Mild Pulmonary Stenosis Research Articles

A Pediatric Cardiology Outreach Clinic in a Local Hospital: An Experience Review from South A'Sharqiyah, Oman.

Specialized pediatric cardiology clinics conducted in local hospitals are an important part of delivering specialized care to patients close to their homes. This study aimed to review our experience with a specialized pediatric cardiology outreach clinic at Jaalan Bani Bu Ali Hospital, South A'Sharqiyah, Oman. Patient records for each individual, seen in the outreach clinic between March 2018 and June 2022, were reviewed to determine demographic information, reason for referral, underlying diagnosis, and clinic visit outcomes. Over the study period, 29 clinics were conducted, with 360 patients seen. Of these, 200 (55.6%) were male with a median age of 13 months. The majority of patients (n = 271; 75.3%) were referred due to a cardiac murmur. Most patients had a normal cardiac evaluation (n = 177; 49.2%). The most common congenital heart diseases detected were mild pulmonary valve stenosis (14.8%) and moderate to large secundum atrial septal defects (13.7%). Significant cardiac lesions detected included severe pulmonary hypertension (2.2%), tetralogy of Fallot (1.6%), and cor triatriatum sinistrum (0.5%). Overall, 70 (19.4%) patients were referred to tertiary care hospitals, and 179 (49.7%) were reassured and discharged. Conducting specialized pediatric cardiology outreach clinics in overpopulated areas is effective and well-received by families. It reassures many families and reduces the need for unnecessary travel to specialized centers. These clinics also play a crucial role in detecting patients with significant cardiac defects requiring urgent care. Implementing specialized clinics in primary and secondary health centers could be beneficial for other subspecialties in reducing long waiting lists.

Whole exome sequencing of a novel homozygous missense variant in PALB2 gene leading to Fanconi anaemia complementation group.

Partner and localiser of BRCA2 (PALB2), also known as FANCN, is a key tumour suppressor gene in maintaining genome integrity. Monoallelic mutations of PALB2 are associated with breast and overian cancers, while bi-allelic mutations cause Fanconi anaemia (FA). In the present study, whole exome sequencing (WES) identified a novel homozygous missense variant, NM_024675.3: c.3296C>G (p.Thr1099Arg) in PALB2 gene (OMIM: 610355) that caused FA with mild pulmonary valve stenosis and dysmorphic and atypical features, including lymphangiectasia, non-immune hydrops fetalis and right-sided pleural effusion in a preterm female baby. WES results were further validated by Sanger sequencing. WES improves the screening and detection of novel and causative genetic variants to improve management of disease. To the best of our knowledge, the present study is the first reported FA case in a Saudi family with phenotypic atypical FA features. The results support the role of PALB2 gene and pathogenic variants that may cause clinical presentation of FA. Furthermore, the present results may establish a disease database, providing a groundwork for understanding the key genomic regions to control diseases resulting from consanguinity.

Williams Syndrome: A Rare Case Report

We report a rare case of Williams Syndrome diagnosed at an age of two months based on subtle facial dysmorphism (elfin facies) and a pulmonary systolic murmur at birth. Echocardiogram revealed mild supravalvular aortic stenosis and mild supravalvular pulmonary stenosis extending into branch pulmonary arteries. Mild developmental delay, hypotonia, feeding problems, constipation and failure to thrive were present. In view of distinctive facial phenotype in combination with cardiac abnormalities, Williams Syndrome was strongly suspected. It was confirmed by fluorescence in situ hybridisation test demonstrating a deletion of elastin gene on chromosome 7. This case highlights the importance of pediatricians awareness about malformation syndromes which can help in early diagnosis, timely genetic counselling to the family and better patient outcomes.

Novel mutation in ELN gene causes cardiac abnormalities and inguinal hernia: case report

BackgroundElastin-driven genetic diseases are a group of complex diseases driven by elastin protein insufficiency and dominant-negative production of aberrant protein, including supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Here, a Chinese boy with a novel nonsense mutation in the ELN gene is reported.Case presentationWe report a 1-year-old boy who presented with exercise intolerance, weight growth restriction with age, a 1-year history of heart murmur, and inguinal hernia. Gene sequencing revealed a novel nonsense mutation in the ELN gene (c.757 C > T (p.Gln253Ter), NM_000501.4). Due to severe branch pulmonary artery stenosis, the reconstruction of the branch pulmonary artery with autologous pericardium was performed. The inguinal hernia repair was performed 3 months postoperatively. After six months of outpatient follow-up, the child recovered well, gained weight with age, and had no special clinical symptoms.ConclusionWe identified a de novo nonsense mutation in the ELN gene leading to mild SVAS and severe branch pulmonary artery stenosis. A new phenotype of inguinal hernia was also needed to be considered for possible association with the ELN gene. Still, further confirmation will be necessary.

Echocardiographic characteristics and clinical outcomes in fetuses with pulmonary stenosis or pulmonary atresia with intact ventricular septum

To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared. Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7%, 18.3%, 26.1% and 13.9%. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2m/s predicted PV pressure ≥40mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11% vs. PA-IVS 36%) and lower rate of developing bi-ventricles (mild 100% vs. moderate 95% vs. critical 89% vs. PA-IVS 36%). Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome.

Generation of iPSC line NCHi012-A from a patient with Alagille syndrome and heterozygous pathogenic variant in the JAG1 gene

Alagille syndrome (ALGS) is an autosomal dominant disease affecting the liver, heart and other organs with high variability. About 95% of ALGS cases are associated with pathogenic variants in JAG1, encoding the Jagged1 ligand that binds to Notch receptors. The iPSC line NCHi012-A was derived from an ALGS patient with cholestatic liver disease and mild pulmonary stenosis, who is heterozygous for a 2 bp deletion in the JAG1 coding sequence. We report here an initial characterization of NCHi012-A to evaluate its morphology, pluripotency, differentiation potential, genotype, karyotype and identity to the source patient.

Pulmonary vein stenosis post cryo AF ablation using single shot 4-minute freeze technique

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Magdi Yacoub foundation Introduction Cryo balloon ablation is widely used for AF ablation. Cryo ablation techniques include regular 2 shots per vein Vs single shot freeze technique. Studies showed that the single shot freeze technique is as effective as the double freezing shot, thus becoming the standard technique (1). There is a debate on whether the single shot freeze should be 3 minutes or 4 minutes. Some data suggest similar efficacy while other data suggest more durable PV isolation with the 4-minute technique (2). Severe pulmonary vein (PV) stenosis is a rare but a serious complication. The only available data on iatrogenic PV stenosis are from studies on the single 3-minute freeze technique (3). Purpose Assessment of the incidence of PVs stenosis using single shot 4-minute freeze technique. Methods We conducted a single center prospective study where we recruited 28 consecutive patients with 102 PVs scheduled for cryo AF ablation using 2nd generation cryo balloon using single shot 4-minute freeze technique as the standard technique. All patients had cardiac CT before ablation and at least 3 months post ablation where each PV’s cross-sectional area was measured. Measurements were validated by a single experienced operator. PV stenosis was classified to minimal, mild, moderate, and severe if the PV’s area decreased by 25%, 25-50%, 50-75% and more than 75% respectively. All patients were assessed regarding the presence of any symptom suggesting PV stenosis or other complications. Results Of the studied 102 PVs, only one vein (0.98%) had severe stenosis (asymptomatic) and one vein (0.98%) had moderate stenosis. Both were left superior pulmonary vein (LSPV). 13 veins (12.7%) developed mild stenosis, 74 veins (72%) showed minimal stenosis and 13 veins had no change in area. None of the studied patients developed phrenic nerve palsy or esophageal injury peri-procedure. Discussion Our results suggest low risk of developing severe PV stenosis using the 4-minute single shot freeze technique (0.98%). However, minimal, and mild pulmonary vein stenosis occurred in 84.7% of the patients denoting that this change is expected with the procedure. Tokutake et al studied the effect of using the single shot 3-minute freeze technique on PV area 3 months post cryo AF ablation and reported mild, moderate and severe (&amp;gt;75%) PV stenosis in 24.6%, 3.8% and 1.3% of PVs respectively (3). This shows that using the 4-minute freeze technique has a similarly low incidence of severe PV stenosis to the published data on the 3-minute technique. It appears that the LSPV is the most liable for this complication and this goes in line with the previous studies. Conclusion Using the 4-minute single shot freeze technique in cryo AF ablation appears to have a comparable low incidence of severe PV stenosis to the published data for the 3-minute freeze technique. The overall risk of severe PV stenosis is low. It appears that the LSPV is the most liable for this complication.

Complexity of dental management of a pediatric patient with lamellar ichthyosis: case report

The purpose of this case report is to describe the complexity of dental management of a pediatric patient with lamellar ichthyosis. A 9-year-old male patient was referred by the pediatrician to the special needs’ clinic with a major complaint of "tooth in the roof of the mouth", according to the person in charge. The patient's mother reported that he was diagnosed with lamellar ichthyosis at birth and regularly followed up with a medical team, including a cardiologist, due to the presence of mild pulmonary valve stenosis. The physical examination revealed presence of skin peeling, everted lip, mouth breathing, cognitive deficit, and speech delay. The intraoral clinical examination showed mixed dentition, absence of caries lesions and presence of supernumerary teeth on the palate, between teeth 11 and 21, which was confirmed by radiographic examination. Although he was cleared by the pediatrician and cardiologist for the procedure under general anesthesia, this procedure in the hospital setting was interrupted at the last minute and contraindicated by the anesthesiologist, as it would be a high risky due to the patient's airway conditions. Thus, one month later, the patient's surgery was performed under local anesthesia on an outpatient basis under sedation and protective stabilization. The patient remains on follow-up and receiving preventive treatment. Thus, it is fundamental that there is a multidisciplinary approach of these patients to prevent possible complications resulting from dental procedures. In addition, regular dental monitoring is essential to avoid subjecting the patient to invasive procedures.

Clinical outcomes of handmade polytetrafluoroethylene trileaflet-valved conduit used for pulmonary valve replacement.

To mitigate the shortage of homograft sources, the use of handmade trileaflet expanded polytetrafluoroethylene valves in pulmonary valve replacement has shown excellent results from multicentre studies conducted in Japan. However, world-wide data outside Japan are relatively insufficient. This study presents the long-term results of a single surgeon's use of flipped-back trileaflet method in a 10-year case series. We have developed an efficient way to make a trileaflet-valved conduit utilizing flipped-back method for pulmonary valve replacement and have employed the technique since 2011. Retrospective data were studied between October 2010 and January 2020. Echocardiography, electrocardiogram, Pro-Brain Natriuretic Peptide and Magnetic Resonance Imaging data were analysed. Fifty-five patients were reviewed and median follow-up duration was 2.9 years. The majority of diagnoses was Tetralogy of Fallot (n = 41), and these patients subsequently underwent secondary pulmonary valve replacement at a median age of 15.6 years. Survival was 92.7% with the longest follow-up period being 10 years. There was no need for reoperation, and freedom from reintervention was 98.0% at 10 years. There were 4 deaths (3 in-hospital and 1 outpatient). One patient eventually received transcatheter pulmonary valve implantation. Postoperative echocardiography showed mild or less pulmonary stenosis and pulmonary regurgitation degree in 92.2% and 92.0% of patients, respectively. Comparable magnetic resonance imaging data (n = 25) showed significant reduction in right ventricular volumes but not in ejection fractions. Our series showed satisfactory long-term function of handmade flipped-back trileaflet-valved conduit used in our patients. The simple design is efficiently reproducible without complex fabrication process.

Prolonged Fever in a Child With Congenital Heart Disease and a Prosthetic Valve.

Prolonged Fever in a Child With Congenital Heart Disease and a Prosthetic Valve.

Prevalence of cardiovascular disease in Sickle cell disease (SCD)- An echocardiographic and pharmacological analysis

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Sickle cell disease (SCD) is an inherited disorder of globin chains that cause haemolysis and chronic organ damage. It is an autosomal recessive condition. SCD is a condition more prevalent in sub-saharan Africa. It is estimated that there are between 12,500 and 15,000 people with SCD in the United Kingdom (UK) in 2014. The prevalence of the disease is increasing due to immigration into the UK and new births. As a result, there is a need to understand cardiovascular complications within this population, as despite a significant increase in longevity for SCD patients over the last few decades, there are significant cardiac complications. Method We present retrospective data from a single center tertiary centre for SCD. A total of 318 patients were followed up from diagnosis to current day with a mean age of 43.4 and median of 42. 136 (43%) were male and 182 (57%) Female. Results A total of 157 (49%) patients underwent a transthoracic echocardiogram (TTE). 56 (17.5%) had a dilated Left Atrium, 36 (11.3%) had Valvular heart disease, 18 (5.7%) Pulmonary Hypertension (PHT) or Right Heart Dysfunction and 17 (5.34%) had a dilated Left Ventricular cavity. 14 patients had Tricuspid regurgitation (13 mild and 1 severe), 15 had Mitral regurgitation (14 mild and 1 moderate), 7 had mild Aortic regurgitation, 6 had Mild Pulmonary regurgitation, 3 had mild Pulmonary Stenosis and 3 were noted to have sclerotic aortic valve. From a Left ventricular systolic function perspective, 151 patients had a normal ejection fraction, 4 had mild Left ventricular systolic dysfunction (LVSD), 1 moderate LVSD and 1 severe LVSD. A normal ejection fraction was defined as &amp;gt;55%, mild 45-55%, moderate 35-45% and severe &amp;lt;35%. 40 (12.60%) Patients were taking Calcium channel blockers (Dihydropyridine), 25 (7.86%) were taking Angiotensin converting enzyme inhibitor/ Angiotensin receptor blocker, 20 (6.29%) taking an anticoagulant (DOAC), 16 (5.03%) were taking a statin, 15(4.72%) taking B blockers, 11(3.46%) taking an antiplatelet, 5(1.57%) patients were taking a loop diuretic, 4 (1.26%) thiazide diuretic, 4 (1.26%) on an alpha blocker and 1(0.3%) patient on digoxin. The prevalence of Atrial Fibrillation was 1.57% (5 patients). Conclusions The Echocardiographic data suggests the commonest findings are of a dilated left atrium, dilated ventricle, valvular heart disease and PHT. The dilated left atrium is secondary to the hyperdynamic states found in sickle cell disease. Despite this being very common the rates of arrhythmia are low. The prevalence of LVSD remains low. The findings of PHT are of particular interest as it guides SCD management with exchange transfusion. The pharmacological data suggests high burden of cardiovascular risk factors i.e. hypertension and hypercholesterolemia despite the young demographic. It is vital to follow up these patients with aggressive primary prevention medications to reduce risk of future cardiovascular events.

959 AN UNEXPECTED DIAGNOSIS BY ECHOCARDIOGRAPHY: APPLICATION IN CLINICAL PRACTICE

Abstract A carcinoid tumore is a rare neuroendocrinologic malignancy that commonly origins from enterochromaffin or Kulchitsky cells in the gastrointestinal tract and secretes bioactive molecules in blood. Up to 60% of patients with carcinoid tumors have cardiac involvement, often on the right-sided heart valves (typically the tricuspid valve and the pulmonic valve). A 66 year old woman was admitted to hospital with congestive heart failure, chronic diarrhea and weight loss. A contrast-enhanced computed tomography scan of the liver showed multiple masses (the largest 70 mm×45mm) compatible with metastases. A transthoracic echocardiogram showed dilatation of right sections of the heart with severe tricuspid regurgitation (RV 64 ml/beat, ERO 1 cmq) and mild pulmonary valve stenosis (Peak gradient 13 mmHg). Patient's aortic and mitral valves were normal in structure and function. The EKG revealed low-voltage QRS complexes of all leads, unspecific ST segment changes and sinusal rhythm. The blood tests showed a significant increase in chromogranin A (17670 ng/ml) and enolase; a 24-hour urine collection showed a total output of 5-hydroxyindoleacetic acid eleveted. A biopsy of the liver with immunostaining for chromogranin and synaptophysin were strongly positive and confirmed the diagnosis of metastatic carcinoid syndrome (grade 1, cells CDX2 positive). The echocardiographic findings of carcinoid heart disease is drumstick-like, rigid, and shortened leaflets with a visible regurgitant orifice, causing torrential tricuspid regurgitation. The pulmonic valve presents more frequently valvular stenosis. A combination of biomarkers and cardiac imaging is useful in screening and diagnosis of carcinoid heart involvement in patients with carcinoid syndrome. The management of carcinoid heart disease is hardful and requires a multidisciplinary approach, medical and surgical treatment, for optimized care.

30 years' experience with the arterial switch operation: risk of pulmonary stenosis and its impact on post-operative prognosis.

Pulmonary stenosis is one of the most common complications in patients with transposition of the great arteries after the arterial switch operation. The reason for this is multifactorial and related to the anterior shift of the pulmonary trunk during the LeCompte manoeuvre, the complex suture line required to fill the gaps after harvesting the coronary arteries, and the need for patch implantation to maintain a tensionless anastomosis.We reviewed all patients with transposition of the great arteries operated on at our institute between 1991 and 2020 to establish the frequency of pulmonary stenosis during post-operative follow-up, reinterventions, and reoperations related to pulmonary stenosis and its potential risk factors.During the analysed period, we performed 848 arterial switch operations for simple and complex cases of transposition of the great arteries. The overall early mortality was 6.96%, and the late mortality was 2.53%. Among all study groups, 243 (28.66%) patients developed mild pulmonary stenosis, 43 patients (5.07%) developed moderate, and 45 patients (5.31%) developed severe pulmonary stenosis. During follow-up, 21 patients required interventions related to pulmonary stenosis. Pulmonary reconstruction with patches, aortic arch anomalies, and ventricular septal defects associated with transposition of the great arteries were significant risk factors. Nine patients required reoperation because of pulmonary artery stenosis with patch reconstruction of the pulmonary artery, aortic arch anomalies, and aortic cross-clamping time, increasing the risk of reoperation.Pulmonary stenosis in patients with transposition of the great arteries after the arterial switch operation is a common complication. If significant, it occurs early after surgery and is the most frequent reason for post-operative interventions and reoperations.

PPP2R1A neurodevelopmental disorder is associated with congenital heart defects.

Protein phosphatase 2A (PP2A) is a heterotrimeric serine/threonine phosphatase that regulates numerous biological processes. PPP2R1A encodes the scaffolding "Aα" subunit of PP2A. To date, nearly 40 patients have been previously reported with 19 different pathogenic PPP2R1A variants, with phenotypes including intellectual disability, developmental delay, epilepsy, infant agenesis/dysgenesis of the corpus callosum, and dysmorphic features. Apart from a single case, severe congenital heart defects (CHD) have not been described. We report four new unrelated individuals with pathogenic heterozygous PPP2R1A variants and CHD and model the crystal structure of several variants to investigate mechanisms of phenotype disparity. Individuals 1 and 2 have a previously described variant (c.548G>A, p.R183Q) and similar phenotypes with severe ventriculomegaly, agenesis/dysgenesis of the corpus callosum, and severe CHD. Individual 3 also has a recurrent variant (c.544C>T, p.R182W) and presented with agenesis of corpus callosum, ventriculomegaly, mild pulmonic stenosis, and small patent foramen ovale. Individual 4 has a novel variant (c.536C>A, p.P179H), ventriculomegaly, and atrial septal defect. To conclude, we propose expansion of the phenotype of PPP2R1A neurodevelopmental disorder to include CHD. Further, the R183Q variant has now been described in three individuals, all with severe neurologic abnormalities, severe CHD, and early death suggesting that this variant may be particularly deleterious.

Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery of an Infant.

A female presented 2 weeks after birth with an unbalanced atrioventricular canal, double outlet right ventricle, mild pulmonary stenosis, and patent ductus arteriosus that eventually caused pulmonary over circulation. After pulmonary artery banding, she experienced myocardial ischemia, suggesting interference with coronary blood flow by the band that had been placed on the main pulmonary trunk. The band was removed and revised to bilateral branch pulmonary artery banding, and cardiac function improved. An anomalous left coronary artery from the underside of the right pulmonary artery was identified. Eight weeks later, the patient underwent coronary transfer and reimplantation of the left coronary artery into the aorta followed by main pulmonary artery banding. She subsequently underwent bidirectional Glenn.

Neonate with Respiratory Distress, Bilateral Microtia, Hypocalcemia, and Lymphopenia.

Neonate with Respiratory Distress, Bilateral Microtia, Hypocalcemia, and Lymphopenia.

Rheumatic Heart Disease with Quadrivalvular Stenosis: A Case Report and Review of Literature

Multivalvular heart disease (MVD), particularly of rheumatic nature, continues to be a widespread condition in developing countries such as Morocco, despite implemented preventive measures. The intricate hemodynamic interactions between coexisting valve lesions may influence the clinical expression of each individual lesion. It makes diagnosis difficult and management often at the discretion of the physician given the lack of specific guidelines to assist in strategy. In the available literature, rheumatic quadrivalvular stenosis is exceptional. Here we describe a very rare case of rheumatic involvement of all four heart valves discovered in a 34-year-old woman with a history of untreated recurrent tonsillitis and joint pain in childhood, who presented with progressively worsening dyspnea and fatigue. Transthoracic echocardiography revealed severe left valves stenosis in addition to severe tricuspid and mild pulmonary stenosis, with evidence of congestive heart failure. Our case highlights the diagnostic difficulties encountered in the context of a developing country with limited access to primary care and treatment options.

NATURAL HISTORY OF CONGENITAL ISOLATED MILD AORTIC VALVE AND MILD PULMONARY VALVE STENOSIS: A SINGLE CENTER FOLLOW-UP STUDY

Objective: Most of the available information on the natural history of aortic stenosis (AS) and pulmonary stenosis (PS) in children is based on studies carried out over the past 35-40 years using cardiac catheterization. This study aimed to reveal the natural history of congenital isolated mild valvular AS and PS in children using serial Doppler echocardiographic examinations. Materials and Methods: A total of 125 children, 50 with mild AS and 75 with mild PS, who underwent Doppler echocardiography were included in this study. The prognoses of mild AS and PS were compared according to age, gender, and valvular gradient at the time of initial diagnosis. Results: The mean age of patients was 26.1±37.6 months at diagnosis. The mean follow-up duration was 27.65±21.60 (1- 120) months. There was a significant decrease in the final gradient of the PS group compared to the baseline (23.58±6.97 vs. 19.88±11.21 mmHg, p=0.001). In the AS group, there was an increase in the final gradient, which was more pronounced in patients ≤1-year-old (22.42±6.12 vs. 27.74±14.12 mmHg, p=0.002). Four percent of patients in the PS group and 12% of patients in the AS group progressed to moderate to severe stenosis. All patients who progressed in the PS group were ≤1-year-old and male. Conclusion: The results showed that mild PS had a better prognosis than mild AS and that the risk of progression in AS was higher. Careful follow-up should be performed in mild PS cases ≤1-year-old, especially in boys, since progression may be detected, even if infrequently. Mild AS should also be followed closely, as the disease may show progressive characteristics in all age groups.

Transcatheter closure of tubular patent ductus arteriosus using muscular ventricular septal defect devices in infants and small children with congestive heart failure

Transcatheter closure of a patent ductus arteriosus in children is widely performed to reduce symptoms and decrease the risk of endocarditis. Most arterial ducts are closed successfully with dedicated devices. However, in a tubular or "type C" patent ductus arteriosus with congestive heart failure, the occlusion is more challenging with these devices, with a higher risk of complications, such as aortic or left pulmonary stenosis and device embolization. To report our experience with muscular ventricular septal defect devices for patent ductus arteriosus occlusion in small children. Retrospective observational series of patients weighing<10kg, with a tubular patent ductus arteriosus (typeC) and congestive heart failure, who underwent transcatheter closure with a muscular ventricular septal defect device between 2017 and 2019. Eight patients were included. The mean age and weight at closure were 6.3 months (range 1-18 months) and 5.3kg (range 2.4-8.2kg), respectively. All patent ductus arteriosus were occluded successfully using Occlutech® (N=3) or Amplatzer® (N=5) muscular ventricular septal defect devices. In four cases, the muscular ventricular septal defect device was used after failure to close the patent ductus arteriosus with a dedicated patent ductus arteriosus device. Two patients had mild left pulmonary artery stenosis, with a maximum velocity on continuous Doppler of 3m/s and 2.7m/s, respectively. After a mean follow-up of 28 months (range 14-41 months), all patients were asymptomatic with excellent results. The mild pulmonary stenosis improved, with a maximum velocity of 2.3m/s in both patients. Closure of tubular patent ductus arteriosus in small children with congestive heart failure using a muscular ventricular septal defect device is safe in this preliminary experience. Further studies with more patients are warranted.

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.