scholarly journals Natural Course of Isolated Pulmonary Valve Stenosis in Pediatric Patients

2020 ◽  
Vol 3 (2) ◽  
pp. 01-07
Author(s):  
Güven Serçin ◽  
Öztarhan Kazım

Aim: The aim of our study is to be able to predict the prognosis of patients with isolated pulmonary valvular stenosis on the basis of age and degree of stenosis. Identification of the course of pulmonary stenosis of different age groups will significantly contribute both to the physicians and the relatives of the patient. Material and Methods: 105 pediatric patients diagnosed with isolated pulmonary valvular stenosis were included in our study. We investigated the impact of the gradient of stenosis and the age at the time of diagnosis on the natural course of pulmonary stenosis. Mean follow-up time of the children was 19 months, 25.45±22.48 months. The patients were divided into four groups over their trans-valvular gradient degrees and<20 mmHg was defined as transient, 20-39 mmHg mild, 40-59 mmHg moderate, 60 mmHg and over as severe pulmonary stenosis. Results: Between two to five months, none of the moderate stenosis cases progressed unlike other patient groups. The decline in the final gradient versus initial gradient was significant in children between two to five months and six months to two years in our study, and yet there was no significant change of initial and final gradients in patients under one month, and at two years and over. Conclusion: It would be reasonable to conclude that the progression of pulmonary valvular stenosis is benign in patients with pulmonary valvular stenosis under 40 mmHg of systolic gradient diagnosed after 6th month of life.

2014 ◽  
Vol 142 (9-10) ◽  
pp. 542-546
Author(s):  
Vojislav Parezanovic ◽  
Milan Djukic ◽  
Sanja Dzelebdzic ◽  
Tamara Ilisic ◽  
Igor Stefanovic ◽  
...  

Introduction. Pulmonary artery stenosis (PS) is a congenital heart defect which occurs in 10% of all congenital heart defects. Pulmonary balloon valvuloplasty (BVP) has been the treatment of choice of PS over the last 30 years. Objective. The purpose of this study was to evaluate the efficacy of this method based on middle-term hospital follow-up, and safety of BVP based on our experience. Methods. The study included 88 patients diagnosed with PS. The patients were divided into three groups based on the severity of the disease. Also, they were divided into two age groups in order to analyze the frequency of complications. Hemodynamic measurements and echocardiography results were recorded before, 24-36 hours after BVP and at the end of follow-up. Results. The studied group involved patients of average age 3.75?4.3 years (20 days to 17 years). Immediately after BVP a significant decrease of pressure gradient across the pulmonary valve (PV) was recorded in all patients; this result was similar in all 3 groups of patients regardless of the severity of stenosis (p<0.001). Complications of BVP occurred most commonly in children up to 12 months of age (ventricular tachycardia 4.5% and supraventricular tachycardia 6.8%). Pulmonary valve insufficiency after dilatation occurred in 6.6% of cases, and was most common in children aged up to 12 months. In 87 (98.9%) patients BVP was a definitive solution, and a significant residual stenosis was not recorded during follow-up. Conclusion. BVP is a safe and effective procedure in the treatment of isolated PS in children, regardless of the severity of stenosis but also regardless of patients? age.


2015 ◽  
Vol 3 (1-2) ◽  
pp. 53-58
Author(s):  
Tahera Nazrin ◽  
CM Shaheen Kabir ◽  
Sahela Nasrin ◽  
Md Rokonujjaman ◽  
M Maksumul Huq ◽  
...  

Congenital pulmonary valve stenosis is a common congenital heart disease andIsolated pulmonary valve stenosis comprises 8-10% of all congenital heart disease. It is an acyanotic heart disease,but can present with severe cyanosis if it is associated with patent foramen ovale (PFO). Severe pulmonary stenosis with cyanosis can be misdiagnosed clinically. Proper evaluation and modern technique of treatment modality can save a life easily. We report a 5 years old boy with severe pulmonary valvular stenosis with PFO who was clinically misdiagnosed as a case of congenital cyanotic heart disease (Tetralogy of Fallot). After taking proper history, clinical examination and investigations we treated the baby by balloon pulmonary valvuloplasty (BPV) successfully without any complication. The short term (6 months) outcome of BPV showed excellent result. Ibrahim Cardiac Med J 2013; 3(1&2): 53-58


2017 ◽  
Vol 27 (8) ◽  
pp. 1566-1570 ◽  
Author(s):  
John J. Parent ◽  
Michael M. Ross ◽  
Edgard A. Bendaly ◽  
John P. Breinholt

AbstractBackgroundPulmonary balloon valvuloplasty is a safe and effective treatment for children with pulmonary valve stenosis. A few studies evaluate the long-term outcomes of the procedure, particularly the degree of pulmonary regurgitation. We evaluated the outcomes of children >1 year following valvuloplasty for pulmonary valve stenosis.MethodsA retrospective analysis of children with pulmonary valve stenosis following pulmonary balloon valvuloplasty at a single institution was performed. Clinic summaries, catheterisation data, and echocardiographic data were reviewed. Inclusion criteria were isolated pulmonary valve stenosis, age <19 years at the time of intervention, and at least one echocardiogram performed at least 1 year after valvuloplasty.ResultsA total of 53 patients met inclusion criteria. The median age at valvuloplasty was 0.4 years (0.01–10.6 years). The last follow-up was 4.8±2.3 years following valvuloplasty. The pre-valvuloplasty peak instantaneous gradient by echocardiography was 60.6±14.6 mmHg. The peak gradient at the first postoperative echocardiography was reduced to 25.5±12 mmHg (p<0.001), and further decreased to 14.8±15.8 mmHg (p<0.001) at the most recent follow-up. The degree of regurgitation increased from before valvuloplasty to after valvuloplasty (p<0.001) but did not progress at the most recent follow-up (p=0.17). Only three patients (5.7%) required re-intervention for increasing pulmonary stenosis (two surgical; one repeat balloon). No significant procedural complications occurred.ConclusionsPulmonary balloon valvuloplasty remains a safe and effective treatment for children with isolated pulmonary valve stenosis, with excellent long-term outcomes and no mortality. A few patients require further intervention. Long-term follow-up demonstrates decreased, residual stenosis. Patients have a small, acute increase in pulmonary regurgitation following valvuloplasty, but no long-term progression.


Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi

Abstract OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Behnood Bikdeli ◽  
David Jimenez ◽  
Jorg Del Toro ◽  
Gregory Piazza ◽  
Augussina Rivas ◽  
...  

Background: Atrial fibrillation (AF) may occur prior to or early in the course of acute pulmonary embolism (PE). The impact of AF on outcomes of patients with PE remains uncertain. Methods: Using the data from a large prospective multicenter registry of patients with objectively-confirmed PE (04/2014 to 01/2020), we identified three patient groups: 1) those with pre-existing AF 2) patients with newly identified AF within 2 days from the index PE (incident AF) and 3) patients without AF. We assessed the 90-day and 1-year risk of mortality and stroke in patients with AF, in unadjusted and multivariable adjusted models considering those without AF as referent. Results: Among 16,497 patients with PE, 792 had pre-existing AF. Compared with those without AF, patients with pre-existing AF, had increased odds of 90-day all-cause (Odds ratio [OR]: 2.81 (95% confidence interval [CI]: 2.33-3.38) and PE-related mortality (OR: 2.38, 95% CI: 1.37-4.14). After multivariable adjustment, pre-existing AF significantly increased the odds of all-cause mortality (OR: 1.91, 95% CI: 1.57-2.32) but not PE-related mortality (OR: 1.50; 95% CI: 0.85-2.66). Pre-existing AF was associated with increased hazard for ischemic stroke at 1-year follow-up (hazard ratio [HR]: 5.48; 95% CI: 3.10-9.69). Among 16,497 patients with PE, 445 developed incident AF within 2 days of acute PE. Incident AF was associated with increased odds of 90-day all-cause (OR: 2.28; 95% CI: 1.75-2.97) and PE-related (OR: 3.64; 95% CI: 2.01-6.59) mortality. Findings were similar in multivariable analyses and at 1-year follow-up (Figure). No patients with incident AF developed ischemic stroke. Conclusion: In patients with acute symptomatic PE, both pre-existing AF and incident AF predict an adverse clinical course, although the type of adverse outcomes may be different depending on the timing of AF onset.


2021 ◽  
Author(s):  
Sonia B. Albanese Albanese

Background: Surgical treatment of isolated pulmonary valve stenosis in infants and children has evolved over the years, shifting from the original exclusive aim at lowering right ventricle pressure to the current concomitant focus on preserving pulmonary valve function. In our study, we sought to analyze the effect of such evolving philosophy on long-term results. Methods: All consecutive patients treated in our center between July 1983 and March 2019 were included. Patients were categorized into 2 groups based on the introduction into treatment practice of sparing valve techniques (1995). Actuarial survival, freedom from reintervention on the right outflow tract, transvalvular mean pressure gradient decrease, and pulmonary valve function at follow-up were analyzed. Results: One hundred twenty-three patients operated before (Group I, n=81) or since (Group II, n=42) 1995 were enrolled in the study. Mean age and weight were 3.0 ± 0.36 years and 16.6 ± 1.7kg, respectively. Early mortality occurred exclusively in 3 patients of Group 1. Transvalvular mean pressure gradient decreased in the entire patient population (from 63.28 ± 12.9mmHg to 16.46 ± 7.9mmHg). At a mean follow-up interval of 4.9 ± 33 years, freedom from death was comparable, but freedom from right ventricular outflow tract reintervention was significantly greater in Group II. Although the transvalvular gradient remained stable over time in non-reoperated survivors (mean value of 16.46 ± 7.9mmHg), pulmonary valve function on 2D-Echo showed severe incompetence in 2 patients of Group I and just mild to moderate incompetence in 20 patients of Group II, with a significantly negative effect of unsuccessful preoperative pulmonary balloon valvuloplasty (14/20 vs 6/20, p=0.025) in the latter. Conclusion: Current pulmonary valve sparing techniques are associated with better results, particularly in terms of freedom from re-interventions and pulmonary valve function at follow-up. Balloon valvuloplasty prior to surgery may worsen operative results, promoting pulmonary insufficiency and therefore should probably be avoided in all patients in whom anatomical characteristics predict failure of percutaneous therapy.


1988 ◽  
Vol 12 (2) ◽  
pp. 476-479 ◽  
Author(s):  
Pablo M. Marantz ◽  
James C. Huhta ◽  
Charles E. Mullins ◽  
Daniel J. Murphy ◽  
Michael R. Nihill ◽  
...  

2006 ◽  
Vol 16 (3) ◽  
pp. 314-315
Author(s):  
S. Viswanathan ◽  
K. English ◽  
M. E. C. Blackburn

Introduction: Repair of Tetralogy of Fallot up until recent decades involved aggressive resection and annular enlargement through a right ventriculotomy. This resulted in ventricular scarring and pulmonary incompetence, with an increased risk of ventricular tachyarrhythmia and sudden death in young adulthood. Following the NICE guidelines, implantation of ICDs as primary prevention in patients with repaired Tetralogy is ever increasing. This study aims to determine the rate of appropriate and inappropriate discharges, the success rate of ICD therapy and the impact of ICD implantation on the use of anti-arrhythmic medication in this population of patients. Materials and Methods: This is a retrospective review of patients with repaired Tetralogy of Fallot (n = 18) and pulmonary stenosis (n = 2) with implantable cardioverter defibrillators managed at our tertiary centre. Patients were identified from our outpatient database, their notes and charts were examined and details regarding indication for ICD implantation, device specifications and complications following implantation were collected. Data was also collected on the incidence of appropriate and inappropriate therapies and the success rate of ICD therapy along with the impact of implantation on the usage of anti-arrhythmic medication in these patients. Results: Of the 20 patients, 18 had previous repair of Tetralogy of Fallot and 2 had pulmonary valvotomy and infundibular resection for pulmonary stenosis between 1969 and 1989. 70% (n = 14) of these patients required reoperation with 10 patients having pulmonary valve replacements (PVR), 3 having redo infundibular resections and 1 requiring aortic valve replacement. At the time of consideration for ICD implantation 80% had moderate to severe pulmonary incompetence and 60% had more than mild right ventricular dilatation on echocardiography. Indications for ICD implantation were symptomatic ventricular tachycardia requiring cardioversion (n = 8), ventricular tachycardia on 24 hr tape/Reveal or electrophysiological study (n = 8), ventricular fibrillation (VF)/pulseless ventricular tachycardia (VT) (n = 2) and syncope with an abnormal EPS other than VT (n = 2, high grade ventricular ectopics, sinus node dysfunction).The median age at implantation was 22 years (16.4–43 years). All our patients had dual chamber devices implanted with either dual (n = 13) or single coil (n = 6) ventricular leads. GEM3 AT (n = 5), Marquis DR (n = 8) and Maximo DR (n = 7) generators (Medtronic Inc.) were implanted in sub pectoral position and both anti-tachycardia pacing and cardioversion modes were programmed as part of individualised VT and VF protocols. Early post procedural complications included atrial lead displacement (n = 1) and pneumothorax requiring drainage (n = 1).During a median follow up of 1.6 years (0.03– 4.5 years) several episodes of inappropriate therapies were noted in 6 patients (30%) especially early after implantation. This was found to be mainly due to atrial tachyarrhythmia, double counting of T waves or inaccurate interpretation of varying PR intervals as AV dyssynchrony which were effectively dealt with by changes in device programming. There were 33 episodes of inappropriate anti-tachycardia pacing (ATP) in 4 patients and 19 episodes of inappropriate cardioversion in 5 patients. Appropriate ATP was instituted in 4 patients (25%) with successful termination of all 20 episodes (100% success rate) of ventricular tachycardia. One patient required cardioversion with successful termination of VF. One patient (5%) with troublesome tachyarrhythmia died suddenly of unknown cause, 10 months after AICD implantation having had no detections or therapies on his device.Prior to ICD implantation 8 patients were on amiodarone therapy. At the time of last follow up after AICD implantation all patients were established on anti-arrhythmic agents and of these 6 patients were on amiodarone with the others being effectively managed on beta-blockers and/or flecainide.Late complications of ICD implantation included lead failure in 1 patient requiring replacement 3.3 years after implantation and generator replacement in a patient who was pacemaker dependent a year after implantation due to an advisory issued by the manufacturer regarding the risk of sudden battery depletion. Conclusions: In our study we found a rate of 0.6 appropriate and 1.4 inappropriate therapies (0.9 episodes of inappropriate ATP and 0.5 episodes of inappropriate cardioversion) per patient-year of follow up following ICD implantation which is in keeping with published literature. The mortality in our study group was 5% which is acceptable given the high risk population. Implantation of an ICD allowed switching over from amiodarone to less toxic anti arrhythmic therapy in a proportion of patients. Anti-tachycardia pacing was very successful in terminating tachyarrhythmia in our population with 100% success in terminating ventricular tachycardia.


2017 ◽  
Vol 27 (7) ◽  
pp. 1314-1322
Author(s):  
Petra Loureiro ◽  
Barbara Cardoso ◽  
Inês B. Gomes ◽  
José F. Martins ◽  
Fátima F. Pinto

AbstractIntroductionPercutaneous balloon valvuloplasty is the primary treatment for critical pulmonary valve stenosis in neonates. Thus far, a few studies have reported long-term results of this technique in neonatal critical pulmonary valve stenosis.MethodsWe carried out a retrospective study of all consecutive newborns with critical pulmonary valve stenosis subjected to percutaneous balloon valvuloplasty at a single centre, between 1994 and 2014, to assess its immediate and long-term safety and efficacy.ResultsA total of 24 neonates presented with critical pulmonary valve stenosis. The mean diameter of the pulmonary annulus was 7 mm (±1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment. Percutaneous balloon valvuloplasty was performed at a mean age of 4.0±4.3 days using, on average, a balloon-to-pulmonary annulus ratio of 1.18 mm (with a range from 0.9 to 1.43). Immediate success was achieved in 22/24 patients (92%) with a reduction in the pulmonary transvalvular peak gradient (p<0.05) and in the right ventricle/systemic pressure ratio (p<0.05). There was one death (4%) 6 days after the procedure, and 29.2% of them had transient rhythm complications. For a mean follow-up time of 8.4 years, the re-intervention rate was 42.9%. In total, 14 re-interventions were performed in nine neonates, including surgery in six. Freedom from re-intervention was 50% at 8 years and 43% at 10 and 15 years.ConclusionThis series, to the best of our knowledge, has had the longest follow-up of neonates with critical pulmonary valve stenosis. Percutaneous balloon valvuloplasty is a safe and effective treatment, and in our study 75% of the patients were exclusively treated using this technique.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 840-840
Author(s):  
Rachael E. Hough ◽  
Clare Rowntree ◽  
Rachel Wade ◽  
Nicholas Goulden ◽  
Chris Mitchell ◽  
...  

Abstract Despite the substantial improvements made in the outcomes of paediatric ALL, with ‘cure' rates now in excess of 90%, survival in teenage and young adult (TYA) patients has remained inferior. The reasons for this are likely multifactorial, including tumour biology, toxicity, compliance, access to clinical trials and protocol (adult or paediatric) used. We report the toxicity profiles observed in children, teenagers and young adults treated on the UK intensive, minimal residual disease (MRD) directed ALL protocol, UKALL2003. Of a total of 3126 patients treated, 1520 patients were under 5 years old, 767 were aged 5-9 years, 610 aged 10-15 years and 229 aged 16-24 years, with a median overall follow-up of 4 year and 10 months. The risk of serious adverse events (SAEs) was higher in patients older than 10 years (56% in 10-15 year olds, 53% in 16-24 year olds) compared to those aged 9 or younger (30% in under 5 years and 31% in 5-9 years)(p<0.0001), with no difference in the those aged 16-24 compared to younger teenagers (p=0.5). The incidence (per number of patients in each group) and distribution of toxicities according to age group is summarised in the table.Table 1Age in years<55-910-1516-24AllTotal number of patients1520767610229 NB: 56 pts≥20 years3126Infection n (%)328 (21.6%)130 (17.0%)145 (23.8%)72 (31.4%)675 (21.6%)Asaparaginase n (%)57 (3.8%)57 (7.4%)64 (10.5%)31 (13.5%)209 (6.7%)Methotrexate n (%)100 (6.6%)74 (9.6%)123 (20.2%)33 (14.4%)330 (10.6%)Steroid n (%)54 (3.6%)37 (4.8%)141 (23.1%)52 (22.7%)284 (9.0%)Vincristine n (%)34 (2.2%)11 (1.4%)22 (3.6%)7 (3.0%)74 (2.4%)Other SAEs94 (6.2%)42 (5.5%)90 (14.8%)25 (10.9%)251 (8.0%) The incidence of certain toxicities including viral infection (5.3%), asparaginase hypersensitivity (1.9%) and vincristine neurotoxicity (2.1%) appeared equivalent across all age groups. Avacular necrosis was seen predominantly in adolescents (83% of 147 events in 10-19 year olds) and was rare in those younger than 10 years (n=18) or older than 20 years (n=7). Asparaginase thrombotic events increased in frequency with increasing age (1.5% in under 5 years, 3.3% in 5-9 years, 4.4% in 10-15 years and 8.3% in 16-24 year olds)(p<0.0001). All other toxicities were more frequently observed in over 10 year olds compared to patients aged 9 or younger, with no difference between 16-24 year olds and 10-15 year olds. The impact of age on SAEs associated with intensive ALL chemotherapy varies according to specific toxicities. In general, toxicity is higher in those over 10 years compared to younger patients, with no excess toxicity in those aged 16-24 compared to 10-15 years. However, specific toxicities may increase with increasing age (thrombosis), be restricted to adolescence (AVN) or be unrelated to age (vincristine neurotoxicity, asparaginase hypersensitivity). Disclosures: No relevant conflicts of interest to declare.


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