Residual Right Ventricular Outflow Tract Research Articles

Study of right ventricular outflow tract gradient in immediate postoperative period following intracardiac repair for tetralogy of Fallot

Background: Surgical repair of tetralogy of Fallot (TOF) consists of adequate ventricular septal defect closure and relief of right ventricular outflow tract (RVOT) obstruction to the greatest extent possible. The residual RVOT gradient can be due to dynamic and fixed obstruction, and high gradient is sometimes seen even after a satisfactory RVOT resection often confirmed with intraoperative TransEsophageal Echocardiogram (TEE). Aim: The present study was conducted to study the changes in RVOT residual gradient in the early postoperative period. We analyzed the change in residual gradient by invasive monitoring intraoperatively after separating from cardiopulmonary bypass (CPB) in a case of intracardiac repair (ICR) for TOF and compared the readings 24 h after extubating the patients in intensive care unit (ICU). Materials and Methods: This was an observation study done in the Department of CTVS, Advanced Cardiac Centre, PGIMER, and Chandigarh from February 2018 to March 2019. A total of thirty patients with preoperative diagnosis of TOF were included in the study. After ICR for TOF, postseparation from CPB, RVOT gradient was measured using 23G needle connected to pressure transducer and compared with RVOT gradient measured 24 h postextubating using invasive line kept intraoperatively in pulmonary artery and RVOT. Results: There was a significant decrease in residual RVOT gradient postoperatively in ICU after 24 h of extubating, in comparison to intraoperative postbypass residual RVOT gradient. Postbypass residual RVOT gradient was 11.33 ± 1.39 that decreased to 7.81 ± 1.29 24 h after extubating (P < 0.05). Patients in whom pulmonary valve was preserved had greater postbypass residual RVOT gradient (12.44 ± 1.13) than patients with transannular patch (10.5 ± 0.90). However, both decreased after 24 h of extubating (9 ± 0.7 and 6.9 ± 0.8, respectively). Conclusion: Once satisfactory RVOT resection for fixed obstruction is done and is confirmed using TEE, the residual gradient, if marginally high, can be ignored as residual gradient significantly decreases after extubation and hemodynamic improvement is seen in postoperative period.

Fate of the Right Ventricular Outflow Tract Following Valve-Sparing Repair of Tetralogy of Fallot

Valve-sparing repair (VSR) of tetralogy of Fallot (TOF) tends to result in higher residual right ventricular outflow tract (RVOT) gradients. We evaluated the progression and clinical implications of RVOT gradients following VSR of TOF. Demographic, clinical, and operative data were retrospectively collected from consecutive TOF patients who underwent VSR at our institution between 01/2010 and 06/2021. RVOT gradient, pulmonary valve annulus (PVA) diameter and Boston Z-scores were recorded from serial echocardiograms. Data are presented as median and interquartile range or number and percentage. A total of 156 children (boys 92, 59%) underwent VSR at 6.5 (4.9-8.4) months of age and 6.6 kg (5.6- 7.7) weight. There was 1 (0.6%) operative mortality. The remaining 155 patients were followed for 69.4 months (4-106.2). RVOT gradient was 2.4m/s (1.7-2.9) at discharge. It transiently increased, then declined and stabilized during follow-up. PVA Z-score was -1.7 (-3.1 to 0.5) at discharge and 'grew' to -0.8 (-1.7 to 0.4) at last follow-up. Freedom from RVOT re-intervention was 97%, 94% and 91% at 1, 5 and 10-year follow-up. Among 67 (43%) patients with PVA Z-score < -2, a similar RVOT gradient pattern was observed and freedom from RVOT re-intervention was 97%, 95% and 95% at 1, 5 and 8-year follow-up. Following VSR of TOF, RVOT gradients transiently increase and then fall as PVA growth catches up, resulting in durable intermediate outcomes. Patients with PVA Z-score < -2 demonstrated a similar pattern of hemodynamics in the RVOT and excellent freedom from reintervention.

Long-term Outcomes of Transatrial-Transpulmonary Repair of Tetralogy of Fallot With Anomalous Coronary Arteries

Repair of tetralogy of Fallot (TOF) can be complicated by the presence of an anomalous coronary artery (ACA) crossing the right ventricular outflow tract (RVOT). This study sought to evaluate the late outcomes of a policy of transatrial-transpulmonary repair for this condition. The transatrial-transpulmonary approach was used in 864 consecutive TOF repairs between 1993 and 2018 at a single institution, of which 55 (6%) patients had an ACA. Nineteen (35%,19/55) patients underwent prior palliation. Late survival and freedom from reoperations were compared with the general cohort of 809 patients who underwent complete repair during the same period. Early mortality was 2% (1/55). Median follow-up was 15.6 years. Late mortality was 6% (3/54). Absence of a preoperative diagnosis of ACA was not a risk factor for worse outcomes in terms of late re-interventions, acute coronary syndrome, residual RVOT gradient, and late mortality. Survival was 91% (95% confidence interval [CI]: 77-96%) at 20 years and was comparable to the general TOF cohort (95%, 95% CI: 90-98%, P = 0.12). Actuarial freedom from any re-intervention was 46% (95% CI: 27-62%) at 20 years, which was also comparable to the general cohort (31%, 95% CI: 20-42%, P = 0.19). The presence of an ACA does not appear to affect late survival or re-intervention rates in patients undergoing transatrial-transpulmonary repair of TOF.

Intracardiac Repair in Late Adolescent and Adult Tetralogy of Fallot - Early and Midterm Results from a Tertiary Care Centre.

IntroductionIn developing countries like India, it is common for late presentation of Tetralogy of Fallot (TOF) patients to a hospital as compared to that of developed countries. The objective of this study is to analyze the surgical outcome of TOF patients with age > 15 years.MethodsThis is a retrospective descriptive study of the surgical outcomes of 45 adult patients undergoing correction for TOF. Epidemiology, symptomology, and preoperative evaluation were performed.ResultsMost of the patients were male (33 [73%]). The median age was 21 years. A total of 42 (93.33%) patients had subaortic ventricular septal defect (VSD), while three (6.6%) patients presented with doubly committed VSD. The most common type of right ventricular outflow tract (RVOT) obstruction was combined infundibular and valvular types, accounting for 34 cases (75.5%). Six patients had infundibular RVOT obstruction, while three patients (6.6%) had predominantly valvular pulmonary stenosis. We performed trans-right atrial repair in 33 patients. Right atrium-pulmonary artery approach was used in five patients (11.1%). The most common postoperative complication was right bundle branch block, seen in 14 patients, with a mortality rate of 2% in the early postoperative period. We achieved excellent early and midterm survival results and significant improvement in functions and disease-free quality of life.ConclusionIntracardiac repair in adult TOF can be performed with low mortality, less residual RVOT obstruction, and need for revision of RVOT far less frequent by using the Jhajhria Infundibular Resection Adequacy Assessment technique (JIRAAT) to assess for adequacy of infundibular resection.

Impact of Patient Prosthesis Mismatch on the Outcome of Transcatheter Pulmonic Valve Implantation

Patient prosthesis mismatch (PPM) is an important factor of the outcome in transcatheter aortic valve implantation. However, the impact of PPM in transcatheter pulmonic valve implantation (TPVI) has not been studied. Based on the narrowest valve stent diameters in two views of fluoroscopy, internal geometric orifice area (GOA) of the valve stent was calculated and indexed by body surface area (BSA), deriving iGOA. To define PPM in TPVI, receiver operating characteristics (ROC) curve analysis for iGOA for predicting significant residual right ventricular outflow tract (RVOT) gradient was used to derive the optimal cut-off value of iGOA. Our cohort were divided into 2 groups: PPM versus non-PPM. The clinical data were compared between 2 groups. TPVI was performed using Melody valve in 101 patients. Significant RVOT residual pressure gradient (≥ 15 mmHg) was observed in 31 patients (39.6%). Over a mean follow up periods of 6.9 ± 2.7 years, 22 patients (21.8%) required repeat interventions (16 transcatheter, 11 surgical, and both in 5 patients). Based on the ROC analysis, the best cut-off value of iGOA was 1.25 cm2/m2 (area under the curve 0.873, p < 0.001) to define PPM. PPM was present in 42 patients (42%). On the Kaplan-Meier survival analysis, PPM was associated with the need of repeat intervention (p = 0.02). In conclusion, in TPVI, PPM was a strong predictor for the need of re-intervention. Considering PPM, target diameter of valve stent would depend on the patient body size and should be taken into account for optimal outcome of TPVI.

Contemporary early results of late repair of tetralogy of Fallot in children: a single-centre case series.

To describe the early results of complete repair of tetralogy of Fallot (TOF) in children older than one year of age. We identified all patients older than one year of age who underwent TOF repair between 2007 and 2017. Data collected included type of surgical repair, survival at 30 days after surgery, reintervention within 30 days of surgery, length of stay in the intensive care unit (ICU), length of stay in hospital, and degree of residual right ventricular outflow tract (RVOT) stenosis and pulmonary insufficiency 30 days after surgery. 125 children (median age 4.4 years, interquartile range [IQR] 3&ndash;5.7) underwent complete repair of TOF, with pulmonary valve preservation in 66%, a transannular patch in 19%, and a valved right ventricular-to-pulmonary artery conduit in 15%. All patients survived. There were twelve (9.6%) reinterventions. Median ICU length of stay was 7 days and median hospital length of stay was 12 days. Significant residual RVOT stenosis was present in 7.2% and significant pulmonary insufficiency in 19.2%. Patients with a transannular patch had a significantly higher proportion of significant residual RVOT stenosis and significant pulmonary insufficiency compared with the other types of surgical repair. Compared with the existing literature on complete late repair of TOF in children, this study showed good early results with no mortality, similar rates of reintervention and valve-preserving surgery, higher ICU and hospital length of stay, higher incidence of significant residual RVOT stenosis, and lower incidence of significant pulmonary insufficiency. Repair with a transannular patch was a risk factor for significant residual RVOT stenosis and significant pulmonary insufficiency.

Comparison of right ventricular outflow tract gradient under anesthesia with post-operative gradient in patients undergoing tetralogy of Fallot repair.

Background:Intra-cardiac repair for tetralogy of Fallot has some degree of residual right ventricular outflow tract (RVOT) obstruction. However, the measurement of this gradient intra-operatively might get affected by the depth of anesthesia which is important for the long-term outcome.Aims:The primary aim was to compare intraoperative RVOT gradient post repair under two different anesthetic depths of 1% and 2% end-tidal sevoflurane. The secondary objective was to follow up the changes in RVOT gradient till 1 month postoperatively. Design: Observational study. Setting : Advanced Cardiac Centre of PGIMER, Chandigarh.Methods:Following intracardiac repair, RVOT gradient was measured directly by placing needle into the right ventricle and pulmonary artery at sevoflurane 1%, and subsequently, at 2% end.tidal concentration while maintaining hemodynamic stability. These gradients were also measured using transesophageal echocardiography (TEE) (ClinicalTrials.gov NCT03234582).Results:Twenty-one patients were included in this study that had intra-cardiac repair, of which pulmonary annulus was preserved for 15 cases. Mean RVOT gradients measured invasively and by TEE at end-tidal sevoflurane concentration of 1% and 2% were not significantly different (6.67 ± 4.16 mmHg vs. 6.76 ± 3.82 mmHg, P > 0.05 invasively and 13.01 ± 7.40 mmHg vs. 12.53 ± 7.11 mmHg, P > 0.05 by TEE, respectively). RVOT gradient measured by trans-thoracic echocardiography (TTE) postoperatively at the time of extubation and during follow-up at 1 month showed significant reduction (11.37 ± 6.00 mmHg, P < 0.05 and 9.23 ± 4.92 mmHg, P < 0.01 respectively). Six patients who underwent repair with transannular patch had significant pulmonary regurgitation (PR) following surgery, with no significant change in PR severity or RVOT gradient on increasing anesthetic depth.Conclusions:Postoperative RVOT gradient was not altered by changing depth of anesthesia provided systemic blood pressure was maintained. One month postrepair RVOT gradients were significantly reduced as compared to the intraoperative values.

A Low Residual Pressure Gradient Yields Excellent Long-Term Outcome After Percutaneous Pulmonary Valve Implantation

ObjectivesThe aim of this study was to report long-term outcomes after percutaneous pulmonary valve implantation (PPVI). BackgroundExcellent short- and mid-term results after PPVI for right ventricular outflow tract (RVOT) dysfunction have been reported. Data on long-term results after PPVI are scarce. MethodsAll 226 patients treated with PPVI at a single institution were prospectively enrolled in a database and included in the study. Follow-up information regarding outcomes was collected. Risk-factor analysis for death and reinterventions was performed. ResultsFollow-up data (up to 11 years) were available in 96% of the patients. At the end of the study period, 219 patients (92.8%) still lived with the initially implanted valve. Seven patients died, 2 of them from procedure-related complications. Seventeen patients (7.2%) needed valve replacement surgically (n = 11) or percutaneously (n = 6) for infectious endocarditis (n = 10) or valve degeneration (n = 7). In the remaining patients, the valves retained excellent function, and right ventricular dimensions and exercise capacity improved. A post-interventional RVOT gradient >15 mm Hg was a risk factor for death (hazard ratio: 7.57; 95% confidence interval: 1.26 to 45.38; p = 0.027) and for valve failure (hazard ratio: 3.76; 95% confidence interval: 1.43 to 9.93; p = 0.007). The best outcome was achieved with RVOT pre-stenting and a post-interventional gradient <15 mm Hg, resulting in an estimated event-free survival rate of 88% at 10 years. ConclusionsPatients after PPVI have excellent long-term outcomes. Right ventricular volumes, function, and exercise capacity improve significantly. A residual RVOT gradient <15 mm Hg was associated with the best outcome.

Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review †.

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score “cut-off” in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was −1.7 (0 to −4.9) with a median re-intervention rate of 4.7% (0–36.8%) during a median follow-up of 2.83 years (1.4–15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of −0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores.

Anesthetic management of a teen with MURCS variant with tetralogy of Fallot for vaginoplasty

MRKH (Mayer Rockytonsky Kuster Houster) Syndrome is a rare congenital disorder of reproductive system affecting females (Incidence is 1:4500 newborn girls). There are 2 types of this syndrome. Type 1 involves maldevelopment of only the reproductive system. Type 2 is also known as MURCS syndrome which involves reproductive, cardiovascular systems, renal agenesis, somatic, vertebral and hearing defects. The MURCS variant with congenital heart defects is very rare type. Morbidity and mortality of patients having corrected Tetralogy of Fallot (TOF) depends upon the severity of residual right ventricular outflow tract (RVOT) obstruction and the amount of pulmonary regurgitation. Successful anaesthetic management of such a case is similar to a tightrope walk. Acute kidney injury (AKI) to the single kidney and deranged coagulation profile were additional challenges that we addressed to during the conduct of this case.

4D flow MRI to assess right ventricular outflow tract in patients undergoing transcatheter pulmonary valve replacement for severe pulmonary regurgitation

Magnetic resonance imaging (MRI) is essential to assess right ventricular enlargement and function as well as the severity of pulmonary regurgitation before pulmonary valve replacement. Transcatheter pulmonary valve replacement (TPVR) has emerged as an alternative to surgical replacement but limited by the size of the right ventricular outflow tract (RVOT). The aim of the study was to compare the measurements of native RVOT between different MRI imaging sequences and catheterization findings. Single-center retrospective study of patients who underwent TPVI for severe pulmonary regurgitation assessed by 4D flow MRI, without significant residual RVOT obstruction. Balloon calibration was used as the reference. Nine adult with repaired tetralogy of Fallot and one patient with Ross procedure were included (mean age: 43.9 ± 12.9). One patient had a too large RVOT to achieve a TPVR, 1 patient had TPVR with Edwards XT 29 mm valve, 7 patients had Melody valve implantation procedure. Mean RVOT measured 25.1 ± 3.0 mm by balloon calibration, 25.0 ± 3.2 mm and 22.5 ± 3.0 mm 4D flow MRI, in respectively in systole and diastole, 22.7 ± 2.4 mm in 2D SSFP MRI. Interventional angiograms dimensions were 26.7 ± 3.3 mm and 24.4 ± 1.3 mm respectively in systole and diastole. No statistical differences were found comparing different measurements dimensions. Good correlation was found between balloon calibration and MRI 4D flow in systole ( r = 0.794, P = 0.01), while no correlation existed between balloon calibration, 2D MRI and 4D flow MRI in diastole. Balloon calibration was also well correlated to angiograms measurements ( r = 0.881; P = 0.0002 and r = 0.775; P = 0.024, respectively). 4D flow MRI sequence allowing 3D measurement through the cardiac cycle, appears as an interesting technique to measure RVOT in daily practice and therefore to plan TPVI.

Optimal Timing for Elective Early Primary Repair of Tetralogy of Fallot: Analysis of Intermediate Term Outcomes

We have previously demonstrated that early primary repair of tetralogy of Fallot with pulmonary stenosis (TOF) can be safely performed without increase in hospital resource utilization or compromise to surgical technical performance scores (TPS). We sought to identify the optimal timing for elective early primary repair of TOF with respect to intermediate-term reintervention. Retrospective review of all patients with TOF undergoing elective primary repair between September 2004 and December 2013 was performed. Patients were stratified into reintervention group or no reintervention group. Multivariable Cox regression analysis identified independent predictors of reintervention. Youden's J-index in receiver operating characteristic analysis identified optimal age cutoff predictive of reintervention. Kaplan-Meier analysis with the log-rank test compared reintervention rates stratified by age and TPS. A total of 129 patients with median (interquartile range) age and weight of 78 days (56 to 111) and 5 kg (4.1 to 5.7), respectively, underwent primary repair. After a median (interquartile range) follow-up of 2.3 years (0.1 to 4.6), 18 patients (14%) required a total of 22 reinterventions. Youden's J-index revealed significantly lower risk of intermediate-term reintervention when repaired after 55 days of age (8% for >55 days old versus 31% for ≤55 days of age). Multivariable Cox regression identified age 55 days and younger (hazard ratio [HR] 4.5, 95% confidence interval [CI] 1.6 to 12.8, p= 0.004), valve sparing repair (HR 15.3, 95% CI 1.8 to 128.5, p < 0.001), residual right ventricular outflow tract (RVOT) gradient (HR 1.11, 95% CI 1.1 to 1.2, p < 0.001), and inadequate TPS (HR 21.5, 95% CI 7.4 to 63, p < 0.001) as independent predictors of overall intermediate-term reintervention. Elective repair in patients greater than 55 days of age, irrespective of size of the patient, can be safely performed without any increase in reintervention rates. Both residual peak RVOT gradient and TPS are effective in identifying patients at increased risk of reintervention.

Impact of residual right ventricular outflow tract obstruction on biventricular strain and synchrony in patients after repair of tetralogy of Fallot: a cardiac magnetic resonance feature tracking study.

Residual right ventricular outflow tract (RVOT) obstruction (RVOTO) is considered beneficial in patients after repair of tetralogy of Fallot (TOF) although underlying mechanisms are unknown. We sought to elucidate differences in myocardial strain and dyssynchrony parameters in patients after TOF repair with and without residual RVOTO using cardiovascular magnetic resonance (CMR) feature-tracking (CMR-FT) analysis. Fifty-four patients (mean age 16.4 ± 8.4 years) were assessed by CMR 14.2 ± 7.3 years after repair of TOF. Residual RVOTO on echocardiography was defined as a peak systolic RVOT gradient >25 mmHg and was present in 27 patients (no RVOTO in n = 27 patients). Right ventricular (RV) and left ventricular (LV) strain measurements were performed using CMR-FT software. The two groups were well matched for age at CMR scan, time and type of surgical repair. There was no difference in the degree of pulmonary regurgitation (PR) and RV end-diastolic volume. Patients with RVOTO showed significant higher RV circumferential strain (CS) (P = 0.02) and RV radial strain (RS) (P = 0.02) values, whereas RV longitudinal strain (LS) did not differ between the two groups (P = 0.39). The degree of RVOTO showed a significant correlation with RV-CS (r = 0.37; P = 0.006) and RV-RS (r = 0.30; P = 0.03) while RV-LS was unrelated to RVOTO (r = 0.06; P = 0.68). Significant relationships between RV and LV strain parameters were only found in the RVOTO group. Interventricular dyssynchrony was significantly higher in the group without RVOTO (P = 0.03) while LV-LS (P = 0.03) and LV intraventricular synchrony (P = 0.05) were impaired in the RVOTO group. In patients after TOF repair, residual RVOTO seems to preserve RV strain and results in stronger RV-LV interactions and less interventricular dyssynchrony and may therefore possess an early protective effect on RV remodelling. However, the potential negative impact of residual pulmonary stenosis on LV strain and intraventricular synchrony needs further investigation.

Results of transcatheter pulmonary valvulation in native or patched right ventricular outflow tracts

Although widely accepted worldwide, indications for percutaneous valve replacement are limited to treatment of dysfunction of prosthetic conduits inserted in the right ventricular outflow tract (RVOT). There has been little evaluation of the use of the Melody(®) valve for patched non-circular pulmonary pathways. To evaluate the outcomes of Melody valve insertion in patients with a patched non-circular RVOT. We analysed procedural and outcomes data from 34 patients who underwent Melody valve implantation for a non-circular RVOT. RVOT preparation was done in all patients, using different techniques (conventional, Russian doll and/or PA jailing). Melody valve insertion was performed concomitantly in most patients. All procedures were successful. Sixteen patients had complex additional procedures, including the jailing technique (n=5), the Russian doll technique (n=6) and multiple stent implantations (Russian jailing; n=5). The remaining patients were treated using the conventional technique with systematic prestenting. Three early complications occurred: one haemoptysis; one residual RVOT obstruction needing recatheterization 48 hours after percutaneous pulmonary valve implantation; and one stent embolization during advancement of the Ensemble(®) delivery system. The mean follow-up period was 2.6 years postprocedure. There was no stent fracture, migration or embolization. Two patients developed a significant paraprosthetic leak and one received a second Melody valve. Careful patient selection, balloon sizing and RVOT preparation with prestenting are required to create a safe landing zone for the Melody valve. Short-term follow-up shows excellent results with no stent fracture or migration and appears promising.

Evolving strategies for preserving the pulmonary valve during early repair of tetralogy of Fallot: Mid-term results

The aim of the study was to evaluate our results with pulmonary valve (PV) preservation in selected patients with tetralogy of Fallot (TOF). From January 2007, 69 patients who underwent early transatrial TOF repair were enrolled in the study. The patients were divided into 2 groups: PV preservation by PV annulus balloon dilation (group 1) and PV cusp reconstruction after annular incision (group 2). Thirty-four patients underwent a successful PV annular preservation (49%). Median age at surgery was 113 days (range, 36-521 days) (group-1 vs group-2, P = not significant). Median preoperative PV Z score was -3.15 (range, -0.95 to -5.62) (group-1 vs group-2, P = .03). Median intensive care unit and hospital stays were 3 and 10 days, respectively (group 1 vs group 2, P = not significant). Median follow-up time was 580 days (range, 189-1940 days) (group 1 vs group 2, P = .08). Two patients were reoperated for residual right ventricular outflow tract (RVOT) obstruction (1 in group 1 and 1 in group 2). The remaining patients are alive and well. Median peak RVOT gradient was 25 mm Hg (range, 8-60 mm Hg) (group 1 vs group 2, P = not significant). The degree of PV regurgitation in group 1 was none/mild in 24 patients (80%) and moderate in 6 (20%) and was none/mild in 8 patients (25%), moderate in 11 (34.4%), and severe in 13 (28.6%) in group 2 (P = .001). Median right ventricular fractional area change was 55% (range, 42%-70%) in group 1 and 50% (range, 40%-63%) in group 2 (P = .003). The integrity and function of the PV can be preserved in selected patients during early repair of TOF by concomitant balloon dilation, leading to a better mid-term right ventricular function.

Preservation of right ventricular structure and function following transatrial-transpulmonary repair of tetralogy of Fallot†

Management strategy of patients with tetralogy of Fallot (TOF)-including timing, as well as surgical technique-remains a controversial topic. We sought to analyse both early and late results of our consistent policy of non-neonatal transatrial/transpulmonary (TA/TP) repair of TOF over almost 14 years, in order to assess preservation or possible deterioration of right ventricular (RV) function. All 245 consecutive patients with TOF, referred to our group for repair between September 1997 and December 2010, have been prospectively followed up. Their clinical and echocardiographic data were retrospectively analysed. All underwent complete TA/TP repair at a median age of 1.6 years (range 0.2 to 55.6 years). Follow-up (median 8.5 years, range 0.5 to 14.6 years) was 100% completed. There was no operative death. There were three early re-operations: one for residual right ventricular outflow tract obstruction (RVOTO), one for intractable arrhythmias and one for remote second ventricular septal defect (VSD). There were three non-cardiac-related late deaths. Actuarial survival at 14 years was 98.8% (242/245). Twenty five patients required late re-operation, including 23 patients (9.4%, 23/245) who had pulmonary valve replacement (PVR). All other patients remained asymptomatic at follow-up. Mean residual right ventricular outflow tract (RVOT) pressure gradients have remained stable (6.8 ± 6.6 mmHg, 95% CI 5.9-7.6, early postoperatively, versus 7.6 ± 7.5 mmHg, 95% CI 6.6-8.6 at follow-up; P = 0.015). The mean qualitative grade of pulmonary valve insufficiency (PVI) increased (from 1.4 ± 0.9, 95% CI 1.3-1.5 at discharge to 2.1 ± 1.2, 95% CI 2-2.3 at follow-up; P < 0.001). Similarly, mean qualitative grade of tricuspid valve insufficiency (TVI) also increased (from 1 ± 0.7, 95% CI 0.9-1 at discharge to 1.5 ± 0.8, 95% CI 1.4-1.6 at follow-up; P < 0.001). However, RV function has remained normal in most patients. No significant arrhythmias have been noted. Our management strategy of non-neonatal TA/TP repair for all patients with TOF is associated with minimal early and late mortality and morbidity, relatively low re-operation rate, preserved RV function and excellent clinical outcomes at follow-up over almost 14 years. Since many patients demonstrated progressive increase in PVI and TVI, a much longer follow-up is necessary to determine the ultimate rates of late re-operation for pulmonary valve replacement (PVR).

Obstetric outcomes in women with congenital heart disease and residual right ventricular outflow tract lesions

BackgroundCurrent evidence suggests that the cardiovascular demands of pregnancy increase fetal morbidity in women with residual right ventricular outflow tract (RVOT) lesions, particularly if there are severe maternal haemodynamic residua....

Pregnancy outcome in women with congenital heart disease and residual haemodynamic lesions of the right ventricular outflow tract

To determine pregnancy outcome and risk factors for adverse events in women with congenital heart disease (CHD) and residual haemodynamic right ventricular (RV) outflow tract (RVOT) lesions. Pregnancy outcome data for women with CHD and residual RVOT lesions have been recorded since 2001. There were 76 pregnancies in 47 women that continued beyond 24 weeks gestation. At conception 20% had RVOT obstruction, 32% had pulmonary regurgitation (PR) and 49% had mixed RVOT obstruction and PR. Moderate-to-severe PR was present in 30 (39%) and RVOT obstruction > or =30 mmHg in 12 (16%) of pregnancies. Seven pregnancies (9%) were complicated by right heart failure (RHF). No arrhythmias were documented. Predictors for RHF were moderate-to-severe PR in combination with at least one additional risk factor (twin pregnancy, branch pulmonary artery stenosis, RV systolic dysfunction, RV hypertrophy). All patients responded to diuretic treatment and had a good pregnancy outcome without foetal complications. In patients with CHD and residual RVOT lesions, the outcome of pregnancy is good. Patients with moderate-to-severe PR were at risk for symptomatic RHF only if additional risk factors were present. When treated by a multidisciplinary team, maternal and foetal outcome was good. The general recommendation that pulmonary valve replacement should be undertaken prior to pregnancy in patients with moderate-to-severe PR and RV dilatation needs to be reconsidered.

Determinants of Exercise Capacity After Arterial Switch Operation for Transposition of the Great Arteries

Patients who undergo the arterial switch operation for transposition of the great arteries (TGA) are at risk of reduced exercise capacity, with most reports focusing on chronotropic incompetence as the cause. Residual right ventricular outflow tract (RVOT) obstruction is relatively common after the arterial switch operation, but its effect on exercise capacity is unknown. We studied 60 patients (44 males, age 13.3 +/- 3.4 years) who had undergone a neonatal arterial switch operation using the cardiopulmonary exercise test and transthoracic echocardiography. The peak exercise oxygen uptake (VO(2)), and heart rate were recorded and are expressed as the percentage of predicted values. The greatest velocity detected by echocardiography across the pulmonary valve, pulmonary trunk, or pulmonary branches was used in the analysis as an index of RVOT obstruction. The peak VO(2)% was 84 +/- 15%, and the peak heart rate percentage was 97 +/- 8%. Of the 60 patients, 29 had an abnormal peak VO(2)% (< or =84%) and 3 (5%) had an abnormal peak heart rate percentage (< or =85%). The maximal RVOT velocity was 2.3 +/- 0.6 m/s, and it correlated with the peak VO(2)% (r = -0.392, p = 0.004). On multivariate analysis, the presence of residual RVOT obstruction (p = 0.0007) was the only variable associated with a reduced peak VO(2)%. Patients with a RVOT maximal velocity > or =2.5 m/s had a lower peak VO(2)% than those with lower velocities (p <0.0001). No relation was found between age at testing and the peak VO(2)%. In conclusion, a reduced exercise capacity is relatively common in children and young adults who have undergone an arterial switch operation, but it does not decrease with age. The presence of residual RVOT obstruction seems to have an effect on exercise capacity.

Management of complications after operation for tetralogy of Fallot on infants and follow-up of one year

Objective To review the early postoperative complications and its treatment in infants with tetralogy of Fallot(TOF).Methods From January 2004 to March 2007,35 infant patients with TOF,aged 4 to 12 months,underwent corrective procedure with hypothermic cardiopulmonary bypass(CPB) and general anaesthesia in Anhui provincial children's hospital.Of all the patients,30 cases received radical surgery and the other 5 cases with initial palliation.Results The early postoperative complications occurred in 10 cases,of which 6 cases comlplicated with low cardiac output,2 cases had severe arrhythmia,2 cases manifested as perfusion lung,2 cases were symptomized by residual right ventricular outflow tract(RVOT) obstruction and 1 case suffered from residual ventricular septal defect(VSD).There were 3 cases of early postoperative death with 8.6% of hospital mortality.Conclusion The operative mortality can be effectively reduced by careful preparations for surgery,reliable surgical procedure,effective myocardial protection and timely treatment for postoperative complications.This study demonstrated that repair of TOF in infancy can be done with excellent results. Key words: Tetralogy of Fallot; Infants; Postoperative complications; Follow-up