Atrial Flutter in a Repaired Tetralogy of Fallot Patient With Unusual Venous Anatomy: Submitted by Reza Ashrafi, MBBS, BSc, MD, MRCP and A.G. Stuart, MBChB, PgCert (Genomics), MSc, FRCP, FRCPCH, FESC

History

A 51-year-old male underwent primary repair of tetralogy of Fallot (ToF) in 1974 at the age of 9 years. The repair included a Dacron patch to close the ventricular septal defect and a pericardial patch in the right ventricular outflow tract. He remained well throughout childhood and early adult life with regular, but infrequent, outpatient follow-up. By the age of 48 he had developed progressive right ventricular dilatation with severe and increasing pulmonary regurgitation on serial echocardiography. He had no exercise-related symptoms and exercise tolerance was good (VO2 max 33 mL/kg/min). Coronary angiography was normal. The right ventricle measured 5.7 cm at the base on echocardiography but there was reasonable long axis function (tricuspid annular plane systolic excursion 18 mm). There was good left ventricular function with no branch pulmonary artery stenosis or other cardiac pathology.

The 12 lead ECG prior to surgery showed sinus rhythm with a broad right bundle branch block pattern with a QRS width of 160 ms and QRS axis of −80 degrees. A preprocedural MRI showed normal biventricular function with severe pulmonary regurgitation and no late gadolinium enhancement. He underwent elective pulmonary valve replacement (26 mm perimount bioprosthesis; Edwards Lifesciences, Irvine, Calif, USA) and a ring annuloplasty was carried out on the tricuspid valve. He made an excellent postoperative recovery ( Fig. 3.1 ).

A year after his procedure he described reduced exercise tolerance with shortness of breath. An echocardiogram showed minimal pulmonary and tricuspid valve regurgitation and normal right ventricular pressures. Left ventricular function was good but his right ventricle had remained dilated with reduced function postoperatively.