Severe Pulmonary Regurgitation Late After Total Repair of Tetralogy of Fallot: Surgical Considerations

Background After total repair of tetralogy of Fallot (TOF-R) with transannular patching (TAP), severe pulmonary regurgitation (PR) is reported to develop in up to 30% of patients at a follow-up of 20 years, and 10–15% or more need pulmonary valve replacement (PVR). In this study, time-related progression of PR and right ventricular (RV) dilatation, and functional recovery of the RV after PVR are analyzed, and the possible causes of PR and timing of PVR are discussed.Methods Eighteen patients, who late after TOF-R with TAP underwent PVR for severe PR, were chosen for the study. NYHA class, QRS duration, RV dilatation index (RVDI = RVEDD/LVEDD), and RV–distal pulmonary artery (PA) peak systolic gradient were reviewed and retrospectively analyzed.Results TOF-R was performed at a mean age of 5.1 ± 3.9 years (range: 0.6–12.8 years); the mean time interval from TOF-R to PR grade 3 onset was 11.8 ± 7.0 years (range: 3.3–27.4 years), and from TOF-R to PVR was 18.5 ± 7.8 years (range: 8.7–37.1 years).At PVR, 11 patients were in NYHA class II–III, all patients had severe PR (grade 3/3) and severe RV enlargement, 4 patients had ventricular arrhythmias, 7 patients significant distal pulmonary artery stenosis, and 2 patients small nonrelevant residual VSD. The mean preoperative RVDI (normal: 0.5) was 0.99 ± 0.14 (range: 0.75–1.3), the mean QRS duration 170 ± 24 ms (140–220 ms), and the mean RV–distal PA peak systolic pressure gradient 33.3 ± 19.0 mmHg (range: 10–60 mmHg). Patients aged at TOF-R> 5 years had considerably longer redo-free intervals than their younger counterparts: mean 23.1 years (range 8.7–37.1 years) vs 14.8 years (range: 9.3–21.2 years), respectively. The redo-free intervals and the duration of severe PR correlated inversely with the RV-PA gradient.At a mean follow-up of 1.3 years (2 weeks–5 years), the mean RVDI decreased from 0.99 ± 0.14 to 0.69 ± 0.15, the mean validity class improved from 2.5 to 1.1. One patient died.Conclusions After TOF-R with TAP, the progression of PR has very individual dynamics, resulting in extremely varying redo-free intervals. Concomitant pulmonary stenosis seems to exaggerate progression of PR. PVR results in effective reduction of diastolic dimensions of severely dilated RV and in improvement of validity class. Referred PVR in no-risk cases seems to be justified.Presented at the 32nd Annual Meeting of the GSTCS, Leipzig, Germany, 24 February 2003.     

Valve-Sparing Tetralogy of Fallot Repair With Intraoperative Dilation of the Pulmonary Valve

Deleterious long-term effects of pulmonary regurgitation after tetralogy of Fallot (TOF) repair have become evident during the last two decades. Subsequently, different groups have developed strategies aimed at preserving the pulmonary valve function. However, the results of these approaches are not well known. From July 2009 through March 2012, 38 patients underwent primary repair of TOF at the authors’ institution. Of these, 12 children (7 boys) underwent attempted pulmonary valve-sparing surgery with intraoperative dilation of the pulmonary valve. The technical details as well as the echocardiographic preoperative and follow-up data for this repair were recorded, with a special focus on the feasibility of the technique and the effects on pulmonary valve function. No patient in the series died. At repair, the median age was 6 months (range 3.4–126 months), and the median weight was 7.6 kg (range 4.7–47 kg). Intraoperative dilation of the pulmonary valve was technically feasible for all the patients. Two patients had unsuccessful dilation and underwent a transannular patch procedure. During a median follow-up period of 22 months (range 6–30 months), the pulmonary valve diameter and z-score improved significantly. Moreover, the annular size normalized, whereas the mean right ventricular outflow tract (RVOT) gradient remained at the mild level (median, 24 mmHg; range 12–36 mmHg). At the most recent follow-up evaluation, three patients showed moderate pulmonary regurgitation. Intraoperative dilation of the pulmonary valve in patients undergoing TOF repair is feasible and provides good relief of obstruction. Moreover, the pulmonary valve annulus grows through the follow-up period. Longer follow-up studies are needed to evaluate the exact role of this strategy in this population.     

Hemodynamic and Electrocardiographic Effects of Early Pulmonary Valve Replacement in Pediatric Patients After Transannular Complete Repair of Tetralogy of Fallot

In adults, pulmonary value replacement (PVR) shows improvement in right ventricular (RV) volume and function and reduces QRS duration. In addition, RV volume correlates with QRS duration and QRS change. This has not been shown in pediatric patients. The purpose of this study was to evaluate serial magnetic resonance imaging (MRI) and electrocardiogram measurements before and after early PVR in a pediatric population with repaired Tetralogy of Fallot and whether QRS duration and QRS change correlated with RV volume. A retrospective review of MRIs and electrocardiograms was conducted on 10 patients. Median age at repair was 2.1 ± 0.7 years, and median age at PVR was 11.5 ± 2.0 years. There were significant decreases in RV end diastolic volume (EDV)/body surface area (BSA) (p < 0.0004), end systolic volume (ESV)/BSA (p = 0.02), RVEDV/left ventricular (LV) EDV (p < 0.001), RV ejection fraction (p < 0.04), RV stroke volume (SV)/BSA (p < 0.0002), and (RVSV - LVSV)/BSA (p = 0.0007). No significant change in QRS duration occurred (p = 0.08). QRS duration (pre-r = 0.44, p = 0.20; post-r = 0.34, p = 0.33) and QRS change (r = −0.08, p = 0.83) did not correlate with RVEDV. We propose early consideration of PVR in pediatric patients. PVR improves RV volumes and function and may provide beneficial electromechanical effects by slowing the progression of QRS duration.     

法洛四联症根治术的远期预后

目前法洛四联症根治手术广泛采用经右心室上部小切口跨瓣环补片 ,并趋向于早期进行 ,远期生存率和患病率是远期疗效判断的重要指标。与之密切关联的病理生理改变主要是肺动脉反流 ,右心室扩张和右心功能不全及限制性右心室功能。针对术后几个改变可再行肺动脉瓣置换术 ,手术指证和时机尚无统一标准 ,术后各项临床实验室指标和主观症状均有明显好转 ,远期预后佳。     

Right Ventricular Diastolic Function After Repair of Tetralogy of Fallot

The objective of this study was quantitate diastolic dysfunction in the postoperative phase of tetralogy of Fallot (TOF) and to correlate it with the type of surgical procedure and clinical parameters. Fifty consecutive patients (mean age, 5.0 years; mean weight, 13.5 kg), operated for TOF during the period November 2004 to May 2005, were prospectively studied [infundibular resection, 23; infundibular resection and transannular patch (TAP), 19; right ventricle→pulmonary artery conduit, 8). Detailed echocardiography was done on postoperative days 3 and 9 with a focus on Doppler indices of right ventricular (RV) function, Antegrade late diastolic flow in the right ventricular outflow tract (RVOT) was taken as the marker of restrictive RV physiology. The previous parameters were correlated to the type of surgery and clinical indices of RV dysfunction. There was no mortality. Twenty-four patients showed restrictive RV physiology. This finding correlated with lower values of E/A ratio (0.98 ± 0.17 vs 1.33 ± 0.49, p < 0.002), tricuspid valve E-wave deceleration time (86.9 ± 21.7 vs 151.4 ± 152 msec, p < 0.05), index of myocardial performance (0.15 ± 0.06 vs 0.26 ± 0.09, p < 0.001), isovolumic relaxation time (19.4 ± 17 vs 39±30 msec, p < 0.009), and a higher central venous pressure (15.1 ± 1.5 vs 12.7 ± 1.9, p < 0.001). Restrictive RV physiology correlated with prolonged intensive case unit (ICU) stay (5.1 ± 3.7 vs 2.8 ± 2 days, p < 0.015), longer duration of inotropic support (108.3 ± 56.2 vs 55.5 ± 28.3 hours, p < 0.02), and higher dosage of diuretics. RV diastolic dysfunction is demonstrable by Doppler echocardiography in the first week following surgery for TOF and tends to be worse with TAP. Restrictive physiology demonstrated by RVOT pulse Doppler predicts longer duration of inotropic support, prolonged ICU stay, and higher dosage of diuretics.     

Risk Factors Associated With Morbidity and Mortality After Pulmonary Valve Replacement in Adult Patients With Previously Corrected Tetralogy of Fallot

Patients with palliated tetralogy of Fallot (TOF) often require pulmonary valve replacement in adulthood, yet the data regarding their outcomes are scarce. This study aimed to identify risk factors associated with postoperative complications in these patients and to establish long-term survival data for this patient group. A retrospective cohort study investigated 153 consecutive patients with a history of TOF repair who underwent pulmonary valve replacement at a single large academic center between March 1996 and March 2010. In part 1 of the study, logistic models were constructed to assess demographic, medical, and surgical risk factors for operative mortality; occurrence of a major adverse event (stroke, renal failure, prolonged ventilation, deep sternal infection, reoperation, or operative mortality); and prolonged hospital stay (>7 days). Risk factors with a p value less than 0.10 by univariate analysis were included in the subsequent multivariate analysis. In part 2 of the study, long-term, all-cause mortality was determined by construction of a Kaplan–Meier curve for the cohort. Seven patients died (4.5%). Significant risk factors for mortality in the multivariable analysis included age older than 40 years (odds ratio (OR) 9.89) and concomitant surgery (OR 6.65). A major adverse event occurred for 22 patients (14.4%). The only significant risk factor in the multivariable analysis for an adverse event was concomitant surgery (OR 6.42). The hospital stay was longer than 7 days for 31 patients (20.3%). The significant risk factors for a prolonged hospital stay included the presence of preoperative arrhythmias (OR 4.17), New York Heart Association class 3 (OR 4.35), and again, concomitant surgery (OR 4.2). Among the 146 hospital survivors, only 5 patients died in the intervening period. The predicted survival rates were 98.5% at 1 year, 96.7% at 5 years, and 93.5% at 10 years. Pulmonary valve replacement in adults with palliated TOF is a safe procedure with excellent long-term survival, but there remain important risk factors for postoperative mortality, prolonged hospital stay, and major adverse events. Awareness and modification of important risk factors may help to improve outcomes.     

Biventricular Long Axis Function After Repair of Tetralogy of Fallot

Right ventricular restrictive physiology is common after repair of tetralogy of Fallot and relates to exercise performance and symptomatic arrhythmias. In this study, we examined biventricular long axis function in an attempt to clarify further the mechanical substrate of this phenomenon. We studied prospectively 95 patients with tetralogy of Fallot (age range 1–44.3 years) at a median of 4.3 years after repair with Doppler and M-mode echocardiography. Pulmonary arterial, tricuspid, and mitral Doppler spectrals and 2-D guided M-mode recordings of ventricular minor and long axes were obtained with simultaneous phonocardiogram and respirometer recordings. Right ventricular restriction was defined by the presence of antegrade pulmonary arterial flow during atrial systole throughout the respiratory cycle. Restrictive right ventricular physiology was demonstrated in 36 (39%) [group 1] of the 92 patients in whom the data were analyzed. Left ventricular function (FS, isovolumic relaxation time and transmitral E wave deceleration time) was not different in the two groups (p < 0.1, p < 0.6, and p < 0.8, respectively). The presence of antegrade diastolic flow shortened the pulmonary regurgitation in the restrictive group (PR duration/√RR 10.7 ± 2.1 vs 12.1 ± 2.1, p < 0.01). There was delayed onset of shortening (97.4 ± 24 vs 88.8 ± 24 ms, p= 0.01), and the amplitude of right atrioventricular ring excursion, corrected for body surface area, was significantly lower during atrial systole in the restrictive group (0.43 ± 0.15 vs 0.54 ± 0.2 cm/m², p < 0.01). There was also a tendency toward a smaller ratio of right to left total atrioventricular ring excursion in the same group (1.14 ± 0.19 vs 1.22 ± 0.23, p= 0.1). Impaired long axis function in patients with restrictive right ventricular physiology following repair of tetralogy of Fallot is associated with abnormal diastolic filling and may contribute to the long-term cardioprotective effect of restrictive physiology by limiting the degree of right ventricular dilatation.     

Takotsubo Cardiomyopathy After Transcatheter Edwards Sapien Pulmonary Valve Placement in a Patient With Tetralogy of Fallot

A 34-year-old woman who had history of repaired tetralogy of Fallot with absent pulmonary valve syndrome presented for transcatheter intervention to reestablish pulmonary valve competence. A 26-mm Edwards Sapien valve was implanted, and coronary angiography after implantation demonstrated no coronary compression. Hemodynamically stable ventricular tachycardia was documented after the procedure. A 12-lead electrocardiogram showed ST segment elevation, and cardiac enzymes were moderately elevated. An echocardiogram demonstrated the stable position of the new pulmonary valve, the marked circumferential hypokinesia of the mid-to-apical left ventricular myocardium, and the normally contracting basal-to-mid myocardial segments. No intervention was required. An echocardiogram obtained 4 days after discharge showed qualitatively improved left ventricular systolic function, particularly in the apical region.     

Tetralogy of Fallot with Aortic Stenosis: Repair Without Aortic Valve Intervention

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.     

Balloon Dilation of the Pulmonary Valve in Premature Infants with Tetralogy of Fallot

Infants with tetralogy of Fallot (ToF) presenting with desaturation may require augmentation of the pulmonary blood flow, usually in the form of a Blalock-Taussig shunt. Shunts may result in a preferential increase in blood flow to one lung. They also may be associated with significant morbidity and possibly mortality of premature infants. Balloon dilation of the pulmonary valve is reported to improve saturation in early infancy. This report describes two premature infants (weighing, respectively, 1.8 and 1.6 kg) with ToF and desaturation for whom balloon dilation of the pulmonary valve showed good results. Neither infant required palliative surgery. At this writing, one infant has already undergone complete repair.     

Course of Right and Left Ventricular Function in Patients with Pulmonary Insufficiency After Repair of Tetralogy of Fallot

Surgical repair of tetralogy of Fallot (TOF) frequently results in pulmonary valve insufficiency. Nevertheless, no serial information is available on the long-term impact of the valvular insufficiency on right and left ventricular function. Right and left ventricular ejection fraction was measured serially by radionuclide angiocardiography in 21 patients with at least moderate pulmonary insufficiency after repair of TOF. A baseline study was obtained an average of 1.2 years after repair, and a follow-up study was performed an average of 10.2 years after surgery. Changes in ventricular function over time and deviations from the normal range were analyzed. At baseline evaluation the mean right ventricular ejection fraction (RVEF; 0.52 ± 0.10) and left ventricular ejection fraction (LVEF; 0.68 ± 0.10) were normal. At the time of follow-up the mean RVEF had significantly decreased to 0.45 ± 0.09 (p < 0.01). The mean LVEF had decreased to 0.60 ± 0.11 (p < 0.02). This change was independent of the RVEF (r=−0.13). Eleven patients (52%) had an abnormal RVEF or LVEF at follow-up. Nineteen patients (90%) showed a decrease of 0.05 or more in RVEF, LVEF, or both between studies. These data suggest a negative impact of long-standing pulmonary insufficiency on right and left ventricular systolic function after repair of TOF. Therefore, continued surveillance of biventricular function in this patient population appears warranted.     

Exercise Capacity in Young Patients After Total Repair of Tetralogy of Fallot

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. Measurement of physical activity is usually performed as a routine part of the patient's cardiac evaluation. The aim of this study was to examine the exercise performance of young patients operated on for tetralogy of Fallot, assessing the possible influence of known negative prognostic factors related to the surgical repair. The study group comprised 41 consecutive patients (29 male and 12 female, ages 11.2 ± 3.9 years, range 6–16 years) operated on for tetralogy of Fallot. Patients in the study group were divided in subgroups in relation to the age of surgical intervention (before or after 2 years of life), the surgical approach (combined transatrial/transpulmonary approach or right ventriculotomy), and the presence of aortopulmonary shunts prior to performing total correction. Their data were compared with those of 33 aged-matched asymptomatic control subjects (19 male and 14 female, ages 11.9 ± 1.3 years, range 11–16 years). Blood pressure and heart rate measured at rest were similar between control and Fallot groups. A normal increase in systolic blood pressure was observed in response to exercise intensity for all subgroups. No significant difference between control and Fallot groups was found under conditions of mild or moderate exercise or for diastolic blood pressure at rest and in response to exercise. Lower maximal heart rate and systolic blood pressure values were recorded in all patients when compared with the control subjects. Significant differences in peak workload were detected between control and Fallot groups and between the control and each subgroup; however, no difference was found between subgroups. In conclusion, despite their very satisfactory clinical status, all patients showed a reduced peak workload, irrespective of the surgical approach, age at surgery, and aortopulmonary shunts prior to performing total correction.     

Tei Index Determined by Tissue Doppler Imaging in Patients with Pulmonary Regurgitation After Repair of Tetralogy of Fallot

The myocardial performance index (Tei index) determined by the pulsed Doppler method is a simple and noninvasive measurement for assessing global right ventricular (RV) function. This index can also be obtained by tissue Doppler imaging (TDI). The effects of significant pulmonary regurgitation (PR) on the determination of the Tei index by these two methods have not been investigated. We examined 15 patients (6.3 ± 2.2 years) with significant PR after repair of tetralogy of Fallot (TOF) and 24 age-matched healthy children. Myocardial wall motion velocities at the tricuspid annulus were assessed during systole (Sa), early diastole (Ea), and late diastole (Aa) from a four-chamber view. Pulsed Doppler–Tei index and TDI–Tei index were measured as reported previously. The Tei index obtained by the pulsed Doppler method in TOF patients did not differ from that in normal children (0.30 ± 0. 12 vs 0.32 ± 0.07, p = not significant). TDI showed that TOF patients had significantly decreased Ea, Aa, and Sa velocities compared to those of normal children. Both isovolumic contraction time and isovolumic relaxation time in TOF patients were significantly longer than those in normal children (88 ± 18 vs 62 ± 23 msec and 46 ± 11 vs 21 ± 12 msec, respectively; p < 0.0001), although the duration of Sa did not differ between the two groups. Consequently, the Tei index as measured by TDI was significantly greater in TOF patients than in normal children (0.48 ± 0.07 vs 0.30 ± 0.07, p < 0.0001). The Tei index measured by the pulsed Doppler method is limited in its ability to assess RV function in patients with significant PR. However, the Tei index determined by TDI is a sensitive indicator of RV function in these patients, and it appears to be a promising new means of assessing global RV function in patients with significant PR.     

Aiming to Preserve Pulmonary Valve Function in Tetralogy of Fallot Repair: Comparing a New Approach to Traditional Management

Pulmonary valve (PV) incompetence following transannular patch (TAP) repair of tetralogy of Fallot (TOF) results in long-term morbidity and mortality. Valve-sparing repairs have recently gained recognition; however, they may be associated with residual pulmonary stenosis (PS) in patients with small PV z scores. We sought to determine whether a repair that increases the PV annulus and augments the valve leaflet with a biomaterial would result in annular growth and in longer duration of valve competence compared with TAP. Eighty patients (median age 136 days, range 4–350) who underwent surgical repair of TOF between 2010 and 2014 were included in the study. Patients were divided into three groups based on the PV intervention: balloon dilation/valvotomy (n = 29), valve-sparing transannular repair (VSTAR) (n = 19) and TAP (n = 32). Intraoperative, early postoperative and midterm follow-up echocardiographic data (median 19 months, range 1–59) were obtained. The primary outcomes were the presence and severity of pulmonary regurgitation and/or PS. Compared with TAP, VSTAR patients demonstrated significantly less severe PR with 100 % freedom of severe PR immediately post-op (vs. 0 % in TAP), 60 % at 6 months and 20 % at 20 months. There were no differences in PS between VSTAR and TAP at follow-up. A subgroup analysis of the VSTAR group was performed. PV z scores were calculated and fit to a random effects model. Patient data fit the model closely, predicting a reproducible increase in valve annulus size over time. With better short-term and comparable midterm results, VSTAR may be appropriate for TOF repair in patients with small PV that would conventionally require a TAP.     

Clinical Applications of Cardiac Magnetic Resonance Imaging After Repair of Tetralogy of Fallot

In the past 15 years, cardiovascular magnetic resonance (MR) has evolved into an imaging technique that provides adequate, and in part unique, information on residual problems in the follow-up of patients operated for tetralogy of Fallot. Spin-echo or gradient-echo cine magnetic resonance imaging allow detailed assessment of intracardiac and large vessel anatomy, which is particularly helpful in Fallot patients with residual abnormalities of right ventricular outflow and/or pulmonary artery. Multisection gradient-echo cine MRI can be used to obtain accurate measurements of biventricular size, ejection fraction, and wall mass. This allows serial follow-up of biventricular function. MR velocity mapping is the only imaging technique available that provides practical quantification of pulmonary regurgitation volume. MR velocity mapping can also be used to quantify right ventricular diastolic function in the presence of pulmonary regurgitation.     

Histopathologic Changes in the Infundibular Septum After Balloon Pulmonary Valvuloplasty in Tetralogy of Fallot

Balloon pulmonary valvuloplasty (BPV) has been applied to tetralogy of Fallot (TOF) as a palliative procedure. To investigate the histopathologic changes by BPV in the infundibular septum of TOF, we performed histopathologic examinations of the infundibular septum resected at corrective surgery. The subjects were 5 patients with TOF, who underwent BPV at the median age of 2.2 months and the corrective surgery at the median age of 15.0 months (BPV group), and 4 patients with TOF who had no prior BPV and who underwent the corrective surgery at the median age of 14.5 months (control group). There was no significant difference between the two groups in the endocardial thickness, myocardial vacuole degeneration, or fraction of fibrous and interstitial space. However, the specimens from 3 patients in the BPV group had localized dense fibrous lesions in the myocardium. The findings in this limited sample suggest that BPV for TOF does not produce overall histopathologic alterations such as fibrosis, thick endocardium, and myocardial vacuole degeneration, but may sometimes damage the myocardium of the infundibular septum resulting in the formation of localized dense fibrous lesions. The clinical significance of this damage is still unknown, and further cases should be investigated.     

Transcatheter Repair of Partial Anomalous Pulmonary Venous Drainage Using an Amplatzer Vascular Plug in a Postoperative Patient With Tetralogy of Fallot

The association of tetralogy of Fallot with a partial anomalous pulmonary vein is rare. Although this combination is generally treated with surgery, in this paper we present the case of an 8-year-old boy whose anomalous venous drainage was successfully closed with an Amplatzer vascular plug after a total correction of a tetralogy of Fallot. The patient was asymptomatic at his last follow-up.