a retrospective study of infants after stage I palliation from 2000 to 2014. Infants with an echocardiogram and catheterization before the second-stage palliative surgery were included. Infants with an echocardiogram >30 days before catheterization were excluded. Doppler-derived gradients were compared to catheterization-derived gradients. Echocardiographic assessment of tricuspid valve (TV) and RV function were recorded. The cohort included 95 infants, and thirty-three (35%) had CoA. Doppler-derived and catheterization-derived gradients correlated weakly in infants with CoA (r = 0.37, p = 0.036) and without CoA (r = 0.35, p = 0.005). Among infants with CoA, 17/33 had none or trivial tricuspid regurgitation (TR) and normal RV function, and Doppler-derived gradients correlated with catheterization gradients in this group (r = 0.71, p = 0.001). In 16/33 infants with ≥moderate TR or RV dysfunction, gradients did not correlate (r = -0.003, p = 0.992). After a stage I palliation in infants with single RV and CoA, Doppler-derived gradients poorly predicted the severity of CoA. Infants with normal TV or RV function had Doppler-derived gradients more predictive of catheterization-derived gradients. Doppler-derived gradients have limited utility in determining the severity of CoA after a stage I palliation.


Subject(s)
Aortic Coarctation/diagnostic imaging , Cardiac Catheterization/methods , Echocardiography, Doppler/methods , Hypoplastic Left Heart Syndrome/surgery , Palliative Care/methods , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Cardiac Surgical Procedures/adverse effects , Cardiac Surgical Procedures/methods , Female , Heart Ventricles/physiopathology , Humans , Infant , Infant, Newborn , Male , ROC Curve , Retrospective Studies , Ventricular Dysfunction, Right/physiopathology
8.
Pediatr Cardiol ; 37(6): 1078-84, 2016 Aug.
Article in English | MEDLINE | ID: mdl-27084382

ABSTRACT

Mortality for the arterial switch operation (ASO) has diminished significantly over the past few decades. Some patients do, however, continue to have protracted and complicated courses after surgery. We attempted to determine which preoperative factors were best associated with prolonged hospital stay after ASO. We retrospectively reviewed all patients that underwent an ASO over a 10-year period. Outcomes of patients with postoperative stays (POS) >14 days (long stay group-LS) were compared with those patients with POS < 7 days (short stay group-SS). The following variables were evaluated: age at surgery, weight, septostomy performed (BAS) and management the day prior to surgery including use of prostaglandin E1 (PGE1), inotropes, intubation status and the establishment of enteral feeds. The SS group had 25 patients and the LS group had 32 patients. Both groups (SS vs. LS) were similar in PGE1 use (48 vs. 69 %), BAS (76 vs. 59 %), age at surgery (6 vs. 7 days) and preoperative inotropes (12 vs. 38 %). The SS group had significantly higher incidence of preoperative feeding (80 vs. 31 %, p < 0.001) and less frequent intubation (12 vs. 47 %, p < 0.001). Patients who are intubated and have not yet begun to receive enteral feeds at the time of their ASO are more likely to have prolonged POS. It is unclear if prolonged stays were a result of operating on patients with worse preoperative hemodynamics or a consequence of a preoperative management strategy that did not allow for extubation and establishment of feeds prior to surgery.


Subject(s)
Enteral Nutrition , Arterial Switch Operation , Humans , Intubation , Length of Stay , Postoperative Period
9.
Pediatr Crit Care Med ; 17(5): 411-9, 2016 05.
Article in English | MEDLINE | ID: mdl-26927939

ABSTRACT

OBJECTIVES: Children with functional single ventricle undergoing the Fontan operation consume considerable resources. The purpose of this study is to evaluate pre- and intraoperative risk factors for longer hospital stay and to describe the perioperative course at a single institution over a 15-year period. DESIGN: Retrospective cohort study. SETTING: A single pediatric cardiac ICU. PATIENTS: All consecutive patients undergoing a first-time Fontan operation from 2000 to 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Prolonged length of stay was defined as hospital stay greater than 75 percentile at our institution after surgery. Of 218 patients who met inclusion criteria, median length of stay was 10 days (interquartile range, 8-14 d); prolonged length of stay was defined greater than or equal to 15 days. Independent pre- and intraoperative risk factors for prolonged length of stay included higher hemoglobin (odds ratio, 1.29; p = 0.003), higher mean pulmonary artery pressure (odds ratio, 1.14; p = 0.037), and lower aortic saturation (odds ratio, 0.92; p = 0.008) in the entire group. When patients with hepatic vein inclusion (following previous Kawashima) were excluded, higher hemoglobin (odds ratio, 1.24; p = 0.027), lower aortic saturation (odds ratio, 0.92; p = 0.017), and placement of a fenestration (odds ratio, 2.438; p = 0.021) were associated with prolonged length of stay. Fifty-eight patients (26.6%) had major complications defined by Pediatric Cardiac Critical Care Consortium. Placement of a fenestration (odds ratio, 2.297; p = 0.014) and longer aortic cross-clamp time (odds ratio, 1.015; p = 0.003) were independently associated with Pediatric Cardiac Critical Care Consortium major complications. CONCLUSIONS: In this series, 75% of patients had a postoperative length of stay less than or equal to 2 weeks. Preoperative factors suggesting worse hypoxemia/decreased pulmonary blood flow (higher hemoglobin and lower oxygen saturation) and increased pulmonary artery pressure were associated with prolonged length of stay. These findings may help risk stratify this complex patient population, provide more accurate family counseling, and provide preliminary data for changes in preoperative timing of the Fontan and/or changes to postoperative management strategies for those at high risk for increased ICU morbidity.


Subject(s)
Fontan Procedure , Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Length of Stay/statistics & numerical data , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Intraoperative Period , Logistic Models , Male , Preoperative Period , Retrospective Studies , Risk Factors , Treatment Outcome , Young Adult
10.
Congenit Heart Dis ; 11(6): 570-577, 2016 Dec.
Article in English | MEDLINE | ID: mdl-26931510

ABSTRACT

OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. SETTING: Tertiary care children's hospital. PATIENTS: Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. RESULTS: One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P < .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P > .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). CONCLUSIONS: In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.


Subject(s)
Arterial Switch Operation , Coronary Vessels/diagnostic imaging , Echocardiography, Doppler, Color , Transposition of Great Vessels/diagnostic imaging , Transposition of Great Vessels/surgery , Female , Hospitals, Pediatric , Humans , Infant , Infant, Newborn , Male , Predictive Value of Tests , Reproducibility of Results , Retrospective Studies , Tertiary Care Centers , Time Factors , Treatment Outcome
11.
Article in English | MEDLINE | ID: mdl-24040624

ABSTRACT

The PediBooster external cardiac compression device is a minimally invasive, non-blood contacting Biventricular Assist Device (BiVAD) intended for pediatric use. It is being developed as a palliative therapy for acute Postcardiotomy Shock (PCS). The PediBooster extracardiac wrap is pneumatically actuated to circumferentially compress the heart, providing co-pulsation support. Attachment is via a novel hydrogel coating. Early versions of the wrap were tested in vivo using a single ventricle congenital heart disease model with postcardiotomy shock, which proved unstable and demonstrated high peri-operative mortality. The final wrap design was tested in 4 acute studies with piglets (5.1 ± 0.3 kg), where the combination of ASD and PA banding induced acute right ventricular dysfunction. Data collected included routine hemodynamic values, TEE, video of the exposed heart, and cardiac histology. The model proved stable for support durations ranging from 2 to 16 hours. The wrap restricted the heart in 3 of the 4 animals, as evidenced by increased diastolic LVP during support compared to the baseline failure condition. TEE and video data showed good attachment and function of the wrap, particularly during the final 16 hr study. This model of congenital heart disease shows promise for chronic (24-72 hr) studies. Ventricular filling during support may be improved by adjusting wrap dimensions to eliminate end diastolic restriction.

13.
J Extra Corpor Technol ; 44(1): 15-20, 2012 Mar.
Article in English | MEDLINE | ID: mdl-22730859

ABSTRACT

This study compares four different activated clotting time (ACT) point-of-care (POC) testing systems used at our institution for the management of patients undergoing heparin therapy. We evaluated these systems under identical conditions to determine their accuracy, reproducibility, ease of use, and cost. Two separate testing stations containing four ACT systems were used. The testing order was randomized for every sample and performed by two trained individuals. Samples of fresh heparinized whole blood were taken at regular intervals and distributed to each station. Each operator tested 50 samples, totaling 400 ACT tests. The ACT value was significantly affected by the type of machine used at both stations 1 and 2 (p < .001). Compared with all systems, the Medtronic ACT Plus Automated Coagulation Timer System (ACT Plus) resulted in the most consistent ACT values (median = 171, Interquartile Range (IQR): 169-175) and least variability (172.17 +/- 5.24). The Hemochron Signature Elite Whole Blood Microcoagulation System had the most variability (221.10 +/- 14.78) and yielded consistently higher ACT values (median = 220, IQR: 210-229.5) compared with other systems. The ACT values reported by the i-STAT Handheld and Test Cartridge Blood Analysis System (153.30 +/- 7.87) were consistently lower (median = 154, IQR: 147-161) in comparison to the ACT Plus and Medtronic HMS Plus Hemostasis Management System (180.60 +/- 7.60, median = 181, IQR: 175-186). There was no statistical difference in results between the two testing sites (p > .05) or the operators (p > .05). The significant finding of this study was the affect each system has on the ACT value. This investigation demonstrates the variability that exists among different ACT monitoring systems at our institution. The discrepant variation in ACT values that exists with the Hemochron system questions the reliability of its use in the management of patients undergoing heparin therapy.


Subject(s)
Blood Coagulation/drug effects , Blood Coagulation/physiology , Drug Monitoring/instrumentation , Heparin/pharmacology , Point-of-Care Systems , Whole Blood Coagulation Time/instrumentation , Anticoagulants/pharmacology , Child , Equipment Design , Equipment Failure Analysis , Humans , Reproducibility of Results , Sensitivity and Specificity
14.
Catheter Cardiovasc Interv ; 78(1): 93-100, 2011 Jul 01.
Article in English | MEDLINE | ID: mdl-21456031

ABSTRACT

OBJECTIVE: We describe a hybrid approach to the treatment of aortic obstruction after stage 1 palliation (S1P) of hypoplastic left heart syndrome. BACKGROUND: Recurrent aortic obstruction is a common problem after S1P of hypoplastic left heart syndrome. Even mild aortic obstruction is poorly tolerated so early and definitive therapy is desirable. Although stent implantation is an effective treatment for aortic obstruction in older children and adults, technical issues due to small vessels and concerns regarding future potential for expansion have generally precluded the use of stents in this setting. METHODS: Six patients underwent hybrid aortic reconstruction (HAR) in the operating room or catheterization laboratory, with the interventional cardiologist and cardiac surgeon working in cooperation. RESULTS: Patients had a mean weight of 5.8 kg (2.9-7.7) and a mean age of 5.6 months (0.5-12.9) at the time of HAR. Five patients had undergone prior balloon angioplasty at a mean age of 2.8 months (2.1-3.5), and five had moderately depressed single ventricular function prior to HAR. The balloons used had a diameter of 7-10 mm and introducer sheath size ranged from 6 to 10 F. There were no immediate or late procedure related complications. Stent redilation was performed in 5 patients for relief of recurrent obstruction or to keep pace with somatic growth. At a median follow up of 4.8 years (0.2-7.9), there were 3 patients progressing well after Fontan palliation and 3 deaths. CONCLUSIONS: HAR allows for placement of stents that can ultimately reach adult size in small infants who have recurrent aortic obstruction after balloon angioplasty following S1P. Advantages include freedom from delivery sheath constraints when determining stent type/size, facilitation of precise stent position, and avoidance of vascular damage or hemodynamic compromise during the procedure. Longer follow-up and larger experience are required to determine if this therapy will provide a long-term solution to this difficult problem.


Subject(s)
Angioplasty, Balloon , Aortic Diseases/therapy , Arterial Occlusive Diseases/therapy , Fontan Procedure/adverse effects , Hypoplastic Left Heart Syndrome/surgery , Norwood Procedures/adverse effects , Angioplasty, Balloon/adverse effects , Angioplasty, Balloon/instrumentation , Angioplasty, Balloon/mortality , Aortic Diseases/etiology , Aortic Diseases/mortality , Aortic Diseases/physiopathology , Arterial Occlusive Diseases/etiology , Arterial Occlusive Diseases/mortality , Arterial Occlusive Diseases/physiopathology , Florida , Fontan Procedure/mortality , Humans , Hypoplastic Left Heart Syndrome/mortality , Hypoplastic Left Heart Syndrome/physiopathology , Infant , Kaplan-Meier Estimate , Norwood Procedures/mortality , Palliative Care , Prosthesis Design , Recurrence , Stents , Time Factors , Treatment Outcome
15.
Pediatr Cardiol ; 32(6): 778-84, 2011 Aug.
Article in English | MEDLINE | ID: mdl-21479823

ABSTRACT

Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit of residual lesions results in a significant number of additional interventional catheterizations and operative procedures but might have an important influence on long-term survival after the Fontan procedure.


Subject(s)
Fontan Procedure , Heart Defects, Congenital/surgery , Postoperative Complications/epidemiology , Adolescent , Cardiac Catheterization , Child , Child, Preschool , Female , Florida/epidemiology , Follow-Up Studies , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/mortality , Humans , Incidence , Infant , Male , Retrospective Studies , Survival Rate/trends , Time Factors , Treatment Outcome
16.
Cardiol Young ; 20 Suppl 3: 128-34, 2010 Dec.
Article in English | MEDLINE | ID: mdl-21087569

ABSTRACT

BACKGROUND: Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution. METHODS: We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography. RESULTS: We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years. CONCLUSIONS: An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies.


Subject(s)
Abnormalities, Multiple/surgery , Pulmonary Artery/abnormalities , Pulmonary Atresia/surgery , Tetralogy of Fallot/surgery , Cardiac Surgical Procedures/methods , Female , Humans , Infant , Male , Pulmonary Atresia/complications , Retrospective Studies , Tetralogy of Fallot/complications
17.
Ann Thorac Surg ; 90(1): 274-6, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20609793

ABSTRACT

The management of complete heart block in premature low birth-weight infants, particularly those with hydrops fetalis, is challenging. We report emergent implantation of permanent epicardial pacemakers in the first 48 hours of life in two premature infants (one with hydrops fetalis) with birth weights of 1,400 grams and 1,000 grams.


Subject(s)
Cardiac Pacing, Artificial , Heart Block/therapy , Adult , Cesarean Section , Critical Illness , Female , Heart Block/complications , Humans , Hydrops Fetalis , Infant, Newborn , Infant, Premature , Infant, Very Low Birth Weight , Pregnancy
18.
Cardiol Young ; 20(5): 477-84, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20456816

ABSTRACT

OBJECTIVE: The purpose of this study was to evaluate the utilisation of a web-based multimedia patient-accessible electronic health record, for patients with congenital cardiac disease. PATIENTS AND METHODS: This was a prospective analysis of patients undergoing congenital cardiac surgery at a single institution from 1 September, 2006 to 1 February, 2009. After meetings with hospital administration, physicians, nurses, and patients, we configured a subset of the cardiac program's web-based clinical electronic health record for patient and family access. The Electronic Health Record continuously measured frequency and time of logins, logins during and between hospitalisations, and page views by type (imaging versus textual data). RESULTS: Of the first 270 patients offered access to the system, 252 became users (93% adoption rate). System uptime was 99.9%, and no security breaches were reported. Users accessed the system more often while the patients were in hospital (67% of total logins) than after discharge (33% of total logins). The maximum number of logins by a family was 440, and the minimum was 1. The average number of logins per family was 25. Imaging data were viewed significantly more frequently than textual data (p 0.001). A total of 12 patients died during the study period and 11 members of their families continued to access their Electronic Health Records after the date of death. CONCLUSIONS: A web-based Patient Accessible Electronic Health Record was designed for patients with congenital cardiac disease. The adoption rate was high, and utilisation patterns suggest that the Electronic Health Record could become a useful tool for health information exchange.


Subject(s)
Electronic Health Records/organization & administration , Family , Health Records, Personal , Internet , Medical Records Systems, Computerized/organization & administration , Multimedia , Patient Access to Records , Adolescent , Attitude to Computers , Child , Female , Heart Defects, Congenital/surgery , Humans , Male , Prospective Studies , Reproducibility of Results
19.
Paediatr Anaesth ; 19(6): 571-6, 2009 Jun.
Article in English | MEDLINE | ID: mdl-19645974

ABSTRACT

AIM: To determine the efficacy and safety of a continuous subcutaneous local anesthetic (LA) infusion in pediatric patients following open heart surgery. BACKGROUND: The use of a continuous LA infusion has been shown to be beneficial following adult cardiac surgery. To date there are no studies in the pediatric population. METHODS/MATERIALS: Using a prospective, randomized, and double blind design, we compared LA, either 0.25% levobupivacaine or bupivacaine (Treatment Group) to saline (Placebo Group) delivered subcutaneously via a continuous infusion for 72 h after open heart surgery in 72 patients. Requirements for postoperative analgesics and pain scores were recorded for 72 h and plasma levels of local anesthetic were measured. Secondary outcomes measures included time to first oral intake, time to first bowel movement, time to urinary catheter removal, length of stay, requirements for antiemetics and additional sedation. RESULTS: Total morphine requirements over the first 24 h were less in the Treatment Group than the Placebo Group (0.05 mg x kg(-1) vs 0.2 mg x kg(-1), P = 0.007); this was true for all patient groups except those patients weighing less than 6.3 kg. The number of patients requiring no morphine was greater in the Treatment Group (7/35 vs 1/37, P = 0.02). The Treatment Group also received less midazolam, lorazepam, and ketorolac than the Placebo Group over 72 h due to the reduced clinical need for these agents in patients weighing less than 31 kg. There were no differences in secondary outcomes. CONCLUSIONS: A continuous incisional infusion of LA reduced postoperative analgesic requirement and sedative use in pediatric patients undergoing a median sternotomy incision. Dosed at a maximum rate of 0.4 mg x kg(-1) x h(-1), a continuous incisional infusion of LA is effective and safe for up to 72 h, with plasma levels of local anesthetic well below the toxic threshold.


Subject(s)
Anesthetics, Local/administration & dosage , Bupivacaine/administration & dosage , Cardiac Surgical Procedures/methods , Adolescent , Anesthetics, Local/blood , Bupivacaine/analogs & derivatives , Bupivacaine/blood , Child , Child, Preschool , Double-Blind Method , Female , Humans , Infant , Infusions, Subcutaneous , Levobupivacaine , Male , Morphine/therapeutic use , Pain, Postoperative/drug therapy , Postoperative Nausea and Vomiting/prevention & control , Prospective Studies , Treatment Outcome
20.
Ann Thorac Surg ; 88(2): 688-9, 2009 Aug.
Article in English | MEDLINE | ID: mdl-19632452

ABSTRACT

Initial palliation for pulmonary atresia with intact ventricular septum continues to evolve in the face of significant early and late morbidity. In patients with suitable anatomy, decompression of the right ventricle may be the first step in treatment. A hybrid approach to right ventricular decompression, combining surgery and interventional catheterization techniques is described. Direct access to the right ventricle through a subxiphoid incision with transventricular sheath placement is used to provide optimum catheter position for radiofrequency perforation of membranous pulmonary atresia followed by balloon dilation. The technique may address key limitations of the traditional surgical and interventional approaches.


Subject(s)
Cardiac Surgical Procedures/methods , Catheter Ablation/methods , Heart Ventricles/surgery , Pulmonary Atresia/surgery , Cardiac Catheterization/methods , Catheterization , Decompression, Surgical , Heart Septum , Humans , Pulmonary Valve
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