Abstract Townes–Brocks syndrome (TBS) is a rare characterized by triad of anal, ear, and thumb anomalies. Further malformations/anomalies include congenital heart diseases, foot malformations, sensorineural and/or conductive hearing impairment, genitourinary anomalies eye nervous system. Definitive diagnosis for TBS confirmed molecular analysis mutations in the SALL1 gene. Only one known case w...

c t a A 74-year-old man, with a history of heavy smoking, presented with unstable angina (Figure 1). Dobutamine stress echocardiography showed ischemia in the right coronary artery territory. Coronary angiography revealed no obstructive epicardial disease in the left dominant circulation (Figure 2A); the right coronary artery (RCA) was a small caliber and tortuous vessel, with only minor athero...

BACKGROUND Patients after repair of tetralogy of Fallot (ToF) frequently have right ventricular (RV) dysfunction and prolonged QRS duration (QRSd) and thus could be candidates for cardiac resynchronization therapy. We aimed to assess the relationship between QRSd and the timing of RV wall motion, including the RV outflow tract (RVOT), in these patients. METHODS AND RESULTS Sixty-seven repaire...

Dr. J. A. DAVIS: The patient (Case No. 298928, P.M. No. 11116) we are about to discuss was a male infant born on 30 December 1964. He was the child of the third pregnancy of a 25-year-old woman with a proved Fallot's tetralogy. The first pregnancy had ended in an abortion at 3 months; the second yielded a live infant who weighed only 1,420 g. at 37 weeks' gestation and died at the age of 30 hou...

OBJECTIVE To clarify the problems in angiographic diagnosis of major coronary arteries crossing the right ventricular outflow tract. DESIGN A retrospective study with clinicomorphological correlations to ascertain any aberrant coronary arteries and variations in distribution of the normal right coronary arterial branches. SETTING Tertiary referral centre. SUBJECTS 36 necropsy specimens to...

Introduction A coronary artery anomaly (CAA) is a congenital defect of one or more coronary arteries of the heart in terms of origin, number, course and termination of the artery. The exact mechanism of CAA development is not elucidated yet. CAA is associated with some other congenital heart diseases like tetralogy of Fallot, transposition of great arteries and pulmonary valve atresia. Most CAA...

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of Fallot can now expect to survive to adulthood. ...

INTRODUCTION Tetralogy of Fallot is one of the most common forms of cyanotic congenital heart disease and in the absence of surgical correction it has an elevated early mortality, with most patients dying in childhood.The authors reported this case because of the unusual course of an uncorrected tetralogy of Fallot. There are only a few reports of patients with an uncorrected tetralogy of Fallo...