Background To determine if RV volume was predictive of survival and cardiovascular adverse event (CAE) after pulmonary valve replacement (PVR). Methods We reviewed the MACHD ( M ayo A dult C ongenital H eart D isease) database for patients with tetralogy of Fallot (TOF) undergoing PVR, 2000–2015. The patients were divided into quartiles based on RV end-diastolic volume index (RVEDVI); those in the lowest quartile (Group A, n = 46) and the top quartile (Group B, n = 42) were selected as the study cohort. Results In comparison to Group A, Group B patients were older at time of PVR (28 ± 4 vs 33 ± 5 years, P = .011) and had larger RV volumes (RVEDVI 127 [117–138] mL/m 2 vs 1 91 [179–208], P 2 vs 122 [103–136], P P = .273) but freedom from CAE was significantly lower in Group B (67% vs 36% at 10 years, P = .002). Combination of RVESVI: >95 mL/m 2 and tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) Conclusion Patients undergoing PVR at larger RV volumes had similar survival but more overall CAE. A larger study population with a longer follow-up will be required to determine if early PVR provides survival benefit in the long-term.
Transcatheter pulmonary valve replacement (TPVR) is an important treatment option in repaired tetralogy of Fallot (TOF) and right ventricular outflow tract (RVOT) dysfunction. Indications for timing of TPVR are extrapolated from surgical pulmonary valve replacement guidelines, which are themselves controversial as published evidence is scarce and expert opinion therefore prevails. We review current indications for PVR following TOF repair, focusing on those for TPVR specifically, and discuss ana...
Background— Right ventricular (RV) dysfunction (RVD) is a poor prognostic factor in heart failure with preserved ejection fraction (HFpEF). The physiological perturbations associated with RVD or RV function indexed to load (RV–pulmonary arterial [PA] coupling) in HFpEF have not been defined. HFpEF patients with marked impairment in RV–PA coupling may be uniquely sensitive to sildenafil. Methods and Results— In a subset of HFpEF patients enrolled in the Phosphodiesteas-5 Inhibition to Improve Cli...
Aims The right ventricle (RV) remodels early after pulmonary valve replacement (PVR) in tetralogy of Fallot (TOF) patients. Previously reported preoperative thresholds to achieve early postoperative RV normalization were consistently close to 80 mL/m2 for end-systolic volume (ESV) and 160 mL/m2 for end-diastolic volume (EDV). Our objective was to determine whether these thresholds were also associated with mid-to-late RV normalization and clinical events. Methods and results Out of a multicentre...
Heart failure and preserved ejection fraction patients (n ¼ 96) and controls (n ¼ 46) underwent right heart catheteriza- tion, echocardiographic assessment, and follow-up. Right and left heart filling pressures, pulmonary artery (PA) pressures, and right-sided chamber dimensions were higher in HFpEF compared with controls, while left ventricular size and EF were similar. Right ventricular dysfunction (defined by RV fractional area change, FAC ,35%) was present in 33% of HFpEF patients and was as...
Background Novel echocardiographic techniques based on myocardial deformation have not been extensively evaluated to assess right ventricular (RV) and left ventricular (LV) response after pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot. Methods Between 2003 and 2012, 133 patients undergoing first-time PVR after tetralogy of Fallot repair underwent echocardiographic assessment at Mayo Clinic. The last echocardiogram before PVR and 1 year after surgery were retrospe...
#1Alexander C. Egbe(ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 8
#2Santosh Uppu(ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 12
Last. Shubhika Srivastava(ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 24
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Current estimates of the incidence of congenital heart disease (CHD) are derived from small clinical studies and metaanalyses. For the true incidence of CHD in the United States of America to be estimated, a single large representative population must be analyzed. All the data in this study were derived from the Nationwide Inpatient Sample database. The study determined the overall and lesion-specific incidences of CHD diagnoses among all birth hospitalizations in 2008, stratified by race, gende...
Last. Darryl F. Shore(NIH: National Institutes of Health)H-Index: 32
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Background—Indications for surgical pulmonary valve replacement (PVR) after repair of tetralogy of Fallot have recently been broadened to include asymptomatic patients. Methods and Results—The outcomes of PVR in adults after repair of tetralogy of Fallot at a single tertiary center were retrospectively studied. Preoperative cardiopulmonary exercise testing was included. Mortality was the primary outcome measure. In total, 221 PVRs were performed in 220 patients (130 male patients; median age, 32...
In contrast to adult patients with acquired heart disease, abnormalities of the right ventricle (RV) are ubiquitous in children and adults with congenital heart disease (CHD). The RV is exposed to volume overload in shunt lesions (eg, atrial septal defect, anomalous pulmonary venous connections), as well as congenital or acquired tricuspid and pulmonary valve regurgitation. RV pressure overload characterizes numerous congenital anomalies, including pulmonary valve stenosis or atresia, large vent...
Objective Right ventricular–pulmonary arterial coupling is an important determinant in the development of right ventricular failure. The purpose of our study was to assess right ventricular–pulmonary arterial coupling in children and adolescents with dilatation of the right ventricle after repair of tetralogy of Fallot. Methods Right ventricular–pulmonary arterial coupling was quantified as the ratio of pulmonary arterial elastance (an index of arterial load) and right ventricular end-systolic e...
OBJECTIVE Median sternotomy has been the standard for pulmonary valve replacement (PVR) in patients with free pulmonary regurgitation (PR) and right ventricular enlargement. With the introduction of transcatheter therapy, the search for an alternate to sternotomy is mandated. We present our early experience with a limited anterior left thoracotomy approach. METHODS We used a left anterior mini-thoracotomy in six male patients (15 ± 1.94 years of age) who developed progressive right ventricular e...
Last. Koji Maemura(Nagasaki University)H-Index: 49
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Abstract A 59-year-old Japanese woman was admitted with heart failure due to severe pulmonary regurgitation and tricuspid regurgitation, in addition to atrial fibrillation 45 years after surgical correction of tetralogy of Fallot (TOF). She had been under treatment with medication and catheter ablation for arrhythmia including ventricular tachycardia for the past 28 years. She underwent pulmonary valve replacement as well as tricuspid and mitral valvuloplasty, which obviously improved her status...
#1Dilachew A. Adebo(University of Texas Health Science Center at Houston)
Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart defects. The diagnosis of TOF is generally made by echocardiography. Cardiac CT may be needed to delineate preoperative anatomy or postoperative complications. Complete surgical repair includes relief of RVOT obstruction or pulmonary stenosis and patch closure of the ventricular septal defect. Surgical approaches emphasize maintaining pulmonary valve competence whenever possible. Residual VSD or residual RVOT obstructi...
AIMS Right atrial pressure (RAP) provides a composite measure of right ventricular diastolic dysfunction, right atrial compliance, and volume status, and these three variables are typically abnormal in adults with repaired tetralogy of Fallot (TOF). RAP is a well-established prognostic metric in patients with pulmonary hypertension, and recent data suggest that RAP is associated with clinical outcomes in TOF. The purpose of this study was to determine the role of inferior vena cava (IVC) haemody...
OBJECTIVES: The objectives of this study were to evaluate long-term outcomes of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (TOF) and to identify the factors associated with adverse clinical events (ACEs). METHODS: A total of 190 patients who underwent PVR between 1998 and 2015 after repair of TOF were retrospectively analysed. ACE was defined as all-cause death, heart transplantation or new-onset sustained arrhythmia. Univariable Cox proportional hazards regr...
The number of rTOF patients who survive into adulthood is steadily rising, with currently more than 90% reaching the third decade of life. However, rTOF patients are not cured, but rather have a lifelong increased risk for cardiac and non-cardiac complications. Heart failure is recognized as a significant complication. Its occurrence is strongly associated with adverse outcome. Unfortunately, conventional concepts of heart failure may not be directly applicable in this patient group. This articl...
Last. Nancy Poirier(UdeM: Université de Montréal)H-Index: 25
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Abstract Background Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. Objectives This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic devi...
Abstract Background Inferior vena cava (IVC) size and collapsibility provides a noninvasive estimate of right heart filling pressures, an important determinant of right heart hemodynamic performance that is not measured by cardiac magnetic resonance imaging (CMRI). We hypothesized that compared to CMRI risk model alone, a combined CMRI-IVC risk model will have better correlation with disease severity and peak oxygen consumption (VO2) in patients with tetralogy of Fallot (TOF). Methods Retrospect...
Abstract Background Left heart filling pressures, as measured by pulmonary artery wedge pressure (PAWP), is associated with heart failure related mortality. Because of the prognostic importance of PAWP, several echocardiographic indices have been proposed for noninvasive assessment of PAWP. However, these indices have not been validated in the congenital heart disease population. The purpose of this study was to determine the correlation between echocardiographic indices of PAWP, and the effect ...