One-Year Follow-up After Tetralogy of Fallot Total Repair Preserving Pulmonary Valve and Avoiding Right Ventriculotomy.

BACKGROUND: We reviewed our revised surgical strategy for tetralogy of Fallot (TOF) total correction to minimize early exposure to significant pulmonary regurgitation (PR) and to avoid right ventriculotomy (RV-tomy). Methods and Results: Since February 2016, we have tried to preserve, first, pulmonary valve (PV) function to minimize PR by extensive commissurotomy with annulus saving; and second, RV infundibular function by avoiding RV-tomy. With this strategy, we performed total correction for 50 consecutive patients with TOF until May 2018. We reviewed the early outcomes of 27 of 50 patients who received follow-up for ≥3 months. Mean patient age at operation was 10.2±5.0 months, and mean body weight was 8.8±1.2 kg. The preoperative pressure gradient at the RV outflow tract and the PV z-score were improved at most recent echocardiography from 82.0±7.1 to 26.8±6.4 mmHg, and from -2.35±0.49 to -0.55±0.54, respectively, during 11.1±1.6 months of follow-up after operation. One patient required re-intervention for residual pulmonary stenosis. Twenty-two patients had less than moderate PR (none, 1; trivial, 8; mild, 13), and 5 patients had moderate PR. There was no free or severe PR. CONCLUSIONS: At 1-year follow-up, the patients who underwent total TOF correction with our revised surgical strategy had acceptable results in terms of PV function. The preserved PV had a tendency to grow on short-term follow-up.

Carcinoid Valve Disease: A Case Report and Review.

In patients with carcinoid syndrome, the development of carcinoid valve disease typically carries an unfavorable prognosis. We present the case of a patient with significant valvular dysfunction secondary to carcinoid valve disease. Valve replacement surgery was complicated by the development of prosthetic valve degeneration, ultimately requiring percutaneous valve implantation in a valve-in-valve fashion. The technical details of the case, as well as a review of carcinoid valve disease, including its pathophysiology, clinical manifestations, diagnostic features and management considerations, are presented.

The Effects of Pulmonary Valve Replacement for Severe Pulmonary Regurgitation on Exercise Capacity and Cardiac Function.

Patients may develop hemodynamic abnormalities after right ventricular outflow tract (RVOT) repair. Re-intervention timing remains a dilemma. This study evaluates exercise capacity and RV function before and after intervention using age-related comparisons. Twenty-six patients with severe pulmonary regurgitation (PR) after initial repair scheduled for pulmonary valve replacement (PVR) were enrolled. Metabolic treadmill testing (EST) and MRI were obtained before and after surgery. EST results were compared with matched controls. Preoperative exercise time and peak oxygen consumption (VO2 max) were significantly diminished compared with controls but were not significantly different postoperatively. The patients were then split into age-related cohorts. When comparing pre-PVR and post-PVR exercise time and VO2 max among themselves, neither cohort showed significant differences. However, patients younger than 25 years had better postoperative results, an age-related difference not seen in the controls. Preoperative MRI showed significantly dilated RV, PR, and low normal function. After PVR, the right to left ventricular end-diastolic volume ratio (RVEDV:LVEDV) and pulmonary artery regurgitant fraction (RF) significantly decreased. There was no change in ventricular ejection fractions (EF). Severe PR, decreased RVEF, and RV dilation can significantly diminish exercise capacity. PVR improves RVEDV:LVEDV and RF, but not EF. Younger patients had better exercise capacity that was maintained postoperatively. This age-related difference was not seen in the controls, indicating that earlier intervention may preserve exercise capacity. Serial ESTs in patients with severe PR following RVOT repair may identify deteriorating exercise capacity as an early indicator for the need for PVR.

The relationship between longitudinal, lateral, and septal contribution to stroke volume in patients with pulmonary regurgitation and healthy volunteers.

Septal systolic motion is towards the left ventricle (LV) in healthy hearts. Patients with pulmonary regurgitation (PR) and right ventricular (RV) volume overload have systolic septal motion toward the RV. This may affect the longitudinal contribution from atrioventricular plane displacement (AVPD) and septal and lateral contribution to stroke volume (SV). The study aimed to quantify these contributions to SV in patients with PR. Cardiac magnetic resonance imaging was used for assessment of cardiac volumes. Patients (n = 30; age 9-59 yr) with PR due to surgically corrected tetralogy of Fallot and 54 healthy controls (age 10-66 yr) were studied. Longitudinal contribution to RVSV was 47 ± 2% (means ± SE) in patients with PR and 79 ± 1% in controls (P < 0.001). Lateral contribution to RVSV and LVSV was 40 ± 1 and 62 ± 2% in patients and 31 ± 1 and 36 ± 1% in controls (P < 0.001 for both). Septal motion contributed to RVSV by 8 ± 1% in patients and by 7 ± 1% to LVSV in controls (P < 0.001). PR patients have decreased longitudinal contribution to RVSV and increased lateral pumping, resulting in larger outer volume changes and septal motion towards the RV. The changes in RV pumping physiology may be explained by RV remodeling resulting in lower systolic inflow of blood into the right atrium in relation to SV. This avoids the development of pendulum volume between the caval veins and right atrium, which would occur in PR patients if longitudinal contribution to SV was preserved. Decreased AVPD suggests that tricuspid annular excursion, a marker of RV function, is less valid in these patients.

CT and MRI of pulmonary valvular abnormalities.

Pulmonary valve disease constitutes a wide spectrum of conditions. Traditionally, echocardiography has been the technique of choice for the evaluation of pulmonary and other valvular disease. However, with advances in technology, computed tomography (CT) and magnetic resonance imaging (MRI) are playing increasingly important roles in the evaluation of these disorders. In this article, we review the normal appearance of the pulmonary valve and then illustrate various variants and pathological entities of the pulmonary valve.

Right-sided valve disease.

In this review, we discuss right-sided heart valve disease, namely tricuspid regurgitation (TR), tricuspid stenosis, pulmonary regurgitation, pulmonary stenosis and right-sided endocarditis. These are frequently seen in conjunction with other diseases, making assessment of their significance more difficult, but it has become increasingly clear that moderate or severe right-sided heart valve disease, in particular TR, is associated with worse prognosis. There remain large gaps in our knowledge of medical and interventional treatment, but in this article we outline what is known about the causes, presentation and management of these commonly seen conditions.

[Minimally invasive pulmonary valve replacement in pediatric patients: importance of imaging]. / Minimal-invasiver Pulmonalklappenersatz bei pädiatrischen Patienten: Stellenwert der Bildgebung.

CLINICAL/METHODICAL ISSUE: Right ventricular outflow tract (RVOT) dysfunction with pulmonary regurgitation or obstruction is a common postsurgical consequence in congenital heart disease. STANDARD RADIOLOGICAL METHODS: Magnetic resonance imaging (MRI) is widely accepted as standard method of imaging in congenital heart disease. It provides anatomical and functional information without radiation exposure and is therefore well suited for serial examinations. METHODICAL INNOVATIONS: Percutaneous pulmonary valve implantation (PPVI) has been shown to be a safe and effective treatment option for patients with pulmonary valve insufficiency or stenosis. Correct patient selection for PPVI is crucial. It is important to be familiar with the indications and anatomical requirements for stent placement and to tailor imaging protocols. PERFORMANCE: Imaging the RVOT, assessment of right ventricular volumes and function and calculation of pulmonary flow and regurgitation are core elements of the MRI examination prior to PPVI. Low interobserver and intraobserver variation allows even small changes to be detected. ACHIEVEMENTS: Imaging provides relevant information for correct patient selection for PPVI and is part of postinterventional follow-up. Imaging is an important tool for documentation of success and for detection of complications. PRACTICAL RECOMMENDATIONS: Several imaging modalities are used for evaluation of RVOTs; however, MRI can provide answers to most questions without radiation exposure.

Effect of pulmonary regurgitation on cardiac functions based on a human bi-ventricle model.

BACKGROUND AND OBJECTIVE: Assessing the severity of pulmonary regurgitation (PR) and identifying optimal clinically relevant indicators for its treatment is crucial, yet standards for quantifying PR remain unclear in clinical practice. Computational modelling of the heart is in the process of providing valuable insights and information for cardiovascular physiology research. However, the advancements of finite element computational models have not been widely applied to simulate cardiac outputs in patients with PR. Furthermore, a computational model that incorporates both the left ventricle (LV) and right ventricle (RV) can be valuable in assessing the relationship between left and right ventricular morphometry and septal motion in PR patients. To enhance our understanding of the effect of PR on cardiac functions and mechanical behaviour, we developed a human bi-ventricle model to simulate five cases with varying degrees of PR severity. METHODS: This bi-ventricle model was built using a patient-specific geometry and a widely used myofibre architecture. The myocardial material properties were described by a hyperelastic passive constitutive law and a modified time-varying elastance active tension model. To simulate realistic cardiac functions and the dysfunction of the pulmonary valve in PR disease cases, open-loop lumped parameter models representing systemic and pulmonary circulatory systems were designed. RESULTS: In the baseline case, pressures in the aorta and main pulmonary artery and ejection fractions of both the LV and RV were within normal physiological ranges reported in the literature. The end-diastolic volume (EDV) of the RV under varying degrees of PR was comparable to the reported cardiac magnetic resonance imaging data. Moreover, RV dilation and interventricular septum motion from the baseline to the PR cases were clearly observed through the long-axis and short-axis views of the bi-ventricle geometry. The RV EDV in the severe PR case increased by 50.3% compared to the baseline case, while the LV EDV decreased by 18.1%. The motion of the interventricular septum was consistent with the literature. Furthermore, ejection fractions of both the LV and RV decreased as PR became severe, with LV ejection fraction decreasing from 60.5% at baseline to 56.3% in the severe case and RV ejection fraction decreasing from 51.8% to 46.8%. Additionally, the average myofibre stress of the RV wall at end-diastole significantly increased due to PR, from 2.7±12.1 kPa at baseline to 10.9±26.5 kPa in the severe case. The average myofibre stress of the LV wall at end-diastole increased from 3.7±18.1 kPa to 4.3±20.3 kPa. CONCLUSIONS: This study established a foundation for the computational modelling of PR. The simulated results showed that severe PR leads to reduced cardiac outputs in both the LV and RV, clearly observable septum motion, and a significant increase in the average myofibre stress in the RV wall. These findings demonstrate the potential of the model for further exploration of PR.

An ovine model of pulmonary insufficiency and right ventricular outflow tract dilatation.

BACKGROUND AND AIM OF THE STUDY: The treatment of pulmonary insufficiency (PI) following reconstructive surgery of the right ventricular outflow tract (RVOT) in repair of the tetralogy of Fallot remains a significant challenge. The study aim was to establish an ovine model of dilated RVOT and PI, and to quantify the degree of PI and right ventricular remodeling over an eight-week period, using magnetic resonance imaging (MRI). METHODS: Five sheep underwent baseline MRI scanning and catheterization. The weight-indexed right and left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF) and pulmonary regurgitant fraction (RF) were measured at baseline. The animals then underwent pulmonary valvectomy and transannular patch repair of the RVOT. Repeat MRI and hemodynamic measurements were obtained after an eight-week period. RESULTS: The indexed RVEDV increased from 49 +/- 4.0 ml/m2 at baseline to 80 +/- 10.3 ml/m2 at eight weeks after valvectomy (p = 0.01), while the indexed RVESV increased from 13 +/- 3.4 ml/m2 to 33 +/- 8.8 ml/m2 (p = 0.01). The indexed RVSV increased from 36 +/- 3.7 ml/m2 to 47 +/- 1.7 ml/m2 (p = 0.01). The RVEF at baseline was 74 +/- 6%, and this decreased to 59 +/- 5% at follow up (p = 0.02). The RF at baseline was 0 +/- 0% and was increased to 37 +/- 3% at eight weeks after valvectomy (p < 0.001). The left ventricular (LV) function was also diminished: LVEF at baseline was 67 +/- 2%, versus 49 +/- 10% at follow up (p = 0.01). Both, the RV and LV end-diastolic pressures were significantly elevated at follow up. CONCLUSION: All five animals developed pulmonary regurgitation sufficient to cause significant RV dilatation and diminished RV and LV functions. This model may be used to investigate novel therapeutic approaches in the treatment of this difficult clinical problem.

Valve-sparing options in tetralogy of Fallot surgery.

Given late outcomes of patients with tetralogy of Fallot repaired in the 1970s and 1980s, as well as a better understanding of the late deleterious effects of pulmonary regurgitation, there is a tendency toward preservation of the pulmonary valve function during primary repair of tetralogy of Fallot. The bar keeps moving downward, to include smaller and more dysmorphic pulmonary valves. This article reviews some useful indications and techniques for valve-sparing options, including intraoperative balloon dilation and cusp reconstruction using a patch. Just like other valve repair techniques, no one technique can be applied uniformly, and surgeons must master a wide armamentarium of techniques.

Impact of percutaneous device implantation for closure of patent foramen ovale on valve insufficiencies.

BACKGROUND: In patients with percutaneous device implantation for closure of patent foramen ovale (PFO), a 10% incidence of new or worsened aortic regurgitation within 12 months has been reported with echocardiography. Cardiac magnetic resonance imaging is a powerful noninvasive tool to quantify volume and fraction of valve insufficiencies. We studied the acute and long-term impact of percutaneous device implantation for PFO closure on valve insufficiencies in cardiac magnetic resonance imaging. METHODS AND RESULTS: Sequential cardiac magnetic resonance imaging studies were performed in 102 patients with cryptogenic ischemic events. Cardiac magnetic resonance imaging was performed before PFO closure, the day after device implantation, and at 12 months of follow-up. There was no difference in volumetric and hemodynamic parameters before PFO closure compared with 12 months of follow-up. With a cutoff for relevant regurgitation fraction of 5%, there were no statistically significant differences in regurgitation fraction of the semilunar and atrioventricular valves. The median fraction of aortic valve insufficiency was 3.9% (interquartile range [IQR] 2.0% to 5.1%) before PFO closure, 5.4% (IQR 4.1% to 5.9%) after device implantation, and 4.3% (IQR 3.3% to 6.0%) at 12 months of follow-up. The size and type of the occluder had no impact on aortic valve insufficiency. Median regurgitation fraction for the pulmonary valve was 3.6% (IQR 2.4% to 6.7%) before intervention, 7.3% (IQR 5.1% to 8.2%) after occluder implantation and 5.8% (IQR 4.8% to 7.4%) at 12 months of follow-up. Values for the mitral valve were 3.1% (IQR 1.4% to 6.0%), 5.5% (IQR 3.5% to 7.3%), and 3.8% (IQR 1.5% to 7.9%) and for the tricuspid valve were 5.4% (IQR 0.1% to 8.8%), 5.8% (IQR 1.4% to 9.2%), and 6.0% (IQR 1.1% to 8.4%), respectively. CONCLUSIONS: Percutaneous PFO closure with device implantation has no impact on valve insufficiencies as determined by cardiac magnetic resonance imaging.

Comparison of bare metal stenting and percutaneous pulmonary valve implantation for treatment of right ventricular outflow tract obstruction: use of an x-ray/magnetic resonance hybrid laboratory for acute physiological assessment.

BACKGROUND: Treatment of right ventricular outflow tract obstruction is possible with a bare metal stent (BMS), although this treatment causes pulmonary regurgitation. In this study, we assessed the acute physiological effects of BMS versus percutaneous pulmonary valve implantation (PPVI) using an x-ray/magnetic resonance hybrid laboratory. METHODS AND RESULTS: Fourteen consecutive children (median age, 12.9 years) with significant right ventricular outflow tract obstruction underwent BMS followed by PPVI. Magnetic resonance imaging (ventricular volumes and function and great vessel blood flow) and hemodynamic assessment (invasive pressure measurements) were performed before BMS, after BMS, and after PPVI; all were performed under general anesthesia in an x-ray/magnetic resonance hybrid laboratory. BMS significantly reduced the ratio of right ventricular to systemic pressure (0.75+/-0.17% versus 0.41+/-0.14%; P<0.001) with no further change after PPVI (0.42+/-0.11; P=1.0). However, BMS resulted in free pulmonary regurgitation (21.3+/-10.7% versus 41.4+/-7.5%; P<0.001), which was nearly abolished after PPVI (3.6+/-5.6%; P<0.001). Effective right ventricular stroke volume (right ventricular stroke volume minus pulmonary regurgitant volume) after BMS remained unchanged (33.8+/-7.3 versus 32.6+/-8.7 mL/m2; P=1.0) but was significantly increased after revalvulation with PPVI (41.0+/-8.0 mL/m2; P=0.004). These improvements after PPVI were accompanied by a significant heart rate reduction (75.5+/-17.7 bpm after BMS versus 69.0+/-16.9 bpm after PPVI; P=0.006) at maintained cardiac output (2.5+/-0.5 versus 2.4+/-0.5 versus 2.7+/-0.5 mL x min(-1) x m(-2); P=0.14). CONCLUSIONS: Using an x-ray/magnetic resonance hybrid laboratory, we have demonstrated the superior acute hemodynamic effects of PPVI over BMS in patients with right ventricular outflow tract obstruction.

Pulmonary valve repair: an option for an emerging problem.

In this report, we present our experience with successful pulmonary valve repair for pulmonary regurgitation in a 15-year-old patient who had undergone percutaneous pulmonary balloon valvuloplasty for pulmonary stenosis in the neonatal period. The pathological findings and the technique of repair are described in detail as the number of such patients is expected to increase in future.

Isolated congenital pulmonary regurgitation with right ventricular outflow tract aneurysm--a rare variant of Uhl's anomaly.

Isolated absent pulmonary valve syndrome is a very rare entity. We report the case of a four-year-old boy who had congenital absent pulmonary valve with a thin and aneurysmal right ventricular infundibulum. The histological picture was suggestive of Uhl's anomaly. This association of partial right ventricular Uhl's anomaly with absent pulmonary valve syndrome has not been described before. We discuss the embryological and clinical significance of this association.

Quantitative assessment of homograft function 1 year after insertion into the pulmonary position: impact of in situ homograft geometry on valve competence.

AIMS: To prospectively evaluate homograft function with cardiac magnetic resonance (CMR) imaging 1 year after insertion into the pulmonary position, and to assess the impact of in situ homograft geometry, surgical factors, and 'intrinsic' homograft properties on early valve incompetence. METHODS AND RESULTS: A total of 60 patients (mean age 21 +/- 10 years; 35 females) with congenital heart disease underwent pulmonary valve replacement with homograft insertion and were prospectively enrolled into a study protocol that included serial echocardiography and CMR 1 year after surgery. None of the patients had homograft stenosis but 10 (17%) had significant homograft incompetence (i.e. pulmonary regurgitation fraction >20% on CMR). A higher incidence of 'eccentric' pulmonary forward flow pattern (P < 0.001, Fisher's exact test), more acute 'homograft distortion angle' (P < 0.001), larger relative 'annular' size (P < 0.01), and greater pre-homograft right ventricular outflow tract (RVOT) diameters (P = 0.01) at CMR was seen in those with worse homograft function. In a backward multivariate linear regression model, 'eccentric' pulmonary forward flow pattern (r(part) = 0.36, P < 0.001), 'homograft distortion angle' (r(part) = 0.31, P = 0.001), and pre-homograft RVOT diameter (r(part) = 0.19, P = 0.03) were independently associated with the degree of pulmonary regurgitation (in %) at 1 year. CONCLUSION: Using CMR, in this prospective cohort study, we have shown that significant valve incompetence is present in one-sixth of patients after homograft insertion into the pulmonary position, and that alterations in the in situ homograft geometry were associated with the likelihood of developing valve incompetence. These findings imply that mechanical factors may have an important impact on homograft performance.

Minimally Invasive Pulmonary Fibroelastoma Resection.

Pulmonary fibroelastomas are a rare primary cardiac tumor with less than 50 cases reported in the literature to date. We performed a minimally invasive valve-sparing tumor resection through a left anterior mini-incision (LAMI). The procedure was performed without cardiac arrest or aortic cross clamp, expediting postoperative recovery and allowing for an uncomplicated discharge on postoperative day 5. LAMI is a safe and reliable alternative to median sternotomy for patients requiring interventions on the right ventricular outflow tract and main pulmonary artery, including pulmonary fibroelastoma resection and pulmonary valve replacement when needed.

Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance.

BACKGROUND: To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. METHODS AND RESULTS: Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42+/-10% to 43+/-10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m2) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: beta=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m2 for normalization of RV end-diastolic volume or 82 mL/m2 for RV end-systolic volume. CONCLUSIONS: Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m2 or RV end-systolic volume was <82 mL/m2.

Evaluation of right ventricular functions and B-type natriuretic peptide levels by cardiopulmonary exercise test in patients with pulmonary regurgitation after repair of tetralogy of Fallot.

Impairment of right ventricular functions, especially due to chronic pulmonary regurgitation (PR), is a well-known entity in patients with tetralogy of Fallot (ToF) after repair. The aim of this study was to examine the relation between B-type natriuretic peptide (BNP) levels and right ventricular dysfunction by cardiopulmonary exercise test (ET) in patients after repair of ToF. Twenty-five patients with a mean age of 14.1 +/- 4.4 years at follow-up who underwent repair of ToF at a mean age of 4.9 +/- 5.1 years and 29 age- and sex-matched healthy children at a mean age of 13.1 +/- 2.8 years were enrolled in this study. Plasma BNP levels were measured at baseline and at maximal exercise. The volume of right ventricle (RV) and the degree of PR were assessed by two-dimensional echocardiography and color Doppler. Plasma BNP levels were significantly higher in patients with ToF than in controls (28.3 +/- 24.1 vs 7.4 +/- 2.3 pg/mL, p = 0.0001). Exercise was associated with increased plasma BNP levels in both groups. A greater increase in BNP was noted in patients with ToF than in controls (37.6 +/- 27.5 vs 11.3 +/- 4.5 pg/mL, p = 0.0001). Forced vital capacity (FVC%) (84.9 +/- 16.9 vs 98.4 +/- 18.2, p = 0.01) and forced expiratory volume during the 1st second (FEV1%) (91.5 +/- 19.3 vs 103.8 +/- 16.1, p = 0.02) were decreased, exercise duration (ED) (10.1 +/- 1.9 vs 11.4 +/- 1.7 min, p = 0.02), maximum heart rate (HRmax) (171.2 +/- 18.9 vs 186.4 +/- 13.9 /min, p = 0.004), and maximum oxygen uptake (VO(2)max) (1.56 +/- 0.53 vs 2.1 +/- 0.6 L/min, p = 0.007) were lower in patients with ToF. There were significant correlations between the degree of PR and ED (r =-0.3, p = 0.009), HRmax (r =-0.4, p = 0.001), and VO(2)max (r =-0.4, p = 0.001). The correlations were significant both before and after exercise, being more pronounced after exercise between BNP level and the degree of PR (r = 0.6, p = 0.0001). As a result, the severity of PR has a negative influence on right ventricular functions and there is significant relation between right ventricular functions and exercise capacity after repair of tetralogy of Fallot.

Three-dimensional analysis of regional right ventricular shape and function in repaired tetralogy of Fallot using cardiovascular magnetic resonance.

BACKGROUND: Patients with surgically repaired tetralogy of Fallot (rTOF) often have residual pulmonic valve regurgitation, leading to abnormal remodeling and dysfunction of the right ventricle often requiring pulmonic valve replacement. We tested the hypothesis that 3D analysis of right ventricular (RV) shape and function may reveal differences in regional adaptive remodeling that occurs in patients with rTOF, depending on whether a transannular patch (TAP) was utilized. METHODS: Forty patients with rTOF who underwent cardiac magnetic resonance imaging (1.5 T), including 20 with and 20 without TAP, and 10 normal controls were studied. Images were analyzed to measure RV endocardial curvature and global and regional volume and function. RESULTS: RV ejection fraction (EF) was 42 ±â€¯11% in TAP and 38 ±â€¯9% in no-TAP (p = 0.19), both lower than 54 ±â€¯3% in controls (p < 0.01). Left ventricular (LV) EF was 54 ±â€¯9% in TAP, 54 ±â€¯8% in no-TAP (p = 0.87) and 61 ±â€¯16% in controls (both p < 0.01). Indexed LV end-diastolic volumes were higher in no-TAP than in TAP subgroup (p = 0.02). With TAP, mid RV septum showed lower curvature during diastole (less convex), than the mid and apical free walls and free wall adjacent to the RV outflow tract (RVOT; more convex). There were no differences in curvature during systole between rTOF subgroups but mid and RVOT free walls showed higher curvature versus controls. CONCLUSIONS: This is the first study to comprehensively describe the influence of TAP on changes in regional RV shape in patients with rTOF. Understanding these differences may help guide therapeutic options for residual pulmonary valve regurgitation in rTOF patients.

The Ross procedure--valvular pathology of synchronous pulmonary autograft and homograft insufficiency.

We report the morphological changes in a patient with combined pulmonary autograft and homograft dysfunction after 9 years of implantation. The case highlights long-term adaptive changes in the autograft to withstand systemic blood pressure with formation of exuberant pannus rich in elastic tissue. In the homograft, maturation of pannus had produced shortening of the 3 cusps with resultant incompetence.