Background: The late postoperative course for children with transposition of the great arteries (TGA) with an intact ventricular septum (IVS) is very important because the coronary arteries may be at risk of damage during arterial switch operation (ASO). We sought to investigate left ventricular function in patients with TGA/IVS by echocardiography.
Methods: From March 2011 to December 2012, totally 20 infants (12 males and 8 females) with TGA/IVS were evaluated via 2-dimensional speckle-tracking echocardiography (2D STE) more than 6 months after they underwent ASO. A control group of age-matched infants and children was also studied. Left ventricular longitudinal strain (S), strain rate (SR), time to peak systolic longitudinal strain (TPS), and time to peak systolic longitudinal strain rate (TPSR) were measured and compared between the 2 groups.
Results: Mean ± SD of age at the time of study in the patients with TGA/IVS was 15 ± 5 months, and also age at the time of ASO was 12 ± 3 days. Weight was 3.13 ± 0.07 kg at birth and 8.83 ± 1.57 kg at the time of ASO. Global strain (S), Time to peak strain rate (TPSR), and Time to peak strain (TPS) were not significantly different between the 2 groups, whereas global strain rate (SR) was significantly different (p value < 0.001).  In the 3-chamber view, the values of S in the lateral, septal, inferior, and anteroseptal walls were significantly different between the 2 groups (p value < 0.001), and SR in the posterior wall was significantly different between the 2 groups (p value < 0.001). There were no positive correlations between S and SR in terms of the variables of heart rate, total cardiopulmonary bypass time, and aortic cross-clamp time. There were no statistically significant differences between the 2 groups regarding S, SR, TPS, and TPSR in the anteroseptal and posterior walls in the 3-chamber view and in the lateral and septal walls in the 4-chamber view.
Conclusion: We showed that between 6 and 18 months after a successful ASO, the parameters of S, SR, and global TPS were normal in our patients with TGA/IVS. However, LV myocardial TPSR did not normalize in this time period.

Background: Hypertrophic cardiomyopathy is a genetic disorder with a prevalence rate of 0.2% in the general population. It comes from mutations in sarcomeric proteins. Cardiac myosin-binding protein C3 is one of the critical genes in hypertrophic cardiomyopathy (HCM) and sudden cardiac death, accounting for about 20% of HCM-causing mutations. Genetic testing is recommended in patients with HCM. The aim of the current study was to find possible disease-causing mutations in 3 exons of the gene myosin-binding protein C (MYBPC3) in patients with HCM.
Methods: Fifty subjects with documented known HCM were enrolled in the study. The patients were referred to the hospitals affiliated to Tehran University of Medical Sciences between 2008 and 2011. Peripheral blood samples were collected, as well as clinical and demographic data. The nucleotide sequences of the exons number 7, 16, and 18 of MYBPC3, whose relevance to the disease was previously reported, were amplified by polymerase chain reaction. Direct DNA sequencing was applied, and the Chromas software was used to analyze the sequences to find possible disease-causing mutations.
Results: The study population comprised 73% male and 27% female patients. The mean age of the patients was 33.9 ± 20.08 years. Family history of sudden cardiac death was reported in 48.2% of the patients. About 79% of the studied subjects had a history of at least 1 other affected relative in their families. Laboratory findings did not show mutations or any nucleotide changes in the sequences of the 3 target exons in the genomic DNA of the studied patients with HCM.
Conclusion: The nucleotide sequences of the exons number 7, 16, and 18 of MYBPC3 were not mutated in the 50 studied subjects with HCM.   

Background: It is not clear whether the latest activation sites in the left ventricle (LV) are matched with infracted regions in patients with ischemic cardiomyopathy (ICM). We aimed to investigate whether the latest activation sites in the LV are in agreement with the region of akinesia in patients with ICM.
Methods: Data were analyzed in 106 patients (age = 60.5 ± 12.1 y, male = 88.7%) with ICM (ejection fraction ≤ 35%) who were refractory to pharmacological therapy and were referred to the echocardiography department for an evaluation of the feasibility of cardiac resynchronization therapy. Wall motion abnormalities, time to peak systolic myocardial velocity (Ts) of 6 basal and 6 mid-portion segments of the LV, and 4 frequently used dyssynchrony indices were measured using 2-dimensional echocardiography and tissue Doppler imaging (TDI). To evaluate the influence of the electrocardiographic pattern, we categorized the patients into 2 groups: patients with QRS ≤ 120 ms and those with QRS >120 ms.
Results: A total of 1 272 segments were studied. The latest activation sites (with longest Ts) were most frequently located in the mid-anterior (n = 32, 30.2%) and basal-anterior segments (n = 29, 27.4%), while the most common sites of akinesia were the mid-anteroseptal (n = 65, 61.3%) and mid-septal (n = 51, 48.1%) segments. Generally, no significant concordance was found between the latest activated segments and akinesia either in all the patients or in the QRS groups. Detailed analysis within the segments indicated a good agreement between akinesia and delayed activation in the basal-lateral segment solely in the patients with QRS duration ≤ 120 ms (Φ = 0.707; p value ≤ 0.001).
Conclusion: The akinetic segment on 2-dimensional echocardiogram was not matched with the latest activation sites in the LV determined by TDI in patients with ICM. 

Background: Recently, stem cells have been considered for the treatment of heart diseases, but no marked improvement has been recorded. This is the first study to examine the functional and histological effects of the transplantation of human amniotic mesenchymal stromal cells (hAMSCs) in rats with heart failure (HF).
Methods: This study was conducted in the years 2014 and 2015. 35 male Wistar rats were randomly assigned into 5 equal experimental groups (7 rats each) as 1- Control 2- Heart Failure (HF) 3- Sham 4- Culture media 5- Stem Cell Transplantation (SCT). Heart failure was induced using 170 mg/kg/d of isoproterenol subcutaneously injection in 4 consecutive days. The failure confirmed by the rat cardiac echocardiography on day 28. In SCT group, 3×10⁶ cells in 150 µl of culture media were transplanted to the myocardium. At the end, echocardiographic and hemodynamic parameters together with histological evaluation were done.
Results: Echocardiography results showed that cardiac ejection fraction in HF group increased from 58/73 ± 9% to 81/25 ± 6/05% in SCT group (p value < 0.001). Fraction shortening in HF group was increased from 27/53 ± 8/58% into 45/55 ± 6/91% in SCT group (p value < 0.001). Furthermore, hAMSCs therapy significantly improved mean diastolic blood pressure, mean arterial pressure, left ventricular systolic pressure, rate pressure product, and left ventricular end-diastolic pressure compared to those in the HF group, with the values reaching the normal levels in the control group. A marked reduction in fibrosis tissue was also found in the SCT group (p value < 0.001) compared with the animals in the HF group.
Conclusion: The transplantation of hAMSCs in rats with heart failure not only decreased the level of fibrosis but also conferred significant improvement in heart performance in terms of echocardiographic and hemodynamic parameters.

The Budd–Chiari syndrome (BCS) is a rare disorder caused by the obstruction of the hepatic veins or the inferior vena cava (IVC) at the suprahepatic level. This syndrome is developed by either hepatic vein thrombosis or mechanical venous obstruction and leads to centrilobular hepatic congestion with the subsequent development of fibrosis and cirrhosis. Intracardiac tumors have been rarely reported as a cause of the BCS. These tumors usually originate from the atrial septum. Very rarely, they arise either from the junction of the IVC and the right atrium or from the Eustachian valve. There are a few case reports in the literature where atrial tumors have caused the BCS. In these cases, the tumors were malignant, and the patients died shortly after being diagnosed.
We describe a 71-year-old female patient who presented with a 3-month history of abdominal pain and protrusion. On physical examination, blood pressure and pulse rate were normal. Jugular venous pressure was about 10 cm. Cardiac examination revealed a systolic murmur, grade IV/VI, in the left sternal border without radiation. Echocardiography showed a large mass (about 6×4 cm) in the right atrium with close contact to the origin of the IVC, obstructing it. Cardiac magnetic resonance imaging, with and without gadolinium, also confirmed the diagnosis. The patient underwent surgery, and the myxoma was removed. The tumor was a large solid mass, 5×4 cm in size, which originated immediately above the entrance of the IVC. The patient is in good condition 1 year afterward. We emphasize that atrial myxomas should be considered in the differential diagnosis of tumors that cause chronic BCS.

The acute coronary syndrome due to the left main coronary artery (LMCA) thrombosis is a clinically rare and catastrophic event.  We describe a young man (smoker, alcoholic, and drug abuser) with a history of recent surgery and typical chest pain who had non-occlusive LMCA thrombosis in coronary angiography. The thrombosis was successfully treated with two 180 µ/kg intracoronary boluses of eptifibatide, which was continued through an intravenous infusion at 2 µ/kg/min for 48 hours postprocedurally. Control angiography, performed 3 days later, revealed that the LMCA was free of thrombosis. The patient had no complaints, including chest pain, and remained completely asymptomatic during the next 30 days' follow-up.

The anomalous origin of the right coronary artery (RCA) as a branch from the left anterior descending artery (LAD) is a very rare variation of the single coronary artery anomaly. The anomalous vessel arises from the proximal or midportion of the LAD and courses anterior to the pulmonary artery trunk in most instances. In this case report, a 61-year-old woman is introduced who underwent coronary angiography following inferoposterior myocardial infarction, in which an anomalous RCA was seen originating from the midportion of the LAD. There was also a separate small artery originating from the right coronary sinus, which was most probably a right atrial branch.

Respiratory failure is a serious complication of H1N1 influenza that, if not properly managed, can cause death. When mechanical ventilation is not effective, the only way to save the patient’s life is extracorporeal membrane oxygenation (ECMO). A prolonged type of cardiopulmonary bypass, ECMO is a high-cost management modality compared to other conventional types and its maintenance requires skilled personnel. Such staff usually comprises the members of open-heart surgical teams.  Herein, we describe a patient with H1N1 influenza and severe respiratory failure not improved by mechanical ventilation who was admitted to Masih Daneshvari Medical Center in March 2015. She was placed on ECMO, from which she was successfully weaned 9 days later. The patient was discharged from the hospital after 52 days. Follow-up till 11 months after discharge revealed completely active life with no problem. There should be a close collaboration among infectious disease specialists, cardiac anesthetists, cardiac surgeons, and intensivists for the correct timing of ECMO placement, subsequent weaning, and care of the patient. This team work was the key to our success story. This is the first patient to survive H1N1 with the use of ECMO in Iran.