Browsing by Subject "Tetralogy of Fallot"
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Item Open Access Comparison of long-term postoperative sequelae in patients with tetralogy of Fallot versus isolated pulmonic stenosis.(Am J Cardiol, 2014-07-15) Zdradzinski, Michael J; Qureshi, Athar M; Stewart, Robert; Pettersson, Gosta; Krasuski, Richard APatients with tetralogy of Fallot (TOF) after complete repair and pulmonic stenosis (PS) after surgical valvotomy often develop significant pulmonic regurgitation (PR) that eventually requires valve replacement. Although criteria exist for the timing of pulmonary valve replacement in TOF, it remains less clear when to intervene in valvotomy patients and whether TOF recommendations can be applied. Our aim was to compare the structural and functional sequelae of valvotomy for PS with complete repair for TOF. We compared the clinical characteristics, electrocardiograms, echocardiograms, cardiac magnetic resonance imaging (MRI), and invasive hemodynamics of 109 adults (34 PS and 75 TOF) newly referred to a congenital heart disease center for evaluation of PR between 2005 and 2012. Both cohorts were similar in terms of baseline demographics and presenting New York Heart Association function class. Valvotomy patients had a slightly greater degree of PR by echocardiogram, although it was similar by cardiac MRI. Electrocardiography QRS width was greater in patients with TOF (114±27 vs 150±28 ms, p<0.001). MRI right ventricular ejection fraction (49±8 vs 41±11%, p=0.001) and left ventricular ejection fraction (59±7 vs 52±10%, p=0.002) were lower in patients with TOF. Pacemaker or defibrillator implantation was significantly greater in patients with TOF (3% vs 23%, p=0.011). In conclusion, patients postvalvotomy and complete repair present with similar degrees of PR and severity of symptoms. Biventricular systolic function and electrocardiography QRS width appear less affected, suggesting morphologic changes in TOF and its repair that extend beyond the effects of PR. These findings suggest the need for developing disease-specific guidelines for patients with PR postvalvotomy.Item Open Access Incremenal Value of Cardiac Magnetic Resonance for Assessing Pulmonic Valve Regurgitation.(The Journal of heart valve disease, 2015-07) Zdradzinski, Michael; Elkin, Rachel; Flamm, Scott; Krasuski, RichardCardiac magnetic resonance (CMR) is the 'gold standard' for quantifying pulmonic regurgitation (PR) in adults with congenital heart disease, but remains costly and is less readily available than echocardiography. Qualitative echocardiographic assessment of PR is challenging, and guiding criteria are limited. It is unknown if echocardiography is sufficient to screen for significant PR. The study aim was to determine whether cardiac MRI provides additional benefit in the assessment of PR in adults with congenital heart disease.Patients with repaired tetralogy of Fallot or congenital pulmonic stenosis after valvotomy undergoing transthoracic echocardiography and CMR with no interval intervention were identified from a prospective registry. Patients with greater than mild pulmonic stenosis, residual ventricular septal defect or poor echocardiographic windows were excluded. Whole-cohort and subgroup (tetralogy of Fallot versus pulmonic stenosis) analyses for inter-modality agreement were performed.A total of 48 patients (24 men, 24 women; mean age 43 +/- 12 years) was included in the analysis. The unweighted kappa value for the two modalities was 0.30, suggesting 'fair' agreement, though only 52% had matching PR assessments. The indexed right ventricular end-systolic volume (RVESVi) correlated closely with cardiac MRI-monitored PR (p = 0.011 by analysis of variance), but not with that monitored with echocardiography (p = 0.081). Subgroup analysis demonstrated less inter-modality agreement in the tetralogy of Fallot population (kappa 0.25) than in the pulmonic stenosis population (kappa 0.35).CMR measurement of PR correlates closely with the RVESVi, and appears superior to echocardiography when assessing patients at risk for PR. The study results suggest a vital role for CMR whenever significant PR is suspected in the adult congenital heart disease population.Item Open Access Left Anterior Mini-Incision for Pulmonary Valve Replacement Following Tetralogy of Fallot Repair.(Innovations (Philadelphia, Pa.), 2020-03) Nellis, Joseph R; Vekstein, Andrew M; Meza, James M; Andersen, Nicholas D; Haney, John C; Turek, Joseph WPulmonary insufficiency is a known complication following Tetralogy of Fallot repair. With over 90% of patients now surviving to adulthood, surgeons are once again faced with the question of when, and more importantly, how to reintervene. We developed a novel approach to pulmonary valve replacement in this population through a 5-cm left anterior mini-incision. The incision is optimized for exposing and operating on the right ventricular outflow tract and the main pulmonary artery in patients with a history of median sternotomy. Early outcomes are reassuring, and we believe our approach is a safe and reliable alternative to median sternotomy within this patient population, with the ability to quickly convert intraoperatively when needed.Item Open Access Left Anterior Mini-Incision for Pulmonary Valve Replacement in a 12-Year-Old.(Innovations (Philadelphia, Pa.), 2020-03) Vekstein, Andrew M; Nellis, Joseph R; Meza, James M; Andersen, Nicholas D; Haney, John C; Turek, Joseph WA 12-year-old, 32 kg male with history of Tetralogy of Fallot status post repair at 8 days of life presented with progressive pulmonary insufficiency and left pulmonary artery stenosis. Surgical options were discussed, and the patient and his family elected to pursue minimally invasive pulmonary valve replacement with left pulmonary artery augmentation through a 5-cm left anterior mini-incision. The procedure was performed without complication, and he was discharged on postoperative day 3. At the time of his last follow-up, the patient was recovering well without evidence of pulmonary stenosis or insufficiency.Item Open Access Modified INOvent for delivery of inhaled nitric oxide during cardiac MRI.(Magn Reson Imaging, 2011-10) Devendra, Ganesh P; Hart, Stephen A; Kim, Yuli Y; Setser, Randy M; Flamm, Scott D; Krasuski, Richard ABACKGROUND: The aim of this study was to assess the feasibility of delivering NO through a modified system to allow clearance of the magnetic field and thus compatibility with cardiac magnetic resonance (CMR). Nitric oxide (NO) is an inhalational, selective pulmonary vasodilator with a wide range of applications in a variety of disease states, including diseases that affect the right ventricle. Accurate assessment of dynamic changes in right ventricular function necessitates CMR; however, delivery of NO is only possible using equipment that is not magnetic resonance imaging (MRI) compatible (INOvent delivery system, Ohmeda, Inc., Madison, WI, USA). METHODS: The INOvent delivery system was modified by using 35 ft. of standard oxygen tubing to allow NO delivery through an electrical conduit and into the MRI suite. The concentrations of oxygen (O(2)), nitrogen dioxide (a harmful byproduct, NO(2)) and NO were measured in triplicate using the built-in electrochemical analyzer on the INOvent. After confirmation of safety, the system was used to administer drug to a patient x, and dynamic MRI measurements were performed. RESULTS: When the standard INOvent was set to administer 40 ppm of NO, the mean/standard deviation of gas delivered was as follows: NO: 42/0 ppm; NO(2): 0.3/0.1 ppm; and O(2): 93/0 ppm. In comparison, the gas delivery of the modified INOvent was follows: NO: 41/0 ppm; NO(2): 0.5/0 ppm; and O(2): 93.7/0.6 ppm. During administration to an index patient with severe pulmonic insufficiency (PI), a measurable reduction in PI was observed by CMR. CONCLUSIONS: Nitric oxide can be administered through 35 ft. of standard oxygen tubing without significantly affecting dose delivery. This technique has potential application in patients with right-sided structural heart disease for determination of dynamic physiological changes.Item Open Access PINOT NOIR: pulmonic insufficiency improvement with nitric oxide inhalational response.(J Cardiovasc Magn Reson, 2013-09-04) Hart, Stephen A; Devendra, Ganesh P; Kim, Yuli Y; Flamm, Scott D; Kalahasti, Vidyasagar; Arruda, Janine; Walker, Esteban; Boonyasirinant, Thananya; Bolen, Michael; Setser, Randolph; Krasuski, Richard ABACKGROUND: Tetralogy of Fallot (TOF) repair and pulmonary valvotomy for pulmonary stenosis (PS) lead to progressive pulmonary insufficiency (PI), right ventricular enlargement and dysfunction. This study assessed whether pulmonary regurgitant fraction measured by cardiovascular magnetic resonance (CMR) could be reduced with inhaled nitric oxide (iNO). METHODS: Patients with at least moderate PI by echocardiography undergoing clinically indicated CMR were prospectively enrolled. Patients with residual hemodynamic lesions were excluded. Ventricular volume and blood flow sequences were obtained at baseline and during administration of 40 ppm iNO. RESULTS: Sixteen patients (11 with repaired TOF and 5 with repaired PS) completed the protocol with adequate data for analysis. The median age [range] was 35 [19-46] years, BMI was 26 ± 5 kg/m(2) (mean ± SD), 50% were women and 75% were in NYHA class I. Right ventricular end diastolic volume index for the cohort was 157 ± 33 mL/m(2), end systolic volume index was 93 ± 20 mL/m(2) and right ventricular ejection fraction was 40 ± 6%. Baseline pulmonary regurgitant volume was 45 ± 25 mL/beat and regurgitant fraction was 35 ± 16%. During administration of iNO, regurgitant volume was reduced by an average of 6 ± 9% (p=0.01) and regurgitant fraction was reduced by an average of 5 ± 8% (p=0.02). No significant changes were observed in ventricular indices for either the left or right ventricle. CONCLUSION: iNO was successfully administered during CMR acquisition and appears to reduce regurgitant fraction in patients with at least moderate PI suggesting a potential role for selective pulmonary vasodilator therapy in these patients. TRIALS REGISTRATION: ClinicalTrials.gov, NCT00543933.Item Open Access Uncorrected Tetralogy of Fallot, Biventricular Dysfunction, and a Large Pericardial Effusion.(J Cardiothorac Vasc Anesth, 2015-10) McCartney, Sharon L; Machovec, Kelly; Jooste, Edmund HItem Open Access When 'blue babies' grow up: What you need to know about tetralogy of Fallot.(Cleve Clin J Med, 2010-11) Fox, David; Devendra, Ganesh P; Hart, Stephen A; Krasuski, Richard AMost babies born with tetralogy of Fallot undergo corrective surgery and survive to adulthood. However, as they get older they are prone to a number of long-term problems, and they often do not receive expert-level follow-up care. This review of the adult complications of tetralogy of Fallot should help primary care practitioners identify these patients, make appropriate and timely referrals, and educate patients and their families.