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OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

Background Repair of the absence of the whole or major parts of pulmonary arteries is a challenge, and the choice of conduit material to reconstruct the pulmonary arteries is under dispute. We used the autologous innominate vein to construct pulmonary arteries. Case presentation l We present a novel technique using the autologous innominate vein as a free graft in a 6-month-old infant with pulmonary atresia and absence of central pulmonary arteries. Double ductus arteriosus were the only source of perfusion of the lungs. The innominate vein was substituted for the central pulmonary artery between the two lung hila. Total repair by using Contegra graft was performed 9 months later. The patient has been followed for 5 years. Conclusions The autologous innominate vein could be used as inter-hilar pulmonary arteries with no calcification and fibrosis in 5-year follow-up.

Introduction The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Material and methods This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery. Results The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients. Conclusion The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs on its own or with some congenital cardiac disorders, particularly tetralogy of Fallot (TOF), while the hidden pulmonary artery might originate from a closed ductus arteriosus (DA) that can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of our nine TOF patients who had the absence of the left pulmonary artery before the operation. The patients were stratified in three groups: group one, whose closed DA were found and stented successfully to the hidden pulmonary artery; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of the other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgically correction, while the pulmonary vein angiography of the group two patients showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of the group three patients, aged 12, 38, 60, and 63 months. Moreover, evaluation of the other six previously corrected patients who were operated with a right pulmonary artery revealed pulmonary artery hypertension of the entire patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

Objectives: TOF is the most common cyanotic CHD. We investigated left ventricular (LV) function after surgical pulmonary valve replacement (sPVR) in patients with repaired Tetralogy of Fallot (rTOF) by Speckle Tracking Echocardiography (STE).Methods: 58 volunteers participated in this study who divided into 3 groups including 22 PVR patients (mean age 18.96±7 year), 16 repaired Tetralogy of Fallot and 20 healthy age match control. For all patients, we performed 2D echocardiography and STE.Results: 2D echocardiography in all groups showed normal LV ejection fraction without a significant statistical difference (64% sPVR, 60% in repaired Tetralogy of Fallot and 62.5% in the control group). However, the mean global longitudinal strains (GLS) of LV were significantly reduced in both sPVR (-17.5±2.5%) and repaired Tetralogy of Fallot (-17.1±4.7%) patients rather than control group (-20.2±0.7%) (P = 0.003). But GLS had no statistically significant difference between repaired Tetralogy of Fallot and sPVR patients (P=0.9). Segmental analysis of longitudinal strain (LS) showed a significant decrease in sPVR patients and repaired Tetralogy of Fallot group in basal anterior, basal septal, basal anterolateral segments, mid-anterior and anterolateral segments. Except for lower LS in the apical-anteroseptal segment, this level was mostly spared in both sPVR and repaired Tetralogy of Fallot patients.Conclusion: LVEF was within normal range after sPVR patients, but the pattern of impaired segmental LS and GLS did not change as compared with rTOF. Surgical PVR in patients with repaired TOF may not have a significant effect on the improvement of LV function assessed by STE. LV damage which happens during surgical correction of TOF may have a permanent deteriorating effect on LV function.

There are limited studies that have focused on the causes for hospitalization as an indicator of morbidity in patients with β-thalassemia major (BTM). A cross-sectional study was conducted to determine the main causes for hospitalization and death in hospitalized BTM patients in a referral hospital in Shiraz, southern Iran. During a 5-year period, 555 BTM patients were admitted to the hospital, of which 390 (67.7%) were 10 to 20 years of age. The most frequent causes for hospitalization were splenectomy (23%), heart failure (22.6%), liver biopsy (22.2%), uncontrolled diabetes (10.9%), arrhythmia (7.2%), cholecystectomy (3.8%), hypoparathyroidism (2.1%), and sepsis (2%). Of the hospitalized patients, 65 (11%), with a mean age of 16.1 ± 4.2 years, died. The most common causes of death were cardiomyopathy (72.3%), infections (17%), malignancies (3.1%), and cerebrovascular accidents (3.1%). Survival of our patients was less than in developed countries and cardiac complications were the most common cause of mortality and morbidity in these patients. Regarding the key role of iron chelation in prevention of different complications in BTM, correction of iron chelation regimen should be well considered.

Absent pulmonary valve (APV) is a relatively rare congenital heart disease, and is mostly associated with tetralogy of Fallot phenotype or ventricular septal defect. APV with intact interventricular septum (IVS) is even less common with case reports or very small series in the literature. Congenital aortic regurgitation with a rudimentary non-coronary cusp is also by itself a rare congenital anomaly and to our knowledge this is the first report of the combination of APV, intact IVS, abnormal aortic valve and ascending aortic aneurysm. The clinical course, possible etiologies and management are discussed.

The patient is a 17-year-old boy who was referred to our center with complaints of easy fatigability and exertional dyspnea. In echocardiography, the left ventricle was identified to be composed of a small functional cavity, and 2 sequestrated apical cavities that communicated with the functional cavity only through few stenotic orifices. The mitral valve apparatus was also abnormal: there was no definite papillary muscle, and chordae tendineae arose from the aberrant fibromuscular ridge that partitioned the left ventricular cavity. Imaging findings and management of this patient with a triple-cavity left ventricle are discussed.

Background: Apolipoprotein E (apoE) with three major alleles E2, E3 and E4 is one of the critical genes in lipid metabolism. Common apoE alleles are in association with an increase in risk for central nervous and cardiovascular diseases such as Alzheimer’s disease, dementia, multiple sclerosis, atherosclerosis, coronary heart disease, hyperlipoproteinemia and stroke. ApoE3 is known as the most frequent allele in all populations, while association of apoE gene polymorphism with reported diseases have mostly been related to other two major alleles especially apoE4. Objective: To determine of apoE alleles frequencies in Southern Iran and comparison of those frequencies with other populations. Methods: DNA was extracted from the whole blood of 198 healthy unrelated candidates from population of Fars Province, Southern Iran, for apoE genotyping who were checked up by a physician. The frequencies of apoE alleles were compared with other populations by χ2 test. Results: The frequencies of E2, E3 and E4 were 0.063, 0.886 and 0.051 respectively. These values were similar to those reported from populations of Kuwait, Oman, Lebanon, India, Turkey, Greece, Spain, Sardinia Islands of Italy and two Iranian populations but were different from South of Italy and Caucasians in other Europe regions, American, American-Indian, African, East Asian and Saudi populations (P

Left ventricular (LV) failure is the main cause of death in thalassemia. Iron overload in patients with thalassemia leads to the formation of oxygen free radicals. Of the various apolipoprotein E (apoE) alleles, apoE4 is the least efficient in conditions of oxidative stress in comparison with apoE2 and apoE3. Our results showed that apoE4 is a genetic risk factor for LV dysfunction in thalassemia.