Upward spontaneous migration of ventriculoperitoneal shunt into the heart: A case report summary.

A male infant with a history of ventriculoperitoneal (VP) implantation due to congenital hydrocephalus presented with fever and lethargy at the age of 8 month-old. Pericardial effusion was detected in transthoracic echocardiography, and he underwent pericardial window operation and was discharged in a stable condition. At 11 months of age, he presented again with fever, lethargy, recurrent vomiting, and respiratory distress. In both plain chest radiography and transthoracic echocardiography, VP shunt migration to the heart cavity was observed. The VP shunt had entered into the right ventricle after perforating the diaphragm and pericardium. The patient underwent open-heart surgery due to vegetation at the tip of the VP shunt inside the right heart. Vegetation was removed and the tip of the shunt was returned to the peritoneal cavity. Two weeks after discharge, the patient presented again with symptoms of tachypnea and lethargy. The imaging revealed the entry of the VP shunt about two centimeters into the anterior mediastinum. The patient was transferred to the operation room and the VP shunt was shortened and re-inserted into the peritoneal cavity. Antibiotic treatment was continued for six weeks and the patient was discharged in stable condition. In follow-up visits after two years, the VP shunt functioned well and no particular complication was observed. This case demonstrates that in patients with VP shunt implantation presenting with pulmonary and cardiac symptoms such as respiratory distress, pericardial effusion, and cardiac tamponade after VP shunt implantation, the possibility of VP shunt catheter migration to the mediastinal cavity should be considered.

New solution for old challenge: Device closure of non-centrally positioned atrial septal defect.

Multiple interatrial defects, termed fenestrated ASDs that require closure are not uncommon. The problem arises when a centrally located defect or a patent foramen oval (PFO) is associated with another peripherally located defect. In cases like this, all attempts at crossing the true defect might totally fail or might be difficult because the wire or the catheter crosses the central defect repeatedly despite the use of a sizing balloon. In order to overcome such an issue, we introduce a new technique by which not only the procedure and the fluoroscopy time will be reduced, but also it ceases the mistakes about the number of defects, their size and location.

A case of pheochromocytoma presenting with cardiac manifestation: case report.

BACKGROUND: Pheochromocytomas are rare tumors originating in chromaffin cells which predominantly are located in adrenal glands. Sustained or paroxysmal hypertension (HT) is the most frequent sign of pheochromocytoma. In some cases, it is associated with the classic triad including episodic headaches, sudoresis, and tachycardia; however, we present a case of pheochromocytoma with first presentation of cardiomyopathy. CASE PRESENTATION: The authors describe a rare case of a pheochromocytoma which was first presented with cardiomyopathy in a 7-year-old patient. The patient was admitted with malaise, abdominal pain, polydipsia, and myalgia. Further evaluations revealed hyperglycemia, mild dehydration and sinus tachycardia but no HT. Echocardiography demonstrated some of the signs of cardiomyopathy which was incorrectly diagnosed as viral myocarditis. The patient was discharged with this diagnosis but he presented again with HT crisis a few months later. A diagnosis of pheochromocytoma was assigned after the evaluation of the HT secondary causes. The diagnosis was confirmed by metanephrine assay and the tumor was localized in the adrenal gland using the abdominal MRI. CONCLUSION: Pheochromocytoma can present itself with normotensive cardiomyopathy. Therefore, the possibility of pheochromocytoma should be considered in patients with cardiomyopathy especially in those with positive familial history.

The correlation between serum level of brain natriuretic peptide and amount of left to right shunt.

Introduction: Natriuretic peptides such as brain natriuretic peptide (BNP), atrial natriuretic peptide (ANP) and pro-BNP are secreted in response to atrial and/or ventricular stretch. Left to right shunts such as ventricular septal defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA), are treated medically or surgically. We aimed to evaluate whether the serum level of pro-BNP would be useful to measure the amount of the shunt. Methods: In this cross sectional study, 60 infants and children, in whom physical examinations approved heart murmur, and had undergone echocardiography by which VSD, ASD, or PDA had been proven, were included in the study. The relationship between serum BNP levels and severity of shunt (Qp/Qs) based on echocardiographic and hemodynamic evaluations, was studied. Results: There was a significant relationship between serum level of pro-BNP and the amount of the shunt in the patients with VSD, ASD, and PDA (P=0.01). A positive correlation was seen between pro-BNP serum level and Qp/Qs ratio. The mean ± SE serum level of pro-BNP in patients with Qp/Qs ratio of less than 1.5, equal to 1.5-2, and more than 2 was 30.83±2.4, 217.88±44.6, and 217.13±51.8, respectively showing a significant relationship (P=0.0001). The cut-off point of pro-BNP demonstrating a Qp/Qs ratio more than 1.5 was measured at the level of 40.36 pg/mL, with a sensitivity and specificity of 92% and 79%, respectively. Conclusion: Based on our study, the cut-off point of 40.36 pg/mL or more for pro-BNP, showing a Qp/Qs ratio more than 1.5, can be considered as an indication for interventional procedures.

Perventricular Muscular Ventricular Septal Defect (VSD) Closure under Epicardial Echocardiography Guidance: A Case Report.

Ventricular septal defects (VSDs) are among the most common congenital cardiac lesions. Large defects at apicomuscular regions, especially in young patients, are far from accessible to surgeons for conventional surgery. Moreover, the transcatheter closure of VSDs in these patients is difficult and carries a high risk of complications because of the large sheath size relative to the patient's size. The periventricular approach simplifies VSD closure and, thus, eliminates the potential complications of cardiac catheterization and fluoroscopy as it is performed under echocardiographic guidance. A 3-year-old girl with a body weight of 11 kg (failure to thrive) was referred to us. She had multiple adjacent apicomuscular VSDs, the largest one being about 19 mm in diameter, and subsystemic pulmonary artery pressure (PAP). The patient underwent periventricular apicomuscular VSD closure with a Lifetech muscular VSD occluder (size 22 mm) under epicardial echocardiography guidance without cardiopulmonary bypass. Post procedure, the PAP was decreased to mild level. The residual shunt was mild across the adjacent small defects. She was discharged after 7 days without complications. At 2 years' follow-up, the patient was hemodynamically stable and had a normal PAP (PAP = about 16 mmHg) by transthoracic echocardiographic assessment.

Misdiagnosis of bland-white-garland syndrome: report of two cases with different presentations.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome is usually an isolated cardiac anomaly but, in rare incidences, has been described with patent ductus arteriosus, ventricular septal defect, and tetralogy of Fallot. This syndrome may cause sudden death in infants and young people but in this case report we present two different types of presentation. First case was a 3 year old girl diagnosed with dilated cardiomyopathy since her infancy. Her electrocardiography showed prominent Q wave in lateral leads. Dilated right coronary artery was revealed by echocardiography. The second case was a girl with prolapsed mitral valve and chest pain but similar to first case she had prominent Q wave in lateral leads at her electrocardiography and dilated right coronary artery but without heart failure. ALCAPA in children may present with ambiguous presentations differing from dilated cardiomyopathy and full blown heart failure to an atypical chest pain attributed to prolapsed mitral valve.

The effect of cyanosis on active clotting time during diagnostic catheterization.

INTRODUCTION: Cardiac catheterization is a common procedure which needs a careful coagulation monitoring. In our study, we aimed to find factors influencing active clotting time (ACT) following heparin therapy. METHODS: ACT of 71 patients who were scheduled to undergo transcutaneous diagnostic catheterization and angiography were measured at baseline, 2 and 60 minutes after 50 IU/kg heparin loading. ACT in two groups of patients (cyanotic and non-cyanotic) was compared. All data were analyzed with Wilcoxon, Mann-Whitney test and Pearson in SPSS 16, P value less than 0.05 was considered significant. RESULTS: ACT following heparin at 2nd and 60th minutes was not significantly different in cyanotic and non-cyanotic groups. At 60th minute following heparin administration, ACT decreased more dramatically in older children. CONCLUSION: Cyanosis does not affect ACT measures following heparin treatment. Moreover, after 60 minutes, heparin efficacy (ACT values) decreased more with increase in patients' age.

Complications of Aortic Stenting in Patients below 20 Years Old: Immediate and Intermediate Follow-Up.

BACKGROUND: Optimal timing and mode of treatment for patients with coarctation of the aorta (COA) remain controversial, particularly in children. Surgery, balloon dilatation, and stent implantation have all proven effective in the treatment of moderate or severe obstruction. The aim of this study was to investigate the complications of COA stenting angioplasty in pediatric patients. METHODS: This retrospective, descriptive study was conducted on patients less than 20 years of age who underwent aortic stenting angioplasty because of congenital COA in the pediatric catheterization laboratory of Rajaie cardiovascular, medical and research Center, Tehran between 2005 and 2010. RESULTS: A total of 26 patients (18 [65.4%] males and 9 [34.6%] females) with congenital COA who had undergone aortic stenting angioplasty were recruited. Nineteen (73.1%) of these patients had native COA and 7 (26.9%) had recurrent COA. Most of the early complications were minor and temporary; only one patient developed early major complications. During the follow-up, whereas none of the native group patients developed late complications, in the re-COA group 28.57% of the patients had re-stenosis and 14.28% had chronic systemic hypertension, requiring drug therapy. CONCLUSION: Our investigation into post-stenting complications in patients with native COA and re-COA showed that endovascular stenting could be an effective and safe method, even in young patients with native COA.