Treatment of severe refractory valvar pulmonary stenosis with primary transcatheter pulmonary valve implantation

2017 ◽  
Vol 27 (6) ◽  
pp. 1232-1234
Author(s):  
Kalyani R. Trivedi ◽  
Laurence Robinson ◽  
Alain Fraisse

AbstractSome patients with pulmonary valve stenosis do not respond to balloon valvuloplasty and must undergo surgical repair. We report the case of a 12-year-old child with pulmonary valve stenosis and Noonan syndrome in whom we performed transcatheter Melody pulmonary valve implantation after balloon dilation failed. The result was excellent. This technique can be proposed as an alternative to surgery in such cases.

2021 ◽  
Vol 2 (2) ◽  
pp. 77
Author(s):  
Tinton Pristianto ◽  
Rosi Amrilla Fagi

Introduction: Noonan syndrome (NS) is a genetic disorder often accompanied by multiple congenital abnormalities. The prevalence of NS at live birth has been reported as one in 1000-2500 individuals. About 80% of patients with Noonan syndrome have abnormalities in the cardiovascular system.Case presentation:41-year-old Javanese male presented with chief complaint shortness of breath.  His Body Mass Index (BMI) was 18,3. He had an oval-shaped face with a short neck, thin hair, and prominent nasolabial fold. Echocardiography showed biventricular hypertrophy alongside pulmonary valve stenosis, pulmonary regurgitation and minimal pericardial effusion. Discussion: In 1962, Jacqueline Noonan, a pediatric cardiologist, identified 9 patients whose faces were very similar, had short stature, significant chest deformities, and with pulmonary stenosis. Noonan syndrome is a relatively common non-chromosomal syndrome that is similar to the phenotype of Turner's syndrome and presents with cardiovascular malformations. Adult with NS has distinctive facial features such as ptosis, wide eyes, low posterior rotation of ears and helical thickening, and a wide neck.Pulmonary stenosis is the most common heartdefect found in NS, besides HCM isalsoquitecommon inabout20% of patients. We reported a case of a patient with typical characteristics of NS such as pulmonary valve stenosis accompanied by biventricular ventricular hypertrophyand its typical face who survived through adulthood.Conclusion: Syndrome Noonan in the adult is quite rare and difficult to diagnose. We reported a case of an adult man with facial appearance and echocardiographic findings identical with Noonan Syndrome.


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.


2021 ◽  
pp. 1-7
Author(s):  
Ryan A. Romans ◽  
Wendy Whiteside ◽  
Sunkyung Yu ◽  
Lori Harris ◽  
Veronika Dottermann ◽  
...  

Abstract Objectives: 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. Background: 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. Methods: 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. Results: 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. Conclusions: 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.


2012 ◽  
Vol 60 (S 01) ◽  
Author(s):  
D Biermann ◽  
J Schönebeck ◽  
M Rebel ◽  
J Weil ◽  
H Reichenspurner ◽  
...  

2020 ◽  
Vol 28 (1) ◽  
pp. 1-6
Author(s):  
Fatema Nurun Nahar ◽  
Jufan Mansur Al ◽  
Shubert Stephan ◽  
Hossain Mir Mahmud

INTRODUCTION Incidence of congenital heart disease is 25 per thousand live birth in Bangladesh which is much higher than other countries. Tetralogy of Fallot, the commonest cyanotic heart disease (5%) and some other complex diseases with right ventricular outflow tract abnormality demand surgical correction and revision in many occasions including percutaneous intervention. As a resource constraint country, it was a difficult task to introduce percutaneous pulmonary valve implantation (PPVI) with MelodyTM. However, it was started on 12th December 2012 in Combined Military Hospital Dhaka, Bangladesh and cases performed till October 2019 were included in this series. METHODS Retrospective analysis of six cases who had PPVI with MelodyTM in Combined Military Hospital, Bangladesh. Patient with dysfunctional conduit between right ventricle (RV) and pulmonary artery causing (a) Symptoms of exceptional dyspnoea of various grade (NYHA II,III, IV) (b) RVEVD >150 ml/m2 ±regurgitant fraction >40% (c) RVOT peak instantaneous gradient > 30 mm Hg. (d) RV dysfunction (RVEF<40%) were accepted for the procedure and outcome were analyzed. RESULTS Mean age was 9.56 ± 2.96 years, weight was 28.75 ± 8.61 kg, height was 137.5 ± 17.52 cm. Mean age at surgery was 4.25 ± 2.72 years. Female were 66.66%. Aortic homograft was used in 66.66% cases. Eighteen mm Ensemble was used in four (66.66%) cases and 20 mm and 22 mm in one each. Immediate result was excellent with no residual PS in two cases and negligible residual flow acceleration across pulmonary valve in four cases. No PR seen in all except one. One patient developed Bacterial endocarditis after 3 years and was treated. CONCLUSION Aim of PPVI is to prolong the life expectancy of conduits which were placed surgically from right ventricle to pulmonary artery. In our case series, we found that Melody valve is functioning well without any complications like infective endocarditis or stent fracture. KEYWORDS PPVI, MelodyTM, RVOT, Outcome


Author(s):  
Osamah Aldoss ◽  
Kaitlin Carr ◽  
Shabana Shahanavaz ◽  
David Balzer ◽  
Daniel S. Levi ◽  
...  

2021 ◽  
Vol 10 (Supplement_1) ◽  
pp. S9-S9
Author(s):  
B Rubbab ◽  
C Talluto ◽  
A Nuibe ◽  
R Levorson

Abstract Background Percutaneous pulmonary valve implantation (PPVI) is being increasingly used as a minimally invasive corrective procedure for right ventricular outflow tract (RVOT) dysfunction. Ten-year survival following PPVI is estimated at over 90% due to the durability of the various bioprosthetic valves. However, infective endocarditis (IE) remains a potential complication of such valves with significant morbidity and mortality. We evaluated the presenting symptoms, clinical features, pathogens, and outcomes of patients with IE following PPVI to identify opportunities to improve early diagnosis and management. Methods A convenience sample of patients at a large Pediatric Cardiology practice in Northern Virginia was queried for PPVI and IE from January 1, 2016, to June 30, 2019. Manual chart review was done to extract clinical points of interest and descriptive analyses were performed. Patients were classified as having IE per modified Duke’s criteria. Results We identified 14 patients who underwent PPVI. Five of these patients (36%) developed IE. All IE patients had underlying Tetralogy of Fallot and none had previous episodes of IE. 60% of patients with IE were male with a median age of 26 years old (IQR 20–30). Four IE patients had a Melody valve and 1 had a SAPIEN valve. The median elapsed time between PPVI and IE diagnosis was 128 days (IQR 32–391) with a median duration of illness prior to the diagnosis of IE of 6 days (IQR 5–9). All IE patients had to present fever. 40% of IE patients had to present chest pain and 20% had presenting musculoskeletal pain. All IE patients had an elevated initial C-reactive protein (CRP) with a median value of 13.1 mg/dL (IQR 12.5–15.2). The median initial white blood cell count was 9.3 × 103/μL (IQR 8.1–10.3). The median duration of bacteremia was 1 day (IQR 1–2). A pathogen was recovered in all five IE patients with different organisms amongst the patients: coagulase-negative Staphylococcus species were recovered in patients who developed IE within 60 days from PPVI (Staphylococcus lugdunensis and Staphylococcus epidermidis) whereas coagulase-negative Staphylococcus species and oral commensal organisms were found in IE patients beyond 60 days from PPVI (Staphylococcus sanguinis, Gamella haemolysans, and Neisseria elongata). The initial echocardiogram did not show vegetations in any of the patients and 40% went on to have sternotomy with valve replacement. There were no deaths. Conclusions With an increase in PPVI, clinicians should have a high index of suspicion for IE in patients with underlying Tetralogy of Fallot who present with fever and elevated CRP, regardless of elapsed time from PPVI or valve type. Empiric antimicrobial therapy for suspected IE following PPVI should remain broad with other possible pathogens beyond coagulase-negative Staphylococcus species.


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