Preoperative Diagnosis of Long QT Syndrome in an Infant With Tetralogy of Fallot

Long QT syndrome is a well-described entity in infants. Its presentation in the context of congenital heart disease is rare and is almost exclusively diagnosed postoperatively. For patients undergoing surgical intervention, preoperative knowledge of the diagnosis and appropriate perioperative management can be life-saving. We present the rare case of an infant with tetralogy of Fallot who was preoperatively diagnosed with long QT syndrome and discuss the implications of this diagnosis for his perioperative management.     

Post-hoc Diagnosis of Congenital Long QT Syndrome in Patients with Tetralogy of Fallot

This report describes two patients diagnosed with congenital long QT syndrome after surgical repair of tetralogy of Fallot. Despite the fact that both patients had preoperative electrocardiograms demonstrating QT prolongation, neither was diagnosed until long after their surgeries, when they or their relatives presented with symptoms of long QT syndrome. A brief discussion highlights the reasons why long QT syndrome may be overlooked in patients with structural heart defects and the clinical importance of identifying these patients preoperatively.     

Enterococcus avium Endocarditis in an Infant with Tetralogy of Fallot

We report a case of infective endocarditis secondary to Enterococcus avium in a 1-year-old infant with tetralogy of Fallot and a Blalock–Taussig shunt. To our knowledge, this is the first case of E. avium endocarditis to be reported.     

Tetralogy of Fallot in a father and son

A family is reported on, in which the tetralogy of Fallot is present in the father and the eldest son. In both the patients a total correction took place. The mother and the second son are healthy.     

Tricuspid Atresia and Tetralogy of Fallot in an Infant of a Diabetic Mother

An infant of a diabetic mother with a prenatal diagnosis of tetralogy of Fallot and tricuspid atresia is presented. To the best of the authors’ knowledge, this is the first report of such an association.     

Renal Dysfunction is Common Among Adults After Palliation for Previous Tetralogy of Fallot

Long-term survival after tetralogy of Fallot (TOF) repair is excellent. However, little is published regarding late noncardiac complications. This study aimed to determine the prevalence and risk factors for renal dysfunction among adults after TOF repair. For this study, 56 adult patients with complete repair of TOF were identified, and their charts were retrospectively reviewed. An estimated glomerular filtration rate (eGFR) for each patient was calculated using the Modification of Diet in Renal Disease formula (MDRD). Using each patient’s eGFR, he or she was classified into stages based on the National Kidney Foundation chronic kidney disease (CKD) staging. Clinical parameters were compared among patients with and those without renal dysfunction to identify risk factors for renal impairment. The median estimated eGFR rate for the cohort was 78 ml/min/1.73 m². Based on the National Kidney Foundation CKD staging system, 54 % of the patients had at least stage 2 chronic renal disease. The risk factors identified were hypertension (p < 0.01), type 2 diabetes mellitus (p < 0.05), longer follow-up evaluation (p < 0.005), older age at complete repair (p < 0.05), and use of daily diuretics (p < 0.05). After repair of TOF, renal dysfunction is common at late follow-up evaluation. The study findings show the importance of routine assessment of renal function and the need to limit or avoid future episodes of acute kidney injury in this at-risk population.     

Congestive Heart Failure with Tetralogy of Fallot Relieved by an Aortopulmonary Shunt

Congestive heart failure occurring in unoperated patients with otherwise uncomplicated tetralogy of Fallot (TF) is rare. We report the case of a boy who was first diagnosed as having TF when he presented at age 3 years with hypoxia and heart failure. Heart failure improved following an aortopulmonary shunt. We attributed his heart failure to cardiomyopathy associated with severe hypoxia. We believe that this report is the first one that attributes heart failure to hypoxia in an unoperated patient with tetralogy of Fallot.     

Parasitic Conjoined Twins with Omphalocele and Tetralogy of Fallot

Human parasitic twins are very rare. Here we report a unique case of a partial twin attached to the host in the midline at the forehead, chin, chest, and epigastrium. The parasite lacked thoracic organs and major neural tube derivatives. However, it had small peripheral nerves and ganglia within perirenal and pericolonic connective tissue. Also present were a well-developed small intestine, colon, and appendix with normal submucosal and myenteric plexuses. These findings may represent either the initial presence of a neural tube that later regressed or migration of autosite neural crest cells. The parasite had a mature, functioning kidney with its ureter opening to skin and complete absence of urinary bladder or genital organs. This raises questions about the embryological development of the ureteric bud, which is an outgrowth of the mesonephric duct. The host had tetralogy of Fallot and omphalocele containing the parasitic kidney and bowel. Parasitic twinning occurs at 3 weeks of gestation, tetralogy of Fallot at 3-7 weeks, and omphalocele at 6-10 weeks. A single noxa acting at 3 weeks could have caused sequential malformations that initially seem unrelated.     

Tetralogy of Fallot with infective endocarditis: an echocardiographic explanation of misleading clinical signs

Echocardiography has a known key role in the diagnosis of infective endocarditis, the diagnosis of complications, follow-up evaluation after therapy, and prognostic assessment Habib (Eur J Echocardiogr 11:202–219, 3). This report describes a boy with tetralogy of Fallot who presented with infective endocarditis and large vegetation occluding the ventricular septal defect, thus resulting in a hemodynamically restrictive ventriculoseptal defect with misleading clinical signs. This case illustrates the role of echocardiography in both explaining clinical signs and providing hemodynamic data.     

Stenting Right Ventricular Outflow in an Infant With Tetralogy of Fallot and Well-Developed Pulmonary Arteries

A right ventricular outflow tract stent was implanted in a 1-month-old (3.5 kg) baby boy with tetralogy of Fallot and worsening cyanosis to relieve infundibular and pulmonary valve stenosis in the setting of well-developed pulmonary artery branches. This management allowed a symmetric and optimal growth of the pulmonary artery branches, with right and left pulmonary arteries measuring 7-mm (z-score, +1.3 SD) and 7.3-mm (z-score, +1.7 SD), respectively, 3 months after stent implantation. No signs of pulmonary overcirculation developed, and complete surgical repair was performed at the age of 6 months.