Surgical Valvuloplasty Research Articles

Initial balloon versus surgical valvuloplasty in children with isolated congenital aortic stenosis: Influence on timing of aortic valve replacement

ObjectiveTo evaluate the influence of initial intervention on the long-term outcomes in congenital aortic stenosis. MethodsTwo hundred forty-three children underwent initial intervention between 1997 and 2022, by surgical valvuloplasty in 92 (32% neonates, 36% infants) and balloon valvuloplasty in 151 (27% neonates, 30% infants). Twenty-eight patients (11.5%) had associated mitral valve stenosis. Competing risk analysis for death, alive after initial intervention, or alive after aortic valve replacement (AVR) was performed and factors influencing survival or AVR examined. ResultsThere were 9 early deaths (3.7%). During a median follow-up of 13.5 years (range, 1.5-26.7), 98 patients had reintervention on the aortic valve (40.3%), whereas 145 had AVR (59.6%) at a median age of 14.0 years (interquartile range, 9.0-17.0), which was by Ross procedure in 130 (89.6%). Of the 12 late deaths, 3 were perioperative and 9 occurred as outpatients. There were no perioperative or late deaths after AVR. AVR occurred earlier in patients who had initial balloon (12.0 years [interquartile range, 5.0-14.5]) rather than surgical (18.5 years [interquartile range, 15.5-21.5]) valvuloplasty (P < .05). Actuarial survival in the cohort was 91.3% at 25 years, with no difference between the 2 initial interventions. Critical aortic stenosis, mitral stenosis, and initial intervention as a neonate were independent risk factors for worse survival. ConclusionsWe demonstrate excellent early and late survival in patients with congenital aortic stenosis after initial balloon or surgical valvuloplasty. Whilst children who had balloon valvuloplasty had AVR earlier than those who had initial surgical valvuloplasty, patient factors had a greater influence on survival than choice of initial intervention.

Postnatal outcome following fetal aortic valvuloplasty for critical aortic stenosis.

To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients. This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation. Sixty-three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3-32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in-utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty-eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0-40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty-eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow-up of 23.3 (2.0-112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who had UVC at birth were still alive at the latest follow-up assessment, at a median (range) gestational age of 24.3 (8.3-48.7) months. FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

SEVERE SYMPTOMATIC MITRAL REGURGITATION AFTER ANNULOPLASTY FAILURE: SUCCESSFUL TRANSCATHETER EDGE TO EDGE REPAIR

Abstract Surgical mitral valve repair is the most common, feasible and preferred procedure to treat primary and secondary mitral regurgitation (MR). However, 30% of patients suffer from residue severe regurgitation 10 years after surgical intervention, resulting in a considerable number of Redo procedures. In elderly and high–risk patients with major comorbidities medical treatment is the only and often insufficient option for symptoms’ relief. Percutaneous Transcatheter Edge to Edge repair technique (TEER) with MitraClip system is emerging as an off–label valid alternative to successfully treat severe mitral regurgitation after surgical valvuloplasty failure. Transesophageal echocardiography (TEE) is essential in diagnosis, planning, intraprocedural device placement, and follow up. In the peculiar setting of failed annuloplasty, with small residual valve area, the main risk is the creation of a gradient across the mitral valve. We present two cases of severe mitral regurgitation after surgical annuloplasty with ring placement, carefully evaluated for TEER and successfully treated with this procedure: 1) a 79–year–old man presented with exertional dyspnea and orthopnea in the setting of previous surgical mitral Annuloplasty (2003) with a 32–mm Carpentier Ring. Transesophageal echocardiography demonstrated severe mitral regurgitation due to P2 prolapse and central V–shaped cut of the valve, mean diastolic gradient 1.8 mmHg. 2) a 83 year–old woman presented with dyspnea (NYHA functional class III) and severe mitral regurgitation after previous Annuloplasty with a 34–mm Edwards Ring and placement of Goretex artificial Chordae (2016). TEE showed prolapse of P2 and P3 leaflets, mean diastolic gradient 2.17 mmHg, valvular area 3.5 cmq. Both patients underwent transfemoral and transseptal implantation of one MitraClip device (XTw and XT, respectively) under Transesophageal echocardiography guidance with successful reduction of mitral regurgitation to mild level and symptoms relief. In the first case post procedural mean diastolic gradient was 3.7 mmHg, in the second case it was 3.16 mmHg.

Case report: Aortic valve endocarditis and recurrent pulmonary valve stenosis

BackgroundWe discuss a rare case of an adult patient with different pathologies involving the aortic and pulmonary valves in need of surgery.Case presentationThe patient had a history of congenital PV stenosis and surgical valvuloplasty. Almost 50 years later the patient underwent a complex second heart surgery due to infective endocarditis of the aortic valve and high-grade restenosis of the pulmonary valve. Replacement of the aortic and pulmonary valve, as well as reconstruction of the RVOT and closure of a persistent foramen ovale, followed. Postoperative course was uneventful and the patient was discharged home a week after surgery.ConclusionSimultaneous surgery of pulmonary and aortic valves due to different pathologies is rare but can be performed successfully even in advanced age and can improve quality of life.

Balloon aortic valvuloplasty in neonates: short- and long-term effects and predictors of successful outcome.

IntroductionBalloon aortic valvuloplasty (BAV) is a common treatment method of aortic valve (AV) stenosis in neonates. Long-term BAV effects are suboptimal, and their predictors are not well acknowledged.AimTo identify predictors of suboptimal short- and long-term BAV results.Material and methodsThe study group comprised forty-three neonates (8 females; weight 3.34 ±0.56 kg) who underwent BAV between 1998 and 2021. Seventeen patients (39.53%) had critical AV stenosis. AV was bicuspid in 22 patients, tricuspid in 12, unicuspid in 2, and undefined in 7 patients. The mean balloon/annulus ratio was 0.9 ±0.07. Catheterization, clinical, and follow-up data were analysed.ResultsThe peak-to-peak gradient decreased from 67.5 ±26.3 to 21.3 ±12.6 mm Hg. Twenty-eight patients (65.1%) had adequate early outcome. Aortic regurgitation (AR) occurred in 13 (30.2%) patients. No predictors of inadequate early outcome were found. Twenty-year survival was 90.7%. Eleven (35.5%) patients underwent reintervention at a median of 12 (1–215) months; BAV in 5 patients, surgical valvuloplasty in 2, Ross operation in 2, AV replacement in 1, and Norwood operation in 1 patient. Fifteen-year freedom from reintervention (FFR) was 48%. Adequate early outcome resulted in higher FFR (71% vs. 22%), and so did no significant AR (60% vs. 30%).ConclusionsBAV provides satisfying early results. AR remains a significant aftermath of BAV. Risk factors and procedural techniques improving the outcome of BAV are unclear. Further research is needed to improve FFR.

Balloon dilation versus surgery for severe neonatal aortic valve stenosis: A matched comparison

Two therapeutic approaches exist for first-line treatment of neonatal severe/critical aortic valve stenosis: percutaneous balloon dilatation and surgical valvuloplasty. For both interventions, the early and long-term results are satisfactory. Previous reports have failed to show clear superiority of one versus the other procedure. In la Timone university hospital (Marseille), balloon dilation is the usual therapeutic approach for neonatal critical/severe aortic valve stenosis. In Necker university hospital (Paris), surgical valvuloplasty remains the preferred option. The aim of our current study is to compare the 2 populations using propensity scores matching. Patients aged < 1 month who underwent percutaneous aortic valve balloon dilation for aortic valve stenosis in Marseille between 2003 and 2020, are included. Patients with borderline left ventricle or hypoplastic left heart syndrome are excluded. Balloon patients will be matched based on age, weight, duct-dependency, left ventricular function, aortic valve morphology and mitral function, with patients who underwent surgical aortic valvuloplasty in Necker (Paris). Early complications, survival, freedom from reintervention, residual stenosis and regurgitation will be retrospectively collected from hospital charts. Initial diagnosis and residual lesions will be confirmed by reviewing echocardiography studies. The primary outcome will be the all-cause mortality. The secondary outcomes will be reintervention, aortic valve replacement, presence of significant valve stenosis or insufficiency at latest follow-up. The results will be available in June 2021. Balloon dilation and surgical valvuloplasty are both efficient procedures for early relief of neonatal aortic stenosis. In case of superiority of one of the techniques, our current practice may need to be questioned and adapted. The complementary aspect of the 2 treatment strategies may also be highlighted.

The Ross-Konno operation for neonates and infants with severe aortic incompetence following treatment for critical aortic stenosis.

Aortic valve stenosis in neonates and infants is associated with congestive cardiac failure, and balloon or surgical valvuloplasty provides relief of stenosis. Occasionally severe aortic insufficiency necessitates urgent aortic valve replacement. We reviewed our experience with the Ross-Konno procedure in patients <1 year. Between October 2013 and May 2020, 36 patients underwent balloon (34) or surgical (2) aortic valvuloplasty for aortic stenosis. Six patients subsequently underwent a Ross-Konno procedure. The median age at operation was 55 (27-116) days and weight was 4.25 (2.5-12) kg. All patients were in severe cardiac failure and had a small aortic annulus with Z-score -3.1 (-1 to -4.4). There were no early or late deaths. At the latest follow-up at 39 (13-60) months, ventricular function had improved in all patients and no patient was on anti-failure medication. On echocardiography, there wasno more than trivial aortic regurgitation and no left ventricular outflow tract obstruction. One patient required right ventricle to pulmonary artery conduit replacement and one patient had homograft stenting. Despite the severe preoperative haemodynamic compromise, the urgent Ross-Konno procedure was associated with excellent operative survival and recovery of ventricular function. The need for reintervention to the pulmonary conduit remains a cause for concern.

Results of balloon and surgical valvuloplasty in congenital aortic valve stenosis: A 19-year, single-center, retrospective study.

Background This study aims to compare the success, complications, and long-term outcomes of aortic balloon valvuloplasty and surgical aortic valvuloplasty in pediatric patients with congenital aortic valve stenosis. Methods Between March 2000 and October 2019, a total of 267 procedures, including 238 balloon valvuloplasties and 29 surgical valvuloplasties, in 198 children (135 males, 63 females; mean age: 57.4±62.6 months; range, 0.03 to 219 months) were retrospectively analyzed. The hospital records, echocardiographic images, catheterization data, angiography images, and operative data were reviewed. Results Aortic regurgitation was mild in 73 patients before balloon valvuloplasty, and none of the patients had moderate-to-severe aortic regurgitation. Compared to surgical valvuloplasty, the rate of increase in the aortic regurgitation after balloon valvuloplasty was significantly higher (p=0.012). The patients who underwent balloon valvuloplasty did not need reintervention for a mean period of 46±45.6 months, whereas this period was significantly longer in those who underwent surgical valvuloplasty (mean 80.5±53.9 months) (p=0.018). The overall failure rate was 8%. Moderate-to-severe aortic regurgitation was the most important complication developing due to balloon valvuloplasty in the early period (13%). All surgical valvuloplasties were successful. The mean length of hospitalization after balloon valvuloplasty was significantly shorter than surgical valvuloplasty (p=0.026). During follow-up, a total of 168 patients continued their follow-up, and a reinterventional or surgical intervention was not needed in 78 patients (47%). Conclusion Aortic balloon valvuloplasty can be repeated safely and helps to eliminate aortic valve stenosis without needing sternotomy. Surgical valvuloplasty can be successfully performed in patients in whom the expected benefit from aortic balloon valvuloplasty is not achieved.

Technical Modifications That Might Improve Long-term Outcomes of the Ross Procedure in Children

BackgroundFailure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children. MethodsSixty-nine patients (median age, 12 years; range, 0.25-17.9) underwent the RP between January 1996 and December 2018. A concomitant Konno procedure was performed on 20 of 69 patients (29%). Prior interventions included balloon valvuloplasty in 30 (44%) and/or surgical valvuloplasty in 39 (57%). Technical modifications included using the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction, and wrapping the native root remnant around the PVA. ResultsOperative mortality was 1 of 69 patients (1.5%), with no late deaths. No patient had neoaortic valvar stenosis and 7 of 68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median, 9.4 years; range, 0.4-21.3) there was no significant change in Z scores of annulus, sinus, or sinotubular junction diameters when compared with those at discharge. Three patients (4.4%) required late autograft replacement, 2 PVA repair, and 2 resection of a pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 years. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80%, respectively. ConclusionsTechnical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients.

Prosthetic repair of aortic valve cusps with autopericardium in children. State of the art and prospects

According to the results of modern researchers, the main techniques used in congenital pathology of the aortic valve in children include balloon catheter dilatation of the aortic valve, surgical valvuloplasty, the Ross procedure and replacement of the aortic valve with a mechanical prosthesis. Many surgeons point out that these techniques in congenital pathology of the aortic valve yield suboptimal results. This is often due to the lack of a clear-cut definition between surgeons as to what operation should be performed in a particular age group. According to the reports of the majority of researchers, biological prostheses undergo early degeneration and structural changes in paediatric cardiac surgery and yield the worst results. Comparing the main techniques, optimal haemodynamics is observed after the Ross procedure. A disadvantage of this operation is the necessity of repeat intervention on the right ventricular outflow tract, which is required in 20 to 40%. Concomitant surgery of the mitral valve and/or aortic arch during the Ross procedure significantly increases the lethality and the risk of postoperative complications. Compared with an adult cohort of patients, children after prosthetic repair of the aortic valve using a mechanical prosthesis are more often found to have postoperative complications and a higher mortality rate. Yet another problem encountered in paediatric valve surgery is the unavailability of commercial prostheses sized ?19 mm. The duration of the intraoperative parameters for reconstructions of the aortic valve, the Ross procedure, and replacement of the aortic valve by the results of many studies averagely amounts to 74±34 min, 100±56 min, and 129±71 min, respectively. Yet another method which can be used for neocuspidization of the aortic valve in reconstructive surgery of the aortic root in paediatric patients is the use of glutaraldehyde-treated autologous pericardium. In our opinion, given the simplicity of the procedure, duration of the intraoperative parameters, and acceptable initial results reported by some researchers, the Ozaki procedure may be performed in children.

Long-term outcome of percutaneous balloon aortic valvuloplasty for children with congenital aortic valve stenosis

Objective: To assess the efficacy and long-term outcome of percutaneous balloon aortic valvuloplasty (PBAV) for children with congenital aortic stenosis (CAS) and to explore risk factors for significant aortic regurgitation (AR) and reintervention after PBAV during follow up. Methods: This was a retrospective study. Children (≤18 years old) with CAS, who underwent PBAV in Guangdong Provincial Hospital from January 2004 to December 2018, were included in this study. Demographic, preoperative transthoracic echocardiography (TTE) and surgical data were collected. Postoperative complications were closely observed, and the patients were followed up at 1, 6, 12 months after the operation, and then at one year interval thereafter. Endpoint events included significant AR and reintervention. Reintervention was defined as any intervention that needed to be performed on the valve for various reasons, including re-PBAV, surgical valvuloplasty and valve replacement. Significant AR was defined as AR grade≥3 by TTE criteria. The results of the last TTE examination before the end of the study were collected. The Kaplan-Meier curve for long-term AR-free and intervention-free survival was plotted. Cox regression model was used to further analyze the risk factors for significant AR and reintervention after PBAV in CAS patients. Results: A total of 55 patients were enrolled in this study, and the age was 4.6(1.6, 6.5) years, with 37(67.3%) males. The peak systolic valve gradient fell from (80.3±30.6)mmHg to (38.5±18.5)mmHg(P<0.001, 1 mmHg=0.133 kPa). Surgical success rate was 89% (49/55). Acute post-PBAV AR occurred in 18 patients, including 3 patients with RA≥3 grade. In-hospital complications occurred in 6 patients (2 deaths, 2 cases of transient arrhythmia, and 2 cases of femoral artery embolization). Fifty patients accomplished the follow-up and the follow-up time was 6.2(3.4, 8.5) years. Significant AR was found in 20 patients. Significant AR-free survival rate was 53% at 5 years and 19% at 10 years. Reintervention was performed in 11 patients (4 with valvuloplasty and 7 with valve replacement), and the 5-year and 10-year intervention-free survival rates were 87% and 62%, respectively. Multivariate Cox regression analysis showed that acute post-PBAV AR was a risk factor for long-term significant AR (HR=2.398, 95%CI 1.007-5.712, P=0.048). Post-PBAV residual pressure gradient ≥ 35 mmHg (HR=4.747, 95%CI 1.116-19.329, P=0.030)and acute post-PBAV AR (HR=5.104, 95%CI 1.083-24.065, P=0.039)were risk factors for re-intervention. Conclusions: PBAV is safe and effective in the treatment of CAS in children, but attention should be paid on significant AR post procedure. Acute post-PBAV AR is a risk factor for re-intervention and significant AR post PBAV, and high post-PBAV residual pressure gradient is a risk factor for re-intervention.

Surgical Valvuloplasty Versus Balloon Dilation for Congenital Aortic Stenosis in Pediatric Patients.

For children with congenital aortic stenosis (AS) who are candidates for biventricular repair, valvuloplasty can be achieved by surgical aortic valvuloplasty (SAV) or by transcatheter balloon aortic dilation (BAD). We aimed to evaluate the longer term outcomes of SAV versus BAD at our institution. We retrospectively reviewed the outcomes of 2 months to 18 years old patients who underwent SAV or BAD at our institution between January 1990 and July 2018. Baseline and follow-up characteristics were assessed by echocardiography. Long-term survival, freedom from reintervention, freedom from aortic valve replacement (AVR), and aortic regurgitation were evaluated. A total of 212 patients met inclusion criteria (SAV = 123; BAD = 89). Age, sex, aortic insufficiency (AI), and aortic valve gradient were similar between the groups. At 10 years, 27.9% (19/68) of SAV patients and 58.3% (28/48) of BAD patients had moderate or worse AI (P = .001), and reintervention occurred in 39.2% (29/74) of SAV patients and 78.6% (44/56) of BAD patients (P < .001). Kaplan-Meier analysis revealed overall survival was 96.8% (119/123) for SAV and 95.5% (85/89) for SAV (P = .87). At 10 years, 35% (23/66) of SAV patients and 54% (23/43) of BAD patients underwent AVR (P = .213). Surgical aortic valvuloplasty demonstrated greater gradient reduction, less postoperative and long-term AI, and a lower reintervention rate at 10 years than BAD. There was no difference in survival or AVR reintervention rate. Surgical aortic valvuloplasty is a durable and efficacious intervention and should continue to be considered a favorable choice for palliation of valvular AS.

Abstract 266: Incidence, Predictors and Outcomes of Permanent Pacemaker Implantation Among Patients With Bradyarrhythmias Following Cardiac Surgery: Insights From the National Inpatient Sample Database

Introduction: Significant atrioventricular blocks and bradyarrhythmias are known complications of open-heart surgery. These are frequently transient, however, some patients go on to need a permanent pacemaker (PP). We sought to describe the incidence, predictors, and outcomes of PP implantation among patients admitted for cardiac surgery who develop bradyarrhythmias. Methods: We queried the National Inpatient Sample (NIS) database from 2010 to 2014 for adults admitted for surgical valve replacement, valvuloplasty or coronary artery bypass grafting (CABG) who had bradyarrhythmias during the admission using the appropriate ICD codes. We identified patients who had permanent pacemaker implantation documented during the admission. Categorical and continuous variables were compared using the chi-square and student's t-test. Predictors of PP implantation and in-hospital mortality were evaluated by logistic regression. Results: Of the 1402930 patients who underwent cardiac surgery, 94748 patients had bradyarrhythmias defined as sinoatrial node dysfunction (SND) and/or atrioventricular block (AVB) during hospitalization. The primary procedure was identified as valve replacement in 50.3% (47615 of 94748), CABG in 29.9% (27622 of 94748) and valvuloplasty in 8.7% (8248 of 94748). SND was found in 29.9% (28372 of 94748) and AVB in 76% (72017 of 94748). Permanent pacemaker implantation was done in 39.3% (37246 of 94748). Valve replacement was the most common surgery associated with PP implantation [58% (21682 of 37246) compared to 21.5% in CABG (8007 of 37246) and 7.7% in valvuloplasty (2882 of 37246), p &lt; 0.001). Female sex aOR 1.36 (95% CI 1.31 - 1.40), young age 18 - 44 years aOR 1.36 (95% CI 1.24 - 1.49), Asiatic and Hispanic origin aOR 1.36 (95% CI 1.23 - 1.51), aOR 1.25 (95% CI 1.17 - 1.34) respectively, diabetes mellitus with chronic complications aOR 1.16 (95% CI 1.09 - 1.24), drug abuse aOR 1.38 (95% CI 1.21 - 1.55) were associated with higher odds of pacemaker implantation. African American origin aOR 0.79 (95CI 0.74 - 0.85), AIDS aOR 0.33 (95% CI 0.17 - 0.67), south hospital region aOR 0.89 (95% CI 0.85 - 0.93), no-charge admissions aOR 0.66 (95% CI 0.49 - 0.89) were associated with a lower odds of PPM implantation. Death during hospitalization was found in 3% of the patients. After multivariable regression, PP implantation was associated with a lower likelihood of in-hospital death aOR 0.45 (95% CI 0.41 - 0.50). Conclusion: Approximately one-third of the patients hospitalized for cardiac surgery related to AVB and/or SND were implanted a permanent pacemaker. Factors like age, sex, race and comorbidities determine the likelihood of this procedure that has a significant impact on mortality. Having a better insight into these predictors would allow a better triage of patients who would benefit from its implantation.

Specific features and survival of French bulldogs with congenital pulmonic stenosis: a prospective cohort study of 66 cases

IntroductionThe objectives of this study were to characterize the epidemiological, clinical, and echocardiographic features of French bulldogs (FBs) with congenital pulmonic stenosis and document their survival times and risk factors for cardiac death (CD). AnimalsThis study included 66 FBs with congenital pulmonic stenosis. MethodsProspective cohort study including a survival analysis to assess time to CD. ResultsIn most cases (53/66, 80%), at least two obstructive lesions were observed, most commonly valvular and supravalvular (42/66, 64%), with pulmonary trunk hypoplasia in 40/66 (61%) of cases. The median Doppler-derived peak trans-stenotic pressure gradient (ΔP) was very high: 170 mmHg (range = 34–291 mmHg), with ΔP ≥ 200 mmHg in 33% of FBs. Among the 51 FBs with an available follow-up and that did not undergo surgical valvuloplasty, 21/51 (41%) died, 67% (14/21) of deaths being CD. The median survival time from diagnosis to CD was 2.8 years (interquartile range = 0.8–4.6 years). Univariate Cox proportional hazard analyses revealed that age (hazard ratio [HR] = 2.3 per 1 year increase; p = 0.02), clinical signs at presentation (HR = 3.7; p = 0.03), ΔP (HR = 1.2 per 10 mmHg increase; p = 0.01), right ventricular dilation (HR = 5.0; p = 0.04), severe tricuspid regurgitation (HR = 7.6; p = 0.001), and right-sided congestive heart failure (HR = 4.8; p = 0.05) were associated with time to CD. After adjustment for age and ΔP, tricuspid regurgitation remained significantly associated with time to CD (HR = 5.1; p = 0.02). ConclusionsPulmonic stenosis in FBs is commonly severe and complex, with at least 2 obstructive lesions in most cases, a high incidence of pulmonary trunk hypoplasia and CD, and strong association between prognosis and tricuspid regurgitation severity.

Fatal heart failure caused by severe pulmonary regurgitation, tricuspid regurgitation and late-onset mitral stenosis in an adult patient with Noonan syndrome: a case report

BackgroundIn patients with Noonan syndrome (NS), cardiac disorders such as pulmonary valve stenosis (PS) or hypertrophic cardiomyopathy (HCM) are common. While some patients can develop heart failure associated with HCM, the long-term outcome of adult patients with NS is reported to be good. Fatal outcomes of heart failure in patients with NS but without HCM are rare.Case presentationWe report a 25-year-old Japanese woman diagnosed with NS in adulthood. She exhibited short stature and minor facial dysmorphism and was diagnosed with PS at 1 year of age. After surgical valvuloplasty for PS at 6 years of age, her general condition became stable without specific medical treatment. She discontinued regular medical follow-up for PS. At 21 years of age, she developed acute decompensated heart failure, which was mainly right-sided heart failure due to severe pulmonary regurgitation (PR) and tricuspid regurgitation (TR). There was no evidence of HCM or PS recurrence. On the basis of the history of PS and characteristic physical features including short stature, webbed neck, and hypertelorism, she was clinically diagnosed with NS. At 25 years of age, she developed heart failure of both sides due to PR, TR and late-onset severe mitral stenosis (MS). The etiology of MS was uncertain. Owing to the patient’s condition, surgical options were considered to be extremely high risk. She was treated with optimal medical treatment as well as the occasional abdominal cavity drainage for recurrent ascites; however, she died of decompensated heart failure at 27 years of age.ConclusionsWe describe an adult patient with NS without HCM who died of heart failure caused by severe PR, TR and MS. Clinicians should recognize that ongoing or late-onset cardiac disorders can develop in patients with NS, and lead to fatal heart failure. Optimal medical follow-up to monitor cardiac function and early identification of heart failure are important.

Comparison of Balloon Dilatation and Surgical Valvuloplasty in Non-critical Congenital Aortic Valvular Stenosis at Long-Term Follow-Up

The two main modalities used for congenital aortic valvular stenosis (AVS) treatment are balloon aortic valve dilatation (BAD) and surgical aortic valvuloplasty (SAV). This study evaluates residual and recurrent stenosis, aortic regurgitation (AR) development/progression, reintervention rates, and the risk factors associated with this end point in patients with non-critical congenital AVS who underwent BAD or SAV after up to 18years of follow-up. From 1990 to 2017, 70 consecutive interventions were performed in patients with AVS, and 61 were included in this study (33 BADs and 28 SAVs). There were no significant differences in age, sex distribution, PSIG, and AR frequency between the BAD and SAV groups. Bicuspid valve morphology was more common in the BAD group than the SAV group. There was no statistically significant difference between PSIGs and AR development or progression after intervention at the immediate postoperative echocardiography of patients who underwent BAD or SAV (p = 0.82 vs. p = 0.29). Patients were followed 6.9 ± 5.1years after intervention. The follow-up period in the SAV group was longer than that of the BAD group (9.5 ± 5.4 vs. 5.5 ± 4.4 years, p = 0.003). There was no statistically significant difference in the last echocardiographic PSIG between patients who underwent SAV or BAD (51.1 ± 33.5 vs. 57.3 ± 35.1, p = 0.659). Freedom from reintervention was 81.3% at 5years and 57.5% at 10years in the BAD group and 95.5% at 5years and 81.8% at 10years in the SAV group, respectively (p = 0.044). There was no difference in postprocedural immediate PSIG and last PSIG at follow-up and the development/progression of AR between patients who were treated with BAD versus SAV. However, long-term results of SAV were superior to those of BAD, with a somewhat prolonged reintervention interval.

Congenital aortic stenosis: treatment outcomes in a nationwide survey

Objective. To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. Design. Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000–2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR. Results. Sixty-one patients underwent either BAV (n = 54) or surgical valvuloplasty (n = 7) for valvar AS at a median age of 29 days (range 6 hours to 16.9 years). The proportion of patients not requiring reintervention at 1, 5, and 10 years was 61%, 50%, and 29% in neonates and 83%, 73%, and 44% in older patients, respectively (p = .02); without difference between treatment groups. Larger proportion of patients remained free from valve surgery after optimal BAV result than after adequate or inadequate result (p = .01). The reason for the first reintervention was AS in 50%, AR in 36%, and combined aortic valve disease in 16% of cases. Early mortality (before hospital discharge) was 4.9%, and associated with critical AS in neonates. There was no late mortality during the follow-up. Conclusions. Although majority of congenital AS patients require more than one intervention during childhood, an optimal BAV result improves long-term outcome by increasing the proportion of patients remaining free from valve surgery. High long-term freedom from reintervention is attainable also in the neonatal population.

Comparison of transthoracic or percutaneous balloon pulmonary valvuloplasty in the therapy of pulmonary valve stenosis

Objective: To compare the clinical outcome of patients with pulmonary valve stenosis underwent transthoracic and percutaneous balloon pulmonary valvuloplasty. Methods: Clinical data of 806 patients diagnosed as pulmonary valve stenosis underwent transthoracic(171 patients as group A)or percutaneous balloon pulmonary valvuloplasty (635 patients as Group B) in Fuwai Hospital from February 2006 to January 2016 were analyzed retrospectively. There were 72 males in group A (42.1%) and 344 males in group B (54.2%). The average age was (1.6±1.1) years in group A and (21.0±18.5) years in group B. The median weight was 7.65 (7.68) kg (M(Q(R))) in group A and 43.75 (47.38) kg in group B. There were 732 (90.9%) patients followed up from 3 months to 10 years, with an average interval of (6.3±3.6) years. Sixty cases were ligated patent ductus arteriosus simultaneously, and 20 cases got Blalock-Taussig shunt at the same time of valvuloplasty in group A. There were 47 cases of transcatheter closure of atrial septal defect and 6 cases of transcatheter closure of patent ductus arteriosus in group B. The t test, rank sum test and χ(2) test were used to compare data of two groups. Results: There were no hospital death or cardiac tamponade and other serious complications for all patients. The postoperative hospital stayin group A was significantly longer than that in group B (8(5) days vs. 2(2) days, Z=-9.404, P=0.000). In every further consultation, patients were reviewed with transthoracic echocardiography to assess transpulmonary gradient and pulmonary regurgitation. There were significant difference between group A and B of preoperative transpulmonary pressure gradient ((80.6±22.4) mmHg vs.(72.6±20.5) mmHg, t=1.611, P=0.032, 1 mmHg=0.133 kPa) and so as transpulmonary pressure gradient reduction value ((55.9±21.0) mmHg vs. (46.6±23.4) mmHg, t=-1.710, P=0.026). Patients in both groups had good cardiac function during follow-up interval. One patient needed surgical valvuloplasty 10 months after percutaneous balloon pulmonary valvuloplasty and 1 case occurred moderate to severe tricuspid regurgitation in group B. During follow-up period, there was no significant difference between group A and B of transpulmonary pressure gradient ((22.3±6.5) mmHg vs. (25.2±12.6) mmHg, t=1.320, P=0.072), the incidence of pulmonary valve regurgitation in patients of group A was significantly lower than patients of group B (56.1% vs.65.2%, χ(2)=4.755, P=0.029). Conclusions: The clinical outcome and complications are similar between patients underwent two different routes of balloon pulmonary valvuloplasty. Transthoracic balloon pulmonary valvuloplasty is more suitable for infant and underweight children patients with pulmonary valve stenosis. Percutaneous balloon pulmonary valvuloplasty is more suitable for the treatment of the elder children or adults.

Insuffisance cardiaque par rétrécissement tricuspidien provoqué par une sonde d’électrostimulation. À propos d’un cas

Tricuspid stenosis (TS) is an uncommon complication of ventricular pacemaker implantation. Mechanisms described by the literature are ventricular inflow obstruction by tricuspid vegetations (endocarditis) or multiple pacemaker leads and fibrosis secondary to mechanical trauma, accounting for perforation or laceration of the TV leaflets, or adherence between redundant loops and valve tissue. We present the case of iatrogenic tricuspid stenosis, observed in a 77-year-old man. Extrinsic tricuspid valve stenosis was detected by transthoracic echocardiography. Further investigations confirmed the intramyocardial lead position. Tricuspid valve stenosis due to transvenous leads are reported to be treated by surgical replacement, surgical valvuloplasty, or percutaneous balloon valvuloplasty.

Surgical Aortic Valvuloplasty Versus Balloon Aortic Valve Dilatation in Children.

Balloon aortic valve dilatation (BAD : is assumed to provide the same outcomes as surgical aortic valvuloplasty (SAV). However, the development of precise modern surgical valvuloplasty techniques may result in better long-term durability of the aortic valve repair. This review of the recent literature suggests that current SAV provides a safe and durable repair. Furthermore, primary SAV appears to have greater freedom from reintervention and aortic valve replacement when compared to BAD.