Severe Valvular Pulmonary Stenosis Research Articles

Balloon pulmonary valvuloplasty with percutaneous transluminal mitral commissurotomy balloon in pulmonary stenosis

Pulmonary stenosis is common and accounts for 5%–12% of all congenital heart diseases. Echocardiography is the mainstay for evaluating pulmonary stenosis due to its easy availability, reproducibility and non-invasive nature. American Heart Association/American College of Cardiology (AHA/ACC) guideline recommends balloon pulmonary valvuloplasty in patients with moderate to severe valvular pulmonary stenosis. Due to its advantages over other balloons, Inoue and Accura balloon, balloons especially designed for Percutaneous Transluminal mitral commissurotomy can be used for successful balloon pulmonary valvuloplasty.

Fenestration: Integrating Wings Into the Atrial Septal Occluder for Navigating a Challenging Terrain.

Background Atrial septal defect (ASD) closure with significant left-to-right shunt and concurrent comorbidities poses challenges for intervention. A fenestrated atrial septal defect (FASD) device is a viable option for patients who cannot undergo complete occlusion due to hemodynamic and medical reasons. This study explores the use of FASD occluders in patients with secundum ASD and associated comorbidities where complete occlusion is difficult. Methodology This retrospective study collected the details of patients recommended for FASD closure diagnosed with significant secundum ASD and who had additional comorbidities between July 2015 and July 2023 in a tertiary cardiac center in eastern India. Among this cohort, patients who underwent FASD device placement were subjected to a comprehensive analysis. Results In total, 16 patients diagnosed with secundum ASD, characterized by significant left-to-right shunt and concurrent comorbidities, were considered for FASD closure during the study period. Ultimately, 13 patients (first group) underwent fenestrated atrial septal occluder implantation. The average age was 45.07 years, with the majority being females (n = 9). Comorbidities among this cohort included substantial left ventricular diastolic dysfunction (n = 7), left ventricular diastolic dysfunction coupled with moderate pulmonary hypertension (n = 1), severe pulmonary hypertension (n = 1), severe pulmonary valvular stenosis with right ventricular diastolic dysfunction (n = 2), and systemic lupus erythematosus (SLE) (n = 2). From this cohort, three patients did not undergo the intervention. The second group consisted of an elderly patient with severe left ventricular diastolic dysfunction, a young adult with a history of left atrial arrhythmia, and achild with Duchenne muscular dystrophy (DMD). The average ASD size among patients who underwent the intervention was 26.38 mm, with a thick-to-thick dimension measuring 31.15 mm. The procedure was successful in all 13 patients, with the most frequently used device being a 34 mm occluder (range = 28-40 mm). All devices, excluding the initial one, were custom-made atrial septal occluders (Lifetech Scientific). Among the patients, 12 exhibited left-to-right fenestration flow, while one patient experienced fenestration constriction, likely due to occluder overcrowding. The first patient hada handmade 5 mm fenestration in a 40 mm Amplatzer septal occluder, which got closed off at the one-year follow-up. The procedure was well-tolerated hemodynamically in all patients, with no major complications during the peri-procedural period. Short-term follow-up indicated favorable patient progress. Conclusions FASD closure emerges as a pivotal alternative for intricate scenarios involving secundum ASD coupled with concurrent comorbidities, offeringindividualized tailored solutions. Alongside the conventional associated comorbidities, such as left ventricular diastolic dysfunction and pulmonary hypertension, FASD devices hold the potential to extend their benefits to patients grappling with other complexities, including severe pulmonary valvular stenosis, SLE, predisposition to left atrial arrhythmia, and conditions like DMD. Ensuring meticulous evaluation of patient suitability and providing ongoing vigilant care becomes paramount for achieving optimal outcomes. The validation of these findings and the broadening of the comprehension of this approach necessitate further comprehensive investigations.

Short term evaluation of right ventricular longitudinal strain in children post balloon pulmonary valvuloplasty

Background Children with isolated severe valvular pulmonary stenosis (sPS) are considered under chronic right ventricle (RV) pressure overload. Little is known about the needed duration for reverse remolding in pediatrics. Objective The study aimed to compare RV function and longitudinal strain in children before and 3 months after balloon pulmonary valvuloplasty (BPV). Patients and methods Thirty-seven children with sPS indicated for BPV were recruited. All patients underwent RV evaluation by Tissue Doppler (TDI) and speckle-tracking echocardiography. RV free-wall longitudinal strain (RV-FW), global RV strain (RV-4C), and global LV strain (GLS-LV) were studied one day before the procedure, 3 months postintervention, and compared with 37 healthy children enrolled as controls. Patient demographic and hemodynamic data during cardiac catheterization was collected. Results Successful BPV of 37 cases (mean age 3.2±4.9 years) showed a significant reduction in transpulmonary gradient from 89.2±23.9 mmHg to 16.1±8.4 mm Hg immediately postprocedure. The RV-FW and RV-4C of patients with sPS and GLS-LV were significantly lower than the control group (P=0.001), respectively. A significant rise of RV-FW and RV-4C occurred 3-month postintervention (−17.5±3.8 vs. 27.4±4.4) and (−15.3±3.8 vs. −23.9±3.9, P=0.001), respectively, and became insignificantly different from the control group. GLS-LV is positively correlated to RV-FW (r=0.342, P=0.038). Cases aged less than 1 year showed a significant rise of RV strains 24 h postprocedure. Conclusion Improvement of RV longitudinal strains of children is highly possible within 3 months of successful BPV up to normalization especially for infants below the age of one year.

Childhood Noonan Syndrome Presenting with Severe Pulmonary Stenosis: A Rare Case Report

Noonan syndrome [NS] is an autosomal dominant inherited condition that can be passed down through families. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. It causes abnormal development in many parts of the body. It is used to be called Turner-like syndrome. Though most of the cases are autosomally inherited some cases may be sporadic. We report a case of 13 year old male child presented to us with features of short stature, dysmorphic facies and severe pulmonary valvular stenosis without any family history of similar illness.
 Keywords: Noonan Syndrome, Pulmonary valvular stenosis.

Long-term results of balloon pulmonary valvuloplasty in pediatric age group in surgical specialty teaching hospital/cardiac center/Hawler

Congenital valvular pulmonary stenosis accounts for 5-10% of all congenital heart disease. Balloon pulmonary valvuloplasty is a safe and effective method in reduction of moderate to severe valvular pulmonary stenosis. This retrospective study done in Hawler surgical specialty cardiac center on pediatric patients who had pulmonary valve stenosis (with echocardiographic peak gradient 2:50mmHg) and treated by balloon pulmonary valvuloplasty. A total of 178 cases were involved, 93(52.25%) patient was male and 85 (47.75%) patients was female. 160 patient (89.89%) were treated with single balloon method 18 patient (10.11%) were managed by double balloon method technique. Follow up period of these patients range from 1 year to 10 years (mean of 5.5 year). In 153 patients (85.96%) pressure across pulmonary valve were below 36 mm Hg, while in 21 (11.80%) pressure gradient across pulmonary valve were (36-50 mm Hg). Four (2.24%) patients had pressure gradient across pulmonary valve more than 50 mm Hg. Forty patients (22.47%) developed mild pulmonary valve regurgitation, while 20 patient (11.24%) developed significant pulmonary valve regurgitation. Balloon pulmonary valvuloplasty provides long-term relief of pulmonary valvular obstruction in the majority of pediatric patients. The long-term results are favorable.

Propofol‐dexmedetomidine continuous infusion in a dog with pulmonic stenosis undergoing balloon valvuloplasty

AbstractA 6‐month‐old, 4 kg, male pomeranian, was referred for investigations of a heart murmur found during a routine clinical examination. A severe valvular pulmonic stenosis was diagnosed and medical management with atenolol was started. One month later, the dog underwent percutaneous balloon valvuloplasty. Anaesthesia was maintained with propofol‐dexmedetomidine continuous infusion, and a minimally invasive cardiac output monitor was used to assess haemodynamic variations. Intraoperatively, lidocaine was started because the frequency of the premature ventricular complexes increased before ballooning. A reduction in pressure gradient across the pulmonic valve was achieved. No other intraoperative complications were recorded. Recovery from general anaesthesia was uneventful, and the dog was discharged from the hospital 26 hours following the procedure.

Simplified art of balloon pulmonary valvuloplasty: Does the presence of mild supravalvular pulmonary stenosis preclude successful balloon pulmonary valvuloplasty?

We present a case of successful balloon pulmonary valvuloplasty (BPV) in a 37-year-old female with severe valvular pulmonary stenosis with peak gradient of 82 mmHg with history of exertional presyncope. The crux of our case was that patient also had associated mild supravalvular pulmonary stenosis which did not preclude a successful BPV outcome. The patient had rare Bombay negative blood group, and the requisite units were not available across all the blood banks in the state to subject the patient for surgical correction. Although the art of BPV is dying among budding young interventionists, this simple procedure is as good as surgical pulmonary valvotomy so far as the immediate and late outcome is concerned and the presence of mild supravalvular pulmonary stenosis does not preclude a successful outcome. Our case is a unique illustration of feasibility of successful balloon pulmonary valvuloplasty in the presence of associated mild supravalvular stenosis.

Pericardial tamponade during balloon pulmonary valvuloplasty in a dog

AbstractA 4‐month‐old entire male Coton de Tulear dog was referred for balloon valvuloplasty for treatment of severe valvular pulmonic stenosis. During the procedure, when attempting to pass the guide wire into the ventricle, the dog developed cardiac arrest. After initial resuscitation with chest compressions, manual ventilation, adrenaline and intravenous fluids, focused cardiac ultrasound revealed pericardial effusion and cardiac tamponade. Pericardiocentesis resolved the haemodynamic emergency. This case report should raise awareness of pericardial tamponade as a possible complication during pulmonic stenosis balloon valvuloplasty. It describes the clinical signs observed during anaesthesia as well as how to treat it with a positive outcome.

Successful balloon valvuloplasty using direct puncture of the heart for pentalogy of Cantrell with complete ectopia cordis, low birth weight, single ventricle and severe pulmonary stenosis.

We present a 31 gestational weeks' premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.

Unconventional deployment of atrial septal occluder in a patient with atrial septal defect, dextrocardia, and interrupted inferior vena cava.

An atrial septal defect is a rare anomaly in patients with interrupted inferior vena cava, which renders the percutaneous intervention more complex; and hence, innovative approaches should be sought. Dextrocardia further complicates the procedure, and traditional atrial septal device deployment methods cannot be employed. We report a successful percutaneous secundum atrial septal defect closure by a novel deployment strategy along with balloon dilation of associated severe valvular pulmonary stenosis in a patient with dextrocardia and interrupted inferior vena cava.

Evaluation of the NuCLEUS-X™ balloon valvuloplasty catheter for severe pulmonic stenosis in dogs

Introduction/objectivesBalloon instability is commonly encountered during balloon pulmonary valvuloplasty (BPV) and may result in an unsuccessful procedure. The NuCLEUS-X™ catheter is a recently developed BPV catheter with a unique barbell shape and an ordered pattern of inflation that stabilizes the balloon to span the valve annulus before expansion of the balloon center. AnimalsTen client-owned dogs with severe valvular pulmonic stenosis (PS). Materials and methodsProspective observational study. The BPV procedure was performed by standard technique with use of NuCLEUS-X™ catheters targeting a balloon-to-annulus ratio between 1.2 and 1.5. Balloon stability, safety, and procedural success were assessed. Procedural success was defined as either a reduction in the Doppler transpulmonic PG by at least 50% of the pre-procedural PG or <80 mmHg one month post procedure. ResultsBalloon stability centered at the pulmonic valve on the first inflation was achieved in 10/10 cases. The mean PG before BPV was 141 mmHg ±41 mmHg, and the PG after BPV at one month was 83 mmHg ±41 mmHg. Procedural success was achieved in 56% of patients. All dogs survived the BPV, and no major procedural complications were encountered using the NuCLEUS-X™ catheter. ConclusionsThe use of the NuCLEUS-X™ catheter is feasible for BPV in dogs with severe PS. The unique balloon shape provided catheter stability on the first inflation in all dogs, which may be beneficial when stabilization of a conventional BPV catheter cannot be achieved.

An unconventional valvuloplasty: severe pulmonary stenosis with 'candy cane' inferior vena cava

Femoral vein is the usual approach to balloon pulmonary valvuloplasty (BPV). However, alternative access may be mandated in some peculiar situations. We hereby report a 10-year-old patient with symptomatic severe...

Complete Transcatheter Repair of Fallot’s Trilogy: A Case Report

Fallot’s trilogy is a relatively uncommon form of cyanotic congenital heart disease characterized by severe valvular pulmonary stenosis (PS), right ventricular hypertrophy and right to left interatrial shunt via an atrial septal defect (ASD) or patent foramen ovale (PFO). Historically, patients with the combination of these lesions were routinely treated by surgical correction. In recent times, transcatheter repair has become an increasingly attractive modality of treating such patients. There is limited experience with such combined transcatheter interventions, and from the scarce data available in published literature it seems that such approach has promising safety and efficacy. Herein, we describe a case of an 18 year old cyanotic male patient who was diagnosed of having severe valvular PS with supra systemic right ventricular pressures and reversed interatrial shunt via a large secundum ASD. We successfully treated him percutaneously with serial balloon pulmonary valvuloplasty, using single balloon and Inoue balloon techniques, followed by Amplatzer device closure of the ASD. The outcome was excellent with marked reduction of trans-pulmonary valvular gradient, complete cessation of shunting across the ASD, dramatic improvement of symptoms and completes disappearance of cyanosis. There were no procedure related complications and the benefits were sustained at follow up of three years. Our case demonstrates that transcatheter repair is a feasible, safe and effective alternative to surgery in selected patients with Fallot’s trilogy. With further experience, it may well become the standard of care in the management of such patients.

Intraoperative Echocardiographic Detection of Septal Aneurysm and Additional Ventricular Septal Defect in a Child with Tetralogy of Fallot

ABSTRACT A 2-year-old child was diagnosed with subaortic ventricular septal defect (VSD) with severe infundibular and valvular pulmonary stenosis (PS) by transthoracic echocardiography. Intraoperative transesophageal echocardiography (TEE) detected aneurysmal interventricular and interatrial septum (IAS), subaortic VSD, and right ventricular outflow tract (RVOT) with an additional midmuscular VSD. The aneurysmal interventricular septum (IVS) was repaired. An accessory tricuspid papillary muscle was attached to RVOT, which was augmented with a transannular pericardial patch to have minimal postoperative gradient without sacrificing the accessory papillary muscle (APM). This rare case poses a challenge to the anesthetist and surgeon in the form of diagnostic differences, severe right ventricle (RV) dysfunction with aneurysmal IVS, and difficulty in reconstruction of RVOT. Intraoperative TEE played a greater role to diagnose the IVS aneurysm, additional VSD and guide for appropriate surgery. How to cite this article Das S, Kumar A. Intraoperative Echocardiographic Detection of Septal Aneurysm and Additional Ventricular Septal Defect in a Child with Tetralogy of Fallot. J Perioper Echocardiogr 2017;5(1):21-24.

Longitudinal function and ventricular dyssynchrony are restored in children with pulmonary stenosis after percutaneous balloon pulmonary valvuloplasty.

The aim of this study was to evaluate the impact of balloon pulmonary valvuloplasty on longitudinal ventricular dysfunction and cardiac dyssynchrony in children with congenital moderately severe pulmonary stenosis (PS). Forty-eight children with congenital moderately severe valvular pulmonary stenosis and 36 age-matched normal children were included. Successful percutaneous balloon pulmonary valvuloplasty was performed for children with PS. Longitudinal Cardiac function and myocardial synchrony was assessed by echocardiography with tissue Doppler imaging (TDI). Mitral annular plane excursion (MAPSE), tricuspid annular plane excursion (TAPSE), left ventricular systolic wave (LVs), right ventricular systolic wave (RVs) and myocardial synchrony was assessed by echocardiography with TDI. TAPSE, RVs, TAPSE/MAPSE ratio and RVs/LVs ratio were significantly lower in children with PS compared to control subjects: (P < 0.001, P < 0.001, P < 0.001 and P < 0.001). All these parameters were significantly improved after balloon valvuloplasty in children with PS. Intra& inter-ventricular dyssynchrony were significantly increased in PS patients than in control group, (P < 0.03, <0.001 and 0.02 respectively). All these variables were significantly reduced after pulmonary valvuloplasty. In children with valvular PS, balloon valvuloplasty restored longitudinal function and cardiac Synchrony. This gives good insights for the importance of early intervention in children with PS to prevent the progression for cardiac deformation.

A fatal complication arising during pulmonic balloon valvuloplasty in a seven-month-old female bulldog

A seven-month-old female bulldog with severe valvular pulmonic stenosis and ventricular septal defect presented for general anaesthesia for repeat balloon valvuloplasty. The first balloon valvuloplasty procedure was not successful. The...

55. Can we assess the success of balloon pulmonary valvuloplasty by ECG

Introduction The gold standard for diagnosis of pulmonary stenosis (PS) is by echocardiography. P wave dispersion (PWD) indicates the spreading of the sinus stimulus in the atria. Aim of the work Study the effect of PS and its treatment by BPV on electrocardiographic (ECG) parameters as PWD and P wave amplitude. Patient & Methods Patients with moderate or severe valvular PS was enrolled in the study. PWD and maximum P amplitude (max P amplitude) on ECG were measured. The peak systolic pressure gradients (SPG) over the pulmonary valve were obtained. All cases were underwent BPV. Reassessment of SPG and ECG parameters were done immediately and one month later. Results Forty two patients were recruited .Their age ranged from 3 to 38 years. PWD before balloon had mean ± SD (0.06 ± 0.017). It decreased significantly to (0.04 ± 0.017) and (0.04 ± 0.013) immediately and One month after balloon respectively ( p value 0.001 ). The max P amplitude had mean ± SD before balloon (0.08 ± .027) and it decreased significantly to (0.07 ± 0.025) 1 month later ( p value 0.013). There was significant correlation between SPG and PWD ( p value p amplitude ( p value 0.041). However, there was non significant correlation between the drop in SPG and the change in PWD. Conclusion PWD and max P amplitude decrease significantly with BPV. The study demonstrated for the first time very simple ECG parameters; PWD and P amplitude that may predict the success of BPV on the short term.

Severe Pulmonary Valvular Stenosis and Balloon Pulmonary Valvuloplasty (BPV): A Case Report

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.&#x0D; Ibrahim Cardiac Med J 2013; 3(1&amp;2): 53-58

Combined severe valvular aortic and valvular pulmonary stenosis and its management – A case report and literature review

Isolated congenital valvular stenosis of either aortic or pulmonary valve is commonly seen yet the presence of both these lesions in the same patient is rare. This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves. We present here a case report of a three and half year old boy diagnosed as a combined congenital severe valvular aortic stenosis with valvular pulmonary stenosis. The patient underwent successful balloon dilatation of both these valves in the same sitting.

Calcified Aorto–Right Ventricular Tunnel in a Patient With Multiple Lentigines Syndrome

From the *Department of Cardiology, C.S.M. Medical University (King George’s Medical College), Lucknow, Uttar Pradesh, India; and the †Department of Radiodiagnosis, C.S.M. Medical University (King George’s Medical College), Lucknow, Uttar Pradesh, India. Manuscript received June 5, 2012; revised manuscript received June 14, 2012, accepted June 26, 2012. A12-year old boy with multiple pigmented nevi and lentigines (A) presented to us with symptoms of effort dyspnea and recent onset cyanosis. He had mild thoracolumbar scoliosis, unilateral sensorineural hearing loss, and growth retardation. The 2-dimensional transthoracic echocardiogram revealed a circular echolucency lateral to the tricuspid annulus (B, Online Video 1) (LA left atrium; LV left ventricle; RA right atrium; RV right ventricle), and color Doppler echocardiogram showed a tunnel-like structure with turbulent jet filling the right ventricle (C, Online Video 2). Two-dimensional echocardiogram with continuous-wave Doppler showed severe valvular pulmonic stenosis (D), and contrast echocardiogram revealed right to left shunt across the foramen ovale (Online Video 3). Cardiac catheterization showed spiral calcification and catheter passage from the aorta to the RV (E, Online Video 4), and the aortogram revealed flow across the tunnel (Online Video 5). Sixty-four slice computed tomography angiography showed a dilated aortic root (DA) with a tortuous aorto–right ventricular tunnel (F). Computed tomography volume-rendered imaging showed a giant tunnel and single left coronary artery giving branches (Bs) to the RV (G), with massive dilation of the aortic root and presence of calcified bands at the origin of the tunnel (H). LAD left anterior descending artery; MPA main pulmonary artery; PA pulmonary artery; RAA right atrial appendage. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jacc.2012.06.066