Mid-Term Outcome of Right Ventricle to Pulmonary Artery Shunt for Older Children and Young Adults With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries

• Published in: Seminars in thoracic and cardiovascular surgery Vol. 31; no. 4; pp. 837 - 844
• Main Authors: Patel, Kartik, Rajan, Suresh Kumar, Garg, Pankaj, Gajjar, Trushar, Mishra, Amit, Kumar, Rahul, Rana, Yashpal, Ananthnarayan, Chadrashekharan, Sharma, Pranav, Patel, Sanjay
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 2019
• Subjects: Adolescent; Age Factors; Cardiac surgery; Cardiac Surgical Procedures - adverse effects; Cardiac Surgical Procedures - methods; Cardioscope: innovation; Child; Coronary Circulation; Female; Heart Septal Defects, Ventricular - diagnostic imaging; Heart Septal Defects, Ventricular - physiopathology; Heart Septal Defects, Ventricular - surgery; Hemodynamics; Humans; Male; Pulmonary Artery - abnormalities; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - physiopathology; Pulmonary Artery - surgery; Pulmonary Atresia - diagnostic imaging; Pulmonary Atresia - physiopathology; Pulmonary Atresia - surgery; Pulmonary Circulation; Recovery of Function; Retrospective Studies; Time Factors; Treatment Outcome; Ventricular Function, Right; Young Adult; MAPCA; RPA; RV; TTE; VSD; Cardioscope: innovation; SpO2; ToF; Cardiac surgery; VSD/PA; PDA; AR; BSA; CT; ICU; LPA; mBT
• ISSN: 1043-0679; 1532-9488
• DOI: 10.1053/j.semtcvs.2019.05.014

Management strategy for patients of ventricular septal defect and pulmonary atresia (VSD/PA) with hypoplastic pulmonary arteries presenting in late childhood or adolescence is still controversial. We present our experience with the use of right ventricle-pulmonary artery shunt (RV-PA) in management of this entity. Between January 2014 and April 2018, 25 patients of VSD/PA underwent valveless RV-PA shunt at our center. The size of the RV to PA shunt was calculated as half the expected diameter of the main pulmonary artery. We retrospectively reviewed the data from hospital records. Follow-up data were recorded from outpatient records or via telephone. Mean age of the cohort was 12.25 ± 3.18 years. There was 1 early and 1 interstage mortality. None of the patient developed acute renal failure, ventricular dysfunction, and arrhythmias. At interstage follow-up of 8.28 ± 3.7 months, both Nakata index (from 66.23 ± 24.12 to 185.8 ± 58 mm2/m2) and McGoon ratio (0.9 ± 0.22 vs 49 1.84 ± 0.4) increased significantly compared to preoperative value, whereas RPA-LPA ratio was not significantly changed (1.095 ± 0.39 vs 1.01 ± 0.56, P = 0.63). Prerepair pulmonary vascular resistance in 17 patients, who underwent complete repair, was 2.9 ± 0.69 woods unit/m2. Postrepair right ventricle-left ventricle pressure ratio was 0.5 ± 0.14. There was no early or late mortality and none of the patient required conduit revision or VSD fenestration. On follow-up of 25.75 ± 17.94 months, 16 patients were in NYHA I and 1 patient was in NYHA II. Appropriate-sized RV-PA shunt is an effective strategy for achieving balanced pulmonary artery growth in VSD/PA with hypoplastic pulmonary arteries presenting late without the risk of pulmonary over circulation or systemic malperfusion.