Case report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement.

Background: Persistent left superior vena cava (LSVC) with connection to the left atrium (LA) is a rare anomaly with serious clinical implications. Depending on the direction of flow through the intracardiac shunt, clinical presentation varies from cyanosis or paradoxical embolism to overt right heart failure. Case summary: A 26-year-old man with repaired ventricular septal defect (VSD) during infancy presented with symptoms of progressive exercise intolerance. Cardiac imaging revealed a large defect at the posterior wall of the LSVC above its entry to the coronary sinus (CS), connecting the LSVC with the LA and resulting in diversion of pulmonary venous return to the CS. All pulmonary veins connect normally to the LA. The large left-to-right intracardiac shunt led to significant right ventricular dilation and tricuspid regurgitation. He underwent successful anatomical repair with transcatheter implantation of covered stent from LSVC to the CS, redirecting pulmonary venous return to the LA. At 1 year follow up, his exercise capacity had improved, and cardiac imaging showed complete seal of the LSVC defect without obstruction to pulmonary venous return. Discussion: Our case is the first to our knowledge to report this unusual anatomic variant of LSVC to LA connection, and complete repair by transcatheter treatment. Previous case reports of other forms of LSVC to LA connection were treated with surgery or device occlusion without reconnection of LSVC. This case highlights the efficacy and safety of innovative percutaneous techniques in the management of complex congenital heart lesions. Meticulous procedural planning through 3D modelling and simulation is vital to mitigate the risks of these innovative procedures.

Renaissance of Cardiac Imaging to Assist Percutaneous Interventions in Congenital Heart Diseases:The Role of Three-Dimensional Echocardiography and Multimodality Imaging.

Percutaneous interventions have completely refashioned the management of children with congenital heart diseases (CHD) and the use of non-invasive imaging has become the gold standard to plan and guide these procedures in the modern era. We are now facing a dual challenge to improve the standard of care in low-risk patients, and to shift our strategies from the classic open chest surgery to imaging-guided percutaneous interventions in high-risk patients. Such rapid evolution of ultrasound technologies over the last 20 years have permitted the integration of transthoracic, transesophageal and intracardiac echocardiography into the interventional workflow to improve image guidance and reduce radiation burden from fluoroscopy and angiography. Specifically, miniaturization of transesophageal probe and advances in three-dimensional (3D) imaging techniques have enabled real-time 3D image guidance during complex interventional procedure, In addition, multimodality and fusion imaging techniques harness the strengths of different modalities to enhance understanding of anatomical and spatial relationship between different structures, improving communication and coordination between interventionalists and imaging specialists. In this review, we aim to provide an overview of 3D imaging modalities and multimodal fusion in procedural planning and live guidance of percutaneous interventions. At the present times, 3D imaging can no longer be considered a luxury but a routine clinical tool to improve procedural success and patient outcomes.

Long term outcomes in CDH: Cardiopulmonary outcomes and health related quality of life.

BACKGROUND: With improvements in clinical management and an increase in CDH survivorship there is a crucial need for better understanding of long-term health outcomes in CDH. AIM: To investigate the prevalence of cardiopulmonary health morbidity and health related quality of life (HRQoL) in CDH survivors. METHODS: We included all studies (n = 65) investigating long-term cardiopulmonary outcomes in CDH patients more than 2 years published in the last 30 years. The Newcastle-Ottawa Scale and the CASP checklist for cohort studies were utilized to assess study quality. Results were reported descriptively and collated by age group where possible. RESULTS: The incidence of pulmonary hypertension was highly variable (4.5-38%), though rates (%) appeared to diminish after 5 years of age. Lung function indices and radiological outcomes were frequently abnormal, and Health Related Quality of Life (HRQoL) reduced also. Long term diseases notably emphysema and COPD are not yet fully described in the contemporary literature. CONCLUSION: This study underscores cardiopulmonary health morbidity and a reduced HRQoL among CDH survivors. Where not already available dedicated multidisciplinary follow-up clinics should be established to support these vulnerable patients transition safely into adulthood. Future research is therefore needed to investigate the risk factors for cardiopulmonary ill health and morbidity in CDH survivors. TYPE OF STUDY: Systematic review of case control and cohort studies.

Infrared thermography as an adjunctive tool for detection of femoral arterial thrombosis after cardiac catheterization: A prospective, pilot study.

OBJECTIVES: To assess the utility of infrared thermography (IRT), to map skin temperature, in the detection of femoral arterial (FA) thrombosis after cardiac catheterization. BACKGROUND: Ultrasound is a validated method for thrombus detection but is generally reserved as a confirmatory test for clinical suspicion due to various constraints. METHODS: Prospective study of infants and children undergoing cardiac catheterization via FA access, comparing IRT and pulse examination. The thermograms, displayed in a color map with each pixel representing a temperature, were examined by qualitative assessment of symmetry in thermal patterns and quantitative image analysis with abnormal thermographic asymmetry defined as a difference of >10% between limbs. RESULTS: In the 20 children enrolled, excellent agreement was found between the two methods with a Kappa value of 0.89. The median thermographic asymmetry in the nine children with pulse loss was 36 (13-76)%. Using receiver operating characteristic analysis, the asymmetrical pattern of ≥18% between limbs predicted the need for anticoagulation with a sensitivity of 100% and specificity of 89%. The area under the curve was 0.97 (95% confidence interval: 0.95-1). Children with absent pulse requiring anticoagulation showed a slower recovery in thermal asymmetry compared to those with a reduced pulse. By qualitative IRT assessment, all children with absent pulse requiring anticoagulation were correctly identified by 10 independent assessors. CONCLUSIONS: This pilot study showed that IRT is feasible and reliable as an adjunctive tool for thrombus detection postcatheterization and treatment monitoring. Specific advantages of IRT include portability, affordability, and contactless image acquisition.

Is there a role for endovascular stent implantation in the management of postoperative right ventricular outflow tract obstruction in the era of transcatheter valve implantation?

BACKGROUND: The optimal management pathway for the dysfunctional right ventricular outflow tract (RVOT) is uncertain. We evaluated the long-term outcomes and clinical impact of stent implantation for obstructed RVOTs in an era of rapidly progressing transcatheter pulmonary valve technology. METHODS: Retrospective review of 151 children with a biventricular repair who underwent stenting of obstructed RVOT between 1991 and 2017. RESULTS: RVOT stenting resulted in significant changes in peak right ventricle (RV)-to-pulmonary artery (PA) gradient (39.4 ± 17.1-14.9 ± 8.3; p < 0.001) and RV-to-aortic pressure ratio (0.78 ± 0.22-0.49 ± 0.13; p < 0.001). Subsequent percutaneous reinterventions in 51 children to palliate recurrent stenosis were similarly effective. Ninety-nine (66%) children reached the primary outcome of subsequent pulmonary valve replacement (PVR). Freedom from PVR from the time of stent implantation was 91%, 51%, and 23% at 1, 5, and 10 years, respectively. Small balloon diameters for stent deployment were associated with shorter freedom from PVR. When additional children without stent palliation (with RV-to-PA conduits) were added to the stent cohort (total 506 children), the multistate analysis showed the longest freedom from PVR in those with stent palliation and subsequent catheter reintervention. Pulmonary regurgitation was well-tolerated clinically. Indexed RV dimensions and function estimated by echocardiography remained stable at last follow up or before primary outcome. CONCLUSION: Prolongation of conduit longevity with stent implant remains an important strategy to allow for somatic growth to optimize the risk-benefit profile for subsequent surgical or transcatheter pulmonary valve replacement performed at an older age.

Transcatheter creation of a Potts shunt with the Occlutech Atrial Flow Regulator: Feasibility in a pig model.

BACKGROUND: Creation of a Potts shunt, a connection between the left pulmonary artery (LPA) and descending aorta (DAo), improves functional status and survival in drug-refractory suprasystemic pulmonary arterial hypertension. We investigated a new approach to transcatheter Potts shunt creation in pigs. METHODS AND RESULTS: In six pigs, a steerable SureFlex sheath was used to optimize the trajectory of perforation from the DAo into LPA using a 0.035″ radiofrequency wire. The combination of a larger perforation, stiffer radiofrequency wire and smooth dilator-to-sheath transition allowed sheath entry into the LPA without requiring an arterio-venous wire circuit. The Occlutech Atrial Flow Regulator (AFR), a double-disc device with a central fenestration, was deployed through this sheath with apposition of the distal disc to the posterior LPA wall and the proximal disc to the anterior DAo wall. The AFR is compliant and crumpling of the central fenestration was resolved by balloon dilation. It was feasible to implant a stent within the fenestration (n = 3). Aortography confirmed a left-to-right shunt through the AFR without contrast extravasation. Autopsy demonstrated anchoring of both discs against the vessel walls, patency of the fenestration and secure placement of the stent with no intra-thoracic bleeding. CONCLUSIONS: In an acute pig model, we have demonstrated the feasibility of creating a transcatheter Potts shunt with a simplified technique using a steerable sheath, a double-disc device with a central fenestration that acts as the shunt channel and optional stenting of the fenestration.

Balloon Angioplasty for Native Aortic Coarctation in 3- to 12-Month-Old Infants.

BACKGROUND: Balloon angioplasty for native coarctation of the aorta (CoA) is successful in children and adults but in neonates results in frequent restenosis. The efficacy of balloon angioplasty for native CoA during infancy beyond the neonatal period was examined in infants aged 3 to 12 months of age. METHODS: A retrospective review of 68 infants who underwent balloon angioplasty for native CoA. 95% CI are in parentheses. RESULTS: Procedural age was (mean±SD) 6±3.4 months and weight was 7±1.8 kg. Balloon angioplasty produced a large decrease in both the noninvasive arm-to-leg blood pressure gradient (41.2±18.7 to 5.6±9.6 mm Hg) and the invasive peak systolic pressure gradient (34±12 to 11±9 mm Hg). Balloon angioplasty increased the CoA diameter from 2.7±1 mm to 4.6±1.2 mm. One patient was lost to follow-up. A catheter reintervention was required in 11.8% and surgery in 10.3%. The hazard of reintervention was highest early. Median freedom from reintervention was 89% (95% CI, 80%-96%) at 1 year, 83% (95% CI, 73%-92%) at 5 years, and 81% (95% CI, 69%-90%) at 10 years. Femoral artery thrombosis was documented in 6 (9%) infants without any long-term consequence. One patient developed a small aortic aneurysm late and has not required treatment. A robust estimate of the frequency of aortic aneurysms remains to be determined as the majority of subjects have not had cross-sectional imaging. CONCLUSIONS: Balloon angioplasty of native CoA is effective and safe in infants aged 3 to 12 months with outcomes comparable to those in older children and adults. Catheter reinterventions can avoid the need for surgery in most patients.

Changes in inflammation and oxidative stress signalling pathways in coarcted aorta triggered by bicuspid aortic valve and growth in young children.

Neonates with coarctation of the aorta (CoA) combined with a bicuspid aortic valve (BAV) show significant structural differences compared to neonatal CoA patients with a normal tricuspid aortic valve (TAV). These effects are likely to change over time in response to growth. This study investigated proteomic differences between coarcted aortic tissue of BAV and TAV patients in children older than one month. Aortic tissue just proximal to the coarctation site was collected from 10 children (BAV; n=6, 1.9±1.7 years, TAV; n=4, 1.7±1.5 years, (mean ± SEM, P=0.92.) Tissue were snap frozen, proteins extracted, and the extracts used for proteomic and phosphoproteomic analysis using Tandem Mass Tag (TMT) analysis. A total of 1811 protein and 76 phosphoprotein accession numbers were detected, of which 40 proteins and 6 phosphoproteins were significantly differentially expressed between BAV and TAV patients. Several canonical pathways involved in inflammation demonstrated enriched protein expression, including acute phase response signalling, EIF2 signalling and macrophage production of IL12 and reactive oxygen species. Acute phase response signalling also demonstrated enriched phosphoprotein expression, as did Th17 activation. Other pathways with significantly enriched protein expression include degradation of superoxide radicals and several pathways involved in apoptosis. This work suggests that BAV CoA patients older than one month have an altered proteome consistent with changes in inflammation, apoptosis and oxidative stress compared to TAV CoA patients of the same age. There is no evidence of structural differences, suggesting the pathology associated with BAV evolves with age in paediatric CoA patients.

Mixed-reality view of cardiac specimens: a new approach to understanding complex intracardiac congenital lesions.

Digital reality is an emerging platform for three-dimensional representation of medical imaging data. In this technical innovation paper, the authors evaluated the accuracy and utility of mixed-reality technology in the morphological evaluation of complex congenital heart disease. The authors converted CT datasets of 12 heart specimens with different subtypes of double-outlet right ventricle to stereoscopic images and interrogated them using a mixed-reality system. The morphological features identified on the stereoscopic models were compared with findings at macroscopic examination of the actual heart specimens. The results showed that the mixed-reality system provided highly accurate stereoscopic display of spatially complex congenital cardiac lesions, with interactive features that might enhance 3-D understanding of morphology. Additionally, the authors found that high-resolution digital reproduction of cardiac specimens using clinical CT scanners is feasible for preservation and educational purposes.

An Overview of Contemporary Outcomes in Fetal Cardiac Intervention: A Case for High-Volume Superspecialization?

Fetal cardiac interventions (FCI) offer the opportunity to rescue a fetus at risk of intrauterine death, or more ambitiously to alter disease progression. Most of these fetuses require multiple additional postnatal procedures, and it is difficult to disentangle the effect of the fetal procedure from that of the postnatal management sequence. The true clinical impact of FCI may only be discernible in large-volume institutions that can commit to a standardized postnatal approach and have sufficient case volume to overcome their FCI learning curve.

Three-dimensional rotational angiography in congenital heart disease: Present status and evolving future.

Three-dimensional rotational angiography (3D-RA) enables volumetric imaging through rotation of the C-arm of an angiographic system and real-time 3D reconstruction during cardiac catheterization procedures. In the field of congenital heart disease (CHD), 3D-RA has gained considerable traction, owing to its capability for enhanced visualization of spatial relationships in complex cardiac morphologies and real time image guidance in an intricate interventional environment. This review provides an overview of the current applications, strengths, and limitations of 3D-RA acquisition in the management of CHD and potential future directions. In addition, issues of dosimetry, radiation exposure, and optimization strategies will be reviewed. Further implementation of 3D-RA will be driven by patient benefits relative to existing 3D imaging capabilities and fusion techniques balanced against radiation exposure.

Bicuspid Aortic Valve Alters Aortic Protein Expression Profile in Neonatal Coarctation Patients.

Coarctation of the aorta is a form of left ventricular outflow tract obstruction in paediatric patients that can be presented with either bicuspid (BAV) or normal tricuspid (TAV) aortic valve. The congenital BAV is associated with hemodynamic changes and can therefore trigger different molecular remodelling in the coarctation area. This study investigated the proteomic and phosphoproteomic changes associated with BAV for the first time in neonatal coarctation patients. Aortic tissue was collected just proximal to the coarctation site from 23 neonates (BAV; n = 10, TAV; n = 13) that were matched for age (age range 4-22 days). Tissue from half of the patients was frozen and used for proteomic and phosphoproteomic analysis whilst the remaining tissue was formalin fixed and used for analysis of elastin content using Elastic Van-Gieson (EVG) staining. A total of 1796 protein and 75 phosphoprotein accession numbers were detected, of which 34 proteins and one phosphoprotein (SSH3) were differentially expressed in BAV patients compared to TAV patients. Ingenuity Pathway Analysis identified the formation of elastin fibres as a significantly enriched function (p = 1.12 × 10-4) due to the upregulation of EMILIN-1 and the downregulation of TNXB. Analysis of paraffin sections stained with EVG demonstrated increased elastin content in BAV patients. The proteomic/phosphoproteomic analysis also suggested changes in inositol signalling pathways and reduced expression of the antioxidant SOD3. This work demonstrates for the first time that coarcted aortic tissue in neonatal BAV patients has an altered proteome/phosphoproteome consistent with observed structural vascular changes when compared to TAV patients.

Recent advances in cardiac catheterization for congenital heart disease.

The field of pediatric and adult congenital cardiac catheterization has evolved rapidly in recent years. This review will focus on some of the newer endovascular technological and management strategies now being applied in the pediatric interventional laboratory. Emerging imaging techniques such as three-dimensional (3D) rotational angiography, multi-modal image fusion, 3D printing, and holographic imaging have the potential to enhance our understanding of complex congenital heart lesions for diagnostic or interventional purposes. While fluoroscopy and standard angiography remain procedural cornerstones, improved equipment design has allowed for effective radiation exposure reduction strategies. Innovations in device design and implantation techniques have enabled the application of percutaneous therapies in a wider range of patients, especially those with prohibitive surgical risk. For example, there is growing experience in transcatheter duct occlusion in symptomatic low-weight or premature infants and stent implantation into the right ventricular outflow tract or arterial duct in cyanotic neonates with duct-dependent pulmonary circulations. The application of percutaneous pulmonary valve implantation has been extended to a broader patient population with dysfunctional 'native' right ventricular outflow tracts and has spurred the development of novel techniques and devices to solve associated anatomic challenges. Finally, hybrid strategies, combining cardiosurgical and interventional approaches, have enhanced our capabilities to provide care for those with the most complex of lesions while optimizing efficacy and safety.

Duct Stenting Versus Modified Blalock-Taussig Shunt in Neonates With Duct-Dependent Pulmonary Blood Flow: Associations With Clinical Outcomes in a Multicenter National Study.

BACKGROUND: Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored. Duct stenting (DS) is emerging as one such alternative with potential for greater early stability and improved survival. METHODS: The purpose of this study was to compare postprocedural outcomes and survival to next-stage palliative or reparative surgery between patients undergoing a modified Blalock-Taussig shunt or a DS in infants with duct-dependent pulmonary blood flow. All patients undergoing cardiac surgery and congenital interventions in the United Kingdom are prospectively recruited to an externally validated national outcome audit. From this audit, participating UK centers identified infants <30 days of age undergoing either a Blalock-Taussig shunt or a DS for cardiac conditions with duct-dependent pulmonary blood flow between January 2012 and December 31, 2015. One hundred seventy-one patients underwent a modified Blalock-Taussig shunt, and in 83 patients, DS was attempted. Primary and secondary outcomes of survival and need for extracorporeal support were analyzed with multivariable logistic regression. Longer-term mortality before repair and reintervention were analyzed with Cox proportional hazards regression. All multivariable analyses accommodated a propensity score to balance patient characteristics between the groups. RESULTS: There was an early (to discharge) survival advantage for infants before next-stage surgery in the DS group (odds ratio, 4.24; 95% confidence interval, 1.37-13.14; P=0.012). There was also a difference in the need for postprocedural extracorporeal support in favor of the DS group (odds ratio, 0.22; 95% confidence interval, 0.05-1.05; P=0.058). Longer-term survival outcomes showed a reduced risk of death before repair in the DS group (hazard ratio, 0.25; 95% confidence interval, 0.07-0.85; P=0.026) but a slightly increased risk of reintervention (hazard ratio, 1.50; 95% confidence interval, 0.85-2.64; P=0.165). CONCLUSIONS: DS is emerging as a preferred alternative to a surgical shunt for neonatal palliation with evidence for greater postprocedural stability and improved patient survival to destination surgical treatment.

Stent Therapy for Aortic Coarctation in Children <30 kg: Use of the Low Profile Valeo Stent.

To report our experience of coarctation stent therapy in small children weighing less than 30 kg, with the low profile dilatable Valeo stent and review the literature on coarctation stent therapy in this patient population. Coarctation stent implantation was undertaken in 14 consecutive children using the Bard Valeo Stent. Demographic, angiographic, echocardiographic and clinical data were reviewed retrospectively. The median age at the time of procedure was 5.1 (2.6-7.5) years and median weight was 20.8 (14.7-27) kg. There was improvement in median coarctation diameter from 4 (1.3-5.2) to 9.5 (5.8-12.7) mm, p < 0.001; and a reduction in the median peak pressure gradient across the coarctation from 35 (20-49) to 9 (0-15) mmHg, p < 0.001. Median stent recoil was 7.9 (0-20)%. There was one case of access related complication that resolved without sequelae. Follow-up was a median of 15 (3.0-57.2) months. CT angiogram performed at a median time of 3.3 (2.6-10.2) months post procedure showed no aortic wall injury and preserved stent integrity in all cases. Two children underwent re-intervention for stent dilation and further stent implantation due to in-stent stenosis and somatic growth after 3 years. Six of fourteen children remained on a single antihypertensive agent post-intervention at last follow-up. Implantation of the dilatable Valeo stent is a feasible treatment strategy in native or recurrent coarctation in small children, accepting that additional stent implantation may be necessary with somatic growth. Further study is required to determine longer-term stent efficacy and clinical outcome.

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.

Clinical Characteristics and Outcomes of Cardiomyopathy in Barth Syndrome: The UK Experience.

Barth syndrome (BTHS) is an X-linked disorder characterised by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. This study describes the UK national clinical experience and outcome of cardiomyopathy in BTHS. The clinical course and echocardiographic changes of all patients with BTHS in the UK were reviewed from 2004 to 2014. In addition, strain analysis using 2D speckle tracking echocardiography was performed to further assess left ventricular function in the most recent follow-up. At last follow-up, 22 of 27 patients were alive with a median age of 12.6 (2.0-23.8) years; seven underwent cardiac transplantation at a median age of 2 (0.33-3.6) years, and five died (18.5%) at a median age of 1.8 (0.02-4.22) years. All deaths were related to cardiomyopathy or its management. Left ventricular diastolic dimension and systolic function measured by fractional shortening tended to normalise and stabilise after the first 3 years of life in the majority of patients. However, patients with BTHS (n = 16) had statistically significant reduction in global longitudinal and circumferential strain compared to controls (n = 18), (p < 0.001), despite apparent normal conventional measures of function. There was also reduced or reversed apical rotation and reduced left ventricular twist. Sustained ventricular arrhythmia was not seen at follow-up. Cardiac phenotype in BTHS is variable; however, longer-term outcome in our cohort suggests good prognosis after the first 5 years of life. Most patients appeared to have recovered near normal cardiac function by conventional echocardiographic measures, but strain analysis showed abnormal myocardial deformation and rotational mechanics.

Longer-term outcome of perventricular device closure of muscular ventricular septal defects in children.

OBJECTIVE: To describe the longer-term clinical experience and follow-up with perventricular device closure of ventricular septal defects (VSD) in children. METHODS: Between January 2005 and December 2013, muscular ventricular septal defect closure with the Amplatzer Muscular VSD Occluder was undertaken using a hybrid perventricular approach. Data including demographic, echocardiographic parameter, and clinical outcome were reviewed retrospectively. RESULTS: Median age at the time of procedure was 8.9 months (range 1.9-31.0 months) and median weight was 6.6 kg (range 4.5-12.9 kg). All had a moderate to large muscular VSD, three had more than one VSD, four had previous coarctation repair, and five had previous pulmonary artery (PA) banding. A single Amplatzer muscular occluder (range 8-18 mm) was deployed in each patient without cardiopulmonary bypass under echocardiographic guidance. Two of ten patients subsequently required a short period of cardiopulmonary bypass for reconstruction of PA after de-banding and closure of atrial septal defect in one. Occluder removal was necessary in one patient due to entrapment of the tricuspid valve and progressive tricuspid regurgitation. This patient underwent surgical repair with a good result and no device-related valve damage. In the remaining nine patients, no severe complications such as device embolization, arrhythmia, or significant valve regurgitation were noted in the post-operative period or follow-up. At a median time of 6.5 years (range 0.9-8.4 years) post device implantation, complete closure was achieved in five patients and four had small residual leaks, which were not hemodynamically significant. CONCLUSION: Perventricular muscular VSD closure is effective in small children with suitable muscular defects and may avoid the morbidity associated with cardiopulmonary bypass and conventional surgical repair. There have been no late complications with this approach.

Foetal supraventricular tachycardia with hydrops fetalis: a role for direct intraperitoneal amiodarone.

INTRODUCTION: Persistent foetal tachyarrythmias complicated by hydrops fetalis carry a poor prognosis, with foetal death reported in excess of a quarter despite treatment. We present our experience with direct intraperitoneal amiodarone administration in eight hydropic foetuses with resistant supraventricular tachycardia. METHODS: Amiodarone was injected slowly into foetal peritoneal cavity under ultrasound guidance. All mothers were loaded with oral amiodarone before the procedure and maintained on it. The procedure was repeated guided by foetal rhythm. RESULT: All eight cases had severe hydrops with a median foetal heart rate of 255 bpm (range 240-300 bpm), and the median gestational age was 27+1 weeks (range 21-33+3 weeks) at presentation. In six cases, the average time for supraventricular tachycardia to revert to sinus rhythm from the first procedure was 11.5 days. In one case, intravascular injection of amiodarone into the umbilical vein was performed before intraperitoneal injection, which resulted in conversion to sinus rhythm sustained until delivery. In the last case, supraventricular tachycardia and severe hydrops persisted and the baby was delivered 5 days later at 34 weeks' gestation. Hydrops resolved in five foetuses with a mean resolution time of 28.4 days. The mean gestational age at delivery was 34+5 days and seven of eight cases survived beyond the neonatal period with good postnatal outcomes. CONCLUSION: Intraperitoneal administration of amiodarone is a relatively simple and effective strategy in refractory supraventricular tachycardia complicated by severe hydrops. The intraperitoneal route assures delivery of the drug to the severely hydropic foetus and enables a bolus dose to be delivered for sustained absorption.