Device closure of atrial septal defect with severe pulmonary hypertension in adults: Patient selection with early and intermediate term results.

OBJECTIVE: To describe a subset of atrial septal defect (ASD) with severe pulmonary hypertension (PHT) that is suitable for closure. BACKGROUND: As per American Heart Association/American College of Cardiology guidelines, ASD with elevated pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >2/3rd systemic is considered as a contraindication for closure. METHODS: Patients with anatomically large ASD measuring >25 mm and a high probability of reversible pulmonary vascular disease were subjected to fenestrated device closure, despite severe PHT and elevated PVR. They were discharged on pulmonary vasodilators and were followed for 39.5 ± 8.5 months. Four patients had repeat cardiac catheterization. RESULTS: Six patients underwent successful device closure of large ASD with severe PHT. Their basal Qp:Qs was 2.8 ± 0.3:1 while the systolic PAP and the PVR index (PVRI) were 102.6 ± 11.5 mm Hg and 9.6 ± 1.6 Wu m2 , respectively. Post 100% oxygen inhalation, the Qp:Qs increased to 3.5 ± 0.3:1, systolic PAP remained 103.5 ± 7.6 mm Hg while the PVRI dropped to 5.4 ± 1.1 Wu m2 . The postballoon occlusion systolic PAP decreased to 86.6 ± 8.8 mm Hg. At the last follow-up, their pulmonary artery systolic pressure by tricuspid regurgitation (TR) jet decreased from 105.6 ± 12.6 mm Hg to 45 ± 7.0 mm Hg. During follow-up cardiac catheterization (n = 4), the systolic PAP and PVRI were 55.7 ± 9.2 mm Hg and 3.2 ± 0.4, respectively. CONCLUSIONS: Patients with anatomically big defect and a large left to right shunt at baseline with a high probability of reversible PVR benefit with ASD closure and pulmonary vasodilators, despite significantly elevated PAP and PVRI.

Transcatheter closure of large atrial septal defects using 40 mm amplatzer septal occluder: Single group experience with short and intermediate term follow-up.

OBJECTIVES: To assess feasibility, safety, and efficacy of the use of 40 mm Amplatzer septal occluder (ASO 40) for the closure of large atrial septal defects (ASD). BACKGROUND: There is very little data available on closure of large ASDs with ASO 40. MATERIALS AND METHODS: Case records of patients who underwent ASD closure with ASO 40 between 2002 and 2014 were retrospectively analyzed. All patients had clinical, transthoracic, and transesophageal echocardiographic (TEE) evaluation prior to device closure. Postclosure follow-up was done at 6 weeks, 6 months, and annually thereafter. RESULTS: 87 patients underwent ASD closure using ASO 40 during the study period. Mean age and weight of the group was 32.4 ± 11.6 years and 59.5 ± 11.3 kg respectively. Mean ASD diameter on TEE was 32 ± 2.8 mm. The balloon stretched diameter (N = 40) was 37.8 ± 1.3 mm. The balloon assisted technique was used in 80/87 patients for device deployment. The procedure was successful in 84/87 patients. Follow-up was available in 77 patients over a period of 44 ± 15.7 months. 3/77 patients had a small residual shunt. The severity of tricuspid regurgitation decreased in 40/77 patients. The pulmonary artery systolic pressure decreased from 49.7 ± 9.2 to 41.2 ± 6.2 mm Hg (N = 61; P < 0.05). The right ventricular diameter decreased from 35.1 ± 2.8 to 26.1 ± 3 mm (N = 77; P < 0.05). CONCLUSION: ASO 40 can be used safely and effectively with promising short and intermediate term results.© 2016 Wiley Periodicals, Inc.

Cardiac catheterization in children with pulmonary hypertensive vascular disease.

The risks associated with cardiac catheterization in children with pulmonary hypertension (PH) are increased compared with adults. We reviewed retrospectively all clinical data in children with PH [mean pulmonary artery pressure (mean PAp) ≥25 mmHg and pulmonary vascular resistance index (PVRI) ≥3 Wood units m(2)] undergoing cardiac catheterization between 2009 and 2014. Our strategy included a team approach, minimal catheter manipulation and sildenafil administration prior to extubation. Adverse events occurring within 48 h were noted. Seventy-five patients (36 males), median age 4 years (0.3-17) and median weight 14.6 kg (2.6-77 kg), underwent 97 cardiac catheterizations. Diagnoses included idiopathic or heritable pulmonary arterial hypertension (PAH) (29 %), PAH associated with congenital heart disease (52 %), left heart disease (5 %) and lung disease (14 %). Mean PAp was 43 ± 19 mmHg; mean PVRI was 9.7 ± 6 Wood units m(2). There were no deaths or serious arrhythmias. No patient required cardiac massage. Three patients who suffered adverse events had suprasystemic PAp (3/3), heritable PAH (2/3), decreased right ventricular function (3/3), and pulmonary artery capacitance index <1 ml/mmHg/m(2) (3/3) and were treatment naïve (3/3). No patient undergoing follow-up cardiac catheterization suffered a complication. In 45 % of cases, the data acquired from the follow-up cardiac catheterization resulted in an alteration of therapy. Three percent of children with PH undergoing cardiac catheterization suffered adverse events. However, there were no intra or post procedural deaths and no one required cardiac massage or cardioversion. Follow-up cardiac catheterization in patients receiving pulmonary hypertensive targeted therapy is safe and provides useful information.

Transcatheter closure of large atrial septal defects in children using the left atrial disc engagement-disengagement technique (LADEDT)-technical considerations and short term results.

OBJECTIVE: To report our experience of transcatheter closure (TCC) of large atrial septal defects (ASDs) in children using the left atrial (LA) disc engagement-disengagement technique (LADEDT). METHODS: This is a retrospective study comprising 21 children diagnosed with large secundum ASDs. All of them had an attempt at TCC with the Amplatzer septal occluder (ASO) using LADEDT. They were assessed by clinical examination, ECG and echocardiography before the procedure, at 24 hours, 6 weeks, 6 months, and every 1 year after the device closure. RESULTS: The median age of the cohort was 3 years (range 1-12 years). The weight ranged from 5 to 40 kg (median = 12 kg). The mean ASD diameter was 16.7 ± 4.3 mm. The median device size used was 22 mm (range 15-40 mm). The device was successfully placed from the superior pulmonary vein (left: 12; right: 4) and the LA appendage (LAA) in 2. In three patients the technique failed and we had to resort to the balloon assisted technique (BAT) for a successful deployment. The number of attempts for deployment ranged from 1 to 4 (median 2). Despite appropriate placement, the device was not released in one patient because of deficient and floppy inferior margin. One patient had worsening of mitral regurgitation from mild to moderate grade without further progression till last follow up. There were no other major complications during the follow up (6.2 ± 2.4 months) period. CONCLUSION: TCC of large ASDs in small children using the LADEDT is predictable, reproducible, relatively simple, safe, and effective.

Transcatheter closure of a large ASD from the femoral vein in a patient with Budd-Chiari syndrome.

Anomalies of the inferior vena cava are rare in patients with isolated atrial septal defect (ASD). When present, they usually preclude successful transcatheter closure of the ASD using the femoral route. We report a case of a large secundum ASD in a 3-year-old child, who had a chronic Budd-Chiari syndrome incidentally detected on the cardiac catheterization table. We were able to successfully deploy the device from the femoral route itself using a collateral channel to reach the heart and then using the pulmonary vein deployment technique to successfully close the defect.

Changing outcomes of stem cell transplantation in primary immunodeficiencies: Results from a tertiary-care charitable trust hospital in Mumbai.

Background: Hematopoietic stem cell transplantation in primary immunodeficiency disorders has come a long way since the first transplant in 1968. In India, pediatric stem cell transplantation long-term survival outcomes range from 62.5% to 75%, compared to 90% in high-income countries. Objective: We present single-center data of primary immunodeficiency transplants with immune-reconstitution evaluation after transplantation from a charitable trust hospital. Methods: Retrospective data of children transplanted for primary immunodeficiency disorders from March 2019 to March 2022 in a newly established transplant unit were collected. Data of pretransplant infections and comorbidities, surveillance for carbapenem-resistant Enterobacteriaceae, transplant characteristics, donor source, graft-versus-host disease, posttransplant infections, immune reconstitution, overall survival at 1 year, and immunodeficiency-free survival were collated. Results: Twenty-one patients underwent transplantation for primary immunodeficiency disorders. The median age at transplantation was 3 years and 5 months (range, 7 months to 17 years). Seventy-five percent of the cohort had organ involvement, with lung being the most common organ involved, followed by central nervous system. Fifty-two percent of children had peritransplant infections, with most of them recognized at the pretransplant assessment. Among 20 of 21 children with engraftment, 94% had complete chimerism initially, with 33% developing mixed chimerism over time. The median duration of immunosuppression was 3 months after transplantation, and only 1 child required systemic graft-versus-host disease treatment for more than a year. Immune-reconstitution showed good T-cell recovery at 3 months and naive T-cell production at 6 months. There was no regimen-related or sepsis-related mortality. Overall survival of the cohort was 95% at 1-year follow-up. Immunodeficiency-free survival was 86% after a median follow-up of 20 months. Conclusions: Immunodeficiency-free and graft-versus-host disease-free survival can be achieved in the majority of children with primary immunodeficiencies using enhanced supportive care and the latest transplantation algorithms.

PICC fracture and embolization into pulmonary artery - a rare but potentially life-threatening complication: A case report.

Peripherally inserted central catheter (PICCs) are popular means of long-term intravenous access in oncology patients. Fracture and embolization are rare but potentially serious complications. Here we present an unusual fracture of the PICC line in a 9-year-old boy with Ewing's sarcoma with embolization to the right ventricle (RV) and right pulmonary artery (RPA) which was retrieved percutaneously by trans-catheter snare assisted retrieval. Adequate care and precautions like handling by trained nursing staff/parental education must be undertaken to prevent such complications.

Multisystem Inflammatory Syndrome in Children With COVID-19 in Mumbai, India.

OBJECTIVE: We describe the presentation, treatment and outcome of children with multisystem inflammatory syndrome with COVID-19 (MIS-C) in Mumbai metropolitan area in India. METHOD: This is an observational study conducted at four tertiary hospitals in Mumbai. Parameters including demographics, symptomatology, laboratory markers, medications and outcome were obtained from patient hospital records and analyzed in patients treated for MIS-C (as per WHO criteria) from 1 May, 2020 to 15 July, 2020. RESULTS: 23 patients (11 males) with median (range) age of 7.2 (0.8-14) years were included. COVID-19 RT-PCR or antibody was positive in 39.1% and 30.4%, respectively; 34.8% had a positive contact. 65% patients presented in shock; these children had a higher age (P=0.05), and significantly higher incidence of myocarditis with elevated troponin, NT pro BNP and left ventri-cular dysfunction, along with significant neutrophilia and lympho-penia, as compared to those without shock. Coronary artery dilation was seen in 26% patients overall. Steroids were used most commonly for treatment (96%), usually along with intra-venous immunoglobulin (IVIg) (65%). Outcome was good with only one death. CONCLUSION: Initial data on MIS-C from India is presented. Further studies and longer surveillance of patients with MIS-C are required to improve our diagnostic, treatment and surveillance criteria.

Biotinidase deficiency.

A three month old baby presented with refractory seizures, dermatosis and persistent metabolic acidosis. Biotinidase deficiency was diagnosed on enzyme assay. Patient responded dramatically to biotin supplementation.

Atrial septal defect with pulmonary hypertension: when/how can we consider closure?

Patients having atrial septal defect (ASD) with moderate and more importantly severe pulmonary arterial hypertension (PAH) pose a clinical dilemma. Closing ASD in those with irreversible PAH and not closing it when the PAH is reversible can cost patients dearly, both in terms of quality of life and longevity. In our experience, there is no single parameter that can help in decision making in this difficult subset of patients and therefore we recommend a multi-dimensional approach, which takes into consideration clinical, radiological, electrocardiographic and hemodynamic variables as a whole. ASD with restrictive left ventricular (LV) physiology can lead to pulmonary venous hypertension, which can manifest as life threatening acute pulmonary edema following device closure. All high-risk candidates prone to having this combination should be prepared with diuretics and vasodilators prior to bringing them to catheterization laboratory and should be assessed with temporary balloon/device occlusion prior to permanent closure of the defect. In those cases of ASD with borderline operability either due to severe PAH or LV restrictive physiology, perforated device may be helpful in preventing acute or long-term complications of complete closure.

Key technical steps in dual-pathway repair of congenital pulmonary vein stenosis.

We present the case of a 2-year-old girl with congenital stenosis of the left inferior pulmonary vein associated with a large perimembranous ventricular septal defect. The child underwent repair of the left inferior pulmonary vein with autologous left atrial appendage as a pedicled tube, followed by closure of the ventricular septal defect. Important technical steps to minimize the restenosis rate are highlighted.

Dual pathology causing severe pulmonary hypertension following surgical repair of total anomalous pulmonary venous connection: Successful outcome following serial transcatheter interventions.

Surgical repair of total anomalous pulmonary venous connection (TAPVC) can be complicated by the development of pulmonary venous stenosis later on. In addition, the vertical vein, if left unligated, can remain patent and lead to hemodynamically significant left to right shunting. We report an infant who required transcatheter correction of both these problems after surgical repair of TAPVC.

The Voice of the Heart: Vowel-Like Sound in Pulmonary Artery Hypertension.

Increased blood pressure in the pulmonary artery is referred to as pulmonary hypertension and often is linked to loud pulmonic valve closures. For the purpose of this paper, it was hypothesized that pulmonary circulation vibrations will create sounds similar to sounds created by vocal cords during speech and that subjects with pulmonary artery hypertension (PAH) could have unique sound signatures across four auscultatory sites. Using a digital stethoscope, heart sounds were recorded at the cardiac apex, 2nd left intercostal space (2LICS), 2nd right intercostal space (2RICS), and 4th left intercostal space (4LICS) undergoing simultaneous cardiac catheterization. From the collected heart sounds, relative power of the frequency band, energy of the sinusoid formants, and entropy were extracted. PAH subjects were differentiated by applying the linear discriminant analysis with leave-one-out cross-validation. The entropy of the first sinusoid formant decreased significantly in subjects with a mean pulmonary artery pressure (mPAp) ≥ 25 mmHg versus subjects with a mPAp < 25 mmHg with a sensitivity of 84% and specificity of 88.57%, within a 10-s optimized window length for heart sounds recorded at the 2LICS. First sinusoid formant entropy reduction of heart sounds in PAH subjects suggests the existence of a vowel-like pattern. Pattern analysis revealed a unique sound signature, which could be used in non-invasive screening tools.

Active right atrial emptying fraction predicts reduced survival and increased adverse events in childhood pulmonary arterial hypertension.

BACKGROUND: Right atrial (RA) function has been studied rarely in childhood pulmonary arterial hypertension (PAH). We sought to determine if RA and right ventricular (RV) area changes measured by echocardiography predicted outcomes. METHODS: We reviewed data from children with PAH undergoing cardiac catheterization and echocardiography. RA and RV areas were obtained from the apical 4-chamber view. Clinical worsening indicated initiation of parenteral prostanoid therapy, heart and/or lung transplantation, Potts shunt surgery or death. RESULTS: We studied 57 children (27 females), median age 3 years (range 0.30-17 years), body surface area 0.56 m2 (0.2-1.8), follow up 3 years (0.21-8.35), time to clinical worsening was 1.14 years (0.03-6.14) and mortality was 1.55 years (range 0.88-4.95). We determined from receiver operator curves that RA active emptying fraction (RA EaF) ≥60% predicted clinical worsening (sensitivity 78%, specificity 69%, AUC 0.7) and mortality (sensitivity 100%, specificity 65%, AUC 0.82). RV fractional area change (RVFAC) <25% predicted clinical worsening (sensitivity 72%, specificity 79%, AUC 0.85) and death (sensitivity 67%, specificity 69%, AUC 0.77). The combination of RA EaF ≥60% and RVFAC <33% were best predictors of clinical worsening (sensitivity 72%, specificity 82%, partial AUC 0.65) and mortality (sensitivity 100%, specificity 77%, partial AUC 0.75). CONCLUSION: In childhood PAH, RA EaF ≥ 60% and RVFAC <25% were associated with poor outcomes. RA EaF ≥60% and RVFAC <33% were best predictors of clinical worsening and may be useful markers in children with PAH who require closer observation and more intensive therapy.

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects.

OBJECTIVES: Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. METHODS: We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. RESULTS: The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). CONCLUSIONS: Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results.

Transposition of the Great Arteries With Total Anomalous Pulmonary Venous Connection.

Transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is a rare association. Very few such cases have been reported. Among them 1 patient underwent anatomic repair. The rest received repair at the atrial level with either a modified Senning technique or a Mustard technique. We report a rare combination of TGA, TAPVC draining to the coronary sinus, and left juxtaposition of the atrial appendages with a diminutive right atrium. The anomalies were successfully repaired by anatomic correction.

Right Posterior Thoracotomy for Open-Heart Surgery in a Rare Morphology.

The closure of atrial septal defects through right-sided limited posterior thoracotomy has been well established in selected subsets. We present a case of large ostium secundum atrial septal defect, pulmonary valvar stenosis, absent right superior vena cava, and isolated left superior vena cava draining to right atrium via coronary sinus. The child successfully underwent total correction through limited posterior thoracotomy with necessary modifications of intraoperative steps.