Faculty Bibliography
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37
Use of echocardiographic subxiphoid five-sixth area length (bullet) method in evaluation of adequacy of borderline left ventricle in hypoplastic left heart complex
Indexed left ventricular end-diastolic volume (ILVEDV) is commonly used in evaluating "borderline left ventricle (LV)" in hypoplastic left heart complex (HLHC) to determine if the LV can sustain adequate systemic cardiac output. Commonly used quantification methods include biplane Simpson or the traditional five-sixth area length "bullet" methods, which have been shown to underestimate true LV volumes, when septal position is mildly abnormal. Subxiphoid five-sixth area length method is proposed as a more accurate estimate of true LV volume in the evaluation of borderline LV.
PMCID:4608205
PMID: 26556974
ISSN: 0974-2069
CID: 1834572
Prenatal Diagnosis of a Persistent Fifth Aortic Arch, Pulmonary-to-Systemic type: An Unusual Association with Evolving Aortic Coarctation
Persistent fifth arch (PFA) is a rare anomaly that is often underdiagnosed and missed. Different PFA types that have been reported in the literature are systemic-to-systemic type (most common), systemic-to-pulmonary artery (PA), and PA-to-systemic types. The designations of systemic-to-PA or PA-to-systemic are based on if the PFA is a source of PA or systemic blood flow, respectively, in the setting of critical proximal obstruction (pulmonary atresia or aortic atresia). This case describes an unusual PFA, which connects the distal PA to distal ascending aorta; however, it is not associated with critical proximal obstruction, and while it appeared to be an incidental finding in early gestation, progressive serial distal obstruction of the left fourth arch was seen to develop. This case highlights that prenatal diagnosis of PFA is possible and that once a diagnosis is made, serial fetal echocardiograms should be performed to evaluate for evolving lesions.
PMID: 25418608
ISSN: 0742-2822
CID: 1359402
Candidacy for device closure of complex muscular ventricular septal defects: Novel application of rapid prototyping and virtual 3d models derived fcardiac CT and MRI [Meeting Abstract]
Background: Complex muscular ventricular septal defects (CMVSD) are often difficult to surgically close and managed by device closure. The pre-intervention imaging is crucial in defining the anatomy and aids in patient selection. We hypothesized that 3D physical and virtual models in patients with CMVSD is feasible, would assist in patient selection and aid in the successful device closure. Methods: Virtual and physical 3D models on 3 patients with CMVSD were generated from CT or MRI data, using Mimics, and 3-Matic software. The first patient had history of complicated and unsuccessful prior device closures, with residual shunt. Two physical models, with and without devices in situ were printed (Figure 1A) for this patient. Two virtual models were generated in the other two patients. Results: The location, size, trabeculations, papillary and muscle bundles were clearly visualized in all patients. The two physical models were extensively studied, resulting in successful device closure of the residual VSD. The virtual model on patient 2 identified RV papillary muscles adjacent to the CMVSD (Figure 1B) precluding device closure. The patient 3 model identified muscle bundles crowding the VSD suggesting potential for spontaneous closure. Conclusion: Construction of 3D models in patients with CMVSD is feasible, assists in appropriate patient selection and allows for extensive examination and planning. This may facilitate a focused and informed procedure and improve the potential for successful closure. (Figure Presented)
EMBASE:71833630
ISSN: 0735-1097
CID: 1561102
PATIENT-SPECIFIC SIMULATION OF RIGHT VENTRICLE OUTFLOW TRACT CONDUIT BALLOON ANGIOPLASTY USING CARDIAC MRI-DERIVED 3D VIRTUAL MODELS TO ASSESS THE RISK OF CORONARY ARTERY COMPRESSION DURING TRANSCATHETER PULMONARY VALVE REPLACEMENT [Meeting Abstract]
ISI:000375328800573
ISSN: 0735-1097
CID: 2962462
Evaluation of pulmonary vasodilators in pediatric congenital cardiovascular care [Meeting Abstract]
Learning Objectives: Pulmonary hypertension (PH) is a serious complication that occurs in some patients following congenital heart surgery (CHS) and increases mortality risk. Inhaled (INH) iloprost is a prostacyclin analog that has been successfully used in pediatric patients but data are limited. We hypothesize that INH iloprost is safe and effective for prevention or treatment of post-operative PH and can be weaned by protocol in pediatric patients following CHS. Methods: This was a retrospective review of pediatric patients who underwent CHS at our institution from January 2013 to May 2014. All patients who received INH iloprost for prevention or treatment of PH were included. There were no exclusion criteria. The primary outcome measures were the efficacy and safety of INH iloprost defined by the effect on the pulmonary to systemic pressure ratio (P/S) and systolic blood pressure (SBP) respectively. Secondary outcomes included evaluation of INH iloprost dosing and weaning strategy. The Wilcoxon signed rank test was used to compare measures. P values less than 0.05 were considered significant. Results: Sixteen patients were included with a median age at surgery of 2 months (0.07 to 136 months) and a median weight of 3.9 kg (2.2 to 25.4 kg). The median starting dose of INH iloprost was 0.5 mcg/ kg (0.2- 1.25 mcg/kg). Two patients had the dose increased during the treatment period. Fifteen patients received the drug every two hours at initiation. Following initiation of INH iloprost median P/S decreased in all patients (0.69 to 0.39; p<0.005) and there was no change in median SBP (71.5 versus 72.9 mmHg; p=0.08). Eight patients were weaned to an every four hour and then every eight hour regimen prior to discontinuation. Median overall wean duration was 48 hours. No patients suffered a pulmonary hypertensive crisis during or after the treatment period. Conclusions: INH iloprost is safe and effective for prevention or treatment of post-operative PH following pediatric CHS. A dose of 0.5 mcg/kg administered every 2 hours is effective, well tolerated and may be safely weaned off over 48 hours
EMBASE:71706907
ISSN: 0090-3493
CID: 1423572
Use of the Aquadexâ„¢ system for ultrafiltration therapy in a hemodynamically unstable pediatric patient [Case Report]
In this case report, we describe the use of the Aquadexâ„¢ system for ultrafiltration therapy in the pediatric cardiac intensive care setting in a patient with fluid overload and acute kidney injury after congenital heart surgery. The patient is an 11-year-old, 25 kg male with complex single ventricle anatomy who underwent a one and a half ventricle repair. The patient experienced multiple organ dysfunction syndrome including acute kidney injury in the early post-operative period secondary to low cardiac output syndrome and tachyarrhythmia. Ultrafiltration using the Aquadexâ„¢ system was utilized to treat fluid overload in the setting of acute kidney injury and hemodynamic instability. Negative fluid balance was safely achieved. It was subsequently possible to wean ventilatory and inotropic support. We conclude that the use of ultrafiltration therapy is feasible in hemodynamically unstable pediatric patients with significant fluid overload in the setting of acute kidney injury following congenital heart surgery.
PMCID:6530742
PMID: 31214453
ISSN: 2146-4618
CID: 3956162
Vascular ring in an infant with cystic fibrosis
A vascular ring (VR) is a rare congenital anomaly that has variable clinical presentation, but may cause trachea-esophageal compressive symptoms. We describe an infant with cystic fibrosis and subtle trachea-esophageal compressive symptoms which were initially attributed to other causes, delaying the diagnosis of a VR
ORIGINAL:0009377
ISSN: 1927-1255
CID: 1431972
Antithrombotic strategies in children receiving long-term Berlin Heart EXCOR ventricular assist device therapy
BACKGROUND: Thromboembolic events while receiving ventricular assist device (VAD) support remain a significant cause of morbidity and mortality despite standard anti-coagulation and anti-platelet therapies. The use of bivalirudin and epoprostenol infusions as an alternate anti-thrombotic (AT) regimen in pediatric VAD patients was reviewed. METHODS: This was a retrospective record review of 6 pediatric patients (aged
PMID: 23465252
ISSN: 1053-2498
CID: 963652
Sudden death after pediatric heart transplantation: analysis of data from the Pediatric Heart Transplant Study Group
BACKGROUND: Sudden death is a well-recognized complication of heart transplantation. Little is known about the incidence and risk factors for sudden death after transplant in children. The purpose of this study was to determine the incidence of and risk factors for sudden death. METHODS: This retrospective multicenter cohort study used the Pediatric Heart Transplant Study Group (PHTS) database, an event-driven registry of children aged <18 at listing undergoing heart transplantation between 1993 and 2007. Standard Kaplan-Meier and parametric analyses were used for survival analysis. Multivariate analysis in the hazard-function domain was used to identify risk factors for sudden death after transplant. RESULTS: Of 604 deaths in 2,491 children who underwent heart transplantation, 94 (16%) were classified as sudden. Freedom from sudden death was 97% at 5 years, and the hazard for sudden death remained constant over time at 0.01 deaths/year. Multivariate risk factors associated with sudden death included black race (hazard ratio [HR], 2.6; p < 0.0001), United Network of Organ Sharing (UNOS) status 2 at transplant (HR, 1.8; p = 0.008), older age (HR, 1.4/10 years of age; p = 0.03), and an increased number of rejection episodes in the first post-transplant year (HR, 1.6/episode; p = 0.03). CONCLUSION: Sudden death accounts for 1 in 6 deaths after heart transplant in children. Older recipient age, recurrent rejection within the first year, black race, and UNOS status 2 at listing were associated with sudden death. Patients with 1 or more of these risk factors may benefit from primary prevention efforts.
PMCID:3210418
PMID: 21996348
ISSN: 1053-2498
CID: 957712
Factors affecting the decision to defer endotracheal extubation after surgery for congenital heart disease: a prospective observational study
BACKGROUND: Fast-tracking and early endotracheal extubation have been described in patients undergoing surgery for congenital heart disease (CHD); however, criteria for patient selection have not been validated in a prospective manner. Our goal in this study was to prospectively identify factors associated with the decision to defer endotracheal extubation in the operating room (OR). METHODS: We performed a prospective observational study of 275 patients (median age 18 months) at the Mount Sinai Medical Center (MSMC), New York, New York, and 49 patients (median age 25 months) at the University of Tokyo Hospital (UTH), Tokyo, Japan, undergoing surgery for CHD requiring cardiopulmonary bypass. These patients were all eligible for fast-tracking, including extubation in the OR immediately after surgery, according to the respective inclusion/exclusion criteria applied at the 2 sites. RESULTS: Eighty-nine percent of patients at the MSMC, and 65% of patients at the UTH were extubated in the OR. At the MSMC, all patients without aortic cross-clamp, and patients with simple procedures (Risk Adjustment for Congenital Heart Surgery [RACHS] score 1) were extubated in the OR. Among the remaining MSMC patients, regression analysis showed that procedure complexity was still an independent predictor for not proceeding with planned extubation in the OR. Extubation was more likely to be deferred in the RACHS score 3 surgical risk patients compared with the RACHS score 2 group (P = 0.005, odds ratio 3.8 [CI: 1.5, 9.7]). Additionally, trisomy 21 (P = 0.0003, odds ratio 9.9 [CI: 2.9, 34.5]) and age (P = 0.0015) were significant independent predictors for deferring OR extubation. We tested our findings on the patients from the UTH by developing risk categories from the MSMC data that ranked eligible patients according to the chance of OR extubation. The risk categories proved to predict endotracheal extubation in the 49 patients who had undergone surgery at the UTH relative to their overall extubation rate, despite differences in anesthetic regimen and inclusion/exclusion criteria. CONCLUSIONS: Preoperatively known factors alone can predict the relative chances of deferring extubation after surgery for CHD. The early extubation strategies applied in the 2 centers were successful in the majority of cases.
PMID: 21490084
ISSN: 0003-2999
CID: 963662
