Right ventricular dilatation score: a new assessment to right ventricular dilatation in adult patients with repaired tetralogy of Fallot.

BACKGROUND: Patients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF. METHODS: Thirty two rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3-6 months post-surgery. RESULTS: RV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI) ≥ 160 ml/m2 or right ventricular end-systolic volume index (RVESVI) ≥ 80 ml/m2 in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI = 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI < 160 ml/m2 and RVESVI < 80 ml/m2, and the two parameters were higher than normal values. Compared with the RV mild dilatation group, patients of RV moderate-severe dilatation have worse cardiac function before surgery (right ventricular ejection fraction, 38.92(9.19) % versus 48.31(5.53) %, p < 0.001; Left ventricular ejection fraction, 59.80(10.26) versus 66.41(4.15), p = 0.021). Patients with RV moderate-severe dilatation faced longer operation time and more blood transfusion during operation (operation time, 271.53(08.33) min versus 170.53(72.36) min, p < 0.01; Intraoperative blood transfusion, 200(175) ml versus 100(50) ml, p = 0.001). Postoperative RV moderate-severe dilatation patients have poor short-term prognosis, which was reflected in a longer postoperative hospital stay (6.59 [2.12] days versus 9.80 [5.10] days, p = 0.024) and a higher incidence of hypohepatia (0[0] % versus 4[26.7] %, p = 0.023). Patients with RV dilatation score > 2.35 were diagnosed with RV moderate-severe dilatation (AUC = 0,882; Sensitivity = 94.1%; Specificity = 77.3%). CONCLUSIONS: RV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score > 2.35, the patient is indicated for further CMR examination and treatment.

Discordance between 2D and 4D flow in the assessment of pulmonary regurgitation severity: a right ventricular remodeling follow-up study.

OBJECTIVES: Pulmonary regurgitation (PR) is common in adult congenital heart disease (ACHD). 2D phase contrast MRI is the reference method for the quantification of PR and helps in the decision of pulmonary valve replacement (PVR). 4D flow MRI can be an alternative method to estimate PR but more validation is still needed. Our purpose was to compare 2D and 4D flow in PR quantification using the degree of right ventricular remodeling after PVR as the reference standard. METHODS: In 30 adult patients with a pulmonary valve disease recruited between 2015 and 2018, PR was assessed using both 2D and 4D flow. Based on the clinical standard of care, 22 underwent PVR. The pre PVR estimate of PR was compared using the post-operative decrease in right ventricle end-diastolic volume on follow-up exam as reference. RESULTS: In the overall cohort, regurgitant volume (Rvol) and regurgitant fraction (RF) of PR measured by 2D and 4D flow were well correlated but with moderate agreement in the overall cohort (r = 0.90, mean diff. -14 ± 12.5 mL; and r = 0.72, mean diff. -15 ± 13%; all p < 0.0001). Correlations between Rvol estimates and right ventricle end-diastolic volume decrease after PVR was higher with 4D flow (r = 0.80, p < 0.0001) than with 2D flow (r = 0.72, p < 0.0001). CONCLUSIONS: In ACHD, PR quantification from 4D flow better predicts post-PVR right ventricle remodeling than that from 2D flow. Further studies are needed to evaluate the added value of this 4D flow quantification for guiding replacement decision. KEY POINTS: • Using 4D flow MRI allows a better quantification of pulmonary regurgitation in adult congenital heart disease than 2D flow when taking right ventricle remodeling after pulmonary valve replacement as a reference. • A plane positioned perpendicular to the ejected flow volume as allowed by 4D flow provides better results to estimate pulmonary regurgitation.

Biventricular Pressure-Volume Loop Assessment Before and After Pulmonary Valve Replacement in Tetralogy of Fallot.

Patients with tetralogy of Fallot (TOF) may undergo pulmonary valve replacement (PVR) after initial full repair. We investigated indices of biventricular function, work and efficiency of TOF patients' using noninvasive pressure-volume (PV) loop analysis on cardiovascular magnetic resonance (CMR) images and compared pre-and post PVR groups. Biventricular segmentations of steady state free precession CMR images were performed using custom validated software (Segment version 2.0 R7067). Brachial cuff pressure estimated left ventricular (LV) systolic pressure. Right ventricular (RV) inputs were obtained from pre-PVR cardiac catheterization data. Biventricular PV loops were then derived using a time-varying elastance model. Twenty seven patients were studied: (22 pre-PVR, 5 post-PVR), mean age of 20±10.5 years and 83% male. RV stroke volume significantly differed before and after PVR (73.2±25 ml vs. 41±10 mL, P =0.01). RV stroke work (SW) and mean external power (MEP) were significantly less post-PVR, but there were no significant differences in the LV hemodynamic indices. TOF patients have reduced RV SW and MEP post-PVR suggesting improved hemodynamics. Noninvasive biventricular PV loop analysis shows potential for integration into standard CMR imaging of TOF and provides hemodynamic data that could influence management decisions.

Eficacia y seguridad de la prótesis valvular pulmonar percutánea para el tratamiento de pacientes pediátricos con antecedente de malformación congénita e historia de cirugía reconstructiva del tracto de salida del ventrículo derecho que presentan regurgitación valvular pulmonar / Efficacy and safety of percutaneous pulmonary valve prosthesis for the treatment of pediatric patients with a history of congenital malformation and history of reconstructive surgery of the right ventricular outflow tract who present pulmonary valve regurgitation

ANTECEDENTES: En el marco de la metodología ad hoc para evaluar solicitudes de tecnologías sanitarias, aprobada mediante Resolución de Instituto de Evaluación de Tecnologías en Salud e Investigación N° 111-IETSI-ESSALUD-2021, se ha elaborado el presente dictamen, el cual expone la evaluación de la eficacia y seguridad de la prótesis valvular pulmonar percutánea (PVPP) para el tratamiento pacientes pediátricos con antecedente de malformación congénita e historia de cirugía reconstructiva del tracto de salida del ventrículo derecho (TSVD) que presentan regurgitación valvular pulmonar. De este modo, el Dr. Miguel Arboleda Torres, siguiendo la Directiva N° 001-IETSI-ESSALUD-2018, envió al Instituto de Evaluación de Tecnologías en Salud e Investigación (IETSI) la solicitud de aprobación del dispositivo médico PVPP. ASPECTOS GENERALES: Aproximadamente un 10 % de pacientes con malformaciones cardiacas congénitas poseen alteraciones anatómicas que obstruyen el tracto de salida del ventrículo derecho (TSVD), el cual conecta el ventrículo derecho del corazón y la arteria pulmonar. La malformación congénita más común que afecta al TSVD es la tetralogía de Fallot y se estima que el 90 % de los pacientes con esta patología son sometidos a una cirugía a corazón abierto durante la infancia temprana para reconstruir el TSVD (que se encuentra obstruido o estrecho [estenótice. Por lo general, la reconstrucción se realiza mediante la colocación de un parche, una prótesis o un conducto entre el ventrículo derecho y la arteria pulmonar. No obstante, con el transcurrir de los años, estos pacientes desarrollan anormalidades de la válvula pulmonar, lo que requiere que se sometan a nuevas intervenciones quirúrgicas para reemplazarla por una prótesis valvular (Alkashkari et al., 2018; Gutiérrez-Larraya et al., 2021, p.). METODOLOGÍA: Se llevó a cabo una búsqueda bibliográfica exhaustiva con el objetivo de identificar la mejor evidencia sobre la eficacia y seguridad del reemplazo valvular pulmonar percutáneo empleando el dispositivo PVPP. La búsqueda bibliográfica se realizó en las bases de datos PubMed, The Cochrane Library y LILACS. Asimismo, se realizó una búsqueda manual dentro de las páginas web pertenecientes a grupos que realizan evaluación de tecnologías sanitarias (ETS) y guías de práctica clínica (GPC) incluyendo el National Institute for Health and Care Excellence (NICE), Canadian Agency for Drugs and Technologies in Health (CADTH), Scottish Medicines Consortium (SMC), Scottish Intercollegiate Guidelines Network (SIGN), Institute for Clinical and Economic Review (ICER), El Instituto de Calidad y Eficiencia en la Atención de la Salud (IQWiG, por sus siglas en alemán), la Base Regional de Informes de Evaluación de Tecnologías en Salud de las Américas (BRISA), la OMS, el Ministerio de Salud del Perú (MINSA) y el Instituto de Evaluación de Tecnologías en Salud e Investigación (IETSI). Además, se realizó una búsqueda de GPC de las principales sociedades o instituciones especializadas en cardiología y cirugía torácica, tales como: American Heart Association (AHA), American College of Cardiology (ACC), European Society of Cardiology (ESC), European Association for Cardio-Thoracic Surgery (EACTS), y la Sociedad Española de Cardiología. Finalmente, se realizó una búsqueda en ClinicalTrials.govpara identificar ensayos clínicos aleatorizados (ECA) en curso o que no hayan sido publicados aún. RESULTADOS: Luego de la búsqueda bibliográfica hasta noviembre del 2021, no se identificaron ECA que compararan las tecnologías de interés. Además, tampoco se encontraron guías de práctica clínica (GPC), evaluaciones de tecnología sanitaria (ETS), revisiones sistemáticas (RS), ni estudios realizados en población pediátrica exclusivamente. En consecuencia, se consideró la inclusión de evidencia científica con una población mixta (adultos y niños) que respondiera a la pregunta PICO. Así, se incluyeron dos GPC (Baumgartner et al., 2021; National Institute for Health and Care Excellence, 2013), dos revisiones sistemáticas (Ribeiro et al., 2020; Zhou et al., 2019), y dos estudios observacionales comparativos que no se encontraban incluidos en ninguna de las dos RS previas (Hribernik et al., 2021; OuYang et al., 2020). CONCLUSIÓN: Por lo expuesto, el IETSI aprueba el uso de prótesis de válvula pulmonar de inserción percutánea en pacientes pediátricos con antecedente de malformación cardiaca congénita e historia de cirugía reconstructiva del tracto de salida del ventrículo derecho que presenten una regurgitación valvular pulmonar moderada a severa, según lo establecido en el Anexo N° 1. La vigencia del presente dictamen preliminar es de un año a partir de la fecha de publicación y la continuación de dicha aprobación estará sujeta a la evaluación de los resultados obtenidos y de mayor evidencia que pueda surgir en el tiempo.

The Safety and Efficacy of Transcatheter Pulmonary Valve Replacement Combined with Electrophysiology Procedures.

The objective of this study was to evaluate the safety and efficacy of combining transcatheter pulmonary valve replacement (TPVR) and electrophysiology (EP) procedures. A retrospective review was undertaken to identify TPVR and EP procedures that were concomitantly performed in the cardiac catheterization laboratory at University of Iowa Stead Family Children's Hospital from January 2011 to October 2019. Procedural and follow-up data were compared between patients who underwent TPVR and EP procedures in the same setting to those who received TPVR or EP procedure separately and that were similar in age and cardiac anatomy. A total of 8 patients underwent combined TPVR and EP procedures. One patient was excluded due to lack of adequate control, leaving seven study subjects (57% female; median age at time of procedure 16 years). The median follow-up time was 11.5 months (range 2-36 months). Patients who received combined TPVR and EP had shorter recovery times (combined: median 18.9 h; IQR 18.35-19.5 vs separate: median 27.98 h; IQR 21.42-39.25; p-value 0.031), shorter hospital length of stay (combined: median 27.5 h; IQR 26.47-31.4 vs separate: median 38.4 h; IQR 33.42-51.50; p-value 0.016), and a 51% reduction in total hospital charges (combined: median $171,640; IQR 135.43-219.22 vs separate: median $333,560 IQR 263.20-400.98; p-value 0.016). There were no significant differences in radiation dose or procedure time between the combined and control groups. The median radiation time for those who had the combination procedure was 30.5 min [IQR 29.6-47.9], and the median dose area product was 215 mGy [IQR 158-935]. In conclusion, combining TPVR and EP procedures is feasible, safe, and economically advantageous.

Assessment of Right Ventricular-Pulmonary Arterial Coupling in Chronic Pulmonary Regurgitation.

BACKGROUND: We hypothesized that noninvasively measured right ventricular (RV) to pulmonary arterial (RV-PA) coupling would be abnormal in chronic pulmonary regurgitation (PR) even in the setting of normal RV ejection fraction, and that RV-PA coupling indices would have a better correlation with peak oxygen consumption (VO2) compared with RV systolic indices alone. METHODS: This was a retrospective study of 129 adults (repaired tetralogy of Fallot [TOF] n = 84 and valvular pulmonic stenosis [VPS] with previous intervention n = 45) with ≥ moderate native PR and RV ejection fraction > 50%. The 84 TOF patients were propensity matched with 84 patients with normal echocardiogram (control); age 28 ± 7 years and male sex n = 39 (46%). RV-PA coupling was measured according to fractional area change (FAC)/RV systolic pressure (RVSP) and tricuspid annular plane systolic excursion (TAPSE)/RVSP. RESULTS: RV systolic function indices were similar between TOF and control groups (FAC 43 ± 6% vs 41 ± 5% [P = 0.164] and TAPSE 22 ± 5 mm vs 24 ± 6 mm [P = 0.263]). However, RV-PA coupling was lower in the TOF group (FAC/RVSP 1.10 ± 0.29 vs 1.48 ± 0.22 [P < 0.001]; TAPSE/RVSP 0.51 ± 0.15 vs 0.78 ± 0.11 [P < 0.001]) because of higher RV afterload (RVSP 42 ± 3 mm Hg vs 31 ± 3 mm Hg [P = 0.012]). FAC/RVSP (r = 0.61; P < 0.001) and TAPSE/RVSP (r = 0.69; P < 0.001) correlated with peak VO2 especially in the patients with impaired exercise capacity whereas FAC and TAPSE were independent of peak VO2. Similar comparisons between VPS and control groups showed no difference in TAPSE and FAC between groups, but lower FAC/RVSP and TAPSE/RVSP in the VPS group. CONCLUSIONS: There is abnormal RV-PA coupling in chronic PR, and noninvasively measured RV-PA coupling might potentially be prognostic because of its correlation with exercise capacity.

3D-assessment of RVOT dimensions prior percutaneous pulmonary valve implantation: comparison of contrast-enhanced magnetic resonance angiography versus 3D steady-state free precession sequence.

To compare contrast-enhanced magnetic resonance angiography (ceMRA) and 3D steady-state free precession (SSFP) during systole and diastole for assessment of the right ventricle outflow tract (RVOT) in patients considered for percutaneous pulmonary valve implantation (PPVI) after tetralogy of Fallot (TOF) repair. We retrospectively evaluated 89 patients (male: 45, mean age 19 ± 8 years), who underwent cardiac-MRI after surgical TOF-repair. Datasets covering the whole heart in systole and diastole were acquired using ECG-gated 3D SSFP and non-gated ceMRA. Measurements were performed in SSFP-sequences and in ceMRA in the narrowest region of the RVOT to obtain the minimum, maximum and effective diameter. Invasive balloon sizing as the gold standard was available in 12 patients. The minimum diameter in diastolic SSFP, systolic SSFP and ceMRA were 21.4 mm (± 6.1 mm), 22.6 mm (± 6.2 mm) and 22.6 mm (± 6.0 mm), respectively. Maximum diameter was 29.9 mm (± 9.5 mm), 30.0 mm (± 7.0 mm) and 28.8 mm (± 8.1 mm) respectively. The effective diameter was 23.2 mm (± 5.7 mm), 27.4 mm (± 6.7 mm) and 24.4 mm (± 6.2 mm), differing significantly between diastole and systole (p < 0.0001). Measurements in ECG-gated SSFP showed a better inter- and intraobserver variability compared to measurements in non-ECG-gated ceMRA. Comparing invasive balloon sizing with our analysis, we found the highest correlation coefficients for the maximum and effective diameter measured in systolic SSFP (R = 0.99 respectively). ECG-gated 3D SSFP enables the identification and characterization of a potential landing zone for PPVI. The maximum and effective systolic diameter allow precise sizing for PPVI. Patients with TOF-repair could benefit from cardiac MRI before PPVI.

Percutaneous Versus Surgical Pulmonic Valve Implantation for Right Ventricular Outflow Tract Dysfunction.

BACKGROUND/PURPOSE: Percutaneous pulmonic valve implantation (PPVI) is an alternative treatment strategy to surgical pulmonic valve implantation (SPVI) for right ventricular outflow tract (RVOT) dysfunction. This study sought to compare outcomes of both treatment strategies. METHODS: The study population was extracted from the 2014 Nationwide Readmissions Database (NRD) using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes for PPVI and SPVI. Study outcomes included all-cause in-hospital mortality, length of index hospital stay (LOS), post-procedural bleeding, mechanical complications of heart valve prosthesis, vascular complications (VC), infective endocarditis (IE), total hospitalization charges, and 30-day readmission rates. RESULTS: A total of 975 patient discharges (176 in PPVI and 799 in SPVI group) were identified (average age 25.7 years; 57.5% male). PPVI was associated with significantly shorter median LOS (1 versus 5 days, p < 0.01), lower risk of bleeding (4.6% versus 26.4%, p < 0.01), and lower total hospitalization charges ($169,551.7 versus $210,681.8, p = 0.02). There was no significant difference between both groups in terms of all-cause in-hospital mortality (0% versus 1.4%, p = 0.12), mechanical complications of heart valve prosthesis (1.7% versus 2.0%, p = 0.78), VC (2.3% versus 2.0%, p = 0.82), IE (1.7% versus 3.1%, p = 0.31), or 30-day readmission rates (4.4% versus 7.6%, p = 0.16). CONCLUSION: Compared with SPVI, PPVI was associated with shorter LOS, lower bleeding, and lower total charges. There was no significant difference between the two strategies in terms of all-cause in-hospital mortality, mechanical complications of heart valve prosthesis, VC, IE, or 30-day readmission rates.

Echocardiographic MRI: an innovative fusion of functional and anatomic assessment strategy for CHD.

We present a pilot case using an innovative fusion of echocardiogram and MRI achieved with a MATLAB-based imaging programme to explore the feasibility of this imaging strategy in the functional and anatomic assessment of a patient with repaired tetralogy of Fallot requiring pulmonary valve intervention. Echocardiogram and MRI neutralises the disadvantages and limitations of each individual imaging modality and yields important anatomic and haemodynamic information crucial to the treatment decision-making process. Future image fusion strategies can apply to three-dimensional images and image-directed therapy for CHD.

Transcatheter pulmonary valve implantation: valve technology and procedural outcome.

PURPOSE OF REVIEW: Procedural technique and short-term outcomes of transcatheter pulmonary valve implantation (TPVI) have been widely described. The purpose of this article is to provide an update on current valve technology, and to focus on recent data surrounding TPVI in the dilated right ventricular outflow tract (RVOT), hybrid interventions, significant outcomes, and procedural costs. RECENT FINDINGS: Transcatheter valve technology has expanded with current trials evaluating self-expandable valves that can be implanted in dilated RVOTs. Until those valves are widely available, hybrid techniques have been shown to offer a potential alternative in these patients, as well as in patients of small size. Although medium-term results of TPVI have shown 5-year freedom from reintervention or replacement of 76%, new data have underlined some concerns relating to bacterial endocarditis after the procedure. Procedural costs remain a concern, but vary greatly between institutions and healthcare systems. SUMMARY: TPVI has emerged as one of the most innovative procedures in the treatment of patients with dysfunctional RVOT and pulmonary valves. Further device development is likely to expand the procedure to patients of smaller size and with complex, dilated RVOTs.

In-hospital cost comparison between percutaneous pulmonary valve implantation and surgery.

Objectives: Today, both surgical and percutaneous techniques are available for pulmonary valve implantation in patients with right ventricle outflow tract obstruction or insufficiency. In this controlled, non-randomized study the hospital costs per patient of the two treatment options were identified and compared. Methods: During the period of June 2011 until October 2014 cost data in 20 patients treated with the percutaneous technique and 14 patients treated with open surgery were consecutively included. Two methods for cost analysis were used, a retrospective average cost estimate (overhead costs) and a direct prospective detailed cost acquisition related to each individual patient (patient-specific costs). Results: The equipment cost, particularly the stents and valve itself was by far the main cost-driving factor in the percutaneous pulmonary valve group, representing 96% of the direct costs, whereas in the open surgery group the main costs derived from the postoperative care and particularly the stay in the intensive care department. The device-related cost in this group represented 13.5% of the direct costs. Length-of-stay-related costs in the percutaneous group were mean $3885 (1618) and mean $17 848 (5060) in the open surgery group. The difference in postoperative stay between the groups was statistically significant ( P ≤ 0.001). Conclusions: Given the high postoperative cost in open surgery, the percutaneous procedure could be cost saving even with a device cost of more than five times the cost of the surgical device.

Short-Term Costs and Hospitalization Rates in Patients With Adult Congenital Heart Disease After Pulmonic Valve Replacement.

In the adult congenital heart disease (ACHD) population, pulmonary valve replacement (PVR) is a common intervention, its benefit, however, has been incompletely investigated. This study investigates short- and intermediate-term outcomes after PVR in ACHD. Using State Inpatient Databases from the Healthcare Cost and Utilization Project, we investigated both hospitalization rate and financial burden accrued over the 12-month period after PVR compared with the 12 months before. Among 202 patients who underwent PVR, per patient-year hospitalization rates doubled in the year after PVR compared with the year before (0.16 vs 0.36, p = 0.006). With the exception of postprocedural complications, the most common reasons for hospitalization were unchanged after surgery: 22% of patients were admitted with equal or greater frequency after PVR. These patients experienced higher inpatient costs both at index admission and in the year after PVR (p = 0.004 and p <0.001, respectively). Univariate predictors of increased hospitalizations after PVR were age ≥50 years (p = 0.016), transposition of the great arteries, or conotruncal abnormalities (p <0.001), lipid disorders (p = 0.025), hypertension (p = 0.033), and number of chronic conditions ≥4 (p = 0.004). Multivariate analysis identified transposition of the great arteries or conotruncal abnormalities as an independent risk factor for increased hospitalization and cost post-PVR (p ≤0.001). In conclusion, short-term costs and hospitalization rates increase after PVR in a small group of patients with ACHD.

Sequential Right and Left Ventricular Assessment in Posttetralogy of Fallot Patients with Significant Pulmonary Regurgitation.

BACKGROUND: The natural history of right ventricular (RV) and left ventricular (LV) size and function among adults with tetralogy of Fallot (TOF) repair and hemodynamically significant pulmonary regurgitation (PR) is not known. The main aim of this study was to determine changes in RV and LV size and function over time in an adult population with TOF repair and hemodynamically significant pulmonary regurgitation. METHODS: Forty patients with repaired TOF and hemodynamically significant PR were included. These patients were identified on the basis of having more than one CMR between January 2008 and 2015. Patients with a prosthetic pulmonary valve or any cardiac intervention between CMR studies were excluded. Rate of progression (ROP) of RV dilation was determined for both indexed right ventricular end-systolic volume (RVESVi) and indexed right ventricular end-diastolic volume (RVEDVi), and calculated as the difference between the last and first volumes divided by the number of years between CMR#1 and CMR#2. Subjects were also divided into two groups based on the distribution of the ROP of RV dilation: Group I-rapid ROP (>50th percentile) and Group II-slower ROP (≤50th percentile). RESULTS: The interval between CMR#1 and CMR#2 was 3.9 ± 1.7 years (range 1-8 years). We did find a significant change in RVEDVi and RVESVi over this time period, although the magnitude of change was small. Nine patients (23%) had a reduction in right ventricular ejection fraction (RVEF) by greater than 5%, 13 patients (33%) had an increase in RVEDVi by greater than 10 mL/m2 and seven patients (18%) had an increase in RVESVi by greater than 10 mL/m2 . Median ROP for RVEDVi was 1.8 (range -10.4 to 21.8) mL/(m2 year); RVESVi 1.1 (range -5.8 to 24.5) mL/(m2 year) and RVEF -0.5 (range -8 to 4)%/year. Patients with a rapid ROP had significantly larger RV volumes at the time of CMR#1 and lower RVEF as compared to the slow ROP group. There was no overall significant change in LVEDVi, LVESVi, or LVEF over this time period. CONCLUSIONS: We have demonstrated, in a small population of patients with hemodynamically significant PR, that there is a small increase in RV volumes and decrease in RVEF over a mean 4-year period. We believe it to be reasonable practice to perform CMR at least every 4 years in asymptomatic patients with repaired TOF and hemodynamically significant PR. We found that LV volumes and function remained stable during the study period, suggesting that significant progressive LV changes are less likely to occur over a shorter time period. Our results inform a safe standardized approach to monitoring adults with hemodynamically significant PR post TOF repair and assist in planning allocation of this expensive and limited resource.

Pulmonary valve replacement and quality-of-life assessment.

BACKGROUND: Chronic pulmonary regurgitation is common after repair of tetralogy of Fallot. Despite the deleterious effects of chronic pulmonary regurgitation on right ventricular function, many patients with repaired tetralogy of Fallot remain asymptomatic. Health is defined not only by the absence of disease but also by physical, mental, and social wellbeing. We sought to examine the impact of pulmonary valve replacement on quality of life in asymptomatic patients with repaired tetralogy of Fallot and chronic pulmonary regurgitation. METHODS: From January 2009 to December 2012, 25 (18 male) asymptomatic patients (mean age 23.4 ± 7.4 years) who underwent pulmonary valve replacement for significant pulmonary regurgitation were recruited. Cardiac magnetic resonance was performed pre- and postoperatively. Quality of life was assessed using the Chinese version of the SF-36v2 evaluation tool. Demographics, clinical data, magnetic resonance findings, and quality-of-life scores were collected and calculated for comparison. RESULTS: After surgery, the indexed right ventricular end-diastolic volume (193 ± 47.3 vs. 105.6 ± 29.6 mL m(-2), p < 0.001) and indexed right ventricular end-systolic volume (108.5 ± 32.9 vs. 61.1 ± 23 mL m(-2), p < 0.001) decreased significantly. The response rates for pre- and postoperative quality-of-life assessments were 100%. Patients demonstrated improvements in all 8 domains of the SF-36v2 assessment. The physical (46.5 ± 6.6 vs. 49.9 ± 6.4, p = 0.012) and mental (43.7 ± 7.8 vs. 51.9 ± 7.6, p < 0.001) component summary scores increased after surgery. CONCLUSION: Pulmonary valve replacement can improve the quality of life in patients with chronic asymptomatic pulmonary regurgitation.