Background: The duration of mechanical ventilation (MV) is one of the most important clinical factors which predict outcomes in pediatric cardiac surgery. The prolonged mechanical ventilation (PMV) following cardiac surgery is a multifactorial phenomenon and there are conflicts regarding its predictors in pediatric population between different centers. Objectives: The current study aimed to describe PMVpredictors in patients undergoing cardiac surgery for congenital heart disease in a tertiary center for pediatric cardiovascular diseases in Iran. Patients and Methods: From May to December 2014, all pediatric patients (less than a month -15 years old) admitted to pediatric Intensive Care Unit (PICU) after congenital heart surgeries were consecutively included. The PMV was defined as mechanical ventila- tion duration more than 72 hours as medium PMV and more than seven days as extended PMV. The demographic data and variables probably related to PMV were recorded during the PICU stay. Results: A total of 300 patients, 56.7% male, were enrolled in this study. Their mean age was 32 ± 40 months .The median duration (IQR) of MVwas 18 hours (8.6 - 48 hours). The incidence of PMV more than 72 hours and seven days was 20% and 10.7%, respectively. Younger age, lower weight, heart failure, higher doses of inotropes, pulmonary hypertension, respiratory infections and delayed sternal closure were independent predictors of PMV in multivariate analyses. Conclusions: The results of this study indicated that PMV predictors could be specific for each center and a good administration program is needed for each pediatric cardiac surgery center for the preoperative management of patients undergoing congenital heart surgeries.

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Background: Women with congenital heart disease (CHD) may experience menstrual disturbances secondary to hemodynamic instability during the mensturation phase. Objectives: We investigated the menstrual bleeding pattern and its relationship with certain clinical findings in adult women with CHD. Patients and Methods: Clinical data and menstrual bleeding pattern of adult women >15 years old who were referred to adult CHD clinic between March and September 2014 were recorded. Patients with syndromic congenital anomalies were excluded. Results: Data of 304 women (151 and 153 with simple and complex CHD groups, respectively) were recorded. Their mean (SD) age was 25.2 (1) years (range, 15-46 years). The median (IQR) age at menarche was 13 (12-14.25) years. Menarche was later in patients with CHD than in the normal population. Furthermore, the simple group showed earlier menarche than the complex group. The most common menstrual abnormality was menorrhagia in both groups (14.5% and 20.5% in the simple and complex groups, respectively). The incidence of menstrual abnormality was higher, though not significantly, in the complex group (40% vs. 25% in the simple group; P = 0.2). Menorrhagia was associated with the severity of oxygen desaturation (P= 0.007). Conclusions: Menstrual abnormalities are common in women with CHD, and therefore this group of patients should be aware of the menstrual function and its abnormalities.

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Introduction: The cardiac manifestations of influenza A are broad, ranging from self-limited pericarditis to fatal cardiomyopathy. The 2009 H1N1 influenza A (H1N1) strain is a rare cause of pericarditis, and its role in developing a pericardial effusion leading to tamponade has infrequently been reported. Case Presentation: We describe a case of a young female with no prior cardiovascular history who presents with a pericardial effusion and shock secondary to cardiac tamponade from pericarditis due to H1N1 influenza A. Conclusions: This case highlights the potential severity of H1N1 infections and the utility of considering cardiac tamponade in patients presenting with influenza symptoms and circulatory collapse.

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Background: Echocardiography is a key screening tool in the diagnostic algorithm of pulmonary hypertension (PH). In addition, tissue doppler imaging (TDI) is a promising method for the noninvasive estimation of pulmonary artery pressure (PAP). Objectives: The aim of this study was to validate the accuracy of measuring the time from the beginning of the right ventricular isovolumetric contraction time (RVIVRT) to the peak of the S wave in the TDI of the base of the RV free wall (time to peak or TTP), as an indicator for the non-invasive estimation of pulmonary hypertension. Patients and Methods: In this diagnostic test study, 60 consecutive patients referred for right heart catheterization (RHC) were enrolled. A pulse-wave TDI was performed before the cardiac catheterization, with a mean interval of 1 hour between the two mea- surements. The TDI variables, such as the RV IVRT, myocardial performance index (MPI), and the new “time to peak” parameter, were measured at the lateral basal RV free wall. The patients were divided into two sub-groups according to the RHC findings: no- PH (mean PAP < 25 mmHg) and PH (mean PAP > 25 mmHg) groups. Then, we calculated the specificity and sensitivity of the TDI parameters (including the TTP) for the diagnosis of PH. Results: In our study, the TTP showed a significant inverse relationship with the PAP. Based on our results, a TTP of less than 127 ms could be used to predict PH, with a sensitivity and specificity of about 70% (AUC = 0.746 ± 0.064). Conclusions: Based on the results of this study, we suggest the use of a novel “time from the beginning of isovolumetric contrac- tion to the peak of the S wave” (TTP) parameter in the TDI of the base of the RV free wall to predict PH with acceptable accuracy in comparison with RHC.

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Introduction: Giant coronary artery aneurysm is an extremely rare form of coronary artery disease. The most common cause of coronary artery aneurysms is atherosclerosis. Although it is usually asymptomatic, it may have various clinical presentations, in- cluding angina, myocardial infarction or sudden death. Case Presentation: A 32-year-old woman presented with edema of the upper and lower limbs, palpitation, and chest pain, and was diagnosed with a giant right coronary artery aneurysm that had initially mimicked a mediastinal cyst. Although computed tomography (CT) suggested a mediastinal cyst, trans-thoracic echocardiography revealed an extra pericardial cyst. The definitive diagnosis of right coronary artery aneurysm was made based on CT angiography and coronary angiography findings. As treatment, aneurysmectomy was performed, and she was discharged on the sixth postoperative day with good general health condition. Conclusions: Coronary artery aneurysm should be a differential diagnosis in cases of mediastinal cyst and mass lesion.

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Introduction: Cardiac inflammatory myofibroblasts tumor is a rare tumor that can cause potentially fatal outcomes. Case Presentation: We describe a case where the tumor originated on the mitral valve and extended through the left ventricular outflow tract and aortic valve and into the left main artery obstructing it. Due to the hemodynamic instability of the patient, we proceeded for cardiac catheterization with the intention to aspirate the mass but were forced to do percutaneous intervention to stabilize the patient and bridge him to surgery. Conclusions: The patient underwent surgery several days later with complete resection of the tumor and coronary stent retrieval but his left ventricular ejection fraction remained poor after several months and he was referred for cardiac transplantation.

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Background: The right ventricular outflow tract (RVOT) palliation has been shown to be a proper interventional procedure for lowering risk of mortality and improving clinical condition in cyanotic congenital heart disease (CHD) patients. Objectives: The present study aimed to assess the consequences of RVOT palliation in patients with TOF. Patients and Methods: This prospective case series was performed on 17 children who suffered from cyanotic CHD. The study end- points were assessed by pulse oximetry, echocardiography, and electrocardiography immediately and also 12 months after RVOT palliation procedure. Results: The mean age of patients was 24.76 (median 10 months). Comparing laboratory and respiratory parameters 12 months after RVOT palliation showed a significant increase in arterial oxygen saturation (from 69.34 ± 13.07 to 86.29 ± 6.64, P= 0.001), RPA index of right pulmonary artery (from 5.49 ± 1.67 mm to 7.59 ± 1.79 mm, P< 0.001), Z score of right pulmonary artery (from-1.56 ± 2.34 to 0.53 ± 2.55, P < 0.001), LPA of left pulmonary artery (from 5.64 ± 1.88 mm to 8.06 ± 2.72 mm, P < 0.001), and also in Z score of left pulmonary artery (from -1.56 ± 2.33 to 0.78 ± 2.15, P = 0.001). Also, a significant decrease in the spell rate (from 88.2% to 17.6%, P < 0.001), and in the rate of tricuspid regurgitation (from 23.5% to 11.8%, P= 0.023) was shown. Cardiac arrhythmia occurred in only one patient that was transient. Stent fracture was found in none of the patients. Stent stenosis was also found in one patient. One-year death occurred only in one child. Conclusions: The right ventricular outflow tract palliation in children with cyanotic CHD leads to long-term favorable outcome regarding improvement in oxygen saturation, increase in Z score of both left and right pulmonary arteries and also considerable decrease in spell. Also, death and complications are rare following RVOT palliation.

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Introduction: The use of extracorporeal membrane oxygenation (ECMO) is considered a risk factor for, or even a potential con- traindication to, lung transplantation. However, only a few pediatric cases have been described thus far. Case Presentation: A 9-year-old boy with idiopathic pulmonary arterial hypertension developed cardiac arrest after the insertion of a central catheter. ECMO was used as a bridge to lung transplantation. However, after prolonged resuscitation, he developed medullary ischemia and medullary syndrome. After 6 weeks of ECMO and triple combination therapy for pulmonary hypertension, including continuous intravenous prostacyclin, he was weaned off support, and after 2 weeks, bilateral lung transplantation was performed. At 4 years post-transplant, he has minimal problems. The medullary syndrome has also alleviated. He is now back to school and can walk with aids. Conclusions: Increasing evidence supports the use of ECMO as a bridge to LT, reporting good outcomes. In the modern era of PAH therapy, it is feasible to use prolonged ECMO support as a bridge to lung transplant, with the aim of weaning off this support; however, its use requires more experience and knowledge of long-term outcomes.

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Background: Thrombolytic therapy in patients with sub-massive pulmonaryembolism (SMPTE) needs further assessment. Objectives: The current study aimed to assess a potential benefit of thrombolytic and non-thrombolytic therapy in patients with SMPTE. Patients and Methods: One hundred-nineteen patients were enrolled with SMPTE from 2006 to 2010 in the tertiary care center of Rajaie medical and research center. The patients who had pulmonary thromboemboli (PTE) and received thrombolytic plus heparin therapy and or non-thrombolytic (unfractionated heparin alone) were evaluated for hemodynamic changes (blood pressure, pulse rate, pulmonary artery systolic pressure, right ventricular failure and right ventricle enlargement), before and after 48 hours of treatment. The mortality rate was also assessed. Results: Forty-five percent of the patients with SMPTE received thrombolytic therapy (streptokinase) and 55% of SMPTE patients received non-thrombolytic therapy (unfractionated heparin). Pulse rate, pulmonary arterial pressure and tricuspid regurgitation gradient in patients receiving thrombolytic therapy reduced significantly (P = 0.001, P = 0.01 and P= 0.001, respectively). There was no significant difference before and after treatment regarding systolic blood pressure (P = 0.4), diastolic blood pressure (DBP) (P = 0.5), systolic arterial pressure (SPAP) (P= 0.1), Right ventricular (RV) function (P = 0.1) and RVsize (P = 0.1). In patients who received a non-thrombolytic therapy, there were no significant differences between the groups regarding SBP(P= 0.2), DBP( P= 0. 4) and PR (P=0.1), SPAP (P = 0.6), TRG (P = 0.4), RV function (P= 0.4) and RVsize (P = 0.2) before and after treatment. There were no significant differences between the groups according to mortality rate. Conclusions: Thrombolytic therapy lead to earlier relief of hemodynamic condition in comparison to non-thrombolytic therapy but no changes were observed in mortality rate.

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