Residual Pulmonary Stenosis Research Articles

Comparing clinical and echocardiographic outcomes following valve-sparing versus transannular patch repair of tetralogy of Fallot: a systematic review and meta-analysis.

Transannular patch (TAP) repair of tetralogy of Fallot (ToF)relieves right ventricular tract obstruction but may lead to pulmonary regurgitation. Valve-sparing (VS) procedures can avoid this situation, but there is a potential for residual pulmonary stenosis. Our goal was to evaluate clinical and echocardiographic outcomes of TAP and VS repair for ToF. A systematic search of the PubMed, Embase, Scopus, Cochrane Central Register of Controlled Trials and Web of Science databases was carried out to identify articles comparing conventional TAP repair and VS repair for ToF. Random-effects models were used to perform meta-analyses of the clinical and echocardiographic outcomes. Forty studies were included in this meta-analysis with data on 11723 participants (TAP: 6171; VS: 5045). Participants who underwent a VS procedure experienced a significantly lower cardiopulmonary bypass time [mean difference (MD): -14.97; 95% confidence interval (CI): -22.54, -7.41], shorter ventilation duration (MD: -15.33; 95% CI: -30.20, -0.46) and shorter lengths of both intensive care unit (ICU) (MD: -0.67; 95% CI: -1.29, -0.06) and hospital stays (MD: -2.30; 95% CI: [-4.08, -0.52). There was also a lower risk of mortality [risk ratio: 0.40; 95% CI: (0.27, 0.60) and pulmonary regurgitation [risk ratio: 0.35; 95% CI: (0.26, 0.46)] associated with the VS group. Most other clinical and echocardiographic outcomes were comparable in the 2 groups. This meta-analysis confirms the well-established increased risk of pulmonary insufficiency following TAP repair while also demonstrating that VS repairs are associated with several improved clinical outcomes. Continued research can identify the criteria for adopting a VS approach as opposed to a traditional TAP repair.

Severe Residual Pulmonary Stenosis after Surgical Repair of Tetralogy of Fallot: What’s Our Next Strategy?

This case report describes a 25-year-old woman with Tetralogy of Fallot (TOF) who underwent surgical repair at age 15. Eight years later, she presented with recurrent chest pain and was found to have residual severe pulmonary stenosis and regurgitation. This is a complication of TOF repair, particularly when the pulmonary valve is not preserved. The paper discusses the management of TOF, including initial palliative procedures and complete repair surgery. It highlights the long-term complications such as pulmonary valve regurgitation and residual stenosis, both of which may require re-intervention. The importance of long-term follow-up for TOF patients is emphasized.

Early outcome of minimally invasive repair of tetralogy of fallot via right vertical infra-axillary incision

Background: Recently, several authors have detailed their experiences with small cohorts of patients in using minimal invasive surgery (MIS) to treat Tetralogy of Fallot (TOF) with innovative approaches and satisfactory results. The goal of this study was to review an new innovative MIS technique that results in using minimal right vertical infra-axillary incision (RVIAI). 
 Methods: Our retrospective study reviewed 45 patients that were diagnosed with TOF and underwent minimally invasive surgery through RVIAI at Children City Hospital from June 2020 to June 2023. 
 Results: Mean age was 9,4 ± 6,6 months, mean body weight was 7,4 ± 1,7 kg, the male/female ratio was 2,1/1. Mean size of pulmonary annulus was 9,58 ± 1,53 mm (-0,47 ± 1,04 Zscore). Central cannulation was accessed in all patients. There were no deaths or conversions to sternotomy. Average durations of aortic cross-clamp and cardiopulmonary bypass (CPB) were 188,7 ± 50,6 min and 124,6 ± 35,8 min, respectively. During the follow-up of 5.4–32.3 months, 11,1% of these patients were found to have moderate residual pulmonary stenosis. There was no severe residual pulmonary stenosis, residual shunt, severe tricuspid regurgitation. No reoperation, no new onset of chest deformities and no sclerosis were observed during the follow-up.
 Conclusions: The right axillary incision allows a safe and effective approach for Tetralogy of Fallot complete repair and is a potential new standard of care for many patients.

Percutaneous approach to residual pulmonary bifurcation stenosis in conotruncal diseases.

Residual stenosis after right ventricle outflow tract surgery represents a major issue to manage in the children and adult patient with conotruncal defects. Despite a detailed multimodality imaging, the anatomy of distal pulmonary trunk and pulmonary artery bifurcation may be challenging in these patients.The aim of this study was to analyse retrospectively the outcome of the percutaneous transcatheter treatment in children with post-surgical stenosis of pulmonary artery bifurcation.We enrolled 39 patients with a median age of 6.0 years. Standard high-pressure balloon dilation was attempted in 33 patients, effective in 5 of them. Pulmonary branch stenting was performed in 10 patients, effective in 6. A kissing balloon approach was chosen in 17 patients (6 after angioplasty or stenting failure), and this technique was effective in 16 cases. Finally, a bifurcation stenting was performed in 10 patients (second step in 9 cases), effective in all the cases. None of the patients approached by kissing balloon needed a bifurcation stenting.In conclusion, standard balloon angioplasty and standard stenting might be ineffective in post-surgical stenosis involving pulmonary artery bifurcation. In this population, kissing balloon or bifurcation stenting, followed by side branch de-jailing, may be more effective in relieving the gradient.

Magnetic resonance imaging of the heart as a method of long-term monitoring of right heart function after tetralogy of Fallot surgery

<p><strong>Introduction.</strong> Cardiac magnetic resonance imaging (CMR) is considered the reference diagnostic method for quantifying right ventricular size and function, and pulmonary regurgitation in patients with tetralogy of Fallot surgery. The aim of this paper is to confirm the importance of magnetic resonance continuous postoperative monitoring of right and left heart function parameters as a diagnostic method that provides the most precise and accurate assessment.</p><p><strong>Methods.</strong> The prospective observational study included subjects with TOF surgery who were diagnosed with residual morphological and/or functional disorders on control postoperative echocardiographic examinations. All subjects underwent magnetic resonance imaging of the heart on a 1.5 T scanner with dedicated coils for the heart surface according to the standard protocol for a period of one year from the beginning of the study. Criteria for exclusion from the study were: significant residual pulmonary stenosis, condition after pulmonary valve replacement, existence of residual shunt lesions, contraindications for cardiac magnetic resonance imaging (pacemaker, ICD, claustrophobia). Depending on the time elapsed since the tetralogy of Fallot surgery, the subjects were divided into groups: more than 15 years, 11−15 years, 6−10 years, less than 5 years.</p><p><strong>Results.</strong> The study included 131 subjects with an average age of 24.18 ± 11.57 years with complete correction of TOF. Intergroup differences in values of right ventricular enddiastolic volume, right ventricular ejection fraction, and left ventricular ejection fraction were demonstrated, but there was no statistically significant intergroup difference in values of pulmonary regurgitation fraction. The negative interaction of the right and left ventricles intensifies during the years of follow-up of patients after TOF surgery, which is especially true fifteen years after surgery.</p><p><strong>Conclusion.</strong> CMR has the most significant role in research efforts aimed at improving the outcomes of operated patients with tetralogy of Fallot.</p>

Surgical Treatment Outcomes of Tetralogy of Fallot: Experience of the Cardiovascular Department of the Mohammed VI University Hospital in Marrakech.

BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. The surgical approach to TOF continues to evolve, with many centers now favoring early repair for TOF. This study aimed to determine the prevalence of postsurgical complications such as pulmonary insufficiency, residual pulmonary stenosis, and interventricular communication.MethodologyWe conducted a cross-sectional, observational study with a descriptive and analytic focus involving 61 patients who were operated on for TOF.ResultsIn total, 39 (63.9%) patients had a regular TOF and 22 (36.1%) had an irregular TOF. In our study, 32 (56.14%) patients had a pulmonary insufficiency, of whom 20 had a minimal pulmonary insufficiency (62.5% of pulmonary insufficiency cases), and 79.4% of patients with right ventricular outflow tract enlargement had pulmonary insufficiency (p < 0.005). Among patients who did not have a right ventricular outflow tract enlargement, three cases of pulmonary insufficiency were identified with a prevalence of 16.6%. Six (10.52%) patients had residual pulmonary stenosis. In addition, two (3.2%) cases of minimal residual ventricular septal defects were identified in this study.ConclusionsPostoperative complications of TOF treatment are frequent and require medical care throughout the lifetime of patients.

Predictive Value of Right Ventricular Pressure Measurement for Residual Pulmonary Stenosis in Tetralogy Repair

Objective: The long-term outcome of tetralogy of Fallot repair depends on an adequate relief of right ventricular outflow tract obstruction and preservation of the pulmonary valve function. Since intraoperative transesophageal echocardiography is not routinely performed in small patients, we postulated that the post-bypass right ventricular pressure measured intraoperatively could predict residual pulmonary stenosis when evaluated by transthoracic echocardiography.Materials and Methods: Of the 187 patients who underwent tetralogy repair between 2012 and 2019 at Siriraj Hospital, Thailand, 95 with right ventricular pressure measurements and pre-discharge echocardiography were included in the study. Their intraoperative parameters, and postoperative outcomes were analyzed. The tolerable pressure cutoff was determined.Results: The median patient age was 3.9 years old (interquartile range 2.75–6). Fifty-three patients (54.6%) required the use of the transannular patch. Ten patients (10.3%) had significant residual pulmonary stenosis with a mean right ventricular systolic pressure of 64.0±10.6 mmHg compared with 48.7±14.4 mmHg for the other patients. There was an association between the pressure figure and the degree of residual pulmonary stenosis (rho=0.391, p=0.01). A systolic pressure above 49 mmHg predicted pulmonary stenosis with a likelihood ratio of 2.18 (1.94-2.80, 95%CI). The likelihood rose to 2.93 (2.44-4.01, 95%CI) if the pressure resulted in a right to left ventricular pressure ratio above 0.62. The patients whose figures did not exceed 49 mmHg experienced no significant residual obstruction, regardless of the pressure ratio.Conclusion: Intraoperative measurement of the right ventricular pressure can predict residual pulmonary stenosis after tetralogy repair with a reassuring cutoff of 49 mmHg

Neurofibromatosis 1-Noonan Syndrome Associated with Pulmonary Stenosis and Hypertrophic Cardiomyopathy

Abstract Neurofibromatosis 1-Noonan syndrome is considered a distinct clinical entity, combining characteristics of both autosomal dominant disorders: neurofibromatosis 1 and Noonan syndrome. We present the case of a 20-year-old patient clinically diagnosed with neurofibromatosis 1-Noonan syndrome, with genetic confirmation-heterozygous mutation of PTPN11 gene and a variant of uncertain significance in NF1 gene (c.2989A&gt;G). Associated congenital heart disease was diagnosed at birth-severe pulmonary valve stenosis and infundibular pulmonary stenosis, surgically corrected at the age of one. At adult age, cardiologic assessment shows severe pulmonary regurgitation post commissurotomy, with residual large pulmonary stenosis and left ventricular apical hypertrophy, suggesting apical hypertrophic cardiomyopathy, confirmed by cardiac magnetic resonance. The patient needs periodical follow-up in order to identify the perfect timing for correction of severe pulmonary regurgitation. As there are no specific genetic therapies for neurofibromatosis 1 or Noonan syndrome, the diagnose and management of associated comorbidities is the main aspect to be taken into considered.

Abstract 12207: Impact of Residual Pulmonary Stenosis on the Prognosis After Pulmonary Valve Replacement for Pulmonary Regurgitation

Introduction: Pulmonary valve replacement (PVR) for pulmonary regurgitation (PR) was reported to provide beneficial effects on patients late after repair of tetralogy of Fallot (TOF). However, the impact of the degree of residual pulmonary stenosis (PS) in patients with severe PR remains unclear. Furthermore, the assessment of residual PS was difficult in patients with reduced RVEF. This study aimed to identify the impact of residual PS on the incidence of cardiovascular adverse events after PVR in preserved RVEF (pEF) or reduced RVEF (rEF) patients with PR late after repair of TOF. Methods: This was a single-center, retrospective review of 64 patients (56 repaired TOF-PS and 8 repaired TOF-pulmonary atresia) who underwent PVR for moderate to severe PR between 2003 and 2020. The threshold dividing pEF and rEF was 40%. Regarding residual PS, the definition the pressure gradient at the pulmonary valve in PSR group was 25 mmHg or more and less than 50mmHg. Patients were divided into 4 groups according to RVEF and the degree of residual PS (20 in PR-pEF, 12 in PSR-pEF, 25 in PR-rEF, 7 in PSR-rEF). Cardiovascular adverse events included cardiac death, prosthetic valve dysfunction, arrhythmia requiring treatment, and hospitalization for heart failure. Based on the above, the influence of hemodynamic parameters before PVR was analyzed. Results: The median follow-up period was 6.1 years [interquartile range 2.7-10]. There was no cardiac death and prosthetic valve dysfunction. Two patients died due to non-cardiac reason. Twenty-two (34%) patients showed cardiovascular adverse events. The cardiovascular adverse event-free rates were the highest in PSR-pEF group (100% at 5 years) and the lowest in PSR-rEF group (43% at 5 years) after PVR (log-rank p &amp;lt; 0.01), and 60% in PR-pEF and 70% in PR-rEF. In comparison between PSR groups, PSR-rEF had higher RVEDP (8±2 vs 13±2 mmHg, p &amp;lt; 0.01) and larger RAVI (71±25 vs 105±42 ml/m 2 , p=0.043). Conclusion: Patients with PSR-rEF had a high incidence of cardiovascular adverse events, high RVEDP, and large RAVI, suggesting RV adverse remodeling. The association between the degree of residual PS and RVEF should be taken into consideration on deciding the timing of PVR for PR.

COMPLETE REPAIR OF TETRALOGY OF FALLOT (TOF) ON BEATING HEART SURGERY WITHOUT AORTIC CROSS CLAMPING : REPORT OF A CASE

Introduction: Reperfusion injury is a well-known phenomenon that occurred in cardioplegic techniques with cardiopulmonary bypass. Therefore great effort is made to prevent reperfusion injury. Beating Heart Continuous Coronary Perfusion (BHCCP) surgery is one of the alternative techniques to improve an ischemic reperfusion injury in open-heart surgery, either in pediatric or in adult. This technique can be done in complete repair of tetralogy of Fallot (TOF). Purpose: To report our first experience in doing complete repair of TOF using beating heart technique without aortic cross-clamping Case Report: A five-year-old boy came on to ER on January 6th, 2012, with a history of cyanotic since birth. His echocardiography and catheterization concluded a tetralogy of Fallot with a McGoon ratio of 1.95 (the diameter of RPA and LPA were 9.14 mm and 8.79 mm, respectively). A complete repair of TOF was done using beating heart surgery without aortic cross-clamping technique. One month post-surgery evaluation, there weren't any neurological or motor disorders. Echocardiography results showed no residual pulmonary stenosis, but 0.2 mm residual VSD was found. Conclusion: This heart rate technique is an alternative method of myocardial protection in cardiac surgery. In several cardiac centres globally, this technique is relatively safe and can be used during surgery, especially in the total correction of TOF.

Evaluation and Cardiac Rehabilitation on a patient with a congenital heart disease

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Severe congenital heart disease (CHD) requiring surgical intervention occurs in 6 of 1000 live-born infants. Substantial improvements in surgical treatment and perioperative care have led to excellent long-term cardiac outcomes for most of these patients and, consequentially, to a significant increase in survival rates. Formal evaluations of preschool, school-aged children and adolescents born with complex CHD demonstrate a pattern of neurodevelopmental sequelae, which may appear alone, or in combination. Purpose F. is a 21 years-old patient with repaired complex transposition of the great arteries with ventricular septal defect, who have already undergone three surgeries: a) an initial palliative surgery (pulmonary artery banding and ductus arteriosus ligation) at the neonatal period; b) a corrective surgery (arterial switch and VSD closure) at the end of the first year of life and c) Pulmonary artery plasty for residual pulmonary stenosis at the age of 16 years-old. Now, he was admitted to our department for urgent wide-complex tachycardia treatment. His initial assessment documented a subjectively impaired motor development, and subsequently, a formal assessment was performed. Methods The Bruininks-Oseretsky Test of Motor Proficiency, second edition (BOT-2) is an individually administrated test that resorts to a series of engaging, goal-directed activities to measure a wide array of motor skills in individuals ages 4 through 21. The BOT-2 uses a subtest and composite structure that highlights motor performance in the broad functional areas of stability, mobility, strength, coordination, and object manipulation. Results Patient"s performance in the four main motor skill areas will be report, namely: Fine Manual Control, Manual Coordination, Body Coordination, and Strength and Agility. Because Fabio completed all four parts of the BOT-2, a comprehensive score from all four areas, the Total Motor Composite, will also be reported. Fabio"s scores were compared to the ones of a control group of age and gender-matched individuals. This Patient started sessions of Cardiac Rehabilitation. The rehabilitation sessions are conducting for 1 hour three times a week for 1 year. This program still in progress, but we speculate that the will improve his motor abilities and developmental skills. Conclusion The risk of a poor developmental outcome varies according to the specific cardiac effect. In addition, there is significant interindividual variation in developmental outcome even among children with the same cardiac defect. Other factors may contribute to neurologic dysfunctions. These factors can be divided into three main categories and time-frames: prenatal, perioperative and post-discharge.

Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot

The choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction. To compare 30-year outcomes of TOF repair after a VS procedure vs TAP. This retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021. The study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis. The primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions. Among 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P < .001) and patients who underwent a VS procedure with at least moderate residual stenosis (1.3 interventions [95% CI, 0.9-1.9 interventions]; MR, 0.65 [0.45-0.93]; P = .02). Results were similar for PVR, with a 30-year cumulative mean 0.3 PVRs [95% CI, 0.1-0.7 PVRs] for patients who underwent a VS procedure without residual pulmonary stenosis (MR, 0.22 [95% CI, 0.12-0.43]; P < .001) and 0.6 PVRs (95% CI, 0.2-1.5 PVRs) for patients with at least moderate residual stenosis (MR, 0.44 [95% CI, 0.21-0.93]; P = .03), compared with 1.4 PVRs (95% CI, 0.8-2.5 PVRs) for the TAP group. No statistically significant difference was found for cardiovascular hospitalizations. This study found that patients who underwent a VS procedure had increased 30-year survival, fewer cardiovascular reinterventions, and fewer PVRs compared with individuals who underwent TAP, even in the presence of significant residual pulmonary stenosis. These findings suggest that it is beneficial to perform a VS procedure when possible, even in the presence of moderate residual stenosis, compared with the insertion of a TAP.

Acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function.

Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by pre-transcatheter pulmonary valve implantation physiology. Transcatheter pulmonary valve implantation is a widely available option to treat residual or recurrent pulmonary stenosis and pulmonary insufficiency. Transcatheter pulmonary valve implantation acutely increases pulmonary artery size and diastolic pressure in patients with pulmonary insufficiency and acute pulmonary edema has been reported after transcatheter pulmonary valve implantation, possibly related to acute left ventricular volume loading. However, the impact of transcatheter pulmonary valve implantation on left ventricular diastolic function has not been established. Patients who underwent transcatheter pulmonary valve implantation from 2010 to 2017 at our centre were grouped by indication for transcatheter pulmonary valve implantation as pulmonary stenosis, pulmonary insufficiency, or mixed disease. Separate analysis was performed on those who underwent transcatheter pulmonary valve implantation for pulmonary stenosis versus pulmonary insufficiency or mixed disease. Intracardiac haemodynamics immediately before and after transcatheter pulmonary valve implantation and echocardiographic assessment of left ventricular systolic and diastolic function at baseline, 1-day post transcatheter pulmonary valve implantation, and 1-year post transcatheter pulmonary valve implantation were compared between groups. In 102 patients who underwent transcatheter pulmonary valve implantation, the indication was pulmonary stenosis in 29 (28%), pulmonary insufficiency in 28 (29%), and mixed disease in 44 (43%). There were no significant differences in left ventricular systolic or diastolic function between groups at baseline, immediately after transcatheter pulmonary valve implantation, or 1-year post implantation. The mean pulmonary artery wedge pressure increased equally across groups. While patients with pulmonary insufficiency likely have acute left ventricular volume loading following transcatheter pulmonary valve implantation, this does not appear to be haemodynamically significant as transcatheter pulmonary valve implantation was not associated with measurable changes in left ventricular systolic or diastolic function acutely or 1-year post implantation.

Early and mid-term outcomes of total repair of tetralogy of Fallot through a right subaxillary thoracotomy

We aimed to investigate the safety, feasibility and early and mid-term outcomes of total repair of elective tetralogy of Fallot through a right subaxillary thoracotomy. Between May 2010 and September 2015, a retrospective review of patients with elective tetralogy of Fallot undergoing primary repair through a right subaxillary thoracotomy was performed. This study included 47 patients [27 males, 20 females; the mean age of 1.7 ± 1.5 years (ranging from 3 to 106 months); body weight of 9.7 ± 2.8 kg (ranging from 5 to 16 kg); McGoon ratio ≥1.5]. The ventricular septal defects were approached through a right atriotomy in 30 patients, a right ventriculotomy in 8 patients and the combination of a right atriotomy and ventriculotomy in 9 patients. Transannular and non-transannular patches were used in 20 and 27 patients, respectively. There was 1 operative death (2.1%), and the others recovered uneventfully. The median cardiopulmonary bypass and aortic cross-clamping times was 79 ± 20.8 (45-136) and 50 ± 15.7 (25-94) min, respectively. The mechanical ventilation time, length of intensive care unit and postoperative hospital stay were 0.9 ± 0.6, 2.6 ± 1.1 and 10.7 ± 2.7 days, respectively. The main complications involved low cardiac output syndrome in 5 patients, arrhythmia in 6 patients, pulmonary infection in 4 patients, small residual shunt in 2 patients, mild residual right ventricular outflow tract obstruction in 2 patients, mild tricuspid regurgitation in 3 patients and atelectasis in 1 patient. The mean follow-up time was 82 ± 22.0 months, and no late deaths occurred during the follow-up. One (2.1%) patient received reintervention for residual significant pulmonary artery stenosis. That was in addition to 2 (4.3%) patients having mild residual right ventricular outflow tract, 1 (2.1%) patient with moderate tricuspid regurgitation and 3 (6.4%) patients with moderate pulmonary valve regurgitation to be followed up. The right subaxillary thoracotomy, providing a safe and feasible alternative, can be performed with a low mortality rate and satisfactory early and mid-term outcomes for total repair of elective tetralogy of Fallot.

Percutaneous atrial septal defect closure in infant weighing &lt;10 kg and having a bilateral superior vena cava: a case report

Percutaneous closure has become a preferred treatment for secundum atrial septal defect (ASD). However, this approach remains challenging in treating small infant weighing &lt;10 kg because of procedure-related complications. The closure of ASD in other congenital anomalies, such as a bilateral superior vena cava (SVC), should be cautiously conducted. A 15-month-old boy with a body weight of 8 kg was diagnosed with secundum ASD (9–11 mm in diameter), residual pulmonary stenosis, and bilateral SVC. Transcatheter closure of ASD was successfully performed by using a 12 mm CeraFlex occluder device (Lifetech Scientific Corporation). Post-procedural examination showed good results without any impingement to the adjacent structure. Therefore,the transcatheter closure of ASD in infant weighing &lt;10 kg and having bilateral SVC is technically feasible and safe.

Effects of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension on remodeling in right-sided heart.

Remodeling in the right-sided heart plays an important role in the management of pulmonary hypertension (PH) patients. However, the effect of balloon pulmonary angioplasty (BPA) on right ventricular (RV) and right atrial (RA) morphology of patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains uncertain. This study involved 45 CTEPH patients who underwent BPA with mean pulmonary artery pressure (mPAP) of 37.0 mmHg (all ≥ 25 mmHg). All patients underwent echocardiography and right-heart catheterization at baseline and 3 months after BPA. RV and RA remodeling was assessed as RV and the RA area, and RV systolic function was calculated by averaging peak speckle-tracking longitudinal strain of the RV free-wall (RV free-wall strain). Significant reverse remodeling in the right-sided heart was observed after BPA, resulting in improvement of mPAP and pulmonary vascular resistance (RV area: from 15.0 ± 5.3 to 9.6 ± 3.0 cm2, p < 0.0001; RA area: from 17.3 ± 6.6 to 13.4 ± 3.8 cm2, p = 0.0002; RV free-wall strain: from 15.9 ± 5.6 to 21.2 ± 4.9%, p < 0.0001). Furthermore, multiple regression analysis showed that the baseline RV area was an independent predictor of post-BPA normalization of RV systolic function defined as RV free-wall strain ≥ 20% (odds ratio = 1.16, p = 0.0305). Interestingly, significant RV reverse remodeling was also observed after additional BPA even in 18 CTEPH patients with residual pulmonary arterial stenosis, whose mPAP was normalized after BPA (RV area: from 11.5 ± 3.8 to 9.2 ± 3.8 cm2, p = 0.0045; RV free-wall strain: from 17.2 ± 4.8 to 22.8 ± 7.4%, p = 0.0216). Significant reverse remodeling in the right-sided heart, as well as hemodynamic improvement, was observed in CTEPH patients after BPA.

Pulmonary Artery Reconstruction After Failed Pulmonary Artery Stents

Pulmonary artery stents are widely deployed in patients with stenoses in the branch pulmonary arteries. However, stents do not address more peripheral sites of stenosis and invariably develop in-stent restenosis. The purpose of this study was to review our experience with pulmonary artery reconstruction after failed pulmonary artery stents. This was a retrospective study of 56 patients who underwent pulmonary artery reconstruction after failed pulmonary artery stents. These patients had undergone a median of 2 (range, 0-5) previous surgical procedures and 2 (range, 1-4) pulmonary artery stents. The median age at stent surgery was 5 (range, 0.3-23.6) years. The majority of stents (79%) were completely removed and patch augmented. The minority of stents (21%) were felt to be unremovable and thus were split longitudinally and reconstructed using a pulmonary artery homograft. There was 1 (1.8%) operative mortality. The mean pulmonary artery-to-aortic pressure ratio decreased from a preoperative value of 0.91 ± 0.21 to a postoperative value of 0.31 ± 0.07 (P < .001). The median hospital length of stay was 10 days. The median durationof follow-up was 1.8 years. There has been no midterm mortality. Six patients have undergone balloon dilation postoperatively for residual pulmonary artery stenosis. Pulmonary artery reconstruction resulted in a significant decrease in pulmonary artery-to-aortic pressure ratios. The subsequent need for reintervention on the pulmonary arteries has been relatively low (11% to date). These results suggest that patients with pulmonary artery stents can be successfully treated with surgical reconstruction.

Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience.

Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery. We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated. Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1). The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Risk factors for infective endocarditis following transcatheter pulmonary valve replacement in patients with congenital heart disease.

We sought to delineate the risk factors for infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TCPVR). Despite the therapeutic benefits of TCPVR for treatment of dysfunctional right ventricular outflow tracts, IE is a major complication of the approach. Specific hemodynamic gradients and patient immune status as predisposing factors for IE are largely unexplored. We performed a retrospective review of patients who had undergone TCPVR at UCLA between October 2010 and October 2017. Cases of IE were diagnosed based on the modified Duke criteria. Two hundred and thirty-five cases of TCPVR were performed with a mean follow-up of 2.6 years (range 0.0-8.0 years). Sixteen distinct IE events developed in 13 patients (Melody™ n = 12, SAPIEN n = 1), with a median time from implant to IE of 3.3 years (range 2.0-7.2 years). Univariate Cox regression showed that immunocompromised status was significantly associated with the development of IE hazard ratios (HR 5.43 [1.80-16.4], p = .003). Kaplan-Meier curves show that the 5-year freedom from IE among immunocompetent patients was 87% (95% CI 78-96%) versus 64% (95% CI 39-89%) among immunocompromised patients (log-rank p = .02). Postimplant right ventricular systolic pressure was higher among immunocompromised patients (p = .03). The risk of IE post-TCPVR in immunocompromised patients with residual pulmonary stenosis was 43%. Among the risk factors examined in this study, immunocompromised status was the most significant predictor of IE development post-TCPVR. Patients with the lowest risk of IE are those with competent immune systems, without a history of IE, and with minimal residual pulmonary valve gradients post-TCPVR.

Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study.

Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS). Long-term data following BPV is limited to small, single center studies. BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were≥moderate PI by echocardiogram and residual PS≥40 mmHg. Risk factors for≥moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis. Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mmHg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had≥moderate PI, 41 patients (17%) had residual PS>40 mmHg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with≥moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention. Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.