Background- Anomalous coronary artery disease is more common among patients with Tetralogy of Fallot (ToF).Methods- In order to determirie the coronary anomalies in patients with ToF, 135 patients (80 males) with ToF, 9 months to 40 years of age vere studied over 7 years (1995-2002) at Modarres Hospitalin Tehran, Iran.Results-Eight out of the 135 patients (4 males) with Tof were associated with a surgically- relevant coronary artery anomaly (5.9%). The anomalous coronary arteries consisted of single ostium of the coronary artery (n =5), the left anterior descending coronary artery originating from the right coronary artery (n= 2) or single righf coronary artery (n = 2) and the right coronary artery arising from the left coronary artery (n = 1). Surgical technique employed in three ToF patients was transverse incision on the right ventricle, and commissurotomy via the pulmonary artery was the technique used for three other patients. In another patient, a composite graft between the right ventricle outflow tract and main pulmonary artery was inserted. In the remaining four patients with single ostium coronaries, routine repairs were done.Conclusions-Anomalous coronary artery disease is more common among patients with ToF. Bearing these anomalies in mind during primary repair could decrease the risk of surgery in patients with ToF

We report two cases of Tetralogy of Fallot with pulmonary valve bacterial endocarditis where one extended to the branch of pulmonary artery (PA). This is a rare occurrence. Aggressive supportive care plus early and radical surgery can be life saving.

Background- Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease (CHD). The aim of this study is determination of demography, associated anomalies, peripheral pulmonary stenosis (PPS), blood group, Rh type, operation results and complications in TOF.Methods- The records of 270 patients were reviewed. These patients had been admitted during 10 years (from 1993 to 2003).Results- 60.37% of patients were male and 39.63% were female. Incidence of patent foramen ovale (PFO), right aortic arch (RAA), coronary artery (CA) anomalies and other anomalies were 44.81%, 21.11%, 9.25% and 36.30%, respectively. Single ostium coronary artery (SOCA) was the most CA anomaly. The most common PPS was bifurcation stenosis. TOF was more common in blood group O patients. The mean age at the first palliative operation was 5.21 years and for total correction (TC), it was 7.19 years. Postsurgical mortality rate was about 3% and morbidity rate was 12.18% (excluding right bundle branch block, RBBB).Conclusion- The trend toward earlier total correction and single stage early total correction of TOF should be recommended as the preferred management strategy.

Background- Pulmonary regurgitation (PR) is the most important residual lesion remaining after the repair of Tetralogy of Fallot (TOF). Through a thorough review of the data, statistics of patients undergoing pulmonary valve replacement following total correction for TOF and analyzing these data, the following study was performed and presented below.Methods- Database search for medical records of patients undergoing pulmonary valve replacement following total correction for TOF was performed and the data gathered, analyzed, and presented.Results- The age of the patients (22.21±6.98 years old), time elapsed between the two operations, right ventricular ejection fraction ( mildly decreased, 18.6%; moderately decreased, 67.9%; and severely decreased, 12.2% of cases), aneurysm in the outflow tract of the right ventricle (20.8%), tricuspid regurgitation (56.6%), tricuspid steno sis (1 case), valve type used for pulmonary valve replacement (biologic, 86.6%; metallic, 11.2%; and homograft, 1.9%), pulmonary artery pressure [<25mmHg, 34 cases (64.2%); 25mmHg - 50mmHg, 7 cases (13.2%); 50mmHg-75mmHg, 1 case (1.9%), and > 75mmHg, 1 case] were evaluated.Conclusions- Although right ventricular volume overload due to severe pulmonary regurgitation after repair of TOF can be tolerated for years, there is now evidence that the compensatory mechanisms of the right ventricular myocardium ultimately fail and that if the volume overload is not eliminated or reduced, this dysfunction may be irreversible. In light of those data and with better understanding of risk factors for adverse outcomes late after TOF repair, many centers are now recommending early pulmonary valve replacement before symptoms of heart failure develop.

The purpose of this study was to evaluate the early and late outcome after total correction of tetralogy of fallot (TOF) in 101 consecutive patients with a mean age of 8.23 ± 4.90 years underwent repair of surgery at one institution between 1995 and 2006. Forty two patients had initial palliative operations. A transannular patch was inserted in 60 (58.5%) patients. Risk factors for operative mortality were analyzed. Followup was obtained from clinical appointments and telephone questionnaires. The operative mortality was 6.9%. Aortic cross-clamp time more than 90 minutes (P<0.01) and cardiopulmonary bypass time more than 120 minute (P<0.01), affected operative mortality, whereas previous palliative procedure, hematocrit level, and use of transannular patch did not. Mean follow-up is 34.08 ± 31.09 months (range, 1 month to 120 months). Actuarial survival is 91% alive 10 years after total correction. On Postoperative echocardiography, 22 patients had mild pulmonary regurgitation, 19 had a right ventricular outflow tract gradient more than 50 mmHg, and 10 had a small residual ventricular septal defect. There were two late deaths. Late sudden death from cardiac causes occurred in one patients. Total correction of TOF can have low operative mortality and provide excellent long-term survival. This experience suggests that the key factor in the total correction of TOF is to correct the pathology completely, to protect the myocardium, and to manage the complication properly.

Background & aim: Fallot tetralogy (TOF) is one of the most common diseases among congenital heart cyanotic abnormalities. 25% of these patients have a right aortic arch. On the other hand, the right aortic arch is often associated with syndromes, heterotaxia, and misplaced origins of the aortic branches. Knowing this is necessary for the surgeon before corrective surgery and for drawing a roadmap for less complicated surgery is very helpful and necessary. Therefore, the aim of present study was to determine and evaluate aortic arch disorders in patients with tetralogy of Fallot. Methods: In the present retrospective study, 332 cases of TOF patients were referred by the pediatric cardiology department was reviewed for preoperative angiography during surgery from 2006 to 2016 to the angiography wards of Namazi and Shahid Faghieh hospitals. Had been reviewed. All patients underwent diagnostic angiography before surgery and their angiographic film was reported and recorded. In this study, all existing preoperative angiography records were carefully reviewed and all available information and how to orient the aortic arch and its abnormalities, if any, were recorded. The collected data were analyzed using. chi-squared. . . test. Results: Out of 332 people included in the study, 312 were children and 20 were adults. The age distribution of patients was determined from one month to 36 years. A total of 77 patients (23. 2%) had right aortic arch among 208 males and 124 females. In one of these patients, an interupted aortic arch was reported. In terms of sex distribution, right aortic arch was observed in 22. 1% of male patients and 24. 9% of female patients, which did not show a clear statistical difference (p=0. 5). No abnormalities of the aortic branches were seen in this study. Conclusion: A significant percentage of patients with TOF have right aortic arch (22%) and aortic arch disorders such as a ruptured aortic arch may be seen with it. Knowing the position of the aorta, arch abnormalities, and abnormal branches of the aorta before surgery will be a great help for the surgeon to have a roadmap to reduce intraoperative and postoperative complications and speed the patient's recovery.

Background:After tetralogy of Fallot (TOF) repair, severe pulmonary insufficiency is known to impair biventricular function. Pulmonary valve replacement (PVR) alleviates symptoms, normalizes right ventricular volumes, and improves ventricular function. Most studies addressing the role of PVR have examined older adolescents or adults. Less is known about the potential benefits of PVR in preadolescents with TOF and significant right ventricular dilatation. Methods: We reviewed the clinical data for all preadolescents (13 years) with TOF who underwent cardiac magnetic resonance imaging (cMRI) or PVR, or both. Serial cMRI data were analyzed to determine the change in indexed right ventricular end-diastolic volume (RVEDV) and biventricular ventricular ejection fractions. Available cMRI data after PVR were compared with data before PVR. Results: During the study period, 101 preadolescents with TOF had cMRI. The median age of complete repair was 6 months (range, 6 days to 3.4 years). The mean RVEDV at the first study was 135 ± 39 mL/m2. For 32 with serial cMRI studies, the RVEDV increased at a mean yearly rate of 9 mL/m2. Ventricular systolic function was impaired in 46 (46%). Forty-two patients underwent PVR at a mean age of 8 ± 3 years. No hospital deaths occurred, and no pulmonary valve reinterventions have been required.Conclusions: Significant right ventricular dilatation is common in preadolescents after transannular patch repair of TOF. Routine follow-up of this population should incorporate cMRI. Further studies will be needed to determine whether a strategy of early PVR might improve intermediate-term outcome.

Background: Tetralogy of Fallot (TOF) refers to a condition in which left ventricular volume is normal or slightly less than normal. Given the differences observed in some Asian patients with TOF، the present study was conducted to investigate left heart by determining Z-scores for the mitral valve in Iranian patients with TOF. Methods: Eligible subjects in this prospective descriptive study comprised all patients with TOF presenting to Shahid Modarres Hospital in Tehran from March 2012 to March 2015 and diagnosed as the candidates for surgery. After determining the need for surgery and the therapeutic method required، the mitral valve size and Z-scores were calculated. We analyzed sex، age، body surface area (BSA)، mitral size (in 2-chamber and 4-chamber view)، mitral Z-score، other cardiac anomalies، number of surgery and previous surgery. Results: Of a total of 80 patients included in the study over 3 years، 29 (36. 3%) were male and 51 (63. 8%) were female. The mean age of the patients was 7. 15± 3. 37 years and their mitral size was found to be 10-27 mm (16. 2± 5. 99 mm) using echocardiography. Z-scores of the mitral valve were also obtained as-3. 09± 2. 11، ranging between-7. 1 SD and +1. 3 SD. 58 (72. 5%) patients have only TOF and 22 (27. 5%) with other cardiac anomalies. 45 patients were for first time underwent surgery and 22 patients for second time، 12 patients for three time and 1 for forth. Of a total of 35 patients had previous surgery، the most common were shunt 15 (42. 9%) and then tetralogy of Fallot total correction (TFTC) in 12 (34. 3%). Of a total of 80 patients، 59 (73. 8%) underwent TFTC، 17 (21. 3%) under pulmonary valve replacement and 4 (5%) shunt. Conclusion: Z-scores of the mitral valve were found to be significantly below the normal value، (i. e. 0± 2 SD)، in the study patients، suggesting the risk of hypoplastic left heart syndrome in Iranian patients with TOF، nevertheless، the type of TOF examined in these patients might have been different from those observed in other races and regions.