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.

Off label use of atrial septal occluder in huge arterio-venous malformation with giant aneurysm.

Pulmonary arterio-venous fistula (PAVF) is an uncommon cause of cyanosis and should be suspected when normal cardiac examination is associated without evidence of intra-cardiac shunt. Off-label use of occluder devices in the catheter laboratories can be helpful and safe when chosen according to the morphology, site, and the size of the fistula, and it is considered a good alternative to surgery as it selectively occludes the PAVF while preserving the normal pulmonary vessels. Our case was a young boy accidentally discovered and diagnosed as having huge PAVF, after false diagnosis of coronavirus disease 2019. The fistula was successfully closed using septal occluder device which is not common to use such device in such lesion. Follow up with computed tomography pulmonary angiogram confirmed the closure results with good device position and no residual shunt. Learning objectives: 1To encourage the use of simple non-invasive tools like pulse oximeter that can help in the diagnosis of clinically un-discovered de-saturated patients.2To be malleable with the different occluder devices and be able to use any, according to the lesion you have to close not only those that they were designed for.

The dilemma after an unforeseen aortic arch anomalies at thoracoscopic repair of esophageal atresia: Is curtailing surgery still a necessity?

BACKGROUND AND OBJECTIVE: There are several forms of relevant epi-aortic branching anomalies, and perhaps that is why different views as to the best approach have been reported. To help resolve this dilemma, we examined the unforeseen arch anomalies found at thoracoscopic repair of esophageal atresia and the outcomes. METHODS: In a retrospective cohort, all consecutive patients who were thoracoscopically approached for esophageal atresia over a 5-year period with unforeseen aortic/epi-aortic branching were identified and grouped. Thoracoscopic views, operative interventions, and outcomes were studied. RESULTS: A total of 121 neonates were thoracoscopically approached for EA, of whom 18 cases with aberrant aortic architecture were selected. Four (3%) cases were diagnosed on a preoperative echocardiography as a right-sided aortic arch, whereas unforeseen anomalous anatomies were reported in 14 cases (11.6%): left aortic arch with an aberrant right subclavian artery (ARSA) (n = 10), right-sided aortic arch with an aberrant left subclavian artery (ALSA) (n = 3), and mirror-image right arch (n = 1). Single postoperative mortality was reported among the group with left arch and ARSA (10%), whereas all the cases with right arch and ALSA died. CONCLUSIONS: In all, 11.6% of the studied series exhibited unexpected aberrant aortic architecture, with higher complication rates in comparison to the typical thoracoscopic repairs. For EA with left aortic arch and ARSA, the primary esophageal surgery could safely be completed. Meanwhile, curtailing surgery-after ligating the TEF-to get advanced imaging is still advised for both groups with the right arch due to the significant existence of vascular rings.

Plasma endoglin in Type2 diabetic patients with nephropathy.

BACKGROUND: Diabetic nephropathy may be a common complication of diabetes mellitus. Endoglin is glycoprotein located on cell surfaces of endothelial cells and is part of the transforming growth factor beta (TGF- ß) receptor. Endoglin expression is enhanced in endothelial cells during injury and inflammation. The aim of this study was to estimate the plasma level of soluble endoglin (sEng) in type 2 diabetic patients (with and without nephropathy). Also to explore its availability as marker for disease progression. METHODS: In this study, sixty eight patients with type 2 diabetes mellitus (T2DM) were included; the patients were sub-grouped to normoalbuminuria without nephropathy and moderately increased albuminuria (microalbuminuria) with nephropathy groups with 13 individuals as control group. Plasma soluble endoglin level was determined using ELISA technique. Fasting plasma glucose (FPG), glycated haemoglobin (HbA1c), lipid profile, and creatinine were determined using colorimetric assay, whereas glomerular filtration rate (GFR) was calculated. RESULTS: The plasma level of sEng of both normoalbuminuria group 1 and microalbuminuria group 2 were significantly higher when compared to control group. While, the plasma level of sEng in microalbuminuria group 2 was nonsignificant lower when compared to normoalbuminuria group 1. Also, there was a significant positive association between plasma level of sEng and HbA1c, HDL-C and urinary albumin concentration in normoalbuminuria group. CONCLUSION: Plasma level of soluble Endoglin is markedly increase prior to alteration in endothelial function, and increases to lesser extent with the developing of diabetic nephropathy which indicated disease progression and development of vascular abnormalities.

Nebulized hypertonic saline to prevent ventilator associated pneumonia in premature infants, a randomized trial.

BACKGROUND/OBJECTIVES: Ventilator associated pneumonia (VAP) is a leading cause of death. Nebulized hypertonic saline solution (HSS) has been used to improve pulmonary clearance and reduce infection in intubated patients. This study examines whether nebulized HSS may reduce VAP in intubated premature infants. METHODS: We analyzed results of 100 intubated premature infants who completed the study. Infants were divided into: (a) the "Control" group, where VAP prevention protocol was implemented and (b) the "Intervention" group, where nebulized HSS twice daily was added to the package of care. Clinical, radiologic, and laboratory evidence of VAP, endotracheal aspirate and blood cultures, and days on mechanical ventilation were compared between groups. RESULTS: VAP occurred in 18% in the intervention group compared to 52% in the control group, relative risk 0.35 (CI:0.18-0.66, p = .001). VAP incidence density was 16/1000 patient-ventilator days in intervention group versus 30/1000 in control group. There was a significant reduction in the days of mechanical ventilation in the intervention group (10.7 ± 8.6 and 16.9 ± 3.4, p < .001). CONCLUSIONS: Nebulized HSS may help preserving lung clearance mechanisms and therefore reduce VAP in premature infants. Multi center, double blinded, randomized, controlled, trial is needed to confirm safety and efficacy of such intervention.

Regression of a large congenital hepatic arteriovenous malformation.

Congenital hepatic arteriovenous malformations are rarely seen in association with persistent neonatal pulmonary hypertension. We report the case of a full-term female newborn who presented with heart failure and respiratory distress soon after birth. Echocardiographic investigation revealed severe persistent pulmonary hypertension and patent ductus arteriosus. Here we report spontaneous regression in size of both the feeder vessel and the vascular bed of the congenital hepatic arteriovenous malformation. We postulate that our conservative use of oral heart failure therapy, in the form of diuretic agents and captopril, decreased the congestion and diameter of the affected vessels.

Imaging of pericardial lymphangioma.

Pericardial cystic lymphangioma is a developmental malformation of the lymphatic system. We report a case of cystic pericardial lymphangioma in the anterior mediastinum in a 1-year-old male child. The lesion was diagnosed with multidetector computed tomography and magnetic resonance imaging (MRI). Histopathological examination showed features of cystic lymphangioma.