Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant

Abstract

Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Infantile Scimitar Syndrome With Contralateral Pulmonary Vein Stenosis and Refractory Pulmonary Hypertension

“…Of the 10 reported cases, there were only two survivors: one required bilateral lung transplantation, and the other required tracheostomy and ventilator dependent [ 2 - 8 ]. Most of the reports did not include information on whether the stenotic segment was distal or proximal, however, the cases described by Argueta-Morales et al.…”

“…and Onalan et al. included a description of the location of the stenosis and in both cases described the stenosis was located proximally [ 7 , 8 ]. Our case is the only reported case of contralateral pulmonary vein stenosis involving the proximal portion.…”

“…Of the 10 reported cases, there were only two survivors: one required bilateral lung transplantation, and the other required tracheostomy and ventilator dependent [2][3][4][5][6][7][8]. Most of the reports did not include information on whether the stenotic segment was distal or proximal, however, the cases described by Argueta-Morales et al and Onalan et al included a description of the location of the stenosis and in both cases described the stenosis was located proximally [7,8]. Our case is the only reported case of contralateral pulmonary vein stenosis involving the proximal portion.…”

The Effects of Inspiratory Muscle Training (IMT) on Patients Undergoing Coronary Artery Bypass Graft (CABG) Surgery: A Systematic Review and Meta-Analysis

“…Annually, more than 200,000 patients undergo CABG in the United States [ 1 ]. Despite the well-known benefits of CABG, many patients suffer from complications such as ventilatory muscle dysfunction, atelectasis, pneumonia, surgical site infection, and psychological disorders (anxiety, fear of death), which contribute to morbidity, mortality, and prolonged intensive care unit (ICU) stays [ 2 , 3 , 4 ]. Various forms of strategies such as aerobic training, endurance training, resistance training, and respiratory muscle training have demonstrated their benefits in inducing morphological and functional changes in the diaphragm while limiting the occurrence of postoperative pulmonary complications (PPCs) [ 5 ].…”