Staged Percutaneous Management of Pulmonary Atresia and Intact Interventricular Septum: Stretching the Limits.

Aims: Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by catheter-based interventions and complemented by various surgical procedures. We aim to determine a long-term treatment strategy to enable patients to be surgery free, depending solely on percutaneous interventions. Methods and Results: We selected five patients from among a cohort of patients with PA/IVS treated at birth with radiofrequency perforation and dilatation of the pulmonary valve. Patients had reached a pulmonary valve annulus of 20 mm or larger on their biannual echocardiographic follow-up, with right ventricular dilatation. The findings, together with the right ventricular outflow tract and pulmonary arterial tree, were confirmed by multislice computerised tomography. Based on the angiographic size of the pulmonary valve annulus, all patients were successfully implanted with either Melody® or Edwards® pulmonary valves percutaneously, regardless of their small weights and ages. No complications were encountered. Conclusion: We managed to stretch the age and weight limitations for performing percutaneous pulmonary valve implantation (PPVI): interventions were attempted whenever a pulmonary annulus size of >20 mm was reached, which was rationalised by the prevention of progressive right ventricular outflow tract dilatation and accommodating valves between 24 and 26 mm, which is enough to sustain a normal pulmonary flow in adulthood.

Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum.

Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

Transcatheter closure of residual patent ductus arteriosus.

INTRODUCTION: Residual patent ductus arteriosus (rPDAs) can occur following surgical or transcatheter treatment, and are indicated for closure because of the risks of infective endarteritis and hemolysis in addition to the hemodynamic effect of the residual left-to-right shunt. METHODS: This retrospective descriptive study describes our experience at two Egyptian centers (Cairo University Children's Hospital & Tanta University Hospital) with transcatheter treatment of rPDAs, from January 2009 to October 2017. RESULTS: Twenty cases were treated: 17/20 postsurgical and 3/20 post-transcatheter, at a mean period of 13.4 ± 9.3 months from the initial procedure. The median rPDA size was 2 mm (range2-3.5 mm). Most common ductal anatomy was the conical shape. All rPDAs were successfully closed with either coils (13/20) or devices (6/20), except one case where the residual flow was within the device mesh material. Coils could be deployed from the antegrade or the retrograde approaches although the latter was associated with a higher incidence of late shunt occlusion. One case with a malpositioned device required simultaneous device and LPA stent deployment. CONCLUSION: Transcatheter closure of rPDAs is feasible in most cases, but may be technically challenging.

Importance of anatomical dominance in the evaluation of coronary dilatation in Kawasaki disease.

Introduction In Kawasaki disease, although coronary dilatation is attributed to vasculitis, the effect of myocardial inflammation is underestimated. Coronary dilatations are determined by Z-scores, which do not take into account dominance. The aim of the present study was to describe the impact of coronary dominance on dilatation in Kawasaki disease. METHODS: We performed a retrospective analysis of coronary dilatations according to angiography categorisation of dominance. RESULTS: Of 28 patients (2.6 [0.2-10.1] years), right dominance was present in 15 patients and left in 13. Early dilatation was present in all patients, of whom 11 were ipsilateral to the dominant segment and 17 contralateral. Ipsilateral dilatations were present at diagnosis (9/11 versus 6/17, p=0.02) compared with contralateral dilatations, which developed 2 weeks after diagnosis (9/11 versus 16/17, p=0.29). Coronary artery Z-scores of patients with contralateral dilatation increased at 2 weeks, before returning to baseline values (2.0±2.2 at diagnosis, 4.1±1.8 at 2 weeks, 1.8±1.2 at 3-6 months, p=0.001), compared with patients with ipsilateral dilatation in whom Z-scores were maximal at diagnosis and remained stable (3.0±0.9, 2.7±1.1 and 2.6±1.5, respectively, p=0.13). Dominant coronary artery Z-scores were higher compared with non-dominant segments at diagnosis (3.0±0.9 versus 1.0±0.8, p<0.001) and at late follow-up (2.6±1.5 versus 0.4±1.4, p=0.002) in patients with ipsilateral dilatation. CONCLUSION: Progression of coronary dilatation after diagnosis may be a sign of dilatation secondary to vasculitis, as opposed to regression of Z-scores in ipsilateral dilatations, probably related to physiological vasodilatation in response to carditis. This needs to be validated in larger studies against vasculitic and myocardial inflammatory markers.

Percutaneous angioplasty used to manage native and recurrent coarctation of the aorta in infants younger than 1 year: immediate and midterm results.

Balloon angioplasty (BAP) used to manage native coarctation of the aorta (CoAo) in infants remains controversial. This study aimed to compare short- and midterm results of BAP between native CoAo (NaCo) and postsurgical recoarctations (ReCo) in infants younger than 1 year. This retrospective study compared the clinical, echocardiographic, hemodynamic, and angiographic data for infants who underwent BAP between July 2003 and September 2012. The 12 NaCo and 13 ReCo patients in this study underwent BAP at 4.61 ± 3.69 and 4.88 ± 3.07 months (p = 0.84) and weighed 5.49 ± 2.57 and 6.10 ± 2.11 kg (p = 0.52), respectively. Their respective heights were 60.58 ± 10.58 and 61.15 ± 6.74 cm (p = 0.87). All the ReCo patients had their initial surgery before the age of 3 months. The minimal CoAo diameter was 2.81 ± 0.96 mm in the NaCo group and 2.86 ± 1.0 mm in the ReCo group (p = 0.90). The relative gradient reduction was 62.79 ± 32.43 % in the NaCo group and 73.37 ± 20.78 % in the ReCo group (p = 0.33). The in situ complication rate (pseudoaneurysm) was 8.33 % in the NaCo group and 7.69 % in the ReCo group (p = 0.74). During the early follow-up evaluation, five NaCo patients (41.66 %) presented with recoarctation requiring BAP reintervention within 1.75 ± 1.41 months (all had their initial BAP before 3 months of age) compared with 1 ReCo patient (7.69 %) (p = 0.165). The mean follow-up period was 3.09 ± 2.69 years for the NaCo patients and 3.6 ± 3.18 years for the ReCo patients (p = 0.69), during which the blood pressure gradient was 12.33 ± 9.67 for the NaCo patients and 7.80 ± 8.78 for the ReCo patients (p = 0.17), with corresponding Doppler peak instantaneous gradients of 21.29 ± 11.19 and 16.20 ± 10.23 (p = 0.24). The resultant diameter ratio between the minimal isthmus diameter and the aortic diameter at the diaphragmatic level was 0.81 ± 0.15 in the NaCo group and 0.85 ± 0.12 in the ReCo group (p = 0.53). The immediate and midterm results of BAP for the NaCo and ReCo infants were comparable. Accordingly, BAP seems to be an acceptable alternative to surgery for infants older than 3 months.

Genetic Variations of ferroportin-1(FPN1-8CG), TMPRSS6 (rs855791) and Hemojuvelin (I222N and G320V) Among a Cohort of Egyptian ß-Thalassemia Major Patients.

Iron overload remains a major cause of morbidity and mortality among ß-thalassemia major (ß-TM) patients. Iron regulatory proteins and their genetic variants together with changes in hepcidin levels in thalassemic patients could affect the disease manifestations. This work aimed to study genetic variations of ferroportin-1 (FPN1-8CG), Transmembrane Serine Protease 6 (TMPRSS6 rs855791) and hemojuvelin (HJV I222N and G320V) genes within a cohort of 97 ß-TM Egyptian patients by Polymerase chain reaction Restriction Fragment Length Polymorphism (PCR-RFLP) in comparison to fifty normal control subjects. Among ß-TM patients; the CG variant of FPN1 was significantly higher, while the TT and TC variants of TMPRSS6 were significantly lower in comparison to controls. Liver Iron Concentration (LIC) was significantly higher among ß-TM patients harboring the FPN1 (GG) genotype and we found that FPN1gene mutation acts as independent predictor of MRI LIC (p = 0.011), Pulmonary artery pressure (PAP) was significantly higher in patients harboring the mutant FPN1 (GG and CG) genotypes (p value 0.04). ß-TM patients having the HJV I222N (AA) genotype were having significantly higher cardiac iron overload (p value = 0.026). The studied genetic variants of iron regulatory proteins could alter the manifestations of iron overload thus resulting in different clinical phenotypes of thalassemic patients, these findings need to be confirmed by larger cohorts of patients with longer follow-up periods. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-022-01580-8.

Exclusively endoscopic management of complicated pineal cysts in young children: Definitive treatment through single burr-hole technique.

Background: The management of complicated symptomatic pineal cysts in the pediatric population is challenging and variable. Surgical management may include treatment of hydrocephalus alone, or direct treatment of the cyst with or without direct hydrocephalus management. This is typically done through craniotomy-based microsurgical approaches to the pineal region or an endoscopic transventricular approach. Methods: We present a stepwise minimally invasive technique to treat complicated pineal cysts in young children associated with an obstructive hydrocephalus in a single procedure through third ventriculostomy combined with an intraventricular marsupialization of the pineal cyst through a single burr-hole using stereotactic navigation. Results: Two young patients with over 2 years of follow-up have done well without complication using this technique. Other literature reports for complex pineal cysts in pediatric patients are reviewed and this technique is not previously described for this population. Conclusion: Endoscopic third ventriculostomy and cyst marsupialization using a single burr-hole and stereotactic navigation for symptomatic or enlarging pineal cysts in children allow for minimally invasive management, a rapid recovery, short hospital stay, and durable outcome owed to redundant CSF flow pathways.

Outcome Predictors in Catheter Interventions for Severe Right Ventricular Outflow Tract Obstructions.

Introduction & aim of work: Transcatheter treatment for critical pulmonary stenosis and membranous pulmonary atresia has become the gold standard of care in many centers. We aimed at evaluating the predictors of outcome in interventions for treatment of duct-dependent right ventricular outflow tract obstruction with intact interventricular septum. Subjects & methods: 68 cases with pulmonary atresia with intact interventricular septum (PA/IVS) and 50 cases with critical pulmonary stenosis (CPS), all younger than 3 months of age, were operated during the period of 10 years; excluding patients with tricuspid valve annulus Z-score smaller than -4, evidence of right ventricular-dependent coronary circulation or additional malformations. Results: Age, weight, body surface area as well as tricuspid & pulmonary valve Z-scores were significantly less in PA/IVS; right ventricular pressure was similar in both groups however procedural success and survival to hospital discharge was higher in the CPS group. Lower age, weight and body surface area were associated with procedural failure. Weight was the only predictor of procedural success; while weight and lower post-procedural right ventricular pressure were independent predictors for survival to hospital discharge. Post-procedural right ventricular pressure and length of stay were less in the CPS group. tricuspid and pulmonary valve annulus Z-scores were the only independent predictors of the post-procedural milrinone duration in PA/IVS. Conclusion: We advocate for the use of larger balloon/pulmonary annulus ratio, to achieve a lower right ventricular pressure not fearing excessive pulmonary regurgitation that might be beneficial for right ventricular growth; and for the combination with ductal stenting in borderline or bipartite right ventricles.

Margin between success and failure of PDA stenting for duct-dependent pulmonary circulation.

OBJECTIVES: Percutaneous patent ductus arteriosus (PDA) stenting is a therapeutic modality in patients with duct-dependent pulmonary circulation with reported success rates from 80-100%. The current study aims to assess the outcome and the indicators of success for PDA stenting in different ductal morphologies using various approaches. METHODS: A prospective cohort study from a single tertiary center presented from January 2018 to December 2019 that included 96 consecutive infants with ductal-dependent pulmonary circulation and palliated with PDA stenting. Patients were divided according to PDA origin into 4 groups: Group 1: PDA from proximal descending aorta, Group 2: from undersurface of aortic arch, Group 3: opposite the subclavian artery, Group 4: opposite the innominate/brachiocephalic artery. RESULTS: The median age of patients was 22 days and median weight was 3 kg. The procedure was successful in 78 patients (81.25%). PDA was tortuous in 70 out of 96 patients. Femoral artery was the preferred approach in Group 1 (63/67), while axillary artery access was preferred in the other groups (6/11 in Group 2, 11/17 in Group 3, 1/1 in Group 4, P <0.0001). The main cause of procedural failure was inadequate parked coronary wire inside one of the branch of pulmonary arteries (14 cases; 77.7%), while 2 cases (11.1%) were complicated by acute stent thrombosis, and another 2 cases with stent dislodgment. Other procedural complications comprised femoral artery thrombosis in 7 cases (7.2%). Patients with straight PDA, younger age at procedure and who had larger PDA at pulmonary end had higher odds for success (OR = 8.01, 2.94, 7.40, CI = 1.011-63.68, 0.960-0.99, 1.172-7.40,respectively, P = 0.048, 0.031,0.022 respectively). CONCLUSIONS: The approach for PDA stenting and hence the outcome is markedly determined by the PDA origin and morphology. Patients with straight PDA, younger age at procedure and those who had relatively larger PDA at the pulmonary end had better opportunity for successful procedure.

In situ clearance of a proximal shunt malfunction in a child with hydrocephalus post cerebral arteriovenous malformation rupture noted intraoperatively.

BACKGROUND: Hydrocephalus shunt malfunctions remain treated with surgical intervention only. Despite efforts at identifying or preventing CSF shunt obstruction, no evidence currently exists to restore CSF flow following proximal occlusion, non-invasively. CASE DESCRIPTION: We present direct intraoperative evidence in the case of a 5-year-old male who developed hydrocephalus subsequent to hemorrhagic presentation post cerebral arteriovenous malformation rupture. After weeks of externalized CSF diversion for clearance of CSF red blood cells, he was taken to the operating room for removal of the external ventricular drain and placement of a ventriculoperitoneal shunt for hydrocephalus. At conclusion of placing his ventriculoperitoneal shunt with ReFlow flusher assist device, his shunt valve reservoir was noted to not refill. Following manual depression of the ReFlow flusher, we identified clearance of debris from the obstructed ventricular catheter allowing reestablished CSF flow through the shunt system under live intraoperative ultrasonography. Subsequently, there was return of brisk refill to the shunt valve reservoir. CONCLUSION: Observations here demonstrate a potentially useful technical strategy toward clearance of proximal shunt obstructions, in situ.

Parameters affecting outcome of paediatric cardiomyopathies in the intensive care unit: experience of an Egyptian tertiary centre over 7 years.

Introduction: Paediatric cardiomyopathies are rare but serious and often life-threatening conditions. In the absence of cardiac transplant and ventricular assist device as treatment options in our region, it is very important to identify patients at higher risk. The aim of this study was to determine the outcome of patients diagnosed with cardiomyopathies and their prognostic indicators. Patients and methods: This study included 92 cases representing all patients diagnosed with cardiomyopathy who were admitted into the pediatric cardiac intensive care unit during the period from January 2012 to September 2018. The patients were classified into two groups according to the outcome: the first group comprised 69 patients who survived, and the second group comprised 23 patients who died. All medical records were reviewed, and data were recorded and analysed. Results: Patients with cardiomyopathies represented 8.6% (92/1071) of all patients with cardiac diseases who were admitted in the study period and in the target age group (0.5-12 years). Dilated cardiomyopathy (DCM) was the most frequent type of cardiomyopathy among the admitted patients (80 patients), while 6 patients were diagnosed with hypertrophic cardiomyopathy (HCM), 4 were diagnosed with restrictive cardiomyopathy (RCM), and only 2 were diagnosed with mixed DCM-RCM. Seventy patients required inotropic support (76.1%). Assisted mechanical ventilation was used on 15 patients (16.3%). Twenty-three patients (25.0%) died during the 7-year study period. Conclusions Conclusions The occurrence of hypotension, abnormally high liver enzymes, the need for mechanical ventilation and the need for multiple inotropic drugs were found to be statistically significant predictors of mortality, while age, sex, fractional shortening, ejection fraction, presence of mitral regurgitation, mural thrombus, electrolyte disturbance and arrhythmias did not predict or affect patients' outcomes.

Percutaneous transcatheter valve-in-valve implantation with the balloon-expandable valve for the treatment of a dysfunctional tricuspid bioprosthetic valve: a pediatric case report.

An inverted Edwards SAPIEN 23 mm valve was implanted in a 14-year-old patient with Ebstein's anomaly who received a Medtronic Mosaic 25 mm valve 8 years earlier and presented with significant progressive symptoms related to severe valvular regurgitation and moderate stenosis. The procedure was performed via the femoral vein using the RetroFlex 3 system and predilatation of the tricuspid valve, under transesophageal echocardiographic guidance. The patient had an immediate drop in right atrial and trans-tricuspid pressures with mild regurgitation, and had stable results on short-term follow-up.

Predictive value of intraoperative diagnosis of residual ventricular septal defects by transesophageal echocardiography.

BACKGROUND: Intraoperative transesophageal echocardiography (IOTEE) is well established as a monitoring tool during ventricular septal defect (VSD) repair to ensure complete closure of the defect. Residual shunts detected by IOTEE are common. The predictive value of IOTEE findings on the long-term course of residual shunts is not well documented, especially in regard to the need for reoperation or bacterial endocarditis prophylaxis. The objective of this study is to determine the predictive value of intraoperative IOTEE diagnosis of residual VSDs and therefore delineating the natural history of these findings. METHODS: Retrospective review of IOTEE reports of 690 consecutive patients with VSD (isolated or part of a complex lesion) was undertaken. Those were compared with transthoracic echocardiographic reports of these patients before their discharge from the hospital, and the most recent transthoracic echocardiographic examination. Positive and negative predictive values, sensitivity, and specificity of such diagnoses were then calculated from predischarge and from follow-up transthoracic echocardiographic data. RESULTS: There were 260 of 690 patients with a residual VSD on IOTEE; 24 required repeat cardiopulmonary bypass for complete closure. There were 573 patients with predischarge transthoracic echocardiographic examination; 296 had residual VSDs (125 not detected by IOTEE), and 13 defects required reoperation during the same hospitalization, 5 of which were detected by IOTEE. The positive and negative predictive values were 78% and 65%, respectively. Follow-up transthoracic echocardiographic examination of 383 local patients showed residual VSD in 57 (37 not detected by IOTEE), with positive and negative predictive values of 15% and 83%, respectively. CONCLUSIONS: Although IOTEE is sensitive enough to detect residual VSD shunts in many patients (37% of this cohort), the majority of these defects are trivial and resolve spontaneously, with a positive predictive value of only 15% on follow-up transthoracic echocardiographic examination and a rare need for reoperation.