Unusual Cardiac Associations with Tetralogy of Fallot��A Descriptive Study

We present a large single-center series (>2200 cases) operated for Tetralogy of Fallot (TOF). We analyzed the incidence of associated unusual and uncommonly described cardiac lesions and their diagnostic and therapeutic implications in TOF patients. This retrospective study was conducted by reviewing records of patients operated for TOF at a large tertiary care pediatric cardiac centre. From 2002 to 2008, a total of 2235 cases of TOF were evaluated with echocardiography, cardiac catheterization, and/or cardiac computed tomography followed by cardiac surgery. Known and well-described associations were excluded from the study. Unusual associations were tabulated. Several unusual associations having an incidence >0.1% were detected. These included subaortic membrane (1%), pulmonary venous abnormalities (0.5%), small left ventricle (0.5%), interrupted inferior vena cava (0.5%), mitral valve abnormalities (0.4%), hemitruncus (0.4%), tricuspid valve abnormalities (0.4%), biventricular dysfunction (0.3%), retroaortic innominate vein (0.3%), bicuspid aortic valve (0.2%), and pericardial effusion (0.2%). This series describes unusual, not previously routinely reported cardiac lesions associated with TOF that may affect management and should be sought on preoperative evaluation. We demonstrate that more unusual associations do exist in not infrequent numbers, i.e., a TET is not just a TET.     

Tetralogy of Fallot in a Patient with Killian–Pallister Syndrome

Killian–Pallister syndrome is a rare dysmorphic condition characterized by specific clinical manifestations and tetrasomy 12p. Although the association of this condition with congenital heart disease has been previously documented, no cases have been reported in association with Fallot's tetralogy. We report one such case.     

Fallot’s tetralogy with macrostoma,quadrilobular tragus and hypertrophy of the conjunctiva

Summary The association of ballot’s tetralogy with associated congenital anomalies: macrostoma, quadriloular tragus with pretragus depression and hypertrophy of the conjunctiva of the lower lid, are interesting features in this case. This association has not been reported before. It is interesting that all the four congenital defects are present on the left side. From the Department of Pediatrics, S. N. Medical College, Agra.     

Tetralogy of Fallot with Congenital Aortic Valvar Stenosis: The Tetralogy–Truncus Interrelationship

Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy–truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.     

Early Postoperative Results After Total Correction of Tetralogy of Fallot in Older Patients: Is Primary Repair Always Justified?

The objective of this study was to compare early postoperative results in primary vs 2-stage repair of tetralogy of Fallot in patients operated on after 5 years of age. Eighty-four patients with tetralogy of Fallot were studied, who were more than 5 years of age and who were repaired in one center by one surgeon. Hospital death and ratio of right-to-left ventricle pressure after correction were compared between the primary and the 2-stage groups as early outcome indices. Overall hospital mortality was 11.9% (10/88). There were 9 (18.4%) hospital deaths in the primary and 1 (2.9%) in the 2-stage group. Mortality was significantly higher in the primary group (p= 0.04 using the 2-tailed Fisher's exact test; relative risk = 6.43; 95% CI = 0.85–48.46). The mean ± standard deviation (SD) for RV/LV ratio was 0.51 ± 0.13. It was 0.61 ± 0.11 in patients who died and 0.49 ± 0.12 in patients who lived (p= 0.01 using the 2-tailed t-test). RV/LV ratio was not significantly different between either group. In the 2-stage group, there was a small inverse correlation between the time interval and the RV/LV ratio (correlation coefficient =−0.235). The TAP group had a statistically significant lower time interval between shunt and total correction than the RVOT-P group (p= 0.02 using 2-tailed t-test). Older patients generally do better on 2-stage repair, because of their age. A long period of low pulmonary blood flow has induced unbalanced ventricles and exaggerated RVOTO for these patients because of muscle hypertrophy. In such patients, shunting will prepare the left ventricle for accepting the extra blood volume that will reach the left ventricle after total correction.     

Non-patch enlargement as a better therapeutic strategy for tetralogy of Fallot with imbalanced pulmonary artery branches北大核心CSCD

Objective: To apply different strategies of pulmonary artery (PA) plasty to seek an optimal treatment for Tetralogy of Fallot (TOF) with unbalanced PA branches. Methods: From January 2006 to December 2017, retrospective review was conducted for 18 consecutive surviving TOF infants with imbalanced PA branches. Regular follow-ups were performed. The relevant clinical data were processed with software SPSS 19.0. Results: Among them, PA plasty was primary (n=14) and secondary (n=4). After an initial PA plasty, ratio of balanced PA branches was much higher in NATIVE group (100%) than that in PATCH & EXTENSION group (100% vs 0, P<0.05); After secondary PA plasty, only 25% of them attained a decent development and balanced PA braches, 28% with patch enlargement had PA branch atresia. Conclusions: For TOF with imbalanced PA branches, leaving HPA in a native state without patch enlargement may be a better strategy. © 2023 Authors. All rights reserved.     

Tetralogy of Fallot with Pulmonic Atresia with Cyclic Occlusion of an Associated Aortic Sinus of Valsalva–Pulmonary Artery Window

A first case of an unusual aortopulmonary window with tetralogy of Fallot associated with pulmonary atresia is presented. The aortopulmonary window was at the aortic sinus of Valsalva. The left aortic leaflet prevented pulmonary hypertension by occluding the window in systole.     

Branch Pulmonary Artery Growth After Blalock–Taussig Shunts in Tetralogy of Fallot and Pulmonary Atresia with Ventricular Septal Defect: A Retrospective, Echocardiographic Study

We performed a retrospective echocardiographic study in tetralogy of Fallot (TOF) or pulmonay atresia with ventricular septal defect (PA&VSD) to evaluate the effects of Blalock–Taussig shunt on branch pulmonary artery growth. There were 35 patients with TOF and 11 with PA&VSD. We measured the right and left pulmonary artery area index and also the combined pulmonary artery area index, both before and after shunt operation. The mean ± SD of these three variables before the shunt operation in the TOF group were 63.5 ± 22.5, 57.8 ± 24.9, and 121.4 ± 42.8 mm²/m²; after shunt operation they were 98.5 ± 33.6, 85.9 ± 31.9, and 184.0 ± 59.8 mm²/m², respectively (p values <0.0001, <0.0002, and <0.0001, respectively). In the PA&VSD group the comparable values before shunt operation were 66.5 ± 16.0, 55.4 ± 10.6, and 120.9 ± 26.9 mm²/m² and after shunt operation were 90.5 ± 22.9, 77.2 ± 24.1, and 166.6 ± 44.4 mm²/m², respectively (p values <0.0006, <0.014, and <0.002, respectively). We also examined the effect of distensibility of pulmonary arteries by comparing the percentage change in size of the combined pulmonary artery area index in the first 4 months after shunt with those after this time (p < 0.023). There were no significant differences between left- and right-sided shunts, origin and distal pulmonary artery growth, and the TOF and PA&VSD groups.