Neglected Partial Anomalous Pulmonary Venous Return during Atrial Septal Defect Device Closure.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital heart malformation in which 1 or more pulmonary veins drain into the systemic venous circulation or directly into the right atrium instead of the left atrium. It may occur alongside other congenital heart defects, including atrial septal defect (ASD). All patients with newly diagnosed ASD must be evaluated thoroughly for the likelihood of PAPVR to select surgical or percutaneous procedures. Here, we describe a 10-year-old girl with PAPVR who underwent percutaneous device closure of her secundum ASD with her PAPVR neglected at 3 years of age. We had to correct the anomalous venous connection by removing the device during an intricate procedure. The typical connection of the pulmonary veins to the left atrium was reported on her postoperative echocardiography 1 day after surgery. The patient was discharged without complications, and her first follow-up visit 7 days after discharge was unremarkable. While the most accurate diagnostic tools for PAPVR are cardiovascular magnetic resonance imaging and computed tomographic angiography, a careful examination of the pulmonary veins during pulmonary angiography or transesophageal echocardiography in children helps identify PAPVR in patients with ASD.

Recommendations for Infantile-Onset and Late-Onset Pompe Disease: An Iranian Consensus.

Background: Pompe disease, also denoted as acid maltase or acid α-glucosidase deficiency or glycogen storage disease type II, is a rare, autosomal recessive lysosomal storage disorder. Several reports have previously described Pompe disease in Iran and considering increased awareness of related subspecialties and physicians, the disease's diagnosis is growing. Objective: This guideline's main objective was to develop a national guideline for Pompe disease based on national and international evidence adapting with national necessities. Methods: A group of expert clinicians with particular interests and experience in diagnosing and managing Pompe disease participated in developing this guideline. This group included adult neurologists, pediatric neurologists, pulmonologists, endocrinologists, cardiologists, pathologists, and physiatrists. After developing search terms, four authors performed an extensive literature review, including Embase, PubMed, and Google Scholar, from 1932 to current publications before the main meeting. Before the main consensus session, each panel member prepared an initial draft according to pertinent data in diagnosis and management and was presented in the panel discussion. Primary algorithms for the diagnosis and management of patients were prepared in the panel discussion. The prepared consensus was finalized after agreement and concordance between the panel members. Conclusion: Herein, we attempted to develop a consensus based on Iran's local requirements. The authors hope that disseminating these consensuses will help healthcare professionals in Iran achieve the diagnosis, suitable treatment, and better follow-up of patients with infantile-onset Pompe disease and late-onset Pompe disease.

Kawasaki Disease and Peripheral Gangrene in Infancy.

INTRODUCTION: Early diagnosis and treatment of Kawasaki disease as the most common cause of acquired heart disease in childhood, may significantly improve the prognosis. Diagnosing infantile Kawasaki (younger than a year) is difficult because of obscure symptoms; at the same time they are at the higher risk of coronary abnormalities. CASE PRESENTATION: We report three infants with prolonged (more than 5 days) fever and peripheral gangrene without any other clinical manifestations of Kawasaki disease. Kawasaki was diagnosed due to dilation of coronary artery and other aortic branches, thrombocytosis, and rising of ESR and CRP. All patients were treated with high dose aspirin, IVIG and pulse therapy with methylprednisolone. Additionally, cytotoxic drugs or infliximab were used for two of them because of severe aneurysms in the aortic branches. All 3 patients received aspirin with anti-platelet aggregation dose and 2 patients heparin as an anti-coagulant agent for longtime. After adequate treatment, peripheral gangrene, arterial dilations and aneurysms improved, but during 12 months follow-up coronary aneurysms did not improve completely. CONCLUSIONS: Peripheral gangrene must be regarded as an important sign of infantile Kawasaki disease early treatment of which can prevent severe permanent coronary involvements and sequels.

Noninvasive Evaluation of Myocardial Systolic Dysfunction in the Early Stage of Kawasaki Disease: A Speckle-Tracking Echocardiography Study.

BACKGROUND: Evaluation of myocardial function by speckle-tracking echocardiography is a new method for the early diagnosis of systolic dysfunction. OBJECTIVES: We aimed to determine myocardial speckle-tracking echocardiography indices in Kawasaki Disease (KD) patients and compare them with the same indices in control subjects. PATIENTS AND METHODS: Thirty-two patients (65.5% males) with KD and 19 control subjects with normal echocardiography participated in this study. After their demographic characteristics and clinical findings were recorded, all the participants underwent transthoracic echocardiography. Strain (S), Strain Rate (SR), Time to Peak Strain (TPS), and Strain Rate (TPSR), longitudinal velocity and view point velocity images in the two, three, and four-chamber views were semi-automatically obtained via speckle-tracking echocardiography. RESULTS: Among the patients, Twenty-four cases (75%) were younger than 4 years. Mean global S and SR was significantly reduced in the KD patients compared to controls (17.03 ± 1.28 vs. 20.22 ± 2.14% and 1.66 ± 0.16 vs. 1.97 ± 0.25 1/second, respectively), while there were no significant differences regarding mean TPS, TPSR, longitudinal velocity and view point velocity. Using repeated measure of analysis of variances, we observed that S and SR decreased from base to apical level in both groups. The change in the pattern of age adjusted mean S and SR across levels was significantly different between the groups (P < 0.001 for both parameters). CONCLUSIONS: We showed changes in S and SR assessed in KD patients versus control subjects in the acute phase of KD. However, we suggest that further studies be undertaken to compare S and SR in the acute phase and thereafter in KD patients.

Usefulness of serum brain natriuretic Peptide level for screening hemodynamically significant patent ductus arteriosus in preterm neonates.

OBJECTIVE: We studied usefulness of serum B-type natriuretic peptide level as a screening tool for detecting hemodynamically significant patent ductus arteriosus in the preterm neonates. METHODS: Sixty admitted preterm neonates with gestational age ≤34 weeks, birth weight ≤2500 gr, and age of >3 days have been enrolled in this study. We measured serum B-type natriuretic peptide levels at the beginning and after completion of drug therapy for ductus occlusion. FINDINGS: Mean±SD gestational age and weight was 31±1.9 weeks and 1680±350 gr, respectively. The peptide levels in the neonates with significant duct (n=13) were significantly higher than in those with insignificant duct (n=17) or no duct (n=30) (1667±821 pg/ml versus 667±666 and 309±171, respectively). The peptide level dropped significantly after ibuprofen administration in the neonates with significant PDA (n=13), (1667±1165 pg/ml to 429±386). CONCLUSION: At a cutoff point of 450 pg/ml, B-type natriuretic peptide level had a sensitivity of 92% and specificity of 87%, the negative predictive value of 98.5%, the positive likelihood ratio of 6.92 and the negative likelihood ratio of 0.089 for detecting significant patent duct. Levels below this can eliminate the need for echocardiography.

Transcatheter atrial septal defect closure using occlutech figulla device: a two-center experience.

BACKGROUND: Despite several reports regarding the use of the Occlutech Figulla® Flex septal occluder (OFFSO) in adults, there are few reports on its use in children. We sought to study the result of the transcatheter closure of atrial septal defect (ASD) using the OFFSO in children ≤12 years. METHODS: We enrolled 45 consecutive patients, ranging from 2.5 to 12 years of age, in two large pediatric cardiovascular centers. All the children underwent complete echocardiographic examination before the procedure. Defect/device ratio and device/weight ratio were measured. The device diameter to the cardiac diameter ratio (DD/CD ratio) in anteroposterior projection after device release and the DD/CD index were calculated by dividing the DD/CD ratio by the body surface area. RESULTS: Of the 45 enrolled patients, 25 (55%) were female. The range and mean ± standard deviation (SD) of age were 2.5 to 12 years and 6.8 ± 2.5 years, respectively. The range and mean ± SD weight were 8.5 to 37.0 kg and 19.7 ± 7.2 kg, respectively. Successful implantation was performed in all the patients. No major complications occurred in any of the subjects. We encountered one cobra head deformity in one patient. Neither residual shunt nor conduction abnormality was observed in any of the cases. CONCLUSION: Transcatheter ASD closure using the OFFSO was effective in our pediatric patients. Although this device needs relatively larger delivery sheaths, its use is safe while closing even large defects in children.