Journal of the College of Physicians and Surgeons Pakistan 2009, Vol. 19 (4): 211-214 211 INTRODUCTION Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease; with an incidence of approximately 0.5/1000 live births making 5-7% of congenital heart lesions. 1-3 TOF is a congenital cardiac anomaly characterised by a large ventricular septal defect, muscular obstrucion within the right ventricular outflow tract, rightward deviation of the aorta with biventricular connection of the valvar leaflets so that its orifice overrides the ventricular septum, and the right ventricular hypertrophy. 4 In the current era, TOF is almost universally amenable to surgical repair with good long-term outcome. This, however, requires a thorough pre-operative anatomic description of central and branch pulmonary arteries and associated defects, like additional muscular VSD, ductus arteriosus, Major Aortopulmonary Collateral Arteries (MAPCA) for better surgical planning and a better outcome. 5,6 Echo- cardiography with Doppler interrogation gives an accurate diagnosis of intracardiac anatomy of these patients. Cine-angiography, however, compliments the echocardiographic study as it allows more accurate evaluation of pulmonary vasculature, coronary arteries and additional ventricular septal defects. 7,8 This invasive study is regarded as necessary to obtain an exact description of cardiac lesions and pulmonary artery variations prior to deciding on surgical management strategies in patients with TOF. There is a limited data on these variations and associations in the local population where patients usually present late and more severe forms have natural attrition. This study was designed to determine various anatomic variations in pulmonary vasculature and other associated cardiac defects in patients with TOF. METHODOLOGY This cross-sectional descriptive study was conducted at the Children’s Hospital and the Institute of Child Health, Lahore, from April 2006 to October 2007. All patients with echocardiographic diagnosis of TOF, undergoing cardiac catheterization during the study period, were selected for evaluation. Patients who had undergone a shunt procedure or had Complete Atrioventricular Septal Defect (CAVSD) with TOF were excluded. Approval of ABSTRACT Objective: To determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot (TOF). Study Design: Cross-sectional, descriptive study. Place and Duration of Study: The Children’s Hospital and the Institute of Child Health, Lahore, from April 2006 to October 2007. Methodology: All patients with TOF, who underwent cardiac catheterization during this period, were included. Standard cine-angiograms were recorded and pulmonary artery sizing was done using z-scoring. Results: A total of 216 patients with TOF were catheterized. Pulmonary Artery (PA) abnormalities were present in 84 (38.9%) patients. The commonest abnormality was isolated Left Pulmonary Artery (LPA) stenosis (n=27, 32.14%) followed by isolated hypoplasia of Main Pulmonary Artery (MPA) (n=18, 21.43%) and supra-valvular stenosis in (n=11, 13.1%) patients. LPA was absent in one patient, while 2 patients had both absent right and left PA with segmental branch pulmonary arteries originating directly from MPA. Associated cardiac lesions included right aortic arch in 34 (15%), additional muscular VSD vary in 13 (5.5%), Patent Ductus Arteriosus (PDA) in 11 (6%) and Major Aortopulmonary Collateral Arteries (MAPCA) in 2 (1.9%) patients. Significant coronary artery abnormality was present in 10 (4.6%) children. Conclusion: Pulmonary artery abnormalities were present in 38.9% of patients with TOF. Isolated LPA origin stenosis and MPA hypoplasia were the most common abnormalities. Significant associated cardiac lesions were present in one-third of the patients and included PDA, additional muscular VSD, coronary artery abnormalities and MAPCA. Key words: Cardiac catheterization. Pulmonary artery variants. Tetralogy of Fallot. Department of Paediatric Cardiology, The Children’s Hospital and Institute of Child Health, Lahore. Correspondence: Dr Sadia Saeed, 11-B-3, Gulberg III, Lahore. E-mail: [email protected] Received March 25, 2008; accepted January 15, 2009. Anatomical Variations of Pulmonary Artery and Associated Cardiac Defects in Tetralogy of Fallot Sadia Saeed, Syed Najam Hyder and Masood Sadiq ORIGINAL ARTICLE
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