Staged interventional and surgical treatment of tetralogy of Fallot with critical stenosis of proximal aortic arch in premature hypotrophic newborn

Introduction

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart defect diagnosed in newborns. Depending on the degree of right ventricle outflow tract stenosis and clinical signs of cyanosis, the pathology necessitates intensive medical, interventional and surgical treatment in early infancy with anatomic correction before the first year of life. ToF is often accompanied by additional cardiovascular defects and congenital abnormalities apart from the circulatory system. Complex stenosis of the right ventricle outflow tract (RVOT) in ToF is rarely accompanied by any stenosis of the left ventricle outflow structures [1]. Regular treatment becomes more problematic in borderline low body weight patients suffering from prematurity and hypotrophy (birth weight < 2.5 kg), who usually do not meet regular criteria for surgery and cardiac interventions.

Case report

A 2-month-old premature hypotrophic newborn boy, 2.3 kg b.w., was referred to the emergency department with severe cyanosis in the course of postnatally diagnosed ToF. Peripheral saturations on air were less than 70%. Apart from dysmorphia features, the boy underwent resection of an additional thumb. Also he had a history of intensive treatment of congenital pneumonia in a different institution. Despite the cyanosis there was an evident pressure gradient of upper-to-lower limbs of 40 mm Hg, with diminished pulse in the femoral artery. Initial echo showed typical morphology of ToF with right aortic arch, malalignment 12 mm ventricular septal defect (VSD), non-restricted right-to-left shunt, 70% overriding aorta, hypertrophied right ventricle (RV) and a severely stenotic, dysmorphic pulmonary valve (PV) in the hypoplastic pulmonary trunk (PA). Peripheral pulmonary arteries had acceptable size with McGoon index > 1.5, although intracardiac defects were accompanied by critical stenosis in the proximal aortic arch of 2 mm width and 40–50 mm Hg echocardiographic pressure gradient. During cardiac catheterization there was revealed right aortic arch, internal carotid arteries right and left extending from the ascending aorta, and the right subclavian artery from the distal part of the aortic arch. The left subclavian artery was filling up to the collateral circulation. Probably the left subclavian artery arose from the closed ductus arteriosus. The patient was qualified for cardiology intervention with balloon plasty of the aortic arch (Figure 1). During the procedure...


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